Neurology 1 Flashcards

1
Q

Pathophysiological background of MS?

A

Autoimmune condition causing episodes of inflammation, demyelination and subsequent sclerosis along CNS nerves (impairing motor and or sensory function), disseminated over space and time

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2
Q

What is the most common type of MS?

A

Relapsing-remitting

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3
Q

What form of MS eventually follows on from relapsing-remitting?

A

Secondary progressive

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4
Q

4 classifications of MS?

A

Relapsing/remitting
Secondary progressive
Primary progressive
Fulminant

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5
Q

Typical patient with MS?

A

Caucasian woman in her early adulthood

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6
Q

What effect does pregnancy have on MS?

A

Less likely to remit during pregnancy

More likely to remit just postpartum

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7
Q

Common visual presentation of MS?

A

Optic neuritis - often unilateral painful movement of the eye, diplopia, decline in central vision

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8
Q

What is Uhthoff’s phenomenon?

A

MS - vision is worse in hot temps or after exercise

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9
Q

What is the most common eye sign of MS?

A

Symmetrical horizontal jerking nystagmus (due to lateral rectus weakness)

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10
Q

What is Lhermitte’s sign?

A

MS - neck flexion in a patient with cervical spinal cord lesions causes ‘electric shocks’ in trunk and limbs

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11
Q

What type of paraesthesia is classical of MS?

A

Perineal/genitalia

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12
Q

What type of motor deficits can occur in MS?

A

UMNL - spastic weakness

Transverse myelitis - loss of everything below level of lesion

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13
Q

What is Devic’s syndrome?

A

Special MS - Neuromyelitis optica + Devic’s syndrome

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14
Q

Common sexual/GU Sx of MS?

A

Perineal/genital paraesthesia
ED
Continence issues or retention

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15
Q

What is the diagnostic criteria of ‘attacks’ in MS?

A

Attacks last > 1hr, >30d between attacks

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16
Q

Is a bilateral optic neuritis suggestive of MS?

A

No not really

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17
Q

What criteria is used in clinical diagnosis of MS?

A

McDonalds criteria

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18
Q

3 investigations that can be used to support a diagnosis of MS?

A

MRI
CSF analysis
Evoked potentials - visual, auditory, somatosensory

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19
Q

What might be visible on CSF analysis in MS?

A

Oligoclonal IgG bands, high Ig index

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20
Q

What CSF finding is highly specific for Devic’s syndrome?

A

NMO-IgG

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21
Q

What does NMO-IgG in CSF point towards?

A

Devic’s syndrome

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22
Q

What is given in acute relapse management of MS?

A

Methylprednisolone

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23
Q

What is the role of interferon use in R/R MS?

A

Reducing relapses

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24
Q

2 alternatives to interferon to reduce relapses in R/R MS?

A

Glatiramer acetate

Dimethyl fumerate

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25
Q

Most commonly used Mab in MS?

A

Natalizumab

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26
Q

What type of motor neurone lesion is MS? (UMNL vs LMNL)

A

UMNL

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27
Q

What is the pathophysiology behind PD?

A

Loss of dopamine at the ventral tier substantia nigra pars compacta leading to degeneration of the nigrostriatal pathway
Lewy body deposition

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28
Q

RFs for PD?

A

Elderly male
Pesticide exposure
Head trauma

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29
Q

PART of PD symptoms?

A

Postural instability
Akinesia/bradykinesia, hypokinesia
Rigidity - lead pipe
Tremor at rest

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30
Q

How does bradykinesia in PD manifest clinically?

A

Slow gait, reduced arm swing
Reduced amplitude of repeated action -> micrographia
Reduced eye blinking, mask like facial expression

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31
Q

How does rigidity manifest in PD?

A

Difficulty initiating movement

Resistance to passive movement - lead pipe, cogwheeling

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32
Q

What is characteristic of the tremor in PD?

A

Starts unilateral, often becomes bilateral and generalised
Slow (4-6Hz) tremor at rest, may be obliterated with concentration
Worse in stress and often absent at night

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33
Q

Describe the gait in PD?

A

Festinant, shuffling - difficulty initiating movement, difficulty in tight spaces
Unsteady with reduced arm swing

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34
Q

3 symptoms of PD that may precede diagnosis by years?

A

Anosmia
Constipation
Sleep disturbance

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35
Q

Non-motor features of PD?

A
Voice - quiet, slurred, monotone
Micrographia
Incontinence
Greasy skin
Drooling and neuromuscular dysphagia
Sleep disturbance 
Pain
Cognitive deficit (inability to multitask) and dementia
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36
Q

What happens to the skin in PD?

A

Becomes greasy -> seborrhoeic dermatitis

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37
Q

What are the 3 types of PD? (In terms of dominance)

A

Tremor dominant
Akinesia dominant
Mixed

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38
Q

Exclusion criteria for PD?

A

Background causes e.g. Stroke, head injury, neuroleptics
SN gaze palsy
Cerebellar signs, Babinski

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39
Q

When is PD dementia more likely than Alzheimer’s dementia in a known PD patient?

A

If it occurs after 1-2 years of diagnosis

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40
Q

Although PD is a clinical diagnosis, what imaging scans may help to support a diagnosis?

A

CT/MRI

DAT/SPECT scans

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41
Q

What must be given alongside LevoDopa for PD and why?

A

A dopamine decarboxylase inhibitor DDCI

Because dopamine can’t cross the BBB

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42
Q

2 levodopa + DDCI combos?

A

Sinemet - Cocareldopa - Ldopa + carbidopa

Madopar - Cobeneldopa - Ldopa + benserazide

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43
Q

Short term SEs of L Dopa treatment?

A

Nausea and vomiting, dizziness
Confusion
Hypotension

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44
Q

Long term SEs of L Dopa treatment?

A

‘Wearing off’
End of dose/peak dose dyskinesia
On and off fluctuation
DA dysregulation syndrome

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45
Q

What are non-ergot DA agonists? Who are they used for?

A

Rapinorole
Cabergoline
For younger patients

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46
Q

What is apomorphine and what is it used for?

A

Powerful DA agonist

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47
Q

What are 2 COMT inhibitors?

A

Entacapone, tolcapone

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48
Q

MAOI inhibitor used in PD?

A

Rasagiline

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49
Q

What does benign essential tremor traditionally affect?

A

Hands and voice

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50
Q

Cerebellar vs PD tremor?

A

Cerebellar is intention tremor causing past pointing

PD is low frequency resting tremor

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51
Q

What limbs does vascular PD typically affect? Why is it interesting in terms of RFs vs normal PD?

A

Affects legs

Normal PD rare in smokers

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52
Q

What is Sydenham’s chorea?

A

Post strep pyogenes (GAS) rheumatic fever infection

Most common cause of acute chorea +/- psych Sx

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53
Q

What are the 2 Parkinson’s plus syndromes?

A

Progressive supranuclear palsy PSNP

Multi-system atrophy

54
Q

What facial signs are suggestive of PSNP?

A

Mega brow lines due to frontalis hyperactivity, as patient can’t voluntarily look up or down so the muscle overcompensated

55
Q

What neck sign is suggestive of multi-system atrophy?

A

Can’t look down voluntarily -> cord involvement, incontinence, autonomic failure

56
Q

Symptoms of brown sequard/cord hemisection?

A

Contralateral pain and temp loss below lesion
Ipsilateral touch vibration proprioception loss below lesion
Ipsilateral spastic paralysis below lesion

57
Q

Symptoms of LMNL? What side are they?

A
Flaccid paraplegia/paralysis
Fasciculations
Muscle wastage
Hypotonia
Hyporeflexia
On ipsilateral side of lesion
58
Q

UMNL symptoms? What side are they?

A
Pyramidal (spastic) weakness/paralysis
Hyperreflexia 
Hypertonia
Positive Babinski
Loss of abdo reflexes 
Clasp knife responses, Hoffmans
Contralateral signs
59
Q

What side of the body are unilateral cerebellar lesions?

A

Ipsilateral

60
Q

DANISH of cerebellar signs?

A
Dysdiadochokinesia and dysmetria
Ataxic gait
Nystagmus
Intention tremor
Speech - dysarthria, scanning
Hypotonia, hyposensitivity
61
Q

What does Rombergs sign indicate? Is it a cerebellar sign?

A

Sensory ataxia

Not a sign of cerebellar dysfunction

62
Q

What is Kernig’s sign?

A

Flex one leg at hip and knee

Then straighten leg -> resisted by spasm in hamstrings, other limb may flex

63
Q

Why is Kernig’s sign so useful in detecting meningeal irritation?

A

Because it’s not positive in localised neck stiffness

64
Q

Brudzinski neck sign?

A

Flexion of neck leads to flexion of hips, indicating meningeal irritation

65
Q

3 classical signs of meningism?

A

Headache
Neck stiffness
Photophobia

66
Q

What is subacute combined degeneration of the spinal cord?

A

B12 deficiency - both UMNL cortiospinal/post column and peripheral nerve degeneration leading to peripheral vibration, proprioception and possibly pain and temp (glove and stocking) loss as well as +ve Babinski and spasticity

67
Q

What is Bells Palsy?

A

A unilateral LMNL of CN7 causing upper and lower facial muscle weakness on the affected side, often preceded by pain

68
Q

What is the characteristic look of a unilateral UMNL of the facial nerve?

A

There is upper facial muscle sparing because there is bilateral cortical representation of these nerves
Lower facial muscles are weak

69
Q

What is Ramsey Hunt syndrome?

A

CN7 invasion by HZV, can affect CN8

70
Q

What side does the uvula deviate to in a CN10 LMNL?

A

Away from the side of the lesion

71
Q

What does a recurrent laryngeal nerve palsy cause?

A

Dysphonia and a bovine cough

72
Q

What does dysphonia and a bovine cough suggest?

A

Recurrent laryngeal nerve damage

73
Q

How do you assess for dysarthria and dysphonia?

A

Listen to speech
Repeat yellow lorry (lingual)
Say baby hippopotamus (labial)
Tongue twister (she sells sea shells…)

74
Q

What is the test for CN9 sensation that isn’t routinely performed in CN exam?

A

Gag reflex

75
Q

What is bilateral weakness and wastage of the sternocleidomastoids characteristic of?

A

Myotonic dystrophy

76
Q

What is myotonia?

A

Slow relaxation of muscles after contraction

77
Q

5 things characteristic of myotonic dystrophy?

A
Myotonia
Bilateral sternocleidomastoid weakness and wastage
Cataracts
Heart defects
Endocrine abnormalities
78
Q

What does a unilateral LMNL of CN12 do?

A

Makes the tongue lick the wound - weakness on side of and deviation towards the side of the lesion

79
Q

What causes a bulbar palsy?

A

LMNL to CNs 9, 10, 11, 12

Often caused by MND (progressive bulbar palsy)

80
Q

What are the characteristic signs of bulbar palsy?

A

Dysarthria NO dysphonia
Dysphagia
Tongue weakness, wastage, fanciful action
No jaw jerk or emotional lability

81
Q

What causes a pseudobulbar palsy?

A

Bilateral UMNL to 9, 10, 11, 12 and often 5 and 7

Often in context of MND, CVA or occasionally MS

82
Q

Symptoms of a pseudobulbar palsy?

A
Dysarthria and dysphonia
Dysphagia
Spastic and conical tongue
\+ve jaw jerk, emotional lability
And CN7 signs of UMNL
83
Q

What is the most common type of MND?

A

ALS Amyotrophic Lateral Sclerosis

84
Q

What is progressive bulbar palsy?

A

A type of MND which affects CNs first

85
Q

What is progressive muscular atrophy?

A

A type of MND that is pure LMNL, affecting the small muscles in hands and feet first

86
Q

What is primary lateral sclerosis?

A

A type of MND causing a pure UMNL of leg muscles

87
Q

MND type affecting CNs first?

A

Progressive bulbar palsy

88
Q

MND type providing pure LMNL of small hand/feet muscles?

A

Progressive muscular atrophy

89
Q

Type of MND affecting leg muscles in UMNL fashion?

A

Primary lateral sclerosis

90
Q

What is the type of motor neurone lesion in MND?

A

Mixed picture of UMNL and LMNL

However LMNL often predominates (think progressive bulbar palsy)

91
Q

What is the most common presentation of ALS?

A

Focal onset affecting one muscle group in upper limb (unless primary lateral sclerosis)

92
Q

What is often the first signs of progressive bulbar palsy?

A

Dysarthria and tongue signs
Dysphagia is late
Emotional lability may be there

93
Q

What sign (of LMNL) is often prominent in MND?

A

Fasciculations

94
Q

Is there any pain or sensory disturbance in MND? What does this differentiate from?

A

Nope, unlike in radiculopathies

95
Q

2 diagnostic pointers of MND?

A

Asymmetrical distal weakness

Brisk reflexes in a wasted limb

96
Q

What are the Charcot Marie Tooth diseases?

A

A group of inherited peripheral neuropathies (LMNLs) that typically cause distal limb muscle wasting and sensory disturbance, progressing proximally over time

97
Q

What are ‘inverted champagne bottle’ legs typical of?

A

CMT disease

98
Q

What is the pathogenesis behind myasthenia gravis?

A

Autoimmune disease causing destruction of the post-synaptic Ach receptors at the nmj

99
Q

When does MG normally present in each sex?

A

Younger women - 20s

Older men - 50s/60s

100
Q

Tumour linked with MG?

A

Thymoma

101
Q

What neurological disorder is associated with thymoma?

A

MG

102
Q

Common presentation of MG?

A

Ocular muscle weakness (class I MG)

103
Q

Worst presentation of MG?

A

Severe weakness in multiple groups (class V/generalised MG)

104
Q

What is the characteristic feature of MG?

A

Easy muscle fatiguability - worsened by exercise e.g. Progressive weakness whilst counting up to 30, ocular muscle weakness and ptosis

105
Q

Are there any abnormal neurological findings on exam in MG?

A

No - just fatiguability

106
Q

Over what sort of time period have most MG patients progressed from grade I -> V?

A

Over a year

107
Q

What is an acute MG attack called and what is the major danger?

A

Myaesthenic crisis, major danger is respiratory inadequacy

108
Q

What simple test is highly sensitive and specific for MG?

A

Ice cube test - putting ice on eyes improved ptosis

109
Q

Two blood tests that are highly specific for MG?

A

AchR Abs

MnSK Abs

110
Q

What medication can be given to transiently improve Sx in MG and may be used in investigation?

A

Achesterase inhibitors

111
Q

Medical management of MG?

A

Immunosuppressants

112
Q

What is Eaton-Lambert myaesthenic syndrome?

A

A paraneoplastic syndrome associated with SCLC, causing proximal muscle fatiguability eased by exercise

113
Q

3 types of neurogenic syncope?

A

Vasovagal
Situational
Carotid sinus hypersensitivity

114
Q

3 major causes of cardiac syncope?

A

LVOT obstruction e.g. Aortic stenosis
Arrhythmias - 3rd degree heart block, VT
Cardiomyopathy (e.g. Hypertrophic obstructive)

115
Q

What type of tongue biting specifically is more indicative of seizure?

A

Lateral tongue biting

116
Q

3 major signs of seizure as opposed to syncope?

A

Not regaining full consciousness/awareness until being in ambulances etc.
Lateral tongue biting
Lip cyanosis (respiratory apnoea)

117
Q

What is a simple partial seizure?

A

Seizure affecting one cerebral hemisphere, retaining full awareness

118
Q

What is a complex partial seizure?

A

Loss of awareness but still only affecting one cerebral hemisphere

119
Q

What is a secondary generalised seizure?

A

A generalised seizure that has began in one cerebral hemisphere before spreading across the corpus callosum

120
Q

What are automatisms?

A

Coordinated, involuntary simple movements associated with seizure activity

121
Q

What features are highly specific of a medial temporal lobe complex partial seizure?

A

Epigastric rising feeling
Odd smells, emotions, déjà vu, automatisms
Loss of awareness

122
Q

What process which may be related to childhood febrile seizures may predispose to medial temporal lobe epilepsy?

A

Hippocampal sclerosis

123
Q

3 major types of idiopathic generalised epilepsy?

A

Tonic-clinic
Absence
Myoclonic

124
Q

In what age groups do absence seizures typically present?

A

Childhood

125
Q

2 visual features highly suggestive of MS eye disease?

A

Blurring of vision

Desaturation

126
Q

What sign on fundoscopy suggests optic neuritis?

A

Swollen discs

127
Q

What is the relevance of oligoclonal bands in MS diagnosis?

A

Unmatched oligoclonal bands in CSF vs. serum is suggestive

128
Q

What cells are responsible for myelin production in the CNS?

A

Oligodendrocytes

129
Q

Why does spasticity happen in UMNLs?

A

Loss of inhibitory fibre activity from above

130
Q

What is autonomic dysreflexia?

A

Spinal cord injury T6 or above eventually causing pale blotchy cold below lesion and hypertension, headache, bradycardia, flushing above

131
Q

Common causes of autonomic dysreflexia?

A

Noxious stimulus - full bladder or bowel