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 ?
These exciting results demonstrate the feasibility of gene therapy to treat limb-girdle muscular dystrophy," said Jane Larkindale, portfolio director with Muscular Dystrophy Association Venture Philanthropy, a program that moves basic research into treatment development.
In addition to indications in FSHD, the Company has selected a specific form of limb-girdle muscular dystrophy, referred to as LGMD 2B, as its next expansion indication and is expected to move forward with a Phase 1b/2 clinical trial of Resolaris(TM) in LGMD 2B beginning in the fourth quarter of 2015.
After evaluating a number of muscular dystrophies (MD), including Duchenne muscular dystrophy and a broad class of MD indications of more than 20 rare genetic conditions known as the limb-girdle muscular dystrophies (LGMD), the Company has selected LGMD 2B as its next expansion indication after early onset FSHD.
CHICAGO, March 26, 2015 /PRNewswire-USNewswire/ -- The Muscular Dystrophy Association announced today that thanks to a grant from Genzyme, a Sanofi company, and in collaboration with Emory Genetics Laboratory, MDA clinics are now offering genetic testing for people experiencing muscle weakness suggesting limb-girdle muscular dystrophy (LGMD) who do not already have a genetic diagnosis.
14, 2014 /PRNewswire-USNewswire/ -- A new guideline from the American Academy of Neurology(AAN) and the American Association of Neuromuscular & Electrodiagnostic Medicine(AANEM) recommends guidance on how doctors should evaluate the full picture-from symptoms, family history and ethnicity to a physical exam and certain lab test results-in order to determine what genetic tests may best diagnose a person's subtype of limb-girdle or distal muscular dystrophy.
Limb-girdle muscular dystrophy most affects muscles close to the center of the body, such as in the areas near the tops of the arms and legs.
Becker muscular dystrophy (BMD) and Limb-Girdle muscular dystrophy (LGMD): Although both BMD and DMD are caused by mutations in the dystrophin gene, BMD is a milder form of dystrophy than DMD.
By the time he was diagnosed in 1992 with limb-girdle muscular dystrophy, or limb-girdle MD, Mathias had already distinguished himself as a food industry executive.
But the real highlight of the day was the moving remarks by Dana Parrott, a teenager and the Connecticut MDA Goodwill Ambassador living with Limb-Girdle Muscular Dystrophy.
Since 2001, federal funding and coordination of muscular dystrophy research has dramatically enhanced discovery and development of potential treatments for the muscular dystrophies, including: Duchenne and Becker muscular dystrophies (DMD/BMD, or also known as DBMD); congenital muscular dystrophy (CMD); facioscapulohumeral muscular dystrophy (FSHD); limb-girdle muscular dystrophy (LGMD); and myotonic muscular dystrophy (MMD).
Joining Triple H in some of the PSAs is Max LaCaria, an 11-year-old from Arizona who has limb-girdle muscular dystrophy.