The Lambert-Eaton myasthenic syndrome

Prinses Beatrix FondsPathophysiologie van aanvalsgewijs en chronisch progressief verlopende aandoeningen van het centrale perifere zenuwstelse

From VGKC to LGI1 and Caspr2 encephalitis: The evolution of a disease entity over time

AbstractA wide variety of clinical syndromes has been associated with antibodies to voltage-gated potassium channels (VGKCs). Six years ago, it was discovered that patients do not truly have antibodies to potassium channels, but to associated proteins. This enabled the distinction of three VGKC-positive subgroups: anti-LGI1 patients, anti-Caspr2 patients and VGKC-positive patients lacking both antibodies. Patients with LGI1-antibodies have a limbic encephalitis, often with hyponatremia, and about half of the patients have typical faciobrachial dystonic seizures. Caspr2-antibodies cause a more variable syndrome of peripheral or central nervous system symptoms, almost exclusively affecting older males. Immunotherapy seems to be beneficial in patients with antibodies to LGI1 or Caspr2, stressing the need for early diagnosis. Half of the VGKC-positive patients lack antibodies to both LGI1 and Caspr2. This is a heterogeneous group of patients with a wide variety of clinical syndromes, raising the question whether VGKC-positivity is truly a marker of disease in these patients. Data regarding this issue are limited, but a recent study did not show any clinical relevance of VGKC-positivity in the absence of antibodies to LGI1 and Caspr2. The three VGKC-positive subgroups are essentially different, therefore, the lumping term ‘VGKC-complex antibodies’ should be abolished

From VGKC to LGI1 and Caspr2 encephalitis: The evolution of a disease entity over time

A wide variety of clinical syndromes has been associated with antibodies to voltage-gated potassium channels (VGKCs). Six years ago, it was discovered that patients do not truly have antibodies to potassium channels, but to associated proteins. This enabled the distinction of three VGKC-positive subgroups: anti-LGI1 patients, anti-Caspr2 patients and VGKC-positive patients lacking both antibodies. Patients with LGI1-antibodies have a limbic encephalitis, often with hyponatremia, and about half of the patients have typical faciobrachial dystonic seizures. Caspr2-antibodies cause a more variable syndrome of peripheral or central nervous system symptoms, almost exclusively affecting older males. Immunotherapy seems to be beneficial in patients with antibodies to LGI1 or Caspr2, stressing the need for early diagnosis. Half of the VGKC-positive patients lack antibodies to both LGI1 and Caspr2. This is a heterogeneous group of patients with a wide variety of clinical syndromes, raising the question whether VGKC-positivity is truly a marker of disease in these patients. Data regarding this issue are limited, but a recent study did not show any clinical relevance of VGKC-positivity in the absence of antibodies to LGI1 and Caspr2. The three VGKC-positive subgroups are essentially different, therefore, the lumping term ‘VGKC-complex antibodies’ should be abolished

Paraneoplastic Syndromes of the Neuromuscular Junction: Therapeutic Options in Myasthenia Gravis, Lambert-Eaton Myasthenic Syndrome, and Neuromyotonia

Neurological Motor Disorder

3,4-diaminopyridine for the treatment of Lambert-Eaton myasthenic syndrome

The Lambert Eaton myasthenic syndrome (LEMS) is an autoimmune disease in which antibodies against voltage-gated calcium channels inhibit cholinergic neurotransmission LEMS is clinically characterized by muscle weakness and autonomic dysfunction 3 4-diaminopyridine (3 4-DAP) blocks potassium channels in nerve terminals resulting in an increase in acetylcholine release This article describes the four randomized placebo-controlled trials of 3 4-DAP in patients with LEMS All trials demonstrated a significant effect on muscle strength and compound muscle action potential amplitude Furthermore the safety and tolerability of 3 4-DAP are reviewed The side effects of 3 4-DAP are generally mild and most frequently consist of paresthesias but epileptic seizures and arrhythmias have been described in patients using high doses Given the efficacy and safety of 3 4-DAP in LEMS, this drug is the mainstay for symptomatic treatment of LEMSStress-related psychiatric disorders across the life spa

Treatment options for Lambert-Eaton myasthenic syndrome

Neurological Motor Disorder

Paraparesis following lumbar puncture in a male with leukemia.

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