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OBJECTIVES AND METHODS Seven families were studied with an axonal form of Charcot-Marie-Tooth disease (CMT) associated with mutations in the peripheral myelin protein zero (MPZ) gene-Thr124Met or Asp75Val. RESULTS Patients with these mutations commonly showed relatively late onset sensorimotor neuropathy predominantly involving the lower limbs. Sensory(More)
  • H Koike, K Misu, M Sugiura, M Iijima, K Mori, M Yamamoto +5 others
  • 2004
BACKGROUND Late-onset type I familial amyloid polyneuropathy (FAP TTR Met30) cases unrelated to endemic foci in Japan show clinical features setting them apart from early-onset cases in endemic foci. OBJECTIVE To compare pathologic features between the early- and late-onset types. METHODS Pathologic findings in FAP TTR Met30 with onset before age 50 in(More)
  • H Koike, K Mori, K Misu, N Hattori, H Ito, M Hirayama +1 other
  • 2001
BACKGROUND Although polyneuropathy related to chronic alcoholism has been reported frequently, its clinical features and pathogenesis remain to be clarified. OBJECTIVE To determine the clinicopathologic features and pathogenesis of alcoholic polyneuropathy associated with pain in patients with normal thiamine status, particularly in comparison to beriberi(More)
A novel mutation (Arg381Cys) in the second zinc-finger domain of early growth response 2 (EGR2) was identified in a late-onset Charcot--Marie--Tooth disease type 1 (CMT1) patient. This patient had initial symptoms of numbness and weakness in the leg at age 59, and a median nerve motor conduction velocity of 27 m/s. A sural nerve biopsy showed a severe loss(More)
Chronic inflammatory demyelinating polyneuropathy (CIDP), which can occur through life from childhood to old age, presents a wide variety of clinical phenotypes. We investigated the relationship between age of onset and phenotype in 124 CIDP patients. Clinical symptoms, pathologic findings and electrophysiologic features were assessed according to age at(More)
OBJECTIVE Polyneuropathy has been reported after gastrectomy performed to treat various lesions. Although thiamine deficiency is a possible cause of this neuropathy, the pathogenesis still remains to be clarified. Seventeen patients with peripheral neuropathy with thiamine deficiency after gastrectomy are described. METHODS Seventeen patients with(More)
Spinal cord sarcoidosis is a rare disorder whose natural history and therapeutic outcome are not fully known. We examined four patients with spinal cord sarcoidosis both clinically and radiologically, particularly in relation to corticosteroid treatment. The initial manifestation was cervical myelopathy in three and uveitis in one. All four patients(More)
  • K Mori, N Hattori, M Sugiura, H Koike, K Misu, M Ichimura +2 others
  • 2002
The authors report five patients with inflammatory demyelinating polyneuropathy with a Guillain-Barré syndrome (GBS)-like onset and initial clinical features, but with persistent symptoms similar to chronic inflammatory demyelinating polyneuropathy (CIDP). Patients in the chronic phase improved with corticosteroid or IV immunoglobulin therapy. Patients with(More)
OBJECTIVES To assess the extent of loss of myelinated nerve fibres and spinal motor neuron loss in chronic inflammatory demyelinating polyneuropathy (CIDP), a clinicopathological study was conducted on biopsied sural nerves and necropsied spinal cords from patients with CIDP. METHODS The myelinated fibre pathology of 71 biopsied sural nerves and motor(More)