TABLE 1.

Diagnostic guidelines for CNS Whipple's disease^*

Definite CNS WD

Must have 1 of the following 3 criteria:

Oculomasticatory myorhythmia (OMM) or oculofacial skeletal myorhythmia (OFSM)

Positive tissue biopsy

Positive PCR analysis

Possible CNS WD

Must have 1 of 4 systemic symptoms, including:

Fever of unknown origin

Gastrointestinal symptoms (steatorrhea, chronic diarrhea, abdominal distention, pain)

Chronic migratory arthralgias or polyarthralgias

Unexplained lymphadenopathy, night sweats, or malaise

Must also have 1 of 4 unexplained neurological signs, including:

Supranuclear vertical gaze palsy

Rhythmic myoclonus

Dementia with psychiatric symptoms

Hypothalamic manifestations

^*Adapted from [20].