Zajicek et al proposed criteria for the diagnosis of central nervous sarcoidosis (neurosarcoidosis). The authors are from the University of Cambridge, St. George's Hospital, Royal Brompton Hospital and the National Hospital for Neurology and Neurosurgery (London).
Parameters:
(1) clinical presentation
(2) nervous system histology
(3) evidence of CNS inflammation
(4) evidence of systemic sarcoidosis
(5) alternative diagnoses
Evidence of CNS inflammation:
(1) CSF: elevated protein and/or leukocytes
(2) oligoclonal bands: present
(3) MRI: compatible with neurosarcoidosis
Evidence of systemic sarcoidosis:
(1) histology: noncaseating granulomas in non-CNS site
(2) Kveim test: positive
(3) >= 2 indirect indicators (Gallium scan, chest X-ray, serum ACE)
where:
• Criteria for systemic sarcoidosis have been modified to remove Kveim test and to add FDG-PET as an indirect indicator.
Definite diagnosis- all of the following:
(1) clinical presentation: suggestive of neurosarcoidosis
(2) nervous system histology: positive for noncaseating granulomas
(5) alternative diagnoses: all excluded
Probable:
(1) clinical presentation: suggestive of neurosarcoidosis
(2) nervous system histology: not available or negative
(3) evidence of CNS inflammation: present (see above)
(4) evidence of systemic sarcoidosis: present (see above)
(5) alternative diagnoses: all excluded
Possible:
(1) clinical presentation: suggestive of neurosarcoidosis
(5) alternative diagnoses: all excluded
NOT definite or probable
