Since it has been recently demonstrated that ketone bodies can replace glucose as the predominant fuel for brain metabolism during starvation, spontaneous hypoglycemia should be classified according to the concomitant changes in the concentration of ketone bodies (and FFA) in plasma. In this paper the following classification of hypoglycemic syndrome is presented. Its efficacy depends on understanding the pathogenesis and severity of brain damage of the hypoglycemic syndrome.
The incidence and the meaning of the metacarpal pseudoepiphysis (PE) found in the hand X-rays of mentally retarded children has yet to be clearly determined. Statistical analysis of examinations of 67 Down's children, 378 non-Down retarded children and 478 normal children with ages ranging from 2 to 9 showed; (a) high incidence of PE among the retarded, 48% in Down's, 16% in non-Down's and less than 3% in normal children; (b) extreme male dominance of PE holders except for Down's; (c) high complications of multiple minor anomalies in PE holders; (d) high occurrence of first or only child in the PE holding retarded; (e) high incidence of late ambulation and speaking, quiet passive behaviors in PE holding retardedz (f) high occurrence of β activity in EEG of PE holding retarded. Several transferring cases are found among the three groups. For examples, one mosaic Down's syndrome without clinical stigmata is found among the PE holders and several students with borderline IQ are seen among the normal PE holders. Thus, this study supports the position that pseudoepiphysis can be a stigmata of mental retardation or developmental aberration and should be included in developmental diagnosis.
Sulfaphenazole, a long acting sulfonamide, was effective on intractable cases of minor seizures of children, especially on those with akinetic seizure or Lennox syndrome. The antiepileptic effect of sulfaphenazole developed rapidly, frequent seizures being abolished about 3 days after the administration and electroencephalograms improved markedly about 10 days after the administration. In two cases, seizures were completely controlled by the sulfaphenazole treatment alone. This drug was found to be ineffective on grand mal seizures.
In preterm and full-term infants, the EEG during quiet sleep is characterized by burst of large amplitude slow waves with sometimes superimposed rapid rhythm (burst), alternating with attenuated periods of various frequencies (suppression) … so called “trace alternant”. This tracé alternant pattern changes with conceptional age. In early conceptional ages, the “suppre ssion” period shows, in practice, no electrical activity. The difference between “burst” and “suppre ssion” becomes less distinct with increasing conceptional age. To demonstrate this difference quantit atively, the frequency analysis was serially performed by means of automatic analyser in 8 preterm infants, for each 5 second epoch of “burst” and “suppression”, The ratios of integrated values for suppression period to those for burst period increased in all frequency bands (σ1: 2-4c/s, θ: 4-8c/s, α: 8-13c/s, β1: 13-20c/s, β2 20-30c/s) with conceptional age. The same maturational change was also found in the ratios of total energy of “suppression” to “burst”. On the contrary, there was no difference in the power spectrum in burst and suppression at all conceptional ages. The gradual increase of the electrical activity in suppressed part of trace alternant, which has been substantiated quantitatively in this study, may be due to the maturation of the cerebral cortex in infants.
A case of classical Niemann-Pick disease was reported. The patient was 16 month old female. At the age of 10 months hepatosplenomegaly was noticed, and thereafter mental and motor functions deteriorated progressively. Rigidospastic quadriplegia, severe mental dementia and extreme enlargement of the liver and spleen were noted at admission. Cherry red spot was recognized in the optic fundi and her chest x ray film showed diffuse miliary shadows. Triglyceride in the serum was increased, and foamy cells were present both in the peripheral blood and in the bone marrow. The liver taken by needle biopsy showed typical appearance of Niemann-Pick disease by light and electronmicroscopically and biochemical study revealed increased sphingomyelin in the liver. At 24 months of age, she expired for bronchopneumonia at home. Autopsy was not permitted.
The children with infantile spasms (West's syndrome) were studied electroencephalographycally and bioc, hemically. 1) Forty-four percent (8 of 18 cases) of infantile spasms, most of which showed no response to the ACTH-Z treatment, changed into the Lennox syndrome. 2) Tn 8 of 18 cases, the electroencephalographic pattern changed from hypsarhythmia to 2 c/s spike nd wave pattern. 3) The cases of infantile spasms with a history of perinatal injuries were associated with cerebral palsy in high percentage. 4) The 5-hydroxyindolacetic acid (5-HIAA) level in lumbar cerebrospinal fluids of these patients with Lennox syndrome is low (29-56 ngm/ml) in comparison with that of the other neurological patients (45-82 ngm/m/).
Electron microscopic study was performed on the brain of an autopsy case of Hallervorden-Spatz disease. The patient developed unsteady gait and night blindness at the age of 4 years, and then dystonia and opisthotonus at the age of 10 years. She died of status epilepticus 4 days after the right stereotaxic thalamotomy performed at the age of 11 years. Frontal section of the brain revealed bilateral rust-brownish discoloration in the globus pallidus and the substantia nigra, which microscopically showed the brown pigment granules and numerous spheroids. Electron microscopic obsevation on the globus pallidus showed the lysosome-like bodies containing ferritin-like granules in neurons and glial cells. Appearance of the dense material and lipid in the lysosome-like bodies was considered to be analogous to the lipofuscins, which were observed as the pigment granules in the light microscopy. Some axons were filled with abundant mitochondria, myelin-like bodies and dense bodies. A few oval bodies composed of the multilamellated bodies and lysosome-like bodies were observed, which were observed as spheroids in the light microscopy. However, the relationship to infantile neurcaxonal dystrophy and significance of the lysosome-like bodies and multilamellated bodies still remained unknown.