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Kearns-Sayre syndrome (KSS) is a subgroup of the mitochondrial encephalomyopathy whose main features are ophthalmoplegia, retinal pigmentary degeneration, heart conduction block with other minor neurological disorders. The most common neuropathological findings for this disorder is spongy degeneration in the white matter of the brain. In this study, we examined the neurological findings and the brain CT or MRI of our seven patients with KSS. CT and MRI revealed cerebral cortical atrophy in five of seven patients with mental deterioration and abnormal intensity lesions in the basal ganglia, midbrain and cerebral and cerebellar white matter of one patient who showed episodic unconsciousness due to lactic acidosis. Neurological and MRI findings differ quantitatively and qualitatively between patients with KSS.

The main symptom of vertebrobasilar insufficiency (VBI) -transient ischemic focal neurological signs due to decreased blood supply to the vertebrobasilar resion- is vertigo, though vertigo without any other neurological signs (solo vertigo) is not enough to diagnose VBI. The etiological factors of VBI are the hemodynamic disorder like postural hypotension, cardiac arrythmia or blood hyperviscosity associated with athelosclerosis in arteries, usually in larger vessels of vertebrobasilar system or fibromuscular dysplasia, cardiac embolism, compression to vertebral arteries with bony deformity etc. It is very important to find out the patients who are in the stage of impending stroke and need antiplatelet therapy among those with solo vertigo. In this purpose, MR angiography is becoming to be a very useful non-invasive tool to verify the arterial stenosis or obstruction.

Mixed connective tissue disease(MCTD) is a syndrome with overlapping clinical features of SLE, scleroderma and polymyositis. Multiple organs like skin, joint, muscle, lung, heart, kidney and so on are affected with this entity. Nervous system disorder is one of the important complications in MCTD. We reported four patients with MCTD who had various neurological complications such as trigeminal neuropathy, meningitis, cerebral infarction and polyneuropathy respectively. The percentage of MCTD patients with neurological complications such as trigeminal neuropathy, meningitis, cerebral infarction and polyneuropathy respectively. The percentage of MCTD patients with neurological complications is reported as 10～50%, in which, the trigeminal neuropathy and meningitis are most frequent. Psychosis, convulsion, cerebellar ataxia and progressive stupor are also reported to be seen in MCTD. Myositis is reported to manifest in 70% of MCTD patients. The clinicopathological feature of myositis in MCTD is not clearly differed from pure polymyositis (PM), however the muscle pathology of the myositis in MCTD tend to show more infiltrated inflammatory cells at perimysium than PM, whearas PM shows larger number of muscle fibers with simple necrosis. Neuropathic changes are frequently seen neurophysiologically and pathologically in MCTD. PM is seen in younger age group. The pharyngeal and respiratory muscles are more frequently affected in PM. PM shows higher CK levels at acute stage. In this article, we focused on an unexpectedly high incidence of various neurological complications in MCTD and compared the features of myositits in MCTD with PM.

This is a report of two patients with subacute cerebellar degeneration associated with cancer whose sera showed antibody activities against nervous tissue elements. Using an immuno-blotting method, the serum of a 68-year-old man with subacute cerebellar degeneration and malignant lymphoma reacted with rat cerebral 250-kd and 110-kd and cerebellar 110-kd acidic cytoplasmic proteins. The serum IgG of the second patient, a 50-year-old woman, who had subacute cerebellar degeneration, Lambert-Eaton myasthenic syndrome and small cell cancer of the lung showed antibody activities to rat cerebral and cerebellar 98-kd neutral cytoplasmic protein and 68-kd membrane protein. These sera did not react with the proteins prepared from human and rat brain obtained several hours after death, which indicated that these antigen proteins were easily degraded and needed to be prepared quickly after death with protease inhibitors. These patients' sera did not react with proteins prepared from rat liver, kidney, spleen, heart muscle and diaphragm and the antigen proteins reacted with these sera might be brain specific. Control sera did not react with any of the brain proteins. The serum of each patients recognized different antigens. Other reported antigens in the literature are also different which indicates those proteins reacting with serum antibody are variable indiviaually among patients with subacute cerebellar degeneration.

近年,保育現場において,子どもの理解や保育の方法を議論する場として注目されている保育カンファレンスは,子どもの理解や支援の検討にあたり,グループダイナミクスによる効果が期待できる。本論では,園外の専門家との協働による保育カンファレンスにより,発達に課題のあるA児に対する支援を検討した実践事例を報告する。, A児は進級当初,他児のモノに興味をもち,無断でこれを取り,思い通りにならないと癇癪を起すといった姿が見られた。これを受けてカンファレンスではルーティンの見直し,声かけの方法の提案などが行われ,根気強い「貸して-いいよ」といった会話スキットの強化が行われた結果,後期には他児の弁当やカバンなどに興味をもった際に言葉で依頼を行うA児の姿が見られるようになり,他児とかかわることに喜びを感じるようになった。, また,本年度は療育機関関係者も招いてカンファレンスを行ったことにより,新たな視点でA児の姿を捉えることができ,園での支援を工夫することにつながった。, 今後は,A児が年長児クラスに進級することから,進学準備・小学校との連携を視野に入れたカンファレンス・支援体制を検討していくことが求められる。