VGKC positive autoimmune encephalopathy mimicking dementia.
Voltage gated potassium channel antibodies (VGKC Abs) are known to cause three rare neurological syndromes- neuromyotonia, Morvan's syndrome and limbic encephalitis although an increasing array of other associated neurological symptoms are becoming recognised. The authors describe the case of a 60-year-old female who presented to the neurology clinic with an apparent early onset dementing process. She was noted to have both extrapyramidal and frontal release signs on examination and was admitted for further evaluation. Her dementia investigation including a neoplastic screen was negative except for VGKC antibody positivity. Her symptoms dramatically improved with commencement of immunosuppression. A non-paraneoplastic VGKC antibody associated dementia-like syndrome has rarely been described. The authors add to the few existing reports of what represents an important reversible cause of cognitive impairment.
Neurology Department, Cork University Hospital, Cork, Ireland. annamolusa@gmail.com
Anna
A
Molloy
Eugene
E
Cassidy
Aisling
A
Ryan
Orna
O
O' Toole
D000906:Antibodies / Q000097:blood*; D001327:Autoimmune Diseases / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D001379:Azathioprine / Q000627:therapeutic use; D001714:Bipolar Disorder / Q000150:complications / Q000188:drug therapy; D001927:Brain Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology*; D003704:Dementia / Q000175:diagnosis; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008875:Middle Aged; D024642:Potassium Channels, Voltage-Gated / Q000276:immunology*
D000906:Antibodies; D007166:Immunosuppressive Agents; D024642:Potassium Channels, Voltage-Gated; D001379:Azathioprine