Sensory involvement in spinal-bulbar muscular atrophy (Kennedy's disease).
Muscle Nerve
; 23(2): 252-8, 2000 Feb.
Article
en En
| MEDLINE
| ID: mdl-10639619
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.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Atrofia Muscular Espinal
/
Neuropatía Hereditaria Motora y Sensorial
/
Fibras Nerviosas Mielínicas
Límite:
Aged
/
Humans
/
Male
/
Middle aged
Idioma:
En
Revista:
Muscle Nerve
Año:
2000
Tipo del documento:
Article
País de afiliación:
Italia
Pais de publicación:
Estados Unidos