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The development of actions is related to the development of cognition and motor function. We propose that in minimal brain dysfunction syndrome the disturbances of actions are regarded as the developmental apraxiaagnosia syndrome. This study was attempted in comparison with Ajuriaguerra and Hecaen's classification of apraxia Piaget's stages of cognitive development.
Ajuriaguerra, Hécaen et al. identify three items in apraxia, namely, 1) sensory motor apraxia, 2) somatospatial apraxia, and 3) apraxia of symbolic formation. These classifications were prepared on the basis of a schematic diagram of the developmental stages, which based on Piaget's theory of cognitive development.
The subjects were children who were diagnosed as having minimal brain dysfunction syndrome with hyperkinetic behavior disorder, learning disabilities and soft neurological signs, and whose total IQ exceeded 80 in the WISC intelligence test. They consisted of 30 children, 26 boys and 4 girls, ranging in age from 7 to 12 years.
They were observed and studied for 15 parameters including drawing right-left cognition, standing on one foot, skipping, loop skipping, use of scissors, dressing and undressing, and tying of string etc. The failure rate for the 15 parameters including constructional actions, motor action and dressing actions averaged 48.5 percent. Scuh disturbances were similar to actions of the preoperational stage in children. Chronologicaly, they reached already in the operational stage but their actions remained in the preoperational stage.
It may be concluded that in minimal brain dysfunction syndrome, such disturbances were caused by the distubances of the ordering, visual space, somatic figure and the somatic coordinate system, i. e., developmental apraxia-agnosia syndrome from the viewpoint of developmental stages. Then, it is noteworthy that in most cases, the disturbances of actions are equivalent for somato-spatial apraxia according to Ajuriaguerra, Hecaen et al. classification of apraxia.

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Fifteen (12.7%) among 118 cases of the West syndrome and three (4.6%) among 65 cases of the Lennox syndrome were effectively treated by high-dose pyridoxal phosphate (PAL-P). Besides these cases, PAL-P was partially effective in eight cases of the West syndrome and five cases of the Lennox syndrome.
Clinical and electroencephalographic investigations including a long-term follow-up study were carried out on them.
1) In 12 effective cases of the West syndrome, clinical seizures were suppressed with PAL-P alone and in three effective cases by an addition of PAL-P to the previous poorly-effective regimen. Twelve cases have continued to be free from seizures, while two cases relapsed to the Lennox syndrome, and one died.
In one effective case of the Lennox syndrome, clinical seizures were suppressed with PAL-P alone and in two effective cases by an addition of PAL-P. Two cases have continued to be free from seizures, while one case relapsed.
2) Hypsarhythmia disappeared by PAL-P in fifteen effective cases of the West syndrome and diffuse slow spike-and-wave discharges disappeared in three effective cases of the Lennox syndrome.
3) The effective daily oral dose of PAL-P was 30 to 400 mg.
4) It is noticeable that PAL-P was effective even in the cases with obvious organic pathology besides idiopathic cases.
5) None revealed the definite vitamin B₆ deficiency state by the tryptophan load test.
6) The prognosis of vitamin B₆ responsive cases was favourable ; as many as seven cases (31.8%) of the West syndrome showed normal development at the follow-up.
7) The response to PAL-P was not predicted by the laboratory data or by clinical features.
The conclusion was that the treatment with a high-dose of PAL-P should be tried in all cases of the West syndrome at first.

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To elucidate the role of the purine nucleotide cycle in muscle, effects of denervation on AMP deaminase activity of rat skeletal muscles were investigated and the following results were obtained,
(1) Activity of AMP deaminase in gastrocnemius and extensor digitorum longus muscles (white musle) were higher than in soleus muscle (red muscle). The difference of AMP deaminase activity between white and red muscles were attributed to that of precipitate fractions.
(2) AMP deaminase activity per mg protein of extensor digitorum longus muscle decreased significantly 2 to 4 weeks after denervation. The enzyme activity in the whole homogenate and the precipitate fraction per whole muscle decreased after denervation, but there was no change in the activity of the supernatant fraction.
(3) AMP deaminase activity in the precipitate fraction per mg protein of soleus muscle decreased 1 week after denervation. The enzyme activity of the whole muscle showed the same changes in extensor digitorum longus muscle.
These results suggested that changes of AMP deaminase activity of precipitate might play a role in developing muscle weakness as the result of denervation.

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Twenty patients with intractable epilepsy including E. I. E. E. (early infantile epileptic encephalopathy with suppression burst), infantile spasms, minor motor seizure, and Lennox- Gastaut syndrome were investigated by monitoring serum cortisol (SC) level during ACTH-Z or hydrocortisone treatment. Fourteen days of consecutive ACTH-Z administration revealed a constant increase of SC levels in patients under 2 years of age. However, SC reached a maximum level around 7 days after initiation of ACTH-Z therapy in older patients followed by no further increase of the SC level despite the continued ACTH-Z administration. The maximum SC level detected during ACTH-Z therapy appeared to be significantly higher (P< 0.001) in the younger age group than the older, 2, 203.1±405.9ng/ml, 750.0±69.9ng/ml, respectively. Older patients showed a poor clinical response to ACTH-Z therapy except for the cases with atypical absence or with a seizure occurring as series form. Obviously, the ability of the younger age group to respond to exogeneously administered ACTH-Z is greater than the older group. This observation suggests a possible use of lower doses of ACTH-Z for controlling intractable epilepsy, mainly consisting of infantile spasms, in order to reduce serious side effects yet maintaining favorable clinical effectiveness.

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Ten patients with infantile spasms were investigated by monitoring serial serum cortisol levels during ACTH-Z treatment. Lower doses of ACTH-Z were used for the purpose of reducing a serious side effects yet maintaining favorable clinical effectiveness. Patients were divided into three groups (group I: 0.025 mg/kg, group II: 0.0125 mg/kg, group III: 0.0125→0.025 mg/kg) according to the doses of ACTH-Z given. Fourteen days of consecutive ACTH-Z administration revealed a significant increase of serum cortisol level in all groups investigated. However, among the three groups, group I demonstrated a significantly higher level of serum cortisol. All the cases in group I showed an excellent clinical response to ACTH-Z therapy. On the other hand 60% of cases in both groups II and III whose treatment was initiated by 0.0125 mg/kg of ACTH-Z showed a poor clinical response. This observation suggests that 0.025 mg/kg of ACTH-Z is effective enough to control infantile spasms favorably with less side effects.

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