Motor neuron disease Flashcards
What is motor neuron disease?
A rapidly progressing neurodegenerative condition causing muscle weakness and wasting, speech and swallowing difficulties and breathing difficulties
What percentage of MND can be described as ‘familial’?
10%
Where is the most common site of onset for MND?
Upper and lower extremities, usually upper
What proportion of cases of MND present as progressive bulbar palsy?
25%
What are the symptoms of progressive bulbar palsy?
Progressive difficulty with chewing, talking, and swallowing
What are the signs associated with progressive bulbar palsy?
Reduced gag reflexes, weak palatal movements, fasciculations, and weak movement of the facial muscles and tongue
What nerves are specifically involved in progressive bulbar palsy?
Glossopharyngeal
Vagus
Hypoglossal
In which age group of patients does progressive bulbar palsy tend to affect?
60-80
Which gender more commonly presents with bulbar palsy?
Female
What treatment can be provided for bulbar palsy?
Early communicator
Nutritional support
Care for upper respiratory tract
What is the diagnosis for this 54 year old lady:
Increased fatigue over 6 months; later dyspnoea at rest
Investigated by cardiology and respiratory teams, unremarkable findings
Then presented with pneumonia, needing emergency intubation
Further investigations revealed: CK 320, EMG: neurogeneic changes in paravertebral muscles, denervation
Thoracic variation of MND
What is the most common type of MND?
Amyotrophic lateral sclerosis
What components of the nervous system does amyotrophic lateral sclerosis affect?
UMN & LMN
What is the prognosis following diagnosis of amyotrophic lateral sclerosis?
3 years life expectancy
How does amyotrophic lateral sclerosis usually present?
Weakness starts in one limb and spreads to others
What component of the nervous system does primary lateral sclerosis affect?
Only UMN
How does primary lateral sclerosis usually present?
Weakness starts in legs, move up to trunk, then arms, head and finally bulbar
Stiffness and pain are associated with PLS
Which MND may present with falls and imbalance?
Primary lateral sclerosis
What component of the nervous system do spinal muscular atrophies affect?
Only LMN
What is an example of a spinal muscular atrophy?
Kennedy’s syndrome
What mode of inheritance is Kennedy’s syndrome?
X-linked recessive
What is the age of onset of spinal muscular atrophies?
20-40
What comorbidities are associated with spinal muscular atrophies?
Type 2 diabetes
Infertility
What kind of dementia is associated with motor neuron disease?
Frontotemporal dementia
