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Neuro > Motor Neurone Disease > Flashcards

Motor Neurone Disease Flashcards

1
Q

What causes MND?

A

Selective loss of neurones in motor cortex , cranial nerve nuclei and anterior horn cells

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2
Q

Prognosis with MND?

A

Average 3 years

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3
Q

Typical onset of MND?

A

Aged 50-75 years old

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4
Q

Who is MND more common in ?

A

Males

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5
Q

Feature of motor neurone disease?

A

UMN and or LMN signs without sensory problems

Muscle weakness -> problems with speech, swallowing, breathing

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6
Q

UMN sings in MND?

A 
Increased tone hyper reflexia
Extensors plantar responses - babinski 
Spastic gait. 
Exaggerated jaw reflex
Slowed movements 
Hoffman reflex
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7
Q

LMN signs in MND?

A

Muscle wasting
Weakness
Fasciculations
Absent or reduced deep tendon reflexes

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8
Q

What is split hand syndrome?

A

Wasting of thenar group - atrophy seen in ALS

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9
Q

Phenotypes of MND?

A 
ALS 
ALS-frontotemporal dementia
Primary lateral sclerosis 
Progressive muscular atrophy
Progressive bulbar palsy
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10
Q

Management of MND?

A

Special services
MDT
Communication needs
Nutritional needs - dieticians, gastrostomy
Respiratory needs - home ventilation
Riluzole - prolongs life by 3 months
Propantheline/ amitriptyline for drooling

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11
Q

What is riluzole?

A

An antiglutamatergic drug

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12
Q

Diagnosis of MND?

A

Clinical criteria
Diagnosis of exclusion
Limb onset, bulbar onset and or cognitive behavioural symptoms
Neuro imaging and lab studies to exclude other diagnoses

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13
Q

What is the diagnostic criteria used for MND?

A

El Escorial

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14
Q

El Escorial definite criteria?

A

UMN and LMN in 3 regions

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15
Q

El Escorial probable criteria?

A

UMN and LMN in 2 regions

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16
Q

El Escorial possible criteria?

A

UMN and LMN in 1 region

17
Q

El Escorial suspected criteria?

A

UMN or LMN in > one region

18
Q

Which is the most common phenotype of MND?

A

ALS

19
Q

Prognosis of ALS?

A

Poor

20
Q

Features of ALS?

A

UMN and LMN features

21
Q

Prognosis of ALS frontotemporal dementia?

A

Poor

22
Q

Features of ALS frontotemporal dementia?

A

UMN and LMN features

23
Q

Prognosis of primary lateral sclerosis?

A

Good : > 5 years

24
Q

Features of primary lateral sclerosis?

A

UMN features

25
Q

Features of progressive muscular atrophy?

A

LMN features

26
Q

Prognosis of progressive muscular atrophy?

A

Variable survival

27
Q

Features of progressive bulbar palsy ?

A

Women more than men.
Affects CN IX -XII
effects tongue and muscles of talking and swallowing

Neuro (19 decks)

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