Unexpected condition in a rare disease: encephalopathy in early-onset sarcoidosis

Background Granulomatous autoinflammatory diseases are monogenic syndromes caused by mutations in the region encoding nucleotide-binding domain of oligomerization domain-containing 2 gene. Blau syndrome and early-onset sarcoidosis familial sporadic forms same disease very rare. Many organ systems may be involved; however, neurologic involvement is infrequent. We reported a case encephalitis 12-year-old girl followed with diagnosis sarcoidosis. Case The patient was diagnosed juvenile idiopathic arthritis at 3 years age. considered druginduced 6 age granulomatous inflammation liver kidney. Small joint camptodactyly developed during follow-up. M315T mutation detected NOD2 gene supporting suffered from encephalopathy when she under methotrexate, infliximab, systemic steroid treatment 12 Cerebrospinal fluid limbic antibody panel negative. Conclusion Encephalopathy not common cause our interpreted as autoimmune encephalitis.