Pilot trial of clenbuterol in spinal and bulbar muscular atrophy

Neurology

Volumn 80, Issue 23, 2013, Pages 2095-2098

  Querin, Giorgia ^a   D'Ascenzo, Carla ^a   Peterle, Enrico ^a   Ermani, Mario ^a   Bello, Luca ^a   Melacini, Paola ^b   Morandi, Lucia ^c   Mazzini, Letizia ^d   Silani, Vincenzo ^e   Raimondi, Monika ^f   Mandrioli, Jessica ^g   Romito, Silvia ^h   Angelini, Corrado ^a   Pegoraro, Elena ^a   Sorarù, Gianni ^a  

^a Neuromuscular Center   (Italy)

^b UNIVERSITY OF PADOVA   (Italy)

^c DEPARTMENT OF NEUROSURGERY   (Italy)

^d UNIVERSITY OF EASTERN PIEDMONT   (Italy)

^e UNIVERSITY OF MILAN   (Italy)

^f REGIONAL HOSPITAL OF LUGANO   (Switzerland)

^g UNIVERSITY OF MODENA AND REGGIO EMILIA   (Italy)

^h UNIVERSITY HOSPITAL OF VERONA   (Italy)

Author keywords

[No Author keywords available]

Indexed keywords

CLENBUTEROL;

ADULT; AGED; AMYOTROPHIC LATERAL SCLEROSIS FUNCTIONAL RATING SCALE; ARTICLE; BLOOD PRESSURE; CLINICAL ARTICLE; COHORT ANALYSIS; CONTROLLED STUDY; CREATINE KINASE BLOOD LEVEL; DRUG DOSE REDUCTION; DRUG EFFICACY; DRUG SAFETY; DRUG TOLERABILITY; FOLLOW UP; FORCED VITAL CAPACITY; HAND TREMOR; HEART PALPITATION; HEART RATE; HEART RHYTHM; HUMAN; KENNEDY DISEASE; MUSCLE CRAMP; MUSCLE STRENGTH; NAMED INVENTORIES, QUESTIONNAIRES AND RATING SCALES; PILOT STUDY; PRIORITY JOURNAL; RISK ASSESSMENT; SIDE EFFECT; WALKING;

ACTIVITIES OF DAILY LIVING; ADRENERGIC BETA-AGONISTS; ADULT; AGED; BULBO-SPINAL ATROPHY, X-LINKED; CLENBUTEROL; EXERCISE TEST; FEMALE; FOLLOW-UP STUDIES; HUMANS; MALE; MIDDLE AGED; MUSCLE STRENGTH; PILOT PROJECTS; SEVERITY OF ILLNESS INDEX; TREATMENT OUTCOME; WALKING;

EID: 84879079114     PISSN: 00283878     EISSN: 1526632X     Source Type: Journal    
DOI: 10.1212/WNL.0b013e318295d766     Document Type: Article

References (10)

1. La Spada AR, Wilson EM, Lubahn DB, Harding AE, Fischbeck KH. Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy. Nature 1991;352:77-79.
2. Katsuno M, Tanaka F, Adachi H, et al. Pathogenesis and therapy of spinal and bulbar muscular atrophy (SBMA). Prog Neurobiol 2012;99:246-256.
3. Palazzolo I, Burnett BG, Young JE, et al. Akt blocks ligand binding and protects against expanded polyglutamine androgen receptor toxicity. Hum Mol Genet 2007;16:1593-1603.
4. Lynch GS, Ryall JG. Role of b-adrenoceptor signalling in skeletal muscle: implications for muscle wasting and disease. Physiol Rev 2008;88:729-767.
5. Takeuchi Y, Katsuno M, Banno H, et al. Walking capacity evaluated by the 6-Minute Walk Test in spinal and bulbar muscular atrophy. Muscle Nerve 2008;38:964-971.
6. ATS Committee on Proficiency Standards for Clinical Pulmonary Function Laboratories. ATS statement: guidelines for the Six-Minute Walk Test. Am J Respir Crit Care Med 2002;166:111-117.
7. Cedarbaum JM, Stambler N, Malta E, et al. The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. J Neurol Sci 1999;169:13-21.
8. Cepero M, Perez-Pertejo Y, Cubrìa JC, et al. Muscle and serum changes with salbutamol administration in aerobically exercised rats. Comp Biochem Physiol C Toxicol Pharmacol 2000;126:45-51.
9. Tomlinson B, Cruickshank JM, Hayes Y, et al. Selective betaadrenoreceptor partial agonist effects of pindolol and xamoterol on skeletal muscle assessed by plasma creatine kinase changes in healthy subjects. Br J Clin Pharmacol 1990;30:665-672.
10. Sorarù G, D'Ascenzo C, Polo A, et al. Spinal and bulbar muscular atrophy: skeletal muscle pathology in male patients and heterozygous females. J Neurol Sci 2008;264:100-105.