Pathobiology of Neurosarcoidosis and Clinicopathologic Correlation

Abstract

SUMMARY:In a retrospective study of 4,294 consecutive postmortem cases, there were 15 with histologically proved sarcoidosis, including 4 with CNS involvement. Whereas in previously reported autopsy series the prevalence of neurosarcoidosis was 15%, it is 27% in this review. Five cases with incidental, clinically inapparent sarcoidosis died at a mean age of 54.6 years; those six with systemic sarcoidosis, not involving the CNS, died at a mean age of 47.0 years; while those four with a variety of neurologic manifestations in addition to systemic sarcoidosis died at a mean age of 38.2 years. Thus, it appears that at one end of the spectrum of morbidity, sarcoidosis may cause no or only trivial symptoms and permit prolonged survival. At the other extreme, sarcoidosis may affect a younger population more severely. CNS involvement occurs relatively early in the course and is rather rapidly progressive, accounting for the poor prognosis, despite appropriate medical and neurosurgical management. In its turn, neurosarcoidosis may present with a variety of signs and symptoms, depending on the site of involvement along the craniospinal axis. This feature is illustrated by the manifestations of compression myelopathy, hydrocephalus with dementia, hydrocephalus with seizures and ataxia, and anosmia, blindness, seizures, and diabetes insipidus. In addition, one patient developed a Nocardia brain abscess as a complication of the altered immune system in sarcoidosis.

Soluble CSF interleukin 2 receptor as indicator of neurosarcoidosis

“…Although the prevalence is said to be 10-50 in 100,000 depending on the ethnical background, the disease might well be underdiagnosed as a post mortem study with an estimated prevalence of more than 600 in 100,000 suggests [1,2]. The nervous system is involved in 5-27% of cases with a broad range of symptoms including headache, aseptic meningitis, visual disturbances, cranial nerve palsy, spinal cord symptoms, brainstem or cerebellar signs and pituitary gland dysfunction [3][4][5][6][7]. However, the diagnosis of neurosarcoidosis remains difficult, especially in the absence of an established diagnosis of systemic sarcoidosis, which is the case in almost half of the patients with NS [5,7].…”

Mycobacteria and sarcoidosis

“…32 33 are likely to be an unrelated late complication. 23 Acid fast coccobacillary bacteria have been identified in tissue…”

Ocular Features in Neurosarcoidosis

“…5 The reported incidence of neurosarcoidosis (NS) is about 5% in patients with systemic sarcoidosis, 6,7 although postmortem studies have shown that the prevalence of the disease may be higher. 8 An incidence of 37% of central nervous system involvement has been reported in sarcoidosis when intraocular involvement is present. 9 In these cases, other causes of retinal phlebitis, such as multiple sclerosis (MS) or Behçet disease, need to be excluded.…”