10. MS and Neurodegeneration

Question 1

What is MS?

Answer 1

Immune-mediated destruction of myelin in the CNS

Question 2

What cells produce myelin in the CNS?

Answer 2

Oligodendrocytes

Question 3

What are the symptoms of MS?

Answer 3

Visual loss 
Acute transverse myelitis
Trigeminal neuralgia
Diplopia, balance disturbance and vertigo
Bladder dysfunction

Question 4

What is Lhermitte’s sign?

Answer 4

Neck flexion causes rapid tingling or electric shock feeling passing from spine to limbs

Question 5

What is Uhtoff’s phenomenon?

Answer 5

Symptoms are exacerbated at increased temperatures

Question 6

What is the classification of MS?

Answer 6

Clinically isolated syndrome
Relapsing and remitting
Primary progressive
Secondary progressive

Question 7

What is the composition of an active MS plaque?

Answer 7

Lymphocytes

Macrophages containing myelin debris

Question 8

How is active MS detected on an MRI?

Answer 8

BBB is disrupted to allow inflammatory cells through, meaning contrast will also seep through, causing enhancement

Question 9

What is the composition of a chronic MS plaque?

Answer 9

Reduced myelin
Astrocytes proliferation
Gliosis

Question 10

What are the genetic components of MS?

Answer 10

HLA

IL-2 and IL-7 receptors

Question 11

What locations are MS plaques commonly found in?

Answer 11

Near ventricles
Optic nerves
Brain stem
Cerebellum
Spinal cord

Question 12

How is MS diagnosed?

Answer 12

Clinically
McDonald criteria: plaques disseminated over time and space
CSF analysis
Visually evoked potential

Question 13

What is seen in CSF in MS?

Answer 13

Mildly increased protein level
Oligoclonal bands
1/3 of cases show moderate pleocytosis

Question 14

What is the acute management of MS?

Answer 14

Steroids

Plasma exchange

Question 15

What is the long term management of MS?

Answer 15

Disease modifying therapy started ASAP

Question 16

What is the definition of neurodegeneration?

Answer 16

Progressive dysfunction and death of neurons

Question 17

What are the features of neurodegeneration?

Answer 17

Neuronal loss
Gliosis
Intracellular accumulation of abnormal proteins

Question 18

What does the presence of ubiquitin suggest?

Answer 18

Increased protein elimination; may be a protein accumulating that hasn’t been recognised yet

Question 19

What is the usual function of tau?

Answer 19

Stabilises microtubules in neurons

Question 20

What part of the brain is affected in Alzheimer’s disease?

Answer 20

Hippocampus

Question 21

What proteins are deposited in Alzheimer’s disease?

Answer 21

B-amyloid

tau

Question 22

What is the macroscopic appearance of the brain in Alzheimer’s disease?

Answer 22

Cortical atrophy and ventricular enlargement

Widening of cerebral sulci

Question 23

What is the microscopic appearance of Alzheimer’s disease?

Answer 23

Neuritic plaques (extracellular)
Neurofibrillary tangles (intracellular)

Question 24

What proteins are deposited in frontotemporal lobar degeneration?

Answer 24

TDP43

Tau

Question 25

What is the difference between delirium and dementia?

Answer 25

Delirium is an acute disorder of attention and global cognition and is reversible Dementia is a progressive decline in at least 2 cognitive areas, irreversible

Question 26

What investigations should be done into dementia?

Answer 26

``` Exclude acute causes Detailed history and cognitive assessment Exclude metabolic causes Autoantibodies Brain imaging to exclude stroke Rarely biopsy ```

Question 27

What are the cardinal features of Parkinson's disease?

Answer 27

Bradykinesia Resting tremor Rigidity

Question 28

What protein is deposited in Parkinson's disease?

Answer 28

a-synuclein, which accumulates to form Lewy bodies within neurons in the substantia nigra

Question 29

What protein accumulates and where in motor neuron disease?

Answer 29

TDP43 in anterior horn cells