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-girdle muscular dystrophy

Etymology: AS, lim, limb, gyrdel
a form of muscular dystrophy transmitted as an autosomal-recessive trait. The characteristic weakness and degeneration of the muscles begins in the shoulder girdle or in the pelvic girdle. The condition is progressive, regardless of the area in which it is first manifest. Kinds of limb-girdle muscular dystrophy are Erb's muscular dystrophy, Leyden-Möbius muscular dystrophy.

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 ?
Instead, scientists in this study "reprogrammed" adult cells from patients with limb-girdle muscular dystrophy into stem cells and were able to induce them to differentiate into mesoangioblast-like cells.
Injections of the alphasarcoglycan gene in the foot muscles of three patients with limb-girdle muscular dystrophy type 2D produced 4-5 times the amount of the alpha-sarcoglycan protein, compared with saline injections.
The researchers point out that limb-girdle muscular dystrophy actually describes more than 19 disorders that occur because patients have a faulty alpha-sarcoglycan gene.
Autosomal recessive limb-girdle muscular dystrophy, LGMD2F, is caused by a mutation in the delta-sarcoglycan gene.
WHAT ARE THE DIFFERENT FORMS OF LIMB-GIRDLE MUSCULAR DYSTROPHY?
At the mildest end of the clinical spectrum, limb-girdle muscular dystrophy (LGMD) patients may present in adult life without associated brain or eye involvement.
Exclusion of identified LGMD 1 loci from four dominant limb-girdle muscular dystrophy families.
Muscular dystrophies include Duchenne, Becker, Emery-Dreifuss, limb-girdle, congenital and myotonic.
2) Inclusion body myositis is marked by painless, slowly progressive, asymmetric muscle weakness and atrophy affecting proximal muscles in a limb-girdle distribution.
Since 1986, scientists have identified genes that, when flawed, can cause myotonic, Duchenne, Becker, limb-girdle (some forms), congenital (one form) and Emery-Dreifuss muscular dystrophies.
He is not only a physician, but also a patient of MDA as he is challenged by limb-girdle muscular dystrophy himself.
Results: In the family, the proband showed limb-girdle pattern of weakness with sparing of ocular, facial, bulbar, and respiratory muscles.