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Flashcards in Motor neurone disease Deck (18):
1

Definition of motor neurone disease

Neurodegenerative disorder of upper and lower motor neurones

2

Combined degeneration of upper and lower motor neurones producing a mix of UMN and LMN symptoms

Amyotrophic lateral sclerosis (ALS)

3

Only show LMN signs, with better prognosis

Progressive muscular atrophy variant

4

Dysarthria and dysphagia, with wasted fasciculating tongue

Progressive bulbar palsy variant

5

UMN pattern of disease (weakness, brisk reflexes) without LMN signs

Primary lateral sclerosis variant

6

Gene implicated in 20% familial cases

SOD1 (superoxide dismutase)

7

MND never affects (4)

Sensation
Sphincters
Eye movements
Cerebellum

8

What does bulbar palsy mean?

Signs affecting IX-XII cranial nerves- nasal speech, nasal regurgitation of food, decreased gag reflex, absent jaw jerk

9

UMN signs (4)

Brisk reflexes; spasticity; plantars up; weakness

10

LMN signs

Wasting, fasciculation, weakness

11

Which other neurological disease is seen in 10-35% of MND patients?

Frontotemporal dementia

12

"Definite" ALS according to diagnostic criteria

LMN + UMN signs in 3 regions

13

What can the following investigations be useful for?
a) MRI
b) LP

Exclude structural (MRI) and inflammatory/infectious (LP) causes

14

Drug which has a modest (3 month) impact on survival

Riluzole (antiglutamergic)

15

End of life management options (2)

Consider PEG tube and home non-invasive ventilation

16

Usual cause of death in MND

Respiratory failure

17

Psychiatric symptoms/complications (4)

Depression
Emotional lability
Disinhibition
Frontal dementia

18

Symptomatic management of:
a) salivation
b) spasticity
c) dypsneoa and anxiety

a) anticholinergics (propantheline)
b) baclofen
c) opiates and benzodiazapenes