Y4 Degenerative Diseases

Question 1

dementia - what two structures differences is there found on biopsy?

Answer 1

neurofibrillary tangles and β amyloid plaques

Question 2

what are three risk factors of dementia?

Answer 2

ApoE4 allele, presenillin 1/2 mutations, Down’s

Question 3

what score does the MMSE have to be to prescribe cholinesterase inhibitors in alkheimers dementia ?

Answer 3

10-20

Question 4

what is the usual presenting complaint of lewy body dementia ?

Answer 4

Fluctuating cognitive dysfunction, visual

hallucinations, parkinsonism

Question 5

what is found on biopsy of frontotemporal dementia?

Answer 5

Pick Bodies

Question 6

what affect does picks bodies have on the brain?

Answer 6

cause cells to die

Question 7

what are reversible causes on dementia ?

Answer 7

infection 
Vascular
􏰀 Chronic subdural haematoma
Inflammation
􏰀 SLE
􏰀 Sarcoid
Neoplasia
Nutritional
􏰀 Thiamine deficiency
􏰀 B12 and folate deficiency
􏰀 Pellagra (B3 / niacin deficiency)
Hypothyroid
Hypoadrenalism
Hypercalcaemia
Hydrocephalus (normal pressure)

Question 8

state 4 parkinsonism conditions ?

Answer 8

Multiple Systems Atrophy
Progressive Supra nuclear Palsy
CorticobasilarDegeneration
Lewy body dementia

Question 9

what destruction causes parkinsons ?

Answer 9

Destruction of dopaminergic neurones in pars compacta

of substantia nigra.

Question 10

what would be found on biopsy of parkinsons causing the pathophysiology?

Answer 10

β-amyloid plaques

􏰀 Neurofibrillary tangles: hyperphosphorlated tau

Question 11

state some reversible causes of parkinsons?

Answer 11

Infection
􏰀 Syphilis, 􏰀 HIV,􏰀 CJD
Vascular: Multiple infarcts in SN 
Drugs: Antipsychotics, metoclopramide Trauma: dementia pugilistica
Genetic: Wilson’s disease

Question 12

what are the four cardinal signs of parkinsons ?

Answer 12

rigidity
gait
bradykinesia
tremor

Question 13

state the characterstics of parkinsons tremor ?

Answer 13

Worse at rest
􏰀 Exacerbated by distraction
􏰀 4-6hz, pill-rolling

Question 14

state the characteristics of the bradykinesia of parksinons?

Answer 14

Slow initiation of movement ̄c reduction of amplitude on repetition
􏰀 Expressionless face
􏰀 Monotonous voice
􏰀 Micrografia

Question 15

what are the signs of multiple systems atrophy?

Answer 15

Autonomic dysfunction: post hypotension, bladder dysfunction
􏰀Cerebellar + pyramidal signs
􏰀Rigidity > Tremor

Question 16

what are the signs of progressive supra nuclear palsy?

Answer 16

Postural instability → falls
􏰀 Speech disturbance (+ dementia)
􏰀 Palsy: vertical gaze

Question 17

what are two signs of lewy body dementia ?

Answer 17

Fluctuating cognition

􏰀 Visual hallucinations

Question 18

what are the signs of Corticobasilar Degeneration?

Answer 18

Aphasia, dysarthria, apraxia
􏰀 Akinetic rigidity in one limb
􏰀 Astereognosis (cortical sensory loss)
􏰀 Alien limb phenomenon

Question 19

what is 􏰀 Astereognosis?

Answer 19

inability to identify an object by active touch of the hands without other sensory input

Question 20

what is dementia pugilistica?

Answer 20

A variant of chronic traumatic encephalopathy (CTE), which is itself a serious type of brain damage resulting from repeated concussions and is found in many professional athletes and military personnel who have been subjected to multiple impacts to the head.

Question 21

state some features of parkinsons ?

Answer 21

Tremor
􏰀 Rigidity
􏰀 Akinesia
􏰀 Postural instability: stooped gait  ̄c festination
􏰀 Postural hypotension
􏰀 Sleep disorders: insomnia, EDS, OSA, RBD
􏰀 Psychosis: esp. visual hallucinations
􏰀 Depression / Dementia

Question 22

sleep disorders what does EDS, OSA and RBD stand for?

Answer 22

EDS - excessive daytime sleepiness
OSA - obstructive sleep apnoea
RBD - rapid eye movement behaviour disorder

Question 23

what could cause Early morning dystonia in parkinsons?

Answer 23

medication is wearing off

Question 24

state some side effects of LDOPA? DOPAMINE

Answer 24

Dyskinesia
􏰀 On-Off phenomena = Motor fluctuations
􏰀 Psychosis
􏰀 ABP↓
􏰀 Mouth dryness
􏰀 Insomnia
􏰀 N/V
􏰀 EDS

Question 25

what is the on off effect in parkinsons ?

Answer 25

unpredictable fluctuations in motor | performance unrelated to timing of dose.

Question 26

state some autonomic dysfunction with parkinsons ?

Answer 26

``` Postural hypotension 􏰀 Constipation 􏰀 Hypersalivation → dribbling (↓ ability to swallow saliva) 􏰀 Urgency, frequency, Nocturia 􏰀 ED 􏰀 Hyperhidrosis ```

Question 27

what scale is used to measure disability in parkinsons ?

Answer 27

UPDRS: Unified Parkinson’s Disease Rating Scale

Question 28

name a MOAB inhibitor?

Answer 28

rasagiline, selegiline

Question 29

name a LDOPA?

Answer 29

co-careldopa or co-beneldopa

Question 30

name a dopamine agonist ?

Answer 30

ropinirole, pramipexole

Question 31

after how long do people loss response to LDOPA?

Answer 31

2-5years

Question 32

what gene is associated with MS?

Answer 32

HLA-DRB1

Question 33

what are the four common symptoms someone with MS presents with?

Answer 33

Tingling (paraesthesia) eyes (optic neuritis) ataxia (loss of control of voluntary movements) motor (spastic paraparesis)

Question 34

what is Lhermitte’s Sign?

Answer 34

when someone flexes their neck and gets shooting pain down arms/legs/trunk

Question 35

what is Uhthoff’s sign in optic neuritis ?

Answer 35

when vision is worse with heat (hot meal, exercise)

Question 36

what is INO?

Answer 36

Disruption of MLF connecting CN6 to CN3 Weak adduction of ipsilateral eye Nystagmus of contralateral eye

Question 37

when is INO seen?

Answer 37

lesion between CN6 and CN3 for example during MS when there is demyelination

Question 38

what type of MRI is used for MS?

Answer 38

T2

Question 39

what is found on LP for MS?

Answer 39

IgG oligoclonal bands (not present in serum)

Question 40

what antibodies can be found with MS?

Answer 40

anti MBP abs | NMO IgG abs

Question 41

what disease is NMO IgE abs specific for ?

Answer 41

Devics disease

Question 42

what criteria is used for T2 MS MRIs?

Answer 42

macdonald criteria

Question 43

what is another name for Devics?

Answer 43

neuromyelitis optica

Question 44

what are two features of neuromyelitis optica?

Answer 44

transverse myelitis | optic atrophy

Question 45

what is the treatment for an acute attack of MS?

Answer 45

steroids

Question 46

what medication is available for treatment of MS?

Answer 46

steroids DMARDS Biologics

Question 47

what medication is used to prevent relapses of MS?

Answer 47

DMARDS - IFN-β: 􏰀- Glatiramer: Biologics Natalizumab: anti-VLA-4 Ab 􏰀 Alemtuzumab: anti-CD52

Question 48

what symptoms can be treated during MS?

Answer 48

``` fatigue bladder incontinence depression pain tremor erectile dysfunction ```

Question 49

on examination what can be found for optic neuritis ?

Answer 49

↓ acuity, ↓ colour vision, white disc, central scotoma, | RAPD (relative afferent pupil defect)