Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002736 (amyotrophic lateral sclerosis)
 19,048 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A clinical and electromyographic study of oculomotor function was carried out in a series of 24 patients with amyotrophic lateral sclerosis (ALS). In 15 cases an alteration of Bell's phenomenon was found. In addition, three patients showed some impairment of conjugate ocular motility in the form of upward gaze paly. All cases had preserved oculocephalic reflexes in the vertical and horizontal planes. On clinical and electromyographic grounds, three degrees of altered Bell's phenomenon are suggested: attenuated (short and unsustained upward displacement of the eyeballs after forced closure of the eyelids), abolished (no upward displacement), and inverted (downward instead of upward displacement of the eyes). These oculomotor alterations were not directly related to the type of ALS at onset of the illness, nor with its duration. However they were correlated with the relative degree of the clinical bilateral pyramidal tract signs at the supraspinal level. The common involvement of the corticogeniculate tract in ALS could explain the unexpectedly high incidence of alteration of Bell's phenomenon found in this disease, but is is non-specific and similar lesions from different causes may also produce it.
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PMID:Abnormalities of Bell's phenomenon in amyotrophic lateral sclerosis: a clinical and electrophysiological evaluation. 68 56

Oculomotor disorders have been considered to be one of the negative symptoms in motor neuron disease (MND). However, recently, ophthalmoplegia, abnormal Bell's phenomenon and disturbance of pursuit movement have been reported. We tried to evaluate 32 patients with MND (19 males and 13 females; age, 35 to 77 years; 52.4 +/- 10.1 years) by bedside examination and electro-oculography (EOG) using an eye tracking method. Twenty-three of them were classified as amyotrophic lateral sclerosis (ALS) and seven as bulbospinal muscular atrophy, and two were unclassified. One hundred healthy persons for bedside examination and twenty-two for EOG were investigated as controls. Findings by bedside examinations were as follows; 1) Slight limitations of upward only, up & downward and upward & horizontal gaze were observed in 5 cases (15.6%), 1 case (3.1%) and 1 case (3.1%), respectively. 2) Incomplete convergence was observed in 11 cases. (34.4%) 3) Horizontal gaze nystagmus was observed in 6 cases. (18.8%) 4) As regards the frequency of limitation of upward gaze and incomplete convergence, there were no statistically significant differences from those in controls. Electrooculographic results were: 1) square wave jerks (SWJs) were recorded in 3 cases. (9.4%) 2) Amplitude ratio of saccade was significantly higher in MND than that in controls with the risk less than 0.1%. 3) The degree of ocular dysmetria was significantly higher in MND than that in controls with the risk less than 0.5%. These abnormalities were not directly related to suffering period. Although the mechanism is not known, several reports of the effectiveness of thyrotropin releasing hormone (TRH) in ALS were recently published.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Analysis of oculomotor disorders in motor neuron disease]. 251 55