Tadao Tsubaki

The Crow‐Fukase syndrome A study of 102 cases in Japan

Clinical manifestations of 102 cases with the Crow-Fukase syndrome (the syndrome of polyneuropathy, anasarca, skin changes, endocrinopathy, dysglobulinemia, and organomegaly), with or without myeloma, were reviewed. Fifty-six cases with myeloma consisted of 31 with osteosclerotic, 17 with mixed osteosclerotic and osteolytic, and 8 with osteolytic. Forty-six cases without myeloma consisted of 2 with extramedullary plasmacytoma, 33 with M protein alone, and 11 with polyclonal protein alone. There was no significant difference in incidence of the major clinical manifestations between the two groups with and without myeloma. They had a common characteristic histologic finding of the lymph node resembling that of Castlemans disease.

Nationwide survey of multiple sclerosis in Japan Clinical analysis of 1,084 cases

Between October 1972 and October 1973, the first nationwide survey of the multiple sclerosis group of diseases in Japan was performed by the Multiple Sclerosis Research Committee of Japan, supported by the Japan Ministry of Health and Welfare. Reports on 1,084 patients with the multiple sclerosis group were collected: 509 patients with multiple sclerosis, 82 with Devics disease, 357 with “multiple sclerosis possible,” and 136 with “other or unclassified demyelinating diseases.” The natural history in the present nationwide series of multiple sclerosis patients showed considerable similarity to that of patients in Western countries, suggesting that multiple sclerosis in Japan is essentially the same as that in the Western countries. However, the previously reported special characteristics of Japanese multiple sclerosis patients, namely, a higher rate of visual impairment at onset, a higher rate of optic nerve involvement during the course of illness, and a higher rate of Devics disease, were reconfirmed in the present series.

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.

Mitochondrial encephalomyopathy: Fluctuating symptoms and CT

We describe a 29-year-old man with mitochondrial encephalomyopathy. The patients disorder was characterized by lactic acidosis, hemiparesis, seizures, aphasia, and hemianopia. CT revealed low-density areas that corresponded to the symptoms. His 56-year-old mother is also involved subclinically, demonstrating that muscle biopsy is an important requisite in the final determination of a familial inheritance pattern in mitochondrial myopathy. Neuronal mitochondrial disorders are suggested as the pathogenesis of his neurologic symptoms.

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.

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

Electroencephalography in subjects sixty years and over.

Abstract 650 EEGs were examined in 466 normal subjects and patients over 60 years old. 1. 1. Abnormal EEGs were observed in 32.7% of normal subjects and in 44.5% of neurologically normal cases. The incidence of EEG abnormalities tended to augment with increasing decades in neurologically normal cases. The existence of high blood pressure did not seem to influence the incidence of EEG abnormalities either in neurological or non-neurological cases. 2. 2. In hemiplegic and hemiparetic patients who had survived more than one year after the apoplectic attack, EEGs were normal in 14.5% and 27%, respectively. No difference in the incidence of abnormal EEG was noted between right and left side paralysis; however, the correlation of the side showing more prominent EEG changes with the clinically paralysed side was significantly higher in right sided than in left sided paralysis in right-handed subjects. 3. 3. Alpha blocking was noted in 57.6% of neurologically normal subjects, decreasing significantly with increasing decade after the 6th, and was significantly lower in neurological patients than in subjects with no neurological manifestations. 4. 4. “Flat” EEGs were found in 8.6% of neurologically normal subjects and their incidence tended to decrease with increasing decade after the 6th. No significant difference was noted between neurologically normal and abnormal cases. 5. 5. The build-up after hyperventilation was poor in general, and was absent in 73% of 401 cases.

Enzymatic analysis of individual anterior horn cells in amyotrophic lateral sclerosis and duchenne muscular dystrophy.

Abstract Five enzyme activities related to glucose metabolism, i.e. glucose-6-phosphate dehydrogenase (G6PDH), isocitric dehydrogenase (ICDH), citrate synthase (CS), ATP citrate lyase (ACL), and pyruvate dehydrogenase complex (PDC), were estimated in anterior horn cells (AHCs) of spinal cords in amyotrophic lateral sclerosis (ALS) and Duchenne muscular dystrophy (DMD) patients by means of the NADP and CoA cycling methods. In ALS, only CS activity was reduced significantly in AHCs, but that of posterior root ganglion cells (PRGCs) remained within the normal range. In DMD, ICDH, CS, ACL and PDC activities were within normal range. However, G6PDH activities were elevated significantly in 2 out of 3 patients examined.

Familial Creutzfeldt-Jakob disease in Japan: Three cases in a family with white matter involvement

Three cases of Creutzfeldt-Jakob disease occurring in one family have been clinicopathologically examined. Although the age at onset, duration, and age at death differed for each case, pathological findings, including diffuse neuronal loss, astrocytosis, spongiform changes and patchy and/or diffuse white matter involvement were similar. Life histories and inheritance patterns of the present 3 cases and 2 other families previously reported in Japan are compared with the general findings for familial cases in western countries.

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.