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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.

Original publication

DOI

10.1136/jnnp.2007.130039

Type

Journal article

Journal

J Neurol Neurosurg Psychiatry

Publication Date

02/2008

Volume

79

Pages

202 - 204

Keywords

Aged, Atrophy, Autoantibodies, Azathioprine, Cyclosporine, Dominance, Cerebral, Epilepsy, Temporal Lobe, Female, Hippocampus, Humans, Hypothalamus, Hypothermia, Immunization, Passive, Immunosuppressive Agents, Limbic Encephalitis, Low Back Pain, Magnetic Resonance Imaging, Male, Middle Aged, Plasma Exchange, Potassium Channels, Voltage-Gated, Prednisolone, Recurrence, Retreatment, Temporal Lobe, Thymoma, Thymus Neoplasms