Shigeo Okinaka

Juvenile muscular atrophy of unilateral upper extremity

IN 1959 WE SUGGESTED a new clinical entity of juvenile muscular atrophy of unilateral upper extremity,l based on our experience of I2 cases. The present communication deals with more detailed observation and laboratory tests on 11 of the 12 patients whose cases were previously reported and 9 additional patients examined during the past three years. One of the 12 patients was excluded in view of the further clinical course. Muscle biopsy specimens taken from 3 patients enabled us to differentiate this syndrome definitely from such myopathies as myositis and muscular dystrophy. Juvenile onset, localized muscular atrophy with unique distribution in the arm of one side, and a nonprogressive course in later stages characterize this disorder. This muscular atrophy is distinctly different from any hitherto described entities of muscular atrophy.

Collateral circulation of the brain: With special reference to atherosclerosis of the major cervical and cerebral arteries

COLLATERAL CIRCULATION is of most concerii in pathologic processes in the brain, particularly in occlusive disease of the cervical and cerebral arteries. The collateral circulation to the brain exists in 3 main areas:l (1) through extracranial arterial connections, ( 2 ) through persisting developmental extracranial to intracranial arteries, and (3) through intracranial cerebral arterial anastomoses. The authors directed efforts toward an analysis of collateral circulation to the brain of aged subjects with some degree of atherosclerosis in the cervical as well as in the cerebral arteries. The present study consists of 3 parts: (1) a study of atherosclerosis of the major cervical and cerebral arteries; (2 ) a demonstration of the collateral circulation between the internal carotid, external carotid, and vertebral arteries. with their intracranial anastomoses, emphasizing the variations of the basal cerebral arteries, including the circle of Willis; and ( 3 ) a clinical consideration of subjects with severe stenosis of the cervical arteries, with regard to cerebral vascular insufficiency due to a fall in blood pressure.

Multiple sclerosis and allied diseases in Japan: Clinical characteristics

MULTIPLE SCLEROSIS is said to be more prevalent in the temperate zones of North America, western Europe, and Australia than in the subtropical or tropical zones of these same There is still some controversy as to whether susceptibility is related to race and national origin or to an environmental factor that affects all races in the same geographic area equally. As early as 1911, multiple sclerosis was said to be almost nonexistent in Japan.a It is the general belief that this disease is also rare among other Oriental populations. In 1929, Woods9 found only 6 cases among the 4,000 neurologic cases in the Peking Union Medical College. In 1955, Imamuralo reviewed the Japanese literature and collected 179 cases of multiple sclerosis and its allied diseases. Although beriberi (thiamine deficiency neuritis) has been regarded as one of the important causes of retrobulbar neuritis, Kuwajima11.12 stressed multiple sclerosis as an important cause of retrobulbar neuritis, a common disease in Japan. One wonders, in view of the general belief that multiple sclerosis is rare or absent in Japan, whether neurologic practice in Japan is going through the same stages as that in the United States some years ago. Even as late as 1921, multiple sclerosis was considered to be less prevalent in the United States than in Europe. However, the studies of MacLean and BerksonI3 in Rochester, Minn., and the population surveys of Ipsen14 and Kurland16 in Boston reveal that multiple sclerosis prevalence is about as high as in equally populated areas in Europe. Whether history will repeat itself in japan remains to be seen.

Studies on electrolyte metabolism in idiopathic and thyrotoxic periodic paralysis

Abstract A study of arteriovenous serum potassium and sodium changes during an induced attack of periodic paralysis was undertaken in 7 patients with idiopathic periodic paralysis and 6 patients with thyrotoxic periodic paralysis. In both types of periodic paralysis, a constant positive arteriovenous difference of potassium was obtained during the development of paralysis. The findings lend further support to the hypothesis that the cellular migration of potassium from the extracellular space is the mechanism of hypokalemia in both idiopathic and thyrotoxic periodic paralysis.

ENCEPHALOMYELOPATHY DUE TO AN ORGANIC MERCURY COMPOUND.

OUR INTEREST in the toxicity of organic metal compounds was stimulated by reports of organic tin intoxication in 19551 and the “Minamata disease,” presumably caused by organic mercury compound,2 which was reported in 1957.3 An excellent review on the clinical, histopathologic, and epidemiologic aspects of this disease has been published.4 A few reports concerning the intoxication of man by organomercury compounds indicate the cause to be the inspiration of the fumes of agricultural drugs in most cases. In 1940, Hunter and associates5 reported 4 cases of intoxication by methylmercury phosphate or nitrate. Fourteen years later, Hunter and Russell6 described detailed histopathologic findings in the brain of one of the patients in the previously reported cases. The microscopic changes in the brain were most pronounced in the occipital pole region of the cerebrum and palleocerebellum. Constriction of the visual fields may be attributed to atrophy of the area striata and, according to their statement, the ataxia could be correlated with changes in the cerebellar cortex. Brown7 reported clinical and autopsy findings in a case of phenylmercury acetate intoxication which showed a clinical picture resembling amyotrophic lateral sclerosis. Microscopic findings showed degeneration of motor cells in the anterior horn and the lateral column of the spinal cord, without changes in the brain. Kantarjians also pointed out the clinical resemblance to amyotrophic lateral sclerosis in 11 cases of definite ethylmercury ptoluene sulfonanilide intoxication. This paper is a report of 3 cases of intoxication caused by the application of a solution containing organic mercury compound for the treatment of a widespread fungus skin infection.

The representation of the pyramidal tract in the internal capsule and basis pedunculi. A study based on three cases of amyotrophic lateral sclerosis.

IT HAS long been established that amyotrophic lateral sclerosis is a disease which invades the pyramidal tracts. Detailed topoanatomic studies of changes in the internal capsule in this disease, however, have not been performed except by Probst’ in 1898, Mane, Bouttier, and Bertrand2 in 1923, and Bertrand and Van Bogaert? in 1925. According to their descriptions, changes were limited to a small part of the area which has been considered to be occupied by the pyramidal tract. Probst, studying horizontal sections, demonstrated a marked atrophy of the pyramidal tracts in the posterior part of the posterior limb of the internal capsule; less marked atrophy was present in the anterior part of the posterior limb. Marie and associates and Bertrand and Van Bogaert found that, in horizontal sections, degenerated fibers were confined to a narrow square within the posterior limb lateral to globus pallidus. Although they stressed the topographic importance of their findings, these observations have strangely escaped the attention of neurologists and neuropathologists. In addition, very little topographic information is available with regard to changes in the basis pedunculi in patients with amyotrophic lateral sclerosis. This study was undertaken to elucidate the localization of degenerating pyramidal tract fibers in the internal capsule and the basis pedunculi, through fiber-analytic studies of 3 cases of amyotrophic lateral sclerosis and 1 case without neurologic manifestations. MATERIALS AND METHODS

Studies on electrolyte metabolism in idiopathic and thyrotoxic periodic paralysis: III. Intra- and extracellular concentrations of potassium and sodium in muscle and their changes during induced attacks of paralysis☆

Abstract Muscle biopsy studies were performed in 8 patients with idiopathic hypokalemic periodic paralysis, 8 patients with thyrotoxic hypokalemic periodic paralysis and 7 patients with thyrotoxicosis without periodic paralysis. Content and intracellular concentration of potassium are signicantly reduced in idiopathic periodic paralysis as compared to thyrotoxic periodic paralysis. There is no constant and characteristic change in intracellular concentration of potassium per se during paralysis, while K i K o ratio increases roughly parallel to the severity of induced paralysis.

Studies on electrolytes metabolism in idiopathic and thyrotoxic periodic paralysis: II. Total exchangeable sodium and potassium

Abstract A study of total exchangeable potassium, sodium and total body water was undertaken in 8 normal subjects, 7 patients with thyrotoxicosis without periodic paralysis, 8 patients with idiopathic periodic paralysis and 7 patients with thyrotoxic periodic paralysis. Our findings show that total exchangeable potassium is modestly but significantly reduced in idiopathic periodic paralysis, but within normal limits in thyrotoxic periodic paralysis.

Studies on the control of coronary circulation Part I. The effect of the stimulation of the nerves on the coronary circulation. Part II. The humoral effect on the coronary circulation

Abstract 1. 1. A study was made of the coronary vasodilatative effects of coronary venous blood samples obtained following the stimulation of the sympathetic and vagal nerves, and of norepinephrine, acetylcholine, and ATP. 2. 2. In the coronary venous blood samples, obtained either during or 15 seconds after the stimulation of the sympathetic nerves, some vasodilatative substances were demonstrated. They may play one of the important roles in sympathetic coronary vasodilatation. The exact nature of the vasodilatative substances are unknown, but they may be the metabolites released following the sympathetic stimulation. 3. 3. The coronary vasodilatative effect of norepinephrine differs in nature from that of acetylcholine, ATP, and effective coronary venous blood. It may result mainly from hemodynamic and metabolic action due to the modification of the heart. The action of norepinephrine on coronary vessels seems to be identical with that of the sympathetic nerves.

Studies on hepatocerebral disease: III. Hepatolenticular degeneration in Japan, with studies on copper metabolism

Age in Years Fig. 1. Age and sex of patients in Japan with Wilson’s disease in accord with American and European studies, that a disturbance of copper metabolism also plays a major role in the pathogenesis of Wilson’s disease in Japan. Similar but rare cases with clinicopathologic findings suggestive of Wilson’s disease but without an abnormality of copper metabolism have also been seen. Wilson’s disease used to be considered a very rare disease in Japan. However, much interest was aroused in this disease recently, and 62 cases have been reported in the past ten years. The inference is well grounded that many more unreported cases must be present in this countrv. The distribution of the patients showed no pnrticular grouping, and the cases were found in almost all sections of Japan. The heredity of 43 families was investigated in regard to this condition. Familial occurrence of the disease or blood relationship between father and mother of the patient was demonstrated in 15 families, or 35%. In the latter cases, most marriages were between cousins.