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Neurology > Motor Neurone Disease > Flashcards

Motor Neurone Disease Flashcards

1
Q

what is ALS

A

amyotrophic lateral sclerosis

is the most common MND phenotype in adults

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2
Q

how does MND present and progess

A

muscle weakness and wasting secondary to motor neuron degeneration
speech, swallowing and breathing problems

upper AND lower MN signs WITHOUT sensory problems

focal onset and continuous spread, finally generalised paresis
cognitive impairment

rapid progression

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3
Q

how much of MND is familial

A

10%

other 90% is sporadic

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4
Q

in scotland what is the median MND survival after symptom onset

A

3 years

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5
Q

how many cases of MND in scotland each year

A

200

incidence 1-3/100000 per year

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6
Q

what is the average age of diagnosis of MND

A

65

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7
Q

who gets MND

A

slightly more common in males
sporadic peaks at ages 50-75
declines after the age of 50
less common in non caucasian populations

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8
Q

what regions of onset can be affected by different types of MND

A

spinal limb (most common)
bulbar
cognitive
respiratory

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9
Q

motor neurone disease is an umbrella term- what does it encompass

A 
amyotrophic lateral sclerosis 
primary lateral sclerosis 
progressive muscular atrophy 
progressive bulbar palsy
congitive impairment
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10
Q

are there upper or lower MN features in ALS and what is the prognosis

A 
both upper and lower
poor prognosis (3-5 years)
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11
Q

are there upper or lower MN features in PLS and what is the prognosis

A

upper

good (>5 years)

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12
Q

are there upper or lower MN features in progressive muscular atrophy (flail arm and leg variants) and what is the prognosis

A

lower MN signs
subclinical upper, clinical upper MN signs in 30%

variable prognosis

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13
Q

are there upper or lower MN features in ALS- frontotemporal dementia and what is the prognosis

A

upper and lower

poor

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14
Q

what are the clinical phenotypes of MND

A

ALS
progressive muscular atrophy
primary lateral sclerosis
progressive bulbar palsy

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15
Q

what are upper motor neurone signs seen in ALS

A 
increased tone 
hyper-reflexia 
extensor plantar responses (babinksi) 
spastic gait
exaggerated jaw jerk 
slowed movements
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16
Q

what are lower motor neurone signs seen in ALS

A 
muscle wasting 
weakness
fasciculations 
absent or reduced deep tendon reflexes 
muscle cramps
17
Q

what is pseudobulbar affect

A

episodes of sudden, uncontrollable and inappropriate laughing or crying
get is in ALS

18
Q

what are the most common presentations of MND

A

extremities 70% (upper>lower)
bulbar 25%
thoracic 2%

UMN 10%
LMN 90%
frontal/ cognitive (5-90%)

MND katabolism 40-50%

19
Q

what is MND katabolism

A

metabolism increased in MND

patients often very underwieght

20
Q

what are the features of bulbar MND

A

affected women more than men
60-80 y/o
always generalises in to ALS
affects tongues, facial and pharyngeal muscles

21
Q

what are the theraputic interventions for bulbar MND

A

early communicator, nutritional support, care for upper respiratory tract

22
Q

what are the features of spinal dysfunction in MND

A

muscle wasting
loss of tone/ contractures
affectes upper and lower limbs

creates difficulty in everyday activities. mobility aids/ hoists often necessary

23
Q

what is split hand syndrome

A

preferential wasting of thenar group (first dorsal interosseous and adbuctor pollicis brevis)
pattern of atrophy seen in ALS

24
Q

which ALS variants have the better prognosises

A

flail arm or leg syndromes (progressive muscular atrophy)

primary lateral sclerosis

25
Q

what is the pathophysiology of MND

A

motor neurone degeneration/ death

genetic link - familial and sporadic
not well understood

26
Q

what investigations into MND

A

electrophysiology

27
Q

what is the diagnostic score for ALS

A

El Escorial

28
Q

what is MND commonly misdiagnosed as

A

carpal tunnel, stroke, neuropathy, mutlifocal motor neuropathy, kennedys disease, myopathy, cervical spondylotic radiculopathy

29
Q

what are the treatment options for MND

A

symptomatic
riluzole
interventions:
-communications (speech therapy, voice banking)
-nutritional (dieticians, grastrotomy)
-respiratory needs (assessment, home ventilation)

30
Q

what interventions might be needed for bulbar dysfunction

A

speech (dysarthria)- communication aids

swallowing (dysphagia)- hydration, nutrition, saliva, gastrostomy

31
Q

why is extra nutrition needed in MND

A

metabolic rate is doubled

need small high energy supplements regularly/ early insertion of gastrostomy/ food thickeners/ liquid drug preparations

32
Q

what treatments can be given for sialorrhoea

A

hyoscine/ buscopan
glycopyrronium
botox
suction/ humidification/ carbocisteine

33
Q

what treatment for muscle cramps

A

baclofen

quinine

34
Q

what treatment for muscle spasms

A

baclofen (dont give too much as high tone may be what is allowing movements and independence)
tizanidine
dantrolene
gabapentin

35
Q

what are the red flags of respiratory failure

A 
breathlessness 
orthopnoea 
recurrent chest infection
disturbed sleep 
non refreshed sleep 
night mares 
daytime sleepiness 
poor concentration
36
Q

what can be used for resp dysfunction

A

BiPAP mask - commenced at night

37
Q

what treatment for SOB/ anxiety

A

lorazepam - palliative

38
Q

what treatment for coughing

A

breath stacking

cough assist

39
Q

what cognitive impairment is seen in MND

A 
in half of patients 
spectrum of severity 
Associated with Frontotemporal Dementia
Apathy, disinhibition, poor planning/decision making
emotional liability

Neurology (35 decks)

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