Linked Life Data

9008527

Source:http://linkedlifedata.com/resource/pubmed/id/9008527

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
1
pubmed:dateCreated
1997-2-26
pubmed:abstractText
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).
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
AIM
pubmed:status
MEDLINE
pubmed:month
Jan
pubmed:issn
0028-3878
pubmed:author
pubmed:issnType
Print
pubmed:volume
48
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
253-7
pubmed:dateRevised
2004-11-17
pubmed:meshHeading
pubmed:year
1997
pubmed:articleTitle
Autosomal dominant hyaline body myopathy presenting as scapuloperoneal syndrome: clinical features and muscle pathology.
pubmed:affiliation
Department of Neurology, Mie University School of Medicine, Tsu City, Japan.
pubmed:publicationType
Journal Article, Case Reports