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NEUROLOGY > Motor Neurone Disease > Flashcards

Motor Neurone Disease Flashcards

1
Q

What is MND in one sentence?

A

-an untreatable, and rapidly progressive neurodegenerative condition

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2
Q

Is MND a UMN or LMN condition?

A

MND has a spectrum of condition with may present with a mix of BOTH UMN and LMN lesion signs or may be Purely LMN lesion/UMN lesion

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3
Q

How different is the muscular atrophy in a UMN lesion vs a LMN lesion?

A
  • UMN lesion: DISUSE ATROPHY
  • LMN lesion: DENERVATION ATROPHY (cutting off of the nerve supply to the skeletal muscle) +fasciculations

THUS BOTH WOULD HAVE WEAKNESS OF MUSCLE —confirm lesion site with BABINSKI Sign

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4
Q

Some symptoms of MND?

A
  • muscle weakness
  • difficulty with speech, swallowing, and breathing
  • cognitive impairment
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5
Q

What are the patient demographics of MND?

A
  • Males in their 50s-70s
  • chance of MND declines after 80yrs
  • more common in Caucasians
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6
Q

What is the spectrum of conditions under MND?

A
  • ALS (Amyotrophic Lateral Sclerosis)
  • PLS (Primary Lateral Sclerosis)
  • PMA (progressive Muscular Atrophy)–10% of MND
  • PBL (progressive bulbar palsy)

+/- cognitive impairment

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7
Q

Which MND condition is the most common?

A
  • ALS

- –and it has the worst prognosis of 3-5years

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8
Q

What are cardinal UMN lesion signs?

A
  • hyperreflexia +exagge. jaw jerk
  • hypertonia
  • spasticity (spastic gait)
  • slowed movements
  • extensor plantar reflexes (+) Babinski
  • MODERATE muscle weakness
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9
Q

LMN lesion signs?

A
  • hypotonia
  • hyporeflexia
  • fasciculation and cramps
  • SEVERE muscle WEAKNESS and WASTING
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10
Q

Which MND condition allows the best lifestyle to be lived?

A

PLS - also a long standing condition with high life expectancy

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11
Q

What are the patient demographics for the BULBAR MND variant?

A
  • women, 60-80s

- —almost always develops ALS

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12
Q

How to treat Bulbar variant of MND?

A
  • early communicator
  • nutritional support
  • care for Upper Respiratory Tract
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13
Q

What is the general cause of death in Bulbar MND?

A
  • d/t inability to swallow
  • aspiration pneumonia
  • choking
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14
Q

What is affected in Bulbar dysfxn?

A

Cranial Nerve 9, 10, 11, 12

  • tongue muscles
  • facial muscles
  • pharyngeal muscles
  • Siallorhea
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15
Q

What is a typical pattern of muscular atrophy seen in ALS?

A

-preferential wasting of the first dorsal interosseous and the ABDuctor pollicis brevis with the PRESERVATION of the abductor digiti minimi

—causes difficulty in daily activities (can’t turnover bed/dress themselves)

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16
Q

Why are patients with ALS more likely to develop UTIs and Pneumonia?

A
  • patients are unable to get as much food in to them as before; with their difficulty in swallowing —their immunity gets weaker
17
Q

What speech changes are noted in ALS?

A
  • speech is NOT AS LOUD and clearly as it used to be
18
Q

How does the LMN variant of MND present as?

A
  • Flail arm/leg $ (limb is just not working)
19
Q

How may a pt coming in with PLS present as?

A
  • “not walking right on my feet”

- they present with severe LOSS of TONE in their legs

20
Q

How to confirm the dx of MND?

A 
- by electrophysiology 
and using the El Escorial criteria 
-only do scans of the head and neck, to r.o myelopathy ----scans SHOULD BE NORMAL in MND
-EMG 
-myelogram 
- MRI of spine and brain 
- LP
21
Q

What ddx could you consider in a pt with more UMN lesion signs?

A
  • Multiple Sclerosis
22
Q

What are common false +ve’s for MND?

A
  • MMN (multifocal motor neuropathy), kennedy disease, myopathy, cervical spondylotic radiculomyelopathy
  • carpal tunnel $, stroke, neuropathy
23
Q

What is one drug could be given for MND?

A
  • riluzole—said to prolong life for 3 extra months in the last few stages of MND
  • has A LOT of s/e’s
24
Q

What ongoing management is given to an MND pt?

A
  • communication needs (SPEECH therapy)
  • nutritional needs (gastronomy, dieticians)
  • respiratory needs (home ventilation)
  • riluzole
25
Q

What care is given to an MND pt?

A
  • designated MND team of 1 consultant and 2 nurse specialists
  • deliver care and advise at HOME and at the clinic
26
Q

How often are pts monitored?

A
  • d/t the rapid progression of the condition, they are asked to be seen 4-6 times/week
27
Q

What is of high importance when managing a patient with MND?

A
  • ensure pt’s nutrition and hydration is thoroughly reviewed
  • early insertion of gastrostomy tubes SHOULD BE CLINICAL PRIORITY
28
Q

What gastrostomy tubes are used?

A
  • PEG, RIG, PIGG, NG tubes
29
Q

How to treat sialorrhea?

A
  • botox injections to salivary glands
  • suction/ humidification (mouth may get too dry—thrush may develop)
  • hyoscine/ buscopan/ Glycopyrronium
30
Q

How to symptomatically treat Muscle cramps?

A
  • quinine, baclofen
31
Q

Muscle spasms rx?

A
  • baclofen
  • tizanidine
  • dantrolene
  • gabapentin
32
Q

What are the signs of Respiratory failure?

A
  • breathlessness
  • orthopnea
  • recurrent CHEST INFECTION
  • disturbed sleep
  • nightmares
  • daytime sleepiness
  • poor concentration
  • non-refreshed sleep
33
Q

What should be done to manage type 2 respiratory failure?

A

non-invasive VENTILATION

BiPAP mask

34
Q

What can be given to control cough?

A

Benzodiazepines

–for SOB and ANXIETY (lorazepam)

35
Q

What kind of dementia is a/w MND?

A

-FRONTOTEMPORAL DEMENTIA

>apathy, disinhibition, poor planning

36
Q

Which MND has no LMN symptoms?

A
  • Primary Lateral Sclerosis
37
Q

What is seen in a MND pt, in terms of Nutritional changes?

A
  • matabolic rate in MND doubles (wgt loss is expected)

- wgt loss > 15% = poor survival ificator

NEUROLOGY (36 decks)

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