Moyamoya disease presenting with singing induced chorea

Sh, Han

doi:10.1136/jnnp.69.6.833

Cited by 26 publications

(22 citation statements)

References 7 publications

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“…5,9,11,12,18,[23][24][25] In our series, 6 (60%) of the 10 patients displayed chorea as the initial presenting sign of their disease. Notably, 3 of these patients were given the diagnosis of Sydenham chorea with suspicion of rheumatic fever despite not meeting the Jones criteria.…”

Section: Discussionmentioning

confidence: 51%

Chorea in the clinical presentation of moyamoya disease: results of surgical revascularization and a proposed clinicopathological correlation

Ahn

Scott²,

Robertson

et al. 2013

PED

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Object Chorea is a movement disorder characterized by brief, irregular, involuntary contractions that appear to flow from 1 muscle to another. There are a limited number of reports in the literature that have linked moyamoya disease and chorea. The authors describe their experience in treating moyamoya disease in patients in whom chorea developed as part of the clinical presentation. Methods The authors conducted a retrospective review of a consecutive series of 316 children who underwent pial synangiosis revascularization for moyamoya disease at the Boston Children's Hospital. Results Of 316 surgically treated patients with moyamoya disease, 10 (3.2%; 6 boys and 4 girls) had chorea as a part of their presentation. The average age at surgical treatment was 9.9 years (range 3.8–17.9 years). All patients had evidence of hypertrophied lenticulostriate collateral vessels through the basal ganglia on preoperative angiography and/or MRI on affected sides. Two patients had cystic lesions in the basal ganglia. Nine patients underwent bilateral craniotomies for pial synangiosis, and 1 patient underwent a single craniotomy for unilateral disease. Follow-up was available in 9 patients (average 50.1 months). The mean duration of chorea was 1.36 years (range 2 days to 4 years), with resolution of symptoms in all patients. One patient developed chorea 3 years after surgical treatment, 4 patients had transient chorea that resolved prior to surgery, and 5 patients experienced resolution of the chorea after surgery (average 13 months). Conclusions The authors describe children with moyamoya disease and chorea as part of their clinical presentation. The data suggest that involvement of the basal ganglia by the hypertrophied collateral vessels contributes to the development of chorea, which can wax or wane depending on disease stage or involution of the vessels after revascularization surgery. In most patients, however, the chorea improves or disappears about 1 year after presentation.

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Section: Discussionmentioning

confidence: 51%

Chorea in the clinical presentation of moyamoya disease: results of surgical revascularization and a proposed clinicopathological correlation

Ahn

Scott²,

Robertson

et al. 2013

PED

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“…Chorea is a comparatively rare clinical feature of moyamoya disease and moyamoya syndrome, which in few cases seems to be the initial manifestation leading to the diagnosis [5,15,16,19,20]. This case demonstrates the need to include this uncommon pathological entity in the differential diagnosis of juvenile or adult onset chorea.…”

Section: Resultsmentioning

confidence: 97%

“…These facts could suggest an underlying ischaemic pathophysiology induced by hyperventilation-mediated vasoconstriction of intracerebral arteries supplying the basal ganglia and a subsequent hypofunction of the striatal indirect pathway [15]. A similar case of singing-induced choreatic movements in a moyamoya patient from Korea has recently been reported [16]. Hyperventilation-induced limb-shaking transient ischaemic attacks have also been attributed to a similar pathogenic mechanism, which however caused hypoperfusion of the frontoparietal cerebral cortex in a Korean patient with moyamoya disease [17].…”

Section: Discussionmentioning

confidence: 94%

Hyperventilation-Enhanced Chorea as a Transient Ischaemic Phenomenon in a Patient with Moyamoya Disease

Spengos¹,

Tsivgoulis²,

Toulas³

et al. 2004

Eur Neurol

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“…It can be a consequence of MCA or carotid stenosis . It has also been reported to be associated with TIAs in Moya‐Moya disease, but commonly induced by hyperventilation.…”

Section: Discussionmentioning

confidence: 99%