MND Flashcards
What is it?
What type of motor neurone is affected - UMN/LMN?
What distinguishes it from MS and polyneuropathies?
What distinguishes it from myasthenia gravis?
A cluster of neurodegenerative diseases, characterised by selective loss of neurons in motor cortex, cranial nerve nuclei and anterior horn cells (spinal cord - that’s why LMN).
Both
There is no sensory loss or sphincter disturbances.
MND doesn’t affect eye movement?
Common symptoms:
Upper limb and spine effects:
- Grip
- Wrist
- Neck
Lower limb effects:
- Foot
- Gait
- What may they have difficulty doing?
Respiratory and bulbar:
- Speech
- Swallowing
- Breathing
- What type of pneumonia are they likely to have?
- Why do they get headaches in the morning?
What type of dementia does 10-20% of them get?
Weak/clumsy grip Wrist drop Neck flexion weakness ----- Foot drop Unsteady spastic gait Balance problems and difficulty rising from chairs ---- Slow/blurred speech Dysphagia SOB Aspiration pneumonia
Fronto temporal dementia
Mixed upper and lower motor neuron signs:
UMN?
LMN?
Hyperreflexia
Hypertonia
Fasciculations
Muscle atrophy
Low power in both UMN and LMN pathology
Types:
(ALS) Amyotrophic lateral sclerosis - most common type:
- Where is the degeneration? - 2
- What is usually affected first?
(PBP) Progressive bulbar palsy - RARE:
- What nerves are affected specifically? - think bulbar
Motor cortex and anterior horn cells
UMN and LMN of cranial nerves 9-12
Types:
(PLS) Primary lateral sclerosis:
- Where is the degeneration:
- It only affects 1 type of MN. Which one is it?
- Symptoms
(PMA) Progressive muscular atrophy:
- Where is the degeneration?
- It only affects 1 type of MN. Which one is it?
- Symptoms
Motor cortex
UMN only - spastic paraparesis, pseudobulbar palsy
Anterior horn cells
LMN only - weakness and wasting
Affects distal muscles first (e.g. small muscles in hands and feet)
DDx:
Neck flexion weakness - 2 - READ
Foot drop is more commonly from peripheral causes. Name 2?
Myasthenia gravis
Guillain Barre syndrome
Peroneal nerve compression
Sciatica
DDx:
What may happen in the cervical spine causing mixed upper/lower motor neuron signs?
What vitamin deficiency can cause this?
Cervical radiculomyelopathy
B12 deficiency
Investigations:
What type of diagnosis is made?
What is done to exclude other causes?
What imaging can be done to look for subclinical denervation?
Clinical diagnosis - using signs without evidence of other causes
MRI and LP
Electromyography (EMG) measures muscle response or electrical activity in response to a nerve’s stimulation of the muscle. The test is used to help detect neuromuscular abnormalities. During the test, one or more small needles (also called electrodes) are inserted through the skin into the muscle.
What can be used for diagnosis and to monitor disease progression?
Respiratory testing:
- Pulse oximetry (+/- ABG)
- Vital capacity
- Sniff nasal inspiratory pressure (SNIP)
Management:
Who should be involved in their care? - think about symptoms
Neurologist Physio OT - adaption for home Dietician - aspiration pneumonia Speech and language Psychology - mind
Management:
Limb symptom relief:
- What can be given to relax the muscles? - B, D, D
- What can be given for spasticity?
- What can be given for muscle cramps?
- What can be given for joint pain?
Baclofen
Dantrolene
Diazepam
Gabapentin
Quinine
Analgesia
Management:
Bulbar symptom relief:
- What can be done for dysphagia?
- What is glycopyrrolate used for?
Soft food
NG tube
PEG
Drooling
Management:
Respiratory symptom relief:
- What type of ventilation be used for respiratory failure or sleep apnoea?
- What are opioids and benzodiazepines used for?
NIV - non-invasive ventilation
SOB
Life-prolonging treatment:
What does Riluzole do?
What is done if NIV is no longer sufficient?
Adds 3 months to life - used in ALS - delays the onset of ventilator-dependence or tracheostomy in some people.
NIV or invasive ventilation via tracheostomy
Prognosis:
How does death usually occur?
What is the prognosis?
Resp failure
3-5 yrs
