Clinical and Neurologic findings in NeuroSjogren's s

Delalande et al. Medicine 83:280-291 2004.

Among patients with Sjogren's syndrome (SS) 20 % have neurologic involvement. Of 82 patients (including 65 women) the mean age at onset was 53. Neurologic involvement preceded diagnosis in 81 %. 56 % had focal or multifocal CNS disorders (acute myelopathy 12, chronic myelopathy 16, MND 1, 33 brain involvement and 13 had optic neuropathy). In 10 patients the disease mimicked relapsing-remitting MS and in 13, PPMS. There were 7 cases of seizures, 9 of cognitive dysfunction and 2 of encephalopathy. 51 patients had involvement of the peripheral nervous system. The neuropathy was a symmetric sensorimotor axonal polyneuropathy with a predominance of sensory symptoms or pure sensory neuropathy (28 patients) or cranial neuropathy affecting cranial nerves V, VII or VIII (n=16). 7 had multiple mononeuropathy, 2 myositis and 1 polyradiculopathy. 30 % had oligoclonal bands, 61 % abnormal VEP's, 70 % had abnormal brain MRI's (white spots) with 40 % meeting criteria for MS. Among the 29 patients with myelopathy, 19 had T2 changes on spinal MRI.

Patients with PNS involvement had frequent (21%) anti Ro/SSA or anti La/SSB antibodies at diagnosis of SS. 52 patients were severely disabled and those were far more likely to have CNS involvement than PNS. Cytoxan treatment resulted in stabilization/partial recovery in 92 % with myelopathy and 100 % with multiple mononeuropathy.

Comment -- the disease is virtually indistinguishable from MS. Check NMO IgG as some are positive in cases of longitudinallly extensive transverse myelitis.