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Show f. Clin. Neuro-ophtha/mo/. 1: 237-246, 1981. Concise Summaries 1. Corectopia appears to be part of a spectrum of genetically related diseases of the iris. 2. If visual acuity were tested better, we might be able to diagnose early foveal receptor disease sooner. There is a better way to test vision! 3. If you find this enzyme, you can make a diagnosis of sarcoidosis more accurately, especially if the clinical diagnosis is in doubt. 4. The flAX text is excellent for the detection of antibodies to herpes simplex or cytomegalovirus. 5. The lack of movement in a myasthenic fetus may be the cause for the later development of arthrogryposis multiplex, according to these authors. 6. The CNS manifestations of systemic lupus erythematosus are the result of antigen-antibody reactions in vessel walls that allow immune complexes to pass the blood-brain barrier. 7. A patient with a Marfans-like body build and prominent corneal nerves has a syndrome With a high incidence of neoplasia. 8. The Churg-Strauss syndrome should not be confused with the usual case of polyarteritis nodosa. It has significant ocular complications in some patients. 9. Cribside neurosonography of the head is a better diagnostic test than is routine CT in neonates with possible hydrocephalus. 10. The stalk sign may be an important CT diagnosis in empty sella synrome. 11. Subdural hematomas are often isodense in anemic patients. This presents a serious diagnostic problem if the hemoglobin is less than 10.0 g/dl. . 12. There is now an animal model avaIlable for the study of pituitary tumor. 13. Skin and subconjunctival biopsies are very effective in establishing a diagnosis in progressive neurologic disease in childhood if the usual biochemical studies fail to do so. 14. Inverse ocular bobbing may be an important sign of metabolic encephalopathy. . 15. Forced upgaze may be an important sIgn of hypoxic encephalopathy. ., 16. Metrizamide is very useful in the confIrmation From the Cullen Eye Institute, Houston, Texas. September 1981 Information Summaries JOHN A. McCRARY, III, M.D. of the empty sella syndrome, if the diagnosis is in question. 17. Meig's syndrome is blepharospasm plus? 18. The value of exploration in a case of progressive severe visual loss with negative neuroradiologic features is verified again. 19. Progressive cerebellar degeneration in Hodgkin's disease is perhaps more common than we thought. 20. Be sure to look at the skin in any patient with uveitis, keratitis, or the other ocular signs of mycosis fungoides described in this paper. 21. Make certain that every patient with ischemic optic neuropathy has a sedimentation rate on the chart. 22. Anderson and Khalil describe the various mistakes that can befall an ophthalmologist confronted by a patient harboring a meningioma. 23. Can ophthalmoplegic migraine occur without headaches? 24. Cimetidine can precipitate a neurologic condition that looks just like alcohol withdrawal. 25. lhermitte's sign may be the clue to vitamin BIZ deficiency. 26. Carotid bruit may be more of a general, rather than specific indicator of stroke risk and potential distribution. 27. Be careful with timolol in any patient with a history of asthmatic bronchitis or other chronic obstructive pulmonary disease. 28. Be sure you follow your patient carefully if you diagnose Tolosa-Hunt syndrome. A neoplasm may still be present. 29. Cerebral angiography may be hazardous to visual function in a patient with marginal renal function. 30. Be sure all patients with transient ischemic attacks have a 24-hour cardiac monitor unless there is specific evidence of obstructive arterial disease to account for the symptoms. 31. Neuron-specific enolase may be a very important new enzymatic way to facilitate the diagnosis of neuroendocrine tumors. 32. Major tranquilizers may predispose to the hyperthermic crisis found in the neurolepti~ malignant syndrome. Death occurs qUIckly If the correct diagnosis is not made. 33. An endarterectomv of the vertebral artery may be feasible treatm~nt for vertebrobasilar transient ischemic attacks, if the patient is totally refractory to all medical treatment. 237 Information Summaries 34. Sekhar and Heros present an excellent review of the current thought on a saccular aneurysm. 35. Yamamoto, Rhoton, and Peace detail the anatomy of the region of the third ventricle, for those with a specific interest in such matters. 36. A spinal tap may lead to very serious complications if the patient is on anticoagulants. 37. Ultrasonically guided percutaneous cephalocentesis may be the safest way to deal with fetal hydrocephalus in advance of a vaginal delivery. 38. Progressive neurologic disease in a child with chronic liver disease may be the result of chronic vitamin E deficiency. 39. Wiebers, Whisnant, and O'Fallon discovered the chance of a bleed from an aneurysm is very low if the diameter is less than 10 mm. 40. Dr. Ojemann comments on aneurysm management. 41. Retinal artery obstruction in children and young adults is most often caused by migraine or coagulation anomalies. 42. Tamoxifen may be the cause for crystalline retinal deposits. 43. Red-green glasses and the duochrome test are very useful in measuring the visual acuity in patients with latent nystagmus. 44. Tuberculosis antigen-antibody interaction may be important as cause for Takayasu's arteritis. 45. This review of optic atrophy by Dr. Wirtschafter is excellent. 46. The sector-CT scan method permits incredible visualization of the anatomic details in the orbit. 47. The best neuroradiologic method for evaluating glioma of the anterior visual pathways in children is the CT. In small lesions, however, the pneumoencephalogram is still better. 48. The nearly occluded internal carotid artery may be a diagnostic trap. 49. Pituitary microadenomas can't be well localized with sella polytomes, according to these authors. 50. Primary cerebral neuroblastoma must now be added to the best of causes for calcified, cystic lesions of the brain in young patients. 1. Reference: Mondino, B.J., and Cohn, H.C.: Corectopia with nystagmus, absent foveal reflexes and corneal changes. Acta. Ophthalmol. 59: 85-93, 1981. Reprints: Bartly J. Mondino, M.D., Dept. of Ophthalmology, 230 Lothrop St., Eye and Ear Hospital, Pittsburgh, Pa. 15213. The authors describe a family whose members have corectopia, nystagmus, absent foveal reflexes, and ((lrnp~1 <1bn(lrm~litil's inherited in an autosomal dominant p.lttern Corneal changes included superficial opac-ities and peripheral vascularization, in younger patients. Stromal thinning was present in the 60-year-old proband. Microcornea, Bitot's spots, and iris colobomas were also features of the anterior segment examination. Stromal corneal opacities have been described in patients with familial and sporadic aniridia. The authors suggest, as have others, that corectopia is part of a spectrum of genetically related diseases of the iris including aniridia, typical and atypical colobomas, and intermediate variations of iris stromal damage. • • • 2. Reference: Frisen L., and Firsen, M.: How good is normal visual acuity? Albrecht Von Graefes Arch. KJin. Exp. Ophthalmol. 2~~: 149-157, 1981. Reprints: Dr. Lars Frisen, Ogonkliniken, Sahlgrenska sjukhuset, S-413 45 Goteberg, Sweden. Visual acuity levels were studied in 100 normal subjects of different ages, using finely graduated letter chart size under carefully optimized conditions. Each line on the chart contained 10 letters of similar difficulty arranged in a random order. Statistical analysis showed the determination of visual acuity limits is better if one accepts 50% correct responses rather than demanding 100% correct responses. Regression analysis showed that depending on age the visual acuity rises toward a peak at 25 years, then slowly declines. The most marked 1055 occurs after age 60. Minor modifications of test strategy are suggested. To accept 20/20 rather than 20/15 as the best visual acuity would appear to be in error and may indeed cause a delay in diagnosis of foveal receptor disease or disconnection of their cortical afferents. • • • 3. Reference: Pertshuk, L.P., Silverstein, E., and Friedland, J.: Immunohistologic diagnosis of sarcoidosis. Detection of Angiotensin-converting enzyme in sarcoid granulomas. Am. }. Clin. Pathol. 75: 350-354, 1981. Reprints: Dr. Louis P. Pertschuk, Box 25, 450 Clarkson Ave., Brooklyn, N.Y. 11203. Angiotensin-converting enzyme is a glycoprotein that .n. catalyzes the conversion of angiotensin I to angiotensin II. Tissue angiotensin-converting enzyme levels are usually elevated in lymph nodes taken from individuals with sarcoidosis. In this study the enzyme was detected by immunofluorescence technique. Angiotenson- converting enzyme was present in granulomas taken from 38 of 39 patients with a clinical diagnosis of sarcoidosis. It was present in none of 37 nonsarcoid granulomas, normal lymph nodes or splenic controls. Tissue immunofluorescence is reduced in proportion to the degree of fibrosis in the granuloma. Detection of angiotensin- converting enzyme appears to be pivotal in helping to establish a diagnosis of sarcoidosis. • • • Journal of Clinical Neuro-ophthalmology 4. Reference: Segar, J.E., Smith, T.F., and IIstrup, D.M.: Evaluation of the flAX test for the detection of antibodies to herpes simplex and cytomegalovirus. Am. I. Clin. Pathol. 75: 387-390, 198\. Reprints: Thomas F. Smith, PhD., M,lyO Clinic, Rochester, Minn. 55901. The flAX test is a fluorescent immunoassay test moHketed by International Diagnostic Technology of Santa Clara, California. In this study it was compared to the Laboratory Branch Complement Fixation test .JS .J determinant of antibodies in human serum to both herpes simplex and cytomegalovirus. The flAX test was 98.0% specific for herpes simplex and 93.8% sensitive. In the evaluation for .Jntibodies to cytomegalovirus the FlAX test was 90.2% specific .md 96.1% sensitive. flAX titers incre.lsed along with complement fixation titers in 93% with herpes simplex and 94% with cytomegalovirus. The flAX test appears to be a rapid, specific, sensitive, and reproducible test for herpes simplex and cytomegalovirus antigens. • • • 5. Reference: Donaldson J.O., Penn, A.S., Lisak, R.P., et .11: AntiacetylchoIine receptor antibody in neonatal myasthenia gravis. Am. I. Dis. Child. 135: 220-226, 1981. Reprints: James O. Donaldson, MD., Dept. of Neurology, University of Connecticut Health Center, Farmington, Ct. 06032. Maternal titers of antiacetylcholine receptor antibody were higher in two mothers with myasthenia gravis who had infants with neonatal myasthenia gravis than was found in two mothers whose children were unaffected. In neonates there appears to be a correlation between the titer of antiacetycholine receptor antibody and the presence and severity of neonatal myasthenia gravis. Very high levels may cause limitation of movement to develop in the fetus, thereby setting the stage for arthrogryposis multiplex. Some neonates may be normal or mildly affected at birth and yet develop myasthenic signs and symptoms later. This may be due to the declining concentration of the high fetal levels of alpha-fetoprotein that occurs postpartum. • • • 6. Reference: Bluestein, H.G., Williams, G.W., and Steinberg, A.D.: Cerebrospinal fluid antibodies to neuronal cells: Association with neuropsychiatric manifestations of systemic lupus erythematosus. Am. I. Med. 70: 240-246, 1981. Reprints: Harry G. Bluestein, MD., University of California Medical Center, 225 Dickinson St., San Diego, Calif. 92103. The hypothesis that some of the neuropsychiatric 1 manifestations of systemic lupus erythematosus are mediated by the direct effects of antibody binding to removal of cell membranes was tested using a radiolabled staphylococcal protein A assay. CSF was tested in 27 patients with SLE and CNS complications and 18 with SLE with SLE without CNS manifestations. The test results indicate that the diffuse CNS manifestations of SLE are indeed the direct result of the interaction of September 1981 McCrary serum antineuronal antibodies with neural cell membranes. The authors suggest that immune complex-mediated changes of vascular integrity could allow transport of immune complexes across the blood-brain barrier. • • • 7. Reference: Spector, B., Klintworth, G.K., and Wells, S.A.: Histologic study of the ocular lesions in multiple endocrine neoplasia syndrome type lIb. Am. ]. Ophth.J/mo/. 91: 204-215, 198\. Reprints: Gordon K. Klintworth, MD., Dept. of Pathology, Duke University Medical Center, Durham, N.C. 27710. Multiple neoplasms of endocrine origin are currently classified in three syndromes. Type lib multiple endocrine neoplasia is characterized by medullary thyroid carcinoma, mucosal neuromas, pheochromocytoma, a Marfan's type body habitus, large lips and other typical facial features, intestinal ganglioneuromas, skeletal abnormality, abnormal intradermal histamine response, and prominent corneal nerves. Thickened eyelids, nasal displacement of the lacrimal puncta. conjunctival thickening, decreased tear formation, and poor pupillary dilation are a few of the other associated ocular findings seen in this condition. • • • 8. Reference: Meisler, D.M., Stock, E.L., Wertz, RD., et .11.: Conjunctival inflammation and amyloidosis in allergic granulomatosis and angiitis (Churg-Strauss syndrome). Am. ]. Ophtha/mo/. 91: 216-219, 1981. Reprints: E. Lee Stock, MD., Dept. of Ophthalmology. Northwestern University Medical School, 303 E. Chicago Ave., Chicago, Ill. 60611. The Churg-Strauss syndrome is a severe, multisystem allergic granulomatosis and angiitis characterized clinically by fever, bronchial asthma, and eosinophilia. Cardiac failure and peripheral neuropathy are commonly present. The syndrome is often confused with polyarteritis nodosa but can be distinguished from it by clinical and histological methods. The patient reported here had conjunctival infiltrates composed of eosinophiles, plasma cells, lymphocytes. and histiocytes. Uveoscleritis, papilledema, and corneal infiltr.ltes have been reported with this condition. ·.. 9. Reference: Edwards, M.K, Brown. D.L.. Muller. ].. et .11.: Cribside neurosonography: Real-time sonography for intracranial investigation of the neonate. Am.]. Roentgenol. 136: 271-270, 1981. Reprints: Dr. M.K. Edwards, Dept. of Pathology, Methodist Hospital Graduate Center, 1604 N. Capital Ave., Inidanapolis, Ind. 47206. A prospective study was done on 56 neonates using real-time sonographic sector scans of the cranial vault. The anterior fontanelle was used as the acoustic window. A CT scan was available for comparison in 17 cases. In no case did the CT scan or sonogram results disagree as to the size of the lateral ventricles. Abnormalities detected by ultrasound included enlarged ven- 239 Information Summaries tricles, intracerebrdl hemdtomds, congenitdl glioma, dnd severdl cysts. Sonographic sector SCdns produce excellent, detailed imdges of dildted Idt£,[dl and third ventricles, use no ionizing radi.ltion, dre less expensive, dnd c.1n be obt,lined in the isolette, thereby reducing the risk of hypoxi.l .md hypothermi.l. Sonogrolphy molY well replolce CT SCdns for the initioll medsurement of ventriculolr size. CT is complementdry in the eVdludtion of the posterior fOSSd, intracrdnioll hemorrhdges, dnd other molSS lesions. • • • 10. Reference: Haughton, Y.M., Rosenbdum, A.E., WilIidms, A.lo, et 011.: Recognizing the empty sella by CT: The infundibulum sign. Am. f. Roentgenol. 136: 293295, 1981. Reprints: Aloin lo Williolms, M.D., Dept. of Roldiology, Milwaukee County Medical Complex, 870 W. Wisconsin Ave., Milwdukee, Wis. 53226. The incorrect diagnosis of the empty sella syndrome by conventional CT methods derives from the numerous intrasella cystic abnormalities are not distinguished from it. In 23 Cdses of presumed empty sella, the pituitdry stalk could be seen within the enlarged sella when thin coronal CT sections were done. Although the diagnosis was not proven by conventional studies, the authors believe that the normal appearance of the infundibulum within the sella excludes the diagnosis of intrasellar cyst. • • • 11. Reference: Smith, W.P., Batinsky, 5., and Rengachary S.5.: Acute isodense subdural hematomas: A problem in anemic patients. Am. f. RoentgenoJ. 136: 543-546, 1981. Reprints: Dr. S. Batinzky, Dept. of Diagnostic Radiology, University of Kansas Medical Center, Kansas City, Ks. 66103. Acute CT isodense subdural hematomas may present .n serious diagnostic problems in persons who Me anemic. This proved to be the case in two patients with blood hemoglobin concentrations of 9.2 and 10.0 gldl. The authors experimentally determined that a blood hemoglobin level between 8 dnd 10 gldl will produce an isodense subdural collection. The CT SCdn may show mass effect, but nothing else, and angiography is needed to make a specific diagnosis. The usual isodense stage for the typiCdlly encountered chronic subdural hemdtoma is 2-6 weeks but may range from 1 week to 3 months. • • • 12. Reference: Schechter, j.E., Felicio, loS., Nelson, j.F., et al.: Pituitary tumorigenesis in aging female C57BLI 6J mice: A light and electron microscopic study. Anat. Rec. 199: 423-432, 1981. Reprints: Dr. joel E. Schechter, Dept. of Anatomy, University of Southern California School of Medicine, The Andrus Gerontology Center, Los Angeles, Calif. 90033. Spontaneous pituitary tumors occur frequently in ..... ( s711l .It'! frm.Jle mice. Tumors .Jre present in over 240 50% of mice 22 months of age or older. The predominant cellular abnormality is in mammotroph cells, though somatotrophs and gonadotrophs appear active and hypertrophied. Metastasis of tumor cells into vascular lakes is common. • • • 13. Reference: Arsenio-Nunes, M.L., Goutieres, F., and Aicardi, J.: An ultramicroscopic study of skin and conjunctival biopsies in chronic neurological disorders of childhood Ann. Neurol. 9: 163-173, 1981. Reprints: Dr. J. Aicardi, INSERM, U-12, Hopital des Enfants-Malades, 149 rue de Sevres, 75730 Paris Cedex IS, France. Skin and conjunctival biopsies were obtained in a group of pediatric patients with progressive neurologic disease. Ultrastructural abnormalities were found in 48 of 72 patients with unequivocally progressive encephalopathies (67 of which could not be diagnosed by biochemical means) but in one of 55 with static or questionably progressive disease. No discrepancies were observed between the analytic results of skin or conjunctival biopsies. Although biochemical testing is the simplest and most effective technique available for diagnosis of progressive encephalopathies, skin or conjunctival biopsy is a valuable procedure in those without detectable enzymatic deficiency. • • • 14. Reference: Knobler, R.lo, Somasundaram, M., and Schutta, H.5.: Inverse ocular bobbing. Ann. Neurol. 9: 194-197, 1981. Reprints: R.L. Knobler, M.D., Dept. of Immunopathology, Scripps Clinic and Research Foundation, 10666 Torrey Pines, Rd., LaJolla, Calif. 90237. Inverse ocular bobbing is a term coined by the authors to describe a slow downward drift of the eyes below the midline followed, after a delay, by a rapid return to the primary position. They describe this sign in two patients who were recovering from status epilepticus. It is suggested that the sign may be a post-ictal phenomenon or may be related to metabolic encephalopathy since the patients had the syndrome of inappropriate antidiuretic hormone secretion and hyperosmolar state, respectively. The authors review the list of ocular movement abnormalities related to inverse ocular bobbing. • • • 15. Reference: Keane, ).R.: Sustained upgaze in coma. Ann. Neural. 9: 409-412, 1981. Reprints: james R. Keane, M.D., 1200 N. State St., Los Angeles, Calif. 90033. Coma with sustained upward gaze deviation followed cardiac arrest in IS patients and prolonged systemic hypotension in two patients. Neuropathological examination confirmed diffuse cerebral and cerebellar damage with relative sparing of the brain stem. No focal lesions were found in the upper midbrain or pretectum. Six patients developed slow, downward-beating nystagmus as forced upward gaze resolved. Journal of Clinical Neuro-ophthalmology Forced upgaze in coma usually is the result of severe hypoxic encephalopathy. Anatomic localization is ob· scure. It may be the result damage to cerebellar p.lth. ways. • • • 16. Reference: lull, D.N., and Falko, J.M.: Metrizamide cistemography in the investigation of the empty sella. Arch. Intern. Med. 141: 487-489, 1981. Reprints: James M. Falko, M.D., Dept. of Endocrinokl~W, Room N-llll, Ohio State University Hospitals, 410 W. Tenth Ave., Columbus, Ohio 43210. Most physicians are reluctant to recommend a pneumoencephalogram to confirm a diagnosis of empty sella syndrome. Metrizamide is a nondissociable, water-soluble glucose amide containing three iodine molecules. This agent is miscible with CSF. It has minimal complications when used in low concentrations in conjuction with CT scanning. Presently metrizamide-CT cistemography appears to be a satisfactory alternative to pneumoencephalography in the diagnosis of empty sella syndrome. • • • 17. Reference: Tolosa, E.: Clinical features of Meige's disease (idiopathic orofacial dystonia). Arch. Neural. 38: 147-151, 1981. Reprints: Eduardo S. Tolosa, M.D., Box 295, University of Minnesota Hospitals, Minneapolis, Minn. 55455. This report deals with 17 patients who manifested 1 idiopathic orofacial dystonia. These patients often have a family history of dystonia or other extrapyramidal disorders, a high incidence of depression, and often have muscles involved beyond the orofacial zone. The course varies from rapid progression to maximal disability to clear evidence of improvement after years of disability. Besides muscle spasms, there were other neurologic abnormalities suggesting extrapyramidal dysfunction. • • • 18. Reference:Spoor, T.e., Kennerdell, J.5., lorub D., et 011.: Progressive visual loss due to glioblastoma. Normal neuroroentgenographic studies. Arch. Neural. 38: 196-197, 1981. Reprints: Thomas e. Spoor, M.D., Division of Ophthalmology, University of South Carolina School of Medicine, 3321 Medical Park Dr., Colombia, S.e. 29203. A60-year-old woman had rapidly progressive visual loss in both eyes over a 6-week period. All neuroradiologic studies on the patient were considered normal. She was neurosurgically explored. The optic nerves and chiasm looked normal except for some slight flattening and widening of the left optic nerve thought to be related to a distended left carotid artery. A biopsy specimen was taken because of the profound visual loss. The histopathologic diagnosis was glioblastoma of the anterior visual pathways. The patient died 5 months later. • • • September 1981 McCrary 19. Reference: Brazis, P. W., Biller, J., Fine, M., et 011.: Cerebellar degeneration with Hodgkin's disease. Computed tomographic correlation and literature review. Arch. Neural. 38: 253-256, 1981. Reprints: Paul W. Brazis, M.D., 2160 S. First Ave., Maywood, III. 60153. A62-year-old male patient developed rather rapid progressive deterioration of cerebellar and corticospinal function as well as dementia. Serial CT scans disclosed progressive cerebellar atrophy. Ultimately the diagnosis of Hodgkin's disease was established. Nine previous reports of similar complications of Hodgkin's disease are reviewed. Any patient with unexplained cerebellar degeneration should have a thorough search for lymphadenopathy. • • • 20. Reference: Stenson,S., and Ramsay, D.L.: Ocular findings in mycosis fungoides. Arch. Ophthalmol. 99: 272-277, 1981. Reprints: Susan Stenson, M.D., 333 E. 34th St., Suite IE, New York, N.Y. 10016. Thirty consecutive patients with mycosis fungoides were examined and speCIfiC ocular abnormalItIes related directly to mycosis fungoides were found in 11. Lid tumors were the most common finding. Keratitis, uveitis, and optic atrophy were also disclosed. The ophthalmologic findings described in isolated case reports seemed to correlate well with the findings in this series of patients. Mycosis fungoides is a malignant lymphomatous disease that arises in the skin. It frequently becomes metastatic to liver, lungs, spleen, lymph nodes, gastrointestinal tract, and the central nervous system. Ocular findings are much more common than previously believed. • • • 21. Reference: Graham, E., Holland, A., Avery, A., et al.: Prognosis in giant-cell arteritis. Br. Med. ]. 282: 269-271, 1981. Reprints: Dr. R.W. Ross Russell, Dept. of Neurology, St. Thomas's Hospital, london SEI 7EH, England. Ninety patients with a proven diagnosis of giant-cell arteritis were followed up. All patients had been diagnosed between 1968 and 1978 and all had the diagnosis confirmed by temporal artery biopsy. Early mortality from the disease was low and most often the result of brain-stem inf.Hction due to vertebral arteritis. late deaths were more often due to myocardial infarction. The patients had a poorer prognosis if high-dose maintenance steroid treatment was required or if visual 1055 occurred. Sixty percent of the patients had ischemic optic neuritis, while only 9% had central retinal artery occlusion. Unilateral or bilateral VI nerve palsy occurred in 9%. Palsy of nerves III and IV occurred in 11%. The pupil was spared in all cranial nerve III palsies. Females tended to fare poorer than male patients. Onethird of patients developed a chronic, relapsing form of 241 Information Summaries the disease but serious late complications were not encountered. • • • 22. Reference: Anderson, D. and Khalil, M.K.: Meningioma and the ophthalmologist: Diagnostic pitfalls. elIl. I. Ophth.l/mol. 16: 10-15, 198!. Reprints: Dr. Duncan Anderson, nm. 4813, Livingston H.11I, Montre.ll General Hospital, 1650 Cedar Ave., M,mtre,ll, PQ H3G IA4, Canada. This report deals with seven patients who had intracranial meningioma and whose opthalmologic presenting signs were misinterpreted, leading to initial misdiagnosis. Three patients had compression of the anterior visual pathways misdiagnosed as optic neuritis, chr,mic optic neuritis, and glaucoma. Others were misdiagnosed as frontal osteoma and migraine. The ophthalmologist must exercise much care in the interpretation of presenting signs and symptoms of his patients in order to avoid such mistakes. · . . 23. Reference: Durkan, G.P., Troost, B.T, Siamovits, TL., et .11.: Recurrent painless oculomotor palsy in children. A variant of ophthalmoplegic migraine? Headache 21: 58-62, 1981. Reprints: B. Todd Troost, MD., V. A. Medical Center (127), 10701 E. Blvd., Cleveland, Ohio 44106. This report concerns two children who developed recurrent, isolated, painless oculomotor palsy with negative neuroradiologic studies. Single attacks were observed from onset to resolution in both children. A diagnosis of painless ophthalmoplegic migraine was suspected. Because permanent deficits of ocular motility may follow repeated attacks of ophthalmoplegic migraine, one child was placed on propranolol. She went on to develop periodic headaches associated with nausea, despite treatment. The pupil function was impaired in both cases. The authors recommend that angiography be deferred in a young child with headache and an oculomotor nerve palsy who is otherwise well. • • • 24. Reference: Weddington, W.W., Muelling, A.E., Moosa, A.H., et .11.: Cimetidine toxic reactions masquerading as delirium tremens. rAM.A 245: 10581059, 198!. Reprints: William W. Weddington, MD., Dept. of Psychiatry, University of Chicago Medical Center, 950 E. 59th. St., Chicago, III. 60637. Cimetidine inhibits gastric acid secretion and thus is usually prescribed as treatment for peptic ulcer and to prevent gastrointestinal bleeding in predisposed patif'n! s. A numhf'r of complications related to the use of till', "".I" .If''"1 .Jr" dps,'..r,ihed. ',T.,hl',i."'.,l.tient reported here had signs and symptOtl'ls ~of,' lirium tremens but .·"C,IO',. worse when treated lO";.b;Ei,'(I! withdrawal with 242 benzodiazepine. Cimetidine slows benzodiazepine clearance. • • • 25. Reference: Butler, W.M., Taylor, H.G., and Diehl, L.F.: Lhermitte's sign in cobalamin (vitamin B-12) deficiency. rAM.A 245: 1059, 1981. Reprints: William M. Butler, MD., Dermatology-Oncology Service, Walter Reed Army Medical Center, Washington, D.C. 20012. Lhermitte's sign (electric shock dysesthesias produced by neck flexion) is often sought for With reference to the diagnosis of multiple sclerosis, tumors of the cervical cord, arachnoiditis, myelitis, and subacute combined degeneration. This paper describes two patients with signs of early subacute combined degeneration and Lhermitte's sign. Treatment with cyanocobalamin caused the Lhermitte's sign to disappear. • • • 26. Reference: Wolf, P.A., Kannel, W.B., Sorlie, P., et .11.: Asymptomatic carotid bruit and risk of stroke. The Flamingham study. rAM.A 245: 1442-1445, 1981. Reprints: Philip A. Wolf, MD., Dept. of Neurology, Boston University School of Medicine, 80 E. Concord St., Boston, Mass. 02118. Carotid bruit was found over an 8-year period in 66 men and 105 women in the Framingham Cohort, all of whom were asymptomatic. The incidence increased with age. The stroke rate was twice that of the normal population of the same age and sex. More often than not the stroke occurred in a vascular supply unrelated to that of the bruit. The incidence of myocardial infarction was also doubled in those with asymptomatic bruits. General mortality was nearly doubled in this group. Carotid bruit is a clear indicator of increased stroke risk but is a general sign of increased risk, mainly as a sign of advanced atherosclerotic disease, but not necessarily an indicator that stroke will occur in the distribution of the artery with the bruit. • • • 27. Reference: Schoene, R. 6., Martin, T R., Charan, N. 6., et .11.: Timolol-induced bronchospasm in asthmatic bronchitis. rAM.A 245: 1460-1461, 1981. Reprints: Robert B. Schoene, MD., Division of Respir, ltory Diseases, University of Washington School of Medicine, RM-12, Seattle, Wash. 98195, Five patients with chronic obstructive pulmonary disease were treated for glaucoma with TimoloL Further deterioration of pulmonary function was discovered when the patients received this topical ocular beta-adrenergic blocking agent. No such decline in function was found after saline was instilled in the conjunctival culde- sacs. This medication must be used carefully if the patient has a history of chronic obstructive pulmonary disease. • • • Journal of Clinical Neuro-ophthalmology 28. Reference; Kline, L.B., and Galbraith, I.G.: Parasellar epidermoid tumor presenting as painful ophthalmoplegia.]. Neurosurg. 54: 113-117, 1981. Reprints: Lanning B. Kline, M.D., Eye Foundation Hospital, 1720 Eighth Ave. South, Birmingham, Ala. 35233. A51-year-old man had a transient right sixth nerve palsy in 1904. In lQ78. he developed painful ophthalmoplegia on the right with paresis of the right oculomotor and trigeminal nerves. Complete neuroradiologic studies were judged to be normal. He was treated with high-dose steroids as the working diagnosis was Tolosa-Hunt syndrome. He experienced temporary improvement on this treatment. In 1979, another CT scan was obtained which revealed a low-density lesion adjacent to the right cavernous sinus. Neurosurgical exploration and biopsy lead to a diagnosis of parasellar epidermoid tumor. • • 29. Reference: Studdard, W.E., Davis, D.O., and Young, S.W.: Cortical blindness after cerebellar angiography. Case report. ]. Neurosurg. 54: 240-244, 1981. Reprints: David O. Davis, M.D., Dept. of Radiology, The George Washington University Medical Center, 901 23rd St., N.W., Washington, D.C 22037. Cortical blindness developed as a complication of cerebral angiography in a 59-year-old female patient. She was neurologically intact at the time of the study but had a history of hypertensive cardiovascular disease, angina, and a right carotid bruit. She also had chronic renal disease. A left nephrectomy had been done in 1972. Serial cr scans revealed persistence of contrast medium in the occipital lobes. This case supports the concept that cortical blindness may be secondary to direct effects of contrast medium on the brain. Persistence of contrast may have related to poor renal function. • • • 30. Reference: DeBono, D.P., and Warlow, CP.: Potential sources of emboli in patients with presumed transient cerebral or retinal ischemia. Lancet 1: 333335, 1981. Reprints: Dr. C P. Warlow, Dept. of Clinical Neurology, University of Oxford, Radcliffe Infirmary, Oxford, 0X2 6HE, England. Potential sources of emboli were sought by non-invasive techniques and, where indicated, by anglographic study in 117 consecutive patients referred with symptoms of transient cerebral or retinal ischemia. Thirty-two percent of the patients had structural cardiac anomalies, 32% had arrhythmias on 24-hour ECG monitoring, 15% had evidence of ischemic heart disease, and 34% were or had been hypertensive. The proportionate similar findings in 104 age and sex controls were 14%, 28%, 12%, and 10%, respectively. Thirty-seven percent who had carotid angiography had significant lesions. Some had both cardiac and carotid lesions. Twenty-five September 1981 McCrary percent had no detectable evidence of any cardiovascular disease. • • • 31. Reference:Tapia, F.I .. Polak, I.M., Barbosa, A.j.A., et al.: Neuron-specific enolase is produced by neuroendocrine tumors. Lancet I: 808-81 I, ]981. Reprints: Dr. j.M. Polak, Histochemistry Unit, Dept. of Histopathology, Royal Postgraduate Medical School, Hammersmith Hospital, DuCane Road, London W]2 OHS, England. Neuron-specific enolase was first found in extracts of brain tissue and was later detected in amine precursor uptake and decarboxylantion cells and neurons of the diffuse neuroendocrine system, but not in other peripheral cells. The antisera to this enzyme react strongly to neuroendocrine neoplasias of all types including pheochromocytoma, carcinoid, and medullary thyroid carcinoma. Neuron-specifiC enolase has been detected in high concentration in human pineal gland and in the monkey pineal and the anterior and posterior lobes of the pituitary gland, according to the reference of Schmechel, Marangos, and Brightman cited in the references of this paper. • • • 32. Reference: Henderson, V. W., and Wooten. CF.: Neuroleptic malignant syndrome: A pathogenetic role for dopamine receptor blockade? Neurology (NY) 31: 132-137, 1981. Reprints: Victor W. Henderson, M.D., Aphasia/Neurobehavioral Unit, Veterans Administration Hospital. 150 South Huntington Ave.. Boston, Mass. 02130. The neuroleptic malignant syndrome is characterized by the onset of extrapyramidal signs and hyperthermia as a complication of treatment with major tranquilizers. Additional manifestations may include pallor, diaphoresis, blood pressure fluctuation, tachycardia. and tachypneic hypoventilation. Respirator support may be required. Death often supervenes but this may be averted with prompt recognition and treatment, The manifestations are attributed to dopamine receptor blockade in the striatum, leading to increased he.!t production, and in the hypothalamus, causing imp.!ired he.!t loss. • • • 33. Reference: Allen. CS., Cohen, R.I., and Preziosi. T./.: Microsurgical endarterectomy of the intracranial vertebral artery for vertebrobasilar transient ischemic attacks. Neurosurgery 8: 56-59, 1981. Reprints: George S. Allen, M.D., Dept. of Neurosurgery, Blalock 1415, The johns Hopkins Hospital, 601 North Broadway. Baltimore, Md. 2]205. This report describes two patients who had typical vertebrobasilar transient ischemic attacks that were refractory to conventional medical treatment. Angiography in both revealed stenotic lesions of the intracranial vertebral artery between the posterior and anterior in- 243 Information Summaries ferior cerebellar arteries. Both patients were operated upon. One patient had the plaque removed while the other did not, as it extended into the basilar artery. The patient whose plaque was removed did well while the other continued to have transient ischemic attacks and eventually had a brain-stem stroke. • • • 34. Reference: Sekhar, L.N., and Heros, R.C.: Origin, growth, and rupture of saccular aneurysms: A review. Neurosurgery 8: 248-260, 1981. Reprints: Laligam N. Sekhar, MD., Dept. of Neurosurgery, 9402 Presbyterian-University Hospital, Desoto at O'Hara Sts., Pittsburgh, Pa. 15213. For those interested in a comprehensive, recent review of this subject including pertinent comments on the pathology and physiology of blood flow in saccular aneurysm, this paper is highly recommended. · . . 35. Reference: Yamamoto, I., Rhoton, A. L., and Peace, D. A.: Microsurgery of the third ventricle: Part I. Microsurgical anatomy. Neurosurgery 8: 334-356, 1981. Reprints: Albert L. Rhoton, MD., Dept. of Neurosurgery, Box J-265, University of Florida Health Center, Gainesville, Fla. 32610. The detailed anatomy of the third ventricle is completely analyzed. Several different microsurgical approaches to the third ventricle are suggested. The deep cerebral venous system is intimately related to the third ventricle. The internal cerebral vein is adjacent to the roof and the basal vein is related to the floor. The junction of these veins with the great vein presents a serious obstacle to the operative approach to the pineal gland and the posterior part of the third ventricle. · . . 36. Reference: Bren, S.s., Hafler, D.A., Van Uitert, R.L., et al.: Spinal subarachnoid hematoma. A hazard of lumbar puncture resulting in reversible paraplegia. N. Engl. ]. Med. 303: 1020-1021, 1981. Reprints: Steven S. Bren, MoO" Dept. of Neurosurgery, Massachusetts General Hospital, Boston, Mass. 02114. This report details the complications encountered when spinal taps were done on two patients who were anticoagulated. Paraplegia developed in both patients due to subarachnoid hematoma. Both patients responded 10 surgical intervention to remove accumulated blood in the subarachnoid space. Caution is advised regarding lumbar puncture in the face of known reasons f()r «().lgul()r,llhv. 244 37. Reference: Birnholz, J.C, and Frigoletto, F.D.: Antenatal treatment of hydrocephalus. N. Engl. f. Med. 303: 1021-1023, 1981. Reprints: Jason c. Birnholz, MD., Dept. of Radiology, Harvard Medical School, Boston, Mass. 02115. Percutaneous cephalocentesis is useful method for reduction of head size in a fetus with massive hydrocephalus so as to aid delivery. The procedure does have significant associated risk of fetal morbidity and i~ used when large fetal head size precludes an uncomplIcated vaginal delivery. Ultrasonically guided percutaneous needle insertion was used in the antenatal management of a fetus with progressive hydrocephalus which was detected at 24 weeks' gestation. Six atraumatic cephalocentesis procedures were done with ultrasonic guidance. The authors suggest that, based on the experience in this single case, ultrasonic guidance for percutaneous tap may be a much safer procedure for the fetus. • • • 38. Reference: Rosenblum, JD., Keating, J.P., Prensky, A.L., et al.: A progressive neurologic syndrome in children with chronic liver disease. N. Engl. ]. Med. 304: 503-508, 1981. Reprints: Jerry L. Rosenblum, MD., 500 S. Kingshigh-way Blvd., P. O. Box 14871, St. Louis, Mo. 63178. Six children with progressive cholestatic disease developed a progressive neurologic syndrome characterized by areflexia, altered gait, diminished proprioceptive and vibratory sensation and gaze paresis. Serum vitamin E levels were very low. Neuropathological studies performed on two of five fatal cases revealed posterior column degeneration, selective loss of large caliber, myelinated axons in peripheral nerve, and spheroids in the gracile and cuneate nuclei. The suspected cause is chronic vitamin E deficiency. • • • 39. Reference: Wiebers, D.O., Whisnaut, J.P., and O'Fallon, W.M.: The natural history of unruptured intracranial aneurysms. N. Engl. f. Med. 304: 696-698, 1981. Reprints: David O. Wiebers, MD., Mayo Clinic, Roch-ester, Minn. 55905. The natural history of a selected group of 65 patients with 81 aneurysms who did not undergo surgery was studied over a mean follow-up interval of 8 years. Multivariate analysis was done to assess the relationship of several independent variables on the frequency of aneurysm rupture. The variables included age, sex, aneurysm size, aneurysm number, presence of multilobed aneurysms, aneurysm symptoms aside from rupture, aneurysm location, and the effect of hypertension. It was found that the only variable of unquestionable importance was aneurysm size. None of the aneurysms less than 1 em in size ruptured, whereas eight of the 29 aneursyms larger than 1 em in diameter did. The appar- Journal of Clinical Neuro-ophthalmology ent probability of rupture of aneurysm less than 1 cm in size is probably very low. • • • 40. Reference: Ojemann, R.G.: Management of unruptured intracranial aneurysm. N. Engl. f. Med. 304: 725-726, 198!. Reprints: Robert G. Ojemann, MD., M,lss,)chuSE'tts General Hospital, Boston, Mass. 02114. This is an editorial response to the paper by WiebE'rs, Whisnaut, and O'Fallon in this issue of the fourn,11. D. Ojemann indicates that this personal experience, the experience in the cited paper ,1bove, ,md thilt obtilined from The Cooperative Study reported Locksley (}. NE'Urosurg. 25: 321-308, 1Qoo) suggests thilt ilneurysms greater than 7 mm in diilmeter should be treilted surgically and all symptomatic aneurysms should be operated. Questions concerning the method of follow-up of asymptomatic, incidentally discovered small aneurysms still remain unresolved. • • • 41. Reference: Brown, G.C, Magargal, L.E., Shields, ].A., et .11.: Retinal arterial obstruction in children and young adults. Ophthlamology 88: 18-25, 198!. Reprints: Larry E. Magargal. MD., Retinal Vascular Unit, Wills Eye Hospital. Ninth and Walnut Sts., Philadelphia, Pa. 19107. The records of 27 patients under 30 years of age were reviewed in order to determine the cause or causes for retinal arterial obstruction. A history of migraine was found in one-third and coagulation abnormalities were also common. Trauma, sickle cell hemoglobinopathies, cardiac disorders, oral contraceptives, pregnancy, lupus, and intravenous drug use were less common causes. Ocular abnormalities included increased intraocular pressure, buried optic nerve drusen, and congenital peripapillary vascular loops. In contrast to older patients with retinal artery obstruction, atheromatous changes were infrequent. The visual prognosis in older or younger patients is, however, the same. • • • 42. Reference: Kaiser-Kupfer, M.I.C, and Rodriques, M.M.: I. Tamoxifen retinopathy. A clinicopathologic report. Ophthalmology 88: 89-93, 1981. Reprints: Muriel I. Kaiser-Kupfer, MD., National Eye Institute, Building 10, Room 125-235, National Institutes of Health, Bethesda, Md. 20205. Superficial white refractile crystals developed in the retina of a 57-year-old female who was bemg treated with tamoxifen citrate for metastatic breast carcinoma. The lesions were predominantly located in the paramacular area. These lesions probably represent products of axonal degeneration secondary to tamoxifen chemotherapy. • • • September 1981 McCrary 43. Reference: Evans, D.E., Biglan, A.W., and Troost, B.T.: Measurement of visual acuity of latent nystagmus. Ophthalmology 88: 134-137, 198 I. [~E'prints: Albert W. Biglan, MoO, 35]8 Fifth Ave., Pitts-burgh, I'a. 15213, Amethod for rapid, accurate measurement of visual acuity in patients with latent nystagmus is described. Monocular visual acuity can be accurately assessE'd using the duochrome test for projected vision test targets and Worth red-green glasses. • • • 44. Reference: I'antell, R.A., and Goodman, B.W,: Takayasu's arteritis: The relationship with tuberculosis. Pediatrics 67: 84-88, 198!. Reprints: Benjamin W. Goodman, Jr., MD., Dept. of Family Practice, Medical College of South Carolina, 171 Ashley Ave., Charleston, S.C 29403. A12-year-old girl developed Takayasu's arteritis (pulseless disease) within a month of the time she manifested a tuberculous cervical adenitis, This arteritis is common in Japan and Korea, rare in U.s.-born people, has an unknown etiology but may be due to an autoimmune process as it responds to steroid treatment. The likelihood of finding tuberculin sensitivity or active tuberculosis in patients with pulseless disease is higher than in the general population in all countries where the disease occurs has been analyzed. The patients described here had a complete remission and return of pulses after antituberculous treatment. The relationship of tuberculosis and pulseless disease may be more than casual. • • • 45. Reference: Wirtschafter, J.D.: Diagnosis of optic atrophy. Perspect, Ophthalmol. 4: 223-252, 1981. Reprints: Jonathon D. Wirtschafter, MD., University of Minnesota School of Medicine, Box 493, Mayo Memorial Building, Minneapolis, Minn. 55455. This is an excellent review of the classification and description of various types of optic atrophy, It would be especially recommended to those individuals who are non-neuro-ophthalmologists or thosE' who m.w feel the need for a concise review of the subject, • • • 46, Reference: Weinstein, M.A., Modic, M,T., Risius, B., et .11.: Visualization of the arteries, veins, and nerves of the orbit by sector computed tomography. Radiology 138: 83-87, 198 I. Reprints: Meredith A. Weinstein, MD., Dept. of Radiology, Cleveland Clinic Foundiltion, 9500 Euclid Ave., Cleveland, Ohio 44106. Achange in the scintillation crystal configuration of a fixed-detector CT scanner increased the spatial resolution from 6.25 to 10.0 line pairs per centimeter. Sector scans of the orbit with this modified scanner demonstrated the ophthalmic artery and superior ophthalmic vein and their branches as well as some of the 245 Information Summaries intr,wrbital components of the third, fifth, and sixth nerves. . . . 47. Reference: Savoiardo, M., Harwood-Nash, D.C., T.1dmor, R., et .11.: Gliomas of the intracranial anterior optic pathways in children. Radio/oxy 138: 601-610, I Q!\ t. Reprints: Derek C. Hdrwood-Nash, M.D., Dept. of Rddiology, Hospital for Sick Children, SSS University Ave., Toronto, Ontario MsG IX8, Canada. Twenty-two gliomas of the anterior optic pathways were reviewed, and the relative merits of computed tomography pneumoencephalography, angiography, dnd isotope brain scans were assessed. Contrast-enhanced CT scans were superior to the other methods in demonstrating the full extent of the lesions and their spread along the optic pathways. Pneumoencephalography better detected small chiasmatic lesions. Angiography was of little help except in intracanilicular or intraorbital optic nerve involvement. A protocol for evaluation of patient with glioma of the anterior visual pathways is proposed. • • • 48. Reference: Gabrielsen, T.O., Seegar, J.F., Knake, J.E., et .11.: The nearly occluded internal carotid artery: A diagnostic trap. Radiology 138: 611-618, 1981. Reprints: Trygve O. Gabrielsen, M.D., Dept. of Radiology, University of Michigan Hospital, Ann Arbor, Mich. 48109. The nearly occluded internal carotid artery may closely stimulate the appearance of total occlusion if standard angiographic methods are utilized for study. Correct diagnosis requires careful study via selective common carotid angiography with a long imaging sequence and subtraction. Carotid endarterectomy is the procedure of choice as the size of the arterial lumen distal to such a repair is often striking. Many cases previously reported as internal carotid artery occlusion 246 with successful repair may have actually had just a severe stenosis. • • • 49. Reference: Raji, M.R., Kishore, P.R.S., and Becker, D.P.: Pituitary Microadenoma: A radiological-surgical correlative study. Radiology 139: 95-99, 1981. Reprints: P.R.5. Kishore, M.D., Dept. of Radiology, Box 61S, Medical College of Virginia, Richmond, Va. 23298. The correlation of radiologic changes of the sella found on thin section, complex motion tomography with the actual location of pituitary microadenomas at the time of surgery was studied. In 14 of S5 patients, a significant discrepancy was found between the radiologic prediction and the actual location of the tumor. The authors caution against absolute reliance on polytomography in making a diagnosis of pituitary microadenoma. • • • 50. Reference: Chambers, E.F., Turski, P.A., Sobel, D., et .11.: Radiologic characteristics of primary cerebral neuroblastomas. Radiology 139: 101-104, 1981. Reprints: Thomas H. Newton. M.D., Dept. of Radiology, Room M-396, University of California School of Medicine, San Francisco, Calif. 94143. Primary cerebral neuroblastoma should be considered in the differential diagnosis of calcified, cystic lesions in the supratentorial compartment of young patients. The authors reviewed the radiologic findings in 11 patients with this tumor. These neoplasms tend to have a high recurrence rate and frequently metastasize along the CSF pathways, although in this series only three patients had recurrences and one had spread of tumor throughout the nervous system. The preferred treatment is surgery combined with Sooo rads of local irradiation of the tumor bed over a 6-week period. • • • Journal of Clinical Neuro-ophthalmology |
