Movement Disorders Vol. 7, No. 4, 1992, pp. 364-366. D 1992 Movement Disorder Society Brief Report Stimulus-Sensitive Paroxysmal Dyskinesias Associated with a Thalamic Infarct P. C. G. Nijssen and C. C. Tijssen Department of Neurology, St. Elisabeth & Maria Hospitals, Tilburg, The Netherlands Summary: We describe a patient with the Dejerine-Roussy syndrome (hemiparesis, hemiataxia, hemidysesthesia, and pain), due to posterolateral thalamic infarction, who also exhibits paroxysmal dyskinesias triggered by action and by sensory stimulation. Key Words: Paroxysmal dyskinesias-Thalamic infarct-Dejerine-Roussy syndrome. of similar involuntary movements occurred. Two weeks later, strength was normal, but hemihypesthesia and severe ataxia of the right leg persisted. Computed tomography (CT) scanning revealed a hypodense lesion of the left posterolateral thalamus and adjacent posterior limb of the internal capsule, without contrast enhancement (Fig. 1). Two months later, she was readmitted because of repeated involuntary movements of the right leg. On examination, higher cortical functions, cranial nerves, reflexes, and strength were normal. Intentional movements of the right arm were disturbed by severe proximal dysmetric flailing movements and kinetic tremor, while precise movements of the fingers and distal diadochokinesis were normal. Intentional movements of the right leg were nearly impossible: attempts to move the right leg induced gross movements of the right leg and trunk, with marked proximal cerebellar dysmetria. Curiously, these movements, with elements of dystonia, ballism, jerks, and stereotypies, usually lasting less than a minute, were also seen directly after sensory stimulation of the right arm and leg. Sensory stimulation of the right limbs was experienced as extremely unpleasant. Sensory stimulation was not mode specific, as vibration, touching, and pricking of hyperpathic dermatomes could all easily induce involuntary movements. Touching the right leg with her own hands was well tolerated, however, inducing neither dysesthesia nor dyskinesias. Walking was possible with support, and showed some circumduction and slight dystonia of the right leg. At this time a videoregistration was made (see videotape). Repeated CT scanning showed the same hypodense lesion. Magnetic resonance imaging (MRI) confirmed the suspicion of an infarct in the left posterolateral thalamus A syndrome consisting of a transient hemiparesis, hemidysesthesia, and hemiataxia (major symptoms), usually combined with pain and choreoathetotic movements (minor symptoms), was named ‘le syndrome thalamique’ by Dejerine and Roussy in 1906 (l), as observed in three patients with infarction of the posterolateral thalamus and the adjacent part of the posterior limb of the internal capsule. We describe a patient with the Dejerine-Roussy syndrome due to posterolateral thalamic infarction, accompanied by dyskinesias that were typically provoked by sensory stimulation of hyperpathic skin regions or by movements of the affected limbs. A videotaped recording is provided demonstrating these findings. CASE HISTORY A 58-year-old woman with a history of migraine, hypercholesterolemia, and hypertension, who was suffering from a continuous headache for 6 weeks, experienced sudden tingling and numb feelings of the right face and arm, which was followed within minutes by involuntary movements of the right leg and arm. On examination, there were gross involuntary movements of the right arm and leg, with right hemihypesthesia, moderate hemiparesis, and hemiataxia. Cranial nerves, speech, and reflexes were normal. The movements stopped spontaneously within minutes and were difficult to describe, having ballistic and dystonic elements. Several days later, an attack A videotape segment accompanies this article. Address correspondence and reprint requests to Dr. P. C. G . Nijssen at Department of Neurology, St. Elisabeth & Maria Hospitals, P.O. Box 90151, 5000 LC Tilburg, The Netherlands. 364 PAROXYSMAL D YSKINESIAS D WE TO THALAMIC INFARCT 365 physiology of this syndrome has been analyzed extensively ( 4 3 ) . Several kinds of dyskinesia have been reported in thalamic lesions: choreoathetosis (1,6), dystonia-chorea (2), hemidystonia (7,8), paroxysmal kinesigenic dystonia (9,10), and hemiballism-hemichorea (1 1). Reflex dystonia induced by tactile stimulation was recently reported in a patient with hypnogenic paroxysmal dystonia (12). As shown in the video, our patient had severe hemiataxia with dysmetria and intention tremor with actioninduced dyskinesias. Most striking in our patient, however, is the remarkable effect of sensory stimulation on the affected side in inducing gross involuntary movements of the right arm, trunk, and right leg. These abnormal movements are complex and hard to describe, as they appear to have elements of ballism, dystonia, jerks, and stereotypies. There was no habituation of the response to sensory stimulation. Touching the hyperpathic regions with her own hands did not induce dysesthesias or involuntary movements. As a result of the difficulty in specifically labeling the abnormal movements, conclusions as to the pathophysiological mechanism are limited. Recent research in hypo- and hyperkinetic movement disorders has led to a functional model of the basal ganglia (13). Increased motor activity may result from decreased inhibitory influences of the internal segment of the globus pallidus and the substantia nigra pars reticulata on the thalamus. In our patient, a similar mechanism may FIG. 1. Computed tomography (CT) scan showing a hypodense lesion of the left posterolateral thalamus and posterior limb of the internal capsule, without contrast enhancement. and internal capsule (Fig. 2). Electroencephalography showed a focal disturbance in the left frontotemporobasal area without epileptic activity. During clinical observation for 4 weeks, attacks of dyskinesias were highly variable in frequency and severity, and were especially increased by distress and hyperventilation. Treatment with clonazepam and pimozide was not successful. After 6-month follow-up, tactile stimulation of the right leg still induced dystonia of the right leg, with inversion of the foot, that lasted for several seconds but was less severe. Recently, 10 months after the first episode, the patient has complained of spontaneous pains in the right extremities. DISCUSSION Thalamic infarcts may lead to at least four different clinical syndromes corresponding to arterial thalamic territories (2). Infarcts in the territory of the inferolateral arteries, including the thalamic ventral posterior nuclear group (and sometimes the internal capsule), may lead to hemiparesis, hemisensory defects, hemiataxia, and painful paroxysms. The symptomatology of Dejerine-Rouw syndrome is quite variable, changing in time, and often under the influence of external factors (3). The patho- FIG. 2. Magnetic resonance imaging (MRI), T2-weighted, showing a hyperintense lesion of the left thalamus and internal capsule. Movement Disorders, Vol. 7, No. 4,1992 P . C . G . NIJSSEN A N D C . C . TIJSSEN 366 be present, due either to destruction of the subthalamic nucleus or its projections to substantia nigrdinternal pallidum, or of projections of the latter nuclei to the thalamus, rendering the thalamus supersensitive to input of either superficial or deep sensory modalities. Disinhibition of thalamic relay nuclei may also result from destruction of the thalamic reticular nucleus. It remains unclear whether hyperpathic tactile stimuli induce involuntary movements directly through the aforementioned circuit, or through an indirect pathway in which hyperpathia leads to reflex withdrawal, which in turn induces further hyperkinesis, as in paroxysmal kinesigenic dystonic choreoathetosis. LEGEND TO VIDEOTAPE The videotape demonstrates the patient walking with an odd hemidystonic gait, attempting to suppress involuntary movements of the right leg with her arm. Spontaneous dyskinesias of the right leg occur while sitting. Finger-nose and heel-shin testing shows a marked cerebellar dysmetria and tremor, whereas precise movements of the right hand are normal. Lifting the right leg induces ballistic jerks, with severe ataxia. Most interesting, however, is the dramatic response to touch of the right arm and leg, leading to complex movements that have elements of ballism, jerks, dystonia, and stereotypies. The patient experienced touching of the right limbs as extremely unpleasant, and tried to avoid external stimulation, whereas touching by her own hands was neither unpleasant nor leading to dyskinesias. Acknowledgment: The authors thank Mr. N. Kempers for his skillful registration of the videotape and preparation of the figures. Movement Disorders, Vol. 7, N o . 4, I992 REFERENCES '1. Deierine J. Roussv G. Le syndrome thalamique. Rev Neurol 1966;12:521-532. 2. Bogousslavsky J, Regli F, Uske A. Thalamic infarcts: clinical syndromes, etiology, and prognosis. Neurology 1988;38: 837-848. 3. Adams RD, Victor M. Principles of neurology, fourth ed. New York, McGraw-Hill, 1989:114-115. 4. Cambier J. Dejerine-Roussy's syndrome. Rev Neurol 1982; 138:979-988. 5 . De Smet Y. The thalamic syndrome of Dejerine-Roussy. Prolegomena. Rev Neurol 1986;142:259-266. 6. Konitzer M. Pathology and clinical features of the postenor thalamic syndrome of Dejerine and Roussy: four cases and a review. 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