Biomaterial Database

Atypical progressive multifocal leukoencephalopathy and primary cerebral malignant lymphoma. 1978

M H GiaRusso, and A H Koeppen

Atypical progressive multifocal leukoencephalopathy occurred in an 82-year-old man with long-standing chronic lymphocytic leukemia. The multifocal white matter lesions revealed demyelination and gliosis, relative preservation of axons and a prominent plasma cell infiltrate. Bizarre astrocytes were uncommon. There were no changes in the oligodendroglial nuclei and no inclusion bodies. Electron and immunohistofluorescence microscopy for papova virus were negative. In addition to this leukoencephalopathy a small primary cerebral lymphoma was present in the right occipital lobe. The tumor cells were plasmacytoid in appearance and their cytoplasm stained well with pyronin. They contained IgG by immunohistofluorescence microscopy. The neoplasm was considered an immunoblastic sarcoma (reticulum cell sarcoma) and thought to arise directly within foci of demyelination. Both disease processes, leukoencephalopathy and lymphoma, may have occurred on a background of immunosuppression.

UI	MeSH Term	Description	Entries
D007968	Leukoencephalopathy, Progressive Multifocal	An opportunistic viral infection of the central nervous system associated with conditions that impair cell-mediated immunity (e.g., ACQUIRED IMMUNODEFICIENCY SYNDROME and other IMMUNOLOGIC DEFICIENCY SYNDROMES; HEMATOLOGIC NEOPLASMS; IMMUNOSUPPRESSION; and COLLAGEN DISEASES). The causative organism is JC Polyomavirus (JC VIRUS) which primarily affects oligodendrocytes, resulting in multiple areas of demyelination. Clinical manifestations include DEMENTIA; ATAXIA; visual disturbances; and other focal neurologic deficits, generally progressing to a vegetative state within 6 months. (From Joynt, Clinical Neurology, 1996, Ch26, pp36-7)	Encephalitis, JC Polyomavirus,Progressive Multifocal Leukoencephalopathy,JC Polyomavirus Encephalopathy,Encephalopathies, JC Polyomavirus,Encephalopathy, JC Polyomavirus,JC Polyomavirus Encephalitis,Leukoencephalopathies, Progressive Multifocal,Multifocal Leukoencephalopathies, Progressive,Multifocal Leukoencephalopathy, Progressive,Progressive Multifocal Leukoencephalopathies
D008223	Lymphoma	A general term for various neoplastic diseases of the lymphoid tissue.	Germinoblastoma,Lymphoma, Malignant,Reticulolymphosarcoma,Sarcoma, Germinoblastic,Germinoblastic Sarcoma,Germinoblastic Sarcomas,Germinoblastomas,Lymphomas,Lymphomas, Malignant,Malignant Lymphoma,Malignant Lymphomas,Reticulolymphosarcomas,Sarcomas, Germinoblastic
D008297	Male		Males
D009778	Occipital Lobe	Posterior portion of the CEREBRAL HEMISPHERES responsible for processing visual sensory information. It is located posterior to the parieto-occipital sulcus and extends to the preoccipital notch.	Annectant Gyrus,Calcarine Fissure,Calcarine Sulcus,Cuneate Lobule,Cuneus,Cuneus Cortex,Cuneus Gyrus,Gyrus Lingualis,Lingual Gyrus,Lunate Sulcus,Medial Occipitotemporal Gyrus,Occipital Cortex,Occipital Gyrus,Occipital Region,Occipital Sulcus,Sulcus Calcarinus,Calcarine Fissures,Calcarinus, Sulcus,Cortex, Cuneus,Cortex, Occipital,Cortices, Cuneus,Cortices, Occipital,Cuneate Lobules,Cuneus Cortices,Fissure, Calcarine,Fissures, Calcarine,Gyrus Linguali,Gyrus, Annectant,Gyrus, Cuneus,Gyrus, Lingual,Gyrus, Medial Occipitotemporal,Gyrus, Occipital,Linguali, Gyrus,Lingualis, Gyrus,Lobe, Occipital,Lobes, Occipital,Lobule, Cuneate,Lobules, Cuneate,Occipital Cortices,Occipital Lobes,Occipital Regions,Occipitotemporal Gyrus, Medial,Region, Occipital,Regions, Occipital,Sulcus, Calcarine,Sulcus, Lunate,Sulcus, Occipital
D001932	Brain Neoplasms	Neoplasms of the intracranial components of the central nervous system, including the cerebral hemispheres, basal ganglia, hypothalamus, thalamus, brain stem, and cerebellum. Brain neoplasms are subdivided into primary (originating from brain tissue) and secondary (i.e., metastatic) forms. Primary neoplasms are subdivided into benign and malignant forms. In general, brain tumors may also be classified by age of onset, histologic type, or presenting location in the brain.	Brain Cancer,Brain Metastases,Brain Tumors,Cancer of Brain,Malignant Primary Brain Tumors,Neoplasms, Intracranial,Benign Neoplasms, Brain,Brain Neoplasm, Primary,Brain Neoplasms, Benign,Brain Neoplasms, Malignant,Brain Neoplasms, Malignant, Primary,Brain Neoplasms, Primary Malignant,Brain Tumor, Primary,Brain Tumor, Recurrent,Cancer of the Brain,Intracranial Neoplasms,Malignant Neoplasms, Brain,Malignant Primary Brain Neoplasms,Neoplasms, Brain,Neoplasms, Brain, Benign,Neoplasms, Brain, Malignant,Neoplasms, Brain, Primary,Primary Brain Neoplasms,Primary Malignant Brain Neoplasms,Primary Malignant Brain Tumors,Benign Brain Neoplasm,Benign Brain Neoplasms,Benign Neoplasm, Brain,Brain Benign Neoplasm,Brain Benign Neoplasms,Brain Cancers,Brain Malignant Neoplasm,Brain Malignant Neoplasms,Brain Metastase,Brain Neoplasm,Brain Neoplasm, Benign,Brain Neoplasm, Malignant,Brain Neoplasms, Primary,Brain Tumor,Brain Tumors, Recurrent,Cancer, Brain,Intracranial Neoplasm,Malignant Brain Neoplasm,Malignant Brain Neoplasms,Malignant Neoplasm, Brain,Neoplasm, Brain,Neoplasm, Intracranial,Primary Brain Neoplasm,Primary Brain Tumor,Primary Brain Tumors,Recurrent Brain Tumor,Recurrent Brain Tumors,Tumor, Brain
D003711	Demyelinating Diseases	Diseases characterized by loss or dysfunction of myelin in the central or peripheral nervous system.	Clinically Isolated CNS Demyelinating Syndrome,Clinically Isolated Syndrome, CNS Demyelinating,Demyelinating Disorders,Demyelination,Demyelinating Disease,Demyelinating Disorder,Demyelinations
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000368	Aged	A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available.	Elderly