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Neurology > UMN, LMN lesions and MND > Flashcards

UMN, LMN lesions and MND Flashcards

1
Q

What is the difference between a bulbar palsy and a pseudo bulbar palsy?

A

A bulbar palsy is a lower motor neuron lesion of cranial nerves IX, X and XII. A pseudobulbar palsy is an upper motor neuron lesion of cranial nerves IX, X and XII.

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2
Q

What is motor neuron disease?

A

untreatable and rapidly progressive neurodegenerative condition, it causes progressive weakness and eventually death usually as a result of respiratory failure or aspiration

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3
Q

MND is more common in men or women?

A

men

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4
Q

People usually present with MND between the ages of _______

A

50-75

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5
Q

Describe genetics of MND?

A

can be familial or sporadic

C9orf72 gene mutation found in many familial cases

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6
Q

List the four types of MND?

A

ALS (amyotrophic lateral sclerosis)
PMA (progressive muscular atrophy)
Progressive bulbar palsy
Primary lateral sclerosis

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7
Q

What is the most common type of MND?

A

ALS

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8
Q

ALS causes ________

A

upper and lower motor neuron damage

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9
Q

Describe presentation of ALS?

A

Progressive focal muscle weakness and wasting with muscle fasciculations due to spontaneous firing of abnormally large motor units formed by surviving axons branching to innervate muscle fibres that have lost their nerve supply. Weakness gradually spreads to other limbs. Cramps are common. On exam there are UMN signs e.g. brisk reflexes, extensor plantar responses and spasticity.

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10
Q

What is the prognosis of ALS?

A

very poor, it is rapidly progressive survive up to 3 years on average usually

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11
Q

PMA causes _____

A

only lower motor neuron degeneration

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12
Q

Describe presentation of PMA?

A

Weakness, muscle wasting, fasciculations that start in one limb and spread

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13
Q

What is prognosis of PMA like?

A

variable

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14
Q

Progressive bulbar palsy causes ________

A

lower cranial nerve nuclei and supranuclear connections degeneration

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15
Q

Presentation of progressive bulbar palsy?

A

dysarthria, dysphagia, nasal regurgitation, choking, fasciculating tongue with slow stiff tongue movements

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16
Q

Primary lateral sclerosis causes _______

A

Upper motor neuron degeneration only

17
Q

What is the least common type of MND?

A

PLS

18
Q

Some common presenting complaints with MND?

A 
Bulbar symptoms
foot drop
difficulty writing, using hands
difficulty breathing
hyper metabolic state > extreme weight loss
19
Q

Describe how diagnosis of MND is made?

A

largely clinical, no diagnostic tests but investigations allow exclusion of other conditions
denervation of muscles due to degeneration of LMNs is confirmed by EMG
certain diagnostic criteria

20
Q

Describe the diagnostic criteria for MND?

A

• Signs of lower motor neuron (LMN) degeneration by clinical, electrophysiological or neuropathologic examination,
• Signs of upper motor neuron (UMN) degeneration by clinical examination, and
• Progressive spread of signs within a region or to other regions, together with the absence of:
• Electrophysiological evidence of other disease processes that might explain the signs of LMN and/or UMN degenerations; and
Neuroimaging evidence of other disease processes that might explain the observed clinical and electrophysiological signs

21
Q

Describe management of MND?

A

No treatment improves outcome substantially
Riluzole by 3-4 months but many don’t take the drug as gives you extra time when most disabled
MDT approach
Communication aids
Dietitician advice
Gastrostomy
Physio
Baclofen for muscle spasm
Non invasive ventilatory support- BiPAP at night

22
Q

Define spasticity?

A

velocity dependent increase in tone, the faster you move the muscle the greater the resistance

23
Q

Define rigidity?

A

Velocity independent increase in tone present throughout muscle movement (usually associated with lesions of basal ganglia)

24
Q

Define Babinksi sign?

A

AKA extensor plantar response

big toe bends up and back and other toes fan out

25
Q

Define clonus?

A

sequence of rhythmic and involuntary muscle contractions

26
Q

Define fasciculations?

A

involuntary twitching of muscles

27
Q

Define hypertonia?

A

increased muscle tone

28
Q

Define hypotonia?

A

decreased muscle tone

29
Q

Define hyporeflexia?

A

under responsive reflexes

30
Q

5 UMN signs?

A
  • Spasticity
  • Babinski sign
  • Hypertonia
  • Hyperreflexia
  • Clonus
31
Q

3 LMN signs?

A
  • Hypotonia
  • Hyporeflexia
  • Fasciculations
32
Q

6 causes of pure UMN signs?

A
  • Stroke
  • Multiple Sclerosis
  • Cerebral palsy
  • Spinal cord injury
  • Huntingtons disease
  • PLS
33
Q

2 causes of pure LMN signs?

A
  • Any kind of peripheral neuropathy

* Progressive muscular atrophy

34
Q

A cause of mixed UMN and LMN signs?

A

ALS

Neurology (22 decks)

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