BILATERAL TRIGEMINAL AMYLOIDOMA - AN UNUSUAL CASE OF TRIGEMINAL NEUROPATHY WITH A REVIEW OF THE LITERATURE - CASE-REPORT

Isolated amyloidomas may, albeit rarely, involve the central nervous s ystem. There are three previous reports of amyloidomas that involved t he gasserian ganglion and caused unilateral trigeminal neuropathies. T he authors report the case of a 49-year-old woman with apparently isol ated amyloidomas that caused slowly progressive bilateral trigeminal n europathies. Magnetic resonance imaging of the brain revealed mild swe lling of the left trigeminal nerve within the cavernous sinus and unif orm enhancement with gadolinium throughout the length of the nerve. At craniotomy, the trigeminal nerve and ganglion were observed to be inf iltrated by a tumor-like mass. Biopsy showed extensive infiltration of the nerve and ganglion by amyloid. Immunocytochemical studies of the amyloid were negative for immunoglobulins, kappa and lambda light chai ns, beta-amyloid A4 protein, transthyretin, beta(2)-microglobulin, cys tatin C, and gelsolin, but weak focal immunoreactivity with antiamyloi d AA antibody was seen in the amyloid in vessels and in some intraneur al deposits. Extensive investigations failed to reveal evidence of eit her systemic amyloidoses or an underlying inflammatory disorder or mal ignancy.