L9 Motor Neuron Flashcards

1
Q

What is the Spinal Cord

A

A long fragile structure that begins at brainstem and continues down the spine.

Consists of nerves responsible for incoming and outgoing messages between the brain and body

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2
Q

Spinal Nerves

A

Emerge in two branches known as anterior and posterior root

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3
Q

Anterior Root

A

Carries motor signals from brain to peripheries

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4
Q

Posterior Root

A

Carries sensory signals from the peripheries to the brain

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5
Q

Cauda Equine

A

Spinal Cord tapers towards the end and carries impulses to and from the legs

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6
Q

What does the CNS control and what neurons does it consist of

A

Controls skeletal muscle and consists of UMN and LMN

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7
Q

Upper Motor Neuron

A

Originate in primary motor cortex and pass through the internal capsule .

In spinal cord they form the lateral corticospinal tract and synapse with LMN .

Axons of upper motor neurons make up the pyramidal tracts and decussate forming the pyramids

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8
Q

Lower Motor Neurons

A

cell body located in spinal cord
Axons emerge from anterior root and joins with sensory fibres and form mixed nerves.

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9
Q

What is a motor unit

A

The motor end plates of each nerve and uscle fibres they support form a motorunit

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10
Q

What is Motor Neuron Disease

A

A group of neurodegeneratice disorders that selectively affect motor neurons, the cells which control the voluntary movement of the body

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11
Q

How is MND classified

A

Inherited or Sporadic

Locus of degeneration

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12
Q

Amytrophic Lateral Sclerosis

A

Both upper and lower motor neurons
Most common form of MND
3-5 years

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13
Q

Primary Lateral Sclerosis (PLS )

A

Upper motor neurons only
Up to 20 years for patients who dont develop ALS

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14
Q

Progressive Muscular Atrophy

A

Lower Motor Neurons only
Variable evolution to ALS
5 years

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15
Q

Progressive Bulbar Palsy

A

Form of ALS, eventually develops ALS
Impacts speech and swallowing first
2-3 years

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16
Q

Clinical presentation of ALS

A

Also known as Lou Gehrigs disease

-onset is asymetrical and distal
-muscle wasting and dasiculaitons
-weight loss and emotional lability
-5 years
long time to diagnose
cognitive and behavioural impairments

17
Q

Bulbar onset (Clinical Representation)

A

25-30% of patients
speech and swallowing
weight loss
difficulty breathing when lying flat
emotional lability
75% ALS will display bulbar

18
Q

Progressive Muslucar Atrophy (clinical representation)

A

Lower motor neuron
males
weakness and arm wasting, power is preserved
Respiration impaired, speech preserved
5 years

19
Q

Primary Lateral Sclerosis (Clinical Presentation)

A

Affects legs first, power is preserved
Urge incontinence
Emotional lability

20
Q

Neuropathology

A

Abnormal protein agregates or inclusions

21
Q

Familial MND

A

10% of cases
Autosomal Dominant
20% of these cases relate to SOD 1

22
Q

Pathogenic Mechanisms of ALS

A

Oxidative Stress
Excitotoxicity
Abnormal Protein precipitation and/or Aggregration
Cytoskeletal Defects
Axonal Transport
Neuroinflammation
Apoptosis
Lack of Growth Factors

23
Q

Pharmacotherapies

A

Riluzole (approved)
¨ Edaravone (approved since 2017)
¨ Ceftriaxone
¨ Tamoxifen
¨ Arimoclomol
¨ Stem cell therapies
¨ Antisense therapies
¨ Antioxidants

24
Q

Non pharacotherapy

A

OT
SLT
Nutritionalist
Auto cough
Suction Machine

25
Q
A