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Flashcards in Neurology 1 Deck (131):
1

Pathophysiological background of MS?

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

2

What is the most common type of MS?

Relapsing-remitting

3

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

Secondary progressive

4

4 classifications of MS?

Relapsing/remitting
Secondary progressive
Primary progressive
Fulminant

5

Typical patient with MS?

Caucasian woman in her early adulthood

6

What effect does pregnancy have on MS?

Less likely to remit during pregnancy
More likely to remit just postpartum

7

Common visual presentation of MS?

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

8

What is Uhthoff's phenomenon?

MS - vision is worse in hot temps or after exercise

9

What is the most common eye sign of MS?

Symmetrical horizontal jerking nystagmus (due to lateral rectus weakness)

10

What is Lhermitte's sign?

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

11

What type of paraesthesia is classical of MS?

Perineal/genitalia

12

What type of motor deficits can occur in MS?

UMNL - spastic weakness
Transverse myelitis - loss of everything below level of lesion

13

What is Devic's syndrome?

Special MS - Neuromyelitis optica + Devic's syndrome

14

Common sexual/GU Sx of MS?

Perineal/genital paraesthesia
ED
Continence issues or retention

15

What is the diagnostic criteria of 'attacks' in MS?

Attacks last > 1hr, >30d between attacks

16

Is a bilateral optic neuritis suggestive of MS?

No not really

17

What criteria is used in clinical diagnosis of MS?

McDonalds criteria

18

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

MRI
CSF analysis
Evoked potentials - visual, auditory, somatosensory

19

What might be visible on CSF analysis in MS?

Oligoclonal IgG bands, high Ig index

20

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

NMO-IgG

21

What does NMO-IgG in CSF point towards?

Devic's syndrome

22

What is given in acute relapse management of MS?

Methylprednisolone

23

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

Reducing relapses

24

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

Glatiramer acetate
Dimethyl fumerate

25

Most commonly used Mab in MS?

Natalizumab

26

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

UMNL

27

What is the pathophysiology behind PD?

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

28

RFs for PD?

Elderly male
Pesticide exposure
Head trauma

29

PART of PD symptoms?

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

30

How does bradykinesia in PD manifest clinically?

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

31

How does rigidity manifest in PD?

Difficulty initiating movement
Resistance to passive movement - lead pipe, cogwheeling

32

What is characteristic of the tremor in PD?

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

33

Describe the gait in PD?

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

34

3 symptoms of PD that may precede diagnosis by years?

Anosmia
Constipation
Sleep disturbance

35

Non-motor features of PD?

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

36

What happens to the skin in PD?

Becomes greasy -> seborrhoeic dermatitis

37

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

Tremor dominant
Akinesia dominant
Mixed

38

Exclusion criteria for PD?

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

39

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

If it occurs after 1-2 years of diagnosis

40

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

CT/MRI
DAT/SPECT scans

41

What must be given alongside LevoDopa for PD and why?

A dopamine decarboxylase inhibitor DDCI
Because dopamine can't cross the BBB

42

2 levodopa + DDCI combos?

Sinemet - Cocareldopa - Ldopa + carbidopa
Madopar - Cobeneldopa - Ldopa + benserazide

43

Short term SEs of L Dopa treatment?

Nausea and vomiting, dizziness
Confusion
Hypotension

44

Long term SEs of L Dopa treatment?

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

45

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

Rapinorole
Cabergoline
For younger patients

46

What is apomorphine and what is it used for?

Powerful DA agonist

47

What are 2 COMT inhibitors?

Entacapone, tolcapone

48

MAOI inhibitor used in PD?

Rasagiline

49

What does benign essential tremor traditionally affect?

Hands and voice

50

Cerebellar vs PD tremor?

Cerebellar is intention tremor causing past pointing
PD is low frequency resting tremor

51

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

Affects legs
Normal PD rare in smokers

52

What is Sydenham's chorea?

Post strep pyogenes (GAS) rheumatic fever infection
Most common cause of acute chorea +/- psych Sx

53

What are the 2 Parkinson's plus syndromes?

Progressive supranuclear palsy PSNP
Multi-system atrophy

54

What facial signs are suggestive of PSNP?

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

55

What neck sign is suggestive of multi-system atrophy?

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

56

Symptoms of brown sequard/cord hemisection?

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

57

Symptoms of LMNL? What side are they?

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

58

UMNL symptoms? What side are they?

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

59

What side of the body are unilateral cerebellar lesions?

Ipsilateral

60

DANISH of cerebellar signs?

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

61

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

Sensory ataxia
Not a sign of cerebellar dysfunction

62

What is Kernig's sign?

Flex one leg at hip and knee
Then straighten leg -> resisted by spasm in hamstrings, other limb may flex

63

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

Because it's not positive in localised neck stiffness

64

Brudzinski neck sign?

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

65

3 classical signs of meningism?

Headache
Neck stiffness
Photophobia

66

What is subacute combined degeneration of the spinal cord?

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

What is Bells Palsy?

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

68

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

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

69

What is Ramsey Hunt syndrome?

CN7 invasion by HZV, can affect CN8

70

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

Away from the side of the lesion

71

What does a recurrent laryngeal nerve palsy cause?

Dysphonia and a bovine cough

72

What does dysphonia and a bovine cough suggest?

Recurrent laryngeal nerve damage

73

How do you assess for dysarthria and dysphonia?

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

74

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

Gag reflex

75

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

Myotonic dystrophy

76

What is myotonia?

Slow relaxation of muscles after contraction

77

5 things characteristic of myotonic dystrophy?

Myotonia
Bilateral sternocleidomastoid weakness and wastage
Cataracts
Heart defects
Endocrine abnormalities

78

What does a unilateral LMNL of CN12 do?

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

79

What causes a bulbar palsy?

LMNL to CNs 9, 10, 11, 12
Often caused by MND (progressive bulbar palsy)

80

What are the characteristic signs of bulbar palsy?

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

81

What causes a pseudobulbar palsy?

Bilateral UMNL to 9, 10, 11, 12 and often 5 and 7
Often in context of MND, CVA or occasionally MS

82

Symptoms of a pseudobulbar palsy?

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

83

What is the most common type of MND?

ALS Amyotrophic Lateral Sclerosis

84

What is progressive bulbar palsy?

A type of MND which affects CNs first

85

What is progressive muscular atrophy?

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

86

What is primary lateral sclerosis?

A type of MND causing a pure UMNL of leg muscles

87

MND type affecting CNs first?

Progressive bulbar palsy

88

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

Progressive muscular atrophy

89

Type of MND affecting leg muscles in UMNL fashion?

Primary lateral sclerosis

90

What is the type of motor neurone lesion in MND?

Mixed picture of UMNL and LMNL
However LMNL often predominates (think progressive bulbar palsy)

91

What is the most common presentation of ALS?

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

92

What is often the first signs of progressive bulbar palsy?

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

93

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

Fasciculations

94

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

Nope, unlike in radiculopathies

95

2 diagnostic pointers of MND?

Asymmetrical distal weakness
Brisk reflexes in a wasted limb

96

What are the Charcot Marie Tooth diseases?

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

97

What are 'inverted champagne bottle' legs typical of?

CMT disease

98

What is the pathogenesis behind myasthenia gravis?

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

99

When does MG normally present in each sex?

Younger women - 20s
Older men - 50s/60s

100

Tumour linked with MG?

Thymoma

101

What neurological disorder is associated with thymoma?

MG

102

Common presentation of MG?

Ocular muscle weakness (class I MG)

103

Worst presentation of MG?

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

104

What is the characteristic feature of MG?

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

105

Are there any abnormal neurological findings on exam in MG?

No - just fatiguability

106

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

Over a year

107

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

Myaesthenic crisis, major danger is respiratory inadequacy

108

What simple test is highly sensitive and specific for MG?

Ice cube test - putting ice on eyes improved ptosis

109

Two blood tests that are highly specific for MG?

AchR Abs
MnSK Abs

110

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

Achesterase inhibitors

111

Medical management of MG?

Immunosuppressants

112

What is Eaton-Lambert myaesthenic syndrome?

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

113

3 types of neurogenic syncope?

Vasovagal
Situational
Carotid sinus hypersensitivity

114

3 major causes of cardiac syncope?

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

115

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

Lateral tongue biting

116

3 major signs of seizure as opposed to syncope?

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

117

What is a simple partial seizure?

Seizure affecting one cerebral hemisphere, retaining full awareness

118

What is a complex partial seizure?

Loss of awareness but still only affecting one cerebral hemisphere

119

What is a secondary generalised seizure?

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

120

What are automatisms?

Coordinated, involuntary simple movements associated with seizure activity

121

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

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

122

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

Hippocampal sclerosis

123

3 major types of idiopathic generalised epilepsy?

Tonic-clinic
Absence
Myoclonic

124

In what age groups do absence seizures typically present?

Childhood

125

2 visual features highly suggestive of MS eye disease?

Blurring of vision
Desaturation

126

What sign on fundoscopy suggests optic neuritis?

Swollen discs

127

What is the relevance of oligoclonal bands in MS diagnosis?

Unmatched oligoclonal bands in CSF vs. serum is suggestive

128

What cells are responsible for myelin production in the CNS?

Oligodendrocytes

129

Why does spasticity happen in UMNLs?

Loss of inhibitory fibre activity from above

130

What is autonomic dysreflexia?

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

131

Common causes of autonomic dysreflexia?

Noxious stimulus - full bladder or bowel