INTRODUCTION Granulomatous inflammation is a distinctive ...

Newer immunomodulatory agents and other modalities of treatment under research include
tacrolimus, sirolimus, anticytokine therapy, anticellular adhesion molecules, T-helper-2 cytokines,
and gene therapy targeting proinflammatory cytokines. No data are available to judge efficacy
(Vital et al., 1982).
Radiation
A small number of patients, specifically those with diffuse encephalopathy and vasculopathy, have
undergone radiation treatment. These patients were treated with total nodal and craniospinal
irradiation with some benefit (Chapelon et al., 1990). The usual recommended total dose is 19.5
Gy. This option is generally considered a last resort for refractory cases. The concomitant use of
steroids or other immunosuppressive agents is generally required.
Surgical treatment of neurosarcoid
Surgery is indicated for hydrocephalus, expanding mass lesions, or lesions that cause increased
intracranial pressure (Andrew, 2006).
If presumed sarcoid lesions do not respond to medical treatment, a biopsy should be considered.
Other surgical indications are mass lesions unresponsive to corticosteroids and hydrocephalus
(Krumholz and Stern, 1998).
For severe hydrocephalus with evidence of raised intracranial pressure, a ventriculoperitoneal
shunt or ventricular drain should be placed promptly. Some clinicians recommend high-dose IV
methylprednisone (20 mg/kg/day for 3 days) to stabilize severe hydrocephalus before surgical
intervention is obtained. Shunt procedures are associated with a significant risk of secondary
infection and obstruction of the shunt from the inflammatory changes (Krumholz and Stern,
1998).
Prognosis
Prognosis of sarcoidosis is highly variable. Spontaneous remissions occur in nearly two thirds of
systemic disease. The course may be chronic or progressive in 10-30% (Andrew, 2006).
The overall prognosis for patients with neurosarcoidosis is poorer than for patients with disease
outside the nervous system; however, certain neurological manifestations have favorable
outcomes. In some patients, the disease is a monophasic illness with a good prognosis, but others
show either a remitting-relapsing disease or a chronic progressive course. Approximately 35% to
50% of neurosarcoid patients improve spontaneously before diagnosis and treatment (Chapelon et
al., 1990). Similarly, treated patients show good improvement in some reports. In contrast, in
some series, the majority worsened despite treatment (Junger et al., 1993). In a recent series of 60
patients, 75% of those treated with steroids deteriorated. There is evidence to suggest that patients
with an acute to subacute course tend to have better outcomes than those with chronic disease.
Approximately one third of patients relapse, mimicking multiple sclerosis, but the relapses tend to
occur in sites of earlier involvement (Junger et al., 1993). Clinical manifestations are the best
predictors of course and prognosis (Agbogu et al., 1995).
Cranial neuropathy and aseptic meningitis carry the best prognosis. More than 90% of patients
experience improvement or recovery. Approximately 32% of patients with neurosarcoidosis,
especially those with cranial neuropathies, relapse after the initial neurological episode. Patients
with the other neurological manifestations, especially those with parenchymal disease, generally
have a prolonged chronic course with significant morbidity (Luke et al., 1987). Only 40% of
individuals with optic nerve involvement make appreciable recovery, and approximately 78% of