myasthenic syndrome

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.

Synonym(s): carcinomatous myopathy, Eaton-Lambert syndrome, Lambert syndrome, Lambert-Eaton syndrome, 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 ?

Myopathic EMG findings and type II muscle fiber atrophy in patients with Lambert-Eaton myasthenic syndrome.

Lambert-Eaton Myasthenic Syndrome with A Twenty-Three-Year Delay in Diagnosis

Diagnosis and treatment of Lambert-Eaton myasthenic syndrome.

Paraneoplastic rheumatic disorders / Paraneoplastik romatizmal hastaliklar

The BioMarin medicine FirDapse - currently licensed for use in patients with Lambert-Eaton Myasthenic Syndrome - costs between pounds 40,000 and pounds 70,000 per patient a year, according to the Muscular Dystrophy Campaign.

Family hit by spiralling cost of new life-saving drugs; MUCH CHEAPER TABLETS MAY BE FORCED OFF THE SHELVES

The presence of a positive family history for autosomal dominant inheritance of a myasthenic syndrome should strongly suggest a SCS.

Decoding pathogenesis of slow-channel congenital myasthenic syndromes using recombinant expression and mice models

LG2 agrin mutation causing severe congenital myasthenic syndrome mimics functional characteristics of non-neural (z-) agrin.

A Novel AGRN Mutation Leads to Congenital Myasthenic Syndrome Only Affecting Limb-girdle Muscle

It was reported yesterday that the agreement has been reached under a Special Protocol Assessment for the protocol design, clinical endpoints, and statistical analysis approach to be taken in the company's upcoming Phase three study evaluating Firdapse (amifampridine phosphate) for the symptomatic treatment of Lambert-Eaton myasthenic syndrome (LEMS).

Catalyst Pharmaceuticals reaches agreement with US FDA for Special Protocol Assessment for Firdapse

And children such as five-year-old Alexander Frizelle, also of Cramlington, who was born with Congenital Myasthenic Syndrome.

Stars shining through adversity; IT'S an event that truly lives up to its name. The Children's Foundation Star Awards recognises the achievements of North East children with a disability, their friends and families and the challenges they face. Ken Oxley joined the celebrations

Family flowers only, but donations in lieu of flowers will be gratefully received towards Lambert and Eaton Myasthenic Syndrome and Cybi Ward, Ysbyty Gwynedd through the Funeral Directors Pritchard a Griffiths Cyf, Dublin Street, Tremadog.

Deaths

Two-year-old Isabella Shotton suffers from the incredibly rare condition Congenital Myasthenic Syndrome (CMS), a terrifying illness that means she could stop breathing at any moment.

Slimmers shedding stones to win pounds for toddler

Congenital insensitivity to pain with anhidrosis associated with congenital myasthenic syndrome.

Hypertelorism in Charcot-Marie-Tooth disease 1A from the common PMP22 duplication: a case report

He had congenital myasthenic syndrome, a rare condition that severely limits the ability to breathe and move limbs.

'Sadly, further treatment is not in RB's interest. It's best to let our dear boy die with dignity' DAD ENDS RIGHT-TO-LIFE FIGHT

Baby RB was born in October last year with a form of congenital myasthenic syndrome (CMS), a rare neuromuscular condition which severely limits the ability to breathe and move limbs.