Biopsy was taken from the tibialis anterior muscle of patients of progressive muscular dystrophy (PMD), spinal progressive muscular atrophy, neural progressive muscular atrophy, spinal cord lesion and spastic paraplegia after compression of spinal cord in 18 cases in total.
Phosphorylase, succinic dehydrogenase, lactic dehydrogenase, cytochrome oxidase, adenosine triphosphatase were stained as well as H-E, PAS and cholinesterase for the motor end plate. Morphologically, the irregularity in diameter of muscle fibers was characteristic in the cases of PMD; majority of fibers were atrophied but some fibers were apparently enlarged. In two cases of neural progressive muscular atrophy, a group of small fibers was separated from another group of hypertrophic fibers. The size of end plate was found to be much reduced as far as 5 cases surveyed in PMD.
In general, the activity of phosphorylase in small fibers was found to be relatively decreased, while ATPase in small fibers was apt to be much increased. It was difficult to find out the relation between the size of fibers and enzymatic activity of succinic dehydrogenase as well as lactic dehydrogenase and/or cytochrome oxidase. In one case of neural progressive muscular atrophy, coarse network of sarcoplasma was found by staining succinic dehydrogenase which is accumulated at mitochondria. In spastic muscle, all of enzyme examined were found to be decreased.

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Exspirograms were measured by McKesson's Vitalor in 45 cases of progressive muscular dystrophy (Duchenne 27 cases, Limbgirdle 15 cases and Facioscapulohumeral 2 cases) and 59 normal pupils.
1) Duchenne (D) group showed a remarkable constrictive difficulty with a low mean value of %VC (70.4%).
2) D-group showed a high mean value of %TVC (94.9%) which was higher than that of normal pupils. This high average was proved to have no relation to the condition of disease or the value of VC.
3) %VC in D-group showed to have a negative correlation with the grade of functional disturbances in the patient's activity. The transformation of chest cage was connected with the low average of %VC.
4) The ventilation of Limbgirdle group showed to be slightly disturbed.
The low average of %VC with the high average of %TVC in the patients of Duchenne type is the most interesting point in this study which will be further investigated in the following series.

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Intelligence scale (WISC) and electroencephalographic record (EEG) were investigated on the 88 patients of progressive muscular dystrophy in Shimoshizu Hospital. Age ranged from 6 to 16 and disability stage was I to III, mainly II. Types of disease were as follows: Duchenne 80, limb-girdle 4, facioscapulohumeral 1, congenital 2, myotonic 1.
Distribution of intelligence quotient (IQ) was similar to normal control except the lowest one (IQ under 79), which was observed in 38% of all cases.
EEG were recorded two to three times about one patient and sleep induction was performed by ingestion of γ-hydroxy butyric acid syrup. The results of EEG examination of muscular dystrophy were as follows: normal 11.2%, borderline 28.8%, abnormal 60.0% in Duchenne; abnormal 1/4 in limb-girdle, 0/1 in facioscapulohumeral, 1/2 in congenital, 1/1 in myotonic and 4/16 (23%) in healthy control. Abnormal EEG findings were negative spike 31, spike & wave complex 9, 14 & 6c/s positive spikes 14, sharp wave 10, theta (sporadic or burst) 57, delta (sporadic or burst) 29, fast rhythm 10, low voltage 11, etc. in Duchenne dystrophy. Fourteen & 6c/s positive spikes were rare in others and healthy controls.
High incidence of intellectual and EEG abnormalities in the muscular dystrophy may be due to the undetermined disturbance in the brain which is caused by the same genetic defect for the dystrophic change in muscle.

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Psychiatric interviews and clinical psychological test have been carried out on 31 patients with progressive muscular dystrophy. Ages are ranged from 8 to 17 years. Correlation between clinical findings and severity of the disease has also been examined.
In these patients have been commonly seen psychological hypofunction, indifference, inability to maintain concentration, poor association, indifferentiation of reality testing, decreased sensitivity to stimulus, supression of emotional expression, hypofunction of examining and criticizing reality, immaturity of verbal expression, and poor experiences of the past. The patients usually tend to consider frustration as inevitable and rarely make efforts for a complete solution of the problems. Their types of adjustment are characterized by passive and introvert stability or instability prone to lead to emotional problems.
On the other hand, in 17 patients have been noted behavior problems such as neurotic habit disorders, restlessness, asthenic and aggressive attitude. It is especially interesting to note transient ill humor and melancholy occurring frequently in the patients of middle school age. In addition, they are unwilling to study hard at the bed school, the disease is always borne deeply in their minds, and they are easily frustrated in the relation to doctors, nurses, therapists and bed school teachers.
No definite correlation between clinical findings and severity of the disease could be elicited. However, it seemed that some of the test results correlated with severity of the disease.

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Case 1: 13-year-old boy, case 2: 15-year-old boy and case 3: 13-year-old boy. All the cases were clinically far advanced Duchenne type of progressive muscular dystrophy (PMD) with about ten years course from onset and were bed-patients.
1) In all the cases, the examined muscles showed typical myopathic changes, but there were marked differences in severity of the histopathological findings according to sorts of muscles. The muscles in the limbs and girdles revealed prominent changes. On histopathological examinations, mild but obvious dystrophic changes were observed in the muscles which showed clinically no abnormalities, such as the tongues or the extraocular muscles.
2) Perivascular cell infiltrations were seen in the only two examined muscles of case 1 and case 3.
3) Atrophy of muscular fibers in group, so called large group of small fibers, which may suggest neurogenic atrophy, was noted besides the myopathic findings in the intercostal muscle of case 2 and in the abdominall rectus muscle of case 3.
4) The muscle-spindles appeared almost normal, but some of them showed thickening of the capsuls and proliferation of the connective tissue surrounding of the intrafusal muscle fibers. Furthermore, fibrosis of the intrafusal muscle fibers was observed not rarely in case 3.
5) In all the cases, the walls of the intramuscular arteries showed marked thickening which consisted of hyperplasia of the media and adventitia with elastosis and fibrosis. These findings of the arterial walls could be recognized not only in the skeletal muscles but also in the digestive tracts, the urinary bladders, the hearts and the nerves. The myocards showed marked patchy fibrosis. In some coronary arteries, proliferation of the intima was observed too.
6) The brains revealed no prominent abnormalities except mild venous stagnation and slight destruction of Purkinje's cells of cerebellums. In the spinal cords, the anterior horn cells decreased a little. In case 3, demyelination was observed extensively in the posterior columns and moderately in the spinocerebellar tracts. There were degenerations of some nerve cells in the sympathetic-and spinalganglia. In the peripheral nerves of all the cases, the pathological changes such as swelling or ballooning of the axons and demyelination were occasionally seen, which changes were not rarely observed especially in case 3.
Though it is difficult to interpret the marked changes in the spinalcord of case 3, the authors would insist possibility that there are involvements of nervous system in some PMD, especially in advanced cases at least.

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During urinalysis of 39 patients with progressive muscular dystrophy (PMD) we have met with a patient who excreted a considerable amount of diphosphopyridin nucleotide (DPN) into his urine. The method employed was column chromatography using Dowex (resin) IXIO (formic type). The isolated and purified substance was identified as DPN by paper chromatography by its absorption spectrum showing the characteristic pattern and absorbancy change when added with potassium cyanide and yeast alcohol dehydrogenase.
In a few other PMD cases we could also observe the presence of a small amount of DPN by enzymatic determination on the elutions from resin which had been suspended in there urines beforehand. We could not, however, prove the presence of DPN in the urine of other diseases.
There have been reports concerning excretion of abnormal substances such as amino acid, creatine, creatinine and pentose into urine of PMD patients but none of DPN except ours. At present nothing definite can be said about the appearance of DPN in the urine of patients with PMD, whether it has some bearings on the pathogenesis of those patients.

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Analysis of aminoaciduria of Duchenne type muscular dystrophy patients and of their mothers was performed.
Relation between the aminoaciduria and activity of serum creatin phosphokinase was also studied.
1) Excretion of arginine was much in dystrophic children and glycine was less than controls.
2) Urinary arginine and histidine-lysine fraction level of mothers, who were possible and probable carriers, was higher than controls and glycine was lower.
3) Mothers especially having higher serum creative phosphokinase seemed to have the above mentioned tendency of aminoaciduria.

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The activities of enzymes, cholinesterase (ChE), creatine phosphokinase (CPK), glutamic oxalactic transaminase (GOT), lactic dehydrogenase (LDH) and inhibiting activity of ChE by dibucaine (DN) in serum were measured in 40 patients of progressive muscular dystrophy (PMD).
Forty cases of PMD were classified in 34 cases of Duchenne type, 5 cases of limb-girdle type and 1 case of facioscapulohumeral type.
Results:
1) The activities of ChE in each PMD were measured for 3 times during 3 months and they were equal to normal human subjects.
2) There were no significant relationship between the activities of ChE in each PMD and the activities of the other enzymes; CPK, GOT and LDH.
3) The activities of DN in each PMD were fixed regardless of the activities of the other enzymes; ChE, CPK, GOT and LDH.

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A study on the activities of creatin-phosphokinase (CPK), lactic dehydrogenase (LDH) and aldolase in sera was carried out for 137 cases of progressive muscular dystrophy (PMD) and their relatives.
In patients of Duchenne type of PMD, it was reconfirmed that an elevation of serum CPK activity was much more remarkable than that of the other enzyme activities.
The frequency of abnormalities in the CPK activity was about 20 per cent in mothers of Duchenne type of this disease, while that in aldolase and LDH was only a few per cent.
A correlation in the increases of CPK and the other enzymes was not found in relatives of the patients.

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