Biomaterial Database

Subacute spongiform encephalopathy (Creutzfeldt-Jakob disease) with amyloid angiopathy. 1985

C Keohane, and R Peatfield, and L W Duchen

A case is reported of Creutzfeldt-Jakob disease associated with amyloid infiltration of cerebral vessels. The duration of progressive dementia was only 4 months. Neuritic plaques were not a feature of the pathology. This report emphasises the association of spongiform encephalopathy with the presence of amyloid in the brain.

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
D008578	Meninges	The three membranes that cover the BRAIN and the SPINAL CORD. They are the dura mater, the arachnoid, and the pia mater.
D008875	Middle Aged	An adult aged 45 - 64 years.	Middle Age
D001921	Brain	The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM.	Encephalon
D002536	Cerebral Arteries	The arterial blood vessels supplying the CEREBRUM.	Arteries, Cerebral,Artery, Cerebral,Cerebral Artery
D002540	Cerebral Cortex	The thin layer of GRAY MATTER on the surface of the CEREBRAL HEMISPHERES that develops from the TELENCEPHALON and folds into gyri and sulci. It reaches its highest development in humans and is responsible for intellectual faculties and higher mental functions.	Allocortex,Archipallium,Cortex Cerebri,Cortical Plate,Paleocortex,Periallocortex,Allocortices,Archipalliums,Cerebral Cortices,Cortex Cerebrus,Cortex, Cerebral,Cortical Plates,Paleocortices,Periallocortices,Plate, Cortical
D002561	Cerebrovascular Disorders	A spectrum of pathological conditions of impaired blood flow in the brain. They can involve vessels (ARTERIES or VEINS) in the CEREBRUM, the CEREBELLUM, and the BRAIN STEM. Major categories include INTRACRANIAL ARTERIOVENOUS MALFORMATIONS; BRAIN ISCHEMIA; CEREBRAL HEMORRHAGE; and others.	Brain Vascular Disorders,Intracranial Vascular Disorders,Vascular Diseases, Intracranial,Cerebrovascular Diseases,Cerebrovascular Insufficiency,Cerebrovascular Occlusion,Brain Vascular Disorder,Cerebrovascular Disease,Cerebrovascular Disorder,Cerebrovascular Insufficiencies,Cerebrovascular Occlusions,Disease, Cerebrovascular,Diseases, Cerebrovascular,Insufficiencies, Cerebrovascular,Insufficiency, Cerebrovascular,Intracranial Vascular Disease,Intracranial Vascular Diseases,Intracranial Vascular Disorder,Occlusion, Cerebrovascular,Occlusions, Cerebrovascular,Vascular Disease, Intracranial,Vascular Disorder, Brain,Vascular Disorder, Intracranial,Vascular Disorders, Brain,Vascular Disorders, Intracranial
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000686	Amyloidosis	A group of sporadic, familial and/or inherited, degenerative, and infectious disease processes, linked by the common theme of abnormal protein folding and deposition of AMYLOID. As the amyloid deposits enlarge they displace normal tissue structures, causing disruption of function. Various signs and symptoms depend on the location and size of the deposits.	Amyloidoses