Dementia and Motor Neuron Diseases

Question 1

What is dementia?

Answer 1

A set of symptoms that may include memory loss and difficulties with thinking, problem solving or language.
- There is a progressive decline in cognitive function.

Question 2

What is the epidemiology and aetiology of Dementia?

Answer 2

Ep: 10% over 65 and 20% over 80
Aetiology: Alzheimer’s disease, Vascular Dementia, Frontotemporal Dementia, Lewy Body Dementia and Vitamin D deficiency

Question 3

What is the main disease profile of Frontotemporal Dementia?

Answer 3

• Frontal Lobe atrophy on MRI
  Associated Disease: Motor Neuron disease
  Symptoms: Behaviour variants: Personality and Behaviour change, Language Variants and overlap with MND

Question 4

What is the main disease profile of Alzheimer’s disease?

Answer 4

• Temporal Lobe is affected
  1st Symptom: Short term memory impairment
• Apraxia, Gait defects, Selective Attention, Language Impairments: Difficulty in naming and understanding
  Histological Signs: Plaques of amyloid and Neuronal reduction
  Diagnoses: Braak staging criteria met and history of Dementia

Question 5

What investigations can be done in primary care to investigate dementia?

Answer 5

1) Good history of symptoms
2) 6CIT
3) Blood tests (Look for reversible causes of B12 deficiency)

Question 6

What secondary investigations can be done to investigate for dementia?

Answer 6

MRI
Amyloid and Tau histopathology
Brain Scans: PET scans and fMRI

Question 7

In what ways can dementia be prevented?

Answer 7

1) Stop smoking
2) Healthy diet
3) Regular exercise
4) Healthy weight
5) Low alcohol intake

Question 8

What medications may be used in dementia?

Answer 8

Acetylcholine esterase inhibitors

Question 9

What 3 diseases are associated with a damaged motor neuron pathway?

Answer 9

1) MND
2) Spinal cord compression
3) Spinal atrophy
4) Poliomyelitis

Question 10

What are the 3 main clinical patterns of MND?

Answer 10

1) Muscular Atrophy: Anterior horn cell lesion -> LMN, fasciculations, weakness, wasting
2) Amyotrophic Lateral Sclerosis: Loss of neurones in motor cortex and the anterior horn spinal cord (LMN and UMN signs) –> Progressive spastic tetraparesis
3) Progressive Bulbar Palsy: Destruction of CN IX-XII

Question 11

What is the long term symptom of ALS?

Answer 11

Progressive spastic tetraparesis

Question 12

What symptoms can be found in Progressive Bulbar Palsy?

Answer 12

1) Dysarthria
2) Dysphagia
3) Wasting and Fasciculations of Tongue

Question 13

What is the main treatment for MND?

Answer 13

Riluzole: Inhibits glutamate release and slows disease progression
Ventilatory support
Feeding by PEG

Question 14

What are the main S/S of hereditary spastic paraplegia?

Answer 14

1) Abnormal gait
2) Bladder problems
3) High arched feet
4) Increased tone in legs and minimal wasting
5) Family History