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J Neurol Neurosurg Psychiatry ; 79(2): 202-4, 2008 Feb.
Article in English | MEDLINE | ID: mdl-18202210

ABSTRACT

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.


Subject(s)
Autoantibodies/blood , Hypothermia/immunology , Limbic Encephalitis/immunology , Potassium Channels, Voltage-Gated/immunology , Aged , Atrophy , Azathioprine/adverse effects , Azathioprine/therapeutic use , Cyclosporine/adverse effects , Cyclosporine/therapeutic use , Dominance, Cerebral/physiology , Epilepsy, Temporal Lobe/etiology , Epilepsy, Temporal Lobe/immunology , Female , Hippocampus/pathology , Humans , Hypothalamus/pathology , Hypothermia/etiology , Immunization, Passive , Immunosuppressive Agents/adverse effects , Immunosuppressive Agents/therapeutic use , Limbic Encephalitis/diagnosis , Limbic Encephalitis/drug therapy , Low Back Pain/immunology , Magnetic Resonance Imaging , Male , Middle Aged , Plasma Exchange , Prednisolone/adverse effects , Prednisolone/therapeutic use , Recurrence , Retreatment , Temporal Lobe/pathology , Thymoma/diagnosis , Thymoma/immunology , Thymus Neoplasms/diagnosis , Thymus Neoplasms/immunology
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