Hypothermia in VGKC antibody-associated limbic encephalitis.
Voltage-gated potassium channel antibody (VGKC-Ab)-associated limbic encephalitis (LE) is a recently described syndrome that broadens the spectrum of immunotherapy-responsive central nervous system disorders. Limbic encephalitis is typically characterised by a sub-acute onset of disorientation, amnesia and seizures, but the clinical spectrum is not yet fully defined and the syndrome could be under-diagnosed. We here describe the clinical profile of four patients with VGKC-Ab-associated LE who had intermittent, episodic hypothermia. One of the patients also described a prodrome of severe neuropathic pain preceding the development of limbic symptoms. Both of these novel symptoms responded well to immunosuppressive therapy, with concurrent amelioration of amnesia/seizures.
Jacob
S
S
Department of Clinical Neurology, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, Headington, Oxford OX3 9DS, UK.
Irani
S R
SR
Rajabally
Y A
YA
Grubneac
A
A
Walters
R J
RJ
Yazaki
M
M
Clover
L
L
Vincent
A
A
D000368:Aged; D001284:Atrophy; D001323:Autoantibodies / Q000097:blood*; D001379:Azathioprine / Q000009:adverse effects / Q000627:therapeutic use; D016572:Cyclosporine / Q000009:adverse effects / Q000627:therapeutic use; D004292:Dominance, Cerebral / Q000502:physiology; D004833:Epilepsy, Temporal Lobe / Q000209:etiology / Q000276:immunology; D005260:Female; D006624:Hippocampus / Q000473:pathology; D006801:Humans; D007031:Hypothalamus / Q000473:pathology; D007035:Hypothermia / Q000209:etiology / Q000276:immunology*; D007116:Immunization, Passive; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use; D020363:Limbic Encephalitis / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D017116:Low Back Pain / Q000276:immunology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D010951:Plasma Exchange; D024642:Potassium Channels, Voltage-Gated / Q000276:immunology*; D011239:Prednisolone / Q000009:adverse effects / Q000627:therapeutic use; D012008:Recurrence; D019233:Retreatment; D013702:Temporal Lobe / Q000473:pathology; D013945:Thymoma / Q000175:diagnosis / Q000276:immunology; D013953:Thymus Neoplasms / Q000175:diagnosis / Q000276:immunology
D001323:Autoantibodies; D007166:Immunosuppressive Agents; D024642:Potassium Channels, Voltage-Gated; D016572:Cyclosporine; D011239:Prednisolone; D001379:Azathioprine