| Budget Amount *help |
¥1,000,000 (Direct Cost: ¥1,000,000)
Fiscal Year 1998: ¥500,000 (Direct Cost: ¥500,000)
Fiscal Year 1997: ¥500,000 (Direct Cost: ¥500,000)
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| Research Abstract |
1). We compared sleep parameters in all-night polysomnography (PSG) with pathological findings of the brainstem in a two-year-old male child, showing the similar neuropathological distribution to that in Leigh disease. In PSG, the percentage of slow wave sleep was reduced, while that of REM sleep was within normal range. The average frequency of submental muscle twitches showed a mild decrease, although the number of rapid eye movements was preserved. The tone of submental muscle was maintained and suppressed during non-REM and REM sleep, respectively. The sleep parameters were less affected than in Leigh disease. Neuropathologically, both the number of neurons and the percentage of tyrosine hydroxylase (TH)-immunopositive neurons were reduced in the locus ceruleus of the patient, however, the brainstem lesions were not so severe as those in Leigh disease. These data suggested that there might be a correlation between physiological dysfunctions and pathological changes in the human brainstem.
2). To investigate the involvement of the brainstem lesion in the pathological mechanism of infantile spasm, we immunohistologically examined the expressions of neurotransmitters and calcium-binding proteins in the brainstem in autopsy cases who suffered from both West and Lennox-Gastaut syndromes (WL), consisting of four subjects each of lissencephaly and a sequela of perinatal hypoxic ischemic encephalopathy. TH-immunopositive neurons were reduced in the dorsal motor nucleus of the vagal nerve in all of the WL patients, while most of tryptophan hydroxylase-immunopositive structures were preserved. In the anti-parvalbumin (PV) immunostaining, the brainstem auditory system was poorly identified in the WL patients. It is more likely that the disturbance of immunoreactivities for TH and PV observed in our WL autopsy cases can be related to the pathogenesis of infantile spasm, because the disease controls did not show any changes in either of immunostainings against TH and PV.
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