Motor Neurone Disease Flashcards Preview

Neurology > Motor Neurone Disease > Flashcards

Flashcards in Motor Neurone Disease Deck (39):
1

what is ALS

amyotrophic lateral sclerosis
is the most common MND phenotype in adults

2

how does MND present and progess

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

3

how much of MND is familial

10%
other 90% is sporadic

4

in scotland what is the median MND survival after symptom onset

3 years

5

how many cases of MND in scotland each year

200
incidence 1-3/100000 per year

6

what is the average age of diagnosis of MND

65

7

who gets MND

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

8

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

spinal limb (most common)
bulbar
cognitive
respiratory

9

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

amyotrophic lateral sclerosis
primary lateral sclerosis
progressive muscular atrophy
progressive bulbar palsy
congitive impairment

10

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

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

11

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

upper
good (>5 years)

12

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

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

variable prognosis

13

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

upper and lower
poor

14

what are the clinical phenotypes of MND

ALS
progressive muscular atrophy
primary lateral sclerosis
progressive bulbar palsy

15

what are upper motor neurone signs seen in ALS

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

16

what are lower motor neurone signs seen in ALS

muscle wasting
weakness
fasciculations
absent or reduced deep tendon reflexes
muscle cramps

17

what is pseudobulbar affect

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

18

what are the most common presentations of MND

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

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

MND katabolism 40-50%

19

what is MND katabolism

metabolism increased in MND
patients often very underwieght

20

what are the features of bulbar MND

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

21

what are the theraputic interventions for bulbar MND

early communicator, nutritional support, care for upper respiratory tract

22

what are the features of spinal dysfunction in MND

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

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

23

what is split hand syndrome

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

24

which ALS variants have the better prognosises

flail arm or leg syndromes (progressive muscular atrophy)
primary lateral sclerosis

25

what is the pathophysiology of MND

motor neurone degeneration/ death

genetic link - familial and sporadic
not well understood

26

what investigations into MND

electrophysiology

27

what is the diagnostic score for ALS

El Escorial

28

what is MND commonly misdiagnosed as

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

29

what are the treatment options for MND

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

30

what interventions might be needed for bulbar dysfunction

speech (dysarthria)- communication aids
swallowing (dysphagia)- hydration, nutrition, saliva, gastrostomy

31

why is extra nutrition needed in MND

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

32

what treatments can be given for sialorrhoea

hyoscine/ buscopan
glycopyrronium
botox
suction/ humidification/ carbocisteine

33

what treatment for muscle cramps

baclofen
quinine

34

what treatment for muscle spasms

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

35

what are the red flags of respiratory failure

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

36

what can be used for resp dysfunction

BiPAP mask - commenced at night

37

what treatment for SOB/ anxiety

lorazepam - palliative

38

what treatment for coughing

breath stacking
cough assist

39

what cognitive impairment is seen in MND

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