Passmed wrong answers Flashcards

(173 cards)

1
Q

What are the typical localising features of focal seizures?

A
  1. Temporal lobe
    May occur with or without impairment of consciousness or awareness

An aura occurs in most patients
- typically a rising epigastric sensation
- also psychic or experiential phenomena, such as déjà vu, jamais vu
- less commonly hallucinations (auditory/gustatory/olfactory)

Seizures typically last around one minute
- automatisms (e.g. lip smacking/grabbing/plucking) are common

  1. Frontal lobe (motor)
    - Head/leg movements, posturing, post-ictal weakness (Todd’s paresis), Jacksonian march
  2. Parietal lobe (sensory) Paraesthesia
  3. Occipital lobe (visual) Floaters/flashes
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2
Q

Classical presentation of absence seizures?

A

Absence seizures, also known as petit mal seizures, are a type of seizure disorder that primarily affects children. The typical clinical features include the child frequently staring off into space and becoming unresponsive during these episodes. These episodes are brief usually lasting only a few seconds and there is no syncope/loss of consciousness. Instead, the child appears to be temporarily ‘absent’ during the episode. Absence seizures also do not cause significant motor movements or convulsions.

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3
Q

Focal seizures

A
  • previously termed partial seizures
  • these start in a specific area, on one side of the brain
  • the level of awareness can vary in focal seizures. The terms focal aware (previously termed ‘simple partial’), focal impaired awareness (previously termed ‘complex partial’) and awareness unknown are used to further describe focal seizures
  • further to this, focal seizures can be classified as being motor (e.g. Jacksonian march), non-motor (e.g. déjà vu, jamais vu; ) or having other features such as aura
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4
Q

Generalised seizure types

A
  • these engage or involve networks on both sides of the brain at the onset
  • consciousness lost immediately.

generalised seizures can be further subdivided into motor (e.g. tonic-clonic) and non-motor (e.g. absence)
specific types include:
- tonic-clonic (grand mal)
- tonic
- clonic
- typical absence (petit mal)
- atonic

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5
Q

What is a focal to bilateral seizure?

A
  • starts on one side of the brain in a specific area before spreading to both lobes
  • previously termed secondary generalized seizures
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6
Q

What are the features of MND?

A

Motor neuron disease is a neurological condition of unknown cause which can present with both upper and lower motor neuron signs. It rarely presents before 40 years and various patterns of disease are recognised including amyotrophic lateral sclerosis (ALS), progressive muscular atrophy and bulbar palsy.

There are a number of clues which point towards a diagnosis of motor neuron disease:
- asymmetric limb weakness is the most common presentation of ALS
- the mixture of lower motor neuron and upper motor neuron signs
- wasting of the small hand muscles/tibialis anterior is common
- fasciculations
- the absence of sensory signs/symptoms
- vague sensory symptoms may occur early in the disease (e.g. limb pain) but ‘never’ sensory signs

Other features
- doesn’t affect external ocular muscles
- no cerebellar signs
- abdominal reflexes are usually preserved and sphincter dysfunction if present is a late feature

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7
Q

What are the important findings when diagnosing MND?

A

The diagnosis of motor neuron disease is clinical, but nerve conduction studies will show normal motor conduction and can help exclude a neuropathy. Electromyography shows a reduced number of action potentials with increased amplitude. MRI is usually performed to exclude the differential diagnosis of cervical cord compression and myelopathy

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8
Q

What are the different types of brain tumour?

A
  1. Metastases
  2. Glioblastoma multiforme
  3. Meningioma
  4. Vestibular schwannoma
  5. Pilocytic astrocytoma
  6. Medulloblastoma
  7. Ependymoma
  8. Oligodendroma
  9. Haemoangioblastoma
  10. Pituitary adenoma
  11. Craniopharyngioma
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9
Q

Which tumours most commonly cause brain metastases?

A

Tumours that most commonly spread to the brain include:
lung (most common)
breast
bowel
skin (namely melanoma)
kidney

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10
Q

What is the most common primary brain tumour in adults, what is the classical histological finding and how is it managed?

A
  • Glioblastoma is the most common primary tumour in adults and is associated with a poor prognosis (~ 1yr).
  • On imaging they are solid tumours with central necrosis and a rim that enhances with contrast. Disruption of the blood-brain barrier and therefore are associated with vasogenic oedema.
  • Histology: Pleomorphic tumour cells border necrotic areas
  • Treatment is surgical with postoperative chemotherapy and/or radiotherapy. Dexamethasone is used to treat the oedema.
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11
Q

What are the second most common primary brain tumours in adults, what is the histological finding and how is it treated?

A
  • The second most common primary brain tumour in adults
  • Meningiomas are typically benign, extrinsic tumours of the central nervous system. They arise from the arachnoid cap cells of the meninges and are typically located next to the dura and cause symptoms by compression rather than invasion.
  • They typically are located at the falx cerebri, superior sagittal sinus, convexity or skull base.
  • Histology: Spindle cells in concentric whorls and calcified psammoma bodies
  • Investigation is with CT (will show contrast enhancement) and MRI, and treatment will involve either observation, radiotherapy or surgical resection.
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12
Q

What is a vestibular schwannoma?

A
  • A vestibular schwannoma (previously termed acoustic neuroma) is a benign tumour arising from the eighth cranial nerve (vestibulocochlear nerve). Often seen in the cerebellopontine angle. It presents with hearing loss, facial nerve palsy (due to compression of the nearby facial nerve) and tinnitus.
  • Neurofibromatosis type 2 is associated with bilateral vestibular schwannomas.
  • Histology: Antoni A or B patterns are seen. Verocay bodies (acellular areas surrounded by nuclear palisades)
  • Treatment may involve observation, radiotherapy or surgery.
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13
Q

What is pilocytic astrocytoma?

A
  • The most common primary brain tumour in children
  • Histology: Rosenthal fibres (corkscrew eosinophilic bundle)
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14
Q

What is a medulloblastoma?

A
  • A medulloblastoma is an aggressive paediatric brain tumour that arises within the infratentorial compartment. It spreads through the CSF system. Treatment is surgical resection and chemotherapy.
  • Histology: Small, blue cells. Rosette pattern of cells with many mitotic figures
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15
Q

What is an ependymoma?

A
  • Commonly seen in the 4th ventricle
  • May cause hydrocephalus
  • Histology: perivascular pseudorosettes
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16
Q

What is an oligodendroma?

A
  • Benign, slow-growing tumour common in the frontal lobes
  • Histology: Calcifications with ‘fried-egg’ appearance
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17
Q

What is a haemangioblastoma?

A
  • Vascular tumour inbrain and spinal cords that often starts in cerebellum
  • Associated with von Hippel-Lindau syndrome
  • Histology: foam cells and high vascularity
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18
Q

What are pituitary adenomas?

A
  • Pituitary adenomas are benign tumours of the pituitary gland. They are either secretory (producing a hormone in excess) or non-secretory. They may be divided into microadenomas (smaller than 1cm) or macroadenoma (larger than 1cm).
  • Patients will present with the consequences of hormone excess (e.g. Cushing’s due to ACTH, or acromegaly due to GH) or depletion. Compression of the optic chiasm will cause a bitemporal hemianopia due to the crossing nasal fibers.
  • Investigation requires a pituitary blood profile and MRI. Treatment can either be hormonal or surgical (e.g. transphenoidal resection).
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19
Q

What is a craniopharyngioma?

A
  • Most common paediatric supratentorial tumour
  • A craniopharyngioma is a solid/cystic tumour of the sellar region that is derived from the remnants of Rathke’s pouch. It is common in children, but can present in adults also. It may present with hormonal disturbance, symptoms of hydrocephalus or bitemporal hemianopia.
  • Histology: Derived from remnants of Rathke pouch
  • Investigation requires pituitary blood profile and MRI. Treatment is typically surgical with or without postoperative radiotherapy.
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20
Q

What are the features of intracranial venous thrombosis?

A
  • can cause cerebral infarction, much lesson common than arterial causes
  • 50% of patients have isolated sagittal sinus thromboses - the remainder have coexistent lateral sinus thromboses and cavernous sinus thromboses

Common features
- headache (may be sudden onset)
- nausea & vomiting
- reduced consciousness

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21
Q

How is intracranial venous thrombosis investigated and managed?

A

Investigation
- MRI venography is the gold standard
- CT venography is an alternative
- non-contrast CT head is normal in around 70% of patients
- D-dimer levels may be elevated

Management
- anticoagulation
- typically with low molecular weight heparin acutely
- warfarin is still generally used for longer term anticoagulation

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22
Q

What are the features of sagittal sinus thrombosis, cavernous sinus thrombosis and lateral sinus thrombosis?

A

Sagittal sinus thrombosis
- may present with seizures and hemiplegia
- parasagittal biparietal or bifrontal haemorrhagic infarctions are sometimes seen
- ‘empty delta sign’ seen on venography

Cavernous sinus thrombosis
- other causes of cavernous sinus syndrome: local infection (e.g. sinusitis), neoplasia, trauma
- periorbital oedema
- ophthalmoplegia: 6th nerve damage typically occurs before 3rd & 4th
- trigeminal nerve involvement may lead to hyperaesthesia of upper face and eye pain
central retinal vein thrombosis

Lateral sinus thrombosis
- 6th and 7th cranial nerve palsies

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23
Q

When should antiepileptics be started?

A

Most neurologists now start antiepileptics following a second epileptic seizure. NICE guidelines suggest starting antiepileptics after the first seizure if any of the following are present:
- the patient has a neurological deficit
- brain imaging shows a structural abnormality
- the EEG shows unequivocal epileptic activity
- the patient or their family or carers consider the risk of having a further seizure unacceptable

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24
Q

AEDs for generalised tonic-clonic seizures

A

males: sodium valproate
females: lamotrigine or levetiracetam
girls aged under 10 years and who are unlikely to need treatment when they are old enough to have children or women who are unable to have children may be offered sodium valproate first-line

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25
AEDs for focal seizures?
first line: lamotrigine or levetiracetam second line: carbamazepine, oxcarbazepine or zonisamide
26
AEDs for absence seizures (petit mal)
first line: ethosuximide second line: male: sodium valproate female: lamotrigine or levetiracetam carbamazepine may exacerbate absence seizures
27
AEDs for myoclonic seizures
males: sodium valproate females: levetiracetam
28
AEDs for tonic or atonic seizures
males: sodium valproate females: lamotrigine
29
What are the criteria for needing a CT head within 1 hour in head injury?
- GCS < 13 on initial assessment - GCS < 15 at 2 hours post-injury - suspected open or depressed skull fracture - any sign of basal skull fracture (haemotympanum, 'panda' eyes, cerebrospinal fluid leakage from the ear or nose, Battle's sign). - post-traumatic seizure. - focal neurological deficit. - more than 1 episode of vomiting
30
What are the criteria for needing a CT head within 8 hours in head injury?
CT head scan within 8 hours of the head injury - for adults with any of the following risk factors who have experienced some loss of consciousness or amnesia since the injury: - age 65 years or older - any history of bleeding or clotting disorders including anticogulants - dangerous mechanism of injury (a pedestrian or cyclist struck by a motor vehicle, an occupant ejected from a motor vehicle or a fall from a height of greater than 1 metre or 5 stairs) - more than 30 minutes' retrograde amnesia of events immediately before the head injury If a patient is on warfarin who have sustained a head injury with no other indications for a CT head scan, perform a CT head scan within 8 hours of the injury.
31
What nerve damage are mid-shaft humeral fractures associated with?
Mid-shaft humeral fractures are associated with radial nerve injury. As such, the radial nerve needs to be tested. This may be achieved in a number of ways, but extending the wrist will test radial nerve function (as the radial nerve supplies innervation to the extensor muscles). Palsy of the radial nerve is associated with 'wrist drop'. Extending the elbow would test the radial nerve, but as the injury is at the shaft of the humerus, innervation of the triceps may arise proximal to a radial nerve injury. This would mean that a more distal injury could be missed. As such, while this will probably be performed, it is not going to give as much information as extension of the wrist.
32
What is the path of the radial nerve?
Continuation of posterior cord of the brachial plexus Path 1. In the axilla: lies posterior to the axillary artery on subscapularis, latissimus dorsi and teres major. 2. Enters the arm between the brachial artery and the long head of triceps (medial to humerus). 3. Spirals around the posterior surface of the humerus in the groove for the radial nerve. 4. At the distal third of the lateral border of the humerus it then pierces the intermuscular septum and descends in front of the lateral epicondyle. 5. At the lateral epicondyle it lies deeply between brachialis and brachioradialis where it then divides into a superficial and deep terminal branch. 6. Deep branch crosses the supinator to become the posterior interosseous nerve.
33
What regions does the radial nerve innervate?
1. Motor (main nerve) - Triceps - Anconeus - Brachioradialis - Extensor carpi radialis 2. Motor (posterior interosseous branch) - Supinator - Extensor carpi ulnaris - Extensor digitorum - Extensor indicis - Extensor digiti minimi - Extensor pollicis longus and brevis - Abductor pollicis longus 3. Sensory The area of skin supplying the proximal phalanges on the dorsal aspect of the hand is supplied by the radial nerve (this does not apply to the little finger and part of the ring finger)
34
what are the effects of radial nerve denervation at different locations?
1. Shoulder Affects Long head of triceps- Minor effects on shoulder stability in abduction 2. Arm affects Triceps - Loss of elbow extension 3. Forearm Affects: Supinator, Brachioradialis, Extensor carpi radialis longus and brevis - Weakening of supination of prone hand and elbow flexion in mid prone position Patterns of damage: - wrist drop - sensory loss to small area between the dorsal aspect of the 1st and 2nd metacarpals - paralysis of triceps in axillary damage
35
What are the three classical features of parkinson's?
Parkinson's disease, characterised by the three classical symptoms: 1. cogwheel rigidity, 2. bradykinesia poverty of movement also seen, sometimes referred to as hypokinesia short, shuffling steps with reduced arm swinging difficulty in initiating movement, and 3. tremor. The tremor seen in Parkinson's disease is often asymmetric, occurs with rest, and improves with voluntary activity. typically 'pill-rolling', i.e. in the thumb and index finger Other symptoms characteristic of Parkinson's disease include mask-like facies, flexed posture, micrographia, drooling of saliva, psychiatric features: depression is the most common feature (affects about 40%); dementia, psychosis and sleep disturbances may also occur, impaired olfaction, REM sleep behaviour disorder, fatigue autonomic dysfunction: postural hypotension
36
What is the cause of parkinson's?
Parkinson's disease is a progressive neurodegenerative condition caused by degeneration of dopaminergic neurons in the substantia nigra. The reduction in dopaminergic output results in a classical triad of features: bradykinesia, tremor and rigidity. The symptoms of Parkinson's disease are characteristically asymmetrical.
37
What are the features of drug-induced parkinsonism?
Drug-induced parkinsonism has slightly different features to Parkinson's disease: 1. motor symptoms are generally rapid onset and bilateral 2. rigidity and rest tremor are uncommon
38
How is parkinson's diagnosed?
Diagnosis is usually clinical. However, if there is difficulty differentiating between essential tremor and Parkinson's disease NICE recommend considering 123I‑FP‑CIT single photon emission computed tomography (SPECT).
39
What are the features of myasthenia gravis?
Myasthenia gravis is an autoimmune disorder resulting in insufficient functioning acetylcholine receptors. Antibodies to acetylcholine receptors are seen in 85-90% of cases (*antibodies are less commonly seen in disease limited to the ocular muscles). Myasthenia is more common in women (2:1) The key feature is muscle fatigability - muscles become progressively weaker during periods of activity and slowly improve after periods of rest: - extraocular muscle weakness: diplopia - proximal muscle weakness: face, neck, limb girdle - ptosis - dysphagia Associations - thymomas in 15% - autoimmune disorders: pernicious anaemia, autoimmune thyroid disorders, rheumatoid, SLE - thymic hyperplasia in 50-70%
40
How is myasthenia gravis investigated?
- single fibre electromyography: high sensitivity (92-100%) - CT thorax to exclude thymoma - CK normal - antibodies to acetylcholine receptors positive in around 85-90% of patients in the remaining patients, about about 40% are positive for anti-muscle-specific tyrosine kinase antibodies - Tensilon test: IV edrophonium (reversible acetylcholinesterase inhibitor) reduces muscle weakness temporarily - not commonly used any more due to the risk of cardiac arrhythmia
41
How is myasthenia gravis and myasthenic crisis managed?
1. long-acting acetylcholinesterase inhibitors - pyridostigmine is first-line - immunosuppression is usually not started at diagnosis, but the majority of patients eventually require it in addition to long-acting acetylcholinesterase inhibitors: *prednisolone initially *azathioprine, cyclosporine, mycophenolate mofetil may also be used - thymectomy Management of myasthenic crisis: - plasmapheresis - intravenous immunoglobulins
42
What are the common precipitants for migraines?
- Chocolate - Hangovers - Orgasms - Cheese, wine, citrus fruit - Caffeine - The oral contraceptive pill - Lie-ins, tiredness, stress - Alcohol - Travel - Exercise -Lack of food/dehydration -Menstruation -Bright lights
43
What are the features of migraine?
Migraine is a common type of primary headache. It is characterised typically by: - a severe, unilateral, throbbing headache - associated with nausea, photophobia and phonophobia - attacks may last up to 72 hours - patients characteristically go to a darkened, quiet room during an attack - 'classic' migraine attacks are precipitated by an aura *these occur in around one-third of migraine patients *typical aura are visual, progressive, last 5-60 minutes and are characterised by transient hemianopic disturbance or a spreading scintillating scotoma
44
What are the formal diagnostic criteria for migraine?
A - At least 5 attacks fulfilling criteria B-D B - Headache attacks lasting 4-72 hours* (untreated or unsuccessfully treated) C - Headache has at least two of the following characteristics: 1. unilateral location in children, attacks may be shorter-lasting, headache is more commonly bilateral, and gastrointestinal disturbance is more prominent. 2. pulsating quality (i.e., varying with the heartbeat) 3. moderate or severe pain intensity 4. aggravation by or causing avoidance of routine physical activity (e.g., walking or climbing stairs) D - During headache at least one of the following: 1. nausea and/or vomiting* 2. photophobia and phonophobia E - Not attributed to another disorder (history and examination do not suggest a secondary headache disorder or, if they do, it is ruled out by appropriate investigations or headache attacks do not occur for the first time in close temporal relation to the other disorder)
45
What is a hemiplegic migraine?
- a variant of migraine in which motor weakness is a manifestation of aura in at least some attacks - around half of patients have a strong family history - very rare - estimated prevalence is 0.01% (i.e. around only 1 in 1,000 migraine patients), more common in adolescent females
46
What are the components of the glasgow coma scale?
Motor response 6. Obeys commands 5. Localises to pain 4. Withdraws from pain 3. Abnormal flexion to pain (decorticate posture) 2. Extending to pain 1. None Verbal response 5. Orientated 4. Confused 3. Words 2. Sounds 1. None Eye opening 4. Spontaneous 3. To speech 2. To pain 1. None
47
What are the types of SAH and what are their causes?
The most common cause of SAH is head injury and this is called traumatic SAH . In the absence of trauma, SAH is termed spontaneous SAH . Causes of spontaneous SAH include: - intracranial aneurysm (saccular ‘berry’ aneurysms) - accounts for around 85% of cases *conditions associated with berry aneurysms include hypertension, adult polycystic kidney disease, Ehlers-Danlos syndrome and coarctation of the aorta - arteriovenous malformation - pituitary apoplexy - mycotic (infective) aneurysms
48
What are the presenting features of SAH?
1. headache - usually of sudden-onset (‘thunderclap’ or ‘hit with a baseball bat’), severe (‘worst of my life’), occipital, typically peaking in intensity within 1 to 5 minutes, there may be a history of a less-severe 'sentinel' headache in the weeks prior to presentation 2. nausea and vomiting 3. meningism (photophobia, neck stiffness) 4. coma 5. seizures 6. ECG changes including ST elevation may be seen this may be secondary to either autonomic neural stimulation from the hypothalamus or elevated levels of circulating catecholamines
49
How is SAH investigated?
1. non-contrast CT head is the first-line investigation of choice: acute blood (hyperdense/bright on CT) is typically distributed in the basal cisterns, sulci and in severe cases the ventricular system. *if CT head is done within 6 hours of symptom onset and is normal new guidelines suggest not doing a lumbar puncture, consider an alternative diagnosis *if CT head is done more than 6 hours after symptom onset and is normal - do a lumber puncture (LP) - timing wise the LP should be performed at least 12 hours following the onset of symptoms to allow the development of xanthochromia (bilirubin from breakdown of RBCs irritating the meninges). - xanthochromia helps to distinguish true SAH from a ‘traumatic tap’ (blood introduced by the LP procedure). - as well as xanthochromia, CSF findings consistent with subarachnoid haemorrhage include a normal or raised opening pressure - if the CT shows evidence of a SAH, referral to neurosurgery to be made as soon as SAH is confirmed After spontaneous SAH is confirmed, the aim of investigation is to identify a causative pathology that needs urgent treatment: - CT intracranial angiogram (to identify a vascular lesion e.g. aneurysm or AVM) +/- digital subtraction angiogram (catheter angiogram)
50
How is an aneurysmal SAH managed?
1. supportive bed rest, analgesia, venous thromboembolism prophylaxis, discontinuation of antithrombotics (reversal of anticoagulation if present) 2. vasospasm is prevented using a course of oral Nimodipine 3. intracranial aneurysms are at risk of rebleeding and therefore require prompt intervention, preferably within 24 hours most intracranial aneurysms are now treated with a coil by interventional neuroradiologists, but a minority require a craniotomy and clipping by a neurosurgeon
51
What are the complications of aneurysmal SAH and the predictive factors?
Complications of aneurysmal SAH: - re-bleeding happens in around 10% of cases and most common in the first 12 hours * if rebleeding is suspected (e.g. sudden worsening of neurological symptoms) then a repeat CT should be arranged * associated with a high mortality (up to 70%) - hydrocephalus - temporarily treated with an external ventricular drain (CSF diverted into a bag at the bedside) or, if required, a long-term ventriculoperitoneal shunt - vasospasm (also termed delayed cerebral ischaemia), typically 7-14 days after onset *ensure euvolaemia (normal blood volume) *consider treatment with a vasopressor if symptoms persist - hyponatraemia (most typically due to syndrome inappropriate anti-diuretic hormone (SIADH) - may cause new headache and muscle cramps) - seizures Important predictive factors in SAH: 1. conscious level on admission 2. age 3. amount of blood visible on CT head
52
What is the triad of normal pressure hydrocephalus?
Normal pressure hydrocephalus is a reversible cause of dementia seen in elderly patients. It is thought to be secondary to reduced CSF absorption at the arachnoid villi. These changes may be secondary to head injury, subarachnoid haemorrhage or meningitis. A classical triad of features is seen 1. urinary incontinence 2. dementia and bradyphrenia 3. gait abnormality (may be similar to Parkinson's disease) It is thought around 60% of patients will have all 3 features at the time of diagnosis. Symptoms typically develop over a few months.
53
How is normal pressure hydrocephalus investigated and managed?
Imaging hydrocephalus with ventriculomegaly in the absence of, or out of proportion to, sulcal enlargement Management - ventriculoperitoneal shunting - around 10% of patients who have shunts experience significant complications such as seizures, infection and intracerebral haemorrhages
54
How do wernicke's and broca's areas interact during conversation?
Spoken word is heard at the ear. This passes to Wernicke's area in the temporal lobe (near the ear) to comprehend what was said. Once understood, the signal passes along the arcuate fasciculus, before reaching Broca's area. The Broca's area in the frontal lobe (near the mouth) then generates a signal to coordinate the mouth to speak what is thought (fluent speech).
55
How do you distinguish the types of aphasia?
When speech is non-fluent (laboured and halting though words may be preserved) -And comprehension is relatively intact = BROCA's APHASIA - And comprehension is impaired = GLOBAL APHASIA When speech is fluent (sentences make no sense, word substitution and neologisms but speech remains fluent) - And comprehension relatively intact = CONDUCTION APHASIA - And comprehension is impaired = WERNICKE'S APHASIA
56
What are the different types of aphasia?
1. Wernicke's (receptive) aphasia - Due to a lesion of the superior temporal gyrus. It is typically supplied by the inferior division of the left MCA - This area 'forms' the speech before 'sending it' to Broca's area. - Lesions result in sentences that make no sense, word substitution and neologisms but speech remains fluent - 'word salad' - Comprehension is impaired 2. Broca's (expressive) aphasia - Due to a lesion of the inferior frontal gyrus. It is typically supplied by the superior division of the left MCA - Speech is non-fluent, laboured, and halting. Repetition is impaired - Comprehension is normal 3. Conduction aphasia - Classically due to a stroke affecting the arcuate fasiculus - the connection between Wernicke's and Broca's area - Speech is fluent but repetition is poor. Aware of the errors they are making - Comprehension is normal 4. Global aphasia - Large lesion affecting all 3 of the above areas resulting in severe expressive and receptive aphasia - May still be able to communicate using gestures
57
What are the 3 broad types of traumatic brain injury and their features?
1. Extradural haematoma Bleeding into the space between the dura mater and the skull. Often results from acceleration-deceleration trauma or a blow to the side of the head. The majority of extradural haematomas occur in the temporal region where skull fractures cause a rupture of the middle meningeal artery. Features: - Raised intracranial pressure - Some patients may exhibit a lucid interval 2. Subdural haematoma Bleeding into the outermost meningeal layer. Most commonly occur around the frontal and parietal lobes. May be either acute or chronic. Risk factors include old age and alcoholism. - Slower onset of symptoms than a extradural haematoma. 3. Subarachnoid haemorrhage Usually occurs spontaneously in the context of a ruptured cerebral aneurysm, but may be seen in association with other injuries when a patient has sustained a traumatic brain injury.
58
What is the pathophysiology of traumatic brain injury?
- Primary brain injury may be focal (contusion/ haematoma) or diffuse (diffuse axonal injury) - Diffuse axonal injury occurs as a result of mechanical shearing following deceleration, causing disruption and tearing of axons - Intra-cranial haematomas can be extradural, subdural or intracerebral, while contusions may occur adjacent to (coup) or contralateral (contre-coup) to the side of impact - Secondary brain injury occurs when cerebral oedema, ischaemia, infection, tonsillar or tentorial herniation exacerbates the original injury. The normal cerebral auto regulatory processes are disrupted following trauma rendering the brain more susceptible to blood flow changes and hypoxia - The Cushings reflex (hypertension and bradycardia) often occurs late and is usually a pre terminal event
59
How is traumatic brain injury managed?
1. Where there is life threatening rising ICP such as in extradural haematoma and whilst theatre is prepared or transfer arranged use of IV mannitol/ frusemide may be required. (Mannitol is an osmotic diuretic that works by increasing the osmolarity of the blood, drawing fluid from the brain parenchyma into the bloodstream and reducing cerebral oedema.) 2. Diffuse cerebral oedema may require decompressive craniotomy 3. Depressed skull fractures that are open require formal surgical reduction and debridement, closed injuries may be managed nonoperatively if there is minimal displacement. 4. ICP monitoring is appropriate in those who have GCS 3-8 and normal CT scan. 5. ICP monitoring is mandatory in those who have GCS 3-8 and abnormal CT scan. 6. Hyponatraemia is most likely to be due to the syndrome of inappropriate ADH secretion. 7. Minimum of cerebral perfusion pressure of 70mmHg in adults. 8. Minimum cerebral perfusion pressure of between 40 and 70 mmHg in children.
60
How should pupillary findings in head injuries be interpreted?
1. Unilaterally dilated pupils with Sluggish or fixed light response - 3rd nerve compression secondary to tentorial herniation 2. Bilaterally dilated pupils with Sluggish or fixed light response - Poor CNS perfusion or Bilateral 3rd nerve palsy 3. Unilaterally dilated or equal with Cross reactive (Marcus - Gunn) light response - Optic nerve injury 4. Bilaterally constricted - May be difficult to assess light response - Opiates, Pontine lesions, Metabolic encephalopathy 5. Unilaterally constricted with Preserved light response - Sympathetic pathway disruption
61
What are psychogenic non-epileptic seizures and what are the features? Which factors favour true epileptic seizures?
Psychogenic nonepileptic seizures are sometimes referred to as pseudoseizures. Factors favouring pseudoseizures: - pelvic thrusting - family member with epilepsy - much more common in females - crying after seizure - don't occur when alone - gradual onset Factors favouring true epileptic seizures - tongue biting - raised serum prolactin
62
Which migraine prophylaxis can be used in asthmatics?
Topiramate instead of propanolol
63
What are the different causes for bitemporal hemianopia when the upper or lower quadrant in predominantly affected?
Bitemporal hemianopia - lesion of optic chiasm - upper quadrant defect > lower quadrant defect = inferior chiasmal compression, commonly a pituitary tumour - lower quadrant defect > upper quadrant defect = superior chiasmal compression, commonly a craniopharyngioma
64
What is lateral medullary syndrome, what are the features and which artery is implicated?
Lateral medullary syndrome, also known as Wallenberg's syndrome, occurs following occlusion of the posterior inferior cerebellar artery. (left or right) Cerebellar features - ataxia - nystagmus Brainstem features - ipsilateral: dysphagia, facial numbness, cranial nerve palsy e.g. Horner's - contralateral: limb sensory loss
65
Which cancer is associated with myasthenia gravis?
Thymoma
66
What is Cushing's triad?
1. widening pulse pressure 2. bradycardia 3. irregular breathing seen in pts with raised ICP
67
How is raised ICP managed?
Management - investigate and treat the underlying cause - head elevation to 30º - IV mannitol may be used as an osmotic diuretic. It is a hypertonic agent which increases systemic osmolality, causing an osmotic shift of water out of the brain parenchyma. - controlled hyperventilation *aim is to reduce pCO2 → vasoconstriction of the cerebral arteries → reduced ICP *leads to rapid, temporary lowering of ICP. However, caution needed as may reduce blood flow to already ischaemic parts of the brain - removal of CSF, different techniques include: *drain from intraventricular monitor (see above) *repeated lumbar puncture (e.g. idiopathic intracranial hypertension) *ventriculoperitoneal shunt (for hydrocephalus)
68
Which dopamine receptor agonists are used in parkinsons and what are the adverse effects?
e.g. bromocriptine, ropinirole, cabergoline, apomorphine - ergot-derived dopamine receptor agonists (bromocriptine, cabergoline) have been associated with pulmonary, retroperitoneal and cardiac fibrosis. The Committee on Safety of Medicines advice that an echocardiogram, ESR, creatinine and chest x-ray should be obtained prior to treatment and patients should be closely monitored - patients should be warned about the potential for dopamine receptor agonists to cause impulse control disorders/disinhibition and excessive daytime somnolence - more likely than levodopa to cause hallucinations in older patients. Nasal congestion and postural hypotension are also seen in some patients
69
What are the adverse effects of levodopa use?
- nearly always combined with a decarboxylase inhibitor (e.g. carbidopa or benserazide): this prevents the peripheral metabolism of levodopa to dopamine outside of the brain and hence can reduce side effects 1. common adverse effects: dry mouth anorexia palpitations postural hypotension psychosis 2. some adverse effects are due to the difficulty in achieving a steady dose of levodopa - end-of-dose wearing off: symptoms often worsen towards the end of dosage interval. This results in a decline of motor activity - 'on-off' phenomenon: large variations in motor performance, with normal function during the ‘on’ period, and weakness and restricted mobility during the ‘off’ period - dyskinesias at peak dose: dystonia, chorea and athetosis (involuntary writhing movements) - these effects may worsen over time with increased use - clinicians therefore may limit doses until necessary 3. it is important not to acutely stop levodopa, for example, if a patient is admitted to hospital. Acute withdrawal can precipitate neuroleptic malignant syndrome. if a patient with Parkinson's disease cannot take levodopa orally, they can be given a dopamine agonist patch as rescue medication to prevent acute dystonia
70
What are the features of a common peroneal nerve palsy?
The sciatic nerve divides into the tibial and common peroneal nerves. Injury often occurs at the neck of the fibula The most characteristic feature of a common peroneal nerve lesion is foot drop. Other features include: - weakness of foot dorsiflexion - weakness of foot eversion - weakness of extensor hallucis longus - sensory loss over the dorsum of the foot and the lower lateral part of the leg - wasting of the anterior tibial and peroneal muscles
71
What are the features and management of essential tremor?
Essential tremor (previously called benign essential tremor) is an autosomal dominant condition which usually affects both upper limbs Features - postural tremor: worse if arms outstretched - improved by alcohol and rest - most common cause of titubation (head tremor) Management - propranolol is first-line - primidone is sometimes used
72
What is subacute combined degeneration of the spinal cord? What are the features?
Subacute combined degeneration (SCD) of the spinal cord is due to vitamin B12 deficiency resulting in impairment of the Spinocerebellar tracts, Corticospinal tracts, Dorsal columns (SCD) Recreational nitrous oxide inhalation may also result in vitamin B12 deficiency → subacute combined degeneration of the spinal cord. Features 1. dorsal column involvement - distal tingling/burning/sensory loss is symmetrical and tends to affect the legs more than the arms - impaired proprioception and vibration sense 2. lateral corticospinal tract involvement - muscle weakness, hyperreflexia, and spasticity - upper motor neuron signs typically develop in the legs first - brisk knee reflexes - absent ankle jerks - extensor plantars 3. spinocerebellar tract involvement - sensory ataxia → gait abnormalities - positive Romberg's sign
73
What are the localising features of focal seizures?
Temporal lobe - May occur with or without impairment of consciousness or awareness - An aura occurs in most patients typically a rising epigastric sensation also psychic or experiential phenomena, such as déjà vu, jamais vu less commonly hallucinations (auditory/gustatory/olfactory) - Seizures typically last around one minute - automatisms (e.g. lip smacking/grabbing/plucking) are common Frontal lobe (motor) - Head/leg movements, posturing, post-ictal weakness, Jacksonian march Parietal lobe (sensory) - Paraesthesia Occipital lobe (visual) - Floaters/flashes
74
What is trigeminal neuralgia?
Trigeminal neuralgia is a pain syndrome characterised by severe unilateral pain. The vast majority of cases are idiopathic but compression of the trigeminal roots by tumours or vascular problems may occur. The International Headache Society defines trigeminal neuralgia as: - a unilateral disorder characterised by brief electric shock-like pains, abrupt in onset and termination, limited to one or more divisions of the trigeminal nerve - the pain is commonly evoked by light touch, including washing, shaving, smoking, talking, and brushing the teeth (trigger factors), and frequently occurs spontaneously - small areas in the nasolabial fold or chin may be particularly susceptible to the precipitation of pain (trigger areas) - the pains usually remit for variable periods
75
How is trigeminal neuralgia managed?
- carbamazepine is first-line - failure to respond to treatment or atypical features (e.g. < 50 years old) should prompt referral to neurology
76
What is a transient ischaemic attack and what are the features?
A transient ischaemic attack (TIA), as the name suggests, is a brief period of neurological deficit due to a vascular cause, typically lasting less than an hour. 'A transient episode of neurologic dysfunction caused by focal brain, spinal cord, or retinal ischaemia, without acute infarction.' Clinical features The clinical features are similar to those of a stroke, i.e. sudden onset, focal neurological deficit but, rather than persisting, the features resolve, typically within 1 hour. Possible features include - unilateral weakness or sensory loss. - aphasia or dysarthria - ataxia, vertigo, or loss of balance - visual problems *sudden transient loss of vision in one eye (amaurosis fugax) *diplopia *homonymous hemianopia
77
How is a TIA managed in the acute phase?
Immediate antithrombotic therapy: give aspirin 300 mg immediately, unless: 1. the patient has a bleeding disorder or is taking an anticoagulant (needs immediate admission for imaging to exclude a haemorrhage) 2. the patient is already taking low-dose aspirin regularly: continue the current dose of aspirin until reviewed by a specialist 3. Aspirin is contraindicated: discuss management urgently with the specialist team Specialist review - if the patient has had more than 1 TIA ('crescendo TIA') or has a suspected cardioembolic source or severe carotid stenosis: *discuss the need for admission or observation urgently with a stroke specialist - If the patient has had a suspected TIA in the last 7 days: *arrange urgent assessment (within 24 hours) by a specialist stroke physician - if the patient has had a suspected TIA which occurred more than a week previously: *refer for specialist assessment as soon as possible within 7 days Advise the person not to drive until they have been seen by a specialist.
78
How are TIAs investigated?
Neuroimaging - NICE recommend that CT brains should not be done 'unless there is clinical suspicion of an alternative diagnosis that CT could detect' - MRI (including diffusion-weighted and blood-sensitive sequences) is preferred to determine the territory of ischaemia, or to detect haemorrhage or alternative pathologies *it should be done on the same day as the specialist assessment if possible Carotid imaging - atherosclerosis in the carotid artery may be a source of emboli in some patients - all patients should therefore have an urgent carotid doppler unless they are not a candidate for carotid endarterectomy
79
How are TIAs managed in the long-term, post-acute phase?
Secondary prevention 1. antiplatelet therapy to follow on from initial aspirin therapy - clopidogrel is recommended first-line (as for patients who've had a stroke) - aspirin + dipyridamole should be given to patients who cannot tolerate clopidogrel 2. lipid modification - high-intensity statin (such as atorvastatin 20–80 mg daily) - the aim of statin therapy is to reduce non-HDL cholesterol by more than 40% With regards to carotid artery endarterectomy: - recommend if the patient has suffered stroke or TIA in the carotid territory and is not severely disabled - should only be considered if *carotid stenosis > 70% according European Carotid Surgery Trialists' Collaborative Group criteria or > 50% according to North American Symptomatic Carotid Endarterectomy Trial criteria
80
What is the mechanism of action and side effects of carbamazepine? When is it contraindicated?
Carbamazepine is chemically similar to the tricyclic antidepressant drugs. It is most commonly used in the treatment of epilepsy, particularly partial seizures, where carbamazepine remains a first-line medication. Other uses include - trigeminal neuralgia - bipolar disorder It makes absence and myoclonic seizures worse. Mechanism of action binds to sodium channels increases their refractory period Adverse effects - P450 enzyme inducer - dizziness and ataxia - drowsiness - headache - visual disturbances (especially diplopia) - Steven-Johnson syndrome - leucopenia and agranulocytosis - hyponatraemia secondary to syndrome of inappropriate ADH secretion Carbamazepine is known to exhibit autoinduction, hence when patients start carbamazepine they may see a return of seizures after 3-4 weeks of treatment.
81
What are the red flags in headache?
- compromised immunity, caused, for example, by HIV or immunosuppressive drugs - age under 20 years and a history of malignancy - a history of malignancy known to metastasis to the brain - vomiting without other obvious cause - worsening headache with fever - sudden-onset headache reaching maximum intensity within 5 minutes - 'thunderclap' - new-onset neurological deficit - new-onset cognitive dysfunction - change in personality - impaired level of consciousness - recent (typically within the past 3 months) head trauma - headache triggered by cough, valsalva (trying to breathe out with nose and mouth blocked), sneeze or exercise - orthostatic headache (headache that changes with posture) - symptoms suggestive of giant cell arteritis or acute narrow-angle glaucoma - a substantial change in the characteristics of their headache
82
What is an arnold-chiari malformation?
Arnold-Chiari malformation describes the downward displacement, or herniation, of the cerebellar tonsils through the foramen magnum. Malformations may be congenital or acquired through trauma. Features - non-communicating hydrocephalus may develop as a result of obstruction of cerebrospinal fluid (CSF) outflow - headache - syringomyelia
83
Which drugs can exacerbate myasthenia gravis?
The most common exacerbating factor is exertion resulting in fatigability, which is the hallmark feature of myasthenia gravis.. Symptoms become more marked during the day The following drugs may exacerbate myasthenia: - penicillamine - quinidine, procainamide - beta-blockers - lithium - phenytoin - antibiotics: gentamicin, macrolides, quinolones, tetracyclines
84
What are the associated stroke effects for different lesion sites? (ACA, MCA, PCA, Weber's syndrome, PICA, AICA, retinal/opthalmic artery , basilar artery)
1. Anterior cerebral artery - Contralateral hemiparesis and sensory loss - Lower extremity > upper 2. Middle cerebral artery - Contralateral hemiparesis and sensory loss - Upper extremity > lower - Contralateral homonymous hemianopia - Aphasia 3. Posterior cerebral artery - Contralateral homonymous hemianopia with macular sparing - Visual agnosia 4. Weber's syndrome (branches of the posterior cerebral artery that supply the midbrain) - Ipsilateral CN III palsy - Contralateral weakness of upper and lower extremity 5. Posterior inferior cerebellar artery (lateral medullary syndrome, Wallenberg syndrome) - Ipsilateral: facial pain and temperature loss/ numbness. Cranial nerve palsy eg Horner's, dysphagia - Contralateral: limb/torso sensory loss/ pain and temperature loss (motor function spared) - Ataxia, nystagmus 6. Anterior inferior cerebellar artery (lateral pontine syndrome) - Symptoms are similar to Wallenberg's (see above), but: - Ipsilateral: facial paralysis and deafness 7. Retinal/ophthalmic artery - Amaurosis fugax 8. Basilar artery - 'Locked-in' syndrome
85
What are lacunar strokes?
- present with either isolated hemiparesis, hemisensory loss or hemiparesis with limb ataxia - strong association with hypertension - common sites include the basal ganglia, thalamus and internal capsule
86
What is an acoustic neuroma and what are the classical features?
Vestibular schwannomas (sometimes referred to as acoustic neuromas) account for approximately 5% of intracranial tumours and 90% of cerebellopontine angle tumours. The classical history of vestibular schwannoma includes a combination of vertigo, hearing loss, tinnitus and an absent corneal reflex. Features can be predicted by the affected cranial nerves: - cranial nerve VIII: vertigo, unilateral sensorineural hearing loss, unilateral tinnitus - cranial nerve V: absent corneal reflex - cranial nerve VII: facial palsy Bilateral vestibular schwannomas are seen in neurofibromatosis type 2.
87
How are acoustic neuromas investigated and managed?
Patients with a suspected vestibular schwannoma should be referred urgently to ENT. It should be noted though that the tumours are often slow growing, benign and often observed initially. MRI of the cerebellopontine angle is the investigation of choice. Audiometry is also important as only 5% of patients will have a normal audiogram. Management is with either surgery, radiotherapy or observation.
88
What is mononeuritis multiplex?
Mononeuritis multiplex is simultaneous or sequential involvement of individual non-contiguous nerve trunks. It typically presents with acute or subacute loss of sensory and motor function of individual nerves. The pattern of involvement is asymmetric, however, as the disease progresses, deficit(s) becomes more confluent and symmetrical, making it difficult to differentiate from polyneuropathy.
89
Which nerve does corneal reflex?
Ophthalmic division of CN 5
90
What is Brown-Sequard syndrome?
caused by lateral hemisection of the spinal cord Features - ipsilateral weakness below lesion (corticospinal tract [UMN] decussates in medulla) - ipsilateral loss of proprioception and vibration sensation (dorsal columns decussate in medulla) - contralateral loss of pain and temperature sensation (spinothalamic tracts decussate in spinal cord)
91
Which type of dementia is associated with MND?
Frontotemporal
92
What drug is given for long-term management after an ischaemic stroke?
Clopidogrel aspirin plus modified-release dipyridamole is now recommended after an ischaemic stroke only if clopidogrel is contraindicated or not tolerated, but treatment is no longer limited to 2 years' duration
93
What is the 'classical' cause of guillain-barre and what is a common initial symptom?
Guillain-Barre syndrome describes an immune-mediated demyelination of the peripheral nervous system often triggered by an infection (classically Campylobacter jejuni). Initial symptoms around 65% of patients experience back/leg pain in the initial stages of the illness
94
What are the classical and other features of guillain barre syndrome?
The characteristic features of Guillain-Barre syndrome is progressive, symmetrical weakness of all the limbs. - the weakness is classically ascending i.e. the legs are affected first - reflexes are reduced or absent - sensory symptoms tend to be mild (e.g. distal paraesthesia) with very few sensory signs Other features - there may be a history of gastroenteritis - respiratory muscle weakness - cranial nerve involvement *diplopia *bilateral facial nerve palsy *oropharyngeal weakness is common - autonomic involvement *urinary retention *diarrhoea Less common findings - papilloedema: thought to be secondary to reduced CSF resorption
95
How is guillain barre investigated?
1. Lumbar puncture - rise in protein with a normal white blood cell count (albuminocytologic dissociation) - found in 66% 2. nerve condution studies may be performed - decreased motor nerve conduction velocity (due to demyelination) - prolonged distal motor latency - increased F wave latency
96
What is Ramsay-Hunt syndrome? How is it managed?
A LMN facial nerve palsy This is caused by reactivation of the varicella zoster virus in the facial nerve and so produces a lower motor neurone (LMN) lesion. A key distinguishing feature is a LMN lesion does not spare the forehead muscles, whereas an upper motor neurone (UMN) lesion would spare the forehead. There is usually otalgia, with a painful and tender vesicular rash in the ear canal, pinna and around the ear on the affected side. This can spread to the anterior 2/3rd of the tongue and hard palate. Treatment is with aciclovir and prednisolone. Patients also require lubricating eye drops.
97
How is spasticity secondary to multiple sclerosis managed?
- baclofen and gabapentin are first-line. Other options include diazepam, dantrolene and tizanidine - physiotherapy is important - cannabis and botox are undergoing evaluation
98
What is the appropriate next step if Bell's palsy shows no improvement after 3 weeks?
if the paralysis shows no sign of improvement after 3 weeks, refer urgently to ENT a referral to plastic surgery may be appropriate for patients with more long-standing weakness e.g. several months
99
How is bladder dysfunction secondary to MS managed?
- may take the form of urgency, incontinence, overflow etc - guidelines stress the importance of getting an ultrasound first to assess bladder emptying - anticholinergics may worsen symptoms in some patients - if significant residual volume → intermittent self-catheterisation - if no significant residual volume → anticholinergics may improve urinary frequency
100
What cause of 3rd nerve palsy causes pain?
Usually a posterior communicating artery aneurysm
101
What are the possible causes of third nerve palsy?
- diabetes mellitus - vasculitis e.g. temporal arteritis, SLE - false localizing sign* due to uncal herniation through tentorium if raised ICP - posterior communicating artery aneurysm: pupil dilated, often associated pain - cavernous sinus thrombosis - Weber's syndrome (branches of PCA): ipsilateral third nerve palsy with contralateral hemiplegia -caused by midbrain strokes - other possible causes: amyloid, multiple sclerosis
102
What are the features of Wernicke's aphasia?
It is a receptive aphasia. Due to a lesion of the superior temporal gyrus. It is typically supplied by the inferior division of the left MCA This area 'forms' the speech before 'sending it' to Broca's area. Lesions result in sentences that make no sense, word substitution and neologisms but speech remains fluent - 'word salad' Comprehension is impaired
103
What are the features of Broca's aphasia?
Due to a lesion of the inferior frontal gyrus. It is typically supplied by the superior division of the left MCA Speech is non-fluent, laboured, and halting. Repetition is impaired Comprehension is normal
104
What are the adverse effects and contraindications of triptans?
Adverse effects 'triptan sensations' - tingling, heat, tightness (e.g. throat and chest), heaviness, pressure Contraindications patients with a history of, or significant risk factors for, ischaemic heart disease or cerebrovascular disease
105
What is cataplexy?
Cataplexy describes the sudden and transient loss of muscular tone caused by strong emotion (e.g. laughter, being frightened). Around two-thirds of patients with narcolepsy have cataplexy. Features range from buckling knees to collapse.
106
What is an ataxic gait?
A wide-based gait with loss of heel to toe walking and instability is called an ataxic gait Ataxic gaits typically occur following cerebellar injury
107
What causes encephalitis and what are the features?
Pathophysiology - HSV-1 is responsible for 95% of cases in adults - typically affects temporal and inferior frontal lobes Features - fever, headache, psychiatric symptoms, seizures, vomiting focal features e.g. aphasia - peripheral lesions (e.g. cold sores) have NO relation to the presence of HSV encephalitis
108
How is encephalitis investigated and managed?
Investigation - cerebrospinal fluid - lymphocytosis, elevated protein, PCR for HSV, VZV and enteroviruses - neuroimaging - medial temporal and inferior frontal changes (e.g. petechial haemorrhages), normal in one-third of patients, MRI is better - EEG - lateralised periodic discharges at 2 Hz Management intravenous aciclovir should be started in all cases of suspected encephalitis
109
What are the features of cluster headaches?
Cluster headaches are more common in men (3:1) and smokers. Alcohol may trigger an attack and there also appears to be a relation to nocturnal sleep. Features -intense sharp, stabbing pain around one eye - pain typical occurs once or twice a day, each episode lasting 15 mins - 2 hours - the patient is restless and agitated during an attack due to the severity - clusters typically last 4-12 weeks accompanied by redness, lacrimation, lid swelling - nasal stuffiness - miosis and ptosis in a minority
110
How are cluster headaches investigated and managed?
Investigations - most patients will have neuroimaging - underlying brain lesions are sometimes found even if the clinical symptoms are typical for cluster headache - MRI with gadolinium contrast is the investigation of choice Management - NICE recommend seeking specialist advice from a neurologist if a patient develops cluster headaches - acute - 100% oxygen (80% response rate within 15 minutes), subcutaneous triptan (75% response rate within 15 minutes) - prophylaxis - verapamil is the drug of choice, there is also some evidence to support a tapering dose of prednisolone
111
What is creutzfeldt-jakob disease and what are the features?
Creutzfeldt-Jakob disease (CJD) is rapidly progressive neurological condition caused by prion proteins. These proteins induce the formation of amyloid folds resulting in tightly packed beta-pleated sheets resistant to proteases. Sporadic CJD accounts for 85% of cases, 10-15% of cases are familial, mean age of onset is 65 years New variant CJD - younger patients, (average age of onset = 25 years), psychological symptoms such as anxiety, withdrawal and dysphonia are the most common presenting features, median survival = 13 months
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What are the features of creutzfeldt-jakob disease and how is it investigated?
Features dementia (rapid onset) myoclonus and rigidity Investigation CSF is usually normal EEG: biphasic, high amplitude sharp waves (only in sporadic CJD) MRI: hyperintense signals in the basal ganglia and thalamus
113
Which scan is used to view demyelinating neurons?
MRI with contrast (brain +/- spinal cord)
114
What are the adverse effects of Phenytoin?
Phenytoin is used in the management of seizures. Acute initially: dizziness, diplopia, nystagmus, slurred speech, ataxia later: confusion, seizures Chronic common: gingival hyperplasia (secondary to increased expression of platelet derived growth factor, PDGF), hirsutism, coarsening of facial features, drowsiness megaloblastic anaemia (secondary to altered folate metabolism) peripheral neuropathy enhanced vitamin D metabolism causing osteomalacia lymphadenopathy dyskinesia Idiosyncratic fever rashes, including severe reactions such as toxic epidermal necrolysis hepatitis Dupuytren's contracture aplastic anaemia drug-induced lupus Teratogenic associated with cleft palate and congenital heart disease Monitoring Phenytoin levels do not need to be monitored routinely but trough levels, immediately before dose should be checked if: adjustment of phenytoin dose suspected toxicity detection of non-adherence to the prescribed medication
115
What is tuberous sclerosis and what are the features?
Tuberous sclerosis (TS) is a genetic condition of autosomal dominant inheritance. Like neurofibromatosis, the majority of features seen in TS are neurocutaneous. Cutaneous features - depigmented 'ash-leaf' spots which fluoresce under UV light - roughened patches of skin over lumbar spine (Shagreen patches) - adenoma sebaceum (angiofibromas): butterfly distribution over nose - fibromata beneath nails (subungual fibromata) - café-au-lait spots may be seen Neurological features - developmental delay - epilepsy (infantile spasms or partial) intellectual impairment Also - retinal hamartomas: dense white areas on retina (phakomata) - rhabdomyomas of the heart - gliomatous changes can occur in the brain lesions - polycystic kidneys, renal angiomyolipomata - lymphangioleiomyomatosis: multiple lung cysts
116
What are the complications of meningococcal meningitis?
Neurological sequalae - sensorineural hearing loss (most common) - seizures - focal neurological deficit - infective - sepsis, intracerebral abscess - pressure - brain herniation, hydrocephalus Patients with meningococcal meningitis are at risk of Waterhouse-Friderichsen syndrome (adrenal insufficiency secondary to adrenal haemorrhage).
117
How is neuroleptic malignant syndrome investigated and managed?
A raised creatine kinase is present in most cases. Acute kidney injury (secondary to rhabdomyolysis) may develop in severe cases. A leukocytosis may also be seen Management - stop antipsychotic - patients should be transferred to a medical ward if they are on a psychiatric ward and often they are nursed in intensive care units - IV fluids to prevent renal failure - dantrolene may be useful in selected cases - thought to work by decreasing excitation-contraction coupling in skeletal muscle by binding to the ryanodine receptor, and decreasing the release of calcium from the sarcoplasmic reticulum - bromocriptine, dopamine agonist, may also be used
118
How is first line treatment for parkinson's decided?
- if the motor symptoms are affecting the patient's quality of life: levodopa - if the motor symptoms are not affecting the patient's quality of life: dopamine agonist (non-ergot derived), levodopa or monoamine oxidase B (MAO‑B) inhibitor (If a patient continues to have symptoms despite optimal levodopa treatment or has developed dyskinesia then NICE recommend the addition of a dopamine agonist, MAO‑B inhibitor or catechol‑O‑methyl transferase (COMT) inhibitor as an adjunct.
119
What are the glasgow coma scale scores?
Motor response 6. Obeys commands 5. Localises to pain 4. Withdraws from pain 3. Abnormal flexion to pain (decorticate posture) 2. Extending to pain 1. None Verbal response 5. Orientated 4. Confused 3. Words 2. Sounds 1. None Eye opening 4. Spontaneous 3. To speech 2. To pain 1. None
120
Neuropathic pain characteristically responds poorly to opioids. However, if standard treatment options have failed which opioid is it most appropriate to consider starting?
Tramadol
121
Which drugs can cause idiopathic intracranial hypertension?
combined oral contraceptive pill steroids tetracyclines retinoids (isotretinoin, tretinoin) / vitamin A lithium
122
What is lambert-eaton syndrome?
Lambert-Eaton myasthenic syndrome is seen in association with small cell lung cancer and to a lesser extent breast and ovarian cancer. It may also occur independently as an autoimmune disorder. Lambert-Eaton myasthenic syndrome is caused by an antibody directed against presynaptic voltage-gated calcium channel in the peripheral nervous system.
123
What are the features of lambert-eaton syndrome?
- repeated muscle contractions lead to increased muscle strength (in contrast to myasthenia gravis) *in reality, this is seen in only 50% of patients and following prolonged muscle use muscle strength will eventually decrease - limb-girdle weakness (affects lower limbs first) - hyporeflexia - autonomic symptoms: dry mouth, impotence, difficulty micturating - ophthalmoplegia and ptosis not commonly a feature (unlike in myasthenia gravis)
124
How is lambert-eaton syndrome investigated and managed?
EMG - incremental response to repetitive electrical stimulation Management - treatment of underlying cancer - immunosuppression, for example with prednisolone and/or azathioprine - intravenous immunoglobulin therapy and plasma exchange may be beneficial
125
tx of bell's palsy
pred eye drops
126
When are antiepileptic drugs started?
Most neurologists now start antiepileptics following a second epileptic seizure. NICE guidelines suggest starting antiepileptics after the first seizure if any of the following are present: - the patient has a neurological deficit - brain imaging shows a structural abnormality - the EEG shows unequivocal epileptic activity - the patient or their family or carers consider the risk of having a further seizure unacceptable Refer to epilepsy clinic after a first seizure.
127
How is idiopathic intracranial hypertension managed?
1. weight loss - whilst diet and exercise are important, medications such as semaglitide and topiramate may be considered by specialists. Topiramate is particularly beneficial as it also inhibits carbonic anhydrase 2. carbonic anhydrase inhibitors e.g. acetazolamide 3. topiramate is also used, and has the added benefit of causing weight loss in most patients 4. repeated lumbar puncture may be used as a temporary measure but is not suitable for longer-term management 5. surgery: optic nerve sheath decompression and fenestration may be needed to prevent damage to the optic nerve. A lumboperitoneal or ventriculoperitoneal shunt may also be performed to reduce intracranial pressure
128
What are the features of a brain abscess?
Brain abscesses may result from a number of causes including, extension of sepsis from middle ear or sinuses, trauma or surgery to the scalp, penetrating head injuries and embolic events from endocarditis The presenting symptoms will depend upon the site of the abscess (those in critical areas e.g. motor cortex) will present earlier. Abscesses have a considerable mass effect in the brain and raised intracranial pressure is common. - headache: often dull, persistent - fever: may be absent and usually not the swinging pyrexia seen with abscesses at other sites - focal neurology e.g. oculomotor nerve palsy or abducens nerve palsy secondary to raised intracranial pressure - other features consistent with raised intracranial pressure: nausea, papilloedema, seizures
129
How is a brain abscess investigated and managed?
Investigations Assessment of the patient includes imaging with CT scanning Management - surgery: a craniotomy is performed and the abscess cavity debrided, the abscess may reform because the head is closed following abscess drainage. - IV antibiotics: IV 3rd-generation cephalosporin + metronidazole - intracranial pressure management: e.g. dexamethasone
130
What are the features of multiple system atrophy?
There are 2 predominant types of multiple system atrophy 1) MSA-P - Predominant Parkinsonian features 2) MSA-C - Predominant Cerebellar features Shy-Drager syndrome is a type of multiple system atrophy. Features - parkinsonism - autonomic disturbance *erectile dysfunction: often an early feature *postural hypotension *atonic bladder - cerebellar signs
131
What are the features of multiple sclerosis?
Patient's with multiple sclerosis (MS) may present with non-specific features, for example around 75% of patients have significant lethargy. Diagnosis can be made on the basis of two or more relapses and either objective clinical evidence of two or more lesions or objective clinical evidence of one lesion together with reasonable historical evidence of a previous relapse. Visual optic neuritis: common presenting feature optic atrophy Uhthoff's phenomenon: worsening of vision following rise in body temperature internuclear ophthalmoplegia Sensory pins/needles numbness trigeminal neuralgia Lhermitte's syndrome: paraesthesiae in limbs on neck flexion Motor spastic weakness: most commonly seen in the legs Cerebellar ataxia: more often seen during an acute relapse than as a presenting symptom tremor Others urinary incontinence sexual dysfunction intellectual deterioration
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What are the features of venous sinus thrombosis?
Overview - can cause cerebral infarction, much lesson common than arterial causes - 50% of patients have isolated sagittal sinus thromboses - the remainder have coexistent lateral sinus thromboses and cavernous sinus thromboses General features Common features headache (may be sudden onset) nausea & vomiting reduced consciousness
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How are venous sinus thromboses investigated and managed?
Investigation - MRI venography is the gold standard: CT venography is an alternative - non-contrast CT head is normal in around 70% of patients - D-dimer levels may be elevated Management - anticoagulation *typically with low molecular weight heparin acutely *warfarin is still generally used for longer term anticoagulation
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What is Stephen-Johnson syndrome?
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are a spectrum of the same pathology, where a disproportional immune response causes epidermal necrosis, resulting in blistering and shedding of the top layer of skin. Generally, SJS affects less that 10% of body surface area whereas TEN affects more than 10% of body surface area. Certain HLA genetic types are at higher risk of SJS and TEN.
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What are the causes of stephen-johnson syndrome/ toxic epidermal necrolysis?
Medications - Anti-epileptics - Antibiotics - Allopurinol - NSAIDs Infections - Herpes simplex - Mycoplasma pneumonia - Cytomegalovirus - HIV
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What are the presenting features of stephen-johnson syndrome/ toxic epidermal necrolysis?
The condition has a spectrum of severity. Some cases are mild whilst others are very severe and can potentially be fatal. Patients usually start with non-specific symptoms of fever, cough, sore throat, sore mouth, sore eyes and itchy skin. They then develop a purple or red rash that spreads across the skin and starts to blister. A few days after the blistering starts, the skin starts to break away and shed leaving the raw tissue underneath. Pain, erythema, blistering and shedding can also happen to the lips and mucous membranes. Eyes can become inflamed and ulcerated. It can also affect the urinary tract, lungs and internal organs.
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How is stephen-johnson syndrome/ toxic epidermal necrolysis managed?
SJS and TEN are medical emergencies and patients should be admitted to a suitable dermatology or burns unit for treatment. Good supportive care is essential, including nutritional care, antiseptics, analgesia and ophthalmology input. Fluid replacement via intravenous and nasogastric access is required, as there is significant fluid loss from the blisters and red, oozing dermis. Treatment options include steroids, immunoglobulins and immunosuppressant medications guided by a specialist.
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What are the complications of stephen-johnson syndrome/ toxic epidermal necrolysis managed?
- Secondary infection: The breaks in the skin can lead to secondary bacterial infection, cellulitis and sepsis. - Permanent skin damage: Skin involvement can lead to scarring and damage to skin, hair, nails, lungs and genitals. - Visual complications: Depending on the severity, eye involvement can range from sore eyes to severe scarring and blindness.
139
What is autonomic dysreflexia?
This clinical syndrome occurs in patients who have had a spinal cord injury at, or above T6 spinal level. Briefly, afferent signals, most commonly triggered by faecal impaction or urinary retention (but many other triggers have been reported) cause a sympathetic spinal reflex via thoracolumbar outflow. The usual, centrally mediated, parasympathetic response however is prevented by the cord lesion. The result is an unbalanced physiological response, characterised by extreme hypertension, flushing and sweating above the level of the cord lesion, agitation, and in untreated cases severe consequences of extreme hypertension have been reported, e.g. haemorrhagic stroke. Management of autonomic dysreflexia involves removal/control of the stimulus and treatment of any life-threatening hypertension and/or bradycardia.
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What is status epilepticus?
Status epilepticus is defined as: a single seizure lasting >5 minutes, or >= 2 seizures within a 5-minute period without the person returning to normal between them This is a medical emergency. The priority is the termination of seizure activity, which if prolonged will lead to irreversible brain damage.
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How is status epilepticus managed?
ABC: airway adjunct, oxygen, check blood glucose First-line drugs are benzodiazepines - in the prehospital setting PR diazepam or buccal midazolam may be given - in hospital IV lorazepam is generally used. This may be repeated once after 5-10minutes. - If ongoing (or 'established') status it is appropriate to start a second-line agent such as levetiracetam, phenytoin or sodium valproate NICE state 'Take into account that levetiracetam may be quicker to administer and have fewer adverse effects than the alternative options.' - If no response ('refractory status') within 45 minutes from onset, then the best way to achieve rapid control of seizure activity is induction of general anaesthesia or phenobarbital
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What is syringomyelia and what are the causes?
Syringomyelia (‘syrinx’ for short) describes a collection of cerebrospinal fluid within the spinal cord. Syringobulbia is a similar phenomenon in which there is a fluid-filled cavity within the medulla of the brainstem. This is often an extension of the syringomyelia but in rare cases can be an isolated finding. Causes include: - a Chiari malformation: strong association - trauma - tumours - idiopathic
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What are the features of syringomyelia?
1. a ‘cape-like’ (neck, shoulders and arms) *loss of sensation to temperature but the preservation of light touch, proprioception and vibration (classic examples are of patients who accidentally burn their hands without realising) this is due to the crossing spinothalamic tracts in the anterior commissure of the spinal cord being the first tracts to be affected 2. spastic weakness (predominantly of the lower limbs) 3. neuropathic pain 4. upgoing plantars 5. autonomic features: 6. Horner’s syndrome due to compression of the sympathetic chain, but this is rare 7. bowel and bladder dysfunction 8. scoliosis will occur over a matter of years if the syrinx is not treated
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How is syringomyelia investigated and managed?
Investigations - full spine MRI with contrast to exclude a tumour or tethered cord - a brain MRI is also needed to exclude a Chiari malformation Treatment will be directed at treating the cause of the syrinx. In patients with a persistent or symptomatic syrinx, a shunt into the syrinx can be placed.
145
Which medication is used to treat cerebral oedema in patients with brain tumours?
Dexamethasone
146
What are the DVLA driving rules for TIA and stroke?
stroke or TIA: 1 month off driving, may not need to inform DVLA if no residual neurological deficit multiple TIAs over short period of times: 3 months off driving and inform DVLA
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How can you differentiate between cerebellar lesions based on ataxia?
Cerebellar hemisphere lesions cause peripheral ('finger-nose ataxia') Cerebellar vermis lesions cause gait ataxia
148
What are the common primary cancers that metastasise to the brain?
The most common secondary brain tumours are lung, breast, kidney, melanoma and colorectal cancers metastases.
149
What is degenerative cervical myelopathy?
Degenerative cervical myelopathy (DCM) is the most common form of spinal cord dysfunction in adults. It occurs when age-related osteoarthritic changes cause narrowing of the cervical spinal canal, leading to chronic spinal cord compression and neurologic disability.
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What are the presenting features of degenerative cervical myelopathy?
DCM symptoms can include any combination of: - Pain (affecting the neck, upper or lower limbs) - Loss of motor function (loss of digital dexterity, preventing simple tasks such as holding a fork or doing up their shirt buttons, arm or leg weakness/stiffness leading to impaired gait and imbalance - Loss of sensory function causing numbness - Loss of autonomic function (urinary or faecal incontinence and/or impotence) - these can occur and do not necessarily suggest cauda equina syndrome in the absence of other hallmarks of that condition - Hoffman's sign: is a reflex test to assess for cervical myelopathy. It is performed by gently flicking one finger on a patient's hand. A positive test results in reflex twitching of the other fingers on the same hand in response to the flick.
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How is degenerative cervical myelopathy diagnosed and managed?
An MRI of the cervical spine is the gold standard test where cervical myelopathy is suspected. It may reveal disc degeneration and ligament hypertrophy, with accompanying cord signal change. All patients with degenerative cervical myelopathy should be urgently referred for assessment by specialist spinal services (neurosurgery or orthopaedic spinal surgery). This is due to the importance of early treatment. The timing of surgery is important, as any existing spinal cord damage can be permanent. Early treatment (within 6 months of diagnosis) offers the best chance of a full recovery but at present, most patients are presenting too late. Currently, decompressive surgery is the only effective treatment. It has been shown to prevent disease progression. Close observation is an option for mild stable disease, but anything progressive or more severe requires surgery to prevent further deterioration. Physiotherapy should only be initiated by specialist services, as manipulation can cause more spinal cord damage.
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What is Miller-Fischer disease?
A variant of Guillain-Barre syndrome The condition is characterised by autoimmune-mediated demyelination of the peripheral nervous system, which may be triggered by a recent infection (especially Campylobacter jejuni). The Miller Fischer variant of Guillian-Barre syndrome classically presents with progressive proximal weakness and a triad of ophthalmoplegia, areflexia and ataxia. This diagnosis is confirmed by nerve conduction studies, which show decreased conduction velocity secondary to demyelination.
153
When are anticoagulants given in stroke following AF?
anticoagulants should not be started until brain imaging has excluded haemorrhage, and usually not until 14 days have passed from the onset of an ischaemic stroke'
154
When is aspirin given in an ischaemic stroke <4.5hrs presentation?
24 hours after the alteplase
155
What GCS indicates emergency intubation?
8 or less
156
What are the features of a fourth nerve palsy?
vertical diplopia - classically noticed when reading a book or going downstairs subjective tilting of objects (torsional diplopia) the patient may develop a head tilt, which they may or may not be aware of when looking straight ahead, the affected eye appears to deviate upwards and is rotated outwards
157
What is a post-lumbar puncture headache and how is it managed?
Headache following lumbar puncture (LP) occurs in approximately one-third of patients. The pathophysiology of is unclear but may relate to a 'leak' of CSF following dural puncture. Post-LP headaches are more common in young females with a low body mass index Typical features usually develops within 24-48 hours following LP but may occur up to one week later may last several days worsens with upright position improves with recumbent position Factors which may contribute to headache: Increased needle size Direction of bevel Not replacing the stylet Increased number of LP attempts Factors which do not contribute to headache: Increased volume of CSF removed Bed rest following procedure Increased fluid intake post procedure Opening pressure of CSF Position of patient Management supportive initially (analgesia, rest) if pain continues for more than 72 hours then specific treatment is indicated, to prevent subdural haematoma treatment options include: blood patch, epidural saline and intravenous caffeine
158
When and how can antiepileptics be stopped?
If seizure free for >2 years, stopped over 2-3 months
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How is status epilepticus managed if not responding to benzos?
In status epilepticus, IV levetiracetam, phenytoin or sodium valproate should be given if not responding to benzodiazepines
160
How is neuropathic pain managed?
- first-line treatment*: amitriptyline, duloxetine, gabapentin or pregabalin if the first-line drug treatment does not work try one of the other 3 drugs in contrast to standard analgesics, drugs for neuropathic pain are typically used as monotherapy, i.e. if not working then drugs should be switched, not added - tramadol may be used as 'rescue therapy' for exacerbations of neuropathic pain - topical capsaicin may be used for - localised neuropathic pain (e.g. post-herpetic neuralgia) pain management clinics may be useful in patients with resistant problems
161
What is 'saturday night palsy'?
Radial nerve palsy
162
Which nerve roots does the triceps reflex correspond to?
C7-C8
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Which nerve roots does the ankle reflex correspond to?
S1-S2
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Which nerve roots does the knee reflex correspond to?
L3-L4
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Which nerve roots does the biceps reflex correspond to?
C5-C6
166
What are the features by type of MND?
1. Amyotrophic lateral sclerosis (50% of patients) typically LMN signs in arms and UMN signs in legs in familial cases the gene responsible lies on chromosome 21 and codes for superoxide dismutase 2. Primary lateral sclerosis UMN signs only 3. Progressive muscular atrophy LMN signs only affects distal muscles before proximal carries best prognosis 4. Progressive bulbar palsy palsy of the tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei carries worst prognosis
167
What are the standout features of a posterior communicating artery aneurysm?
PAINFUL 3rd nerve palsy Pupil dilated
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How is disability after stroke measured?
Disability is most commonly measured using the Barthel index (BI), an outcome measure for stroke - Describes 10 tasks, and is scored according to amount of time or assistance required by the patient for each given task - Tasks: feeding, moving from wheelchair to bed, personal toileting, getting on/off toilet, bathing, walking on level surface, ascending/descending stairs, dressing, controlling bowels and controlling bladder - The total score is from 0 to 100, with 0 being completely dependent, and 100 being completely independent
169
What are the features of charcot-marie-tooth disease?
Charcot-Marie-Tooth Disease is the most common hereditary peripheral neuropathy. It results in a predominantly motor loss. There is no cure, and management is focused on physical and occupational therapy. Features: There may be a history of frequently sprained ankles Foot drop High-arched feet (pes cavus) Hammer toes Distal muscle weakness Distal muscle atrophy Hyporeflexia Stork leg deformity
170
What is thoracic outlet syndrome?
Thoracic outlet syndrome (TOS) is a disorder involving compression of brachial plexus, subclavian artery or vein at the site of the thoracic outlet. TOS can be neurogenic or vascular; the former accounts for 90% of the cases. TOS develops when neck trauma occurs to individuals with anatomical predispositions neck trauma can either be a single acute incident or repeated stresses anatomical anomalies can either be in the form of soft tissue (70%) or osseous structures (30%) a well-known osseous anomaly is the presence of cervical rib examples of soft tissue causes are scalene muscle hypertrophy and anomalous bands there is usually a history of neck trauma preceding TOS
171
What is the ROSIER score?
ROSIER is an acronym for 'Recognition Of Stroke In the Emergency Room'. It is the tool recommended by NICE to assess stroke symptoms in an acute setting. Exclude hypoglycaemia first, then assess the following: Assessment Scoring Loss of consciousness or syncope: - 1 point Seizure activity: - 1 point New, acute onset of: * asymmetric facial weakness: + 1 point * asymmetric arm weakness: + 1 point * asymmetric leg weakness: + 1 point * speech disturbance: + 1 point * visual field defect: + 1 point A stroke is likely if > 0.
172
What is the mechanism and side effect of lamotrigine?
Lamotrigine is an antiepileptic used second-line for a variety of generalised and partial seizures. Mechanism of action sodium channel blocker Adverse effects Stevens-Johnson syndrome
173
What are 5HT-3 antagonists and what are the complications?
5-HT3 antagonists are antiemetics used mainly in the management of chemotherapy-related nausea. They mainly act in the chemoreceptor trigger zone area of the medulla oblongata. Examples ondansetron palonosetron second-generation 5-HT3 antagonist main advantage is reduced effect on the QT interval Adverse effects prolonged QT interval constipation is common