Motor neurone disease and the life of motor neurones

1997 ◽  
Vol 166 (2) ◽  
pp. 109-109
Author(s):  
Amolak S Bansal ◽  
Jacqueline A Bansal
Keyword(s):  
Motor Neurone ◽  
Motor Neurone Disease ◽  
Motor Neurones

Finger extension weakness and downbeat nystagmus motor neurone disease (FEWDON-MND)

2019 ◽  
Vol 19 (5) ◽  
pp. 424-426 ◽  
Author(s):  
Wladimir B V R Pinto ◽  
Igor Braga Farias ◽  
Bruno de Mattos Lombardi Badia ◽  
Luiz Henrique Libardi Silva ◽  
Mario Teruo Yanagiura ◽  
...  
Keyword(s):  
Neurodegenerative Disorders ◽  
Clinical Picture ◽  
Motor Neurone ◽  
Motor Neurone Disease ◽  
Downbeat Nystagmus ◽  
Upper Limbs ◽  
Finger Extension ◽  
Motor Neurones ◽  
Secondary Causes

Atypical motor neurone disease (MND) represents a challenging and expanding group of neurodegenerative disorders involving the upper or lower motor neurones, and rarely both. Neuro-ophthalmological disturbances such as gaze-evoked downbeat nystagmus are extremely rare in the context of typical and atypical MND. Finger extension weakness and downbeat nystagmus motor neurone disease (FEWDON-MND) syndrome has been recently recognised as a distinct syndromic phenotype of MND, with a characteristic clinical picture. We describe a 63-year-old woman with long-standing lower motor neurone involvement of the upper limbs, who on examination had gaze-evoked downbeat nystagmus. After extensive negative investigation for secondary causes of MND and downbeat nystagmus, we diagnosed FEWDON-MND syndrome.

Motor neurone disease

2020 ◽  
Vol 13 (12) ◽  
pp. 702-711
Author(s):  
Lisa Knight
Keyword(s):  
Primary Care ◽  
Early Diagnosis ◽  
Neurodegenerative Disorders ◽  
Motor Neurone ◽  
Neurological Symptoms ◽  
Motor Neurone Disease ◽  
Motor Neurones ◽  
Primary Care Clinicians ◽  
Prolonged Delay

Motor neurone disease describes a group of rare, fatal, neurodegenerative disorders. They are devastating conditions that cause the loss of upper and/or lower motor neurones, leading to a variety of progressive neurological symptoms that can develop over months to years. Due to the rarity of these conditions and the differing and often insidious symptoms there is typically a prolonged delay between presentation and diagnosis of between 15 and 18 months on average. There is often a short prognosis of 3 years, though this can vary significantly depending on the type of syndrome diagnosed. Primary care clinicians play a key role in both facilitating early diagnosis and subsequent management and co-ordination of care in the community.

Occupational Exposure to Electric Shocks and Extremely Low-Frequency Magnetic Fields and Motor Neurone Disease

2020 ◽  
Author(s):  
Grace X Chen ◽  
Andrea’t Mannetje ◽  
Jeroen Douwes ◽  
Leonard H Berg ◽  
Neil Pearce ◽  
...  
Keyword(s):  
Occupational Exposure ◽  
Magnetic Fields ◽  
Low Frequency ◽  
Population Based ◽  
Motor Neurone ◽  
Motor Neurone Disease ◽  
Cumulative Exposure ◽  
Spine Injury ◽  
Extremely Low Frequency ◽  
Electric Shocks

Abstract In a New Zealand population-based case-control study we assessed associations with occupational exposure to electric shocks, extremely low-frequency magnetic fields (ELF-MF) and motor neurone disease using job-exposure matrices to assess exposure. Participants were recruited between 2013 and 2016. Associations with ever/never, duration, and cumulative exposure were assessed using logistic regression adjusted for age, sex, ethnicity, socioeconomic status, education, smoking, alcohol consumption, sports, head or spine injury and solvents, and mutually adjusted for the other exposure. All analyses were repeated stratified by sex. An elevated risk was observed for having ever worked in a job with potential for electric shocks (odds ratio (OR)=1.35, 95% confidence interval (CI): 0.98, 1.86), with the strongest association for the highest level of exposure (OR=2.01, 95%CI: 1.31, 3.09). Analysis by duration suggested a non-linear association: risk was increased for both short-duration (<3 years) (OR= 4.69, 95%CI: 2.25, 9.77) and long-duration in a job with high level of electric shock exposure (>24 years; OR=1.88; 95%CI: 1.05, 3.36), with less pronounced associations for intermediate durations. No association with ELF-MF was found. Our findings provide support for an association between occupational exposure to electric shocks and motor neurone disease but did not show associations with exposure to work-related ELF-MF.

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