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Neurology > Motor Neuron Disease > Flashcards

Motor Neuron Disease Flashcards

1
Q

What is motor neuron disease?

A

Untreatable and rapidly progressive neurodegenerative disease = mainly clinical diagnosis, variable clinical phenotypes

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2
Q

What are the main features of motor neuron disease?

A

Muscle weakness
Cognitive impairment
Issues with speech, swallow or breathing
UMN and/or LMN signs without sensory problems

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3
Q

What is the progression of motor neuron disease?

A

Focal onset and continuous spread = results in generalised paraesthesiae
Median survival is 3 years

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4
Q

What is the most common subtype of motor neuron disease?

A

Amyotrophic lateral sclerosis = worst prognosis and has mixed UMN and LMN signs, less common in non-Caucasians

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5
Q

What is the epidemiology of motor neuron disease?

A

Slightly more common in men
90% sporadic and 10% familial
Sporadic peaks at age 50-75 (declines after age 80)

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6
Q

What is the genetic occurrence of motor neuron disease?

A

Genetic aetiology has been identified in up to 20% of sporadic and 60% of familial cases

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7
Q

What are the different kinds of motor neuron disease?

A

Primary lateral sclerosis
Progressive muscular atrophy
Progressive bulbar palsy

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8
Q

What are some features of primary lateral sclerosis?

A

Rarest subtype

Presents with mostly UMN signs

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9
Q

What are some features of progressive muscular atrophy?

A

LMN variant of primary lateral sclerosis

Has variable prognosis

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10
Q

What is progressive bulbar palsy?

A

Bulbar variant = mixed upper and lower motor neuron signs but only confined to mouth

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11
Q

What are the UMN signs of motor neuron disease?

A

Pseudobulbar affect
Moderate weakness
Spasticity and hyperreflexia
Extensor plantar reflexes

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12
Q

What are the LMN signs of motor neuron disease?

A

Severe weakness and muscle cramps
Fasciculations
Hypotonicity and hyporeflexia
Muscle atrophy

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13
Q

What part of the body do most patients present with symptoms in?

A

The limbs = extremities (70%, upper>lower), bulbar (25%) and thoracic (2%)

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14
Q

What signs are most prominent?

A

LMN signs

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15
Q

What is motor neuron disease katabolism?

A

Increased metabolic rate = occurs in 40-50%

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16
Q

What are some features of progressive bulbar palsy?

A

More common in women aged 60-80
Always generalises into ALS
Affects tongue, facial and pharyngeal muscles
May need early communicator and nutritional support

17
Q

What is split hand syndrome?

A

Preferential wasting of thenar group = seen occasionally in ALS

18
Q

What mutation has been implicated in motor neuron disease?

A

C9orf72 expansion = present in 41% of familial ALS and 5-10% of sporadic ALS

19
Q

What are some variants of ALS?

A

Flail arm/leg syndrome = one limb affected
Focal distal spinal muscular dystrophy
All have more benign prognosis

20
Q

What is used to diagnose ALS?

A

Diagnosis of exclusion

Use neuroimaging and El Escorial criteria

21
Q

How is suspected motor neuron disease investigated?

A

Electrophysiology

22
Q

What is the underlying disease process of motor neuron disease?

A

Motor neuron degeneration and death

23
Q

What are some conditions motor neuron disease is misdiagnosed as?

A

Carpal tunnel syndrome, stroke and neuropathy

24
Q

How common are false positive diagnoses of motor neuron disease?

A

7-10% of cases

25
Q

What are some conditions which are mistaken for motor neuron disease?

A

Multifocal motor neuropathy, Kennedy’s disease, myopathy, cervical spondylotic radiculomyelopathy

26
Q

What is an example of a drug used to treat motor neuron disease?

A

Riluzole = gives patient extra three moths at end of disease, needs blood monitoring as affects kidneys and liver

27
Q

Where are patients with motor neuron disease referred?

A

Specialist MND services = allocated to one consultant and two nurse specialists at time of diagnosis, seen 4-6 weekly if needed

28
Q

What is a poor survival indicator in motor neuron disease?

A

Weight loss > 10% at diagnosis

29
Q

How are communication difficulties managed?

A

Using AACs

30
Q

What are some features of a gastrostomy?

A

Either PEG, RIG, PIGG or NG used
Carers may need to be involved in maintenance
Narrow window for effectiveness

31
Q

What are some treatments for sialorrhoea?

A

Hyoscine or buscopan orally
Glycopyrronium if cognitive impairment
Botox and suction also options

32
Q

How are muscle cramps treated?

A

Quinine or baclofen

33
Q

How are muscle spasms treated?

A

Baclofen, tizanidine, dantrolene or gabapentin

34
Q

What is one of the main causes of death in motor neuron disease?

A

Weakness of respiratory muscles = treated with non-invasive ventilation, BiPAP commenced at night initially and gradually increased

35
Q

What is cognitive impairment associated with?

A

Frontotemporal dementia

36
Q

How does cognitive impairment present in motor neuron disease?

A

Apathy, disinhibition, poor planning/decision making, inappropriate laughing or crying

37
Q

What are some respiratory red flags?

A

SOB, orthopnoea, recurrent chest infections, disturbed or non-refreshed sleep, nightmares, daytime sleepiness, poor concentration

Neurology (48 decks)

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