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Query: UMLS:C0008489 (chorea)
 2,102 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report the case of a 13-year-old boy with disabling chorea due to moyamoya disease. His chorea seemed to improve with steroid therapy. We conclude that steroid therapy may ameliorate moyamoya-associated chorea, and perioperative steroids can confound neurosurgical outcome. We are unable to assess the effect of cerebrovascular bypass procedures on the outcome of chorea in this patient.
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PMID:Steroid-responsive chorea in moyamoya disease. 175 54

Three children with moyamoya disease are reported whose initial and predominant manifestations were choreic movements. Two of the patients presented with unsteady gait and the other with clumsiness. Choreic movements were recurrent and were often triggered by excitement, emotional tension, or crying. They occurred unilaterally or bilaterally and often alternated between the right and left. Moyamoya disease must be considered in the differential diagnosis of acquired chorea in children.
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PMID:Moyamoya disease presenting with chorea. 231 Apr 35

An 11-year-old girl with Down syndrome is reported with moyamoya syndrome; she presented with chorea and mental regression, but had no hemiplegia or convulsions. Magnetic resonance imaging and magnetic resonance angiography were valuable for diagnosis of moyamoya syndrome. It is suggested that moyamoya syndrome be considered as a possible cause of involuntary movements in Down syndrome patients.
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PMID:Moyamoya syndrome in a patient with Down syndrome presenting with chorea. 829 17

We reported a 12-year-old boy with unilateral moyamoya disease whose initial and predominant manifestation was hemichorea. Neurological examinations revealed chorea in his left upper extremity and muscle hypotonia in his left upper and lower extremities. Cranial MRI showed moyamoya vessels only in the right basal ganglia and infarction in the white matter of the right frontal lobe. Right carotid angiography revealed stenosis in the distal part of internal carotid artery, and in the proximal part of anterior and middle cerebral arteries with moyamoya vessels. Left carotid angiography showed normal findings. He was diagnosed as a suspected case of moyamoya disease (unilateral moyamoya disease) according to the diagnostic criteria proposed by the Research Committee on Moyamoya Disease of the Ministry of Health and Welfare of Japan. His chorea responded to haloperidol but encephalo-duro-arterio-synangiosis on the right side improved all symptoms. Chorea occurs in some patients with moyamoya disease. Hypofunction of the striatal indirect pathway is suggested as the cause of chorea. In this case an ischemic lesion in the right striatum may have caused hypofunction of the pathway and developed chorea and hypotonia.
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PMID:[A case of unilateral moyamoya disease presenting with hemichorea]. 1126 Sep 20

A 54-year-old woman presented choreic movements in left face and extremities for three months. Cerebral angiography revealed occlusions of bilateral internal carotid arteries (right: after the furcation of posterior communicating artery; left: after the furcation of opthalmic artery) and net-like collaterals around the basal ganglia region (Moyamoya vessels). Haloperidol rapidly resolved the choreic movements and no recurrence was observed. PET demonstrated misery perfusion at bilateral temporo-parietal cortices. Especially, right peri-sylvian region showed the most severe ischemia. MRI demonstrated no infarcts. Therefore, ischemia of the right striatum was suspected to be the cause of the left-sided hemichorea. Previously, Moyamoya disease presenting chorea was infrequently reported in young people of less than 20 years of age. This is the first report of the aged patient of Moyamoya disease presenting with hemichorea and severe misery perfusion.
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PMID:[An adult case of Moyamoya disease presenting with transient hemichorea]. 1235 53

Moyamoya disease (MMD) is an uncommon intracranial vasculopathy that typically presents with ischemic or hemorrhagic stroke. Persistent choreoathetosis has been identified as a rare early manifestation of MMD. We present 2 patients with paroxysmal dyskinesia as the initial symptom of MMD, one resembling paroxysmal kinesigenic dyskinesia (PKD) and the other paroxysmal non-kinesigenic dyskinesia (PNKD). We also review the cases of moyamoya-induced chorea reported previously, none of which resembled PKD or PNKD. We hypothesize that both hormonal and ischemic factors may be implicated in the pathogenesis of these abnormal involuntary movements. These cases suggest that MMD should be included in the differential diagnosis of PKD and PNKD.
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PMID:Moyamoya-induced paroxysmal dyskinesia. 1450 75

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.
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PMID:Chorea and Broca aphasia induced by diabetic ketoacidosis in a type 1 diabetic patient diagnosed as Moyamoya disease. 1564 79

Chorea gravidarum is uncommon movement disorder of pregnancy, characterized by involuntary, abrupt, non-rhythmic movements. It can be idiopathic or secondary to the underlying pathology. A 28-year-old, primigravida woman who was 8 weeks and 6 days of gestation presented with a history of involuntary choreiform movements in the left side limbs and facial twitch for 2 weeks. The symptoms started just after onset of severe emesis gravidarum. There was no meaningful medical history or family history, and she was taking no regular medication. Magnetic resonance imaging of the brain revealed moyamoya disease. The symptoms, as well as the hyperemesis gravidarum, improved with gestational age; however, they were sustained up to 30 weeks of gestation. She delivered 2.61 kg, healthy male neonate at 40 weeks' pregnancy by vaginal delivery under epidural analgesia. After 3 years later, she got pregnant again, and delivered at 41 weeks of pregnancy without complication. She experienced similar but milder symptoms to that of the first pregnancy until the late second trimester. After the delivery, full evaluation was lost due to her refusal. In this report, we present the case of a patient with chorea gravidarum in which moyamoya disease acts as an etiologic factor and a review of literature with management proposal.
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PMID:Consecutive pregnancy with chorea gravidarum associated with moyamoya disease. 1932 53

A seven year-old male presented to his pediatrician with choreiform movements and a recent history of sore throat. He was diagnosed with Sydenham's chorea based on clinical criteria and laboratory evidence. Worsening symptoms prompted a magnetic resonance imaging (MRI) of the brain which demonstrated evidence of Moyamoya disease. Sydenham's chorea is a common and well-documented complication of post-streptococcal infection, but has not been previously reported in association with Moyamoya disease. This case raises the quandary of causality of chorea in this patient and the need for neuroimaging in children with movement disorders.
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PMID:Sydenham's chorea as a presentation of Moyamoya disease. 2043 90

The aim of this study was to define the clinical characteristics of patients who developed movement disorders in association with moyamoya disease (MMD). Using PubMed and medical records of our hospital from 1985 to 2008, we searched for patients who developed movement disorders in association with MMD. This study included 38 patients described in previous studies and 4 patients found in the medical records. The onset of movement disorders was thought to be sudden. In 13 patients, the movement disorders were precipitated by hyperventilation or emotional stress. Twenty-seven of the 42 patients developed chorea, 4 patients developed dystonia, and 4 developed a mixture of both. The movement disorders of the remaining 7 patients were described as dyskinesia. A third of the 42 patients developed bilateral movement disorders, and their mean age was younger than that of those with unilateral movement disorders. In 37 of the 42 patients, brain imaging studies showed ischemic lesions, but the remaining 5 patients showed no parenchymal lesions. Cerebral perfusion studies showed hypoperfusion in the basal ganglia and in the cerebral cortical areas. Most patients improved whether they were treated or not. MMD must be included in the differential diagnosis of the sudden onset of dyskinesias, particularly chorea and focal dystonia. Even in patients with no parenchymal lesion in brain imaging studies, cerebral angiography and cerebral blood perfusion studies must be performed, if they develop a sudden onset or recurrent movement disorders preceded by emotional stress or hyperventilation.
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PMID:Movement disorders associated with moyamoya disease: a report of 4 new cases and a review of literatures. 2062 62


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