Rasmussen's syndrome and new-onset narcolepsy, cataplexy, and epilepsy in an adult.
We report a case of new-onset seizures and narcolepsy in a previously healthy 40-year-old man. He developed severe daytime somnolence and cataplexy over the course of a few months. Brain MRI was normal, and polysomnography with multiple sleep latency testing confirmed a diagnosis of narcolepsy. His HLA haplotype is DQB1*0602 and cerebrospinal fluid analysis showed no detectable hypocretin. Approximately 18 months later, he developed complex partial seizures. Further MRI showed a progressively enlarging lesion involving the left frontotemporal and insular areas. Pathology from a partial resection was consistent with Rasmussen's syndrome. Evaluation for tumor, infectious, and paraneoplastic etiologies was negative. There was no further progression of the residual lesion on serial MRI. Although the pathophysiologic bases of narcolepsy and Rasmussen's syndrome are unknown, they may have an autoimmune basis. This unique case of both disorders in a single patient suggests the possibility of a common underlying disease process.
Lagrange
A H
AH
Department of Neurology, Vanderbilt University, 465 21st Avenue S, 6144 MRB III, 37232-8552, Nashville, TN, USA. andre.h.lagrange@vanderbilt.edu
Blaivas
M
M
Gomez-Hassan
D
D
Malow
B A
BA
D000328:Adult; D001931:Brain Mapping; D002352:Carrier Proteins / Q000134:cerebrospinal fluid; D004569:Electroencephalography; D004660:Encephalitis / Q000150:complications* / Q000473:pathology; D004827:Epilepsy / Q000175:diagnosis / Q000209:etiology*; D005911:Gliosis; D006801:Humans; D047908:Intracellular Signaling Peptides and Proteins; D008214:Lymphocytes / Q000473:pathology; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D009290:Narcolepsy / Q000175:diagnosis / Q000209:etiology*; D009460:Neurologic Examination; D009479:Neuropeptides / Q000134:cerebrospinal fluid; D000068797:Orexins; D017286:Polysomnography / Q000379:methods; D012895:Sleep, REM; D013702:Temporal Lobe / Q000166:cytology / Q000473:pathology
D002352:Carrier Proteins; D047908:Intracellular Signaling Peptides and Proteins; D009479:Neuropeptides; D000068797:Orexins