Presence of voltage-gated potassium channel complex antibody in a case of genetic prion disease.
BMJ Case Rep
; 20142014 Jun 05.
Article
in En
| MEDLINE
| ID: mdl-24903967
ABSTRACT
Voltage-gated potassium channel (VGKC) complex antibody-mediated encephalitis is a recently recognised entity which has been reported to mimic the clinical presentation of Creutzfeldt-Jakob disease (CJD). Testing for the presence of this neuronal surface autoantibody in patients presenting with subacute encephalopathy is therefore crucial as it may both revoke the bleak diagnosis of prion disease and allow institution of potentially life-saving immunotherapy. Tempering this optimistic view is the rare instance when a positive VGKC complex antibody titre occurs in a definite case of prion disease. We present a pathologically and genetically confirmed case of CJD with elevated serum VGKC complex antibody titres. This case highlights the importance of interpreting the result of a positive VGKC complex antibody with caution and in the context of the overall clinical manifestation.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Autoantibodies
/
Prion Diseases
/
Potassium Channels, Voltage-Gated
/
Dementia
Type of study:
Diagnostic_studies
Limits:
Aged
/
Female
/
Humans
Language:
En
Journal:
BMJ Case Rep
Year:
2014
Document type:
Article
Affiliation country:
Estados Unidos