Objectives To analyze the main epidemiological and clinical characteristics of patients with neurological involvement related to sarcoidosis.
Methods In May 2013, the Study Group on Autoimmune Diseases (GEAS-SEMI) created the national registry of patients with sarcoidosis (SARCOGEAS-SEMI Registry). Patients presenting with neurological features were specifically evaluated.
Results In December 2013, a total of 719 patients with sarcoidosis were included in the SARCOGEAS-SEMI Registry; 52 (7.2%) had neurosarcoidosis, 22 men and 30 women, with a mean age at diagnosis of neurosarcoidosis of 50.1 years (range 23-84 years). The main neurological involvement consisted of cranial neuropathies in 27 (52%) patients, followed by CNS involvement in 23 (44%), peripheral neuropathies in 6 (10%) and muscular involvement in 4 (8%). The most frequent cranial neuropathies included involvement of VII pair (17 cases) followed by VIII pair (5 cases) and V and VI pairs (4 cases each); other cranial nerves affected included IX (n=3), III (n=3), XI (n=2), I (n=1), II (n=1) and XII (n=1); 12/27 (44%) patients showed multiple cranial neuropathies. With respect to CNS involvement, meningeal involvement (15%) and vascular/demyelinating lesions (10%) were the most frequent involvements; other features consisted of parenchymatous cerebral lesions (n=4), encephalopathy (n=4), hypothalamic involvement (n=3), seizures (n=2) and hydrocephalia (n=1). All patients received corticosteroids (10 received methylprednisolone pulses) and 9 immunosuppressants (methotrexate in 5, azathioprine in 4, cyclophosphamide in 2). One patient refractory to standard therapy was treated with infliximab. Nearly half the cases showed improvement or stabilization (46%), 34% showed complete resolution, 16% worsened and 4% died.
Conclusions Nearly 10% of patients with sarcoidosis present with neurological involvement. The main involvement of neurosarcoidosis consisted of cranial neuropathies in half the cases. Twenty percent of patients showed a worse outcome, with a mortality rate of 4%. Neurological involvement is a significant cause of morbidity and mortality in patients with sarcoidosis.
Disclosure of Interest : None declared