Journal of Neuro-Ophthalmology, September 1991, Volume 11, Issue 3

Delayed oculogyric crises associated with striatocapsular infarction.

Oculogyric crises are dystonic, usually upward, conjugate eye deviations. We describe an 11-year-old girl who developed oculogyric crises 3 1/2 years after infarction of the right caudate, putamen, and internal capsule. Her abnormal eye movements responded to anticholinergic agents. This is the first reported association between oculogyric crises and striatocapsular infarction.

Journal of Clinical Neuro- ophthalmo[ ogy 11( 3): 198- 201,1991. Delayed Oculogyric Crises Associated with Striatocapsular Infarction Grant T. Liu, M. D., Enrique J. Carrazana, M. D., Jeffrey D. Macklis, M. D., and Mohamad A. Mikati, M. D., F. C. P. © 1991 Raven Press, Ltd., New York Oculogyric crises are dystonic, usually upward, conju­gate eye deviations. We describe an ll- year- old girl who developed oculogyric crises 31/ 2 years after infarction of the right caudate, putamen, and internal capsule. Her abnormal eye movements responded to anticholinergic agents. This is the first reported association between oc­ulogyric crises and striatocapsular infarction. Key Words: Basal ganglia- Dystonia- Oculogyric cri­ses- Stroke- Varicella. From the Department of Neurology, Children's Hospital and the Harvard- Longwood Neurology Program, Harvard Medical School, Boston, Massachusetts, U. S. A. Please address correspondence and reprint requests to Dr. Liu at Division of Neurology, Brigham and Women's Hospital, 7~ !=,~!" j< Sh" pet Boston. MA 02115. 198 Oculogyric crises are a dystonia of ocular mus­cles ( 1) characterized by dramatic involuntary con­jugate deviation of the eyes, usually upward, last­ing seconds to hours ( 2,3). Frequently they are accompanied by other dystonic or dyskinetic movements such as tongue protrusion, lip smack­ing, blepharospasm, choreoathetosis and an­terocollis ( 4- 7). In the 1920s the French first de­scribed crises ( crises oculogyres) as complications of postencephalitic parkinsonism ( 2,8), but today such crises are thought to be usually acute ( 3) or tardive ( 4- 7) extrapyramidal reactions to neurolep­tics. In contrast, we describe a patient who devel­oped a striatocapsular infarction and delayed ocu­logyric crises, a heretofore unreported association. CASE REPORT An ll- year- old right- handed girl presented to the neurology service complaining of uncontrolla­ble annoying eye movements. She was born with a double outlet right ventricle and pulmonary steno­sis. At age 5 she underwent a right atrium to pul­monary artery anastomosis and intra- atrial baffling of the pulmonary venous return ( modified Fontan procedure). Her postoperative course was compli­cated by persistent pericardial and pleural effu­sions, requiring chronic steroid treatment and Denver pleuroperitoneaI shunting. At age 7 years, S months, while still on steroids, she contracted a primary varicella infection treated with acyclovir. Two months later she developed a right frontal headache, then acute weakness of the left face, arm, and leg. Initially, computerized to­mography of the brain was normal; however, a repeated study 11 days later documented an in­farction in the right basal ganglia and internal cap­sule. A work- up for the etiology of her stroke was unrevealing. OCULOGYRIC CRISES 199 Within weeks her hemiparesis improved, al­though she developed dystonic posturing of the left hand, marked by finger hyperextension that was worsened by hand or finger activity. There was no family history of dystonia or tics. Eight months after the stroke, the posturing spontane­ously resolved, and the hemiparesis was mild. At age 11 she began experiencing rolling eye movements which gradually increased in fre­quency and intensity over 4 days and interfered with her schoolwork and play. She had no head­ache, diplopia, or abnormal thoughts. The eye movements were not preceded by any irresistable urge, and she did not exhibit coprolalia, echolalia, stuttering, or spasmodic dysphonia. Her medica­tions included aspirin and Inderal for migraine headaches, and Lasix, Aldactone, and digoxin. Her neurological exam was remarkable for left hemihypoplasia, hemiparesis, and hyperreflexia. There was neither dysmetria nor gait ataxia. Visual acuity and fields and funduscopic exam were nor­mal. Her pupils were equal and normally reactive. Eye movements. Primary gaze was conjugate, and vergence movements were normal. Randomly and independent of position, her eyes deviated conju­gately upward and obliquely to the right or left. The episodes lasted 1- 3 seconds, and then her eyes returned to primary position. During a timed typical I- minute period, S events were oblique to the right, and 6 were oblique to the left. She was unable to suppress them, and they persisted dur­ing venipuncture while she cried. During observa­tion without her awareness, the eye movements continued. Frequently she manifested brief left and rightward spasmodic, dystonic head turning which was temporally and directionally unrelated to her abnormal eye deviations. Electroencephalography ( EEG) during the eye movements showed no epileptiform activity. Sed­imentation rate, antinuclear and antistreptolysin­a antibodies, copper and ceruloplasmin levels, and cerebrospinal fluid and toxicology screen were all normal. Magnetic resonance imaging of the head demonstrated the old infarction within the right caudate, putamen, and internal capsule ( Fig. 1). The patient was given intramuscular placebo ( 3 cc 0.9% NaCl) and then Benadryl ( 50 mg/ 3cc 0.9% NaCl). The eye movements did not respond to pla­cebo despite suggestion. However they responded to Benadryl gradually over 10- 15 minutes. Their rate decreased from &- 10 movements per minute to 0- 1 per minute. Cogentin ( 0.5 mg bid) was pre­scribed. Two months later eye movements contin­ued to occur at a rate of 2 or 3 per hour, and the FIG. 1. Cranial magnetic resonance imaging ( TR 2000 msec, TE = 80 msec) revealed previous infarc­tion of the right internal capsule, caudate, and puta­men ( arrow). head turning resolved. Five months after their on­set, the eye deviations were completely controlled. After a subsequent taper- off of Cogentin, the ab­normal eye movements recurred; therefore, the Cogentin was restarted. DISCUSSION Our patient developed a right striatocapsular in­farction either from a cardiac- source embolus in the setting of congenital heart disease ( 9) or a varicella- related delayed vasculopathy, which typ­ically involves the lenticulostriate arteries 1- 3 months after the initial infection ( 10). However, neither etiology could be verified with certainty. Her spasmodic involuntary upward eye move­ments 31/ 2 years later are reminiscent of oculogyric crises ( 2,3), and the head turning ( which we feel was torticollis), as well as the response to anti­cholinergic agents, suggest such eye movements were indeed dystoniC ocular deviations. They probably are not hysterical, because the eye move­ments persisted while she was unaware of obser­vation and while she cried during venipuncture, and they did not respond to administration of pla­cebo. The absence of a repetitive to- and- fro quality makes it unlikely that they are nystagmus, and they are too slow to be considered ocular flutter, opsoclonus, or ocular myoclonus. Reverse ocular bobbing is primarily vertical rather than oblique JClin Neuro- ophthalmol, Vol. 11, No. 3, 1991 200 G. T. LIU ET AL. and occurs in comatose patients ( 11). Dysmetric overshoot is related to pursuit. The normal EEG during the eye deviations rules out epilepsy. Dystonic eye rolling and torticollis have been de­scribed in tic disorders ( 12- 14), but for several rea­sons we feel that that is an unlikely possibility in our patient. Tourette's syndrome is inherited, and children with this disorder have vocal tics and be­havioral disturbances in addition to motor tics ( 14). Ninety percent of Tourette's patients develop their first motor tic by age 10 ( 15). Isolated motor tics can be idiopathic or secondary to drugs or basal gan­glia injury (' acquired tourettism') ( 14). Unlike most individuals with tics, however, the patient lacked a typical preceding irresistible urge and an ability to suppress the eye movements ( 14). Furthermore, tics respond to dopamine antagonists, whereas our patient's eye movements improved with Co­gentin, which worsens tics ( 14). Although oculogyric crises are normally associ­ated with a thought or emotional disturbance, are preceded by a brief stare, and last for hours ( 2,3,5,8), they can occur without these features and last only a few seconds ( 2,3,6). The eyes usu­ally deviate straight upwards or up and to the left or right, and the position can change from crisis to crisis ( 2). Oculogyric crisis are features of symp­tomatic rather than idiopathic dystonias ( 1). In ad­dition to postencephalitic parkinsonism, oculogy­ric crisis have been associated with Parkinson's disease ( 16), familial Parkinson- dementia syn­drome ( 17), neurosyphilis, multiple sclerosis ( 18), ataxia- telangiectasia ( 19), Rett's syndrome ( 20), cerebellar disease, trauma ( 21), acute herpetic brain stern encephalitis ( 22), and a third ventricular cystic glioma ( 23). Our patient had none of these disorders. Besides neuroleptics ( 3- 7,24), Tegretol ( 25), tetrabenazine ( 26), and several other drugs ( 21) can cause oculogyric crisis, but our patient did not use any of these. Hereditary risk factors for dystonia such as Wilson's disease, Huntington's disease, Hallervorden- Spatz disease, and meta­bolic disorders ( 1) were notably absent. Rather, we attribute our patient's oculogyric cri­ses and torticollis to delayed effects of her original striatocapsular injury. Acutely she acquired a left hemiparesis and subacutely a dystonic posturing of the left arm, both of which eventually resolved. Contralateral focal and hemidystonias are known sequela of striatal infarction ( 27). As in our patient, dystonias can manifest themselves several years after a nonprogressive cerebral insult, perhaps as a result of aberrant neuronal sprouting ( 28). In ad­dition, caudate lesions can cause torticollis ( 27). To our knowledge oculogyric crises have not previ-ously been associated with basal ganglia infarc­tion. Injury to the striatum can cause dystonias by interrupting extrapyramidal pathways from frontal association, premotor, and sensorimotor cortices to globus pallidus and substantia nigra, both of which then send fibers to the ventrolateral thala­mus ( 29). Unfortunately we are unable to explain how a unilateral basal ganglia lesion can lead to upward and leftward as well as rightward devia­tion or to torticollis with bilateral head turning. The direction of the torticollis is usually only con­tralateral to the lesion ( 27). The etiology of oculogyric crises is not certain. In postencephalitic patients, some authors believe they result from a release of supranuclear control of oculomotor centers due to injury to the corpus striatum or subthalamic nucleus ( 2). Onuaguluchi ( 8) hypothesized that these crises were due to an abnormal vestibulo- ocular reflex in the setting of brain stern lesions involving vestibular pathways. Leigh et a1. ( 5) attributed the deviations to an in­correct efference copy of eye position. Based on the response to anticholinergic agents in neurolep­tic- induced crises, they alternatively invoked a de­fect in mesencephalic vertical gaze- holding mech­anisms normally dependent on balanced cholin­ergic and dopaminergic systems ( 5). In our patient the association between oculogyric crises and a le­sion in the striatum implies that this balance may be mediated within the cortico- striato- pallido/ nigral- thalamic loop ( 27,29). The findings in our patient suggest that striato­capsular infarction should be added to the list of disorders associated with oculogyric crises. REFERENCES 1. Fahn 5, Marsden CD, Caine DB. Oassification and inves­tigation of dystonia. In: Marsden CD, Fahn 5, eds. Move­mrnt disorders 2. London: Butterworths, 1987: 332- 58. 2. McCowan PK, Cook LC, Cantab BA. Oculogyric crises in chromc epIdemic encephalitis. Brain 1928; 51: 285-- 309. 3. Miller NR. Walsh and Hoyt's clinical neuro- ophthalmology. 4th ed. Baltimore: Williams & Wilkins, 1985: 7~ 1. 4. Munetz MR. Oculogyric crises and tardive dyskinesia. Am J Psychiatry 1980; 137: 1628. 5. Leigh RJ, Foley JM, Rernler BF, Civil RH. Oculogyric crisis: a syndrome of thought disorder and ocular deviation. 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JCIin Nellra- ophtha/ ma/' Va/. 11, No. 3, 1991