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CNS CLERKSHIP > MND Disease > Flashcards

MND Disease Flashcards

1
Q

MND patients present with what signs?

A

Muscle weakness and wasting secondary to motor neuron degeneration.

  • UMN and/or LMS signs without sensory problems.
  • Focal onset and continuous spread with final generalised paresis.
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2
Q

MND causes what symptoms?

A
  • Muscle weakness (and wasting).

- Difficulties with speech, swallowing and breathing.

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3
Q

Are cases of MND more often familial or sporadic?

A
  • 10% familial.

- 90% sporadic.

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4
Q

What is the most common motor neuron disease phenotype in adults?

A

Amyotrophic lateral sclerosis - ALS = “Lou Gehrig’s disease”.

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5
Q

How is MND diagnosed?

A

Mainly clinical based on symptoms and signs.

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6
Q

Prognosis for MND?

A

Untreatable and rapidly progressive neurodegenerative condition.

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7
Q

What is average survival time of MND?

A

3 years.

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8
Q

What is the lifetime risk of developing MND?

A

1:400.

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9
Q

Sporadic MND peaks at what ages?

A

50-75 years and declines after 80.

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10
Q

ALS is more common in which populations?

A

Caucasians.

Less common in non-caucasian populations.

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11
Q

What UMN signs may be seen in a patient with amyotrophic lateral sclerosis?

A
  • Increased tone.
  • Hyper-reflexia.
  • Extensor plantar responses.
  • Spastic gait.
  • Exaggerated jaw-jerk.
  • Slowed movements.
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12
Q

What LMN signs may be seen in a patient with amyotrophic lateral sclerosis?

A
  • Muscle wasting and cramp.
  • Weakness.
  • Fasciculations.
  • Absent or reduced deep tendon reflexes (hyporeflexia).
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13
Q

what is the Babinski sign?

A

On stroking the sole of the foot, the big toe then moves superiorly.

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14
Q

what is a Bulbar palsy?

A

Signs and symptoms reflecting impaired function of CN IX, X, XI and XII.

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15
Q

What are bulbar UMN signs e.g. seen in MND?

A
  • Exaggerated snout reflex.
  • Clonic jaw jerk.
  • Emotional lability.
  • Forced yawning.
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16
Q

What signs of UMN disease will be seen in the cervical and lumbar region?

A
  • Clonic deep tendon reflexes.
  • Preserved reflex in a weak, wasted limb.
  • Hoffmann reflex.
  • Hyperreflexia.
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17
Q

What is the Hoffman reflex?

A

Indicates UMN lesion affecting cervical and lumbar regions. Or can be entirely normal.

  • Hold middle finger and flick fingernail downward: positive if there is flexion and adduction of the thumb on the same hand.
18
Q

UMN signs which may be clinically detected?

A
  • Spasticity.
  • Babinski sign.
  • Bulbar UMN signs.
  • Cervical and lumbar region symptoms.
  • Loss of superficial abdominal reflexes.
19
Q

Give a typical pattern of atrophy in ALS?

A
  • Preferential wasting of thenar group of hand.

(First dorsal interosseous, abductor pollicis brevis wasting and preservation of abductor digiti minimi).

20
Q

Primary bulbar onset occurs in what percentage of MND patients?

A

-25%.

21
Q

Is the bulbar variant of MND more common in men or women?

A

Woman.

22
Q

In whihc age group is the bulbar variant of MND most common?

A

60-80 years.

23
Q

What therapeutic interventions may be offered to those with bulbar variant MND?

A
  • Early communicator.
  • Nutritional support.
  • Care for URT.
24
Q

There is always a generalisation of Bulbar Variant MND into what?

A

ALS.

25
Q

Other than ALS, what are the other phenotypes of MND?

A
  • Progressive muscular atrophy (PML).
  • Primary lateral sclerosis (PLS).
  • Progressive bulbar palsy (PBP).
26
Q

There is a link between ALS and what form of dementia (discovery of C9ORF72 hexanucleotide repeat expansions)?

A

Frontotemporal dementia.

27
Q

Prognosis of ALS (MND)?

A

Poor, median survival 3-5 years.

28
Q

Prognosis of primary lateral sclerosis (MND)?

A

Good, survival >5 years.

29
Q

Prognosis of ALS-frontotemporal dementia (MND)?

A

Poor - test for C90RF72 gene.

30
Q

Prognosis of progressive muscular atrophy (MND)?

A

Variable for both flail arm and flail leg variant ALS.

31
Q

What are the diagnostic criteria for ALS?

A
  • LMN degeneration signs.
  • UMN degeneration signs.
  • Progressive spread of signs within a region or to other regions with the absence of: evidence of other disease processes responsible for LMN and/or UMN degeneration and neuroimagining suggesting disease processes possibly explaining clinical and electrophysiological signs.
32
Q

Clinically definite ALS criteria?

A
  • UMN and LMN signs in bulbar and at least two spinal (lumbosacral, thoracic or cervical regions OR:
  • UMN and LMN signs three spinal regions.
33
Q

Clinically probable ALS criteria?

A
  • UMN and LMN signs in at least two regions (bulbar or spinal).
    + UMN signs rostral to the LMN signs.
34
Q

Clinically probably ALS = laboratory supported?

A
  • Clinical evidence of UMN and LMN signs in one body region and EMG findings of LMN involvement in at least two body regions.
35
Q

Clinically possible ALS?

A 
- UMN and LMN signs in only bulbar or only one spinal region.
OR
- UMN signs in 2 or more regions.
OR
- LMN signs rostral to UMN signs.
36
Q

How is MND diagnosed?

A
  • Consensus clinical critera + UMN/LMN signs and an absence of sensory symptoms.
  • Limb onset, bulbar onset and/or cognitive behavioural symptoms.
  • Diagnosis of exclusion: neuroimaging and laboratory studies to rule out other diseases.
37
Q

What is the most frequent surgical intervention in MND?

A
  • Decompressive spinal surgery.
38
Q

Management of MND?

A
  • Access to specialist MND services.

- On-going management.

39
Q

What is part of the on-going management of MND?

A
  • Key worker assessment (needs & care co-ordination).
  • Communication needs (speech therapy etc).
  • Nutritional needs (dietitians, gastrostomy).
  • Resp. needs (assessment, home ventilation).
  • Riluzole.
40
Q

What has been identified as the pathological signature in 95% of cases of MND?

A

TDP43 inclusions.

CNS CLERKSHIP (43 decks)

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