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Sensory involvement in spinal‐bulbar muscular atrophy (Kennedy's disease)
Author(s) -
Antonini G.,
Gragnani F.,
Romaniello A.,
Pennisi E.M.,
Morino S.,
Ceschin V.,
Santoro L.,
Cruccu G.
Publication year - 2000
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/(sici)1097-4598(200002)23:2<252::aid-mus17>3.0.co;2-p
Subject(s) - spinal and bulbar muscular atrophy , medicine , jaw jerk reflex , sural nerve , sensory system , anatomy , trigeminal nerve , reflex , sensory nerve , atrophy , nervous system , neuroscience , pathology , masseter muscle , psychology , androgen receptor , anesthesia , prostate cancer , cancer , psychiatry
Spinal‐bulbar muscular atrophy (SBMA) is a rare X‐linked neuronopathy associated with an abnormal representation of androgen receptors in the nervous system. Standard nerve conduction and histopathological studies have disclosed the involvement of large myelinated sensory fibers in the spinal nerves of SBMA patients. Little is known about the involvement of small sensory neurons and trigeminal nerves. Laser evoked potentials (LEPs) were studied in 6 unrelated patients with SBMA; 5 of these patients also underwent trigeminal reflex recordings, and 3 a sural nerve biopsy. LEPs were markedly abnormal, indicating a dysfunction in pain pathways. Given the sparing of small fibers in the sural nerve specimens, we hypothesize a dysfunction in spinothalamic cells, possibly due to an abnormal representation of the androgen receptors. Except for the jaw‐jerk, all the trigeminal reflexes were markedly abnormal. Since the afferents for the jaw‐jerk have their cell body within the central nervous system instead of the ganglion, the selective sparing of the jaw‐jerk indicates a trigeminal ganglionopathy. © 2000 John Wiley & Sons, Inc. Muscle Nerve 23: 252–258, 2000.