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MS4 - Neurology > Motor Neuron Disease > Flashcards

Motor Neuron Disease Flashcards

1
Q

Definition of MND

A

Upper and lower motor neurons affected

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2
Q

How to distinguish MND from MS or polyneuropathy

A

MND has no sensory loss and no sphincter disturbance

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3
Q

How to distinguish MND from myasthenia gravis

A

MND never affects eye movements

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4
Q

Inheritance of MND

A

Autosomal dominant

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5
Q

Types of MND

A

Amyotrophic lateral sclerosis (ALS)

Progressive bulbar palsy

Progressive muscular atrophy

Primary lateral sclerosis

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6
Q

ALS definition

A

Loss of motor neurons in motor cortex and anterior horn of cord

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7
Q

Sx of ALS

A

Weakness

UMN signs (upgoing plantars)

LMN signs (fasciculations)

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8
Q

Progressive bulbar palsy definition

A

Only affects cranial nerves

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9
Q

Types of progressive blubar palsy

A

Bulbar palsy

Corticobulbar palsy (pseudobulbar palsy)

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10
Q

Bulbar palsy definition

A

Disease of nuclei of cranial nerves 9-12 in medulla

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11
Q

Signs of bulbar palsy

A

LMN lesions of cranial nerves 9-12

Flaccid fasciculating tongue

Jaw jerk normal or absent

Quiet hoarse voice

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12
Q

Causes of bulbar palsy

A

MND

Guillain Barre

Myaesthenia gravis

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13
Q

Corticobulbar palsy definition

A

UMN lesion of muscles of swallowing and talking

Due to bilateral lesions above mid-pons (corticobulbar tracts)

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14
Q

Causes of corticobulbar palsy

A

MS

MND

Stoke

Central pontine myelinolysis

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15
Q

Signs of corticobulbar palsy

A

Slow tongue movements

Slow deliberate speech

Jaw jerk hyperreflexia

Pseudobulbar affect

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16
Q

Pseudobulbar affect

A

Emotional incontinence without mood change

Eg. weeping w/o sorrow

Eg. incongruent giggling

17
Q

Progressive muscular atrophy definition

A

Anterior horn cell lesion only

18
Q

Signs of progressive muscular atrophy

A

No UMN signs

Affects distal muscles before proximal

19
Q

Primary lateral sclerosis definition

A

Loss of Betz cells in motor cortex

20
Q

Signs of primary lateral sclerosis

A

UMN signs

Spastic leg weakness

Pseudobulbar palsy

No cognitive decline

21
Q

Association to MND

A

Fronto-temporal dementia

22
Q

Classic MND patient

A

> 40 yrs

Stumbling spastic gait

Foot drop

Proximal myopathy

23
Q

Diagnostic criteria for MND

A

Definite

Probable

Probably with lab support

Possible

Suspected

24
Q

Definite diagnostic criteria for MND

A

LMN + UMN signs in 3 regions

25
Q

Probable diagnostic criteria for MND

A

LMN + UMN signs in 2 regions

26
Q

Probably with lab support diagnostic criteria for MND

A

LMN + UMN signs in 1 region

OR

UMN signs in 1 or more regions

+ EMG shows acute denervation in 2 or more limbs

27
Q

Possible diagnostic criteria for MND

A

LMN + UMN signs in 1 region

28
Q

Suspected diagnostic criteria for MND

A

UMN or LMN signs only

1 or more regions

29
Q

Prognosis of MND

A

Often fatal in 2-4 yrs

30
Q

Mx of MND

A

MDT

Riluzole

Propantheline

Blend food/NG tube/PEG for dysphagia

Non-invasive ventilation

31
Q

Riluzole

A

Antiglutamatergic drug

Prolongs life ~ 3 months

Expensive

32
Q

SEs of riluzole

A

Raises LFTs

Vomiting

Tachycardia

Headache

33
Q

Propantheline use

A

For drooling in MND

MS4 - Neurology (11 decks)

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