Kennedy’s disease: an under-recognized motor neuron disorder
Loading...
Date
Journal Title
Journal ISSN
Volume Title
Publisher
Springer Science and Business Media Deutschland GmbH
Abstract
Kennedy’s disease or spinal bulbar muscular atrophy is a rare, inherited and slowly progressive multisystem disease mostly manifesting with a motor neuron disease phenotype leading to disability. The slow progression, partial androgen insensitivity, electrophysiological evidence of sensory neuronopathy, and relatively spared central nervous system pathways help differentiate it from amyotrophic lateral sclerosis. To date, there is no treatment or cure with clinical care mainly focused on accurate diagnosis, symptom management, patient education, and genetic counselling. © 2020, Belgian Neurological Society.
Description
Keywords
Kennedy’s disease, Motor neuron disease, Spinal bulbar muscular atrophy, Bulbo-spinal atrophy, x-linked, Humans, Androgen receptor, Biological marker, Ethambutol, Follitropin, Leuprorelin, Levodopa, Lomustine, Mitochondrial dna, Polyglutamine, Androgen insensitivity syndrome, Cognitive defect, Daily life activity, Disease severity, Dysarthria, Dysphonia, Dystonia, Electromyography, Electrophysiology, Exercise intensity, Gene mutation, Genetic counseling, Grip strength, Gynecomastia, Human, Huntington chorea, Kennedy disease, Mini mental state examination, Morphometry, Motor evoked potential, Motor performance, Muscle action potential, Muscle atrophy, Muscle hypertrophy, Muscular dystrophy, Myalgia, Myelooptic neuropathy, Nerve degeneration, Nerve potential, Neuroimaging, Neuropsychological test, Nuclear magnetic resonance imaging, Paresthesia, Parkinson disease, Patient education, Periodic limb movement disorder, Phenotype, Pleocytosis, Polysomnography, Prevalence, Prognosis, Quality of life, Rem sleep, Review, Six minute walk test, Spine fracture, White matter