Shozo Nakano

Age related incidence of cavum septi pellucidi and cavum vergae on CT scans of pediatric patients.

The computed tomography scans of 1.050 infants and children with various degrees of neurological involvement were reviewed to determine the incidence of cavum septi pellucidi (CSP) and cavum Vergae (CV). The incidence was 10% in patients under 1 year, gradually decreasing to 5 to 6% at 2 to 5 years, 2.7% at 6 to 9 years, and 2.3% at 10 to 14 years, with an average rate of 5.59%. An attempt was made to assess possible relationships between clinical syndromes (convulsive disorders, developmental delays, and others) and the presence of CSP-CV. No solid statistical evidence of such relationships could be established.

Landau-Kleffner syndrome EEG topographic studies

Spectral and historical topographic mapping of EEG was done on 2 siblings with Landau-Kleffner syndrome. The clinical features of the elder sister were acquired aphasia developed at the age of 5, followed by convulsions a year later, and those of the younger brother were progressive ataxia, hemiparesis, urinary incontinence and convulsions at the age of 4 years and 10 months, followed by acquired aphasia a year later. The most prominent spectral mapping features were high spectral powers of delta, theta and alpha waves over the fronto-centro-parietal area. The power of alpha and beta wave bands, reflecting sharp or spike waves, varied spatio-temporally over the central, parietal, temporal and frontal areas. The historical mapping revealed variabilities of paroxysmal discharges in modes of propagation. These results suggested that electrophysiological dysfunction of the fronto-centro-parietal areas associated with markedly unstable paroxysmal discharges is the main feature of Landau-Kleffner syndrome.

MR imaging of a group I case of Hallervorden-Spatz disease.

Magnetic resonance (MR) imaging of a case of Hallervorden-Spatz disease (HSD) using a 1.5 T system is described. The patient showed progressive spastic diplegia with equinovarus deformity of the feet, dystonic postural movements, dysarthria, dysphagia, mental deterioration, optic nerve atrophy, and peripheral neuropathy. These clinical features were compatible with HSD. Symmetrical, decreased signal intensity was seen on both proton density weighted and T2-weighted spin echo images in the globus pallidus as well as in the substantia nigra (Group I). This MR finding suggests an increased iron deposition in these subcortical nuclei, which is characteristic of HSD. The characteristic MR imaging, together with the relevant clinical features, was considered to be useful for establishing the diagnosis of HSD.

18p− syndrome associated with hemivertebrae, fused ribs and micropenis

SummaryAn 11-month-old boy was found to have deletion of the short arm of chromosome 18. He was a short hypotonic infant with thoracic hemivertebrae, fused ribs and micropenis in addition to developmental delay, ptosis of the eyelids, epicanthal folds, hypertelorism, large prominent and low-set ears, micrognathia and short neck.Banding analysis showed the patients karyotype to be 46, XY,del(18) (p1103).

Computed Tomography In Postinfluenzal Encephalitis

A 2-year-old girl developed encephalitis following influenza A virus infection. Brain computed tomography (CT) showed symmetrical hypodense areas in the putamina. Contrast enhancement was noted surrounding the hypodense areas 2 days after the onset of encephalitis. These localized lesions demonstrated on CT have not been reported in the literature of postinfluenzal encephalitis.

Carbamazepine Therapy and LongTerm Prognosis in Epilepsy of Childhood

Summary: Sixty‐seven of 90 patients (74% who had been treated with carbamazepine (CBZ) alone were seizure‐free for > 3 years. The EEG of the patients given CBZ monotherapy was more often normal in those without neurologic abnormalities other than mental retardation or a genetic predisposition. The prognosis of patients with partial seizures secondarily generalized was poorer than that of the other patients. Patients without mental retardation more often had monotherapy CBZ. The lowest blood level of CBZ for maintenance was considered to be 4 μg/ml, although the therapeutic blood level was between 6 and 12 μ.g/ml. Most of the side effects were mild.

Cerebellum and brain stem atrophy in a child with Huntington's chorea

An 11-yr-old girl with Huntingtons chorea since the age of 4 had mental deterioration, chorea, rigidity, generalized convulsions and cerebellar ataxia. Computerized tomography (CT) showed atrophy of the cerebellum and brain stem in addition to atrophy of the caudate nucleus and cerebral cortex.

MRI in infantile neuroaxonal dystrophy

A 6-year-old boy with the typical clinical features of infantile neuroaxonal dystrophy was examined with magnetic resonance imaging. The findings suggested increased metal deposition in the globus pallidus. Magnetic resonance imaging findings of Hallervorden-Spatz syndrome and infantile neuroaxonal dystrophy are similar, suggesting that these two disease entities overlap. Magnetic resonance imaging findings, as well as relevant clinical features, may be useful in the diagnosis and classification of infantile neuroaxonal dystrophy.

Tuberous sclerosis with increasing calcification in the brain during a 15-month period: A case report

We report here a patient with tuberous sclerosis who, over a 15-month period, had markedly increasing brain calcification on CT (computed tomography). When first seen, the 2-month-old boy had a white spot on the right thigh. His mother, maternal uncles, grandfather and great-grandfather had not only white spot but also sebaceous adenoma. Infantile spasms were noted 2 days after birth. His first brain CT at 3 months of age showed a slightly higher than normal density area in the right occipital lobe. Subsequent CT scans at 6 and 18 months of age revealed progressively increasing density in this area (CT number above 60).