Transcranial magnetic stimulation (TMS) has been used to describe cortical plasticity after unilateral cerebral lesions. The objective of this study was to find out whether cortical plasticity occurs after bilateral cerebral lesions. We investigated central motor reorganization for the arm and leg muscles in cerebral palsy (CP) patients with bilateral cerebral lesions using TMS. Seventeen patients (12 with spastic diplegia, 1 with spastic hemiplegia, and 4 with athetoid CP) and 10 normal subjects, were studied. On CT/MRI, bilateral periventricular leukomalacia was observed in all spastic patients with preterm birth. In two normal subjects, motor responses were induced in the ipsilateral tibialis anterior, but no responses were induced in any normal subject in the ipsilateral abductor pollicis brevis (APB) or biceps brachii (BB). Ipsilateral responses were more common among CP patients, especially in TMS of the less damaged hemisphere in patients with marked asymmetries in brain damage: in 3 abductor pollicis brevis, in 6 BBs, and in 15 tibialis anteriors. The cortical mapping of the sites of highest excitability demonstrated that the abductor pollicis brevis and BB sites in CP patients were nearly identical to those of the normal subjects. In patients with spastic CP born prematurely, a significant lateral shift was found for the excitability sites for the tibialis anterior. No similar lateral shift was observed in the other CP patients. These findings suggest that ipsilateral motor pathways are reinforced in both spastic and athetoid CP patients, and that a lateral shift of the motor cortical area for the leg muscle may occur in spastic CP patients with preterm birth.

Twenty hemiplegic patients were studied with transcranial magnetic stimulation (TMS). Motor evoked potentials (MEPs) of the biceps brachii (BB) and the abductor pollicis brevis muscles (APB) were recorded on both sides simultaneously. TMS was carried out with an 8-shaped coil over different scalp positions in the intact hemisphere. Bilateral MEPs of BB were elicited in patients with later childhood lesions as well as early lesion, but those of APB were only elicited in the latter (up to 2 years). In patients with prenatal or birth lesion on BB and in all patients on APB, cortical maps of MEP amplitude of paretic and non-paretic sides showed similar distributions. There were no remarkable differences in mean latency between both sides, and correlation coefficients of MEP amplitude between both sides were high in these patients. In patients with postnatal lesion on BB, MEP maps of both sides showed different distributions, ipsilateral MEP latencies were delayed and correlation coefficients were low. We suspect that ipsilateral MEPs after early lesion derive from axonal sprouting both in the proximal and the distal muscles. After postnatal lesion, other mechanisms of ipsilateral motor projection take place in the proximal muscles, but not in the distal ones.

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After the DNA diagnosis, we evaluated the prevalence of Huntington''s disease (HD) in the San-in area of Japan, and confirmed the founder effect. There were 10 patients with HD in the San-in area, who were diagnosed clinically. The expansion of the CAG repeat was observed in 9 patients with HD members in their families, although those family members of the patients had already died. In the patient who had no positive family history, expansion of the CAG repeat was not seen. The prevalence of HD was 0.65/100,000 in this area. The common haplotype studied by the polymorphism marker of D4S136 was shown in all 9 HD patients, although they were observed in only 2.7% of the normal population. These results suggested a common ancestor of these HD patients.

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Summary:Purpose: To report a previously undescribed adverse effect, IgA/IgG subclass deficiency associated with zonisamide (ZNS) therapy.Methods: Serum IgA and IgG subclass levels were determined by the turbidimetric immunoassay and enzymelinked immunosorbent assay, respectively, in a 2-year-old boy with postmeningitis sequelae who was treated by ZNS.Results: Four months after initiation of ZNS, combined deficiency of IgA and IgG2 was noted. After cessation of ZNS, serum IgA level was promptly increased. IgG2 level was gradually increased, but remained subnormal after 7 months.Conclusions: This case documents, for the first time, the action of ZNS on IgG immune system as well as IgA system.If patients with ZNS therapy showed IgA deficiency and recurrent infections, it is preferable to check serum IgG subclass concentrations as well. Key Words: ZonisamideEpilepsy-IgA deficiency-IgG subclass-Phenytoin.Secondary IgA deficiency has been reported in patients treated with various drugs such as epileptic drugs (AEDs), D-penicillamine, gold, and sulfasalazine (1,2). Combined deficiency of IgA and IgG subclasses has been observed in association with phenytoin (PHT) and sulfasalazine therapies (3,4). There have been only a few reports of IgA deficiency or low IgA levels in children treated with a new benzisoxazole AED: zonisamide (ZNS) (5,6). We report a patient who developed IgA and IgG2 subclass deficiency associated with ZNS therapy. CASE REPORTAt age 17 months, an otherwise healthy boy developed bacterial meningitis. He was treated with antibiotic agents and phenobarbital (PB). At that time, serum immunoglobulin levels were normal (Table 1). Because the boy had postmeningitis sequelae, i.e., spastic quadriplegia, epilepsy, and mental retardation, he was transferred to our division for rehabilitation. After discharge, he was treated with PB (40 mg/ day; serum concentration, 12.3 pglml) and muscle relaxants. At age 2 years 3 months, ZNS (80 mg/ day) was added to his regimen because of secondarily generalized seizures and increases in epileptic discharges on EEG. Four months after initiation of ZNS therapy, a deficiency in serum IgA (<5 mg/dl) and IgG2 (21.0 mg/dl; normal range at age 2-4 years; 58.5-292.1 mg/dl) levels was noted, with decreased serum levels of total IgG (379 mg/dl) ( Table 1). Serum IgG1, IgG3, and IgG4 concentrations were within normal ranges for children of his age (7) (normal range of IgG1, 390.2-955.2 mg/dl; normal range of IgG3,11.4-98.8 mg/dl; normal range of IgG4,1.2-76.7 mg/dl). Total IgG, IgA, and IgM concentrations were measured by turbidimetric immunoassay (8). IgG subclasses were determined by enzyme-linked immunosorbent assay (ELISA) (9). Three examinations during ZNS therapy consistently showed IgA deficiency and decreased total IgG levels. Serum concentration of ZNS was not in the toxic range (15.2 pglml). Because the patient showed no susceptibility to infections, intravenous y-globulin therapy was not initiated. Three weeks after discontinuation of ZNS therapy, serum IgA levels were nor...

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The case of a patient who developed thrombocytopenia during treatment with carbamazepine (CBZ) is described. The platelet count recovered soon after discontinuation of CBZ, Lymphocyte stimulation test with carbamazepine-10, 11-epoxide (CBZ-10, 11-EPOX), a major metabolite of CBZ, was positive, although with CBZ it was negative. These findings suggest that CBZ-10, 11-EPOX was possibly causative in the pathogenesis of CBZ-induced thrombocytopenia in this case.