Motor Neuron Diesease

Question 1

What are motor neuron diseases?

Answer 1

A group of progressive neurological disorders affecting nerve cells responsible for controlling muscle movement.

Question 2

What are the main types of motor neuron diseases?

Answer 2

• Amyotrophic Lateral Sclerosis (ALS)
• Primary Lateral Sclerosis (PLS)
• Progressive Muscular Atrophy (PMA)

Question 3

What are possible causes of motor neuron diseases?

Answer 3

-Genetics
-environmental factors (toxins, viral infections)
-immune system dysfunction.

Question 4

What is ALS also known as?

Answer 4

Lou Gehrig’s disease.

Question 5

What are common symptoms of ALS?

Answer 5

-Progressive muscle stiffness, weakness, and wasting (early stages)

-Difficulty eating, speaking, moving, and breathing (progression)

-frontotemporal dementia, and minor difficulties
with thinking and behavior.

Question 6

What is the most common form of motor neuron disease?

Answer 6

Amyotrophic Lateral Sclerosis (ALS).

Question 7

Define ALS

Answer 7

It is a rare terminal neurodegenerative disease that results in the progressive loss of both the upper and lower motor neurons

Question 8

How is ALS classified based on symptom onset?

Answer 8

-Limb-onset: Weakness begins in the arms or legs
-Bulbar-onset: Difficulty speaking or swallowing

Question 9

What are the pathological hallmarks of ALS?

Answer 9

-Degeneration of pyramidal Betz cells in the motor cortex
-Degeneration of anterior horn cells in the spinal cord
-Degeneration of the lower motor nuclei of the brain stem

Question 10

In ALS, What causes the spinal cord to atrophy along
with the affected muscles?

Answer 10

Gliosis replacing lost neurons

Question 11

Which neurons are spared in ALS?

Answer 11

Sensory neurons

Question 12

What is the typical age group affected by ALS?

Answer 12

Late middle-aged individuals (possibly with an affected relative)

Question 13

Weakness in often first noticed in what part in patients with ALS?

Answer 13

Upper limb, They may complain of clumsiness,
dropping things more often or tripping over and fatigue when exercising

Question 14

How is ALS diagnosed?

Answer 14

Through exclusion of other diseases. Because no single definitive test exists.

Question 15

What is the treatment for ALS?

Answer 15

(No know cure, just slowing down the progression)

-Medications like riluzole to slow progression
-Non-invasive ventilation (NIV) for respiratory support

Question 16

What is the role of physical therapy in the management of ALS?

Answer 16

-delaying loss of strength
-maintaining endurance
-limiting pain

Question 17

What neurons are affected in PLS?

Answer 17

Upper motor neurons

Question 18

Unlike ALS, PLS shows no evidence of…?

Answer 18

-Degeneration of spinal muscle neurons
-muscle wasting (amyotrophy)

Question 19

How does the onset of PLS usually occur?

Answer 19

spontaneously after age 50 and progresses gradually

Question 20

What is the clinical presentation of PLS?

Answer 20

-gradual-asymmetrical-progressive-onset.

-lower-extremity stiffness and pain due to muscle spasticity.

Question 21

Initially in PLS muscles do not atrophy, then what is the disabling aspect of this disease?

Answer 21

Joint immobility as a result of muscle spasticity and cramping, and intense pain when those muscles are stretched.

Question 22

Describe the gait pattern of PLS patients

Answer 22

tiny step shuffle with related instability and falling.

Question 23

How is PLS diagnosed?

Answer 23

Similar to ALS, there are no specific tests for the
diagnosis of PLS. Therefore, the diagnosis occurs as
the result of eliminating other possible causes of the
symptoms

Question 24

What medications can reduce muscle spasticity in PLS?

Answer 24

Baclofen and tizanidine.

Question 25

What drugs may reduce cramps in PLS?

Answer 25

Quinine and phenytoin

Question 26

What are the goals of physical therapy for PLS patients?

Answer 26

• Reducing muscle tone
• Maintaining joint mobility
• Improving coordination and strength

Question 27

PMA is also called?

Answer 27

Duchenne–Aran muscular atrophy

Question 28

What characterizes PMA?

Answer 28

Degeneration of lower motor neurons, leading to muscle weakness, atrophy, and fasciculations.

Question 29

What are “flail limb” cases in PMA?

Answer 29

Symptoms restricted to arms or legs s (or in some
cases just one of either)

Question 30

Which one has a better prognosis, ALS or PMA?

Answer 30

PMA

Question 31

Answer 31