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Titolo:
Atypical motor neuron disease and related motor syndromes
Autore:
Verma, A; Bradley, WG;
Indirizzi:
Univ Miami, Sch Med D45, Dept Neurol, Miami, FL 33136 USA Univ Miami Miami FL USA 33136 h Med D45, Dept Neurol, Miami, FL 33136 USA
Titolo Testata:
SEMINARS IN NEUROLOGY
fascicolo: 2, volume: 21, anno: 2001,
pagine: 177 - 187
SICI:
0271-8235(2001)21:2<177:AMNDAR>2.0.ZU;2-X
Fonte:
ISI
Lingua:
ENG
Soggetto:
AMYOTROPHIC-LATERAL-SCLEROSIS; HEREDITARY SPASTIC PARAPLEGIAS; JUVENILE MUSCULAR-ATROPHY; FRONTOTEMPORAL DEMENTIA; PARALYTIC POLIOMYELITIS; NEUROLOGICAL DISORDERS; MOLECULAR-GENETICS; CONDUCTION BLOCK; PRION DISEASES; PARKINSONISM;
Keywords:
atypical amyotrophic lateral sclerosis; motor neuron disease; progressive muscular atrophy; spinal muscular atrophy; postpolio muscular atrophy; monomelic amyotrophy; lathyrism; dementia;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
90
Recensione:
Indirizzi per estratti:
Indirizzo: Bradley, WG Univ Miami, Sch Med D45, Dept Neurol, 1501 NW 9th Ave, Miami, FL 33136 USA Univ Miami 1501 NW 9th Ave Miami FL USA 33136 mi, FL 33136 USA
Citazione:
A. Verma e W.G. Bradley, "Atypical motor neuron disease and related motor syndromes", SEM NEUROL, 21(2), 2001, pp. 177-187

Abstract

There is an imperative need for the early diagnosis of amyotrophic lateralsclerosis/motor neuron disease (ALS/MND) in the current era of emerging treatments. When evaluating the patient with ALS/MND, the neurologist must consider a number of other motor neuron disorders and related motor syndromesthat may have clinical features resembling ALS/MND, The revised Airlie House-El Escorial diagnostic criteria have been established through the consensus of experts meeting at workshops. However, by definition, using these criteria a patient is likely to have fairly advanced disease at the time of adefinitive ALS/MND diagnosis. The reasons for the difficulty in making an early ALS/MND diagnosis are several. No surrogate diagnostic marker currently exists for ALS/MND, ALS/MND at its onset is heterogeneous in clinical presentation, its clinical course is variable, and several clinical variants are recognized. In addition, certain motor syndromes, such as monomelic amyotrophy, postpolio muscular atrophy, and multifocal motor neuropathy, can clinically mimic ALS/MND. Therefore, not only may the diagnosis of ALS/MND be clinically missed in the early stages, but worse, the patient may be wrongly labeled as having ALS/MND, The diagnosis of ALS/MND requires a combination of upper motor neuron (UMN) and lower motor neuron (LMN) involvement. Motor syndromes in which the deficit is restricted to the UMN or LMN throughthe entire course of the disease are described as atypical MND in this review Approximately 5% of patients with ALS/MND have overt dementia with a characteristic frontal affect. ALS/MND with parkinsonism and dementia is rareoutside the western Pacific region. The clinical course of motor disorder in these overlap syndromes does not differ from that in typical ALS/MND.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 17/09/19 alle ore 23:13:25