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Isaacs Syndrome (Neuromyotonia)

A rare neuromuscular disorder with onset usually in late childhood or early adulthood, characterized by intermittent or continuous widespread involuntary muscle contractions; FASCICULATION; hyporeflexia; MUSCLE CRAMP; MUSCLE WEAKNESS; HYPERHIDROSIS; TACHYCARDIA; and MYOKYMIA. Involvement of pharyngeal or laryngeal muscles may interfere with speech and breathing. The continuous motor activity persists during sleep and general anesthesia (distinguishing this condition from STIFF-PERSON SYNDROME). Familial and acquired (primarily autoimmune) forms have been reported. (From Ann NY Acad Sci 1998 May 13;841:482-496; Adams et al., Principles of Neurology, 6th ed, p1491)
Also Known As:
Neuromyotonia; Isaacs-Mertens Syndrome; Pseudomyotonia; Acquired Neuromyotonia; Continuous Muscle Activity Syndrome; Gamstorp-Wohlfart Syndrome; Isaacs' Syndrome; Myokymia, Myotonia, Muscle Wasting, And Hyperhidrosis; Pseudomyotonia Syndrome of Isaacs; Quantal Squander; Syndrome of Continuous Muscle Activity; Continuous Myokymia; Continuous Myokymias; Gamstorp Wohlfart Syndrome; Isaac Syndrome; Isaacs Mertens Syndrome; Isaacs Pseudomyotonia Syndrome; Myokymias, Continuous; Neuromyotonia, Acquired; Myokymia, Continuous
Networked: 305 relevant articles (18 outcomes, 10 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Isaacs Syndrome (Neuromyotonia)
2. Thymoma (Thymic Carcinoma)
3. Glioma (Gliomas)
4. Myasthenia Gravis
5. Sleep Initiation and Maintenance Disorders (Insomnia)

Experts

1. Vincent, Angela: 18 articles (01/2020 - 08/2002)
2. Watanabe, Osamu: 12 articles (12/2018 - 01/2002)
3. Vincent, A: 7 articles (01/2019 - 03/2000)
4. Arimura, Kimiyoshi: 7 articles (12/2018 - 01/2002)
5. Lancaster, Eric: 6 articles (07/2022 - 08/2010)
6. Dalmau, Josep: 6 articles (01/2018 - 08/2010)
7. Irani, Sarosh R: 5 articles (10/2020 - 09/2010)
8. Waters, Patrick: 5 articles (10/2020 - 09/2010)
9. Buckley, Camilla: 5 articles (12/2019 - 09/2003)
10. Lang, Bethan: 5 articles (12/2018 - 03/2003)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Isaacs Syndrome:
1. AntibodiesIBA
2. Carbamazepine (Tegretol)FDA LinkGeneric
3. Intravenous Immunoglobulins (IVIG)FDA Link
4. SteroidsIBA
5. MethylprednisoloneFDA LinkGeneric
6. Voltage-Gated Potassium Channels (Voltage-Gated Potassium Channel)IBA
7. Proteins (Proteins, Gene)FDA Link
8. AutoantibodiesIBA
9. Leucine (L-Leucine)FDA Link
09/01/2010 - "Of the 19 patients with contactin-associated protein-antibody-2, 10 had neuromyotonia or Morvan's syndrome, compared with only 3 of the 55 leucine-rich, glioma inactivated 1 protein-antibody positive patients (P < 0.0001), who predominantly had limbic encephalitis. "
06/01/2023 - "Leucine-rich Glioma-inactivated 1 Encephalitis Followed by Isaacs Syndrome: Alternating Presence of Pathogenic Autoantibodies to Leucine-rich Glioma-inactivated 1 and Contactin-associated Protein-like 2."
01/01/2022 - "Six months after thymectomy, his nephrotic syndrome and MG symptoms were relieved; however, he presented with typical manifestations of Morvan's syndrome, including neuromyotonia, severe insomnia, abnormal ECG activity, and antibodies against leucine-rich glioma-inactivated 1 (LGI1) and γ-amino-butyric acid-B receptor (GABABR). "
10/01/2020 - "Autoantibodies to leucine-rich glioma-inactivated 1 (LGI1) and contactin-associated protein like-2 (CASPR2) are associated with clinically distinctive syndromes that are highly immunotherapy responsive, such as limbic encephalitis, faciobrachial dystonic seizures, Morvan's syndrome and neuromyotonia. "
07/16/2022 - "Neuromyotonia is a rare peripheral nerve hyperexcitability syndrome often associated with antibodies directed against contactin-associated protein-like 2 and leucine-rich, glioma inactivated 1. The quadrivalent human papilloma virus vaccine Gardasil®, first approved in 2006, is known to be a highly effective prophylaxis against papillomavirus types 6, 11, 16, and 18. "
10. MercuryIBA

Therapies and Procedures

1. Plasmapheresis
2. Radiotherapy
3. Thymectomy
4. Cytoreduction Surgical Procedures
5. Analgesia