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[The application of guanidine hydrochloride to the treatment of degenerative nervous and muscular diseases. I. Clinical results (author's transl)].

This paper reports the results obtained on using guanidine hydrochloride in the treatment of patients with amyotrophic lateral sclerosis, degenerative diseases of the spinocerebellar system or the peripheral nervous system and dystrophic muscle diseases. A long-term effect of the substance was a diminution in the rate of progression of the diseases, with the exception of the group with dystrophic muscle diseases. Initial clinical improvement occurred in certain patients of both groups. The substance seems to be more effective in less-advanced cases than on administration in the later stages of the disease. The therapeutic dosage was 20 to 40 mg/kg/day. The most frequent side-effect was paraesthesia and sometimes gastric disturbance was reported. Therapy had to be discontinued in 3 patients due to leucopenia. In these patients the symptoms rapidly increased in severity after discontinuation of treatment. This supports the assumption that guanidine hydrochloride treatment slows down the progress of the disease.

Adult↗

[Macrophagic myofasciitis: inflammatory, vaccination-associated muscular disease].

HISTORY AND ADMISSION FINDINGS: A 67-year-old man presented with a 6-week history of slowly progressive muscle pain and generalized muscular weakness. Neurological examination showed symmetrical proximal weakness (MRC grade 4/5). INVESTIGATIONS: Laboratory tests revealed an elevated CK level of 124 U/l (normal < 80). Needle EMG showed pathological spontaneous activity in proximal muscle groups. A diagnostic muscle biopsy taken from the left deltoid muscle revealed a characteristic infiltrate of PAS-positive and acid phosphatase-positive macrophages. Electron microscopic analysis of muscle tissue failed to demonstrate aluminium hydroxide inclusion. Serological analysis excluded antibodies against HAV and HBV but was compatible with a previous tetanus vaccination (antibody titre of 2.3 IE/ml). TREATMENT: A 2-year treatment with steroids and azathioprine led to complete recovery of muscle strength without relapse after discontinuing the immunosuppression. CONCLUSION: Macrophagic myofasciitis (MMF) is a rare inflammatory muscle disorder characterized by a characteristic infiltration of muscle tissue by PAS-positive macrophages, which is caused by pathological persistence of vaccine-derived aluminium hydroxide. The diagnosis can only be established by an open muscle biopsy from the muscle that was used for intramuscular vaccination.

Adrenal Cortex Hormones↗

[Subvalvular aortic stenosis, dysmorphic familial syndrome and peripheral muscular disease].

Two related cases are reported, those of two sisters aged 7 and 13, who had an aortic subvalvular stenosis in the form of a fibro-muscular channel, associated with mitral incompetence, a triangular facies with a pointed chin, pinched lips, a divergent squint, hypertelorism, a mask-like face, normal intelligence, and a peripheral muscular disorder with arthrogriposis. The description of such a combination of malformations is original, and this is discussed.

Abnormalities, Multiple↗