[A case of intravascular lymphomatosis (IL) with diffuse cerebral hypoperfusion detected by SPECT]

A 66-year-old male experienced the sudden onset of amnesia and generalized convulsions, and during the subsequent month developed consciousness disturbance, disorientation, memory disturbance, abnormal speech and behavior, pseudobulbar palsy, unsteadiness and urinary/fecal incontinence. The initial brain CT scan was unremarkable, and the MRI findings were also unremarkable, except for evidence of several scattered lacunar strokes in the cerebral white matter. 99mTc-HM-PAO-SPECT, however, revealed multifocal large hypoperfused lesions in the cerebrum. The erythrocyte sedimentation rate and serum CRP, LDH and gamma-globulin levels were elevated. CSF studies showed mild pleocytosis and increased protein levels. The patient's subsequent course was marked by fever, SIADH, adrenal enlargement, splenomegaly, pulmonary infiltration and pancytopenia. The neurological signs progressively worsened, until the patient lapsed into an apallic state and died 3 months after the onset of symptoms. Autopsy disclosed diffuse intravascular B lymphomatous proliferation in the brain, lungs, kidneys, adrenals, spleen and pancreas. Until now, the brain SPECT findings of IL were not very well known, but in our patient, SPECT was capable of demonstrating the pathophysiologic changes very precisely. SPECT was able to provide valuable information on the pathophysiology of IL and may therefore become another powerful tool in the diagnosis of IL.