RESUMO
INTRODUCTION: Isolated Whipple disease of the central nervous system is a rare occurrence. Migratory arthralgias and gastrointestinal problems, including malabsorption, abdominal pain, diarrhea, and weight loss, are common presenting symptoms. DISCUSSION: For those patients with systemic signs and symptoms of Whipple disease, 6% to 43% will have clinically manifested CNS involvement that may include alterations in personality, ataxia, and dementia. We report our experience with a patient, who was successfully treated for Whipple disease 12 years prior to presentation and had a magnetic resonance image of the brain that revealed two solitary lesions resembling a tumor upon presentation.
Assuntos
Erros de Diagnóstico/prevenção & controle , Encefalite/microbiologia , Encefalite/patologia , Lobo Temporal/microbiologia , Lobo Temporal/patologia , Doença de Whipple/patologia , Anti-Infecciosos/uso terapêutico , Neoplasias Encefálicas/diagnóstico , Doença Crônica/terapia , Transtornos da Consciência/etiologia , Diagnóstico Diferencial , Encefalite/cirurgia , Cefaleia/etiologia , Humanos , Hipotálamo/microbiologia , Hipotálamo/patologia , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Procedimentos Neurocirúrgicos , Lobo Temporal/cirurgia , Resultado do Tratamento , Combinação Trimetoprima e Sulfametoxazol/uso terapêutico , Tropheryma/fisiologia , Doença de Whipple/fisiopatologia , Doença de Whipple/cirurgiaRESUMO
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.