It is now generally recognized that excessive amounts of fluid may be collected in the subdural space after bacterial meningitis in infancy. During the past year three cases, whose ages were 2 weeks, 5 months and 6 months respectively, with subdural effusion after purulent meningitis due to Alkalescens dispar or Escherichiacoli were admitted to our clinic. Convulsive seizures, hemiparesis, irritability and fever were seen in all cases after apparently sufficient tretment of acute meningitis with broad spectrum antibiotics, and diagnostic subdural tap, carotid angiography and subdural pneumography revealed the presence of subdural effusions. Out of three cases 2 were successfully treated by repeated subdural tappings only and the remaining case was cured by subdural-peritoneal shunt following evacuation of the effusion. In order to prevent severe neurologic deficits or mental retardation after postmeningitic subdural effusion in infancy, the early diagnosis is essential and for this purpose diagnostic subdural tap should be performed routinely when patients do poorly after apparently satisfactory treatment of the acute stage of meningitis. Finally, the literatures so far reported were reviewed and incidence, diagnosis, and treatment of postmeningitic subdural effusion in infancy were discussed.
A boy, aged 13 years, was hospitalized because of polydipsia, polyuria, speech disorder and waddling gait. Birth and early motor and psychologic development had been normal. At age 10 years, a decline in his school record appeared. At age 11 years, he became hesitant to walk and unable to run. The parents noted his constant excessive thirst. On admission, small testes and gynecomastia were noted. The gait was somewhat ataxic;neurologic examination showed hyperactive knee jerk, cerebellar ataxia and bilateral horizontal nystagmus. Carotid and vertebral angiography were normal, but ventriculography showed slight dilatation of the 3 rd ventricle. An EEG was borderline normal. Visual fields and funduscopic examination revealed normal. The testes biopsy revealed aspermatogenesis, but the chromosome analysis indicated normal male karyotype. The cerebrospinal fluid contained 100mg/dl protein and Ig-G was elevated in serum and spinal fluid. Extensive endocrinologic studies provided definte evidence of malfunction of the hypothalamichypophysial system such as central diabetes insipidus, hypogonadotrophic hypogonadism and secondary hypothyroidism. These findings differred from brain tumor, xanthomatosis, subacute encephalitis, hereditary spinocerebellar ataxia and multiple sclerosis. Two similar cases have heretofore been reported by Toyokura et al. (1967) All these cases have the following symptoms in common; (1) cerebellar ataxia, (2) progressive pyramidal signs, (3) central diabetes insipidus, (4) mental retardation with euphoria and (5) no remarkable cause of onset followed by gradual deterioration. This set of symptoms and signs might be a new clinical entity. The author discussed the similarity of this syndrome to “Scrapie”of sheep and“Kuru”in New Guinea and suggested that these cases might be due to slow virus infection.
A 4 years old boy complained of gait and speech disturbances which occured from about six months before. Carotid angiography revealed cerebral arteriovenous malformation (fistula) which had two afferent arteries from internal carotid artery and basilar artery. So we could not performed surgical treatment to him. The continuous murmur was heard on the surface of his cranium. ECG showed left ventricular hypertrophy with strain pattern. EEG showed diffuse but r-hemispheric dominant slow activity.
Serial electroencephalographic examinations were performed during the course of intermittent peritoneal dialysis in a 10 year old uremicpatient who had no family or past history of epilepsy. At the height of uremia with maximal biochemical deviation, EEG showed marked slow wave dysrhythmia and occasional bursts of bilaterally synchronous high voltage slow waves, which gradually improved to show normal basic rhythm in the course of treatment. However, with clinical and electro-encephalographic improvement, bilaterally synchronous atypical spike and wave complexes appeared in all leads. Marked slowing of EEG at the height of uremia may reflect a primary cortical involvenment and bilaterally synchronous high voltage slow wave bursts may reflect involvement of brain stem mechanisms of presumed reticularorigin. Later development of bilaterally synchronous atypical spike and wave complexes with improvement of cortical background activity is consistent with the known pathology of uremia in which brain stem reticular formation is particularly involved together with cerebral cortex. The above finding is one of evidences to support the concept that origination of bilaterally synchronous spike and wave complex will require the involvement of cortico-reticular system.
Our study was undertaken to investigate the in vivo effects of several amino acids on both the uptake of 14C-l-leucine into the brain and the incorporation of 14C-l-leucine into the brain protein by using 17-day old rats. The rats with hyperaminoacidemia, previously produced by intraperitoneal loadings of 0.15 or 0.30 m-moles of a single amino acid twice a day, were intraperitoneally injected with 14C-leucine, and then, sacrificed one hour following the 14C-leucine injection. It was found under the above-mentioned experimental condition, that phenylalanine in excess inhibited not only the uptake of 14C-leucine into the brain by 50% but also the incorporation of 14C-leucine into brain protein by 48%. The similar inhibitory effect was observed in case of loading of valine, histidine or methionine, too. The present results suggest that a few specific amino acids in excess influence both the intracellular accumulation and the incorporation into protein of other amino acids in the brain. It is entirely possible that disturbed transport of amino acids may induce a disruption of the normal balance of free amino acid pools in the brain. The reduction of protein synthesis may partially be accounted for the deficient substances normally present in optimal concentrations in the brain. Decreased protein synthesis in brain may, in part, explain a cause of neurological dysfunction seen in most of inborn errors of amino acidmetabolism such as PKU.
During recent 12 years, 16 children with severe head injuries were admitted to our clinic. On admission deep coma, unequality of pupillary size, and decerebrate rigidity were seen in all cases. In these cases, mortality and restitution were studied and compared with those of the adult cases with severe head injuries. Finally, the influences of severe head injuries on the further physical and mental development in infancy and childhood were discussed. Out of 7 cases whose ages were under 3 years, 4 died during the acute stage and the remaining 3 cases showed more or less retardation in both physical and mental development in the 1 to 4 years' observation after head injuries. It was conspicuous that in the cases whose ages were ranging from 4 to 15 years the mortality was quite low and all the survived cases including 4 cases having had prolonged coma over 4 weeks showed markedly good recovery in neurological and mental functions within the relatively early period after the injury and most of them were able to spend fairly normal school lives. On the contrary, it was evident that the surviving adult cases after severe head injuries showed various kinds of neurologic deficits and marked retardation of mental functions, that made the patients difficult to return to the former occupations or normal social lives.
For the last three years the authors noticed the fact that patients of infantile spasms exhibited a marked but transient improvementof epileptic status concurrent with infection of exanthema subitum. In all three cases, epileptic fits disappeared in 2 or 3 days afterfever-rising of exanthema subitum, and such favorable condition continued for 1.5 to 7 months afterwards. By contrast, another 18 cases with infantile spasma, suffered from high fever caused by acute pharyngitis or acute pneumonia, showed no desirable improvement of convulsive episode. It is difficult to interpret the observation here reported. The relation between infantile spasm and exanthema subitum is briefly discussed.
An autopsy case of 11-year-old girl with Hallervorden-Spatz disease was reported Her family history was noncontributory. Following a normal gestation and birth, her development had been normal until the age of 4. when unsteady gait, athetoid movements of upper extremities and night blindness were noticed. These symptoms slowly progressed. Physical examination, at 9 years of age, revealed retinal degeneration, mild dysarthria, hypertonus of neck muscle, athetoid movements and incoordination of extremities. Mental deterioration was not observed. Laboratory investigations showed nothing significant. Dystonia and opisthotonus developed at 10 years of age. She suffered from frequent attacks characterized by opisthotonus and extension of extremities, which were easily evoked by a small stimulus. Although right stereotaxic thalamotomy was done at 11 years of age, she died of status epilepticus 4 days after the operation. The brain weighted 1, 320g. and there were bilateral rust-brownish discoloration of the globus pallidus and the substantia nigra. On microscopic examination, a number of brown pigment granules were found intracellularly and freely in the parenchyma of the pallidum, and a few pigment granules were found only in some nerve cells of the substantia nigra. The pigment gave a positive iron reaction. Spheroids were distributed numerously in the pallidum, and were rare in the substantia nigra, the putamen, the thalamus and the medulla oblongata. Diffuse gliosis was found in the pallidum where the neuronal loss was marked. There was degeneration or loss of Purkinje cells accompanied by torpedoes in the cerebellum. There was degeneration of nerve cells also in the olive and dentate nuclei.
Galacosemia is one of the inherited metabolic disorder which may lead to death early in in fancy or survival with varing degrees of mental retardation. Kalckar have found the defect in galactosemia as a deficiency of the enzyme, galactose-1-phosphate uridyl transferase. However, the full explanation of mental retardation in this disorder have not yet appeared because of difficulties and lacks of studies on the problem. The author analyzed brain lipids of galaco semic rats, which were made galactosemic with feeding the diet Containg 35% galactose, for elucidating the pathogenosis of mental retardation in this condition. There are low levels of. free myo-inositol and phosphatidyl inositol in the brains 1 to 3 weeks old rats fed with galactose containing diet. Further, analyses of diand triphosphoinositides from frozen rat brains with liquid nitrogen showed some changes in developing brains of both groups of animals. The increasing amount of sphingomyelin and cerebroside during the course of growth in galactosemic rat brains were considerably lower than that of the sucrose fed rat brains, and these results indicated 1 week retardation of myelination in the former. It has been noted that the inositol phosphatide may act an important role on ion trnsport or menbrane permeability. Especially, interesting work that triphosphoinositide-protein-ion complex release, the ions by adding acetylcholin in vitro (Hayashi, 1968), suggests some relation of this lipids to the functions of neural tissues. The low levels of these lipids in' brains of experimental galactosemic rats might support the idea that these relate to mental retardation.
Three cases of Tay-Sachs disease were studied. The onset in Case 1 was at about 6 months of age 10 months general hypotonia with some spasticity in the legs and typical cherry red spots on the maculae were noticed. Light and electron microscopy, together with chemical, studies proved the case to be ge-le ralized lipidosis. Abnormalities were suspeoted in Case 2 at about 8 months of age. At age 1 year 3 moths, slight touches of light brown colour were noticed on the maculae. They were not symmetrical. Two months later, the spots on the maculae became spherical and of dark brown colour-typical cherry red spots. Case 3 was a premature baby. Developmen tal delay was obvious. Frequent convulsions commenced at age 7 months. At age 1 year 10 months typical cherry red spots were observed on the maculae. Light microscopic study con firmed cerebral lipidosis.
Experimental studies using rats were con.- ducted to study the effects of alcohol administration on the occurrence of INAH-induced convulsions. As a result of this experimental studies, it was observed that alcohol, when it was administered simultaneously with INAH, significantly repressed the occurrence of convulsions. It might be considered that the large doses of acetoacetic acid producing as metabolite of alcohol minimizes the convulsive acting of IN AH, combining with it in a living body. Then, it might be considered that the metabolic speed of alcohol was interfered by coexisting INAH in vivo.