Neurology Flashcards
What is the classification of strokes?
Oxford stoke classification AKA the Bamford classification
The following criteria should be assessed:
1. unilateral hemiparesis and/or hemisensory loss of the face, arm & leg
2. homonymous hemianopia
3. higher cognitive dysfunction e.g. dysphasia
What indicates a total anterior circulation infarct?
Total anterior circulation infarcts (TACI, c. 15%)
involves middle and anterior cerebral arteries
all 3 of the classification criteria are present
- unilateral hemiparesis and/or hemisensory loss of the face, arm & leg
- homonymous hemianopia
- higher cognitive dysfunction e.g. dysphasia
What indicates a Partial anterior circulation infarct?
involves smaller arteries of anterior circulation e.g. upper or lower division of middle cerebral artery
2 of the classification criteria are present
- unilateral hemiparesis and/or hemisensory loss of the face, arm & leg
- homonymous hemianopia
- higher cognitive dysfunction e.g. dysphasia
What indicated a lacunar infarct?
Lacunar infarcts (LACI, c. 25%)
involves perforating arteries around the internal capsule, thalamus and basal ganglia
presents with 1 of the following:
1. unilateral weakness (and/or sensory deficit) of face and arm, arm and leg or all three.
2. pure sensory stroke.
3. ataxic hemiparesis
What indicates a Posterior circulation infarct?
Posterior circulation infarcts (POCI, c. 25%)
involves vertebrobasilar arteries
presents with 1 of the following:
1. cerebellar or brainstem syndromes
2. loss of consciousness
3. isolated homonymous hemianopia
what is lateral medullary syndrome?
Lateral medullary syndrome (posterior inferior cerebellar artery)
aka Wallenberg’s syndrome
ipsilateral: ataxia, nystagmus, dysphagia, facial numbness, cranial nerve palsy e.g. Horner’s
contralateral: limb sensory loss
What is Weber’s syndrome?
Weber’s syndrome
ipsilateral III palsy
contralateral weakness
What is Wernike’s aphasia?
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
What is Broca’s aphasia?
expressive aphasia
Due to a lesion of the inferior frontal gyrus. Typically supplied by the superior devision of the left MCA.
Speech in non-fluent, laboured and halting. Repetition is impaired
Comprehension is normal
What is condition 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
What is 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
What are the features of stroke?
motor weakness
speech problems (dysphasia)
swallowing problems
visual field defects (homonymous hemianopia)
balance problems
Cerebral hemisphere infarcts may have the following symptoms:
contralateral hemiplegia: initially flaccid then spastic
contralateral sensory loss
homonymous hemianopia
dysphasia
what may a brainstem infarct lead to?
may result in more severe symptoms including quadriplegia and lock-in-syndrome
What are lacunar infarcts and how do they present?
small infarcts around the basal ganglia, internal capsule, thalamus and pons
this may result in pure motor, pure sensory, mixed motor and sensory signs or ataxia
what symptoms are patients with hemorrhagic stroke more likely to have?
decrease in the level of consciousness: seen in up to 50% of patients with a haemorrhagic stroke
headache is also much more common in haemorrhagic stroke
nausea and vomiting is also common
seizures occur in up to 25% of patients
what are the investigations for stroke?
Initially - Non contact CT (this will help rule out haemorrhage stroke, and can show early signs of ischaemic stroke) such as a loss of insular ribbon or hyper dense middle cerebral artery sign)
MRI - with diffusion weight imaging - more sensitive for detecting acute ischaemia
bloods - fbc, clotting profile, electrolytes,, lipid profile, BM
Further imaging
Echo
Carotid imaging
24 hour egg monitoring
Cerebral angiography
Differential Diagnosis for Stroke?
Migraine with Aura
Bell’s palsy (weakness will include the forehead where as in stroke it would be forehead sparing)
Hypoglycaemia
What is the management of stroke?
- blood glucose, hydration, oxygen saturation and temperature should be maintained within normal limits
- BP should not be lowered in the acute phase of an ischaemic stroke unless there are complications e.g. hypertensive encephalopathy or they are being considered for thrombolysis
Aspirin 300mg should be given orally/rectally ASAP if haemorrhage stroke has been excluded
If AF - anticoagulants should not be started until 14 days has passed from the onset of the stroke
If cholesterol > 3.5mmol/l patients should be commenced on statin (delay treatment for 48 hours due to risk of haemorrhagic transformation
Thrombolysis
Thrombectomy
When is thrombolysis indicated for acute ischaemic stroke ?
The standard criteria for thrombolysis with alteplase or tenecteplase are as follows:
it is administered within 4.5 hours of onset of stroke symptoms
haemorrhage has been definitively excluded (i.e. Imaging has been performed)
The 2023 National Clinical Guideline for Stroke broadened the potential inclusion criteria.
it recommends that patients with an acute ischaemic stroke, regardless of age or stroke severity, who were last known to be well more than 4.5 hours earlier, should be considered for thrombolysis with alteplase if:
treatment can be started between 4.5 and 9 hours of known onset, or within 9 hours of the midpoint of sleep when they have woken with symptoms, AND
they have evidence from CT/MR perfusion (core-perfusion mismatch) or MRI (DWI-FLAIR mismatch) of the potential to salvage brain tissue
this should be irrespective of whether they have a large artery occlusion and require mechanical thrombectomy.
there are specific criteria in the guidelines that determine the imagine criteria that determine whether thrombolysis should be performed
Blood pressure should be lowered to 185/110 mmHg before thrombolysis.
Absolute contraindications for thrombolysis
- Previous intracranial haemorrhage
- Seizure at onset of stroke
- Intracranial neoplasm
- Suspected subarachnoid haemorrhage
- Stroke or traumatic brain injury in preceding 3 months
- Lumbar puncture in preceding 7 days
- Gastrointestinal haemorrhage in preceding 3 weeks
- Active bleeding
- Oesophageal varices
- Uncontrolled hypertension >200/120mmHg
Relative contraindications for thrombolysis?
- Pregnancy
- Concurrent anticoagulation (INR >1.7)
- Haemorrhagic diathesis
- Active diabetic haemorrhagic retinopathy
- Suspected intracardiac thrombus
- Major surgery / trauma in the preceding 2 weeks
When is thrombectomy used in acute Ischaemic stroke?
NICE recommend a pre-stroke functional status of less than 3 on the modified Rankin scale and a score of more than 5 on the National Institutes of Health Stroke Scale (NIHSS)
Offer thrombectomy as soon as possible and within 6 hours of symptom onset, together with intravenous thrombolysis (if within 4.5 hours), to people who have:
acute ischaemic stroke and
confirmed occlusion of the proximal anterior circulation demonstrated by computed tomographic angiography (CTA) or magnetic resonance angiography (MRA)
Offer thrombectomy as soon as possible to people who were last known to be well between 6 hours and 24 hours previously (including wake-up strokes):
confirmed occlusion of the proximal anterior circulation demonstrated by CTA or MRA and
if there is the potential to salvage brain tissue, as shown by imaging such as CT perfusion or diffusion-weighted MRI sequences showing limited infarct core volume
Consider thrombectomy together with intravenous thrombolysis (if within 4.5 hours) as soon as possible for people last known to be well up to 24 hours previously (including wake-up strokes):
who have acute ischaemic stroke and confirmed occlusion of the proximal posterior circulation (that is, basilar or posterior cerebral artery) demonstrated by CTA or MRA and
if there is the potential to salvage brain tissue, as shown by imaging such as CT perfusion or diffusion-weighted MRI sequences showing limited infarct core volume
Secondary prevention for stroke?
clopidogrel
Carotid endarterectomy is recommend if the patient has suffered a stroke or TIA in the carotid territory and is not severely disabled
should only be considered if the stenosis > 50% according to North American Symptomatic Carotid Endarterectomy Trial (NASCET) criteria
what environmental factors may lead to epilepsy?
Prenatal and perinatal risk factors - maternal drug use, infection during pregnancy, preterm birth, low birth weight, neonatal seizures, Hypoxic ischaemic encephalopathy
CNS infections
Cerebrovascular disease - e.g. stroke
Traumatic brain injury
Brain tumours
Mixed genetic and environmental - neurocutaneous syndromes - conditions such as tuberous sclerosis complex, neurofibromatosis type 1 and Struge weber syndrome have genetic and environmental tigers
what are the different classifications of seizures?
Focal Onset Seizure
Generalised onset seizure
Unknown onset seizure
What are the different types of focal seizures?
Focal aware seizure (previously simple partial seizures) - individual remains conscious and can recall events during the seizure.
Focal Impaired awareness seizure (previously complex partial seizure) - involves alteration in consciousness - they may start as focal aware seizures and then progress
Focal to Bilateral Tonic-Clonic Seizures: Begin in one hemisphere and spread to involve both, resulting in a tonic-clonic seizure.
What are the different types of generalised onset seizures?
These arise at some point within, and rapidly engage, bilaterally distributed networks.
Tonic-Clonic Seizures: Characterised by stiffening (tonic phase) followed by rhythmic muscle jerking (clonic phase).
Absence Seizures: Brief lapses in consciousness, often with staring. They can be sub-classified into typical and atypical absence seizures.
Tonic Seizures: Cause stiffening.
Atonic Seizures: Lead to loss of muscle control, often resulting in falls (‘drop attacks’).
Clonic Seizures: Involves repetitive jerking movements.
Myoclonic Seizures: Quick, sudden jerks of a muscle or group of muscles.
How can epilepsy be classified based on epilepsy syndromes?
Childhood Absence Epilepsy (CAE): Characterised by typical absence seizures, with onset usually between 4-10 years.
Juvenile Myoclonic Epilepsy (JME): Marked by myoclonic jerks, typically shortly after waking.
Dravet Syndrome: Severe epilepsy beginning in infancy, initially presenting as prolonged seizures with fever.
Lennox-Gastaut Syndrome: Characterised by multiple seizure types, cognitive dysfunction, and a specific EEG pattern.
What investigations for epilepsy?
Bloods - FBC, Electrolytes, glucose, LFTs U&E’s
EEG - electoencephalogram
MRI
LP, Genetic testing, prolonged EEG monitoring
Differential diagnosis for Epilepsy?
Syncope
Psychogenic non-epileptic seizures
TIA
Alcohol withdrawal seizure
Seizures secondary to metabolic/toxic disturbance
Management of epilepsy
Normally start anti-epileptics after a second seizure
Sodium valproate is considered the first line treatment for patients with generalised seizures with carbamazepine used for focal seizures.
Generalised tonic-clonic seizures
sodium valproate
second line: lamotrigine, carbamazepine
Absence seizures* (Petit mal)
sodium valproate or ethosuximide
sodium valproate particularly effective if co-existent tonic-clonic seizures in primary generalised epilepsy
Myoclonic seizures**
sodium valproate
second line: clonazepam, lamotrigine
Focal seizures
carbamazepine or lamotrigine
second line: levetiracetam, oxcarbazepine or sodium valproate
*carbamazepine may exacerbate absence seizures
**carbamazepine may exacerbate myoclonic seizures
Driving advice following seizure
Can’t drive for 6 months following a seizure. If patients have established epilepsy then they must be seizure free for 12 months.
when would anti-epileptics be started after one seizures?
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
What is the acute management of seizures?
For people having a tonic-clonic seizure lasting more than 5 minutes, or who have more than three seizures in an hour
- Buccal midazolam as first-line treatment in the community.
Rectal diazepam if preferred, or if buccal midazolam is not available.
Intravenous lorazepam if intravenous access is already established and resuscitation facilities are available.
Cerbebellar signs
Dysdiadochokinesia & Dysmetria (past pointing)
Inability to perform and sustain a series of rapidly alternating muscle movements. The patient overshoots when attempting to reach a point with their finger
Ataxia
Gross incoordination of muscle movements. The patient may be very unsteady on their feet towards the side of the lesion. The patient may also show rebound, where pushing down on the outstretched upper limb causes it to rebound up past its original position
Nystagmus
A repetitive, involuntary oscillation of the eyes. The patient may also complain of blurred vision
Intention tremor
A wide tremor during voluntary movements, such as holding out the hands
Slurred speech
Speech may be imprecise, slow and distorted
Hypotonia
The patient may have muscle weakness on the side of the lesion
How are acute stroke services run?
The focus on management in hyperacute stroke units and the specialist services they offer (i.e. thrombolysis and thrombectomy)
Understanding that not every hospital has a hyperacute stroke unit - and hence knowing where and how to refer in the event of a suspected stroke
The existence of specialist and dedicated acute stroke teams in hospitals - including consultants, registrars and specialist nurses, enabling rapid responses and immediate specialist expertise for suspected strokes
“Time is brain” - investigations should not delay transfer to hyperacute stroke units
what is Myasthenia Gravis?
chronic autoimmune neuromuscular disease
The hallmark of the condition is muscle weakness that increases during periods of activity and improves after rest. It primarily affects the ocular muscles, leading to ptosis and diplopia, but can also involve bulbar, limb and respiratory muscles.
what are the autoantibodies seen in Myasthenia gravis?
postsynaptic nicotinic acetylcholine receptors (AChRs) at the neuromuscular junction, impairing neuromuscular transmission. Other patients may have antibodies against muscle-specific kinase (MuSK)
Aetiology of myasthenia gravis?
Genetic predisposition - HLA-B8, DR3 haplotype has been associated with early onset MG, HLA b27/ DR15/16 linked to late onset
Environmental triggers - certain infections can trigger an autoimmune response leading to MG.
- Thymoma is found in 10-15% of patients - these tumours can induce an immune response resulting in the productive of autoantibodies against acetylcholine receptors
Hormonal influnces
Myasthenia Gravis pathophysiology
autoantibodies against components of the neuromuscular junction (NMJ), particularly the nicotinic acetylcholine receptor (nAChR).
= reduction in the number and functionality of nAChRs at the postsynaptic membrane, thereby disrupting neurotransmission at the NMJ. The diminished nAChR density leads to a decreased safety factor for neuromuscular transmission, which manifests as muscle weakness in patients with MG.
Also AB against muscle specific kinase and lipoprotein-related protein 4 (LRP4) - both essential for proper NMJ formation and maintenance.
The presence of these autoantibodies and their respective pathogenic mechanisms contribute to the heterogeneity observed in MG clinical presentation.
Clinical features of MG?
The hallmark of MG is fluctuating muscle weakness that worsens with repetitive activity and improves with rest.
ptosis (drooping of the upper eyelid), diplopia (double vision), dysarthria (difficulty speaking), dysphagia (difficulty swallowing), and proximal limb weakness.
Myasthenic crisis - respiratory involvement - life threatening
which drugs may exacerbate myasthenia?
penicillamine
quinidine, procainamide
beta-blockers
lithium
phenytoin
antibiotics: gentamicin, macrolides, quinolones, tetracyclines
Investigations for myasthenia gravis?
Repetitive nerve stimulation
Single-fiber electromyography (most sensitive test)
Anti-acetylcholine receptor (AChR) antibodies
Anti-muscle-specific kinase (MuSK) antibodies
Anti-lipoprotein-related protein 4 (LRP4) antibodies
CT chest or MRI - to assess for thymoma or thymic hyperplasia
Myasthenia Differentials ?
Lambert-Eaton Syndrome (autoimmune disorder affecting the neuromuscular junction. However, it primarily affects voltage-gated calcium channels at the presynaptic nerve terminal). LES typically presents with proximal muscle weakness that improves with repeated use (unlike MG where symptoms worsen with use). - Commonly get dry mouth.
GBS - GBS is an immune-mediated polyradiculoneuropathy characterised by rapidly progressive bilateral limb weakness and loss of tendon reflexes. GBS often presents with ascending paralysis starting from the lower extremities. Sensory disturbances may also be present.
The progression of symptoms in GBS is usually over days to weeks, whereas in MG it is more variable and often fluctuates during the day.
Pupillary responses remain intact in GBS unlike in some cases of MG where pupillary involvement can occur due to cranial nerve involvement.
Botulism - a neurotoxic disorder caused by Clostridium botulinum that blocks acetylcholine release at the neuromuscular junction.
Unlike MG, botulism often presents with a descending paralysis starting from cranial nerves, leading to symptoms such as diplopia and dysphagia.
Autonomic symptoms including dry mouth, constipation and urinary retention are common in botulism but rare in MG.
In contrast to MG, muscle weakness in botulism does not improve with rest or anticholinesterase medications.
Management of Myasthenia Gravis?
Acetylcholinesterase inhibitors such as pyridostigmine.
If symptoms persist - consider immunosuppressive agents - Steroids are often first line - other options = azathiorprine, mycophenolate mofetil, cyclosporine.
IVIG or plasma exchange may be consider in severe cases or during myasthenia crisis
Surgical - thymectomy
Side effects of pyridostigmine?
abdominal cramps, diarrhoea and excessive salivation
Management of Myasthenia crisis?
n a myasthenic crisis, immediate hospitalisation is required. Intensive respiratory support may be necessary, including intubation and mechanical ventilation if there is impending respiratory failure.
Rapid short-term symptom control can be achieved by plasma exchange or IVIg.
What is Guillain-Barré syndrome?
acute polyradiculoneuropathy characterised by rapidly evolving ascending muscle weakness with mild sensory changes
Autoimmune
Precipitated by infection - most common Campylobacter, EBV, CMV
Occasionally vaccines have been associated with it
Some studies suggest an increased risk of GBS following surgical procedures
Pathophysiology of GBS?
cross reaction of antibodies with gangliosides in the peripheral nervous system
correlation between anti-ganglioside antibody (e.g. anti-GM1) and clinical features has been demonstrated
anti-GM1 antibodies in 25% of patients
Clinical features of GBS?
progressive weakness of all four limbs
Weakness is classically ascending i.e in the lower extremities
there may be a history of gastroenteritis
areflexia
cranial nerve involvement e.g. diplopia
autonomic involvement: e.g. urinary retention, diarrhoea
Investigations for GBS?
LP - rise in protein with a normal white blood cell count (albuminocytologic dissociation) found in 66%
Nerve conduction studies may be performed
What is Miller Fisher syndrome?
variant of Guillain-Barre syndrome
associated with ophthalmoplegia, areflexia and ataxia. The eye muscles are typically affected first
usually presents as a descending paralysis rather than ascending as seen in other forms of Guillain-Barre syndrome
anti-GQ1b antibodies are present in 90% of cases
Differentials for BGS?
- acute transverse myelitis (ATM) - apid onset of spinal cord dysfunction, which may mimic the ascending weakness seen in GBS. Key distinguishing features include:
Sensory level: A clear sensory level is indicative of ATM and not typically seen in GBS.
Bladder or bowel dysfunction: More commonly associated with ATM than GBS at onset.
MRI findings: In ATM, T2-weighted images often show hyperintense lesions in the spinal cord. - Chronic Inflammatory demyelinating polyneuropathy - similar to GBS - important differences:
Course of disease: CIDP has a more protracted course, developing over at least two months, while GBS typically presents acutely over days to weeks.
Response to treatment: CIDP patients usually respond well to immunosuppressive therapy and may require long-term treatment; whereas GBS is generally self-limiting once treated.
Botulism - can resemble but differences are -
Eyelid ptosis and fixed pupils: These are early signs in Clostridium botulinum poisoning but are not common in GBS.
History of food ingestion: A history of ingestion of home-canned, preserved or fermented foods can point towards botulism.
Descending paralysis: Botulism typically presents with a descending pattern of weakness, whereas GBS is classically ascending.
Management of GBS?
plasma exchange
IV immunoglobulins (IVIG): as effective as plasma exchange. No benefit in combining both treatments. IVIG may be easier to administer and tends to have fewer side-effects
steroids and immunosuppressants have not been shown to be beneficial
FVC regularly to monitor respiratory function
Complications of BGS?
Respiratory complications (mechanical ventilation is required in 30% of patients)
Autonomic dysregulation (labile BP, arrhythmias, cardiac arrest due to involvement of autonomic fibres)
DVT
Muscle atrophy
Contractures
pressure sores
Persistent numbers, tingling or pain in the limbs
Poor prognostic features in GBS?
age > 40 years
poor upper extremity muscle strength
previous history of a diarrhoeal illness (specifically Campylobacter jejuni)
high anti-GM1 antibody titre
need for ventilatory support
What is Bells palsy?
Bell’s palsy is an acute neurological condition presenting with rapid onset of unilateral facial paralysis. The exact cause is unknown but it has been linked to the herpes simplex virus.
risk factors of Bell’s palsy?
Pregnancy
Severe pre-eclampsia
Hypertension
Obesity
Diabetes mellitus
Upper respiratory tract infections
Clinical Features of Bells palsy?
unilateral facial paralysis
acute onset - 72 hours
the inability to wrinkle forehead and close eye fully on the affected side
oss of the nasolabial labial fold, and drooping of the mouth, which is more pronounced when the patient tries to smile
Pain in the ear and surrounding area
loss of taste in the anterior tongue
Hyperacusis (increases sensitivity to noise
**forehead sparing suggests an upper motor neurone cause
Differentials for Bell’s palsy?
Stoke or TIA
Brain tumour
Ramsey Hunt - reactivation of VZV which can cause unilateral facial paralysis but typically also has painful vesicular rash
Management of Bells palsy?
Good eye care
- artificial eye drops, use tape overnight, wearing sunglasses
Support nutrition with straws and soft diet
Prednisoolone if present within 72 hours of onset
Antivirals
Ensure follow up appointment to monitor recovery
complications of Bell’s palsy
Synkinesis: This is characterised by involuntary muscular movements accompanying voluntary movements. It occurs due to aberrant regeneration of the facial nerve and may present as crocodile tears syndrome (lacrimation during eating), gustatory sweating or hemifacial spasm.
Persistent facial weakness
Corneal keratitis
Lagophthalmos
Abrasive keratopathy
Prognosis of bells palsy?
Most patients can expect to see signs of recovery within 3 weeks and complete recovery within 4 months.
What is Neuroleptic malignant syndrome?
a rare, life-threatening condition triggered by neuroleptic or antipsychotic medication
a neurological emergency characterised by hyperthermia, altered mental status, muscle rigidity and autonomic dysregulation
Causes of Neuroleptic malignant syndrome?
ntipsychotics: Both typical (e.g., haloperidol) and atypical (e.g., risperidone) antipsychotics are primary offenders.
Other Medications: Antiemetics like metoclopramide, which have dopamine-blocking effects, may also trigger the syndrome.
Clinical features of Neuroleptic malignant syndrome?
It occurs within hours to days of starting an antipsychotic (antipsychotics are also known as neuroleptics, hence the name) and the typical features are:
- pyrexia
- muscle rigidity
- autonomic lability: typical features include hypertension, tachycardia and tachypnoea
- agitated delirium with confusion
Investigations for Neuroleptic malignant syndrome?
- primarily a clinical diagnosis based on the presence of cardinal symptoms: hyperthermia, autonomic dysregulation, altered mental status, and lead-pipe rigidity.
Bloods
Leukocytosis is commonly seen in NMS
May have elevated CK levels
If suspicion of neuroinflammatory condition which might mimic NMS - may need LP to exclude this
CT or MRI to exclude space occupying lesion
Management of Neuroleptic malignant syndrome?
stop offending agent
ICU admission
Hydrations and electrolyte management
Treatment of hyperthermia
Dopamine agonists if symptoms persist despite supportive care, considering dopamine agonists like bromocriptine and amantaide
Dantrolene can be considered in severe cases resistant to other treatments
Complications of Neuroleptic malignant syndrome?
Cardiovascular complications: These are amongst the most serious and frequent complications. They include arrhythmias, hypotension, myocardial infarction and cardiac arrest.
Pulmonary complications: Acute respiratory distress syndrome (ARDS), aspiration pneumonia, pulmonary oedema and respiratory failure may occur due to dysphagia and altered consciousness.
Renal complications: Acute kidney injury is a common complication of NMS. It is typically caused by rhabdomyolysis leading to myoglobinuria.
Musculoskeletal complications: Severe muscle rigidity can lead to rhabdomyolysis which can further cause renal impairment or failure.
Hepatic complications: Hepatotoxicity or liver dysfunction may occur due to drug-induced liver injury or secondary to systemic inflammatory response syndrome (SIRS).
causes of serotonin syndrome?
monoamine oxidase inhibitors
SSRIs
St John’s Wort, often taken over the counter for depression, can interact with SSRIs to cause serotonin syndrome
tramadol may also interact with SSRIs
ecstasy
amphetamines
Features of Serotonin syndrome?
neuromuscular excitation
hyperreflexia
myoclonus
rigidity
autonomic nervous system excitation
hyperthermia
sweating
altered mental state
confusion
Management of serotonin syndrome?
supportive including IV fluids
benzodiazepines
more severe cases are managed using serotonin antagonists such as cyproheptadine and chlorpromazine
what is MS?
Multiple sclerosis (MS) is an inflammatory demyelinating disease, clinically defined by two episodes of neurological dysfunction (brain, spinal cord or optic nerves) separated in space and time.
clinical features of MS?
Vision Problems (optic neuritis - temporary vision loss , scotoma, colour blindness and painful eye movement, pale optic disc on fundoscopy.
Fatigue
Rain and altered sensation - trinomial neuralgia, chest tightness, Lhermitte’s phenomenon (shock like sensation radiating dow the spine induced by neck flexion)
Muscle spasticity, stiffness and weakness
Mobility issues
bladder and bowel dysfunction
Sexual dysfunction
Depression and enxiety
Cognitive impairment
Speech and swallowing issues
Investigations for MS
Usual bloods to rule out alternatives
MRI brain
MRI brain
CSF examination if MRI is not diagnostic
Management of MS?
Acute relapse -High dose steroids
Disease modifying drugs Typical indications for disease-modifying drugs include:
relapsing-remitting disease + 2 relapses in past 2 years + able to walk 100m unaided
secondary progressive disease + 2 relapses in past 2 years + able to walk 10m (aided or unaided)
Options
Natalizumab
Ocrelizumab
fingolimod
Trial of amantadine for fatigue
Baclofen and gabapentin for spasticity
Causes of headache?
meningitis
encephalitis
subarachnoid haemorrhage
head injury
sinusitis
glaucoma (acute closed-angle)
tropical illness e.g. Malaria
Temporal arteritis?
Typically patient > 60 years old
Usually rapid onset (e.g. < 1 month) of unilateral headache
Jaw claudication (65%)
Tender, palpable temporal artery
Raised ESR
common causes of Meningitis?
Neisseria meningitidis, Streptococcus pneumoniae, or Haemophilus influenzae.
Signs of meningitis?
Classic Triad
- fever
- Neck stiffness (Nuchal rigidity)
- altered mental state
Malaise, headache, myalgia,
Kernig’s sign: In a supine patient with hips and knees flexed at 90 degrees, pain on straightening the knee suggests meningeal irritation.
Brudzinski’s sign: Passive flexion of one hip leading to involuntary flexion of the other hip is another indicator of meningeal irritation.
Investigations for meningitis?
Blood cultures
FBC
CRP and ESR
Blood glucose
LP
- microscopy and gram stain
- culture
- PCR
- CSF glucose and protein
Differentials for Meningitis?
Encephalitis - focal neurological signs such as hemiparesis or aphasia are more common in encephalitis.
In contrast to the nuchal rigidity frequently seen in meningitis, this is less commonly observed in encephalitis.
The presence of seizures at onset is more suggestive of encephalitis than meningitis.
SAH
Brain Abscess - Focal neurological deficits are common in brain abscess and less so in meningitis.
Brain abscess often has a more gradual onset of symptoms over days to weeks, while meningitis typically presents acutely.
Treatment for meningitis?
Empirical therapy with 3rd generation cephalosporin (ceftriaxone or cefotaxime) plus ampicillin
Add acyclovir
Complications of meningitis?
hearing loss
Cognitive impairment
seizures
Sepsis
Waterhouse-friderichsen syndrome - This is a severe complication of Neisseria meningitidis infection characterised by adrenal gland failure due to haemorrhage, leading to shock and disseminated intravascular coagulation (DIC).
Visual loss
Subdural effusions and empyemas
Cerebral abscesses
What are the causes of syncope?
Vasovagal syncope: This is the most common cause of syncope and occurs due to a sudden drop in blood pressure and heart rate. It is often triggered by emotional stress, pain, or standing for long periods.
Cardiac syncope: This type of syncope occurs due to a problem with the heart’s electrical system, such as an arrhythmia or heart block. It is more common in older adults and those with a history of heart disease.
Orthostatic hypotension: This is a sudden drop in blood pressure that occurs when a person stands up from a sitting or lying position. It can be caused by dehydration, medication side effects, or underlying medical conditions.
Neurological syncope: This type of syncope occurs due to a problem with the brain or nervous system, such as a seizure disorder or stroke. It is less common than the other types of syncope but should be considered in patients with a history of neurological conditions.
Hypoglycemia: Low blood sugar levels can cause syncope, especially in patients with diabetes who take insulin or other medications that lower blood sugar levels. It is less common than the other types of syncope but should be considered in patients with a history of diabetes or other metabolic disorders.
what is situational syncope?
Situational syncope: This occurs during specific situations like micturition, defecation or coughing. The pathophysiology involves an abrupt drop in blood pressure due to a strong vagal response.
what is carotid sinus hypersensitivity?
Carotid sinus hypersensitivity: It’s characterised by an exaggerated response to carotid sinus baroreceptor stimulation leading to bradycardia and/or vasodilation. It often presents in elderly patients who turn their head or wear tight collars.
what is BPPV?
Benign paroxysmal positional vertigo (BPPV) is one of the most common causes of vertigo encountered. It is characterised by the sudden onset of dizziness and vertigo triggered by changes in head position. The average age of onset is 55 years and it is less common in younger patients
Features of BPPV?
Features
vertigo triggered by change in head position (e.g. rolling over in bed or gazing upwards)
may be associated with nausea
each episode typically lasts 10-20 seconds
positive Dix-Hallpike manoeuvre
rapidly lower the patient to the supine position with an extended neck
a positive test recreates the symptoms of benign paroxysmal positional vertigo
rotatory nystagmus
BPPV prognosis?
BPPV has a good prognosis and usually resolves spontaneously after a few weeks to months. Symptomatic relief may be gained by:
Epley manoeuvre (successful in around 80% of cases)
teaching the patient exercises they can do themselves at home, termed vestibular rehabilitation, for example Brandt-Daroff exercises
Medication is often prescribed (e.g. Betahistine) but it tends to be of limited value.
Around half of people with BPPV will have a recurrence of symptoms 3-5 years after their diagnosis
what is viral labyrinthitis?
Viral labyrinthitis is an inflammatory condition of the inner ear, specifically affecting the labyrinth, which comprises the vestibular system and the cochlea. It is characterized by a sudden onset of vertigo, hearing loss, and tinnitus, typically following a viral infection.
clinical features of viral labyrinthitis ?
vertigo accompanied by N&V
Hearing loss
Tinnitus
spontaneous unidirectional horizontal nystagmus towards the unaffected side
sensorineural hearing loss: shown by Rinne’s test and Weber test
abnormal head impulse test: signifies an impaired vestibulo-ocular reflex
gait disturbance: the patient may fall towards the affected side
treatment of Viral labyrinthitis?
episodes are usually self-limiting
prochlorperazine or antihistamines may help reduce the sensation of dizziness
what is vestibular neuronitis?
Vestibular neuronitis is a cause of vertigo that often develops following a viral infection.
Features of vestibular neuronitis?
recurrent vertigo attacks lasting hours or days
nausea and vomiting may be present
horizontal nystagmus is usually present
no hearing loss or tinnitus
Differential diagnosis?
viral labyrinthitis
posterior circulation stroke: the HiNTs exam can be used to distinguish vestibular neuronitis from posterior circulation stroke
management of vestibular neuronitis?
buccal or intramuscular prochlorperazine is often used to provide rapid relief for severe cases
a short oral course of prochlorperazine, or an antihistamine (cinnarizine, cyclizine, or promethazine) may be used to alleviate less severe cases
vestibular rehabilitation exercises are the preferred treatment for patients who experience chronic symptoms
what is Meziere’s disease?
Meniere’s disease is a disorder of the inner ear of unknown cause. It is characterised by excessive pressure and progressive dilation of the endolymphatic system.
what are the risk factors and underlying causes of Ménière’s disease?
Age - 20-50
Female
family history
Underlying causes
Viral infections - HSV
Allergies
Autoimmune disorder
vascular factors
Genetic factors
Clinical features of Ménière’s disease?
recurrent episodes of vertigo, tinnitus and hearing loss (sensorineural). Vertigo is usually the prominent symptom
Aural fullness
Nystagmus and +ve Romberg test
Managemt on Ménière’s disease?
ENT assessment
Inform DVLA
Acute attacks - buccal or IM prochlorperazine.
Prevention - betahistine and vestibular rehabilitation exercises may be of benefit
what examination can be used to determine between peripheral or central vertigo?
HINTS examination - positive HINTS = likely central cause of vertigo
Only used in normal full euro exam, persistent vertigo and nystagmus
head impulse test, evaluation of nystagmus, and a test of skew.
What is Romberg’s test?
This test assesses the patient’s ability to maintain balance. The patient stands with feet together and eyes closed. A positive test, indicating proprioceptive or vestibular dysfunction, is signalled by swaying or falling.
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.
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
Management -
carbamazepine is first-line
failure to respond to treatment or atypical features (e.g. < 50 years old) should prompt referral to neurology
Red flag symptoms in trigeminal neuralgia?
Sensory changes
Deafness or other ear problems
History of skin or oral lesions that could spread perineurally
Pain only in the ophthalmic division of the trigeminal nerve (eye socket, forehead, and nose), or bilaterally
Optic neuritis
A family history of multiple sclerosis
Age of onset before 40 years
Features of Temporal arteritis?
typically patient > 60 years old
usually rapid onset (e.g. < 1 month)
headache (found in 85%)
jaw claudication (65%)
vision testing is a key investigation in all patients
anterior ischemic optic neuropathy accounts for the majority of ocular complications. It results from occlusion of the posterior ciliary artery (a branch of the ophthalmic artery) → ischaemia of the optic nerve head. Fundoscopy typically shows a swollen pale disc and blurred margins
may result in temporary visual loss - amaurosis fugax
permanent visual loss is the most feared complication of temporal arteritis and may develop suddenly
diplopia may also result from the involvement of any part of the oculomotor system (e.g. cranial nerves)
tender, palpable temporal artery
around 50% have features of PMR: aching, morning stiffness in proximal limb muscles (not weakness)
also lethargy, depression, low-grade fever, anorexia, night sweats
What is Temporal Arteritis?
Temporal arteritis (also known as giant cell arteritis: GCA) is a vasculitis of unknown cause that affects medium and large-sized vessels arteries. It occurs in those over 50 years old, with a peak incidence in patients who are in their 70s.
Investigations for temporal arteritis?
raised inflammatory markers
ESR > 50 mm/hr (note ESR < 30 in 10% of patients)
CRP may also be elevated
temporal artery biopsy
skip lesions may be present
note creatine kinase and EMG normal
