Cerebral amyloid angiopathy and motor neurone disease presenting with a progressive supranuclear palsy‐like syndrome
Identifieur interne : 004162 ( Main/Exploration ); précédent : 004161; suivant : 004163Cerebral amyloid angiopathy and motor neurone disease presenting with a progressive supranuclear palsy‐like syndrome
Auteurs : Robert A. Weeks [Royaume-Uni] ; Francisco Scaravilli [Royaume-Uni] ; Andrew Lees (neurologue) [Royaume-Uni] ; Camille Carroll [Royaume-Uni] ; Masud Husain [Royaume-Uni] ; Peter Rudge [Royaume-Uni]Source :
- Movement Disorders [ 0885-3185 ] ; 2003-03.
Descripteurs français
- Pascal (Inist)
- Wicri :
- topic : Personne âgée.
English descriptors
- KwdEn :
- ALS, Aged, Amyloidosis, Case study, Cerebral Amyloid Angiopathy (complications), Cerebral Amyloid Angiopathy (diagnosis), Cerebrovascular disease, Diagnosis, Differential, Differential diagnostic, Elderly, Fatal Outcome, Female, Humans, Immunohistochemistry, MND, Motor Neuron Disease (complications), Motor Neuron Disease (diagnosis), Motor neuron disease, Protein, Steele‐Richardson‐Olszewski, Supranuclear Palsy, Progressive (diagnosis), Supranuclear Palsy, Progressive (etiology), Supranuclear ophthalmoplegia, Syndrome, cerebral amyloid angiopathy, motor neurone disease, progressive supranuclear palsy.
- MESH :
- complications : Cerebral Amyloid Angiopathy, Motor Neuron Disease.
- diagnosis : Cerebral Amyloid Angiopathy, Motor Neuron Disease, Supranuclear Palsy, Progressive.
- etiology : Supranuclear Palsy, Progressive.
- Aged, Diagnosis, Differential, Fatal Outcome, Female, Humans, Syndrome.
Abstract
We describe a 68‐year‐old woman who presented with falls, mild limb bradykinesia, axial rigidity, and a severe supranuclear gaze palsy, which failed to benefit from levodopa. She subsequently developed severe apraxia, progressive dysarthria, dysphagia, and a frontal cognitive impairment. Pyramidal weakness with fasciculations and widespread chronic partial denervation appeared shortly before her death from bronchopneumonia, 6 months after disease onset. A severe cerebral amyloid angiopathy diffusely involving the cerebral hemispheres and cerebellum was present at autopsy as well as a second pathological condition indicative of motor neurone disease. Cerebral amyloid angiopathy may rarely present with a progressive supranuclear palsy–like phenotype.
Url:
DOI: 10.1002/mds.10347
Affiliations:
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Disord.</title><idno type="ISSN">0885-3185</idno><idno type="eISSN">1531-8257</idno><imprint><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><pubPlace>New York</pubPlace><date type="published" when="2003-03">2003-03</date><biblScope unit="vol">18</biblScope><biblScope unit="issue">3</biblScope><biblScope unit="page" from="331">331</biblScope><biblScope unit="page" to="336">336</biblScope></imprint><idno type="ISSN">0885-3185</idno></series><idno type="istex">70430785ED156B34C0ACCA359B6FFCFE07F3337E</idno><idno type="DOI">10.1002/mds.10347</idno><idno type="ArticleID">MDS10347</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>ALS</term><term>Aged</term><term>Amyloidosis</term><term>Case study</term><term>Cerebral Amyloid Angiopathy (complications)</term><term>Cerebral Amyloid Angiopathy (diagnosis)</term><term>Cerebrovascular disease</term><term>Diagnosis, Differential</term><term>Differential diagnostic</term><term>Elderly</term><term>Fatal Outcome</term><term>Female</term><term>Humans</term><term>Immunohistochemistry</term><term>MND</term><term>Motor Neuron Disease (complications)</term><term>Motor Neuron Disease (diagnosis)</term><term>Motor neuron disease</term><term>Protein</term><term>Steele‐Richardson‐Olszewski</term><term>Supranuclear Palsy, Progressive (diagnosis)</term><term>Supranuclear Palsy, Progressive (etiology)</term><term>Supranuclear ophthalmoplegia</term><term>Syndrome</term><term>cerebral amyloid angiopathy</term><term>motor neurone disease</term><term>progressive supranuclear palsy</term></keywords><keywords scheme="MESH" qualifier="complications" xml:lang="en"><term>Cerebral Amyloid Angiopathy</term><term>Motor Neuron Disease</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Cerebral Amyloid Angiopathy</term><term>Motor Neuron Disease</term><term>Supranuclear Palsy, Progressive</term></keywords><keywords scheme="MESH" qualifier="etiology" xml:lang="en"><term>Supranuclear Palsy, Progressive</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Aged</term><term>Diagnosis, Differential</term><term>Fatal Outcome</term><term>Female</term><term>Humans</term><term>Syndrome</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Amyloïdose</term><term>Cérébrovasculaire pathologie</term><term>Diagnostic différentiel</term><term>Etude cas</term><term>Femelle</term><term>Immunohistochimie</term><term>Neurone moteur maladie</term><term>Ophtalmoplégie supranucléaire</term><term>Personne âgée</term><term>Protéine</term></keywords><keywords scheme="Wicri" type="topic" xml:lang="fr"><term>Personne âgée</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">We describe a 68‐year‐old woman who presented with falls, mild limb bradykinesia, axial rigidity, and a severe supranuclear gaze palsy, which failed to benefit from levodopa. She subsequently developed severe apraxia, progressive dysarthria, dysphagia, and a frontal cognitive impairment. Pyramidal weakness with fasciculations and widespread chronic partial denervation appeared shortly before her death from bronchopneumonia, 6 months after disease onset. A severe cerebral amyloid angiopathy diffusely involving the cerebral hemispheres and cerebellum was present at autopsy as well as a second pathological condition indicative of motor neurone disease. Cerebral amyloid angiopathy may rarely present with a progressive supranuclear palsy–like phenotype.</div></front></TEI><affiliations><list><country><li>Royaume-Uni</li></country><region><li>Angleterre</li><li>Grand Londres</li></region><settlement><li>Londres</li></settlement><orgName><li>National Hospital for Neurology and Neurosurgery</li></orgName></list><tree><country name="Royaume-Uni"><region name="Angleterre"><name sortKey="Weeks, Robert A" sort="Weeks, Robert A" uniqKey="Weeks R" first="Robert A." last="Weeks">Robert A. Weeks</name></region><name sortKey="Carroll, Camille" sort="Carroll, Camille" uniqKey="Carroll C" first="Camille" last="Carroll">Camille Carroll</name><name sortKey="Husain, Masud" sort="Husain, Masud" uniqKey="Husain M" first="Masud" last="Husain">Masud Husain</name><name sortKey="Lees, Andrew J" sort="Lees, Andrew J" uniqKey="Lees A" first="Andrew J." last="Lees">Andrew Lees (neurologue)</name><name sortKey="Lees, Andrew J" sort="Lees, Andrew J" uniqKey="Lees A" first="Andrew J." last="Lees">Andrew Lees (neurologue)</name><name sortKey="Rudge, Peter" sort="Rudge, Peter" uniqKey="Rudge P" first="Peter" last="Rudge">Peter Rudge</name><name sortKey="Scaravilli, Francisco" sort="Scaravilli, Francisco" uniqKey="Scaravilli F" first="Francisco" last="Scaravilli">Francisco Scaravilli</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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