Revise Notes Neurology Flashcards
1
Q
Brain Metastases
Key learning
Brain metastasis are the most common form of brain tumour
Most commonly spread from:
Lung (especially small-cell)
Breast
Melanoma
Renal cell
Colorectal cancer
Investigation:
Gold standard MRI head with contrast
Management:
Dexamethasone to reduce oedema
MDT- surgery vs radiotherapy vs best supportive care
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Epidemiology
Most common type of brain tumour
Metastases spread via bloodstream
Around 15% of cancer patients develop brain metastases
Most commonly spread from:
Lung (especially small-cell)
Breast
Melanoma
Renal cell
Colorectal cancer
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Q
Clinical Features of brain mets
Headaches
Focal neurological deficits (weakness, sensory changes)
Seizures
Cognitive or personality disturbances
Signs of increased intracranial pressure (vomiting)
Examination Findings
Focal deficits corresponding to the location of the metastatic lesion:
Hemiparesis
Cranial nerve palsies
Ataxia
Signs of increased intracranial pressure:
Papilloedema
A
Management
Initial:
Oral or IV steroids (dexamethasone) commonly used to reduce intracranial oedema
Long term:
Oncology MDT- neurosurgeon, neuro-oncologist, neuro-radiologist, palliative care
Options include:
Surgery- if limited brain metastases, surgically accessible, patient has good performance status
Stereotactic radiosurgery- large dose of radiation to targeted area usually in single fraction
Whole-brain radiotherapy- if poor performance status or many brain metastases
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Q
Brain mets investigation
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Investigations
Initially CT head
MRI head with contrast- gold standard
CT-TAP /tumour markers/PET if brain metastasis found and unknown primary
Biopsy may be performed for histological confirmation and to guide treatment decisions
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Q
Normal pressure hydrocephalus (NPH)
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Pathophysiology
Impaired CSF absorption at the arachnoid villi results in excess CSF within the ventricles.
Clinical features
A triad of:
Urinary incontinence
Abnormal Gait
Festinating, resemblant of parkinsonism
Slow, short-stepped
Feet “glued to the floor”
Confusion / dementia
Mnemonic: wet, wobbly & ‘wacky’
Investigations
CT /MRI - Enlarged 4th Ventricle
Management
Trial lumbar puncture and if benefit, patient is for VP shunt
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Q
Wernicke’s encephalopathy
A neurological condition resulting from depletion of thiamine (B1) reserves, which causes petechial haemorrhages affect the mamillary bodies and ventricles.
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Clinical Features
A classic triad of:
Ophthalmoplegia with nystagmus
Ataxia
Confusion
Causes
Thiamine (B1) deficiency secondary to alcohol excess, malnourishment / refeeding
Diagnosis
WE is a clinical diagnosis
Investigations might include MRI which can confirm petechial haemorrhages as above.
Management
B1 replacement (pabrinex)
Complications
If untreated, thiamine deficiency can result in korsakoff’s dementia characterised by antero + retrograde amnesia with confabulation
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Q
Delirium
An acute, fluctuating, confusional state. Patients may have delusions or hallucinations.
Consciousness is impaired, and patients may be hyperactive, hypoactive or mixed.
A
Management
Conservative management strategies to reorientate the patient
Only if a patient is at risk to themselves or others pharmacological
management can be used: 1st line: Haloperidol
If the patient is on the SCP (Supportive Care Plan) – Midazolam is 1st line for agitation/restlessness
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Q
Creutzfeldt-Jakob disease (CJD)
A neurodegenerative disorder caused by abnormal prion proteins which cause the formation of amyloid folds and beta-pleated sheets.
Clinical Features
Dementia
Myoclonic jerks
Ataxia
Investigations
EEG - Sporadic CJD - biphasic high sharp spikes
MRI: basal ganglia/ thalamus - hyperintense signals
A
Types of CJD
Sporadic CJD – 85%
CSF – 14 – 3 -3 proteins present
New variant CJD
Affects younger patients (25 yrs.)
Features: Anxiety, social withdrawal, dysphonia
‘Hockey stick’ MRI
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Q
Frontotemporal lobar degeneration
Clinical features
Young age of onset - commonly <65 yrs
Insidious onset of personality change + socially inappropriate behaviours
Memory is typically preserved
A
Subtypes of frontotemporal dementia
- Pick’s Disease (most common)
Focal gyral atrophy – ‘knife-blade’ appearance on neuroimaging
Microscopic changes: Pick bodies, gliosis, NF tangles + plaques
2.Chronic Progressive Aphasia/non-fluent aphasia
Non-fluent, halting speech. Preserved understanding.
- Semantic dementia – Progressive Fluent aphasia
Fluent but nonsensical speech
Management
AChIs/memantine are NOT recommended by NICE
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Q
Lewy Body Dementia
Lewy body dementia is a cause of dementia characterised by the presence of alpha-synuclein cytoplasmic inclusions (lewy bodies) in the substantia nigra / paralimbic regions / neocortex.
Note - 40% of patients with Alzheimer’s dementia also have Lewy bodies
A
Clinical features
In-attention and impairment of executive function (not just memory loss)
Fluctuating cognitive impairment
Parkinsonism
Visual hallucination
Diagnosis
DaT scan (SPECT)
Management
Acetylcholinesterase inhibitor / memantine as per AD
Caution: Neuroleptics must be avoided - can lead to irreversible parkinsonism
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Q
Vascular Dementia
Progressive cognitive decline resulting from vascular related brain injury. It is classically described as ‘stepwise’ in nature, whereby each small ischaemic injury causes a further decline in function.
A
Classification
Vascular dementia (VD) can be further categorised as:
Stroke related VD – 1 or several large strokes/ TIAS
Subcortical VD – small vessel disease
Mixed - VD + Alzheimer’s
Management
Optimisation of risk factors (BP, smoking, hypercholesterolaemia, diabetes)
Memantine and acetylcholinesterase inhibitors should only be used in cases of MIXED dementia (NOT if pure vascular dementia)
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Q
Conservative management
Group cognitive stimulation therapy
Pharmacological management
Mild-Moderate Dementia: Acetylcholinesterase inhibitors:
Donepezil (Relative contraindications: Bradycardia ; Side effects -insomia); rivastigmine; galantamine
2nd line: Memantine = NMDA receptor antagonist
Add memantine to 1st line treatments in moderate AD or if AChI are not tolerated
Severe AD - memantine monotherapy
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Alzheimer’s Disease
Alzheimer’s disease is the most common cause of dementia
Pathophysiology
Genetics
95% of cases are sporadic
5% of cases are autosomal dominant
Patients who have the apoprotein E allele E4 are at increased risk
Macroscopic pathology: Global cerebral atrophy, particularly affecting the cortex/hippocampus
Microscopic pathology:
Cortical plaques (formed by type A Beta-Amyloid protein)
Interneuronal neurofibrillary tangles
(Hyperphosphorylated TAV protein)
Chemical pathology: Acetylcholine deficiency
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Q
Dementia
A neurological syndrome characterised by progressive and irreversible cognitive decline and behavioural changes.
Diagnosing dementia
NICE recommend the following tools:
10-CS (10-point cognitive screener)
6-CIT (6-item cognitive impairment test)
A
Investigations: Assessment must rule out reversible causes of cognitive impairment
Neuroimaging - CT/MRI - rule out causes such as subdural haematoma, normal pressure hydrocephalus
Bloods: FBC, LFTs, ESR, CRP, UE, Calcium, HbA1c, TFTs, B12 and folate
Consider, if appropriate: urine MCS, CXR, ECG, syphilis and HIV testing
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Q
Brain Abscess
Aetiology
Can occur as a complication following sinusitis, otitis media or sepsis.
MCQs might suggest a history of nasal congestion/frontal headache followed by worsening of headache/fever/confusion etc.
A
Investigations & Management
Diagnosis: CT head
Surgery - craniotomy with debridement
IV Cephalosporin (ceftriaxone) + Metronidazole
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Q
Encephalitis
Inflammation of the brain which is most commonly caused by infection. Typical symptoms include confusion, headache, seizures, and symptoms of meningism.
Herpes Simplex Encephilitis
Cause
HSV-1 - affects the temporal + frontal lobes
A
Presentation
Fever, confusion, headache
Wernicke’s aphasia due to temporal lobe involvement
Investigations
LP – Lymphocytosis, raised protein, PCR - HSV
MRI - temporal + frontal lobe - inflammation/oedema.
T2 weighted MRI - increased signal in temp/frontal lobes.
Management
IV Aciclovir
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Q
A
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Q
Essential Tremor
A neurological disorder characterised by a bilateral tremor. It often presents with a familial pattern and affects classically improves with alcohol consumption. Propranolol or primodine are first line management options.
Pathophysiology
ET is an autosomal dominant disorder with variable penetrance, though the exact genetics remain unclear.
A
Clinical Features
Tremor:
Bilateral tremor: Most prominent when the hands are outstretched or during goal-directed movements (6-8 Hz).
Titubation: Head bobbing tremor may also be present.
Family History: Often a positive family history, given its autosomal dominant inheritance.
Improvement with alcohol - many patients report a reduction in tremor severity after consuming alcohol. This is a classic clue in SBAs.
Management
Pharmacological treatment:
Propranolol often used as first-line treatment to reduce tremor amplitude.
Primidone can be used as an alternative or adjunct to propranolol.
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Q
Genetic Neurological Disorders
Von Hippel Lindau
Pathology
A multisystem genetic disorder characterised by the formation of cysts and tumours
Genetics
Autosomal dominant inheritance
Mutation of VHL tumour suppressor gene (TSG) on Chr 3
A
Clinical Features
Renal cysts (pre-malignant) and renal clear cell carcinoma
Hemangioblastomas:
Cerebellar –> ataxia, balance difficulties or SAH
Retinal –> vitreous hemorrhage
Pheochromocytoma - sweating, hypertension, palpitations
Other - pancreatic cysts, epididymal cysts
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Q
Tuberous Sclerosis
Pathology
A rare neurocutaneous disorder characterised by the formation of benign tumours
Inheritance: Autosomal dominant
Clinical Features
A
Neurological: Learning disability, epilepsy
Cutaneous: Café au lait, Ash leaf spots, Shagreen Patch, Adenoma sebaceum
Tumours: Retinal Hamartomas, Cardiac rhabdomyomas, Renal angiomyolipomas
- can result in haemorrhage in kidneys (abdominal pain, heamaturia)
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Q
Neurofibromatosis
Pathology
An autosomal dominant genetic disorder characterised by the development of tumours within the nervous system
Clinical Features
Lisch nodules (iris hamartomas)
NF1 - Mutation on Chr 17:
Café au lait spots
Axillary freckling
Peripheral neurofibromas
Phaeochromocytoma
A
NF2 - Mutation on Chr 22:
Bilateral acoustic neuromas (vestibular schwannomas) - tinnitus, problems with balance, hearing loss
Cafe au lait patches (fewer than NF1)
Cataracts
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Q
Friedreich’s Ataxia
Genetics
Expansion of GAA trinucleotide repeats on the FXN gene on Chromosome 9
Friedrich’s AtaxiA - FAA = GAA
Inheritance: Autosomal recessive
Clinical Features
Presents between 5-15 years with Gait ataxia + Kyphoscoliosis
O/E: Absent reflexes - ankle jerk/extensor planters
A
Complications
HOCM - cause of death in 40% of patients
Diabetes mellitus (20%)
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Q
Ataxic telangiectasia
Inheritance
Autosomal recessive
Clinical features
Present at between 1-5 years of age (younger than FA) with:
A
Ataxia
Telangiectasia
Recurrent infections (especially chest inf) due to IgA deficiency
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Q
Myotonic Dystrophy
Pathology
Expansion of the C T G repeat on:
DM1: Chromosome 19 on DMPK gene (myotonic dystrophy protein kinase) gene
DM2: Chromosome 3 on ZNF9 gene
Inheritance: Autosomal dominant
Complications
Cardiomyopathy
Heart blockage
A
Clinical Features
Myotonic facies
Frontal balding
Cataracts
Dysarthria
Weakness:
DM1 – distal weakness
DM2 -Proximal weakness
Myotic handshake - prolonged, slow to relax
ECG: Long PR interval (20%)
Tip: Myotonic dystrophy - long handshake, long PR
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Q
A
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Q
Hereditary Motor & Sensory neuropathy (HMSN) (including Charcot Marie Tooth)
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Pathology
Axonal or demyelinating pathology affecting peripheral myelin protein (PMP)
Inheritance: Autosomal dominant
Clinical Features
Muscular weakness and atrophy
Inverted “champagne bottle” legs
Pes cavus
Claw toes
Foot drop is common
Epilepsy
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Gbs
GBS is an immune mediated neurological condition, characterised by acute ascending, symmetrical weakness.
Pathophysiology
Immune-mediated demyelinating disorder, resulting in a subacute neuropathy
Antibodies: Anti-Ganglioside antibodies– Anti-GM1
Clinical Features
60% of patients experience a preceding infection - most commonly this is gastroenteritis (so a history of diarrhoea in MCQs is common), or an infection of the respiratory tract.
Campylobacter jejuni - most commonly implicated pathogen
Cytomegalovirus can also cause GBS
Ascending paralysis, progressive, symmetrical, affecting the feet and legs first
Hyporeflexia - reflexes are absent or reduced
Very mild sensory loss might be noted
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Miller fisher variant
Clinical Features
Ophthalmoplegia
Ataxia present
Descending paralysis
Areflexia
Antibodies
Anti–GQ1B Abs
Investigation and management of Guillain-Barré/Miller fisher
Lumbar puncture - raised protein and normal wcc
Close monitoring of FVC - at risk of respiratory compromise
Management options
IV Immunoglobulins
Plasma exchange
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Subarachnoid haemorrhage
Causes
Traumatic SAH is the most common cause
Spontaneous SAH: Berry/Saccular Aneurysms – associated with ADPKD, Ehlers danlos, coarctation of the aorta
Investigations
1st line: CT head
SAH Findings: CT head shows a high-attenuating substance within the subarachnoid space, including the subarachnoid cisterns and sulci.
In the case of a negative CT:
NICE : If CT head done within 6 hours of symptom onset shows no evidence of SAH, do not routinely offer an LP and consider alternative causes
If CT head is done > 6 hours and is negative, consider LP
LP should be performed at least 12 hours after injury to identify xanthochromia
Management
Medical management:
Nimodipine - Prevents vasospasm and reduces secondary ischaemia
Refer to neurosurgery urgently and investigate for underlying cause - CT angiogram
Neurosurgical management:
Treat aneurysmal bleeding with endovascular coiling (interventional radiology),
or if innappropriate consider clipping as an alternative.
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Subdural haematoma
Clinical Features
Elderly patients or history of alcohol excess (brain atrophy increases risk of torn bridging veins),
Fluctuating consciousness
History of fall
Investigations
CT head - crescent shaped collection
Acute = hyperdense (light)
Chronic = hypodense (dark)
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Extradural haematoma
Clinical Features
Raised ICP - papilloedema, vomiting etc.
Lucid interval is common, and is a typical clue in MCQs
Initial brief LOC, followed by an asymptomatic period, followed by deterioration in symptoms/ coma onset etc.
The middle meningeal artery is the most common artery implicated
Investigations
CT head: Biconvex / lentiform appearance, most commonly adjacent to temporal bone
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Head Injuries & Intracranial Bleeds
NICE guidance: CT head guidelines (Adults - 16+)
Criteria for immediate CT head (within 1 hour)
Consciousness level
GCS of 12 or less during initial assessment
GCS <15 two hours post injury
Suspected fracture - open/depressed/basal skull #
Haemotympanum, panda eyes, battle sign, CSF leakage
Seizure or focal neurology
More than 1 vomit
Criteria for CT head within 8 hours (or 1 hour if presenting > 8hrs post-injury)
History of loss of consciousness or Amnesia plus any one of the following:
65+ yrs
Clotting disorders - e.g. liver cirrhosis
Retrograde amnesia of > 30 minutes immediately before injury
Dangerous mechanism - bike/pedestrian vs motor vehicle, fall from >1m/5 stairs
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Subdural haematoma
Clinical Features
Elderly patients or history of alcohol excess (brain atrophy increases risk of torn bridging veins),
Fluctuating consciousness
History of fall
Investigations
CT head - crescent shaped collection
Acute = hyperdense (light)
Chronic = hypodense (dark)
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Extradural haematoma
Clinical Features
Raised ICP - papilloedema, vomiting etc.
Lucid interval is common,
and is a typical clue in MCQs
Initial brief LOC, followed by an asymptomatic period, followed by deterioration in symptoms/ coma onset etc.
The middle meningeal artery is the most common artery implicated
Investigations
CT head: Biconvex / lentiform appearance, most commonly adjacent to temporal bone
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Head Injuries & Intracranial Bleeds
NICE guidance: CT head guidelines (Adults - 16+)
Criteria for immediate CT head (within 1 hour)
Consciousness level
GCS of 12 or less during initial assessment
GCS <15 two hours post injury
Suspected fracture - open/depressed/basal skull #
Haemotympanum, panda eyes, battle sign, CSF leakage
Seizure or focal neurology
More than 1 vomit
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Criteria for CT head within 8 hours (or 1 hour if presenting > 8hrs post-injury)
History of loss of consciousness or Amnesia plus any one of the following:
65+ yrs
Clotting disorders - e.g. liver cirrhosis
Retrograde amnesia of > 30 minutes immediately before injury
Dangerous mechanism - bike/pedestrian vs motor vehicle, fall from >1m/5 stairs
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Anticoagulated pts
Anticoagulated patients
For anticoagulated patients with a head injury, if no other indications:
Consider a CT head within 8 hours
OR CT head within 1 hour
if they present > 8 hours after the injury
Nb. 'anticoagulated' includes patients on antiplatelet treatment (except aspirin monotherapy)
References and Further Reading
NICE: Head injury: assessment and early management. Clinical guideline [CG232]. MAY 2023 URL: https://www.nice.org.uk/guidance/ng232/chapter/Recommendations#investigating-clinically-important-traumatic-brain-injuries
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Pituitary Apoplexy
Pathology
Sudden enlargement of pituitary tumour secondary to infarct or haemorrhage
Clinical Features
Similar to SAH - sudden onset, thunderclap headache
Bitemporal superior quadrantanopia
Extra-ocular nerve palsy
Electrolyte abnormalities
Features of pituitary insufficiency - hypotension
Management
IV hydrocortisone
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Secondary Headache
Idiopathic intracranial hypertension
Clinical Features
The patient is classically a young overweight female with headache, blurred vision.
Examination findings
Papilledema
CN6 Palsy
Enlarged blindspot
Diagnosis
CT head
LP with opening pressures
MRI
Management
Acetazolamide
LP (therapeutic/repeated)
Surgical measures
Optic nerve sheath decompression + fenestration
VP shunt
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Medication overuse headaches (MOH)
Clinical Features
Headache present on 15 days per month or more,
Occurring in a patient who frequently uses analgesia such as opiates/triptans.
Management
Simple analgesics and triptans should be stopped abruptly
Opiates should be gradually withdrawn
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Trigeminal Neuralgia
Clinical Features
Severe unilateral ‘electric-shock’ pains which affect divisions of the trigeminal nerve (CN5 - V1/V2/V3).
Pain is commonly triggered by touch (e.g washing hair, shaving etc.)
Red flags
Concomitant sensory changes
Deafness
Skin or oral lesions
OPHTHALMIC division ONLY affected
FHx of MS, optic neuritis
Age <40 yrs
Management
1st Line: Carbamazepine
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Chronic Paroxysmal Hemicrania
Clinical Features
20 attacks per day
Severe headaches each lasting 5-10 minutes
Associated eye signs - tearing, redness
Management
Indomethacin
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Cluster Headache
Clinical Features
A 'cluster' of severe headaches - 1-2 attacks per day for 2-6 weeks
Duration: approximately 30 minutes
Tip: the frequency and duration of attacks are important differentiating factors from CPH
Character: Severe pain which is sharp, and stabbing, often worst around eye
Normally associated with redness of eye, lacrimation, swelling, nasal congestion
Management
100% O2+ subcutaneous triptan
Prophylaxis: Verapamil
Lifestyle advice: Avoid alcohol – can trigger attacks
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Migraine
Clinical Features
Severe unilateral headache
Throbbing or pulsating in nature
Worsened by activity and eased by rest (e.g. lying in a darkened room)
Photophobia, phonophobia
Often associated with aura, most commonly visual
Management
1st line: Oral triptan (5-HT1 Agonist) PLUS either NSAID or paracetamol
Triptans are 5-HT1 agonists
If patients are aged 12-17 yrs, offer a nasal triptan)
2nd line: Add Metoclopramide/Prochlorperazine/non-oral NSAID or triptan
Pregnant patients – 1st line = paracetamol
Menstruation related migraine (occuring predictably during cycle) – Mefenamic acid
Avoid COCP - complete CI if migraine with aura
Prophylaxis
Prophylaxis for migraine is usually indicated if the patient experiences 2 or more migraines per month
1st Line – Propranolol, Topiramate (note - topiramate is teratogenic so propranolol is preferable in women of child bearing age)
2nd Line – Acupuncture 10 sessions over 5-8/52
Predictable Menstrual Migraine: Frovatriptan/Zolmitriptan 2.5mg BD - “mini-menstrual-prophylaxis”
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Primary Headaches
Headaches are an extremely common presenting complaint.
The most common causes of primary headache include tension type headaches, migraines and cluster headaches.
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Motor Neurone Disease
Motor neurone disease (MND) is a condition of unknown aetiology which affects the function of both upper and lower motor neurons. It typically affects patients > 40 years.
Clinical features
Commonly presents with asymmetrical weakness
Weakness in legs - tripping, difficulty climbing stairs
Weakness in hands - poor grip strength, unable to open jars, dropping things
Fasciculations are a key finding
Classically mixed upper and lower motor neuron signs (ALS)
No sensory loss (MOTOR neurone disease)
Atrophy/wasting of the tibialis anterior and thesmall muscles of the hand
MND does NOT affect the extra-ocular muscles, cerebellum, abdominal reflexes
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Patterns of MND
There are 4 main subtypes of MND.
🥳Amyotrophic lateral sclerosis (ALS)
Accounts for 50% of cases of MND
UMN signs predominantly affecting the lower limbs, LMN signs predominantly affecting the upper limbs
Familial MND can occur (Chromosome 21 defect affecting superoxide dismutase)
🥳Primary Lateral Sclerosis (PLS)
Affects the UMNs of the arms, legs and face.
🥳Progressive Muscular Atrophy (PMA)
Affects LMNs only
🥳Progressive bulbar palsy
Dysfunction of the brainstem motor nuclei
Affects the muscles of mastication/swallowing and tongue
Carries the worst prognosis
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Motor Neurone Disease
Motor neurone disease (MND) is a condition of unknown aetiology which affects the function of both upper and lower motor neurons.
It typically affects patients > 40 years.
Investigations
MND is a clinical diagnosis
NCS & EMG can aid diagnosis
Management
Riluzole - blocks glutamate receptors - 2-4 month survival benefit
NIV at night - 6 month survival benefit
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Multiple Sclerosis
Multiple sclerosis (MS) is a chronic neurological disorder characterised by plaques of demyelination which are disseminated in both time and space.
The pathology is autoimmune in nature.
Disease courses
Relapsing remitting MS 85% - acute attacks followed by periods of remission (65% will later develop secondary progressive MS, where symptoms and signs persist between relapses)
Primary progressive MS - progressive neurological deterioration from the onset of disease (without periods of remission) - accounts for 10%
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MS
Clinical Features
MS commonly presents at between 20-50 years of age.
Visual:
Optic neuritis - loss of/impaired vision in one eye, with pain on eye movements
INO – conjugate lateral gaze disorder - the affected eye does not aDduct
Sensory - paraesthesia
Motor - spastic weakness
Autonomic dysfunction
Urinary incontinence
Sexual dysfunction
Cerebellar - ataxic gait, unsteadiness
MS phenomena:
Uhthoff phenomenon - increase in temperatures worsen symptoms - e.g. classically symptoms are worse after getting out of a warm bath
Lhermitte’s phenomenon - neck flexion ---> paraesthesia
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Ms investigation
Investigations
MRI brain & spinal cord: High Signal lesions, periventricular plaques - identify demyelinating lesions within the CNS
CSF: Oligoclonal bands - reflect increased intrathecal IgG synthesis
VEPs (visual evoked potentials) - delayed, but preserved
Management
Acute attack
Corticosteroids - methylprednisolone 0.5g daily is standard (for 5 days)
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Disease modifying drugs
DMDs are indicated if 2 or more attacks within 2 years and significant functional limitation (e.g. unable to walk > 100m)
1st line: Natalizumab, ocrelizumab
Monoclonal antibodies which inhibit movement of inflammatory cells across the BBB into the CNS.
Alternatives
Fingolimod
Beta-Interferon: reduces relapses by 30%
Glatiramer acetate
Symptom management
Fatigue: Amantadine
Spasticity: 1st line: Baclofen, gabapentin
2nd line
Bladder dysfunction: Bladder emptying must be assessed first to guide Mx:
High residual vol - intermittent self – catheterisation
Low residual vol - anticholinergics
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Lambert-Eaton Myasthenic syndrome (LEMS)
A rare autoimmune condition in which patients develop proximal muscle weakness which improves initially with muscle use.
Pathophysiology
A paraneoplastic phenomenon due to the development of autoantibodies vs voltage gated calcium channels
in pre-synaptic neurons of peripheral nervous system
Causes:
Small Cell lung cancer
Ovarian ca.
breast ca.
Clinical features
Limb girdle weakness / proximal muscle weakness and aching - repeated contractions improves strength initially-
but then if sustained, strength worsens.
Hyporeflexia
Investigations
EMG - Incremental response to repetitive stimulation
Management
Treat malignancy, Immunosuppression
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Myasthenia Gravis
MG is a chronic autoimmune neuromuscular disorder characterised by fatigable weakness
Epidemiology
Peak incidence in women - 30-40 yrs
Peak incidence in men - 50-60 yrs
Pathophysiology
IgG antibodies vs nicotinic Acetylcholine receptor at the neuromuscular junction (90%) - results in fatiguable weakness
Clinical Features
Diplopia, ptosis, ophthalmoplegia
Dysphagia
Dysphonia – voice becomes quieter during talking (e.g. when counting to 100)
Girdle muscles weakness
Features worsen with fatigue ‘e.g. at the end of the day’, or improve following rest.
Investigations
1st line: serum anti-acetylcholine receptor antibody testing (90% positive)
If negative, 40% are positive for Anti-muscle specific kinase (anti-MuSK) antibodies
Single fibre EMG – decremental response (92-100% sensitivity) to repetitive nerve stimulation
CT Thorax - to identify the presence of a thymoma (15%)
The edrophonium test is rarely performed due to complications
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MG mng
Management
1st Line: Pyridostigmine (Long-acting acetylcholinesterase inhibitors)
Prednisolone may be used if there is limited benefit from pyridostigmine
Thymectomy
Crisis - plasmapheresis can be used, IV Igs
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Clinical Features
Excessive daytime sleepiness (EDS) - overwhelming urge to sleep during the day, regardless of the amount of nighttime sleep.
Disrupted nocturnal sleep: frequent awakenings during the night.
May be associated with cataplexy - sudden, brief episodes of muscle weakness, paralysis and loss of tone (may cause collapse) triggered by strong emotions, such as laughter or anger.
Sleep paralysis
Temporary inability to move or speak while falling asleep or waking up.
Often associated with hallucinations:
vivid, often frightening, dream-like experiences occurring while falling asleep (hypnagogic) or waking up (hypnopompic).
Narcolepsy & Cataplexy
Narcolepsy is a chronic neurological disorder caused by the brain's inability to regulate sleep-wake cycles normally.
The cause is commonly a deficiency of hypocretin (also known as orexin), a neurotransmitter that promotes wakefulness.
Investigations
Multiple sleep latency EEG
Measures the time it takes to fall asleep in a quiet environment during the day,
typically showing a short sleep onset and rapid entry into REM sleep.
Management
EDS:
1st line: Modafinil.
2nd line: Methylphenidate.
Sodium oxybate, though not widely available in Europe, is also effective.
Sodium oxybate can be combined with modafinil for severe cases of EDS.
Cataplexy:
Sodium oxybate and clomipramine are recommended.
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Sleep paralysis
Paralysis often in the presence of hallucinations occurring before/during/after sleep
Pharmacological Management: Clonazepam
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Holmes-Adie Pupil
Pathophysiology
Holmes-Adie pupil is due to postganglionic parasympathetic denervation of the pupil, often idiopathic. It is a benign condition.
Clinical Features
It classically affects young women (often a clue in questions).
Unilateral dilated pupil that reacts sluggishly to light but better to accommodation.
Hence, a common differential diagnoses in questions vs Argyll-Robertson pupil
May be associated with diminished or absent deep tendon reflexes, particularly the Achilles reflex.
H-A pupil is part of the Holmes-Adie syndrome, which includes absent deep tendon reflexes.
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Argyll-Robertson Pupil
Pathophysiology
Argyll-Robertson pupil is associated with lesions in the pretectal region of the midbrain.
It is classically linked to neurosyphilis, a manifestation of tertiary syphilis.
Other causes include diabetes mellitus and multiple sclerosis
Clinical Features
Small, irregular pupils.
Pupils react to accommodation but not to light.
Mnemonic: Argyll-Robertson Pupil: ARP LRA
Accommodation Reflex Present, Light Reflex Absent
Usually bilateral but can be asymmetric.
Often associated with other neurological signs of syphilis, such as tabes dorsalis and general paresis (dementia and motor weakness).
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Internuclear Ophthalmoplegia (INO)
Pathophysiology
INO results from a lesion in the medial longitudinal fasciculus (MLF)
The MLF coordinates horizontal eye movements by connecting the abducens nucleus of one side with the oculomotor nucleus of the other.
Common causes include multiple sclerosis in young patients and stroke in older individuals.
Alongside optic neuritis, INO is often a buzzword/key phrase suggesting MS in question vignettes.
Clinical Features
Impaired adduction of the ipsilateral eye.
Nystagmus of the contralateral eye when it abducts, i.e. while attempting horizontal gaze.
Diplopia during horizontal eye movements.
Bilateral INO can occur, especially in multiple sclerosis.
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Horner's Syndrome
Pathophysiology
Horner's syndrome results from disruption of the sympathetic nerves supplying the eye and face.
This can occur due to lesions at various levels:
Central - e.g. stroke, tumour
Preganglionic - e.g. lung apex tumour, cervical rib
Postganglionic - e.g. carotid artery dissection, cluster headaches
Clinical Features
Classic triad of:
Ptosis: Mild drooping of the upper eyelid due to loss of sympathetic innervation to the Muller muscle.
Miosis: Constricted pupil from unopposed parasympathetic activity.
Anhidrosis: Decreased sweating on the affected side of the face.
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Oculomotor (CN3) Palsy
Pathophysiology
Oculomotor nerve palsy involves the disruption of the third cranial nerve, which innervates most of the extraocular muscles, levator palpebrae superioris, and the pupillary sphincter.
This disruption can be caused by aneurysms, tumours, infections, or microvascular disease (commonly in diabetes).
The parasympathetic fibres, which control pupil constriction, are located peripherally on the nerve and are thus more susceptible to compressive lesions.
Clinical Features
Eye positioned down and out due to unopposed action of the lateral rectus and superior oblique muscles
Ptosis from loss of function in the levator palpebrae superioris
Diplopia (double vision)
Pupil involvement helps differentiate the cause:
Surgical third nerve palsy:
Pupil is dilated due to loss of parasympathetic function
Suggests a compressive lesion such as a posterior communicating artery aneurysm.
Medical third nerve palsy:
Pupil sparing suggests a medical (non-compressive) lesion such as microvascular pathology caused by diabetes or hypertension.
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Neuropathic Pain
Pain that results from abnormalities of the PNS or CNS. It is frequently described as sharp, shooting pain or may be described as 'burning', or electric shock like.
NICE advise the following pharmaceutical management options (this also applies to the management of painful diabetic neuropathy) :
1st line: gabapentin OR pregabalin OR amitriptyline OR duloxetine
2nd line: Stop the 1st line drug and try an alternative 1st line medication
Rescue Therapy: Tramadol / Topical caspaicin can be used if localised pain (e.g. post-herpetic neuralgia)
Consider capsaicin 0.075% cream for patients with localised neuropathic pain, who do not want/can't tolerate PO treatment options
Exceptions to the above NICE guidelines include:
Sciatica - use NSAIDs 1st line - there is no good evidence behind gabapentinoids
Trigeminal neuralgia - 1st line - carbamazepine
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Parkinsonism & Disorders of Movement
Parkinsonsim
Parkinsonism is a clinical syndrome characterised by a triad of:
Bradykinesia
Tremor
Rigidity
Other features include a festinating gait (small shuffling steps, stooped forward gait, absent arm swing), hypomimia (mask-like facial expression), micrographia.
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Parkinson’s causes
Causes
There are many different causes of parkinsonism, including:
Idiopathic Parkinson's disease
Drug induced parkinsonism
Lewy body dementia
Parkinson plus disorders including PSP, MSA, CBD (see later)
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Causes include
Antipsychotics – chlorpromazine/Haloperidoll/Droperidol
Dopamine receptor antagonist – Metoclopramide (unlike domperidone which does NOT cross blood brain barrier)
Treatment - Antimuscarinics: Procyclidine, Benzatropine, Benzhexol
Drug induced parkinsonism
Suspect drug induced parkinsonism if motor symptoms are of rapid onset and symmetrical
Akinesia with loss of arm swing is an early symptom
Bradykinesia is common, manifesting as hypomimia, difficulty with speech and slowness of movement.
Tremor is less common, and more suggestive of idiopathic PD
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Idiopathic Parkinson's disease
Pathology
Degeneration of dopaminergic neurones in substantia nigra
Clinical Features
As above - classically asymmetrical in nature (differentiating factor from drug induced parkinsonism) rigidity, tremor and bradykinesia.
Diagnosis
The diagnosis of PD is clinical
I-FP SPECT can aid diagnosis
Management
If motor symptoms affect quality of life:
1st Line: Levodopa
If motor symptoms do NOT affect quality of life, treatment options include
Non-ergot dopamine agonists
MAO-B inhibitors
Levodopa
If pt is already on Levodopa + has ongoing symptoms, consider an adjunct such as:
Dopamine agonist
MAO-B inhibitor
COMT inhibitor
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Levodopa
Levodopa is a precursor to dopamine
It is typically combined with a decarboxylase inhibitor –
inhibiting the peripheral conversion of levodopa to dopamine (optimising the amount of levodopa which is ‘available’ to the central nervous system).
Decreasing effect after 2 yrs
SEs:
SEs:
On-Off effect
NICE (NG71. 2017) recommends considering a protein redistribution diet, where the majority of dietary protein is consumed in the evening, for patients with motor fluctuations/on-off symptoms.
Dyskinesias (involuntary writhing)
Postural hypotension
Palpitations
Psychosis
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Dopamine Receptor Agonists
Non-ergot derived = Ropinirole/Apomorphine
Ergot derived = Cabergoline/Bromocriptine
Complications of ED DAs include: Pulmonary fibrosis + Cardiac fibrosis + Retroperitoneal fibrosis
Before commencing treatment, patients should undergo a baseline Echo, CXR, ESR, creatinine
Regular echocardiograms should be performed during treatment
SEs: Impulse control disorder, daytime Somnolence
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MAO – B Inhibitors
Selegiline - Inhibit dopamine breakdown
Amantadine
Functions via multiple mechanisms
SEs: cerebellar symptoms including ataxia
COMT-inhibitors
Entacapone, tolcapone
Mechanism: Prevent peripheral methylation of levodopa to 3-O-methyldopa – this increases the availability of levodopa to the CNS.
Other drugs
Glycopyrronium Bromide - Effective to reduce drooling in patients with PD
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Neuroleptic malignant syndrome
Causes
Antipsychotic medications
Or suddenly stopping Levodopa/ DA / PD meds
Clinical Features
Pyrexia + Rigidity + Delirium + ANS stimulation (tachycardia/hypertension)
Myoclonus is NOT present in NMS (this is suggestive of serotonin syndrome)
Investigations
High CK with subsequent AKI due to rhabdomyolysis
Management
Stop Antipsychotics
Start PD meds if missed
IV fluids
Dantrolene
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Parkinson’s Plus Syndromes
A group of neurological conditions characterised by the presence of parkinsonism, plus additional distinguishing features.
They include corticobasal degeneration (CBD), multiple system atrophy (MSA), and progressive supranuclear palsy (PSP).
Corticobasal degeneration
Parkinsonism
Difficulty controlling the limbs - alien hand, “limbs act independently”
Numbness and loss of motor coordination - apraxia
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Multiple system atrophy
Multiple system atrophy (MSA)
Parkinsonism with autonomic nervous system dysfunction
Postural hypotension and instability
Erectile dysfunction
Loss of bladder control, urinary retention due to atonic bladder
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Progressive Supranuclear Palsy (PSP)
Parkinsonism
Vertical gaze impairment (e.g. difficulty going down stairs)
Postural instability and an unsteady, broad based gait
Frequent falls, often backwards
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Pathology of the spinal cord tracts
Brown sequard syndrome
Brown-Séquard Syndrome
Pathology: Hemisection of the spinal cord
Clinical features:
Corticospinal tract damage: Ipsilateral weakness below the lesion
Dorsal columns damage: Ipsilateral loss of fine touch/proprioception
Lateral spinothalamic tract damage: Contralateral loss of pain/temperature sensation
Segmental anaesthesia: Loss of all sensory modalities at the level of the lesi
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Subacute Combined Degeneration of the Cord
Causes:
B12 deficiency (therefore associated with pernicious anaemia)
Friedreich's ataxia
Clinical features:
Spastic paresis: Bilateral corticospinal tracts affected
Loss of fine touch/proprioception/vibration: Bilateral dorsal columns affected
Limb ataxia: Spinocerebellar involvement
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Friedreich's Ataxia
Pathology/Cause: Genetic mutation (FXN gene) leading to degeneration of spinal cord and cerebellum
Clinical Features:
Spastic Paresis: Bilateral corticospinal tract involvement
Loss of Fine Touch/Proprioception/Vibration: Bilateral dorsal columns affected
Limb Ataxia: Involvement of spinocerebellar tracts
Lateral Spinothalamic Tract: Loss of pain and temperature sensation
Cerebellar Features - DANISHP:
Dysarthria, Ataxia, Nystagmus, Intention tremor, Scanning speech, Hypotonia, Past pointing
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Anterior Spinal Artery Occlusion
Pathology/Cause: Occlusion of the anterior spinal artery, e.g. thromboembolic disease
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Clinical Features:
Lateral corticospinal tract affected: Bilateral spastic paresis
Lateral spinothalamic tracts affected: Bilateral loss of temperature/pain sensation
Onset: Typically sudden, characteristic of a vascular cause
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Syringomyelia
Pathology: Formation of a cyst (syrinx) within the spinal cord causing compression on decussating nerves in the ventral white commissure
Clinical features:
Spinothalamic tract affected: Loss of pain and temperature sensation
Dissociated sensory loss: 'Cape-like' distribution or involving both arms
Anterior horn involvement: Bilateral flaccid paresis (lower motor neuron) and diffuse muscle atrophy starting in the hands, progressing proximally. Clawhand may develop.
Association: Strongly associated with Arnold-Chiari malformation
Diagnosis: MRI spine and brain (to rule out Chiari malformation)
Treatment: Surgery if symptomatic/progressive (including restoration of normal CSF flow and drainage of syrinx)
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Arnold-Chiari Malformation
Pathology: Downward displacement of cerebellar tonsils through the foramen magnum
Associations:
Syringomyelia
Hydrocephalus
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Transverse Myelitis
Causes: Often follows viral infections such as VZV, CMV, EBV, HIV
Presentation: Constitutional symptoms (fever, pain) plus cord compression-like symptoms
Diagnosis: MRI
Management: Steroids
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Tabes Dorsalis
Cause: Syphilis
Pathology: Damage to the dorsal column-medial lemniscus pathway
resulting in loss of proprioception and fine touch
Clinical features: High-stepping gait
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Restless leg syndrome
Restless Leg Syndrome (RLS) is a neurological disorder characterised by an uncontrollable urge to move the legs, usually due to an uncomfortable sensation.
It is believed to be linked to dysfunction in the brain's dopamine pathways, which play a role in controlling movement. Iron deficiency is also implicated, as it affects dopamine metabolism.
Clinical Features
Irresistible urge to move one's legs
Often accompanied by uncomfortable sensations described as crawling, tingling, or itching.
Symptoms are worse during periods of inactivity and are more pronounced in the evening or at night, leading to sleep disturbances.
Movement provides temporary relief
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Restless leg syndrome
Management
Iron Supplementation:
If serum ferritin levels are below 75 mcg/L, iron supplementation is recommended.
Pharmacological Treatment - 1st Line: Dopamine agonists OR alpha-2-delta ligands
Dopamine agonists (e.g. ropinirole, pramipexole, rotigotine patches)
Complications: Impulse control disorders – unable to resist sudden urges – can result in pathological gambling, alcohol/drug misuse, binge eating sexual misconduct.
Should therefore be avoided in patients at risk of the above.
Alpha-2-delta ligands (e.g. gabapentin, pregabalin)
Preferred for people with severe sleep disturbance, pain, anxiety, or a history of an ICD.
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Localising features
Certain features during seizures are suggestive of involvement of certain areas within the brain:
Frontal lobe = Motor features eg Jacksonian march/posturing/movements
Parietal lobe = Sensory features - paraesthesia
Occipital = Visual - floaters/flashes
Temporal = Hallucinations, emotions, deja vu.
Focal seizures
Summary
Seizure activity which starts in a specific part of brain, consciousness remains intact (+/- awareness)
Can become generalised - focal to bilateral seizure
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Generalised Seizures
Both sides of brain affected, consciousness lost
Features
Motor: Tonic-clonic, tonic, clonic
Non-motor: Absence (petit mal)
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Mng of epilepsy
Management of Epilepsy
Commencing AEDs - NICE Guidance
AEDs (anti-epileptic drugs) are generally commenced after a 2nd seizure, or after the 1st with the following indications:
EEG = consistent with epilepsy
Abnormal MRI/ brain imaging
Focal neurology
Unacceptable risk to pt./family
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Generalised tonic-clonic seizures
1st Line: Sodium Valproate
In males and women who are unable to have children
Except - AVOID in women of child bearing age - teratogenic
2nd Line (1st line for women of child bearing age) - Lamotrigine or Levetiracetam
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Absence seizures
1st Line: Ethosuximide
2nd line: sodium valproate (for boys/ men/ women not of child-bearing potential)
Avoid carbamazepine - can worsen absence seizures
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Myoclonic seizures
Clinical Features
Often present with brief twitching/ jerking - can lead to ‘throwing cup of tea’ or falls
Classically occur soon after waking up and can be exacerbated by alcohol, lack of sleep, stress
Management
1st Line: Sodium valproate
2nd Line (or 1st line in W of CB potential) - Levetiracetam
Avoid carbamezapine, phenytoin, gabapentin - worsen seizures
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Focal seizures (with or without evolution to bilateral tonic-clonic seizures)
1st Line: Lamotrigine or levetiracetam
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Anti-epileptic Drugs
Phenytoin
Side effects:
Acute: cerebellar-like symptoms - nystagmus/dizziness/Ataxia/slurred speech
Others: A vast number… some important ones include:
Peripheral neuropathy
Megaloblastic anaemia
Gingival hyperplasia
Hepatitis
Phenytoin is Teratogenic - cleft palate, congenital heart disease, clotting disorders in newborn
P450 INDUCER (remember – PC BRAS)
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Sodium Valproate
Side effect profile – Mnemonic: VALPROATE
Vomiting
Alopecia
Liver – Hepatotoxicity
Pancreatitis/Pancytopenia
Return Fat (weight gain) + appetite
Oedema
Ataxia
Tremor
Electrolyte disturbance ( hyponatraemia)
Sodium valproate is also teratogenic: Neurodevelopmental delay
P450 INHIBITOR (remember – OAK DEVICES)
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Carbamazepine
Side effect profile
Blurred vision – Diplopia
Agranulocytosis
Autoinduction - the commencement of carbamezapine can exacerbate seizures for the first 3-4 weeks of use
SIADH - hyponatraemia
P450 INDUCER (remember pC bras)
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Status Epilepticus
Treatment for SE is recommended after a seizure does not self terminate after 5 minutes, or if there > 1 seizure with incomplete resolution between seizures.
Management
0-5 minutes A-E approach: 02, Check BM
5-15 minutes - 1st step: BZDs - e.g. Lorazepam 4mg
Wait 5 minutes - Then repeat BZD (lorazepam 4mg) if not resolved
2nd step: Then give intravenous Levetiracetam, phenytoin or sodium valproate
In the case of refractory status consider general anaesthesia
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Epilepsy In pregnancy
Epilepsy in Pregnancy
Prescribe high dose folic acid - 5mg daily
Lamotrigine and levetiracetam – safer in pregnancy
Non -epileptic attack disorder
Often suggested by seizures consisting of thrashing like movements or complete motionless
1st Line investigations: video telemetry
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Intracranial Venous Sinus Thrombosis
General symptoms
Sudden onset headache
N&V
Papilloedema
Stroke
More specific clinical features of IVST depend on the site of the thrombosis:
Isolated sagittal sinus thrombosis
Seizures
Hemiplegia
EMPTY DELTA sign
Cavernous Sinus thrombosis
Periorbital oedema, erythema
Ophthalmoplegia - CN6 affected first, then CN3, 4 +/- CNS (face/eye pain +/- retinal vein thrombosis
Lateral/Transverse Sinus thrombosis
CN6 + CN7 Palsies
CT head: Empty delta sign
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Subclavian Steel Syndrome
Pathology: Proximal Subclavian artery stenosis results in retrograde flow into vertebral artery.
Features: Exercise with the arm above head results in neurological symptoms (e.g. paraesthesia) - a typical cause might be painting for example.
Diagnosis: MRA (duplex US)
Management: Endarterectomy/Stent
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CADASIL
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy
Inheritance: Autosomal dominant
Genetics: Mutation in NOTCH3 gene (Chr19)
Features:
Family history
History of migraine with aura
History of multiple infarcts (stroke/TIA/vascular dementia) - often at a young age
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Transient Ischaemic Attack (TIA)
A transient episode of neurological dysfunction without infarction, characterised by resolution of neurological deficit within 24hrs.
Management
1. Antiplatelets
Load with aspirin 300mg OD (unless already on regular aspirin)
CT head should be performed if the patient has an underlying bleeding disorder or is on an anticoagulant, to rule out haemorrhage.
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2. Assessment and follow up
If crescendo TIA (>1 TIA) OR cardioembolic source is suspected (e.g. infective endocarditis) OR severe carotid stenosis
Discuss case with stroke team - consider admission
If single TIA which occurred within the last 7 days
Urgent assessment should be performed in less than 24hrs by stroke specialist
If single TIA which was more than 7 days ago
Assessment by stroke specialist should be within 7 days
Do NOT use scoring systems such as ABCD2
3. Secondary Prevention
Antiplatelets:
1st Line: Clopidogrel
2nd line: ASA + modified release dipyridamole (DP)
3rd line: DP
Carotid imaging (as above per stroke) for consideration of endarterectomy
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Management of stroke
Thrombolysis - Alteplase
The use of 'clot busting' drugs to break down the thrombosis and restore cerebral perfusion.
The primary drugs used are tissue plasminogen activators such as alteplase which convert plasminogen into the enzyme plasmin. Plasmin lyses fibrinogen and fibrin.
Indications for thrombolysis - Alteplase is recommended if:
Patient presents within 4.5 hours onset of symptoms
AND intracranial bleed has been ruled out
Absolute contraindications for thrombolysis:
Ever had an intracranial haemorrhage
SAH
Seizure at the onset of stroke
Intracranial neoplasm
History of stroke/traumatic brain injury within last 3 months
Lumbar puncture within last 7 days
Active bleeding
History of a GI bleed in the last 3/52 or known oesophageal varices
Pregnant
BP > 200/120
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Stroke thrombectomy
Thrombectomy
To qualify for thrombectomy, patients must meet the following criteria
Rankin scale < 3 AND
NIHSS > 5
Indications:
Offer thrombectomy within 6 hours (PLUS thrombolysis if < 4.5 hrs) for patients with
Confirmed proximal anterior circulation occlusion (as demonstrated by CTA/MRA)
Offer thrombectomy within 6-24 hours for patients with:
Confirmed proximal anterior circulation occlusion
AND potential to salvage brain tissue (eg limited infarct core volume) as shown by CT or DW-MRI
Consider thrombectomy within 24 hrs (and thrombolysis if < 4.5hrs) for patients with:
Proximal posterior circulation occlusion (basilar/posterior Cerebral) with salvageable Potential
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Antiplatelet treatment
Antiplatelet treatment
In acute stroke patients should be commenced on Aspirin 300 mg OD for 2 weeks (plus PPI), after which they should commence secondary prevention:
1st Line: Clopidogrel 75mg OD (or NOAC if AF)
2nd Line: Aspirin + MR dipyridamole 200mg BD
3rd. Modified release Dipyridamole alone
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Carotid imaging
Carotid Imaging
Patients should undergo carotid imaging to assess suitability of endarterectomy.
Recommended in stroke or TIA patients who are NOT severely disabled AND stenosis:
50% (North American SCET)
Or >70% (European CST)
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Stroke & TIA
Stroke
An acute vascular event in which there is inadequate blood flow to the brain, resulting in cell death. This is most commonly caused by vascular occlusion/infarct or haemorrhage.
Stroke by anatomy
Certain signs/symptoms are characteristic of infarcts of specific territories.
Anterior Cerebral Artery
Supply the anteromedial region of the cerebrum
Features: Motor/sensory symptoms more pronounced in lower limbs than upper limbs
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Middle Cerebral Artery
Supply the lateral cerebrum
Features:
Motor / sensory - UL + Face affected more than lower limbs
Aphasia
Contralateral homonymous hemianopia
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Posterior Cerebral Artery
Contralateral homonymous hemianopia
Visual agnosia
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Oxford stroke classification (Bamford)
The Bamford classification categorises stroke according to the following criteria:
Unilateral weakness/sensory loss affecting the face/arm/leg
Homonymous hemianopia
Higher cortical dysfunction - e.g. dysphasia
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Total anterior circulation stroke = TACS
3/3 of the above features
Vascular involvement: Middle and anterior cerebral arteries
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Partial anterior circulation stroke = PACS
2/3 features
Vascular involvement:
Partial compromise to the anterior circulatory blood supply
Divisions of the middle/anterior cerebral arteries (e.g. the upper middle cerebral artery)
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Posterior circulation stroke = POCS
Features:
Loss of consciousness
Lateral medullary or lateral pontine syndrome
Isolated homonymous hemianopia
Vascular involvement:
A compromise of the posterior circulation which supplies the brainstem and cerebellum
Vertebrobasilar arteries
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Lacunar stroke (LACS)
A subcortical stroke, occurring due to small vessel disease
Major risk factor: Hypertension
Features
Characterised by the presence of 1 of:
Purse motor or pure sensory symptoms
Ataxic hemiparesis
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Stroke syndromes
Wallenberg's/Lateral Medullary Syndrome
Cause: Posterior Inferior Cerebellar Artery lesion
Features:
Ipsilateral facial loss of Temperature / Pain sensation
Loss of Temperature /Pain in contralateral torso and limbs
Ataxia, Nystagmus, vertigo
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Lateral Pontine Syndrome
Cause: Anterior inferior cerebellar Artery
Ipsilateral Features:
Ipsilateral paralysis upper and lower face
Ipsilateral loss of lacrimation/sensation, corneal reflex (effects) and 2/3rd taste)
Facial hemianesthesia - loss of pain / temperature
Cochlear nuclei lesion: ipsilateral deafness
Horner’s syndrome – descending SNS affected
Contralateral:
Loss of of Temperature /Pain in torso and limbs
Vestibular nuclei involvement - nausea, vomiting, nystagmus, vertigo
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Weber's Syndrome
Cause: Posterior Cerebral A. branches supplying midbrain
Features:
Ipsilateral CN3 Palsy (down and out, ptosis)
Contralateral hemiparesis of upper and lower limbs
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Stroke syndrome investigation
Investigations
Diagnosis - 1st Line imaging - Non-contrast CT head
Gold standard: MRI brain
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Tension-type Headache
Key learning
Most common primary headache disorder
Band-like pattern, tight, bilateral, non-pulsating, non-disabling
No associated neurological deficit or vomiting
Management is reassurance, reducing triggers and simple analgesia
Pathophysiology
Unknown mechanism likely secondary to peripheral and central pain mechanisms and increased sensitive to pain
Provoking factors
Stress
Alcohol
Dehydration
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Tension headache mng
Clinical features
Subcategories:
Infrequent- < 1 day a month and self-limiting
Frequent- > 10 episodes on fewer than 15 days per month on average for more than 3 months
Chronic- 15 or more days of headache per month for more than 3 months in absence of medication overuse
Typical symptoms:
Band-like pattern, bilateral, non-pulsating
Last 30 minutes to 7 days
Develop and resolve gradually
Can have one of photophobia or phonophobia
No nausea or vomiting
Not worsened by routine physical activity (walking or climbing stairs)
Examination findings
Normal neurological exam
Management
Reassurance
Recognise and reduce triggers
Simple analgesia- paracetamol or ibuprofen
Avoid opioids (increased risk of medication overuse headache)
Chronic subtype:
Consider trial amitriptyline
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Brachial neuritis
Upper Limb Neuropathies
Brachial neuritis
Pathophysiology
Inflammation of the brachial plexus with resultant pain and weakness.
The cause is often unknown but can be associated with viral infections, trauma, or surgery.
Clinical features
Sudden, severe shoulder, scapular and upper arm pain
Pain is typically followed by weakness and atrophy of the affected muscles.
Sensory disturbances/paraesthesia may be present
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Erb’s palsy
Pathophysiology
Injury to the upper brachial plexus (C5-C6 roots)
Causes include trauma, excessive lateral traction on the head during childbirth.
Clinical features
Characteristic "waiter's tip" position: the arm hangs by the side and is internally rotated, with the forearm extended and pronated.
There is weakness or paralysis of the deltoid, biceps, brachialis, and brachioradialis muscles. Sensory loss may occur along the lateral aspect of the arm.
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Klumpke Palsy
Pathophysiology
Injury to the lower brachial plexus (C8 and T1 nerve roots).
Often caused by excessive abduction of the arm, trauma, or obstetric complications.
Clinical Features
Weakness and atrophy of intrinsic hand muscles.
Claw hand deformity: hyperextension of the metacarpophalangeal joints and flexion of the interphalangeal joints.
Sensory loss in the medial aspect of the forearm and hand.
Horner's syndrome may occur if the T1 sympathetic fibres are affected.
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Cervical myelopathy
Pathophysiology
Cervical myelopathy is a condition resulting from compression of the spinal cord in the cervical spine.
Causes include cervical spondylosis and disc herniation. The compression leads to ischemia and neuronal damage within the spinal cord.
Spondylotic myelopathy: OA of Cervical vertebral bodies causes spinal canal narrowing with resultant cord compression
Clinical features
Unilateral or bilateral neck, shoulder, and/or arm pain following a dermatome; often severe and nocturnal.
Neck pain
Neurological signs/symptoms of compression (weakness, sensory changes, ataxia).
Sensory symptoms: shooting pains, numbness, hyperaesthesia (more common than motor).
Motor symptoms: muscle weakness and spasm; gradual or abrupt onset.
Most commonly affects C7 and C6 nerve roots.
Hoffman sign
Spurling's test positive: Axial compression with neck in extension, lateral flexion reproduces symptoms radiculopathy.
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Cervical myelopathy
Pathophysiology
Cervical myelopathy is a condition resulting from compression of the spinal cord in the cervical spine.
Causes include cervical spondylosis and disc herniation. The compression leads to ischemia and neuronal damage within the spinal cord.
Spondylotic myelopathy: OA of Cervical vertebral bodies causes spinal canal narrowing with resultant cord compression.
Investigations
MRI C-spine is the investigation of choice.
Management
Conservative Management (for symptoms <4-6 weeks and NO objective neurological signs)
Reassurance: Prognosis is good. Most cases improve without surgery.
Analgesia and physiotherapy.
Advanced Management (for symptoms ≥4-6 weeks or with objective neurological signs)
Diagnostic Confirmation: Refer for MRI to confirm diagnosis.
Invasive Mx/ Surgical decompression is often required in moderate to severe cases to prevent further neurological deterioration.
Consider interlaminar cervical epidural injections, transforaminal injections
Surgical options include anterior cervical discectomy and fusion (ACDF) or posterior decompression with laminectomy.
Indications - unremitting radicular pain despite 6–12 weeks of conservative treatment, disabling symptoms, progressive motor weakness, and MRI evidence of nerve root compression.
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Visual field defects
Visual Field Defects
Area of Visual Field Loss
Common Patterns
Site of Lesion
Aetiology
——Central Scotoma
Round or oval shape at fixation point
Macula
Macular degeneration, diabetic retinopathy, optic neuritis
——-Superior Quadrantanopia
Loss of upper quarter of visual field
Temporal lobe (inferior aspect of optic radiation)
Stroke, tumour, trauma
———Inferior Quadrantanopia
Loss of lower quarter of visual field
Parietal lobe (superior aspect of optic radiation)
Stroke, tumour, trauma
———Bitemporal Hemianopia
Loss of temporal visual fields in both eyes
Optic chiasm
Pituitary tumours (more upper quadrant loss) craniopharyngioma (more lower quadrant loss)
——Homonymous Hemianopia
Loss of the same side of the visual field in both eyes
Optic tract, optic radiation, occipital lobe (if macular sparing)
Stroke, brain tumors, trauma, multiple sclerosis
——Peripheral Field Loss
General constriction or reduction in peripheral vision
Retina, optic nerve
Glaucoma, retinitis pigmentosa, optic nerve damage
—-Monocular Blindness
Complete loss of vision in one eye
Eyeball,
optic nerve
Retinal detachment, retinal artery/vein occlusion, B12 deficiency
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