limb-girdle muscular dystrophy

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limb-·gir·dle mus·cu·lar dys·tro·phy

[MIM*253600]
a group of muscular dystrophies, probably heterogeneous in nature. Onset usually in childhood or early adulthood and both sexes affected. Characterized by weakness and wasting, usually symmetric, of the pelvic girdle muscles, the shoulder girdle muscles, or both, but not the facial muscles. Muscle pseudohypertrophy, heart involvement, and mental retardation are absent. Autosomal dominant and recessive inheritance have been described.

limb-girdle muscular dystrophy

n.
Any of several forms of muscular dystrophy that chiefly affect the skeletal muscles of the shoulder and pelvic girdles, with symptoms beginning at any age.

limb-gir·dle mus·cu·lar dys·tro·phy

(lim'gĭr'dĕl mŭs'kyū-lăr dis'trŏ-fē)
One of the less well-defined types of this disorder, it is characterized by weakness and wasting, usually symmetric, of the pelvic girdle muscles, the shoulder girdle muscles, or both, but not the facial muscles. Muscle pseudohypertrophy, heart involvement, and mental retardation are absent. Variable inheritance.

Limb-girdle muscular dystrophy (LGMD)

An autosomal recessive form of muscular dystrophy that appears anywhere from late childhood to middle age and is characterized by progressive muscular weakness beginning either in the shoulder or pelvic girdle; the disease usually progresses slowly with cardio-pulmonary complications in the later stages

Leyden,

Ernst V. von, German physician, 1832-1910.
Charcot-Leyden crystals - see under Charcot
Leyden ataxia - Synonym(s): Westphal-Leyden syndrome
Leyden crystals - Synonym(s): Charcot-Leyden crystals
Leyden disease
Leyden-Möbius muscular dystrophy - Synonym(s): limb-girdle muscular dystrophy
Leyden neuritis - fatty degeneration of the fibers of the affected nerve.
Westphal-Leyden syndrome - see under Westphal

Möbius,

Paul J., German physician, 1853-1907.
Leyden-Möbius muscular dystrophy - Synonym(s): limb-girdle muscular dystrophy
Möbius sign - impairment of ocular convergence in Graves disease.
Möbius I syndrome - moderate migraine accompanied by extraocular palsy. Synonym(s): occasional oculomotor paralysis; ophthalmoplegic migraine
Möbius II syndrome - a developmental bilateral facial paralysis usualy associated with oculomotor or other neurological disorders.Synonym(s): congenital facial diplegia; developmental bilateral facial paralysis
References in periodicals archive ?
MRI in the assessment of muscular pathology: a comparison between limb-girdle muscular dystrophies, hyaline body myopathies and myotonic dystrophies[J].
Bruno et al., "Mutations in the caveolin-3 gene cause autosomal dominant limb-girdle muscular dystrophy," Nature Genetics, vol.
Emery, "A population study of adult onset limb-girdle muscular dystrophy," Journal of Medical Genetics, vol.
Westra et al., "Limb-girdle muscular dystrophy in the United States," Journal of Neuropathology & Experimental Neurology, vol.
The clinical presentation of our patients with LOPD was classical, characterized by progressive limb-girdle muscle weakness in all cases and diaphragm involvement in 2 patients, leading to respiratory insufficiency and early death in the patient number 2 in agreement with previous studies [19, 35].
| ON THE UP: Brothers Ben (left) and Sam Booth who are planning the Olympus Mons Challenge at Rokt Climbing Gym (left) in aid of Limb-Girdle Muscular Dystrophy.
However, the authors found that they could not get a sufficient number of mesoangioblasts from patients with limb-girdle muscular dystrophy because the muscles of the patients were depleted of these cells.
In a separate study, he and his associates transferred the large limb-girdle muscular dystrophy type 2D gene via the adeno-associated virus (Ann.
There are several forms of muscular dystrophy (MD) that affect children including Duchenne, facioscapulohumeral, limb-girdle, and myotonic muscular dystrophy.
Described as the first gene therapy trial in muscular dystrophy demonstrating promising findings, researchers from the University of Florida (UF), Nationwide Children's Hospital in Columbus, Ohio, and The Ohio State University report how they safely transferred a gene to produce a protein necessary for healthy muscle fiber growth into three teenagers with limb-girdle muscular dystrophy.
Washington, April 16 (ANI): Researchers from the University of Florida (UF), Nationwide Children's Hospital in Columbus, Ohio, and The Ohio State University have safely transferred a gene to produce a protein necessary for healthy muscle fibre growth into three teenagers with limb-girdle muscular dystrophy.
It is worth noting that mutations in the [delta]-sarcoglycan gene have been also reported in patients with idiopathic dilated cardiomyopathy and limb-girdle muscular dystrophy-2F (28-31).