Bulbospinal Neuronopathy

Kennedy's disease, also known as bulbospinal muscular atrophy (BSMA), is a rare, adult-onset, X-linked, recessive trinucleotide… Expand

SummaryA girl with rapid-onset, bulbospinal muscular atrophy and deafness is described. The patient's mother and brother showed… Expand

OBJECTIVE To characterize the earliest symptoms of X-linked bulbospinal neuronopathy (Kennedy disease [KD]) during the course of… Expand

The autonomic regions of the thoracolumbar spinal cord receive a dense enkephalinergic (ENK) innervation from supraspinal sources… Expand

There is increasing evidence that acetylcholinesterase is organised in a lattice-like fashion in the intermediate layers of the… Expand

CAG repeat expansion is thought to be a causative genetic mutation in several neurodegenerative diseases, including Huntington’s… Expand

In some patients with amyotrophic lateral sclerosis (ALS), the thenar hand is more severely affected than the hypothenar hand. To… Expand

We describe four men from two kinships affected with X-linked recessive bulbospinal neuronopathy, and one sporadic case. All… Expand

A 32 year old male is described with an onset of upper limb postural tremor in adolescence followed by muscle cramps. Progressive… Expand

The organization of the axonal pathways of reticulospinal and vestibulospinal projections in the 11‐day chicken embryo was… Expand