INTRODUCTION Granulomatous inflammation is a distinctive ...

the appearance of pulmonary lesions is relatively specific for sarcoidosis. Sites of inflammation
can be accessed for directed biopsy. The combination of gallium 67 scanning and Serum ACE
levels yields a specificity of 83% to 99%, thus making the combination an excellent, minimally
invasive evaluation for sarcoidosis (Corse and stem, 1990).
Ultimately, in cases where the diagnosis of neurosarcoidosis is in doubt despite the above
evaluation, tissue biopsy from neural lesions should be considered. The common sites for biopsy
are the meninges and mass lesions. The sensitivity of meningeal biopsy improves if there is
enhancement on contrast imaging. In the case of focal meningeal enhancement, the imaging
studies should be used to choose the site for biopsy. In case the biopsy from a cerebral mass lesion
shows evidence of noncaseating granulomata, cultures should be obtained to rule out infection as a
cause. A generous sample of tissue should be obtained, because granulomatous changes can be
seen around primary cerebral tumors (Peeples et al., 1991).
Treatment
Unlike pulmonary sarcoidosis, where a period of observation is recommended for mild and
asymptomatic cases (Hunninghake et al., 1994), neurosarcoidosis patients almost always should be
treated on diagnosis. Much of the information regarding specific treatment of neurosarcoidosis
comes from multiple case reports and small case series. Otherwise, most treatment choices are
made based on studies of systemic sarcoidosis (Agbougu et al., 1995).
Immunosuppression is the principal method of controlling the disease, and corticosteroids are the
cornerstone of therapy. Spontaneous remission has been observed. Relapses may respond poorly,
however, requiring chronic steroid therapy. Before initiating immunosuppressive therapy, an
infectious etiology for the symptoms must be excluded (Bucurescu, 2006).
Role of lmmunotherapies
Treatment with corticosteroids has shown impressive responses in many cases. Postulated
mechanisms of action include inhibition of lymphocyte and mononuclear phagocytic activity,
inhibition of transcription of proinflammatory cytokines, down regulation of important cellular
receptors, and interference with collagen synthesis. Although steroids have a biological use in
sarcoidosis, there is no evidence that they change the natural history and outcome of the illness.
The main goal of treatment is to salvage tissue function and prevent progression of the disease,
especially fibrosis and ischemia. Corticosteroids should be started in high doses and, after a
clinical response occurs, gradually tapered, similar to their use in other inflammatory conditions
(Chapelon et al., 1990). Complete withdrawal can be attempted if the clinical course permits. In
case of relapse, a repeat course of high-dose steroid treatment should be attempted. Patients
should be monitored for complications and treated appropriately. It is very important to reduce
the morbidity related to the treatment to avoid discontinuation of the medication because of side
effects. In one study, only 29% of patients with neurosarcoidosis continued long-term steroids; the
remainder discontinued treatment because of intolerable side effects or failure to respond. (Lower
et al., 1997)
Johns Hopkins prednisone treatment schedule (for management of symptomatic pulmonary
sarcoidosis)
Initial treatment 40 mg daily or 20 mg twice daily if fever is prominent, then 30 mg daily in
single morning dose, then 25 mg daily. Then 20 mg daily, each dose level lasting 2 weeks.
Daily maintenance (after 8 wk) 15 mg daily (10 mg may be adequate later)