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Washington University Experience | MISCELLANEOUS | Amyloidoma | 3A0 Case 3 History
Case 3 History (AANP Diagnostic Slide session 1991, Case 2) ---- The patient was a 32 year old male with no significant past medical history prior to developing seizures when he was 28 years old and optic disc swelling without loss of visual acuity. CT showed diffuse enhancement predominantly in the white matter of the left frontal and right parietal areas. MRI showed multiple areas of increased T2 signal in the same distribution. An LP was done. The opening pressure was not recorded. No cells were present. The protein was 35mg. glucose 69 mg%, and IgG was 6.17mg/d! (normal up to 3.7mg/dl). Isoelectric focusing showed multiple bands. Visual evoked potentials and EEG were normal. Over the ensuing years his seizures were difficult to control and he developed progressive cognitive decline, followed by reflex asymmetries. On the present admission, he had moderate dementia characterized mainly by frontal lobe type behavior, accompanied by marked reflex asymmetries and bilateral ankle clonus. At this time, there was no papilledema or optic disc pallor. CT showed large ring enhancing lesions that on MRI appeared to have progressed to a mild degree over the course of his illness. EEG's on several occasions remained normal. A left frontal craniotomy revealed a diffusely firm cortical surface. Two 3 x 3 x 2.5cm biopsies were taken, which included cerebral cortex and underlying white matter.