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[A case of intravascular lymphomatosis (IL) with diffuse cerebral hypoperfusion detected by SPECT]
T Namikawa1, H Moriyasu, Y Tsubota
1Department of Neurology, Wakayama Rosai Hospital, Japan.
Insights
This case study highlights intravascular lymphoma (IL), a rare condition. Technetium-99m-HMPAO-SPECT imaging precisely revealed brain hypoperfusion, aiding in diagnosing this aggressive lymphoma.
Area of Science:
- Neurology
- Oncology
- Radiology
Background:
- Intravascular lymphoma (IL) is a rare extranodal non-Hodgkin lymphoma characterized by proliferation of malignant lymphocytes within blood vessels.
- Diagnosis of IL can be challenging due to its diverse clinical manifestations and nonspecific initial findings.
- Neuroimaging plays a crucial role in evaluating central nervous system involvement in lymphoproliferative disorders.
Observation:
- A 66-year-old male presented with acute amnesia, convulsions, and progressive neurological decline, including consciousness disturbance, disorientation, and pseudobulbar palsy.
- Initial CT and MRI scans were largely unremarkable, showing only scattered lacunar strokes.
- Technetium-99m-HMPAO-SPECT revealed multifocal, large hypoperfused lesions in the cerebrum, alongside systemic signs of inflammation and organ involvement.
Findings:
- Elevated inflammatory markers (ESR, CRP, LDH), increased gamma-globulin, CSF pleocytosis, and pancytopenia were noted.
- SPECT imaging demonstrated significant cerebral hypoperfusion, correlating with the patient's severe neurological symptoms.
- Autopsy confirmed diffuse intravascular B-lymphomatous proliferation across multiple organs, including the brain.
Implications:
- Brain SPECT imaging can precisely visualize pathophysiological changes in IL, offering valuable diagnostic information.
- SPECT may serve as a powerful tool for the early diagnosis and management of intravascular lymphoma.
- This case underscores the importance of considering rare lymphoproliferative disorders in patients with unexplained neurological and systemic symptoms.
Abstract:
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.