Neurosarcoidosis manifestations:
- Myelopathy
- Cranial neuropathies
- Pituitary disease
- Dural based lesion
- Encephalopathy
- CNS sarcoid angiitis
- Peripheral sensory neuropathy
- Myopathy
Diagnosis:
Histology: +typical findings +excluding other diseases confirms the diagnosis
Histology: noncaseating granuloma
Findings on Investigations:
CXR:
- Stage 0: normal
- Stage I: bilateral hilar lymphadenopathy or paratracheal lymphadenopathy
- Stage II: hilar lymphadenopathy +pulmonary infiltrates
- Stage III: pulmonary infiltrates without hilar lymphadenopathy
- Stage IV: ‘honey comb lung appearance’
MRI:
- T2: high signal. Around pituitary stalk, periventricularly, myelopathy
- +gadolinium: meningeal enhancement (perform this before LP)
CSF:
- High immunoglobulins
- Cell cound: 10-200, mainly lymphocytes
- Glucose: normal or mildly reduced
NCS and EMG: neuropathies or myopathies
- In neuropathy:
- LMN VII palsy/recurrent/bilateral,
- Rarely sensorimotor neuropathy
- NCS: axonal, multifocal, sensorimotor
Muscle biopsy in myopathy:
- Interstitial epithelioid and giant cell granulomas.
Tests for hypopituitarism if pituitary/hypothalamic disease is found
Blood tests:
- FBC: lymphopenia
- ESR: raised
- ACE levels: high, not sensitive or specific
- Immunoglobulins i.e. gammaglobulins: high
- Ca++: hypercalcemia
- Hypercalciuria
BAL: increased lymphocytes
PFTs: normal or restrictive
Note, Kveim tests are obsolete
X-rays: punched out lesions in terminal phalanges
Pathology, nerve biopsy, rare:
Epineurium: Noncaseating granuloma (multinucleated giant cells), vasculitis can occur. Perineurium: inflammatory infiltrate i.e. perineuritis. Endoneurium: inflammatory infiltrate, granuloma, asymmetrical axonal loss.
Treatment:
Consider depending on complications:
- Prednisolone 40 mg daily for 2 weeks, then taper over 2 weeks, then maintenance for at least 6 months
In neurosarcoid:
- Prednisolone as above
- 2nd line: cyclosporin, also steroid sparing
Treat eye disease