Thirty bive cases of inpatients of progressive muscular dystrophy (PMD) were divided into five groups and given with ATP, Co-carboxylase, Vitamin B₁₂, Aspara, Vitamin E, Adenosine, Hoscorin and Ilotycine for 6 to 9 months. The study on the chage of the activity of Creatin-Phosphokinase (CPK), Lactic Dehydrogenase (LDH) and the degree of disability were carried out.
The results were as follows:
1) The activity of CPK in serum decreased in groups of the administration of ATP for 1 month, and Co-carboxylase and Ilotycin for 4 to 6 months.
2) The degree of the disability in PMD was progressive during administration of drugs in 17% (6 among 35 cases), On the other hand, there were progression in 43% (15 among 35 cases) at 6 to 9 months after D/C of all drugs.
3) There were significant correlation between the activities of LDH and CPK in serum, and the activity of LDH and the degree of disability.

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The urinary creatinine and creatine were measured in 40 cases of progressive muscular dystrophy (Duchenne: 34 cases and Limb girdle: 6 cases).
The increased excretion of creatine and the decreased excretion of creatinine were observed in the patients with progressive muscular dystrophy.
The excretion of cratinine and creatine in LG group were almost similar.
Duchenne group showed remarkable excretion of creatine and striking daily variations. These tendency were also found with severe patients.

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Since Minot et al. reported the excretion of compounds containing pentowe and phosphor rus into the urines of patients with progressive muscular dystrophy (PMD) in 1949, several papers were published on this problem, but no definite conclusion has been obtained whether the pentosuria represents a characteristic of PMD.
I was able to identify that 6 out of 70 PMD patients excreted a substance of nucleotide nature in urine by employing a conventional column chromatographic technique with Dowex resin instead of applying the osazon test which had been used by the previous investigators. The substance could succesfully be isolated and partially purified. Analytical data showed that the compound contained ribose, phosphorus and a base component. The base component did not coincide with any known bases of nucleic acid. A second sugar component other than ribose was also detected in the sample. Further investigations are required for the identification of the extract formula of the compound (s).
The amount of the compound excreted in the urine fluctuated significantly from day to day.

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We studied on serum CPK activities in Duchenne type progressive muscular dystrophy and carrier.
1) Serum CPK activities of the earlier stage group decrease faster than the later stage group during about 2 years period we observed.
2) Serum CPK activities of Duchenne type muscular dystrophies increase after exercise but it does not always rise in proportion to amount of exercise in all cases.
3) Serum CPK activities in probable carriers and their daughters are significantly higher than possible carriers and their daughters.

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It was suggested for a long time that the abnormality of autonomic nervous system was one of the pathogenesis of progressive muscular dystrophy(PMD)by Kure and Okinaka.
We found clinical, morphological and histochemical changes in the skeletal muscle of the infant dogs suffered from muscular dystrophy-like state, caused by cervical sympathetic gangiionectomy, which were about the same with those of PMD.
And it was attempted to observe the pattern of autonomic nerve function by several agents on about 60 cases of PMD (Duchenne type, 7∼18 years old). In pilocarpine test, 91.5% of the cases were revealed positive reaction (+1ff), and in mecholyl test, many cases (44.4%) belonged to S type (sympathetic hyperreactor) and none of P type (sympathetic hyporeactor).

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Nine typical Duchenne type PMD patients and much the same aged normal 9 children had carried out exercises of flexing their right elbows, pulling up the 1/2 weights of their 1 repetition maximum power—100 times every day for 4 weeks. Their ergograms were checked on every week-end and they verified that the increased proportions of the 1 R. M, of the pulling up frequency, of the total height and of the fatigue quatient in the cases of PMD patients were more than those of normal children.
After 4 weeks rest of the exercise their ergograms showed that the decreased proportions of the 1 R. M and the pulling up frequency in the PMD patients were almost in same degree as of normal children, while the fallen off rates in the total of pulling up heights and in the fatigue quatient of the formers were more remarkable than those of the latters.
Consequently it is concluded that the muscular endurance of a PMD patient is conspicuously increased by the exercise and is markedly decreased by a rest of the exercise.
Searching for the upper limit of the increase in the muscular endurance by exercises and the most adequate exercising time and hardness still remain as the problems of future.

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It is now well known that disturbance of gait in progressive muscular dystrophy (PMD) is mainly due to the weakness of musculature of the lower extremities & the trunk, and to the contracture caused by imbalance between the flexors and the extensors. In twenty-eight cases of hospitalized Duchenne type the relationship between the contracture of their lower extremities and the ability of walking was investigated. It was elucidated from the study of each joints of lower extremities that contracture of the hip and knee joints played the main role in the disability of walking. Therefore, the method of manipulation of those joints is important in the management of PMD. After six months of treatment, about half of the cases showed some improvement thcugh all the cases showed gradual loss of ability in the activities of daily living. Then to make secure the evidence of the effect of manipulation, we selected four cases as a control group to stop manipulation, whose condition were not so severe. After five months all the cases of the control group showed deterioration in both contracture and ability of walking. About one-third out of the remaining sixteen cases under the treatment showed deterioration in regard to contracture and about two-thirds showed deterioration in ability of Walking.
From those facts it may be considered that manipulation will prevent or delay the occurence of contracture and the disability of walking.

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Twenty-six patients of progressive muscular dystrophy were examined on muscle testing for the purpose of studying the disturbance of gait. The relationship between the muscle power, grading of this disease and joint contractures were investigated.
On the hip joint, the weakening of the extensor power was inversely proportional to the grading of this disease and the degree of contracture of joint. The walkable patients had stronger muscle power than the not walkable patients and no joint contracture.
On the knee joint, the weakening of the extensor power was also inversely proportional to the grading of this disease and the degree of contracture of joint. But no considerable differences of the extensor power was presented between these patients. While no contracture was observed on the walkable patients, the patients with contracture of this joint could not walk even when they had stronger muscle power than some walkable patients. By this reason, the prevention of contracture of the knee joint is a very important problem of this disease.

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I. The Yatabe-Guilford Personality Inventory was carried out on three groups: 48 patients with progressive muscular dystrophy, 77 patients with other chronic diseases and 343 normal children. They were all elementary and junior high school pupils; the former two groups were in bed school. The PMD group was different significantly from the other two groups in the distribution of the five profile types. The E type had the largest frequency in the former and this tendency was more remarkable in the junior high school children than in the elementary school children. While in the elementary school children the PMD groups showed more intensive introversion than the other two groups, in the junior high school children the PMD groups showed more intensive emotional instability in addition to introversion.
II. Inquiries were made by mail about family circumstances and life histories of 29 PMD children. We had found, in the previous paper, 17 out of 29 as the problem children from results of some clinical psychological tests, psychiatric interviews and behavior observations. From the family inquiries some features were found in the problem children group as compared with the another normal PMD children group; many families of the former live with grandparents, or have some family troubles or some change of them between the time before and after entering the sanatorium. Remarkable trends were also found in the former, that onset of the disease had been noticed earlier but it had taken longer time till the diagnosis of PMD was confirmed, and they had visited the many medical facilities. In their parents attitudes toward child rearing, somewhat bipolar tendencies such as dotage and rejection, strictness and indulgence were founnd.

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The electrocardiograms recorded on 40 patients with the Duchenne type tf ptogiessite muscular dystrophy were analyzed. A first EKG of serial EKGs from each patient was used for the statistical analysis. The ages of the patients at the first obser' aticn ranged from 5 to 20, with a mean age of 11.4. The EKGs were analyzed with reference to the normal standards for the EKG of the Japanese children (6∼11 years) that were proposed by Kuriyama. The other normal standards for children including the ones proposed by Shirai, Ziegler, and Alimurung were referred to, when necessary. Sequential changes which occurred during 0.5 to 2.5 years follow-up periods were analyzed in 37 cases in which serial EKGs were available. EKGs were being taken with a frequency of at least every 6 months.
With the analysis of the 40 EKGs representing each patient, 87.5 per cent had some EKG abnormalities, though the findings such as sinus tachycardia, sinus arrhythmia, and slight deviation from the normal range of P-R or QRS interval were not counted as abnormal. Sinus tachycardia (>100/min.) was found in 9 (22.5%), and significant sinus arrhythmia was found in 2 (5%). In one patient atrial and ventricular premature beats were observed concomitantly.
A P-R interval equal to or shorter than 0.11 sec. was observed in 6 (15.0%), including 5 with 0.11 sec. and 1 with 0.10 sec. A slightly prolonged QRS interval was observed in 6 (15.0%), including 5 with 0.10 sec. and 1 with 0.11 sec.
The commonest abnormality was an increased R/S ratio in the right precordial leads; R/SV₁ exceeding 1 was observed in 19 (47.5%), tlit exceeding 1.5 in 12 (30.0%), and that exceeding 2 in 8 (20.01).
The next common abnormality was the presence of R' in the right precordial leads. When an R' in V₁ was present regardless of its amplitude, it was classified as incomplete right bundle branch block (RBBB), provided that the QRS interval was less than 0.12 sec.; incomplete RBBB defined as such was present in 14 (35.0%), among which an R'V₁ greater than 5.0 mm was observed in 3 (7.5 9, ).
A high R in V₁ exceeding its upper normal limit 12.0 mm (according to Kuriyama) was present in 4 (10.00), and an abnormally small S in Yi less than its lower normal limit 4.0mm (Kuriyama), regardless of presence or absence of R'V₁, was present in 15 (37.5%). Also, the mean of RV₁ of the 40 cases was close to the mean of normal, while the mean of SV₁ was significanlty smaller than the mean of normal. Therefore, a short Svi rather than a high RV₁, appears to be a major deviation from normality, contributing to an increased R/S ratio in V₁.
Abnormal Q waves in I, aV_L, V₄, V₅, and/or V₆ were observed in cases with relatively advanced muscle function impairment; Q/R ratio greater than 1/3 in I, V₄, V₅, and, or V₆, or a QS deflection in these leads, a pattern simulating a lateral wall myocardial infarction, were observed in 3 (7.5%). When Qv5 greater than 4.0 mm (upper normal limit by Kuriyama) was used as a criterion, regardless of Q /R ratio, it was found in 5 (12.5 %), including 3 cases with mild impairment of muscle function. In contrast, high voltage in the left precordial leads was rare, and there was only one case with a Rv5 exceeding 40.0mm. The mean amplitude of RV₅ of the present series was 16.95 mm, and this value was significantly lower than the mean of normals (24.70 mm for 11 years old boys).
There were 3 cases in which T wave changes were found. The changes consisted of flat T in V₅ and V₆ or small inverted T in V₄ through V₆, which were found also in relatively advanced cases.

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I observed forty-two doub:e handicapped juvenile patients and got the following results.
1. C. P. is the largest in number, the second one is the sequela of encephalitic process, and they are extremely backward in intellectuality as well as advancing of speech ability.
2. The disturbances at perinatal stage (immatureness, asphyxia, icterus) influence greatly on these pathogenesis.
3. Unhealthiness of the mothers at pregnancy is also one of the cause.
4. The occuring time of the pathogenesis is presumed at perinatal stage in a half of the number.
5. C. P. often coexists with epilepsy as wall as feeble mindedness.
6. The dislocation of hip joint, the abnormality of testicle and microcephalus are often seen.
7. Paralysis and other motor disturbances are remarkable.
8. The difficulty of eating or disturbance of sphincter control is also serious.
9. Behavior problem are pronounced mostly in mental deficiencies.
10. The family environments of the patients are bad.

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1. The neuropathological findings in a boy suffering from progressive muscular dystrophy and mental retardation with EEG abnormalities (5.6 c/s slow wave bursts in all leads and spikes in the right parietal and occipital areas) were presented. Two brothers of the patient also had progressive muscular dystrophy, mental retardation and EEG abnormalities. Moreover, their mother was mentally retarded.
2. Macroscopically, the cerebrum was rounded at the frontal lobe, but other areas of the brain were normal.
There was no particular histological finding corresponding to the clinical findings of mental retardation and EEG abnormalities.
3. There was a marked dysplasia of the spinal cord (the lower cervical cord, the thoracic and lumbar cord).
4. The meaning of the malformation of the GNS, mental retardation and EEG abnormalities in progressive muscular dystrophy was briefly discussed in comparing with these cases reported in the literature.

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