Posttraumatic paroxysmal nocturnal hemidystonia

Movement Disorders v0i. 9, NO. 1, 1994, pp. 9a99 0 1994 Movement Disorder Society

Brief Report

Posttraumatic Paroxysmal Nocturnal Hemidystonia N. Biary, B. Singh, Y. Bahou, S. M. A1 Deeb, and H. Sharif Department of Clinical Neurosciences, Riyadh Armed Forces Hospital, Riyadh, Saudi Arabia

Summary: The term “nocturnal paroxysmal dystonia” has been used to describe pa-

tients who display paroxysmal episodes of dystonic-dyskinetic movements arising during nonrapid eye movement sleep, in particular stages 2-3 (Lugaresi E, Cirignotta F. Hypnogenic paroxysmal dystonia: epileptic seizure or a new syndrome. Sleep 1981 ;4: 129-138). The pathogenesis of these attacks has remained controversial. We describe a patient with posttraumatic paroxysmal nocturnal hemidystonia. Acetazolamide led to improvement. Key Words: Nocturnal paroxysmal dystonia-Acetazolamide-Trauma.

creased to 250 mg twice daily, resulting in a significant reduction of the severity of the attacks as well as their frequency to once every other night.

CASE REPORT A 15-year-old boy was hit by a car at the age of 4 years. He sustained a head injury, was comatose for 24 h, and had a right hemiplegia that improved during 1 year. A few months after the injury he developed episodes of exclusively nocturnal abnormal movements on the right side without enuresis or tongue biting. Examination revealed a minimal right hemiparesis with hyperreflexia and Babinski’s sign. The right upper and lower limbs were smaller and 1 cm shorter than the left. The rest of the neurological examination results were normal. Electroencephalographic (EEG) and computed tomography (CT) brain scan findings were normal. Magnetic resonance (MR) imaging of the brain revealed two small areas of high-signal intensity on the heavily weighted T2 image in the left posterior putamen (Fig. 1). Routine laboratory tests, including screening for Wilson’s disease and brucellosis, were negative. Polysomnographic monitoring under audiovisual control revealed episodes of nocturnal right hemidystonia lasting 3-12 min each, occurring two to five times per night during nonrapid eye movement (NREM) sleep with normal EEG findings. During the attacks, he had extension of the right wrist and fingers with fanning and writhing movements of the fingers. His right foot was flexed and inverted (see videotape). Trihexyphenidyl, baclofen, and carbamazepine were not effective. Acetazolamide therapy, 250 mg daily, was begun and the dose was in-

DISCUSSION Nocturnal paroxysmal dystonias (NPD) are charactensed by short- or long-lasting attacks. The former are more common, last up to 2 min, occur during NREM sleep, and show a good response to carbamazepine (1). In three cases EEG has shown clear-cut evidence of a frontal lobe origin of the seizures (2), and a positron emission tomography scan in one further patient disclosed focal areas of hypometabolism localized in the frontal or frontotemporal lobes (3). Nine other patients with shortlasting NPD had no distinguishable clinical features compared with patients with nocturnal motor attacks of known epileptic origin or with daytime frontal lobe seizures (4). NPD with long-lasting attacks (2-50 min) was reported in two patients (1) with normal EEG findings and unresponsiveness to anticonvulsant drugs. One patient later developed Huntington’s chorea, suggesting a sleeprelated movement disorder rather than an epileptic process. Our case had some similarity with the long-lasting NPD, having a normal EEG and being refractory to carbamazepine; however, it was posttraumatic and not spontaneous. Trauma has been reported to induce paroxysmal choreoathetotic-dystonic movements. Robin ( 5 ) described a 33-year-old man with left-sided attacks of choreoathetotic movements lasting 5-10 s with normal EEG and CT brain scan findings and responsiveness to phenobarbitone. Drake et al. (6) described three cases of paroxysmal choreoathetosis lasting several minutes after a closed head injury with efficacy of anticonvulsant drugs

A videotape segment accompanies this article. Address correspondence and reprint requests to Dr. N. Biary, Department of Clinical Neurosciences, Riyadh Armed Forces Hospital, P.O. Box 7897, Riyadh 11 159, Kingdom of Saudi Arabia.

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documented MR imaging abnormality in the basal ganglia and a good response to acetazolamide. Acknowledgment: We thank Kathleen Harrison for typing the manuscript.

LEGEND TO VIDEOTAPE A 15-year-old boy with posttraumatic paroxysmal nocturnal right hemidystonia is shown. The patient was unaware of these attacks, during which he had extension of the right wrist and fingers, with fanning and writhing movements of the fingers and flexion and inversion of the right foot with occasional writhing movements of the toes. During the episodes, the EEG did not show any seizure activity.

REFERENCES 1. Lugaresi E, Cirignotta F, Montagna P. Nocturnal paroxys-

2.

3. FIG. 1. Axial magnetic resonance (spin echo, TR = 3,000 ms, TE = 90 ms) at the level of basal ganglia shows focal areas of high signal intensity in the posterior putamen on the left side (arrowheads).

and no evidence of basal ganglia lesions on CT brain scan. Richardson et al. (7) described a young man with episodes of left-sided dystonic spasms induced by sudden movements after a severe, unilateral, frontal lobe contusion. The attacks were relieved by phenytoin. Our patient resembles these aforementioned patients in the fact that the head trauma was severe and the abnormal movements were paroxysmal. However, his attacks were exclusively nocturnal. In posttraumatic dystonias, neuroimaging usually reveals some abnormality in the caudate nucleus, lentiform nucleus, thalamus, or in a combination of these structures (8,9). Although acetazolamide was reported to be effective in the treatment of paroxysmal dystonia in central demyelinating disease (lo), this is the first reported case of posttraumatic paroxysmal nocturnal dystonia with a

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7. 8. 9. 10.

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Movement Disorders, Vol. 9, No. 1 , 1994