Flashcards in Motor Neurone Disease Deck (70)
What is the median survival upon onset of symptoms?
At what age is motor neurone disease likely to present?
What is the epidemiology of MND?
10% Familial link
What are the four types of MND
Amyotrophic Lateral Sclerosis
Primary Lateral Sclerosis
Progressive Muscular Atrophy
Progressive Bulbar Palsy
What is the most common subtype of MND?
ALS - Amyotrophic lateral sclerosis
How does ALS present?
With both Lower and Upper motor neurones features
How does PLS present?
Upper motor neurone features
Good survival >5yrs
How does Progressive Muscular Atrophy present?
30% have UMN features
UMN - Clinical signs
Extensor Plantar Response
Exaggerated Jaw jerk
LMN - Clinical signs
Fasciculations(spontaneous muscle twitch)
Absent or reduced tendon reflexs
What form of MND affects the lower cranial nerves?
progressive bulbar palsy
Who is affected by progressive bulbar palsy?
60 - 80 years
What muscles are affected by Progressive bulbar palsy?
Facial Tongue Pharyngeal
What are the main issues surrounding Progressive bulbar Palsy?
Dysphagia - risk of choking
Dysarthia - Can't communicate
Why is it called progressive bulbar palsy?
As the disease always progresses to become ALS.
What are the treatments for Progressive Bulbar Palsy?
Therapeutic - communication devise, nutritional support, care for the URT.
What common syndrome presents in ALS?
Split Hand syndrome - selective wasting of muscles within the hand
What muscles show wasting in Split Hand syndrome?
First dorsal Interosseus
What muscles are preserved in Split Hand syndrome?
Abductor Digit minimi
What form of dementia is linked to ALS?
What are the diagnostic criteria for ALS?
Signs of UMN LMN degeneration
Electrophysiological and Neuropathological investigation
Signs in 2/3 vertebral segments on MRI
What is the primary treatment for ALS?
Rilozole 50mg x2
Why is nutrition and hydration support in ALS so impotent?
Metabolic rate is doubled and weight loss indicates a very poor prognosis.
What is a clinical priority in regards to ALS?
Insertion of a Gastrostomy
What is sialorrhoea?
What is the treatment for sialorrhoea?
In ALS what is used to treat Muscle Cramps?
In ALS what is used to treat Muscle Spasms?
Why is respiratory dysfunction one of the main causes of death in ALS?
Respiratory muscles fail.