Hypersomnia in Whipple disease: case report.

• Main Author: MAIA, L.
• Publication Date: 2006
• Other Authors: MARTA, M., LOPES, V., ROCHA, N., LOPES, C., MARTINS‐DA‐SILVA, A., MONTEIRO, L.
• Format: Article
• Language: eng
• Source: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
• Download full: http://hdl.handle.net/10400.16/839

Arq Neuropsiquiatr. 2006 Sep;64(3B):865-8. Hypersomnia in Whipple disease: case report. Maia LF, Marta M, Lopes V, Rocha N, Lopes C, Martins-da-Silva A, Monteiro L. SourceDepartment of Neurological Disordes and Senses, Hospital Geral de Santo António, Largo Prof. Abel Salazar, 4099-001 Porto, Portugal. Abstract Whipple disease (WD) is a rare systemic infection caused by Tropheryma whippelii. Neurological involvement has been recognised in 40% of patients, either as initial manifestations or during the course of the disease. We report on a 45 years-old man with WD with initial, persistent and irresistible episodes of daytime somnolence. The patient was HLA-DQB1*0602 positive (genetic marker for narcolepsy). WD diagnosis was suspected on clinical and MRI basis and confirmed by histological and immunohistochemical study of duodenal biopsy. Forty months later all clinical features improved, narcoleptic-like episodes disappeared and cerebral MRI and CSF normalised. Longitudinal neurophysiological studies revealed persistent sleep pattern abnormalities with sleep fragmentation, paucity of slow wave and of REM sleep. The disruption of the hypocretin circuitry in the hypothalamic - diencephalic region triggered by the infection was the probable cause of the hypersomnia and narcopleptic symptoms. WD should be added to the list of causes of secondary hypersomnia.