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Primary progressive myoclonus of aging.

Identifieur interne : 002125 ( PubMed/Checkpoint ); précédent : 002124; suivant : 002126

Primary progressive myoclonus of aging.

Auteurs : Maria Alvarez [États-Unis] ; John N. Caviness

Source :

Movement disorders : official journal of the Movement Disorder Society [ 1531-8257 ] ; 2008.

RBID : pubmed:18709679

English descriptors

KwdEn :
- Aged, Aged, 80 and over, Aging, Brain (pathology), Female, Humans, Magnetic Resonance Imaging, Male, Myoclonic Epilepsies, Progressive (metabolism), Myoclonic Epilepsies, Progressive (pathology), Myoclonic Epilepsies, Progressive (physiopathology), Neuropsychological Tests, Retrospective Studies, Tomography, X-Ray Computed.
MESH :
- metabolism : Myoclonic Epilepsies, Progressive.
- pathology : Brain, Myoclonic Epilepsies, Progressive.
- physiopathology : Myoclonic Epilepsies, Progressive.
- Aged, Aged, 80 and over, Aging, Female, Humans, Magnetic Resonance Imaging, Male, Neuropsychological Tests, Retrospective Studies, Tomography, X-Ray Computed.

Abstract

Myoclonus in older individuals usually occurs in the context of associated neurologic features which allow the diagnosis of the underlying disorder. We encountered 7 patients with a newly recognized myoclonus syndrome; we use the term primary progressive myoclonus of aging (PPMA) for this syndrome. Our purpose was to characterize the clinical and electrophysiological properties of this syndrome. Our database was searched for the presence of "myoclonus" in the physical examination. Medical records and laboratory data were retrospectively reviewed, including electrophysiology data. We applied our criteria for PPMA: (1) asymmetric symptomatic action myoclonus, (2) >/=65 years of age, (3) cortical myoclonus physiology, (4) no dementia, (5) no associated features of defined neurodegenerative disorders, and (6) no secondary cause found. Seven patients fulfilled criteria. Age at presentation ranged from 70 to 87 years. Mean duration from myoclonus onset to last follow-up was 2.9 years. Electrophysiology showed positive-negative back-averaged transients, consistent with cortical myoclonus. No patient demonstrated dementia. Brain imaging in all cases was unremarkable. PPMA is a unique syndrome with characteristic findings that differentiate it from dementias and defined neurodegenerative syndromes. It is important to distinguish primary PPMA from other syndromes seen in older individuals to avoid diagnostic confusion. Some cases showed a response to levetiracetam.

DOI: 10.1002/mds.22085
PubMed: 18709679

Affiliations:

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pubmed:18709679

Le document en format XML

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<author><name sortKey="Alvarez, Maria" sort="Alvarez, Maria" uniqKey="Alvarez M" first="Maria" last="Alvarez">Maria Alvarez</name>
<affiliation wicri:level="2"><nlm:affiliation>Department of Neurology, Wilford Hall Medical Center, San Antonio, Texas, USA.</nlm:affiliation>
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<term>Myoclonic Epilepsies, Progressive (metabolism)</term>
<term>Myoclonic Epilepsies, Progressive (pathology)</term>
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<front><div type="abstract" xml:lang="en">Myoclonus in older individuals usually occurs in the context of associated neurologic features which allow the diagnosis of the underlying disorder. We encountered 7 patients with a newly recognized myoclonus syndrome; we use the term primary progressive myoclonus of aging (PPMA) for this syndrome. Our purpose was to characterize the clinical and electrophysiological properties of this syndrome. Our database was searched for the presence of "myoclonus" in the physical examination. Medical records and laboratory data were retrospectively reviewed, including electrophysiology data. We applied our criteria for PPMA: (1) asymmetric symptomatic action myoclonus, (2) >/=65 years of age, (3) cortical myoclonus physiology, (4) no dementia, (5) no associated features of defined neurodegenerative disorders, and (6) no secondary cause found. Seven patients fulfilled criteria. Age at presentation ranged from 70 to 87 years. Mean duration from myoclonus onset to last follow-up was 2.9 years. Electrophysiology showed positive-negative back-averaged transients, consistent with cortical myoclonus. No patient demonstrated dementia. Brain imaging in all cases was unremarkable. PPMA is a unique syndrome with characteristic findings that differentiate it from dementias and defined neurodegenerative syndromes. It is important to distinguish primary PPMA from other syndromes seen in older individuals to avoid diagnostic confusion. Some cases showed a response to levetiracetam.</div>
</front>
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<DateCompleted><Year>2009</Year>
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<Title>Movement disorders : official journal of the Movement Disorder Society</Title>
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<Abstract><AbstractText>Myoclonus in older individuals usually occurs in the context of associated neurologic features which allow the diagnosis of the underlying disorder. We encountered 7 patients with a newly recognized myoclonus syndrome; we use the term primary progressive myoclonus of aging (PPMA) for this syndrome. Our purpose was to characterize the clinical and electrophysiological properties of this syndrome. Our database was searched for the presence of "myoclonus" in the physical examination. Medical records and laboratory data were retrospectively reviewed, including electrophysiology data. We applied our criteria for PPMA: (1) asymmetric symptomatic action myoclonus, (2) >/=65 years of age, (3) cortical myoclonus physiology, (4) no dementia, (5) no associated features of defined neurodegenerative disorders, and (6) no secondary cause found. Seven patients fulfilled criteria. Age at presentation ranged from 70 to 87 years. Mean duration from myoclonus onset to last follow-up was 2.9 years. Electrophysiology showed positive-negative back-averaged transients, consistent with cortical myoclonus. No patient demonstrated dementia. Brain imaging in all cases was unremarkable. PPMA is a unique syndrome with characteristic findings that differentiate it from dementias and defined neurodegenerative syndromes. It is important to distinguish primary PPMA from other syndromes seen in older individuals to avoid diagnostic confusion. Some cases showed a response to levetiracetam.</AbstractText>
<CopyrightInformation>(c) 2007 Movement Disorder Society.</CopyrightInformation>
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	Movement Disorders (revue)
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Movement Disorders (revue)

Primary progressive myoclonus of aging.

Primary progressive myoclonus of aging.

Source :

English descriptors

Abstract

Links toward previous steps (curation, corpus...)

Links to Exploration step

Le document en format XML

Pour manipuler ce document sous Unix (Dilib)

Pour mettre un lien sur cette page dans le réseau Wicri

Pour générer des pages wiki