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Flashcards in Neurology Deck (196)
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1

What gives you a hemiplegic gait?

UMN
Stroke, MS, tumour, SOL

See circumduction or drag

2

What give's you a bilateral / diplegia gait

UMN
Bi-hemispheric = MS or cerebral palsy

Cord = compression, tumour, syringomyelia

3

What will give you a peripheral motor neuropathy foot drop?

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

4

Peripheral sensory neuropathy features and causes?

Broad based, stamping gait with sensory ataxia, rombergs positive

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


5

Myopathy features and causes?

Waddling, difficulty in rising, Gower's sign

Causes = muscular dystrophies, thyroid, Cushings and myositis

6

Motor part of GCS

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

7

Verbal response GCS?

5 = orientated
4 = confused
3 = Words
2 = sounds
1 = none

8

Eyes GCS

4 = spontaneous
3 = speech
2 = pain
1 = none

9

Olfactory nerve palsy causes?

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

10

In bitemporal hemianopia what affects superior fields first?

Pituitary tumours / temporal one lesions = upper fields

Lower = Craniopharyngeal lesions / parietal lesions

11

What gives inferior or superior homonymous quadrantopias?

Parietal lesion = inferior
Temporal = superior

PITS

12

What will give you a macular sparing visual loss?

Occipital lobe lesion

13

How does a CN3 lesion present?

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

Reduced response of elevator palpable superiors = ptosis

14

CN3 medical vs surgical?

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

Cause = Diabetes, MS

15

Classifying horners lesions?

Investigations?

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

16

What is INO, wheres the lesion and causes?

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

17

Trochlear CN palsy?

Paralysis of SO

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

Affected eye turns up and out when looking laterally

18

CN6 abducens palsy?

Innervates lateral rectus so eye cannot abduct = strabismus

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

19

Trigeminal palsy?

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

20

Afferent and effort pathways of corneal reflex?

Afferent = CN5 so get bilateral loss of reflex

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

21

Clinical features of Bell's palsy?

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

22

LMN vs UMN facial nerve lesion?

LMN affects whole face, UMN lesion is forehead sparing

23

Management of facial nerve palsy?

Eye protection, lubricant and tape eyes shut at night

High dose predinisolone

24

What is Ramsay hunt sydrome and management?

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

25

Bilateral facial palsy causes?

Sarcoidosis, GBS and limes disease

Can also see bilateral acoustic neuromas in NFT2

26

Webers lateralises to the right side, this means?

Ipsilateral conductive hearing loss = right conductive

Or contralateral sensorineural

27

Taste to anterior 2/3rds of the tongue?

Facial

28

Taste to Posterior 2/3rds of tongue?

Glossopharyngeal

29

What do you see in a vagus nerve palsy?

Uvula deviates away from the lesion
Loss of gag reflex

30

Hypoglossal palsy?

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

31

Resting tremor features and causes?

Increase with distraction, abolished on voluntary movement

Seen in Parkinson's, treat with dopamine agonists

32

Action / postural tremor features and causes?

Absent at rest, worse on movement

Causes = BEAT

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

33

What is acute dystonia, causes and management?

Prolonged muscle contractor causing unusual joint posture / repetitive movements

Torticollis, trismus, oculogyric crisis

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

Management = procyclidine

34

Whats athetosis?

Slow sinuous writhing movements

Seen in cerebral palsy

35

Syncope causes?

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

36

Causes and features of cardiac syncope?

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

37

Features of reflex syncope?

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

38

Investigations for syncope?

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

39

Differential for vertigo?

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

40

Management of labrynthitis?

Vestibular suppressants = Promethazine
Prednisolone
If bacterial = Topical ofloxacin

41

management of menieres?

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

If ongoing can have surgery

42

BPPV management?

Educate and reassure not subside within 6 months

Epley manœuvre for treatment
If this fails Semont repositioning

43

What is a seizure?

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

44

Different seizure presentations maintaining consciousness?

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

45

Different types of seizures losing consciousness?

Absences

Complex partial / focal awareness impaired



Tonic clonic / generalised

46

Features of an absence seizure?

management?

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

47

Complex partial features and management?

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

Carbamazepine or lamotrigine

2nd line = Levetiracetam or sodium valproate

48

Simple partial features and management?

Emotional disturbance and automatisms
Post-ictal phase

Carbamazepine or lamotrigine

2nd line = Levetiracetam or sodium valproate

49

Generalised seizure features and management?

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

50

Management of myoclonic seizures?

Sodium valproate
Second line = lamotrigine

51

Common side effects of anti-epileptics?

Lamotrigine = skin hypersensitivity

Valproate = teratogenic and weight gain

Carbamazepine = Skin hypersensitivity, vision, and SIADH

Ethosuximide = GI effects, insomnia and psychotic episodes

52

Driving regulations epilepsy?

First seizures reported to DVLA

Can drive once 12 month seizure free

If bus / lorry driver = ten years free

53

status epilepticus definition?

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

54

Status epileptics management?

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

55

What is West syndrome and its features?

<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

56

Lennox-Gastaut?

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

57

What is benign rolandic epilepsy?

Paraesthesia e.g. unilateral face on waking up

58

Juvenil myoclonic epilepsy features?

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

Responds well to sodium valproate

59

Migraine without aura criteria

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

60

Migraine with aura criteria?

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

61

Chronic migraine criteria?

>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

62

Migraine management?

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

63

What is absolutely contraindicated in migraines?

COC due to increased risk of TIA

64

Cluster headache criteria?

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

65

Management of cluster headaches?

Acutely 1005 O2
Sumitriptan 6mg subcut

Prophylaxis = Verapamil

66

What is trigeminal neuralgia, causes and management

Paroxysmal unilateral stabbing pain

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

1st line = carbamazepine 100mg BD

67

GCA features and management?

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

68

Features of raised ICP?

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

69

Causes of raised ICP?

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

70

Management of raised ICP?

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

71

IIH features and management?

As for ICP

1st line = weight loss
Acetazolamide 500mg BD

72

Kernigs and Brudzinskis sign?

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

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

73

Lumbar puncture signs in bacterial meningitis?

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

74

LP in viral meningitis?

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

75

TB LP in meningitis?

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

76

Empirical meningitis management?

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

77

Management for meningococcal meningitis?

IV BenPen 2.4g IV 4 hourly

78

Management for pneumococcal / haemophilia meningitis ?

IV cefotaxime

79

Management for listeria meningitis?

IV gentamicin and ampicillin

80

Fungal meningitis management?

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

Aspergillus = Voriconazole 6mg/kg IV BD

81

What is encephalitis?

Causes?

Inflammation of the brain and meninges

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

82

Clinical features of encephalitis and management?

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

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

83

Investigations for encephalitis?

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

84

Stroke definition?

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

85

Stroke causes?

80% = ischaemia e.g. atheroma, embolism

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

86

Oxford classifications of strokes 4 types?

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

What vessel causes dysphasia?

MCA

88

What is Wernickes and where is affected?

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

Superior temporal gyrus

89

What is brooks?

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

Inferior frontal gyrus

90

Conduction dysphasia?

Associative dysphasia, fluent but with abnormal comprehension + abnormal repetition

Arcuate nucleus = connection between Broca's and wernickes

91

What will ACA stroke show?

Contralateral motor / sensory. legs > arms. Face spared

92

MCA stroke?

Contralateral motor / sensory. Face and arms > leg

93

PCA stroke?

Contralateral homonymous hemianopia, macula sparing

94

Webers stroke?

Branches of the posterior artery that supply midbrain

Ipsilateral CN3 and contralateral hemiparesis

95

Wallenberg stroke?

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

Lateral pontine syndrome?

Anterior inferior cerebellar artery.

Similar to Wallenberg but get ipsilateral facial paralysis and deafness

97

Millard Gubler Syndrome

Lesion in the pons
CN6/7 and corticospinal tracts

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

98

Locked in syndrome

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

Cerebelopontine angle syndrome causes and features

Causes = Acoustic neuromas, meningioma, metastasis

Ipsilateral CN5/6/7/8 + cerebellar signs

100

Subclavian steal syndrome

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

Stroke investigations?

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

ECG

CT head

102

Management of acute ischaemic stroke?

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

Management of acute haemorrhage stroke?

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

Long term management of stroke?

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

If ischaemic = lifelong anticoagulation

105

What is a TIA?

Stroke symptoms which resolve with 24 hours

106

TIA management?

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

What is a SAH and its causes?

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

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

108

Where do berry aneurysms occur?

Branch points

Junction of posterior communicating artery with internal carotid

Junction of ACA and anterior cerebral artery

109

Clinical features of SAH?

Sudden onset headache
Collapse

LP = bloodstained CSF

CT = Well defined round hyper attenuated lesion

110

SAH management?

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

What is a subdural haemorrhage?

Features?

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

Subdural investigations and management?

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

What is an extradural?

Clinical features?

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

Extradural investigations and management?

CT = lens shaped haematoma + skull fracture

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

115

Two types of intracranial venous thrombosis?

Causes?

Dural venous, Cortical Vein

Pregnancy, OCP, head injury, dehydration and thrombophilia

116

Types of Dural venous sinus thrombosis type and PC?

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

Investigations and management of intracranial venous thrombosis?

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

Delirium definition?

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

119

Clinical features and management of neuroleptic malignant syndrome?

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

Stop anti-psychotic
Fluids
Bromocriptine or Dantrolene

120

What is dementia?

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

121

Pathology of Alzheimers?

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

122

Classification of Alzheimers severity?

MMSE:

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

123

Alzheimers management?

Structured cognitive stimulation

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

Severe = Memantine

124

Clinical features and management of vascular dementia?

Step wise progression, with vascular risk factors

Manage the predisposing RF's

125

Lewy body dementia pathology, PC and management?

Lewy bodies in occipital-parietal cortex

PC = fluctuating cognition, visual hallucinations and Parkinsonism

Management = AChE inhibitors

126

Fronto-temporal dementia pathology, PC and scan findings?

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

Parkinsons pathology?

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

Parkinsons features?

Tremor, rigidity and akinesia / bradykinesia

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

129

Management of Parkinson's?

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

SE's of L-DOPA?

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

131

What class is pramipexol / rotigotine and SE's?

Dopamine agonist

Impulse behaviour
Oedema
Fibrosis

132

How do MAO inhibitors and COMT inhibitors work?

Both inhibit the breakdown of dopamine

133

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

Stop drug

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

134

What three conditions make ups MSA?

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

Clinical features of MSA?

Autonomic dysfunction = postural hypotension and bladder dysfunction

Cerebellar signs
Rigidity > tremor

136

PC of corticobasal degeneration?

Parkinsonism
Speech disturbances
Alien limb

Tau +ve

137

PSP clinical features?

Supranuclear gaze and postural instability = can't look down

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

138

What is multiple sclerosis?

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

139

MS classification?

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

140

MS relapses vs progression

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

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

141

Clinical features of MS?

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

MS investigations?

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

Management of MS?

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

Management of specific MS problems?

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

145

Clinical features of cord compression?

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

146

Management of cord compression?

Trauma = immobilise, surgery and methylpred.

Malignancy = Methylpred. ± surgery ± radiation

Abscess = Vancomycin + metronidazole + cefotaxime

147

Cauda equina signs and management?

Saddle anaesthesia, back pain and poor anal tone

bilateral flaccid and areflexic lower limbs

Incontinence / retention

Mx = Laminectomy

148

What is spondylosis?

Degenerative change of intervertebral discs

149

Cervical spondylosis symptoms?

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

Lumbosacral spondylosis symptoms?

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

Spondylosis investigations and management?

MRI is definitive investigation

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

152

Cervical myelopathy causes, clinical features and management

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

PC of musculocutaneous lesion?

Weak flexion at elbow, absent biceps reflex.
Numbness over lateral forearm

154

PC of axillary nerve damage?

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

155

PC of radial nerve damage?

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

PC of median nerve lesion?

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

PC of ulnar nerve lesion?

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

Causes of polyneuropathies?

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

159

Investigations for polyneuropathy?

B12 levels.
MRI may show you subacute combined degeneration

Diabetes = HbA1c

Alcohol = AST:ALT ratio, GGT

160

What is GBS?

Causes?

An acute inflammatory post-infectious neuropathy, antibodies to gangliosides

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

161

Clinical features of GBS?

Prodromal = malaise, vomtiing, headache, limb pain

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

162

Types of GBS?

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

GBS management?

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

85% full recovery

164

What is CMT and the two types?

Hereditary sensory and motor neuropathy. Autosomal dominant

CMT1 = commonest, autosomal dominant, demyelination

CMT2 = second commonest, autosomal dominant, axonal degeneration

165

Clinical features of CMT?

FHx is key = pes cavus, neuropathy or abnormal gait

Walking difficult with foot drop / high stepping gait

Pes cavus

Reduced reflexes, sensation,

166

Management of CMT?

Physiotherapy with low impact exercise and stretching

Bracing

Orthopaedic surgery to correct foot deformities

167

Types of MND?

UMN AND LMN

Amyotrophic lateral sclerosis

Primary lateral sclerosis

Progressive muscular atrophy

Bulbar or pseudo bulbar palsy

168

Features of ALS?

Most common MND
Lesion to corticospinal tracts

UMN in legs, LMN in arms

progressive and unrelenting with 3-5 year survival

169

features of PLS

Isolated UMN with progressive weakness and spasticity

Slower disease progression vs ALS

170

Features of progressive muscular atrophy

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

Median survival = 4.6 years = BEST PROGNOSIS

171

Features of pseudobulbar palsy

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

Bulbar palsy

Impairment of CN9/10/12

Get dysarthria, dysphagia and poor swallow
Nasal speech
LMN signs

173

MND management?

Riluzole 50mg PO BD =
Prevents stimulation of glutamate receptors

Can prolong life by 3 months

174

Myopathy vs neuropathy?

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

175

Different causes of myopathies?

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

Metabolic e.g. thyroid, graves

Drug induced e.g. alcohol, statins and steroids

176

clinical features and management of Duchennes

X-linked, non-functional dystrophin

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

Management = prednisollone, creatine and supportive

177

Clinical features and management of Becker's?

X-linked, partially functioning dystrophin.

Same as Duchennes

178

features of fascioscapulohumeral dystrophy?

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

Features of myotonic dystrophy?

PC = Cataracts, hypogonadism, frontal balding and cardiac disorders

Wasting of sternocleidomastoids = Swan necked appearance.

180

What is myasthenia gravis?

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

Antibodies vs. the Ach receptor

181

Myasthenia associations in men vs women?

Men = thyme atrophy / tumour

Women = autoimmune disease e.g. RA, DM

182

Clinical feature of Myasthenia?

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

Normal reflexes

183

What can precipitate Myasthenia?

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

184

Investigations and Management of Myasthenia?

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

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

185

What is LEMs?

Causes?

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

Clinical features of LEMs and management?

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

movement IMPROVES symptoms

Mx:
Treat underlying cause
Amifampridine
Refractory = IVIG

187

Clinical features of NFT1?

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

Clinical features NFT2?

Bilateral acoustic neuromas = SNHL, vertigo

189

NFT management?

MDT
Genetic counselling
Surgery for any underlying malignancies

190

What is tuberous sclerosis?

Autosomal dominant condition causing tumours throughout he body.

191

Clinical features of tuberous sclerosis?

Cutaneous
Neurological
Other

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

What is syringomyelia?

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

193

Causes of syringomyelia?

Spina bifida

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

Secondary to cord trauma / tumours

194

Clinical features and management of syringomyelia?

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

What is Friedrich's ataxia?

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

196

Clinical features of Friedrich's ataxia?

Neurological:
Ataxia
Absent reflexes
Dysarthria
Weakness and wasting (relatively late sign)

Non-neuro:
Pes cavus
Scoliosis
Cardiac = HOCM
DM