Epidemiological survey of X-linked bulbar and spinal muscular atrophy, or Kennedy disease, in the province of Reggio Emilia, Italy

European Journal of Epidemiology

Volumn 17, Issue 6, 2001, Pages 587-591

  Guidetti, D ^a   Sabadini, R ^a   Ferlini, A ^b   Torrente, I ^c  

^a S MARIA NUOVA HOSPITAL   (Italy)

^b S ANNA HOSPITAL   (Italy)

^c CASA SOLLIEVO DELLA SOFFERENZA HOSPITAL   (Italy)

Author keywords

Epidemiology; Incidence; Kennedy disease; Prevalence; X linked spinal and bulbar muscular atrophy (X BSMA)

Indexed keywords

ADULT; AGED; AMYOTROPHIC LATERAL SCLEROSIS; ARTICLE; CLINICAL ARTICLE; CLINICAL FEATURE; DIAGNOSTIC ACCURACY; FAMILIAL DISEASE; FEMALE; HUMAN; HUMAN TISSUE; INCIDENCE; ITALY; KENNEDY DISEASE; MALE; MORTALITY; MUSCLE BIOPSY; ONSET AGE; PREVALENCE; SEX DIFFERENCE; SURVIVAL TIME; SYMPTOM;

ADULT; AGED; AMYOTROPHIC LATERAL SCLEROSIS; BIOPSY; ELECTROMYOGRAPHY; FEMALE; HUMANS; ITALY; LINKAGE (GENETICS); MALE; MIDDLE AGED; MUSCULAR ATROPHY, SPINAL; NEUROLOGIC EXAMINATION; PEDIGREE; X CHROMOSOME;

EID: 0035725264     PISSN: 03932990     EISSN: None     Source Type: Journal    
DOI: 10.1023/A:1014580219761     Document Type: Article

References (23)

1. Progressive proximal spinal and bulbar atrophy of late onset. A sex linked recessive trait
2. Hereditary proximal spinal and bulbar neuron disease of late onset
3. Kennedy disease in an Italian kindred
4. X-linked bulbar and spinal muscular atrophy, or Kennedy disease: Clinical, neurophysiological, neuropathological, neuropsychological and molecular study of a large family
5. Subclinical phenotypic expression in heterozygous females of X-linked recessive bulbospinal neuronopathy
6. Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy
7. Clinical and molecular aspects of androgen receptor defects
8. Meiotic stability and genotype-phenotype correlation of the trinucleotiderepeat in X-linked spinal and bulbar muscular atrophy
9. Molecular analysis of the androgen receptor gene in Kennedy's disease
10. Nuclear inclusions of the androgen receptor protein in spinal and bulbar muscular atrophy
11. Nonneural nuclear inclusions of androgen receptor protein in spinal and bulbar muscular atrophy
12. Kennedy's disease: Caspase cleavage of the androgen receptor is a crucial event in cytotoxicity
13. Caspase-3 cleaves the expanded androgen receptor protein of spinal and bulbar muscular atrophy in a polyglutamine repeat length-dependent manner
14. The rediction of androgen receptor mRNA in motoneurons of X-linked spinal and bulbar muscular atrophy
15. The lateral corticospinal tract and spinal ventral horn in X-linked recessive spinal and bulbar muscular atrophy: A quantitative study
16. Primary sensory neurons in X-linked recessive bulbospinal neuronopathy: Histopathology and androgen receptor gene expression
17. Epidemiological survey of amyotrophic lateral sclerosis in the province of Reggio Emilia, Italy: Influence of environmental exposure to lead
18. Androgen receptor gene CAG repeat analysis in the differential diagnosis between Kennedy disease and other motoneuron disease
19. Calculating confidence intervals for rates and ratios. Simplified method utilizing tabular values based on the poisson distribution
20. Spinobulbar muscular atrophy can mimic ALS: The importance of genetic testing in male patients with atypical ALS
21. Atypical clinical presentations of X-linked spinal and bulbar muscular atrophy in patients with mild CAG expansion in androgen receptor gene
22. X-linked spinal and bulbar muscular atrophy with myasthenic symptoms