Neurology Flashcards
What are the two main types of stroke?
Ischaemic (85%): blockage in BV
Haemorrhagic (15%): burst in BV
What are the types of ischaemic stroke?
Thrombolic stroke: thrombosis from large vessels
Embolic stroke: Blood/fat/bacteria breaks away and translocates
–> Think AF
What are the types of haemorrhagic stroke?
Intracerebral haemorrhage: bleeding within the brain
Subarachnoid haemorrhage: bleeding on surface of brain
Which of the following is not a risk factor for an ischaemic stroke?
A. Age
B. Hypertension
C. Smoking
D. AV malformation
D - this is a RF for haemorrhagic stroke
Which of the following is not a risk factor for haemorrhagic stroke?
A. Age
B. Hypertension
C. Anticoagulation
D. Diabetes mellitus
D - this is a RF for ischaemic stroke
Which of the following is a feature of a brainstem infarction?
A. Contralateral hemiplegia
B. Quadriplegia
C. Pure motor
D. Dysphagia
B - Brainstem infarction = quadriplegia and locked-in-syndrome
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
D
What classification system can be used for strokes?
What are the criteria assessed?
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
What are the categories of stroke according to the Oxford Stroke Classification?
TACS 3/3
PACS 2/3
LACS
POCS
What are the features of TACS?
3/3 of criteria
Hemiparesis/hemisensory loss
Homonymous hemianopia
Loss of higher cerebral function
What are the features of PACS?
Which arteries are typically affected?
2/3
Smaller arteries of anterior circulation e.g. upper or lower division of MCA
What is a LACS?
Lacunar stroke involving perforating arteries around internal capsule, thalamus and basal ganglia
1 of the following:
Hemiparesis/ hemisensory loss
Pure sensory stroke
Ataxic hemiparesis
What is a POCS?
Posterior stroke involving vertebrobasilar arteries with 1 of the following:
Cerebellar syndrome
Loss of consciousness
Isolated homonymous hemianopia
Which artery is affected in a POCS?
Vertebrobasilar artery
What artery is affected in a TACS?
ACA or MCA
Which artery is affected in a PACS?
Smaller artery of anterior circulation e.g. upper/lower division of MCA
Which artery may be affected in a LACS?
Perforating arteries around internal capsule, thalamus and basal ganglia
What features may be more common or suggestive of a haemorrhagic stroke?
Symptoms alone cannot distinguish between haemorrhagic and ischaemic, need imaging.
N/V
Headache
Reduced consciousness (50%)
Seizures (25%)
What investigation may be used to distinguish between a haemorrhagic or ischaemic stroke?
CT-Head
MRI-Head
How is an ischaemic stroke managed?
Confirmed ischaemic stroke
Thrombolysis (Alteplase) within 4.5 hours of onset of symptoms
Aspirin 300mg
Thrombectomy within 6 hours
What scoring system can be used in a TIA to examine risk of stroke?
ABCD2
Age >60 years
BP >140/90
Clinical features
Duration of Sx
Diabetes mellitus
What is the management for TIA?
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
How is a haemorrhagic stroke managed?
Stop anticoagulants
Lower blood pressure
Surgical intervention
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
D - this is a relative contraindication
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
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
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
When should you consider intervention for carotid artery stenosis?
Following a stroke/TIA in carotid territory
Stenosis >70% (ECST criteria) or >50% (NASCET criteria)
What are the absolute contraindications for thrombolysis management?
Mnemonic: NIC BLS
Neoplasm
ICH
CVA
Bleeding
Lumbar puncture (7 days)
Seizure
Surgery (3 months)
What is a crescendo TIA?
two or more TIAs within a week
What is the gold-standard imaging for a stroke?
Diffusion-weighted MRI
Draw out the Circle of Willis.
Mnemonic: Man with many limbs…
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
What radiographic features may you see in a CT-Head of a haemorrhagic stroke?
Hyperdense material (blood) surrounded by low density (oedema)
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
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
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
What are the features of Weber’s Syndrome?
Ipsilateral CN III palsy
Contralateral weakness of upper and lower extremity
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
What are the symptoms when a stroke affects the ophthalmic artery?
Amaurosis fugax
What occurs when a stroke affects the Basilar artery?
Locked-in-Syndrome
State 5 RFs for an intracranial bleed.
Head injury/trauma Hypertension Aneurysms Neoplasms Anticoagulation Ischaemic stroke progressing
What are the layers of the meninges?
Mnemonic: DAP
Skull Dura mater Arachnoid mater Pia mater Brain
How does a subdural haematoma appear on CT-Head?
Crescenteric shape - not limited by cranial sutures
What is the appearance of an extradural haemorrhage on CT?
Bi-convex e.g. egg shaped
How does a subarachnoid haemorrhage appear on CT-head?
Blood in basal cisterns, sulci and ventricular system
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
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)
What is the management of a SAH?
Surgery: Coil/Craniotomy and clipping
±
CCB: Nimodipine 21 days
± Hydrocephalus
Surgery: Ventricular drain / VP Shunt
What are the complications of aneurysmal SAH?
Re-bleeding (10% in 12 hours)
Vasospasm
Hyponatraemia (SIADH)
Seizures
Hydrocephalus
Death
Which of the following is not a risk factor for a SAH?
A. FHx
B. Cocaine use
C. Diabetes
D. Hypertension
C
What causes xanthochromia observed in SAH?
RBCs in the CSF with bilirubin present
Who is most at risk of Multiple Sclerosis?
Females
20-40 years old
Higher latitudes
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
How is MS diagnosed?
MRI with 2+ lesions
2+ relapses with objective clinical evidence
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
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
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.
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)
How is Oscillopsia managed in MS?
Gabapentin
What is the most common type of MND?
Amyotrophic Lateral Sclerosis (50%)
What are the classical features of Amyotrophic Lateral Sclerosis?
LMN signs in arms
UMN signs in legs
Gene lies on chromosome 21
What are the clinical features of Primary lateral sclerosis?
UMN signs only
What are the clinical features of Progressive Muscular Atrophy?
LMN signs only, affecting distal before proximal
What are the clinical features of progressive bulbar palsy?
Palsy of tongue, muscles of mastication and facial muscles
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.
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
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
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
What is the management for MND?
Riluzole - slows progression
Supportive: MDT; Advanced directives; EoL care
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
Outline the pathophysiology of Parkinson’s Disease.
Reduced DA in the substantia nigra of the basal ganglia therefore reduced coordination of movement patterns
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
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
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
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
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
What is the MOA of Entacapone?
COMT inhibitor to reduce enzyme metabolising levodopa
What is the MOA of Bromocriptine?
Give a notable side effect of it.
Dopamine agonists
Sexual dysfunction Sleep disorders Movement disorders Hallucinations Pulmonary fibrosis
What is the MOA of Selegiline?
MAOb inhibitors
Which of the following is most associated with impulse control disorders?
A. Cabergoline
B. Entacapone
C. Rasagiline
D. Levodopa
A - DA agonist therapy
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
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
State 3 potential side effects of Levodopa.
Dyskinesias…
Dry mouth Anorexia Palpitations Postural hypotension Psychosis Drowsiness Impulse control disorder
Dystonia
Chorea
Athetosis
How should Levodopa be stopped?
Gradually - not acutely
Can use TD patch to prevent acute dystonia
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
How is BET managed?
Propanolol
Primidone
What is the cause of Epilepsy?
Abnormal electrical activity resulting in hyper excitation of cerebral neurones
What investigations are used in Epilepsy?
FBC; U+Es; TFTs; LFTs; CRP;
EEG
MRI-Brain
ECG
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
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
When is the peak time for alcohol withdrawal seizures to occur?
36 hours
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
What may be seen in Lennox-Gustaut Syndrome?
Atypical absences, falls, jerky
Occur in severely mentally handicapped
EEG: slow spike
Improved with ketogenic diet
What may be seen in a benign rolandic seizure?
Paraesthesia of the face
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
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
What is the MOA of Sodium valproate?
GABA agonist
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
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
Which of the following is not a side effect of Carbamazepine?
A. P450 enzyme inducer
B. Leucopnia
C. SIADH
D. Alertness
D - drowsiness
What is the MOA of Carbamazepine?
Anti-epileptic which inactivates NaVg to increase refractory period
Which of the following is a side effect of Lamotrigine?
A. P450 enzyme inducer
B. Visual disturbances
C. Agranulocytosis
D. SJS
D
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
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
how long should a patient stop driving after a single stroke?
A. 3 months
B. 1 months
C. 6 months
D. 2 weeks
B
how long should a patient stop driving after a single TIA?
A. 3 months
B. 1 months
C. 6 months
D. 2 weeks
B
how long should a patient stop driving after a three strokes?
A. 3 months
B. 1 months
C. 6 months
D. 2 weeks
A
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
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
What is the definition of Status Epilepticus?
defined as seizures lasting more than 5 minutes or more than 3 seizures in one hour.
How do you manage Status Epilepticus?
A-E
Help
Safe environment
Secure airway
IV Lorazepam 4mg, repeat after 10 minutes
What are the clinical features of neuropathic pain?
Burning
Tingling
Pins and needles
Electric shocks §
What tool may be used to assess neuropathic pain?
DN4 questionnaire
What medication can be used for Neuropathic pain?
Amitryptiline
Duloxetine
Gabapentin
Pregabalin
Carbamazepine (Trigeminal neuralgia)
Tramadol
Describe Complex Regional Pain Syndrome.
Affected by abnormal nerve functioning causing neuropathic pain and abnormal sensation - often triggered by an injury to the area
What are the clinical features of Fibromyalgia?
Chronic pain at multiple sites (9/18 tender points)
Lethargy
Cognitive impairment (“fibrofog”)
Sleep disturbance, headaches, dizziness
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
Where does the facial nerve exist the brainstem?
Cerebellopontine angle
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
What is the sensory innervation of the facial nerve?
Anterior 2/3 of tongue
What is the parasympathetic innervation of the facial nerve?
Submandibular and sublingual glands
Lacrimal gland
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
Give an example of a bilateral upper motor neurone lesion.
Pseudobulbar palsy
Motor neurone disease
How are Bell’s palsies managed?
If presenting within 3 days
Prednisolone 50mg 10/7
+
Eye drops (avoid exposure keratopathy)
What is the management for Ramsay-Hunt syndrome?
Within 72 hours give:
Prednisolone
+
Aciclovir
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
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
What are the most common cancers to metastasise to the brain?
Lung Breast Bowel Kidney Melanoma
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
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
What condition is associated with bilateral vestibular schwannomas?
NF type 2
A tumour is seen at the cerbellopontine angle. Histology shows Antoni A patterns.
What is your differential?
Vestibular schwannoma
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
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
Where is an Ependymoma commonly seen?
4th ventricle
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
What type of brain tumour is VHL associated with?
Hamaengioblastoma
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
How is Huntington’s chorea transmitted?
Autosomal dominant from HTT gene on Chromosome 4
What are the clinical features of Huntington’s chorea?
Chorea
Eye movement disorders
Dysarthria
Dysphagia
How is Huntington’s Chorea managed?
Supportive: MDT; SLT; Genetic counselling; advanced directives; EoL care
+
Medical: Benzodiazepines; SSRs; Antipsychotics
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.
which antibodies can cause Myaesthenia Gravis?
Anti-acetylcholine receptor antibodies
MuSK
LRP4 Abs
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
What drugs may exacerbate myasthenia gravis?
Penicillamine Procainamide Beta blockers Lithium Phenytoin ABX: Gentamicin; Macrolides; Quinolones; Tetracyclines
What is Myasthenia gravis associated with?
Thymomas
Autoimmune conditions
Thymic hyperplasia
What is the management of Myasthenia Gravis?
Pyridostigmine
Thymectomy
What is the management of a myasthenic crisis?
Plasmapheresis
IVIG
In which patients does Lamberton-Eaton Syndrome occur?
Cancers: SCLC; Breast and Ovarian cancer
Abs agains CaVg channels
What is the main distinguishing feature between Myasthenia gravis and Lambert-Eaton syndrome?
Increased contractions lead to increased muscle strength
How is Charmot-Marie-Tooth disease inherited?
Autosomal dominant pattern
What are the clinical features of CMT disease?
Pes cavus Inverted champagne bottle legs Atrophy Reduced tone Bradyreflexia Peripheral sensory loss
LMN signs essentially…
Give 5 causes of Peripheral Neuropathy.
A-E
Alcohol B12 deficiency Cancer/CKD Diabetes/Drugs (isoniazid; amiodarone; cisplatin) Every vasculitis
How is CMT managed?
Supportive management: MDT; Physio; OT; podiatry; Tx Sx
Which of the following pathogens is not associated with Guillain Barre Syndrome development?
A. C jejuni
B. S pyogenes
C. EBV
D. CMV
B
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
What is Miller Fisher syndrome?
Guillain-Barre syndrome variant featuring ophthalmoplegia, areflexia and ataxia
Anti-GQ1b Abs present
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
What investigations are conducted in Guillain-Barre syndrome?
LP: Rise in protein with normal WBC
Nerve conduction studies: reduced motor nerve conduction velocity
How is Guillain-Barre syndrome managed?
IVIG/ Plasma exchange
VTE prophylaxis
Tx symptoms
What is the prognosis of Guillain-Barre syndrome?
80:20
80% fully recover
15% left with impairment
5% will die
What does the NF1 gene code for?
Neurofibromin (Chr 17) - a TS protein
What does the NF2 gene code for?
Merlin (Chr 22) - a TS protein important in Schwann cells
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
How is NF type 1 diagnosed/
A. Genetic
B. XR
C. CT
D. Clinical
D
What are the clinical features of NF type 2?
Acoustic neuromas
Hearing loss
Tinnitus
Balance problems
Schwannomas
Meningiomas
Ependymomas
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
How is Tuberous Sclerosis managed?
Supportive
+ Tx Sx
What chromosome is the TSC 1 gene on?
Chromosome 9 - hamartin
What chromosome is TSC 2 gene on?
Chromosome 16 - codes for tuberin
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
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
What is a secondary headache?
Occurs following another cause
Medical condition
Alcohol
Head injury
CO poisoning
What is an analgesic headache?
Medication overuse - using analgesia 15 times in 1 month
What is a hormonal headache?
Relates to oestrogen levels - low oestrogen thus pre-menstrual/around menopause or in pregnancy
How is Trigeminal neuralgia managed?
Carbamazepine
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
How is a cluster headache managed acutely?
Triptans
+
High flow Oxygen
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
Name the types of Migraine.
Migraine with aura
Migraine without aura
Hemiplegic migraine
Silent migraine
What are the clinical features of migraine?
Moderate-severe unilateral pain Pounding/throbbing Photophobia Phonophobia With or without aura N/V
What are the potential triggers of a migraine? Give 5.
Chocolate Cheese Caffeine Dehydration Menstruation Abnormal sleep patterns Trauma Stress Bright lights
What can be used as migraine prophylaxis?
Propanolol
Topiramate
Amitriptyline
What can be used to treat an acute Migraine?
Triptans - 5HT3 agonists
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.
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
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
Which type of aphasia occurs following damage to the arcuate fasciculus?
Conduction aphasia. Speech fluent but repetition poor. Comprehension normal
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
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
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
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
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
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
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
Following a stroke, what is the threshold regarding commencement of Statins in secondary prevention?
> 3.5mmol/L
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
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
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
What are the clinical features of a Temporal lobe seizure?
Mnemonic: HEAD
Hallucinations
Epigastric rising/Emotional
Automatisms
Deja vu/ Dysphasia post-ictal
What are the clinical features of a Parietal lobe seizure?
Paraesthesia
What are the clinical features of an Occipital lobe seizure?
Visual changes
What are the clinical features of a Frontal lobe seizure?
Weakness in hands or legs
Jacksonian march
Post-ictal weakness
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
