Motor Neurone Disease Flashcards
What is MND in one sentence?
-an untreatable, and rapidly progressive neurodegenerative condition
Is MND a UMN or LMN condition?
MND has a spectrum of condition with may present with a mix of BOTH UMN and LMN lesion signs or may be Purely LMN lesion/UMN lesion
How different is the muscular atrophy in a UMN lesion vs a LMN lesion?
- UMN lesion: DISUSE ATROPHY
- LMN lesion: DENERVATION ATROPHY (cutting off of the nerve supply to the skeletal muscle) +fasciculations
THUS BOTH WOULD HAVE WEAKNESS OF MUSCLE —confirm lesion site with BABINSKI Sign
Some symptoms of MND?
- muscle weakness
- difficulty with speech, swallowing, and breathing
- cognitive impairment
What are the patient demographics of MND?
- Males in their 50s-70s
- chance of MND declines after 80yrs
- more common in Caucasians
What is the spectrum of conditions under MND?
- ALS (Amyotrophic Lateral Sclerosis)
- PLS (Primary Lateral Sclerosis)
- PMA (progressive Muscular Atrophy)–10% of MND
- PBL (progressive bulbar palsy)
+/- cognitive impairment
Which MND condition is the most common?
- ALS
- –and it has the worst prognosis of 3-5years
What are cardinal UMN lesion signs?
- hyperreflexia +exagge. jaw jerk
- hypertonia
- spasticity (spastic gait)
- slowed movements
- extensor plantar reflexes (+) Babinski
- MODERATE muscle weakness
LMN lesion signs?
- hypotonia
- hyporeflexia
- fasciculation and cramps
- SEVERE muscle WEAKNESS and WASTING
Which MND condition allows the best lifestyle to be lived?
PLS - also a long standing condition with high life expectancy
What are the patient demographics for the BULBAR MND variant?
- women, 60-80s
- —almost always develops ALS
How to treat Bulbar variant of MND?
- early communicator
- nutritional support
- care for Upper Respiratory Tract
What is the general cause of death in Bulbar MND?
- d/t inability to swallow
- aspiration pneumonia
- choking
What is affected in Bulbar dysfxn?
Cranial Nerve 9, 10, 11, 12
- tongue muscles
- facial muscles
- pharyngeal muscles
- Siallorhea
What is a typical pattern of muscular atrophy seen in ALS?
-preferential wasting of the first dorsal interosseous and the ABDuctor pollicis brevis with the PRESERVATION of the abductor digiti minimi
—causes difficulty in daily activities (can’t turnover bed/dress themselves)
Why are patients with ALS more likely to develop UTIs and Pneumonia?
- patients are unable to get as much food in to them as before; with their difficulty in swallowing —their immunity gets weaker
What speech changes are noted in ALS?
- speech is NOT AS LOUD and clearly as it used to be
How does the LMN variant of MND present as?
- Flail arm/leg $ (limb is just not working)
How may a pt coming in with PLS present as?
- “not walking right on my feet”
- they present with severe LOSS of TONE in their legs
How to confirm the dx of MND?
- by electrophysiology and using the El Escorial criteria -only do scans of the head and neck, to r.o myelopathy ----scans SHOULD BE NORMAL in MND -EMG -myelogram - MRI of spine and brain - LP
What ddx could you consider in a pt with more UMN lesion signs?
- Multiple Sclerosis
What are common false +ve’s for MND?
- MMN (multifocal motor neuropathy), kennedy disease, myopathy, cervical spondylotic radiculomyelopathy
- carpal tunnel $, stroke, neuropathy
What is one drug could be given for MND?
- riluzole—said to prolong life for 3 extra months in the last few stages of MND
- has A LOT of s/e’s
What ongoing management is given to an MND pt?
- communication needs (SPEECH therapy)
- nutritional needs (gastronomy, dieticians)
- respiratory needs (home ventilation)
- riluzole
What care is given to an MND pt?
- designated MND team of 1 consultant and 2 nurse specialists
- deliver care and advise at HOME and at the clinic
How often are pts monitored?
- d/t the rapid progression of the condition, they are asked to be seen 4-6 times/week
What is of high importance when managing a patient with MND?
- ensure pt’s nutrition and hydration is thoroughly reviewed
- early insertion of gastrostomy tubes SHOULD BE CLINICAL PRIORITY
What gastrostomy tubes are used?
- PEG, RIG, PIGG, NG tubes
How to treat sialorrhea?
- botox injections to salivary glands
- suction/ humidification (mouth may get too dry—thrush may develop)
- hyoscine/ buscopan/ Glycopyrronium
How to symptomatically treat Muscle cramps?
- quinine, baclofen
Muscle spasms rx?
- baclofen
- tizanidine
- dantrolene
- gabapentin
What are the signs of Respiratory failure?
- breathlessness
- orthopnea
- recurrent CHEST INFECTION
- disturbed sleep
- nightmares
- daytime sleepiness
- poor concentration
- non-refreshed sleep
What should be done to manage type 2 respiratory failure?
non-invasive VENTILATION
BiPAP mask
What can be given to control cough?
Benzodiazepines
–for SOB and ANXIETY (lorazepam)
What kind of dementia is a/w MND?
-FRONTOTEMPORAL DEMENTIA
>apathy, disinhibition, poor planning
Which MND has no LMN symptoms?
- Primary Lateral Sclerosis
What is seen in a MND pt, in terms of Nutritional changes?
- matabolic rate in MND doubles (wgt loss is expected)
- wgt loss > 15% = poor survival ificator
