Hypersomnia in Whipple disease: case report.

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.

journal_name

Arq Neuropsiquiatr

authors

Maia LF,Marta M,Lopes V,Rocha N,Lopes C,Martins-da-Silva A,Monteiro L

doi

10.1590/s0004-282x2006000500030

subject

Has Abstract

pub_date

2006-09-01 00:00:00

pages

865-8

issue

3B

eissn

0004-282X

issn

1678-4227

pii

S0004-282X2006000500030

journal_volume

64

pub_type

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