Transient total sleep loss in cerebral Whipple's disease: a longitudinal study.
A case with transient, almost complete sleep loss caused by cerebral manifestation of Whipple's disease (WD) is presented. Cerebral WD is rare and in most cases occurs after gastrointestinal infection. In our case, a progressive and finally almost complete sleep loss was the initial and predominant symptom. Polysomnographic studies in several consecutive nights and over 24 h showed a total abolition of the sleep-wake cycle with nocturnal sleep duration of less than 15 min. Endocrine tests revealed hypothalamic dysfunction with flattening of circadian rhythmicity of cortisol, TSH, growth hormone and melatonin. Cerebrospinal fluid (CSF) hypocretin was reduced. [18F]Deoxyglucose positron emission tomography (FDG-PET) revealed hypermetabolic areas in cortical and subcortical areas including the brainstem, which might explain sleep pathology and vertical gaze palsy. In the course of treatment with antibiotics and additional carbamazepine for 1 year, insomnia slowly and gradually improved. Endocrine investigations at 1-year follow-up showed persistent flattening of circadian rhythmicity. The FDG-PET indicated normalized metabolism in distinct regions of the brain stem which paralleled restoration of sleep length. The extent of sleep disruption in this case of organic insomnia was similar to cases of familial fatal insomnia, but was at least partially reversible with treatment.
Voderholzer
Ulrich
U
Department of Psychiatry and Psychotherapy, University of Freiburg, Freiburg, Germany. ulrich_voderholzer@psyallg.ukl-frieburg.de
Riemann
Dieter
D
Gann
Horst
H
Hornyak
Magdolna
M
Juengling
Freimut
F
Schumacher
Martin
M
Reincke
Martin
M
Von Herbay
Axel
A
Nishino
Seiji
S
Mignot
Emmanuel
E
Berger
Mathias
M
Lieb
Klaus
K
D000328:Adult; D001921:Brain / Q000378:metabolism / Q000503:physiopathology*; D002352:Carrier Proteins / Q000134:cerebrospinal fluid / Q000378:metabolism; D002940:Circadian Rhythm / Q000502:physiology; D004569:Electroencephalography; D005403:Fixation, Ocular / Q000502:physiology; D019788:Fluorodeoxyglucose F18; D005500:Follow-Up Studies; D006801:Humans; D006854:Hydrocortisone / Q000378:metabolism; D007031:Hypothalamus / Q000378:metabolism / Q000503:physiopathology; D047908:Intracellular Signaling Peptides and Proteins; D008297:Male; D008550:Melatonin / Q000378:metabolism; D009479:Neuropeptides / Q000134:cerebrospinal fluid / Q000378:metabolism; D009483:Neuropsychological Tests; D000068797:Orexins; D017286:Polysomnography; D019275:Radiopharmaceuticals; D012892:Sleep Deprivation / Q000150:complications / Q000378:metabolism / Q000503:physiopathology*; D020178:Sleep Disorders, Circadian Rhythm / Q000209:etiology / Q000378:metabolism; D013972:Thyrotropin / Q000378:metabolism; D014055:Tomography, Emission-Computed; D008061:Whipple Disease / Q000378:metabolism / Q000503:physiopathology*
D002352:Carrier Proteins; D047908:Intracellular Signaling Peptides and Proteins; D009479:Neuropeptides; D000068797:Orexins; D019275:Radiopharmaceuticals; D019788:Fluorodeoxyglucose F18; D013972:Thyrotropin; D008550:Melatonin; D006854:Hydrocortisone