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.
Publication types
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Case Reports
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Research Support, Non-U.S. Gov't
MeSH terms
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Aged
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Atrophy
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Autoantibodies / blood*
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Azathioprine / adverse effects
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Azathioprine / therapeutic use
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Cyclosporine / adverse effects
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Cyclosporine / therapeutic use
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Dominance, Cerebral / physiology
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Epilepsy, Temporal Lobe / etiology
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Epilepsy, Temporal Lobe / immunology
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Female
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Hippocampus / pathology
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Humans
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Hypothalamus / pathology
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Hypothermia / etiology
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Hypothermia / immunology*
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Immunization, Passive
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Immunosuppressive Agents / adverse effects
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Immunosuppressive Agents / therapeutic use
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Limbic Encephalitis / diagnosis
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Limbic Encephalitis / drug therapy
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Limbic Encephalitis / immunology*
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Low Back Pain / immunology
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Magnetic Resonance Imaging
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Male
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Middle Aged
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Plasma Exchange
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Potassium Channels, Voltage-Gated / immunology*
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Prednisolone / adverse effects
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Prednisolone / therapeutic use
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Recurrence
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Retreatment
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Temporal Lobe / pathology
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Thymoma / diagnosis
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Thymoma / immunology
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Thymus Neoplasms / diagnosis
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Thymus Neoplasms / immunology
Substances
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Autoantibodies
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Immunosuppressive Agents
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Potassium Channels, Voltage-Gated
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Cyclosporine
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Prednisolone
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Azathioprine