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Titolo:
Primary lateral sclerosis: clinical, neurophysiological, and magnetic resonance findings
Autore:
Kuipers-Upmeijer, J; de Jager, AEJ; Hew, JM; Snoek, JW; van Weerden, TW;
Indirizzi:
Univ Groningen Hosp, Dept Neurol, NL-9700 RB Groningen, Netherlands Univ Groningen Hosp Groningen Netherlands NL-9700 RB ningen, Netherlands Nij Smillinghe Hosp, Dept Neurol, Drachten, Netherlands Nij Smillinghe Hosp Drachten Netherlands Neurol, Drachten, Netherlands Univ Groningen Hosp, Dept Neuroradiol, Groningen, Netherlands Univ Groningen Hosp Groningen Netherlands adiol, Groningen, Netherlands Martini Hosp, Dept Neurol, Groningen, Netherlands Martini Hosp GroningenNetherlands Dept Neurol, Groningen, Netherlands
Titolo Testata:
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY
fascicolo: 5, volume: 71, anno: 2001,
pagine: 615 - 620
SICI:
0022-3050(200111)71:5<615:PLSCNA>2.0.ZU;2-0
Fonte:
ISI
Lingua:
ENG
Soggetto:
MOTOR-NEURON DISEASE; SOMATOSENSORY-EVOKED POTENTIALS; PYRAMIDAL TRACT; FEATURES; PARAPROTEINEMIA; STIMULATION; SPASTICITY; RESPONSES; PROTEIN;
Keywords:
primary lateral sclerosis; motor neuron disease;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
30
Recensione:
Indirizzi per estratti:
Indirizzo: de Jager, AEJ Univ Groningen Hosp, Dept Neurol, POB 30-001, NL-9700 RB Groningen, Netherlands Univ Groningen Hosp POB 30-001 Groningen Netherlands NL-9700 RB
Citazione:
J. Kuipers-Upmeijer et al., "Primary lateral sclerosis: clinical, neurophysiological, and magnetic resonance findings", J NE NE PSY, 71(5), 2001, pp. 615-620

Abstract

Objective-To describe the clinical, neurophysiological, and MRI findings in 10 patients with primary lateral sclerosis (PLS). Results-The course of the disease was very slowly progressive. Spasticity due to upper motor neuron dysfunction was the most prominent sign, but EMG showed slight lower motor neuron signs, such as a mixed pattern on maximal voluntary contraction and enlarged motor unit potentials. One patient had clinically mild lower motor neuron involvement. Central motor conduction times (CMCT) were more prolonged in PLS than is the case in ALS. Minor sensorysigns were found on neurophysiological examination, comparable with those in ALS. In four patients serum creatine kinase activity was raised. On MRI cortical atrophy was seen, most pronounced in the precentral gyrus and expanding into the parietal-occipital region. Conclusions-PLS is a distinct clinical syndrome, part of the range of motor neuron diseases. Besides pronounced upper motor neuron symptoms, mild lower motor neuron symptoms can also be found, as well as (subclinical) sensory symptoms. PLS can be distinguished from ALS by its slow clinical course, a severely prolonged MEP, and a more extensive focal cortical atrophy.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 27/11/20 alle ore 13:49:58