myasthenic syndrome


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Lam·bert-Ea·ton myasthenic syn·drome (LEMS),

(lam'bert ē'tŏn),
a generalized disorder of neuromuscular transmission caused by a defect in the release of acetylcholine quanta from the presynaptic nerve terminals; often associated with small cell carcinoma of the lung, particularly in elderly men with a long history of cigarette smoking. In contrast to myasthenia gravis, weakness tends to affect solely axial muscles, girdle muscles, and less often the limb muscles; autonomic disturbances, for example, dry mouth and impotence, are common; the deep tendon reflexes are unelicitable; on motor conduction studies, responses on initial stimulation are quite low in amplitude, but they show marked posttetanic facilitation after a few seconds of exercise. Lambert-Eaton syndrome is due to loss of voltage-sensitive calcium channels located on the presynaptic motor nerve terminal. See: myasthenic syndrome.

myasthenic syndrome

Lambert-Eaton syndrome, see there.

my·as·then·ic syn·drome

(mī-as-then'ik sin'drōm)
A disorder of neuromuscular transmission marked primarily by limb and girdle weakness, absent deep tendon reflexes, dry mouth, and impotence; due to an immunologic disorder; often, especially in males, a paraneoplastic syndrome linked to small cell carcinoma of the lung.
References in periodicals archive ?
The gene, GFPT1, is crucial in causing a variation of Congenital Myasthenic Syndrome which gained media attention recently with the plight of one-year-old baby RB, who was at the centre of a "right-to-life" legal dispute.
In particular the Complaint asserts that Catalyst failed to disclose during the Class Period that one of its competitors has already been manufacturing a drug biologically equivalent to Firedapse-a drug Catalyst has claimed to be developing and marketing-and providing it to patients to treat Lambert-Eaton Myasthenic Syndrome free of charge, through a compassionate use program.
Called Congenital Myasthenic Syndrome, the incredibly rare disorder affects only around 20 families in the country and parents are often told to prepare for the worst.
The tot has congenital myasthenic syndrome - a rare disorder that means his muscles are too weak for him to move effectively or breathe unaided.
Catalyst) announced that the independent Data Monitoring Committee (DMC) overseeing Catalyst's ongoing pivotal Phase III clinical trial in the US and Europe that evaluated Firdapse for the treatment of Lambert-Eaton Myasthenic Syndrome (LEMS) has recommended continuation of the trial by Catalyst, as planned, based on the committee's review of safety and clinical data from the trial.
Baby RB was born with congenital myasthenic syndrome (CMS), a rare neuromuscular condition which severely limits the ability to breathe and move limbs.
BioMarin: Firdapse - Lambert-Eaton myasthenic syndrome - Firdapse Phase III results - H2 2012
Approved products include Naglazyme[R] (galsulfase) for mucopolysaccharidosis VI (MPS VI), a product wholly developed and commercialized by BioMarin; Aldurazyme[R] (laronidase) for mucopolysaccharidosis I (MPS I), a product which BioMarin developed through a 50/50 joint venture with Genzyme Corporation; Kuvan[R] (sapropterin dihydrochloride) Tablets, for phenylketonuria (PKU), developed in partnership with Merck Serono, a division of Merck KGaA of Darmstadt, Germany; and Firdapse[TM] (amifampridine), which has been approved by the European Commission for the treatment of Lambert Eaton Myasthenic Syndrome (LEMS).
Approved products include Naglazyme (galsulfase) for mucopolysaccharidosis VI (MPS VI), a product wholly developed and commercialized by BioMarin; Aldurazyme (laronidase) for mucopolysaccharidosis I (MPS I), a product which BioMarin developed through a 50/50 joint venture with Genzyme Corporation; Kuvan (sapropterin dihydrochloride) Tablets, for phenylketonuria (PKU), developed in partnership with Merck Serono, a division of Merck KGaA of Darmstadt, Germany; and Firdapse(TM) (amifampridine), which has been approved by the European Commission for the treatment of Lambert Eaton Myasthenic Syndrome (LEMS).
Among specific topics are histological and histochemical stains and reactions, how to read a biopsy, muscular distrophies and allied disorders, congenital myopathies and related disorders, myopathies associated with systemic disorders and aging, myasthenic syndromes, and toxic and drug-induced myopathies.
Subjects with probable congenital myasthenic syndromes, i.