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Titolo:
Dystonia in corticobasal degeneration
Autore:
Vanek, Z; Jankovic, J;
Indirizzi:
Baylor Coll Med, Dept Neurol, Parkinsons Dis Ctr, Houston, TX 77030 USA Baylor Coll Med Houston TX USA 77030 nsons Dis Ctr, Houston, TX 77030 USA Baylor Coll Med, Dept Neurol, Movement Disorders Clin, Houston, TX 77030 USA Baylor Coll Med Houston TX USA 77030 isorders Clin, Houston, TX 77030 USA
Titolo Testata:
MOVEMENT DISORDERS
fascicolo: 2, volume: 16, anno: 2001,
pagine: 252 - 257
SICI:
0885-3185(200103)16:2<252:DICD>2.0.ZU;2-2
Fonte:
ISI
Lingua:
ENG
Soggetto:
BASAL GANGLIONIC DEGENERATION; PROGRESSIVE SUPRANUCLEAR PALSY; CORTICONIGRAL DEGENERATION; NEURONAL ACHROMASIA; REFLEX MYOCLONUS; LIMB;
Keywords:
corticobasal degeneration; dystonia; botulinum toxin;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
33
Recensione:
Indirizzi per estratti:
Indirizzo: Jankovic, J Baylor Coll Med, Dept Neurol, Parkinsons Dis Ctr, 6550 Fannin,Suite 1801, Houston, TX 77030 USA Baylor Coll Med 6550 Fannin,Suite 1801 Houston TX USA 77030 SA
Citazione:
Z. Vanek e J. Jankovic, "Dystonia in corticobasal degeneration", MOVEMENT D, 16(2), 2001, pp. 252-257

Abstract

OBJECTIVE: To characterize the clinical features, particularly dystonia. in patients with clinically diagnosed or pathologically proven corticobasal degeneration (CBD). BACKGROUND: Although dystonia has been reported in many neurodegenerative disorders, it has not been studied in CBD. Dystonia, often accompanied by painful rigidity and fixed contractures, is one of the most disabling features of CBD. METHODS: The medical records, imaging studies, and videotapes of 66 patients who satisfied the clinical criteria of CBD, evaluated between 1988 and 1998, were reviewed. The occurrence, nature, and distribution of dystonic features were analyzed and correlated with other features of CBD. RESULTS: Of the 66 patients with CBD, 39 (59.0%) had dystonia. The mean age at onset of initial symptoms was 63.9 years (range 44-75). In 20 (51.0%) patients, dystonic symptoms began in one arm, while 13 (33.0%) patients hadinitial leg involvement. At least one arm was affected in 36 (92.0%) dystonic patients. Although 11 (28.0%) patients had leg dystonia, the leg was the predominant site of involvement in only 1 patient. Only 12 (31.0%) patients had dystonia involving the head, neck, or trunk in the course of the disease. The diagnosis of CBD was confirmed in all 4 patients who had autopsies. CONCLUSION: In this large series of CBD patients we found that asymmetric limb dystonia, particularly affecting one arm, is a common manifestation ofCBD; dystonia may be the initial manifestation of this neurodegenerative disorder. Axial or leg dystonia. without significant involvement of an arm, is rare. There is no effective treatment for this relentless disorder, except for temporary relief of dystonia and pain, with local botulinum toxin injections. (C) 2001 Movement Disorder Society.

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Documento generato il 26/01/20 alle ore 22:21:36