A retrospective study of Creutzfeldt-Jakob disease in North of Portugal 1993-2002: demographic, clinical and neuropathological features
Description of the demographic, clinical and neuropathological features of 11 cases of Creutzfeldt-Jakob disease (CJD). Review of the clinical and neuropathological features of patients with CJD diagnosed in hospitals in the North of Portugal between 1993 and 2002. Eleven patients were identified, 4...
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| Veröffentlicht in: | Arquivos de neuro-psiquiatria 2003-12, Vol.61 (4), p.950-956 |
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| container_title | Arquivos de neuro-psiquiatria |
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| creator | Silva, Ana Martins Pires, Manuel Melo Bastos Leite, Antonio J Honavar, Mrinalini Mendes, Alexandre Correia, Manuel Nora, Manuel Silva, Mário Rui Costa, Manuela Guimarães, Antonio Monteiro, Luís |
| description | Description of the demographic, clinical and neuropathological features of 11 cases of Creutzfeldt-Jakob disease (CJD).
Review of the clinical and neuropathological features of patients with CJD diagnosed in hospitals in the North of Portugal between 1993 and 2002.
Eleven patients were identified, 4 females: mean age of onset of symptoms--64 years, mean duration of disease--8 months. All presented with a syndrome of progressive dementia with myoclonus, with four patients presenting with cerebellar signs. Neuropathological examination of brain at autopsy showed spongiosis and reactive gliosis associated with neuronal loss. In eight cases immunocytochemistry for prion protein (PrP) was carried out and was positive.
The group of patients described represents the heterogeneity of the clinical phenotypes possible in CJD. Neuropathological examination is still indispensable to make the definitive diagnosis of the disease. |
| doi_str_mv | 10.1590/S0004-282X2003000600012 |
| format | Article |
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Review of the clinical and neuropathological features of patients with CJD diagnosed in hospitals in the North of Portugal between 1993 and 2002.
Eleven patients were identified, 4 females: mean age of onset of symptoms--64 years, mean duration of disease--8 months. All presented with a syndrome of progressive dementia with myoclonus, with four patients presenting with cerebellar signs. Neuropathological examination of brain at autopsy showed spongiosis and reactive gliosis associated with neuronal loss. In eight cases immunocytochemistry for prion protein (PrP) was carried out and was positive.
The group of patients described represents the heterogeneity of the clinical phenotypes possible in CJD. Neuropathological examination is still indispensable to make the definitive diagnosis of the disease.</description><identifier>ISSN: 0004-282X</identifier><identifier>DOI: 10.1590/S0004-282X2003000600012</identifier><identifier>PMID: 14762597</identifier><language>por</language><publisher>Brazil</publisher><subject>Age of Onset ; Aged ; Atrophy ; Brain - pathology ; Creutzfeldt-Jakob Syndrome - genetics ; Creutzfeldt-Jakob Syndrome - pathology ; Diagnosis, Differential ; Electroencephalography ; Female ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Portugal ; Prions - analysis ; Retrospective Studies ; Tomography, X-Ray Computed</subject><ispartof>Arquivos de neuro-psiquiatria, 2003-12, Vol.61 (4), p.950-956</ispartof><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780,860,27901,27902</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/14762597$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Silva, Ana Martins</creatorcontrib><creatorcontrib>Pires, Manuel Melo</creatorcontrib><creatorcontrib>Bastos Leite, Antonio J</creatorcontrib><creatorcontrib>Honavar, Mrinalini</creatorcontrib><creatorcontrib>Mendes, Alexandre</creatorcontrib><creatorcontrib>Correia, Manuel</creatorcontrib><creatorcontrib>Nora, Manuel</creatorcontrib><creatorcontrib>Silva, Mário Rui</creatorcontrib><creatorcontrib>Costa, Manuela</creatorcontrib><creatorcontrib>Guimarães, Antonio</creatorcontrib><creatorcontrib>Monteiro, Luís</creatorcontrib><title>A retrospective study of Creutzfeldt-Jakob disease in North of Portugal 1993-2002: demographic, clinical and neuropathological features</title><title>Arquivos de neuro-psiquiatria</title><addtitle>Arq Neuropsiquiatr</addtitle><description>Description of the demographic, clinical and neuropathological features of 11 cases of Creutzfeldt-Jakob disease (CJD).
Review of the clinical and neuropathological features of patients with CJD diagnosed in hospitals in the North of Portugal between 1993 and 2002.
Eleven patients were identified, 4 females: mean age of onset of symptoms--64 years, mean duration of disease--8 months. All presented with a syndrome of progressive dementia with myoclonus, with four patients presenting with cerebellar signs. Neuropathological examination of brain at autopsy showed spongiosis and reactive gliosis associated with neuronal loss. In eight cases immunocytochemistry for prion protein (PrP) was carried out and was positive.
The group of patients described represents the heterogeneity of the clinical phenotypes possible in CJD. Neuropathological examination is still indispensable to make the definitive diagnosis of the disease.</description><subject>Age of Onset</subject><subject>Aged</subject><subject>Atrophy</subject><subject>Brain - pathology</subject><subject>Creutzfeldt-Jakob Syndrome - genetics</subject><subject>Creutzfeldt-Jakob Syndrome - pathology</subject><subject>Diagnosis, Differential</subject><subject>Electroencephalography</subject><subject>Female</subject><subject>Humans</subject><subject>Magnetic Resonance Imaging</subject><subject>Male</subject><subject>Middle Aged</subject><subject>Portugal</subject><subject>Prions - analysis</subject><subject>Retrospective Studies</subject><subject>Tomography, X-Ray Computed</subject><issn>0004-282X</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2003</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFkMlOwzAQhn0A0VJ4BfCJEwEvSRxzQxWrKkACJG6RY0_aQBoHL0jlBXhtAhSuHEb_LJ_-GQ1C-5Qc0UyS43tCSJqwgj0xQvhQ5ENQtoHGf4MR2vb-mRCWSim20IimImeZFGP0cYodBGd9Dzo0b4B9iGaFbY2nDmJ4r6E1IblWL7bCpvGgPOCmwzfWhcUXdTckca5aTKXkyXAAO8EGlnbuVL9o9CHWbdM1egBUZ3AH0dlehYVt7fy7W4MK0YHfQZu1aj3srnWCHs_PHqaXyez24mp6Okt6KkhIpBLMQGV4pWiRq9romlSZKIblBedVUTOhhWSaFVyonAHnxqSUyypPtSKQ8Qk6-PHtnX2N4EO5bLyGtlUd2OhLQTOWF4z9CzJCKeF5PoB7azBWSzBl75qlcqvy98f8E99NfrM</recordid><startdate>20031201</startdate><enddate>20031201</enddate><creator>Silva, Ana Martins</creator><creator>Pires, Manuel Melo</creator><creator>Bastos Leite, Antonio J</creator><creator>Honavar, Mrinalini</creator><creator>Mendes, Alexandre</creator><creator>Correia, Manuel</creator><creator>Nora, Manuel</creator><creator>Silva, Mário Rui</creator><creator>Costa, Manuela</creator><creator>Guimarães, Antonio</creator><creator>Monteiro, Luís</creator><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>7TK</scope><scope>7U9</scope><scope>H94</scope><scope>7X8</scope></search><sort><creationdate>20031201</creationdate><title>A retrospective study of Creutzfeldt-Jakob disease in North of Portugal 1993-2002: demographic, clinical and neuropathological features</title><author>Silva, Ana Martins ; Pires, Manuel Melo ; Bastos Leite, Antonio J ; Honavar, Mrinalini ; Mendes, Alexandre ; Correia, Manuel ; Nora, Manuel ; Silva, Mário Rui ; Costa, Manuela ; Guimarães, Antonio ; Monteiro, Luís</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-p170t-9a72debd3ba186afdcf0b578993833b8f27c792c2837a62e33dd4139b64ca0e53</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>por</language><creationdate>2003</creationdate><topic>Age of Onset</topic><topic>Aged</topic><topic>Atrophy</topic><topic>Brain - pathology</topic><topic>Creutzfeldt-Jakob Syndrome - genetics</topic><topic>Creutzfeldt-Jakob Syndrome - pathology</topic><topic>Diagnosis, Differential</topic><topic>Electroencephalography</topic><topic>Female</topic><topic>Humans</topic><topic>Magnetic Resonance Imaging</topic><topic>Male</topic><topic>Middle Aged</topic><topic>Portugal</topic><topic>Prions - analysis</topic><topic>Retrospective Studies</topic><topic>Tomography, X-Ray Computed</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Silva, Ana Martins</creatorcontrib><creatorcontrib>Pires, Manuel Melo</creatorcontrib><creatorcontrib>Bastos Leite, Antonio J</creatorcontrib><creatorcontrib>Honavar, Mrinalini</creatorcontrib><creatorcontrib>Mendes, Alexandre</creatorcontrib><creatorcontrib>Correia, Manuel</creatorcontrib><creatorcontrib>Nora, Manuel</creatorcontrib><creatorcontrib>Silva, Mário Rui</creatorcontrib><creatorcontrib>Costa, Manuela</creatorcontrib><creatorcontrib>Guimarães, Antonio</creatorcontrib><creatorcontrib>Monteiro, Luís</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>Neurosciences Abstracts</collection><collection>Virology and AIDS Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>MEDLINE - Academic</collection><jtitle>Arquivos de neuro-psiquiatria</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Silva, Ana Martins</au><au>Pires, Manuel Melo</au><au>Bastos Leite, Antonio J</au><au>Honavar, Mrinalini</au><au>Mendes, Alexandre</au><au>Correia, Manuel</au><au>Nora, Manuel</au><au>Silva, Mário Rui</au><au>Costa, Manuela</au><au>Guimarães, Antonio</au><au>Monteiro, Luís</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>A retrospective study of Creutzfeldt-Jakob disease in North of Portugal 1993-2002: demographic, clinical and neuropathological features</atitle><jtitle>Arquivos de neuro-psiquiatria</jtitle><addtitle>Arq Neuropsiquiatr</addtitle><date>2003-12-01</date><risdate>2003</risdate><volume>61</volume><issue>4</issue><spage>950</spage><epage>956</epage><pages>950-956</pages><issn>0004-282X</issn><abstract>Description of the demographic, clinical and neuropathological features of 11 cases of Creutzfeldt-Jakob disease (CJD).
Review of the clinical and neuropathological features of patients with CJD diagnosed in hospitals in the North of Portugal between 1993 and 2002.
Eleven patients were identified, 4 females: mean age of onset of symptoms--64 years, mean duration of disease--8 months. All presented with a syndrome of progressive dementia with myoclonus, with four patients presenting with cerebellar signs. Neuropathological examination of brain at autopsy showed spongiosis and reactive gliosis associated with neuronal loss. In eight cases immunocytochemistry for prion protein (PrP) was carried out and was positive.
The group of patients described represents the heterogeneity of the clinical phenotypes possible in CJD. Neuropathological examination is still indispensable to make the definitive diagnosis of the disease.</abstract><cop>Brazil</cop><pmid>14762597</pmid><doi>10.1590/S0004-282X2003000600012</doi><tpages>7</tpages><oa>free_for_read</oa></addata></record> |
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| source | MEDLINE; DOAJ Directory of Open Access Journals; Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals |
| subjects | Age of Onset Aged Atrophy Brain - pathology Creutzfeldt-Jakob Syndrome - genetics Creutzfeldt-Jakob Syndrome - pathology Diagnosis, Differential Electroencephalography Female Humans Magnetic Resonance Imaging Male Middle Aged Portugal Prions - analysis Retrospective Studies Tomography, X-Ray Computed |
| title | A retrospective study of Creutzfeldt-Jakob disease in North of Portugal 1993-2002: demographic, clinical and neuropathological features |
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