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Phase 3A Medicine > Neuro > Flashcards

Neuro Flashcards

1
Q

Define a headache

A

Pain or discomfort between the orbits and the occiput, arising from pain-sensitive structures

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2
Q

List 4 intracranial pain-sensitive structures

A
  • Venous sinuses
  • Cortical veins
  • Basal arteries
  • Dura of anterior, middle and posterior fossae
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3
Q

List 6 extracranial pain-sensitive structures

A
  • Scalp vessels and muscles
  • Orbital contents
  • Mucous membranes of nasal and paranadal spaces
  • External and middle ear
  • Teeth
  • Gums
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4
Q

Examination of headache?

A

Full general examination

  • Ocular - acuity, tenderness, stabismus
  • Teeth and scalp
  • Percussion over frontal and maxillary sinuses
  • Fever?
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5
Q

Primary causes of headache?

A
  • Migraine
  • Tension
  • Cluster
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6
Q

Secondary causes of headache?

A
  • Meningitis
  • Subarachnoid haemorrhage (SAH)
  • Medication overuse
  • Raised ICP
  • Low CSF pressure
  • Trigeminal neuralgia
  • GCA
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7
Q

What is the most common type of headache in children?

A

A headache accompanying febrile illness or infection of the nasal passages or sinuses

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8
Q

What red flags are you looking for in children and what could these infer?

A
  • neck stiffness
  • impaired conscious level

–> meningitis, encephalitis or cerebral abscess

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9
Q

What would acute obstructive hydrocephalus and a headache suggest to you in a child?

A

Intracranial tumour, occurring in the midline (medulloblastoma, pineal region tumour)

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10
Q

Red flags for urgent investigation of a headache?

A
  • New headache with history of cancer
  • Cluster headache (pituitary)
  • Seizure
  • Significantly altered consciousness levels, memory loss, confusion, co-ordination changes
  • Papilloedema
  • Neck stiffness
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11
Q

Duration of tension headaches?

A

30 mins to 7 days

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12
Q

Frequency of tension headaches?

A
  • Infrequent or daily
  • Worse towards end of the day
  • May persist over years
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13
Q

Mechanism of tension headache?

A
  • ‘Muscular’
  • Due to persistent contraction, e.g. clenching teeth, head posture, furrowing of brow
  • Some overlap with transformed migraine
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14
Q

Treatment of tension headache?

A
  • Reassurance
  • Short term analgesia but reduce over-use
  • Stress relief
  • Amitriptyline and other tricyclic antidepressants
  • Beta-blockers
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15
Q

Characteristics of tension headaches?

A
  • Bilateral
  • Non-pulsatile
  • Diffuse, dull, aching
  • Band-like
  • Worse on touching scalp
  • May be aggravated by noise
  • ASSOCIATED WITH DEPRESSION and anxiety
  • NOT aggravated by physical activity
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16
Q

Define migraine

A

A common, often familial disorder characterised by UNILTERAL THROBBING HEADACHE
- aggravated by physical activity

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17
Q

Onset of migraine?

A

Childhood or early adult life

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18
Q

Incidence of migraine?

A

5 - 10% of population

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19
Q

Who do migraines affect more?

A

Females

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20
Q

Family history of migraines?

A

70%

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21
Q

What are the two forms of migraine?

A

Migraine WITH aura, and migraine WITHOUT aura

Aura = visual, sensory of motor type followed by headache - throbbing, unilateral, worsened by bright lights, relieved by sleep, associated with nausea and sometimes vomiting

Without aura can sometimes sound like a tension headache!

Can also get aura by itself.

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22
Q

Duration of migraines?

A

2 - 72 hours, rarely occur more frequently than twice a week

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23
Q

What are the two types of migraine with aura?

A
  • Basilar: bilateral visual symptoms, unsteadiness, dysarthria, vertigo, limb paraesthesia. Possible loss of consciousness. Affects young women.
  • Hemiplegic: aura of unilateral paralysis which persists for some days after settling of headache. Often misdiagnosed as stroke. Dominant inheritance is noted.
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24
Q

Triggers of migraine?

A 
CHOCOLATES:
C: Chocolate
H: Hangovers
O: Orgasm
C: Cheese/caffeine
O: Oral contraceptive pill/premestrual
L: Lie ins
A: Alcohol/anxiety
T: Travel
E: Exercise
S: Stress
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25
Q

Management of migraines?

A

Identify and avoid triggers

Acute attacks:

  • Simple analgesics (aspirin) with metoclopramide
  • Anti-emetics (domperidone/prochlorperazine)
  • Sumatriptan and other triptans (PO or SC)
  • Ergotamine
  • Methylprednisolone

Prophylaxis:

  • Propranolol (NOT in asthmatics)
  • Topiramate
  • Pizotifen
  • Verapamil
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26
Q

What is a medication overuse headache?

A

When some patients with episodic tension or migraine have headaches all the time so they start taking regular medication. This overuse itself can lead to worse headaches.
Using meds 10-15 days in a month.

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27
Q

Which drugs commonly cause drug overuse headaches?

A
  • Analgesia - mixed (paracetamol + codeine/opiates)
  • Triptans
  • Ergotamine
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28
Q

Management of drug overuse headaches?

A

Stop the medication!

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29
Q

What is a transformed migraine?

A

When patients with migraine go on to develop chronic daily headaches without overusing medication. Usually responds to migraine prophylactic agents.

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30
Q

What is a cluster headache?

A
  • The most disabling primary headache
  • Caused by a pituitary tumour
  • More common on middle age men
  • May be precipitated by alcohol
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31
Q

Duration of cluster headaches?

A
  • The headache itself lasts 15 mins - 2 hours
  • Clusters last 4-12 weeks
  • Then followed by pain-free months or 1-2 years before next attack
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32
Q

Mechanism of cluster headaches?

A
  • Superficial temporal artery smooth muscle hyper-reactivity to 5-HT
  • Hypothalamic grey matter abnormalities
  • Autosomal dominant gene
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33
Q

Diagnosis of cluster headached?

A

At least 5 headaches fulfilling:

  • Severe or very severe UNILATERAL ORBITAL, SUPRAORBITAL or TEMPORAL pain lasting 15 - 180 mins
  • Accompanied by IPSILATERAL cranial autonomic features and/or agitation or restlessness
  • Can be from 1 every other day to 8 a day
  • No attributed to another disorder
  • “Alarm clock headache”: wakes them at night

Extra info:

  • May have watery red eye and vomiting
  • May be pacing and restless
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34
Q

Management of cluster headaches?

A

ACUTE:

  • 100% O2 for 15 mins through non-rebreathable masks
  • Sumatriptan SC at onset

PREVENTION:

  • Suboccopital steroid injections
  • Intravenous CIVAMIDE
  • Veramapril
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35
Q

Define trigeminal neuralgia

A
  • Chronic pain disorder that affects the third branch of the trigeminal nerve = MANDIBULAR
  • Believed to be due to demyelination of the trigeminal nerve
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36
Q

Causes of trigeminal neuralgia?

A
  • Inflammation
  • Aneurysm
  • Tumour!!
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37
Q

Triggers for trigeminal neuralgia?

A
  • Shaving
  • Washing
  • Eating
  • Talking
  • Dental prostheses
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38
Q

Signs and symptoms of trigeminal neuralgia?

A

At least 3 attacks of UNILATERAL facial pain fulfilling:

  • Occurring in 1 or 2 distributions of the trigeminal nerve
  • Pain has at least three of: reoccurring paroxysmal attacks from seconds - 2 mins; severe intensity; electric-shock-like; precipitated by stimulus to face
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39
Q

Management of trigeminal neuralgia?

A

Carbamazepine
OR Lamotrigine
If meds fail -> microvascular decompression

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40
Q

What is giant cell arteritis?

Describe its epidemiology?

A
  • Autoimmune disease of unknown cause
  • Throbbing headache with general malaise
  • Common in the ELDERLY
  • Associated with POLYMYALGIA RHEUMATICA
  • Secondary causes: SLE, RA, HIV

Consider in all OVER 50s with HEADACHE!!!

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41
Q

Signs and symptoms of GCA?

A
  • Headache (gradual onset)
  • Temporal artery = tender and thickened but non-pulsatile
  • Tender scalp
    = JAW CLAUDICATION
  • Amaurosis fugax (blind drawing down) or diplopia
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42
Q

Diagnosis of GCA?

A
  • Raised ESR and CRP
  • Raised platelets and alkaline phosphatase
  • Low Hb
  • Temporal artery biopsy: within 7 days of steroids
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43
Q

Mechanism of GCA?

A
  • Large and medium-sized arteries undergo ‘giant cell’ infiltration with fragmentation of the lamina and narrowing of the lumen
  • Results in distal ischaemia as well as stimulating pain sensitive fibres
  • Occlusion of important end arteries, e.g. ophthalmic
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44
Q

Treatment of GCA?

A

URGENT PREDNISOLONE: 60 mg daily

if complications already occurred -> give parenteral high dose steroids

Maintenance steroids = 5 mg daily: monitor the ESR to get down to maintenance dose

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45
Q

Characteristics of a raised ICP headache?

A
  • Aggravated by bending/coughing
  • Worse in morning upon awakening or may awaken patient
  • Severity progresses gradually
  • Vomiting in later stages
  • Transient loss of vision when sudden change in posture
  • Papilloedema
  • Focal signs
    MRI or CT = Essential!
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46
Q

Describe sinus headache

A
  • Well localised, worse in morning, affected by posture
  • XR: opacified sinuses
  • Treatment: decongestants or drainage
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47
Q

Describe ocular headache

A
  • Refraction errors may result in ‘muscle contraction’ headaches
  • Resolves when corrected with glasses
  • Acute glaucoma can produced headache but accompanied by misting of vision
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48
Q

Describe dental disease headaches

A
  • Discomfort localised to teeth
  • Check for malocclusion
  • Check temporomandibular joints
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49
Q

GCS

A

Eye opening:
1 = none; 2 = to pain, 3 = to speech, 4 = spontaneous

Verbal response:
1 = none; 2 = incomprehensible, 3 = inappropriate words, 4 = confused speech, 5 = orientated

Motor response:
1 = none; 2 = extension to pain, 3 = flexion to pain, 4 = withdraw from pain, 5 = localises pain, 6 = obeys commands

Max score = 15, Min score = 3

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50
Q

Definition of a stroke

A

Rapid onset focal CNS signs and symptoms, with presumed vascular origin, lasting more than 24 hours

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51
Q

Two types of stroke and how common is each?

A

Ischaemic (85%) - Infarct-thromboembolism
Haemorrhagic (15%) - Bleeding:
- Hypertension (BERRYS ANEURYSM)
- Lobar

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52
Q

Causes of stroke?

A
  • Small vessel occlusion
  • Cardiac emboli (AF/prosthetic valve/MI)
  • Atherothromboembolism
  • CNS bleeds
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53
Q

Risk factors for stroke?

A
  • HTN
  • Smoking
  • Alcohol excess
  • DM
  • Heart disease
  • PVD
  • Previous TIA
  • Carotid bruit
  • Combined pill
  • Hyperlipidaemia
  • Increased clotting
  • Syphilis
  • Age and sex
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54
Q

Diagnosis of stroke

A
  • URGENT CT HEAD: rule out haemorrhage before thrombolysis
  • Pulse, BP and ECG - look for AF
  • FBCs - look for thrombocytopenia and polycythaemia
  • Glucose - rule out hypoglycaemia
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55
Q

Describe CT scans and how different materials present. What would you see in each stroke?

A

WHITE: Fresh blood, calcium, bone
DARK: Fluid (e.g. CSF), air, fat (e.g. lipomas)

Haemorrhagic stroke: fresh blood = bright white! White -> Isodense -> hypodense

All stroke eventually goes DARK (subacute - chronic stage) as the space get filled with CSF

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56
Q

List some general management of an acute stroke to maximise reversible ischaemic tissue

A
  • Hydration
  • Oxygenation (> 95%)
  • Consider antihypertensive if BP > 185/110 (20% drop may compromise cerebral perfusion)
  • Maintain glucose between 4 - 11 mmol/l
  • Treat chest infections and cardiac failure/dysrhythmias
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57
Q

Describe thrombolysis

A
  • Intravenous recombinant tissue plasminogen activator (ALTEPLASE)
  • Give within 4.5 hours of anterior circulation ischaemic stroke
  • Start antiplatelet therapy 24 hrs after thrombolysis (CLOPIDOGREL)
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58
Q

CIs of thrombolysis?

A
  • Uncertain time of onset
  • Spontaneously improving
  • Head injury or previous stroke in last 3 months
  • GI surgery in last 21 days
  • BP > 180/110
  • On an anticoagulant
  • Seizure
  • Hypodensity on CT
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59
Q

Management of stroke if thrombolysis not an option?

A
  • 300 mg ASPIRIN daily for 2 weeks (or Clopidogrel if CI)
  • Then lifelong Clopidogrel
  • Anticoagulants should be avoided if possible as increase risk of haemorrhagic transformation
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60
Q

Non-medical management of stroke?

A
  • Transfer to stroke unit
  • Assess swallow (SALT)
  • Early mobilisation
  • Physiotherapy
  • Home modifications
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61
Q

Management of haemorrhagic stroke?

A
  • Frequent GCS monitoring
  • Anitplatelets = contraindicated
  • Any anticoagulation should be reversed
    (e. g. Beriplex or Vit K for warfarin)
  • Control hypertension - BETA-BLOCKERS
  • Manual decompression if raised ICP
  • Or diuretics to reduce ICP (MANNITOL)
  • Maybe surgery
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62
Q

How to calculate stroke risk (post-TIA)?

A

ABCD2
1-3: 0.4%
4-5: 12%
6: 31.4%

HIGH RISK IF:

  • ABCD2 > 4
  • > 1 TIA in previous 7 days
  • New arrhythmia
  • Carotid stenosis
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63
Q

Prevention of stroke?

A
  • Lifelong platelet treatment if already had stroke: Aspirin + Dipyridamole + Clopidogrel
  • Cholesterol treatment, e.g. SIMVASTATIN
  • AF treatment, e.g. WARFARIN
  • BP treatment, e.g. RAMIPRIL
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64
Q

Define meningitis

A

Inflammation of the meninges (membrane of the brain and spinal cord)

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65
Q

Common meningitis organisms?

A

Common:

  • Meningococcus (Neisseria meningitides): Gram-negative bacteria, diplococcus
  • Pneumococcus (streptococcus pnuemoniae): Gram-positive bacteria, diplococcus
  • Listeria monocytogenes (In immunocompromised and babies): Gram-positive bacteria, Bacilli

NEONATES: GBS

Others:
- E.coli, Haemophilus influenzae, TB, cytomegalovirus, cryptococcus

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66
Q

Clinical presentation of meningitis?

A

EARLY:

  • Headache
  • Leg pains
  • Cold hands and feet
  • Abnormal skin colour

LATE:

  • MENINGISM - NECK STIFFNESS, PHOTOPHOBIA, +VE KERNIGS SIGN
  • Reduced consciousness
  • Coma
  • Seizures
  • Focal CNS signs
  • Papilloedema
  • Brudzinski’s sign
  • Petechial rash (non-blanching)
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67
Q

What is Kernig’s sign?

A

With hip at 90 degrees, can’t straighten leg > 135 degrees without pain

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68
Q

What is Brudinski’s sign?

A

Flex patients neck -> patient compromised by flexing hips and knees

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69
Q

What is different in the presentation in viral meningitis?

A

Does not present with a rash!

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70
Q

Diagnosis of meningitis?

A
  • Bloods: FBC, UE, LFT, glucose, coagulation screen, lactate
  • Blood cultures: before antibiotics
  • Throat swabs
  • CT head - check for space-occupying lesions and ICP
  • Lumbar puncture - If not sign of ICP

LP:

  • Opening pressure is HIGH
  • Send CSF for gram stain, MCS, protein, glucose, vriology/PCR, lactate
  • CSF in bacteria: high neutrophils, high protein, low glucose
  • CSF in viral: high lymphocytes, high or normal protein, normal glucose
  • If septic –> do NOT attempt LP
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71
Q

Treatment of meningitis?

A 
MEDICAL EMERGENCY if any 2 of:
- Pyrexia
- Headache
- Neck stiffness
- Altered mental state
Give immediate IV FLUIDS and IM/IV BEN PEN

Immediate antibiotics:

  • If < 55yrs –> CEFOTAXIME
  • If > 55yrs or IC –> CEFOTAXIME and AMPICILLIN
  • If returned traveller –> VANCOMYCIN
  • If listeria –> add AMOXICILLIN

If viral –> ACYCLOVIR

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72
Q

How would you manage a close contact of a meningitis patient?

A

PROPHYLAXIS:

  • 1st line = CIPROFLOXACIN
  • 2nd line = RIFAMPICIN - NOT in pregnancy
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73
Q

What is meningococcal septicaemia?

A

Meningococcal septicaemia is the cause of the classic “non-blanching rash” that everybody worries about

This rash indicates the infection has caused:

  • Disseminated intravascular coagulopathy (DIC)
  • Subcutaneous haemorrhages
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74
Q

Describe the facial nerve pathway

A
  • Exits brainstem at cerebellar pontine angle
  • passes through the TEMPORAL BONE and PAROTID GLAND
  • Divides into 5 branches:
    o Temporal
    o Zygomatic
    o Buccal
    o Marginal mandibular
    o Cervical
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75
Q

Describe the function of the facial nerve

A

Motor, Sensory and Parasympathetic nerve supply

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76
Q

Describe motor function of facial nerve

A
  • Supplies muscles of facial expression
  • Stapedius of inner ear
  • Posterior digastric, stylohyoid and platysma muscles of the neck
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77
Q

Describe sensory function of facial nerve

A

Taste from ANTERIOR 2/3rds of tongue

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78
Q

Describe parasympathetic supply of facial nerve

A
  • Supply to SUBMANDIBULAR and SUBLINGUAL salivary glands

- Supply to LACRIMAL gland (tear production)

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79
Q

Upper vs lower motor neuron facial palsy?

A

UPPER spares UPPER

- Must refer anyone with UMN facial palsy urgently with suspected stroke

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80
Q

Name some UMN facial nerve lesions

A 
Unilateral:
- Cerebrovascular event, e.g. stroke
- Tumours
Bilaterally:
- Pseudobulbar palsies
- MND
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81
Q

What is Bells and what is the epidemiology?

A
  • Idiopathic
  • Unilateral
  • Peak = 20 - 40 yrs
  • More common in pregnant women
  • LMN so forehead involvement
  • May also notice altered taste, dry eyes, hyperacusis
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82
Q

How would you manage Bells palsy?

A
  • Most recover fully within few weeks though can take 12 months and some will have residual weakness forever
  • If presents within 72 hours: PREDNISOLONE
  • Lubricating eye drops
  • If eye pain -> review for exposure keratopathy
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83
Q

What is Ramsay-Hunt syndrome?

A
  • Caused by herpes-zoster virus
  • Painful and tender vesicular rash in ear canal, pinna and around ear on affected side
  • Rash may extend to anterior 2/3rds of tongue
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84
Q

Management of Ramsay-Hunt syndrome?

A

Ideally within 72 hours

  • Prednisolone
  • Acyclovir
  • Lubricating eye drops
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85
Q

What is encephalitis?

A

Inflammation of the brain matter itself

- Typically affects the temporal and inferior frontal lobes

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86
Q

What are the common causes of encephalitis?

A 
VIRAL:
- HSV 1 and 2 and varicella-zoster
(HSV1 responsible for 95% in ADULTS)
- HIV/EBV/measles/mumps/rabies/jap enc/arbovirus
NON-VIRAL:
- Bacterial meningitis
- TB
- Malaria
- Listeria
- Lyme disease
- Legionella
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87
Q

Sign and symptom of encephalitis?

A
  • Fever
  • Headache
  • Lethargy
  • Seizures
  • Off behaviour
  • Psychiatric symptoms
  • Vomiting
  • Focal neurological features, e.g. aphasia
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88
Q

Investigations and results for encephalitis?

A
  • LP/CSF: lymphocytosis and elevated protein; low/normal glucose
  • viral PCR for HSV
  • CT: Petechial haemorrhages - white areas of enhancement in temporal and inferior frontal lobes. Normal in 1/3. Do before LP.
  • MRI is better
  • EEG: lateralised periodic discharges at 2 Hz
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89
Q

Management of encephalitis?

A
  • IV ACYCLOVIR within 30 mins of admission for 2 weeks

- Should be started in all cases of suspected encephalitis

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90
Q

What is hydrocephalus?

A
  • An excessive volume of CSF within the ventricular system of the brain
  • Caused by an imbalance between CSF production and draining or absorption
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91
Q

Presentation of hydrocephalus in adults?

A

Symptoms of raised ICP:

  • Headache (Raised ICP)
  • Nausea and vomiting
  • Papilloedema
  • Coma (in severe cases)
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92
Q

Presentation of hydrocephalus in children?

A
  • Bulging and tense anterior fontanelle
  • Increased head circumference
  • Failure of upward gaze (‘sunsetting eyes’) due to compression of the superior colliculus of the midbrain
  • Poor feeding and vomiting
  • Poor tone
  • Sleepiness
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93
Q

Two categories of hydrocephalus? The pathology of each?

A

Obstructive (non-communicating)

  • Structural pathology blocking the flow of CSF
  • Dilatation of the ventricular system is seen superior to site of obstruction
  • Causes: tumours, acute haemorrhage, developmental abnormalities (e.g. AQUEDUCTAL STENOSIS = most common or Arnold-Chiari malformation)

Non-obstructive (communicating):

  • Imbalance of CSF production/absorption
  • Either increased production of CSF (e.g. choroid plexus) but RARE
  • OR failure of reabsorption at the arachnoid granulations, e.g. meningitis or post-haemorrhage (MORE COMMON)
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94
Q

Investigations in hydrocephalus?

A
  • CT head = FIRST LINE
  • MRI offers more detail - useful is suspected underlying lesion
  • LP:
    o ONLY if NON-OBSTRUCTIVE due to risk of HERNIATION
    o Diagnostic and therapeutic as can allow you to sample CSF, measure opening pressure, and drain CSF to reduce pressure
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95
Q

Management of hydrocephalus?

A
  • External ventricular drain (EVD) in acute, severe cases. Inserted into R lateral ventricle.
  • Ventriculoperitoneal shunt (VPS) = long-term CSF diversion technique - drains CSF from ventricles to the peritoneum
  • OBSTRUCTIVE: may need surgical treatment of the obstructing pathology
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96
Q

What are the complications of a VPS shunt?

A
  • Infection
  • Blockage
  • Excessive drainage
  • Intraventricular haemorrhage
  • Outgrowing them (need replacing every 2 yrs in children)
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97
Q

What is normal pressure hydrocephalus?

A
  • A form of non-obstructive hydrocephalus
  • Characterised by large ventricles but normal ICP
  • Classical triad = dementia, incontinence and disturbed gait (similar to Parkinson’s)
  • Reversible cause of dementia
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98
Q

Diagnosis of normal pressure hydrocephalus?

A

Imaging:

  • enlarged fourth ventricle
  • absence of substantial sulcal atrophy
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99
Q

Management of normal pressure hydrocephalus?

A
  • VPS shunt

- 10% experience significant complications, e.g. seizures infection and intracerebral haemorrhages

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100
Q

What is a subarachnoid haemorrhage (SAH)?

A

Bleeding into the subarachnoid space

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101
Q

Causes of SAH?

A
  • MOST COMMON (85%) = BERRY ANEURYSM (associated with PKD, Ehler-Danlos and CoA)
  • AV malformation (15%)
  • Others: arterial dissection, mycotic infective aneurysms, vasculitis, encephalitis, tumours
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102
Q

Risk factors for SAH?

A
  • HTN
  • Smoking
  • Excess alcohol
  • Cocaine use
  • Family history
  • Black patients
  • Female patients
  • Age 45-70
  • Sickle cell anaemia
  • Neurofibromatosis
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103
Q

Presentation of SAH?

A
  • Sudden-onset occipital headache: ‘worst of my life’, thunderclap headache, ‘like being hit on the back of the head with a bat’
  • Neck stiffness
  • Photophobia
  • Vision changes
  • Neurological symptoms, e.g. weakness, seizures, LOC
  • Nausea and vomiting
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104
Q

Investigation of SAH?

A
  • CT = FIRST LINE: STAR SHAPE (blood in fissures), hyperattenuation in subarachnoid space
  • LP: If CT is negative and no CIs. Must be done after 12hrs of headache onset.
    o Red cell count will be raised (early on)
    o Xanthochromia (yellow caused by bilirubin)
  • Cerebral angiography once SAH confirmed to locate source of bleeding
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105
Q

Management of SAH?

A

Maintain cerebral perfusion

  • Hydration
  • Bed-rest
  • Well-controlled BP (Systolic 160)

NIMODIPINE

  • 21-day course
  • Reduces vasospasm (cerebral artery)

Endovascular coiling
- Using catheter/angiography
- Blocks off the blood vessel
Sometimes requires surgical clipping

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106
Q

Complications of SAH?

A
  • Rebleeding (10% of cases, < 12hrs usually)
  • Vasospasm (delayed cerebral ischaemia): 7-14 days
  • Hyponatraemia (due to SIADH)
  • Seizures (treat with antiepileptics)
  • Hydrocephalus (treat with LP or VPS shunt)
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107
Q

Associations of SAH?

A
  • PKD
  • Ehler-Danlos (or Marfans)
  • CoA
  • Sickle cell
  • Cocaine use
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108
Q

Risk factors for intracranial bleeds?

A
  • Head injury
  • HTN
  • Aneurysms
  • Ischaemic stroke can progress to haemorrhage
  • Brain tumours
  • Anticoagulants, e.g. Warfarin
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109
Q

Define a subdural haemorrhage (SDH)

A

CONSIDER IN ALL WITH FLUCTUATING CONSCIOUSSNESS

  • Rupture of the bridging veins between the cortex and venous sinuses
  • Results in haematoma between the dura and arachnoid mater
  • Gradually rising ICP
  • Midline structures shift away from the side of the clot
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110
Q

Risk factors for SDH?

A
  • ELDERLY and ALCOHOLICS: Have cerebral atrophy which increases tension on cerebral veins
  • Infants have fragile bridging so at risk (shaken baby syndrome)
  • Trauma
  • Falls
  • Anticoagulation
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111
Q

Presentation of an SDH?

A
  • Fluctuating consciousness level
  • Sleepiness
  • Headaches
  • Personality/mood changes
  • Raised ICP
  • Seizures
  • Localising neurological symptoms
  • Vomiting

Sometimes get LATENT PERIOD: after head injury. 8-10 weeks later, clot breaks down –> increased oncotic pressure –> water sucked into hematoma by osmosis

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112
Q

Diagnosis of SDH?

A
  • CT/MRI scan: CRESCENT SHAPE, clot and midline shift, not limited by suture lines
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113
Q

Treatment of SDH?

A
  • 1st line = Clot evacuation (If develops weeks after a head injury)
  • Irrigation/burr hole
  • 2nd line = Craniotomy (if develops soon after a head injury)

Minor: observed conservatively, may need decompressive craniectomy

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114
Q

What is an extradural haemorrhage (EDH)?

A
  • Collection of blood between SKULL and DURA
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115
Q

Where is an EDH most common and why?

A

Often in the temporal region (thin skull overlying MIDDLE MENINGEAL ARTERY)

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116
Q

Causes of EDH?

A
  • Fractured temporal/parietal bone (middle meningeal artery) - typical after trauma to temple just lateral to eye
  • Tear in dural venous sinus
  • Rapid rise in ICP –> ventricles get rid of CSF to prevent this rise
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117
Q

Sign and symptoms of EDH?

A
  • Fall in consciousness
  • Followed by lucid interval (temp. improvement)
  • Severe headache
  • Vomiting
  • Confusion
  • Brisk reflexes
  • Seizures
  • Late signs: raised BP, bradycardia
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118
Q

What happens if left untreated and what later signs might you see if bleeding continues in an EDH?

A

Coning (squeezing of brain and brainstem through the foramen magnum because of swelling)

  • Ipsilateral pupil dilates (compression of parasymp fibres of 3rd cranial nerve
  • Coma deepens
  • Bilateral limb weakness
  • Breathing = deeper and irregular (brainstem compression)
  • -> Death
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119
Q

Diagnosis of EDH?

A
  • CT: LENS-SHAPED (round). Limited to suture lines of skull.

- XR: Fracture lines crossing the course of middle meningeal artery

120
Q

Treatment of EDH?

A
  • Clot evacuation
  • Ligation of bleeding
  • Decrease ICP
121
Q

Define coma

A

Unconsciousness = not aware and not rousable

122
Q

Commom causes of coma?

A
  • Drugs/toxins (opiates/EtOH/CO)
  • Anoxia (post-arrest)
  • Mass lesions (bleeds - head injury)
  • Infections (bacterial meningitis)
  • Metabolic (hypoglycaemia/DKA/uraemia)
  • SAH
  • Epilepsy
123
Q

Uncommon causes of coma?

A
  • Mass lesions (tumour)
  • Venous sinus occlusions
  • Hypothermia
  • Psychiatric (catatonia)
124
Q

History of an unconscious person?

A
  • Collateral history from relatives/friends/ambulance/nursing staff/GP
  • Previous events, e.g. suicide notes, travel history
  • PMH: general, psychiatric, history of head injury, alcohol abuse,
  • Drug history: recreational, insulin, antiepileptics
125
Q

General examination of an unconscious person?

A
  • Trauma - skull#, head injury
  • Fever
  • Hypothermia
  • Breath odour (alcohol/ketoacidosis)
  • Needle marks
  • Stigmata of liver disease
  • Evidence of convulsion
  • Skin colour
  • ASSESSMENT OF GCS
  • Check for MENINGISM
126
Q

Brainstem examination of an unconscious patient?

A
  • Pupil size
  • Pupil reactions
  • Eye movements
  • Corneal reflexes
127
Q

Focal examination of an unconscious patient?

A
  • Asymmetry of motor function?
  • Tendon reflexes
  • Plantar responses
128
Q

Investigations in an unconscious patient?

A
  • Bloods: FBC, UE, glucose, Ca, Phosphate, LFT, clotting, toxicology (incl. alcohol), ABGs, anion gap
  • Imaging: CT head, MRI head, ECG
  • LP
  • EEG: Only if strongly indicated!
  • Rarely: cortisol, TFTs
129
Q

Differentials of sudden or subacute new headache?

A
  • SAH
  • Cerebral venous thrombosis
  • Dissection: carotid/vertebral
  • Infection: meningitis, encephalitis, cerebral abscess
  • Acute haemorrhage
130
Q

Management of acute headache?

A
  • Need to know when it escalated to 10/10 pain
  • 2 commonly used treatments:
    o Surgical clipping
    o Endovascular coiling
131
Q

Define status epilepticus

A

Official definition: Persistent seizure activity for 30 mins+

  • Continuous
  • Intermittent attacks without the recovery of consciousness

Practical definition: convulsive seizure activity for > 10 mins

= MEDICAL EMERGENCY

132
Q

Causes of status epilepticus?

A
  • Anticonvulsant withdrawal (21%)
  • Cerebrovascular disease (21%)
  • Alcohol withdrawal (18%)
  • Metabolic disorders (13%)
  • Haemorrhage (7%)
  • Infectious disorders (4%)
  • Hypotension (4%)
  • Tumours (3%)
  • Anoxia (2%)
133
Q

Why is status epilepticus a medical emergency?

A

Because if the seizure activity is prolonged, it can lead to irreversible brain damage

134
Q

Management of status epilepticus?

A
  • ABCDE + GLUCOSE (oxygen, airway adjunct, IV access)
  • 1ST LINE: BENZODIAZEPINES
    e. g. Lorazepam IV 4 mg, repeated after 10 mins if continues
  • If seizures persist: PHENYTOIN or PHENOBARBITAL IV infusion
  • If not response within 45 mins - general anaesthetic (e.g. Propofol)
135
Q

List 5 neurological emergencies

A
  1. Coma
  2. Sudden/subacute new headache
  3. Weakness
  4. Visual loss
  5. Status epilepticus
136
Q

Basic management of neurological emergencies?

A

ABCDE(f)G
(DEFG = Don’t Ever Forget Glucose)

A: Airway
B: Breathing
C: Circulation
D: Deficits
E: Environment (hypo/hyperthermic)
(f)
G: Glucose
137
Q

What is myasthenia gravis?

A

AUTOIMMUNE disease against NICOTINIC ACETYLCHOLINE RECEPTORS in the neuromuscular junction –> reduced muscle contraction (85%)

In 15% of those with myasthaenia gravis:

  • Muscle-specific kinase
  • Low-density lipoprotein receptor-related protein 4 (LRP4)
138
Q

Presentation of myasthenia gravis (MG)?

A
  • WEAKNESS: Worse with muscle use, improves with rest (better in the morning)

Mainly in head and neck:

  • Extraocular muscles –> diplopia
  • Eyelid muscles –> partial ptosis
  • Facial muscles –> reduced facial movements (snarly smile)
  • Bulbar muscles –> swallowing/chewing problems and slurred speech
139
Q

What does MG only cause partial ptosis?

A

Because the levator palpebrae superioris is 50/50 smooth muscle and striatal muscle

140
Q

Examination of MG?

A
  • Repetitive blinking to elicit ptosis
  • Count to 50 to elicit speech tiring
  • Upward gazing to elicit double vision
  • Repetitive adduction of arm X20 will elicit unilateral weakness
  • Check for thymectomy scar
  • Can check FVC to check breathing muscle strength
141
Q

Epidemiology of MG?

A

Peak in women < 40 yrs

Peak in men > 60 yrs

142
Q

Pathophysiology of MG?

A
  • Autoimmune disease mediated by ANTIBODIES to the acetylcholine receptor (Anti-AChR)
  • Interferes with neuromuscular junction via depletion of working post-synaptic receptor sites
  • Both T-cell and B-cells are implicated
  • Blocks the excitatory effect of ACh on the nicotinic receptors –> MUSCLE WEAKNESS
143
Q

Diagnosis of MG?

A

ANTIBODIES:

  • Anti-AChR in 85%
  • Anti-MUSK in 10%
  • Anti-LRP4 in <5%

CT/MRI of Thymus gland to look for hyperplasia, atrophy or tumour (thymoma)

Single fibre electromyography:
- High sensitivity (92-100%)

Edrophonium test:

  • IV dose of Edrophonium Chloride
  • Briefly and temporarily improve weakness
144
Q

Management of MG?

A

Symptom control:
- Anticholinesterase inhibitors (ORAL PYRIDOSTIGMINE)

Immunosupression:
- ORAL PREDNISOLONE

Steroids may be combined with:
- ORAL AZATHIOPRINE or METHOTREXATE

Thymectomy:
- Removal of the thymus if onset <50yrs and poorly controlled

Monoclonal antibodies:

  • Rituximab (B-cells)
  • Eculizumab (C5 cells) (NOT on NHS)
145
Q

What is a myasthenic crisis?

A

Severe and potentially life-threatening complication

  • Acute worseing of symptoms
  • Including RESPIRATORY muscles –> can lead to RESP FAILURE
  • Causes: infection, natural disease cycle, under/over dosing on meds
146
Q

Management of MG crisis?

A
  • Urgent neurologist review
  • Non-invasive ventilation with BiPAP
  • Or full intubation and ventilation
  • IV IMMUNOGLOBULINS
  • PLASMAPHERESIS (plasma exchange and antibody removal)
147
Q

Associations with MG?

A
  • THYMOMAS (15%)
  • Autoimmune disorders: pernicious anaemia, autoimmune thyroid disorders, RA, SLE
  • Thymic hyperplasia (50-70%)
148
Q

What is Guillian Barre syndrome (GBS)?

A
  • An acute inflammatory demyelinating polyneuropathy –> SCHWANN CELLS
  • Often triggered by an infection
149
Q

Pathophysiology of GBS?

A
  • Molecular mimicry
  • B cells of the immune system create antibodies against pathogens that cause the preceding infection
  • These antibodies also match the proteins on the myelin sheath of motor nerve cells or nerve axons
  • Cross reaction of antibodies with gangliosides in the peripheral nervous system
  • Correlation between the anti-ganglioside antibody (e.g. anti-GM1) and clinical features has been demonstrated
  • anti-GM1 antibody in 25% of patients
150
Q

What are the most common causes of GBS?

A
  • CAMPYLOBACTER JEJUNI

- Also CMV, or EBV

151
Q

Presentation and course of GBS?

A
  • Few weeks after infection (~4 weeks)
  • Symmetrical ascending muscle weakness
  • Reduced reflexes
  • May be peripheral loss of sensation or neuropathic pain
  • May progress to cranial nerves and cause facial nerve weakness
  • If autonomic dysfunction (involvement of heart): sweating, increased pulse, BP changes, arrhythmias
152
Q

Investigations of GBS?

A
  • Brighton criteria for a clinical diagnosis
    Supportive investigations:
  • Nerve conduction studies: SLOW conduction
  • LP of CSF: high protein, normal WCC and glucose
    (change in WCC would indicate a different diagnosis)
  • Monitor FVC and ECG (resp failure and autonomic dysfunction are biggest GBS killers)
153
Q

Management of GBS? And prognosis?

A
  • IV IMMUNOGLOBULINS for 5 DAYS
  • Plasma exchange (Plasmapheresis)
  • If resp involvement –> ventilation and measure FVC
  • 85% will make a good recovery, however, 10% are unable to walk after 1 year
154
Q

What is myasthenic Lambert-Eaton syndrome?

A
  • Progressive muscle weakness with increased use as result of damage to the NMJs
  • Antibodies directed against the PRE-synaptic VOLTAGE-GATED CALCIUM CHANNELS
  • Similar set of symptoms to MG but more insidious and less pronounced
155
Q

What is LE syndrome associated with?

A
  • Small cell lung cancer

- Less so: Breast/ovarian cancer

156
Q

Presentation of LE syndrome?

A
  • Same as MG
  • Proximal leg muscle weakness is the key feature
  • Reduced reflexes
  • Sometimes see an increase in muscle strength and normal reflexes (not seen in MG) = post-tetanic potentiation
  • Autonomic features: dry mouth, impotence, difficult micturating
157
Q

Management of LE syndrome?

A
  • Tx of underlying cancer
  • Immunosuppression: Prednisolone +/- Azathioprine
  • AMIFAMPRIDINE
  • Plasma exchange
158
Q

What is cataplexy?

A

The sudden and transient loss of muscular tone caused by strong emotion, e.g. laughter, being frightened

  • Around 2/3rds of patients with narcolepsy have cataplexy
  • Features range from buckling knees to collapse
159
Q

What is narcolepsy?

A
  • Associated with HLA-DR2
  • Associated with low levels of orexin (hypocretin) - a protein responsible for controlling appetite and sleep patterns
  • Early-onset of REM sleep
160
Q

Features of narcolepsy?

A
  • Typical onset in teenage years
  • Hypersomnolence
  • Cataplexy
  • Sleep paralysis
  • Vivid hallucinations on going to sleep or waking up
161
Q

Investigation for narcolepsy?

A
  • Multiple sleep latency EEG
162
Q

Management of narcolepsy?

A
  • Daytime stimulants (e.g. modafinil)

- Nighttime sodium oxybate

163
Q

Define multiple sclerosis (MS)?

A
  • Chronic and progressive
  • Inflammatory plaques of demyelination in the central nervous system
  • Disseminated in space and time
  • Occurring at multiple sites
164
Q

In whom and when is MS most common?

A
  • Young adults < 50 yrs
  • Peak = 30 yrs
  • More common in women
  • Symptoms tend to improve in pregnancy and the postpartum period
  • Much more common at higher latitudes
165
Q

Pathophysiology of MS?

A
  • T-cell mediated immune response
  • T-lymphocyte is primed -> gets through blood-brain barrier -> starts immune response cascade -> discrete plaques of demyelination at multiple CNS sites -> heals poorly -> remitting and relapsing symptoms
  • Demyelination: some recovery but THIN
  • Prolonged demyleination -> Axonal loss: these do NOT recover -> progressive symptoms/disability
166
Q

MS disease patterns?

A
  • Clinically isolated syndrome: first episode. May or may not go on to develop MS. If lesions are seen on MRI they are more likely to progress to MS
  • Relapsing-remitting: 85%. Acute episodes (1-2 months) followed by recovery periods.
  • Secondary-progressive: Started as relapsing-remitting but now deteriorated to have neuro symptoms between relapses. Around 65% of RR go on to develop SP within 15 yrs. May see gait/bladder problems.
  • Primary progressive: worsening of the disease and neuro symptoms without initial relapses and remissions. More common in older people.
167
Q

Causes of MS

A
  • Genetics + chance + environment
  • EBSTEIN BARR VIRUS (EBV)
  • Low vitamin D
  • Smoking
  • Obesity
168
Q

Presentation of MS

VISUAL

A

Visual:

  • optic neuritis (UNILAT)
  • optic atrophy
  • Internuclear ophthalmoplegia = conjugate lateral gaze disorder (6th cranial nerve): affected eye will not be able to ADDUCT when looking laterally
169
Q

Presentation of MS

SENSORY

A

Sensory:

  • Numbness, tingling in limbs
  • Trigeminal neuralgia
  • Pins and needles (paraesthesia)
  • Lhermitte’s sign: electric shock sensation down the spine when flexing the neck - indicates disease in the cervical spinal cord in dorsal column
170
Q

Presentation of MS

MOTOR

A
  • Spastic weakness, most commonly seen in legs
  • Bells Palsy
  • Horners syndrome
171
Q

Presentation of MS

CEREBELLAR

A
  • Ataxia: more often seen during acute relapse

- Tremor

172
Q

Presentation of MS

OTHERS

A
  • Urinary incontinence
  • Sexual dysfunction
  • intellectual deterioration
  • CHARCOTS TRIAD:
    o Nystagmus
    o Intention tremor
    o Dysarthria
173
Q

Diagnosis of MS

A
  • Clinically: 2 or more lesions of CNS disseminated in time and space
  • MRI: high signal T2 lesions; periventricular plaques; Dawson fingers - hyperintense lesions perpendicular to the corpus callosum
  • LP/CSF: OLIGOCLONAL BANDS of IgG
  • Visual evoked potential: delayed but well-preserved waveform
174
Q

Treating relapses of MS

A
  • High dose steroids, e.g. PO/IV methylprednisolone for 5 days
  • Shorten relapse but do not alter the degree of recovery
175
Q

Disease-modifying drugs in MS

A
  • BETA-INTERFERON
  • Monoclonal antibodies, e.g. NATALIZUMAB or Alemtuzumab
  • Immunosuppression: Azathioprine
176
Q

Symptom control in MS

A

Spasticity:

  • Baclofen or Gabapentin (or Diazepam)
  • Physiotherapy

Fatigue:
- Amantadine once other possible causes excluded

Incontinence:
- If not residual volume (do US) -> anticholinergic (e.g. oxybutinin)

Depression: SSRIs

Neuropathic pain:
- Gabapentin or Amytryptilline

Tremor:
- Botulinum Toxina

Exercise to maintain activity and strength

IF SEVERE –> bone marrow transplant

177
Q

What is cerebellar syndrome? Features?

A
  • Unilateral cerebellar lesions cause ipsilateral signs

DANISH:
D: Dysdiadokinesia, Dysmetria (past-pointing), ‘Drunk’ appearance
A: Ataxia (limb, truncal)
N: Nystagmus (horizontal = ipsilateral hemisphere)
I: Intention tremor
S: Slurred staccato speech
H: Hypotonia

178
Q

Causes of cerebellar syndrome?

A
  • Toxic (alcohol/lithium/phenytoin/lead poisoning)
  • Idiopathic
  • Neurodegenerative
  • MS
  • Hypothyroidism
  • Paraneoplastic (2ndry to lung or breast cancer) -

Friedrich’s ataxia:

  • Autosomal recessive
  • Presents early in childhood
  • Patient at increased risk of CVD disease

Spino-cerebellar ataxia 6 (SCA6)

  • Autosomal dominant
  • Episodic ataxia
  • Difficulty focusing and migraines
179
Q

Diagnosis of cerebellar syndrome?

A
  • MRI: cerebellar atrophy and excludes other causes
180
Q

Management of cerebellar syndrome?

A
  • Speech therapy

- Physiotherapy

181
Q

What is Wernicke’s encephalopathy (WE)

A
  • Neuropsychiatric illness caused by THIAMINE (Vit B1) deficiency
  • Mainly seen in ALCOHOLICS, but rarely in persistent vomiting, stomach cancer or dietary deficiency
  • Petechial haemorrhages occur in the brain
182
Q

Features of WE?

A

CLASSIC TRIAD - Confusion, Ataxia and Nystagmus (CAN)

  • Ophthalmoplegia
  • Peripheral sensory neuropathy
183
Q

Investigations of WE?

A
  • Decreased red cell transketolase

- MRI

184
Q

Treatment of WE?

A

Urgent replacement of THIAMINE

Medical emergency - high mortality rate if untreated!

185
Q

What is Korsakoffs syndrome? Management?

A
  • Complication of WE if it is left untreated
  • Addition of antero- and retrograde amnesia and confabulation in addition to symptoms of WE
  • Often irreversible and requires full-time institutional care
  • Can give thiamine supplementation and abstain from alcohol
186
Q

What is shingles?

A

An acute, unilateral, painful blistering rash caused by reactivation of the Varicella Zoster Virus (VZV)
- Occurs in ONE DERMATOME

187
Q

Management of shingles?

A
  • Oral aciclovir = first line

- Treatment reduces the incidence of post-herpetic neuralgia (more common in elderly)

188
Q

Describe the shingles vaccine

A
  • Offered to all patients aged 70 - 79 years
  • Is a live-attenuated vaccine given sub-cutaneously
  • CI = immune suppression
  • SEs = injection site reactions, chickenpox (1 in 10,000)
189
Q

What is Horner’s syndrome?

A
  • Damage to the sympathetic nervous system supplying the face
190
Q

Features of Horner’s syndrome?

A
  • Ptosis
  • Miosis (small pupil)
  • Anhidrosis (loss of sweating on one side)
    +/- Enopthalmos (sunken eye)
191
Q

Causes of Horner’s and how to distinguish?

A 
Central: 4S's
Stroke
Swelling (tumour/encephalitis)
multiple Sclerosis
Syringomyelia (cyst in the spinal cord)
Pre-ganglionic: 4T's
Trauma
Tumour (Pancoast's)
Thyroidectomy
Top rib (cervical rib above clavicle)
Post-ganglionic: 4C's
Carotid artery dissection
Carotid aneurysm
Cavernous sinus thrombosis
Cluster headache

Anhidrosis:
Central: face, trunk, arms
Pre-ganglionic: face
Post-ganglionic: NONE

Hereditary cause:
Heterochromia

192
Q

Diagnosis of Horner’s syndrome?

A
  • Cocaine eye drop WON’T cause any changes to the pupil (would normally)
  • Adrenaline eye drop will cause DILATION of the pupil (would NOT normally)
193
Q

What is Huntington’s?

A
  • Inherited neurodegenerative genetic condition
  • Autosomal dominant
  • Progressive and incurable
  • Degeneration of cholinergic and GABAnergic neurons in the striatum of basal ganglia
  • Due to a defect in the huntingtin (HTT) gene on chromosome 4
  • Presents in middle age
194
Q

What is genetic anticipation in Huntington’s?

A
  • A feature of TRINUCLEOTIDE REPEAT DISORDERS
  • Successive generations have more repeats in the gene, resulting in:
    o Earlier age of onset
    o Increased severity of the disease
195
Q

Presentation of Huntington’s?

A

Prodrome:

  • Irritability
  • Depression
  • Incoordination
  • Personality changes

Progresses to:

  • Chorea: abnormal involuntary movements
  • Athetosis: twisting and writhing
  • Abnormal eye movements: broken pursuit
  • Ataxia: problems with heel to toe walking
  • Some Parkinsonism

Within 15 yrs:

  • Dementia
  • Psychiatric problems - personality change, depression, psychosis
  • Fits
  • Death
196
Q

Management of Huntington’s?

A

No treatment that prevents progression

Supportive:

  • MDT involvement
  • SALT
  • Genetic counselling
  • Advanced life directives
  • End of life care planning

Symptom management:

  • Chorea: dopamine antagonists, e.g. TETRABENAZINE
  • Depression: SSRIs, e.g. SEROXATE
  • Psychosis: Olanzapine, Haloperidol
  • Aggression: Risperidone
  • Benzodiazepines, e.g. Diazepam
197
Q

Prognosis of Huntington’s?

A
  • Typically results in death after 20 yrs after initial symptoms
  • Suicide is a more common cause of death than for the general population
198
Q

What is epilepsy?

A
  • Umbrella term for conditions where there is a tendency to have unprovoked epileptic seizures
  • Seizure = paroxysmal event in which changes of behaviour, sensation or cognitive processes are caused by excessive, hypersynchronous neuronal discharges in the brain
199
Q

Which conditions have an association with epilepsy?

A
  • Cerebral palsy (30% have epilepsy)
  • Tuberous sclerosis
  • Mitochondrial diseases
200
Q

Characteristics of an epileptic seizure?

A
  • Duration: 30 - 120 seconds
  • Positive ictal symptoms: seeing/hearing/feeling things that are not there
  • Postictal symptoms
  • Stereotypical seizures - follow patterns
  • May occur from sleep
  • May be associated with other brain dysfunction
  • Lateral tongue bite
  • Deja vu
201
Q

Basic classification questions in epilepsy?

A
  1. Where the seizures begin in the brain
  2. Level of awareness during a seizure (important as can affect safety during seizures)
  3. Other features of the seizure
202
Q

Features of a generalised tonic-clonic (grand mal) seizure?

A

Involves networks on both sides of the brain at onset

  • LOC
  • Tonic: muscle tensing; Clonic: muscle jerking
  • May be associated with tongue biting, incontinence, groaning, irregular breathing
  • Post-ictal confusion, drowsiness or irritability
203
Q

Features of an absence (petit mal) seizure?

A
  • Brief pauses (<10 secs), e.g. stop talking mid-sentence
  • Presents in childhood
  • Unaware of surroundings and won’t respond
  • Last 10-20 seconds
  • EEG: 3Hz spike & wave oscillations
204
Q

Features of an atonic seizure?

A
  • Sudden loss of muscle tone, causing a fall
  • No loss of consciousness
  • Don’t usually last > 3 mins
  • May be indicative of Lennox-Gastaut syndrome
205
Q

Management of generalised tonic-clonic seizure?

A

1st line = Sodium Valproate (AVOID in pregnancy)

2nd line = Lamotrigine or Carbamazepine

206
Q

Features of a focal seizure/partial seizure?

A
  • Specific area on one side of the brain
  • Level of awareness can vary
  • Focal aware (simple partial), focal impaired awareness (complex partial) and awareness unknown can further describe focal seizures
  • Can also be classed as motor, non-motor or as having other features, e.g. aura
207
Q

Management of focal seizures?

A

1st line = CARBAMAZEPINE

2nd line = LAMOTRIGINE, sodium valproate, oxcarbamazepine, topimerate

208
Q

Management of absence seizures?

A
  • Most (>90%) will stop having these as they get older

- 1st line = Sodium Valproate or Ethosuximide

209
Q

Management of atonic seizures?

A
  • 1st line = Sodium Valproate

- 2nd line = Lamotrigine

210
Q

Feature of myoclonic seizures?

A
  • Sudden brief muscle contractions
  • Like a sudden jump
  • Patient usually remains awake during these episodes
  • Happen in children as part of Juvenile myoclonic epilepsy
211
Q

Management of myoclonic seizures?

A
  • 1st line = Sodium Valproate

- 2nd line = Lamotragine, Topiramate, Levetiracetam

212
Q

Temporal lobe seizure features?

A
  • Lip-smacking
  • Chewing
  • Fumbling
  • Grabbling
  • Abdomen rising sensation
  • Dysphasia
  • Hallucinations
  • Deja vu
  • Postictal confusion
  • Impaired awareness
213
Q

Frontal lobe seizure features?

A
  • Asymmetrical posturing
  • Pedalling legs
  • Post-ictal weakness
  • Jacksonian march
  • Do NOT lose consciousness
214
Q

Parietal lobe seizure features?

A
  • Paraesthesia
215
Q

Occipital lobe seizure features?

A
  • Floaters/flashers
216
Q

Which antiepileptics should not be used in pregnancy and why?

A
  • Sodium valproate - neural tube defects

- Phenytoin - cleft palate

217
Q

Briefly describe Infantile spasms (West’s syndrome)

A
  • Brief spasms starting in the first few months of life
  • More common in males
  • Usually secondary to a serious neurological condition
  • Poor prognosis
  • Vigabatrin/steroids
  1. Flexion of the head, trunk, limbs –> extension of arms
    Lasts 1-2 seconds, repeat up to 50 times
  2. Progressive mental handicap
  3. EEG: hypsarrthymia
218
Q

Briefly describe Lennoz-Gastaut syndrome

A
  • May be an extension of infantile spasms
  • Onset 1-5 years
  • Atypical absences, falls, jerks
  • Usually have a history of more than one seizure type. For example, both atonic & myoclonic seizures.
  • 90% moderate-severe mental handicap
  • EEG: 1.5-2.5Hz (SLOW) spike & wave pattern
  • Ketogenic diet may help
219
Q

Briefly describe Juvenile myoclonic epilepsy

A
  • Onset = teens
  • More common in females
  1. Infrequent generalised seizures, often in the morning
  2. Daytime absences
  3. Sudden, shock-like myoclonic seizures

Usually good response to sodium valproate

220
Q

What differences might you see in non-epileptic seizures to help differentiate them from epilepsy?

A
  • Last longer (10-20 mins)
  • Eyes and mouth closed
  • No cyanosis
  • May get crying/weeping/speaking
  • Recovery is quicker
  • Pelvic thrusting
  • History of psychiatric illness
  • Situational
221
Q

Diagnosis of epilepsy?

A
  • Detailed description of attack by eye witness
  • EEG
  • CT scan: to look for space-occupying lesions
222
Q

What is motor neurone disease?

A

An umbrella term encompassing neurological conditions of unknown cause that can present with both upper and lower motor neurone signs

  • A progressive, ultimately fatal condition
  • NO effect on SENSORY neurones
223
Q

Epidemiology of MND?

A
  • More common in MALES
  • Median age of onset = 60yrs
  • Prevalence = 6/100,000
224
Q

Pathophysiology of MND?

A
  • Genetic component - so good family history is important
  • There’s an increased risk associated with smoking, exposure to heavy metals and certain pesticides
  • Cluster of major degenerative diseases
  • Upper AND Lower MNs affected
  • Selective loss of neurons in the motor cortex, cranial nerve nuclei and anterior horn cells
  • Sensory nerves are spared
225
Q

How might you differentiate MND from other differentials?

A
  • No sensory loss or sphincter disturbances: differentiate from MS and polyneuropathies
  • No eye movement disturbances: differentiate from MG
  • Wasting of the small hand muscles/tibialis anterior is common in MND
  • No cerebellar signs in MND
  • No sensory signs in MND
  • Nerve conduction studies will show normal motor conduction and can help exclude a neuropathy
  • MRI can exclude the differential diagnoses of cervical cord compression and myelopathy
226
Q

What are the four clinical patterns of MND?

A
  • ALS (80%)
  • Progressive bulbar palsy (10-20%)
  • Progressive muscle atrophy (<10%)
  • Primary lateral sclerosis (rare)
227
Q

Features of ALS?

A
  • Loss of motor neurons in motor cortex AND anterior horns SO combined UMN and LMN signs
  • Typically LMN signs in arms and UMN signs in legs
  • Progressive spastic tetra-paresis
  • Worse prognosis if:
    Bulbar onset
    Increased age
    Low FVC
228
Q

Features of progressive bulbar palsy?

A
  • ONLY affects CRANIAL NERVES
  • LMN lesion of tongue and muscles of talking/swallowing
  • Flaccid, fasciculating tongue
  • Jaw jerk = normal/absent
  • Speech = quiet, hoarse or nasal
  • Dysarthria
  • Dysphagia
  • Carries the worst prognosis
229
Q

Features of progressive muscle atrophy?

A
  • Anterior horn cell lesions ONLY
  • So LMN signs ONLY
  • Affects distal muscle groups before proximal
  • Fasciculations, weakness, wasting
230
Q

Features of primary lateral sclerosis?

A
  • Loss of Betz cells in the motor cortex
  • Mainly UMN signs
  • Marked spastic leg weakness and pseudobulbar palsy
  • No cognitive decline
231
Q

Diagnosis of MND?

A

Mainly clinical

  • Nerve conduction studies: normal motor conduction (can help exclude a neuropathy)
  • Electromyography: reduced number of action potentials with increased amplitude
  • MRI: can exclude cervical cord compression and myelopathy
  • LP: may exclude inflammatory causes
232
Q

Management of MND?

A

Antiglutamatergics, e.g. RILUZOLE

  • Used mainly in ALS
  • Prolongs life by ~ 3 months

For resp failure:

  • Non-invasive ventilation (BIPAP) at night/home
  • Survival benefit of ~ 7 months

Death:

  • Usually in sleep due to hypercapnia
  • Give LORAZEPAM (prevent choking) and MORPHINE

Drooling: Amitryptiline
Dysphagia: Blend food/NG tube/PEG
Spasticity: Baclofen/Diazepam
Joint pain: Analgesic ladder

233
Q

What is hereditary spastic paraplegia?

A

UMN problems:

  • Abnormal gait
  • Bladder problems
  • High arched feet
  • Increased tone in legs and minimal muscle wasting
  • Family history
234
Q

Define cerebral palsy (CP)

A
  • Permanent neurological problems resulting from damage to the brain around birth
  • A disorder of movement and posture due to a non-progressive lesion of the motor pathways in the developing brain
  • NOT progressive, however, the nature of the symptoms and problems may change over time during growth and development
235
Q

Causes of cerebral palsy?

A

Antenatal (80%):

  • Maternal infections (TORCH)
  • Trauma during pregnancy

Perinatal (10%):

  • Birth asphyxia
  • Pre-term birth
  • Trauma

Postnatal (10%):

  • Intraventricular haemorrhage
  • Meningitis
  • Head trauma
  • Severe neonatal jaundice
236
Q

Types of cerebral palsy?

A
  • Spastic (70%): Hypertonia and reduced function from UMN damage.
  • Dyskinetic: problems controlling muscle tone (hypo- and hypertonia), athetoid movements and oro-motor problems. Basal ganglia damage.
  • Ataxic: problems with coordinated movement from cerebellar damage
  • Mixed: Mix of some or all of the above
237
Q

Patters of spastic CP?

A
  • Monoplegia: one limb affected
  • Hemiplegia: one side of the body affected
  • Diplegia: four limbs affected, but mostly legs
  • Quadriplegia: four limbs affected more severely, often with seizures, speech disturbance and other impairments
238
Q

Presentation of CP?

A
  • UPPER MOTOR NEURONE (differentials: acquired brain injury or a tumour)
  • HAND PREFERENCE BEFORE 18 MONTHS
  • Failure to meet milestones
  • Feeding or swallowing problems
  • Learning difficulties
  • Abnormal gait
  • Abnormal tone in early infancy
  • Leg will be extended with PLANTAR FLEXION
239
Q

Complications of CP?

A
  • Learning difficulties
  • Epilepsy (30%)
  • Squints (30%)
  • Hearing impairment (20%)
  • GORD
  • Kyphoscoliosis
  • Muscle contractures
240
Q

Management of CP?

A
  • MDT approach: PT/SALT/OT
  • Spasticity: Baclofen, Diazepam, Botulim toxin type A
  • Anticonvulsants and analgesia as required

MDT approach:

  • PT/OT/SALT
  • Dieticians
  • Ortho surgeons
  • Paediatricians
  • Charities and support groups
241
Q

Briefly discuss Benign Rolandic epilepsy

A
  • Also known as Childhood Epilepsy with Centrotemporal Spikes (CECTS)
  • Centrotemporal spike formations
  • Daytime symptoms: tingling, numbness and twitching (often of the face)
  • Night symptoms (more common and noticeable) are often full body convulsions
242
Q

How would a third nerve palsy present?

A
  • Diplopia
  • Ipsilateral fixed pupil
  • Ptosis
  • Inability to adduct or supraduct the eye
243
Q

When might an extended period of time for treating a stroke with a thrombectomy be considered?

A

For thrombectomy in acute ischaemic stroke, an extended target time of 6-24 hours may be considered if there is the potential to salvage brain tissue, as shown by imaging such as CT perfusion or diffusion-weighted MRI sequences showing limited infarct core volume

NICE recommends thrombectomy should be considered as soon as possible for people who fulfil the following criteria:

  • Last known to be well up to 24 hours previously (including wake-up strokes)
  • Who have acute ischaemic stroke and confirmed occlusion of the proximal posterior circulation (that is, basilar or posterior cerebral artery) demonstrated by CTA or MRA
  • There is the potential to salvage brain tissue, as shown by imaging such as CT perfusion or diffusion-weighted MRI sequences showing limited infarct core volume
244
Q

What is peripheral neuropathy?

A
  • CHRONIC and SLOWLY progressive
  • Starts in the LEGS and lower limbs
  • SENSORY or MOTOR or BOTH
  • Symmetrical!
245
Q

Predominantly motor loss types of peripheral neuropathy?

A
  • GBS
  • Porphyria
  • Lead poisoning
  • Hereditary sensorimotor neuropathies (HSMN): Charcot-Marie-Tooth
  • Chronic inflammatory demyelinating polyneuropathy (CIDP)
  • Diptheria
246
Q

Predominantly sensory loss types of peripheral neuropathy?

A
  • Diabetes
  • Uraemia
  • Leprosy
  • Alcoholism
  • Vit B12 deficiency
  • Amyloidosis
247
Q

What is alcoholic neuropathy?

A
  • Secondary to both direct toxic effects and reduced absorption of B vitamins
  • Sensory symptoms typically present prior to motor symptoms
248
Q

What is Vit B12 deficiency neuropathy?

A
  • Subacute combined degeneration of the spinal cord

- Dorsal column usually affected first (joint position, vibration) prior to distal paraesthesia

249
Q

What is Charcot-Marie-Tooth disease?

Management?

A
  • The most common hereditary peripheral neuropathy
  • Predomaninatrly motor loss
  • No cure so management = PT/OT/podiatrists/ortho surgeons

Features:

  • History of a frequently sprained ankle
  • Foot drop (high-stepping gait)
  • High-arched feet (pes cavus)
  • Hammer toes
  • Distal muscle weakness
  • Distal muscle atrophy
  • Hyporeflexia
  • Stork leg deformity
250
Q

Briefly describe porphyria

A
  • Photosensitive rash with blistering on face and dorsal of hands
  • Hyperpigmentation
  • Hypertrichosis
  • Treat with chloroquine
251
Q

Briefly describe lead poisoning

A

Features:

  • Abdominal pain
  • Peripheral neuropathy (mainly motor)
  • Fatigue
  • Constipation
  • Blue lines on gum margin (only 20% of adult patients, very rare in children)

The blood lead level is usually used for diagnosis: > 10 mcg/dl

Management:

  • Dimercaptosuccinic acid (DMSA)
  • D-penicillamine
  • EDTA
  • Dimercaprol
252
Q

Describe carpal tunnel syndrome

A

Caused by compression of median nerve in carpal tunnel

  • Pain/pins and needles in THUMB, INDEX FINGER, MIDDLE FINGER
  • Wake and shake - patient shakes hand to obtain relief, classically at night
253
Q

Examination of carpal tunnel syndrome

A
  • Weakness of thumb abduction (abductor pollicis brevis)
  • Wasting of thenar eminence (NOT hypothenar)
  • Tinel’s sign: tapping causes paraesthesia
  • Phalen’s sign: flexion of wrist causes symptoms
254
Q

Causes of carpal tunnel syndrome

A
  • Idiopathic
  • Pregnancy
  • Oedema (HF)
  • Lunate fracture
  • RA
255
Q

Treatment of carpal tunnel syndrome

A
  • Corticosteroid injection
  • Wrist splints at night
  • Surgical decompression (flexor retinaculum division)
256
Q

Presentation of ulnar nerve damage

A

Damage at wrist:

  • ‘Claw hand’
  • Wasting and paralysis of intrinsic hand muscles (except lateral two lumbricals)
  • Wasting and paralysis of hypothenar muscles
  • Sensory loss to the medial 1 1/2 fingers (palmar and dorsal aspects)

Damage at the elbow:

  • Same as above
  • Clawing is more severe in distal lesions
  • Radial deviation of the wrist
257
Q

Presentation of radial nerve damage

A
  • Wrist drop
  • Sensory loss to small area between the dorsal aspect of 1st and 2nd metacarpals

Axillary damage:

  • As above
  • Paralysis of triceps
258
Q

Presentation of common peroneal nerve damage

A

–> FOOT DROP

259
Q

What is neurofibromatosis?

A
  • Genetic condition
  • Autosomal dominant
  • Tumours of nerves (neuromas)
  • Benign but cause neurological symptoms
  • NF1 and NF2
260
Q

Briefly describe NG1 gene neurofibromatosis?

A
  • Also known as Recklinghausen’s syndrome

- CHROMOSOME 17 - codes for NEUROFIBROMIN

261
Q

Presentation of NG1 gene neurofibromatosis?

A

CRABBING. For diagnosis, need 2:

  • C: Cafe-au-lait spots (at least 6, >5mm in children, >15mm in adults
  • R: Relative with NF1
  • A: Axillary and inguinal freckles
  • BB: Bone dysmorphia, e.g. Bowing of long bones or sphenoid wing dysplasia
  • I: Iris hamartoma/Lisch nodules (at least 2)
  • N: Neurofibromas (at least 2) or one plexiform neurofibroma
  • G: Gliomas of the optic nerve
262
Q

Investigations into NG1 gene neurofibromatosis?

A

CLINICAL DIAGNOSIS so do not need investigations but some could be helpful:

  • CT/MRI for lesions in the brain/spinal cord/anywhere
  • Gene testing if unsure
  • XR for bone lesions/pain
263
Q

Management of NG1 gene neurofibromatosis?

A
  • No treatment for the underlying condition or to prevent complications
  • Management = control symptoms, treat complications, monitor the disease
264
Q

Complications of NG1 gene neurofibromatosis?

A
  • Renal artery stenosis (leads to HTN)
  • Malignant peripheral nerve sheath tumours
  • GI stromal tumour (type of sarcoma)
  • Migraines
  • Epilepsy
  • Learning difficulties/behavioural problems
  • Scoliosis
  • Vision loss (2ndry to gliomas of optic nerve)
  • Brain tumours
  • Spinal cord tumours with associated neurology
  • Increased risk of cancers. e.g. breast
  • Leukaemia
265
Q

Briefly discuss aetiology, presentation and management of NG2 gene neurofibromatosis?

A
  • Found on CHROMOSOME 22 which codes for MERLin
  • Important in SCHWANN CELLS
  • Present with BILATERAL ACOUSTIC NEUROMAS/SCHWANNOMAS
  • May, therefore, have hearing loss, tinnitus or balance problems
  • Also: schwannomas in brain, meningiomas, ependymomas
  • SURGERY can be used to resect tumours but carries a risk of permanent nerve damage
266
Q

What is tuberous sclerosis?

A
  • A GENETIC condition
  • AUTOSOMAL DOMINANT inheritance
  • Characesterist feature = HAMARTOMAS (abnormal growth and size of cells
267
Q

Where does tuberous sclerosis most commonly affect?

A
  • Brain
  • Kidneys
  • Eyes
  • Lungs
  • Skin
  • Heart
268
Q

Types of tuberous sclerosis?

A
  • TSC1 on CHROMOSOME 9, encodes for HAMARTIN

- TSC2 on CHROMOSOME 16, encodes for TUBERIN

269
Q

Skin signs in tuberous sclerosis?

A
  • Ash leaf spots
  • Shagreen patches
  • Angiofibromas
  • Subungual fibromata
  • Cafe-au-lait spots
  • Poliosis
270
Q

Neuro signs in tuberous sclerosis?

A
  • Developmental delay
  • Epilepsy
  • Intellectual impairment
271
Q

Other signs in tuberous sclerosis?

A
  • Retinal hamartomas
  • Rhabdomyomas of the heart
  • Gliomas
  • Polycystic kidneys
  • Lymphangioleiomyomatosis
272
Q

What is the classical presentation of tuberous sclerosis?

A
  • A CHILD presenting with EPILEPSY with the characteristic SKIN FEATURES of TS
  • Can also present in adults
273
Q

Causes of brain abscess?

A
  • Extension of sepsis from middle ear or sinuses
  • Trauma or surgery to the scalp
  • Penetrating head injuries
  • Embolic events from infective endocarditis
274
Q

Features of a brain abscess?

A
  • Depends upon the site if the abscess
  • Those in critical areas, e.g. motor cortex will present earlier
  • Raised ICP is common
  • Fever, headache and focal neurology
  • Fever may be absent and isn’t like the pyrexia seen in other abscesses
275
Q

Investigation of a brain abscess?

A
  • CT
276
Q

Management of a brain abscess?

A
  • Surgery: craniotomy with debribement of abscess
  • Antibiotics: IV 3rd-gen CEPHALOSPORIN and METRONIDAZOLE
  • ICP management, e.g. Dexamethasone
277
Q

Briefly describe neoplastic spinal cord compression

A
  • Oncological emergency and affects up to 5% of cancer patients
  • Extradural compression accounts for the majority of cases, usually due to vertebral body metastases.
  • It is more common in patients with lung, breast and prostate cancer
278
Q

Features of neoplastic spinal cord compression

A
  • Back pain - the earliest and most common symptom; may be worse on lying down and coughing
  • Lower limb weakness
  • Sensory changes: sensory loss and numbness

Neurological signs depend on the level of the lesion:

  • Lesions above L1 usually result in upper motor neuron signs in the legs and a sensory level.
  • Lesions below L1 usually cause lower motor neuron signs in the legs and perianal numbness.
  • Tendon reflexes tend to be increased below the level of the lesion and absent at the level of the lesion
279
Q

Investigation of neoplastic spinal cord compression

A

URGENT MRI: whole spine MRI within 24hrs of presentation

280
Q

Management of neoplastic spinal cord compression

A
  • High-dose oral dexamethasone

- Urgent oncological assessment for consideration of radiotherapy or surgery

281
Q

Features of myopathies?

A
  • Symmetrical muscle weakness (Proximal > distal)
  • Common problems rising from chair/getting out of bath
  • Sensation = normal, reflexes = normal
  • No fascisulations
282
Q

Causes of myopathies?

A
  • Inflammatory: poliomyelitis
  • Inherited: Duchenne’s/Becker muscular dystrophy, myotonic dystrophy
  • Endocrine: Cushing’s (Steroids), thyrotoxicosis
  • Alcohol
283
Q

Duchenne vs Becker muscular dystrophy

A

Duchenne muscular dystrophy

  • Progressive proximal muscle weakness from 5 years
  • Calf pseudohypertrophy
  • Gower’s sign: child uses arms to stand up from a squatted position
  • 30% of patients have an intellectual impairment
  • Associated with dilated cardiomyopathy

Becker muscular dystrophy

  • Develops after the age of 10 years
  • Intellectual impairment much less common
284
Q

Define brain tumours

A

Abnormal growths within the brain

Many different types from benign to highly malignant

285
Q

What are the three cardinal symptoms of a brain tumour?

A
  1. Symptom of a raised ICP
    - Headache, reduced GCS, nausea and vomiting
    - Papilloedema
  2. Progressive neurological defects
    - Depending on the location of the tumour
  3. Epilepsy
    - Focal seizures
    - Sinister in adults when new-onset
286
Q

What does papilloedema look like on fundoscopy?

A
  • Loss of crisp white optic nerve head margins
  • Venous engorgement
  • Retinal oedema
  • Retinal haemorrhages
287
Q

Diagnosis of brain tumours?

A
  • CT/MRI
  • Consider biopsy
  • PET-scan: good for primary cause
  • DO NOT LP –> RISK OF CONING
288
Q

What is a secondary brain tumour?

A
  • Metastases
  • Spherical and multiple lesions
  • Most commonly from: LUNG, breast, bowel, skin, prostate, kidney
289
Q

What kind of headache may follow a LP and how would you manage this?

A
  • Low pressure headache
  • Manage with fluids and caffeine
  • If does not improve: blood patch
290
Q

What is idiopathic intracranial hypertension (IIH)?

A
  • Idiopathic ICP

- Classically seen in young, overweight females

291
Q

What are the risk factors for IIH?

A
  • Obesity
  • Female
  • Pregnancy
  • Drugs: OCP, Steroids, tetracycline, Vit A, Lithium
292
Q

What are the features of IIH?

A
  • Headache
  • Blurred vision
  • Papilloedema
  • Enlarged blind spot
  • Sixth nerve palsy may be present
293
Q

How would you manage IIH?

A
  • Weight loss
  • Diuretics, e.g. ACETAZOLAMIDE
  • TOPIRAMATE
  • Repeated LP
  • Surgery: optic nerve sheath decompression and fenestration may be needed to prevent damage to the optic nerve
  • A lumboperitoneal or ventriculoperitoneal shunt may also be performed
294
Q

What is multiple system atrophy?

A
  • Multiple system atrophy (MSA) is a progressive neurodegenerative disorder characterized by a combination of symptoms that affect both the autonomic nervous system and movement.
    Two types:
  • MSA-P: predominantly Parkinsonian features
  • MSA-C: predominantly Cerebellar features
295
Q

What are the features of Multiple system atrophy?

A
  • Parkinsonism
  • Autonomic disturbance:
    o Erectile dysfunction
    o Postural hypotension
    o Atonic bladder
  • Cerebellar signs
296
Q

How to differentiate multiple system atrophy from Parkinson’s?

A

Key features to help you differentiate are the presence of UNILATERAL symptoms, and more severe/early onset AUTONOMIC dysfunction (postural hypotension/erectile dysfunction).

Drug-induced Parkinsonism can also cause unilateral symptoms so this would present with history of drug use.

Phase 3A Medicine (17 decks)

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