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Stage 3 > Neurology: Parkinsonism, Dementia, MND, MS > Flashcards

Neurology: Parkinsonism, Dementia, MND, MS Flashcards

1
Q

What is Parkinsonism?

A

Triad of:
1) Resting tremor
2) Hypertonia e.g. cogwheel rigidity
3) Bradykinesia e.g. slow, shuffling gait

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2
Q

What are the four main parkinson-plus syndromes?

A

1) Progressive supranuclear palsy
2) Multiple system atrophy
3) Cortico-basal degeneration
4) Lewy body dementia

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3
Q

What is a parkinson-plus syndrome?

A

Parkinsonism + additional clinical features

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4
Q

What are the clinical features of progressive supranuclear palsy (PSP)?

A

Parkinsonism + vertical gaze palsy

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5
Q

What are the clinical features of multiple system atrophy (MSA)?

A

Parkinsonism + early autonomic clinical features e.g. postural hypotension > 20/10, incontinence, impotence

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6
Q

What are the clinical features of cortico-basal degeneration?

A

Parkinsonism + spontaneous activity by an affecting limb OR akinetic rigidity of that limb

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7
Q

What are the clinical features of lewy body dementia?

A

Parkinsonism + fluctuations in cognitive impairment, visual hallucinations, often before Parkinsonian features occur

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8
Q

What is chorea?

A

Uncontrolled, dance-like movements
- May be unilateral depending on the cause, not typically sequential (i.e. from leg to arm), not associated with epilepsy

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9
Q

What is perseveration?

A

The repetition of gestures, words or phrases in the absence of a stimulus - typically associated with brain injuries

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10
Q

What is vascular dementia?

A

An umbrella term for a group of syndromes of cognitive impairment that are caused by cerebrovascular disease

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11
Q

What are types of vascular dementia?

A

1) Stroke related vascular disease
2) Subcortical vascular dementia
3) Mixed dementia

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12
Q

How does vascular dementia present?

A

1) Progressive stepwise deterioration in cognition e.g. memory/recognition which usually occurs over a period of several months to years
2) Distinct episodes of sudden, marked deterioration in memory - as mini-infarcts occur
3) Vascular risk factors/disease e.g. HTN, AF, history of stroke

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13
Q

How do you diagnose vascular dementia?

A

1) Comprehensive history and examination
2) Formal cognition screen
3) Medication review
4) Rule out reversible organic causes
5) Imaging - MRI is preferred

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14
Q

Which imaging modality is preferred in vascular dementia?

A

MRI - brain atrophy

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15
Q

How do you manage vascular dementia?

A

1) Treatment is symptomatic
2) Detect and address cardiovascular risk factors
3) Advanced care planning

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16
Q

How is the cognitive decline in Alzheimer’s dementia?

A

Gradual and insidious

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17
Q

What is pseudodementia?

A

1) When depression presents as cognitive impairment initially suggesting a neurodegenerative disorder
2) Once depression is treated the cognitive impairment is often reversible

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18
Q

What is important to remember about depression and dementia?

A

It is always important to consider and address mood in a patient presenting with memory loss, as depression rates are very high in patients experiencing true forms of dementia, and identifying and treating this appropriately can improve cognition and quality of life

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19
Q

How does fronto-temporal lobe dementia (FTLD) present?

A

Personality change or inappropriate social behaviour prior to any significant impact on memory (cognitive decline comes later in disease course) - due to area of brain affected

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20
Q

What is the difference in the type of patients affected by FTLD compared to other types of dementia?

A

FTLD tends to develop in younger patients < 65 years compared with other forms of dementia with increase in incidence with increasing age

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21
Q

How does delirium present?

A

Fluctuating cognition and inattention (cognitive impairment), and is usually reversible once the trigger has been addressed e.g. concurrent infection

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22
Q

What is the mean age onset for motor neurone disease in sporadic cases (90%)?

A

50-60 years (2:1 male predominance)

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23
Q

Which genes are associated with MND (10% cases are familial)?

A

SOD1, FUS, C9ORF72

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24
Q

What is motor neuron disease?

A

1) A spectrum of heredodegenerative diseases of the peripheral and central motor nerves
2) Can be classified clinically and pathological according to the distribution of motor neuron involvement

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25
Q

Which nerves are affected in MND?

A

Peripheral and central motor nerves

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26
Q

What is the most common variant of MND?

A

Amyotrophic lateral sclerosis (ALS)

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27
Q

How does ALS present/what is the classical MND presentation?

A

Mixed clinical picture of upper and lower motor neuron signs - affects both upper and lower motor neurons

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28
Q

What are the four clinical groups of MND?

A

1) Spinal ALS - the classic MND syndrome
2) Bulbar ALS
3) Progressive muscular atrophy
4) Primary lateral sclerosis

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29
Q

What features of MND carry the poorest prognosis?

A

1) Early bulbar and respiratory muscle involvement
2) Older age

30
Q

What features of MND are associated with more protracted survival?

A

More prominent LMN features

31
Q

What are upper motor neuron signs in MND?

A

Spasticity, hyperreflexia and upgoing plantars (though often down going in MND)

32
Q

What are lower motor neuron signs in MND?

A

Fasciculations and later atrophy

33
Q

Which muscles are spared in MND until late in the disease course?

A

Eye and sphincter muscles

34
Q

What is not seen in MND?

A

Sensory disturbance - should prompt consideration of an alternative diagnosis

35
Q

What is the key feature of bulbar ALS?

A

Early tongue and bulbar involvement

36
Q

What is the key feature of progressive muscular atrophy?

A

Only LMN features

37
Q

What is the key feature of primary lateral sclerosis?

A

Only UMN features

38
Q

What is the only disease modifying treatment available for MND which can prolong life by 3 months?

A

Riluzole - antiglutaminergic drug which dampens motor nerve firing

39
Q

What treatment can prolong survival in MND patients with type 2 respiratory failure?

A

Non-invasive ventilation (NIV)

40
Q

What are the important features of MND treatment?

A

1) Treatment focus is supportive and best coordinated via and MDT approach
2) Early discussion of advanced care planning should be initiated to minimise distress and complications as disease progresses

41
Q

What symptomatic treatments can be given for MND?

A

1) Pain relief - simple analgesia
2) Baclofen and botox injections - to treat spasticity and contractures
3) Anticholinergics incl. TCAs - to help drooling
4) Eventually supportive feeding via an NG or PEG may be indicated as bulbar disease progresses

42
Q

What is a disease-modifying drug used to treat multiple sclerosis?

A

Beta-interferon

43
Q

Which medication is used to treat myasthenia gravis?

A

Pyridostigmine - cholinesterase inhibitor

44
Q

What is used to treat Guillain-Barré syndrome?

A

IVIG

45
Q

What is the diagnostic test for myasthenia gravis?

A

Anti-acetylcholine receptor antibody

46
Q

How does myasthenia gravis present?

A

1) Diplopia + ptosis (pupils spared)
2) Weakness, which worsens throughout the day on repeated movements

46
Q

How does myasthenia gravis present?

A

1) Diplopia + ptosis (pupils spared)
2) Weakness, which worsens throughout the day on repeated movements

47
Q

What are important pathological features of multiple sclerosis?

A

1) CD4-mediated destruction of oligodendroglial cells
2) Humoral response to myelin binding protein

48
Q

What are the radiological and pathological hallmarks of MS?

A

Plaques of demyelination and eventual axonal loss

49
Q

What are the two types of MS?

A

1) Relapsing-remitting (80%) - may become secondarily progressive
2) Primary progressive (<10%)

50
Q

What is multiple sclerosis?

A

A multifocal condition which causes wildly variable clinical features dependent on the affected area of brain or spinal cord (not unlike cerebrovascular disease)

51
Q

What are typical presentations of multiple sclerosis?

A

1) Sensory disease - patchy paraesthesia
2) Optic neuritis - loss of central vision and painful eye movements
3) Internuclear ophthalmoplegia (blurred and double vision, limited movement of adducting eye and nystagmus in abducting eye) - lesion in the medial longitudinal fasciculus of the brainstem
4) Subacute cerebellar ataxia
5) Spastic paraparesis - transverse myelitis, including Lhermitte’s sign

52
Q

What is Lhermitte’s sign?

A

An electric shock-like sensation that occurs on flexion of the neck - this sensation radiates down the spine, often into the legs, arms, and sometimes to the trunk

53
Q

What is not seen in MS?

A

LMN signs - patterns of weakness or sensory loss that suggests a peripheral lesion are not consistent with MS as it is a disease of the CNS

54
Q

How do you diagnose MS?

A

At least two of:
1) Clinical history/examination
2) Imaging findings - MRI
3) Lumbar puncture

55
Q

What are imaging findings of MS on MRI?

A

Periventricular white matter lesions

56
Q

What CSF findings (lumbar puncture) are very sensitive for MS?

A

Oligoclonal bands - reflect various immunoglobulins seen on CSF electrophoresis and indicate the presence of an autoimmune process in the CNS

57
Q

In what other conditions can oligoclonal bands be found in CSF?

A

Other autoimmune and infectious conditions incl. Lyme disease, SLE and neurosarcoid

58
Q

Which imaging modality is used to diagnose MS?

A

MRI

59
Q

Which criteria are used to diagnosis MS?

A

McDonald criteria

60
Q

What is first line management for an acute attack of MS?

A

Glucocorticoids - 1g of IV or oral methylprednisolone every 24h for 3 days
(In practice, usually necessary to involve the local neuro team for guidance of when to start steroids for patients with an acute flare of MS)
Discuss with MS specialist before starting

61
Q

What must be excluded before offering first line management for MS and how?

A

Infections - routine bloods + urine dip

62
Q

What is second line management for severe acute attacks of MS which do not respond to glucocorticoids?

A

Plasma exchange

63
Q

What does acute treatment of MS achieve?

A

Reduces the duration and severity of individual attacks (does not appear to affect long term outcome)

64
Q

What are the two groups of drugs used in the long term management of relapsing remitting MS?

A

1) Disease modifying therapies (DMTs) - the extent to which long-term use of DMTs reduces risk of secondary progressive MS is not yet clearly established
2) Symptomatic therapies

65
Q

What is the first line disease modifying treatment for MS?

A

Injectables e.g. interferon and glatiramer

66
Q

What are other/second line treatments for MS?

A

1) New oral agents such as dimethyl fumarate, teriflunomide and fingolimod
2) Biologics such as natalizumab and alemtuzumab

67
Q

What is the problem with MS treatment?

A

As a general rule, increasingly effective treatments are associated with increasingly significant side effects

68
Q

What are symptomatic therapies used in MS?

A

1) Physiotherapy
2) Baclofen and botox - for spasticity
3) Modafinil and exercise therapy - for fatigue
4) Anticholinergics - for bladder dysfunction
5) SSRIs - for depression
6) Sildenafil - for erectile dysfunction
7) Clonazepam - for tremor

69
Q

When is admission to hospital required for acute MS?

A

Hospital admission is typically only required for very severe relapses or where oral steroids have not been effective

Stage 3 (61 decks)

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