Chapter 42: Tumors

CHAPTER 42 Tumors CONTENTS A. Summary ........................ B. General Signs of Tumors within the Central Nervous System . ................... 1. Increased Intracranial Pressure and Obstruction of Ventricles ........... 2. Brain Shifts and Herniations ........ 3. "General Tumor Signs" . . . . . . . . . . . . C. Tumors That Impair the Sympathetic Pathways to the Iris . . . . . . . . . . . . . . . . D. Tumors That Impair the Pupillary Light Reflex Pathways ................... 1. Damage to the Rostro-dorsal Midbrain 2. Damage to the Ventral Midbrain . 1455 . 1457 . 1457 . 1457 . 1458 . 1461 . 1464 1464 Speaking generally, tumor of the central nervous system constitute a bleak but vitally important chapter of neurologic medicine. AJ o in general, it may be said of them that they occur in great variety; produce neurologic symptoms because of size, location, and invasive qualitie • usu- Tegmentum ........ • • • • •• • • • • 3. Damage to the Afferent and Efferent Light Reflex Pathways ... • • • • • • • • 4. Orbital and Ocular Tumors .•.• • • • • • E. Other Tumors That May Affect the Pupils 1. Diencephalic Tumors: Thalamic, Hypothalamic, Subthalamic, or Basal Ganglion Tumors, or Tumors That Occupy the Third Ventricle . . 2. Occipital Lobe Tumors .. 3. Diffuse Tumor Syndromes 4. Pheochromocytoma ...... . 1468 1468 1473 1474 1474 1476 1476 1476 alJy destroy the tissues in which they are situated and displace those around them; are frequently cause of increased intracranial pressure, and are often lethal. But this dismal prospect is beginning to change, thanks to perfections in neurosurgical technique and advances in medical therapeutics. (Adams and Victor, 1977) A. Summary Many different kinds of tumor can interfere with pupillary innervation. They differ in everal re pects: in their frequency of occurrence (in general and among different age group ; see Table 42-1), in their di po ition to grow in particular locations, their growth rate, their invasive tendency, their sensitivity to radiation and other treatment, and their acces ibility to surgery. Different nerve paths or centers may be invaded by the tumor directly, or they may be damaged econdarily by pre sure of a neighboring ma s, by edema, or by embarra ment of their blood upply. Pupillary defect vary a great deal because all component of the pupillary innervation may be involved: the afferent pathway in the optic nerve, chiasm, or tracts; the midbrain neuron and efferent intracranial or orbital para ympathetic fiber ; or the sympathetic center or pathway within the brain, spinal cord, or peripheral neuron . The mo t characteristic of the e pupil defect and main a ociated ign are summarized in Table 42-2. It mu t be remembered that such signs frequently overlap: a thalamic tumor may invade or exert pres ure upon the optic tract or midbrain; a peduncular tumor upon the tectum lower brainstem, or diencephalon; a pituitary tumor may extend into the cavernou inu or hypothalamu • and o forth. And secondary hemorrhage and vascular occlusions may complicate the clinical picture further. In addition to focal ign , "general tumor ign " al o may be present. With the development of modern canning methods the pupillary ign cau ed by brain tumor have lo t ome of their diagnostic importance. Nevertheless, they can serve as early signs and are easily (and cheaply) recognized. Table 42-1. Types of intracranial tumor in the combined series of Ziilch, Cushing, and Olivecrona, expressed in percentage of total (approximately 15,000 cases) TUMOR PERCENT Glioma glioblastoma multiforme as troc_ytoma 10 20} epend~moma 6 medulloblastoma 4 oligodendroc;ytoma 5 Meningioma Pituitary adenoma Neurinoma Metastatic carcinoma Craniopharyngioma, dermoid , eEidermoid, teratoma Angioma Sarcoma U~classified (mostl_y gliomas! Miscellaneous (pinealoma, chordoma granuloma) 45 15 7 7 6 4 4 4 5 • TOTAL From Adams & Victor, 1977, 3 100 Table 30-l. 1455 1456 I V. Pupillary Pathology: Pupillary Signs in Various Diseases Table 42-2. Pupillary deficit caused by tumors at various sites TUMORS PUPILLARY AND IMPORTANT OTHER SIGNS (in addition or signs of increased intracranial pressure) A. TUMORS WITHIN SYSTEM Hemispheric Thalamic, tumors THE CENTRAL "tumor signs" NERVOUS Tumors subthalamic to general No direct effect on pupils; secondary impairment of parasympathetic fibers in the 3rd nerve and of the oculomotor nuclear area due to brain shifts and herniations (see section on Trauma); general signs vary with size and location of tumor and 3rd ventricle General signs vary with kind and site: thalamic, hypothalamic, endocrine, sensorimotor defects, etc. ; pupils involved in three different syndromes: (1) In thalamic and subthalamic syndromes, ipsilateral sympathetic deficit (often incomplete), with vasomotor and sudomotor loss including the ipsilateral half of the body; and with crossed sensory and motor impairment; (2) Bouts of autonomic excitation ( especially sympathetic): Penfield's autonomic epilepsy; (3) General paresis-like mental syndromes with dissociated "Argyll Robertson"like pupils, due to caudal extension or pressure on the midbrain Rostro-dorsal midbrain tumors (pineal or col1 icular ) ; or upward or downward extensions to the midbrain of diencephalic, pituitary, or lower brain stem tumors; or pressure from cerebellar tumors Relatively large, dissociated or poorly reactive or fixed pupils, usually bilateral; often combined with impaired upward gaze (Parinaud's syndrome), or with other gaze palsies or nystagmus, and sometimes lid retraction; defective hearing if lesion extenclsto inferior colliculi; general sensori-motor and cerebellar signs depending on site and extent of mass Tumors in midbrain vading or pressing ventricle, pineal, tegmentum, or tumors inupon the area, such as 3rd cerebellar or pituitary tumors Ipsilateral pupillary sphincter palsy as part of third nerve damage ; crossed motor and sensory impairment and cerebellar signs, according to extent and position of the lesion Pon tine, medullary down to T2 - T3 and spinal Ipsilateral sympathetic deficit, with vasomotor and sudomotor defects including the ipsilateral half of the body; general sensori-motor, cranial nerve- and cerebellar defects according to size and position of tumor B. EXTRACRANIAL Tumors invading brachia! plexus Tumors cord tumors, spinal roots TUMORS or engulfing in the neck ( Horner's and Ipsilateral sympathetic deficit, with vasomotor and sudomotor signs extending to the arm, hand, neck and face; paralysis and sensory loss in the ipsilateral arm and hand (Klumpke's or Dejerine-Klumpke's syndrome) ; arm pain syndrome) Ipsilateral preganglionic sympathetic deficit, with sudomotor and vasomotor signs in the ipsilateral face and head; other defects , depending on nature of mass Tumors of the retro-parotid space (Villaret's syndrome); or at the angle of the jaw, including the subcranial pericarotid area Ipsilateral paralysis of lower group of cranial nerves, and impairment of pre/or postganglionic sympathetic neurons (depending on exact site and anatomic variations and C. INTRACRANIAL TUMORS AFFECTING EXTRACEREBRAL PUPILLARY PATHS AT THE BASE Half of base of skull (Garcin's or GuillaumeAlajouanine-Garcin's syndrome), caused by primary, metastatic or nasopharyngeal tumors Ipsilateral loss of function may include all 12 cranial nerves ; pathetic andpostganglionic sympathetic fibers may be affected, sudomotor deficit limited to area above the ipsilateral brow Apex of petrous bone (Gradenigo's syndrome), to cholesteatoma, chondroma, meningioma, or 2° sarcoma at base, etc. 6th nerve palsy and oculo-sympathetic postganglionic motor loss above brow; larger tumors may damage due 1° Middle Iossa (Raeder's syndrome), due to neurinoma of Gasserian ganglion, meningioma or other 10 or invading or metastatic tumors Subclinoid para-sellar area, caused by lateral extension of pituitary tumors, craniopharyngiomas, middle fossa chondromas, meningiomas, neurinoor metastatic mas, invading nasopharyngeal tumors; when limited to cavernous sinus these cause the "cavernous sinus }>~ndromes" ______ Supraclinoid expansion of the same tumors with impairment of optic nerve, chiasm or tract; or with invasion of diencephalon pupillary parasym with vasomotor and deficit; vasomotor and sudo3rd, 4th and 7th nerve Pain, dysesthesias and anesthesia in territory of 5th nerve and postganglionic sympathetic deficit, with vasomotor and sudomotor loss above brow; other defects according to type and size of mass Ophthalmoplegia and facial pain or numbness due to impairment of the 3rd, 4th, 6th damage to parasympathetic pupillary fibers in the 3rd and ophthalmic 5th nerves; nerve and to sympathetic fibers running with the 5th; general signs (for example endocrine) depend on type of tumor and on size and direction of expansion Visual disturbances: reduced acuity, restricted fields, scotomata, hemianopsias, uni- or bilateral blindness, combined with the same signs as in subclinoid lesions afferent, sympathetic and parasympathetic pupil defects or added diencephalic signs; Superior orbital fissure (Foix's syndrome) due to 10 sphenoid osteomas, meningiomas, sarcomas, or metastatic carcinomas, etc. , or extension of orbital or of nasopharyngeal tumors Ipsilateral ophthalmoplegia dysesthesias or anesthesia damage to parasympathetic Orbital apex, due to optic nerve gliomas, orbital or other tumors listed for the SOF syndrome Ipsilateral impairment of vision, varying from small scotomata to blindness; papilledema and optic atrophy; often combined with sensori-motor defects of SOF syndrome. Afferent pupillary defects plus sympathetic -parasympathetic efferent loss C. ORBITAL AND OCULAR due to paralysis of the 3rd, 4th and 6th nerves; pain, in territory of the ophthalmic 5th nerve, and proptosis; and to postganglionic sympathetic pupillary innervation TUMORS Primary orbital tumors or tumors invading from cavernous or nasal sinuses, metastatic tumors or ocular tumors of various types Defects as listed for SOF and orbital apex syndromes; rarely tonic pupil syndrome; with ocular tumors destruction of effector muscles of the iris I 42. Tumors / 1457 B. General Signs of Tumors within the Central Nervous System In addition to direct destruction of particular areas of the brain by growing tumors, two main pathologic processes participate in the production of tumor signs, namely (1) increased intracranial pressure and (2) brain shifts or herniations. 1. Increased lntracranial Pressure and Obstruction of Ventricles The skull after closure of the sutures is a rigid chamber and its contents-the brain, cerebrospinal fluid, and blood-are fairly incompressible. Increased brain volume due to a tumor and surrounding edema will, at first, be compensated for by displacement of cerebrospinal fluid through the foramen magnum to the subarachnoid space, and through the optic foramina into the perioptic subarachnoid space. With continued tumor growth this ability to compensate reaches a limit-relatively sooner in more rapidly growing than in slow-growing tumors-and then the pressure will rise throughout the whole dural volume, intracranial as well as spinal. In addition, the tumor and associated edema may interfere with the flow of cerebrospinal fluid by obstructing or compressing the ventricles (Figures 42-1 and 42-2). Frontal lobe tumors tend to block the interventricular forarnen of Monro; temporal tumors the third ventricle; and subtentorial tumors the aqueduct and fourth ventricle. Consequently, pressure will build up in the ventricular system proximal to the obstruction, as a consequence of the resulting internal hydrocephalus (Figure 42-3). Compression of veins may hinder venous outflow and thereby raise intravenous pressure proximal to the occluded area. Occlusion of dural sinuses will decrease absorption of cerebrospinal fluid and raise intracranial pressure even more, which-in turn-will aggravate the venous embarrassment, initiating a vicious circle. In the eye, high peri-optic subarachnoid pres ure impedes venous drainage and axonal transport from the retina and optic nerve head, leading to papilledema and secondary hemorrhages. 2. Brain Shifts and Herniations A tumor located in the midline exerts its effect symmetrically. Unilateral or asymmetric lesion , however, will push the brain toward the oppo ite side of the skull. For hemispheric tumors uch lateral shifts are limited to a degree by the tough membrane of the falx; but when sufficient mass develops in the tumor, brain substance will be squeezed below the falx to the other side. In addition, the mesial part of the temporal lobe, the uncus (which may normally overhang the upporting tentorium), will be pushed through the tentorial gap; and the entire brain tern will be shifted laterally and downward ("tentorial pressure cone"). With marked downward shift, the caudal brainstem and cerebellar tonsils may become wedged into the foramen magnum ("cerebellar pressure cone"; Cu hing, 1911). The e brain shifts and their consequence are de cribed in connection with trauma in Chapter 43. FALX VENTRICLES Figure 42-l. Diagram showing the relations between tentorium, falx, and ventricles. 1458 V. Pupillary Pathology: Pupillary Signs in Various Diseases 3. "General Tumor Signs" General tumor signs usually (but not always) precede the development of focal signs for variable periods, up to everal years. They may be only slight; and indeed, a tonishingly large tumors may grow slowly without any symptoms at all. Or subtle changes in behavior are explained a personality quirks, mental depression, or responses to external stress. Often the patient complains only about feeling tired, vague dizzy, or giddy; observant relatives and friends may notice increasing "psychomotor asthenia": a lack of attention and spontaneity, resourcefulness, and persistence; slowing and reduction of mental activity; a decrease in memory, emotional responsiveness, and accuracy of judgment. The patient may become listless, withdrawn, and indifferent or restless, irritable, and emotionally unstable. With increasing intracranial pressure, deepening torpor, somnolence, and incontinence will be recognized as pathologic, and may develop fairly quickly into stupor and coma. Headaches, often among the earliest symptoms, usually are light and dull at first. With supratentorial lesions they are felt above the area of the tumor; with posterior fossa tumors they occur around the ear and occiput on the same side. These headaches are nonspecific and tend to come and go. They usually are worst at night and in the early morning, and sometimes are ?ccentuated by certain head positions. Coughing, strainmg, and other reactions that raise intracranial pressure also may exacerbate them. As the tumor grows the headaches may become more prominent. With raised intracranial pressure the regional quality may no longer be present. Vomiting is unrelated to food and occurs with or without nausea. Giddiness and (nonrotatory) dizziness or a "confused feeling in the head" may get worse with changes in head position. Abdominal pains, generalized convulsions, or a stiff neck are other "general" tumor signs. Finally, papilledema will indicate a rise in intracranial pressure and the need for urgent therapeutic action. On the background of these "general" signs, focal defects point to the location of the lesion. Topical location of a lesion is discussed in various monographs concerned with neurologic examination. Ocular signs include uni- or bilateral loss of visual acuity, enlarged blind spots, scotomata, or other visual field defects; accommodative loss, diplopia and ptosis due to impairment of the third, fourth, or sixth nerve; and proptosis, nystagmus, gaze palsies, and other disorders of ocular motility, and pupillary disturbances. With some tumors, pupillary defects are early and reliable localizing signs. The main pupillary syndromes found in patients with tumors arc collected in Table 42-2 (see also Figures 42-4, 42-5, and 42-6). Figure 42-2. Normal circulation of cerebrospinal fluid (semidiagrammatic). A shows the coronal section of the forebrain at the level of the optic chiasm, as seen from in front, and B shows the lateral view; the stippled area outlines the ventricular system. (From N.M. Dottin Thomson and Miles's Manual of Surgery, 9th ed. [London: Humphrey and Milford, 1939], Vol. 2) 42. Tumors 1459 Figure 42-3. Internal hydrocephalus due to ventricular obstruction below the third ventricle. Note the compression of the hypothalamus and hypophysis due to the enlarged third ventricle, and the ballooned lateral ventricles. (From .M. Dott, in Thomson and Miles's Manual of Surgery, 9th ed. [London: Humphrey and Milford, 1939], Vol. 2) ·,I I I I I Optic 'Pltullary ch.la mn ·A-i= ------------ 87~ IR . 6 ------ I -....\1 I ' I B ___ _, __\"\,. I ........ ::.,..-,, __ -~---- '1./, 5&------------------1-Jr+I I 2 I --;.;;-;._-,1 \. '- .. .J. __ ........ --:-- ,.,-----~,.---<1----·<= 1--,,~----~ ,1-------1 -lr+l- 1 ~-._E_ --=~-..!.=,:-.\~'--=.............._===1=====_ ~11==7=_/"==-:::--~--~~-=--====-=·::-_ -__ -_-_-_-_-_-__J-3 NV. - ~ n=i-G-1 FY. 1 1 I 1 ''--•-'2 I j_____ 1~--~ ---------=----___ ! ____ !---~----1-.1---------'-------_-_--l-;-_,.._-_-=---_-_-:-,.....;.;:__-_-_~----_-_-_-___ ,1---+-----_-_-_-___ I---..J-------~ ~ 5 I d.r.l ld.r. d.l. d.l. t 8'-f-1 FY. O. lsec. ➔ Figure 42-4. Dissociated pupillary defect in pinealoma. The patient, a 27-year-old plumber's helper, began to see double when he looked to the right and up. There was no other symptom. The general neurologic examination as well as blood and cerebrospinal fluid tests were negative. The blood cells showed no sign of lead poisoning. Corrected vision was 20/20+ in each eye. At first the --- diagnosis of isolated right superior rectus paresis was made. However, the pupils reacted asymmetrically and only poorly to light and darkness (A, B, C, D, F, and G), while the near-vi ion contractions were brisk and equal on the two sides (E). On further investigation the patient was found to have a pinealoma. '1 -- s , ✓--'------I n. o.,se~. Figure 42-5. Internal ophthalmoplegia mistaken for "Adie's syndrome" in a patient with pinealoma. About a year before examination the patient, a 24-year-old man, noticed that vision in his right eye was blurred. He had difficulty focusing with that eye, and the right pupil wa larger than the left. He saw an ophthalmologist who diagnosed "Adie's syndrome." During the last 2 to 3 months before examination he began to feel restless, tired, and excitable. He slept poorly. When he walked, both legs tired quickly. On examination, both ankle reflexes and the deep tendon reflexes in the upper extremities were abolished. The right patellar reflex was overactive, the left normal. Abdominal and cremasteric reflexes were present. There was no ataxia. but when the patient walked on the lateral edges of his feet, both big toes went into dorsiflexion, the right more than the left. Best vision was 20/15 on the right and 20/20 on the left side. The visual fields, fundi, and extraocular movements were normal. When the patient tried to look near he developed accomodative spasms. The right pupil was large and reacted only minimally to light. It contracted about as well as the left pupil to near vision. While the reactions were thus dissociated, the residual light reflexes were neither de- O. lsec.-+ Figure 42-6. Bilateral efferent deficit due to a cyst in the posterior fossa. The patient was 47 years old when he was hospitalized because of right parietal headaches, together with tearing and blurred vision in her left eye of about 5 months duration. The headaches had become increasingly severe, with bouts of nausea and projectile vomiting during the last 3 months, and with a stiff neck, unsteady gait, and veering to the right for 2 months. On admission to the hospital there was bilateral papilledema with tortuous vessels and flame-shaped retinal hemorrhages. Best vision was 1460 F. ~ layed nor tonic, and the movements were not smoothed out as is typical for tonic pupils, even though the condition existed already almost a year. Further, central inhibition of the light reflexes was abolished: the left pupil was quite small for the patient's age and its light reflexes had square shape. They were not improved after psychosensory stimulation (sudden sound, at arrows), while reflex dilation was present on both sides. It was felt that these findings were not compatible with a peripheral lesion. Skull X-rays showed the sella to be very unusual in shape: it was shallow, measured 6 x 16 mm, and had a short, thick dorsum. The radiologist thought this suggested a lesion above, in the hypothalamus. The cerebrospinal fluid was normal and under normal pressure, and the EEG was normal. The patient was asked to return for further workup but he broke all appointments. Eighteen months later his family physician called to report that he had died rather suddenly without having developed further symptoms. On autopsy a pinealoma wa found. Comment: Mydriasis and deep tendon reflex anomalies looked superficially like "Adie's" syndrome, and so did the accommodative difficulty. This case illustrates that atypical cases of this kind need more elaborate investigation and followup. 15/20 in the right and 15/200 in the left eye. The visual fields were constricted peripherally, and the blind spots enlarged. The extraoeular movements were unimpaired. Hearing was diminished on the left. There were no abnormal reflexes, ataxia, or Romberg sign. Skull X-rays showed erosion of the sella, and the EEG was diffusely abnormal. A ventriculogram revealed obstruction of the caudal part of the fourth ventricle, with marked enlargement of the lateral ventricles as well as dilation of the third ventricle, the aqueduct, and the rostral part of the fourth ventricle. A sub-occipital craniotomy was done and a large pia-arachnoid cyst was found in the midline. It measured 3.5 x 4 cm and had compressed the cerebellar vermis and displaced the cerebellar hemispheres laterally. The cerebellar ton ils had herniated partially through the foramen magnum. There were no signs of inflammation or neoplasm, and after removal of the cyst recovery was uneventful and complete. Two and a half years later ophthalmologic examination showed slight peripapillary changes, probably due to the previous papilledema. The fields were normal except for enlarged blind spots, and visual acuity was 20/20 in the right and 20/40 in the left eye. Both disks were slightly pale. Neurologic examination was negative. In darkness the pupils were normal in size for the patient's age. They both contracted only slowly and inextensively to light stimulation of either eye (A and B) as well as during near vision (not shown). The left pupil's contractions lagged behind those of the right pupil. The darkness reflexes also were slow and inextensive (C and D). 42. Tumors Table 42-3. AUTHOR 1878 Eulenburg Guttmann Schulze Ferrier Bourneville Isch-Wall Ruel 1890 1894 1894 1895 1898 1898 1900 1905 1910 1911 1012 1916 1920 1922 1928 1929 1929 1461 Tumors affecting the central sympathetic pupillary pathways YEAR 1878 1884 1887 / KIND OF TUMOR & spinal cord tumors SEinal cord tumors thalamic-hyEothalamic & Jackson Schiff oe12enneim PE;Y:bikoff Schmidt-RimEler Jacobsen SQiiler 1f.2 Pfeifer Uhthoff Nonne Mondonesi SQiller Barath Rossi Barre & Alfandarv Penfield tuberculoma cord tumor; AUTHOR KIND OF TUMOR 1935 1935 1936 Ceni DeJong: Foerster; Gagel Barre& al Lichtenstein Roberts Ehlers Freeman & Russell McKissok & Paine Sorsby Serra trice Tori & al. echinococcal cyst in thalamus spinal core! tumor cystic cran1opharyng10ma invadin~ 3rd ventricle * spinal cord tumor multiele core! ~encl~oma (assumed) cord tumor seinal core! tumor tumor 1938 1941 1942 1953 1955 in the pons collicular tumor pressing on subthalamus (vasomotor signs) spinal cord tumors medullary ano core! tumors sarcoma in SEinal cord cord sarcoma thalamic lesion tumor engulfing thalami eontine glioma thalamic tumor SQi nal cord tumor cervical cord tumor thalamic gliosarcoma cord cyst; eontine tuberculoma medullari cancer metastases glioma invading pons & medulla* hypophyseal tumor prolongea into basal ganglia * cbolesteatoma in 3rd ventricle* * signs of sympathetic stimulation ADDITIONS: 1976, Saver &Levinsohn_, YEAR 1958 1958 1961 ~ 1966 1971 1974 1933 1958 1979, Torres &al., Cheek & Taveras Huber Huber Baumann Giles & Henderson cystic cavitation of cord 3 cases with thalamic tumors and iQsilateral miosis 12ontine & medulla!:Y tumors cervical cord tumors 15 cases with thalamic tumors and ipsilateral miosis 17 patients with thalamic tumors and iJ2Silateral miosis hypothalamic tumor; meoullaE;Y: tumors "tumorous meningitis" of brain , reaching down to T3, with ipsilateral Horner's svndrome brain stem and cord tumors cord tumor. C. Tumors That Impair the Sympathetic Pathways to the Iris Reports of sympathetic damage in patients with tumors in the thalamic-hypothalamic area, lower brainstem, and cervical cord have been relatively scarce, compared to sympathetic deficit due to vascular accidents in these areas (Table 42-3). This may reflect the fact that tumors usually are not restricted to the small areas of the brain and spinal cord that give rise to oculosympathetic deficit. Patients with tumors in these vital areas of the central nervous system have so many major defects that not much interest is aroused by the slight and harmless pupillary sign. In contrast, sympathetic deficit (and occasionally irritation) has been observed often in patients with tumors of the upper chest, brachial plexus, or neck (Table 42-4). In such patients the pupillary syndromes have served as valuable early signs of trouble. It should, therefore, never be forgotten that "peripheral Homer's syndrome" does not necessarily indicate a benign process; and a conscientious search for thoracic or neck neoplasm is mandatory in all patients with an unexplained Homer's syndrome. Tumors causing postganglionic sympathetic impairment can be found both extracranially (for example, parotid gland tumors or neoplasms at the base of the skull) or near the apex of the petrous bone in the lateral fossa, or in the cavernous sinus (Tables 42-2 and 42-5). Raeder's original patient (1918) had a tumor involving the Gasserian ganglion and adjacent lateral fossa. For this reason the term "Raeder's para-trigeminal syndrome" should be used for lesions in this area ( ee Chapter 25). Pain, anesthesia, or paresthesias in the ipsilateral eye and face may be caused by involvement of the ophthalmic division fibers. But sometimes cephalic pain is caused by pressure or traction on sensory nerve twigs innervating intracranial blood vessels or dura. It is therefore important to demonstrate the pre ence of objective fifth nerve sensory or motor deficit in such cases. Failure to make this distinction has contributed to the confusion that has arisen about "Raeder's syndrome" and some forms of migraine. Reports of intracranial postganglionic sympathetic damage due to tumors have not been numerous (Tables 42-2 and 42-5). Doubtless the sympathetic path is injured by such lesions in the lateral fossa or the parasellar area more often than is appreciated. A sympathetic defect is easily overlooked because of coinciding third nerve damage. The pupil tends to be larger than the normal one when studied in light because the parasympathetic impulses to the pupillary sphincter are lost, and the lid hangs flaccid due to paralysis of the levator palpebrae. Slight sympathetic lid droop is thereby overshadowed. It has, however, been observed by many authors that the fixed pupil seen in patients with parasellar lesions is smaller than one would expect from pure parasympathetic paralysis. 1462 / V. Pupillary Pathology: Pupillary Signs in Various Diseases Table 42-4. Tumors affecting the preganglionic sympathetic neuron in the chest and neck YEAR AUTHOR 1838 1839 1850 1856 1858 1858 1858 1860 1862 1863 1864 1864 1864 1866 1866 1868 1868 1868 1869 1869 1869 1869 1869 1873 1873 Hare Reid Mc Donnel Butcher Colby Mc Donnel Ogle Walshe Demme Cockle Eulenburg Heinecke Panas Czermak Hutchinson Hutchinson Steinberger Tr~lat Eulenburg Horner Ogle Poiteau Rossbach Carson Eulenburg & Guttmann Jany Baerwinkcl Foerster Schwabach Seeli~i.iller Eulenburg Beigel Chavasse Leeser Mobius Uhthoff Israel Pfeifer {WS} Rosenfeld Jackson Pick HitZ!,g Jacobsohn He_yligenthal OpEenheim Best Conzen 1873 1873 1876 1876 1877 1878 1880 1881 1881 1884 1885 1888 1892 1892 1894 1896 1897 1899 1900 1903 1904 1904 1904 1905 1906 1906 1909 1911 1911 KIND OF TUMOR tumor in lower neck neck cancer {cit. Poiteau, 1868) chest tumor {cit. Walshe, 1860) brachial ,elexus carcinoma thoracic tumor intrathoracic tumors neck abscess; neck & chest tumor• intrathorac ic tumors cystic goitre * neck tumors cervico-<lorsal abscess* cancer involving neck and chest cancer of neck and axilla neck tumor* neck tumors neck tumors neck sarcoma neck chrondroma struma struma neck tumors and abscesses neck tumors and abscesses mediastinal tumor* neck tumor neck tumors struma goitre struma; necl< tumor neck 1:1:'.!!!Ehoma thyroid tumor neck tumor intrathoracic (lung) tumor neck sarcoma goitre neck tumor abscessed neck !?land neck tumor {surgery) mediastinal sarcoma oeso,ehageal tumor neck tumor mediastinal tumor oesoEhageal cancer Ca metastases to brachial 121. struma struma neck & brachial olexus tumors lymphogranuloma in neck ossified }Mendel Bumke Guthrie Boinett {WS} Cramer Sebilcau & Lemaitre struma ossified struma* goitre ,eleuro.eulmonari sarcoma enlaq~ed neck glands* pharyngeal tumor (WS) = cited after *= signs of sympathetic excitation; Wilbrand & Saenger, 1922; Tbc = tuberculosis. ADDITIONS Todd & Brooks 1973 Gaillard & al. 1974 Strauss & al. 1974 Jaffe &al. 1975 1975 1975 Beckerman Seaver Pilley & Thompson & ganglioneuroma of vagus sympathetic ganghoneuroma upper cliest Scliwanno-ghoma mediastmal neuroblastoma cervical neuro blastoma thoracic Pancoast neuroblastoma tumor YEAR AUTHOR 1913 1916 1917 1918 1918 1918 1920 1921 1921 1925 1925 1925 1925 1925 1926 1927 1927 1928 Pamoeil Langdon Hesse Herrenschwand Waardenburg Blumenthal Samaja Samaja Merida Alguier Angelucci Gilbert Scarlett Troell Ballan!=xne Verderaume Weizenblatt Bonnano 1928 Brickner & Zemans)sy Lutz Angelucci Otuki Horning & Horning -Wenger Lohlein Courcoux & Lereboullet Russi Marsigli Pancoast Lee De Jong Patroni & Giarto Friboure: & al. Giacanelli Guillain & Sterne Jaensch Menzani Fribourg & Blanc Custodis Giraud &al. Tamman Monteiro-Sales Otuki Jaffe Pomerantz Ehlers Manning Giles &Henderson Serratrice Robinson & al. Reeves & Posner Walsh & Hoyt Cole & Berghuis Lentini & al. Johnson & Moore Sarma 1928 1929 1930 1931 1931 1931 1931 1932 1932 1933 1935 1935 1936 1936 1936 1936 1936 1937 1938 1938 1938 1939 1939 1950 1951 1953 1953 1958 1961 1965 1969 1969 1970 1970 1974 1974 1976 1976 1977 1979 1980 1983 1983 1977 Demirel & al. Sauer & Levinsohn Thom.eson Torres &al. Maloner Sa:):'.ed& al. Shewmon Riggs &al. goitre goitre thyroid tumor struma struma; neck tumors struma; neck tumors* goitre goitre neck l:):'.m,ehoma cervico-sca2ular cellulitis* tubercular neck ~lands* struma subclavicular cellulitis thyroid adenoma ,eha!:,Yngeal abscess thyroid cancer struma; neck tumor chest tumor; Tbc lymphogran. subclavicular lrmEhosarcoma actinomycosis of lung and chest wall* goitre Tbc neck glands* Ca metastases in necK tonsillar abscess* goitre cancer of lungs and brachia.l ,elexus neck l:):'.mphoma cervical l:):'.m,ehoma bronchogenic cancer goitre neck and chest tumors neck carcinoma goitre goitre lung Ca s,ereading to brachial 121. struma lY!!!,,ehos:Eanuloma in neck goitre epipharyngcal tumor a,eical lung tumor pulmonary carcinoma Ca metastasis in tlie necK Ca metastases in the neck calcified struma subclavicular tumor metastases goitre; neck & mediastinal tumors neurofibroma in ,eharrnx & neck bronchogcnic, metastatic & other tumors 12ancoast tumor; neck tumor cystic mass in chest and neck neck and chest tumors tumors of brachial .elexus and neck mediastinal tumor* neck tumor thoracic-brachial plexus mass intrathoracic cystic mass mali1?11ant pleural mesothelioma chest and brachial plexus tumors • cervical adenitis neck tumor necK tumor cliest !1ineclc tumors paravertebral tumor steilate gangnoneuroma cervical neurogenic tumors 42. Tumors Table 42-5. YEAR Tumors affecting the postganglionic sympathetic neuron AUTHOR Verneuil Poiteau Elschnig 1902 Hirschberg Best 1904 ~ Sieckel 19()7 Fuchs 191() Laignel - Lavas tine & Cantonnet 1918 Raeder Sommerfeldt 1920 1921 Shelden (2) 1922 Behr 1922 Foix 1922 New (21) 1924 Raeder (1) 1926 Favorv 1927 Dejean Herzau 1930 1932 Schlivek (16) 1935 Borghesan 1935 Po2ek 1935 Roger & Alliez (2) 1938 Cairns 1938 Cushing & Eisenhardt 1938 Custodis 1938 Jefferson 1939 Biemond 1939 Rubino & Stagnelli 1939 Sales 1940 Weinberger & al. 1942 Michaescu 1949 Bardram 1950 Lyle 1959 Paganoni 1953 Manning 1955 Paganoni 1956 Bonnet 1957 Etienne 1960 Lasco & Arseni 1965 Thomas & Waltz 1965 Vasquez-de-ParaSantamarina 1966 Godtfredsen & Gelderman 1970 Thrush 1975 Spector & al.( It) 1977 Grimson & Thompson 1864 1869 1898 KIND OF TUMOR parotid tumor parotid & other tumors at angle of jaw th_yroid cancer metastasis in CS orbital apex tumor sarcoma of upper jaw SOF tumor OA tumor tumor at angle of jaw middle fossa tumor involving GG (N5) ganglion neuroma of SCG tumors involving GG (N5) tumors of CS. SOF OA tumors of CS 2 SOF, OA cranio12har;}:'.ngioma invading CS 12aratr!geminal tumor tumor in CS Ca invading orbit via SOF naso12haringioma; metastatic tumors in CS naso12haringioma invading CS otogenous cholesteatoma and abscess in MF temporal lobe tumor (N5) tumors in SOF in orbit ana MF involving GG (N5) tumor (cit. by Walsh&Hoyt) meningioma of sheath of GG or of dural sinuses middle fossa tumor tumors in CS, SOF, OA craniopharyngioma invading CS SOF tumor metastatic tumor in middle fossa pituitary tumor invading CS naso12har;y:ngioma invading CS pituitary tumor invading CS Ca metastasis in CS tumor in OA neuroHoroma 01 SCG tumors in SOF meningioma involvmg uu (N5) craniopharyngioma in CS cranio2ha!:,lngioma in CS naso2haryngeal tumor in CS SCG neurinoma (N5) nasopharyngeal to CS tumors spreading N5 tumor; other middle fossa tumors glomus tumors invading MF Ca metastases in middle fossa (N5) CS= cavernous sinus; SOF = superior orbital fissure; OA = orbital apex; GG = Gasserian ganglion; SCG = superior cervical ganglion; N5 = pain and impairment of fifth nerve; numbers in brackets = num bers of patients described. MF= middle fossa; Ca= cancer; cit.= cited ADDITIONS 1928 SeEQ!ini 1934 Ellsberg& Duke 1953 Jefferson 1975 Boniver 1976 Sauer & Levinsohn OA tumor SOF mening!oma tumor in CS metastatic tumor of the petrous bone rabdosarcoma involving the middle ear / 1463 1464 Table 42-6. V. Pupillary Dissociated Pathology: AUTHOR 1 85 1887 1888 1895 1889 1901 1906 1909 1909 1910 Moeli ~? Moeli Q'F 57 Q'F 19 Ho2e Guthrie & Turner 0"'23 Weiss 61 24 Hem~l Wilson #2 Q'F 24 Chozen 0"'48 Kolein Buzzard #1 11 #2 Rorschach #1 27 Frank O"' 20 Mey r #1 42 fl #4 48 Ricaldoni 18 Wilson & Rudolf O"' 23 DeMonchy O"' 14 Escard6 y ~10 Amava Kennedy ~ Barre & Metzger 13 Brunnow 30 Ford 35 Alajouanine & al ~26 Rabinowitsch O"'22 Dimitri & Balaclo (abst)_ Ehnmach & d'61 Jacobowski Alajouanine & 0"'27 Gilbert ~52 Barre & Leyy Haldemann #2 45 25 Guillain & al. O"'66 Meyer O'"'26 Glaser #6 0"'27 Maxwell #2 #1 Wilson & -g.17 28 Gerstle ~ 09'23 Moreau &al. Alajouanine & ....!±.!_ O"'57 #2 ? Thurel #1 0.-13 Globus & 09'13 Silbert #3 Q'F35 #5 15 de Martel & al. #2 6.5 #3 O"' 20 Harris & Cairns 32 Baile:y 1141 47 Alajouanine & al. II 11 1922 1922 1923 1923 1923 1924 1924 1924 1925 1925 ""I926 1927 1927 1927 1927 1928 1928 1929 1929 1929 1930 1931 1931 1931 1932 1933 1934 Signs in Various Diseases pupil syndromes caused by tumors in the rostro-dorsal YEAR 1913 1921 1922 Pupillary AGE SEX g. lr §; midbrain KIND OF TUMOR PUPIL a22le-sized tumor filling 3rd ventricle, fused to walls large tumor in 3rd ventricle Qigeon-egg sized collicular tumor obstructing ac;iueduct angiosarcoma incl. colliculi, central grei, thalamus collicular tumor on right side Qineal tumor destro:i::ing colliculi, 12ushing into third V eeendimal tumor in 3rd V with 12ressure on midbrain cisticerus wedged between brain stem and cerebellum large metastatic Ca with 2ressure on midbrain choroid 2lexus cyst in 3rd ventricle cyst in 3rd V, imQinging on 2ost. commissure & colliculi oineal tumor distending aqueduct &flattening colliculi Qineal teratoma flattened colliculi Ca metastases, 2 foci in midbrain lateral to agueduct diffuse Ca invading brain stem & cranial nerves Qineal tumor eng!!,lfing colliculi choroid Qlexus in 3rd V; ant. colliculi destroyed Qineal tumor; colliculi destro:i::ed nn 5mm L 4.5 R 5 mm semiclilatecl R>Y: large variable; D, E large R> L; D R>L nn medium, R<L nn large average, R<L medium, R>L 2.5 mm OU medium no defect noted ptosis R t 2aresis 6th N; t:2aresis 1& conv. :2ares is EOM free fpalsv 6th N, bilateral Qalsy t 12a1si; 6th N 2alsy EOM normal EOM normal EOM free 't & QQnV. :QfilSY EOMfree EOM free EOM normal ;:& ...... I,>aresis palsy; conv. spasm large EOM normal tem2oral tumor causing 2ressure on midbrain tumor in coll icular area fibrosarcoma in cerebello-2ontine angle cholesteatoma in 3rd V, occludi~ agueduct collicular or tefil!!ental tumor ~ ray} thalamic gliosarcoma growing into 3rd ventricle micl6rain tumor (ventriculogram) diffuse melanoma filling lateral and 3rd ventricles, invading brain stem substance alo~ vessels collicular or tegmental tumor (X-ray evidence) n.s. (cl) nn R)L large, large, R>L R=L R>L (abst} R>L t 2alsy 2alsy EOM normal-. EOM normal paresis conv. s2asm; (abstract) R=L t palsy nut-sized rostro-dorsal midbrain tumor tumor filling 22sterior 3rd V and ac;iueduct rostral midbrain tumor ~ ray evidence) pineal tumor soreading over meso-diencenhalon 2a2illoma of choroid :2lexus, filling 3rd ventricle Qineal tumor (X-ray treatment) s12ongioblastoma destroyed colliculi tumor & cyst: dorsal midbrain, thalamus, cc small tegmental abscess lateral to agueduct collicular or te~ental tumor ~-rai treatment) rectal Ca metastasis to rostral midbrain Qineaioma infil tratccl colliculi & cere6eIIum Qineal tumor pinealoma large cistic cerebellar glioma mesencephalic glioma filli~ aqueduct walnut sized pinealoma removed 12ineal tumor neuroglioma engulfing post. commissurc R. aqueouct small, D R>L unegual §; g.. g. g.. mandarine-sized cerebellar gliosarcoma - SIZE D large nn large medium R=L R=L R>L R>L nn large large large R > L, lare:e R= L, moderate ns EXTRAOCULAR MUSCLES :t + 1 3rd N EOM 2ar. EOM normal t palsy :!:12aresis 112alsy , EOM deficit conv. I,>arcsis 6th N; 11alsy -2aresis; EOM 12ares. t paresis Ny .,_.Ny;+ t & conv. J2alS;):'. t palsy; conv. pares is 6tn N; t 2aresis R 2tosis; t2aresis bilat. 6th N palsy no defect noted no defect noted t 12aresis conv., t & L 6thN pal. t palsy Numbers marked # are case numbers in reports; nn = not noted by reviewer; ns = not said by author; (abst) not reported in abstract; (d) = not reported in discussion ; V ~ ventricle; CC = corpus callosum; in column PUPIL SIZE, R = right; L = left; OU= both eyes; D = pupils distorted; E = pupils ectopic; in column EXTRAOCULAR MUSCLES, t= up-gaze; down-gaze; += both up and down-gaze; - left-gaze; conva= convergence; 6th N = sixth nerve; EOM = extraocular movements; Ny= nystagmus; -. = later development. + TUMORS were verified by autopsy, unless otherwise stated. D. Tumors That Impair the Pupillary Light Reflex Pathways 1. Damage to the Rostro-dorsal Midbrain One of the most characteristic localizing pupillary syndromes is found in patients with tumors that affect the rostro-dorsal midbrain: pineal or collicular tumors; tumors of the caudal corpus callosum, or meningiomas in the area; thalamic tumors or third ventricle tumors that extend caudally; lower brainstem tumors expanding rostrally; or cerebellar tumors growing from the dorsal side. Such tumors may invade the tectum and pretectum; they may obstruct or compress the Sylvian aqueduct and the vein of Galen, bringing about a rapid rise in intracranial pressure with papilledema and retinal hemorrhages; or they may flatten the colliculi and injure the dorsal tegmentum by pressure. They destroy the pretec- I 42. Tumors Table 42-6 (continued) AGE SEX YEAR AUTHOR Lagrange & Lagrange sdrensen & Bush Springob Gabriel #2 1935 1465 KIND OF TUMOR corpus callosum tumor, pressing on midbrain PUPIL SIZE EXTRA.OCULAR MUSCLES large, D no defect noted Q>r23 Q 15 tumor occupying 4th ventricle semidilated divergent squint 1935 collicular tumor (suspected) ns t palsy 1935 -('yr---rf cvstic pinealoma nushed into 3rd ventricle R = L poor1 ; conv. spasm 1936 11 23 cases with pineal tumors and dissociated nupil syndromes - bo detailed case reports Jaensch Q>r24 large thalamic tumor with pressure on midbrain large -. 2 .5 mm t==-=P'-=•al~s.,;:.y _______ _ 1936 Araki #1 9 57 meningioma from velum interpos~tor~um 3. 5 mm OU EOM free 1937 11 #2 ~44 meningioma from velum interpos1tonum R = L EOM free Morax Q>r36 nrobablv oinealoma (surgery) large -. small normal _.. t palsy 1937 Moore & Stern Q 43 large temporal tumor with secondary midbrarn damage small, unequal ..,.t_p~al_s~y'-7 _______ 1938 1 Roger & al. c,. 32 1~c~o~l~l~ic~ul=a~r~tum~~o'.!.r'----....,.,------:-----:-----,,------,---..:---.-:---l ·-d::=e::cfo~r~m ______ 1938 1 7 e::.:d::_:---- 1""t_.._p-=a=-r-=e-=s-"is::_ Smyth JQ__ _9 43 thalamic glioma spreadin!f to tegmentum and c~llicu~i 5 ~ 3 mm t & conv. paresis 1938 &Stern E__ _Q 44 thalamic sarcoma, softenrng central grey of m1dbrarn R4 L3, irregul. 1_t-'-'p.__al..,;...s_.y'-------,,------I ___ _____ __:#,_.,6"----1--'o>r'"--=3=-3 diffuse glioma in colliculi, extending to thalamus moderate t palsy, +Paresis 1 1941 Thurcl #2 Q>r? pinealoma pushing anteriorly into 3rd ventricle immobile to light t palsy 1=p,_o_s_n_e_r---=-&--#""1=--lr-----::?-1'""94~6cno details given; all were pinealomas large, R >L t paresis, 6lhN palsy ~ ---?with more or less typical signs normal R 6thN; -Ny Horrax ~ ---?large t paresis; -+-Ny ~ ---?normal EOM normal Tio---?normal R 6thN; ++Ny 1940 Bender & in central grey in collicular area; normal --+ large y; divergence palsy 0 18 small hemangioma Savitzky Cf 1------------1 1_~a~q~uc::e:,-.::d~u~c~t..'.d~i~l~a~te~d~an~d~d~is~to:'..:r~t~e~d~.,----,----,-,-,------I----:-;------1942.-. Al_p_e_r_s~~----t--o..-='"'2s-4· ___:e::,tp::.:e::::n:::d::.Y:.:m=o.::m:::.a:::.?.., ...!P:::O:::S:::t::::e:.::r:.:i_::oc::r_c=o~ll:.:i_::c:.::ul:::.=.i-=an=d=-=c..::e.::n:::tr:..al=-.2gc::r..::e,,.y ___ , ...:.t_an:.;.:__d_c:...o:...n_v_. _.p_a_r_e_s_1..;:· s'-- I 1....:s::cm=all=----1 1943 Paufique & ? tumor in post. 3rd ventricle and colliculi large t palsy Chavanne 1944 Russell & Sachs _0""="'1'--'7_ 1__.p-=in=e-"'al=-oc:cm=a"----------------------unequal 6th palsy; t pares is 1946 Ehni ---'0""=---=1-=8_ 1 _.o:P-=inc:ce-=-al==--=te-=-r=-a:;t.::co::.:m=a'--"(s=-u=r°"'g"'-e=-r=-y,,_,__) ----~--------large t paresis Maspes #5 g;12 cyst in 3rd ventricle removed surgically norm.~ large L--dev1ahon~ norm. 1949 Fanta & Reisner 56 large craniopharvngioma removed from 3rd ventricle small-. larger ...- & rotatory Ny 1952 1952 Kornblueth ___ 1_3_1-~p_in_e_al_tu_m_o_r'----------------------•--D-'-, _E ______ ,...:.t_p:._al_s__,y:._:_; _c_o_n_v_._s~p_as_m __ 1 & ~ Las1erra Arroyo 2 pts mesencephalic tumors (surgery) "AR" ns (abst) 10 t 1958 1958 1958 Arseni Kawai & al. 1959 1959 Cogan #4 0""31 large pinealoma projecting into 3rd ventrocle Sanchez-Salorio o 17 mesencephalic tumor reaching 3rd ventricle & Molina-Negro 'F Sokolova 54 pts 22 pinealoma; 14 other pineal tumors, 18 collicular Bourlouts}Y -3-6-pts pinealoma (proliably same cases as So7<:olova) Huber ~ pinealoma or tumors in collicular area Arnould & al. --,~:-::--2..,.3_1-~pin_e_al_o_m-,-a~-~------------------·•--~?'-('-'c..;:i..:.t'-. Poppen & Marino 20 pts tumors in pineal area Loewenfeld 9~ pinealoma ( surgically removed ) Walsh &Hoyt ~ pinealoma 1 Seybold & al. #1 ~p..,.in_e_al"""o~m--'a--------------------" " ~ prnealoma ]~5~1959 1961 1965 1968 1969 1969 1971 " M~~~ock Abbreviations indicated; Additional 3 pts & ~ 22 tumors affecting midbrain and median thalami pineal teratoma filled lateral and 3rd ventricles #1, #4 thalamic tumors spreading as in Part 1 of this Table; -Ny= horizontal (cit. WH) = cited after Walsh & Hoyt, 1969. cases: 1960, Tytus: 17 patients with pinealoma; to brain nystagmus; 14 had loss tal pupillary light reflex neuron, but during the early stages leave the oculomotor nuclei intact. In such early cases the pupils are "dissociated": they fail to react to light while the contractions to near vision remain active (Table 42-6, Figures 42-4 and 19-17). Because of the poor or absent light reflexes these pupils may look large when they are examined in light. In dim illumination it can be seen, however, that they are really not "enlarged" but often are quite small for the patient's age. These findings were important in the discussions about the mechanism of the Argyll Robertson syndrome, as described in Chapter 19. 1 ? large, stem Pts in column of light reflex, R = L ? t. t paresis t palsy gaze defects 3 mm medium defects t ns (abs t) 1 26 "AR" }f and other gaze palsies gaze palsies _W:.:..:.H::,):....__ t palsy & retractory Ny ? (cit. WH) Parinaud's syndrome medium tpalsy; conv. Ny "AR", D gaze palsies &spasms abnormal Parmaud 1s syndrome abnormal Parinaud•s syndrome j'--~~~--- no details AGE,SEX 9 loss = several of upward gaze, patients, or number 6 3rd nerve deficit. The dissociated pupil syndrome often occurs together with gaze palsies, especially with weaknes or paralysi I. Early reports of patients with collicular tumors helped to settle the controversy regarding the location of the "center of vision." Until the turn of the century many authors believed the colliculi to be this center in man, as they are in some lower species. The fibers of the optic tracts and nerves were said to arise from the colliculi and to travel from there to the eye, with sensory endings in the retina. But here were patients whose colliculi were entirely destroyed, and yet, until they lapsed into unconsciousness, their vision remained normal (Piltz, 1870; Nothnagel, 1889; Lichtheim, 1892; Goldzieher, 1893; and others). 1466 / V. Pupillary Pathology: Pupillary Signs in Various Diseases Table 42-7 • Impaired pupillary reactions to light and to near vision due to verified tumors affecting the rostro-dorsal midbrain YEAR 1837 1 37 1861 1864 1864 1870 1871 1 72 1883 AUTHOR Schmidt Stanley Seidel Henoch Steffen Piltz* Blanguingue Ferrier Parinaud #4 It #6 It 1883 1886 1885 1885 1885 1886 1886 1887 1888 1888 1889 1889 1889 1889 1 89 1890 1890 1892 1893 1893 11 1893 1896 1896 1899 1899 1899 1901 1901 1903 1905 1906 1906 1906 1909 1910 II SEX AGE }Bristowe Feilchenfeld Ponto22ian Fisher Reinhold Schultz Leube Daly Nothnagel Gauderer Kny Nothn~el Ogle Preston Christ Ruel Lichtheim Golaziener Bruns #1 II w- p KIND OF TUMOR -s 22 4 pineal tumor; colliculi destroyed .JL cvstic oineal tumor· colliculi destroyed B nn Qineal tumor B collicular tuberculoma B ~;-5 collicular tuberculoma B jutting int.o 3rd V B ..Q_L_ collicular tuberculoma O"'39 pineal tumor pressing on midbrain B 0"'14 thalamic e:lioma incl. rostral midbrain B O"'67 tumor si!i!!S but no auto2sy B O"'37 tumor of R cerebellar 2eduncle --B ~7 tuberculoma involving colliculi B O"'18 O"'31 0"'24 ()'I' 19 O"'28 50 23 O"'20 O"'12 O"'32 O"'15 O"'6 ()'I' 36 0"'18 ()'I' 20 g. ()"' ()"' 5 0"'2 .5 ()"' 11 Kolisch Q 8 Gutzeit 0"'7 ()"' 9 Hoesslin Biancone 0"'18 Lawrence O"'16 Oestereicher &Slawskv #1 o"'4 Nissen O"' 5 Neumann <.j? 28 Jankowskv <l Gruner & ~36 Bertolotti Avtokratov 0"'40 Askanazy 0"'19 Wilson #1 0"'24 Frankla"' Hochwart Howell #3 0"'20 11 ~ 0"'42 EYE MOVEMENT ...... ..1..+ - -- -++ - --- -- + -- - --- -- -- + -- - J2ineal gliosarcoma B pineal tumor compressing colliculi B --+ sarcoma in Qineal & collicular area .B -:pineal gliosarcoma B walnut-sized pineal tumor re -pineal tumor & tegmental hemorrhage B -pineal tumor: colliculi flattened B -collicular glioma, compressing agueduct B Qineal teratoma growing into 3rd V B ---:pineal round cell sarcoma B collicular tumor B -large :pineal tumor growing into 3rd V B -tumor from CC, flattening colliculi B -large pineal gliosarcoma B collicular glioma pressing on tegmentum B -collicular glioma extending to thalamus B -tuberculoma u2per pons & colliculi re collicular tubercul. extending to root N5 re -cerebellar tumor re -collicular tumor extended to 4th V B :pineal teratoma protruding into 3rd V re Ny oineal sarcoma iuttine: into 3rd V B collicular sarcoma orotrudine: into 3rd V B -large pineal sarcoma growing into 3rd V B -large pineal tumor filled 3rd V Ny B tubercle occupied colliculi tumor & cyst in colliculi & diencephalon cystic Qinealoma; extreme hydroceehalus tuberculoma in thalamus, central grey; 3rd nerve nuclei destroyed glioma of SUE. colliculi &diencephalon oost. commissure & cerebellum tumor large pineal tumor including tegmentum pineal teratoma including post. commissure and colliculi pineal t. including 3rd V & tegmentum pineal tumor extending to med. thalamus B B B re B B B ++ Xi --- - -- - + - ++ - -- -- -- + ++ -- Ny ++ -- -- + - -- - ++ -- -- - -- - -- - -++ ++ ---+ - ~ No -- 30 ++ ++ ~ -- -t - - R+ - - 7fo --:;-- ..BQ._ -_o_ -- --0 -- - -- + + - -++ + -L60 -- ----- --- -- - --- --go- - --- ~ ++ ++ - -- -- - -- 60 - --- ++ +- - _o_ - ...!ill... -+ --- -- -- -- -- 60 ++ -++ -- ___Q_ - +R + 30 - -- -- ++ ++ -- + -- I--- -- ++ - - -- - -- ++ -++ -- ++ -- + -- ++ - ---+ --- ++ - -- -- -- -- + -- --- -- + - -- --- ++ + - B B B + -- DEFICIT EO Pt ? -- - -- + -- -H- - -0-0- - -- -- - -- -- - 60 -- #=case number; in column P (pupil): B = bilaterally disturbed; re= reactive; in EYE MOVEMENT column:+-+= lateral conjugate gaze; f = upward gaze; +=downward gaze;--= convergence; EO = extraocular muscle palsy: 30 = third nerve only; 6° = sixth nerve only; F,~ = forced downward gaze; in Pt (ptosis) column+= ptosis; = lid retraction; in last column nn = not noted by reviewer; 0= no abnormal movements or deficit mentioned. No = normal; + = paresis; ++=palsy. t of upward gaze (Table 42-6). This combination of defects is almost always caused by tumors or cerebrovascular lesions. Rare patients with infective cysts, or with degenerative plaques (in multiple sclerosis), have been described, and patients with trauma are exceptional. This is easily understood when the anatomic relations of the region are considered: destruction by trauma, limited to this small, inaccessible area, is most unusual; and extensive trauma to the rostral brainstem usually leads to death. Because of their early appear- ance, and because they pinpoint the lesion quite precisely, these signs are of great diagnostic value. Convergence palsy also is common in such patients, and with slightly more caudal damage down-gaze becomes impaired. Horizontal nystagmus is often present, and occasionally rotatory nystagmus is seen. Nystagmoid convergence and retractory movements of the globe may be elicited when the patient tries to look up. Larger lesions in the same area will include the midbrain tegmentum, so that the pupils will lose their 42. Tumors I 1467 Table 42-7 (continued) YEAR AUTHOR SEX AGE KIND OF TUMOR P EYE MOVEMENT DEFICIT ,l. -MEO Pt ? ..... t ....:1:.:9:..::1:..::0_ 1...:Wc:..il=· =..:li::::a:::m:::s:...;o""n'-~_:::14.=.... c:.::ul::,:a~rc....::s,:P::in:.:dl=e ..:c:..::e-=-11:;_;;_s_ar_c_o_m_a ___ _ B ----------~ 1_c::_:o::;l=li::::· 1 1911 Bailey & d' 12 large pineal teratoma filling 3rd V B + _4_0____ _ ___ ,~J_ell_if_fe __ ~.,---=-=-l------------------1-~B + + __ _ 1911 Weisenburg#l Q 17 brain stem glioma B + ~ - -+ 11 11 if2 n~ 20 brain stem glioma -,.,-,,.-•-=------t B + + - - -+ --- _ __19::..:1~2_ _H_o,....p ...p~e-~-1 ~ 17 glioma in midbrain,pons,cerebellum __________ l9l6 Warren & O"'18 pineal tumor invading midbrain & thalami re No Tilney 1--1---1--1----1921 Jacobi <,j.> 21 ovarian metastases disnlaced aaueduct B ++ __ _!__ 1922 Collier ? midbrain tumor B ++ survey of many cases from literature and 12 cases with histopathology: pineal tumors; 1922} IIorrax & Bailey not much detail about pupillary and eye movement deficits 1925 1926 Giannuli ? (a.bs) mesencephalic -pontine glioma B __:!:_ + ....:!:.._ _ -;,o ___ _ 1926 Marburg O"'22 collicular tumor growing ccphalad B ~ ++ _6_ 1926 Neidig O"' collicular tumor B 6° 1927 Castex &: ~ _Q 12 nut-sized glioma in collicular area B ++ _______ _ Camliuer #3 9 33 glioma in collicular area B ++ _____ - --1927 Horrax ill -6-"'52 oinealoma extending to CC B ~ ____ R __ _ 1928 Foerster O"'26 orange-sized tumor in collicular area B + + _ _ _ __ 1928 Meyer 0"'66 midbrain-pineal-thalamic tumor B ____ _+_ + 1929 Fulton&Bailev O"' 15 cm pinealoma filling 3rd V B _ --- -- ---0 60 1929 Glaser ii 1 ~0"':'...:...:6~ 1...!:la~r~g~e::.p~m~· ~e~a::lo~m=a....!f::ils=l::in,,.g"--"3=-rd"--:-'V'-:---:--:,,----.r=-e=1 11 60 ~ O"'19 glioma invading thalami and 3rd V re --O"'17 pinealoma B ~ 6° + 1931 Globus & #2 Silbert ~ 0"'30 P.inealoma B ~ ,.;.+_, __ __. B -- ___ _Q__ 1931 de Martel #1 0"'28 large cerebellar tubercle & arachn. cyst II 11 11 is" O"'6 medulloblastoma in 4th V B ___________ o__ ++ 1932 Allen &Lovell 0"'6 pineal tumor B 1933 Guillain & al O"'32 1...:::c.:::::o!!llc!.:ic::;ul=a=-r....::t::um=o:.:r...:s:c,pt::r::.:e::..:a:.:d::::in?gc.:-=:to:::..:t-=-egm=::::e n=..:tu=m=-'B:::--__ ++ _ + _____ _ 7 11 1935 Zeitlin #1 ()'I' 14 2x3 cm pineal tumor occluding aqueduct B _liy ++ _ _ __ _ __ II " ~ O"'45 pineal tumor blocking aqueduct B ~ ±... II 11 ~ 0"'21 meningioma compressing pineal & thalam. B ++ 1936 v. Gehuchten 0"'3 pineal spongioblastoma obs tr. aqueduct B + L6° - --1936 Dandy #1 O"'10 pineal teratoma B -6° + II 11 #2 .Q 28 pinealoma (surgery) B _ __ __ __ + + __ _ 11 #3 (}1' 15 4 gr. pinealoma destroying colliculi B ++ 1937 Alajouanine 0"'29 pinealoma engulfing midbrain B ++ ++ L6° - --1~7::0:.:g:.::r::o:p::;i::n::e...:al;.:o:i:m=a:.:e::xg_te-=n=d;.:i:::n:..:g..::t:.::o:.:,3;:-r-d;-::-:V,---1-:B;:-...:l:..:9c.::3..:.7_ -=H=..:o:..::rc.::r..::ax=----l-"O"''--l-'--0 6° 11 t --- = 1 Baggenstoss d"'33 pinealoma filling 3rd V, compressed ++ B & Love R thalamus & midbrain _1..:9_3_9_ ,~B_i_e_m_o_n_d ___ 1_0"'_2_1_.bg::li""o.!!m::ae...f::r'-'o'-"m~f"'o""rnee.ix:::· ,_fl=a""tt"'e"'n"ced"'--"c'-"o~ll"'i"-cul=i __ l-:BB=__ ..±±.. _____ 1941 Tburel !±l___ ~i..i::P.::in::..:e:.::al=-o=-=m=a'-'g2.:r:...;o..:.w:...:i:::n.,g--'i""n"-to'--"3=r~d_V~-----•-:=__ ++ _ ++ _____ 11 !±1.._ ~--=3-=-3_ ______ --::B:_ ++ _______ 1_,g:,:l:.::.io::..:b::..:l=as=-t.:.:o:.:m=a=-=in:.:....r.P:..:i:::n-=-eal=-=ar=-e::..:ac.._ 1 11 #4 y O"' pineal tumor (ventriculogram) B __ ________ ++ 1944 Russell & pinealoma (surgery) B Sachs 1949 Feld &Pecker 10 cerebellar cyst compressing colliculi B _ ++ _ ++ _____ 1949 Maspes #3 12 pinealoma B '' ~ ~1~7-1-'p'-in-e~al~o-m_a _____________ B + ++ ++ ++ + 1955 Newmann Y: ()'I' pinealoma anterior midbrain B ---1956 Cogan #2 Q_ 25 tumor involvine: anterior midbrain B _ ++ ________ 1965 Arnoud & al. ~ 23 pinealoma B ++ ++ 1939 s --- _ _ _ 0 _ --No _ Note: The cases in this Table resemble those of Table 42 - 6, except that the lesions extended slightly more ventrally so that bath the light reflexes and near vision reactions were impaired. contractions to near vision in addition to those to light, and will react feebly or not at all to all these stimuli (Table 42-7 and Figures 42-5 and 42-6). As the mass grows further, extraocular muscle pareses develop, and soon signs of high intracranial pressure and extensive brainstem compression will ensue. According to Bailey and Jelliffe (1911), the area can tolerate growths to about walnut size, but further expansion will lead to rapid death. Children (especially boys) with pineal tumors sometimes show precocious sexual development. This was thought to be related to an endocrine function of the pineal gland. It was, however, later recognized that the changes were secondary to pressure upon the neighboring diencephalon due to the pineal tumor. It has been said that the dissociated or fixed pupils in these patients and disturbed upward gaze are related to dilation of the posterior part of the third ventricle, and not to local pressure effects of the tumor (Adams and Victor, 1977, page 604). The ocular signs, however, develop very early, in many cases Jong before the aqueduct is occluded and hydrocephalus develops; and we have seen the pupillary syndrome persist after the hydrocephalus was relieved by ventricular shunt (see Figure 6 in Chapter 20). 1468 / V. Pupillary Pathology: Pupillary Signs in Various Diseases 2. Damage to the Ventral Midbrain Tegmentum As just stated, unrelieved expansion of pineal or cerebellar tumors, downward growth of tumors in the third ventricle, or upward enlargement of lower brainstem or fourth ventricle tumors will eventually encroach upon the midbrain tegmentum ( directly or by pressure). Pituitary tumors breaking out of the sella posteriorly (Figure 42- 7), tumors of the hypophyseal duct, or other middle fossa tumors also may extend sufficiently to reach this area from the rostro-ventral side; and occasionally primary midbrain tumors develop (mostly gliomas). These tumors cause pupillary light reflex and accommodative loss as part of destruction of the oculomotor nucleus and of the third nerve fibers in their fascicular course (see Weber's and Benedict's syndromes in Chapter 22). In addition, visual and pupillary field defects have been described in such cases, resulting from involvement of the optic tract as it embraces the brainstem. Long tract, cranial nerve, and cerebellar signs are found, and vary from case to case. Sympathetic pupillary impairment due to tumors confined to the midbrain must be rare. We have not found mention of such cases in the literature, though they were observed after vascular lesions in this area. Small tumors may spare the sympathetic fibers which are laterally situated. With larger lesions sympathetic deficit may be overlooked, partly because the patients are very ill or comatose, and partly because efferent parasympathetic loss dominates the pupillary picture, so that sympathetic impairment will not be noticed unless specially searched for. Figure 42-7. Extension of pituitary tumors. The arrows indicate directions of extension of pituitary adenomata: downward through the bone into the sphenoidal sinus; more often upward through the sellar diaphragm, exerting pressure upon the chiasm from below; sometimes invading the third ventricle from below'. occluding one or both foramina of Monro or the aqueduct of Sylv1us; or pos- 3. Damage to the Afferent and Efferent Light Reflex Pathways The afferent and efferent light reflex pathways can be damaged by tumors at the base of the skull, the more common being pituitary tumors, craniopharyngiomas, meningiomas, chordomas, osteomas, nasopharyngiomas, or metastatic tumors (Table 42-8). Some features of regional anatomy are important in relation to the effects of such tumors. (1) The nasal sinuses are variable among individuals. As seen in Figure 42-8, the part of the sphenoid sinus that underlies the sella may be small and bordered by a thick wall of bone. Conversely, it may be huge, and its walls paper-thin. In such patients an expanding nasopharyngeal tumor can break through the fragile floor of the sella and surrounding base into the cranial cavity; or a pituitary or craniopharyngeal tumor may expand downward and fill the sinus. The lateral walls of the sella are not composed of bone but of the dural lining of the cavernous sinus. Pituitary tumors therefore can spead laterally from the sella and compress the contents of the cavernous sinus, including the parasympathetic pupillary fibers in the third nerve and the sympathetic fibers running with the ophthalmic branch of the fifth nerve. (2) The afferent pupillary pathway can be interrupted in the optic nerves, chiasm, or tracts when tumors extend upward from the sellar region. For example, a meningioma arising from the tuberculum sellae will deform and stretch the chiasm, as shown by Cushing and Eisenhardt's illustration in Figure 42-9. Similarly, pituitary tcriorly along the base. Lateral spread (not shown) is common, involving the cavernous sinus; this may cause proptosis, oculomotor deficit, and fifth nerve deficit, including the pupillary sympathetic innervation. Further lateral spread may damage the temporal lobe. (Redrawn from F.B. Walsh and W.F. Hoyt, Clinical Neuro-Ophthalrnology [Baltimore:Williams and Wilkins, 1969]) 42. Tumor Table 42-8. YEAR 1884 ~ 1890 1895 1895 1896 1896 1898 1900 1902 1906 1907 1907 1907 1907 1912 1914 1916 1916 1921 1922 1922 1922 1922 AUTHOR Thomsen (Richter 1887) Leclerc Riscoldi Kar12lus Thomas Broadbent Sachs Elschnig Pichler Hirschberg Sieckel Dupuy -Dutemps & Lejenne Fuchs V. Rad & Neuburger Saenger Jess Oppenheim Kablmcter Meggendorfer Hirsch Foix 1926 1927 1928 Bunge 1928 1928 1928 1928 1929 Lutz Magnus Nemlicher Se~ini Barre & Alfandary Igersheimer Mankowsky Wenger Herzau 1923 1924 1924 1924 1925 1925 1929 192"9 1929 1930 -mo 1930 Jecl<eln Lutz KIND OF TUMOR YEAR AUTHOR KIND OF TUMOR intrinsic 1930 1931 1931 pituitary stalk tumor pituitary tumor hypophyseal tumors naso126aryngoma mvaaing: Rathke's pocket tumor meningioma at SOF 1940 1942 Mylius Butler Nordmann ScliliveK Busacca Elsberg & Dyke Roger & Alliez Pomfret Hartmann & iil. Yealland Cairns Jefferson Strebel Biemond Meyer Rubino & Stafil!elli Weinberger & al. Michaescu 1943 1949 1949 1950 1950 1951 1953 1955 Coxon Bardram Walsh Lyle Paganoni Huber Jefferson Chamlin & al. 1955 1955 1955 Paganoni Norstrand O'Rourke & Schlezinger Samojlov Etienne Marcello & Antonio Lasco & Arscni Liegl Liegl &Kohn Levatin Symonds Green &al. 3rd N fibrochrondroma J2ituitary tumor J2ituitary tumor neurofibroma of 3rd nerve eituitary tumor 12ituitary tumor orbital glioma thyroid Ca invading CS orbital adenocarcinoma tumor at OA tumor at SOF pituitary tumor tumor at OA pituitary tumor pituitary tumor tumor damaging OJ2tiC tract hypophyseal tumors hy12012hyseal tumor hypophyseal tumors hYJ20J2hyseal tumors hypophyseal, metastatic or tem12oral lobe tumors tumors in CS, SOF or OA 2ituita!:_Y tumor craniopharyngioma tumor in CS hYJ20J2hyseal tumor hy202hyseal tumor hypophyseal tumor s12enoid sinus tumor hypophyseal tumor pituitary and iniundibular tumors hypophyseal tumors tumor invading orbit via SOF; tumors & metastases at OA pituitary and other subtentorial tumors hYJ20J2hyseal tumors hYJ20J2hyseal tumors hy:e212hyseal tumors tumor at OA ~ 1933 1934 1935 1936 1937 1937 1938 1938 1938 1939 1939 1939 1956 1957 1958 1960 1960 1961 1961 1962 1964 1965 tumor 1967 su12rasellar tumors su12rasellar meningioma 1969 craniopha!):'.ngioma nasopharyngeal, craniopbaryngeal, 1970 pituitary, metastatic tumors in CS 1971 mefasfafac ovarian rumor in CS ~ subtentor ial tumor 1973 hypophyseal * = several or many cases, with exact number not known to reviewer; Ca= cancer; Cs= cavernous sinus; SOF = superior orbital fissure; ADDITIONAL 1469 Tumors affecting the afferent and efferent pupillary pathways Behr di Marzio New Behr Uhthoff Bussy di Marzio Torrigani Hessberg Orzechowski & Mitkus Favory Dejean 1923 I CASES; Thomas & Waltz Newman Walsh & Hoyt Thrush & Small Huber Robinson g;;al. Narasaki & al. tumors CS at SOF hypopnysea.1 tumor oHacto!}'. mening10ma pituitary aaenoma tumors at :,,uJ< , uA or 1n c:s tumors in CS, at SOF or OA intrasellar [!ineal tumor craniopharyngioma in CS J2ituitary aaenoma tumor in area of SOF (169) pituitary adenoma nasopharyngwma invadrng CS or orbit pituitary tumor w1tli fiemorrliage J2ituita!}'. adenoma !90) oituitarv adenoma metastatic Ca in CS tumor in OA cavernous sinus synarome tumors in cavernous sinus* pituitary tumors and cranio2hari!!S:ioma {156) tumor at SOF cranio12haryngioma pituitary tumors; nasopharyngioma; parasellar meningioma* epiilielioma of splienoia wing cranio121ia:n'.ngioma in CS pituitary tumor with hemorrhage cranio12ha!:_Yngioma cboroidal metastases of } mammillary carcinoma pituitary aaenoma pituitary adenoma pituitary tumors; bronchial Ca metastases in CS* nasopharyngeal tumor in CS pituitary adenoma; cranioplia!:_Yngioma; meningioma * pituitary tumors; craniopharyn* geal and other tumors at SOF, OA, CS nasopharyngeal carcinoma pituitary & other tumors* 121fii1tary tumors w1ffi fiemorrliage pituitary tumors numbers in brackets OA = orbital apex. are numbers of cases in report; 1861, Seidel: basal tumor (area of tuber cinereum) 1924, Schaeffer: hypophyseal tumors 1951, Fischer-Brilgge: supratentorial tumors pressing the 3rd nerve upon the clivus edge 1964, Hermans & al. : reticulosarcoma in middle fossa 1975, Shuangshoti: neurolemmoma of 3rd nerve 1976, David: parachiasmal tumor 1983, Hall & al. : pupil sparing 3rd nerve palsy due to cancer metastasis in the cavernous sinus NOTE: Slow-growing tumors that compress the oculomotor nerve, such as cavernous sinus meningiomas, may bring about ~ development of "primary oculomotor misdirection", that is, associated synkinetic movements of extra- and intra-ocular 1:1uscles without history of obvious palsy (Schatz & al., 1977; Boghen & al .• 1979, and others). In these patients, destruction of small nerve bundles and regenerative sprouting of injured fibers proceed at the same time as described elsewhere in this book (see Chapter 11). • 1470 V. Pupillary Pathology: Pupillary Signs in Various Diseases A --<irf;;~ . d B . Figure 42-8. Relation of the phenoid sinus and parasellar part of the base of the kull. A: Diagram of a sagittal midline view. B: agittal cuts through the region of the sphenoid sinus. Note the marked individual variations in size of the sinus and in thickness of its bony walls. The sphenoid sinus is shown in black (s); the stippled areas represent bone; h, hypophyseal fossa; d, dorsum sellae. (A and B modified from J.P. Schaeffer,Anat. Rec., 28 [1924]:243) I -- _c. N Figure 42-9. Four stages of chiasmal deformation caused by meningiomas of the tuberculum sellae. I: Initial stage. II: Early stage, found at autopsy, and probably pre-symptomatic. Ill: Early clinical stage; and IV: Large tumor, weighing 20 g. The symbols mean A.C., anterior commissure; C., chiasm; Hy., hypophysis; I., infundibulum; O.R., optic recess. (From H. Cushing and L. Eisenhardt, in Meningiomas: Their Classification, Regional Behavior, Life History, and Surgical End Results [Springfield, Ill: C.C. Thomas, 1938]) 42. Tumors tumors often expand upward (as well as rostrally and caudally, as seen in Figure 42- 7) to compress the chiasm, or the optic nerves or tracts from below. This upward extension may occur late in patients who have a thick, tough, complete sellar diaphragm. If they also have a large, thin-walled sphenoid sinus the tumor may first expand down into the sinus, as just described. In other patients, however, the sellar diaphragm has a large infundibular foramen, and the diaphragm consists of no more than a peripheral rim of thickened tissue, so that only the arachnoid covers the pituitary gland (Schaeffer, 1924). This favors upward expansion of the tumor. (3) The visual and pupillary field defects brought about by pituitary tumors vary further with the relation between the optic chiasm and these/la in each individual. As described by Schaeffer, the optic chiasm never lies wholely in the chiasmatic sulcus but more caudally (Figure 42-10). In only 5% of 125 bodies examined were the intracranial optic nerves so short that part of the / 1471 chiasm rested on the sulcus. In all other the optic nerves were much longer, o that the chia m wa located entirely over the sellar diaphragm (12% ); or partly over the diaphragm and partly behind it, over the dor um sellae (79% ); or even more caudally, partly over and partly behind the dorsum cllae (4%). A pituitary tumor expanding upward can therefore first come in contact with the ro tral edge of the chia m, it middle, or it caudal part, depending upon anatomic placement of the chia m. And sometimes the optic nerve or tract will be affected by the le ion. In addition, marked enlargement of the third ventricle by tumor invading it from the ba e may push the optic nerve laterally again t the carotid arteries, leading to bina al pupillary and i ual defect . (4) In ome patient the brains/em ride high, extending above the tentorium and causing the optic nerve , chiasm and tracts to )ant teeply upward from the ba e of the kull a they encircle the brain tern (Figure 42-11,A). In uch anatomic variant there i pace Ls cs In Pt Ds 12. % Ls Ts pt In 30 79% Figure 42-10. Rostro-caudal relation between the optic chiasm and the sellar diaphragm. Ls, limbus sphenoidalis; cS, chiasmatic sulcus; Ts, tuberculum sellae; Ds, dorsum sellae; In, infundibulum; Pt, pituitary gland; 3°, third nerve. A: The optic chiasm rests pa_rtlyon_the chiasmatic sulcus, partly upon the sellar diaphragm; this relation was found in only 5% of the bodies examined. B: The chiasm is located wholly over the scllar diaphragm (12%). C: The chiasm i placed almost entirely over the sellar diaphragm but a small portion project posteriorly over the dorsum sellae (79% ). D: The chiasm is located caudal to the sella (4%). (Modified from J.P. Schaeffer, Anat. Rec., 28 ( 1924]:243) 1472 / V. Pupillary Pathology: Pupillary Signs in Various Diseases Table 42-9. Expanding lesions of the orbit among 230 consecutive cases LESIONS Th;yroid o;ehthalmo;eath;y Hemangioma L;ym;ehos arcoma Chronic granuloma {:eseudotumors} Lacrimal gland e;eithelial tumors Meningioma Lymphangioma Glioma of 012tic nerve Metastasis of malignant tumors Peripheral nerve tumors Dermoid c;yst Sinusitis or mucocoele Ra.bdom;yosarcoma Vascular aneur;ysm or fistula Angiosarcoma Osteoma His tioc;ytoma Sarcoid Other Figure 42-11. Vertical relation between the sellar diaphragm and the optic chiasm. A: Note the relatively great distance between the basal surface of the optic chiasm and the superior surface of the ellar diaphragm. The brainstem is placed high and the optic tract lant steeply upward as they embrace it. The infundibulum is long. B: The anterior cranial fossa is low and the dor um sellae relatively high, and there is little space between the base and the chiasm. (A and B modified from J.P. Schaeffer,Anat. Rec., 28 [1924):243) - -A-- - --.,. -2- ..----\- ,,---. '----,- --- ---• t •----1. 37 28 22 18 17 11 10 8 8 7 7 6 5 5 5 2 2 2 6 Patients seen by Reese & Jones; from H.M. Moss. Amer. J. Ophthal. 54(1962):761. Reprinted with permission of the Ophthalmic Publishing Co. •,-_- _- _-_--:.,-_ - -2- ---- ,,=...... _-:,,.,~-.,, - ·--~--l-~~~~-----1- NUMBER -3- - - ~- .,. ~-======= I ~ ½l:~•II::II::ii:=c~·m::n::Jllc::::::::cj~m!ll:Il!!!!::I!:~ Figure 42-12. Pupillary reactions in a case with a large orbital lesion. The patient was a 6-year-old boy. He had a cavernous hemangioma in his left orbit. His left eye was proptosed and displaced temporally and down. Nevertheless the pupils were equal and they reacted symmetrically to all stimuli. The pupillogram showed the ups and downs and the rapid changes in reflex pattern typical 1 :ib::::==:::II:l!lm·~n::JI!!!!Im:::i:::!!!l!!Ill:~ in small children, and there was not the slightest sign of sympathetic or parasympathetic deficit. An apparent left-sided afferent loss was artefactual: due to the displacement of the globe the stimulating light beam hit the eye obliquely (compare A with B and C with D). The child had no visual defect. Right eye, solid lines; left eye, broken lines. 42. Tumors between the sellar diaphragm and the chiasm, so that upward expansion of an intrasellar mass can be tolerated longer than when the brainstem lies low and the optic pathways run flatly along the base, with the chiasm directly above the sellar diaphragm (Figure 42-11,B). Depending on size, location, and direction of expansion of these basal tumors, as well as on the anatomic details of the area, the pupillary defects may, then, include (1) only afferent fibers, which occurs, for example, when a pituitary tumor expands upward and rostrally, or with primary tumors of the optic nerve and chiasm; (2) only the sympathetic and parasympathetic motor pathways, as with tumors confined to the cavernous sinus; or (3) both afferent and efferent paths, as with large parasellar tumors or in the orbital apex syndrome. The pupillary afferent deficit matches the visual loss, and generally both the visual and the pupillary dysfunction follow a time course of slow, relentless progression, except when a hemorrhage into the tumor brings about sudden worsening of afferent conduction. Tumors of the c~vernous sinus and the superior orbital fissure (mening1omas, osteomas, sarcomas, or invading nasopharyngeal or metastatic tumors) may cause proptosis and paralysis of the extraocular muscles by injuring the third, fourth, and sixth nerves. There may be pain, numbness, or dysesthesias due to involvement of the ophthalmic branch of the fifth nerve. Quite similar pain can be caused by traction and pressure upon the blood vessels a~d d~ra at the base. Objective sensory impairment of tngemmal function must therefore be demonstrated before the pain can be blamed on damage to the ophthalmic fifth nerve. Slow-growing tumors that affect the oculomotor nerve such as cavernous sinus meningiomas, may bring abou; development of "primary oculomotor misdirection " that is, associated (synkinetic) movements of vario~s extra- and intraocular muscles without a history of obvious ~alsy (Schatz et al., 1977; Boghen, 1979). In these patients, destruction of small nerve bundles and regene_rative sprouting of injured fibers proceed at the same time, as described in Chapters 11 and 23. Tumors extending upward may also affect the sense of sm~ll (Foster Kennedy syndrome). Third nerve damage with the pupil spared is very rarely due to tumors. Walsh (1957) mentioned that he had seen such a case and Hall_and c<;>-workers(1983) observed a patient with metastatic carcmoma in the cavernous sinus. Pituita:Y. tumors will, of course, bring about their charactenst1c endocrine disturbances. As the tumor expands, additional general and focal signs will appear. The hy~o~halamus is especially vulnerable to pressure fro~ pituitary tumors because of its close proximity, leading to defect_s su~h as diabetes insipidus, obesity, somnolence, and impaired temperature regulation. / 1473 4. Orbital and Ocular Tumors It makes no sense to consider all kinds of orbital tumors that may cause pupillary deficit (Table 42-9), since any orbital mass can interfere with the afferent and efferent reflex fibers and with the sympathetic fiber in the nasociliary and long ciliary nerves. From the point of view of pupillary pathology the interesting points are these. (1) Orbital tumor , especially when they are oft, may become quite large and proptose and di place the globe remarkably without the slighte t impairment of the pupillary reflexes (Figure 42-12). Often all voluntary movements are abolished in such ca es while the pupil continues to react to light, and this has led some author to say that oculomotor damage due to orbital le ion may "spare" the pupil. However, the fixed globe i due mainly to the marked propto is and other mechanical effects of the tumor and not nece arily to paraly i of the oculomotor nerves. The iris i protected within the globe from these mechanical problem and therefore it remains free to re pond. To differentiate such case of orbital tumors, or of dysthyroid infiltration of extraocular muscle , from myasthenic or diabetic "pupil- paring" ophthalmoplegia the forced duction te t i u eful: pushing or pulling the eye in a direction in which it cannot be moved voluntarily by the patient will reveal whether a mechanical hindrance exi t . (2) Orbital tumors, pseudotumors, and other condition leading to pressure within the orbit may cause the tonic pupil syndrome when the ciliary ganglion or hort ciliary nerves are engulfed or otherwise damaged ( ee Figu:e. :4-39). This mu t be kept in mind as an etiologic po~s1b1hty when a patient with a tonic pupil complain of pam or unpleasant sensations around the eye ( other than ciliary spasm). In such case careful measurement with the exophthalmometer and X-ray, ultrasound or computerized axial tomography studies are indicated. . Tu~ors within the eye can, of course, damage the iri itself, its muscles, or their nerve supply. In a single ca e Liegl (1960) and Liegl and Kohn (1961) de cribed the development of a tonic pupil in an eye with a slowgrowing ch_o_roidal tumor. The tumor had interrupted the short c1hary nerves in their intraocular course and on autopsy corresponding retrograde changes were found !n the cilia1?' ~anglion. Simultaneous regenerative sproutmg of surv1vmg terminal accommodative fibers brought about the development of the tonic near-vision responses. 1474 / V. Pupillary Pathology: Pupillary Signs in Various Diseases E. Other Tumors That May Affect the Pupils 1. Diencephalic Tumors: Thalamic, Hypothalamic, Subthalamic, or Basal Ganglion Tumors, or Tumors That Occupy the Third Ventricle of the lesion, together with vasomotor and sudomotor loss in the ipsilateral face and body and with contralateral hemiplegia and sensory disturbances. It is interesting that few of these patients had spontaneous "thalamic pain," though sensory hypersensitivity and paresthesias have been observed (Orzechowski and Mitkus, 1925; Ludecke, 1927; Smyth and Stern, 1938). (2) An extremely rare phenomenon of autonomic excitation was described by Penfield (1929) under the name of "diencephalic autonomic epilepsy." This case is described in Chapter 25. It was a woman with a movable, perfectly round cholesteatoma within the third ventricle that may have acted as a ball valve, causing intermittent rises of cerebrospinal fluid pressure. Episodic autonomic discharges were observed also by Bittorf (1920) in a patient with a large ependymal cyst in the third ventricle that was attached to and compressed the right thalamus; by Barre and Alfandary (1929) in a patient with hypophyseal tumor that had grown laterally toward the basal ganglia; by McLean in a patient with an astrocytoma arising from the floor and walls of the third ventricle; and by Foerster and Gagel (1936) in a patient with a craniopharyngioma that had invaded the third ventricle. In all these patients maximal mydriasis accom- Primary tumors of the thalamus are relatively rare (mo tly glioblastomas or astrocytomas), and usually are not confined olely to the thalamus. More commonly, the diencephalon is invaded secondarily by metastatic or nearby intrinsic tumor such as pineal or upper braintern tumors from the caudal side, by pituitary, craniopharyngeal, or other basal tumors from below (Figure 42-13) or laterally by temporal lobe tumors. Third ventricle tumor ( colloid cyst , choroid plexus papilloma , ependymomas, or other ) tend to spread to the ylvian aqueduct and the lateral ventricles. And presure from neighboring tumor upon the diencephalon, or econdary hemorrhages or vascular occlusions complicate the clinical picture further. Aside from regional tumor signs and those due to increa ed intracranial pressure, if present, three diff rent combinations of pupillary with other defects have be n observed in patients with tumors in the thalamicubthalamic area. (1) A already mentioned, occasionally the central ympathetic neuron is affected in cases with lateroventral thalamic or subthalamic tumors (Table 42-3). Such patients had oculo-sympathetic deficit on the side Figure 42-13. Hypophyseal duct cyst, compressing the hypophyseal stalk and basal diencephalon. Larger masse in this area extend to the brainstem. Hydrocephalus is caused by compression of the aqueduct. (From W.E. Dandy, in Practice of Surgery, D.D. Lewis, Ed. LHagerstown, Md.: W.F. Pryor, 1936], Vol. 12) ltypol'hyeu/ ~JlyML dyer <;,:j-1 w,th ch .. ,-::,r,-y.j, cry ·ab 42. Tumor Table 42-10. YEAR 18 -l 1899 1900 1900 1902 1903 1910 1911 1925 1927 1927 1927 ~ 1929 1929 1931 1932 1936 1938 1938 1952 1954 1958 1958 1475 Oculomotor disturbances in patients with thalamic and third ventricle tumors PUPIL TUl\lOH AUTHOR o"'l-l, thalamic tumor Sieveking c":r58. 3rd ventricle sarcoma * l\lever _d' 13, bilateral thalam ic tumor gro_wing caudally * Jacobsohn <1'3-! choroid olexus tumor above infund1bulum Mott & Barrett . * B ie lsc ho wsky _Q 18, choroid plexus tumor growing caudally * papilloma of choroid plexus, fill mg the third ventricle lt3 Henneberg bilateral thalamic tumor Pfeifer#61 =<1'50, with small <1'50, tumor in caudal 2/3 of L thalamus, lt62 extension into R thalamus eigeon-egg-sized tumor in R th_alamus "'63 -<1'32, extending into Q 17, glioma from walls of 3rd ventricle, Weis en burg lateral ventricle and aqueduct Orzechowski 48, hypophyseal cystic tumor extending into 3rd ventricle & Mitkus tumors of ventricles and their walls (abst.) Hogner tumor oI 3ra and lateral ventricles (a6st.l LUdecke Seletzki _a' 34 1 thalamic glioma, invading R thalamus Meyer ~66, cystic pineal tumor distorting thalamus & rostral brain stem Igersheimersuprasellar tumors Fuiton ~Bailey tumors oI 3ra ventricle, ancl oiliers in aiencephalic-mesen _ cephalic border zone Stertz * #1 <1'35, cist from pes ~dunculi, flattening the chiasm jg__ _Q 42, no auto2sy . . . #3 Q 47, apple-sized tumor in L basal nuclei, f11lrng the 3rd _ ventricle and extending caudally * #4 a' 55, tumor at base of diencephalon, infiltrating thalamus and comeressing 3rd ventricle and colliculi * 4f.7 craniopharyngioma invading 3rd ventricle ~ -<1'42, post-traumatic abscess in R pes pedunculi, extending to thalamus * Allen & Lovell <1'15 1 glioma extending into 3rd ventricle* Foerster & invading the 3rd ventricle & <;? , craniopharyngioma Gagel agueduct * Nevin #1 7, gliomatosis of the thalamus ~ 46 irliomatosis of the thalamus Smyth & #1 ~50, glioma left thalamus & midbrain tegmentum * Stern #2 !;?43, R thalamic tumor extending to mid brain * 2 -8. :/&._ _astrocY!Qma reelacing left thalamus #4 _d' 67, astrocY!oma L thalamus, irrowing caudally ~ d' 68 1 diffuse thalamic glioma invading mid brain #6 <;?44, tumor R thalamus, habenulae and colliculi 1939 / Hyndman & Van E1;rns Fanta & Reisner Skorodumova Arseni McKissock & Paine 3 cases with thalamic <;?56, large suprasellar * * tumor large and fixed reactions of left eueil diminished pupils unequal; convergence palsi both pueils sluggish; Pt; D pupils L> R I fixed to light & near; ! eueils L RI almost fixed to light pupils L > R; light reflexes R sluggish DEFECTS both pupils sluggish pupils R disturbed pupils to light ( L worse) ; conv. ; .-. __ L !IQ§f!lt : Pt L, fixed to light light reflexes unequal, irregular shape, fixed to light unequal, large, poor-ly reactive pupils bilaterally on some clays clisturbea light rel1ex; Pt; D pupils R ) L, fixed to light (patient blind!; D; Ny disturbed light reflexes hemiopic pupillar~ defects pupils large ancl s~uggis!i, sometimes clissociatecl pupils unequal, distorted & fixed OU; Pt·,t. R~ L, ixea to lignt OU i EOM i Ny unequal, distorted, reactions to light reduced pupils distorted reduced n.s. bilaterally, pupils distorted; unequal, unequal; EOM R>> L, L fixed to light; Pt; Nt; extreme mydriasis during light reflexes 6th n. palSJ:'. epileptiform attacks left euEil reacted only sluggishlJ:'. to l!ght light reflex diminished, eseecially R pupils 5 mm R L, fixed to light, good to near; convergence ealsy; ++ R4, L3 mm; irregular, fixed to light &active to near· Pt· converirence palsy 4 mm R zL, reacting briskli:: 3 mm R =1. almost fixed to light; 2 mm OU. trace of light reflex conv. • D R - L, moderate size; fixed to light, Ieebly reactive to near;;; conv.; Ny; ➔ #2 had right pupil fixed to light craniopharyngioma third ventricle craniopharyngioma among 2000 tumors, 36 thalamic (mostly gliomaJ among :.1500 tumors, :,:4 thalamic, but seldom conlined thalamus: only 3 isolated thalamic tumors * 1961 Tovi & al. among 5000 tumors, 49 thalamic, usually beyond thalamus, often to midbrain extending 1966 Cheek & Taveras among 6048 tumors, astrocytoma; * glioblastoma 51 thalamic, AND OTHER OCULOMOTOR R eueil mostly to or pupils small, hxed to hght and well reactive to near vision; D· Ny small i1 aqueduct is blocked 3 cases with AR eueils and loss of ueward S!!:ze among 36 patients, 6 with anisocoria, 6 with abnormal pupil reactions; 3 of these poor to light, good to near; t; D; 3rd n. i Ny 10 wHht, 3 with 3rd nerve deficit; 16 anisocoria; 14 D; 14 light reflex lost or sluggish; 15 larger oupil oooosite lesion 11 with ipsilateral miosis, some with slight ptosis 1n column "AUTHOR", numbers marked JF are individual case numbers of original; in "TUMORS", V =ventricle; (abst.) = only abstract read by reviewer; R = right, L = le ft; 0 67, O 17, etc. = patients' sex and age; * = anatomic proof of caudal ex tens ion of tumor; In column "PUPTLLARY AND OTHER DEFECTS", R = right, L = left, and OU= both eyes; EOM = extraocular muscle defect; Pt= ptosis D = diplopia; conv. = convergence palsy or paresis; AR = Argyll Robertson pupils ;'t = loss of upward gaze:t = loss of up and down gaze; ._. = horizontal gaze palsy; -+ or+= right or left gaze palsy; Ny = nus ta gm us; n .s. = not reported. NOTE: Signs in these cases overlap with those of rostro-dorsal midbrain panied paroxysms of autonomic activity (chiefly sympathetic). In Pcnfield's and in Foerster and Gagel's cases no somatic movements took part in the seizures, while Bittorfs patient had tonic spasms and Barre and Alfandari's patient developed full-blown Jacksonian seizures. Rare tumors that affect the medial portions of the temporal lobe by invasion or pressure have caused tumors extending to tho dicncephalon or 3rd ventricle. pupillary dilation and other autonomic signs during psychomotor seizures. (3) In patients with tumors involving the dorsomedial portions of the thalamus, sensory disturbances are often absent or develop later, and hemipare is is mild or absent at first. Instead, there is a peculiar mental syndrome that-together with pupillary pathology- 1476 / V. Pupillary Pathology: Pupillary Signs in Various Diseases caused ome of the ca es to be mi diagnosed as general paresi . The patients complained of las itude and no initiative or drive. They became dull, inattentive, and apathetic and-with progression of the tumor--<:onfused and di oriented. OccasionalJy they were irritable and negative, crying and re isting examination. At other times a flat euphoria and Kor akoff-like excitement were prominent. Their pupil reacted poorly to light, while the reaction to near vision were relatively well preserved (Table 42-10). The mental change were not the result of elevated intracranial pres ure, which developed later and wa accompanied by increasing drowsiness and tupor, leading to coma and death. They appeared early and were een in patients with small tumors without increased intracranial pres ure. They may have been related to interruption of impul e from the midbrain and diencephalic reticular formation to higher brain centers just as the cerebellar sign in these patients (vertigo, cerebellar gait, ataxia, retropul ion) were explained by interruption of the cerebello-thalamic-cortical path (Smyth and Stern, 1939). In rare ca es with third ventricle tumors a picture of akinetic mutism developed, without cortical injury. A patient de cribed by Cairns and co-workers (1941) regained full consciousness 10 minutes after pre ure of a dermoid cyst in the third ventricle was relieved. No pupillary defects were mentioned in this patient. The pupillary defects that accompanied the mental ign in most of these patients--especially the early appearance of Argyll Robertson pupils-doubtless were due to midbrain involvement, as was proven at autop y in many of the patients, where a distended aqueduct, flattened colliculi, or other anatomic damage in the rostral midbrain were found. The pupillary defects were frequently associated with other ocular signs of midbrain damage: gaze palsies, convergence weakness or loss, nystagmus, ptosis, or diplopia due to paresi of the third or the sixth nerve (Table 42-10). Tinnitus and deafness were ascribed to destruction of the inferior colliculi or their connections to the temporal lobe, while hemianopia, tremor of the face, tongue, or extremities, extrapyramidal disturbances, sensory loss, and hemiplegia were thought to be due to damage in the thalamus, basal ganglia, and internal capsule. raised thre holds for pupillary light reflexes. These findings were exaggerated by claiming that they had "pupillary hemiakinesia," as is found after damage to the optic tract. However, direct or secondary involvement of the optic tract was not ruled out with certainty in any of these cases; and retrograde (trans-synaptic) degeneration of the primary optic pathways after destruction of the central visual neuron has been described (see Chapters 3 and 11). Further, patients with occipital tumors often have additional parasympathetic pupillary deficit on the same or on the opposite side, due to pressure or vascular impairment that affects the third nerve or the upper brainstem, as described in more detail in the section on supratentorial mass lesions (see below). 3. Diffuse Tumor Syndromes Tumors that cause multiple metastases or invade large areas of the central nervous system by perivascular or meningeal infiltration, or by shedding malignant cells into the cerebrospinal fluid (medulloblastomas, carcinomas, melanomas, multiple myelomas, reticulum cell sarcomas, lymphomas) may affect any part of the pupillary reflex paths. Neoplastic diseases that bring about recurrent migratory phlebitis, endocarditis, or anemia (such as the leukemias) may cause pupillary and other neurologic deficits by way of secondary ischemia, hemorrhages, or vascular occlusions. In addition, from 2 to 5% of patients with neoplasm develop neurologic deficit due to toxic or metabolic effects and not to invasion of the brain or cord by the tumor (Victor and Adams, 1977; Gilroy and Meyer, 1975). Often carcinoma of the lung or stomach is responsible, but every type of tumor has been found. Polyneuropathy, myasthenia-like syndromes (Eaton-Lambert syndrome), secondary amyloidosis, cerebellar and ponto-cerebellar degeneration, diffuse encephalopathy, and encephalomyelitis-like changes occur. Such patients will have pupillary changes if the pathology involves the central or peripheral parts of the pupillary reflex arcs. Beallo (1965) described bilateral tonic pupils in a patient with polyneuropathy due to oat cell carcinoma of the lung. More such cases probably will be discovered now that these syndromes have come to general attention. 4. Pheochromocytoma 2. Occipital Lobe Tumors Cases with tumors in the occipital lobe are mentioned here because they have been used to support the erroneous assumption that the pupillary light reflex path runs via the occipital cortex to the midbrain rather than by the brachia of the superior colliculi (see Chapter 17). Some of the patients with occipital mass lesions had The pupils participate in the global paroxysms of adrenergic activity seen in patients with pheochromocytoma. These attacks are caused by episodic outpouring of catecholamines into the blood. No doubt such cases played a role in early reports about episodic sympathetic hyperactivity. During intervals between attacks the pupils are normal.