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Welcome to Neuroscience Pearls: A publication from the UW Medicine Neurosciences Institute. Our goal is to provide useful information pertinent to your practice. Here we bring you key points related to Amyotrophic Lateral Sclerosis - ALS.

Richard G. Ellenbogen, MD, Professor and Chairman, Department of Neurological Surgery, Director, UW Neurosciences Institute
Bruce R. Ransom, MD, PhD, Professor and Chair, Department of Neurology, Co-Director, UW Neurosciences Institute, Adjunct Professor, Department of Physiology and Biophysics
Contributing Author:
B. Jane Distad, MD, Associate Professor, UW Department of Neurology

Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig’s disease, is a progressive neurodegenerative disorder affecting the motor neuron pathways in the spinal cord, brainstem and brain, leading to weakness and progressive muscle loss. Medical and supportive care improves quality of life and life expectancy, but individuals usually succumb to the disease within 5 years.

Amyotrophic refers to muscle loss from damage to the motor neurons; lateral indicates the area of the spinal cord affected; and sclerosis means hardening. ALS causes progressive loss of motor neurons, leading to weakness and muscle atrophy in arms, legs - and elsewhere adversely affecting all motor functions including swallowing, speaking and breathing. It is the most common motor neuron disorder. Absolute diagnosis of the disease relies on autopsy features of motor neuron loss and sclerosis. However, the findings on physical examination of upper motor and lower motor signs in multiple regions are pathognomonic of ALS (Table 1). These findings in combination with abnormalities in bulbar (the region of the brainstem controlling swallowing and speaking) muscles are highly associated with this particular disease. No laboratory or radiologic tests confirm the diagnosis. (Specific diagnostic criteria for research studies are included in Table 2).

The possible causes of ALS include genetic factors, inflammation, the environment, smoking, low body mass index and heavy metals. Other diseases which may present similarly to ALS include arthritic changes in the neck (spondylosis) causing spinal cord and nerve root compression, inclusion body myositis and rarely multifocal motor neuropathy and spinal muscular atrophy.

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Novmeber 2015 | Volume 3: Issue 3