[A case of neuromyelitis optica associated with anti-aquaporin 4 antibody and other autoantibodies].

We report a patient with optic neuropathy and longitudinally extensive myelitis associated with anti-aquaporin 4 (AQP4) antibody and other autoantibodies. An 89-year-old woman presented with progressive numbness and weakness of the extremities which had acutely developed. She also complained of neck pain and gait disturbance.

[Autopsy case of dural thickening caused by widespread dural vein thrombosis associated with disseminated bone marrow carcinomatosis].

A 64-year-old woman was referred to our hospital because of disturbance of consciousness. She had undergone distal gastrectomy for gastric carcinoma 17 years previously. General physical examination was unremarkable, neurologic examination disclosed hyperactive deep tendon reflexes in the upper limbs.

[Support of home care and rehabilitation].

With the aging of society in Japan, the figure of Parkinson's disease is gradually increasing and the percentage of patients with severe disease has also increased. Among intractable neurological diseases, the possibility of bed confinement is the highest for this disease. Therefore, in addition to appropriate control of the disease using anti-parkinsonism drugs, maintenance of QOL, rehabilitation, the daily life therapy and treatment at home are necessary in terms of long-term management.

[A patient with von Recklinghausen's disease associated with polymyositis, asymptomatic pheochromocytoma, and primary hepatic leiomyosarcoma].

A 72-year-old woman with von Recklinghausen's disease was referred to our hospital because of pain and muscle weakness in her thighs. She had elevated serum values of creatine kinase, aspartate aminotransferase, alanine aminotransferase, lactate dehydrogenase, and aldolase. Based on these results, a diagnosis of polymyositis was made.

Decreased total nitric oxide production in patients with duchenne muscular dystrophy.

Plasma nitric oxide (NO) levels in Duchenne muscular dystrophy (DMD) patients were significantly lower than those observed in both healthy controls and in patients with other neuromuscular disorders. The correlation between NO level and ejection fraction was significant (r = -0.384, p = 0.

[Intractable neurological diseases and neurology].

In intractable neurological diseases, there are often no appropriate treatment methods even after admission and the course is frequently chronic. Therefore treatment at home is a major choice. In particular, in Parkinson's disease and amyotrophic lateral sclerosis, care at home while symptoms are stable is appropriate in terms of extension of ADL and the QOL of the patient and family.

Diagnosis of dystrophinopathy by skin biopsy.

We studied the expression of dystrophin in skin biopsy samples from 19 patients with neuromuscular diseases. Immunohistochemical procedures for dystrophin analyses were performed using monoclonal antibodies for three different domains. Arrector pili muscles, which are smooth muscles in the skin, expressed dystrophin in the patients with limb-girdle muscular dystrophy (5), facioscapulohumeral muscular dystrophy (1), and spinal muscular atrophy (3), and in normal controls (2).