Eijiro Satoyoshi

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.

Synthesis and secretion of nerve growth factor by mouse astroglial cells in culture

Astroglial cells cultured from the mouse brain have been found to synthesize and secrete a material(s) with nerve growth factor-like immunoreactivity (NGF-LI) into their culture medium. A material(s) with NGF-LI showed identical properties to those of beta NGF purified from the mouse submaxillary gland in immunoreactivity, molecular weight, isoelectric point, and neurite outgrowth stimulatory activity. These results indicate that astroglial cells cultured from mouse brain are able to synthesize and secrete beta NGF in culture.

Synthesis/secretion of nerve growth factor is associated with cell growth in cultured mouse astroglial cells

Astroglial cells cultured from 8-day-old mouse brain synthesized and secreted nerve growth factor (NGF). An increase in cell density or the withdrawal of serum from the culture medium caused a drastic decrease in the rate of NGF secretion which could be reversed by reculturing at a low cell density or by refeeding with serum-containing culture medium. The cells cultured for two weeks without serum entered the quiescent phase without loss of the activity of an astroglial marker enzyme, glutamine synthetase. These results suggest that NGF secretion by astroglial cells in vitro is regulated in a growth phase-dependent manner. Evidence is also presented to show that NGF secretion is not phase-specific in the cell cycle.

Catecholamines increase nerve growth factor mRNA content in both mouse astroglial cells and fibroblast cells

Previous studies have shown that catecholamines increase the nerve growth factor (NGF) content in medium conditioned by mouse L‐M fibroblast cells and mouse astroglial cells. In this study, the NGF mRNA levels in these cells were measured by Northern blot analysis. In astroglial cells treated with epinephrine (EN), the cellular NGF mRNA level increased prior to accumulation of NGF in the culture medium. 3‐Hydroxytyramine (DA) and norepinephrine (NE) also increased the cellular NGF mRNA content. An increased level of NGF mRNA elicited by EN was also observed in mouse L‐M cells. These results indicate that catecholamines enhance NGF synthesis of L‐M fibroblast cells and astroglial cells by increasing the cellular content of NGF mRNA. The present results also indicate that the effects of catecholamines are not mediated by adrenergic receptors.

A syndrome of progressive muscle spasm, alopecia, and diarrhea.

A syndrome of progressive muscle spasm, alopecia, and diarrhea was seen in 15 patients. The syndrome was characterized by painful intermittent muscle spasm, alopecia, amenorrhea, and malabsorption, and was sometimes associated with epiphyseal destruction and retarded growth. Symptoms began at age 10 and were more common in women than men. Muscle cramps affected the limbs first and then, several years after onset, the neck, trunk, and masticatory muscles. The course was progressive and led to malnutrition. Four patients died from 5 to 18 years after onset. Autopsy revealed polypoid changes throughout the gastrointestinal tract.

MYOPATHY IN THYROTOXICOSIS. WITH SPECIAL EMPHASIS ON AN EFFECT OF POTASSIUM INGESTION ON SERUM AND URINARY CREATINE.

HEIGHTENED METABOLIC ACTIVITY of hyperthyroidism may be reflected in a diversity of clinical features. Of these, some degree of weakness, often most noticeable at hips or trunk, is extremely common. Occasionally this proximal weakness, frequently accompanied by disproportionate muscle wasting, is sufficiently disabling functionally to be a principal presenting symptom of the disorder. Accordingly the “myopathy” of thyrotoxicosis has been a subject of several communications since the end of the last century.l-ll Admittedly, however, the number of reported cases with muscular involvement sufficiently severe to merit clinical designation as “chronic thyrotoxic myopathy”4 is small. A completely satisfactory explanation for the discrepancy between this relative scarcity and the high frequency with which weakness is encountered in hyperthyroidism is not forthcoming. It has been repeatedly emphasized, however, that thyrotoxic patients with severe muscle involvement are usually older than those without it, they have had symptoms longer, and their other clinical manifestations of thyroid disorder are less overt.”6.s,o I t has been suggested, therefore, that frank myopathy results from prolonged unrecognized hyperthyroidism. For this to be convincing, some alteration in muscle, even though subtle, from shorter periods of thyrotoxicosis should be demonstrable. This could conceivably be in power, electromyographic pattern, histologic appearance, or metabolism. However, reported data, on all of these are either scanty or contradictory. Dynamometry, for example, has not been reported in a large series of thyrotoxic patients before and after treatment. Electromyograms, recorded in the presence of frank weakness, .have , been reported as norma1,lO or alternatively as indicating myopathy.1l Histologic abnormalities have been frequent in some series,5J1-zgJO but absent in others.7J0 Granted that creatinuria, common in muscle disease, is also a feature of hyperthyroidism,”h6.‘“-‘“ it has not been present in all groups with chronic thyrotoxic myopathy.6 Moreover, muscle taken from thyrotoxic patients has not been subjected to more than cursory chemical analysis. I t is the purpose of the present communication to report briefly on the incidence, in a large series of unselected hospitalized thyrotoxic patients, of weakness and wasting of sufficient degree to warrant a clinical diagnosis of myopathy. In a smaller number of these patients and a number of persons judged to be suitable controls, muscle taken at biopsy was subjected to histologic and chemical study.

Aliphatic side chain of catecholamine potentiates the stimulatory effect of the catechol part on the synthesis of nerve growth factor

Catecholamines are potent in stimulating nerve growth factor (NGF) synthesis in mouse L‐M cells. The relationship between the structure of catecholamines and their stimulatory effect on NGF synthesis has been studied using various 3,4‐dihydroxyphenyl derivatives or their analogues. All 3,4‐dihydroxyphenyl derivatives with two saturated carbons on the side chain were potent stimulators, whereas those with only one carbon on the side chain were weak stimulators. Drugs lacking the catechol ring were not effective. These results suggest that the catechol part of catecholamines is essential for the stimulatory effect and that the aliphatic side chain potentiates this effect. The present results also suggest the terminal amino residue on the side chain is not critical for the effect.

Action‐induced rhythmic dystonia An autopsy case

We studied a patient with action-induced rhythmic dystonia that followed a stroke. Postmortem studies showed an infarct in the right posterolateral ventral part of the thalamus. Electrophysiologic analysis indicated that the eliciting factor of the involuntary movement was an impulse, promoting voluntary contraction of muscle. CSF 5-HIAA content was low, and HVA was high. Administration of 5-HTP and clonazepam abolished the involuntary movements.

HLA antigens and myasthenia gravis in Japan.

Recent studies have noted an increased association of the histocompatibility antigen HLA-B8 with myasthenia gravis in Caucasian patients. The HLA types of 63 Japanese patients with myasthenia were compared to those of 271 controls. The present study was designed to assess correlations between HLA antigens, sex, age at onset, the presence of autoantibodies and thymic morphology. HLA-B12 was increased Japanese patients, especially females with early age at onset, and in addition it was significantly correlated with thymic hyperplasia. HLA-B5 was frequently found in the patients with thymoma. Statistically the most frequent haplotype found in this disease was HLA-A10-HLA-B12.

Familial Type I Fiber Atrophy

An 11-year-old boy and his 40-year-old mother with congenital, non-progressive muscular weakness and wasting are described. Muscle biopsies from both cases showed a selective atrophy of Type I fibers without any structural change except for very few nemaline bodies. Probably, the neuromuscular disorder in this family is identical to the congenital fiber type disproportion described by Dubowitz and Brooke, but familial Type I fiber atrophy (hypotrophy, or hypoplasia) is considered to be a more appropriate descriptive term for a family with such distinct histochemical characteristics.