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Show journal of Natro- Ophthalmohxn 14(. 3): 160- 162, 1994. *',• 1994 Raven Press, Ltd., New York Involuntary Ocular Deviations and Generalized Dystonia in Multiple Sclerosis A Case Report Jason J. S. Barton, M. D., F. R. C. P. C, Terry A. Cox, M. D., F. R. C. S. C, and Donald A. Calne, M. D., F. R. C. P. C. A man with chronic relapsing multiple sclerosis developed involuntary ocular deviations, blepharospasm, torticollis, and chorea of the right arm and head during relapses precipitated by urinary tract infections. An MR scan showed lesions adjacent to the right caudate and lentiform nuclei. Key Words: Multiple sclerosis- Involuntary ocular deviations- Internuclear ophthalmoplegia. Movement disorders in multiple sclerosis ( MS) are unusual. Paroxysmal dystonias, or tonic spasms, are most frequently described, although pathophysiological^ they probably differ from other movement disorders ( 1,2). Among nonparoxysmal forms ballismus ( 3), chorea ( 4,5), dystonia ( 6), and blepharospasm ( 7) have been reported. We describe a case of limb and craniocervical dystonia associated with involuntary ocular deviations. From the Division of Neurology, University of British Columbia, Vancouver, British Columbia, Canada. Dr. Barton is currently at the Department of Neurology, University of Toronto, Ontario, Canada. Address correspondence and reprint requests to Dr. Jason J. S. Barton, Department of Neurology, Toronto Western Hospital, 399 Bathurst Street, Toronto, Ontario, Canada M5T 2S8. CASE REPORT A 59- year- old man had relapsing and remitting MS starting at age 32 with left leg weakness and imbalance. Since age 44 he had a chronic relapsing course with episodes of incontinence, internuclear ophthalmoplegia and fatigue superimposed on chronic weakness in the left arm and both legs, culminating in tetraplegia for the last 10 years. In the last year he had several admissions with fever, confusion, and right arm weakness associated with urinary tract infections. Each time he was noted to have continuous involuntary movements of his head, which resolved as the infection was treated and other symptoms abated. He was admitted 2 months after his last bout because of increasing right arm weakness and clumsiness and slurred speech. Medications included baclofen, ferrous gluconate, ascorbic acid, folate, triazolam, pilocarpine eyedrops, and Dulcolax. He had never used neuroleptics, metaclopramide, carbamazepine, or lithium. On admission he had spastic dysarthria, mild right internuclear ophthalmoplegia, decreased oc- 260 INVOLUNTARY OCULAR DEVIATIONS IN MS 161 ular pursuit, dysmetric saccades, and left facial weakness. Mild choreiform head movements were present. He had spastic tetraplegia with pyramidal weakness of the right arm. Vibration and joint position sense were abnormal in all limbs, and reflexes were increased with upgoing plantar responses. He had a condom catheter system. The day after admission he had a seizure with unresponsiveness, right head deviation, and right arm twitching. Computed tomography ( CT) scan showed periventricular low densities and suggested mild cortical tissue loss. Phenytoin was started intravenously. The next day he developed a fever and leukocytosis, and a urinary tract infection with Streptococcus faecalis was found. He was confused, swore, and reported auditory and visual hallucinations, often with paranoid ideation. His involuntary head movements increased in vigor with added torticollis, most frequently to the right, spontaneous blepharospasm, and dystonic movements in his right arm. Involuntary ocular deviations most often to the right or upward were seen. These were fast movements followed by brief periods of sustained eccentric position for 1 to 3 seconds. He was able to suppress both head and ocular involuntary movements partially for periods of 10 to 20 seconds. Movements were almost continuous while he was awake. Treatment with ampicillin and nozinan was followed by resolution of fever, confusion, and involuntary movements after 1 week. When questioned afterward, he denied manic, depressive, or obsessive features. An electroencephalogram ( EEG) during the movements showed abnormal slowing in the fron-totemporal regions but no epileptiform abnormalities. Cerebrospinal fluid showed normal pressure, 4 white cells, 6 red cells, normal glucose, and mildly elevated protein at 0.689 g/ L. Serum ammonia, bilirubin, alkaline phosphatase, lactate dehydrogenase, and VDRL were normal. Magnetic resonance imaging showed multiple periventricular lesions, a lesion near the right caudate head, and a right parietal lesion adjacent to the lenticular nucleus ( Fig. 1). No lesions were seen in the brainstem. DISCUSSION Nonparoxysmal movement disorders in MS are rare. Hemiballismus was described in 1930 by Guillain and Mollaret ( 3) and by Mouren and associates ( 8), who found plaques in the contralateral subthalamic and red nuclei. Chorea is more fre- FIG. 1. MR image ( TR 2800, TE 20). High- intensity signal in white matter adjacent to the posterior lentiform nucleus on the right. quently described ( 4,5,9- 12), although the ocular motor findings if any are seldom mentioned. Mao and colleagues ( 10) had one patient with limb chorea and ocular ataxia, nystagmus, and slowed sac-cades. Dystonia in MS has been the subject of few reports ( 6,7,12- 14), some of which mention ocular motor findings. Jankovic and Patel ( 7) reported two patients in whom blepharospasm developed in association with an INO. One of Bachman's cases had generalized dystonia with normal eye movements ( 12). Recently Coleman and colleagues ( 14) described two patients whose first symptoms were writer's cramp and left hemidystonia: both had nystagmus, and the first had bilateral internu-clear ophthalmoplegia ( 14). Our patient had clinically definite MS by history and examination ( 15). He had dystonic and choreiform movements of the head, neck, and right arm, suggesting a generalized form of dystonia that may have been masked in the other limbs by tetraplegia. His movement disorder occurred with exacerbations of other MS symptoms brought on by infections and subsiding with antibiotics. He had no prior exposure to drugs associated with dystonic reactions. Although he had a seizure prior to the increase in dystonia during this admission, EEG did not show ongoing seizure activity during these movements. Although phenytoin may cause movement disorders, the occurrence of similar movements during previous hospitaliza- ; Neuro- Ophthalmol, Vol 14, No. 3, 2994 162 ]. /. S. BARTON ET AL. tion, the chorea evident prior to his seizure, and the abating of his symptoms despite continuing phenytoin would imply that the drug did not cause his symptoms. The involuntary ocular deviations were reminiscent of oculogyric crises, except that they were continuous while awake. Some texts state that such deviations can occur in MS ( 16,17), but we have been unable to find a description in the original references ( 18,19). None of the reports mentioned above noted ocular deviations. Oculogyric crises are a feature of postencephalitic parkinsonism, but today are seen most commonly in acute dystonic or tardive reactions from neuroleptics or similar drugs. The onset of oculogyric movements has been associated with mood changes, obsessive thought disorders, and compulsive behavior ( 20,21). Other disorders with ocular deviations include Rett syndrome ( 22), ataxia- telangectasia ( 23), and neurosyphilis ( 19). Tourette's syndrome has been associated with involuntary oculogyric movements and blepharo- tics ( 24). However, no tics were seen in our patient, and his obscenities did not have the tic- like distortions of Tourette's coprolalia, but seemed consistent with his delirium. He also lacked the obsessive- compulsive features seen in Tourette's syndrome. For these reasons we felt that there was insufficient evidence to diagnose acquired tourettism. It is possible that involuntary ocular deviations may be underreported rather than nonexistent in dystonia. These must not be confused with compensatory vestibulo- ocular responses during head turning, which may resemble ocular deviation. Jankovic and Patel ( 7) have pointed out that Bell's phenomenon occurring in blepharospasm may also be confused with true eye deviation. Likewise, patients who cannot inhibit reflex visual sac-cades may appear to have involuntary ocular deviations ( 25). The anatomic substrate of involuntary ocular deviations is unknown. Dopaminergic pathways are suggested by its occurrence in neuroleptic- induced states. Its occurrence in postencephalitic parkinsonism also suggests a midbrain site involving the pars reticulata of the substantia nigra and the ventral tegmental area ( 19). The MR image in our patient did not reveal any midbrain abnormality, although his internuclear ophthalmoplegia indicates some brainstem pathology. It is unclear what role the lesions adjacent to his lenticular and caudate nuclei might play. Mao's group ( 10) studied a patient with chorea who also had similar lesions in the vicinity of the caudate nuclei. REFERENCES 1. Zeldowicz L. Paroxysmal motor episodes as early manifestations of multiple sclerosis. Can Med Assoc / 1961; 84: 937- 41. 2. Berger R, Sheremata WA, Melamed E. Paroxysmal dystonia as the initial manifestation of multiple sclerosis. Arch Neurol 198441: 747- 50. 3. Guillain G, Mollaret P. 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Neurology 1983; 33: 1268- 75. / Ncuw- Ophthalmol. Vol. 14, Nu. .3, 1994 |
