Neurology Specific Literature Search   
 
[home][thesaurus]
    
Click Here to return To Results

 

Clinical and Electrodignostic Features of X-Linked Recessive Bulbospinal Neuronopathy
Neurol 41:823-828, Olney,R.K.,et al, 1991
See this aricle in Pubmed

Article Abstract
We describe four men from two kinships affected with X-linked recessive bulbospinal neuronopathy,and one sporadic case.All developed postural tremor,weakness,and fasciculations,with onset from age 25 to 39 years. Weakness began in the pelvic girdle or hands,with dysphagia or dysarthria occurring years later in two.Sensory symptoms were present in only one,who also had diabetes mellitus.In contrast,sural nerve action potentials were small or absent in all.Needle EMG showed widespread chronic partial denervation with reinnervation.The characteristic twitching of the chin produced by pursing of the lips consisted of repetitive or grouped motor unit discharges,rather than fasciculations.Broader awareness of the distinctive features of bulbospinal neuronopathy will probably increase the frequency of its recognition.Diagnosis is important for purposes of providing a prognosis for affected men and genetic counseling for affected families.
 
Related Tags
(click to filter results - removes previous filter)

amyotrophic lateral sclerosis,differential diagnosis
amyotrophic lateral sclerosis,misdiagnosis
areflexia
dysarthria
dysphagia
electromyogram
familial
fasciculation
gene
genetic counselling
genetic neurologic disorders
gynecomastia
hyporeflexia
muscle cramp
neuroendocrinology
neuronopathy
tongue,fasciculations of
tremor
tremor,postural
weakness
X-linked bulbospinal neuronopathy

Click Here to return To Results