A case of Lafora's disease was reported. The clinical course could be divided into four stages as follows. The first stage or subclinical stage began at about the age of 7, and during this stage the electroencephalogram abnormality without clinical seizure was shown. Its duration was about 3 years. In the second atage appeared some change in personality. The duration was 1-2 years. The third stage began with the onset cf epileptic seizures. During this stage the clinical course were progressive though slow. The epileptic and myoclonic seizures, cerebellar signs and dementia developed with occasional remission. Its duration was about 6-7 years. The course of the fourth or end stage was rapid. The status epilepticus or status myoclonicus developed. This stage lasted for about four years. The patient died the age of 19. Autopsy was performed. The Lafora's bodies in the central nervous tissue, and the PAS-positive deposits in the cytoplasm of liver cells and in the heart muscle were observed.
Brain edema was produced in rats and cats by freeze treatment, implanted balloons or triethyltin (TET) injection and metabolic activities were studied. An increase in water and sodium and a decrease in potassium content of white matter, resulting in a sharp rise of Na: K ratio, were observed. In total protein, nucleic acid and lipid content no significant changes were observed between normal and edematous brains. However, level of ganglioside was elevated markedly in both gray and white matter of cold induced brains. On the other hand the level of proteolipid protein showed a marked increase in the gray matter and a decrease in the white matter of both cold induced and TET intoxicated brains. In the edematous brains the concentrations of the energy-rich phosphates, creatine phosphate and adenosine triphosphate, were decreased markedly whereas inorganic phosphate was increased. The increased level of lactate was observed in the cold induced brains which suggested an increase of glycolysis in this condition. However, in the cold induced and epidural compressed gray and white matter, the most remarkable fact was that the protein synthesis was increased markedly after production of edema. In the experiments, protein synthesis increased for twenty-four hours following injury, reached a maximum between seventy-two and one hundred and twenty hours, and then gradually decreased. These findings correlate with the macroscopic changes of swelling of the brain. The same phenomenon was observed in the white matter of TET intoxicated cat brains to which TET was injected at the daily dose of 1 mg/kg, that is, the protein synthesis of the white matter increased remarkably on the fifth day, when its prominent edematous changes had already been confirmed. The proteolipid protein synthesis was also increased markedly in the cold induced gray and white matter and in the TET intoxicated white matter of the brains. These findings suggest that turnover of the structural protein may be increased and the structural component of the membrane may be changed. Incorporation of [5-3H] uridine into RNA was also increased in this condition. However, there was a sharp decrease in respiratory response to cationic and electrical stimulation. These findings are discussed in this paper in relation to other aspects of metabolic activity and to the mechanism of brain edema.
This report is based on the observation of 206 children with increased intracranial pressure at the Department cf Pediatrics cf Nagasaki University from January 1965 to December 1970. 1) The subject were 9% cf the total admission number during that period. 2) Etiologically, the 206 patients consist of 50 cases cf sercus meningitis, 33 of bacterial menigitis, 27 of encephalitis, 26 of intracranial bleedings and head injuries, 24 cf brain tumor, 24 cf acute encephalopathy, 9 of acute infantile hemiplegia, 3 of cerebro-vascular disorder, 3 of hydrccephalus, 3 of benign intracranial hypertension and 1 of leukemic meningitis. 3) The onset was more frequent at a younger age. The infant group occupied 75. 2 % of all subjects. 4) The pathogenetic factors of acute brain edema concerned mostly hyperthermia, fluid and elektrolyte metabolism, acid-base equilibrium, adreno-cortical function, liver function and the function of autonomic nervous system. 5) Three cases of acute brain edema with liver dysfunction-Reye syndrome, acute encephalcpathy following relatively minor burns and hepatic encephalcpathy-were reported. The metabolic disturbances caused by liver dysfunction might be the main pathogenetic factor cf acute brain edema in childhood. 6) From this point cf view, we discussed several problems concerning the mechanism cf acute brain edema in childhood. 7) From the analysis cf the relationship between the under living diseases and mechanisms cf acute brain edema, we discussed its treatment presenting a case of Reye syndrome successfully treated. 8) In view of a remarkable advance in developmental biology and pediatric neurology, further studies on the treatment of acute brain edema in childhood are to be expected.
It is well known that cevebral herniation is one of the disastrous-results in case of intracranial space-taking disorders. The authors discussed pathological classifications as well as clinical manifestations of cerebral hernition, and demonstrated few patterns of shear strain, which take place in the brain when it is put aside by expanding lesion in the cranial cavity, with the aid cf a 2-dimensional gelation model and polarized light. It was shown, in regards to transtentorial herniation, that anteri or herniation was easily seen when the expanding mass was located in a parietal region, whereas, posterior herniation was observed when the mass was in a frontal region. A lateral view of carotid angiosram was thought not to be so reliable in diagnosing transtentorial herniation. Because directions of posterior communicating and posterior cerebral arteries along the edge of the incisura tentrii showed some individual variations i a themselves. However, the foraminal sign on vertebral angiogram (by Sano) was in good accordance with foraminal herniation verified during a craniotomy. The extensive decompressive craniotomy has been advocated not only in Japan but also in other countries. among many treatments to ameliorate intracranial hypertension. The operation may be most effectiveto relieve brainstem lesion by cerebral herniation, when it is undertaken as the prophylactic procedure prior to the on set of marked decerebration.
1) From the etiological point of view, hydrocephalus is not the definite disease, but the symptome complex, that is the reason why the causative treatment for hydrocephalus is usually difficult. The ventriculoatrial shunt, the ventriculoperitoneal shunt with Pudenz-Heyer tube or Holter-Spitz tube are the most common surgical treatment for hydrocephalus. 2) In order to make shunting procedures for hydrocephalus i) the lateral ventricles must be symmetric. (Patency of Foramen Monro must be identified.) ii) the slowly progressive hydrocephalus must be differentiated from the real arrested hydrocephalus. iii) the chemical and the bacteriological examination of cerebrospinal fluid must be very carefully. iv) The priority for surgical treatments must be considered, when hydrocephalus is combined with malformations (eg. myelomeningocele, craniosynostosis etc.) or other diseases (eg. subdural hematom etc.) 3) For the past 4 years, the author has treated 121 hydrocephalus by ventriculoatrial shunt and/or ventriculo-peritoneal shunt, the rate of surgical mortality, the initial success immediately after surgery and the late complications are essentially the same with these procedures. 4) Comparative studies of hydrocephalus with or without operation are discussed.
Comparative studies were performed on effects, complications and shunt impairments of ventriculo atrial shunt (V-A shunt) and ventriculo-peritoneal shunt (V-P shunt) operations, mainly on the basis of our experiences with 62 V-A shunt and 19 V-P shunt operations. From the standpoint of shunt effect and durability, V-A shunt was definitely superior. From the standpoint of safety, V-P shunt was superior. We consider that, in hydrocephalic cases where both V-A shunt and V-P shunt have good indications, V-A shunt will be the operation of choice. However, in cases where any occult infection of CSF is suspected for, V-P shunt will be the operation cf choice. Various attempts hitherto performed by us for preventing postoperative complications and shunt impairments following V-A shunt, were mentioned. Remote prognosis of our hydrocephalic cases treated with shunt operations, as revealed by enquete method, was also reported.
A follow up study was made on 24 infants with acute purulent meningitis, admitted to the Hokkaido University Hospital from 1960 to 1970. Fifteen infants were under 2 months of age and remaining 9 were in ages between 3 and 15 months. Diplococcus pneumoniae, Staphylococcus aureus and Pseudomonas aeruginosa were the most frequent pathogens. Seven cases were caused by Gram-negative bacilli, i. e. 5 Pseudomonas aeruginosa and 2 Citrobacter, all of which occurred in infants under 2 months of age. In younger infants the initial symptoms were so obscure that in one third of patients the diagnosis was first made more than 4 weeks after the suspected onset of illness. During the course of meningitis hydrocephalus occurred in 8 patients, ventriculitis in 7, cerebral abscess in 3 and subdural effusion in 2. Thus, intracranial complications were observed in 47. 8% of patients. Of all 24 cases 4 died in the course of acute illness, and other 4 died during the following 2 years due to intracranial complications. Nine patients had permanent neurological sequelae and 7 (29.0%) recovered completely. Of 15 patients under 2 months of age 7 died, and among 8 survivors only one patient recovered without any recognizable deficit.