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Biomedical subjects

O Narazaki

Publications and source records attributed to O Narazaki.

15 recordsLinked to original sources
PubMed1999

Treatment of infantile spasms with zonisamide.

We determined the efficacy of and tolerability to zonisamide (ZNS) in newly diagnosed patients with infantile spasms. ZNS, 4-20 mg/kg per day, was introduced as an add-on therapy or monotherapy in 27 children with infantile spasms (cryptogenic, 2; symptomatic, 25). The dosage was initially 2-4 mg/kg per day, and then was increased by 2-5 mg/kg every 2-4 days until the seizures disappeared. Nine (33.3%) out of the 27 patients who were administered ZNS exhibited the disappearance of seizures. ZNS was effective in all the cryptogenic cases and seven (28.0%) of the symptomatic cases. The effective daily doses were 5-12.5 mg/kg (mean, 7.8 mg/kg), and the daily dosages were 40-100 mg (mean, 61.1 mg). The steady-state plasma ZNS concentrations were almost within the therapeutic range. The mean time interval between the start of ZNS therapy and the seizure disappearance was 5.0 days. Six (75.0%) of eight effective cases, the exception being one for whom EEG was not performed after the therapy, showed the disappearance of hypsarrhythmia. The recurrence of seizures were observed in four of the nine cases. No adverse reaction to ZNS was noted in any patient. In conclusion, ZNS treatment is considered to be worthwhile trying, for early stage therapy for infantile spasms.

Anticonvulsants↗
PubMed1998

[A case of hereditary motor and sensory neuropathy (HMSN) with excessive myelin outfolding with autosomal recessive inheritance].

A 3-year-old girl with rare and unique myelin abnormality in the sural nerve is described. Her parents are first cousins. Her clinical features were similar to those of HMSN I. Therefore, a diagnosis of autosomal recessive form of HMSN was made. However, in histopathologic studies of the sural nerve, it was observed that the folded myelin did not extend into the axon but into the outer portion of Schwann cell cytoplasm, so called excessive myelin out folding. The density of both the myelinated and unmyelinated fibers was less in the patient than in controls. In recent years, the patient described above is usually classified under HMSN IVB or (Charcot-Marie-Tooth disease) CMT4B, and molecular genetic studies are now in progress.

Child, Preschool↗
PubMed1997

Hypogammaglobulinaemia in a patient with ring chromosome 21.

An 8 year old boy with ring chromosome 21 who was susceptible to sinorespiratory infections due to hypogammaglobulinaemia is reported. He presented with the characteristic features of monosomy 21 syndrome, such as psychomotor retardation, hypertonia, large saccular ears, prominent nasal bridge, micrognathia, thrombocytopenia, and patent ductus arteriosus. His serum IgG concentration was less than 1.5 g/l at 3 years and 6 months of age after repeated hospitalisations with pneumonia, otitis media, and convulsions. Regular replacement of intravenous gammaglobulin effectively reduced such infectious episodes. A predisposition to infection in patients with ring chromosome 21 may be explained by hypogammaglobulinaemia and merit treatment with gammaglobulin.

Agammaglobulinemia↗
PubMed1986

Brainstem auditory evoked potentials in children with neurodegenerative diseases.

We assessed the usefulness of brainstem auditory evoked potentials (BAEPs) for differentiating types of degenerative diseases in children. The findings of BAEPs were abnormal in one out of two with polioencephalopathies and in seven of the eight with leukoencephalopathies, showing prolonged interpeak latencies of waves I-III, I-V or disappearance of wave components. On the other hand, normal responses were observed in four with corencephalopathies and one with spinocerebellopathy. The serial BAEPs in eight patients had deteriorated with progression of the clinical symptoms. These data on 16 children suggest that BAEPs are indeed a useful tool for monitoring pathophysiologic processes of neurodegenerative diseases.

Adolescent↗
PubMed1986

A case of hereditary motor and sensory neuropathy type III with a decrease in unmyelinated fibers.

We report a 3-year-old girl with hereditary motor and sensory neuropathy type III with a decrease in unmyelinated fibers. There have been few reports of such cases. The present findings suggest the possibility that the primary lesion in this disease is in the axons. We consider that more attention should be paid to the changes in unmyelinated fibers and axons in further studies on this disease.

Child, Preschool↗
PubMed1985

Menkes disease: is vitamin C treatment effective?

The pathogenesis of Menkes disease seems to be linked to metallothionein which binds to copper trapped within cells in some tissues. The only known therapy for this disease is parenteral administration of copper, but the effects are equivocal. We treated a patient with Menkes disease by giving vitamin C orally. The clinical manifestation and bone changes improved and the plasma copper and ceruloplasmin levels gradually increased. Vitamin C may prevent the binding of copper and metallothionein by its reducing effect, and excess copper would be released from the cells. Vitamin C treatment is a simple and physiological method, and should aid in clarifying the pathogenesis of the disease.

Ascorbic Acid↗
PubMed1983

Ocular manifestations of congenital muscular dystrophy (Fukuyama type).

Ocular manifestations in two cases of congenital muscular dystrophy of Fukuyama type were reported. This disease is characterized by early onset of hypotonia, generalized muscle weakness and atrophy, mental retardation, and elevated serum creatine-phosphokinase activity. The symptoms include entropion of lower lids, pathological myopia with astigmatism, optic nerve pallor, and irregular grayish subretinal mottling. Case 1 showed additional features of posterior staphyloma, dragged papillomacular vessels, peripheral grayish-white discoloration of the retina, and rete mirabile as well as abnormal vascular anastomosis.

Astigmatism↗
PubMedDate not supplied

Central nervous system disorders and possible brain type carnitine palmitoyltransferase II deficiency.

We describe two male infants with central nervous system disorders, i.e. infantile spasms in one and athetotic quadriplegia in the other, and with recurrent attacks of high plasma creatine kinase levels induced by viral infections. Although carnitine palmitoyltransferase I (CPT I) activity in biopsied muscle was normal in both cases, that of carnitine palmitoyltransferase II (CPT II) was decreased to 37% and 25% of the control value, respectively. Meanwhile, to determine whether or not and how CPT exists in the central nervous system (CNS), we studied animal brain tissues. CPT activity was demonstrated in almost all regions, especially in the brainstem, cerebellum and spinal cord. Although CPT deficiency can be classified into hepatic (CPT I) and muscular (CPT II) presentations, these data suggest that another symptomatology of CPT II deficiency with CNS involvement (brain type?) might exist.

Animals↗
PubMedDate not supplied

Prognosis of occlusive disease of the circle of Willis (moyamoya disease) in children.

The prognosis of 27 patients with moyamoya disease was studied. The ages at onset ranged from 11 months to 4-11/12 years. Follow-up study was performed within 4 years from the onset in 13, 5 to 9 years in 5, and 10 to 15 years in 9. Transient ischemic attacks (TIA) occurred most often during the first four years and decreased thereafter. Intellectual deterioration and neurologic deficits increased with time. Outcome included no sequelae in five (19%), occasional TIA or headache alone in nine (33%), mild intellectual and/or motor impairment in seven (26%), requirement for special school or care by parents or institutions after reaching the teen years in three (11%), continuous 24-hour care in two (7%), and death in one (3%). Poor prognosis was correlated with an early age at onset and hypertension.

Adolescent↗