Table 2.

Diagnostic criteria for neurosarcoidosis (central and peripheral nervous system involvement) [7^▪▪]

Possible

The clinical presentation and diagnostic evaluation suggest neurosarcoidosis, as defined by the clinical manifestations and MRI, CSF, and/or EMG/NCS findings typical of granulomatous inflammation of the nervous system and after rigorous exclusion of other causes

There is no pathological confirmation of granulomatous disease

Probable

The clinical presentation and diagnostic evaluation suggest neurosarcoidosis, as defined by the clinical manifestations and MRI, CSF, and/or EMG/NCS findings typical of granulomatous inflammation of the nervous system and after rigorous exclusion of other causes

There is pathological confirmation of systemic granulomatous disease consistent with sarcoidosis

Definite

The clinical presentation and diagnostic evaluation suggest neurosarcoidosis, as defined by the clinical manifestations, MRI, CSF, and/or EMG/NCS findings typical of granulomatous inflammation of the nervous system, after rigorous exclusion of other causes

The nervous system abnormality is consistent with neurosarcoidosis

Type a. Extraneural sarcoidosis is evident

Type b. No extraneural sarcoidosis is evident (isolated neurosarcoidosis)

CSF, cerebrospinal fluid; EMG, electromyography; MRI, magnetic resonance imaging; NCS, nerve conduction studies.