Neurological Progressive Disorders Flashcards
What is Motor Neurone Disease?
A clinically and pathologically heterogenous group of neurologic diseases characterised by progressive degeneration of motor neurons. Can be either or both UMN and/or LMN.
What are the common types of motor neurone disease?
Amyotropic lateral sclerosis (ALS), Spinal Muscular atrophy (SMA), progressive bulbar palsy and primary lateral sclerosis.
What is Amyotrophic lateral sclerosis and what is it known as in the USA?
Amyotrophic - muscle wasting, sclerosis - breakdown of muscle fibre. It is a progressive neurological disease that affects both UMN and LMN. In Australia, there are 2 deaths per day (respiratory muscle complications) and 2 new cases diagnosed per day. It also know in the USA as Lou Gehrig’s disease (after famous baseball player).
Does Amyotrophic lateral sclerosis have a cure?
No
What causes Amyotrophic lateral sclerosis?
Several factors: genetic, toxicity to glutamate, damage to mitochondria, environmental causes, infections, auto-immune, inflammatory process, smoking.
What LMN signs may you see in amyotrophic lateral sclerosis?
Muscle atrophy, hypotonia, hyporeflexia, muscle weakness, fasciculation (muscle twitching), cramps and fatigue.
What UMN signs may you see in Amyotrophic lateral sclerosis?
Hypertonia, hyperreflexia, spasticity, muscle weakness, muscle atrophy, bulbar symptoms.
What is primary lateral sclerosis or upper motor neuron predominant ALS?
Clinical signs of UMN problems. Slow progression but involves up to all regions. Is diagnosed if there is no LMN signs after 4 weeks.
What is progressive muscular atrophy or lower motor neuron predominant ALS?
Clinical signs of LMN problems. Slow progression but can involve all regions. Confirmed if there has been no UMN signs after 4 weeks.
What is progressive bulbar palsy and pseudobulbar palsy?
PBP: involves bulbar region and predominantly LMNs.
Pseudobulbar palsy: involves bulbar region and predominantly UMNs.
What is the bulbar region?
Consists of the cerebellum, medulla oblangatta and pons. Bulbar palsy affects CN’s 9, 10, 11, 12. (glossopharyngeal, vagus, accessory, hypoglossal). Therefore, loss of taste to posterior 1/3 of tongue and troubles swallowing. Parasympathetic sensory and motor issues. Weakness of sternocleidomastoid and trapezius. Tongue motor loss.
What are the stages of amyotrophic lateral sclerosis?
1-5. Mod (functional gait, ADLs and speech), mod, severe (increased dependency in ADLs and transfers, dysphagia and/or dysphasia), severe (complete loos of function in 2 areas + mod-severe deficit in 3rd area), death (life expectancy 2-5 years).
What are the characteristics of Spinal/progressive Muscular Atrophy?
Muscle weakness and wasting, loss of weight and muscle twitching (LMN impacts).
How is Spinal/Progressive Muscular Atrophy classified?
By age of onset and clinical onset. Type 4 is onset at 30-70 years old.
What are the risk factors for Spinal/Progressive Muscular Atrophy?
Exposure to chemicals: agrotoxics, solvents, mercury, lead. Farm work. Electrical shock. Strenuous physical activity.