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Neuro & Special Senses > Neurology Conditions > Flashcards

Neurology Conditions Flashcards

1
Q

What is epilepsy?

A

Occasional sudden excessive rapid and local discharges of grey matter

Abnormal electrical activity

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2
Q

What are convulsions?

A

Motor signs of electrical discharges

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3
Q

What causes epilepsy?

A

2/3 idiopathic

Cortical scarring
Developmental causes
Space occupying lesions
Stroke
Hippocampal sclerosis
Vascular malformations
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4
Q

What can patients experience before an epileptic event?

A

Prodrome lasting days/hours - change in mood or behaviour

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5
Q

What is an aura indicative of?

A

Focal seizure in the temporal lobe

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6
Q

What can aura involve?

A

Déjà vu
Strange feeling in gut
Strange smell
Flashing lights

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7
Q

What is the post-ictal phase?

A

Altered state of consciousness after an epileptic seizure

Typically 5-30 mins

Headache
Myalgia
Confusion
Temporary weakness - motor cortex
Dysphagia - temporal seizures
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8
Q

What is important about an epileptic history? (long answer)

A 
GET ONE FROM A WITNESS
Rule out other causes - pseudoseizure
Family history
Previous head injury
Birth problems
Ask what happened before, during and after the episode
Before
Illness?
Medications?
Triggers
During
Headaches - migraines can manifest in similar ways
Loss of consciousness?
Lose control of bladder/bowels?
Bite tongue/cheeks?
Could you talk, move etc?

After
Confused, headache, myalgia?

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9
Q

What investigations would you ask for if you suspect epilepsy?

A

EEG

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10
Q

What are types of seizures?

A

Focal

Generalised - Absence, Tonic-clonic, Myoclonic, Atonic, (Tonic, Clonic)

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11
Q

What is the difference between focal and generalized seizures?

A

Focal seizures only affect one hemisphere and are usually associated with structural disease

Generalised originate at some point but spread bilaterally and rapidly distribute. They have no localising features

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12
Q

How are focal seizures managed?

A

Carbamazepine -1st line

Lamotrigine

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13
Q

What is used to manage generalised seizures?

A

Sodium valproate

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14
Q

What seizures can carbamazepine exacerbate?

A

Myoclonic and Absence

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15
Q

What is an absence seizure?

A

Brief (usually <10s) event where subject stop talking mid sentence then carry on where they left off

Often seen in childhood

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16
Q

What happens in a tonic-clonic seizure?

A

Lose consciousness

Limbs stiffen - tonic
Then jerk - clonic

Often lose continence, have aura before and severe headache after

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17
Q

What happens in a myoclonic seizure?

A

Sudden jerk of limb, face or trunk - suddenly thrown to ground

Violently disobedient limb

No loss of consciousness and continue as normal after

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18
Q

What happens in an atonic seizure?

A

Sudden loss of muscle tone –> fall

No loss of consciousness

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19
Q

What symptoms are indicative of temporal lobe seizures?

A 
Complex motor phenomena
Impaired awareness
Oral movements - lip smacking, chewing, swallowing
Deja vu
Emotional disturbance
Sound, smell, taste hallucination
Delusional behaviour
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20
Q

What symptoms are indicative of frontal lobe seizures?

A 
Motor feature - posturing or peddling movement
Jacksonian march
Motor arrest
Subtle behavioural disturbance
Speech arrest
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21
Q

What is a jacksonian march?

A

Simple partial seizure spreads from distal part of the limb towards the ipsilateral face

Progression of location leads to march of motor presentation of symptoms

Tingling sensation in fingers, then moves up proximally

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22
Q

What symptoms are indicative of parietal lobe seizures?

A

Sensory disturbances
Tingling numbness
Pain
Motor symptoms

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23
Q

How do occipital lobe seizures present?

A

Visual phenomena - spots, lines and flashes

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24
Q

What things are important when giving management advice for epilepsy?

A

Pharmacological side effects

CBT can be recommended

Driving advice

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25
Q

What is the driving advice for a first unprovoked seizure?

A

Can’t drive for 6 months if no structural abnormality and no abnormality on EEG, if not 12 months off

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26
Q

What is the driving advice for patients with epilepsy?

A

Fit free for 12 months

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27
Q

What is the driving advice when withdrawing from anti-epileptic medication?

A

No driving until 6 months after last dose

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28
Q

Over what time period should anti-epileptic drugs be stopped?

A

Decreased slowly over 2-3 months

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29
Q

Over what time period should barbituates and benzodiazepines be stopped?

A

Decreased slowly over 6 months

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30
Q

What patients is it especially important to carefully consider management of epilepsy for?

A

Patients on other medication - CYP inducers/inhibitors

Women wishing to get pregnant

Women on contraception

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31
Q

Name some ADR’s associated with carbamazepine

A 
Leucopenia
Visual disturbance
Balance issues
SIADH
Erythematous rash

P450 Inducer

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32
Q

What is important to consider about dosage with Lamotrigine?

A

Dose changes depending on valproate and carbamazepine use alongside

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33
Q

What are some ADR’s associated with Lamotrigine?

A

Maculopapular Rash

Steven Johnson Syndrome - warn them to see a doctor if they have flu like symptoms

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34
Q

Name some ADR’s associated with Sodium Valproate

A 
Nausea
Teratogenic
Liver failure
Pancreatitis
Hair loss
Obesity
Ataxia
Tremor
Thrombocytopenia
Encephalopathy

P450 inhibitor

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35
Q

How does carbamazepine work?

A

Bind to sodium channels to increase refractory period

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36
Q

How does lamotrigine work?

A

Sodium channel blocker

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37
Q

How does sodium valproate work?

A

Enhance GABA receptors

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38
Q

How does phenytoin work?

A

Bind to sodium channels to increase refractory period

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39
Q

Give some ADR’s of Phenytoin

A 
Nystagmus
Diplopia
Tremor
Dysarthria
Dizzy/drowsy
Peripheral neuropathy
Gingival hyperplasia

CYP450 Inducer

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40
Q

What medication is used for pregnant women?

A

Lamotrigine

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41
Q

What is important to remember about the contraceptive pill and lamotrigine?

A

Lamotrigine reduce effectiveness of Contraceptive

Contraceptive reduce lamotrigine levels

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42
Q

What must women of child bearing age on Anti-epileptics take?

A

5mg Folate OD

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43
Q

How should patients having a fit be managed immediately?

A

Most resolve within 5 minutes.

If not resolving after 5-10 mins, administer benzodiazepine (4mg lorazepam)

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44
Q

What is the management of status epilepticus?

A

In hospital:
ABCDE

Secure airway
High conc oxygen
Assess cardiac and resp function
Check blood glucose levels
Gain IV access
IV lorazepam 4mg, repeat after 10 mins if continues

If persists - IV phenobarbital or phenytoin

In the community - buccal midazolam or rectal diazepam

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45
Q

What is status epilepticus?

A

Seizures lasting more than 5 minutes

Or more than 3 seizures in one hour

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46
Q

What is a focal seizure?

A

A partial seizure - affect initially only one hemisphere of the brain, or part of a lobe

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47
Q

What are the types of focal seizure?

A

Simple partial - only affect small region of brain, often temporal lobe or e.g. hippocampi
Remain conscious

Complex partial - unilateral cerebral hemisphere involvement, causes impairment of awareness or responsiveness i.e. altered consciousness

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48
Q

What is a petit mal?

A

Absence epilepsy
Starts in childhood

EEG abnormalities - 3Hz generalised, symmetrical spike wave complexes

Treat with sodium valproate, ethosuximide or both

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49
Q

What should be included in a headache history?

A 
Mode of onset
Duration
Nature of headache
Site
Pattern and timing
Provoking and relieving factors
Associated symptoms including aura
Drug history
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50
Q

What drugs can cause medication overuse headaches?

A

Paracetamol, aspirin, NSAID use for 15 days per month or more
Triptans, opioids, ergot preparations for 10 days a month or more

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51
Q

What are the red flags for a headache?

A 
Thunderclap
Associated fever
Meningism signs
RICP
New neurological deficit
New cognitive dysfunction
Personality change
Impaired/deteriorating conscious level
Recent head injury
New onset headache in elderly - GCA
Significant change in pattern of chronic headache
History of malignancy or impaired immunity
Postural headaches - worse on lying, standing, post LP
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52
Q

What are the features of a headache due to raised ICP?

A

Present on waking, or wakes patient at night
Exacerbated by sneezing, straining, bending, lying down
Intracranial tumour - short history

Effortless vomiting

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53
Q

What are the features of idiopathic benign intracranial hypertension?

A

Common in young, obese women
Signs of RICP but no mass

Morning headache
Vomiting
Visual disturbances - diplopia, visual obscurations (sudden transient bilateral visual loss with changes in posture)

Bilateral papilloedema

CSF examination by LP confirms RICP

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54
Q

What is the management of idiopathic intracranial hypertension?

A

Self-limiting, resolves with weight loss and LPs

Chronic - carbonic anhydrase inhibitors e.g. acetazolamide, diuretics and corticosteroids

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55
Q

What are the signs of meningism?

A 
Severe global or occipital headache
Vomiting
Photophobia
Nuchal rigidity - stiff neck
Resistance to passive neck flexion
Kernig's sign - pain and resistance to passive knee extension with flexed hip
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56
Q

What are the clinical features of GCA?

A

Granulomatous inflammatory changes in branches of external carotid

Headache, can localise to temples
Scalp tenderness on combing hair
Intermittent claudication of the jaw - impairment of blood supply to muscles of mastication
Amaurosis fugax - transient loss of vision in one eye

Constitutional symptoms - low grade fever, night sweats, shoulder and/or pelvic girdle pain

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57
Q

What are the investigations for GCA?

A 
ESR grossly elevated
CRP high
Normochromic normocytic anaemia
Abnormal LFTs, raised ALP
Temporal artery biopsy - may be skip lesions
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58
Q

What is the management for GCA?

A

IV steroids once blood has been taken for ESR i.e. before biopsy

40-60mg pred per day

Aspirin 75mg daily decreases visual loss and strokes
PPI for gastric prevention whilst on steroids

Referral to rheum, vascular surgeons, ophthalmology

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59
Q

What are complications of GCA?

A

Relapse
Steroid SEs
Aortitis leading to aortic aneurysm and aortic dissection

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60
Q

What is a tension headache and treatment?

A

Mild ache across forehead in band like pattern

Due to stress, depression, alcohol, skipping meals, dehydration

Treatment - reassurance, basic analgesia, hot towels and relaxation techniques

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61
Q

What are secondary headaches?

A

Give similar presentation to tension headache, but clear cause

Underlying medical condition e.g. infection, OSA, pre-eclampsia
Alcohol
Head injury
Carbon monoxide poisoning

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62
Q

What is the diagnostic criteria for migraine?

A

At least 5 headaches that:

  • Last 4-72 hours
  • Are severe, unilateral, pulsating and interrupt daily activity
  • Are associated with N&V or photo/phonophobia
  • Are not due to a secondary cause
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63
Q

What are some migraine triggers?

A 
Oestrogen (COCP and menstruation)
Foods (cheese, red wine, citrus fruits)
Stress
Bright lights 
Alcohol
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64
Q

How are migraines managed?

a) Acute
b) prophylaxis + when is prophylaxis offered?
c) menstrual induced

A

ACUTE:

  1. oral triptan + NSAID or paracetamol
  2. prochlorperazine + nasal triptan

PROPHYLAXIS if >2/month:

  1. propranolol (preferred in women) or topiramate (teratogenic)
  2. acupuncture

MENSTRUAL MIGRAINE
frovatriptan

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65
Q

Describe the aura associated with some migraines

A
  • transient hemianopic disturbance

- spreading scintillating scotoma

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66
Q

Describe the signs and symptoms of a cluster headache and the timing/ frequency of attacks

A

Severe, sudden onset, unilateral pain around the eye

Ipsilateral autonomic features - rhinorrhoea, sweating, partial horner’s, lacrimation, lid swelling

Typically occur at night
1-2 hour bouts daily over 6-12 weeks

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67
Q

What can trigger cluster headaches?

A

Triggers - alcohol, histamine, heat, exercise

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68
Q

How are cluster headaches managed?

A

Acute - subcutaneous sumatriptan, 100% O2

Prophylaxis - verapamil (some evidence for prednisolone)

Surgery - trigeminal nerve blockade

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69
Q

What secondary causes of headaches would you consider?

A 
V: temporal arteritis 
I: meningitis, sinusitis, malaria, HIV
T: head injury, SAH
A: 
M: hypothyroid 
I:
N: brain metastasis, primary tumour 
D: medication induced, CO poisoning

Other: dental/ jaw

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70
Q

Explain the pathophys of idiopathic parkinsons disease

A
  • loss of dopaminergic neurones in substantia nigra
  • leads to increased activity of basal ganglia and so hyperkinesia
  • most are sporadic with many gene loci identified
  • mean onset is in 60s
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71
Q

What are the 4 cardinal features of parkinsons?

A
  • TREMOR: worse at rest, pinrolling
    At 4-6Hz, or can be induced by concentration, usually apparent in one limb or limbs on one side for months
  • HYPERTONIA: rigidity (lead pipe- not velocity dependent), when combined with tremour gives cogwheeling during rapid pronation/ supination
  • BRADYKINESIA: slow to initate movement, actions slow and decreased in amplitude with repitition, slow blink rate and micrographia
  • GAIT: shuffling, pitched forward, loss of arm swing, en bloc turning
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72
Q

Give 4 other (non cardinal) features of parkinsons

A
  • expressionless face
  • autonomic dysfunction (postural hypotension, constipation, urinary frequency, urgency, drooling)
  • sleep disturbance (REM sleep disorder)
  • reduced sense of smell
  • neurpsychiatric
    complications: dementia, depression, psychosis
  • signs are almost always worse on one side- if bilateral look for other causes
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73
Q

Describe the pharmacological management of parkinsons disease (7)

A
  • Levodopa: efficacy reduced over time so start it late as poss
  • dopa decarboxylase inhibitors (carbidopa/ co benledopa) will increase levo dopa’s efficacy
  • dopamine agonists like ropinerole can help delay starting levodopa in early PD
  • apomorphine: potent DA agonists used to even out end of dose effects or as rescue pen for dose effects
  • anticholinergics: young pts only as confuse old ppl
  • MAO- B inhibitors: alternative to DA’s in early PD
  • COMT inhibitors eg etacapone- help motor complications in late disease and lessen off time in end of dose wearing off
  • clonazepam and melatonin for REM sleep disorder
  • fludrocotisone if postural hypotension
  • quetiapine and clozapine for distressing hallucinations (worsens parkinsons)
  • Acetylcholinesterase inhibitor for dementia (donepezil and rivastigmine)
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74
Q

Describe the surgical management options for parkinsons disease

A
  • deep brain stimulation: may help but only if partially dopamine responsive still
  • surgical ablation of basal ganglia circuits eg subthalamic nucleus
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75
Q

What are the 4 parkinsons plus syndromes and their other features

A
  • Progressive supranuclear palsy: early postural instability, vertical gaze palsies +/- falls, rigidity of trunk> limbs, symmetrical onset, little tremor, speech and swallowing problems
  • Multiple systems atrophy: early autonomic features eg incontinence, postural hypotension + cerebellar + pyramidal signs, rigidity > tremor
  • Corticobasal degeneration: akinetic rigidity involving one limb, cortical sensory loss, apraxia

-Lewy body dementia
Early onset dementia <1 yr with features of parkinonism
Dementia usually proceeding feature prior to motor symptoms, with visual hallucinations and fluctuating consciousness

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76
Q

Give 5 secondary causes of parkinsonism

A
  • vascular parkinsonism: diabetic/ HTN pt with postural instability and falls
  • drugs: antipsychotics, metoclopramide
  • toxins: manganese
  • wilsons disease
  • trauma
  • encephalitis
  • neurosyphilis
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77
Q

What is the anatomy and physiology behind parkinson’s?

A

Basal ganglia - gray matter cell bodies - contains caudate nucleus, putamen, globus pallidus, substansia nigra and subthalamic nucleus

Basal ganglia helps kick start and fine tune movement initiated by the motor cortex

Inhibition of muscle tone
Coordinated slow sustained movement
Suppression of useless patterns of movement
Initiation of movement

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78
Q

What are the pathways within the substantia nigra?

A

Process of modulation dependent on direct and indirect pathways

Basal ganglia needed for the modulation of pyramidal motor output

Direct - mostly stimulatory pathway, shorter, mostly off, predominantly associated with D1 receptors
Dopamine released from SN via dopaminergic neurones to activate D1 receptors

Indirect - inhibitory pathway
Longer, mostly on, D2 receptors
Allows inhibition of muscular tone to prevent unnecessary movement

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79
Q

What is the UK Parkinson’s Disease Society Brain Bank Criteria?

A

Step 1: diagnosis of Parkinsonian syndrome
Bradykinesia + at least one:
muscular rigidity, 4-6Hz resting tremor, postural instability

Step 2: exclusion criteria for PD - history of repeated strokes, history of repeated head injury, definite encephalitis, sustained remission, cerebellar signs, dementia, Babinski’s, hydrocephalus, negative response to LDOPA

Step 3 - supportive prospective positive criteria of Parkinson’s
Three or more for definite:
Unilateral onset, rest tremor present, progressive disorder, persistent asymmetry, excellent response to LDOPA, hyposmia, visual hallucinations

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80
Q

What are the features of Parkinson’s disease dementia?

A

Occurs more than one year after diagnosis
Similar to Alzheimer’s

Presence of Parkinsonism in the limbs
Frequent visual hallucinations
Frequent fluctuations in lucidity

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81
Q

What are the differentials for Parkinson’s?

A 
Benign essential tremor
Drug or toxin induced
Huntington's
Wilson's
Corticobasal degeneraiton
Creutzfeldt Jakob disease
Multi-infarct dementia
Lewy body dementia
Pick's disease
Cerebellar tumour
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82
Q

What are the investigations for Parkinson’s?

A

CT or MRI if fail to respond to therapeutic (600mg/day) of L-DOPA

MRI to rule out rare secondary causes

PET with flurodopa to localise dopamine deficiency

Genetic testing - Huntington’s

Olfactory testing

Measurement of ceruloplasmin levels - Wilson’s, or syphilis serology

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83
Q

What is cogwheel rigidity?

A

Resistance to passive movement of a joint

Take their hand, passively flex and extend arm at the elbow, creates a tension in their arm that gives way to movement in small increments - little jerks

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84
Q

How can a Parkinson’s tremor be differentiated from a benign essential tremor?

A

Parkinson’s is asymmetrical, worse at rest, improves with intentional movement, no change with alcohol

Benign essential tremor is symmetrical, improves at rest, worse with intentional movement, improves with alcohol

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85
Q

What is levodopa?

A

Synthetic dopamine
Given orally to boost own dopamine levels

Combined with other drug that stops levodopa being broken down in the body before it gets to the brain

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86
Q

What are the main side effects of dopamine treatment?

A

Develops dyskinesias - abnormal movements associated with excessive motor activity

Dystonia - excessive muscle contraction leads to abnormal postures or exaggerated movements

Chorea - abnormal involuntary movements that can be jerking and random

Athetosis - involuntary twisting or writhing movements usually in the fingers, hands or feet

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87
Q

What is the action of COMT inhibitors?

A

Inhibitors of catechol-o-methyltransferase

COMT enzyme metabolises levodopa in the body and brain

e.g. entacapone taken with levodopa and a decarboxylase inhibitor to slow breakdown of levodopa in the brain

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88
Q

What is a notable side effect of dopamine agonists?

A

Pulmonary fibrosis

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89
Q

What are dopamine agonists used in Parkinson’s?

A

Mimic dopamine in the basal ganglia
Stimulate dopamine receptors

Less effective than levodopa
Used in combination with levodopa to reduce the dose of levodopa required

Bromocryptine
Pergolide
Carbergoline

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90
Q

What are monoamine oxidase B inhibitors used in Parkinson’s?

A

Monoamine oxidase B enzyme - specific to breakdown of dopamine

So these medicatoins bllock this enzyme, increasing circulating dopamine

Usually used to delay use of levodopa

Examples - selegiline, rasagiline

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91
Q

How can you clinically distinguish MND from

other polyneuropathies?

A
  • Both may have mixed UMN and LMN signs
  • There is NO sensory loss or sphincter disturbance in MND
  • It also NEVER effects eye movements- helps distinguish from MG
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92
Q

Describe the 2 commonest clinical patterns of MND, state the other two.

A
  • amylotrophic lateral sclerosis (ALS): Loss of motor neurones in motor cortex AND anterior horn of cord (so UMN +LMN signs). Definite if in 3 regions, probably if in 2.
  • Progressive bulbar palsy: bulbar palsy (LMD) , often mixed with pseudobulbar palsy (UMN)
    Affects primarily the muscles of talking and swallowing
  • progressive muscular atrophy: only affects anterior horn cells so LMN signs only, distal before proximal muscle groups affected
  • primary lateral sclerosis: rare, loss of betz cells in motor cortex, mainly UMN
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93
Q

How does MND tend to present and describe its clinical features

A
  • age >40
  • stumbling spastic gain, foot drop +/- proximal myopathy, weak grip, poor shoulder strength or aspiration pneumonia are usually 1st presentations
  • UMN signs AND LMN signs (tongue fasiculation common)
  • frontotemporal dementia occurs in 25%
  • no sensory disturbance, eyes movements not effected

Wasting usually begins at hands then spreads, may initially be unilateral
Fasciculations, cramps
Usually NO PAIN

Bulbar and pseudobulbar features - progressive
Dysphagia, dysarthria, nasal regurg, choking
Swallowing solids difficult

Reflex problems, lost or hyperreflexic (due to loss of corticospinal neurons)

Sphincters not affected

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94
Q

How should suspected MND be investigated?

A
  • no diagnostic test
  • MRI brain/ cord to exclude structural cause
  • LP excludes inflammatory causes
  • neurophysiology studies can detect subclinical denervation and exclude mimicking motor neuropathies
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95
Q

Describe the management of MND

A
  • Riluzole: inhibits glutamate release and NMDA receptor antagonist, is the only drug proven to improve survival but only by 2-4 months but has significant impact on tracheostomy free survival
  • Positioning, oral care and anti muscarinic (glycopyrronium) can help with the excess saliva
  • Blended and thickened foods as well as gastrostomy can help with dysphagia
  • Exercise, orthotics can help with spasticity
  • Augmentative and alternative communication equipment can aid comms difficulty
  • Involve palliative care from point of diagnosis as median survival from diagnosis is only 2-4 yrs
  • Positive pressure ventilation can aid with breathing greatly
  • Muscle cramps- quinine, baclofen, gabapentin, diazepam
  • communication aids
  • Early discussion about end of life care are esp important with ALS as many will develop frontotemportal dementia and not be able to express wishes later on in the disease
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96
Q

What is the pathophysiology of MND?

A

Progressive degeneration of both upper and lower motor neurones

The sensory neurones are spared

Genetic component
Take a good family history as 5-10% inherited

Oxidative neuronal damage
Aggregation of abnormally large amounts of protein inside a cell
Glutamate problems
Apoptosis
Prolonged caspase activity - contributing to the apoptosis

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97
Q

What are some of the known risk factors of MND?

A

Smoking
Exposure to heavy metals
Certain pesticides

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98
Q

What is the difference between bulbar and pseudobulbar palsies?

A

Bulbar palsy - LMN palsy that affects CNs 9, 10, 11 and 12

Pseudobulbar palsy - UMN palsy affecting the corticobulbar tracts of 5, 7, 9, 10, 11, 12

Any condition which disrupts or damages the cranial nerve nuclei or corticobulbar tracts can cause this - stroke, MS, infections, tumours

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99
Q

What are features seen in ALS?

A

Amyotrophic lateral sclerosis
Lateral sclerosis refers to damage to the lateral corticospinal tracts

Leads to spastic parapesis

Amyotrophic - loss of muscle tone

May be pyramidal weakness

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100
Q

What are differentials for MND?

A

Motor neuropathy - will cause distal weakness and LMN signs
There will be conduction block
Spinal muscular atrophy
Kennedy’s syndrome
Hyperthyroidism and hyperparathyroidism can both result in muscle wasting and hyperreflexia
Pseudobulbar palsy
Cervical spondylitis and spinal tumours both exhibit both UMN and LMN signs

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101
Q

What clinical features is it important to remember ARE NOT affected in MND?

A

Bladder never affected
No sensory signs
Occular muscles never affected

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102
Q

What is the action of Riluzole?

A

Sodium channel blocker
Can slow progression very slightly, especially if bulbar features

Thought that by blocking sodium channels, effect of glutamate greatly reduced

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103
Q

What is Baclofen?

A

GABA agonist

Helps reduce spasticity

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104
Q

What can amitriptyline help with in MND?

A

Drooling

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105
Q

What is the pathophysiology of multiple sclerosis?

A

Cell mediated autoimmune demyelination of the white matter CNS. This leads to reduced conduction velocity
Disseminated (over time and space) - lesions vary in their location over time, meaning different nerves affected, symptoms change over time

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106
Q

Where does MS typically only affect?

A

The central nervous system (the oligodendrocytes)

Inflammation around myelin and infiltration of immune cells

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107
Q

What are believed to be some causes of MS?

A 
Multiple genes
EBV
Low Vitamin D
Smoking
Obesity
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108
Q

What are some poor prognostic signs for MS?

A

Older patient
Motor signs at onset
Many MRI lesions

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109
Q

What are common first symptoms of MS?

A

Optic Neuritis - pain on eye movement with greying and blurring of vision
Odd sensations such as wetness or burning

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110
Q

What pathway does ALS commonly involve?

A

Corticospinal path - motor pathway

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111
Q

What sensory symptoms are seen in MS?

A

Dysaesthesia (abnormal unpleasant feeling when touched)
Pins and needles
Decreased vibration sense
Trigeminal neuralgia
Lhermitte’s syndrome - electric shock sensation that travels down the spine and into the limbs when flexing the neck, indicates disease in cervical spinal cord in DC

Up to 75% report bladder dysfunction, may occur leading to incontinence, poor bladder emptying or urinary retention

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112
Q

What motor symptoms are seen in MS?

A

Spastic weakness
Myelitis
Hyper-reflexia

Transverse myelitis - local inflammation within the spine, leads to sensory and motor symptoms below the level of the lesion

Cerebellar syndrome - disease of the cerebellum causes ataxia, slurred speech, intension tremor, nystagmus, vertigo, clumsiness

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113
Q

What GU symptoms are seen in MS?

A

Erectile dysfunction
Anorgasmia
Urinary frequency
Incontinence

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114
Q

What GI symptoms are seen in MS?

A

Swallowing difficulty

Constipation/diarrhoea

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115
Q

What eye symptoms are seen in MS?

A 
Diplopia
Optic neuritis
Nystagmus
Opthalmoplegia
Uhthoff's Phenomenon - transient worsening of neurological symptoms in increase in temp
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116
Q

What cerebellar signs are seen in MS?

A

Trunk/limb ataxia
Intention tremor
Scanning speech (ataxic dysarthria)

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117
Q

What cognitive/visiospatial signs are seen in MS?

A

Amnesia
Executive dysfunction - emotional and behavioural difficulty
Depression/mood disorders
cognitive impairment

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118
Q

What are some subtypes of MS?

A

Relapsing-remitting (most common)
Secondary progressive disease
Primary progressive disease

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119
Q

What is relapsing-remitting MS like?

A

Acute 1-2 month attacks followed by periods of remission

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120
Q

What is secondary progressive disease in MS?

A

R-R patients don’t fully remit and over time the disability accumulates (around 65% of R-R patients progress within 15 years)

See gait and bladder problems

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121
Q

What is primary progressive disease in MS?

A

Progressive deterioration seen from the onset

122
Q

What lifestyle advice is given for patients with MS?

A

Regular exercise
Stop smoking
Avoid stressful situations

123
Q

How are acute relapses of MS managed?

A

High dose IV methylprednisolone

124
Q

What criteria have to be met for beta interferon to be used in MS?

A

Not suitable for primary progressive.

Have to have had 2 relapses in past 2 years.

125
Q

What is the effect of beta interferon on MS?

A

Reduce number of relapses and MRI changes

No effect on disability

126
Q

What disease modifying drugs are used in MS?

A 
Interferon-B
Glatiramer acetate
Dimethyl fumerate
Alemtuzumab
Natalizumab
Fingolimod
127
Q

How is spasticity in MS controlled?

A

Baclofen and gabapentin

Physio

128
Q

How is tremor in MS controlled?

A

Botulinum toxin

Primidone

129
Q

How is bladder dysfunction managed in MS?

A

If significant residual volume - self catheterise

Frequency symptoms - Anticholinergics

130
Q

How is oscillopsia (visual fields oscillating) in MS. managed?

A

Gabapentin

131
Q

How is fatigue in MS managed?

A

Amantadine and CBT

132
Q

How is MS diagnosed? What are the results of these investigations?

A

MRI contrast: demyelination plaques (hyperintense plaques in the periventricular region)
Electrophoresis of CSF: oligoclonal bands of IgG
Evoked potentials: prolonged conduction

133
Q

What are some differentials for MS?

A

Demyelinating diseases:
neuromyelitis optica, tranverse myelitis

Infections:
Lyme’s, tertiary syphillis

Metabolic diseases:
B12 deficiency, DM, copper deficiency

SLE, sarcoidosis

Neoplasia, vasculitis, stroke

134
Q

Optic neuritis and intranuclear opthalmoplegia are common in MS and often how it presents initially. Describe these two conditions

A
  • Optic neuritis: pain on eye movement, reduced central vision, red desensitisation- esp centrally, usually unilateral, sometimes swollen disc on fundoscopy acutely and after the disc appears white (scarred), RAPD
  • Intranuclear ophthalmoplegia (MLF syndrome)- when one eye looks laterally the other doesn’t turn medially or lags, often with nystagmus of the laterally moved eye, due to demyelination of medial longitudinal fasciculus connecting the IV and VI cranial nerves in the brain stem, can be bilateral
135
Q

Other than intranuclear opthalmoplegia and optic neuritis, give 7 other deficits commonly caused by MS and state when the symptoms tend to get worse

A
  • worsen with heat (hot weather, shower, bath)
  • SENSORY: dysaesthsia, pins n needles, decreased vibration sense, trigeminal neuralgia
  • MOTOR: spastic weakness, myelitis, CN palsies-> diplopia
  • SEXUAL/ GU: ED, incontinence, urinary retention
  • EYE: diplopia, hemianopia, pupil defects, visual phenomena on exercise
  • cerebellum signs
  • cognitive: visual spacial neglect, accidnet, low mood, poor executive functioning
136
Q

How is MS diagnosed?

A
  • clinical- McDonald criteria can be used
  • MRI sensitive but not specific for detecting lesions and can help exclude other causes
  • oligoclonal bands of IgG on electrophoresis of CSF can help indicate MS
  • Visual evoked response - measuring electrical activity via a transducer over the occipital cortex in response to a light stimulus
137
Q

How should relapsing remitting and primary and secondary progressive MS be managed?

A
  • Regular exercise, stop smoking and avoid stress may help
  • Legal obligation to inform DVLA
  • Dimethyl fumarate then interferon B for mild- moderate RR MS
  • Natalizumab if more active RR MS
  • Interferon B for primary and secondary progressive MS
  • Cannaboids effective in some
  • Metylprednisolone IV for 3-5 days shortens acute relapses but use sparingly (only for attacks lasting >24 hrs and moderate- severe), doesn’t alter overall progress
  • Baclofen/ gabapentin/ benzos for spasticity
  • Botulinum toxin injections for tremors
  • Self catheterisation if retainer
  • Amantadine, CBT and exercise can help with the fatigue
  • gabapentin for oscillopsia
  • exercise, cbt then amantadine for chronic fatigue
138
Q

Where are the classic plaque sites in MS?

A

Optic nerves
Spinal cord - majority associated with concomitant brain lesions
Brainstem - may present with ophthalmoplegia
Cerebellum - ataxia and gait disturbance
Juxtacortical white matter
Periventricular white matter near the ventricular system

139
Q

What is the most common type of MS?

A

90% characterised by relapsing-remitting disease course

140
Q

What is clinically isolated syndrome CIS?

A

First clinical episode of suspected MS
No previous evidence of demyelination clinically or on neuroimaging

Oligoclonal bands in CSF can be used as supportive criteria to help come to diagnosis

141
Q

What palsy is commonly seen in MS?

A

Abducens palsy - absence of lateral abduction of the eye

If the right abducens nerve is affected, when looking to the right, the right eye will remain central

142
Q

What are the general principles of the revised McDonald criteria?

A

Diagnosis based on MRI findings - brain +- spinal cord and clinical presentation

> 2 attacks with objective clinical evidence of >2 lesions - MS diagnosed

143
Q

What is neuromyelitis optica?

A

NMO spectrum disorders

Series of neuroinflammatory demyelinating conditions preferentially affecting the optic nerve and spinal cord

Hallmark is acute attack of bilateral optic neuritis or transverse myelitis
In NMOSD the presentation is more severe

Associated with formation of IgG autoantibody to aquaporin 4

Treatment is similar to MS with corticosteroids, but plasma exchange may be required for refractory cases

144
Q

Name two motor and two non motor complications of Parkinsons Disease

A

Motor - Freezing of Gait, Falls

Non Motor - Aspiration Pneumonia, Pressure Sores

145
Q

Name four causes of Neuromuscular Weakness

A

Guillaine Barre Syndrome
Myasthenia Gravis
Motor Neurone Disease
Muscular Dystrophy

146
Q

Define Guillaine Barre Syndrome (GBS)

A

Acute inflammatory polyneuropathy characterised by progressive ascending neuropathy with weakness and reduced reflexes

Often triggered by infections such as Campylobacter

147
Q

Describe the pathophysiology of GBS

A

Immune mediated damage to peripheral nerves (proposed antigenic mimicry)

148
Q

What are the four subtypes of GBS?

A

Acute Inflammatory Demyelinating Polyneuropathy (90%)
Acute Motor Axonal Neuropathy
Acute Motor Sensory Neuropathy
Miller Fisher Syndrome

149
Q

How does the AIDP subtype of GBS present?

A

Progressive symmetrical weakness (often ascending from distal muscles)
Reduced/absent reflexes
Reduced sensation

150
Q

What is the destructive target in Acute Motor Axonal Neuropathy (GBS)?

A

The Axons

151
Q

How does the Miller Fisher subtype of GBS present?

A

Ataxia
Areflexia
Opthalmoplegia

152
Q

Name five investigations for suspected GBS

A
  • Viral Screen
  • CXR (Rule out Sarcoidosis)
  • Electrophysiology (confirms diagnosis and differentiates axonal and demyelinating)
  • LP (increased protein and normal WCC)
  • AB for Miller Fisher
153
Q

The management for GBS is normally supportive, if required, what management can be given?

A

FVC<20ml/kg - ITU and intubation

IVIG/Plasma Exchange

154
Q

What is Hughes Disability Score?

A 
Severity scoring for GBS 
0 - Healthy
4 - Bedridden
5 - Assisted Ventilation
6 - Death
155
Q

Define Myasthenia Gravis

A

Antibody mediaed blockage of Neuromuscular Transmission due to ACh receptor AB

156
Q

Describe the pathophysiology of Myasthenia Gravis

A
  • Type II Hypersensitivity reaction
  • Cross links receptors causing destruction and activates –Membrane Attack Complexes to destroy membrane
  • Disease fluctuates as NMJ can repair itself and create more receptors easily
157
Q

What are the three stages of Myasthenia Gravis?

A

1 - Fluctuating muscle weakness with most severe symptoms happening here
2 - Persistent but stable symptoms
3 - Remission

158
Q

What is the relation of Myasthenia Gravis to the Thymus?

A

10-15% have a Thymoma
Up to 85% have Thymic Hyperplasia

There are myoid cells in the thymus which may start the target for autoimmunity

159
Q

What are the two clinical subtypes of Myasthenia Gravis?

A

Ocular

Generalised

160
Q

Name three antibodies associated with Myasthenia Gravis

A

AChR - Ab
Anti MuSK
Anti - LRP4

161
Q

Name four ocular features of Myasthenia Gravis

A

Diplopia (improved on occluding one eye)
Ptosis
Weak eye movements
Pupillary sparing

162
Q

Name four features of Generalised Myasthenia Gravis

A
  • Bulbar - Fatiguable chewing
  • Facial - Expressionless
  • Neck - Dropped head towards EOD
  • Proximal limb weakness more than distal
163
Q

What is the Ice Pack Test for Myasthenia Gravis?

A

Improvement of ptosis after ice applied to closed eye for one minute
Neuromuscular transmission is better at lower temperatures

164
Q

Other than the Ice Pack Test, name four investigations for Myasthenia Gravis

A
  • Tensilon Test (improvement after AChesterase inhibitors)
  • Serological Antibodies
  • Repetitive Nerve Stimulation (showing decline)
  • CT/MRI thymus
165
Q

Name three options for treating Myasthenia Gravis

A

Pyridostigmine
Prednisolone
Azathioprine

Could also do a Thymectomy

166
Q

What is a Myasthenic Crisis?

A

Worsening of weakness requiring respiratory support

Precipitants include warmth/surgery/stress/infection

167
Q

What is the action of pyridostigmine?

A

Acetylcholinesterase inhibitor

Blocks the action of acetylcholinesterase and increases the levels of acetylcholine

168
Q

How are Myasthenic Crises managed?

A

IVIG
Plasma Exchange
Steroids

169
Q

Define Muscular Dystrophy

A

Umbrella term that causes gradual wasting and weakness of muscles

170
Q

Describe Duchennes Muscular Dystrophy

A
  • X linked inherited abnormal gene
  • If mother is a carrier, son has 50% chance of being affected
  • Boys present at 3-5y with pelvic muscular weakness
  • Life expectancy of 25-35y
171
Q

How is Duchennes MD managed?

A

Steroids to slow progression and creatine supplements

172
Q

Describe Beckers MD

A

Dystrophin gene is less severely affected than in Duchennes

Symptoms start around 8-12y and may require a wheelchair by 20-30y

173
Q

Describe Myotonic Muscular Dystrophy

A
  • Genetic disorder presenting in adulthood

- Progressive muscle weakness, prolonged contractions, cataracts, cardiac arrhythmia

174
Q

Describe Facioscapulohumeral MD

A

In childhood, weakness around face progressing to shoulders and arms

Sleep with eyes open, can’t purse lips, can’t blow out cheeks

175
Q

Describe Oculopharyngeal MD

A

Bilateral Ptosis
Restricted eye movements
Swallowing problems

176
Q

Describe Emery Dreifuss MD

A
  • Contractures (usually at elbow and ankles)

- Progressive weakness and muscle wasting

177
Q

Define Benign Essential Tremor

A

Relatively common condition characterised by fine trmor affecting voluntary muscles, most noticable in hands but can affect other areas

178
Q

Name four features of Benign Essential Tremor

A

Fine Tremor
Symmetrical
Improved by alcohol
More prominent on voluntary (eg outstretched)

179
Q

How is Benign Essential Tremor managed?

A

No definitive management

Propranolol or Primidone can be tried

180
Q

What is Bell’s Palsy?

A

Idiopathic facial nerve palsy

181
Q

Name four symptoms of Bell’s Palsy

A

Difficulty chewing
Tingling
Ear Pain
Hyperacusis

182
Q

Name four signs of Bell’s Palsy

A

Loss of nasolabial folds
Drooping corner of mouth
Drooping eyebrow
Asymmetric smile

183
Q

How can Bell’s Palsy be differentiated from a stroke?

A

A stroke affected UMN so is forehead sparing

184
Q

What is the grading system for Bell’s Palsy?

A

House Brackmann Grading System

185
Q

When would you consider an alternative diagnosis to Bell’s Palsy?

A

Overt pain
Systemic Illness
Hearing abnormalities

186
Q

How is Bell’s Palsy managed?

A

Prednisolone if within 72 hours
Eye drops and tape for protection

May take several months to recover

187
Q

Name two complications of Bell’s Palsy

A

Corneal Abrasion

Aberrant Reinnervation (voluntary contraction of one muscle leads to involuntary contraction of another muscle)

188
Q

Name five presenting features of Cervical Spondylosis

A
  • Cervical pain worsened by movement
  • Cervical stiffness
  • Vague numbness/tingling/weakness of upper limbs
  • Radiculopathy
  • Retro orbital/Temporal pain
189
Q

How can you demonstrate dural irritation in Cervical Spondylosis?

A

Lateral flexion and pressure on top of head

190
Q

Name three read flags for Cervical Spondylosis

A
  • Age of onset <20 ir >55
  • Weakness/ Sensory loss in more than one Dermatome/Myotome
  • Constitutional symptoms
191
Q

How would you manage Cervical Spondylosis initially?

A

First 3-4 weeks reassure patient that it is common and will resolve
Stay active
One firm pillow at night

192
Q

After four weeks how should Cervical Spondylosis be managed?

A

Physiotherapy, Occupational health referral, Pain Clinic, Surgery

193
Q

What is a Radiculopathy?

A

Sensory/Motor symptoms in response to nerve root damage by any cause

194
Q

How does Posterior Sciatica present?

A

Pain along posterior thigh and posterolateral leg due to L5/S1 radiculopathy

195
Q

How does Anterior Sciatica present?

A

Pain along anterior leg/thigh due to L3/L4 radiculopathy

196
Q

Patients with Diabetic Neuropathy may not notice themselves. What are the five different types?

A 
Peripheral Sensorimotor
Acute Diffuse Painful
Autonomic
Mononeuropathy
Diabetic Amyotrophy
197
Q

Diabetic Neuropathy is the most common cause of Peripheral Neuropathy. Give three risk factors

A

Smoker
>40
Periods of poor glycaemic control

198
Q

How does Peripheral Sensorimotor Diabetic Neuropathy present?

A

Sensory nerves affected more (glove and stocking)
Loss of ankle jerks and later knee
Hands only in long standing

199
Q

How does Acute Diffuse Painful Diabetic Neuropathy present?

A

Abrupt onset but can resolve completely
Burning foot pain (worse at night)
Associated with poor glycaemic control

200
Q

How does Autonomic Diabetic Neuropathy present?

A

Cardiac abnormalities
Exercise intolerance
Reduced respiratory drive
Reduced baroreceptor sensitivity

201
Q

How does Mononeuropathic Diabetic Neuropathy present?

A

E.G Carpal Tunnel

202
Q

How does Diabetic Amyotrophy present?

A

Pain and paraesthesia in upper legs with weakness and wasting of muscle

203
Q

How is Diabetic Neuropathy investigated?

A

Diabetic Control
Measuring BP
Nerve conduction studies
Electromyography

204
Q

How is Diabetic Neuropathy managed?

A

Regular surveillance and foot care
Good diabetic control
bed foot cradles
Neuropathic pain relief if required

205
Q

What are the two types of ADLs?

Activities of daily living

A

Personal - Washing/Dressing/Toileting/Continence

Domestic - Cooking/Cleaning/Shopping

206
Q

Define Alzheimer’s

A

Progressive neurodegenerative disorder that causes reduced mental performance and impairment in social and occupational function

207
Q

What causes Alzheimer’s?

A

A combination of factors

Older Age, Genetics (Sporadic/APP/Presenilin), CVS Disease

208
Q

What are the two main pathological features associated with Alzheimer’s Disease?

A

Senile Plaques (Beta Amyloid deposits extracellularly, they are seen in normal ageing)

Neurofibrillary Tangles (Hyperphosphorylated Tau Proteins in regions involved in memory, promoting cell death)

209
Q

Name two cognitive and two non cognitive symptoms of Alzheimer’s disease

A

Cognitive - Poor Memory, Language

Non Cognitive - Agitation, Emotional Lability

210
Q

Name four cognitive assessments

A
  • Mini - Cog (three item word memory and clock drawing)
  • AMT (ten item tool)
  • MMSE (eleven item tool)
  • MoCA (several domains including executive function, attention, language, memory and visuospatial)
211
Q

How is Alzheimer’s Disease diagnosed?

A 
Functional Inability (and decline from previous)
Cognitive (impairment in >2 domains)
Differentials excluded (via neuroimaging etc)
212
Q

Name three non pharmacological managements of Alzheimer’s Disease

A

Advanced Care Planning
Inform DVLA
Encourage groups and activities

213
Q

What are the pharmacological options for Alzheimer’s management?

A

Mild to Mod - AChesterase inhibitors (Donepazil)

Mod to Severe - NMDA Antagonists (Memantine)

214
Q

Frontotemporal dementia has prominent disturbances in social behaviour, language and personality. What are the subtypes?

A

Behavioural Variant - Progressive personality and behavioural change

Primary Progressive Aphasia (Non Fluent or Semantic)

215
Q

Describe the pathophysiology of Frontotemporal Dementia

A

Phosphorylated Tau Proteins/Pick Bodies

Some familial cases with AD Inheritance

Atrophy of frontal and temporal lobes

216
Q

Frontotemporal Dementia can present in many different ways. How does the Behavioural Variant present?

A

Disinhibition, Loss of empathy, Apathy

217
Q

Frontotemporal Dementia can present in many different ways. How does the Primary Progressive Aphasia present?

A

Effortful/Halting Speech
Speech Apraxia
Difficulty finding words

218
Q

Frontotemporal Dementia can present in many different ways. How do the Motor Syndromes present?

A
  • MND
  • Corticobasal Syndrome (Dystonia, Asymmetrical Akinesia, Alien Limb)
  • Progressive Supranuclear Palsy (difficulty looking up)
219
Q

What would the MRI of Frontotemporal Dementia show?

A

Frontal and temporal lobe atrophy

220
Q

How would you manage Frontotemporal Dementia?

A

Financial advice, SALT input, Supervision

SSRIs (decrease impulsivity)/Atypical Antipsychotics

221
Q

State the five subtypes of Vascular Dementia

A
  • Subcortical
  • Stroke Related (Large Cortical)
  • Single/Multiple Infarct
  • Mixed (with Alzheimers)
  • Autosomal Dominant
222
Q

How does Vascular Dementia present?

A

Stepwise cognitive decline

Can have focal neurological symptoms

223
Q

How is Vascular Dementia managed?

A

Donepazil/Memantine

Control CVS factors

224
Q

What is Horner’s Syndrome?

A

Classic triad of Miosis/Ptosis and anhidrosis/enopthalmos due to a lesion along oculosympathetic pathway

225
Q

What is the Oculosympathetic Pathway?

A

First Order - Hypothalamus to Spinal Cord
Second Order - Preganglionic (spinal cord to sympathetic chain)
Third Order - Post Ganglionic (within adventitia of IC, travelling to dilator pupillae and muller’s muscle)

226
Q

What are the causes of Horner’s Syndrome if first order neurones are affected? (4S’s)

A

Stroke
multiple Sclerosis
SOL
Syringomyelia

Anhidrosis on one half of body

227
Q

What are the causes of Horner’s Syndrome if second order neurones are affected? (4T’s)

A

Tumour
Trauma
Thyroidectomy
Top Rib

228
Q

What are the causes of Horner’s Syndrome if third order neurones are affected? (3C’s)

A

Carotid Aneurysms
Carotid Artery Dissection (no other sx)
Cavernous Sinus Thrombosis

No Anhidrosis

229
Q

How can Horner’s Syndrome be investigated?

A

CT Angiography (exclude dissection)
MRI (if brainstem features)
CXR (Pancoast)

230
Q

How can Horner’s Syndrome be confirmed?

A

Cocaine Eye Drops - blocks NA reuptake, only unaffected eye constricts

Apraclonidine - Alpha agonist causing affected pupil to dilate and normal pupil to constrict

Hydroxyamphetamine - all dilate except post ganglionics

231
Q

Name two cerebellopontine angle lesions

A

Acoustic Neuroma

Meningioma

232
Q

What are Acoustic Neuromas?

A

Tumours of the vestibulocochlear nerve arising from schwann cells
Typically benign and slow growing but causes pressure on surrounding tissues

233
Q

Name two risk factors for Acoustic Neuromas

A

Neurofibromatoses

High dose ionising radiation

234
Q

What initial symptoms can Acoustic Neuromas present with?

A

Unilateral/Asymmetric hearing loss/tinnitus
Impaired facial sensation
Balance problems without explanation

235
Q

As an Acoustic Neuroma grows, what further symptoms can it cause?

A

Facial Pain/Numbness
Earache
Ataxia
Hydrocephalus

236
Q

What investigations are required for Acoustic Neuromas?

A

Audiology

MRI

237
Q

If the Acoustic Neuroma is small, you can just manage with serial scans. What other management options are there?

A

Microsurgery (can cause CSF leak or stroke)

Radiotherapy (sterotactic, fractionated, proton beam)

238
Q

What are Meningiomas?

A

Slow growing benign tumours arising from Dura Mate

Normally well circumscribed

239
Q

How are Meningiomas graded?

A

I - Generally Benign
II - Higher rate of recurrence post surgery
III - Anaplastic

240
Q

How do Meningiomas present?

A 
Seizures
Raised ICP
Changes in personality
Nerve palsies
Can be spinal - Brown Sequard
241
Q

How do Meningiomas appear on MRI?

A

Well defines
Central Cystic Degeneration
Oedema of nearby white matter

242
Q

What are the management options for Meningiomas?

A

Endovascular Embolisation (Coil or Glue)
Surgical Removal
Stereotactic Radiotherapy

243
Q

What is Subacute Combined Degeneration of the Cord?

A

Degeneration of the posterior and lateral columns as a result of B12/Vitamin E/Copper deficiency

244
Q

Name three underlying causes of Subacute Combined Degeneration of the Cord

A

Dietary Deficiency
Lack of IF
Low Gastric pH

245
Q

What would be the underlying investigative abnormality in Subacute Degeneration of the Spinal Cord?

A

B12 and folate deficiency leading to megaloblastic anaemia

246
Q

How does Subacute Degeneration of the Spinal Cord present?

A

Weakness of leg/arms/trunk
Progressive tingling and numbness
Posterior Column - reduced vibration and proprioception
Lateral Column - UMN signs, Ataxia

247
Q

How is Subacute Degeneration of the Spinal Cord managed?

A

Replace B12

If folate also deficient, treat B12 first

248
Q

What is Cavernous Sinus Syndrome?

A

Any pathology involving Cavernous Sinus which may present as a combination of unilateral opthalmoplegia, autonomic dysfunction, or sensory loss

249
Q

Give four causes of Cavernous Sinus Syndrome

A

Meningioma
Sarcoidosis
Basal Skull Fracture
Cavernous Sinus Thrombosis

250
Q

How does Cavernous Sinus Syndrome present?

A

Can compress any nerves leading to isolated palsies or post ganglionic Horners
May get Proptosis and Chemosis secondary to pressure

251
Q

How is Cavernous Sinus Syndrome investigated?

A

Bloods
MRI
CT

252
Q

How is Cavernous Sinus Syndrome managed?

A

Tumour - Surgery or Radio
Traumatic - Orbital Decompression
Inflammatory - Steroids
Vascular - Embolisation/Clipping

253
Q

What is Wernicke’s Encephalopathy?

A

Spectrum of diseases resulting from Thiamine deficiency (usually related to alcohol abuse although can be secondary to Bariatric Surgery/Hyperemesis Gravidarum etc)

254
Q

Describe the pathophysiology of Wernicke’s Encephalopathy

A

Inadequate nutritional thiamine/decreased absorption from GI tract/impaired thiamine utilisation

Thiamine is a cofactor required by three enzymes for carbohydrate synthesis so causes metabolic disruption

255
Q

What is the classic triad of Wernicke’s Encephalopathy? Name two other symptoms

A

Ataxia, Opthalmoplegia, Confusion

Unexplained hypotension/hypothermia
Hallucinations

256
Q

What is required to diagnose Wernicke’s Encephalopathy?

A

At least 2 of:

Dietary Deficiency
Oculomotor Abnormalities
Cerebellar Dysfunction
Altered Mental State/Impairment

257
Q

How is Wernicke’s Encephalopathy managed?

A

Thiamine + Pabrinex

Alcoholics should have prophylactic thiamine as long as they are malnourished/have decompensated liver disease

May have to use Mental Health Act

258
Q

One of the complications of Wernicke’s Encephalopathy is Korsakoff Syndrome. Name three features of this

A

Confabulation
Anterograde and Retrograde Amnesia
Psychosis

259
Q

What is an Argyll Robertson Pupil?

A

Small irregular pupils that have little/no constriction to light but do accommodate

Classically caused by neurosyphilis

260
Q

Describe the pathophysiology behind an Argyll Robertson Pupil

A

Damage to dorsal aspect of EWN disrupts inhibitory neurones causing constant constriction

261
Q

What is Creutzfeld-Jakob Disease?

A

Best known Human Prion Disease

Accumulation of small infectious pathogens, containing protein but lacking nucleic acids

262
Q

Name the four main variants of CJD

A
  • Sporadic (85% of cases)
  • Hereditary (Familial clusters with dominant inheritance)
  • Iatrogenic (Neurosurgery, tissue grafts)
  • New Variant (linked to eating BSE infected cattle products)
263
Q

CJD presents very non specifically and can’t be reliably diagnosed until death. Give some presentations

A
  • Myoclonus
  • Progressive Ataxia/Choreiform
  • Visual Disturbance
  • Rapidly progressing cognitive and functional impairment
264
Q

How is CJD investigated?

A
  • Brain Biopsy (can use tonsil biopsy if new variant)
  • CSF markers
  • EEG (Periodic wave complexes in sporadic)
  • MRI (increased intensity in certain brain regions depending on subtype)
265
Q

Define Syringomyelia

A

Fluid filled tubular cyst in central spinal cord that can elongate/enlarge and expand into grey/white matter, compressing tracts

266
Q

Define Syringobulbia

A

Where Syringomyelia has extended into brainstem

Can cause nerve palsies

267
Q

Describe the aetiology of Syringomyelia

A

Blockage of CSF (normally secondary to Chiari malformation)

SOL, Arachnoiditis, Post Traumatic

268
Q

How does Syringomyelia present in terms of sensory features?

A

Spinothalamic lost in a shawl like distribution
Extends into Dorsal Column

Dysaesthesia - Pain when skin is touched

269
Q

How does Syringomyelia present in terms of motor features?

A

As it extends and damages LMN of anterior horn

Muscle wasting and weakness beginning in hand
Reduced tendon reflexes
Claw hand

270
Q

How does Syringomyelia present in terms of autonomic features?

A

Can affect bowel/bladder/sexual organs

Horners

271
Q

How can Syringomyelia be investigated?

A

MRI - shows soft tissue causes

CT - shows bony causes

272
Q

How can Syringomyelia be managed?

A

Physio and rehab
Taught to avoid damage which may occur in the absence of pain

Surgery - Shunt/Laminectomy/Syringotomy

273
Q

What is Hypoxic Ischaemic Encephalopathy?

A

Entire brain is derived of adequate oxygen supply but loss is not total. Normally associated with neonates but can occur in adults (eg post cardiac arrest)

274
Q

How does HIE present?

A

Cyanosis as blood is redirected

Fainting/Coma/Seizures/Brain Death

275
Q

What would be seen on CT of HIE?

A

Diffuse oedema

Reduced cortical grey matter

276
Q

What Syndrome can Pituitary Tumours be associated with?

A

MEN1

277
Q

Name four types of Pituitary Tumour

A

Non functioning adenoma
Prolactinoma
GH secreting
ACTH secreting

278
Q

Describe the local effects of a Pituitary Tumour

A

Headache (retro-orbital or bitemporal, worse on waking)
Visual Field Defects
Facial Pain

If extending into hypothalamus - Diabetes Insipidus etc

279
Q

How can the cause of Bitemporal Hemianopia be distinguished?

A

If initially lower quadrants affected - Craniopharyngoma

If upper - Pituitary Adenoma

280
Q

What is the order of hormones affected in Hypopituitarism

A

LH, GH, TSH, ACTH, FSH

281
Q

The management for Pituitary Adenomas is often Trans-sphenoidal surgery. Name four complications

A

SIADH
DI
Addisons
CSF leak

282
Q

How can you manage Pituitary Adenomas medically?

A

Acromegaly - Octreotide

Prolactinoma - Bromacriptine

283
Q

When would you use Radiotherapy for Pituitary Adenoma?

A

Incomplete resection

284
Q

Name three causes of Olfactory Nerve Damage

A

Trauma
Frontal Lobe Tumour
Meningitis

285
Q

Name three causes of Oculomotor Nerve Damage

A

DM
GCA
PCA

286
Q

Name two causes of Trochlear Nerve Damage

A

Rare -Orbital trauma, Diabetes

287
Q

Name three causes of Abducens Nerve Damage

A

MS
Pontine CVA
Raised ICP

288
Q

Name two causes of Facial Nerve Damage

A

Upper - Stroke

Lower - Bells

289
Q

Name three causes of Vestibulocochlear Nerve Damage

A

Loud Noises
Pagets
Acoustic Neuroma

290
Q

Name three causes of Glossopharyngeal Nerve Damage

A

Trauma
GBS
Polio

291
Q

Name two causes of Vagus and Accessory Nerve Damage

A

Trauma

Brainstem pathology

292
Q

Name three causes of Hypoglossal Nerve Damage

A

Polio
Syringomyelia
TB

293
Q

What AED is tolerated really well?

A

Levetiracetam (Keppra)

294
Q

Name three stroke mimics

A

Migraines
Todd’s Paresis (post seizure)
Bells Palsy

295
Q

Name three Chameleons (atypical strokes)

A

Bilateral thalami stroke
Bilateral occipital stroke
Limb shaking TIA

296
Q

What can affect the seizure threshold?

A

Medications
Drugs
Sleep
Flashing Lights

297
Q

What is the pregnancy prevention programme for epilepsy?

A

At least 1 form of contraception (ideally 2)

Should be started Atleast one month before starting the medication

298
Q

MCA infarcts make up 2/3 of Ischaemic strokes. How would ACA strokes present differently?

A

Contralateral Lower limb > upper limb

Disorder of executive function

299
Q

What is characteristic on an epileptic EEG?

A

Spike and wave discharge

Hz is how many spikes and waves in a second

300
Q

What are the four recognised stages of Parkinson’s

A

Early
Maintenance
Advanced
Palliative

301
Q

What are the main neurotransmitters in the CNS?

A

Glutamate - main excitatory transmitter in the central nervous system - NMDA are glutamate receptors
GABA - inhibitory in the brain
Glycine - inhibitory in the spinal cord

Neuro & Special Senses (12 decks)

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