Motor neuron diseaseals

Progressive bulbar palsy

The symptoms and signs are due to a disturbance of the motor cranial nuclei rather than corticobulbular tracts. The condition is distinguished from pseudobulbar palsy by the presence of lower motor neuron (nuclear) signs.

Atrophy and fasciculations are present in cranial nerve innervated muscles.

Fasciculations are visible muscle twitches which occur spontaneously and represent groups of discharging motor units.

The tongue appears wasted and folded; fasciculations produce a writhing appearance. --- " Jaw jerk and gag reflex are absent.

Corticospinal involvement

Signs of corticospinal tract disturbance with:

- Increased tone.

- Brisk reflexes.

- Extensor plantar responses.

- Distinctive distribution of weakness (extensors in upper limbs; flexors in lower limbs). Spasticity is rarely severe (intact extrapyramidal inhibition). (Primary lateral sclerosis is a slowly progressive form of ALS restricted to the cortical spinal tract.)

Progressive muscular atrophy

Signs and symptoms are due to anterior horn cell involvement. Atrophy, weakness and fasciculations are the cardinal features.

The patient is often aware of fasciculation. Muscle cramps are common.

Weakness is not as severe as the degree of wasting suggests.

In the hand: wasting is evident.

-----1st dorsal interosseous muscle and

-----tendons become prominent as hand muscles waste, giving 'guttered' appearance -skeleton hand.

As the disease progresses, all levels of the motor system become involved. Respiratory muscle weakness ultimately occurs and is the usual cause of death.

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