Clinical, Laboratory and Neuroimaging Findings in Patients with Sarcoidosis Involving the CNS: Study of 29 Cases

Abstract

Introduction: Sarcoidosis is a rare idiopathic systemic inflammatory disorder characterized by the formation of the non-caseating granulomas. Neurological complications involve approximately 5% of the patients with systemic sarcoidosis. Most literatures on neurosarcoidosis are anecdotal case reports. Studies of a series of cases of neurosarcoidosis are sparse. In this report, we studied clinical, laboratory and neuroimaging findings in patients with sarcoidosis involving the central nervous system (CNS). Methods: Clinical charts were retrospectively reviewed for the past 10 years to identify patients with a clinically conformed diagnosis of sarcoidosis, which was proven by a previously biopsied histological evaluation. Subjects with neurological presentation and clinically diagnosed neurosarcoidosis were collected. Subjects with identifiable etiologies causing a CNS structural lesion and/or dysfunction other than sarcoidosis, incomplete data, or sarcoidosis without nervous system complications were excluded. The collected data of clinical, laboratory and neuroimaging findings were analyzed. Results: Twenty-nine patients (age: 51.7 ± 9.0 years, range: 36 - 77, female / male = 21 / 8) were studied. Common clinical presentations were seizures (34.5%); Bell’s palsy (17.2%) with facial hypoesthesia (13.8%); limb weakness (13.8%) and/or numbness (6.9%); abducens nerve paresis (6.9%); hydrocephalus (6.9%), nystagmus (3.4%), and optical neuritis (3.4%). MRI showed abnormalities in discrete areas in the brain and spinal cord involving the parenchyma in different anatomic locations, leptomeninges and cranial nerves. Abnormal laboratory findings showed elevated ACE level in plasma and cerebrospinal fluid, impaired liver functions with reduced level of triglycerides, and elevated protein with decreased glucose and mild lymphocytosis in cerebrospinal fluid. Conclusion: Diagnosis of neurosarcoidosis remains a clinical challenge. Seizures were most frequently seen at presentation in patients with sarcoidosis involving the CNS. Laboratory studies of impaired liver function with reduced triglycerides, and CSF elevated levels of ACE and protein with decreased glucose and mild lymphocytosis may aid in differentiating neurosarcoidosis from other CNS inflammatory demyelinating disorders.