Although bacterial meningitis has been mentioned to be one of the causative diseases of infantile spasms, there are very few reports of infantile spasms following bacterial meningitis. Out of 33 patients suffering from bacterial meningitis from 1977 to 1980, three showed hypsarhythmia on electroencephalogram (EEG) as a sequela.
Case 1:
A 4-month-old female infant suffered from H. influenzae meningitis. She was attacked by head nodding without series formation 4 months and 15 days later. Serial computed tomography (CT) findings illustrated the progressive cortical and ventricular atrophy following the absorption of subdural effusion. Asymmetric slow activity on EEG at the early stage of the illness was changed into hypsarhythmia via multifocal spikes.
Affter ACTH-Z therapy, clinical seizures ceased and hypsarhythmia disappeared. Her motor development has also become normal.
Case 2:
A 1-year-9-month-old boy had H. influenzae meningitis. Asymmetric slow activity on EEG was developed into hypsarhythmia 2 months later. Although there was no clinical attacks, involuntary movements appeared. CT findings indicated the progressive hydrocephalus without cortical damage and the hemorrhage into the basal ganglia of the cerebrum.
He could stand without support but the neurological sequelae such as deafness, mental deficiency and right hemiparesis remained.
Case 3:
A 4-month-old female infant suffered from D. pneumoniae meningitis. The quadriplegia associated with involuntary movement of the upper extremity followed the comatose state for the first two weeks.Ventriculoperitoneal shunt operation was done one month later but the ventricles on CT picture extremely dilated. Five months later, typical infantile spasms ensued.
ACTH-Z injection therapy converted infantile spasms into adversive seizures. But quadriplegia, deafness and blindness remained unchanged.
These results suggested that infantile spasms were mainly initiated by cortical lesions in some stage of the development and hypsarthythmia, which seemed to arise 2 to 5 months after the onset of meningitis with certain silent intervals, was likely to be induced from the brain stem lesion.

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Recently the pathological involvement of sensory neurons and thalamus in Werdnig-Hoffmann (W-H) disease has been reported. The brains of five autopsy cases with severe infantile form of W-H disease were studied to ascertain pathological changes of sensory neurons and thalamus. All the cases examined died before 4 months of age. Four of them showed unilateral or bilateral drop hands and two cases showed delay of motor conduction velocity of median the nerve.
The present study disclosed severe loss, chromatolytic degeneration and empty cell beds of the spinal anterior horn cells and cranial motoneurons (V, VII, X, XII), and glial bundles in the anterior roots. As to the sensory neurons, some ghost cells were noticed in Clark's column and a small amount of glial bundles in the posteriorroots in two cases. Posterior column appeared to be normal. Thalamus showed evidences of considerable neuronal degeneration such as chromatolytic changes and neuronophagia in all four cases examined. These lesions were dominant in the lateral formation of thalamus (ventralis posterior, dorsalis posterior), while the pulvinar and the ventralis anterior were less affected. Thus, it was demonstrated that not only degeneration of lower motor neurons and glial bundles in the anterior roots, but also degeneration of sensory neurons and thalamus were present in W-H disease. Degeneration of thalamus and Clark's column can not be explained by the traction mechanism which has been proposed by Chou et al. We are in agreement with the opinion of Iwata et al. (1978) that W-H disease is a disorder of multi-systemic degeneration of neurons.
It was suggested that the drop hand might be considered as a clinical sign suggestive of peripheral nerve involvement.

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A systematic neuropsychological study was performed to clarify the mechanism of the learning disorders in minimal brain dysfunction syndrome (MBD) and the relationship between MBD and the behavior disorder withepilepsy. Fifty-three children with MBD (group I), aged 4 to 12 years, and thirty hyperkinetic children with grand mal seizures (group II), aged 5 to 13 years, were examined. Intelligence quotients were above 90 in all patients. Except for the neurological examination, soft neurological signs and the EEG study, neuropsychological studies consisted of 1) lateral dominance of hand, foot and eye, 2) Illinois test of psycholinguistic abilities (ITPA) (Japanese edition), 3) Bender-Gestalt test, and 4) Frostig developmental test of visual perception (Japanese edition).
The results were as follows: 1) The incidence of mixed dominance was 49.0% in group I and 43.3% in group II and was significantly higher than in normal children (23.3%). 2) The psycholinguistic quotient (PLQ) with ITPA was lower than the intelligence quotient and this difference was especially definite in children with learning disorders. Therefore, the PLQ was a more accurate index for the learning ability in MBD. The evaluation with 10 subtests revealed that there was a patchy defect in automatic function but not in representative function. Significantly abnormal low scores were found in visual sequencing memory (34.0% in group I and 26.7% in group II), grammatic closure (24.5% in group I and 16.7% in group II), and auditory sequencing memory (15.1% in group I and 16.7% in group II). There was a high. correlation between learning disorders and patchy defects in automatic function. 3) In Bender- Gestalt test (Koppitz's method), 18 cases of 49 in gruop I and 10 cases of 30 in group II had high scores beyond the normal range. There was a high correlation between the scores of Bender- Gestalt test and ability of visual sequencing memory in ITPA in these 28 high score patients. Also they had mixed dominance, dyslexia and dysgraphia, and learning disorders frequently. 4) Total perceptual quotient in Frostig test was below 90 in 16 of 29 patients. There was a parallel correlation between PQ of Frostig test and the results of Bender- Gestalt test. 5) In EEG, both group showed frequently similar abnormalities, i. e., anterior theta bursts, 6 c/s wave and spike phantoms, and 6 & 14 c/s positive spikes which suggested diencephalic dysfunction. These EEG abnormalities showed a correlation with patchy defects in automatic function.
Children with MBD and epileptic children with behavior disorders had mixed dominance and patchy defects frequently in automatic function such as sequencing memory, closure and visual motor perception. It was concluded that these defects were the causes of learning disorders in MBD. It was emphasized that epileptic children with behavior disorders need medical and educational care as in MBD.

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Serial cranial computed tomography (CCT) was studied in 20 children with acute infantile hemiplegia. These children were devided into two groups: hemiplegia following fever, hemiconvulsion and unconsiousness (the convulsive group) and hemiplegia without convulsion (the non-convulsive group). There were 15 cases in the convulsive group and 5 cases in the non-convulsive group. Two cases in the convulsive group developed episodes during purulent meningitis. As underlying diseases, a case of tuberous sclerosis, a case of osteogenesis imperfecta and a case of febrile convulsion were found in the convulsive group, and a case of cyanotic heart disease in the non-convulsive group. The others were normal until the illness.
Results
1) Acute phase
We could investigate the CCT immediately after the onset in 6 convulsive cases and 3 non-convulsive cases, but the immediate CCT revealed no abnormalities in both groups. Within several days after the onset the abnormally low density area appeared on the CCT in both groups. In three cases there were abnormally high density areas complicationg these abnormalities. We divided those low density and high density areas into five types I. hemispheric low density area type, II. lobar low density area type (dominantly in the temporal area), III. small or lacunar low density area type, N. hemorrhagic infarction type and V. normal.
I. II. III. IV. V.
Convulsive group 7 4 0 1 3
Non-convulsive group 0 0 3 2 0
2) Recovery phase
Over more than a month, the hemispheric low density area changed into the hemispheric atrophy and the lobar low density area changed into the focal wedge-shaped atrophy or diminished. The small or lacunar low density area changed into the low density spot and the hemorrhagic infarction into the porencephaly.
3) Angiographic abnormalities
The occlusion of the internal carotid artery were found in two non-convulsive cases and the stenosis of the internal carotid artery in a convulsive case with purulent meningitis.

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This paper reported the result of a long-term clinical and electroencephalographic study of a case of Aicardi syndrome involving a 6-year- old girl. She started having flexor spasms at 5 months of age; the case was diagnosed as infantile spasms, and she received anticonvulsants. At 9 months of age, flexor spasms recurred, and she was treated with ACTH. The seizures was cotrolled for almost 4 years thereafter, but at 4 years and 10 months of age tonic seizures and astatic seizures, chinical manifestations of Lennox syndrome, were noted and these seizures are still intractable.
All patients so far reported with Aicardi syndrome have had severe psychomotor retardation. On the other hand our case did not have psychomotor retardation until 2 years of age; she was able to walk alone at 1 year 1 month of age and speak a few words at 1 year 4 months of age.
Nineteen EEG records taken during the 6 years were studied. At the onset, the records showed hypsarhythmia, but not the, suppression-burst pattern. At 8 months of age, after about 3 months on the anticonvulsants, hypsarhythmia diminished, and sporadic multifocal spikes appeared. ACTH therapy improved the EEG findings for a time. However, multifocal spikes reappeared soon and gradually increased in frequency. At 6 years 7 months of age, the EEGs showed slow spike & wave complexes and rapid rhythms which sometimes revealed complete asynchrony between right and left hemispheres. We think that this findings is a characteristic electroencephalographic pattern of Aicardi syndrome with Lennox syndrome.

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A 2-year-6-month-old Japanese boy with Ullrich syndrome was described. Clinical findings included distal dominant generalized muscle weakness and atrophy, acroatonia, contracture of hip joints, scoliosis, arefrexia, high arched palate, prominent calcaneus, and soft and elastic skin. These were consistent with Ullrich syndrome. Electromyographic examination of the left tibialis revealed neurogenic pattern with high amplitude potential of 4 mV. The right quadriceps femoris muscle showed a myopathic changes consisting of variations in the fiber size, and degeneration and redegeration fibers. Type I fiber predominance and hypotrophy were also found. Therefore this patient was diagnosed as having congenitai fiber type disproportion myopathy as well. Biochemical analysis of collagen in the skin revealed that the soluble fraction by pepsin digestion was increased, although the total amount of collagen was reduced, suggesting the presence of immature collagen.

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Diencephalic syndrome of infancy was first described by Russell in 1951 as a rare syndrome wite characteristic clinical features, associated with tumors of hypothalamus and optic chiasm. The features of the syndrome include emaciation, euphoria, and hyperkinesis. Endocrinological abnormalities were also reported. This report described a case of the syndrome and the pathogenesis of the emaciation was discussed.
Case. T. I., a 15-month-old infant, was admitted because of extreme emaciation. He was normal at birth, weighing 3, 330 g, and his neonatal course was uneventful. At 3 months of age the parents noticed rotatory nystagmus and the patient had no weghit gain thereafter. His developmental milestones were normal. On admission, his weight was 5, 940 g (-4 S. D. below the mean). He was strikingly emaciated with almost complete abesence of subcutaneous fat. His face was pale but he, was alert, hyperkinetic, and even euphoric. Except for rotatory nystagmus and delayed light reaction, no other neurological abnormalities were found. Intestinal absorption tests of fat and xylose were all normal, and the basal metabolic rate was high. Endocrinological studies showed that the plasma concentration of GH was constantly elevated. TSH, T 3 and T 4 were all within normal range. The daily profile of GH was absent. CT scan revealed very large low density area from hypothalamic region to the base of the frontal lobes, which was markedly enhanced after contrast infusion. This low density area was considered to be a hypothalamic tumor. Radiotherapy (5, 000 rads) to the tumor site was given for 6 weeks. After radiotherapy the tumor size was markedly decreased. When his hyperkinesis was much improved, the patient recovered hi body weight, but plasma GH level was not reduced to normal. We speculated that the emaciation in this case was partly related to the excessive consumption of energy.

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This report described a case with Smith-Lemli-Opitz syndrome, in which the intracranial calcification was found by CT.
The patient showed the typical clinical manifestations for this syndrome; short stature, microcephaly, specific countenance, mental retardation, hypospadias, cryptorchism, polydactyly and syndactyly. He was suffered from acute lymphocytic leukemia at 14 years of age, and died in spite of anti-ALL chemotherapy. The skull-CT, taken 2 weeks after the initiation of therapy, revealed the symmetric calcification on the portions of caudate nucleus and globus pallidus.
This finding suggested that there might occur the intracranial calcification in Smith-Lemli-Opitz syndrome, and further suggested that CT of the skull is of great help to find it.

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[in Japanese]

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[in Japanese]

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