TABLE 1.
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].