Authors have already reported that ACTH combined with sodium valproate (VPA) was more effective therapy than ACTH only for infantile spasms.
The present study was carried out to examine the effects of VPA and ACTH on GABA metabolism of rat's brain. The experiments were carried out on female rats of Wistar strain, weighing 150-170g. The rats were divided into seven groups: group A, controls: group B, fast 5 days: group C₁, intraperitoneal injection of VPA (400 mg/kg); group C₂, oral administration of VPA (150 mg/kg a day) for 20 days: group C₃, oral administration of VPA (100 mg/kg a day) for 20 days: group D, intraperitoneal injection of ACTH-Z (0.1 mg/kg a day) for 14 days: group E, intraperitoneal injection of ACTH-Z (0.1 mg/kg a day) for 14 days combined with oral administration of VPA (150 mg/kg a day) for 20 days. Results were obtained as follows.
The mean values+SD (μmole/g) of GABA contents in brains were 2.46±0.23 in group A, 2.29+0.23 in group B, 3.13±0.27 in group C₁, 2.82±0.15 in group C2 2.80±0.13 in group C₃, 2.20±0.08 in group D and 3.71±0.18 in group E respectively. There were significant increases of GABA contents in group C₁ (P<0.025), group C2 (p<0.05), group C3 (P<0.05) and group E (P<0.005).
These results suggest that ACTH has no effect on GABA metabolism but increases the action of VPA. We speculate that an increase of GABA content in the brain contributed to the effectiveness of ACTH and VPA therapy.

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Clinical and neuropathological studies were done on the brain stem of 88 prematurely born infants. There were 12 cases with neuronal loss and astrogliosis in the inferior olivary nuclei, 2 cases with neuronal lossin the tegmentum of the brain stem, and 11 cases with recent or old necrosis in the pontine nuclei.
The neuronal loss and astrogliosis in the inferior olivary nuclei was closely related with the cerebellar hemisphere lesions such as cerebellar hemorrhage or loss of Purkinje cells and astrogliosis in the cerebellar white matter. This olivary lesion may be a secondary change to the damage of the cerebellar hemisphere.
The clinical history and the distribution of the lesions suggested that the neuronal loss in the tegmentum of the brain stem was due to hypotensive brainstem necrosis, and that the necrosis in the pontine nuclei was a hypoxic change such as pontosubicular necrosis in most cases or a secondary change to cerebellar hemispheric lesions in some cases.

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Although the main clinical symptoms ataxic type of cerebral palsy include remarkable truncal unsteadiness, incoordination of the limbs and intention tremor, an appropriate diagnosis is often difficult because of poor definition and widespread etiologies in this disorder. However, it seems to be necessary to confirm whether the infants have nonprogressive ataxia as early as possible, because early diagnosis and treatment can help to prevent advancing ataxic handicaps from these infants.
In the present study, we tried to classify this type of cerebral palsy (non-progressive ataxic syndromes) into two categories ; congenital cerebellar ataxic syndrome (CCA) and dysequilibrium syndrome (DES) in conformity with a classification by Hagberg et al. The former is characterized by the presence of dyssynergia, dysmetria and incoordination of the limbs, the latter disturbance of posture and equilibrium.
On examination of twelve cases with this disorder, five belonged to CCA, four to DES, and three infants remained to be classified to either type because of their younger ages. The patients of both groups were slow in developmental milestones and were floppy in infantile and early childhood stages mimicking those with mental retardation or so-called floppy infant syndrome. In DES the motor development was extremely delayed; none of four patients in the present study learned to walk by five years of age and they had remarkable impediment in speech development as well as visual recognition. On the other hand, three of five patients with CCA were able to walk by the age of five years and were less tardy in developmental motor skills in comparison with those of DES.
Since early diagnosis and early physical therapy might reduce or prevent future child's handicaps of cerebellar ataxic, one should consider a possbility of this non-progressive ataxic syndromes when examining the patients with floppy infant syndrome.

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Subarachnoid hemorrhage of the newborn is known to be the most common variaty of neonatal intracranial hemorrhage, but ist difinite diagnosis is very difficult by the usual diagnostic technique.
Although computed tomographic brain scan makes is possible to know the accurate site of the intracranial hemorrhage of the newborn, especially the intraventricular hemorrhage, the CT finding of the subarachnoid hemorrhage has not been fully evaluated.
Computed tomographic brain scan was performed on twenty-five high risk newborns with suspected intracranial hemorrhage and the high density area of the falx (falx image) was seen with or without intraventricular and intracerebral hemorrhage.
1) The falx image disappeared on the follow-up CT.
2) Intracranial hemorrhage was confirmed by CSF spectrophotometry (eighteen cases).
3) Subarachnoid hemorrhage was revealed in eight autopsy cases.
From these data, it was concluded that the falx image on the computed tomographic brain scanning was of diagnostic value on subarachnoid hemorrhage of the newborn.
Although the calcification of the falx is rarely seen in the newborn period, the falx image does not always mean the subarachnoid hemorrhage. In the newborn period, hematocrit is high and brain shows diffusely of low density on the CT scanning. The combination of high hematocrit and low density of the brain may make the higher incidence of the non-hemorrhagic falx image in the newborn period.
The points of differential diagnosis of the hemorrhagic and non-hemorrhagic falx image were:
1) The hemorrhagic falx image was wide and irregular on edges. On the other hand, the non-hemorrhagic falx image was recognized as straight and regular line on the CT scanning.
2) The mean EMI unit of twenty picksels of the hemorrhagic falx image was more than 40, but that of the non-hemorrhagic falx image was less than 40.

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Intraventricular hemorrhage (IVH) in the neonatal period is readily demonstrated by CT of the cranium. In our clinic an infant of very low birth weight with IVH was treated conservatively with success. Information about the site and extent of the IVH, and size of the ventricular system, was provided once a week for 28 days by the use of CT.
The male infant, weighing 1, 300 g., was born on 5 Oct. 1978, with spontaneous delivery following a 29 week gestation, Apgar score was 7 at 1 minute after birth. Oxygen inhalation was started after birth, because grunting and acrocyanosis were noted. on the 4 th postnatal day, CT was performed and IVH was diagonsed, as the hematocrit fell to 36% suggesting IVH. CT revealed hematomas in the trigonum of bilateral lateral ventricles with slight ventricular dilation. CT on the 3 rd week showed that the hematomas had disappeared, but moderate ventricular dilation was still observed. On the 4 th week, the size of the ventricles became normal. Thereafter, no ventricular dilation was observed by CT. From the 4 th to 19 th postnatal days, lumbar punctures were performed four times in order to reduce intraventricular pressure. CSF pressures were always 110-120 mmH₂O.

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A case of thalamic hemorrhage in a 4-year-old girl was reported. The patient began to vomit suddenly without any provoking cause. After several hours, her grasp of the right hand became weak and right hemiplegic gait appeared. Her consciousness was clear. She was admitted to our hospital the next day. On neurological examination, right hemiparesis and Kernig sign were present. Cerebrospinal fluid was xanthochromic. Electroencephalogram showed lazy activity on the left parieto-occipital area. Computed tomography (CT) revealed left thalamic hemorrhage surrounded by edema extending to the left internal capsule. Cerebral angiography showed dilatation of the left thalamusperforating artery and intense visualization of choroid plexus. But the nature of the lesion was not clear. She was treated conservatively by phenobarbital and prednisolone. On the CT picture obtained 18 days after the onset of the hemorrhage, the hemorrhagic lesion of the left thalamus disappeared, and cerebral angiography 30 days after the onset showed no abnormality. Clinical symptoms of right hemiparesis almost recovered within month. We concluded that clinical symptoms of our case was that of acute infantile hemiplegia, proven to be caused by thalamic hemorrhage by CT, and that it was regarded as idiopathic intracranial hematoma because the cause of the hemorrhage was unknown in spite of various investigations.

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The influence of 3R-(-)-4-amino-3-hydroxy-butanoic acid (l-GABOB) on spinal reflex was investigated by observing the sequential changes of Hoffmann reflex (H-reflex) after therapeutic intrathecal injection of l-GABOB in a patient (2-year-old boy) with spastic quadriplegia as sequela of measles encephalopathy. The injection was repeated twice a week with a starting dose of 20mg, increasing gradually up to 160 mg. When the dose was raised up to 80mg or over, muscle tone of the patient, decreased profoundly, and both deep tendon reflexes and Babinski reflex were abolished.
Recruitment curve of the H-reflex became flat after l-GABOB administration, the ratio of the largest amplitude of H wave to that of M wave decreased, and the ratio of the threshold of H wave to that of M wave increased. Both early and late facilitation in the recovery curve of H reflex diminished markedly after injection. However, clinical as well as electrophysiological changes described above were just transient, lasting for 10 hours at maximum.
The authors concluded that clinical and electrophysiological changes observed in our patinet after 1-GABOB i. t. were based upon a generalized suppression of the excitability of spinal motor neurons through the inhibition of not only monosynaptic, but oligo- and poly-synaptic reflexes.

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The results of clinical, electrophysiological and histopathological studies in a 6-year-old female with ataxic gait were presented. She had a history of inability to stand with, poor balance since early childhood. Those signs were slowly progressive. Clinical examination revealed ataxic gait, loss of deep tendon reflexes, pes cavus, markedly positive Romberg test and decreased deep sensations, while superficial senses were intact. Electrophysiological examinations showed a marked decrease in MCV and SCV of the ulnar nerve. Sural nerve biopsy showed a moderate loss of total myelinated fibers and a selective loss of large myelinated fibers on microscopic examination. Electronmicroscopic studies showed onion-bulb formation and proliferated collagen fibers. There was no neurological disease in family history and MCV and SCV of her parents and sister were within normal range.
The presented case could be considered as a type of peripheral neuropathy with ataxic gait and was suggested as Roussy-Levy syndrome except negative family history. However further follow up studies are necessary for differentiation of this case from Friedreich's ataxia and Charcot-Marie-Tooth disease.

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Congenital indifference to pain with anhidrosis is a rare syndrome consisting of the following ;(1) generalized analgesia from birth;(2) recurrent unexplained high fever;(3) neurogenic anhidrosis; and (4) mental retardation.
This syndrome is now classified as “familial sensory neuropathy type-IV”. Only ten or more cases have been reported since Nishida described the first case in 1951. Loss of small neurons of dorsal root ganglia, lack of small fibers in the dorsal roots, and absence of Lissauer's tract were described at autopsy case by Swanson at 1965, but its pathogenesis is poorly understood.
The present report described 2 mentally retarded sibling girls, 4-year-8-month and 2-year-10-month old, with recurrent episodes of unexplained high fever, repeated traumatic injuries and burns, and self-mutilating behavior. They were slender and hypotonic. The elder sister had only one tooth, because of spontaneous extraction. She had Charcot's joints at ankles. Her anhidrosis was not improved by acetylcholine injection. Motor and sensory conduction velocities were at the lowest level of the normal range. The skin biopsy was performed without anesthesia, and showed histologically a diminution of secretory portion of sweat glands, which was considered to be a secondary change of the sensory neuropathy.
Clinical symptoms of both patients, including recurrence of high fever, self-mutilation and delayed mental development, were improved with age.

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