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PURPOSE To quantify impairment of the basal ganglia (globus pallidus and thalamus) in adult-onset dentatorubral-pallidoluysian atrophy (DRPLA). METHODS Five patients with genetically definite adult-onset DRPLA (aged 51 to 65 years, mean 55.6 years) and 5 age- and sex-matched healthy controls underwent conventional magnetic resonance imaging (MRI) and(More)
To determine whether eosinophils play a critical role in muscle fiber damage in patients with eosinophilic myositis (EM). We investigated expression of eosinophilic major basic protein (MBP) and interleukin (IL)-5 at the protein and mRNA levels in muscle biopsies from three patients with idiopathic EM. MBP deposits were found on the surface of eosinophils(More)
OBJECTIVE : The objective of this study was to characterize the involvement of costimulatory molecules in patients with polymyositis (PM). METHODS : A multiparameter flow cytometry analysis was used to identify peripheral blood mononuclear cell (PBMNC) subsets expressing CD8, CD4, and CD28 in 14 patients with PM, 10 patients with Duchenne muscular(More)
BACKGROUND Recently, paraneoplastic encephalitis associated with ovarian teratoma has been described and related to an autoantibody. METHODS We describe four patients with ovarian teratoma-associated encephalitis (OTE) and compared their clinical pictures with those of 17 previously reported patients with OTE. RESULTS Clinically, OTE was characterized(More)
We investigated expression of costimulatory molecules BB-1, B7-1 (CD80), B7-2 (CD86), and their counter-receptors CD28 and CTLA-4 (CD152) in muscle biopsy specimens of patients with scleroderma-polymyositis overlap syndrome (SSc-PM), primary polymyositis (PM), and other related diseases to examine whether the muscle fibers in patients with SSc-PM behave as(More)
This report describes a case of a 17-year-old girl with Charcot-Marie-Tooth disease (CMT) representing rigid spine and respiratory failure. At age 11, she tended to walk on her toes and had difficulty in getting up from the floor without support. She became aware of flexion limitation of the neck at the age of 12. At 15 years of age, She began to have(More)
Serial T2-weighted magnetic resonance imaging in a 29-year-old woman with juvenile type dentatorubral-pallidoluysian atrophy (DRPLA) demonstrated that a cerebral white matter hyperintensity appeared within 2 months after status epilepticus and persisted for more than 20 months. The patient had rapidly progressive mental regression and became akinetic after(More)
We reported a 68-year-old man with anti-phospholipid antibody syndrome who presented slowly progressive pure motor monoparesis(PMM) in left upper extremity as a sign of cerebral infarction. He had history of hypertension and hyperlipidemia. He first noticed clumsiness in left fingers, then weakness of left fingers with drop hand developing gradually in 2 to(More)
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