Motor Neuron Disease Flashcards
What is MND
Umbrella term for progressive neurodegenerative diseases affecting the motor neurones
State the 4 main forms of MND
- Amyotrophic lateral sclerosis - mc
- Progressive bulbar palsy
- Progressive muscular atrophy
- Primary lateral sclerosis
Which form of MND is an isolated lower motor neuron disorder
progressive muscular atrophy
Which form of MND is an isolated upper motor neuron disorder
primary lateral sclerosis
What does progressive bulbar palsy primarily affect
Muscles of speech and swallowing - cranial nerves 9-12
Does ALS typically present with UMN or LWM signs
Usually a combination of both
Give 3 RFs for MND
- Male >40
- FHx
- Exposure to Pesticides, Herbicides etc
Give 4 signs of UMN lesions
- Hypertonia
- +ve Babinski sign
- Hyperreflexia
- Spasticity
Give 4 signs of a LMN lesion
- Fasciculations
- Muscle wasting
- Hyporeflexia
- Hypotonia
Where is weakness first noticed in MND
Upper limbs
Give 6 ways ALS may present
- Spastic unsteady gait
- Foot drop
- UMN + LMN signs
- Dysphagia and dysarthria
- Proximal muscle weakness
- Dyspnoea/ orthopnoea
(Progressive painless paralysis)
How is MND diagnosed
Clinical diagnosis - presence of UMN/LMN signs, disease progression and absence of any other aetiology
* EMG and nerve conduction studies - diagnostic when presentation is limited to <3 limbs
How is ALS treated
- Riluzole - slow progression (3m)
- Non-invasive ventilation
- Supportive care
- PEG tube - help maintain stable weight
How does riluzole slow the progression of ALS
Sodium channel blocker that inhibits glutamate release protecting motor neurones
Give 2 complications of ALS
- Respiratory failure
- Aspiration pneumonia
