KEGG   DISEASE: Isaacs syndrome
Entry
H01804                      Disease                                
Name
Isaacs syndrome
Description
Isaacs syndrome is a rare neuromuscular disorder of continuous muscle fibre activity resulting from peripheral nerve hyper excitability. Symptoms commonly include myokymia, pseudomyotonia, muscle cramps and stiffness. It is caused by voltage-gated potassium channel dysfunction and may be inherited or acquired. Recent evidences suggest that autoantibodies against voltage-gated potassium channels (VGKC-Abs) are associated with this disease. Treatment commonly includes anticonvulsants, immunosuppressive therapy and plasma exchange.
Category
Nervous system disease; Musculoskeletal disease
Brite
Human diseases in ICD-11 classification [BR:br08403]
 08 Diseases of the nervous system
  Diseases of neuromuscular junction or muscle
   Primary disorders of muscles
    8C71  Myotonic disorders
     H01804  Isaacs syndrome
Other DBs
ICD-11: 8C71.4
ICD-10: G71.1
MeSH: D020386
Reference
  Authors
Lide B, Singh J, Haeri S
  Title
Isaacs' syndrome in pregnancy.
  Journal
BMJ Case Rep 2014:bcr2014206704 (2014)
DOI:10.1136/bcr-2014-206704
Reference
  Authors
Takahashi H, Mori M, Sekiguchi Y, Misawa S, Sawai S, Hattori T, Kuwabara S
  Title
Development of Isaacs' syndrome following complete recovery of voltage-gated potassium channel antibody-associated limbic encephalitis.
  Journal
J Neurol Sci 275:185-7 (2008)
DOI:10.1016/j.jns.2008.07.034
Reference
  Authors
Meyniel C, Ollivier Y, Hamidou M, Pereon Y, Derkinderen P
  Title
Dramatic improvement of refractory Isaacs' syndrome after treatment with dronabinol.
  Journal
Clin Neurol Neurosurg 113:323-4 (2011)
DOI:10.1016/j.clineuro.2010.11.006
Reference
  Authors
Panzer J, Dalmau J
  Title
Movement disorders in paraneoplastic and autoimmune disease.
  Journal
Curr Opin Neurol 24:346-53 (2011)
DOI:10.1097/WCO.0b013e328347b307
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