Amyotrophic Lateral Sclerosis

Also referred to as Motorneuron Disease in the UK, or Lou Gehrig’s Disease. It

attacks the upper and/or lower motor neurons (not the sensory system) and leads to
weakness, muscle wasting, loss of mobility in limbs and later difficulty in speech,
swallowing and breathing. There are four sub-classifications: ALS, Progressive
Bulbar Palsy, Progressive Muscular Atrophy and Primary Lateral Sclerosis.

Generally occurs in 40-70 age group but can occur at any stage. 5-10% of cases are
familial and all others are idiopathic (cause not known). Some risk factors are
mechanical/electrical trauma, military service, expose to heavy metals, agricultural
chemicals, high levels of exercise, but data is not robust.

The primary motor cortex makes a decision about what muscle to move. The upper
motor neuron runs down the spine to the anterior horn and the lower motor then
connects to the skeletal muscle. PMA affects the lower motor neuron. PBP affects
the corticobulbar tract from the motor cortex to pons and medulla oblongata. These
coordinate complex action like facial movement, swallowing and throwing and are
the first points of diagnosis (particularly swallowing).

There are three types of lower motor neuron: alpha, beta and gamma.
Alpha are the most common and contact extrafusal muscle fibres which are
responsible for muscle contraction. Beta and gamma innervate intrafusal muscles:
spindles inside the muscle covered in collagen which regulate sensitivity to stretch
and feedback to the CNS. Beta also sends collaterals to extrafusal muscle. Motor
neurons matched to muscle type: type 1 (slow twitch) and type 2 (fast twitch).

Diagnosis is difficult in early stages as symptoms are generic but usually muscle
weakness and lack of coordination. Differences between UMN and LMN lesions can
be distinguished. UMN lesions disrupt the regulation of muscle contraction, such as
that required in bending of the elbow (bicep contracts, tricep relaxes). This means no
atrophy occurs in UMN lesion patients but does in LMN lesions. Fasciculations
(twitches) also do not occur in UMN lesions because only the LMNs are involved.
Reflexes and tone are actually increased in UMN lesions because of the lapse in
regulation, but decreased in LMN lesions.