|Year : 2020 | Volume
| Issue : 4 | Page : 222-224
Acute hemiballismus in the absence of subthalamic nucleus lesion
Shreya Gudi, Tushar Kanti Biswas, Rucha Sane
Department of Geriatrics, MGM Medical College and Hospital, Kamothe, Navi Mumbai, India
|Date of Submission||27-Jun-2020|
|Date of Decision||07-Sep-2020|
|Date of Acceptance||07-Sep-2020|
|Date of Web Publication||09-Dec-2020|
Dr. Tushar Kanti Biswas
Department of Geriatrics, MGM Medical College and Hospital, Kamothe, Navi Mumbai 410209.
Source of Support: None, Conflict of Interest: None
A 68-year-old male, known hypertensive on irregular medication presented with sudden onset of involuntary movements of the left upper limb, in the form of severely violent, arrhythmic, and flinging large amplitude movements for 2 days, suggestive of hemiballismus. Magnetic resonance image of brain revealed a subacute nonhemorrhagic infarct in the posterior limb of the right internal capsule, but no lesion was noted in the subthalamic region. In traditional teaching, hemiballismus has been characterized as almost pathognomonic of a lesion in the subthalamic nucleus (STN). While a lesion occurring directly in the subthalamic nuclei can explain the onset of the movement disorder, a lesion occurring anywhere along the cortico-striatal pathway can also produce similar symptoms. The prognosis is benign in most cases, with almost all patients responding well to treatment and many having spontaneous remission. Our patient improved significantly following Tetrabenazine. Hemiballismus is as such a rare movement disorder. An elderly patient with sudden onset hemiballismus, indicating a vascular insult confirmed by subacute infarct in the posterior limb of the internal capsule, without any lesion in STN merits the report of this rare case.
Keywords: Hemiballismus, subthalamic nucleus, tetrabenazine
|How to cite this article:|
Gudi S, Biswas TK, Sane R. Acute hemiballismus in the absence of subthalamic nucleus lesion. MGM J Med Sci 2020;7:222-4
| Introduction|| |
Hemiballismus, classified as a Choreoathetoid movement disorder, is seen most commonly in patients with subthalamic nuclei lesions. Patients present with subacute onset of violent involuntary flinging large amplitude movements often involving either or both the contralateral limbs. Infarcts or hemorrhagic lesions in the thalamus, putamen, or parietal cortex can also develop brief self-resolving episodes of hemiballismic movements. While most commonly due to vascular insults, other pathologies including intracranial neoplasms, demyelinating disorders, vasculitis, trauma, infections like HIV, and even hyperglycemic ketotic states have been documented to cause hemiballismus., The diagnosis is a purely clinical one and involves carefully studying the amplitude, direction, frequency, and recurrence of the movements., Similar to other jerky hyperkinetic movement syndromes, laboratory testing for metabolic disturbances and genetic mutations often pre-dominates the workup of such patients. Imaging when performed is done by MRI and is often directed toward identifying the underlying cause of the presenting disorder. Hemiballismus has been documented to be very often self-resolving in most of the cases, except intra-cranial space occupying lesion, demyelination, etc. In cases requiring intervention, treatment is initiated with dopamine blocking or depleting agents and has shown excellent clinical results. We hereby describe a patient who presented with hemiballismus but eventually had no identifiable basal ganglia lesion on neuro-imaging, attributable to the same. We intend to highlight the rarity of distant ischemic lesions causing probable temporary surge and precipitating a movement disorder.
| Case report|| |
A 68-year-old male, known hypertensive on irregular medication, came to the hospital with sudden onset of involuntary movements of the left upper limb for 2 days. The patient did not report any limb weakness, facial deviation, dysarthria, aphasia, cranial nerve deficits, or sensory neural loss. There was no history of any seizure or self-resolving episodes of involuntary limb movements in the past. On examination, the patient was found to be in accelerated hypertension with blood pressure (BP) of 200/100 mmHg. In view of the same, the patient was initially managed with a targeted reduction of BP. On neurologic examination, the patient was found to have swinging movements of the left upper and lower limbs. There was no evidence of any focal neurologic deficit. The patient had severe, violent, arrhythmic, large-amplitude movements at the proximal part of the limb, admixed with flexion, extension, rotation, and crossing seen over the distal limbs, suggestive of hemichorea–hemiballismus type of movements. The patient’s routine metabolic profile was found to be normal. A cardiology work-up in the form of ECG and 2D echocardiography was normal. MRI brain was suggestive of a subacute nonhemorrhagic infarct in the posterior limb of the right internal capsule. However, no ischemic areas were noted in the subthalamic region [Figure 1][Figure 2][Figure 3]. MR brain angiography was found to be normal [Figure 4]. The patient was initiated on a single antiplatelet therapy along with tablet Clozapine but did not show adequate improvement. The patient was then initiated on tablet Tetrabenazine which resulted in significant improvement. The patient was discharged and currently remains symptom-free.
|Figure 1: T2 image shows hyperintensity in the posterior limb of right internal capsule|
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|Figure 2: T1 image showing same hyperintense signal in the right internal capsule with the absence of any enhancement in the subthalamic nucleus|
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|Figure 3: Diffusion-weighted image showing enhancement in right internal capsule|
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|Figure 4: MR angiogram showing normal flow in bilateral intracerebral regions|
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| Discussion|| |
Hemichorea–hemiballismus is an unusual complication of ischemic and hemorrhagic strokes with a reported incidence of less than 1%. Imaging in acute stroke has now become advanced enough to provide imaging within acute ischemic stroke window periods. Our patient presented with the acute onset of a movement disorder suggestive of an acute vascular insult or stroke.
The acute onset of movement disorders warrants reliable imaging of the basal ganglia to rule out causative primary lesions and clearly distinguish true basal ganglia lesions from lesions occurring elsewhere in the cortico-thalamic tracts and cortico-thalamo-striatal tracts. While a lesion occurring directly in the subthalamic nuclei could explain the onset of the movement disorder, a lesion occurring anywhere along the pathway can also produce similar symptoms. Our patient had an ischemic infarction of the posterior limb of the right internal capsule, which caused a probable imbalance in the dopaminergic pathway and produced the patient’s symptoms. Hemichorea–Hemiballismus onset after stroke has a good prognosis. Treatment options vary and include both typical and atypical catecholamine depletion agents. At first, the therapy includes neuroleptic agents like haloperidol and perphenazine. Refractory cases have been found to benefit from the use of clozapine. Tetrabenazine or reserpine must be used with caution in view of their significant side effects of hypotension, extrapyramidal symptoms, and depression. Our patient improved significantly following tetrabenazine, which was then tapered off.
| Conclusion|| |
Hemiballismus is the most common movement disorder seen in post-stroke patients. It has a subacute onset in patients aged greater than 70 years of age. While the subthalamic area is most commonly affected, Physicians and Radiologists must be aware of clinical symptoms being produced due to physiological aberrations occurring as a result of distant lesions along pathways connected to the striatum.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]