J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.46.6.566 on 1 June 1983. Downloaded from

Journal of , Neurosurgery, and Psychiatry 1983 ;46:566-569

Short report Postictal blindness in adults

MENACHEM SADEH,* YOCHANAN GOLDHAMMER, ARIEH KURITSKY From the Departments ofNeurology, The Chaim Sheba Medical Center and Beilinson Medical Center, Sackler School of Medicine, Tel-A viv University, Israel

SUMMARY Cortical blindness following grand mal occurred in five adult patients. The causes of seizures included idiopathic , vascular accident, brain cyst, acute and chronic encephalitis. Blindness was permanent in one patient, but the others recovered within several days. Since most of the patients were either unaware of or denied their blindness, it is possible that this event often goes unrecognised. Cerebral is considered the most likely mechanism.

Cortical blindness is a rare complication of epileptic otherwise the neurological examination was normal. The seizures. In 1903 Ashby and Stephenson' summar- EEG showed diffuse high voltage slow waves and irregular ised the clinical features of this entity in 11 children bifrontal sharp wave activity. Results of lumbar puncture Protected by copyright. and infants (five in their own series). In addition to were normal. A CT head scan showed dilated ventricles blindness some patients were and enlarged sulci and subarachnoid cisterns. Bilateral aphasic and hemi- carotid and vertebral angiography was normal. Routine plegic; vision returned in all except one. Since this blood and urine analysis were also normal. The patient early report there have been only a few descriptions remained listless and generally hypoactive throughout 3 of postictal blindness2- and six cases of postictal months of follow-up. Repeated examinations confirmed hemianopia.5 8-10 Blindness has been noted in her persistent total blindness. Subsequently she died for infants and children, except for one adult case, while unknown reasons elsewhere, but a necropsy was not hemianopia has been described in adults and chil- performed. dren. We report the sudden development of blind- Case 2. A 70-year-old woman had a transient left ness in five adults immediately after grand mal seiz- hemiparesis seven years before admission. Three months ures prior to admission she presented with convulsions involv- of various etiologies. The postictal blindness ing her right arm and leg and right hemiparesis. EEG was transient in four of the five cases. showed irregular slow waves in the left temporal area. CT head scan and lumbar puncture findings were normal. An embolus was observed in the right ocular fundus involving Case reports the lower branch of the central retinal artery. She was admitted with an acute confusional state. During the http://jnnp.bmj.com/ Case 1. A 33-year-old woman had grand mal seizures since examination a grand mal was witnessed. After the age of 18. She was treated with drugs regaining consciousness she became surprisingly alert and and had a seizure approximately once a year. She was well oriented. However, while denying any visual problem, admitted to another hospital in . Upon examination revealed her to be totally blind. She did not regaining consciousness she was restless and confused. She respond to any visual stimulus such as optokinetic flag. was transferred to the Department of Neurology when she Pupillary reactions were normal. An embolus was seen in was found to be disoriented to time and place. Pupillary the lower branch of the right retinal artery as noted previ- reactions and fundoscopic examination were normal but ously. The neurological examination was otherwise nor- she was totally blind. She did not respond to any visual mal. The next morning she could count fingers at a distance on September 27, 2021 by guest. stimuli and no optokinetic nystagmus could be elicited. She of 2 m with a gradual improvement of her vision in three denied her blindness. Plantar reflexes were extensor but days to 20/100 in the right eye and 20/50 in the left. There was no record of her previous . Perimetry revealed an *Present address and address for reprint requests: Dr M Sadeh, upper temporal field defect in the right eye. On Department of Internal Medicine, University of Arizona Health follow-up there was no change in her visual acuity, visual Sciences Center, Tucson, Arizona 85724, USA. fields and fundoscopic findings. Case 3. A 25-year-old woman developed a grand mal Received 22 November 1982 seizure disorder at-the age of 20. Paranoid behaviour and Accepted 5 January 1983 brief episodes of total blindness were reported by her fam- 566 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.46.6.566 on 1 June 1983. Downloaded from

Postictal blindness in adults 567 ily following several of the epileptic seizures. She was fundoscopic examination. It may be denied by the admitted for evaluation of a mild right hemiparesis and patient (Anton syndrome). Cortical blindness has treatment of frequent seizures. She was alert and well rarely been reported as a consequence of seizure.'-8 oriented. General physical examination was normal. Some Walsh and Hoyt5 compared the blindness to postic- slow activity was evident in the EEG in the left temporal tal (Todd's) paralysis, the cause of which was area. A CT head scan showed a small low density lesion in the left temporal lobe not enhancing after contrast injec- thought to be exhaustion of neurons by hyperactiv- tion. Left carotid angiography was normal. During hos- ity; this analogy, however, raises several questions. pitalisation she had frequent adversive and grand "mal seiz- Postictal paralysis appears in patients suffering from ures. Visual testing was normal until the tenth day when focal seizures. A transient paralysis occurs after she was found to be completely blind following a pro- many of these seizures for minutes or hours, with longed grand mal seizure. Although alert and well oriented full recovery and without remaining pathological she appeared undisturbed by her visual loss. She did not neurological signs. The severity and the duration of respond to any visual stimuli and no optokinetic nystagmus the paralysis are not correlated with the severity of could be elicited. Pupils were equal and reactive and fun- the convulsions or with the occurrence or absence of doscopic examination was normal. Vision cleared gradu- ally after four days with return to normal vision and visual loss of consciousness. In fact, with abortive seizures fields. On follow-up CT head scan the lesion was the palsy may even be more pronounced. unchanged and appeared to be a low-grade astrocytoma. As opposed to postictal paralysis, postictal blind- Case 4. In July 1976 a 23-year-old previously healthy ness follows a generalised grand mal seizure and woman suffered a grand mal seizure. On regaining con- often a prolonged episode of status epilepticus. sciousness she complained that she could not see. She was Blindness is bilateral and continues for days, weeks alert, well oriented and cooperative. General physical and or months and only exceptionally for hours, some- neurological examinations were normal, except for visual times being accompanied by other neurological testing which confirmed her blindness. Pupils reacted well deficits such as weakness, deafness, or to light and the ocular fundi were normal. EEG showed diffuse high voltage slow waves. Cerebral angiography was . If postictal blindness were analogous to Protected by copyright. normal (CT scan was not then available). A day after postictal paralysis, a typical case would be one with a admission her temperature rose to 39°C and she became unilateral focus having "positive somnolent and developed a mild . Lum- phenomena" during activation of the occipital lobe bar puncture was normal except for slightly elevated pro- and the adjacent areas, such as light flashes, colour tein level of 0-56 g/l. Over the next two days the patient visions and visual ." These events became gradually more alert. Her aphasia cleared and her would perhaps be followed by the "negative vision returned to normal. Subsequent hospital course was phenomena" hemianopia and blindness, or by sec- unremarkable. Her fever abated over the ensuing several ondary generalisation to a grand mal seizure. Some- days and she made a complete recovery. The presumed diagnosis was a although this was never times the negative phenomena would occur without confirmed. the positive ones. Case 5. A 20-year-old woman had presented a year pre- Cases similar to this idealised description have in viously for investigation of mental changes over the previ- fact been documented. Penfield" reported a case ous year. These included several episodes of confusion. On with post-traumatic scar in the right occipital pole. that admission she was disoriented and had a markedly Seizures generally started with light flashes which impaired memory function. The EEG showed periodic dis- were followed by left hemianopia, blindness, stiff- charges of generalised high amplitude slow waves every 10 ness and occasionally by a grand mal seizure. http://jnnp.bmj.com/ to 20 seconds. The CT head scan was normal. Measles of blindness not accompanied by the posi- antibody titre was 1/256 in the blood and 1/32 in the CSF. Episodes A diagnosis of subacute sclerosing panencephalitis was tive phenomena have been described by Strauss'2 in made and she was treated with isoprenosine. The current an 11-year-old boy. EEG showed bilateral syn- admission was precipitated by a grand mal seizure which chronous occipital discharges; however, no record- occurred at her home. At the hospital she was awake and ing was obtained during the ictal phase. Huott'3 alert but completely blind although unaware of this. No described five cases with epileptic activation of the response to optokinetic flag or any other visual stimuli was occipital lobes. The ictal events included flashing observed. Pupils reacted normally to light and the ocular lights, visual hallucinations, fixation of gaze, oculo- on September 27, 2021 by guest. fundi were normal. When examined five hours after the clonic movements and visual field defects or blind- seizure, normal vision returned. Subsequent hospital ness. In one case, blindness preceded loss of con- course was slowly progressive with typical clinical and laboratory findings of subacute sclerosing parencephalitis. sciousness and in another it either preceded a grand mal seizure or occasionally followed it. Engel"' Discussion described a transient cortical blindness during pro- longed partial complex status epilepticus. In these Cortical blindness is associated with normal pupil- cases, the use of the term "ictal blindness" appears lary reactions and absence of abnormal findings on to be adequate since blindness occurred during an J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.46.6.566 on 1 June 1983. Downloaded from

568 Sadeh, Goldhammer, Kuritsky epileptic activity of the occipital lobes and rep- that the irreversibility of the blindness was due to resented an inhibitory seizure. Blindness may severe prolonged anoxia. Permanent postictal appear during any stage of the seizure as there is no blindness was described by Ashby and Stephenson distinct border between ictal or postictal inhibi- in one of their cases.' tion.'5 Anton's syndrome was present in at least three The hypothesis that postictal paralysis is caused patients and only one complained spontaneously of by an active inhibitory mechanism was first sug- visual loss. It is therefore possible that postictal gested by Gowers,'6 who objected to the "exhaus- blindness occurs more frequently than has been tion theory" that had been proposed by Todd." reported, going unrecognised with the accompany- Efron'5 criticised the vague term "exhaustion" and ing behavioural changes being attributed to postictal claimed that no evidence supporting this idea with- confusion. stood critical analysis. He offered further evidence for Gowers' arguments and suggested that a hyper- The authors acknowledge the critical comments and polarisation mechanism developing during the suggestions provided by Dr Lawrence Z Stern of the epileptic activity was responsible for ictal or postic- Department of Internal Medicine (Neurology), tal inhibition. This mechanism could well explain Arizona Health Sciences Center, The University of ictal blindness as well as a short transient postictal Arizona. blindness or hemianopia, such as those described by Kosnik et al.8 In their cases, vision loss for several hours was associated with focal seizures and epilep- tic discharges involving the occipital lobe. However, References this mechanism can not account for the relatively long duration of blindness following grand mal seiz- 'Ashby H, Stephenson S. Acute amaurosis following infantile convulsions. Lancet 1903;1:1294-6. ures or status epilepticus. 2 Pritchard E. Case of amaurosis following violent convul- Protected by copyright. Fiume' and later Olurin7 proposed intra-ictal sions. Proc R Soc Med 1918;11:1-2. anoxia as the basis for postictal blindness. During Mecca M. Reperti oculari nell'epilepssia essentiale con seizures, severe hypoxia, acidosis, rise in PCO2 and particolare riguardo allo spasmo vasale. Annali di lactic acidemia have been found.'8 These changes Ottalmologia e Clinica Oculistica 1938;66:457-69. are induced mostly by respiratory spasm, obstruc- 4 Fiume S. L'Amaurosi nell'epilessia. Riv Neurol 1958; tion of airways and muscular activity during the 28:43-55. seizure. Prevention of hypoxia and muscle contrac- Walsh FB, Hoyt WF. Clinical Neuro-opthalmology. Bal- tions timore: The Williams & Wilkins Co., 1969:127-29. by artificial respiration and curarization have 6 Barnet AB, Manson JI, Wilner E. Acute cerebral blind- markedly reduced the mortality of convulsing cats ness in childhood. Neurology (Minneap) 1970; and rats and avoided acidosis, accumulation of lactic 20:1147-56. acid and cardiac arrhythmias.'9 Adequate oxygena- Olurin 0. Cortical blindness following violent convul- tion enabled the brain temporarily to meet the sions and fever in Nigerian children. Pediatrics increasing energetic demands during seizures.20 1970;46:102-7. Brain anoxia could well account for the bilateral- 8 Kosnik E, Paulson GW, Laguna JF. Postictal blindness. ity of the blindness and its relatively long duration Neurology (Minneap) 1976;26:248-50. since the occipital lobes are especially sensitive to Thiebaut MF, Matavulj N. Hemianopsies passageres. http://jnnp.bmj.com/ in Rev Otoneuroopthalmol 1960;32:376-8. anoxia, being located the cerebral blood supply 10 Salmon JH. Transient postictal hemianopsia. Arch Oph- border zone.2' In the last two decades with the wide thalmol 1968;79:523-5. use of cardio-pulmonary resuscitation, many severe Penfield W, Erickson TC. Epilepsy and cerebral localisa- hypoxaemic episodes and hypotensive events have tion. Springfield: Charles C. Thomas, 1961. occurred. Not uncommonly they have been compli- 12 Strauss H. Paroxysmal blindness. Electroencephalogr cated with cortical blindness.622 Thus, anoxia has Clin Neurophysiol 1963;15:1047. been recently considered to be one of the most fre- 3 Huott AD, Madison DS, Niedermeyer E. Occipital lobe quent immediate causes of bilateral cortical blind- epilepsy. A clinical and electroencephalographic on September 27, 2021 by guest. ness.23 study. Eur Neurol 1974;11:325-39. In two of our the cerebral '4Engel J, Ludwig BI, Fetell M. Prolonged partial complex patients underlying dis- status epilepticus: EEG and behavioural observations. ease was focal and unilateral and therefore it is not Neurology (Minneap) 1978; 28:863-9. possible to relate directly the location of the lesions "5 Efron R. Post epileptic paralysis: Theoretical critique to the bilateral blindness. Four patients were blind and report of a case. Brain 1961;84:381-94. for hours or days after a single grand mal seizure. 16 Gowers WR. Epilepsy and other chronic convulsive dis- Patient 1 was permanently blind after status epilep- eases: their causes, symptoms and treatment. London: ticus lasting several hours, and it may be assumed Old Hickory Bookshop, 1901. J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.46.6.566 on 1 June 1983. Downloaded from

Postictal blindness in adults 569

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