Motor Neurone Diseases Specialty Group
Chair: Matthew Kiernan
e-mail: matthew.kiernan@sydney.edu.au
About
Motor neurone disease (amyotrophic lateral sclerosis; Lou Gehrig's disease) encompasses a spectrum of neurodegenerative conditions that affect the brain, spinal cord, peripheral nerves and muscles. The clinical presentations of MND/ALS include: limb-onset ALS with a combination of upper and lower motor neurone (UMN and LMN) signs; bulbar-onset ALS, presenting with speech and swallowing difficulties, with limb and sometimes cognitive features developing later in the course of the disease; the less common primary lateral sclerosis (PLS) with pure UMN involvement; and progressive muscular atrophy, with pure LMN signs.
The Specialty Group (SG) represents an international network and is engaged in patient care, research and the development of new therapies, including targeted gene therapy approaches.
The SG recently simplified diagnostic criteria1 - MND/ALS is defined when there is:
- Progressive motor impairment as documented by history or repeated clinical assessment, preceded by normal motor function.
- The presence of upper and lower motor neuron dysfunction in at least one body region (with upper and lower motor neuron dysfunction noted in the same body region if only one body region is involved), or lower motor neuron dysfunction in at least two body regions.
- Appropriate investigations undertaken to exclude alternative disease processes.
The Specialty Group hosts an educational WFN session at the International Symposium and engages in educational initiatives and advocacy.
The journal of the WFN Specialty Group is Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration (https://www.tandfonline.com/toc/iafd20/14/1)
1 Shefner JM et al. A proposal for new diagnostic criteria for ALS. Clin Neurophysiol 2020 Aug;131(8):1975-1978.
Specialty Group Members
- Adriano Chio (Italy)
- Jeffery Rosenfeld (US)
- Liying Cui (China)
- Martin Turner (UK)
- Patricia Lillo (Chile)
- Ryuji Kaji (Japan)
- Timothy Miller (US)
