Epilrpiru. 3otl):11f-l20. 1989 Raven Preas. Ltd.. New York i‘ International League Agan\t Eprlep)

Ictal Cortical Blindness with Permanent Visual Loss

Michael S. Aldrich, Chris W. Vanderzant, Anthony G. Alessi, Bassel Abou-Khalil, and J. Chris Sackellares

Depiirtment of Neitrology. Unitvrsiu of Michigan Medical Center, Ann Arbor, Michigan, U.S.A.

Summary: Cortical blindness is rarely an ictal manifesta- esthesias. When he was 32, transient blindness occurred tion. We report the case of a man who developed tran- during several days of repeated occipital . Per- sient cortical blindness followed by permanent visual def- manent left homonymous hemianopia, right homonymous icits during repeated partial seizures. Intermittent visual central scotoma, dyschromatopsia, and altered stereopsis impairment began at age 14 years. After he had the first followed these seizures. Brain CT demonstrated a new generalized at age 18 years, neurologic, ophthal- right occipital lesion. Partial seizures arising posteriorly mologic, angiographic. and brain computed tomographic may cause transient cortical blindness and result in per- (CT) examinations were normal. Several EEGs showed manent visual deficits. Key Words: -Partial sei- almost continuous biposterior spike-waves. Over the next zures--Blindness-- several years, frequent partial seizures were associated Electroencephalography. with transient visual loss and left body twitching or par-

Cortical blindness is an unusual manifestation of CASE REPORT epilepsy. Although children, and less commonly adults, may transiently be blind after generalized A 32-year-old, right-handed man had frequent ep- tonic-clonic seizures (Ashby and Stephenson, 1903; isodes of visual disturbance without be- Pritchard, 1918; Olurin, 1972; Kosnik et al., 1976; ginning at age 14 years. Depth perception would Sadeh et al., 1983), permanent postictal blindness is become distorted and objects appeared “wavy” or

rare (Radhakrishnan et al., 1979; Sadeh et al., “faded out. ” Occasional biposterior 1983). Ictal blindness has been reported in children were not associated with visual disturbance. There and adults (Russel and Whitty, 1955; Strauss, 1963; was no personal or family history of . Kooi, 1970; Huott et al., 1974; Engel et al., 1978; At age 28 years, first a generalized tonic-clonic Gastaut, 1982; Olivier et al., 1982), but permanent seizure occurred, followed by frequent partial sei- visual loss following partial seizures has not been zures associated with twitching movements or par- described except in occasional patients with preex- esthesias of the left arm or left leg. EEG showed isting visual deficits (Russel and Whitty, 1955; nearly continuous bioccipital and right posterior Kooi, 1970). We report a man with many years of temporal spikes and spike-and-slow-wave com- episodic visual disturbance who developed tran- plexes. Neurologic and ophthalmologic examina- sient cortical blindness during repeated partial sei- tions were normal, including , visual zures, followed by a permanent left homonymous fields by perimetry, and color vision. Brain com- hemianopia, right homonymous central scotoma, puted tomography (CT), (CSF) dyschromatopsia, and altered stereopsis. analysis, and carotid and vertebral angiography were normal. Transient visual difficulties and partial motor sei- zures continued despite treatment with Rhenvtoin Received July 1986; revision accepted April 1988. (PHT), phenobarbital (PB), primidone (PRM); and This work was presented in part at the American EEG Society meetings, Salt Lake City, Utah. September 1984. and published Or in He had in Elrcrromcephulogr Clin Neurophysiol 1985;61:37P. moments of almost total blindness and episodes of Address correspondence and reprint requests lo Dr. M. S. Al- darkened peripheral vision around a white drich at Department of . IS00 East Medical Center Dr.. 192010316 Taubman Center. Ann Arbor. MI48109-0316. spot. Reading was often difficult because print ‘‘ran U.S.A. together” or “disappeared.” During one examina-

I I6 ICTAL CORTICAL BLINDNESS 117

tion, his vision suddenly worsened, and he briefly eyes deviated to the left with clonic movements of had only light perception. Repeated EEGs all the left arm and leg. Ictal EEGs showed rhythmic showed nearly continuous biposterior epileptiform repetitive sharp waves and spikes beginning over activity (Fig. 1) unaffected by eye movements or the right posterior quadrant and spreading to in- reading. Pattern-reversal visual evoked potentials volve both hemispheres. Interictal EEGs demon- were normal. strated frequent bioccipital and left posterior tem- When the patient was 32, PRM dosage was ta- poral spikes and bursts of rhythmic slow waves. pered owing to complaints of sedation; PHT and Videotaped examinations, both ictal and interictal, VPA were continued. Ten days after PRM was dis- documented blindness with inability to count fin- continued, a right posterior headache developed as- gers, see hand motion, or identify objects or famil- sociated with a 45-min of “red flash- iar faces. ing lights’’ in the left hemifield. The following morn- Partial seizures continued despite oral carbarnaz- ing, a generalized tonic-clonic seizure occurred and epine (CBZ) and intravenous (i.v.) PHT and PB. he came to the hospital. When first examined, he Intubation and continuous i.v. diazepam were re- had repeated partial seizures with twitching of the quired on the second hospital day to control the right arm and jerking of the head and eyes to the left seizures. There were no episodes of or hy- without altered consciousness or disorientation. A potension. After extubation, on the seventh day, he dense left homonymous hemianopia and impaired had confusion, right gaze preference, sensory ex- voluntary gaze to the right were apparent. Vision tinction on the left, and occasional of was limited to finger counting in the right upper “cartoon figures.” Repeated examinations demon- quadrant and possible hand motion and light per- strated visual acuity <20/400 O.U., left homony- ception in the right lower quadrant. Intermittently, mous hemianopia, and intermittent ability to count his eyes deviated tonically to the right side. Serum fingers in the right visual field. He could not read electrolytes and CSF analysis were normal. Serum the Ishihara color control card. Brain CT on the 9th PHT level was 16.5 pghl and VPA level was 31.7 day and radionuclide cerebral blood flow scan on CLdml. the eighteenth day were normal. By the 18th hospi- Repeated partial seizures occurred over the next tal day, he was alert and oriented. Repeat EEG 6 days, but there were no additional generalized showed frequent biposterior spikes and spike- seizures. EEG with closed circuit television moni- waves with right posterior temporal emphasis. toring on the first hospital day recorded many sei- Two months after discharge, he still had simple zures of 40-150-s duration during which head and partial seizures with left-sided jerking, and white

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T*”1- FIG. 1. lctal EEG showing continuous serial spikes and spike-and-slow-waves over both posterior regions. Epilepsia, Vol. 30, No. I. 1989 118 M. S. ALDRICH ET AL. spots appeared frequently in the left visual field. A partial seizures. Although up to 20% of epileptic dense left homonymous hemianopia persisted, vi- patients with occipital EEG foci may have visual sual acuity was 20/60 O.U., and stereopsis was field deficits (Ludwig and Marsan, 1975), these def- poor. He could read the Ishihara color control card icits usually predate the seizures. Gastaut (1982) but none of the 10 test cards. Repeat brain CT emphasized the benign character of partial epilepsy showed a right medial occipital lucency (Fig. 2). of childhood associated with occipital spike-waves: Two years later, seizure control was improved 92% of cases had complete remission of seizures by with a regimen of PHT, VPA, and PB. There were age 19 years. A benign course was also reported in no motor or sensory symptoms, but involuntary four adolescents with seizures, basilar migraine, jerking of the eyes occurred occasionally. Visual and occipital spike-waves (Camfield et al., 1978). acuity was 20/40 O.U. Perimetry demonstrated a Our report documents that such cases are not lim- complete left homonymous hemianopia and a small ited to childhood and that the prognosis is not uni- homonymous right central scotoma (Fig. 3). Color formly benign. vision was unchanged. EEG showed frequent Our patient shows some clinical similarities to pa- spikes and spike-waves occurring mainly over the tients with the syndrome of mitochondrial myopa- left parietal-occipital and posterior temporal re- thy, , lactic acidosis, and - gions. like episodes (MELAS) (Pavlakis et al., 1984). A variant of this syndrome has been reported in which DISCUSSION mitochondria1 encephalopathy and are as- sociated with migraine headaches and occipital sei- This man’s epilepsy is unusual because (a) tran- zures (Dvorkin et a]., 1987), and several of the pa- sient blindness occurred both ictally and postic- :ients developed permanent hemianopias or cortical tally; (b) fixed visual deficits, including dyschro- blindness. Although our patient did not have head- matopsia, occurred after protracted occipital sei- aches with his episodes of visual disturbance, or the zures; and (c) CT evidence of right occipital short stature typical of MELAS syndrome, his con- encephalomalacia eventually developed. Acquired dition may be related to these syndromes. We did dyschromatopsia is a rare phenomenon which is not measure serum or CSF lactic acid and did not usually caused by bilateral lesions involving the in- obtain a muscle biopsy to look for ragged red fibers. ferior occipital regions (Meadows, 1974; Green and The cause of the permanent visual loss cannot be Lessell, 1977). Also striking was the high incidence stated with certainty. In two reported cases of per- of biposterior epileptiform activity on every EEG manent blindness following generalized seizures, obtained over several years. blindness was attributed to the effects of anoxia The onset of permanent left homonymous hemi- (Radhakrishnan et al., 1979; Sadeh et al., 1983). Our anopia and right homonymous superior central patient had no evidence of an anoxic or hypotensive scotoma coincided with the prolonged period of episode and had only one generalized tonic-clonic seizure. Although infarction resulting from vaso- spasm or vascular occlusion may have induced the prolonged partial seizures, the previously normal angiogram and the absence of migraine or other risk factors for make this pos- sibility unlikely. Furthermore, evidence of infarc- tion would usually be apparent on brain CT by the ninth hospital day, yet CT of our patient was normal at that time. Not until 2 months later did the CT lesion appear. That the repeated occipital seizures induced irre- versible cerebral damage from metabolic compro- mise is more likely. Primate studies have shown that prolonged seizures can produce neuronal dam- age independent of the effects of hypotension, aci- dosis, and hypoxia (Meldrum and Brierly, 1973). Moreover, a preexisting epileptogenic focus may have rendered the patient’s occipital cortex more vulnerable to the deleterious metabolic effects of FIG. 2. Brain computed tomography, 2 months after dis- charge from hospital, shows an area of right medial occipital repeated seizures. encephalomalacia. Although the ictal manifestations of epilepsy are Epilepsiu. Vol. 30. No. 1. 1989 ICTAL CORTICAL BLINDNESS 119

LEFT RIGHT 20/60 20/60

Vle FIG. 3. Visual fields 2 years after discharge from hospital, showing a left homonymous hemianopia and right central scotoma.

usually brief, clinical and EEG data suggest that our childhood with occipital spike-waves. Clin Electroencepha- patient's episodes of prolonged but reversible visual logr 1982;I3;13-22. Green GJ, Lessell S. Acquired cerebral dyschromatopsia. Arch disturbance occurring during 18 years were almost Ophthalmol 1977;95:121-8. continuous simple partial seizures involving occip- Huott AD, Madison DS, Niedermeyer E. Occipital lobe epi- ital cortex. Our report underscores the fact that vi- lepsy. A clinical and electroencephalographic study. Eur Neurol 1974;11:325-39. sual hallucinations, reading impairment, or cortical Kooi K. Episodic blindness as a late effect of head trauma. Elec- blindness may all be manifestations of epileptic sei- trophysiological study of 3 cases. Neurology 1970;20:569-73. zures. Occipital seizures should be considered in Kosnik E, Paulson GW, Laguna JF. Postictal blindness. Neu- the differential diagnosis of any patient with inter- rology 1976;26:248-50. Ludwig BI, Ajmone-Marsan C. Clinical ictal patterns in epileptic mittent or fluctuating visual complaints. The poten- patients with occipital electroencephalographic foci. Neurol- tial for partial seizures to lead to permanent cere- ogy 1975;25:463-71. bral damage extending beyond the region of the Meadows JC. Disturbed perception of colours associated with original epileptogenic focus emphasizes the need localized cerebral lesions. Brain 1974;97:615-32. Meldrum BS, Brierly JB. Prolonged epileptic seizures in pri- for prompt treatment of frequent or protracted par- mates. Arch Neurol 1973;28:10-7. tial seizures. Olivier A, Gloor P, Andermann F, Ives J. Occipitotemporal ep- ilepsy studied with stereotaxically implanted depth elec- trodes and successfully treated by temporal resection. Ann REFERENCES Neurol 1982; I I :428-32. Olurin 0. Cortical blindness following convulsions and fever in Ashby H, Stephenson S. Acute amaurosis following infantile Nigerian children. Sum Ophthalmol 1972;16:341-2. convulsions. Lancet 1903;l:1294-6. Pavlakis SG, Phillips PC, DiMauro S. et al. Mitochondrial my- Camfield PR, Metrakos K, Andermann F. Basilar migraine, sei- opathy, encephalopathy. lactic acidosis, and stroke-like epi- zures, and severe epileptiform EEG abnormalities. Neurol- sodes: a distinctive clinical syndrome. Ann Neurol 1984; ogy 1978;28:584-8. 16:481-8. Dvorkin GS, Andermann F, Carpenter S, et al. Classical mi- Pritchard E. Cases of amaurosis following violent convulsions. graine, intractable epilepsy and multiple strokes: a syndrome Proc Soc R Med 1918;11:1-2. related to mitochondria1 encephalomyopathy. In: Andermann F, Lugaresi E, eds. Migraine and epilepsy. Boston: Butter- Radhakrishnan K, Razdan S, Chopra JS. Postictal blindness. worths 1987:203-32. Neurology (India) 1979;27:196-8. Engel J, Ludwig BI, Fetell M. Prolonged partial complex status Russel WR. Whitty CWM. Studies in traumatic epilepsy. 3. Vi- epilepticus: EEG and behavioral observations. Neurology sual fits. J Neurol Neurosurg Psychiatry 1955;18:79-%. 1978;28:863-9. Sadeh M. Goldhammer Y, Kuritsky A. Postictal blindness in Gastaut H. A new type of epilepsy: benign partial epilepsy of adults. J Neurol Neurosurg Psychiatry 1983;46:566-9.

Epilepsia, Vol. 30, No. I, 1989 I20 M. S. ALDRICH ET AL.

Strauss H. Paroxysmal blindness. Elecrroencephulogr Clin Neu- neurol6gicos, oftalmol6gicos, angiograficos y de tomografia rophysiol 1%3; 1 S:92 1. computarizada fueron normales. Varios EEGs mostraron com- plejos biposteriores punta-onda, practicamente continuos. Du- rante 10s siguientes anos ataques parciales frecuentes se asocia- RkSUME ron a perdida de vision transitoria y parestesias o movimientos La cecite corticale est rarement une manifestation critique. en el lado izquierdo de su cuerpo. A la edad de 32 anos tuvo una Les auteurs rapportent I’observation d’un homme qui a present6 ceguera transitoria durante varios dias de ataques occipitales une cecite cortical transitoire suivie de deficits visuels perma- repetidos. Estos ataques se siguieron de una hemianopsia nents a I’occasion de crises partielles rtp6tees. Les episodes de hom6nima izquierda permanente, escotoma central hom6nimo gene visuelle intermittente ont commence a I’lge de 14 ans. derecho, discromatopsia y estereopsia alterada. La CT cerebral Apres une premiere crise gtneralisee a I’lge de 28 ans, un bilan mostro una lesi6n occipital derecha nueva. Los ataques parciales complementaire comprenant examen neurologique, ophtal- que se originan en regiones posteriores pueden causar ceguera mologique, angiographie et scannographie ckrtbrale s’est rkvkle cortical transitoria y producir deficits permanentes. normal. Les EEG successifs ont montre I’exsistence de pointes- (A. Portera-Sanchez, Madrid) ondes biposterieures pratiquement continues. Pendant les an- nees suivantes. le patient a prksente de nombreuses crises par- tielles avec perte transitoire de la vision et secousses ou pares- ZUSAMMENFASSUNG thesies de I’hemicorps gauche. A I’lge de 32 ans. une cCcite Kortikale Blindheit gilt als seltenes Anfallssymptom. Wir ber- transitoire se produisit lI’occasion de crises occipitales rep6tCes ichten uber einen Patienten mit transienter kortikaler Blindheit, pendant plusieurs jours. Aprks ces crises, on a constate la per- die von bleibender Vissusstorung gefolgt wurde. Eine intermit- sistance d’une hemianopsie homonyme gauche, d’une scotome tierende visuelle Verschlechterung stellte sich mit 14 Jahren ein. central homonyme droit, d’une dyschromatopsie et d’une alter- Nach einem ersten generalisierten Anfall im Alter von 28 Jahren ation de la vision stereoscopique. La scannographie cerebrale a fanden sich neurologisch, ophthalmologisch, angio- und comput- mis en evidence une lesion occipitale droite rkcente. Les crises ertomographischein Normalbefunde. Mehrere EEG’s zeigten partielles posterieures peuvent entrainer une cecite corticale kontinuierliche biposteriore spike-wave. In den folgenden Jahren transitoire et kvoluer vers des deficits visuels permanents. waren haufige Partialanfalle mil voriibergehendem Visusverlust (P. Genton, Marseille) und linksseitigen Korperzuckungen und Paraesthesien ver- bunden. Mit 32 Jahren trat eine vorubergehende Blindheit wahrend mehrerer Tage bei wiederholten Anfallen auf. Darauf RESUMEN stellte sich eine homonyme Hemianopsie links, ein homonymes La ceguera cortical es una rara manifestaci6n de 10s ataques. Zentralskotum rechts, Dyschromasie and verindertes Stereose- Se publica el caso de un hombre que desarroll6 ceguera cortical hen ein. Das CT zeigte eine frische occipitale Laesion rechts. transitoria seguida de un dtficit visual permanente durante Partialanfalle mit occipitalem Ursprung konnen voriibergehende ataques parciales repetidos. Los ataques de trastorno visual in- kortikale Blindheit verursachen und in eine permanente termitente empezaron a la edad de 14 anos. DespuBs de un Visuseinbupe munden. primer ataque generalizado a la edad de 28 anos 10s estudios (C. G. Lipinski, HeidelberglNeckargemund)

Epilepsia, Vol. 30, No. 1. 1989