Neurology Flashcards

1
Q

What gives you a hemiplegic gait?

A

UMN
Stroke, MS, tumour, SOL

See circumduction or drag

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2
Q

What give’s you a bilateral / diplegia gait

A

UMN
Bi-hemispheric = MS or cerebral palsy

Cord = compression, tumour, syringomyelia

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3
Q

What will give you a peripheral motor neuropathy foot drop?

A

High stepping gait

Anterior horn = Polio

Radicular = L5 weak dorsiflexion (can’t stand on heels), S1 weak plantar flexion (can’t stand on toes)

Sciatic or common peroneal = foot drop

Bilateral = GBS or Charcot Marie Tooth

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4
Q

Peripheral sensory neuropathy features and causes?

A

Broad based, stamping gait with sensory ataxia, rombergs positive

Causes = Diabetes, B12, drugs e.g. vincristine and phenytoin
GBS and CMT

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5
Q

Myopathy features and causes?

A

Waddling, difficulty in rising, Gower’s sign

Causes = muscular dystrophies, thyroid, Cushings and myositis

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6
Q

Motor part of GCS

A
6 = obey commands
5 = Localise to pain
4 = Withdraws to pain
3 = Abnormal flexion to pain
2 = extension to pain
1 = none
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7
Q

Verbal response GCS?

A
5 = orientated
4 = confused
3 = Words
2 = sounds
1 = none
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8
Q

Eyes GCS

A
4 = spontaneous
3 = speech
2 = pain
1 = none
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9
Q

Olfactory nerve palsy causes?

A
Bilateral = URTI, meningioma of olfactory groove
Unilateral = Head trauma, early meningioma
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10
Q

In bitemporal hemianopia what affects superior fields first?

A

Pituitary tumours / temporal one lesions = upper fields

Lower = Craniopharyngeal lesions / parietal lesions

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11
Q

What gives inferior or superior homonymous quadrantopias?

A

Parietal lesion = inferior
Temporal = superior

PITS

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12
Q

What will give you a macular sparing visual loss?

A

Occipital lobe lesion

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13
Q

How does a CN3 lesion present?

A

Down and out pupil as only lateral rectus and superior oblique left

Reduced response of elevator palpable superiors = ptosis

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14
Q

CN3 medical vs surgical?

A

Medical affects vaso vorum causing an ischaemic core = pupillary sparing as not affecting the outer parasympathetic fibres

Cause = Diabetes, MS

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15
Q

Classifying horners lesions?

Investigations?

A

1st order = central = MS / stroke / brainstem lesion
- Trunk, arms and face

2nd order = pre-ganglionic = pan coasts, apical TB, cervical rib, previous chest drain, thoracic/neck surgery
-Face
3rd order = Post-ganglionic = herpes zoster, carotid pathology
-Sweating unaffected

Investigations = CXR, MRA if brain and neck

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16
Q

What is INO, wheres the lesion and causes?

A

Lesion to the medial longitudinal fasciculus between midbrain and pons

Imapired adduction of ipsilateral, nystagmus on contralateral abduction

Causes = MS, vascular brainstem lesion, pontine glioma and encephalitis

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17
Q

Trochlear CN palsy?

A

Paralysis of SO

Diplopia maximal when looking down and in e.g. stars

Affected eye turns up and out when looking laterally

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18
Q

CN6 abducens palsy?

A

Innervates lateral rectus so eye cannot abduct = strabismus

Easily affected due to long course = Tumours, trauma and CVA e.g. Millard Gubler, Wernickes

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19
Q

Trigeminal palsy?

A

Lose sensation in ophthalmic, maxillary and mandibular regions.
Lose motor function of masseter and pterygoids

No jaw jerk

Corneal reflex = Afferent is CN5 ophthalmic branch, so if both eyes don’t close it is CN5

Causes: Midbrain lesions, trigeminal ganglion lesion e.g. acoustic neuroma. Lesion in cavernous sinus

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20
Q

Afferent and effort pathways of corneal reflex?

A

Afferent = CN5 so get bilateral loss of reflex

If only one side it is due to efferent pathway = facial nerve

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21
Q

Clinical features of Bell’s palsy?

A

Hyperacusis
Loss of motor supply to face
Cold sores if due to HSV

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22
Q

LMN vs UMN facial nerve lesion?

A

LMN affects whole face, UMN lesion is forehead sparing

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23
Q

Management of facial nerve palsy?

A

Eye protection, lubricant and tape eyes shut at night

High dose predinisolone

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24
Q

What is Ramsay hunt sydrome and management?

A

Reactivation of VZV in geniculate ganglion of CN8

PC = ear pain and neck stiffness
Vesicular rash in auditory canal
Ipsilateral facial weakness

Management = Aciclovir and steroids

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25
Q

Bilateral facial palsy causes?

A

Sarcoidosis, GBS and limes disease

Can also see bilateral acoustic neuromas in NFT2

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26
Q

Webers lateralises to the right side, this means?

A

Ipsilateral conductive hearing loss = right conductive

Or contralateral sensorineural

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27
Q

Taste to anterior 2/3rds of the tongue?

A

Facial

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28
Q

Taste to Posterior 2/3rds of tongue?

A

Glossopharyngeal

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29
Q

What do you see in a vagus nerve palsy?

A

Uvula deviates away from the lesion

Loss of gag reflex

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30
Q

Hypoglossal palsy?

A

Controls motor component of tongue
So will deviate towards the side of the lesion
May see wasting / fasciculations

Like facial tongue has bilateral UMN innervation so only lost of LMN

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31
Q

Resting tremor features and causes?

A

Increase with distraction, abolished on voluntary movement

Seen in Parkinson’s, treat with dopamine agonists

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32
Q

Action / postural tremor features and causes?

A

Absent at rest, worse on movement

Causes = BEAT

Benign essential tremor = alcohol improves
endocrine e.g. thyrotoxicosis
Alcohol withdrawal
Toxins e.g. B agonists

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33
Q

What is acute dystonia, causes and management?

A

Prolonged muscle contractor causing unusual joint posture / repetitive movements

Torticollis, trismus, oculogyric crisis

Often a drug reaction e.g. neuroleptics, L-DOPA

Management = procyclidine

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34
Q

Whats athetosis?

A

Slow sinuous writhing movements

Seen in cerebral palsy

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35
Q

Syncope causes?

A

CRASH

Cardiac = Stoke Adams attacks / CV syncope

Reflex = vagal overactivity e.g. vasovagal syncope, carotid sinuous hypersensitivity
OR
Sympathetic under activity e.g. postural hypotension

Arterial = Vertebrobasillar insufficiency

Systemic = hypoglycaemic

Head = epilepsy

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36
Q

Causes and features of cardiac syncope?

A

Bradycardia e.g. Heart block, long QT

Tachycardia e.g. SVT or VT

Structural e.g. LVF, tamponade

Before = palpitation, pain, SOB
During = Short LOC, pale and pulseless
After = quick recovery and flushed
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37
Q

Features of reflex syncope?

A
Before = slow onset, sweaty, clammy and tunnel vision 
During = pale, grey, bradycardia, may have clonic tonic jerks but NO TONGUE BITING
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38
Q

Investigations for syncope?

A
Postural BP, cardio and neuro exam
ECG ± 24 hour tape
U&E's, FBC
Echo
CT
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39
Q

Differential for vertigo?

A

IMBALANCE

Infection = labrynthitis

  • associated URTI
  • Acute and short lived
  • otorrhoea

Menieres

  • Episodic vertigo with roaring tinnitus
  • Lasts minutes to hours
  • sensation of pressure discomfort

BPPV
-Sudden, <30 seconds, head movements precipitate

Aminoglycosides and furosemide

Lymphatic fistula = tulles phenomenon where vertigo induced by nosie

Arterial = stroke, TIA

Nerves = Acoustic neuroma

Central = MS, tumour

E = epilepsy

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40
Q

Management of labrynthitis?

A

Vestibular suppressants = Promethazine
Prednisolone
If bacterial = Topical ofloxacin

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41
Q

management of menieres?

A

Low salt diet
Vestibular suppressant e.g. Promethazine, and corticosteroids
Menniet device TDS = delivers intermittent pulse pressures through ear

If ongoing can have surgery

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42
Q

BPPV management?

A

Educate and reassure not subside within 6 months

Epley manœuvre for treatment
If this fails Semont repositioning

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43
Q

What is a seizure?

A

Clinical manifestation of presumed / proven abnormal electrical activity in the brain

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44
Q

Different seizure presentations maintaining consciousness?

A

Myoclonus = irregular jerk caused buy involuntary muscle contraction

Aura = Simple partial seizure only lasting seconds

Simple partial motor = clonic (regular shaking), tonic (stiffness) or dystonic (spasm) lasting seconds

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45
Q

Different types of seizures losing consciousness?

A

Absences

Complex partial / focal awareness impaired

Tonic clonic / generalised

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46
Q

Features of an absence seizure?

management?

A

Last seconds, occurring multiple times in one day
3-10 years
Stimulated by hyperventilation
EEG = characteristic bilateral symmetrical 3Hz spike and wave pattern

90% seizure free by adulthood

Ethosuximide or sodium valproate

Second line = Lamotrigine

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47
Q

Complex partial features and management?

A

Impaired awareness / memory
Automatisms
Involve one side of the brain
Rapid recovery, no sleepiness

Carbamazepine or lamotrigine

2nd line = Levetiracetam or sodium valproate

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48
Q

Simple partial features and management?

A

Emotional disturbance and automatisms
Post-ictal phase

Carbamazepine or lamotrigine

2nd line = Levetiracetam or sodium valproate

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49
Q

Generalised seizure features and management?

A

No warning if generalised, aura if focal with secondary generalisation

Lateral tongue biting, incontinent, cyanosed
Can last 1-2 minutes
Post-ictal up to ten

Sodium valproate
Second line = Lamotrigine

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50
Q

Management of myoclonic seizures?

A

Sodium valproate

Second line = lamotrigine

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51
Q

Common side effects of anti-epileptics?

A

Lamotrigine = skin hypersensitivity

Valproate = teratogenic and weight gain

Carbamazepine = Skin hypersensitivity, vision, and SIADH

Ethosuximide = GI effects, insomnia and psychotic episodes

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52
Q

Driving regulations epilepsy?

A

First seizures reported to DVLA

Can drive once 12 month seizure free

If bus / lorry driver = ten years free

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53
Q

status epilepticus definition?

A

Continuous seizure lasting > 5 minutes, or repeated seizures lasting > 5 minutes with no regain of full consciousness in between

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54
Q

Status epileptics management?

A

ABC, 100% O2 and suction
IV access and bloods

  1. Reverse potential causes
  2. IV lorazepam 2-4mg / Rectal diazepam 10mg if no IV access. Second dose if no response after 10 minutes
  3. 2nd line = phenytoin IV 20mg/kg at a rate not exceeding 50mg/minute
    OR
    Phenobarbitol IV 10mg/kg at 100mg/minute
    Call anaesthetist
  4. RSI
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55
Q

What is West syndrome and its features?

A
<1 year
Salaam attacks = Flexion of head, trunk and limbs. then extension of arms
Last 1-2 seconds, repeated 50 times
Progressiv mental handicap
EEG = hypsarrhythmia
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56
Q

Lennox-Gastaut?

A
Onset 1-5 years
Atypical absences, jerks and falls
90% moderate to severe metal handicap
EEG = slow spike
Ketogenic diet may help
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57
Q

What is benign rolandic epilepsy?

A

Paraesthesia e.g. unilateral face on waking up

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58
Q

Juvenil myoclonic epilepsy features?

A
Onset in teens
Female
Infrequent generalised seizure, often in morning
Daytime absences
Sudden shock like myotonics

Responds well to sodium valproate

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59
Q

Migraine without aura criteria

A

At least 5 attacks, lasting 4-72 hours

Headache is 2 of:
Unilateral
Pulsating
Moderate to severe 
Aggravated by exercise

During the headache 1 of:
N&V, photophobia, phonophobia

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60
Q

Migraine with aura criteria?

A

Same as without but aura must fulfil…

1 of:

Fully reversible +ve / -ve symptoms
Dysphasic speech disturbance

2 of:
Homonymous visual or unilateral sensory symptoms
One aura symptom develops over at least 5 minutes
Each last 5-60 minutes

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61
Q

Chronic migraine criteria?

A

> 15 days a month, for at least 3 months +

Patient has had > 5 migraine attacks ± aura

On >8 days a month for 3 months fulfilling a migraine

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62
Q

Migraine management?

A

Acute reliever = Paracetamol / NSAIDs with an oral triptan
If under 17 = nasal triptan

Prophylaxis:
Avoid triggers
Topiramate or propranolol if > 2 attacks per month
2nd line = gabapentin

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63
Q

What is absolutely contraindicated in migraines?

A

COC due to increased risk of TIA

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64
Q

Cluster headache criteria?

A

5 attacks fulfilling below:

Severe unilateral orbital, supraorbital or temporal pain, lasting 15 minutes to 3 hours

Accompanied by one of:
Lacrimation
Rhinorrhoea
Facial oedema
Miosis/ptosis

Attacks happen at least every other day, up to 8/day

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65
Q

Management of cluster headaches?

A

Acutely 1005 O2
Sumitriptan 6mg subcut

Prophylaxis = Verapamil

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66
Q

What is trigeminal neuralgia, causes and management

A

Paroxysmal unilateral stabbing pain

Cause = Vessel compressing it commonly superior cerebellar, MS, varicella zoster

1st line = carbamazepine 100mg BD

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67
Q

GCA features and management?

A

Associated with PMR in 50%

ESR > 60

Temporal artery biopsy is gold standard

Management = Oral prednisone 60mg one of if visual symptoms, then refer to ophthalmology
If no visual = 40-60mg daily

Assess steroid response in 48 hours

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68
Q

Features of raised ICP?

A
Headache
Vomiting + seizures
Papilloedema
GCS reduced
Cushings reflex = Triad of increased BP, bradycardia and irregular breathing
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69
Q

Causes of raised ICP?

A
Vascular = haemorrhage, haematoma, AVM
Infection = Abscess, cyst, meningitis
Malignancy
TB granuloma
Hydrocephalus
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70
Q

Management of raised ICP?

A

ABC and 100% O2
Tilt bed to 45 degrees
Consider mannitol if head injury / bleed 0.25g/kg IV stat
IV dexamethasone if SOL 0.25mg/kg daily IV

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71
Q

IIH features and management?

A

As for ICP

1st line = weight loss
Acetazolamide 500mg BD

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72
Q

Kernigs and Brudzinskis sign?

A

Kernigs = Flex thigh, and straighten knee slowly. =pain

B sign = Patient supine and examiner flexes neck = involuntary knee and hip flexion

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73
Q

Lumbar puncture signs in bacterial meningitis?

A

Cloudy
Low glucose
high protein (0.5-3)
Raised WCC - neutrophils

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74
Q

LP in viral meningitis?

A

Clear / cloudy
raised leucocytes
Raised protein,
normal / high glucose

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75
Q

TB LP in meningitis?

A

Clear / cloudy. Fibrin web
Raised monocytes
Protein raised
Glucose normal

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76
Q

Empirical meningitis management?

A

Empirical = Cefotaxime 2g IV BD (+ ampicillin if <3 month, >50 or immuncomp)

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77
Q

Management for meningococcal meningitis?

A

IV BenPen 2.4g IV 4 hourly

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78
Q

Management for pneumococcal / haemophilia meningitis ?

A

IV cefotaxime

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79
Q

Management for listeria meningitis?

A

IV gentamicin and ampicillin

80
Q

Fungal meningitis management?

A

Cryptococcal and candididal = Amphotericin B 5mg/kg/day IV

Aspergillus = Voriconazole 6mg/kg IV BD

81
Q

What is encephalitis?

Causes?

A

Inflammation of the brain and meninges

Viruses e.g HSV!/2, CMV, EBV, West nile
Also any bacterial meningitis

82
Q

Clinical features of encephalitis and management?

A

Prodromal fever and rash
Altered mental state, meningism and Neuro signs
Seizures

Empirical = acyclovir 10mg/kg IV 8 hourly for three weeks

83
Q

Investigations for encephalitis?

A

Bloods = cultures, smears and FBC
Viral PCR
CT brain = late changes = hypodense regions
LP

84
Q

Stroke definition?

A

Rapidly developing clinical signs of focal disturbance of cerebral function, > 24 hours.

85
Q

Stroke causes?

A

80% = ischaemia e.g. atheroma, embolism

20% = haemorrhage e.g. HTN, aneurysm, anticoagulation

86
Q

Oxford classifications of strokes 4 types?

A

Total anterior
-highest mortality
Large infarct of carotid/MCA or ACA
-All 3 of higher dysfunction, contralateral sensory / motor deficit and contralateral homonymous hemianopia

Partial anterior
-same as above but only 2/3

Posterior circulation

  • Vertebrobasillar territory
  • Any of cerebellar syndrome, brainstem syndrome, LOC and contralateral homonymous hemianopia

Lacunar strokes

  • Small infarcts around basal ganglia, internal capsule
  • Pure motor
  • Pure sensory
  • Mixed
  • Ataxic hemiparesis
87
Q

What vessel causes dysphasia?

A

MCA

88
Q

What is Wernickes and where is affected?

A

Receptive dysphasia = speech fluent but cannot understand
Sentences make no sense
Good repetition

Superior temporal gyrus

89
Q

What is brooks?

A

Expressive dysphasia = understand things, just cannot express fluently
Speech is laboured, non-fluent and halting

Inferior frontal gyrus

90
Q

Conduction dysphasia?

A

Associative dysphasia, fluent but with abnormal comprehension + abnormal repetition

Arcuate nucleus = connection between Broca’s and wernickes

91
Q

What will ACA stroke show?

A

Contralateral motor / sensory. legs > arms. Face spared

92
Q

MCA stroke?

A

Contralateral motor / sensory. Face and arms > leg

93
Q

PCA stroke?

A

Contralateral homonymous hemianopia, macula sparing

94
Q

Webers stroke?

A

Branches of the posterior artery that supply midbrain

Ipsilateral CN3 and contralateral hemiparesis

95
Q

Wallenberg stroke?

A

Posterior inferior cerebellar artery

Ipsilateral facial pain and temperature loss
Contralateral limb/torso pain and temperature loss

A-HAND:
Ataxia
Horners
Anaesthesia (as above)
Nystagmus and vertigo
Dysphagia
96
Q

Lateral pontine syndrome?

A

Anterior inferior cerebellar artery.

Similar to Wallenberg but get ipsilateral facial paralysis and deafness

97
Q

Millard Gubler Syndrome

A

Lesion in the pons
CN6/7 and corticospinal tracts

Diplopia
LMN lesion facial loss and loss of corneal reflex
Contralateral hemiplegia

98
Q

Locked in syndrome

A

Ventral pons infarct = basilar artery

Patient is aware, but completely paralysed except for vertical gaze and upper eyelid movement
Due to sparing of the mid-brain tectum

99
Q

Cerebelopontine angle syndrome causes and features

A

Causes = Acoustic neuromas, meningioma, metastasis

Ipsilateral CN5/6/7/8 + cerebellar signs

100
Q

Subclavian steal syndrome

A

Subclavian artery stenosis proximal to origin of vertebral artery = blood is stolen from here due to retrograde blood flow

Syncope and neurology on use of arm

101
Q

Stroke investigations?

A

Bloods = exclude hypoglycaemia, U&E’s, cardiac enzymes + clotting

ECG

CT head

102
Q

Management of acute ischaemic stroke?

A

No venous sinus thrombosis (VST) within 4.5 hours:
-Alteplase 0.9mg/kg IV 10% dose as bolus, rest 90% over 1 hour
Max 90mg
300mg aspirin for 2 weeks

If no VST, >4.5 hours = 2 weeks aspiring 300mg OD. no rTPA

If VST = Heparin full dose, then warfarin until INR 2-3

103
Q

Management of acute haemorrhage stroke?

A

ITU, airway

BP control = labetalol 10mg IV over 2 minutes

Correct any coagulopathy:
Warfarin = Stop, phytomenadione and FFP + PT complex
Heparin = stop and protamine sulphate
Dagibatran = Idarucizumab (Praxbind)

If deteriorating = External ventricular drainage of CSF / haematoma removal surgically

104
Q

Long term management of stroke?

A

Clopidogrel lifelong
RF control = BP, statins
Physio, salt, OT and Neuro-rehabilitation

If ischaemic = lifelong anticoagulation

105
Q

What is a TIA?

A

Stroke symptoms which resolve with 24 hours

106
Q

TIA management?

A

Clopidogrel 75mg immediato and lifelong
RF control
Assess stroke risk with ABCD2

TIA clinic referral

Cant drive for 1 month, or three if multiple over a short period of time

107
Q

What is a SAH and its causes?

A

Bleeding into the SA space between the arachnoid membrane and Pia mater

Cause = Rupture of berry aneurysms (80%) and AVMs

108
Q

Where do berry aneurysms occur?

A

Branch points

Junction of posterior communicating artery with internal carotid

Junction of ACA and anterior cerebral artery

109
Q

Clinical features of SAH?

A

Sudden onset headache
Collapse

LP = bloodstained CSF

CT = Well defined round hyper attenuated lesion

110
Q

SAH management?

A

Unruptured <7mm can be observed

Surgical:

Clipping = more suitable if large parenchymal haematomas
- craniotomy and clip

Endovascular coil via femoral:
- Better suited for old / frail

Medical:
Nimodipine 60mg four hourly for three weeks = vasospasm prophylaxis

Stool softeners

111
Q

What is a subdural haemorrhage?

Features?

A

Haematoma between the dura and the arachnoid
Caused by damage to the riding veins between cortex and sinus

Old alcoholic, lots of falls
Headache and slowly progressive mental/physical decline

112
Q

Subdural investigations and management?

A

CT = crescenteric, midline shift

Management:
<10mm or midline shift <5mm:

Prophylactic anti epileptics e.g. phenytoin
Correct any coagulopathy
ICP management = tilt, ventilate and mannitol

> 10mm or >5mm midline shift or significant neuro:
Same as above
Burr hole craniotomy and irrigate clot with saline and suction

113
Q

What is an extradural?

Clinical features?

A

Collection of blood between inner layer of the skull and outer layer of the dura

Middle meningeal artery bleed, with parietal/temporal fracture

Lucid interval, can be hours/days
Raised ICP symptoms
Brainstem compression = Cushings triad

114
Q

Extradural investigations and management?

A

CT = lens shaped haematoma + skull fracture

Neuroprotective ventialtion, mannitol and craniotomy for clot evacuation and vessel ligation

115
Q

Two types of intracranial venous thrombosis?

Causes?

A

Dural venous, Cortical Vein

Pregnancy, OCP, head injury, dehydration and thrombophilia

116
Q

Types of Dural venous sinus thrombosis type and PC?

A

Sagittal = 45% of IVT. Headache, vomiting, seizures and visual

Transverse = 35% of IVT
Headache ± mastoid pain, focal neuro signs, seizures

Cavernous sinus
Spread from facial pustules / folliculitis
Often involves CN3-6

117
Q

Investigations and management of intracranial venous thrombosis?

A

Exclude SAH and meningitis with CT/MRI contrast = convex bowing of lateral walls and increased dural enhancement
LP = increased opening pressure

LMWH then warfarin INR 2-3
Thrombolysis
For cavernous = Vancomycin and ceftriaxone

118
Q

Delirium definition?

A

Acute fluctuating confusional state, with clouding of consciousness affecting the sleep wake cycle

119
Q

Clinical features and management of neuroleptic malignant syndrome?

A
Young male, within 10 days of starting anti-psychotics
Pyrexia
Rigidity
Tachycardia
Rasied CK

Stop anti-psychotic
Fluids
Bromocriptine or Dantrolene

120
Q

What is dementia?

A

Progressive mental decline interfering with ADLs, insidious in onset with clear consciousness

121
Q

Pathology of Alzheimers?

A
Altered APP metabolism = amyloid B deposition causing:
Extracellular plaques
NFT's
Cerebral amyloid angiopathy
Cerebral atrophy
122
Q

Classification of Alzheimers severity?

A

MMSE:

mild = 21-26
moderate = 10-20
Moderately severe = 10-14
Severe = <10

123
Q

Alzheimers management?

A

Structured cognitive stimulation

Mild to moderate = AChE inhibitors e.g. Donepezil, Galantamine and rivastigmine

Severe = Memantine

124
Q

Clinical features and management of vascular dementia?

A

Step wise progression, with vascular risk factors

Manage the predisposing RF’s

125
Q

Lewy body dementia pathology, PC and management?

A

Lewy bodies in occipital-parietal cortex

PC = fluctuating cognition, visual hallucinations and Parkinsonism

Management = AChE inhibitors

126
Q

Fronto-temporal dementia pathology, PC and scan findings?

A

Fronto-temporal atrophy, marked gloss and neuronal loss, balloon neutrons + tau+ve pick bodues

PC = disinhibition, personality change, early memory preservation

Scan = fronto-temporal atrophy

127
Q

Parkinsons pathology?

A

Loss of dopaminergic neutrons that originate in substantial migration and project to the striatum = Lewy body inclusion, +ve for alpha-synuclein
NFT’s = hyper-phosphorylated tau

128
Q

Parkinsons features?

A

Tremor, rigidity and akinesia / bradykinesia

Minimal facies
Postural instability
50% get dementia
90% = sleeping disorders

129
Q

Management of Parkinson’s?

A

1st line = L-DOPA, most effective for motor signs.
Can give with decarboxylase inhibitor e.g. Sinemet / Madopar

2nd line = Pramipexol, Rotigotine

MAO-B inhibitors = Rasagiline

COMT inhibitors = entacapone

130
Q

SE’s of L-DOPA?

A

On-off motor fluctuations
End of dose deterioration
Permanently induce dyskinesia

131
Q

What class is pramipexol / rotigotine and SE’s?

A

Dopamine agonist

Impulse behaviour
Oedema
Fibrosis

132
Q

How do MAO inhibitors and COMT inhibitors work?

A

Both inhibit the breakdown of dopamine

133
Q

What do you use to treat L-DOPA induced dyskinesia?

A

Stop drug

Amantidine
SE’s = ataxia, slurred speech and lived reticularis

134
Q

What three conditions make ups MSA?

A

Olivopontocerebellar atrophy
Shy-drager syndrome
Striatonigral degeneration

Generally now classed as MSA-P or MSA-C dependant on the predominant cerebella or Parkinsonism symptoms

135
Q

Clinical features of MSA?

A

Autonomic dysfunction = postural hypotension and bladder dysfunction

Cerebellar signs
Rigidity > tremor

136
Q

PC of corticobasal degeneration?

A

Parkinsonism
Speech disturbances
Alien limb

Tau +ve

137
Q

PSP clinical features?

A

Supranuclear gaze and postural instability = can’t look down

Rocket sign = when asked to get out of a chair they fly up

138
Q

What is multiple sclerosis?

A

Chronic inflammatory demyelinating disease of the CNS = CD4 mediated destruction of oligodendrocytes

139
Q

MS classification?

A
Relapsing remitting = 80%
Secondary progressive (following R-R)
Primary progressive = 20%
Progressive relapsing
140
Q

MS relapses vs progression

A

Relapses = acute onset with complete / partial recovery. Last > 24 hours

Progression = Insidious, relentless and irreversible . For at least 1 year

141
Q

Clinical features of MS?

A

Most common at onset = optic neuritis, motor weakness and sensory disturbances

UMN ONLY

Optic neuritis = pain on eye movement and reduced vision. Uhthoffs = vision reduced with heat

Spinal cord lesion = spastic paraparesis, Lhermittes sign where electric shock triggered by neck flexion, urinary incontinence

Brainstem lesion = INO, vertigo, nystagmus

Cerebellar = ataxic

142
Q

MS investigations?

A

Lesions must be separated in time and space
4 typical MRI lesions = SIPS

Spinal cord
Infratentorial
Subcortical
Periventricular

CSF = Increased production of IgG oligoclonal bands

143
Q

Management of MS?

A

Acute attack = 1g prednisolone for 3 days

Preventing relapses in R-R: IFN-B 30ug once weekly OR Glatiramer 20mg subcut. OD
2nd line = Alemtuzumab 12mg for 3 days, then head later 12mg for 5 days

Secondary progressive = Methylprednisolone 1g IV monthly

Primary progressive MS = Ocrelizumab 300mg initial dose

144
Q

Management of specific MS problems?

A

Spasticity = baclofen

Bladder dysfunction = may require regular self catheterisation and anti-cholinesterase

145
Q

Clinical features of cord compression?

A

Spinal pain
Numbness / paraesthesia below level
Weakness
Bladder and bowel dysfunction

146
Q

Management of cord compression?

A

Trauma = immobilise, surgery and methylpred.

Malignancy = Methylpred. ± surgery ± radiation

Abscess = Vancomycin + metronidazole + cefotaxime

147
Q

Cauda equina signs and management?

A

Saddle anaesthesia, back pain and poor anal tone

bilateral flaccid and areflexic lower limbs

Incontinence / retention

Mx = Laminectomy

148
Q

What is spondylosis?

A

Degenerative change of intervertebral discs

149
Q

Cervical spondylosis symptoms?

A

C5 = Deltoid, supraspinatus and reduced supinator jerk
+ numb elbow

C6 = Biceps, brachioradialis, reduced biceps reflex
+ Numb index

C7 = Triceps, finger extension and triceps reflex
+ numb middle finger

C8 = Finger flexors and intrinsic hand muscles
+ numb ring and little

150
Q

Lumbosacral spondylosis symptoms?

A

L4 = Quad wasting / weakness. Impaired knee jerk. Sensory impairment over medial calf.
+ve femoral stretch test

L5 = Wasting and weakness of dorsiflexors - some degree of foot drop. Sensory over dorsum of foot.
INTACT REFLEXES
+ve sciatic nerve test

S1 = Impaired ankle jerk. Sensory impairment over lateral aspect of foot.
+ve sciatic nerve test.

151
Q

Spondylosis investigations and management?

A

MRI is definitive investigation

Management = bed rest and NSAIDs
Surgery indications = Abnormal neurology, recurrent pain or severe pain.

152
Q

Cervical myelopathy causes, clinical features and management

A

Causes = spondylosis, trauma and congenital

PC = Pain affecting neck and limbs, loss of motor function is reduced dexterity, loss of outonomic function = incontinence / retention.

MRI = gold standard

Management = Urgent surgical referral for spinal decompression

153
Q

PC of musculocutaneous lesion?

A

Weak flexion at elbow, absent biceps reflex.

Numbness over lateral forearm

154
Q

PC of axillary nerve damage?

A

Weak shoulder abduction and external rotation. Sensory loss over regimental patch

155
Q

PC of radial nerve damage?

A

Wrist drop as cannot extend wrist, weak elbow extension.
Cannot abduct thumb
Absent triceps and supinator reflex.
Sensory loss over dorsal aspect of dorsal aspect of thumb and lateral two fingers

156
Q

PC of median nerve lesion?

A

Cannot flex wrist, weak flexion of fingers BUT can still flex at DIPJ of ring finger and little finger. All PIPs gone as lose FDS.
Lose sensation over palmar aspect of thumb and medial two fingers.

157
Q

PC of ulnar nerve lesion?

A

Can’t abduct or adduct fingers = paper test.
Weak wrist flexion, weak flexion of ring and little fingers.
Numbness over hypothenar eminence and lateral 1.5 fingers.

158
Q

Causes of polyneuropathies?

A
Metabolic = Diabetes, B12
Vasculitis
Inflammatory = GBS, sarcoidosis
Inherited = CMT
Infection = HIV, syphilis
Drugs = Isoniazid, vincristine, phenytoin
159
Q

Investigations for polyneuropathy?

A

B12 levels.
MRI may show you subacute combined degeneration

Diabetes = HbA1c

Alcohol = AST:ALT ratio, GGT

160
Q

What is GBS?

Causes?

A

An acute inflammatory post-infectious neuropathy, antibodies to gangliosides

Viral e.g. SMV, EBV (HHV4),
Bacterial = Campylobacter

161
Q

Clinical features of GBS?

A

Prodromal = malaise, vomtiing, headache, limb pain

Followed by ascending paralysis = reduced reflexes, weakness, respiratory distress

162
Q

Types of GBS?

A

Acute inflammatory demyelinating polyradiculopathy = most frequent in western world

Acute motor axonal neuropathy = purely motor

Acute motor and sensory = very rare

Miller-Fisher Syndrome = Ophthalmoplegia, ataxia and areflexia.
Descending paralysis
Anti-GQ1b antibodies

163
Q

GBS management?

A

Plasma exchange or IVIG 400mg/kg/day for 5 days
- Plasma exchange if IgA deficiency or renal failure

85% full recovery

164
Q

What is CMT and the two types?

A

Hereditary sensory and motor neuropathy. Autosomal dominant

CMT1 = commonest, autosomal dominant, demyelination

CMT2 = second commonest, autosomal dominant, axonal degeneration

165
Q

Clinical features of CMT?

A

FHx is key = pes cavus, neuropathy or abnormal gait

Walking difficult with foot drop / high stepping gait

Pes cavus

Reduced reflexes, sensation,

166
Q

Management of CMT?

A

Physiotherapy with low impact exercise and stretching

Bracing

Orthopaedic surgery to correct foot deformities

167
Q

Types of MND?

A

UMN AND LMN

Amyotrophic lateral sclerosis

Primary lateral sclerosis

Progressive muscular atrophy

Bulbar or pseudo bulbar palsy

168
Q

Features of ALS?

A

Most common MND
Lesion to corticospinal tracts

UMN in legs, LMN in arms

progressive and unrelenting with 3-5 year survival

169
Q

features of PLS

A

Isolated UMN with progressive weakness and spasticity

Slower disease progression vs ALS

170
Q

Features of progressive muscular atrophy

A

Isolated LMN = lesions of anterior horn cell
Asymmetrical limb wasting and weakness
Affects distal then proximal

Median survival = 4.6 years = BEST PROGNOSIS

171
Q

Features of pseudobulbar palsy

A

Degeneration of corticobulbar tracts to CN5/7/10/11/12

Difficulty in mastication, facial expressions, weak swallowing and weak tongue 
Labile emotions
Brisk jaw jerk
UMN signs
SPARES 3/4/5 = EYES
172
Q

Bulbar palsy

A

Impairment of CN9/10/12

Get dysarthria, dysphagia and poor swallow
Nasal speech
LMN signs

173
Q

MND management?

A

Riluzole 50mg PO BD =
Prevents stimulation of glutamate receptors

Can prolong life by 3 months

174
Q

Myopathy vs neuropathy?

A

Myopathy = muscle dysfunction. Proximal weakness with no sensory loss.

175
Q

Different causes of myopathies?

A

Muscular myopathies e.g. Duchennes, Beckers, Facioscapulohumeral, myotonic

Metabolic e.g. thyroid, graves

Drug induced e.g. alcohol, statins and steroids

176
Q

clinical features and management of Duchennes

A

X-linked, non-functional dystrophin

PC = delayed walking, falls, waddling gait and Gower’s.
Calf pseudohypertrophy

Management = prednisollone, creatine and supportive

177
Q

Clinical features and management of Becker’s?

A

X-linked, partially functioning dystrophin.

Same as Duchennes

178
Q

features of fascioscapulohumeral dystrophy?

A

Weakness in second or third decade.
Mainly facial, periscapular and humeral.

Characteristic unlined facies, pouting lips and transverse smile.

foetal myoglobin and sarcolemma are increased

179
Q

Features of myotonic dystrophy?

A

PC = Cataracts, hypogonadism, frontal balding and cardiac disorders

Wasting of sternocleidomastoids = Swan necked appearance.

180
Q

What is myasthenia gravis?

A

Chronic autoimmune disorder of post-synaptic membrane at the NMJ in skeletal muscle

Antibodies vs. the Ach receptor

181
Q

Myasthenia associations in men vs women?

A

Men = thyme atrophy / tumour

Women = autoimmune disease e.g. RA, DM

182
Q

Clinical feature of Myasthenia?

A

Muscle fatigueablilty = bilateral ptosis, diplopia. Myasthenia snarl.
Proximal limb weakness

Normal reflexes

183
Q

What can precipitate Myasthenia?

A

BB’s, lithium, phenytoin, antibiotics e.g. gentamicin

184
Q

Investigations and Management of Myasthenia?

A

Single fibre EMG, serum AchR antibodies, muscle specific tyrosine kinase antibodies

Mx:
Cholinetserase inhibitors = Pyridostigmine
Prednisolone
Consdier thymectomy
refractory = IVIG
185
Q

What is LEMs?

Causes?

A

Auto-immune disorder of the NMJ with antibodies vs. the voltage gated calcium channel = no presynaptic fusion and therefore no excretion of Ach

Cause = paraneoplastic pulse with small cell carcinoma or autoimmune.

186
Q

Clinical features of LEMs and management?

A

Leg weakness before eyes.
Autonomic symptoms e.g. dry mouth

movement IMPROVES symptoms

Mx:
Treat underlying cause
Amifampridine
Refractory = IVIG

187
Q

Clinical features of NFT1?

A

CAFE-NOIR

Cafe au lait spots >6 15mm in diameter

Axillary freckling

Fibromas = subcutaneous or plexiform (nerve trunk)

Eye = Lisch nodules

Neoplasia e.g. CNS meningioma, phaeo, CML/AML, GI tumours

Orthopaedic = scoliosis

IQ reduced

Renal = RAS = HTN

188
Q

Clinical features NFT2?

A

Bilateral acoustic neuromas = SNHL, vertigo

189
Q

NFT management?

A

MDT
Genetic counselling
Surgery for any underlying malignancies

190
Q

What is tuberous sclerosis?

A

Autosomal dominant condition causing tumours throughout he body.

191
Q

Clinical features of tuberous sclerosis?

Cutaneous
Neurological
Other

A

Cutaneous:
Depigmented ash leaf spots. Roughened shagreen patches over lumbar region
Adenoma sebaceum
Subungal fibratoma

Neuro:
Developmental delay
Epilepsy
Intellectual impairment

Other features = retinal hamartomas (NFT HAS IRIS HAMARTOMAS)
Gliomatous changes in brain lesions

192
Q

What is syringomyelia?

A

Slowly progressive syndrome, I which cavitation (called a syrinx) occurs in central segments of the spinal cord, often cervical

193
Q

Causes of syringomyelia?

A

Spina bifida

Arnold-Chiari malformation in which cerebellum extends through foramen magnum into first part of cervical spine.

Secondary to cord trauma / tumours

194
Q

Clinical features and management of syringomyelia?

A

Sensory loss due to spinothalamic damage = temperature and pain, often in arms and shoulders

Anterior horn cell damage = LMN signs = wasting and weakness

Management = surgery to promote free flow of CSF

195
Q

What is Friedrich’s ataxia?

A

Hereditary progressive ataxia, due to degeneration of spinocerebellar and corticospinal tracts + dorsal root ganglion and cerebellar cells

196
Q

Clinical features of Friedrich’s ataxia?

A
Neurological:
Ataxia
Absent reflexes
Dysarthria
Weakness and wasting (relatively late sign)
Non-neuro:
Pes cavus
Scoliosis
Cardiac = HOCM
DM