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Časopis Neurology – Článok Amifampridine – an „orphan therapy“ in the treatment of Lambert-Eaton myasthenic syndrome: results of four years’ experience in two patients

Neurology

Reviewed, postgraduate scietific medical journal.
Period 3x per year
1336-8621
The journal is indexed in the Slovak National Bibliography, Bibliographiia Medica Slovaca (BMS) and listed to citation database CiBaMed. All articles are reviewed. The publisher does not bear any responsibility for data and opinions of particular authors of the articles or advertisements. The articles on grey pages are company promotions or non reviewed information, an author is responsible for the content. Any reproduction of the content is allowed only with direct consent of the editorial office.
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Neurology
Neurology
Reviewed, postgraduate scietific medical journal.
Period 3x per year
Predplatné
Téma: Original works

Amifampridine - an „orphan therapy“ in the treatment of Lambert-Eaton myasthenic syndrome: results of four years’ experience in two patients

Peter Špalek, František Cibulčík, Angela Vincent

Lambert-Eaton myasthenic syndrome (LEMS) is a rare but debilitating autoimmune neuromuscular junction disorder. Muscle weakness in LEMS is caused by autoantibodies against presynaptic voltage-gated P/Q calcium channels (VGCCs). This decreases calcium entry into the presynaptic terminal, which prevents binding of acetylcholine vesicles to presynaptic membrane and inhibits acetylcholine release. Over 80% of patients are aged ˃ 40 years at the onset of symptoms. LEMS occurs in about 50% patients as a paraneoplastic disorder most often associated with small cell lung cancer. Diagnosis of LEMS is frequently missed or delayed. Symptoms usually begin insidiously and many patients go for months or even years before a diagnosis is made. The most common initial complaint is proximal muscle weakness involving the lower extremities more than the upper extremities. Muscle weakness has a substantial impact on patients’ ability to lead a normal life, affecting their ability to walk, climb stairs and get up from a chair. Absent or depressed tendon reflexes and autonomic dysfunction are frequently present. Involvement of bulbar and respiratory muscles is rare. Clinical presentation of LEMS can resemble myasthenia gravis, spinal amyotrophy, limb-girdle muscular dystrophies and other myopathies. Diagnosis is confirmed by electrophysiological testing, which demonstrates a marked (˃70%) increment in compound motor action potential amplitude with 30-Hz repetitive stimulation, and by serological test for VGCCs autoantibodies. In LEMS immunotherapy (prednisone, immunosuppressants, plasmapheresis, intravenous immunoglobulin) has a key role in the treatment. However, some LEMS patients do not respond sufficiently to immunotherapy and have long-lasting debilitating symptomatology. A new orphan drug amifampridine was recently authorised by Europen Medical Agency for the European Union countries. Amifampridine is recommended as the only first-line symptomatic treatment for LEMS. Amifampridine blocks voltage-dependent potassium channels, thereby prolonging presynaptic cell membrane depolarisation. Prolonging the action potential enhances the transport of calcium into the nerve terminal and consequently acetylcholine release, thus relieving the LEMS symptoms. We report our four years’ experience with amifampridine treatment in two Slovak LEMS patients. The clinical symptomatology improved markedly in both patients and there are no side effects.

LEMS, imunopathogenesis, diagnosis, immunotherapy, amifampridine
Neurológia 2019; 14 (1): 5-11

Ročník 2019  Témy časopisu Neurology 1 / 2019

Original works

Peter Špalek, František Cibulčík, Angela Vincent
Daša Viszlayová, David Školoudík, Miroslav Brozman, Kateřina Langová, Roman Herzig, Lukáš Pátrovič, Silvia Királová
René Opšenák, Branislav Kolarovszki, Pavol Snopko, Martin Hanko, Kristián Varga

Overview works

Ivan Martinka, Peter Špalek
Ema Kantorová, Ján Grossmann, Kryštof Kantor, Štefan Sivák, Robert Ružinák, Slavomíra Kováčová, Egon Kurča
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The journal is indexed in the Slovak National Bibliography, Bibliographiia Medica Slovaca (BMS) and listed to citation database CiBaMed. All articles are reviewed. The publisher does not bear any responsibility for data and opinions of particular authors of the articles or advertisements. The articles on grey pages are company promotions or non reviewed information, an author is responsible for the content. Any reproduction of the content is allowed only with direct consent of the editorial office.
Predplatné
Neurology
Neurology
Reviewed, postgraduate scietific medical journal.
Period 3x per year
Predplatné