An unusual presentation of anti-Hu-associated paraneoplastic limbic encephalitis.
Paraneoplastic limbic encephalitis is a rare disorder characterized by personality changes, seizures, memory loss, and psychiatric symptoms often associated with antineuronal antibodies. It is well described in the adult literature but is still underreported in the pediatric literature. Symptoms are usually multifocal and subacute in presentation, occurring over days to weeks; however, in rare cases, symptom onset can be more gradual. We report the case of a 9-year-old male with anti-Hu-associated paraneoplastic limbic encephalitis that presented as episodic ataxia and behavioral changes evolving to intractable epilepsy and worsening behavioral changes over the course of a year. This case highlights the importance of considering a paraneoplastic disorder in the differential diagnosis for unexplained multifocal neurological symptoms of subacute or chronic onset as earlier detection and treatment may result in an improved outcome.
Department of Neurology, University of Virginia Health System, Charlottesville, VA 22908-0394, USA.
Jennifer E
JE
Langer
M Beatriz S
MB
Lopes
Nathan B
NB
Fountain
Michael R
MR
Pranzatelli
Elizabeth A
EA
Thiele
Robert S
RS
Rust
Howard P
HP
Goodkin
D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001323:Autoantibodies / Q000097:blood*; D002648:Child; D003907:Dexamethasone / Q000627:therapeutic use; D004334:Drug Administration Schedule; D004359:Drug Therapy, Combination; D051959:ELAV Proteins / Q000276:immunology*; D004833:Epilepsy, Temporal Lobe / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology; D006801:Humans; D016130:Immunophenotyping; D007262:Infusions, Intravenous; D020363:Limbic Encephalitis / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D008279:Magnetic Resonance Imaging; D008297:Male; D000069283:Rituximab; D013702:Temporal Lobe / Q000473:pathology
D000893:Anti-Inflammatory Agents; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D001323:Autoantibodies; D051959:ELAV Proteins; D000069283:Rituximab; D003907:Dexamethasone