Neurology Flashcards

1
Q

What are the two main types of stroke?

A

Ischaemic (85%): blockage in BV

Haemorrhagic (15%): burst in BV

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2
Q

What are the types of ischaemic stroke?

A

Thrombolic stroke: thrombosis from large vessels

Embolic stroke: Blood/fat/bacteria breaks away and translocates
–> Think AF

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3
Q

What are the types of haemorrhagic stroke?

A

Intracerebral haemorrhage: bleeding within the brain

Subarachnoid haemorrhage: bleeding on surface of brain

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4
Q

Which of the following is not a risk factor for an ischaemic stroke?

A. Age

B. Hypertension

C. Smoking

D. AV malformation

A

D - this is a RF for haemorrhagic stroke

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5
Q

Which of the following is not a risk factor for haemorrhagic stroke?

A. Age

B. Hypertension

C. Anticoagulation

D. Diabetes mellitus

A

D - this is a RF for ischaemic stroke

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6
Q

Which of the following is a feature of a brainstem infarction?

A. Contralateral hemiplegia

B. Quadriplegia

C. Pure motor

D. Dysphagia

A

B - Brainstem infarction = quadriplegia and locked-in-syndrome

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7
Q

Which of the following is not a typical feature of a lacunar infarct?

A. Pure motor

B. Pure sensory

C. Mixed motor and sensory

D. Dysphagia

A

D

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8
Q

What classification system can be used for strokes?

What are the criteria assessed?

A

Oxford Stroke Classification

Criteria:
1) Unilateral hemiparesis ± hemisensory loss of face/arms/legs

2) Homonymous hemianopia
3) Higher cognitive function e.g. dysphagia, dysarthria, planning, execution of complex events

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9
Q

What are the categories of stroke according to the Oxford Stroke Classification?

A

TACS 3/3

PACS 2/3

LACS

POCS

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10
Q

What are the features of TACS?

A

3/3 of criteria

Hemiparesis/hemisensory loss

Homonymous hemianopia

Loss of higher cerebral function

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11
Q

What are the features of PACS?

Which arteries are typically affected?

A

2/3

Smaller arteries of anterior circulation e.g. upper or lower division of MCA

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12
Q

What is a LACS?

A

Lacunar stroke involving perforating arteries around internal capsule, thalamus and basal ganglia

1 of the following:
Hemiparesis/ hemisensory loss

Pure sensory stroke

Ataxic hemiparesis

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13
Q

What is a POCS?

A

Posterior stroke involving vertebrobasilar arteries with 1 of the following:
Cerebellar syndrome

Loss of consciousness

Isolated homonymous hemianopia

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14
Q

Which artery is affected in a POCS?

A

Vertebrobasilar artery

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15
Q

What artery is affected in a TACS?

A

ACA or MCA

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16
Q

Which artery is affected in a PACS?

A

Smaller artery of anterior circulation e.g. upper/lower division of MCA

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17
Q

Which artery may be affected in a LACS?

A

Perforating arteries around internal capsule, thalamus and basal ganglia

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18
Q

What features may be more common or suggestive of a haemorrhagic stroke?

A

Symptoms alone cannot distinguish between haemorrhagic and ischaemic, need imaging.

N/V
Headache
Reduced consciousness (50%)
Seizures (25%)

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19
Q

What investigation may be used to distinguish between a haemorrhagic or ischaemic stroke?

A

CT-Head

MRI-Head

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20
Q

How is an ischaemic stroke managed?

A

Confirmed ischaemic stroke

Thrombolysis (Alteplase) within 4.5 hours of onset of symptoms

Aspirin 300mg

Thrombectomy within 6 hours

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21
Q

What scoring system can be used in a TIA to examine risk of stroke?

A

ABCD2

Age >60 years

BP >140/90

Clinical features

Duration of Sx

Diabetes mellitus

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22
Q

What is the management for TIA?

A

Aspirin 300mg

± Tx other causes
e.g. Carotid stenosis
/
Assessment by stroke physician within 24 hours if stroke within 7 days; stroke >7 days, see within 7 days

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23
Q

How is a haemorrhagic stroke managed?

A

Stop anticoagulants

Lower blood pressure

Surgical intervention

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24
Q

Which of the following is not an absolute contraindication to thrombolysis in a stroke?

A. Intracranial neoplasm

B. Lumbar puncture in last 7 days

C. GI bleed in last 3 months

D. Active diabetic haemorrhagic retinopathy

A

D - this is a relative contraindication

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25
Which of the following is not an absolute contraindication to thrombolysis in a stroke? A. Previous ICH B. Surgery in last 2 weeks C. Seizure at onset of stroke D. Uncontrolled hypertension >200/120mmHg
B - this is a relative contraindication
26
When should thrombectomy be offered?
Offer within 6 hours if confirmed occlusion of proximal anterior circulation Offer to those who were well between 6 and 24 hours but potential to save brain tissue as shown with limited infarct core volume Offer to those well 24 hours previously if confirmed occlusion of proximal posterior circulation and potential to salvage brain tissue evidence by limited infarct core volume
27
What is the recommended secondary prevention following a stroke?
``` Antiplatelets: Clopidogrel 75mg + Antihypertensives: Ramipril + Anti-lipids: Simvastatin 80mg ``` Note: Aspirin + Dipyridamole (200mg) recommended if clopidogrel not tolerated
28
When should you consider intervention for carotid artery stenosis?
Following a stroke/TIA in carotid territory Stenosis >70% (ECST criteria) or >50% (NASCET criteria)
29
What are the absolute contraindications for thrombolysis management?
Mnemonic: NIC BLS Neoplasm ICH CVA Bleeding Lumbar puncture (7 days) Seizure Surgery (3 months)
30
What is a crescendo TIA?
two or more TIAs within a week
31
What is the gold-standard imaging for a stroke?
Diffusion-weighted MRI
32
Draw out the Circle of Willis.
Mnemonic: Man with many limbs...
33
What changes may you see on a CT-head in an ischaemic stroke?
Low density in grey and white matter of the territory Hypersense artery sign
34
What radiographic features may you see in a CT-Head of a haemorrhagic stroke?
Hyperdense material (blood) surrounded by low density (oedema)
35
Which of the following is a feature of a stroke affecting the ACA? A. Contralateral hemiparesis and sensory loss with lower > upper B. Contralateral hemiparesis and sensory loss with upper > lower C. Homonymous hemianopia with macular sparing D. Amaurosis fugax
A
36
Which of the following is a feature of a stroke affecting the MCA? A. Contralateral hemiparesis and sensory loss with lower > upper B. Contralateral hemiparesis and sensory loss with upper > lower C. Homonymous hemianopia with macular sparing D. Amaurosis Fugax
B
37
Which of the following is a feature of a stroke affecting the PCA? A. Contralateral hemiparesis and sensory loss with lower > upper B. Contralateral hemiparesis and sensory loss with upper > lower C. Homonymous hemianopia with macular sparing D. Amaurosis fugax
C
38
What are the features of Weber's Syndrome?
Ipsilateral CN III palsy Contralateral weakness of upper and lower extremity
39
What are the features of Lateral Medullary Syndrome? Which arteries affected in this?
Ipsilateral facial pain and temperature loss Contralateral limb/torso pain and temperature loss Ataxia Nystagmus Artery: Posterior inferior cerebellar artery
40
What are the symptoms when a stroke affects the ophthalmic artery?
Amaurosis fugax
41
What occurs when a stroke affects the Basilar artery?
Locked-in-Syndrome
42
State 5 RFs for an intracranial bleed.
``` Head injury/trauma Hypertension Aneurysms Neoplasms Anticoagulation Ischaemic stroke progressing ```
43
What are the layers of the meninges?
Mnemonic: DAP ``` Skull Dura mater Arachnoid mater Pia mater Brain ```
44
How does a subdural haematoma appear on CT-Head?
Crescenteric shape - not limited by cranial sutures
45
What is the appearance of an extradural haemorrhage on CT?
Bi-convex e.g. egg shaped
46
How does a subarachnoid haemorrhage appear on CT-head?
Blood in basal cisterns, sulci and ventricular system
47
How and when is a SAH confirmed, in the absence of CT-head?
LP after 12 hours - allows Xanthochromia to develop and raised opening pressure
48
What are the causes of spontaneous SAH?
Intracranial aneurysm: PCKD; CT diseases; Coarctation of aorta ``` AV Malformation Pituitary apoplexy Arterial dissection Mycotic aneurysms Perimesencephalic (idiopathic venous bleed) ```
49
What is the management of a SAH?
Surgery: Coil/Craniotomy and clipping ± CCB: Nimodipine 21 days ± Hydrocephalus Surgery: Ventricular drain / VP Shunt
50
What are the complications of aneurysmal SAH?
Re-bleeding (10% in 12 hours) Vasospasm Hyponatraemia (SIADH) Seizures Hydrocephalus Death
51
Which of the following is not a risk factor for a SAH? A. FHx B. Cocaine use C. Diabetes D. Hypertension
C
52
What causes xanthochromia observed in SAH?
RBCs in the CSF with bilirubin present
53
Who is most at risk of Multiple Sclerosis?
Females 20-40 years old Higher latitudes
54
What are the types of MS?
Relapsing-remitting: acute attacks with period of remission Primary progressive disease: progressive from onset Secondary progressive disease: relapsing and remitting patient deteriorated and developed neurological signs - gait and bladder disorder seen
55
How is MS diagnosed?
MRI with 2+ lesions 2+ relapses with objective clinical evidence
56
What are the features of MS?
Visual: Optic neuritis Optic atrophy Uhthoff's phenomenon: worsened vision with rise in body temperature ``` Sensory: Pins/needles Numbness Trigeminal neuralgia Lhermitte's syndrome: neck flexion causing paraesthesia in limbs ``` ``` Motor: Motor weakness (commonly legs) ``` Cerebellar: Ataxia Tremor Other: Urinary incontinence Sexual dysfunction Intellectual deterioration
57
What investigations may be used to confirm MS?
MRI: High signal T2 lesions; periventricular plaques CSF: Oligoclonal bands; increased intrathecal synthesis of IgG Visual evoked potentials: Delayed but well preserved waveform
58
Outline the pathophysiology of MS.
In MS there is inflammation around the myelin with immune cell infiltration thus demyelination occurs resulting in neural symptoms. Some repair and re-myelination may occur but in later stages of the disease, re-myelination is incomplete with symptoms becoming permanent.
59
What is the management of MS? Talk through the management of acute relapse, DMD, and treating any symptoms.
Acute relapse Medical: High dose steroids PO/IV 5/7 DMDs ß-interferon + Tx symptoms Fatigue Medical: Amantadine Spasticity Medical: Baclofen; Gabapentin Urinary retention Medical: Anticholinergics / Intervention: Self-catheterisation (if US shows full bladder)
60
How is Oscillopsia managed in MS?
Gabapentin
61
What is the most common type of MND?
Amyotrophic Lateral Sclerosis (50%)
62
What are the classical features of Amyotrophic Lateral Sclerosis?
LMN signs in arms UMN signs in legs Gene lies on chromosome 21
63
What are the clinical features of Primary lateral sclerosis?
UMN signs only
64
What are the clinical features of Progressive Muscular Atrophy?
LMN signs only, affecting distal before proximal
65
What are the clinical features of progressive bulbar palsy?
Palsy of tongue, muscles of mastication and facial muscles
66
A 60 year old man presents with a slowly progressing speech problem. They have been more clumsy over the last few months, dropping things more often. They notice a weakness too. He says his uncle suffered from something similar. O/E you observe increased tone and brisk reflexes in the legs. Examination of the arms reveals muscle wasting and reduced tone with bradyreflexia. What is your diagnosis? A. Primary lateral sclerosis B. Progressive muscular atrophy C. Progressive bulbar palsy D. Amyotrophic lateral sclerosis
D - the feature of LMN in arms and UMN in legs.
67
A 60 year old man presents with a slowly progressing speech problem. They have been more clumsy over the last few months, dropping things more often. They notice a weakness too. He says his uncle suffered from something similar. O/E you observe increased tone and brisk reflexes in the legs. What is your diagnosis? A. Primary lateral sclerosis B. Progressive muscular atrophy C. Progressive bulbar palsy D. Amyotrophic lateral sclerosis
A - only UMN signs
68
A 60 year old man presents with a slowly progressing speech problem. They have been more clumsy over the last few months, dropping things more often. They notice a weakness too. He says his uncle suffered from something similar. O/E the hands and wrists reveals muscle wasting and reduced tone with bradyreflexia. What is your diagnosis? A. Primary lateral sclerosis B. Progressive muscular atrophy C. Progressive bulbar palsy D. Amyotrophic lateral sclerosis
B - LMN signs from the distal muscles
69
A 60 year old man presents with a slowly progressing speech problem. They have been more clumsy over the last few months, dropping things more often. They notice a weakness too. He says his uncle suffered from something similar. O/E you observe the tongue deviating as well as a facial palsy. What is your diagnosis? A. Primary lateral sclerosis B. Progressive muscular atrophy C. Progressive bulbar palsy D. Amyotrophic lateral sclerosis
C - palsy of the tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei
70
What is the management for MND?
Riluzole - slows progression Supportive: MDT; Advanced directives; EoL care
71
What is the MOA of Riluzole? A. SERM B. Aromatase inhibitor C. Glutamatergic antagonist D. Glutamatergic agonist
C - prevents stimulation of glutamate receptors Can prolong life by 3 months
72
Outline the pathophysiology of Parkinson's Disease.
Reduced DA in the substantia nigra of the basal ganglia therefore reduced coordination of movement patterns
73
What are the clinical features of Parkinson's Disease?
Triad: Bradykinesia + Rigidity + Unilateral tremor Tremor: 4-6Hz (per second) e.g. Pill rolling tremor Cogwheeling rigidity: tension in arm gives way to movement Bradykinesia: Shuffling gait; Simian swing; Hypomimia; Festinant gait ``` Other: Depression Anosmia Sleep disturbance and insomnia Postural instability Cognitive impairment and memory problems ```
74
State 5 differences between Parkinson's Tremor and Benign Essential Tremor.
PD is asymmetrical cf BET is symmetrical PD is 4-6Hz cf BET is 6-8Hz PD is at rest; BET is at movement PD worse at rest; BET worse with movement PD has other features BET improves with alcohol
75
Give 5 causes of Parkinsonism
``` Drug induced (Anti-psychotics; Metoclopramide) Toxins Wilson's Post-encephalitis Parkinson's disease Dementia pugilistica (secondary to chronic head trauma) Multiple system atrophy Progressive supra nuclear palsy ```
76
What is the management of Parkinson's Disease?
Synthetic dopamine: Levodopa + Peripheral carboxylase inhibitor: Carbidopa MAO-Bi: Selegiline; Rasagiline COMT-i: Entacapone DA agonist: Cabergoline
77
What is the MOA of Levodopa? Give the main side effects.
Synthetic dopamine to boost dopamine levels Side effects are dyskinesias: - Dystonia: excessive muscle contraction causes abnormal postures - Chorea: abnormal rhythmic jerky movements - Athetosis: involuntary twisting or writhing movements in fingers, hands or feet
78
What is the MOA of Entacapone?
COMT inhibitor to reduce enzyme metabolising levodopa
79
What is the MOA of Bromocriptine? Give a notable side effect of it.
Dopamine agonists ``` Sexual dysfunction Sleep disorders Movement disorders Hallucinations Pulmonary fibrosis ```
80
What is the MOA of Selegiline?
MAOb inhibitors
81
Which of the following is most associated with impulse control disorders? A. Cabergoline B. Entacapone C. Rasagiline D. Levodopa
A - DA agonist therapy
82
A patient who has recently started Levodopa therapy for their Parkinsons presents with dizziness on standing. What can be done to treat this?
Medicines review with potential reconciliation Midodrine (a1 agonist) thus increases arterial resistance
83
A patient has recently been prescribed a drug for their Parkinsons disease. They report with a lace-like rash on their legs which is red and white. Which drug was the patient most likely given? A. Levodopa B. Entacapone C. Rasageline D. Amantadine
D - Amantadine ``` SEs: Ataxia Slurred speech Confusion Dizziness Livedo reticularis ```
84
State 3 potential side effects of Levodopa.
Dyskinesias... ``` Dry mouth Anorexia Palpitations Postural hypotension Psychosis Drowsiness Impulse control disorder ``` Dystonia Chorea Athetosis
85
How should Levodopa be stopped?
Gradually - not acutely Can use TD patch to prevent acute dystonia
86
Which of the following is not a clinical feature of BET? A. 6-8Hz tremor B. Worsens when tired C. Symmetrical D. Present when sleeping
D - absent during sleep. Also improves with alcohol; worsens with fatigue, stress or caffeine
87
How is BET managed?
Propanolol Primidone
88
What is the cause of Epilepsy?
Abnormal electrical activity resulting in hyper excitation of cerebral neurones
89
What investigations are used in Epilepsy?
FBC; U+Es; TFTs; LFTs; CRP; EEG MRI-Brain ECG
90
List as many types of seizures as you know.
Generalised Tonic-Clonic Focal seizures Absence seizures Atonic seizures Infantile spasms (West's Syndrome) Lennox-Gustaut syndrome Benign rolandic epilepsy Juvenile myoclonic epilepsy Alcohol withdrawal seizures Febrile convulsions Psychogenic
91
Which of the following is false regarding febrile convulsions? A. 3% children have one febrile convulsion B. Seizures are brief and generalised tonic C. Occur usually in viral infection D. Occur up to 10 years old
D - occur up to 5 years old
92
When is the peak time for alcohol withdrawal seizures to occur?
36 hours
93
What may be seen in West's Syndrome?
Flexion of head, trunk and limbs Extension of arms (Salaam attack) lasting 1-2 seconds and repeated up to 50 times EEG shows Hypsarrhythmia
94
What may be seen in Lennox-Gustaut Syndrome?
Atypical absences, falls, jerky Occur in severely mentally handicapped EEG: slow spike Improved with ketogenic diet
95
What may be seen in a benign rolandic seizure?
Paraesthesia of the face
96
What may be seen in Juvenile myoclonic epilepsy?
Infrequent, generalised seizures (generally in am) Daytime absences Sudden shock like myoclonic seizures More common in girls
97
How is epilepsy managed?
Treat after 2nd seizure Generally: Generalised seizure = Sodium valproate Focal seizure = Carbamazepine Supportive: Stop driving till 12 months free; Pregnancy counselling; Medicine reconciliation
98
What is the MOA of Sodium valproate?
GABA agonist
99
Which of the following is not a side effect of Sodium valproate? A. Weight gain B. Alopecia C P450 enzyme inducer D. Pancreatitis
C - enzyme inhibitor
100
Which of the following is not a side effect of Sodium valproate? A. Weight gain B. Alopecia C P450 enzyme inhibitor D. Thormbocythemia
D - thrombocytopenia
101
Which of the following is not a side effect of Carbamazepine? A. P450 enzyme inducer B. Leucopnia C. SIADH D. Alertness
D - drowsiness
102
What is the MOA of Carbamazepine?
Anti-epileptic which inactivates NaVg to increase refractory period
103
Which of the following is a side effect of Lamotrigine? A. P450 enzyme inducer B. Visual disturbances C. Agranulocytosis D. SJS
D
104
Which of the following is Phenytoin not associated with? A. P450 enzyme inhibitor B. Ataxia C. Gingival hyperplasia D. Peripheral neuropathy
A - P450 enzyme inducer
105
How long should a patient experiencing a seizure not drive for?
First seizure in absence of investigation finds = 6 months Epilepsy = 12 months from any seizure Withdrawing epilepsy medication: 6 months after last dose
106
how long should a patient stop driving after a single stroke? A. 3 months B. 1 months C. 6 months D. 2 weeks
B
107
how long should a patient stop driving after a single TIA? A. 3 months B. 1 months C. 6 months D. 2 weeks
B
108
how long should a patient stop driving after a three strokes? A. 3 months B. 1 months C. 6 months D. 2 weeks
A
109
how long should a patient stop driving after a craniotomy for meningioma? A. 3 months B. 1 months C. 6 months D. 12 months
D
110
how long should a patient stop driving after a transsphenoidal pituitary adenectomy? A. 3 months B. 1 months C. 6 months D. 2 weeks
C
111
What is the definition of Status Epilepticus?
defined as seizures lasting more than 5 minutes or more than 3 seizures in one hour.
112
How do you manage Status Epilepticus?
A-E Help Safe environment Secure airway IV Lorazepam 4mg, repeat after 10 minutes
113
What are the clinical features of neuropathic pain?
Burning Tingling Pins and needles Electric shocks §
114
What tool may be used to assess neuropathic pain?
DN4 questionnaire
115
What medication can be used for Neuropathic pain?
Amitryptiline Duloxetine Gabapentin Pregabalin Carbamazepine (Trigeminal neuralgia) Tramadol
116
Describe Complex Regional Pain Syndrome.
Affected by abnormal nerve functioning causing neuropathic pain and abnormal sensation - often triggered by an injury to the area
117
What are the clinical features of Fibromyalgia?
Chronic pain at multiple sites (9/18 tender points) Lethargy Cognitive impairment ("fibrofog") Sleep disturbance, headaches, dizziness
118
What are the clinical features of complex regional pain syndrome?
``` Progressive, disproportionate symptoms to original injury Allodynia Temperature and skin colour changes Oedema and sweating Motor dysfunction ```
119
Where does the facial nerve exist the brainstem?
Cerebellopontine angle
120
Which of the following is not a motor supply from the facial nerve? A. Stapedius B. Posterior digastric C. Orbicularis oculi D. Sphincter dilator pupillae
D
121
What is the sensory innervation of the facial nerve?
Anterior 2/3 of tongue
122
What is the parasympathetic innervation of the facial nerve?
Submandibular and sublingual glands Lacrimal gland
123
How can you discriminate between an UMN and LMN lesion of the facial nerve?
UMN lesion forehead is spared due to contralateral UMN innervation. LMN lesion forehead not spared due to whole neuronal innervation knocked out
124
Give an example of a bilateral upper motor neurone lesion.
Pseudobulbar palsy Motor neurone disease
125
How are Bell's palsies managed?
If presenting within 3 days Prednisolone 50mg 10/7 + Eye drops (avoid exposure keratopathy)
126
What is the management for Ramsay-Hunt syndrome?
Within 72 hours give: Prednisolone + Aciclovir
127
Give 5 causes of a Lower Motor Neurone Facial Nerve palsy
Infection: Otitis media HIV Lyme's disease ``` Systemic disease: Diabetes Sarcoidosis Leukaemia MS Guillain-Barre syndrome ``` Tumours: Acoustic neuroma Parotid tumour Cholesteatomas Trauma: Direct nerve trauma Damage during surgery Base of skull fracture
128
How may a brain tumour present?
Raised ICP - headaches; visual defects; ptosis; CN3 and CN6 palsies; seizures and papilloedema Sx dependent on where lesion is e.g. frontal lobe = behavioural change
129
What are the most common cancers to metastasise to the brain?
``` Lung Breast Bowel Kidney Melanoma ```
130
An adult has a CT with contrast, showing a solid tumour with central necrosis. The rim is enhanced. Biopsy of a brain tumour shows pleomorphic tumour cells with bordering necrotic areas. What is your differential? A. Glioblastoma multiforme B. Meningioma C. Pilocytic astrocytoma D. Medulloblastoma
A
131
An adult has a CT-head showing contrast enhancement of a lesion located at the falx cerebri. Histology shows spindl cells and calcified psammoma bodies. What is your differential? A. Ependyoma B. Meningioma C. Pilocytic astrocytoma D. Medulloblastoma
B
132
What condition is associated with bilateral vestibular schwannomas?
NF type 2
133
A tumour is seen at the cerbellopontine angle. Histology shows Antoni A patterns. What is your differential?
Vestibular schwannoma
134
A brain tumour in a child is biopsied which shows Rosenthal fibres of eosinophilic bundles. What is your differential? A. Glioblastoma multiforme B. Meningioma C. Pilocytic astrocytoma D. Medulloblastoma
C
135
A child has a suspected SOL. Biopsy shows small, blue cells in a Rosette pattern. What is your differential? A. Glioblastoma multiforme B. Meningioma C. Pilocytic astrocytoma D. Medulloblastoma
D
136
Where is an Ependymoma commonly seen?
4th ventricle
137
A biopsy of a SOL in the brain shows calcifications with a fried-egg appearance. What is your DDx? A. Meningioma B. Glioblastoma multiforme C. Ependymoma D. Oligodendroma
D
138
What type of brain tumour is VHL associated with?
Hamaengioblastoma
139
What is the most common paediatric supratentorial tumour? A. Craniopharyngioma B. Medulloblastoma C. Pilocytic astrocytoma D. Ependymoma
A - solid/cystic tumour derived from Rathke's pouch
140
How is Huntington's chorea transmitted?
Autosomal dominant from HTT gene on Chromosome 4
141
What are the clinical features of Huntington's chorea?
Chorea Eye movement disorders Dysarthria Dysphagia
142
How is Huntington's Chorea managed?
Supportive: MDT; SLT; Genetic counselling; advanced directives; EoL care + Medical: Benzodiazepines; SSRs; Antipsychotics
143
Outline the pathophysiology of Myaesthenia Gravis.
In around 85% of patients with myasthenia gravis, acetylcholine receptor antibodies are produced by the immune system. These bind to the postsynaptic neuromuscular junction receptors. This blocks the receptor and prevents the acetylcholine from being able to stimulate the receptor and trigger muscle contraction. As the receptors are used more during muscle activity, more of them become blocked up. This leads to less effective stimulation of the muscle with increased activity. There is more muscle weakness the more the muscles are used. This improves with rest as more receptors are freed up for use again.
144
which antibodies can cause Myaesthenia Gravis?
Anti-acetylcholine receptor antibodies MuSK LRP4 Abs
145
What are the clinical features of Myaesthenia gravis?
Extraocular muscle weakness causing double vision (diplopia) Eyelid weakness causing drooping of the eyelids (ptosis) Weakness in facial movements Difficulty with swallowing Fatigue in the jaw when chewing Slurred speech Progressive weakness with repetitive movements
146
What drugs may exacerbate myasthenia gravis?
``` Penicillamine Procainamide Beta blockers Lithium Phenytoin ABX: Gentamicin; Macrolides; Quinolones; Tetracyclines ```
147
What is Myasthenia gravis associated with?
Thymomas Autoimmune conditions Thymic hyperplasia
148
What is the management of Myasthenia Gravis?
Pyridostigmine Thymectomy
149
What is the management of a myasthenic crisis?
Plasmapheresis IVIG
150
In which patients does Lamberton-Eaton Syndrome occur?
Cancers: SCLC; Breast and Ovarian cancer Abs agains CaVg channels
151
What is the main distinguishing feature between Myasthenia gravis and Lambert-Eaton syndrome?
Increased contractions lead to increased muscle strength
152
How is Charmot-Marie-Tooth disease inherited?
Autosomal dominant pattern
153
What are the clinical features of CMT disease?
``` Pes cavus Inverted champagne bottle legs Atrophy Reduced tone Bradyreflexia Peripheral sensory loss ``` LMN signs essentially...
154
Give 5 causes of Peripheral Neuropathy.
A-E ``` Alcohol B12 deficiency Cancer/CKD Diabetes/Drugs (isoniazid; amiodarone; cisplatin) Every vasculitis ```
155
How is CMT managed?
Supportive management: MDT; Physio; OT; podiatry; Tx Sx
156
Which of the following pathogens is not associated with Guillain Barre Syndrome development? A. C jejuni B. S pyogenes C. EBV D. CMV
B
157
Outline the pathophysiology of Guillain-Barre syndrome.
B cells produce Abs against antigen of pathogen however these mimic the proteins on nerve cells which target myelin sheath causing demyelination of the peripheral nervous system
158
What is Miller Fisher syndrome?
Guillain-Barre syndrome variant featuring ophthalmoplegia, areflexia and ataxia Anti-GQ1b Abs present
159
How does Guillain-Barre syndrome present?
Symmetrical ascending weakness Bradyreflexia Paresthesia/Pain The Sx occur 4 weeks after preceding infection Recovery period last months to years
160
What investigations are conducted in Guillain-Barre syndrome?
LP: Rise in protein with normal WBC Nerve conduction studies: reduced motor nerve conduction velocity
161
How is Guillain-Barre syndrome managed?
IVIG/ Plasma exchange VTE prophylaxis Tx symptoms
162
What is the prognosis of Guillain-Barre syndrome?
80:20 80% fully recover 15% left with impairment 5% will die
163
What does the NF1 gene code for?
Neurofibromin (Chr 17) - a TS protein
164
What does the NF2 gene code for?
Merlin (Chr 22) - a TS protein important in Schwann cells
165
What are the clinical features of NF type 1?
Mnemonic: CRABBING ``` Cafe-au-lait spots: 6+ Relative with NF1 Axillary/Inguinal freckles Bony dysplasia/Bowing Iris hamartomas (Lisch nodules) Neurofibromas: 2+ Glioma of optic nerve ```
166
How is NF type 1 diagnosed/ A. Genetic B. XR C. CT D. Clinical
D
167
What are the clinical features of NF type 2?
Acoustic neuromas Hearing loss Tinnitus Balance problems Schwannomas Meningiomas Ependymomas
168
What are the clinical features of Tuberous Sclerosis?
ASHLEAF ``` Ashleaf spots (depigmented areas) Shagreen patches (thick, dimpled pigmented patches) Heart rhabdomyomas Lung lymphangioleiomyomatosis Epilepsy Angiomyolipoma (in kidney) Facial angiofibromas ``` Poliosis
169
How is Tuberous Sclerosis managed?
Supportive + Tx Sx
170
What chromosome is the TSC 1 gene on?
Chromosome 9 - hamartin
171
What chromosome is TSC 2 gene on?
Chromosome 16 - codes for tuberin
172
What are the red flags of headache?
``` Visual changes Dizziness Vomiting Worse on coughing or straining Worse position change Focal neurological symptoms Wakes from sleeping Trauma Pregnancy ```
173
What are the clinical features of tension headaches?
Distributed band-like across head Gradual onset and relieve Associated with: stress/MHD/alcohol/skipping meals/dehydration
174
What is a secondary headache?
Occurs following another cause Medical condition Alcohol Head injury CO poisoning
175
What is an analgesic headache?
Medication overuse - using analgesia 15 times in 1 month
176
What is a hormonal headache?
Relates to oestrogen levels - low oestrogen thus pre-menstrual/around menopause or in pregnancy
177
How is Trigeminal neuralgia managed?
Carbamazepine
178
What are the clinical features of cluster headache?
Come in clusters of attacks then disappear Middle aged male smoker ``` Red, swollen and watering eye Miosis Ptosis Nasal discharge Facial sweating ```
179
How is a cluster headache managed acutely?
Triptans + High flow Oxygen
180
Which of the following is not a common prophylactic treatment of Cluster headaches? A. Verapamil B. Lithium C. Prednisolone D. Triptans
D - Triptans TREAT thus acute
181
Name the types of Migraine.
Migraine with aura Migraine without aura Hemiplegic migraine Silent migraine
182
What are the clinical features of migraine?
``` Moderate-severe unilateral pain Pounding/throbbing Photophobia Phonophobia With or without aura N/V ```
183
What are the potential triggers of a migraine? Give 5.
``` Chocolate Cheese Caffeine Dehydration Menstruation Abnormal sleep patterns Trauma Stress Bright lights ```
184
What can be used as migraine prophylaxis?
Propanolol Topiramate Amitriptyline
185
What can be used to treat an acute Migraine?
Triptans - 5HT3 agonists
186
An 81-year-old woman is brought to her general practitioner by her concerned son who reports that she has been unable to cope at home following an acute ischaemic stroke she suffered 8 weeks ago. The son supplies the general practitioner with a CT brain report that confirms a lesion was identified within the M1 segment of the left middle cerebral artery. During the consultation, the general practitioner performs a mini-mental state examination (MMSE). The woman appears to understand all of the instructions and performs well. She speaks fluently but has difficulty repeating 'apple, table, penny' immediately after hearing the phrase. What is the most accurate description of the woman's language deficit? A. Broca's dysphasia B. Conduction dysphasia C. Dysarthria D. Wernicke's dysphasia
B - speech fluent but repetition poor. Comprehension intact.
187
Which type of aphasia occurs due to a lesion of the superior temporal gyrus?
Wernicke's (receptive): Lesion in superior temporal gyrus supplied by inferior division of left MCA. Forms speech before sending it to Broca's area. Comprehension impaired
188
Which type of aphasia occurs following a lesion to the inferior frontal gyrus?
Broca's (expressive) aphasia due to lesion of inferior frontal gyrus supplied by superior division of left MCA Note: Supplied by the superior division as it is better to speak than think Speech non-fluent, laboured and halting. Repetition impaired. Comprehension normal
189
Which type of aphasia occurs following damage to the arcuate fasciculus?
Conduction aphasia. Speech fluent but repetition poor. Comprehension normal
190
What type of aphasia occurs if all 3 areas, inferior temporal gyrus, superior temporal gyrus and arcuate fascicles is affected?
Global aphasia with severe expressive and receptive aphasia. May still be able to communicate with gestures
191
A 23-year-old man presents to his GP. He describes episodes of leg weakness following bouts of laughing whilst out with friends. The following weekend his friends described a brief collapse following a similar episode. What is the most likely diagnosis?
Cataplexy
192
Explain Autonomic dysreflexia.
Clinical syndrome following SCI at T6 or above. Sympathetic spinal reflex via thoracolumbar outflow because parasympathetic response is prevents thus unbalanced physiological response with: hypertension, flushing, sweating above the level of lesion and agitation. Stimuli could be faecal impaction, urinary retention etc
193
How does Syringomyelia present?
Classically presents with cape-like loss of pain and temperature sensation due to compression of the spinothalamic tract fibres decussating in the anterior white commissure of the spine
194
A 21-year-old woman presents to the emergency department with temporary loss of vision in her right eye. This occurred gradually over the last few hours however her vision has now returned. A lumbar puncture is performed during her diagnostic workup. Following this, she develops a headache which is worse on standing. What is the most appropriate treatment for her headache over the next few days?
Caffeine and fluids for the low pressure headache
195
A 65-year-old male presents to the emergency department with severe pain behind his right eye for the last 40 minutes. He states that his right eye has also been ‘tearing excessively’ for the last three hours. The man has had similar episodes like this over the last few weeks, but this time the pain is significantly worse. His past medical history is significant for coronary artery disease for which he had a coronary artery bypass graft (CABG) last year and he also suffers from asthma. He is not compliant with his medication regimen. He has normal vital signs. Ophthalmic examination demonstrates conjunctival injection and miosis of the right pupil. Neurological examination is normal. A computed tomography (CT) scan of the brain and erythrocyte sedimentation rate (ESR) are unremarkable. Which of the following is the most appropriate treatment for this patient?
High flow O2 Both O2 and Triptans may be used in cluster headaches but Triptans CI in CAD due to risk of coronary vasospasm
196
A 71-year-old man is reviewed following an ischaemic stroke. He is known to be intolerant of clopidogrel. What is the most appropriate therapy to help reduce his chance of having a further stroke?
Aspirin + Dipyramidole lifelong No longer a 2 year limit
197
Following a stroke, what is the threshold regarding commencement of Statins in secondary prevention?
>3.5mmol/L
198
A 34-year-old lady is blue-lighted to the emergency department with sudden onset left sided weakness of her arm and leg. Her past medical history consists of anxiety and depression. An urgent CT head scan was performed which showed no acute intracranial abnormality and no evidence of any intracranial haemorrhage. A subsequent MRI brain scan was performed to rule out any underlying inflammatory process but this was also unremarkable. What clinical sign could be examined for in this patient to further evaluate whether this is an organic or functional presentation?
Hoover's Sign
199
A 48-year-old gentleman was blue lighted to the nearest emergency department with sudden onset right sided weakness, slurred speech and right facial droop. An urgent CT head scan was performed which showed no acute intracranial abnormality. His ECG showed sinus rhythm and an x-ray of his chest showed clear lung fields. He presented within the thrombolysis window and received IV Alteplase. He demonstrated a considerable improvement in his symptoms 24 hours later. His past medical history was nil. What other tests would be important to further investigate the cause of a stroke given his age?
Thrombophilia screen Antibody screen
200
A 28-year-old female presents with a two-day history of a gradual onset severe headache associated with nausea and three episodes of vomiting. She comes across as blunted and states she is having difficulty in finding the right words. She has no significant past medical history and her only medication is the combined oral contraceptive pill. The only thing of note in her family history is that her mother had an unprovoked DVT in her 30s. Given the likely diagnosis, what is the gold standard test to diagnose her condition?
MR Venogram Done after CT Head
201
What are the clinical features of a Temporal lobe seizure?
Mnemonic: HEAD Hallucinations Epigastric rising/Emotional Automatisms Deja vu/ Dysphasia post-ictal
202
What are the clinical features of a Parietal lobe seizure?
Paraesthesia
203
What are the clinical features of an Occipital lobe seizure?
Visual changes
204
What are the clinical features of a Frontal lobe seizure?
Weakness in hands or legs Jacksonian march Post-ictal weakness
205
A 32-year-old window cleaner is admitted after falling off the roof. He reports that he had slipped off the top of the roof and was able to cling onto the gutter for a few seconds. The patient has Horner's syndrome What is the name of this palsy and which nerve roots are affected?
Klumpke's palsy Brachial trunks C8-T1