Potassium channel antibody-associated encephalitis with hypothalamic lesions and intestinal pseudo-obstruction.
A subgroup of limbic encephalitis is associated with antibodies against voltage-gated potassium channels (VGKC), and responds well to immuno-modulating therapies. Anti-VGKC antibodies are also found in Isaacs' syndrome and Morvan's syndrome, both of which are sometimes complicated by thymoma. We describe a 52-years-old man with limbic encephalitis, thymoma, and anti-VGKC antibodies, who presented with autonomic dysfunctions such as severe intestinal pseudo-obstruction, hyperhidrosis and hypertension. Thymectomy and corticosteroid therapy remarkably improved his symptoms. Brain magnetic resonance imaging showed hypothalamic lesions, in addition to the bilateral involvement of the medial temporal lobes. This patient had severe autonomic dysfunctions resembling those of Morvan's syndrome. This case may represent a subgroup of VGKC-antibody associated syndromes with a wide spectrum of symptoms, including Isaacs' syndrome, Morvan's syndrome, and limbic encephalitis.
Sekiguchi
Yukari
Y
Department of Neurology, Graduate School of Medicine, Chiba University, 1-8-1 Inohana, Chuo-ku, Chiba-city, Chiba 260-8677, Japan. syukari36@nifty.co.jp
Takahashi
Hirokatsu
H
Mori
Masahiro
M
Ito
Shoichi
S
Shimada
Hitoshi
H
Hattori
Takamichi
T
Kuwabara
Satoshi
S
D000906:Antibodies / Q000097:blood*; D006801:Humans; D007031:Hypothalamus / Q000473:pathology*; D007418:Intestinal Pseudo-Obstruction / Q000150:complications*; D020363:Limbic Encephalitis / Q000150:complications / Q000276:immunology / Q000473:pathology; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D024642:Potassium Channels, Voltage-Gated / Q000276:immunology*; D014057:Tomography, X-Ray Computed / Q000379:methods
D000906:Antibodies; D024642:Potassium Channels, Voltage-Gated