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Medicine Finals > Neurology > Flashcards

Neurology Flashcards

1
Q

What is the maximum score a patient can get on the Glasgow coma scale?

A

15

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2
Q

What is the lowest score a patient can get on the Glasgow coma scale?

A

3

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3
Q

What are the two types of stroke?

A

Ischaemic and haemorrhagic

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4
Q

What is the most common type of stroke?

A

Ischaemic

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5
Q

What causes an Ischaemic stroke?

A

Thrombus or embolus

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6
Q

What are causes of a haemorrhagic stroke?

A

Burst blood vessel
Subarachnoid haemorrhage
Intracerebral bleed

Most common cause = Hypertensive vasculopathy

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7
Q

What are the 3 main criteria assessed in stroke?

A

Unilateral hemiparesis
Homonymous hemianopia
Higher cognitive dysfunction (eg dysphasia)

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8
Q

How does a total anterior circulation infarction present?

A

All 3 - unilateral hemiparesis, homonymous hemianopia, higher cognitive dysfunction

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9
Q

How does a partial anterior circulation infarction present ?

A

Only 2 of unilateral hemiparesis/homonymous hemianopia/higher cognitive dysfunction

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10
Q

How does a lacunar infarct present?

A

1 of:
Unilateral weakness
Pure sensory stroke
Ataxic hemiparesis

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11
Q

How does a posterior circulation infarction present?

A

1 of:

Cerebellar/brain stem syndrome

Loss of consciousness

Isolated homonymous hemianopia

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12
Q

What is the first investigation conducted in stroke? What is the gold standard?

A

Non contrast CT - rule out haemorrhagic stroke

Gold standard = diffusion weighted MRI

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13
Q

How is an ischaemic stroke managed

A

300mg aspirin (continue for 2 weeks)

Thrombolysis with alteplase (if within 4.5 hours of stroke symptoms)

Thrombectomy (if within 6 hours and proximal anterior circulation stroke)

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14
Q

How long within onset of stroke symptoms does alteplase (thrombolysis) need to be administered?

A

Within 4.5 hours

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15
Q

What are contraindications to thrombolysis with alteplase in ischaemic stroke?

A 
Previous intracranial haemorrhage
Seizure at onset of stroke
Suspected subarachnoid haemorrhage 
Lumbar puncture in the last 7 days
Active bleeding
Pregnancy 
Uncontrolled HTN
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16
Q

What kind of stroke can a thrombectomy be done in?

A

A proximal anterior circulation infarct

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17
Q

How soon within onset of stroke symptoms does thrombectomy need to occur?

A

Within 6 hours

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18
Q

What is the medical management of stroke?

A

300mg Aspirin daily for 2 weeks
If AF diagnosed on inpatient, wait the 2 weeks before commencing anticoagulants

After 2 weeks is up…

1) Clopidogrel 75mg
Or Aspirin + Dipyridamole

2) Statin
3) Anti-hypertensives

If AF –> Start a DOAC

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19
Q

What is a subdural haemorrhage?

A

Rupture of the bridging veins between the dura mater and the arachnoid mater

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20
Q

How is a subdural haemorrhage seen on CT?

A

Crescent shaped
Blood crosses suture lines
If acute - bright lesion (hyper dense)
If chronic - dark lesion (hypodense)

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21
Q

What is the cause of an acute subdural haemorrhage?

A

High impact trauma

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22
Q

Which people are at risk of a chronic subdural haemorrhage?

A

Alcoholics and the elderly

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23
Q

How is a subdural haemorrhage managed?

A 
Chronic = Decompression with burr holes
Acute = decompressive craniotomy
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24
Q

What is an extradural haematoma?

A

Rupture of the middle meningeal artery

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25
Q

How does an extradural haematoma look on a CT?

A

Hyperdense area - Bi-convex/lens shaped

Blood does not cross the suture lines

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26
Q

How does an extradural haematoma usually present?

A

Loss of consciousness followed by a lucid interval and then worsening of symptoms

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27
Q

Which type of intracranial bleed is associated with a lucid period?

A

Extradural haematoma

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28
Q

What is an intracerebral haemorrhage ?

A

A bleed within the brain tissue

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29
Q

What is the cause of a subarachnoid haemorrhage?

A

Ruptured cerebral aneurysm (Berry aneurysm)

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30
Q

How does a subarachnoid haemorrhage present?

A

Sudden severe occipital headache
Nausea and vomiting
Photophobia
Neck stiffness

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31
Q

What is seen on lumbar puncture in subarachnoid haemorrhage?

A

Xanthochromia due to increased bilirubin

Normal /raised opening pressure

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32
Q

How long after presentation of SAH can you do a lumbar puncture?

A

Need to wait at least 12 hours from onset of symptoms

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33
Q

How is an SAH managed?

A

Insertion of coil

21 day course of Nimodipine

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34
Q

What is multiple sclerosis?

A

An autoimmune disorder causing demyelination of myelinated neurones

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35
Q

What is the most common form of multiple sclerosis?

A

Relapsing remitting

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36
Q

What are some eye features of multiple sclerosis?

A

Optic neuritis - reduced vision, pain on eye movement

Uhthoff’s phenomenon - worsening of vision following rise in body temp

Internuclear opthalmoplegia

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37
Q

What are some sensory symptoms of multiple sclerosis?

A

Pins and needles
Numbness
Trigeminal neuralgia
Lhermitte’s syndrome - electric shock sensation in limbs on neck flexion

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38
Q

How is multiple sclerosis seen on MRI?

A

Peri ventricular plaques

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39
Q

How is multiple sclerosis see on lumbar puncture?

A

Oligoclonal bands in the CSF

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40
Q

How is an acute relapse of multiple sclerosis managed?

A

High dose steroids (oral or IV methylprednisolone)

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41
Q

What is a disease modifying drug used in multiple sclerosis?

A

Beta interferon

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42
Q

What is motor neurone disease?

A

Umbrella term for condition that leads to destruction of motor neurones

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43
Q

What is the most common type of motor neurone disease?

A

Amyotrophic lateral sclerosis

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44
Q

How does amyotrophic lateral sclerosis present?

A

LMN signs in the arms

UMN signs in the legs

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45
Q

What are upper motor neurone signs?

A

Increased tone
Spasticity
Brisk reflexes
Upgoing plantar reflexes

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46
Q

What are lower motor neurone signs?

A

Muscle wasting
Reduced tone
Muscle twitching
Reduced reflexes

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47
Q

How is motor neurone disease managed?

A

Riluzole may extend survival by a few months

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48
Q

What is Parkinson’s disease?

A

Condition where there is progressive reduction of dopamine in the substantia nigra

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49
Q

What is the triad of symptoms seen in Parkinson’s ?

A
  1. Resting tremor - asymmetrical, pill-rolling
  2. Bradykinesia - shuffling gait, micrographia, reduced arm swing
  3. Rigidity
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50
Q

What other symptoms are seen in Parkinson’s?

A 
Depression
Drooling of saliva
Reduced facial expressions 
Fatigue 
Postural hypotension
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51
Q

What is the first line management of Parkinson’s?

A

If motor symptoms affecting quality of life - levodopa

If motor symptoms not affecting quality of life - can also use a dopamine agonist eg cabergoline/bromocriptine

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52
Q

What are side effects of levodopa?

A 
Dyskinesia (uncontrollable muscle movements)
Dry mouth
Anorexia
Palpitations
Red discolouration of urine
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53
Q

How can you tell whether a tremor is an essential tremor or due to Parkinson’s ?

A

Essential tremor is worse on movement and better on resting

Essential tremor is symmetrical

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54
Q

How can an essential tremor be managed?

A

Propranolol

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55
Q

How does a generalised tonic clonic seizure present?

A

Loss of consciousness
Tongue biting
Tonic and clonic episodes

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56
Q

What is first line for a tonic clonic seizure?

A

Sodium valproate

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57
Q

What is first line for absence seizures?

A

Sodium valproate or ethosuximide

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58
Q

What is first line for atonic seizures?

A

Sodium valproate

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59
Q

What is first line for myoclonic seizures?

A

Sodium valproate

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60
Q

How does a temporal lobe focal seizure present?

A

Hallucinations, memory flashbacks, deja vu

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61
Q

How does a frontal lobe focal seizure present?

A

Head/leg movements, Jacksonian March, post-ictal weakness

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62
Q

What is the first line management for focal seizures?

A

Carbamazepine/lamotrigime

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63
Q

How long must you be seizure free to drive?

A

Single seizure - 6 months
Established epilepsy/multiple seizures - 12 months

bus drivers etc - 5 years one seizure, 10 years more than one

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64
Q

Which anti epileptic drug is used in pregnancy?

A

Lamotrigine

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65
Q

What is status epilepticus?

A

Either
A single seizure more than 5 minutes
Or
2 or more seizures within 5 minutes without the patient returning to normal in between

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66
Q

How do you manage a patient with status epilepticus?

A 
ABCDE
Secure the airway
Administer oxygen
Insert cannula
Check blood glucose

Administer IV Lorazepam/ Rectal diazepam / Buccal midazolam
This can be repeated in 10 mins but only once

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67
Q

How do you manage status epilepticus which is still ongoing after two doses of 4mg IV Lorazepam?

A

IV Phenytoin or Phenoarbital

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68
Q

What kind of genetic pattern is Huntington’s?

A

Autosomal dominant

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69
Q

What phenomenon do you witness with future generations of Huntington’s chorea?

A

Genetic anticipation - successive generations have

1) earlier age of onset
2) increased severity of disease

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70
Q

How does Huntington’s present?

A

Initially cognitive/psychiatric/mood problems

Then movement disorders:

Chorea

Eye movement disorders

Dysarthria

Dysphagia

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71
Q

What is myasthenia gravis?

A

An autoimmune disorder against acetylcholine receptors in the neuromuscular junction

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72
Q

What type of tumour is myasthenia gravis associated with?

A

Thymoma

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73
Q

Which autoantibody is seen in myasthenia gravis?

A

Acetylcholine receptor antibodies

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74
Q

How does myasthenia gravis present?

A

Muscle fatigabilty - muscles get weaker the more they’re used

Extraocular muscle weakness - Diplopia

Eyelid weakness - ptosis

Facial weakness

Jaw fatigue when chewing

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75
Q

How is myasthenia gravis managed?

A

Pyridogstigmine (acetylcholinesterase inhibitor)

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76
Q

What is a myasthenia crisis?

A

Acute worsening of symptoms in someone with myasthenia gravis
Respiratory failure

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77
Q

How is a myasthenic crisis managed?

A

Non invasive ventilation

IV Immunoglobulins

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78
Q

How does Lambert Eaton myasthenic syndrome present?

A

Someone with small cell lung cancer

Muscle weakness - sometimes improves with use

Usually in the proximal leg muscles

Can also cause diplopia and ptosis

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79
Q

How is Lambert Eaton managed? (Including specific drug)

A

Treat underlying malignancy

Amifampridine (symptomatic)

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80
Q

What is a normal intracranial pressure?

A

7-15

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81
Q

What are causes of a raised ICP?

A 
Idiopathic intracranial hypertension 
Traumatic head injury
Meningitis 
Encephalitis
Brain Abscess
Tumour - endocrine, metastases, primary
Hydrocephalus
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82
Q

How does raised ICP present?

A

Headache worse in the morning
Vomiting
Reduced consciousness
Papilloedema

Cushing’s triad - Bradycardia, Raised BP, Bradypnoea

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83
Q

How is raised ICP managed? (3 steps)

A

IV mannitol

Head elevation to 30 degrees

Controlled hyperventilation

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84
Q

How does a parietal lobe lesion present?

A

Apraxia (inability to perform movements on command)

Astereognosis (inability to identify objects by feel)

CONTRALATERAL Inferior homonymous quadrantanopia

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85
Q

How does an occipital lobe lesion present?

A

Homonymous hemianopia with macular spearing

Cortical blindness (vision loss)

Visual agnosia - inability to recognise familiar people/objects

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86
Q

How does a temporal lobe lesion present?

A

Wernickes aphasia - fluent speech but with word substitution

CONTRALATERAL Superior homonymous qudrantanopia

Auditory agnosia - inability to recognise familiar sounds/voices

Difficulty recognising faces

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87
Q

How does a frontal lobe lesion present?

A

Brocas aphasia - non fluent halting speech

Disinhibition

Perseveration

Anosmia

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88
Q

What is the most common cancer that metastasises to the brain?

A

Lung

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89
Q

What is the most common primary brain tumour in adults?

A

Glioblastoma

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90
Q

What are first line treatments for neuropathic pain?

A

Amitripytyline/pregabalin/gabapentin/duloxetine

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91
Q

How does trigeminal neuralgia present?

A

Severe unilateral pain on the face

Triggered by washing/talking/smoking/brushing teeth

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92
Q

How is trigeminal neuralgia managed?

A

Carbamazepine

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93
Q

How does a migraine present?

A

Severe unilateral throbbing headache
Nausea
Photophobia
Phonophobia

94
Q

How does aura present with migraine?

A

Blurred vision
Lines across vision
Loss of visual fields
Floaters/flashes

95
Q

What is the acute management for migraine?

A

Triptan + paracetamol/NSAID

96
Q

What is the prophylactic medication for migraine?

A

Topiramate or propranolol

Topiramate is teratogenic

97
Q

What are side effects of triptans?

A

Tightness

Heaviness

Pressure

98
Q

What are contra indications for Triptans?

A

Ischaemic Heart disease or cardiovascular disease

Severe or uncontrolled hypertension

Concurrent use of an SSRI

99
Q

What is the main risk group for cluster headaches?

A

Male smokers

100
Q

How do cluster headaches present?

A 
Severe unbearable unilateral headaches around the eye 
Red, swollen watering eye
Constricted pupils
Ptosis
Nasal discharge

Presents in clusters eg 3/4 a day for 4-12 weeks then 1-2 years without

101
Q

What is the acute management for cluster headaches?

A

Subcutaneous sumatriptan

High flow oxygen

102
Q

What is the prophylactic medication for cluster headaches?

A

Verapamil

103
Q

What are the two most common causes of bacterial meningitis in adults?

A

Neisseria meningitidis and streptococcus pneumoniae

104
Q

What is the most common cause of meningitis in neonates?

A

Group B strep

105
Q

How does meningitis present?

A 
Headache
Fever
Nausea and vomiting 
Photophobia
Neck stiffness
Drowsiness
106
Q

How does meningococcal septicaemia present?

A

Same as meningitis + non-blanching rash

107
Q

What is seen on lumbar puncture in bacterial meningitis?

A

Cloudy appearance

Raised protein

Low glucose

High neutrophils

Positive bacterial culture

108
Q

What is seen on lumbar puncture in viral meningitis?

A

Clear appearance

Normal protein

Normal glucose

High lymphocytes

109
Q

How can bacterial meningitis be managed in the community?

A

IM Benzylpenicillin

110
Q

How is bacterial meningitis treated in infants below three months?

A

IV Cefotaxime + amoxicillin

111
Q

How is bacterial meningitis treated?

A 
In the community:
IM Benzylpenicillin (if suspected due to meningococcal disease)

In hospital:
3months-50yrs: Cefotaxime/ceftriaxone

<3 months / >50 years - Cefotaxime + Amoxicillin

112
Q

How is bacterial meningitis treated in those over 50 years of age?

A

IV Cefotaxime + amoxicillin

113
Q

How are contacts of those with bacterial meningitis treated prophylactically?

A

All close contacts within seven days must be given oral ciprofloxacin or oral rifampicin

114
Q

What is encephalitis?

A

Acute inflammation of the brain

115
Q

How does encephalitis present?

A

Fever

Headache

Altered mental status (confusion, agitation, irritability)

Acute onset of focal neurological symptoms

116
Q

What is the most common cause of encephalitis?

A

HSV-1

117
Q

What is seen on lumbar puncture in encephalitis?

A

Raised protein

Raised lymphocytes

Normal glucose

118
Q

How is HSV-1 encephalitis managed?

A

IV Aciclovir

119
Q

What is Guillan barre syndrome?

A

An immune mediated demyelination of the peripheral nervous system

Triggered by an infection

120
Q

Which infections most commonly cause Guillan-Barre syndrome?

A

Campylobacter jejuni

Cytomegalovirus

Epstein-Barr virus

121
Q

What are characteristic features of Guillan-Barre syndrome?

A

History of gastroenteritis

Initial back/leg pain

Progressive symmetrical weakness of all limbs which starts in the legs

Reduced reflexes

122
Q

What is seen on nerve conduction studies in Guillan-Barre syndrome?

A

Decreased motor conduction velocity

123
Q

What is seen on lumbar puncture in Guillan-Barre syndrome?

A

Raised protein, normal white cell count

124
Q

How is Guillan-Barre syndrome managed?

A

IV Immunoglobulins

Supportive care

125
Q

What is Charcot-Marie-Tooth disease?

A

Autosomal dominant cause of peripheral neuropathy

126
Q

What are classic features seen in someone with Charcot-Marie-Tooth disease?

A

(Most common hereditary cause of peripheral neuropathy)

History of frequently sprained ankles

High foot arches

Distal muscle wasting - inverted champagne bottle legs

Hammer toes

Peripheral sensory loss

127
Q

How does a lower motor neurone facial nerve palsy present?

A

Affects whole face

128
Q

How does an upper motor neurone facial nerve palsy present?

A

Forehead sparing

129
Q

What are you worried of when you see facial nerve palsy with forehead sparing?

A

Stroke

130
Q

What is Bell’s palsy?

A

An acute unilateral facial nerve paralysis caused by an UMN lesion of the facial nerve

131
Q

Is Bell’s palsy LMN or UMN?

A

Almost always LMN - NO forehead sparing

132
Q

How is Bell’s palsy managed?

A 
Oral prednisolone (if presents within 72 hours of onset)
If no improvement within 3 weeks - ENT referral
133
Q

A lesion in which nerve commonly presents with foot drop?

A

Common peroneal nerve

134
Q

What is neurofibramatosis?

A

A genetic condition that causes nerve tumours to develop throughout the nervous system

135
Q

What is the inheritance pattern of neurofibramatosis?

A

Autosomal dominant

136
Q

How does neurofibramatosis type 1 present?

A

Cafe-au-lait spots

Lisch nodules (pigments around the iris)

Phaeochromocytoma

Axillary/groin freckles

137
Q

How does neurofibramatosis type 2 present?

A

Bilateral acoustic neuromas(vestibular schwannomas)

Leading to hearing loss, tinnitus and balance problems

138
Q

What is tuberous sclerosis?

A

An autosomal dominant condition causing hamartomas (benign neoplastic growth of tissue)

139
Q

What are skin features seen in tuberous sclerosis?

A

Depigmeted ash leaf spots

Shagreen patches (roughed patches of skin over the lumbar spine)

Adenoma sebaceum (butterfly distribution over the nose)

Subungal fibromata

140
Q

What neurological features are seen in tuberous sclerosis?

A

Developmental delay

Epilepsy

Intellectual impairment

141
Q

How does a brain abscess present?

A

Headache

Fever

Focal neurology eg nerve palsy

Papilloedema

Nausea

142
Q

How is a brain abscess managed?

A

Craniotomy + debridement of cavity

IV Ceftriaxone + metronidazole

143
Q

What is normal pressure hydrocephalus?

A

A reversible cause of dementia due to decrease CSF absorption

Causes dementia + urinary incontinence

144
Q

How does normal pressure hydrocephalus present?

A

Urinary incontinence

Dementia

Gait abnormality

145
Q

How is normal pressure hydrocephalus managed?

A

Ventriculoperitoneal shunt

146
Q

What is multiple system atrophy?

A

Parkinson’s plus syndrome

Parkinson’s + autonomic dysfunction e.g. postural hypotension, constipation

147
Q

What is important to consider in patients taking Levodopa?

A

Don’t stop suddenly - if can’t take suddenly then prescribe as patch

If they stop suddenly can cause neuroleptic malignant syndrome

148
Q

How is postural hypotension treated?

A

Midodrine

149
Q

Which drugs can cause drug-inducted Parkinson’s?

A

Anti-psychotics (especially typical anti-psychotics)

Metoclopramide

Drug induced Parkinson’s is usually bilateral

150
Q

Which lobe is Wernicke’s associated with?

A

Temporal lobe

151
Q

Which lobe is Broca’s associated with?

A

Frontal lobe

152
Q

How do you treat a post lumbar puncture headache?

A

Caffeine and fluids

153
Q

How does a third nerve palsy present?

A

Down and out position of the eye

Ptosis

Pupil may be dilated

154
Q

What are causes of a third nerve palsy?

A

Herniation

Aneurysm

Weber’s syndrome (stroke of the posterior cerebral artery branches) - which causes ipsilateral third nerve palsy and contralateral hemiplegia

155
Q

Can cranial bleeding cause contralateral or ipsilateral third nerve palsy?

A

Ipsilateral

156
Q

How does a fourth nerve palsy present (trochlear nerve)?

A

Defective downward gaze

Vertical Diplopia

157
Q

How does a fifth nerve palsy present? (Trigeminal nerve)

A

Trigeminal neuralgia

Loss of corneal reflex

Loss of facial sensation

Paralysis of the mastication muscles

deviation of jaw muscles to weak side

158
Q

How does a sixth nerve palsy present? (Abducens nerve)

A

Defective abduction in the affected eye

Horizontal diplopia

159
Q

How does a stroke in the anterior cerebral artery present?

A

Contralateral hemiparesis and sensory loss

Affects legs more than arms

160
Q

How does a stroke in the middle cerebral artery present?

A

Contralateral hemiparesis and sensory loss

Affects arms more than legs

Contralateral homonymous hemianopia

Aphasia

161
Q

How does a stroke in the posterior cerebral artery present?

A

Contralateral homonymous hemianopia with macular spearing

Visual agnosia (unable to visually recognise objects)

No sensory loss/hemiparesis

162
Q

What is Weber’s syndrome and how does it present?

A

Stroke of the branches of the posterior cerebral artery that supply the midbrain

Ipsilateral third nerve palsy

Contralateral arm and leg weakness

163
Q

What is Wallenberg’s syndrome and how does it present?

A

Posterior inferior cerebellary artery stroke

AKA Lateral medullary syndrome

Ipsilateral facial pain and temperature loss

Contralateral limb/torso pain, and temperature loss

Ataxia

Nystagmus

164
Q

How does anterior inferior cerebellar artery stroke present?

A

Ipsilateral facial pain and temperature loss

Contralateral limb/torso pain and temperature loss

(Same as posterior) except also

Ipsilateral facial paralysis and deafness

165
Q

How does a stroke of the ophthalmic artery present?

A

Amaurosis fugax

166
Q

How does a stroke in the basilar artery present?

A

Locked in syndrome (complete paralysis except for eyes)

167
Q

What is autonomic dysreflexia?

A

Occurs in patients who have had a spinal cord injury at or above T6

Hyperstimulation of the nervous system

Often triggered by faecal impaction/urinary retention

Causes extreme HTN, flushing and sweating, dilated pupils, headache, lightheadedness

Can cause haemorrhagic stroke

168
Q

What is Creutzfeldt-Jakob disease?

A

Rapidly progressive neurological condition due to prion proteins

169
Q

How does Creutzfeldt-Jakob disease present?

A

Rapid onset dementia

Myoclonus

170
Q

What is spyringomyelia?

A

Collection of cerebrospinal fluid within the spinal cord

171
Q

How does spyringomyelia present?

A

Loss of pain and temperature sensation to the neck and arms (cape distribution)

172
Q

How is spyringomyelia investigated?

A

Full spine MRI with contrast

173
Q

Neuro presentation: patient with loss of pain and temperature sensation to neck and arms?

A

Spyringomyelia

Full spine MRI with contrast

174
Q

How does degenerative cervical myopathy present and how is it diagnosed?

A

Pain

Loss of motor function (digital dexterity, holding a fork, doing up buttons)

Numbness

Urinary/faecal incontinence

Hoffmann’s sign

Diagnosed with MRI cervical spine

175
Q

What inheritance pattern does Freidrich’s Ataxia have?

A

Autosomal recessive

176
Q

How does Freidrich’s Ataxia present?

A

Gait ataxia

Kyphoscoliosis

Optic atrophy

Hypertrophic obstructive cardiomyopathy

Pes cavus (high arch)

177
Q

What inheritance pattern does Ataxia telangiectasia have?

A

Autosomal recessive

178
Q

What are features of ataxia telangiectasia?

A

Cerebellar ataxia

Telangiectasia

IgA deficiency (recurrent infections)

179
Q

What is idiopathic intracranial hypertension?

A

Idiopathic raised intracranial pressure classically seen in young obese females

180
Q

How does idiopathic intracranial HTN present?

A

Headache - worse on waking, worse when coughing/mvoing head

Blurred vision

Papilloedema

(Presents like raised ICP)

Hears a ‘whoosh’

6th nerve palsy (inability to abduct affected eye)

181
Q

What drugs can cause idiopathic intracranial HTN?

A

Tetracyclines

Steroids

COCP

Lithium

Vitamin A (isotretinoin)

182
Q

How is idiopathic intracranial HTN managed?

A

Weight loss

Acetazolamide (reduces pressure - also used in open angle glaucoma)

183
Q

How does someone with myotonic dystrophy present?

A

Myotonic face (long, haggard appearance)

Progressive weakness and muscle wasting

DM1 - distal weakness most prominent

DM2 - proximal weakness most prominent

Frontal balding

Bilateral ptosis

Cataracts

Dysarthria

Myotonia

184
Q

What are the two dystrophinopathies and what is their inheritance pattern?

A

Duchenne muscular dystrophy and Becker muscular dystrophy

Both X-linked recessive

185
Q

How do you differentiate between Duchenne and Becker muscular dystrophy?

A

Duchenne develops at around 5 years and causes intellectual impairment and calf pseudo hypertrophy

Becker muscular dystrophy develops after 10 years and intellectual impairment is less common

186
Q

What is narcolepsy?

A

A rare condition where the brain loses its ability to regulate the sleep wake cycle

Type 1 is associated with cataplexy (sudden loss of muscle tone often triggered by emotion)

187
Q

What is a pituitary apoplexy?

A

The sudden enlargement of a pituitary tumour (usually a non-functioning adenoma e.g. prolactinoma)

This enlargement is secondary to haemorrhage or infarction

188
Q

How does a pituitary apoplexy present?

A

Sudden onset severe headache (similar to SAH)

Vomiting

Neck stiffness

Bitemporal superior quadrantinopia

Severe, acute hypopituitarism - Features of pituitary insufficiency - hyponatraemia, hypotension (secondary to adrenal insufficiency)

189
Q

How is a pituitary apoplexy treated?

A

Urgent steroid replacement - IV hydrocortisone

Careful fluid balance with IV fluids

Surgical resection

190
Q

How is restless legs syndrome managed?

A

Ropinirole

191
Q

What is thoracic outlet syndrome?

A

Compression of the brachial plexus/subclavian artery/subclavian vein at the site of the thoracic outlet - can be neurogenic or vascular

192
Q

How does neurogenic thoracic outlet syndrome present?

A

Painless muscle wasting of hand muscle

Hand weakness

Numbness and tingling of hand

193
Q

How does vascular thoracic outlet syndrome present?

A

Vein -> painful diffuse arm swelling

Artery -> painful arm claudication

194
Q

What is Wernicke’s encephalopathy and how does it present?

A

Disorder caused by a thiamine deficiency (most commonly seen in alcoholics)

Ophthalmoplegia/nystagmus

Ataxia

Confusion

195
Q

How is chemotherapy related nausea managed?

A

Ondansetron

196
Q

How is nausea in Parkinson’s managed?

A

Domperidone

197
Q

What electrolyte abnormality is commonly associated with subarachnoid haemorrhage?

A

Hyponatraemia

Due to SIADH

198
Q

Which cancer is Lambert Eaton Syndrome associated with?

A

Small cell lung cancer

199
Q

What is autoimmune encephalitis and how does it present?

A

A form of non-infectious brain inflammation

Confusion

Seizures

Behavioural changes

Emotional lability (can switch from laughing to crying easily)

Psychosis

200
Q

What is seen on lumbar puncture in autoimmune encephalitis?

A

Raised lymphocytes in the CSF

201
Q

How is autoimmune encephalitis treated?

A

First line = IV methylprednisolone + IV immunoglobulins

If no response within two weeks – rituximab/Cyclophosphamide 

202
Q

What type of inheritance pattern does myotonic dystrophy have?

A

Autosomal dominant

203
Q

What type of pituitary tumour is most likely to have a pituitary apoplexy?

A

Prolactinoma

204
Q

What is nystagmus?

A

Involuntary rhythmic side-to-side, up and down or circular motion of the eyes

Can be horizontal, vertical or rotary

205
Q

Which cancer is Lambert Eaton associated with?

A

Small cell lung cancer

206
Q

What can be given for spasticity in multiple sclerosis?

A

Baclofen or Gabapentin

207
Q

How do you manage bladder dysfunction in MS?

A

First do a bladder ultrasound to assess bladder emptying

If poor emptying - intermittent catheterisation

If emptying is fine - try an anticholinergic

208
Q

x

A

x

209
Q

How to medically manage someone with AF who has had a stroke?

A

Stop their anticoagulants
Give Aspirin 300mg for 2 weeks
then re-start their anticoagulants

210
Q

Which cranial nerve supplies sensory innervation to the eye?

A

CN V1 (Opthalmic nerve)

211
Q

What is Hoover’s sign?

A

Manoevure to distinguish between organic and non-organic paresis of leg

212
Q

What is paroxysmal hemicrania? How is it managed?

A

Attacks of severe unilateral headache
Often associated with autonomic features – Nasal congestion, tearing
Management – Indomethacin

213
Q

What blood marker can be used to differentiate a true seizure from a pseudoseizure?

A

Prolactin – will be rasied in true seizures

214
Q

How to differentiate postural hypotension related to Parkinson’s and postural hypotension related to multi-system atrophy?

A

In multi system atrophy they occur earlier in the disease

215
Q

What feature on an EEG is defining for an absence seizure?

A

3Hz spike and wave pattern

216
Q

What is spontaneous intracranial hypotension?

A

Rare cause of headaches due to CSF leak
Headache worse when stood upright

RF = Marfan’s

217
Q

When should trigeminal neuralgia be referred for urgent specialist assessment?

A

<40 years
Deafness/hearing loss
Optic neuritis
Family history of MS

218
Q

At what GCS should a patient be intubated?

A

Less than 8

219
Q

LMN and UMN lesions - contralateral or ipsilateral?

A

UMN = contralateral

LMN = ipsilateral

220
Q

Is Bell’s palsy contralateral or ipsilateral?

A

Ipsilateral

221
Q

Is a UMN or LMN facial palsy more worrying?

A

UMN more worrying (forehead sparing)

222
Q

Does MS need CT with contrast or without contrast?

A

With contrast (contrast needed to visualise demyelinated lesions)

223
Q

How can you differentiate between subdural and extradural haematoma by presenting features?

A

Extradural – BRIEF lucid period and then sudden onset deterioration
Subdural – can present several weeks after injury

224
Q

How is cerebral oedema in patients with brain tumours treated?

A

Dexamethasone

225
Q

What needs to be urgently ruled out in status epilepticus?

A

Hypoxia and hypoglycemia (check sats and do a capillary blood glucose)

226
Q

Is meningitis or encephalitis more likely to cause seizures?

A

Encephalitis

227
Q

What antibodies are associated with Lambert Eaton syndrome?

A

Voltage-gated calcium-channel antibodies

228
Q

What’s the difference between an UMN lesion and LMN lesion? (Pathophysiology)

A

UMN lesion = lesion above the anterior horn of the spinal cord or motor nuclei or cranial nerves

LMMN lesion = lesion which affects nerve fibres travelling from anterior horn of spinal cord to muscles

229
Q

What are causes of papilloedema?

A

Space occupying lesion – neoplastic or vascular
Malignant HTN
IIH
Hydrocephalus
Hypercapnia (build up of CO2 – this is because it triggers vasodilation and cerebral blood volume)

230
Q

How does cerebellar syndrome present?

A

DANISH

Dysdiadokinesia
Ataxia
Nystagmus (Horizontal)
Intentio tremor
Slurred speech
Hypotonia
231
Q

What are causes of cerebellar syndrome?

A 
Freidrich’s ataxia
Ataxic telangiectasia
Stroke
Alcohol
MS
Paraneoplastic – secondary to lung cancer
232
Q

How does Brown-Sequard syndrome present?

A

Ipsilateral weakness below lesion

Ipsilateral loss of proprioception and vibration sensation

Contralateral loss of pain and temperature sensation

Medicine Finals (32 decks)

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