INTRODUCTION Granulomatous inflammation is a distinctive ...

y central punctuate nodules. It is treated by praziquantel or oxamniquine (David et al., 2004).
Sarcoidosis is a granulomatous inflammation of unknown etiology. And neurological
manifestations occur in 5 – 16% of patients, usually with correlation to disease activity. Isolated
neurosarcoidosis is reported in less than 10 % of cases (James, 1998). Although neurosarcoidosis
can affect any part of the central or peripheral nervous system, the most common presentations
are, aseptic basal meningitis, multiple cranial neuropathies usually facial and optic nerves, focal
supratentorial parenchyma involvement, axonal polyneuropathy and polyneuritis multiplex
(Waxman and Sher, 1979).
Neurosarcoidosis is a diagnosis of exclusion. It relies on the systemic features if present. The most
diagnostic investigation is tissue biopsy (Transbronchial mediastinal biopsy, skin, superficial
lymph nodes, parotid). The combination of positive gallium scan and elevated serum ACE is a
highly diagnostic non invasive tool (83 to 99% specificity). In cases of isolated neurosarcoidosis,
routine CSF studies and Neuroimaging are not conclusive. ACE level in CSF is elevated in 50 % of
cases, otherwise we should rely on tissue biopsies for solid diagnosis (Bourahoui et al., 2004).
Treatment of neurosarcoidosis should start immediately with corticosteroids. Other steroid
sparing agents (cyclosporine, azathioprine, methotrexate, cyclophosphamide) can be used to
decrease maintenance doses of steroids, and in refractory cases. Newer agents as tacrolimus,
sirolimus, anticytokine therapy are under trial (Vital et al., 1982).
Wegener granulomatosis (WG) is a unique clinicopathological disease entity characterized by
necrotizing granulomatous vasculitis of the upper and lower respiratory tract, segmental
necrotizing glomerulonephritis, and small vessel vasculitis (Carol, 2003).
The reported frequency of neurologic involvement in WG ranges from 22% to 54%., and its
manifestations are extremely varied. the most common neurological presentations are
mononeuritis multiplex, cranial neuropathies, seizures, altered cognition (cerebritis), focal motor
and sensory complaints, stroke syndromes, or diabetes insipidus (Metwally, 2006).
Routine laboratory investigations are not specific, yet testing for c-antineutrophil cytoplasmic
antibodies (ANCA) is 97% specific for WG, and 90% sensitive for classical systemic disease. MRI
findings include diffuse or focal thickening of the meninges with contrast enhancement,
Nonhemorrhagic infarcts and occasionally Nonspecific white matter lesions (Joseph et al., 1999)..
Glucocorticoids combined with cyclophosphamide or methotrexate are the only two regimens that
have been shown to induce remission of active WG affecting a major organ (Hoffman et al., 1992).
Primary angiitis of the CNS is a rare disease characterized by vasculitis of small and medium
arteries in the brain and spinal cord. Presentations are highly variable, but the triad of headache,
organic brain syndrome, and multifocal neurologic deficits is most suggestive. Most affected
patients do not have systemic symptoms, signs, or abnormal routine blood tests, including ESR
(Bettoni et al., 1984).
Noninvasive tests have limited sensitivity and specificity. MRI is most sensitive, being abnormal in
more than 80% of patients. The diagnosis is very rare when both the lumbar puncture (with
regards to cells and protein level) and the MRI are normal. Angiography showing beading
produced by alternating stenosis and dilation suggests the diagnosis but is not specific. Analysis of
biopsy specimens of brain cortex and leptomeninges can help establish the diagnosis. In the
absence of positive results on biopsy specimen analysis, the diagnosis of primary angiitis of the
CNS should be doubted (Lie, 1992).
The best therapy has not been established many patients are treated with prednisone and