Paraneoplastic limbic encephalitis in two teenage girls.
Paraneoplastic neurological disorders represent remote effects of cancer without invasion of tumor cells into the nervous system. Limbic encephalitis is a distinct entity mostly associated with small-cell carcinoma of the lung. We present the cases of two teenage girls who were admitted with clinical symptoms typical for limbic encephalitis. In the course of the disease, they exhibited characteristic evolutionary changes of brain MRI abnormalities. Onset of neurological symptoms and type of underlying neoplasia were different in both patients. In one girl the initial workup led to the diagnosis of nodular sclerosing Hodgkin disease which so far had not caused any symptoms besides the described neurological abnormalities. A diagnostic brain biopsy showed inflammatory changes and excluded invasion of malignant cells into the central nervous system. The other patient had been diagnosed with a small cell carcinoma of the ovary several months before neurological and brain MRI abnormalities were observed. This is the first report in which clinical picture, evolution of MRI abnormalities, and--in one case--characteristic neuropathological changes are suggestive of paraneoplastic limbic encephalitis in two adolescent girls.
Department of Neuropediatrics, Heinrich-Heine-Universität Düsseldorf, Germany.
T
T
Rosenbaum
J
J
Gärtner
D
D
Körholz
G
G
Janssen
D
D
Schneider
V
V
Engelbrecht
U
U
Göbel
H G
HG
Lenard
D000293:Adolescent; D001921:Brain / Q000473:pathology / Q000503:physiopathology; D018288:Carcinoma, Small Cell / Q000150:complications; D018450:Disease Progression; D004660:Encephalitis / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology; D005260:Female; D006689:Hodgkin Disease / Q000150:complications; D006801:Humans; D008032:Limbic System; D008207:Lymphatic Metastasis; D008279:Magnetic Resonance Imaging; D010051:Ovarian Neoplasms / Q000150:complications; D010257:Paraneoplastic Syndromes / Q000209:etiology* / Q000503:physiopathology; D018458:Persistent Vegetative State / Q000209:etiology