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Diagnostic Guidelines in Central Nervous System Whipple's Disease
Ann Neurol 40:561-568, Louis,E.D.,et al, 1996
See this aricle in Pubmed

Article Abstract
Many cases of central nervous system(CNS)Whipple's disease are not diagnosed until postmortem.Few reviews of CNS Whipple's disease have delineated the frequencies of abnormalities on neurological examination, cerebrospinal fluid studies,neuroimaging,and intestinal biopsy studies. Guidelines for diagnosis and treatment have not been proposed.In this review we present 3 new cases of CNS Whipple's disease and summarize the literature to determine the frequencies of neurological signs and abnormalities on diagnostic testing.We propose guidelines for diagnostic screening,selection for biopsy,and treatment.Review of the 84 cases of CNS Whipple's disease(81 in the literature,3 new)revealed that 80%of the patients had systemic signs.Cognitive changes were frequent(71%),and 47% with cognitive changes also had psychiatric signs.Oculomasticatory myorhythmia and oculo-facial-skeletal myorhythmia,pathognomic for CNS Whipple's disease,were present in 20%of patients and were always accompanied by a supranuclear vertical gaze palsy.Tissue biopsy was a sensitive technique;89%of those who had biopsies had positive biopsy results.Diagnosis and treatment of definite CNS Whipple's disease should be based on the presence of pathognomic signs(oculomasticatory myorhythmia or oculo-facial-skeletal myorhythmia)or positive biopsy or polymerase chain reaction results.Possible CNS Whipple's disease should be diagnosed in the setting of unexplained systemic symptoms and neurologic signs (supranuclear vertical gaze palsy,rhythmic myoclonus,dementia with psychiatric symptoms,or hypothalamic manifestations).Those with possible CNS Whipple's disease should undergo small-bowel biopsy.
 
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abdominal distention
arthralgia
ataxia
bacterial infection
brain biopsy
cerebrospinal fluid,abnormal
dementia
diarrhea
facial movement disorder
fever
gaze palsy
gaze palsy,supranuclear
hypothalamus,disturbance of
intellectual deficit
intestinal biopsy
level of consciousness,decreased
lymphadenopathy
malaise
myoclonus
neurologic disease,diagnoses of
night sweats
oculomasticatory myorhythmia
ophthalmoplegia
pain,abdominal
PAS positive
polymerase chain reaction
psychiatric disorder
psychiatric problems in neurologic disorders
review article
seizure
steatorrhea
weight loss
Whipple's disease

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