A case of late-onset chorea

Background A 75-year-old woman with rheumatoid arthritis presented with a 4-year history of chorea to a hospital movement disorder clinic. The involuntary movements were initially mild, affecting only the right side of the body, but gradually worsened and became bilateral. There was no relevant family history. Medications included hormone replacement therapy (HRT), diclofenac sodium, vitamin D, folic acid, methotrexate and zopiclone. On examination, bilateral choreiform movements were seen, affecting the face and limbs, with the right side more severely affected than the left. Investigations Neuropsychological testing, laboratory blood and DNA testing, echocardiogram, MRI of the brain, and brain perfusion single-photon emission computed tomography (SPECT) scanning.Diagnosis HRT-related chorea, possibly caused by a predisposition secondary to rheumatoid arthritis and small-vessel ischemic disease, or subclinical childhood rheumatic fever. Management Discontinuation of HRT.     

A case of senile chorea

Senile chorea, a late-onset, slowly progressive disorder without mental changes, is a poorly characterized entity. It is often thought to represent late-onset Huntington's disease in which the chorea precedes the mental changes and the family history is absent due to parental death before symptoms. The following case differs pathologically from Huntington's disease and from previous reports of senile chorea.     

Methylphenidate-induced chorea: case report and pharmacologic implications

In a child with minimal brain dysfunction, we found that chorea was related to the major central effect of methylphenidate and probably to the effect of the drug on central catecholaminergic systems. Also, after 3 weeks of treatment with methylphenidate, guinea pigs showed a hypersensitive response to apomorphine, suggesting that chronic administration of methylphenidate leads to hypersensitivity of receptor sites. Chorea beginning shortly after initiation of methylphenidate therapy probably is related to the central dopaminergic effect of the drug; when choreic movements appear after chronic methylphenidate administration, altered responsiveness of striatal dopamine receptor sites may be responsible.     

Anti-Yo,chorea and hemiballismus: A case report

68-year-old female presented with involuntary movements. MRI was normal. Cerebrospinal fluid analysis was normal. whole body CT and biopsy confirmed diagnosis of metastatic adenocarnimoa. The autoimmune panel was positive for anti-Yo antibodies.     

Sydenham's chorea in the age of MRI: a case report and review

Mitochondrial ornithine transporter deficiency, or HHH syndrome, is a metabolic disorder resulting in various neurologic symptoms, including mental retardation, spastic paraparesis with pyramidal signs, cerebellar ataxia, and episodic disturbance of consciousness or coma caused by hyperammonemia. Several mutations have been reported in the ORNT1 gene encoding mitochondrial ornithine transporter of patients with this disorder. In this article, we report a new patient, a male 15 years of age, who had typical clinical features of HHH syndrome. Because the patient did not have any of the three mutations previously described in other Japanese patients with HHH syndrome, and the only material available from the patient was peripheral leukocytes, we established a genomic polymerase chain reaction method using intronic primers to amplify every exon of the ORNT1 gene, and we directly sequenced the polymerase chain reaction products. Using this method, we documented a novel mutation in this patient, P126R, and demonstrated that HHH syndrome is genetically heterogeneous, even in the Japanese population.     

A case of thyrotoxicosis with chorea: association with anti-phospholipid antibody

We report a case of thyrotoxicosis associated with chorea and detectable anti-phospholipid antibodies in which a structural abnormality could not be detected on routine imaging but cerebral perfusion single photon emission computed tomography scans suggested a reversible alteration of basal ganglia perfusion. The pathophysiological mechanisms of phospholipid antibody causing chorea in thyrotoxicosis are discussed.     

Cerebral vasculitis in a case of Sydenham's chorea

A 10-year-old girl with acute-onset hemichorea had multiple areas of abnormal signal seen on magnetic resonance imaging of the brain, associated with middle and anterior cerebral artery vasculitis seen on cerebral angiography. Her serology and clinical course were supportive of the diagnosis of Sydenham's chorea. Other causes of cerebral vasculitis were excluded. Follow-up studies revealed resolution of changes seen on magnetic resonance imaging and partial resolution of angiographic abnormalities. This is the first report of abnormal cerebral angiography in Sydenham's chorea.     

A case of chorea due to polycythaemia vera

The history of a 76 years-old woman suffering from chorea caused by polycythaemia vera is reported. Initial treatment with repeated phlebotomy followed by intravenous administration of P 32 led to the disappearance of both the polycythaemia and the chorea. Since abnormal CBF in the region of the basal ganglia has been postulated to be responsible for this syndrome, a dynamic CT examination was performed, but no significant abnormalities were found.     

A case of moyamoya disease presenting with chorea

Case report We describe a 15-year-old girl with moyamoya disease whose initial manifestation was chorea-like involuntary movements. T2-weighted magnetic resonance imaging showed high signal intensity lesions in the left frontal lobe, right parieto-occipital lobes, and frontal subcortical white matter. Single-photon emission computed tomography (SPECT) showed diffuse hypoperfusion of the whole brain. Bilateral direct and indirect cerebrovascular bypass surgeries were performed. Chorea disappeared 2 days after the surgery. Follow-up SPECT demonstrated increased cerebral perfusion in the bilateral frontal, temporal, and parietal regions.Conclusions Chorea accompanied with moyamoya disease can be properly managed by revascularization surgery. Moyamoya disease should be remembered as being one of the differential diagnoses of chorea, which is treatable by surgery.     

Benign hereditary chorea 2: Pathological findings in an autopsy case

Two Japanese families with benign hereditary chorea (BHC) 2 have recently been reported. BHC 2 is characterized by adult‐onset non‐progressive chorea, and by genetic abnormality in the locus of chromosome 8q21.3‐q23.3. This differs from the genetic abnormality previously reported in BHC. Here we report the first autopsied case of a member of one of two known families with BHC 2. A normally developed woman recognized choreiform movements of her bilateral upper extremities beginning approximately at age 40. The movements had slowly spread to her trunk and lower extremities by approximately age 60. Generalized muscular hypotonia was also observed. The symptoms persisted until her death at the age 83, but had not worsened. Neuropathological examination revealed mild to moderate neuronal loss and astrocytosis in the striatum and decreased volume of cerebral white matter with astrocytosis bilaterally. Additionally, sparse but widely distributed neurofibrillary tangles and argyrophilic threads as well as scattered tufted astrocytes immunoreactive for 4‐repeat isoform of tau were observed in the cerebrum, brainstem and cerebellum, showing 4‐repeat tauopathy similar to that of progressive supranuclear palsy (PSP). Unique neuronal cytoplasmic inclusions were observed in the oculomotor nuclei; however, any specific immunoreactivities (e.g. ubiquitin and p62) were not detected, suggesting the presence of previously undescribed protein intracellular inclusions. Clinicopathologically, chorea accompanied with generalized muscular hypotonia seemed to be associated with mild degeneration of the striatum and cerebral white matter. The significance of PSP‐like changes in the pathogenesis of BHC 2 remains to be elucidated.     

Bilateral Charcot arthropathy of shoulder secondary to syringomyelia: An unusual case report

Neuropathic arthropathy of the shoulder is a relatively rare disorder characterized by destruction of joint secondary to loss of sensory innervation. Bilateral Charcot arthropathy is an even rarer disorder, with very few cases reported in the English literature. We herein present a case of bilateral shoulder arthropathy secondary to syringomyelia with classical clinical and radiological findings. Radiological finding on one side was of resorptive type and resorptive mixed with productive on the other side.