Lecture 12: Motor Neuron Disease Flashcards

1
Q

ALS breakdown

A
  • Amytrophy: muscle wasting LMN

- Lateral sclerosis: loss of lateral corticospinal tract UMN

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Treatment

A

Riuzole

  • increases lifespan by 3 months
  • NMDA antagonist
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Features of MND

A
  • characterised by upper +/- lower MN loss
  • sensation is preserved
  • Few groups of MN spared: sphincter control, extraocular muscles
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Symptoms

A

(-) muscle weakness, cramps, flaccid paralysis

(+) hyper reflexia, spastic paralysis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Diagnosis

A
  • Not definitive
  • Rule out other diseases
  • Motoneuronal degeneration determined by:
  • clinical tests
  • electrophysiological tests
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Types

A
  • sporadic: 95%

- familial: 5%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Sporadic

A
  • middle-late onset
  • Slight preference for males
  • UMN +/- LMN loss
  • muscle weakness, cramps, wasting, paralysis
  • death within 2 yrs due to resp-related illnesses
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Spinal Muscular Atrophy

A
  • progressive loss of LMNs
  • Types: infantile and juvenile
  • recessive mutation in SMN1
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Hypotheses on Causes of MND

A
  1. genetics
  2. excitotoxicity
  3. oxidative stress
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Excitotoxicity

A
  • Loss of glial glutamate transporters -> excessive glutamate -> act on NMDA receptors -> cell death
    Although:
  • only some reports of decreased glutamate transporters and increased glutamate in CSF
  • NMDA antagonist only extends like by 90 days:?
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Oxidative Stress

A
  • SOD-1 scavenges free-radicals
    -> mutation in SOD-1 -> increase in free radicals -> cell injury
    Although:
  • SOD1 mutation kill other cells too -> not specific to MN death as seen in MND
How well did you know this?
1
Not at all
2
3
4
5
Perfectly