Case Of Hemichorea Research Articles

A case of hemichorea in RNF213-related vasculopathy

BackgroundInternal carotid artery (ICA) stenosis has been recently reported to cause hemichorea, mainly in East Asia. The East Asian-specific p.R4810K variant of RNF213, a susceptibility gene for moyamoya disease (MMD), accounts for up to 25% of sporadic ischemic stroke with ICA stenosis cases in East Asia. However, as RNF213-related vasculopathy does not meet the diagnostic criteria for MMD, the creation of a new disease category has been suggested. Here, we report the first case of hemichorea in RNF213-related vasculopathy.Case presentationAn 81-year-old woman was admitted to our hospital with choreic movements in the periphery of the right extremities at rest. Though head magnetic resonance imaging showed no fresh or old cerebral infarction, 123I-iodoamphetamine-single photon emission computed tomography showed cerebral blood flow of < 80% in the anterior territory of the left middle cerebral artery (MCA) in a resting state and cerebrovascular reactivity of < 10% in the broader area supplied by the left MCA after acetazolamide challenge. Head magnetic resonance angiography and digital subtraction angiography revealed left ICA C1 portion stenosis with compromised collateral vessels. Involuntary movements resolved with haloperidol administration within 3 days, without apparent recurrence from continuation of the medication for a year. Genetic testing revealed the presence of the heterozygous RNF213 p.R4810K variant.ConclusionsChorea is thought to be caused by damage to circuitry connecting the basal ganglia with the cerebral cortex, as found in cases of MMD, which possess aberrant vessels in the basal ganglia. However, aberrant vessels and cerebral infarctions were not observed in the basal ganglia in the current case, decreasing the likelihood of a role in chorea. Alternatively, as RNF213 regulates vascular endothelial function and angiogenesis, dysregulation may impair the neurovascular unit and damage basal ganglia circuitry, contributing to the development of chorea. This case may renew interest in the concept of RNF213-related vasculopathy and the pathophysiological mechanisms behind chorea in ICA stenosis.

A case of hemichorea caused by right internal carotid artery stenosis

This is a case of a 71 years old man. Hemichorea appeared in the left half of his body in the middle of November, 2014. Minute hyperintense areas in the white matter near the posterior horn of the right lateral ventricle and in the right parasagittal frontal cortex was shown in MRI diffusion weighted image. Severe stenosis was seen in the right internal carotid artery, and reduction in cerebral blood flow of the right cerebral hemisphere including the basal ganglia was shown in single photon emission computed tomography (SPECT). After having carotid endarterectomy of the right internal carotid artery in January, 2015, hemichorea disappeared, and the cerebral blood flow of the right cerebral hemisphere improved. It is known that hemichorea is caused by the infarction of the basal ganglia. In recent years, some hemichorea cases are reported around East Asia caused by internal carotid artery stenosis with reduction in cerebral blood flow.

Hemichorea caused by nonketotic hyperosmolar state: A case report and review of the literature

Our report involves a case of hemichorea caused by the nonketotic hyperosmolar state. We have analyzed the clinical data and relevant features of a patient who presented herself to the Affiliated Hospital of Xuzhou Medical University. The patient had unilateral involuntary movements for 1 month. We discovered that her blood glucose levels were very high. The patient underwent computed tomography (CT), magnetic resonance imaging (MRI), and magnetic resonance angiography (MRA), indicating right basal ganglia lesion. Control of the patient’s blood glucose plus supportive treatment resulted in a significant improvement of her clinical state.

DBS reduced hemichorea associated with a developmental venous anomaly and microbleeding in STN

Developmental venous anomaly (DVA) is the most common vascular malformation and is usually asymptomatic. Movement disorders associated with DVA have rarely been reported, except a case of hemichorea and hemiballism caused by DVA in the contralateral putamen associated with hyperglycemia.1 The abnormal movements in that case nearly resolved after treatment with insulin and risperidone. We report a case of DVA who presented with hemichorea without metabolic derangement but microbleeding surrounding the DVA in the contralateral subthalamic nucleus (STN) area. The lesion was unresectable and deep brain stimulation (DBS) of globus pallidus interna (GPi) helped control the pharmacologically refractory symptoms.

A Case of Hemichorea with Primary Somatosensory Cortical Infarction

A Case of Hemichorea with Primary Somatosensory Cortical Infarction

One case of hemichorea associated with non-diabetic hyperglycemia

One case of hemichorea associated with non-diabetic hyperglycemia

Hemichorea improved by extracranial–intracranial bypass surgery for middle cerebral artery occlusion

Hemichorea is often associated with structural ischemic lesions located in the motor frontosubcortical circuit of Alexander [1, 2]. However, the precise pathogenic link between the symptom and lesions remains uncertain [2, 3]. Recent reports have described patients with hemichorea caused by contralateral extraor intracranial arterial stenosis. Improvement of the hemichorea by surgical revascularizations and preand postsurgical single-photon emission computed tomography (SPECT) analyses suggested functional hypoperfusion, not a structural infarct per se, as an important etiology of hemichorea [4–8]. Here we report a case of hemichorea that improved following extracranial–intracranial (EC–IC) bypass surgery to treat hypoperfusion secondary to a middle cerebral artery (MCA) occlusion. A 67-year-old man acutely developed an involuntary movement in his left hand that spread to his left arm and leg within a few days. He had a smoking habit and hypercholesterolemia without any medication history including antipsychotics. The involuntary movement did not interfere with his daily activities and walking, but persisted for 2 months. Neurologic examination was unremarkable except for the hemichorea and mild leftsided facial dyskinesia. Hemiballism and other extrapyramidal signs were absent. The hemichorea was persistent and unrelated to the patient’s posture. Orthostatic hypotension was absent. Blood tests revealed hypercholesterolemia (LDL-cholesterol 192 mg/dl; the normal range 70–140 mg/dl). Antinuclear antibody, lupus anticoagulant activity, and anticardiolipin antibodies were absent. Thyroid function and plasma osmolarity were normal. Brain diffusion-weighted magnetic resonance imaging (MRI) showed no acute infarct (Fig. 1a, b). T2-weighted MRI revealed an old infarct in the right frontal white matter (Fig. 1c) but no lesion in the basal ganglia (Fig. 1d). Cerebral angiography revealed an occlusion at the stem of the right MCA (Fig. 1e). Collateral arterial networks linking the right anterior cerebral artery, posterior cerebral artery (PCA), and branches from the right external carotid arteries supplied the large part of the right cerebral hemisphere (Fig. 1f). The right deep cerebral structures were supplied by collaterals originated from the occluded MCA stem and the right PCA (Fig. 1e, f). No abnormal net-like collateral vessels (moyamoya vessels) were present (Fig. 1f), and atherosclerosis was postulated to be a cause of the MCA occlusion. SPECT using I-labeled N-isopropyl iodoamphetamine (I-IMP) suggested no basal hypoperfusion (Fig. 1g), but acetazolamide-challenged I-IMP SPECT revealed a decrease of vascular reserve [9] in the right MCA territory, including the right basal ganglia (Fig. 1h). The patient was treated with aspirin (300 mg daily), atorvastatin (20 mg daily), and cilostazol (200 mg daily), but the hemichorea persisted. Three months after onset, an EC–IC bypass (superficial temporal artery-MCA anastomosis) operation was performed. Surprisingly, the hemichorea improved a few days after the surgery. Follow-up SPECT 1 week after the surgery T. Irioka (&) Department of Neurology, Yokosuka Kyosai Hospital, 1-16 Yonegahama-dori, Yokosuka, Kanagawa 238-8558, Japan e-mail: t-irioka.neuro@ykh.gr.jp

Chorea and Broca aphasia induced by diabetic ketoacidosis in a type 1 diabetic patient diagnosed as Moyamoya disease

We here report one case of hemichorea and Broca aphasia occurred with diabetic ketoacidosis. A 20-year-old woman with type 1 diabetes mellitus had experienced diabetic ketoacidosis fourth time after the onset of diabetes. At the third ketoacidotic episode, the patient was admitted to our hospital for the first time to show hemichorea of the left extremities. Brain computed tomography (CT) demonstrated a high-density area in the right caudate head and low-density area in the right putamen. Magnetic resonance angiography (MRA) demonstrated a stenosis at the root of the bilateral middle and anterior cerebral arteries. The hemichorea disappeared within 3 days. At the fourth ketoacidotic episode, not hemichorea but unconsciousness was there for 2 days even after ketoacidosis disappeared. After the unconscious state, Broca aphasia was demonstrated for 15 days. The cerebral angiography showed a finding compatible to Moyamoya disease. These findings support that chorea and Broca aphasia induced by diabetic ketoacidosis was developed in addition to blood vessel abnormalities such as Moyamoya disease. We suggest that poorly controlled diabetic patients with hemichorea should undergo cerebral angiography.

A Case of Hemichorea With Post-Mortem Examination

A Case of Hemichorea With Post-Mortem Examination