Changes in the lymphocyte subsets during high dose immunoglobulin therapy for intractable childhood epilepsy.
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Biomedical subjects
Publications and source records attributed to N Michihiro.
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Two patients with slowly progressive muscle atrophy limited to only one leg are reported. They had pes equinovarus deformity and muscle weakness in the affected leg but no symptom in the other limbs. Muscle biopsies from the affected leg showed dystrophic changes consisting of variation in muscle fiber size, endomysial fibrosis, and necrotic and regenerating fibers. Dystrophin was normally expressed at the surface membrane of the muscle fibers. These two patients possibly had a variant of distal muscular dystrophy, though a neural influence could not be completely excluded.
Single photon emission computed tomography (SPECT) with N-isopropyl-p-[123I]-iodoamphetamine was performed twice at different times in 18 children suffering from partial seizures to evaluate the time-course changes of the hemodynamics in the focal region. Comparison of the 2 SPECT images revealed that a decreased regional cerebral blood flow (CBF) in the first was normalized in 4 cases (22.2%) and contracted in 7 cases (38.9%). The region of decreased blood flow in the second was contralateral to that in the first in 1 case (5.6%) and unchanged in 6 cases (33.3%). Most of the normalized and contracted cases were those in which clinical seizures disappeared and the EEG findings were normalized. In these cases which responded to treatment, the decreased regional CBF was also found to be improved. Repeated SPECT appears to be useful for evaluating the therapeutic efficacy. On the other hand, most of the unchanged cases were those in which clinical seizures were frequent or uncontrolled, or which persistently showed the abnormalities in their EEG. SPECT was considered to reflect well the conditions of the epileptic foci. These results indicate that repeated SPECT for observing the time-course changes of the regional CBF represents a useful technique with can be applied in evaluating the therapeutic efficacy or deciding the time to discontinue treatment and for pathogenetic elucidation of the epileptic foci.
An examination was made of high dose non-treated immunoglobulin (NTIG) therapy at an early stage of the West syndrome (WS). Six patients with cryptogenic WS who suffered attacks ranging from 15 days to 6 months (mean 70 days) and 5 patients with symptomatic WS who suffered attacks ranging from 14 days to 4 months (mean 32 days), were administered NTIG 6-10 times intravenously at 100-200 mg/kg of body weight at intervals of 2 or 3 weeks. All patients with cryptogenic WS showed complete remission in accordance with normalized electroencephalogram (EEG) without following anticonvulsants medication. Of the 5 patients with symptomatic WS one patient showed cessation of clinical seizures in agreement with EEG improvement and 2 other patients revealed transient cessation of clinical seizures with recurrence. In cases of complete remission, the energy percentage of the power spectrum for each frequency band displayed a tendency toward gradual decrease of delta wave band in correspondence with the increase of theta wave band percentage after NTIG therapy, suggesting that high dose NTIG may be useful for early treatment of cryptogenic WS and for inhibiting brain deterioration owing to epileptic encephalopathy.
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