Hyaline bodies are rare subsarcolemmal aggregates in type 1 fibers of the skeletal muscle, stain pale pink with hematoxylin-eosin and pale green with the modified Gomori trichrome, and lack reactivity for glycogen and oxidative enzymes. We report clinical findings of autosomal-dominant hyaline body myopathy in seven members in four generations and muscle biopsy findings in two of them. Slowly progressive muscle weakness and atrophy developed with scapuloperoneal distribution; age at onset was from the first to the fifth decade. Muscle biopsy showed subsarcolemmal hyaline bodies in approximately 20% of type 1 fibers. Hyaline bodies showed myofibrillar ATPase activity after acid pre-incubation. Immunohistochemically, they stained intensely with myosin heavy chain (slow), but not with myosin heavy chain (fast). Ultrastructurally, they consisted of granules sometimes in linear array, filaments, and amorphous materials. These findings suggest that hyaline bodies may be products of degeneration of myosin heavy chain (slow).
Clinical significance of the globus pallidus signal intensity ratio in patients with liver cirrhosis The object of this study was to evaluate the clinical value of the globus pallidus signal intensity ratio for the subclinical detection of hepatic encephalopathy. This study comprised 25 patients with liver cirrhosis without overt hepatic encephalopathy. There was a high frequency (56%) of patients exhibiting increased signal in the globus pallidus. The pallidal signal was related to the severity of the liver disease. The auditory brain stem reaction was not correlated with the pallidal intensity and laboratory parameters. During the follow-up study, 3 out of 5 patients presenting overt hepatic encephalopathy showed strong pallidal signals. The results of this investigation suggest that abnormal globus pallidus signal may constitute a useful method for the subclinical detection of hepatic encepalopathy.
We report one case of bullous pemphigoid that did not respond to the standard western medical treatment with topical and oral steroid therapy. This case achieved complete remission after the application of Yizongjinjion seihijoshitsuinkagen (Yizongjinjion Qing Pi Chu Shi Yin Hua Cai), a spleen-dampness-eliminating beverage. The patient was a 78-year old female who had soy bean or 5 mm to 25 mm sized blisters covering a wide area of her back since two years ago. She had visited a neighborhood physician who diagnosed her with bullous pemphigoid and initiated the standard treatment. Although some improvements were observed after the start of western medical treatment, her symptoms eventually grew worse. Therefore, she visited our clinic and requested the traditional Chinese treatment. We diagnosed her skin blisters as the heart-fire spleendampness type of bullous pemphigoid using the four diagnostic examinations, and treated her with the modulated spleen-dampness-eliminating beverage which resulted in a complete remission within 10 months. Taken together, our results suggest that the modulated spleen-dampness-eliminating beverage (Yizongjinjion seihijoshitsuinkagen) is effective for the treatment of the heart-fire spleen-dampness type of bullous pemphigoid. bullous pemphigoid, seihijoshitsuin, heart fire spleen dampness, treatment of traditional Chinese medicine
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