The characteristic electrodiagnostic features of Kennedy's disease

Mark Ferrante, Asa J. Wilbourn

Research output: Contribution to journalArticle

72 Citations (Scopus)

Abstract

To define the electrodiagnostic (EDX) features of Kennedy's disease, their distribution, their clinical correlation, and to determine whether they are unique to this disorder, we retrospectively evaluated the EDX and clinical features of 19 patients with Kennedy's disease and found that: (1) the percentage with sensory nerve action potential abnormalities is high (95%); (2) compound muscle action potential abnormalities are less frequent (37%) and less pronounced; (3) the needle electrode examination is always abnormal (100%), revealing acute and chronic motor axon loss, with the latter predominating; (4) the clinical onset is heterogeneous for both the site of onset (bulbar, upper extremity, lower extremity, combination) and the symptomatology (sensory, motor, sensorimotor); (5) focal onsets were reported in the majority (79%); and (6) there is a strong correlation between the clinical onset (both site and symptomatology) and the maximal EDX abnormalities. Thus, the EDX features of Kennedy's disease are consistent with a slowly progressive and very chronic degeneration of the anterior horn cells and dorsal root ganglia. Although the clinical onsets are heterogenous, the EDX features are homogenous and unique, consisting of a diffuse, very slowly progressive anterior horn cell disorder coupled with a sensory neuropathy/neuronopathy that mimics an acquired process.

Original languageEnglish (US)
Pages (from-to)323-329
Number of pages7
JournalMuscle and Nerve
Volume20
Issue number3
DOIs
StatePublished - Mar 18 1997
Externally publishedYes

Fingerprint

X-Linked Bulbo-Spinal Atrophy
Anterior Horn Cells
Action Potentials
Hospital Distribution Systems
Spinal Ganglia
Upper Extremity
Needles
Axons
Lower Extremity
Electrodes
Muscles

All Science Journal Classification (ASJC) codes

  • Clinical Neurology
  • Neuroscience(all)
  • Cellular and Molecular Neuroscience
  • Physiology (medical)
  • Physiology

Cite this

The characteristic electrodiagnostic features of Kennedy's disease. / Ferrante, Mark; Wilbourn, Asa J.

In: Muscle and Nerve, Vol. 20, No. 3, 18.03.1997, p. 323-329.

Research output: Contribution to journalArticle

Ferrante, Mark ; Wilbourn, Asa J. / The characteristic electrodiagnostic features of Kennedy's disease. In: Muscle and Nerve. 1997 ; Vol. 20, No. 3. pp. 323-329.
@article{4b48a299e05f41079ddb1f6e57996bfb,
title = "The characteristic electrodiagnostic features of Kennedy's disease",
abstract = "To define the electrodiagnostic (EDX) features of Kennedy's disease, their distribution, their clinical correlation, and to determine whether they are unique to this disorder, we retrospectively evaluated the EDX and clinical features of 19 patients with Kennedy's disease and found that: (1) the percentage with sensory nerve action potential abnormalities is high (95{\%}); (2) compound muscle action potential abnormalities are less frequent (37{\%}) and less pronounced; (3) the needle electrode examination is always abnormal (100{\%}), revealing acute and chronic motor axon loss, with the latter predominating; (4) the clinical onset is heterogeneous for both the site of onset (bulbar, upper extremity, lower extremity, combination) and the symptomatology (sensory, motor, sensorimotor); (5) focal onsets were reported in the majority (79{\%}); and (6) there is a strong correlation between the clinical onset (both site and symptomatology) and the maximal EDX abnormalities. Thus, the EDX features of Kennedy's disease are consistent with a slowly progressive and very chronic degeneration of the anterior horn cells and dorsal root ganglia. Although the clinical onsets are heterogenous, the EDX features are homogenous and unique, consisting of a diffuse, very slowly progressive anterior horn cell disorder coupled with a sensory neuropathy/neuronopathy that mimics an acquired process.",
author = "Mark Ferrante and Wilbourn, {Asa J.}",
year = "1997",
month = "3",
day = "18",
doi = "10.1002/(SICI)1097-4598(199703)20:3<323::AID-MUS9>3.0.CO;2-D",
language = "English (US)",
volume = "20",
pages = "323--329",
journal = "Muscle and Nerve",
issn = "0148-639X",
publisher = "John Wiley and Sons Inc.",
number = "3",

}

TY - JOUR

T1 - The characteristic electrodiagnostic features of Kennedy's disease

AU - Ferrante, Mark

AU - Wilbourn, Asa J.

PY - 1997/3/18

Y1 - 1997/3/18

N2 - To define the electrodiagnostic (EDX) features of Kennedy's disease, their distribution, their clinical correlation, and to determine whether they are unique to this disorder, we retrospectively evaluated the EDX and clinical features of 19 patients with Kennedy's disease and found that: (1) the percentage with sensory nerve action potential abnormalities is high (95%); (2) compound muscle action potential abnormalities are less frequent (37%) and less pronounced; (3) the needle electrode examination is always abnormal (100%), revealing acute and chronic motor axon loss, with the latter predominating; (4) the clinical onset is heterogeneous for both the site of onset (bulbar, upper extremity, lower extremity, combination) and the symptomatology (sensory, motor, sensorimotor); (5) focal onsets were reported in the majority (79%); and (6) there is a strong correlation between the clinical onset (both site and symptomatology) and the maximal EDX abnormalities. Thus, the EDX features of Kennedy's disease are consistent with a slowly progressive and very chronic degeneration of the anterior horn cells and dorsal root ganglia. Although the clinical onsets are heterogenous, the EDX features are homogenous and unique, consisting of a diffuse, very slowly progressive anterior horn cell disorder coupled with a sensory neuropathy/neuronopathy that mimics an acquired process.

AB - To define the electrodiagnostic (EDX) features of Kennedy's disease, their distribution, their clinical correlation, and to determine whether they are unique to this disorder, we retrospectively evaluated the EDX and clinical features of 19 patients with Kennedy's disease and found that: (1) the percentage with sensory nerve action potential abnormalities is high (95%); (2) compound muscle action potential abnormalities are less frequent (37%) and less pronounced; (3) the needle electrode examination is always abnormal (100%), revealing acute and chronic motor axon loss, with the latter predominating; (4) the clinical onset is heterogeneous for both the site of onset (bulbar, upper extremity, lower extremity, combination) and the symptomatology (sensory, motor, sensorimotor); (5) focal onsets were reported in the majority (79%); and (6) there is a strong correlation between the clinical onset (both site and symptomatology) and the maximal EDX abnormalities. Thus, the EDX features of Kennedy's disease are consistent with a slowly progressive and very chronic degeneration of the anterior horn cells and dorsal root ganglia. Although the clinical onsets are heterogenous, the EDX features are homogenous and unique, consisting of a diffuse, very slowly progressive anterior horn cell disorder coupled with a sensory neuropathy/neuronopathy that mimics an acquired process.

UR - http://www.scopus.com/inward/record.url?scp=0031035995&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0031035995&partnerID=8YFLogxK

U2 - 10.1002/(SICI)1097-4598(199703)20:3<323::AID-MUS9>3.0.CO;2-D

DO - 10.1002/(SICI)1097-4598(199703)20:3<323::AID-MUS9>3.0.CO;2-D

M3 - Article

VL - 20

SP - 323

EP - 329

JO - Muscle and Nerve

JF - Muscle and Nerve

SN - 0148-639X

IS - 3

ER -