Sensory involvement in X-linked spino-bulbar muscular atrophy (Kennedy's syndrome): An electrophysiological study

Journal of Neurology

Volumn 243, Issue 5, 1996, Pages 388-392

  Polo, Alberto ^a   Teatini, Francesco ^a   D'Anna, Sebastiano ^b   Manganotti, Paolo ^a   Salviati, Alessandro ^a   Dallapiccola, Bruno ^c   Zanette, Giampietro ^a   Rizzuto, Nicolò ^a  

^a POLICLINICO BORGO ROMA   (Italy)

^b City Hospital   (Italy)

^c UNIVERSITY OF ROME TOR VERGATA   (Italy)

Author keywords

Neuronopathy; Somatosensory evoked potentials; X linked recessive spino bulbar muscular atrophy

Indexed keywords

ACTION POTENTIAL; ADULT; ARTICLE; CLINICAL ARTICLE; DISEASE CARRIER; ELECTROMYOGRAPHY; EVOKED BRAIN STEM AUDITORY RESPONSE; EVOKED SOMATOSENSORY RESPONSE; FEMALE; HUMAN; LATENT PERIOD; MALE; MOLECULAR GENETICS; MOTOR NERVE CONDUCTION; MOTOR UNIT; NERVOUS SYSTEM ELECTROPHYSIOLOGY; PRIORITY JOURNAL; SENSORY NEUROPATHY; SPINAL MUSCULAR ATROPHY;

ADULT; CARRIER STATE; ELECTROMYOGRAPHY; EVOKED POTENTIALS, AUDITORY, BRAIN STEM; EVOKED POTENTIALS, SOMATOSENSORY; FEMALE; HUMANS; LINKAGE (GENETICS); MALE; MIDDLE AGED; MUSCULAR ATROPHY, SPINAL; PEDIGREE; SYNDROME; X CHROMOSOME;

EID: 0029898097     PISSN: 03405354     EISSN: None     Source Type: Journal    
DOI: 10.1007/BF00868997     Document Type: Article

References (18)

1. Amato AA, Prior TW, Barohn RJ, Snyder P, Papp A, Mendell JR (1993) Kennedy's disease: a clinicopathologic correlation with mutations in the androgen receptor gene. Neurology 43: 791-794
2. De Ferron E, Le Roux MG, Pascal O, Moisan JP, Fève JR (1994) Etude génétique moléculaire d'une famille atteinte du syndrome de Kennedy avec une hétérozygote symptomatique. Rev Neurol (Paris) 150:854-857
3. Doyu M, Sobue G, Mukai E, Kachi T, Yasuda T, Mitsuma T, Takahashi A (1992) Severity of X-linked recessive bulbospinal neuronopathy correlates with size of tandem CAG repeat in androgen receptor gene. Ann Neurol 32: 707-710
4. Fischbeck KH, Ionasescu V, Ritter A, Ionasescu R, Davies K, Ball S, Bosch P, Burns T, Hausmanova-Petrusewicz I, Borowska J, Ringel PS Stern LZ (1986) Localization of the gene for X-linked spinal muscular atrophy. Neurology 36:1595-1598
5. Fischbeck KH, Sounders D, La Spada AA (1991) Candidate gene for X-linked spinal muscolar atrophy. In: Rowland LP (ed) Advances in neurology: Amyotrophic lateral sclerosis and other motor neuron diseases, vol 56, Raven Press, New York, pp 209-213
6. Harding AE, Thomas PK, Baraitser M, Bradbury PG, Morgan-Hughes JA, Ponsford JR (1982) X-linked recessive bulbospinal neuronopathy: a report of ten cases. J Neurol Neurosurg Psychiatry 45:1012-1019
7. Jeanmond K, Sindou M, Mauguiere F (1991) The human cervical and lumbosacral evoked electrospinogram. Data from intra-operative spinal cord surface recordings. Electroencephal Clin Neurophysiolograph 80:447-489
8. Igarashi S, Tanno Y, Onodera O, Yamazaky M, Sato S, Ishikawa A, Miyatani N, Nagashima M, Ishikawa K, Sahashi K, Ibi T, Miyatake T, Tsuji S (1992) Strong correlation between the number of CAG repeats in androgen receptor genes and the clinical onset of features of spinal and bulbar muscular atrophy. Neurology 42:2300-2302
9. Kachi T, Sobue G, Sobue I (1992) Central motor and sensory conduction in X-linked recessive bulbospinal neuropathy. J Neurol Neurosurg Psychiatry 55:394-397
10. Kennedy WR, Alter M, Sung JH (1968) Progressive proximal spinal and bulbar atrophy of late onset. A sex-linked recessive trait. Neurology 18: 671-680
11. La Spada AR, Wilson EM, Lubhan DB, Harding AE, Fischbaeck HK (1991) Androgen receptor mutation in X-linked spinal and bulbar muscular atrophy. Nature 352:77-79
12. Li M, Sobue G, Doyu M, Mukai E, Hashizume Y, Mitsuma T (1995) Primary sensory neurons in X-linked recessive bulbospinal neuronopathy: histopathology and androgen receptor gene expression. Muscle Nerve 18: 301-308
13. Richards RI, Sutherland GR (1992) Heritable unstable DNA sequences. Nature Genet 1:7-9
14. Ross CA, McInnis MG, Margolis RL, Shi-Hua Li (1993) Genes with triplet repeats: candidate mediators of neuropsychiatric disorders. Trends Neurosci 16:254-260
15. Schoenen J, Delwaide PJ, Legros JJ, Franchimont P (1979) Mototorneuropathie héréditaìre: la forme proximale de l'adulte liée au sexe (ou maladie de Kennedy). J Neurol Sci 41: 343-357
16. Sobue G, Hashizume Y, Mukai E, Hirayama M, Mtsuma T, Takahashi A (1989) X-linked recessive bulbospinal neuronopathy: a clinicopathological study. Brain 112:209-232
17. Stefanis C, Papapetroupolos T, Scarpalezos S, Lygidakis G, Panayiotopoulos CP (1975) X-linked spinal and bulbar muscular atrophy of late onset: a separate type of motor neuron disease? J Neurol Sci 24:493-503
18. Zanette G, Polo A, Gasperini M, Bertolasi L, De Grandis D (1990) Farfield and cortical somatosensory evoked potentials in motor neuron disease. Muscle Nerve 13:47-55