Motor Neurone Disease Flashcards
MND is untreatable. TRUE/FALSE?
TRUE
the treatments we have are not effective at improving progosis
Do patients with MND have Upper motor neuron symptoms, lower motor neuron symptoms or sensory symptoms?
Can have both UMN and LMN symptoms
NO SENSORY SYMP
What si the most common subtype of MND?
Amyotrophic lateral sclerosis
What is the median prognosis of MND after prognosis?
3 years
MND is becoming more common. TRUE/FALSE?
TRUE
mechanism unknown
Is MND slightly more common in males or females?
Males
Are most cases of MND sporadic or familial?
90% sporadic
10% Familial
The diagnosis of sporadic MND peaks at what ages?
50-75
if not diagnosed by 80, likelihood goes down
ALS is less common in non-Caucasian populations. TRUE/FALSE?
TRUE
=> more common in caucasians
How is MND usually classified?
UMN - Primary lateral sclerosis
LMN - Primary Muscular Atrophy
Mix of the two = ALS
If patients with ALS exhibit signs of upper and lower motor neuron dysfunction, what symptoms can they have?
Brisk reflexes (UMN) Muscle wasting
If MND has a bulbar onset, what activities are affected?
Speech
Swallowing
What is the prognosis of ALS compared to other types of MND?
ALS - poor prognosis (3-5 years)
Primary Lateral Sclerosis (>5yrs)
Progressive Muscular atrophy (variable)
What gene should you test for in ALS with frontotemporal dementia (FTD-MND)
C9 ORF 72
Give examples of UMN signs seen in MND?
Increased tone Hyper-reflexia Extensor plantar responses Spastic gait Slow movement
What LMN signs could be seen in MND?
Muscle wasting
Weakness
Fasiculation
Absent/reduced reflexes
What is MND katabolism and what percentage of patients does this affect?
- patients are cahectic and struggle to maintain body weight despite eating normal amounts
- due to progressive muscle break down
- 40-50% of patients have this
What tasks do MND patients with limb dysfunction often complain they are struggling to complete?
Writing
Holding objects
Turning keys in the door
What is the largest complication risk in MND patients with bulbar onset?
aspiration due to poor swallow
=> respiratory complications
What patient group normally gets primary bulbar onset and what does this progress to?
Woman > Men (60-80 years of age)
Always generalises into ALS
What early interventions are given to patients with bulbar involvement?
- Early communicator => voice banking
- nutritional support
- care for upper respiratory tract
Other than muscles in the tongue, what does bulbar onset MND affect?
facial muscles and pharyngeal muscles
What symptoms are seen in spinal dysfunction MND?
- Muscle wasting
- Loss of tone or contractures
- Problems with washing, dressing, feeding, turning in bed
MND “Split hand syndrome” indicates what group of muscles wastes more than the other?
Thenar muscle wasting (1st dorsal interosseous and abductor pollicis brevis)
hypothenar = spared (adbductor digiti minimi)
How is a diagnosis of MND made?
- clinical criteria UMN/LMN signs and no sensory symptoms
- Limb/bulbar/spinal onset +/- cognitive symptoms
- El-Escorial criteria and EMG findings
- Imaging/ lab studies to rule out other diseases
Why are a lot of patients with MND referred to another speciality first?
Bulbar onset can mimic potential ENT tumour
=> referred urgently there first
What can cause a false positive diagnosis of MND?
Multifocal motor neuropathy (MMN)
Kennedy’s disease
cervical spondylotic radiculomyelopathy
What drug can be used in MND and what does it do?
Riluzole - prolongs life by 3 months in very late stage of illness
many pts choose not to take the drug for this reason
What interventions can be used to help with breathing and nutrition in MND?
BiPAP - non-invasive ventilation
PEG tube
Why can BiPAP ventilation be a problem for patients who have reduced upper limb function?
If mask falls off during the night, patient cant use their hand to fix this
How many months are usually between symptom onset and diagnosis?
12 months
Most MND care is delivered in hospital. TRUE/FALSE?
FALSE
Most in community/ at patients homes
Bulbar dysfunction can also cause problems with saliva. How can these be treated?
Over salivation- Tx: buscopan, botox
Under salivation- Tx: Salivation pastilles
What are the different methods of augmentative and alternative communication (AAC)?
Low-tech: Paper and pen, Word Boards
High-tech: iPads, Lightwriter, Eye-Gaze
Weight loss is a red flag symptom in MND prognosis. TRUE/FALSE?
TRUE
- 6 months left after patient begins to become cachexic
What drugs can be given to patients to help with muscle cramps and spasms?
Cramps
- Quinine (not widely used now)
- Baclofen
Spasms
- Baclofen
- Tizanidine
- Dantrolene
- Gabapentin
What are the red flags of respiratory failure in MND?
- SOB
- Orthopnoea
- Recurrent chest infection
- Disturbed sleep/nightmares
- Poor concentration
If patients breathlessness cannot be controlled by non-invasive ventilation, how are they managed?
- Sedation - lorazepam
- coughing techniques
Certain MND patients can be emotionally labile. What does this mean?
- Inappropriate crying or laughing
- Part of the disease (Upper Motor Neuron sign)
