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Volumn 69, Issue 19, 2007, Pages 1828-1835

Pathophysiologic insights into motor axonal function in Kennedy disease

Author keywords

[No Author keywords available]

Indexed keywords

ABDUCTOR POLLICIS BREVIS; ACTION POTENTIAL; ADULT; AGED; ARTICLE; CLINICAL ARTICLE; CONTROLLED STUDY; CORRELATION ANALYSIS; FEMALE; HUMAN; KENNEDY DISEASE; MALE; MOTOR PERFORMANCE; MUSCLE; NERVE EXCITABILITY; NERVE FIBER; PATHOPHYSIOLOGY; PERIPHERAL NERVE; PRIORITY JOURNAL; STATISTICAL SIGNIFICANCE;

EID: 35948960336     PISSN: 00283878     EISSN: None     Source Type: Journal    
DOI: 10.1212/01.wnl.0000279521.81846.59     Document Type: Article
Times cited : (23)

References (58)
  • 1
    • 0014310582 scopus 로고
    • Progressive proximal spinal and bulbar muscular atrophy of late onset. A sex-linked recessive trait
    • Kennedy WR, Alter M, Sung JH. Progressive proximal spinal and bulbar muscular atrophy of late onset. A sex-linked recessive trait. Neurology 1968;18:671-680.
    • (1968) Neurology , vol.18 , pp. 671-680
    • Kennedy, W.R.1    Alter, M.2    Sung, J.H.3
  • 2
  • 5
    • 0025735846 scopus 로고
    • Clinical and electrodiagnostic features of X-linked recessive bulbospinal neuronopathy
    • Olney RK, Aminoff MJ, So YT. Clinical and electrodiagnostic features of X-linked recessive bulbospinal neuronopathy. Neurology 1991;41:823-828.
    • (1991) Neurology , vol.41 , pp. 823-828
    • Olney, R.K.1    Aminoff, M.J.2    So, Y.T.3
  • 6
    • 0033646625 scopus 로고    scopus 로고
    • Complex fasciculations and their origin in amyotrophic lateral sclerosis and Kennedy's disease
    • Hirota N, Eisen A, Weber M. Complex fasciculations and their origin in amyotrophic lateral sclerosis and Kennedy's disease. Muscle Nerve 2000;23:1872-1875.
    • (2000) Muscle Nerve , vol.23 , pp. 1872-1875
    • Hirota, N.1    Eisen, A.2    Weber, M.3
  • 7
    • 0032737074 scopus 로고    scopus 로고
    • Distinguishing clinical and electrodiagnostic features of X-linked bulbospinal neuronopathy
    • Meriggioli MN, Rowin J, Sanders DB. Distinguishing clinical and electrodiagnostic features of X-linked bulbospinal neuronopathy. Muscle Nerve 1999;22:1693-1697.
    • (1999) Muscle Nerve , vol.22 , pp. 1693-1697
    • Meriggioli, M.N.1    Rowin, J.2    Sanders, D.B.3
  • 9
    • 0028296724 scopus 로고
    • X-linked recessive bulbospinal neuronopathy (Kennedy's syndrome): A neurophysiological study
    • Trojaborg W, Wulff CH. X-linked recessive bulbospinal neuronopathy (Kennedy's syndrome): a neurophysiological study. Acta Neurol Scand 1994;89:214-219.
    • (1994) Acta Neurol Scand , vol.89 , pp. 214-219
    • Trojaborg, W.1    Wulff, C.H.2
  • 10
    • 0031035995 scopus 로고    scopus 로고
    • The characteristic electrodiagnostic features of Kennedy's disease
    • Ferrante MA, Wilbourn AJ. The characteristic electrodiagnostic features of Kennedy's disease. Muscle Nerve 1997;20:323-329.
    • (1997) Muscle Nerve , vol.20 , pp. 323-329
    • Ferrante, M.A.1    Wilbourn, A.J.2
  • 11
    • 0020070530 scopus 로고
    • Pathophysiology of fasciculations in ALS as studied by electromyography of single motor units
    • Conradi S, Grimby L, Lundemo G. Pathophysiology of fasciculations in ALS as studied by electromyography of single motor units. Muscle Nerve 1982;5:202-208.
    • (1982) Muscle Nerve , vol.5 , pp. 202-208
    • Conradi, S.1    Grimby, L.2    Lundemo, G.3
  • 13
    • 0028046650 scopus 로고
    • The origin of muscle fasciculations and cramps
    • Layzer RB. The origin of muscle fasciculations and cramps. Muscle Nerve 1994;17:1243-1249.
    • (1994) Muscle Nerve , vol.17 , pp. 1243-1249
    • Layzer, R.B.1
  • 14
    • 0020463456 scopus 로고
    • The origin of fasciculations
    • Roth G. The origin of fasciculations. Ann Neurol 1982;12:542-547.
    • (1982) Ann Neurol , vol.12 , pp. 542-547
    • Roth, G.1
  • 15
    • 0021272423 scopus 로고
    • Fasciculations and their F-response. Localisation of their axonal origin
    • Roth G. Fasciculations and their F-response. Localisation of their axonal origin. J Neurol Sci 1984;63:299-306.
    • (1984) J Neurol Sci , vol.63 , pp. 299-306
    • Roth, G.1
  • 16
    • 0018341370 scopus 로고
    • The origin of fasciculations in motoneuron disease
    • Wettstein A. The origin of fasciculations in motoneuron disease. Ann Neurol 1979;5:295-300.
    • (1979) Ann Neurol , vol.5 , pp. 295-300
    • Wettstein, A.1
  • 17
    • 15044345732 scopus 로고    scopus 로고
    • Threshold electronus in the assessment of motor neuron disease
    • Mauguiere F, ed, Amsterdam: Elsevier
    • Kiernan MC, Burke D. Threshold electronus in the assessment of motor neuron disease. In: Mauguiere F, ed. Handbook of clinical neurophysiology. Amsterdam: Elsevier, 2004.
    • (2004) Handbook of clinical neurophysiology
    • Kiernan, M.C.1    Burke, D.2
  • 18
    • 0031898458 scopus 로고    scopus 로고
    • Strength-duration properties of sensory and motor axons in amyotrophic lateral sclerosis
    • Mogyoros I, Kiernan MC, Burke D, Bostock H. Strength-duration properties of sensory and motor axons in amyotrophic lateral sclerosis. Brain 1998;121:851-859.
    • (1998) Brain , vol.121 , pp. 851-859
    • Mogyoros, I.1    Kiernan, M.C.2    Burke, D.3    Bostock, H.4
  • 19
    • 33645075174 scopus 로고    scopus 로고
    • Altered axonal excitability properties in amyotrophic lateral sclerosis: Impaired potassium channel function related to disease stage
    • Kanai K, Kuwabara S, Misawa S, et al. Altered axonal excitability properties in amyotrophic lateral sclerosis: impaired potassium channel function related to disease stage. Brain 2006;129:953-962.
    • (2006) Brain , vol.129 , pp. 953-962
    • Kanai, K.1    Kuwabara, S.2    Misawa, S.3
  • 20
    • 33745418427 scopus 로고    scopus 로고
    • Axonal excitability properties in amyotrophic lateral sclerosis
    • Vucic S, Kiernan M. Axonal excitability properties in amyotrophic lateral sclerosis. Clin Neurophysiol 2006;117:1458-1466.
    • (2006) Clin Neurophysiol , vol.117 , pp. 1458-1466
    • Vucic, S.1    Kiernan, M.2
  • 21
    • 0032777485 scopus 로고    scopus 로고
    • Strength-duration properties of peripheral nerve in acquired neuromyotonia
    • Maddison P, Newsom-Davis J, Mills KR. Strength-duration properties of peripheral nerve in acquired neuromyotonia. Muscle Nerve 1999;22:823-830.
    • (1999) Muscle Nerve , vol.22 , pp. 823-830
    • Maddison, P.1    Newsom-Davis, J.2    Mills, K.R.3
  • 22
    • 0032703182 scopus 로고    scopus 로고
    • Potassium current suppression in patients with peripheral nerve hyperexcitability
    • Nagado T, Arimura K, Sonoda Y, et al. Potassium current suppression in patients with peripheral nerve hyperexcitability. Brain 1999;122:2057-2066.
    • (1999) Brain , vol.122 , pp. 2057-2066
    • Nagado, T.1    Arimura, K.2    Sonoda, Y.3
  • 23
    • 0035136014 scopus 로고    scopus 로고
    • Excitability properties of motor axons in patients with spontaneous motor unit activity
    • Kiernan MC, Hart IK, Bostock H. Excitability properties of motor axons in patients with spontaneous motor unit activity. J Neurol Neurosurg Psychiatry 2001;70:56-64.
    • (2001) J Neurol Neurosurg Psychiatry , vol.70 , pp. 56-64
    • Kiernan, M.C.1    Hart, I.K.2    Bostock, H.3
  • 24
    • 0030700891 scopus 로고    scopus 로고
    • Rethinking genotype and phenotype correlations in polyglutamine expansion disorders
    • Andrew SE, Goldberg YP, Hayden MR. Rethinking genotype and phenotype correlations in polyglutamine expansion disorders. Hum Mol Genet 1997;6:2005-2010.
    • (1997) Hum Mol Genet , vol.6 , pp. 2005-2010
    • Andrew, S.E.1    Goldberg, Y.P.2    Hayden, M.R.3
  • 26
    • 0034005355 scopus 로고    scopus 로고
    • Multiple measures of axonal excitability: A new approach in clinical testing
    • Kiernan MC, Burke D, Andersen KV, Bostock H. Multiple measures of axonal excitability: a new approach in clinical testing. Muscle Nerve 2000;23:399-409.
    • (2000) Muscle Nerve , vol.23 , pp. 399-409
    • Kiernan, M.C.1    Burke, D.2    Andersen, K.V.3    Bostock, H.4
  • 27
    • 0031914185 scopus 로고    scopus 로고
    • Threshold tracking techniques in the study of human peripheral nerve
    • Bostock H, Cikurel K, Burke D. Threshold tracking techniques in the study of human peripheral nerve. Muscle Nerve 1998;21:137-158.
    • (1998) Muscle Nerve , vol.21 , pp. 137-158
    • Bostock, H.1    Cikurel, K.2    Burke, D.3
  • 28
    • 0000004378 scopus 로고
    • Sur la possibilité de rendre comparables entre eux les appareils servant l'excitation électrique
    • Weiss G. Sur la possibilité de rendre comparables entre eux les appareils servant l'excitation électrique. Arch Ital Biol 1901;35:413-446.
    • (1901) Arch Ital Biol , vol.35 , pp. 413-446
    • Weiss, G.1
  • 29
    • 0029874995 scopus 로고    scopus 로고
    • Strength-duration properties of human peripheral nerve
    • Mogyoros I, Kiernan MC, Burke D. Strength-duration properties of human peripheral nerve. Brain 1996;119:439-447.
    • (1996) Brain , vol.119 , pp. 439-447
    • Mogyoros, I.1    Kiernan, M.C.2    Burke, D.3
  • 31
    • 0035049255 scopus 로고    scopus 로고
    • Effects of temperature on the excitability properties of human motor axons
    • Kiernan MC, Cikurel K, Bostock H. Effects of temperature on the excitability properties of human motor axons. Brain 2001;124:816-825.
    • (2001) Brain , vol.124 , pp. 816-825
    • Kiernan, M.C.1    Cikurel, K.2    Bostock, H.3
  • 32
    • 0031037308 scopus 로고    scopus 로고
    • Latent addition in motor and sensory fibres of human peripheral nerve
    • Bostock H, Rothwell JC. Latent addition in motor and sensory fibres of human peripheral nerve. J Physiol (Lond) 1997;498 (Pt 1):277-294.
    • (1997) J Physiol (Lond) , vol.498 , Issue.PART 1 , pp. 277-294
    • Bostock, H.1    Rothwell, J.C.2
  • 33
    • 0020642921 scopus 로고
    • The strength-duration relationship for excitation of myelinated nerve: Computed dependence on membrane parameters
    • Bostock H. The strength-duration relationship for excitation of myelinated nerve: computed dependence on membrane parameters. J Physiol (Lond) 1983;341:59-74.
    • (1983) J Physiol (Lond) , vol.341 , pp. 59-74
    • Bostock, H.1
  • 34
    • 0023162189 scopus 로고
    • Function and distribution of three types of rectifying channel in rat spinal root myelinated axons
    • Baker M, Bostock H, Grafe P, Martius P. Function and distribution of three types of rectifying channel in rat spinal root myelinated axons. J Physiol (Lond) 1987;383:45-67.
    • (1987) J Physiol (Lond) , vol.383 , pp. 45-67
    • Baker, M.1    Bostock, H.2    Grafe, P.3    Martius, P.4
  • 35
    • 0029871264 scopus 로고    scopus 로고
    • Queer current and pacemaker: The hyperpolarization-activated cation current in neurons
    • Pape HC. Queer current and pacemaker: the hyperpolarization-activated cation current in neurons. Annu Rev Physiol 1996;58:299-327.
    • (1996) Annu Rev Physiol , vol.58 , pp. 299-327
    • Pape, H.C.1
  • 36
    • 0023822152 scopus 로고
    • Evidence for two types of potassium channel in human motor axons in vivo
    • Bostock H, Baker M. Evidence for two types of potassium channel in human motor axons in vivo. Brain Res 1988;462:354-358.
    • (1988) Brain Res , vol.462 , pp. 354-358
    • Bostock, H.1    Baker, M.2
  • 37
    • 0029782542 scopus 로고    scopus 로고
    • Differences in the recovery of excitability in sensory and motor axons of human median nerve
    • Kiernan MC, Mogyoros I, Burke D. Differences in the recovery of excitability in sensory and motor axons of human median nerve. Brain 1996;119:1099-1105.
    • (1996) Brain , vol.119 , pp. 1099-1105
    • Kiernan, M.C.1    Mogyoros, I.2    Burke, D.3
  • 38
    • 0020005026 scopus 로고
    • Intracellular recording from vertebrate myelinated axons: Mechanism of the depolarizing afterpotential
    • Barrett EF, Barrett JN. Intracellular recording from vertebrate myelinated axons: mechanism of the depolarizing afterpotential. J Physiol (Lond) 1982;323:117-144.
    • (1982) J Physiol (Lond) , vol.323 , pp. 117-144
    • Barrett, E.F.1    Barrett, J.N.2
  • 39
    • 0027398432 scopus 로고
    • Molecular dissection of the myelinated axon
    • Waxman SG, Ritchie JM. Molecular dissection of the myelinated axon. Ann Neurol 1993;33:121-136.
    • (1993) Ann Neurol , vol.33 , pp. 121-136
    • Waxman, S.G.1    Ritchie, J.M.2
  • 40
    • 0026456689 scopus 로고
    • Severity of X-linked recessive bulbospinal neuronopathy correlates with size of the tandem CAG repeat in androgen receptor gene
    • Doyu M, Sobue G, Mukai E, et al. Severity of X-linked recessive bulbospinal neuronopathy correlates with size of the tandem CAG repeat in androgen receptor gene. Ann Neurol 1992;32:707-710.
    • (1992) Ann Neurol , vol.32 , pp. 707-710
    • Doyu, M.1    Sobue, G.2    Mukai, E.3
  • 41
    • 0027023516 scopus 로고
    • Meiotic stability and genotype-phenotype correlation of the trinucleotide repeat in X-linked spinal and bulbar muscular atrophy
    • La Spada AR, Roling DB, Harding AE, et al. Meiotic stability and genotype-phenotype correlation of the trinucleotide repeat in X-linked spinal and bulbar muscular atrophy. Nat Genet 1992;2:301-304.
    • (1992) Nat Genet , vol.2 , pp. 301-304
    • La Spada, A.R.1    Roling, D.B.2    Harding, A.E.3
  • 42
    • 0034094873 scopus 로고    scopus 로고
    • Glutamine repeats and neurodegeneration
    • Zoghbi HY, Orr HT. Glutamine repeats and neurodegeneration. Annu Rev Neurosci 2000;23:217-247.
    • (2000) Annu Rev Neurosci , vol.23 , pp. 217-247
    • Zoghbi, H.Y.1    Orr, H.T.2
  • 43
    • 77957099017 scopus 로고    scopus 로고
    • Threshold electrotonus in the assessment of motor neuron disease
    • Daube J, Mauguiere F, eds, Amsterdam: Elsevier
    • Kiernan M, Burke D. Threshold electrotonus in the assessment of motor neuron disease. In: Daube J, Mauguiere F, eds. Handbook of clinical neurophysiology. Amsterdam: Elsevier, 2004:359-366.
    • (2004) Handbook of clinical neurophysiology , pp. 359-366
    • Kiernan, M.1    Burke, D.2
  • 44
    • 0029787778 scopus 로고    scopus 로고
    • Relationships between motor-unit number estimates and isometric strength in distal muscles in ALS/MND
    • Bromberg MB, Larson WL. Relationships between motor-unit number estimates and isometric strength in distal muscles in ALS/MND. J Neurol Sci 1996;139 suppl:38-42.
    • (1996) J Neurol Sci , vol.139 , Issue.SUPPL. , pp. 38-42
    • Bromberg, M.B.1    Larson, W.L.2
  • 45
    • 0037379363 scopus 로고    scopus 로고
    • Muscle cramp in Machado-Joseph disease: Altered motor axonal excitability properties and mexiletine treatment
    • Kanai K, Kuwabara S, Arai K, Sung JY, Ogawara K, Hattori T. Muscle cramp in Machado-Joseph disease: altered motor axonal excitability properties and mexiletine treatment. Brain 2003;126 (Pt 4):965-973.
    • (2003) Brain , vol.126 , Issue.PART 4 , pp. 965-973
    • Kanai, K.1    Kuwabara, S.2    Arai, K.3    Sung, J.Y.4    Ogawara, K.5    Hattori, T.6
  • 46
    • 0029440344 scopus 로고
    • X-linked recessive bulbospinal neuronopathy (SBMA)
    • Sobue G. X-linked recessive bulbospinal neuronopathy (SBMA). Nagoya J Med Sci 1995;58:95-106.
    • (1995) Nagoya J Med Sci , vol.58 , pp. 95-106
    • Sobue, G.1
  • 47
    • 0029811875 scopus 로고    scopus 로고
    • The effect of stimulus duration on the latency of submaximal nerve volleys
    • Mogyoros I, Kiernan MC, Gracies JM, Burke D. The effect of stimulus duration on the latency of submaximal nerve volleys. Muscle Nerve 1996;19:1354-1356.
    • (1996) Muscle Nerve , vol.19 , pp. 1354-1356
    • Mogyoros, I.1    Kiernan, M.C.2    Gracies, J.M.3    Burke, D.4
  • 48
    • 0019402912 scopus 로고
    • Electrical properties of isolated demyelinated rat nerve fibres
    • Brismar T. Electrical properties of isolated demyelinated rat nerve fibres. Acta Physiol Scand 1981;113:161-166.
    • (1981) Acta Physiol Scand , vol.113 , pp. 161-166
    • Brismar, T.1
  • 49
    • 0017685107 scopus 로고
    • Androgen concentration in motor neurons of cranial nerves and spinal cord
    • Sar M, Stumpf WE. Androgen concentration in motor neurons of cranial nerves and spinal cord. Science 1977;197:77-79.
    • (1977) Science , vol.197 , pp. 77-79
    • Sar, M.1    Stumpf, W.E.2
  • 50
    • 0025232316 scopus 로고
    • Distribution of androgen and estrogen receptor mRNA-containing cells in the rat brain: An in situ hybridization study
    • Simerly RB, Chang C, Muramatsu M, Swanson LW. Distribution of androgen and estrogen receptor mRNA-containing cells in the rat brain: an in situ hybridization study. J Comp Neurol 1990;294:76-95.
    • (1990) J Comp Neurol , vol.294 , pp. 76-95
    • Simerly, R.B.1    Chang, C.2    Muramatsu, M.3    Swanson, L.W.4
  • 51
    • 0022462204 scopus 로고
    • Androgen receptor levels in cranial nerve nuclei and tongue muscles in rats
    • Yu WH, McGinnis MY. Androgen receptor levels in cranial nerve nuclei and tongue muscles in rats. J Neurosci 1986;6:1302-1307.
    • (1986) J Neurosci , vol.6 , pp. 1302-1307
    • Yu, W.H.1    McGinnis, M.Y.2
  • 52
    • 0027161059 scopus 로고
    • Up-regulation of androgen receptor immunoreactivity in the rat brain by androgenicanabolic steroids
    • Menard CS, Harlan RE. Up-regulation of androgen receptor immunoreactivity in the rat brain by androgenicanabolic steroids. Brain Res 1993;622:226-236.
    • (1993) Brain Res , vol.622 , pp. 226-236
    • Menard, C.S.1    Harlan, R.E.2
  • 53
    • 0030464919 scopus 로고    scopus 로고
    • Changes in excitability and impulse transmission following prolonged repetitive activity in normal subjects and patients with a focal nerve lesion
    • Kiernan MC, Mogyoros I, Burke D. Changes in excitability and impulse transmission following prolonged repetitive activity in normal subjects and patients with a focal nerve lesion. Brain 1996;119:2029-2037.
    • (1996) Brain , vol.119 , pp. 2029-2037
    • Kiernan, M.C.1    Mogyoros, I.2    Burke, D.3
  • 54
    • 0030895327 scopus 로고    scopus 로고
    • Excitability changes in human cutaneous afferents induced by prolonged repetitive axonal activity
    • Kiernan MC, Mogyoros I, Hales JP, Gracies JM, Burke D. Excitability changes in human cutaneous afferents induced by prolonged repetitive axonal activity. J Physiol (Lond) 1997;500 (Pt 1):255-264.
    • (1997) J Physiol (Lond) , vol.500 , Issue.PART 1 , pp. 255-264
    • Kiernan, M.C.1    Mogyoros, I.2    Hales, J.P.3    Gracies, J.M.4    Burke, D.5
  • 55
    • 4043094759 scopus 로고    scopus 로고
    • Differences in activity-dependent hyperpolarization in human sensory and motor axons
    • Kiernan MC, Lin CS, Burke D. Differences in activity-dependent hyperpolarization in human sensory and motor axons. J Physiol (Lond) 2004;558:341-349.
    • (2004) J Physiol (Lond) , vol.558 , pp. 341-349
    • Kiernan, M.C.1    Lin, C.S.2    Burke, D.3
  • 56
    • 0033635346 scopus 로고    scopus 로고
    • Effects of membrane polarization and ischaemia on the excitability properties of human motor axons
    • Kiernan MC, Bostock H. Effects of membrane polarization and ischaemia on the excitability properties of human motor axons. Brain 2000;123:2542-2551.
    • (2000) Brain , vol.123 , pp. 2542-2551
    • Kiernan, M.C.1    Bostock, H.2
  • 57
    • 0025925116 scopus 로고
    • Na(+)-Ca2+ exchanger mediates Ca2+ influx during anoxia in mammalian central nervous system white matter
    • Stys PK, Waxman SG, Ransom BR. Na(+)-Ca2+ exchanger mediates Ca2+ influx during anoxia in mammalian central nervous system white matter. Ann Neurol 1991;30:375-380.
    • (1991) Ann Neurol , vol.30 , pp. 375-380
    • Stys, P.K.1    Waxman, S.G.2    Ransom, B.R.3
  • 58
    • 0031985806 scopus 로고    scopus 로고
    • Anoxic and ischemic injury of myelinated axons in CNS white matter: From mechanistic concepts to therapeutics
    • Stys PK. Anoxic and ischemic injury of myelinated axons in CNS white matter: from mechanistic concepts to therapeutics. J Cereb Blood Flow Metab 1998;18:2-25.
    • (1998) J Cereb Blood Flow Metab , vol.18 , pp. 2-25
    • Stys, P.K.1


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