Biomaterial Database

[Subacute spongiform encephalopathy and Creutzfeldt-Jakob disease. Apropos of 1 case]. 1975

J Morice, and J C Turpin, and M Pluot, and G Level

The authors describe a case of sub-acute spongiform encephalopathy with an ultrastructural study in a man of 65 with associated dementia, extra-pyramidal syndrome, myoclonia, optical disorders, pyramidal syndrome and speech disorders, rapidly leading to death one and a half months later. Electroencephalograms were at first disorganized with persistent delta and theta activity and only in the last record did short runs of repetitive activity appear. Histologically, there was severe status spongiosus, a moderate degree of neuronal depopulation with a noticeable astrocytary gliosis. Under the electron microscope, the neuropile in the astrocytes was found to be pitted with numerous cavities and so were, to a lesser extent, the axonal projections. The vessels were normal. Nothing was found to indicate viral structures. Attention is drawn to the nosological connection between this type of observation and Creutzfeldt-Jakob disease.

UI	MeSH Term	Description	Entries
D007562	Creutzfeldt-Jakob Syndrome	A rare transmissible encephalopathy most prevalent between the ages of 50 and 70 years. Affected individuals may present with sleep disturbances, personality changes, ATAXIA; APHASIA, visual loss, weakness, muscle atrophy, MYOCLONUS, progressive dementia, and death within one year of disease onset. A familial form exhibiting autosomal dominant inheritance and a new variant CJD (potentially associated with ENCEPHALOPATHY, BOVINE SPONGIFORM) have been described. Pathological features include prominent cerebellar and cerebral cortical spongiform degeneration and the presence of PRIONS. (From N Engl J Med, 1998 Dec 31;339(27))	New Variant Creutzfeldt-Jakob Disease,Spongiform Encephalopathy, Subacute,CJD (Creutzfeldt-Jakob Disease),Creutzfeldt Jacob Disease,Creutzfeldt-Jakob Disease,Creutzfeldt-Jakob Disease, Familial,Creutzfeldt-Jakob Disease, New Variant,Creutzfeldt-Jakob Disease, Variant,Familial Creutzfeldt-Jakob Disease,Jakob-Creutzfeldt Disease,Jakob-Creutzfeldt Syndrome,V-CJD (Variant-Creutzfeldt-Jakob Disease),Variant Creutzfeldt-Jakob Disease,CJD (Creutzfeldt Jakob Disease),Creutzfeldt Jakob Disease,Creutzfeldt Jakob Disease, Familial,Creutzfeldt Jakob Disease, New Variant,Creutzfeldt Jakob Disease, Variant,Creutzfeldt Jakob Syndrome,Creutzfeldt-Jakob Diseases, Familial,Disease, Creutzfeldt Jacob,Disease, Creutzfeldt-Jakob,Disease, Familial Creutzfeldt-Jakob,Disease, Jakob-Creutzfeldt,Encephalopathies, Subacute Spongiform,Encephalopathy, Subacute Spongiform,Familial Creutzfeldt Jakob Disease,Familial Creutzfeldt-Jakob Diseases,Jacob Disease, Creutzfeldt,Jakob Creutzfeldt Disease,Jakob Creutzfeldt Syndrome,New Variant Creutzfeldt Jakob Disease,Spongiform Encephalopathies, Subacute,Subacute Spongiform Encephalopathies,Subacute Spongiform Encephalopathy,Syndrome, Creutzfeldt-Jakob,Syndrome, Jakob-Creutzfeldt,V CJD (Variant Creutzfeldt Jakob Disease),Variant Creutzfeldt Jakob Disease
D008297	Male		Males
D011596	Psychomotor Disorders	Abnormalities of motor function that are associated with organic and non-organic cognitive disorders.	Psychomotor Impairment,Developmental Psychomotor Disorders,Psychomotor Disorders, Developmental,Developmental Psychomotor Disorder,Impairment, Psychomotor,Impairments, Psychomotor,Psychomotor Disorder, Developmental,Psychomotor Impairments
D003072	Cognition Disorders	Disorders characterized by disturbances in mental processes related to learning, thinking, reasoning, and judgment.	Overinclusion,Disorder, Cognition,Disorders, Cognition
D003937	Diagnosis, Differential	Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures.	Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
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
D012897	Slow Virus Diseases	Diseases of viral origin, characterized by incubation periods of months to years, insidious onset of clinical manifestations, and protracted clinical course. Though the disease process is protracted, viral multiplication may not be unusually slow. Conventional viruses produce slow virus diseases such as SUBACUTE SCLEROSING PANENCEPHALITIS, progressive multifocal leukoencephalopathy (LEUKOENCEPHALOPATHY, PROGRESSIVE MULTIFOCAL), and AIDS. Diseases produced by unconventional agents were originally considered part of this group. They are now called PRION DISEASES.	Disease, Slow Virus,Diseases, Slow Virus,Slow Virus Disease,Virus Disease, Slow,Virus Diseases, Slow
D013064	Speech Disorders	Acquired or developmental conditions marked by an impaired ability to comprehend or generate spoken forms of language.	Aprosodia,Aprosodic Speech,Cluttering,Dysglossia,Dyslalia,Rhinolalia,Verbal Fluency Disorders,Aprosodias,Clutterings,Dysglossias,Dyslalias,Rhinolalias,Speech, Aprosodic,Verbal Fluency Disorder
D014786	Vision Disorders	Visual impairments limiting one or more of the basic functions of the eye: visual acuity, dark adaptation, color vision, or peripheral vision. These may result from EYE DISEASES; OPTIC NERVE DISEASES; VISUAL PATHWAY diseases; OCCIPITAL LOBE diseases; OCULAR MOTILITY DISORDERS; and other conditions (From Newell, Ophthalmology: Principles and Concepts, 7th ed, p132).	Hemeralopia,Macropsia,Micropsia,Day Blindness,Metamorphopsia,Vision Disability,Visual Disorders,Visual Impairment,Blindness, Day,Disabilities, Vision,Disability, Vision,Disorder, Visual,Disorders, Visual,Hemeralopias,Impairment, Visual,Impairments, Visual,Macropsias,Metamorphopsias,Micropsias,Vision Disabilities,Vision Disorder,Visual Disorder,Visual Impairments