Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0022116 (ischemia)
 91,303 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hemichorea associated with carotid artery occlusive disease is extremely rare. It has been recently suggested that carotid artery stenosis should be considered in the differential diagnosis of chorea, even in the absence of a preceding stroke or transient ischemic attack. Although the pathophysiology of this condition is still under discussion, some reports suggest that impaired cerebral blood flow in the basal ganglia is a key contributing factor. We herein report a case of hemichorea related to severe stenosis of the left internal carotid artery with no basal ganglia lesions on brain MRI. After carotid revascularization, hemichorea gradually subsided and reversible left thalamic and putaminal hypoperfusion were demonstrated by functional neuroimaging. This case report supports the hypothesis about the central role of hemodynamic ischemia in the pathophysiology of hemichorea associated with carotid artery stenosis, and highlights the importance of vascular imaging studies for the early identification of carotid disease in patients with chorea, even in the absence of other clinical signs.
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PMID:Reversible hemichorea associated with extracranial carotid artery stenosis. 2088

It is not unusual to observe hemichorea in patients with diabetes mellitus, with origins attributable to recent ischemia. Our patient was a 66-year-old female with diabetes mellitus who suddenly developed right hemichorea, mild muscle weakness of the right upper extremity, ideational apraxia, and acalculia. Her blood glucose was 600 mg/dL, and HbA1c was 13.3%. After the patient underwent head magnetic resonance imaging (MRI), a new cerebral infarction was observed in the left frontal lobe, and treatment was started with edaravone and cilostazol. At the same time, insulin treatment was also started for hyperglycemia. The acalculia and ideational apraxia improved approximately 1 week after treatment initiated, and the hemichorea also decreased. ECD-SPECT was performed on admission, and it was observed that blood flow was decreased in the left frontal lobe and striatum, but increased in the thalamus; two weeks later on follow-up ECD-SPECT, blood flow had increased slightly in the left forebrain and striatum, while it had decreased slightly in the thalamus. This suggests that the cause of hemichorea was related to ischemia. When the activity of the pallidum is impaired, it is presumed that the inhibitory activity towards the thalamus weakens and the thalamic cells become over-excited, causing chorea.
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PMID:Hemichorea in a diabetes mellitus patient following acute ischemic stroke with changes in regional cerebral blood flow. 2574 12

Hyperglycemia-associated chorea-ballism (HCB) is an infrequent neurological syndrome whose pathophysiology remains poorly understood. Positron emission tomography (PET) studies have offered valuable information regarding regional glucose metabolism. The studies included were published between 1980-2017 and reported demographic, clinical, laboratory and imaging data from patients with HCB in whom a PET scan had been performed. Eleven patients were evaluated (women 82%, Asian origin 91%, mean age 71 years). The main findings were an increase in glucose metabolism at the contralateral motor cortex related to recent episodes of hemiballism-hemichorea in 2 patients, and an altered metabolism in the affected basal ganglia in all of them: decreased in 10 patients (91%) and increased in 1 (9%). However during the acute period the patients showed only an increased metabolism, or even no changes. Contrary to what has previously been suggested in a metabolic failure hypothesis, changes in glucose metabolism in the basal ganglia may not be a key factor in the pathogenesis of HCB, and may potentially be a direct result of histological changes such as cellular ischemia and gliosis related to HCB development.
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PMID:Understanding the pathophysiology of hyperglycemia-associated chorea-ballism: a systematic review of positron emission tomography findings. 2998 83

Hemichorea has been well-reported in association with nonketotic hyperosmolar hyperglycemia (NKHH), but reports of concurrent temporal lobe involvement are rare. We present the case of a man with NKHH who developed hemichorea in the setting of rapidly progressive memory and cognitive impairments. He demonstrated the unilateral striatal T1 hyperintensities expected for NKHH-induced hemichorea but was also found to have fluid-attenuated inversion recovery hyperintensity, contrast enhancement, and eventual atrophy of his ipsilateral temporal lobe. A review of similar case reports and radiologic findings was performed. His temporal lobe injury shows a progression mimicking that seen in cortical laminar necrosis, suggesting transient ischemia to this lobe as a consequence of either blood hyperviscosity or vasoconstriction; atypical infections or parainfectious processes cannot fully be excluded, however. In addition to hemichorea or focal neurologic deficits, NKHH may also be associated with a rapidly progressive dementia and temporal lobe injury, with deficits that may not fully reverse after glycemic control.
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PMID:Rapidly Progressive Dementia and Temporal Lobe Atrophy in a Case of Nonketotic Hyperglycemic Hemichorea. 3254 49


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