Neurology Flashcards
Stroke (ischaemic, haemorrhage) definition
Ischaemic stroke = vascular occlusion or stenosis cuts off blood supply to the brain
- most common type of stroke (87%)
Haemorrhagic stroke = vascular rupture, resulting in intraparenchymal and/or subarachnoid haemorrhage - bleeding in brain causes stroke
- 13% of strokes
Stroke aetiology
Risk factors both
- age >55
- family history
- HTN, T2DM
- smoking
Ischaemic
- Afib
- history of: TIA, stroke
- sickle cell disease
Haemorrhagic
- male
- asian/black
- alcohol
- anticoagulant use
Stroke pathophysiology
Other types = subarachnoid haemorrhage and cavernous sinus thrombosis
Stroke clinical manifestations
Ischaemic - key
- vision loss
- unilateral muscle weakness (often face, arm, leg)
- impaired language function (aphasia)
- impaired coordination (ataxia)
Ischaemic - other
- sensory loss
- headache
- diplopia
- dysarthria
- gaze paresis
- arrhythmia
Haemorhhagic
- visual disturbance (homonymous hemianopia - visual field loss on the left or right side of the vertical midline on the same side of both eyes may be due to haemorrhage in visual pathways, diplopia may result from brain stem haemorrhage)
- photophobia
- unilateral muscle weakness/paralysis (often face, arm, leg)
- dysarthria/dysphasia
- ataxia
- sensory loss
Stroke uncommon clinical manifestations
Both
- vertigo
- nausea/vomiting
- coma
Ichaemic
- neck/facial pain
Haemorrhagic
- gaze paresis
Stroke 1st line investigations
Both
- non-contrast CT head
- serum glucose - hyperglycemia associated with worse prognosis, hypo
= stroke mimc
- serum electrolytes - exclude electrolyte disturbance
- serum urea and creatinine - exclude renal failure
- FBC - exclude thrombocytopenia
- ECG - may indicate MI
Ischamic
- cardiac enzymes - exclude MI
- PT and PTT - exclude coagulopathy
Haemorrhagic
- liver func
- clotting screen - exclude coagulopathy
Stroke general investigations
Both: serum toxicity screen - exclude alcohol/drug causes
Ischaemic
- CT/MRI - show areas at risk of subsequent infarction
- US - shows carotid stenosis
Stroke management
INITIAL - suspected ischaemic/haemorrhagic stroke
- first line stabilize and refer to hyperacute/acute stroke unit
- consider endotracheal intubation
- give supplementation oxygen only if oxygen saturation drops below 93%
ACUTE - confirmed ischaemic stroke
- 1st line - monitor: consciousness (Glasgow coma scale), blood glucose, BP, oxygen saturation, hydration, temperature (antipyretic high temp), cardiac rhythm and rate, intracranial pressure
- IV alteplase
- mechanical thrombectomy and antiplatelet drug (aspirin)
- venous thromboembolism prophylaxis and high intensity statin (atorvastatin)
ACUTE - confirmed haemorrhagic stroke
- 1st line - monitor (as above) and immediate referral to neurosurgery assessment
- rapid BP control
- reversal of anticoagulation
- warfarin/vit K antagonist reversal (prothrombin complex concentrate, phytomenadione)
- dibigatran reversal (idarucizumab)
- factor Xa reversal (prothrombin complex concentrate)
- venous thromboembolism prophylaxis
Transient ischaemic attack (TIA) definition
Focal, sudden onset, neurological deficit lasing <24 hours, with complete clinical recovery
15% of first strokes are preceded by TIA
Transient ischaemic attack epidemiology
M > F
Black ethnicity is at greater risk due to their hypertension and atherosclerosis predisposition
20, 000 people have a TIA
Risk factors
- age
- hypertension
- smoking
- diabetes
- heart disease - valvular, ischaemic or AF
- past TIA
- peripheral arterial disease
Transient ischaemic attack aetiology
Inadequate cerebral or ocular blood supply due to
- reduced blood flow
- ischaemia
- embolism associated with disease of: blood vessels, heart or blood
Reduced blood flow can be caused by
- atherothromboembolism from the carotid artery
- small vessel occlusion
- cardioembolism resulting in microemboli from
- mural thrombus post-MI or in AF
- valve disease
- prosthetic valve
- hyperviscocity eg polycythaemia, sickles cell anaemia, extremely raised white cell count, myeloma
Transient ischaemic attack pathophysiology
Common cause is from cerebral ischaemia - lack of O2 and nutrients to the brain - cerebral dysfunction
- ischaemia is short lived, with symptoms only lasting a maximum if 5-15 mins after onset and then resolves before irreversible cell death occurs
Gradual progression of symptoms suggests a different pathology eg demyelination, tumour or migraine
90% of TIA’s affect the anterior circulation (carotid artery)
10% affect posterior circulation (vertebrobasilar artery)
Transient ischaemic attack clinical manifestations
Sudden loss of function with complete recovery
Stroke symptoms (focal neurological deficit)
- slurred speech
- facial droop
Transient ischaemic attack other diagnostic features
Associated with both anterior and posterior circulation TIAs
- unilateral weakness/paralysis
Associated with anterior circulation TIAs
- dysphasia
- amaurosis fugax
Aossictaed with anterior circulation TIAs
- ataxia, vertigo, loss of balance
- homonymous hemianopia
- diplopia
Transient ischaemic attack investigations
First line:
Blood glucose - exclude hypoglycaemia
FBC/platelet count = normal
PT/PTT/INR - exclude coagulopathy
Fasting lipid profile - normal or hyperlipidemia
Serum electrolytes - exclude electrolyte disorders
ECG - evaluate atrial fibrillation and other arrhythmias (rule out MI)
CT angiography - look for stenosis
Transient ischaemic attack differential diagnosis
Impossible to differentiate from a stroke until there is a full recovery
Hypoglycaemia
Labyrinthine disorders - labyrinthitis/vestibular neuronitis, BPPV, Meniere’s disease
Migrainous aura
- typical - visual symptoms, sensory symptoms, dysphagia
- atypical - motor weakness “hemiplegic migraine”
Mass lesions
- sundural hematoma
- cerebral abscess
- tumours
Postictal weakness (also known as Todd’s paralysis)
Transient ischaemic attack management
INITIAL - for suspected transient ischaemic attack 1st line - antiplatelet therapy Primary: aspirin Secondary: clopidogrel Referral
ACUTE - confirmed TIA 1st line - Antiplatelet therapy Primary: clopidogrel Secondary: aspirin
High intensity statin - atorvastain - simvastatin Anticoagulant - LMW heparin - direct thrombin inhibitor - factor Xa inhibitor
Subarachnoid, subdural, extradural haemorrhage definition
EDH - bleeding external to dura
SDH - bleeding beneath the dural membrane
SAH - bleeding within the subarachnoid space
Subarachnoid, subdural, extradural haemorrhage epidemiology
EDH - adolescents/young adults
- 10% severe head injuries -> EDH
SDH
- elderly
- blood thinning medication
- haemophilia
- epilepsy
- alcoholics (chronic alcohol consumption can gradually cause brain to shrink and make blood vessels more vulnerable to damage
SAH - uncommon
Subarachnoid, subdural, extradural haemorrhage risk factors
SAH
- smoking
- high blood pressure
- excessive alcohol consumption
- a family history of the condition
- some rarer conditions, such as autosomal dominant polycystic kidney disease (ADPKD)
Subarachnoid, subdural, extradural haemorrhage aetiology
EDH
- head strike (sport/motor vehicle accident)
- fractured temporal or parietal damaging the middle meningeal artery or vein
SDH
- usually trauma causes tearing of subdural cortical bridging veins = blood between dura matter
SAH
- after trauma where cortical surface vessels are damaged
- non-traumatic follows the rupture of a cerebral (Terry) aneurysm = blood in circle of willis cisterns and fissures
- less common - brain tumour; encephalitis; vasculitis
Subarachnoid, subdural, extradural haemorrhage pathophysiology
SDH
Bleeding into the subdural space caused by damage to cranial vessels/brain.
As blood starts to build up, it can place pressure on the brain (intracranial hypertension) -> brain damage
SAH
A brain aneurysm is a bulge in a blood vessel, caused by weakness in the blood vessel wall, usually at a point where the vessel branches off. As blood passes through the weakened vessel, the pressure causes a small area to bulge outwards like a balloon and rupture
Subarachnoid, subdural, extradural haemorrhage clinical manifestations
EDH
- initial loss of consciousness -> lucid interval -> second loss of consciousness
- hemiparesis
- nausea/vomiting
- unequa pupils
- bradycardia
SDH
- level of consciousness gradually decreases
- gradual increase of headache and confusion/slurred speech
- confusion
- double vision
- seizures
SAH
- extremely painful headache
- thunderclap headache, maximum severity within seconds, worse ever, potential focal symptoms/coma as a result of aneurysms
- sight problems
Subarachnoid, subdural, extradural haemorrhage management
EDH
- expident evacuation via a craniotomy
- ABC/2 (haemorrhage volume measurement method)
- give oxygen
- a full trauma assessment must be made
- intravenous (IV) fluids may be required to maintains the circulation and preserve cerebral perfusion
- if IC pressure is raised -> osmotic diuretics (IV mannitol)
- Burr holes may be required to evacuate a hematoma
SDH
- conservative (monitor with serial CT)
- surgical - craniotomy (main treatment after severe head injury); burr holes (main treatment for hematomas)
SAH - nimodipine - analgesioa anticonvulsant (phenytoin) - antiemetic (promethazine) - surgery - neurosurgical clippings, endovascular coiling
Subarachnoid, subdural, extradural haemorrhage complications
SAH
- rebleeding
- delayed cerebral ischaemia
- coma
- hydrocephalus
- epilepsy
- cognitive dysfunction
Epilepsy definition
A recurrent tendency to spontaneous episodes of abnormal electrical activity within the brain which manifest as seizures
Epilepsy aetiology
Electrical signals become scrambled and there are sometimes sudden bursts of electrical activity causing seizures.
Very often idiopathic with no clear cause found
Associated with
- underlying structural lesions (trauma, neoplasms, malformations, stroke)
- metabolic conditions (alcohol, electrolyte disorders)
- infections
- rare genetic diseases (eg ion channel mutations)
Epilepsy pathophysiology
Classification:
Partial seizures:
- features attributable to a localised part of one hemisphere
- simple partial seizures, consciousness is unimpaired
- complex partial seizures, consciousness is impaired
Generalised seizures:
- originating bilaterally distributed networks -> simultaneous onset of widespread electrical discharge. No localising features referable to a single hemisphere
- motor seizures are bilateral
- more commonly seen in children
- consciousness is always impaired
- absence seizures = brief (<10s) pauses eg stopping talking in mid-sentence and then carrying on where left off
- tonic-clonic seizures = sudden loss of consciousness with stiffening (tonic) of limes and then jerking (clonic)
- myoclonic jerks = sudden violent movement of the limbs
- atonic (akinetic) seizures: sudden loss of muscle tone causing a fall, no LOC
- tonic: sudden stiffening of the body, no jerking
- infantile spasms: commonly associated with TB
Focal seizures: originating in networks linked to 1 hemisphere and often seen with underlying structural disease
- subclasses include:
- without conscious impairment: no post-ictal syndrome
- with impairment of consciousness: most commonly arise from temporal lobe; post-ictal confusion
- evolving to bilateral, convulsive seizure
Epilepsy clinical manifestations
ICTAL: seizures can present in a number of ways
PREICTAL: preceding prodrome (an early sign or symptom) lasting hours or days in which there may be a change in mood or behaviour
- aura: maybe a strange feeling in the gut or flashing lights (implies a focal seizure, often from the temporal lobe
POSTICTAL
- headache
- confusion
- myalgia (pain in a muscle or group of muscles)
Focal lobe specific postictal symptoms include:
- temporal lobe: emotional disturbance, dysphagia, hallucinations, bizarre associations
- frontal lobe: motor features such as pedaling movements of the legs. Motor arrest, dysphagia or speech arrest; Postictal Todd’s Palsy: limb paralysis in a seizure for several hours
- parietal lobe: sensory disturbances - tingling, numbness, pain. Motor symptoms
- occipital lobe: visual phenomena such as spots, lines, flashes
Epilepsy differential diagnosis
- migraine
- TIA
- cardiogenic syncope
- parasomnia
Epilepsy management
Pharmacology: anti-epileptic drugs (main treatment)
Do not give sodium valproate to pregnant women. Women on childbearing age should be on contraception (not just condoms)
Psychological therapies:
- relaxation
- CBT
Surgical intervention
- neurosurgical resection
- vagus nerve stimulation is a palliative treatment option for refractory epilepsy
Lifestyle: ketogenic diet can help control seizures
Parkinsons definition
Chronic progressive neurological disorder characterised by motor symptoms of resting temor rigidity, bradykinesia and postural instability
Parkinsons key clinical manifestations
- presence of risk factors (age, family PD, mutations in GBA gene)
- bradykinesia (progressive slowness of movement)
- resting trmor
- rigidity
- postural instability
Parkinsons other clinical manifestations
- masked facies (loss of spontaneous face movement)
- hypophonia (reduced voice vol)
- hypokinetic dysarthria
- micrographic (decreased amplitude of handwriting)
- stooped posture
- shuffling gait
- fatigue
- constipation
- depression
- anxiety
- dementia
Parkinsons 1st line investigations
Dopaminergic agent trial
Parkinsons 2nd line investigations
- Brain MRI
- Functional neuroimaging (dopamine transporter imaging) - look for decreased basal ganglia presynaptic dopamine uptake
- olfactory testing looks for hyposmia or anosmia
- genetic testing
- neuropsychometirc testing
- serum ceruloplasmin excludes Wilson’s disease
- 24 urine copper excludes Wilson’s disease
- postmortem brain pathology
Parkinsons management
Mild Parkinson’s:
1st line - MAO-B inhibitor, dopaminergic agent, amantadine or trihexyphenidyl
Primary options:
- rasagline (MAOi)
- pramipexole (dopamine agonist)
- ropinirole (dopamine agonist)
- carbidopa/levodopa (converts to dopamine)
- rotigotine transdermal (dopamine agonist)
Secondary:
- selegiline (MAOi)
- trihexyphenidyl (anticholinergic)
- amantadine (increase amount of dopamine)
- also physical activity
- additional carbidopa or domperidone (dopamine antagonist) for nausea
Moderate Parkinson’s:
First line - MAO-B inhibitor, dopaminergic agen, amantadine or trihexyphenidyl
- pharmacotherapy/deep brain stimulation
- propanolol
- primidone
- reduce dopaminergic medications
Advanced Parkinson’s:
First line - MAO-B inhibitor, dopaminergic agent, amantadine or trihexyphenidyl
- deep brain stimulation and intrajejunal infusion of levodopa
General
- physical therapy
- occupational therapy
- speech and language therapy
- diet advice
Headaches - migraine, tension cluster, drug overdose epidemiology
Early-mid life
Headaches risk factors
Family history
- high caffeine intake
- female sex/obesity
- stressful life events/sleep disorders
Mental tension
- stress
- missing meals
- fatigue
- female sex
Male sex
- family history
- head injury
- smoking
- heaving drinking
Headaches pathophysiology
Classifications
- Primary (have no diagnostic test, diagnose based on symptoms)
- migraine - w/wihtout aura
- tension - mild/moderate ‘squeezing’, not aggregated by physical activity
- cluster - Secondary (consider if older, immunosuppressed, previous cancers)
- meningitis
- subarachnoid haemorrhage
- giant cell arthritis
- idiopathic intracranial hypertension - can affect vision
- medication overused headache - Other
- trigeminal neuralgia (facial pain)
Headaches clinical manifestations (migraine)
Episodic/20% with aura or chronic
Moderate to sever pain, lasting 4-72 hours
Aggravated by routine physical injury
Unilateral, nausea/vomiting, photophobia/phonophobia
Headaches clinical manifestations (tension)
Bilateral, pressing, tightening, mild.moderate intensity,
Stress = common trigger, not aggravated by physical activity
No nausea/vomiting
Potential photophobia/phonophobia
Headaches clinical manifestations (cluster)
Unilateral, orbital, supraorbital and temporal
Extremely painful
Attacks same time for several weeks
Accompanied with ipsilateral cranial autonomic features
Sense of relentlessness or agitation
Generally shorter episodes than migraine
Headaches clinical manifestations (medicine overuse)
Present on >15 days a month
Common medicines
- ergotamine, triptans, opioids, analgesics.
Headahce develops/markedly worsens during drug use.
Can lead to chronic daily headache
- (if headache happens over 50% of the time)
Headaches clinical manifestations (trigeminal neuralgia)
Short paroxysmal attacks, electric shock like, precipitated by innocuous stimuli to affected side of face
Headaches clinical manifestations (secondary)
Postural headaches are suggestive of secondary
Neck stiffness - possibly meningitis
Sudden onset thunderclap (suggests vascular)
Headaches clinical manifestations (red flags - brain tumour)
New headache with Hx of cancer Cluster headache Seizure Significantly altered consciousness, memory, confusion, coordination Papilloedema
Headaches investigations
Primary - no diagnostic tests - classify based on symptoms
May do other tests to rule out other causes (not diagnostic), all these tests should be normal if patient has a migraine/tension/cluster
- ESR - rule out temporal arteritis
- Lumbar puncture - rule out: SAH, meningitis
- CFR culture - rule out systemic/NS infection
- MRI brain - rule out ischaemic lesions
- CT of head - rule out lesions, SAH
- pituitary func test - rule out pituitary adenoma
- ECG - rule out conduction abnormalities
Headaches differential diagnosis
Tension/cluster headaches, MOH, SAH, giant cell arteritis, CVT
Chronic migraine, sphenoid sinusitis
Migraine, paroxysmal hemicrania, SUNCT
Headaches management
MIGRAINE: lifestyle modification and trigger management
- lying in a dark room
Pharmacological treatments:
- offer topiramate or propanolol (for avoiding attacks, taken regularly)
- botulinum toxin A injections can work in chronic cases
- NSAIDS (aspirin, ibuprofen, naproxen)
- pain killers (paracetamol, ketorolac)
- triptan (migraine specific pain killer) (sumatriptan, almotriptan, eletriptan); often effective to combine with another painkiller)
- anti-sickness medication (metoclopramide, prochlorperazine, promethazine, compazine, thorazine, reglan)
- antihistamine (in acute M) (diphenhydramine, promethazine)
Hydration
High flow O2
Surgical treatment
- decompression (removes tissue putting pressure on peripheral sensory nerves)
- neurectomy (cuts nerve in head contributing to migraine symptoms)
- acupuncture
- TMS = brain stimulation
TENSION HEADACHES Non-pharmacological treatment - relaxation techniques: yoga/massage - exercise - EMG biofeedback - CBT - acupuncture - physiotherapy Pharmacological treatment: - Acute: simple analgesics (aspirin, paracetamol, ibuprofen, naproxen) - chronic: - antidepressants (amitriptyline, doxepin, venlafaxine); muscle relaxants (tizanidine)
CLUSTER HEADACHES Pharmacological - 1st line: sumatriptan, oxygen - zolmitriptan - anaesthetic (lidocaine) - CCB (verapamil) - AEDs (topiramate) Non-pharmalogical - greater occipital nerve block - surgery (deep brain stimulation) MOH Stop taking medication
Multiple sclerosis definition
Inflammatory autoimmune demyelinating central nervous system condition characterised by the presence of episodic neurological dysfunction in at least two areas of the central nervous system
Multiple sclerosis epidemiology
Caucasian
Women (F:M, 3:1)
20-40 years
Multiple sclerosis pathophysiology
Oligodendrocytes affected not Schwann cells
Classification
1. macrophage mediated demyelination
2. antibody mediated demyelination
3. distal oligodendrogliopathy and apoptosis
4. primary oligodendroglial degeneration
Type of MS progression
- relapsing/remitting (attacks are in discrete time sets, go back to normal after each attack or disability will progressively get worse between attacks)
- primary progressive (patient gets progressively worse, or at accelerated/decelerated rate)
- secondary progressive (following an initial attack the disease will get progressively worse)
Multiple sclerosis clinical manifestations
Typical:
- optic neuritis (painful progressive visual loss)
- spasticity and other pyramidal signs
- sensory symptoms and signs
- Ihermitte’s signs (electric shock down back of spine)
- Nystagmus (involuntary movements)
- double vision and vertigo (spinning / dizziness)
- bladder and sexual dysfunction
Atypical:
- aphasia (can’t comprehend language)
- hemianopia
- extrapyramidal movement disturbances
- severe muscle wasting
- muscle fasciculation (all grey matter issues or peripheral nerve issues)
Patients more commonly present with weakness, paraesthesia, visual loss, as opposed to incontinence.
Multiple sclerosis 1st line investigations
MRI-brain (sagittal FLAIR)
MRI-spine (demyelinating cervical spinal cord lesions are common)
FBC - excludes alternative diagnosis (should be normal)
Comprehensive metabolic panel - excludes alternatve dianosis (should be normal)
Thyroid stimulating hormone - excludes alternative diagnosis (should be normal)
Vit B12 - excludes alternative diagnosis (should be normal)
Anti-neuromyelitis optica antibody - present in neuromyelitis optica
Cerebrospinal fluid evaluation - elevated CSF IgGs in 80% of MS
Multiple sclerosis differential diagnosis
Autoimmune disorders
- systemic lupus erythamtous
- primary sjogrens syndrome
- Bhcet’s disease
- polyarteritis nodosa
- acute disseminated encephalomyelitis
Infectious disease - lyme - syphilis - AIDS Adrenomyeloneuropathy Mitochondrial encephalopathy Arnold-chirari malformation Olivopontocerebellar atrophy Cardiac emboli event
Multiple sclerosis management
Acute relapsing MS:
- immunimodulatory therapy for acute relapse (5 day course of corticosteroid prednisolone - symptomatic modifying; anti-inflammatory cytokine Betaferon - disease modifying)
- oral/IV methylprednisolone
- plasma exchange
Relapse-remitting MS:
- immunomodulatory (interferon B)
Symptomatic treatment:
- medications for fatigue (amantadine, modafinil, armodafinil)
- visual problems (steroids - gabapentin)
- muscle spasms (physiotherapy
- mobility problems (exercise program, mobility aids)
- neuropathic pain (gabapentin, crabamezapine)
- MSK pain (exercise techniques, painkillers, TENS machine)
- emotional problems (antidepressant, benzodiazepines, CBT)
- sexual problems (viagra)
- bladder problems (catheter)
- bowel problems (changing diet, laxative)
- temor (propanolol)
Secondary progressive:
- 1st line - siponimod or methylprednisolone
- cladribine
Progressive:
- ocrelizumab
Motor neurone disease definition
A cluster of neurodegenerative disease
Motor neurone disease epidemiology
Most cases are sporadic. More common in middle aged men
Motor neurone disease classification
Amylotrophic later sclerosis (80%):
- loss of neurons in the motor cortex and the anterior horn
- combined UMN and LMN signs
Progressive bulbar/pseudobulbar palsy (10-20%)
- only affects cranial nerves IX-XII
- worst prognosis
Progressive muscular atrophy (<10%)
- anterior horn cell lesion
- best prognosis
- LMN signs only and distal muscle groups affected first
Primary lateral sclerosis (rare)
- progressive tetraparesis
With all there is no sensory loss and it never affects eye movements
Motor neurone disease pathophysiology
Characterised by selective loss of neurons in motor cortex, cranial nerves nuclei and anterior horn cells
Motor neurone disease clinical manifestations
UMNL
- lesion above the anterior horn in the spinal cord or above the nuclei of the cranial nerves
- ‘everything goes up’
- increased spasticity +/- clonus (involuntary muscle contractions)
- increased reflexes
- extensor plantar response
- abscent fasciculations ( muscle twitch)
LMNL
- lesion in anterior horn, motor nerve fibre or neuromuscular junction
- ‘everything slows down’
- reduced tone
- deep tendon reflexes absent or reduced
- plantar response normal or absent
- fasciculations may be present
- muscle wasting usually present
Bulbar palsy - lower motor neuron lesion to CN IX, X, XI, XII
- dysphagia, chewing difficulties
- flaccid, fasciculation tongue
- quiet/nasal/hoarse speech
- normal/absent jaw jerk reflex
Pseudobulbar palsy - upper motor neuron lesion to CN IX, X, XI, XII
- dysphagia
- slow tongue movements
- small, stiff, spastic tongue
- slow, indistinct speech
- increased jaw jerk reflex
Emotional liability - difficulty stopping crying/laughing in inappropriate situations Cognitive changes Generalised paralysis (bilateral)
Motor neurone disease investigations
Clinical diagnosis
Electromyography (EGM) shows muscle denervation but not specific
Brain/cord MRI to exclude structural causes
LP to exclude inflammatory causes
Motor neurone disease management
Incurable so instead symptom management
Occupational therapy
Physio
Riluzole, can slow progression of condition
Medicine to relieve muscle stiffness and help saliva problems
Motor neurone disease prognosis
Poor, death within 3yrs in 50%
Infections - meningitis definition
Inflammation of lepto-meninges and underlying subarachnoid CSF
Infections - meningitis epidemiology
Mainly the young and elderly
3,200 cases of bacterial meningitis occur in the UK ps,
3,000 cases of viral meningitis
Infections - meningitis aetiology
Viral - around 80% of meningitis cases
- enterovirus (echo virus, coxsackie virus)
- herpes simplex virus
- Varicella-zoster virus
- EBV (epstien-barr)
- CMV (cytomegalovirus)
Bacterial
- Neisseria meningitidis (meningococcus) - gram-ve diplococci
- Streptococcus pneumpniae - gram+ve diplococci
- Listeria monocytogenes
- Hamophilius influenzae
- Escherichia coli and Group B Streptococcus - in neonates
Intrathecal drug
Microorganism reach meninges by (direct from ears, nasopharynx, cranial injury, bloodstream)
Risk factors
- CSF shunts
- immunocompromised (HIV, cancer)
- Bacterial endocarditis, T2DM, alcohol and cirrhosis, IV druguse, renal insufficiency, adrenal insufficiency, malignancy, hyperparathyroidism, thalassaemia, CF
- splenectomy and sickle cell disease
- age (<5yrs, >60yrs)
Infections - meningitis pathophysiology
Viral meningitis = more common and benign than bacterial meningitis
Meningococcal disease (illnesses caused by N.meningitidis)
- presents as bacterial meningitis (15%)
- septicaemia (25%)
- combination of 2 presentations (60%)
Infections - meningitis clinical manifestations
Early:
- fever, headache
- leg pain
- cold hands/feet
Later:
- stiff neck, back rigidity, bulging fontanelle
- photophobia
- altered mental state, unconsciousness, coma
- Kernig’s sign
- Brudzinski’s sign (forced flexion of the neck elicits a reflex flexion of the hips)
- paresis
- raised ICP - low GCS, seizure, focal deficits
- septic shock - (suggestive of meningococcal)
- vomiting
- papilledema
- progressive dorwsiness, lateralizing signs
Viral m. features may be milder and complications less frequent
Meningisn:
- neck stiffness
- photophobia
- non-blanching petechial rash/purpura (menigococcal only)
- Kernig’s sign (thigh is flexed at the hip and knee, subsequent extension in knee is painful
Infections - meningitis investigations
1st line: bloods - culture, FBC, CRP, glucose, lactate, VBG, nose and throat swabs
2nd line: lumbar puncture - contraindicated if raised ICP, shock, coagulation abnormalities
Infections - meningitis management
IV fluids - prevent dehydration
Oxygen if breathing difficulties
Steroids - can reduce swelling in the brain
Prevention by:
- meningococcal vaccine (same for meningococcal disease)
- Hib vaccine
- pneumococcal vaccine
Infections - meningitis complications
Bacterial:
Spetic shock, disseminated intravascular coagulation, coma with loss of protective airway reflexes, cerebral oedema and raised intracranial pressure, spetic arthritis, pericardial effusion and haemolytic anaemia (H, influenzae)
Subdural effusions: reported in 40% of children aged 1-18 months with bacterial meningitis. Risk factors include young age, rapid onset of illness, low peripheral white cell count and high CSF protein.
Syndrome of inappropriate antidiuretic hormone secretion (SIADH)
Seiures
Decreased hearing, or deafness; other cranial nerve dysfunction, multiple seizures, focal paralysis, subdural effusions, hydrocephalus, intellectual deficits, ataxia, blindness, Wtarehouse-Friderchsen syndrome and peripheral gangrene.
Venous sinus thrombosis, severe cerebral oedema, cerebral abscess
Viral: rarely occur
Infections - meningitis prognosis
Meningitis kills more UK children under the age of 5 than any other infectious disease
Encephalitis defintion
Inflammation of the brain
Encephalitis epidemiology
Age (<1, >65)
Immunodeficient
HSV (herpes simple virus)
Encephalitis aetiology
An infection spreads to the brain - usually viral
Herpes viruses:
- herpes simple virus (most common cause)
1. cold sores/fever
2. genital herpes - Epstein barr
- Varicella zoster (chickenpox/shingles)
Enterovirus:
- polio
- coxsackievirus (flu, eye inflammation, ab pain)
Childhood infections:
- measles, mumps, rubella
Animal spread:
- tick-born, Japanese encephalitis, rabies, West Nile virus)
USUALLY viral but can be bacterial, fungal and parasitic:
- bacteria: mycoplasma, meningococcal, pneumococcal listeria.
- fungi: histoplasma, cryptococcus, candida
- parasites: malaria, toxoplasma
Encephalitis risk factors
- viral infections
- organ transplant
- animal/insect bites
- location
- season
Encephalitis pathophysiology
Infection and non-infectious causes
Infectious (primary):
- agent directly infects the brain
- viral/bacterial/fungal.parasitic
Non-infectious (secondary)
- faulty immune system reaction to infection elsewhere in body - immune system accidentally also attacks health cells in the brain
- autoimmunity
Encephalitis clinical manifestations
- headache
- seizures
- cough
- GI infection
- focal neurological signs
- fever
- rash
- altered mental state
- meningismus
- parotitis
- lymphadenopathy
- optic neurtisi
- acute flaccid paralsis
- movement disorder
- bisphasic illness
- myocarditis/pericarditis
- jaundice
- arthirits (lyme disease)
- retinitis (Wets Nile virus)
- Parkinsonism (arbovirus)
Encephalitis 1st line investigations
- blood cultures (confirms Bacterial E. and most arboviral infections)
- FBC (elevated WCC)
- peripheral blood smear (detects P. falciparum/ehrlichia)
- serum electrolytes (hyponatraemia)
- liver function tests = elevated
- throat swab - detect viruses
- nasopharyngeal aspirate (detects resp viruses); go on to do PCR to confirm adenovirus/influenza
- sputum culture (detect mycoplasma, TB and fungal infections
- chest x-ray (detect non infectious/infectious causes)
Neuroimaging:
- MRI - will differ depending on aetiology
- CT scan
- CSF (lumbar picture) - analysis; culture; serology; PCR
- ECG
Encephalitis 2nd line investigations
- urine culture - detects polio, varicella zoster, mumps, measles
- stool enteroviral culture
- IgG/IgM antibodies in blood - may identify aetiology
- PCR
- HIV serology /RNA test
- CSF biomarkers
- whole body CT/PET - identify cancers
Encephalitis management
Treat underlying cuase:
- antiviral medication; acyclovir, ganciclovir, foscarnet
- anti-inflammatory drugs; corticosteroid - if E. is caused by a problem with the immune system
- immunoglobulin therapy
- plasmapheresis
- surgery to remove tumur
- antibiotics or antifungals
Encephalitis puts strain on the body and relieves symptoms
- fluids to prevent dehydration
- painkillers to reduce discomfort or high temp
- medicine to control seizure or fits
- medicine to help the person relax
- oxygen to support lungs
- medicine - prevent a build up of pressure
Acyclovir - for viral E.
Encephalitis complications
- seizures
- hydrocephalus
- neurological sequelae
Herpes zoster definition
Shingles also known as herpes zoster is caused by reactivation of varicella-zoster virus (VZV) that was acquired during a primary varicella infection
Herpes zoster aetiology
Reactivation of latent VZV from dorsal root or cranial nerve ganglia present since primary infection. Herpes zoster and varicella (chicken pox) are both caused by VZV but varicella usually follows he initial infection and causes a generalised rash whereas HZ occurs after activation of a previous infection and tends to be localised to a specific nerve distribution.
Herpes zoster pathophysiology
Immunocompromisation such as HIV, malignancy, chemo and chronic use of corticosteroids.
Infection spreads from a ganglion to the corresponding cutaneous neural tissue
Most common dermatomes are T1 to L2
Herpes zoster clinical manifestations
Characterised by dermatomal pain and papular rash. Pain typically precedes the rash by several days and can persist for months after the rash resolves. Rash usually presents in a single dermatome and typically resolves within 4-5 weeks
Key:
- localised burning, stinging, itching or tingling in a dermatome
- pruritus may resent in affected dermatome
- erythematous maculopapular rash
- corneal ulceration (if trigeminal nerve is affected)
Herpes zoster investigations
Usuaully clinical diagnosis
May need PCR
Herpes zoster management
Antiviral therapy: famciclovir if immunicompetent aciclovir if immunocompromised
Herpes zoster complications
HZ ophthalmicus
Superinfection of skin lesions
Postherpetic pain is common
Dementia - AD, vascular, lewy body, frontotemporal definition
Group of chronic, progressive neurogenerative disorders
Dementia aetiology
Alzheimer’s (50%): atrophy, neurofibrillary tangles, amyloid plaques, neuronal loss
Vascular (25%): brain damage due to cerebrovascular disease
Lewy Body (15%): deposition of abnormal protein in brain stem and neocortex
Frontotemporal (5%): atrophy of frontal and temporal lobes. Most common dementia in user 65s
Dementia risk factors
AD: old age, family history, Down’s
Vascular: old age, obesity, HTN, smoking
Frontotemporal: genetic mutations
Dementia pathophysiology
AD: extracellular deposition of beta-amyloid plaques, tau intracellular neurofibrillary tangles, damaged synapses and atrophy are the pathological hallmarks
Lewy body: deposition of levy body inclusions, neurofibrillary tangles and a deficit of ACh and dopamine (causing Parkinsonism)
Dementia clinical manifestations
Alzheimer’s:
- progressive short term memory loss
- disorintation
- nominal dysphasia
- getting lost
- decline in ADLs (activities of daily activity)
Vascular: stepwise progression (stable symptoms followed by sudden increase in severity), stroke history, poor attention, abnormal reflex responses (jaw-jerk,glabella tap)
Lewy body: fluctuating conginition, recurrent visual hallucinations, REM sleep behaviour disorder, parkinsonism
Frontotemporal: park incidence = 50-60s behavioural/personality change, loss of language fluency/comprehension, abandonment of work and activities
Dementia management
Alzheimer’s: supportive
Vascular: treat atherosclerosis, lifestyle modifications, antiplatelets, statins, antihypertensive
Lewy Body:
- behavioural disturbances: benzodiazepines
- psychotic symptoms: cholinesterase inhibitors
- REM sleep problems: SSRI
- motor symptoms: clonazepam, melatonin, carbidopa
Frontotemporal
- no cure
- SSRIs for behavioural symptoms
- Levodopa/carbidopa if parkinson’s symptoms present
Primary and secondary tumours classification
Classification by cell of origin
Glial cells: astrocytoma, oligodendrocytoma, ependymoma, glioblastoma
Primitive neuroectodermal cells: medulloblastoma, neuroblastoma
Arachnoidal cell: meningioma
Nerve sheath cell: schwannoma, neurofibroma
Lymphoreticular cells: lymphoma (primary CNS lymphoma)
Histological classification
- Neuroepithelial (neuroepithelial cells are stem cells that differentiate into neurons and glial cells)
- astorcytic - glioblastoma, pilocytic astrocytoma
- oligodendroglioma - Meningeal - meningioma (mostly benign)
- Metastatic - commonly in lung, breast, colorectal, testicula, renal cell, malignant melanoma
- Others - pituitary oedema, CNS lymphoma, germ cell tumours (rare paediatric, usually malignant), sella region (eg craniopharyngiomas, cystic tumours)
- Nerve sheath - schwannoma (can affect cranial nerves eg CN VIII - acoustic neuroma), neurofibroma
Primary and secondary tumours epidemiology
Gliomas - most common primary brain tumour (glioblastoma are the most common adult brain tumour, astrocytomas are the most common in children), high grade gliomas (grade III and IV) make up 85% of all new cases of all new cases of malignant primary brain tumours
Primary and secondary tumours clinical manifestations
Cardinal Features
Features of raised ICP:
- headache - worse in morning and lying down, associated with N&V, exacerbated by coughing, sneezing, drowsiness - bear in mind headaches are very common and not much more common than in general population red flags: features of raised ICP (papilloedema and CN VI palsy)
- papilloedema
Progressive focal neurological deficits:
- hemiparesis
- hemisensory loss
- visual field defect
- dysphasia
Epileptic seizures
Reg flags: new focal symptoms, unilateral, awakes at night
Primary and secondary tumours management
Dexamethasone - reduce oedema around tumour
Resective craniotomy +/- biopsy
Radio/chemo available but limited
Vasculitis definition
Conditions where there is inflammation of blood vessels. Most cases are idiopathic. Classified into large, medium, small vessel variable vessel, signal organ and systemic disease-associated
Vasculitis presentation
Large vessel: HTN, anurysms, dissection, end-organ ischaemia
Medium vessel: ulcers, nodules, lesions, HTN
Small vessel: purpura, ting papules, splinter haemorrhages, urticaria
Vasculitis diagnosis
Full history
Bloods
Creen for Antinuclear antibodies (ANA)
Vasculitis management
Corticosteroids - control acute symptoms
Immunosupression - methotrexate, azathioprine
Plasmapheresis
Giant cell artiritis (GCA) definition
A granulomatous vasculitis of large and medium sized arteries. It primarily affects branches pf the external carotid artery and its branches such as the superficial temporal artery and is the most common form of systemic vasculitis in adults.
Giant cell artiritis epidemiology
Typically occurs in people >50yrs and is more common in women. Associated with polymyalgia rheumatica
Giant cell artiritis aetiology
Likely genetic and possibly infective factors
Giant cell artiritis pathophysiology
Extracranial branches of the carotid artery are preferentially involved although the aorta and its branches may be affected. Affected arteries contain granulomas and may have multinucleate giant cells present although they are not needed for diagnosis of GCA. Inflammation leads to proliferation of myofibroblasts, new vessel formation and marked thickening of the intimal or inner layer. This process leads to narrowing or occlusion of the vessel lumen, ultimately causing tissue ischaemia.
Symptoms derive from ischaemia to organs such as the eye and brain leading to blindness and storke.
Giant cell artiritis clinical manifestations
Headache - usually temporal or occipital areas
Polymyalgia rheumatica symptoms - aching and stiffness in neck and joints after a period of inactivity and with movement
Extremity claudication
Cranial artery abnormalities - occipital, postauricular or facial arteries may be
Can cause jaw claudication, visual symptoms, tender temporal arteries
Giant cell artiritis investigations
Bloods: ESR & CRP raised, often have normochromic normocytic anaemia with normal WBC and elevated platelets
Temporal artery USS and Biopsy
Giant cell artiritis management
1st line: Prednisolone - start immediately if suspected
- may want to start with methylprednisolone pulse therapy
Adjunct: aspirin (lower risk of vision loss and stroke)
Consider osteoporosis prevention (glucocorticoid use): calcium carbonate
Giant cell artiritis complications
Large vessel stenosis (especially subclavian and axillary arteries) Aortic aneurysm (thoracic are much more likely)
Spinal cord compression and cauda equina definition
Spinal cord compression: results from processes that compress or displace arterial, venous or cerebrospinal fluid spaces as well as the cord itself
Brown-Sequard syndrome: hemisection of spinal cord usually due to penetrating trauma
Cauda equina syndrome: occurs when the nerves below the end of the spinal cord (cauds equina) is damaged. It is a surgical emergency
Spinal cord compression and cauda equina aetiology
Spinal cord compression:
Trauma
- usually vertebral fractures or facet joint dislocation
- complete transection of the spinal cord
- hemisection (brown-sequard syndrome) usually caused by a penetrating trauma
Tumours (benign and malignant)
- bone tumours, primary/metastatic tumours
- common sites of primary tumours are breats, prostate and lung
- lymphoma, myeloma
- neuro tumours eg schwannoma, neurofibroma
Prolapsed intervertebral disc
- L4/5 and L5/S1 are the most common site for disc prolapse
- large disc herniation can cause Cauda equina syndrome
Epidural/subdural hematoma
Inflammatory disease, especially RA
Infection
Spinal cord compression and cauda equina pathophysiology
Spinal cord compression:
Injury to the spinal cord or nerve roots arises from stretching or from pressure. This results in injury to the white matter (myelinated tracts) and grey matter (cell bodies) in the cord with loss of all or some of the sensory modalities (pinprick, joint position sense, vibration, hot/cold, pressure) and motor function
Spinal cord compression and cauda equina clinical manifestations
Spinal cord compression Red flag signs: - loss of bladder or bowel function - UMN signs in lower limbs (eg clonus, hyperreflexia) - LMN signs in upper limbs (eg atrophy)
Symptoms depend on injury type and sit:
- paraplegia
- pain
- paraesthesia
- changes to tendon reflexes
Cauda equina syndrome
- bilateral neurogenic sciatica (pain going down the leg from the lower back, along the course of the sciatic nerve)
- reduced perineal sensation
- altered bladder function with painless retention
- loss of anal tone (bowel dysfunction)
- loss of sexual function
Brown-Sequard Syndrome
- ipsilateral hemiplagia with contralateral pain and temperature sensation deficits (due to crossing of fibres in spinothalamic tracts)
- this is a theoretical injury, it is almost impossible to get a spinal cord injury where you damage the spinal cord exactly in half like this
Spinal cord compression and cauda equina investigations
Spinal cord compression:
- spine X-ray is 1st line
- MRI spine - imaging of choice
Cauda eqina:
- medical emergency - immediate referral
- rectal exam - loss of anal tone/sensation
- MRI is the preferred investigation although often shows no abnormality
Brown-Sequard Syndrome:
- plain radiographs (X-ray) is 1st line
- MRI for extent of injury
Spinal cord compression and cauda equina management
Spinal cord compression:
- immobilisation essential
- decompressive/stabilisation surgery
- dexamethosone until treatment plan confirmed
Cauda equina
- surgical decompression is essential
Brown-Sequard
- spine immobilisation
- steroids for swelling
Myasthenia gravis definition
Autoimmune destruction of Ach (nicotinic) receptors at the neuromuscular junction (NMJ) mediated by ACh receptor antibodies (anti-AChR)
Peak incidence around 30 and 60 years. Twice more common in women
Associated with: SLE, RA, thyroid disease, pernicious anaemia, thymic hyperplasia (wmone>50yrs), thymic atrophy and tumours
Myasthenia gravis clinical manifestations
Muscle weakness that is worse on exertion and gets better with rest. Ocular muscles are usually involved first (ptosis and diplopia). Often partial or unilateral, improves after sleep and improves with putting an ice pack on the lid.
Fatigable ptosis (eyelid droop) and mysathenia snarl (snarling expression when attempting to smile) may be present.
Progression of weakness is usually top down: extraocular -> bulbar -> face -> limb girdle -> trunk
Weakness is more marked in proximal muscles. No muscle wasting, sensation is unimpaired.
Seizures can occur
Myasthenia gravis investigations
Anti-AchR antibodies (specific for MG)
Nevre conduction studies (NCS): characteristic decrement in potential
Mediastinal imaging with CT or MRI to look for thymoma
Tensilon test (IV of anticholinesterase) - rarely required
Myasthenia gravis management
Symptomatic control: anticholinesterase eg pyridostigmine
Relapse treatment:
- immunosuppression with prednisolone (and osteoporosis prophylaxis) - azathioprine etc may be used
- plasmapheresis and IV Ig
- thymectomy if needed (thymoma)
Exacerbating drugs:
- ciproflaxin, azithromycin
- propanolol, atenolol
- verapamil
- lithium
- statins
- chloroquine
- prednisolone
Peripheral neuropathies definition
Peripheral neuropathy refers to any disorder of the peripheral nervous system.
Acute or chronic.
Acute neuropathies (such as Guillian-Barre syndrome) eveolve very rapidly.
Chronic neuropathies can be further classified into small fibre and large fibre neuropathies. Neurophysiology helps to divide large fibre into axonal or demyelinating. Chronic demyelinating neuropathies are usually genetic or inflammatory (eg CIDP). These are generally seen by specialist neurologists whereas axonal neuropathies are seen more in primary care and hospital.
Nerve fibres:
- Aa (large myelinated) - proprioception signals
- Ab (large myelinated) - light touch, pressure, vibration
- small myelinated - pain, cold sensation
- C (small unmyelinated) - pain, warm sensation
Mononeuropathy = problem with one nerve
- carpal tunnel syndrome (median nerve)
- ulnar neuropathy (entrapment at the cubital terminal)
- peroneal neuropathy (entrapment at the finular head)
- cranial mononeuropathies (III or VIII CN palsy) - idiopathic, immune mediated, ischaemic
Polyneuropathy = problem with many nerves Axonal: - systemic disease: diabetes mellitus - infectious (hep, HIV, lyme) - ischaemic (vasculitis) - metabolic (Fabry's, porphyria) - hereditary (CHarcot-Marie-Tooth) - toxins Demyelinating - Guillain Barre syndrome
Mononeuritis Multiplex (multifocal neuropathy) - two or more nerves in disparate parts of the body
Peripheral neuropathies aetiology
Diabetes is the most common cause
Can also be due to: dietary deficiencies, medicines, alcohol excess, CKD, injury, infection, connective tissue disorders, inflammatory conditions, some hereditary diseases
Segmental demyelination: from guillain barre syndrome and charcot-marie-tooth disease
Peripheral neuropathies pathophysiology
Several mechanisms can cause nerve dysfunction
Demyelination: when Schwann cells are damaged the myelin sheath is damaged. This causes marked slowing of conduction, seen for example in Guillain-Barre syndrome.
Axonal degeneration: primary damage is in the axons which dies back from the peripheries
Wallerian degeneration: changes following nerve section (cut). Both axon and distal myelin sheath degenerate over several weeks.
Compression: focal demyelination at the point of compression. This occurs particularly in entrapment neuropathies (eg Carpal Tunnel Syndrome)
Infarction: microinfarction of the vasa nervorum (supplies nerves with blood) occurs in arteritis occurs in diabetes mellitus.
Inflitration: inflammatory cell inflitration due to leprosy, malignant cells or granulomas in sarcoidosis.
Essential tremor definition
Most commo type of tremor. Involuntary and rhythmic shaking, trembling often occurs in your hands, especially when you do simple tasks such as drinking from a glass or tying shoelaces.
Intention tremors are slower, zigzag-like movements which are evident during deliberate and visually guided movement.
Essential trmor can lead to trmors without accompanying intentional movements.
Essential tremor risk facotrs
Advanced age, familial history, exposure to toxins.
Essential tremor presentation
Usually absent at rest
Essential tremor diagnosis
Clinical
Essential tremor management
Propanolol, primidone
Huntingdon’s disease definition
Autosomal dominant neurogenerative disorder
Huntingdon’s disease pathophysiology
Expanded CAG repeat on huntingtin protein (chromosome 4) - toxic protein fragment production - cross-linking occurs - aggregation - resistance to degradation - interference with normal cellular function. Essentially, too much dopamine.
Huntingdon’s disease presentation
Typical onset 35-45, more common northern europeans
Non-motor: irritability, impulsivity, personality change
Motor: main sign is hyperkinesia, chorea, twitching, loss of coordination
Huntingdon’s disease diagnosis
Clinical diagnosis
MRI/CT - loss of striatal volume
Huntingdon’s disease management
Non-pharmacological: counselling, carer support, physiotherapy, exercise
Pharmacological: antipsychotics, antidepressants, SSRIs dependent on symptoms
Huntingdon’s disease complications
Prognosis is poor, concurrent disease and suicide are the most common causes of death
Guillain-Barre syndrome definition
An acute immune-mediated demyelinating polyneuropathy. Immune response against peripheral nerve myelin. Most common cause of acute polyneuropathy.
Guillain-Barre syndrome aetiology
Usually post-infective
- campylobacter
- EBV
- CMV
- mycoplasma
- HIV
Antibodies attack nerves, symptoms present a few weeks after infection
Other causes include vaccination, surgery, trauma
Guillain-Barre syndrome presentation
Progressive onset of distal limb weakness/numbness - symmetrical, ascending, TOES TO NOSE WEAKNESS
Absent tendon reflexes (LMN sign)
Sesnory deficit distally - paraesthesia
Respiratory/facial muscle involvement
Occasionally autonomic - HR/BP/urinary control
Guillain-Barre syndrome diagnosis
Bloods - B12, FBC, LFTs, TFTs Lumbar puncture - raised protein - normal WCC Spirometry and ECG for resp and cardiac functions respectively
Guillain-Barre syndrome management
IV Immunoglobulins for 5 days Plasma exchange (plasmapheresis) Avoid corticosteroids
Lambert-Eaton syndrome definition
Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune disorder of the neuromuscular junction. Caused by impaired presynaptic release of ACh.
Autoimmune attack on presynaptic calcium channels.
Lambert-Eaton syndrome aetiology
It occurs in association with an underlying cancer (CA-LEMS) (commonly small cell lung carcinoma) or without cancer and part of a more general autoimmune state (NCA-LEMS)
Lambert-Eaton syndrome presentation
Insidious and gradual onset of fatigue, weakness and a dry mouth
Limb weakness - begins in the proximal legs, typically hip flexion and hip abdunction and subsequently usually affects the proximal arms
Weakness may lead to a ‘waddling gait’
Ptosis
Dysarthria, areflexia, ptosis, diplopia, impotence, dysphagia may be present
Lambert-Eaton syndrome diagnosis
DDx: presents similarly to Myasthenia Gravis - check for ACh receptor antibodies to rule out MG
Nerve conduction studies, 50Hz
MRI for malignancy - 2yrs after diagnosis if cancer not already present
Lambert-Eaton syndrome management
Acetylcholinesterase inhibitors
Amifampridine - improves muscle strength
