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Show }. Clin. Neuro-Ophthalmol. 1: 67-75, 1981. The purpose of this section is to provide the reader with the latest clinically applicable reference information. Over 100 sources were reviewed for this section. Most of the references cited are not from the usual ophthalmological literature. The main thrust here is to provide the reader with information from sources not often consulted by ophthalmologists. There are occasional exceptions, of course. It is my hope that the reader will enjoy having access to the newest clinical information as much as I have enjoyed the research and preparation. The name and address of the author responsible for providing reprints is included in each of the summaries for those who wish to request reprints. Concise Summary 1. Penicillamine can produce a reversible form of myasthenia gravis. 2. Reformation of computerized tomograms is the best way to judge the true size of the extraocular muscles or the optic nerve. 3. Chronic uveitis in conjunction with neurologic signs could be the key to the diagnosis of Whipple's disease. Biopsy is diagnostic and antibiotics will save the patient's life. 4. Astereognosis may not always be a parietal lobe sign. It can occur with brain-stem tumors. 5. Temporal artery biopsy may be needed in late management of giant-cell arteritis to determine the effectiveness of treatment, even with a normal sed rate. 6. The sudden onset of seborrheic dermatitis (Leser-Trelat sign) is an important sign of internal malignancy. 7. Opsoclonus can be a sign of thalamic hemorrhage. 8. A history of progressive neurologic deterioration in a patient with bilateral internuclear ophthalmoplegia may not be caused by the progressive form of multiple sclerosis. 9. Oval pupils can be a feature of an evolving cerebrovascular event. This sign is of brainstem origin. 10. Lithium carbonate is apparently of value in the management of patients with cluster headache. 11. Ocular drift is an important sign of medullary dysfunction. From Baylor University School of Medicine, Houston, Texas. March 1981 Information. Summaries JOHN A. McCRARY, III, M.D. 12. Acquired ocular retraction movements are a sign of orbital pathology. It may not be Duane's syndrome after all in that patient with diplopia! 13. Gliomas and meningiomas of the optic nerve require more flexibility in management than we thought, depending on their behavior. 14. Two reports suggest that the oculomotor nerve palsy that sometimes occurs with migraine is not the result of compression of the nerve by the carotid artery. 15. Cimetidine is reported to cause a reversible motor neuropathy. 16. Yet another author relates that recurrent attacks of ischemia may occur in an eye with anterior ischemic optic neuropathy. 17. Aqueous LDH levels are important in the diagnosis of retinoblastoma, if doubt exists. 18. Late-life migraine can cause stroke-like symptoms. Special features help differentiate these attacks from events produced by thrombosis or embolism. 19. A transient lower motor neuron paralysis can occur with an attack of asthma and look just like a case of polio. Asthma can produce serious neurologic consequences. 20. Some xanthomas can be associated with serious visual complications. 21. Livedo reticularis may be associated with severe cerebrovascular disease. This skin sign is important. 22. Hyperprolactinemia is not always caused by a pituitary adenoma. 23. There are specific CT changes which can differentiate Graves' orbitopathy from pseudotumor of the orbit. 24. Palinopsia is an important clinical diagnostic tool. 25. Cataplexy can be a feature of midbrain neoplasia. 26. Fractures of the sella are best diagnosed with plain films and polytomes of the sella, not the CT scan. A fractured sella is potentially dangerous to the patient. 27. What seems to be Reye's syndrome may really be carnitine deficiency. Recurrent attacks are the clinical key. 28. The fetal alcohol syndrome may be the precursor of neural crest tumors. 29. Delayed high-dose contrast-enhanced CT scans may be more useful in the diagnosis of 67 Information Summaries brain tumors of all types than the more usual immediate postinfusion scan done with a standard dose of contrast agent. Reference: Rousseau, J.J., and Dieudonne, L.G.: Myasthenia gravis syndrome following administration of a-penicillamine. Acta. Neurol. Belg. 80: 165-173, 1980. Reprints: Dr. J-J. Rousseau, Institut de Medecme, ~ecteur de Neurologie, Hopital Universitaire de Bavlere, B4020 Liege, Belgium. Over 40 cases of myasthenia gravis have thus far been reported in the literature concerned with the complications encountered in treating patients with a-penicillamine. The authors describe the onset of myasthema in a patient being treated with a-penicillamine for 6 months. The medication was used to treat advanced rheumatoid arthritis. The myasthenic symptoms cleared rapidly after the treatment with a-penicillamine was discontinued. • Reference: Unsold, R., Newton, TH., and Hoyt, W.F.: CT-Evaluation of extraocular muscles-AnatomicCT- correlations. Alb. v Graefes. Arch. Klin. Ophthalmol. 214: 155-180, 1980. Reprints: T.H. Newton, M.D., Dept. of Radiology, University of California Medical Center, San Francisco, Calif. 94143. The extraocular muscles cannot be properly evaluated by computerized tomography if standard axial and coronal sections are studied. By analysis of anatomic and computerized tomographic sections of the orbit, it was found that reformatting was the ideal method of determining true muscle size. As a rule, visualization of any ocular muscle is optimal if the plane of section or reformation is parallel to the course of the muscle. Any judgment of the size of an ocular muscle should be based only on true cross sections obtained from reformatted tomograms known to be perpendicular to that muscle. • • Reference: Johnson, L., and Diamond, I.: Cerebral Whipple's disease: Diagnosis by brain biopsy. Am. ]. Clin. Pathol. 74: 486-490, 1980. Repnnts: Lewis Johnson, M.D., Dept. of Pathology, Roger Williams General Hospital, 825 Chalkstone Ave., Providence, R.1. 02908. Whipple's disease is a rare, multisystem, chronic granulomatous condition which usually presents with gastrointestinal or arthralgic symptoms. It is treatable with antibiotics. The diagnosis is usually made by biopsy of the mucosa of the small intestine. The biopsy shows multiple, small, PAS-positive inclusions in the cytoplasm of macrophages in the lamina propria. The causative agent is a small bacteria. Whipple's disease may rarely present with predominantly neurologic findings. Such a patient is described in this report. The diagnosis was made by brain biopsy. The patient was a 51-year-old male who presented with weakness of the left arm and leg, cataracts, uveitis, and polyarthralgia. A CT scan showed an abnormal right parietal focus. The 68 patient improved on treatment. Untreated Whipple's disease quickly causes death in most cases. • • • Reference: Unsold, R., DeGroot, J., and Newton, TH.: Images of the optic nerve: Anatomic-CT correlation. Am. ]. Roentgenol. 135: 767-773, 1980. Reprints: TH. Newton, M.D., Dept. of .R.adiology, University of California School of MediCine, San Francisco, Calif. 94143. The features of the intraorbital portion of the optic nerve were correlated with computerized tomographic sections taken in several different planes, respectively. The anatomic and CT appearances of the optic nerve were analyzed. It was appare~t that the sinuous course of the optic nerve In the orbit, whether viewed in the axial or sagittal plane, made the nerve seem to be thinned in some tomographic sections. Computerized tomographic images of the optic nerve taken with the angulation -100 to the orbitomeatal line will make axial studies of the nerve optimal. Volume averaging with 8.0-mm sections makes the image of the nerve more uniform. Reformatting, especially with sagittal views, is the best way to reveal the course and thickness of the optic nerve. • • • Reference: Feinsod, M., Bentin, S., Moscovitch, M., et al.: Brain-stem tumor presenting with unilateral astereognosis. Ann. Neural. 8: 191-192, 1980. Reprints: Moshe Feinsod, M.D., Dept. of Neurosurgery, Hadassah University Hospital, Ein-Kerem, Jerusalem, Israel. Astereognosis is generally regarded as a sign of parietal lobe dysfunction. This case report describes a 13year- old female patient who had astereognosis as the predominant neurologic sign. The patient had a brainstem neoplasm as the cause for this sign. This case and those previously reported with brain-stem tumors and astereognosis confirm that a brain-stem etiology for this important sign is, though not common, worth remembering. • • • Reference: Thompson, H.5., and Corbett, J.J.: Spasms of the iris sphincter. Ann. Neurol. 8: 547-549, 1980 Reprints: H. Stanley Thompson, M.D., CS. O'Brien Library, Dept. of Ophthalmology, University of Iowa Hospitals and Clinics, Iowa City, Iowa 52242. The authors report a patient with periodic spasms of pupil constriction and accommodation. Both the pupil sphincter and ciliary muscle were contracting several times per minute. The patient complained of sporadic dimness of vision during these events. Only one eye was involved. The spasms lasted for only 11 days and then disappeared. Pupillographic documentation of this phenomenon was obtained. The mechanism for this unusual uniocular parasympathetic activity is unknown, but transient irritability of the ciliary ganglion is suggested as a possible cause. Journal of Ginical Neuro-ophthalmology • • • Reference: Fisher, J.P., Costin, '.A., and Smith, j.L.: Perspectives in neuro-opthalmology: Pulmonary edema in giant cell arteritis. Ann. Ophthalmol. 12: 779-781, 1980. Reprints: Jerome P. Fisher, M.D., Bascom P.llmer Eye Institute, P.O. Box 016880, Miami, Fla. 33101. A79-ye~r-old physician who had been on continuous sterOid therapy for 3 years for treatment of giant cell arteritis developed severe recurrent pulmonary edema. Despite normal monthly sedimentation rates while on continous steroid therapy and no headache complaints, a temporal artery biopsy was done. It revealed severe active inflammatory disease. The patient had the steroid dose increased and experienced resolution of most of his cardiorespiratory symptoms. • • • Reference: Curry, 5.5., and King, L.E.: The sign of LeserTrelat. Report of a case with adenocarcinoma of the duodenum. Arch. Dermatol. 116: 1059-1060, 1980. Reprints: Lloyd E. King, M.D., Dermatology Research, V.A. Medical Center, Nashville, Tenn. 37203. The sign of Leser-Trelat is a rare cutaneous manifestation of internal malignancy. Although adenocarcinoma is the most. common malignant neoplasm associated with this sign, this may be the first report of duodenal adenocarcinoma as a cause. The sign of LeserTrelat is the sudden appearance of a large number of seborrheic dermal lesions. If seborrheic dermatosis which is severe and generalized does suddenly develop, a search for malignancy should be performed. • • • Reference; Solomon, L.M., and Keuer, E.J.: The ectodermal dysplasias. Problems of classification and some newer syndromes. Arch. Dennatol. 116: 1295-1299, 1980. Reprints: Lawrence M. Solomon, M.D., Abraham lincoln School of Medicine, Dept. of Dermatology, Chicago, Ill. 60680. This is an excellent short review of the newest classification of the disorders grouped under the banner of ectodermal dysplasia. Many of the diseases and syndromes so classified have important ocular signs. For those with a special interest in such matters and particularly for the "syndromo!ogists," this paper is recommended. • • • Reference: Keane, J.R.: Transient opsoclonus with thalamic hemorrhage. Arch. Neurol. 37: 423-424, 1980. Reprints: James R. Keane, M.D., 1200 North State St. Los Angeles, Calif. 90033. This report deals with three patients with thalamic hemorrhage who manifested opsoclonus in connection with pupillary and other ocular motility signs of pretectal brainstem dysfunction. Opsoclonus is a rare ocular movement disorder that is most often caused by a benign encephalitis after viral infection. The author March 1981 McCrary suggests that the mechanism for the ocular movement in these patients with thalamic hemorrhage may be the release of spontaneous saccades of cerebellar system origin which follows a loss of normal inhibition of these movements as the result of brain-stem dysfunction. • • • Reference: Troost, B.T., Martinez, J., Abel, L., et al.: Upbeat nystagmus and internuclear ophthalmoplegia with brainstem glioma. Arch. Neurol. 37: 453-456, 1980. Reprints: B. Tood Troost, M.D., V.A. Medical Center (127), University Drive C, Pittsburgh, Pa. 15240. The authors describe the findings in a 16-year-old girl with a progressive neurologic condition. 5he developed a bilateral internuclear ophthalmoplegia and a primary position upbeating nystagmus. A CT scan revealed a lesion in the region of the fourth ventricle and pneumoencephalography an infiltrative neoplasm of the brain stem. When a portion of the tumor was exposed within the fourth ventricle at the time of surgery, biopsy specimens confirmed that the neoplasm was a glioma. Irradiation therapy was begun. • • • Reference: Fisher, C. M.; Oval pupils. Arch. Neurol. 37: 502-503, 1980. Reprints: C. Miller Fisher, M.D., Neurology Service, Massachusetts General Hospital, Fruit 5t., Boston, Mass. 02114. This report deals with 17 patients who, during the course of their neurologic illness, developed an oval configuration of one or both pupils. 5ixteen of the patients had serious cerebrovascular disease and one was recovering from a third nerve palsy. In the group with cerebrovascular disease, there were five with hypertensive cerebral hemorrhage, five with ruptured aneurysms, two with bilateral cerebral infarction, two with brainstem stroke, and one each with epidural hemorrhage and cerebral anoxia. Oval pupils were most often found during a stage in evolution of brainstem compression. The pupils tended to become round as further neurologic deterioration occurred. • • • Reference: Medina, J,L., Fareed, J.. and Diamond, 5.: Lithium carbonate therapy for cluster headache. Changes in number of platelets and serotonin and histamine levels. Arch. Neurol. 37: 559-563, 1980. Reprints: 'ose L. Medina, M.D., Diamond Headache Clinic, 5252 North Western Ave., Chicago, 111. 60625. Lithium carbonate was given to one of three groups of patients as treatment for cluster headache. The dose was 900 mg/day and on that dose there was a significant decline in headache complaints. There was a rise in the platelet count and a decline in platelet-rich plasma histamine and platelet serotonin while the patients were taking lithium. When the treatment with this agent was discontinued, the headaches returned as serotonin and histamine rose to pretreatment levels. • • • 69 Information Summaries Reference: Meyer, K. T, Baloh, R.W., Krohel, C.B., et al.: Ocular lateropulsion. A sign of lateral medullary disease. Arch Ophthalmol. 98: 1614-1616, 1980. Reprints: Kenneth T Meyer, M.D., Jules Stein Eye In-stitute, Los Angeles, Calif. 90024. Four patients with clinically localized lesions in the lateral medulla exhibited tonic bias of their eyes toward the side of the neurologic injury despite the fact that they could generate full ocular movements. Each reported that there was a feeling that the eyes were being pulled toward the involved side. Although tonic bias was most prominent when fixation was inhibited, it also occurred with fixation and interfered with saccadic and smooth-pursuit movements. Saccades were hypometric when directed against the tonic bias, whereas they were hypermetric when directed toward the side of the lesion. Smooth pursuit toward the affected side was severely impaired; pursuit in the direction of the bias was normal or near normal. · . . Reference: Osher, R. H., Schatz, N.}., and Duane, TO.: Acquired orbital retraction syndrome. Arch. Ophthalmol. 98: 1798-1802,1980. Reprints: Robert H. Osher, M.D., Bascom Palmer Eye Institute, 900 N.W. 17th St., Miami, Fla. 33152. Four patients with infiltrative orbital disease involving extraocular muscles revealed a characteristic retraction- motility pattern. The cardinal feature was retraction of the globe on attempted gaze to the side opposite the field of action of the restricted muscle. When motility was impaired in this way, the forced duction test was positive. Modest proptosis and episcleral vascular congestion over the extraocular muscle insertions were associated findings. Dysthyroid eye disease, inflammatory myositis, and neoplasms are among the infiltrative myopathies that may produce an acquired orbital retraction syndrome. • • • Reference: O'Day, }., Billson, F., and King, J.: Ophthalmoplegic migraine and aberrant regeneration of the oculomotor nerve. Br]. Ophthalmol. 64: 534-536 1980. ' Reprints: Dr. John King, Royal Melbourne Hospital, Melbourne, Victoria 3050, Australia. Apatient with ophthalmoplegic migraine developed aberrant regeneration of the oculomotor nerve. This discovery supports the view that the oculomotor nerve lesion in this condition is peripheral. The extreme rarity of aberrant regeneration suggests that the mechanism of oculomotor nerve dysfunction in migraine is ischemic rather than being caused by compression of the nerve by an edematous intracavernous carotid artery. • • • Reference: Wright, I.E., McDonald, W.I., and Call, N.B.: Management of optic nerve gliomas. Br. ]. Ophthalmol. 64: 545-552, 1980. Reprints: Mr. John E. Wright, Moorfields Eye Hospital City Rd., London ECIV 2PD, England. ' 70 T his report deals with 17 patients with a diagnosis of orbital optic nerve glioma when first seen. After being followed for up to 12 years, there was enlargement of the optic canal in 15 of 16 patients. This finding was an unreliable indicator of the posterior extent of the tumor. Nine patients had a stable course with little change over a period of up to 8 years; there was optic atrophy in all and neurofibromatosis was relatively common (7/9). Eight patients had progressive enlargement of the tumor; six had swollen discs, and the incidence of neurofibromatosis was relatively low (3/8). The optic nerve was excised in seven of the latter group. Biopsies of the optic nerve taken from the region of maximal enlargement were difficult to interpret. Radical surgery (excision of the orbital optic nerve) should be reserved for those with evidence of progressive deterioration (about half of patients) while the remainder should be followed. The optic nerve should be excised in progressive disease only if the chiasm is spared. • • • Reference: Wright, J. E., Call, N.B., and Liaricos, S.: Primary optic nerve meningioma. Br. ]. Ophthalmol. 64: 553-558, 1980. Reprints: Mr. John E. Wright, Moorfields Eye Hospital, City Rd., London ECIV 2PD, England. T wenty-seven patients with meningiomas arising from within the optic nerve sheath were reported. Twenty-one were female patients, the majority aged 3964 years. The men were younger with most between ZO and 41 years when first seen. There were no patients under the age of 20 years. Twenty-two had progressive loss of visual function as the initial complaint. The optic nerve was abnormal in all patients; 14 had swollen discs and in 13 there was optic atrophy. Neurofibromatosis was not associated with optic nerve meningioma. Treatment was surgical. The authors recommend that biopsy or any other procedure which transgresses the optic nerve sheath be avoided unless the rate of growth suggests a malignant type of meningioma or CT scans show the. tumor to be very anterior and vision is good, in which case exploration can yield a good result. For all others, they recommend waiting until vision is very poor so that the optic nerve and meningioma can be removed by a single total excision. • • • Reference: Walls, T.}., Pearce, S.J., and Venables, C.S.: Motor neuropathy associated with cimetidine. Br. Med.]. 281: 974-975, 1980. Reprints: TJ, Walls, MRCP, Dept. of Medicine, Dryburn Hospital, Durham DHI 5TW, England. Two patients wit~ asthma developed a transient motor neuropathy while on treatment with cimetidine. The neuropathy, affecting all four extremities, cleared rapidly ~hen the drug was discontinued. This drug has been Implicated In the production of arrhythmias, thrombocytopema, and coma in other reports. • • • Reference: Abramson, D.H., and Ellsworth, R.M.: Andl- Journal of Clinical Neuro-ophthalmology lary tests for the diagnosis of retinoblastoma. Bull. N. Y. Acad. Med. 56: 221-231,1980. Reprints: David H. Abramson, M.D., 70 East 66th St., New York, N.Y. 10021. The diagnosi.s .of retinoblastoma is usually easy to make on cllmcal grounds. Occasionally, though, a whitish mass may be present in the fundus of an eye which is difficult to differentiate from Coat's disease, Toxocara canis, or other clinical entities. The authors suggest the use of skull films, CT scans, and ultrasound testing to look for calcification within the eye which is typical in retinoblastoma. Measurement of LDH levels on intraocular fluids is also proposed as a very useful method for differentiating retinoblastoma from other disorders. Most patients with retinoblastoma have very high aqueous LDH levels, as compared to normals where LDH measures 250 IU/L in children and 150 IU/L in adults. Best correlation of LDH with retinoblastoma occurs when levels are over 6000 IU/L. Only 7"l0 of retinoblastoma cases have normal LDH levels, and the false-positive rate is 1%. • • • Reference: Miller, N.R.: Anterior ischemic optic neuropathy: Diagnosis and management. Bull. N. Y. Acad. Med. 56: 643-654,1980. Reprints: Neil R. Miller, M.D., The Wilmer Ophthal-mological Institute, Baltimore, Md. 21205. The author stresses the importance of steroid therapy in the management of this condition when it is associated with temporal arteritis. If temporal arteritis is suspected clinically and an initial temporal artery biopsy fails to confirm the diagnosis, biopsy of the other temporal artery is suggested for consideration. The idiopathic form of ischemic optic neuropathy does not have a suitable treatment at this time. The author mentions the poSSibility that treatment with aspirin might have potential benefit. He further notes that recurrent attacks of ischemia may occur in an affected eye, despite common opinion to the contrary in other reports. • • • Reference: Fisher, CM.: Late-life migraine accompaniments as a cause of unexplained transient ischemic attacks. Can. f. Neural. Sci. 7: 9-17, 1980. Reprints: C Miller Fisher, M.D., Dept. of Neurology, Massachusetts General Hospital, Fruit St., Boston, Mass. 02114. Certain patients in the stroke-prone age group, over 40 years of age, have transient cerebral ischemic attacks in association with normal cerebral angiograms. A review of 120 such patients was undertaken in whom there were transient episodes suggestive of migraine. Typical of this group of patients is a buildup and migration of visual scintillations, a march of paresthesias, and progression from one neurologic accompaniment to another, which are not characteristic of thrombosis or embolism. Diagnosis is facilitated by two or more similar episodes or migraine-like scintillations. Headache occurred in 5()%. Other cerebrovascular processes, coagulation disorders, and seizures must be ruled out. Arteriography is advised in all but classic cases. March 1981 McCrary • • • Reference: Vijayan, N.: Ophthalmoplegic migraine: Ischemic or compressive neuropathy? Headache 20: 300-304, 1980. Reprints: N. Vijayan, M.D., University of California, Davis Medical Center, 2315 Stockton Blvd., Sacramento, Calif. 95817. Popular current opinion suggests that the oculomotor nerve impairment seen in some cases of migraine is the result of compression of the cranial nerve by a swollen carotid artery. The basilar artery has also been implicated. A patient with ophthalmoplegic migraine was seen and the pupil was completely spared. A review of the literature showed that complete paralysis of the pupil occurs in about 33% of patients with ophthalmoplegic migraine and nearly 40% have completely spared pupils. The remainder have partial impairment of pupil function. The high frequency of normal pupil function or only partial involvement suggests that the etiology of the ophthalmoplegia is an ischemic neuropathy not a compressive process. • • • Reference: Mimura, 0., Shiraki, K., and Shimo-oku, M.: Fundus controlled perimetry in the tilted disc syndrome. fpn. f. Ophthalmol. 24: 105-111,1980. Reprints: Osamu Mimura, M.D., Dept. of Ophthalmology, Hyogo College of Medicine, 1-1, Mukogawa-cho, Nishinomiya, Japan, 663. The tilted disc syndrome is one of the congenital anomalies of the optic nerve which often has a visual field defect associated with it. The visual field anomaly is often bitemporal and suggests an intracranial lesion. Local ectasia in the fundus is the cause for the visual field defect. The results of this study indicated that the visual field defect is not the result of an ectatically induced local refractive error, as previously reported, but is instead the result of localized neuroretinal hypoplasia. • • • Reference: Beede, H.E., and Newcomb, R.W.: Lower motor neuron paralysis in association with asthma. fohns Hopkins Med. f. 147: 186-187, 1980. Reprints: Richard W. Newcomb, M.D., La Rabida Children's Hospital. E. 65th St. at Lake Michigan, Chicago, Ill. 60649. The author reports a case of an 8-year-old boy who developed seizures, altered mentation, apneic episodes, and a lower motor neuron quadriparesis in association with an attack of asthma. The child slowly improved. All testing for poliovirus and other known causes for this neurologic condition was negative. Other reports of this phenomenon have uniformly failed to identify a cause. • • • Reference: Kossard, S., and Winkelmann, R.K.: Necrobiotic xanthogranuloma with paraproteinemia. f. Am. Acad. DermJtol. 3: 257-270, 1980. Reprints: Steven Kossard, M.D., c/o Section of Publi- 71 Information Summaries cations, Mayo Clinic, 200 First St., S.W., Rochester, Minn. 55901. The authors describe the findings in eight patients who had multiple xanthomatous plaques and subcutaneous nodules that had a predilection for the periorbital area, flexures of the extremities, and the trunk. The lesions tended to ulcerate. Skin biopsy showed xanthogranuloma with necrobiosis. All patients had a dysproteinemia; in six patients it was a monoclonal IgG paraprotein. Bone marrow examination showed myeloma in two patients, atypical plasma cells in two and a Iymphoproliferative process in one. Episcleritis, uveitis, keratitis, ulceration, orbital cellulitis, and proptosis may occur in these patients. These threats to vision are not a feature of the more common normolipemic xanthomas and other necrobiotic granulomas. • • • Reference: Quimby, S.R., and Perry, H.O.: Livedo reticularis and cerebrovascular accidents. f. Am. Acad. Dermatol. 3: 377-383, 1980. Reprints: S.R. Quimby, M.D., c/o Section of Publications, Mayo Clinic, 200 First St., S.W., Rochester, Minn. 55901. Tivedo reticularis is a clinical pattern of cutaneous L vascular dysfunction that can be observed in connection with a large number of diseases. It has rarely been reported in association with cerebrovascular accidents. The authors relate the clinical and histopathologic findings in a 52-year-old female who had cerebrovascular disease and severe, generalized livedo reticularis. Skin biopsy revealed extensive vasculopathic changes which may hold a relationship with the cerebrovascular disorder. The authors suggest that this possible relationship bears further study.. . . Reference: Kapcala, L.P., Molitch, M.E., Post, K.D., et al.: Galactorrhea, oligo/amenorrhea, and hyperprolactinemia in patients with craniopharyngiomas. f. Clin. Endocrinol. Metab. 51: 798-808, 1980. Reprints: Mark E. Molitch, M.D., Box 268, New England Medical Center, 171 Harrison Ave., Boston, Mass. 02111. This report deals with three patients who had galactorrhea, oligo/amenorrhea, and abnormal tomograms of the sella suggestive of prolactinoma. One patient had an intrasellar craniopharyngioma and two had suprasellar craniopharyngiomas. Thus, hyperprolactinemia may be found in association with nonpituitary parasellar neoplasms. • • • Reference: Damasio, H., and Lyon, L.: Lithium carbonate in the treatment of cluster headaches. f. Neural. 224: 1-8, 1980. Reprints: Dr. Hannah Damasio, Dept. of Neurology, University of Iowa College of Medicine, Iowa City, Iowa 52242. Twenty-one patients with cluster headache participated in an open trial to assess the effectiveness of 72 lithium carbonate in the acute treatment and prevention of cluster headaches. In 11 patients there was absolute improvement, in five there was partial improve~ent, and five others had no improvement or unsustamed benefit. Improvement was obtained on doses lower than those used for the effective management of bipolar depression. It appears that lithium can be useful in the management of cluster headaches provided drug levels and renal function are carefully watched. No serious side effects were noted. Treatment was started with 600 mgi day of lithium carbonate and was increased by 300 mg weekly until the patient improved or side effects or toxicity occurred. Renal toxicity is the major concern with this medication. • • • Reference: Rowland, L.P.: Controversies about the treatment of myasthenia gravis. f. Neurol. Neurosurg. Psychiatry 43: 644-659, 1980. Reprints: L.P. Rowland, M.D., Neurological Institute, 710 W. 168th St., New York, N.Y. 10032. Those clinicians who treat patients with myasthenia gravis must choose from a variety of therapies which include cholinergic drugs, corticosteroids, immunosuppressive agents, and thymectomy. Plasmapheresis is another mode of treatment currently under intense scrutiny. The sequence and combination of different treatments must also be decided upon. Because controlled trials have not been done to determine the long-term benefits of these modes of treatment, controversy abounds. This author attempts to brin~ order to the subject of treatment with an excellent summary of the current state of knowledge concerning the treatment of myasthenia gravis. • • • Reference: Ottomo, M., and Heimburger, R.F.: Alternating Homer's syndrome and hyperhidrosis due to dural adhesions following cervical spinal cord injury.]. Neurosurg. 53: 97-100, 1980. Reprints: Michinori Ottomo, M.D., Dept. of Neurosurgery, Hirosaki University School of Medicine, 5 ZaifuCho, Hirosaki-Shi, Aomori-Ken, 036, Japan. AltematingHomer's signs and hyperhidrosis appeared .l"'\. In a patIent 8 years after a spinal cord injury at the C6-7 level. An intramedullary cyst was suspected but was not found at operation. There was striking improvement in both the symptoms and signs after adhesions between the spinal cord and the dura were freed. • • • Reference: Muhr, c., Bergstrom, K., Enoksson, P., et al.: Follow-up study with computerized tomography and clinical evaluation 5 to 10 years after surgery for pituitary adenoma. f. Neurosurg. 53: 144-148, 1980. Reprints: Carin Muhr, M.D., Dept. of Neurology, Uni-versity Hospital, 5-750 14, Uppsala, Sweden. Afull clinical, endocrinological, perimetric, and computerized tomographic examination was performed on 19 of 20 patients who had been operated upon previously for pituitary adenoma. Plain films of Journal of Clinical Neuro-ophthalmology .~ i the sella were obtained on all patients. The computerized tomograms showed evidence of tumor recurrence in four patients while perimetry disclosed evidence for recurrence in only one patient. Plain films of the sella were of no value in deciding whether recurrence had developed. Prolactin determination had limited value unless the levels were extremely high. It was concluded th.lt computerized tomography was the most valuable way to detect tumor recurrence. • • • Reference: Nemet, P., GodeL V., Branch, E., et a1.: Benign palsy of superior oblique muscle. ]. Pedidtr. Ophth<l/mol. Strab. 17: 320-322, 1<)80. Reprints: Pinhas Nemet, M.D., Dept. of Ophthalmology, Ichilov Hospital, Tel Aviv, Israel. Thirteen patients with nontraumatic superior oblique muscle palsy were examined during a 4- to 7-year period of follow-up. Extensive investigation disclosed no underlying systemic or neurologic disease. In all patients a rapid improvement occurred spontaneously in about 10 weeks, suggesting a benign prognosis in this condition. • • • Reference: Sternberg, I., Ronen, S., and Amon, N.: Recurrent, isolated postfebrile abducens nerve palsy. ]. Pediatr. Ophthalmol. Strab. 17: 323-324, 1980. Reprints: Ido Sternberg, M.D., Dept. of Ophthalmology, Shaare Zedek Hospital, Jerusalem, Israel. A patient is reported who had three episodes of recurrent sixth nerve palsy apparently related to febrile illness. All investigations were negative and the condition fully recovered within 3 months after each episode. • • • Reference: Unsold, R., Ostertag, Ch., and Newton, T.H.: Differential diagnosis of Graves' disease and orbital pseudotumor: CT findings. Klin. Mol. Augenheilk. 177: 31-47, 1980. Reprints: Dr. R. Unsold, Univ.-Augenklinik, Killian-strasse 5, 7800 Freiburg i Br. Computerized tomographic scans were taken of 25 typical cases of Graves' disease and 15 cases of orbital pseudotumor and an analysis revealed some valuable differential criteria. The CT findings do overlap and should be viewed as two opposite poles of a continuous spectrum with Graves' disease and pseudotumor having important differential features if specific changes can be identified. Massive swelling of ocular muscles, involving several muscles and usually without density changes in orbital fat, suggests Graves' disease. More or less circumscribed masses of soft tissue density surrounding the globe, ocular muscles, or the optic nerve or severe diffuse increase of density in the retrobulbar space masking orbital structures indicates orbital pseudotumor. • • • Reference: Rush, ].A.: Pseudotumor cerebri. Clinical pro- March 1981 McCrary file and visual outcome in 63 patients. Mayo C1in. Proc. 55: 541-546, 1980. Reprints: James A. Rush, M.D., Dept. of Ophthalmology, Neuro-ophthalmology Service, University of South Florida College of Medicine, Tampa, Fla. Few current reports in the ophthalmic literature address the visual prognosis in patients with pseudotumor cerebri. The visual status of 63 patients with pseudotumor cerebri seen at the Mayo Clinic from 1961 through 1978 was retrospectively analyzed. Visual loss was not statistically related to the presence of visual obscurations, durations of symptoms, degree of papilledema, or the number of recurrences. No patient with visual loss had substantial improvement regardless of the type of treatment. • • • Reference: Michel, E.M., and Troost, B.T.: Palinopsia: Cerebral localization with computed tomography. Neurology 30: 887-889, 1980. Reprints: B. Todd Troost, M.D., V.A. Medical Center (127). University Drive C. Pittsburgh, Pa. 15240. The authors describe three patients with bizarre visual symptoms which were initially misinterpreted by some as being of psychiatric or metabolic origin. Each patient had historical complaints typical of palinopsia. Each patient described repetitive visual images of previously viewed objects or scenes, not always confined to the area of the homonymous visual field defect. Each patient had a large occipital lobe lesion documented by computer tomography. The fact that the visual images experienced by the patient related to an object or scene recently viewed indicated that the visual perceptions were visual transpositions in time or space. • • • Reference: Savino, P., Glaser, J., and Schatz, N.J.: Traumatic chiasmal syndrome. Neurology 30: 963-970, 1980. Reprints: Peter J. Savino, M.D., Neuro-ophthalmology Unit, Wills Eye Hospital. 9th and Walnut Sts., Philadelphia, Pa. 19107. Eleven patients with the uncommon finding of traumatic chiasmal syndrome after closed-head trauma were studied. Visual field defects varied from complete monocular blindness and contralateral temporal hemianopsia to subtle bitemporal visual field defects. The degree of visual loss was not necessarily related to the severity of the trauma. Diabetes insipidus was present in half of the patients but, unlike the visual abnormalities, this complication was transient. • • • Reference: Stahl, S.M., Layzer, R.B., Aminoff, M.J., et a1.: Continuous cataplexy in a patient with a midbrain tumor: The limp man syndrome. Neurology 30: 11151118,1980. Reprints: Robert B. Layzer, M.D., Dept. of Neurology, School of Medicine, Univiersity of California, San Francisco, Calif. 94143. A patient with a glioblastoma of the rostral midbrain and hypothalamus exhibited internuclear ophthal- 73 Information Summaries moplegia and vertical nystagmus; he suffered from episodes of cataplexy, narcolepsy and sleep paralysis. A peculi.u fluctuation of posture and tone ("limp man syndrome") proved to be a feature of continuous cateplexy, as documented by H-reflex recordings. This is the second report of cataplexy associdted with d rostral midbrain tumor and the first report of this peculiar movement disorder. • • Reference: Young, H.A., Olin, M.s., and Schmidek, H.H.: Fractures of the sella turcica. Neurosurgery 7: 23-29, 1980. Reprints: Henry H. Young, M.D., Neurosurgical Service, Medical Center of Vermont, University of Vermont College of Medicine, Burlington, Vt. 05405. Five cases of sella turcica fracture are described by the authors. These fractures are often associated with frontal or maxillofacial trauma and are complicated by cranial nerve palsies, chiasmatic injury, and cerebrospinal fluid rhinorrhea and otorrhea. The mortality rate is high because of associated brain injury. A detailed endocrine evaluation is very important in these cases. Plain films and polytomes are the best x-ray studies for confirmation of sellar fracture. Computer tomography is unreliable as a diagnostic tool. Four-vessel angiography is essential in all cases to rule out significant vascular injury. High-dose steroids given early may mask adrenal insufficiency produced by ACTH reduction after trauma, thus the need for follow-up endocrine testing. Meningitis and direct brain damage often are the causes of death. • • Reference: Chapoy, P.R., Angelini, C, Brown, J., et al.: Systemic camitine deficiency-A treatable inherited lipid storage disease presenting as Reye's syndrome. N. Engl. ]. Med. 303: 1389-1394, 1980. Reprints: Paul R. Chapoy, M.D., Dept. of Pediatrics, UCLA Health Sciences Center, Los Angeles, Calif. 90024. This report details the observations made on a 3.5year- old male patient who presented with lethargy, somnolence, hypoglycemia, hepatomegaly, and cardiomegaly. Restoration of blood sugar levels to normal yielded no significant improvement, and it was noted that there was no ketonuria during subsequent attacks of hypoglycemia. The lack of ketone production prompted a search for a defect in fatty acid metabolism. Camitine deficiency was identified. Replacement camitine was beneficial. This treatable disorder is characterized by recurrent attacks and has features suggestive of Reye's syndrome. Camitine deficiency is the leading cause of Type [ lipid storage myopathy. • • Reference: Kinney, H., Faix, R., and Brazy, J.: The fetal alcohol syndrome and neuroblastoma. Pedidtrics 66: 130-132, 1980. Reprints: Jane Brazy, M.D., Box 3967, Dept. of Pediatrics, Duke University Medical Center, Durham, N.C 27710. 74 Apatient with adrenal carcinoma and fetal. alcohol syndrome was reported in 1977. The patient described by the authors had a neuroblastoma and fetal alcohol syndrome. The possible significance of a relationship between fetal alcohol syndrome and the potential development of neural crest neoplasms deserves further investigation in their opinion. A significant connection already exists between the fetal hydantoin syndrome and neuroblastoma. • • • Reference: Forbes, G.5., Sheedy, P.F., and Waller, R.R.: Orbital tumors evaluated by computed tomography. Rildiology 136: 101-111, 1980. Reprints: Glenn S. Forbes, M.D., Dept. of Radiology, Mayo Clinic, Rochester, Minn. 55901. The results obtained from computed tomographic studies taken on 90 orbital tumors were analyzed. Tumors were both benign and malignant and included hemangiomas, lacrimal gland tumors, lymphomas, carcinomas, metastatic tumors, pseudotumors, and neoplasms of the optic nerve. The important computerized tomographic features of a variety of orbital tumors are described. Computerized tomography was more useful than any other test in providing information on the type of tumor and was the best means for detection of tumor. • • Reference: Hayman, L.A., Evans, R.A., and Hinck, V.L Delayed high iodine dose contrast computed tomography: Cranial neoplasms. Rildiology 136: 677-684, 1980. Reprints: L. Anne Hayman, M.D., Dept. of Radiology, University of Texas Medical School, 6431 Fannin, Houston, Tex. 77030. A I-year prospective study was done with 78 patients who had suspected intracranial neoplasms. Immediate and delayed high-dose contrast postinfusion computerized tomographic scans were compared in 15 cases of primary and 32 cases of metastatic neoplasm. The 1hour delayed scan was of the same diagnostic value as the immediate scan in all patients. In 10 cases, additional lesions were demonstrated on the delayed studies. The results of this study suggest that delayed high-dose computerized tomography should be done instead of the usual immediate postinfusion scan in all patients suspected of harboring a neoplasm. • • • Reference: Daniels, D.L., Haughton, V.M., Williams, A.L, et al.: Computed tomography of the optic chiasm. Rddiology 137: 123-127, 1980. Reprints: David L Daniels, M.D., Dept. of Radiology, Milwaukee County Medical Complex, 8700 West Wisconsin Ave., Milwaukee, Wis. 53226. Axial and coronal computerized tomographic scans n were done on a cadaver, 41 normal people, and six patients with symptoms consistent with chiasmal glioma. The size and shape of the normal chiasm was determined. It typically appears V-shaped in axial and coronal Journal of Clinical Neuro-ophthalmology studies. The chiasm was globular and larger in all the patients with chiasmaI gliomas. An abnormal chiasm could be accurately differentiated from a normal chiasm by computed tomography. Optimal views of the chiasmal area are obtained if sections are 0.15-0.5 cm thick and if they are taken nearly parallel and perpendicular to the Reid base line. Coronal sections are best for March 1981 McCrary visualizing suprasellar tumors extrinsic to the chiasm. Contrast enhancement is advised. The optic tracts can be seen best if the plane of tomographic section is 0 to +30 degrees to the Reid base line. The optic nerve, on the other hand, is best seen in -10 degree sections. • • • 75 |
