Motor Neuron Diesease Flashcards
What are motor neuron diseases?
A group of progressive neurological disorders affecting nerve cells responsible for controlling muscle movement.
What are the main types of motor neuron diseases?
• Amyotrophic Lateral Sclerosis (ALS)
• Primary Lateral Sclerosis (PLS)
• Progressive Muscular Atrophy (PMA)
What are possible causes of motor neuron diseases?
-Genetics
-environmental factors (toxins, viral infections)
-immune system dysfunction.
What is ALS also known as?
Lou Gehrig’s disease.
What are common symptoms of ALS?
-Progressive muscle stiffness, weakness, and wasting (early stages)
-Difficulty eating, speaking, moving, and breathing (progression)
-frontotemporal dementia, and minor difficulties
with thinking and behavior.
What is the most common form of motor neuron disease?
Amyotrophic Lateral Sclerosis (ALS).
Define ALS
It is a rare terminal neurodegenerative disease that results in the progressive loss of both the upper and lower motor neurons
How is ALS classified based on symptom onset?
-Limb-onset: Weakness begins in the arms or legs
-Bulbar-onset: Difficulty speaking or swallowing
What are the pathological hallmarks of ALS?
-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
In ALS, What causes the spinal cord to atrophy along
with the affected muscles?
Gliosis replacing lost neurons
Which neurons are spared in ALS?
Sensory neurons
What is the typical age group affected by ALS?
Late middle-aged individuals (possibly with an affected relative)
Weakness in often first noticed in what part in patients with ALS?
Upper limb, They may complain of clumsiness,
dropping things more often or tripping over and fatigue when exercising
How is ALS diagnosed?
Through exclusion of other diseases. Because no single definitive test exists.
What is the treatment for ALS?
(No know cure, just slowing down the progression)
-Medications like riluzole to slow progression
-Non-invasive ventilation (NIV) for respiratory support
What is the role of physical therapy in the management of ALS?
-delaying loss of strength
-maintaining endurance
-limiting pain
What neurons are affected in PLS?
Upper motor neurons
Unlike ALS, PLS shows no evidence of…?
-Degeneration of spinal muscle neurons
-muscle wasting (amyotrophy)
How does the onset of PLS usually occur?
spontaneously after age 50 and progresses gradually
What is the clinical presentation of PLS?
-gradual-asymmetrical-progressive-onset.
-lower-extremity stiffness and pain due to muscle spasticity.
Initially in PLS muscles do not atrophy, then what is the disabling aspect of this disease?
Joint immobility as a result of muscle spasticity and cramping, and intense pain when those muscles are stretched.
Describe the gait pattern of PLS patients
tiny step shuffle with related instability and falling.
How is PLS diagnosed?
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
What medications can reduce muscle spasticity in PLS?
Baclofen and tizanidine.
What drugs may reduce cramps in PLS?
Quinine and phenytoin
What are the goals of physical therapy for PLS patients?
• Reducing muscle tone
• Maintaining joint mobility
• Improving coordination and strength
PMA is also called?
Duchenne–Aran muscular atrophy
What characterizes PMA?
Degeneration of lower motor neurons, leading to muscle weakness, atrophy, and fasciculations.
What are “flail limb” cases in PMA?
Symptoms restricted to arms or legs s (or in some
cases just one of either)
Which one has a better prognosis, ALS or PMA?
PMA
