Motor Neuron Disease Flashcards

Supplementary to 3MB Neurology

1
Q

9/10 MND diagnoses are familial and 1/10 are sporadic. True/false?

A

False - 9/10 are sporadic and 1/10 familial

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2
Q

At what age range does sporadic MND tend to occur?

A

50-75 years

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3
Q

ALS is less common in caucasian/non-caucasian populations?

A

Less common in non-caucasian

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4
Q

PLS is a very ____ type of MND. It presents with ______ signs and without muscle ______.

A

PLS is a very rare type of MND. It presents with UMN signs and without muscle wasting.

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5
Q

Progressive Muscular Atrophy is a very ____ type of MND. It presents with ____ signs and with muscle ____ and ____.

A

Progressive Muscular Atrophy is a very rare type of MND. It presents with LMN signs and with muscle wasting and fasciculations.

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6
Q

ALS is the ____ ______ type of MND. It presents with ____ and ____ signs.

A

ALS is the most common type of MND. It presents with UMN and LMN signs.

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7
Q

Describe presentation of ALS patient.

A
muscle wasting, 
fasciculations,
increased tone,
brisk reflexes,
So UMN & LMN
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8
Q

Outline the presentation of progressive bulbar palsy MND?

A
wasted, fasciculated tongue,
brisk reflex, 
hypotonic nasal speech,
mixed UMN and LMN in mouth,
normal peripheries
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9
Q

Which type of MND is fastest killer?

A

ALS

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10
Q

What is PLS and is it common/rare?

A

Primary lateral sclerosis - is rare

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11
Q

List 6 signs of UMN disease.

A
  • spasticity
  • stronger reflexes
  • increased plantar response (Babinski sign)
  • spastic gait
  • slow movements
  • exaggerated jaw-jerk
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12
Q

List 4 signs of LMN disease.

A
  • muscle wasting
  • fasciculations
  • weakness
  • absent/reduced reflexes
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13
Q

What is the difference between bulbar and pseudobulbar palsy?

A

Bulbar is an LMN lesion of CN IX, X and XII and pseudobulbar is UMN lesion of CN IX, X and XII

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14
Q

What is MND Catabolism?

A

sudden weight loss that occurs in 50% patients with MND

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15
Q

Bulbar onset occurs in approx. 2%/25%/70% patients?
Limb onset occurs in approx. 2%/25%/70% patients?
Thoracic onset occurs in approx. 2%/25%/70% patients?

A

Bulbar - 25%
Limb - 70%
Thoracic - 2%

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16
Q

Is bulbar onset MND more common in men or women?

A

Women

17
Q

What are the 3 types of less fatal MND?

A
  • Flail arm syndrome
  • Flail leg syndrome
  • PLS
18
Q

What is the investigation for MND?

A
  • electrophysiology

- diagnosis of exclusion

19
Q

What type of drug is an option for MND patients and why do most patients refuse it?

A

Riluzole - it only extends life by 3 months and lots side effects (esp. liver)

20
Q

What is the management for MND patients with dysphagia?

A

ensuring hydration and nutrition,
saliva,
gastrostomy

21
Q

Name 4 management options for sialorrhoea.

A

hyoscine/buscopan,
glycopyrronium (especially if cognitively impaired),
botox,
suction

22
Q

Name 2 drugs for muscle cramps.

A
  • quinine

- baclofen

23
Q

Name 4 drugs for muscle spasms.

A
  • baclofen
  • tizanidine
  • dantrolene
  • gabapentin
24
Q

What non-invasive treatment can be offered for respiratory problems?

A

biPAP mask

25
Q

Approx. what percentage of patients have cognitive impairment with MND?

A

50%