Hypersomnia in Whipple disease: case report

Arquivos de Neuro-Psiquiatria

Luís F. Maia²  Mónica Marta²  Vitor Lopes¹  Nelson Rocha¹  Carlos Lopes¹  Antônio Martins-da-silva²  Luís Monteiro² 

[1] ,Hospital Geral de Santo António Department of Neurological Disorders and Senses Porto,Portugal

关键词: Whipple disease;    hypersomnia;    multiple sleep latency test;    HLA (DQB1*0602);    doença de Whipple;    hipersónia;    teste de latências múltiplas;    HLA (DQB1*0602);   

DOI  :  10.1590/S0004-282X2006000500030

来源: SciELO

【 摘 要 】

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.

【 授权许可】

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