Journal of Neuro-Ophthalmology, March 1982, Volume 2, Issue 1

Concise summaries.

f. C}in. Neuro-ophthalmol. 2: 65-71, 1982. Concise Summaries 1. What does this paper suggest is needed in .In evaluation of all patients with Raeder's para­trigeminal syndrome? ., Is the corneal reflex affected by timolol maleate O.SOlo? 3. What are the CT manifestations of radiother­apeutic complications in the central nervous system? . 4. Gallium citrate isotope scans may be an Im­portant adjunctive test in the diagnosis of sar­coidosis. 5. An afferent pupil defect may be found in patients with asymmetric severe macular de­generation. 6. Why should a sebaceous nevus of Jadassohn be followed carefully? 7. Here is an excellent review of the various Causes of organic brain syndrome. 8. The onset of hypopituitary endocrine function during pregnancy or during the immediate postpartum state suggests a diagnosis of lym­phocytic hypophysitis. 9. Papilloarterial ischemia is much like anterior ischemic optic neuropathy, but has some im­portant differences. 10. Here is further evidence that the ipsilateral hemisphere controls smooth pursuit move­ments of the eyes. 11. Lowenfeld and Thompson find more support for the reinnervation theory for the constric­tion of the tonic pupil during the near synki­nesis. 12. What is the most common cause of unilateral proptosis in childhood? 13. Acute syphylitic optic neuritis may occur with­out other signs, such as uveitis or retinal vas­culitis. 14. Chronic papilledema will clearly affect the ERG and the YEP, based on this study of two patients. 15. Skin biopsy studies to look for giant pigment granules and macromelanosomes are of more value than iris transillumination or examina­tion of the fundus in the detection of affected males or carrier females with x-linked albin­ism. 16. This paper recommends that surgery not be done on pineal and ectopic pineal tumors. Instead, the patient should be treated WIth March 1982 Information Summaries JOHN A. McCRARY, III, MD. whole brain irradiation with a boost dose to the tumor site. 17. Skew deviation may be a sign of a ball-valve hydrocephalic complication of a third ventric­ular cyst. 18. Does the YEP correlate well with the severity of optic nerve complications in experimental allergic encephalomyelitis? 19. The vascular retinopathic complications en­countered in the optic neuritis of experimental allergic encephalomyelitis were found to be the result of compression of the intraneural central retinal vein. 20. Have you ever wondered how often papille­dema occurs in meningitis? 21. Is the hot bath test of any real clinical value? 22. Here is a thoughtful editorial on the diagnosis of multiple sclerosis. 23. Ocular bobbing can have some unusual causes. This report describes one of them. 24. If confronted by "idiopathic" cerebellar atro­phy, these eye signs suggest a high probability of dominant heredity, even though the patient may have no family history for the disease.. 25. What is the neuroanatomic locus for selectIve acquired downgaze paralysis? 26. What is the best method of treatment of cran­iopharyngioma? Is irradiation important or not? 27. Does opening the optic nerve sheath in a pa­tient with chronic papilledema cause a reduc­tion in intracranial pressure? 28. Recurrent retinal ischemia can have some un­usual causes, and may require specialized sur­gical consideration. 29. This review of neuromuscular transmission disorders is excellent. 30. Old anatomy teaching is reconfirmed. 31. Here is an unusual migraine syndrome. 32. Is meningitis after lumbar puncture a signifi­cant risk in d child with bacteremia? I. [~ef('r('nc(': Celmers, H.I.: The Pericarotoid syn­drome: A combination of hemicrania, Horner's syn­drome, and internal carotid artery waH lesion. Acta Neufllchir. 57: 37-42, 1981. Reprints: H.I. Gelmers, MD., Department of Neurology, Pr. Irenr Ziekenhaus, 7607 P W, Almelo, The Neth­erlands. Two patients developed ocu)osympathetic paresis and ipsilateral faCIal pam. AnglOgraphlc evaluatIOn re- 65 Information Summaries vealeda lesion in the cervical extracranial cMotid artery on the side ipsilateral the ocular lesion. In one patient the cause W,IS fibromuscular dysplasia, while the other had spont,mN1us dissection of the media within an in­ternal cMotid ,lrtery. Good recovery from pain was en­countered postoperatively in both cases. The oculosym­pathetic defect persisted in each patient. • • 2. Reference: Calissendorff, B.: Corneal anesthesia after topical treatment with timolol maleate: A case report. Acta Ophthalmol. 59: 347-349, 1981. Reprints: Berit Calissendorff, MD., Dept. of Ophthal­mology, Karolinska Institute, Huddinge University Hospital, S- 14 1 86, Huddinge, Sweden. This report describes the detection of transient corneal sensitivity depression in a 58-year-old patient treated with timolol maleate, 0.5% once daily. The de­pression of the corneal reflex in the treated eye was striking-almost to the point of complete anesthesia. The medication was discontinued; corneal sensation gradually returned to normal and the intraocular pres­sure rose. Treatment with timolol was restarted, and 3 weeks later corneal sensation was normal: 6 weeks later corneal sensitivity was reduced. No other explanation for the anesthesia could be found aside from the effect of timolol. If a patient fails to give the physician a complete history of drug use and a decrease in corneal sensation is detected, one should be certain to inquire about a history of glaucoma and the possibility of the use of timolol maleate to avoid misinterpretation of a decrease in corneal sensitivity. This information is especially use­ful to the neurologist or neurosurgeon who might see a patient who is on treatment for a unilateral glaucoma. • • 3. Reference: Kingsley, D.P.E., and Kendall, B.L Re­view: CT of the adverse effects of therapeutic radia­tion of the central nervous system. Am. ]. Neurora­diol. 2: 453-460, 1981. Reprints: D.P.E. Kingsley MD., Dept. of Radiology, London Hospital, Whitechapal, London Ell BB, Eng­land. Clinical deterioration during or after radiation therapy of the central nervous system may be due to pro­gression of, or a complication induced in, the lesion being treated. Deterioration m,ly ,llso be due to an adverse effect of the radiation on nervous tissue or vessels within the beam, to alteration of the blood-br,lin barrier, thus influencing the effects of drug therapy, or a superimposed catastrophe unrelated to the tre,ltnH'nt. Transient effects, which occur within the first 3 months of radiotherapy, include increase in symptoms suggest­ing enlMgement of thp tumor ,md thp soml1(llpl1l'P syn­drome that occurs in children. Classical permanent radiation effects includp necrosis, atrophy, calcification, necrotizing Ipukopm'ephalopathy, mineralizing miuoangiopathy, ,mpurysm form,ltic1n, tu­nwr Indll( tion, and (prpbrosplnal fluid fistul,l('. R,HPly, demyelination occurs with higher doses than are cur­rpntly used. The permanent effects tend to occur later than the transient ones; some are progressive and many have serious consequences, The appearance of mineral­izing microangiopathy is specific on CT; but the n,ature of other complications is usually only made by clInICal criteria, although the differentiation of radiatIon necrosIs from further growth of intracerebral tumor may be difficult. Selected cases are used to illustrate the CT features of these entities. • • • 4. Reference: Weinreb, R,N., Yavitz, E.Q., and O'Connor, C.R.: Lacrimal gland uptake of gallium citrate, Am. ]. Ophthalmol. 92: 16-20, 1981. Reprints: Robert N. Weinreb, MD" Clinical Research Center for Eye Disease, 350 Pamassus Ave" SUIte 307, San Franscisco, Calif. 94117. An increased gallium citrate Ca 67 uptake was detected n in the lacrimal gland in 53 out of 61 patients (87%) with sarcoidosis. The uptake was increased in 21% out of 68 (31%) on nonsarcoid patients. The difference be­tween groups was significant, but because the uptake is increased in any disorder that causes inflammation of the lacrimal gland, it cannot be considered specific for sarcoidosis. The test may be useful for investigational direction indicating the need to rule out sarcoidosis by other measures. • • • 5. Reference: Newsome, D.A., Milton, R.C., and Cass, JD.M.: Afferent pupillary defect in macular degen­eration. Am. ]. OphthalmoJ. 92: 396-402, 1981. Reprints: David A. Newsome, M.D., Building 10, Room 10 D 19, National Eye Institute, National Institute of Health, Bethesda, Md 20205. An evaluation for the afferent pupil defect was done n in 101 consecutive patients with senile macular degeneration. The pupil sign was found in 39 patients. It was observed most often when there was a large difference in the size of the macular lesion in both eyes. The afferent pupil defect is not only a sign of optic nerve disease but can occur in diseases of the retina, • • • b. Reference: Wilkes, S.R., Campbell, R.J" and Waller, R.R.: Ocular malformation in association with ipsi­lateral facial nevus of Jadassohn. Am, ]. OphthalmoJ. 92: 344-352, 1981. Reprints: Robert R. Waller, MD" c/o Mayo Clinic, 200 First St., S.W" Rochester, Minn. 55905, The ocular and associated neurologic manifestations of a sebaceous nevus of Jadassohn are described in ,1 13-year-old boy. Any patient with this condition which consists of an excess or deficiency of skin appendages, including sebaceous glands, together with nevi or nevoid tumors, should be kept under periodic evaluation, as malign,mt tumors may arise in the area of the nevus, Neurologic complications include skull bony defects, cerebral atrophy and seizures, while the ocular defects usually comprise conjunctival anomalies, • • • Journal of Clinical Neuro-ophthalmology 7. Reference: McEvoy, I.P.: Review: Organic brain syndromes. Ann. Int. Med. 95: 212-220, IQ~ I. Reprints: loseph P. McEvoy, MD., Western I'syrhi.ltril· Institute, 3811 O'H,lra St., Pittsbur~h, I'mll. I52t) I. For those persons interested in .1 surlinlt review <If lhe current classification, deslriptive .In.llysis, .lnd diffN­entiaI diagnosis of the v.Hious l)rg.lnil br.lin svndrllTl1l's, this paper is highly recommended. • • 8. Referenle: As.I, S.L.. Bilb.lo, l.M., KlW.llS, K., et .11.: lymphocytic hypophysitis of pregnancy resulting in hypopituitarism: A distinct clinicopathologic entity. Ann. Intern Med. 95: 1(>(>-171, 1'181. Reprints: Sylvia l. Asa, MD., Dept. of P.lthology, St. Michael's Hospital, 30 Bond St., Toronto, Ontario M5B 1W8. Canada. Two patients who presented with clinical evidence of pituitary adenoma associated with pregnancy, or the postpartum period, are described. Pathologic examina­lions revealed extensive pituitary gland destruction by Iymphoplasmacytic infiltrates, with no tumor identified, in each case. Both patients eventually developed hypo­pituitarism. Histologic features suggest an autoimmune cause for the condition that has been reported in women during pregnancy or the postpartum state. Prior reports on this condition are reviewed. Lymphocytic hypophysitis must be considered as a potential diagnosis when the clinical evidence for an aggressive pituitary neoplasm is present in conjunction with hypopituitarism and pregnancy or the postpartum state. • • 9. Reference: Kottow, M.H.: Papilloarterial ischemia. Ann. Ophthalmo/. 13: 963-969, 1981. Reprints: Michael H. Kottow, MD., Elisabethen Str. 15, 7000 Stuttgart I, West Germany. This report describes the findings in six patients with an unusual type of vascularly induced disc edema. All patients were fairly young (median age 43! years) and the condition caused slight, mostly transient, visual decrease. The pupils were consistently abnormal and altitudinal visual field defects were commonly present. Bilateral involvement occurred in three patients. The fundus showed a pale disc with edema and se­quential constriction of emerging arteries associated with signs of inflammation, peripapillary hemorrhages, and cotton-wool spots. The fluorescein angiographic char­acteristics showed slow arterial flow with disc and cho­roidal ischemia and leakage of dye. This condition ap­pears to be clinically different than arterior ischemic optic neuropathy for a number of reasons. The cause is unknown. • • • March 1982 McCrary 10. I< cfl'rC'11lC': Sh.lrpC', I.A., and Lo, A. W.: Voluntary and visual control of the vestibuloocular reflex after cerebral hemidecortication. Ann. Neural. 10: 164-172, IQ~I. 1~('prints: J.lmes A. Sh.lrpe, M.D., Division of Neurology, T<lronto Western Hospital, 399 Bathurst St., Toronto, Ont.lrio M5T 258 Canad.l. Horizontal Vl'stibulooculM reflex function (VOR, was studil'd in fivl' patil'nts after cl'rl'bral hl'midecor­lilation. In dMkness, vl'stibuloocular reflex gain was slightly higher contralatl'ral to thl' decorticate hemi­sphere. Voluntary modul.ltion of the reflex by attempted fixation of imaginary targets in darkness increased to vestibuloocular reflex asymmetry. Defective visual mod­uldtion corresponded to paresis of ipsilateral smooth pursuit. One hemisphere regulates ipsilateral smooth ocular movements that achieve voluntary and visual control of the vestibuloocular reflex. • • • 11. Reference: Lowenfeld, I.E., and Thompson, H.5.: Mechanism of tonic pupil. Ann. Neurol. 10: 175-176, 1981. Reprints: Irene E. Lowenfeld, PhD., Kresge Eye Institute, Wayne State University, Detroit, Mich. 48201. Recently, some authors have suggested that the con­striction of the tonic pupil to near stimulation might not be due to axonal sprouting and reinnervation cou­pled with hypersensitivity, but instead might be due to an outpouring of acetylcholine from the ciliary body causing the hypersensitive postdenervated pupil to con­strict. New evidence has now surfaced to refute the latter argument and clearly support the original suggestion that imperfect reinnervation of the pupil sphincter in association with postdenervational hypersensitivity is indeed the cause for the enhanced response of the tonic pupil to near reflex stimulation. • • 12. Reference: O.lkhill, A., Willshaw, H., and Mann, l.R.: Unilateral proptosis, Arch. Dis. Child. 56: 549­551, 1981. Reprints: I. R. M.lnn, MD., Children's H0spital, Lady-wood Middleway, Birmingh.lm B 10 8ET, England. Twenty-seven children with unil.lter.ll proptosis were investig,lted over .1 period of ~! ye.HS. Seventeen (03%) had local or dissl'mill.lted m.llignalKY. This fre­quency of m.llign.lnlY is higher than in prior reports. The frequency of inflamm.ltory causes appears to have declined due to the liberal use of antibiotics. The most common malign.lncy was rhabdomyosarcoma with glioma being next most common. • • • 67 Inforn1<ltion Summ.lries 13. Reference: Weinstein, I.M., Lexow, S.s., .lnd Ho, P., et ,\1.: Acute syphilitic optic neuritis. Arch. OphthJ/- • mol. 99: 1392-1395, 1981. Reprints: 101'1 M. Weinstein, MD., l1C'pl. of Ophth'll­moll1gy, V,lIlderbih UniVl'rsity, N.lshville, Tenn. 37232. Two p,ltients experiC'nced ,Kute unil.lteral loss of vi­sil> ll ,IS ,I complil,ltion of second<lry neurosyphilis. In both L1SC'S, the optic nerve infl.lmmdtion WdS preceded by dl,H,lderistic derm.ltologic signs ,l11d signs of mild ~eninge.ll irrit.ltion. No uv('itis or rC'tindl vdsculitis W.lS discovered. It is import.lnt to remembC'r th.lt optic neu­ritis C.ln be found in pdtients with meninge.ll infl.lm­mdtion <lccompdnying seconddry syphilis. It is importdnt thdt CNS involvement be confirmed dnd thdt dppropridte .lntibiotic ther<lpy be given. • 14. Reference: Kirkhdm, TH., dnd Coupldnd, SB.: Ab­normal electrorelinograms and visual evoked poten­tials in chronic papilledema using time-difference analysis. Can. j. Neurol. Sci. 8: 243-248, 1981. Reprints: Trevor H. Kirkhdm, M.D., 3801 Universite, Suite 201, Montredl, Quebec H3A 2B4 Cdnadd. ViSUdl function is usudlly thought to be normdl in pdpilledema. This report dedls with the previously undescribed observdtion of dbnormdl scoptopic dnd pho­topic electroretinogrdm (ERG) and ViSUdl evoked poten­tidl (VEP) in two patients with chronic pdpilledemd. These studies revedled an apparent relative vulnerability of a high frequency sensory signdl system to raised intracranial pressure. The abnormalities resolved rapidly once the intracranial pressure was normalized. The exact Cduse for these ERG and YEP is unknown. • 15. Reference: Cortin, P., Tremblay, M., dnd Lemange, ].M.: X-linked ocular albinism: Relative value of skin biopsy, iris transillumination and funduscopy. Can. j. Ophthalmol. 16: 121-123, 1981. Reprints: Paul Cortin, M.D., Department d'ophthalmologie, Centre Hospitalier de l'universite wval, 2705 boul. Ldurier, Ste-Foy, Quebec G1V 4G2 Candda. Ina group of 15 males with x-linked oculdr dlbinism dnd 13 obligdte carriers of the gene for the condition, skin biopsy was done to search for gidnt pigment grdn­ules dnd mdcromeldnosomes by light .lnd electron mi­croscopy. Gidnt pigment grdnules dnd m.lcromeldnl~­somes were more often found to confirm the presence of the gene thdn W.lS iris tr.lnsillumin.ltion llf fundus­copy. Thus, skin biopsy should signific.lntly improve the detection of dffected mdles and cMrier fem.lles. 16. Reference: Rdo, Y.TR., Medini, E, H.ls('low, R.E, et al.: Pineal and ectopic pineal tumors: The role of radiation therapy. c'lI1cer 48: 708-713, 1981. Reprints: Y.lshod.l TR Rao, MD., Dept. of Ther.lpeutic Radiology, VAMC, 54th SI. ,lIld 48th Av('. South, MirllH',lpolis, Minn. 55417. Seventeen patients with pineal tumors anJ one pati~nt with a suprasellar germll10ma were treated wl~h radiation therapy. Surgical decompression was done III 16 pdtients and two had resection of the tumor. No age or dose dependence in survival was discovered, but those patients who received whole brain irradiation or gener­ous fields thdt included the ventricular system had better survival. The possibility of increased incidence of men­ingedl tumor cell seeding following surgical intervention is considered. The current recommenddtions from this series in­cludes whole brain irrddiation with 4000 rads followed by a boost dose of 5000 rdds to the tumor site. Surgery CJn be reserved for diagnosis and/or treatment of pa­tients who show a deldyed response to irradiation. • • 17. Reference: Kempf, D., and Szepan, B.: Skew devia­tion as presenting ocular sign of a third ventricle colloid cyst. Eur. Neural. 20: 326-329, 1981. Reprints: D. Kempf, M.D., Neurologische Klinik, Med­izinische Hochsclurle Lubeck, Ratzeburger Allee 166, D-2400 Lubeck, (FRG). The authors describe recurrent attacks of difficulty with vertical gaze impairment and skew deViatIOn 111 a patient with d colloid cyst of the third ventricle. The attacks of skew deviation coincided with deterioration in the pdtient's level of consciousness. The attacks occurred dS the result of acute hydrocephalus produced by a ball­valve effect of the cyst periodically obstructing the fo­ramina of Monro. • • 18. Reference: Hayreh, 5.5., Massanari, R.M., Yamdda, T, et dl: Experimental allergic encephalomyelitis. I. Optic nerve and central nervous system manifesta­tions. Invest. Ophthalmol. 21: 256-269, 1981. Reprints: S.s. Hayreh, M.D., Dept. of Ophthalmology, University Hospitals and Clinics, Iowa City, Iowa 52242. Experimental allergic encephalomyelitis was produced in six adult rhesus monkeys. They were carefully evaluated with repeated clinical observation of ocular and neurologic status. Special attention was focused on optic nerve complications of experimental allergic en­cephalomyelitis. Laboratory evaludtion via fluorescein fundus photography, visual evoked potential recording, and studies of cerebrospinal fluid were also done. Visual evoked potentials were recorded by the bright flash method. Four of the six monkeys survived longer than 1 month .lnd all developed chronic relapsing experimental allergic encephalomyelitis. These monkeys were sacrificed from 3 to 14 months after sensitization. All 12 eyes from the six monkeys developed optic neuritis with variable ,lmounts of optic disc edema and visual 1055. The animals that survived to develop chronic experimental allergic encephalomyelitis developed optic atrophy with com­plete or nearly complete blindness. Histopathologic examination of the optic nerve and central nervous system in all of the monkeys revealed inflammatory infiltrates, extensive 1055 of myelin, and Journal of Clinical Neuro-ophthalmology axonal degeneration, without infl.lmmation in the retin.l or optic nerve head. The visu.ll evoked potenti.ll did not correlate well with less th.m severe llptic nervl' dise.lsl' that was clinically obvious. • • 19. Reference: H.lyreh, 5.5.: Experimental allergic en­cephalomyelitis. II. Retinal and other ocular m.mi­festations. Invest. Ophth.llnwl. 21: 270-281, IQ81. Reprints: S.s. Hayreh, M.D.. Dept. of Ophth.llmolo~y, University Hospit.11s .md Clinics. low.l City, 10w.1 52242. E xperiment.ll.lller~ic enceph.llomyelitis W.IS produced in six .ldult rhesus monl..eys. All ot the .lI1imals developed .Kute EAE .lI1d llptic neuritis in both eyes. A vaso-occlusive retinopathy develt'ped in five llf the 12 eyes. Conjunctivitis .md iridocyclitis were seen in some eyes. The retinopathy appeMed to be the result of intra­neural compression of the central retinal vein second.lry to the inflamm.ltory condition of the nerve, not by direct retinal-vascular inflammatory changes. There was no histopathologic evidence for direct inflamm.ltory change in the retina or its vascular tree. • 20. Reference: Hannd. L.S., Girgis, N.J.. Yassin, M.M.W., et dl.: Incidence of papilloedema and optic atrophy in meningitis. jpn. j. Ophthalmol. 25: 69-73, 1981. Reprints: Publications Editor, NAMRU-3, FPO New York (NY) 09527. The ophthalmoscopic changes thdt occurred dS d com­plication of meningitis were studied in Cdiro, Egypt, dUring the period from August 1969 to August 1978. The incidence of papilloedema in 2,178 cases of menin­gitis was 21% and optic atrophy occurred in 1!%. Papil­loedemd and optic atrophy occurred more often in tu­berculous meningitis than in other types of bacterid I meningitis. Primary optic atrophy was more common than post-papilloedema optic atrophy. Papilloedema was observed on admission in 36 out of 485 patients with tuberculous meningitis (7.4%). 15 out of 853 patients with meningococcal meningitis (1.8%). and 4 out of 614 with purulent meningitis of unknown cause (0.7%). None of the 66 patients with virdl menin­gitis had papilledema on ddmission. • • 21. Reference: Malhotrd, AS, and Goren, H.: The hot bath test in the diagnosis of multiple sclerosis. j.A.M.A. 246: 1113-1114,1981. Reprints: Hershel Goren, M.D., The C1evl'ldnd Clinic Foundation, 9500 Euclid Ave., Cleveland, Ohio 44106. This study was designed to test the sensitivity and specificity of the hot bath test in the diagnosis of multiple sclerosis. New neurological signs developed in 60% of the patients with multiple sclerosis, but not in the control patients. The multiple sclerosis study group had 18 patients. Eight developed a significant drop in visual acuity de­spite the fact that none of the eight had prior visual March 1982 McCrary complaints. 5ix patients with no prior history of diplopia developed an internuclear ophthalmoplegia. • • 22. 1~('f('f(,Ill'I': Cilman, 5.: The diagnosis of multiple sclerosis (editorial). /.A.M.A. 246: 1122-1123,1981. I~l'prillts: Sid eilman, MD., University of Michigan, Dept. of Neurology, Ann Arbor, Mich. 48109. There is no IJboratory test that conclusively makes the di.,~nosis of multiple sclerosis. The diagnosis must still bl' made on clinical ~rounds and supported by labor.ltory tests. Thl' use of thl' CT scan, visual-evoked potentials, and auditory-evoked brain-stem potentials may be of help. Spindl fluid eVdluation can also be useful, especidlly if there is .In elevdted IgG level, oli­goclonal bands, or myelin basic protein elevation. These tests are, though, not specific. This is an excellent, thoughtful commentary on the real status of the diag­nosis of multiple sclerosis. • • • 23. Reference: Rudick, R., Satran, R., dnd Eskin, T.A.: Ocular bobbing in encephalitis. rNeural. Neurosurg. Psychiatry 44: 441-443, 1981. Reprints: R. Rudick, MD., 601 Elmwood Ave., Roches-ter, N.V. 14642. Ocular bobbing is generally found in the context of d severe pontine vascular insult. This report de­scribes the observation of ocular bobbing in a 19-year­old female patient who developed a pharyngitis followed by encephalitis, proven by brain biopsy. The patient slowly recovered. This is the second case report of ocular bobbing with encephalitis and the first with histopath­ologic assessment reported. • 24. Reference: Harding, A.E.: "Idiopathic" late onset cerebellar ataxia: A clinical and genetic study of 36 cases. j. Neural. Sci. 51: 259-271, 1981. Reprints: A.E. Harding, M.D., Dept of Neurology, The Middlesex Hospital, Mortimer Street, London WI. England. The clinical features of 36 patients with late onset cerebellar ataxid were studied. The age of onset was from 30 to 74 yeus, and there were more males than females. The pdtients were divided into three groups on clinical grounds. Group A hold 12 patients in whom truncal ataxia was greater than limb dtdxia, and there WdS a mean time of onset of nearly 55 years. Group B hold six pdtients with more tremor in the upper limbs at rest and during .lction. Group C h.ld \8 patients similu to sporadic.llly developing olivopllntocerebellar atrophy. This latter c.lte~ory h.ld a large excess of males. All 30 pJtients in the thrl'e groups were compared with 30 patients known to havl' dominantly inherited late onset cerebeliM ataxi.l, to determine what clinicdl signs indi­cdte that a "spor.ldic" case might indeed indicdte J new mutation of d dominant cerebeliM atrophy that would require special family counseling. The discovery of optic atrophy, ophthalmoplegia, and retinopathy indicated a higher probdbility of a dominant disorder. • • 69 Infllrmation Summaries 25. Reference: Trojanowski, J.Q., Jnd Lafontaine, M.H.: Neuroanatomical correlates of selective downgaze paralysis. j. Neuro/. Sci. 52: 91-101, 1981. I~cprints: John Q. Troj.lI1owski. MD., Dept. of Pathol­l'gy, Division of Ncurop.lthology, University of Penn­sylv. lI1i.1 School of Medicine, 454 Johnson I'Jvilion, I'hilJdclphiJ PJ. 19104. A5b-ye.H-old femJle hJd the sudden onset of a selec­tive p.H.Ilysis of downgdze With a pdrtldl third nerve p.llsy on the right. Over neMly 4 years this gaze defect W.IS stJble. At Jutopsy, the lesions Jccounting for the g.lze .1I10maly were bilJteral, Jsymmetricdl cavitdry lesions locJted at the thalJmomesencephalic junction. This case, together with previous reports of downgaze paralysis, suggests that the pertinent locus for downgaze extends from the oculomotor nucleus to the rostral pole of the red nucleus and slightly dorsomedial to the latter structure. This is analogous to the "prerubral field" of the monkey which is thought to mediate vertical gaze, and especiJlly downgaze. 26. Reference: Cabezudo, J.M., Vaquero, J.. Areito, E., et al.: Craniopharyngiomas: A critical approach to treatment.]. Neurosurg. 55: 371-375, 1981. Reprints: Michael E. Cohen, M.D., Dept. of Neurology, Children's Hospital, 219 Bryant St., Buffalo, N.Y. 14222. The authors conducted a retrospective statistical anal­ySIs of the results obtamed m a senes of 45 patients with craniopharyngiomas to assess the value of differing therapeutic approaches. All patients were placed in one of three groups according to the method of treatment: 1) less total excision; 2) less subtotal excision; and 3) less surgery followed by a course of radiotherapy. Of the patients treated by total removal, 30% had tumor recur­rence after a mean time of 2 years. Tumors recurred in 71% of those treated with subtotal excision in a mean time of 2.6 years. There was a 6% recurrence in those receiving radiation therapy after subtotal resection. The latter treatment is recommended for elective treatment of craniopharyngiomas. • • 27. Reference: Kaye, A.H., Galbraith, J.E.K., and King, /.: Intracranial pressure following optic nerve de­compression for benign intracranial hypertension. /. Neurosurg. 55: 453-450, 1981. Reprints: Andrew A. Kaye, M.B., B.S., Dept. of Neuro­logical Surgery, Radcliff Infirm.1ry, Oxford OX2 oHE, England. The authors report the resultsuf intracr.1niJI pressure monltormg m d p.1tlent With benign mtrJcrJnial hypertension who underwent .1 bilaterJI optic nerve sheath decompression for severe pJpilledema. No change in intracranial pressure was produced as b.1sed on continuous intraeraniisl pressure monitoring in the preoperative and postoperative stdte. The decredse in papilledemd c.1used by this operJtion is strictly the result of a pressure deere.Ise within the optic nerve sheJth, not d central pressure decline, as previously suggested by some .Iuthors. • 28. Reference: Countee, R.W., Vijanath.,':. T., and Chavis, P.: Recurrent retinal ischemia beyond cervi­cal carotid occlusions. ]. Neurosurg. 55: 532-542, 1981. Reprints: Roger Countee, M.D., Section of Neurological Surgery, New Jersey Medical School. 100 Bergen St., Room A 592, Newark, N. J. 07107. Seventeen patients with persistent ocular transient ischemic attacks ipsilateral to angiographically dem­onstrated internal carotid artery occlusions in the neck have been treated by the authors over the past 5 years. Complete cerebral angiography demonstrated in each case that the ophthalmic artery was perfused by the ipsilateral external carotid artery which invariably had an obstructive and/or ulcerative lesion at its origin. An adjacent residual "stump" of the occluded internal ca­rotid artery was often present and was often an embolic source. Retinal artery emboli were observed in 9 patients on fundoscopy. Endarterectomy of the external carotid artery with resection of the "stump" of internal carotid artery terminated symptoms in 10 of 11 patients. Anti­coagulent drugs stopped symptoms in four nonsurgical patients and in two with failed operations. It was con­cluded that recurrent retinal ischemia beyond cervical carotid occlusions frequently results from microembo­lism from the external carotid artery. External carotid artery endarterectomy is recom­mended for control of recurrent retinal ischemic symp­toms. Anticoagulant drugs are effective in the patients who are poor surgical risks. • • 29. Reference: Spector, R.A., Glaser, J.5., David, N.]., et al.: Occipital lobe infarctions: Perimetry and com­puted tomography. Neurology (NY) 31: 1098-1106, 1981. Reprints: Robert H. Spector, M.D.. Emory University Clinic, 1365 Clifton Rd., N.E., Atlanta, Ga. 30322. This report deals with the visual field anomalies found in seven patients with either unilateral or bilateral occipital lobe infarction. The patients were studied by computed tomography and the visual field defects were correlated with the tomographic findings. The retino­topic representation of the occipital lobe is confinned to be dS previously stated according to the older literature on the subject. • • • .10. Reference: Swift, T.R.: Disorders of Neuromuscular transmission other than myasthenia gravis. Muscle Nerve 4: 334-353. 1981. Reprints: Thomas R. Swift, M.D., Dept. of Neurology, Medical College of Georgia, Augusta, Ga. 30912. Congenital myasthenia gravis is probably a group of diseases that disrupt neuromuscular transmission. Serum acetylcholine receptor antibodies are not present and other evidence for immunologic abnonnalities at the neuromuscular junction is Idcking. This condition and many others of interest to neuroophthalmologists, in­cluding mydsthenid gravis, are reviewed and updated. The microphysiology, microanatomy, and microphar­macology of these diseases is discussed. This review is highly recommended. • • • Journal of Clinical Neuro-ophthalmology 31. Reference: Bartleson, '.D., Swanson, ,. W., and Whis­nant, J.P.: A migrainous syndrome with cerebrospinal fluid pleocytosis. Neurology (NY) 31: 1257-12b2, 1981. Reprints: John D. Bartleson, M.D., M<,yo Clinic, Roch-ester, Minn. 55901. A bnorrnalities of the spinal fluid are rare in migraine, 1"\. even when accompanied by focal neurologic signs and symptoms. Seven patients without a prior history of migraine experienced a limited series of migraine-like attacks associated with dbnormalities of the CSF. The episodic, severe headaches preceded or accompanied sensory, motor, speech, or visual disturbances. All seven patients recovered. The authors postul<,te th.lt migr<line was only a symptom of an underlying infl.lmmatory condition, the exact cause for which is currently un­known. The distinctive pattern of involvement in these patients suggests a benign syndrome. Prior reports of patients with similar conditions are reviewed. • • • March 1982 McCrary 32. Reference: Teele, D.W., Dashefsky, B., Rakusan, T., et .11.: Meningitis after lumbar puncture in children with bacteremia. N Engl. /. Med. 305~ 1079-1081. 1981. Reprints: David W. Teele, M.D., Maxwell Finland lab­oratory for infectious diseases, Boston City Hospital, Boston, Mass. 02118. These authors report yet another complication of lumbar puncture. The authors report on 277 epi­sodes of bacteremia that occurred in 271 children over a 9-year period. Lumbar punctures were done on the first visit in 46 children with normal results. Seven of the 46 children went ahead to develop meningitis, whereas only two out of 231 who did not have lumbar punctures developed meningitis. All those who devel­oped postlumbar puncture meningitis were under 1 year of age. The authors suggest cautious selection of these children with bacteremia before considering lumbar puncture. Clinical evidence for meningitis should be the deciding point for recommending lumbar puncture. • • • 71