Y4 Degenerative Diseases Flashcards

1
Q

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

A

neurofibrillary tangles and β amyloid plaques

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2
Q

what are three risk factors of dementia?

A

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

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3
Q

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

A

10-20

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4
Q

what is the usual presenting complaint of lewy body dementia ?

A

Fluctuating cognitive dysfunction, visual

hallucinations, parkinsonism

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5
Q

what is found on biopsy of frontotemporal dementia?

A

Pick Bodies

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6
Q

what affect does picks bodies have on the brain?

A

cause cells to die

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7
Q

what are reversible causes on dementia ?

A
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)
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8
Q

state 4 parkinsonism conditions ?

A

Multiple Systems Atrophy
Progressive Supra nuclear Palsy
CorticobasilarDegeneration
Lewy body dementia

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9
Q

what destruction causes parkinsons ?

A

Destruction of dopaminergic neurones in pars compacta

of substantia nigra.

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10
Q

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

A

β-amyloid plaques

􏰀 Neurofibrillary tangles: hyperphosphorlated tau

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11
Q

state some reversible causes of parkinsons?

A
Infection
􏰀 Syphilis, 􏰀 HIV,􏰀 CJD
Vascular: Multiple infarcts in SN 
Drugs: Antipsychotics, metoclopramide Trauma: dementia pugilistica
Genetic: Wilson’s disease
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12
Q

what are the four cardinal signs of parkinsons ?

A

rigidity
gait
bradykinesia
tremor

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13
Q

state the characterstics of parkinsons tremor ?

A

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

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14
Q

state the characteristics of the bradykinesia of parksinons?

A

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

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15
Q

what are the signs of multiple systems atrophy?

A

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

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16
Q

what are the signs of progressive supra nuclear palsy?

A

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

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17
Q

what are two signs of lewy body dementia ?

A

Fluctuating cognition

􏰀 Visual hallucinations

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18
Q

what are the signs of Corticobasilar Degeneration?

A

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

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19
Q

what is 􏰀 Astereognosis?

A

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

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20
Q

what is dementia pugilistica?

A

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.

21
Q

state some features of parkinsons ?

A
Tremor
􏰀 Rigidity
􏰀 Akinesia
􏰀 Postural instability: stooped gait  ̄c festination
􏰀 Postural hypotension
􏰀 Sleep disorders: insomnia, EDS, OSA, RBD
􏰀 Psychosis: esp. visual hallucinations
􏰀 Depression / Dementia
22
Q

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

A

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

23
Q

what could cause Early morning dystonia in parkinsons?

A

medication is wearing off

24
Q

state some side effects of LDOPA? DOPAMINE

A
Dyskinesia
􏰀 On-Off phenomena = Motor fluctuations
􏰀 Psychosis
􏰀 ABP↓
􏰀 Mouth dryness
􏰀 Insomnia
􏰀 N/V
􏰀 EDS
25
Q

what is the on off effect in parkinsons ?

A

unpredictable fluctuations in motor

performance unrelated to timing of dose.

26
Q

state some autonomic dysfunction with parkinsons ?

A
Postural hypotension
􏰀 Constipation
􏰀 Hypersalivation → dribbling (↓ ability to swallow saliva)
􏰀 Urgency, frequency, Nocturia
􏰀 ED
􏰀 Hyperhidrosis
27
Q

what scale is used to measure disability in parkinsons ?

A

UPDRS: Unified Parkinson’s Disease Rating Scale

28
Q

name a MOAB inhibitor?

A

rasagiline, selegiline

29
Q

name a LDOPA?

A

co-careldopa or co-beneldopa

30
Q

name a dopamine agonist ?

A

ropinirole, pramipexole

31
Q

after how long do people loss response to LDOPA?

A

2-5years

32
Q

what gene is associated with MS?

A

HLA-DRB1

33
Q

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

A

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

34
Q

what is Lhermitte’s Sign?

A

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

35
Q

what is Uhthoff’s sign in optic neuritis ?

A

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

36
Q

what is INO?

A

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

37
Q

when is INO seen?

A

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

38
Q

what type of MRI is used for MS?

A

T2

39
Q

what is found on LP for MS?

A

IgG oligoclonal bands (not present in serum)

40
Q

what antibodies can be found with MS?

A

anti MBP abs

NMO IgG abs

41
Q

what disease is NMO IgE abs specific for ?

A

Devics disease

42
Q

what criteria is used for T2 MS MRIs?

A

macdonald criteria

43
Q

what is another name for Devics?

A

neuromyelitis optica

44
Q

what are two features of neuromyelitis optica?

A

transverse myelitis

optic atrophy

45
Q

what is the treatment for an acute attack of MS?

A

steroids

46
Q

what medication is available for treatment of MS?

A

steroids
DMARDS
Biologics

47
Q

what medication is used to prevent relapses of MS?

A

DMARDS
- IFN-β:
􏰀- Glatiramer:

Biologics
Natalizumab: anti-VLA-4 Ab

􏰀 Alemtuzumab: anti-CD52

48
Q

what symptoms can be treated during MS?

A
fatigue 
bladder incontinence 
depression 
pain 
tremor 
erectile dysfunction
49
Q

on examination what can be found for optic neuritis ?

A

↓ acuity, ↓ colour vision, white disc, central scotoma,

RAPD (relative afferent pupil defect)