Motor Neuron Disease Flashcards
What is motor neuron disease?
A term that encompasses a variety of specific diseases affecting the motor nerves.
Motor neurone disease is progressive and eventually fatal.
What is the typical progression of motor neuron disease?
Progressive, eventually fatal condition where the motor neurons stop functioning.
It presents with both upper and lower motor neuron signs.
Does motor neuron disease affect sensory neurons?
No, sensory neurons are never affected.
Sensory symptoms suggest an alternative diagnosis.
What percentage of motor neuron disease patients have amyotrophic lateral sclerosis (ALS)?
50% of patients.
ALS typically shows LMN signs in arms and UMN signs in legs.
What are the signs of lower motor neuron disease?
- Muscle wasting
- Reduced tone
- Fasciculations (twitching muscles)
- Reduced reflexes
These signs indicate damage to the lower motor neurons.
What are the signs of upper motor neuron disease?
- Increased tone or spasticity
- Brisk reflexes
- Upgoing plantar reflex
These signs indicate damage to the upper motor neurons.
What is the typical demographic of a motor neuron disease patient?
Late middle-aged (60 years) male, possibly with an affected relative.
Insidious, progressive weakness of muscles is common.
What is the typical initial symptom of motor neuron disease?
Weakness often noticed first in upper limbs.
Patients may also experience increased fatigue and clumsiness.
What is the diagnosis process for motor neuron disease?
Needs to be made carefully by a specialist, based on clinical presentation and exclusion of other conditions.
Nerve conduction studies will be normal, ruling out neuropathies.
What is the role of electromyography in diagnosing motor neuron disease?
Shows reduced action potentials with increased amplitudes.
This helps in confirming the diagnosis.
What is Riluzole used for in motor neuron disease management?
Prevents stimulation of glutamate receptors, mostly used in ALS.
It can prolong life by around 3-7 months.
What is the purpose of non-invasive ventilation in motor neuron disease management?
Supports breathing when respiratory muscles weaken, usually BIPAP.
It can increase survival by several months.
What supportive care options are available for motor neuron disease patients?
- Nutrition - percutaneous gastrostomy tube (PEG)
- Benzodiazepines for breathlessness caused by anxiety
- Symptom control - baclofen for muscle spasticity, antimuscarinics for excess saliva
- Advanced directives
Supportive care is crucial for quality of life.
What is the prognosis for progressive bulbar palsy?
It has the worst prognosis.
This condition affects the tongue and muscles of chewing/swallowing.
Fill in the blank: The gene associated with familial ALS is located on chromosome _______.
21
This gene codes for superoxide dismutase.
True or False: Progressive muscular atrophy affects proximal muscles before distal muscles.
False
It affects distal muscles before proximal muscles and has the best prognosis.
What are the main clinical features of motor neuron disease?
Insidious, progressive weakness affecting limbs, trunk, speech, and face.
Symptoms may include slurred speech (dysarthria) and increased clumsiness.
