Selected Notes neuro 1 Flashcards
What is a subarachnoid haemorrhage?
<ul><li>Blood within the subarachnoid space(under arachnoid mater)</li><li><img></img><br></br></li></ul>
Describe the epidemiology of SAH
<ul><li>F>M</li><li>Peak incidence: 40-50 years</li><li>80% without trauma due to a ruptured berry aneurysm</li></ul>
What is the most common cause of a SAH
<ul><li>Head injury</li></ul>
Name some risk factors for developing a Berry aneurysm?
<ul><li>Hypertension</li><li>Adult polycystic kideny disease</li><li>EDS</li><li>Coarctation of the aorta</li></ul>
Name the symptoms of a SAH
<ul><li>Sudden onset 'thunderclap' headache, peaks in intensityin 1-5 minutes</li><li>May have history of previous less severe 'sentinel' headache</li><li>Altered consciousness</li><li>Nausea and vomiting</li><li>Seizures</li><li>Meningism: photophobia and neck stifness</li></ul>
Name the signs of an SAH
<ul><li>Fundoscopy: retinal haemorrhage</li><li>Positive Kernig's/Brudzinksi's sign</li><li>Focal neurological deficits</li></ul>
<div>CN3/4/6-diplopia</div>
<div>Hemiparesis/hemiplegia</div>
What investigations should be done in a patient with a SAH
<ul><li>Non contrast CT head->hyperdense blood in basal cistern</li><li>If normal >6 hours post onset:</li><li>LP if >12 hours post onset: xanthochromia </li><li>CT angiogram to check for aneurysms or vascular abnormalitis</li></ul>
Describe the treatment for an SAH
<ul><li>Oral nimodipine to prevent vasospasm-> ischaemic damage</li><li>Coiling, stenting or clipping of aneurysms: neurosrugery</li></ul>
Name some complications of an SAH
<ul><li>Re-bleeding</li><li>Hydrocephalus</li><li>Vasospasm</li><li>Hyponatraemia->SIADH</li><li>Seizures</li></ul>
Describe the prognosis of a patient with an SAH
<ul><li>If untreated: 50% mortality</li><li>Of those who survive the 1st month: 50% will beocme dependent, 85% recovery in those admitted to neurosurgical unit</li></ul>
Name some predictive factors for the outcome of a patient with an SAH
<ul><li>Age</li><li>Consciousness level on admission</li><li>Amount of blood visible on CT head</li></ul>
Describe the pathophysiology of a patient with a TIA
<ul><li>Transient disruption of blood flow to a specific region of the CNS resulting in ischaemia</li></ul>
Describe the presentaiton of a patient with a TIA
<ul><li>Completely resolves within 24 hours</li><li>Stroke symptoms</li></ul>
<div>Aphasia/dysarthria</div>
<div>Unilateral weakness/sensory loss</div>
<div>Ataxia, vertigo, balance issues</div>
<div>Visual: amaurosis fugax, diplopia, HH</div>
What investigations should be done in a patient with a suspected TIA?
<ul><li>Neuroimaging: MRI(ischaemia, haemorrhage, other pathologies)</li><li>Carotid dopple USS-> atherosclerosis</li><li>Echo: cardiac thrombus</li><li>24hr ECG: AF</li><li>Bloods: glucose, lipid profile, clotting</li></ul>
What is the immediate treatment for a patient with a TIA?
<ul><li>Immediate antithrombotic therapy: aspirin 300mg unless CI</li></ul>
Name some contraindications for aspirin therpay in a patient with a TIA
<ul><li>Bleeding disorder</li><li>Already on aspirin</li></ul>
For a patient with a TIA in the last 7 days, how urgently should they be reviewed by a specialist?
<ul><li><7 days: urgent assessment within 24 hours</li></ul>
For a patient with a TIA over 7 days ago, how urgently should they be reviewed by a specialist
<ul><li>Within 7 days</li></ul>
For a patient with a crescendo TIA or multiple TIAs, how urgently should they be reviewed by a specialist?
<ul><li>Admitted immediately</li><li>Liekly cardioembolic source</li></ul>
Describe the secondary management of TIA
<ul><li>Antiplatelet therapy: clopidogrel</li><li>Lipid moidification: atorvastatin 20-80mg daily</li><li>Carotid endartectomy if severe carotid stenosis</li></ul>
What is an extradural haemorrhage?
<ul><li>Blood collects between the dura mater(outermost meningeal layer) and inner surface of the skull</li></ul>
What is the most common blood vessel implicated in an extradural haemorrhage?
<ul><li>Middle meningeal artery-> thin skull at pterion</li></ul>
<div>Usually arterial</div>
Describe the typical presentaiton of a patient with an extradural haemorrhage
<ol><li>Initial brief los sof consciousness post initial trauma</li><li>Lucid interval(regianed consciousness and apparent recovery)</li><li>Subsequent deterioration of consciousness and headache onset</li></ol>
Describe the pathophysiology of an extradural haemorrhage
<ul><li>Haematoma expands-> uncus of temporal lobe herniates around the tentorium cerebelli-> parasympathetic fibres of CN3 compressed-> fixed and dilated pupil</li></ul>
Name some differentials for a extradural haematoma
<ul><li>Subdural haemorrhage</li><li>SAH</li><li>Intracerebral haemorrhage</li><li>Cerebral contusion</li></ul>
What investigations should be done in a patient with an extradural haematoma?
<ul><li>CT scan: biconvex/lentiform hyperdense collection limited by the suture lines of the skull</li><li>Assess for midline shift/uncal herniation</li><li><img></img><br></br></li></ul>
Describe the management of an extradural haematoma
<ul><li>No neurological deficits-> conservative: supportive therapy and radiological observation</li><li>Definitive: craniotomy and haematoma evacuation</li></ul>
What is a subdural haemorrhage?
<ul><li>Accumulation of venous blood in the potential space between the dura mater and arachnoid mater of the brain</li></ul>
How can subdural haemorrhages be classified?
<ul><li>Acute</li><li>Subacute</li><li>Chronic</li></ul>
Describe the timeline of an acute subdural haemorrhage
<ul><li>Sx develop within 48 hours of injury-> rapid neurological deterioration</li></ul>
Describe the timeline of a subacute subdural haemorrhage
<ul><li>Sx present days-> weeks post injury-> gradual progression of neurological symptoms</li></ul>
Describe the timeline of a chronic subdural haemorrhage
<ul><li>Elderly: weeks-> months</li><li>Might not remember specific head injury</li></ul>
Name some risk factors for developing a subdural haemorrhage
<ul><li>Increasing age</li><li>Anticoag use</li><li>Chronic alcohol use</li><li>Recent trauma</li><li>Infants (shaken baby)</li></ul>
What kind of vessels are implicated in a subdural haemorrhage?
<ul><li>Rupture of bridging veins within subdural space</li></ul>
Describe the typical way in which a patient with a subdural haemorrhage would present?
<ul><li>History of head trauma-> lucid interval-> decline in consciousness</li></ul>
Name some clinical neurological signs you might find in a patient with a subdural haemorrhage
<ul><li>Altered/fluctuating mental status</li><li>Focal neurological deficits</li><li>Headache</li><li>Memory loss</li><li>Cognitive impariment</li><li>Seizures</li><li>Personality changes</li></ul>
Name some possible exam abnormalities in a patient with a subdural haemorrhage
<ul><li>Papilloedema (raised ICP)</li><li>Pupillary changes: unilateral dilated pupil-> CNS compression</li><li>Gait abnormalities</li><li>Hemiparesis/hemiplegia</li><li>Bradycardia, hypertension, irregular respirations (Cushing's triad)</li></ul>
What investigations should be done in a patient with a subdural heamorrhage?
<ul><li>CT scan: screscent shaped, not restricted by suture lines</li><li>Hyperacute(<1hr): isodense</li><li>Acute (<3 days): hyperdense</li><li>Subacute(3d-3 weeks): idosense</li><li>Chronic (>3 weeks): hypodense</li></ul>
<div><img></img><br></br></div>
Describe the management of a patient with a subdural haemorrhage
<ul><li>Conservative: monitor ICP etc</li><li>Acute: decompressive craniotomy</li><li>Chronic: Burr holes</li></ul>
What is an ischaemic stroke?
<ul><li>Sudden onset neurological deficit of vascular aetiology with symptoms lasting >24 hours</li></ul>
Describe the pathophysiology of an ischaemic stroke
<ul><li>Decrease in blood flow-> low O2 and glucose-> energy failure and disruption of cellular ion haemostasis-> exotoxicity, oxidative stress, inflammation and apoptosis-> irreversible neuronal damage</li><li>Can lead to cerebral oedema-> raised ICP-> secondary neuronal damage</li></ul>
Name sone strong risk factors for an ischaemic stroke
<ul><li>Increasing age</li><li>Male</li><li>Family history</li><li>Hypertension</li><li>Smoking</li><li>Diabetes</li><li>AF</li></ul>
Name some weak risk factors for an ischaemic stroke
<ul><li>High cholesterol</li><li>Obesity</li><li>Poor diet</li><li>Oestrogen therapy</li><li>Migraine</li></ul>
How is an ischaemic stroke diagnosed?
<ul><li>Non contrast CT head</li><li>Areas of low density/'hyperdense artery' sign</li></ul>
What is alteplase?
<ul><li>Thrombolytic: tissue plasminogen activator</li></ul>
Describe the secondary prevention of an ischaemic stroke
<ul><li>Clopidogrel 75mg </li><li>Aspiring if clopidogrel CI or not tolerated</li><li>Carotid endartectomy within 7 days if severe carotid stenosis</li><li>Atorvastatin 20-80mg OD</li><li>Smoking cessation and lifestyle advice</li><li>Hypertension treatment and diabetes check</li></ul>
What artery supplies the lateral cerebral cortex?
<ul><li>Middle cerebral artery</li></ul>
What artery supplies the anterior cerebral cortex?
<ul><li>Middle cerebral artery</li></ul>
Describe the symptoms of lateral pontine syndrome and name the implicated artery
<ul><li><div>Ipsilateral:</div><div>-CN3 palsy</div><div>-Vertigo/nystagmus/deafness</div><div>-Poor coordination/tone/balance</div></li><li><div>Anterior inferior cerebellar artery</div></li></ul>
Describe the symptoms of Weber’s syndrome
<ul><li>Ipsilateral CN3 palsy</li><li>Contralateral hemiparesis</li></ul>
Name some cerebellar signs
<ul><li>Nystagmus</li><li>Vertigo</li></ul>
Name some signs of bulbar muscle weakness
<ul><li>Dysphagia </li><li>Dysarthria</li></ul>
What is Horner’s syndrome?
<ul><li>Ptosis</li><li>Miosis</li><li>Anhidrosis</li><li>Damage to sympathetic nerve supply to the eye</li></ul>
What are the criteria for a total anterior circulation stroke?
<div>3/3 of:</div>
<div><ul><li>Unilateral weakness and/or sensory deficit of face, arm and leg</li><li>Homonymous hemianopia</li><li>Higher cerebral dysfunction(dysphasia, visuospatial disorder)</li></ul></div>
What are the criteria for a partial anterior circulation stroke
<div>2/3 of:</div>
<div><ul><li>Unilateral weakness and/or sensory deficit of face, arm and leg</li><li>Homonymous hemianopia</li><li>Higher cerebral dysfunction(dysphasia, visuospatial disorder)</li></ul></div>
What are the criteria for a posterior circulation stroke?
1/5 of:<br></br><ul><li>Cranial nerve palsy and contralateral motor/sensory deficit</li><li>Bilateral motor/sensory deficit</li><li>Conjugate eye movement disorder(horizontal gaze palsy)</li><li>Cerebellar dysfunction (vertigo, nystagmus, ataxia)</li><li>Isolated homonymous hemianopia</li></ul>
What causes a lacunar stroke and which part of the brain does it affect?
<ul><li>Subcortical</li><li>Occurs secondary to small vessel disease</li></ul>
How can a lacunar stroke be differentiated from other strokes?
<ul><li>NO loss of higher cerebral function</li></ul>
What are the criteria for a lacunar stroke?
1/4 of:<br></br><ul><li>Pure sensory stroke</li><li>Pure motor stroke</li><li>Sensori-motor stroke</li><li>Ataxic hemiparesis</li></ul>
What can a CN5 palsy result in?
Trigeminal <br></br><ul><li>Trigeminal neuralgia</li><li>Loss of corneal reflex</li><li>Loss of facial sensation</li><li>Paralysis of mastication muscles</li><li>Deviation of jaw to weak side</li></ul>
What can a CN6 palsy result in?
Abducens<br></br><ul><li>Defective abduction-> hortizontal diplopia</li></ul>
What can a CN7 palsy result in?
Facial<br></br><ul><li>Flaccid paralysis of upper and lower face</li><li>Loss of corneal reflex(efferent)</li><li>Loss of taste</li><li>Hyperacusis</li></ul>
What can a CN8 palsy result in?
Vestibulocochlear<br></br><ul><li>Hearing loss</li><li>Vertigo, nystagmus</li><li>Acoustic neuromas</li></ul>
What can CN9 palsy result in?
Glossopharyngeal<br></br><ul><li>Hypersensitive carotid sinus reflux</li><li>Loss of gag reflex (afferent)</li></ul>
What can a CN10 palsy result in?
Vagus<br></br><ul><li>Uvula deviates away from site of lesion</li><li>Loss of gag reflex(efferent)</li></ul>
What can a CN11 palsy result in?
Accessory<br></br><ul><li>Weakness turning head to contralateral side</li></ul>
What can a CN12 palsy result in?
Hypoglossal<br></br><ul><li>Tongue deviates towards the side of lesion</li></ul>
What is encephalitis?
<ul><li>Inflammation of the brain parenchyma</li></ul>
Describe the epidemiology of encephalitis
<ul><li>Peak: >70 yrs, <1 year</li><li>M:F 1:1</li></ul>
Describe the symptoms of encephalitis
<ul><li>Fever</li><li>Headaches</li><li>Seizures</li><li>Psych sympotms</li><li>Vomiting</li><li>Focal features</li><li>Flu-like prodromal illness</li></ul>
Name some differentials for encephalitis
<ul><li>Hypoglycaemia</li><li>HE</li><li>DKA</li><li>Uremic/drug induced encephalopathy</li></ul>
What investigations should be done in a patient with suspected encephalitis?
<ul><li>CSF testing: lymohocytosis, hgih protein, viral PCR analysis of CSF</li><li>MRI</li><li>EEG</li><li>CT</li></ul>
How is encephalitis treated?
<ul><li>10mg/kg aciclovir TDS for 2 weeks</li><li>Broad spectrum antibiotics e.g. ceftriaxone</li><li>Supportive-seizure management</li></ul>
Name some side effects of aciclovir
<ul><li>GI changes</li><li>Photosensitivity and rashes</li><li>Acute renal failure</li><li>Hepatitis</li></ul>
Describe the prognosis of encephalitis
<ul><li>10-20% mortality of treatment started promptly</li><li>80% mortality if untreated</li></ul>
What is meningitis?
<ul><li>Inflammation of the meninges (dura, arachnoid, pia)</li><li>Can be infective or non infective</li></ul>
Describe the epidemiology of meningitis
<ul><li>Viral(enteroviruses) most common</li><li>Bacterial: associated with increased morbidity and mortality</li><li>Fungal/parasitic: rare except in immunosuppressed</li></ul>
Name the most common bacterial causes of meningitis and the groups they are present in
<ul><li>S.pneumoniae</li><li>N.meningitidis</li><li>H. influenza: infants</li><li>Listeria monocytogenes: Elderly</li></ul>
Name some viral causes of meningitis
<ul><li>Enteroviruses</li><li>Herpes</li><li>VZV</li><li>Measles/rubella</li></ul>
What is the most common fungal cause of meningitis?
<ul><li>Cryptococcus neoformans</li></ul>
Name the parasitic causes of meningitis
<ul><li>Amoeba</li><li>Toxoplasma gardii</li></ul>
Name some non infective causes of meningitis
<ul><li>Malignancies: leukaemia, lymphoma</li><li>Drugs: NSAIDs, trimethoprim</li><li>Systemic inflammatory diseases: Sarcoidosis, SLE, Behcets</li></ul>
Describe the symptoms of meningitis
<ul><li>Headahce</li><li>Fever</li><li>Nausea and vomiting</li><li>Seizures</li><li>Decreased consciousness</li><li>Photophobia</li><li>neck stuffness</li><li>Non blanching petechial/purpuric rash-> DIC</li></ul>
Name some signs in a patient with meningitis
<ul><li>Kernig's sign: Pain and resistance to knee extension</li><li>Brudzinski's sign: passive neck flexion results in involuntary hip and knee flexion</li></ul>
Name some differential diagnoses for meningitis
<ul><li>Encephalitis</li><li>SAH</li><li>Brain abscess</li><li>Sinusitis</li><li>Migraine</li></ul>
What investigations should be done in a patient with suspected meningitis?
<ul><li>Bloods: FBC, CRP, coag screen, cultures, PCR, glucose</li><li>ABG</li><li>CT head</li><li>LP and CSF analysis </li></ul>
Describe the treatment of a patient with suspected meningitis if presenting to a GP
<ul><li>IM benzylpenicillin and urgent hospital transfer</li></ul>
Describe the treatment of a patient with suspected bacterial meningitis in hospital
<ul><li>IV cefotaxima/ceftriaxone and IV dexamethasone</li><li>Add amoxicillin for listeria cover(age extremes)</li></ul>
Describe the treatment of suspected viral meningitis
<ul><li>If enteroviruses: nothing</li><li>If HSV/VZV: aciclovir</li></ul>
What are the prophylaxis recommendations for contacts of meningitis
<ul><li>If in contact 7 days prior to onseet: One off dose of oral ciprofloxacin</li></ul>
Name some complications of meningitis
<ul><li>Sepsis</li><li>DIC</li><li>SIADH</li><li>Seizures</li><li>Waterhouse friedrichsen syndrome</li><li>Delayed: hearing loss, cranial nerve dysfunction, intellectual deficits, ataxia, blindness</li></ul>
What is Waterhouse friedrichsen syndrome?
<ul><li>Adrenal insufficiency caused by intra-abdominal haemorrhage from DIC</li></ul>
Name the causes of meningitis is neonates(0-3 months)
<ul><li>Group B strep</li><li>E.Coli</li><li>Gram negative bacilli</li><li>Listeria</li><li>S.pneumoniae</li></ul>
Name the causes of meningitis in infants(3 months-6 years)
<ul><li>S.pneumoniae</li><li>N.meningitidis</li><li>H. influenzae</li></ul>
Name the causes of meningitis in adults(6-60 years)
<ul><li>S.pneumoniae</li><li>N.meningitidis</li></ul>
Name the causes of meningitis in the elderly (>60yrs)
<ul><li>S.pneumoniae</li><li>N.meningitidis</li><li>Listeria</li><li>Gram negative bacilli</li></ul>
How does S.pneumoniae appear on microbiology?
<ul><li>Gram positive diplococcus in chains</li></ul>
How does Group B strep appear on microbiology?
<ul><li>Gram positive coccus in chains</li></ul>
What age groups is most likely to have meningitis cauased by Group B strep and why?
<ul><li>Neonates</li><li>Colonises in maternal vagina</li></ul>
What age group is most likely to get meningitis from listeria monocytogenes?
<ul><li>Extremes of age, pregnant</li><li>Found in cheese</li></ul>
What does listeria monocytogenes appear like on microbiology?
<ul><li>Gram positive bacillus</li></ul>
What is neurofibromatosis?
<ul><li>Genetic condiiton that causes nerve tumours(neuromas) to develop in the nervous system</li><li>Benign but can cause neurological/structural problems</li></ul>
What are the types of neurofibromatosis and which is more common?
<ul><li>Type 1-more common</li><li>Type 2</li></ul>
What is neurofibromatosis type 1 also called?
<ul><li>Von Recklinghausen's syndrome</li></ul>
Describe the inheritance pattern of neurofibromatosis type 1
<ul><li>Autosomal dominant</li></ul>
Describe the features of neurofibromatosis type 1<br></br><div>CRABBING</div><div><ul><li>C{{c1::afe au lait spots(>15mm)}}</li><li>R{{c2::elative with F1}}</li><li>A{{c3::xillary/inguinal freckles}}</li><li>B{{c4::ony dysplasia-bowing of long bones or sphenoid wing dysplasia}}</li><li>I{{c5::ris hamartomas(Lisch nodules)-yellow/brown spots on iris}}</li><li>N{{c6::eurofibromas(over 2 significant unless more than 1 plexiform)}}</li><li>G{{c7::lioma of optic pathway}}</li></ul></div>
How is neurofibromatosis diagnosed?
<ul><li>Diagnostic criteria and genetic testing</li></ul>
Describe the treatment of neurofibromatosistype 1
<ul><li>Monitor and manage symptoms, treat complications</li></ul>
Name some complications of neurofibromatosis type 1
<ul><li>Malignant peripheral nerve sheath tumours(MPNST)</li><li>Gastrointestinal stromal tumours(GIST)</li><li>Migraines</li><li>Epilepsy</li><li>Hypertension from renal artery stenosis</li><li>Scoliosis</li><li>Brain.spinal tumours</li><li>High cancer risk</li></ul>
What is the inheritance pattern of neurofibromatosis type 2
<ul><li>Autosomal dominant</li></ul>
What is the main symptom/complication of neurofibromatosis type 2?
<ul><li><b>Bilateral</b> acoustic neuromas</li></ul>
What is giant cell arteritis/temporal arteritis?
<ul><li>Vasculitis of unknown cause that affects medium-large sized vessel arteries, especially at the temples</li><li>Overlap with PMR</li></ul>
Describe the epidemiology of giant cell arteritis
<ul><li>Most common primary vasculitis</li><li>>50 years, peaks in 70s</li><li>M:F 1:3</li></ul>
Name some risk factors for giant cell arteritis
<ul><li>Genetics</li><li>Environmental</li><li>Age-fmales</li><li>Sex</li><li>Ethnicity(Mc caucasian-scandinavian)</li></ul>
Describe the presentation of a patient with giant cell arteritis
<ul><li>Usually rapid onset: <1 month</li><li>Temporal headache</li><li>Jaw claudication</li><li>Amaurosis fugax, diplopia</li><li>Tender, palpable temporal artery, scalp tenderness, bruits(rare)</li><li>50% have PMR features(aching, morning proximal limb weakness, lethargy)</li></ul>
What investigations should be done in a patient with giant cell arteritis?
<ul><li>High ESR/CRP</li><li>Normal creatine kinase and EMG</li><li>Temporal artery biopsy-> granulomatous inflammatin and infiltration of giant cells</li><li>Doppler USS: 'halo' sign</li></ul>
Describe the managmeent of giant cell arteritis
<ul><li>Urget high dose steroids: 40-60mg prednisolone OD to prevent blindness</li><li>Then taper(use bisphosphonates/PPI)</li><li>Low dose aspiring to reduce risk of stroke/blindness</li></ul>
Name some complications of giant cell arteritis
<ul><li>Permanent monocular blindness</li><li>Diplopia</li><li>Stroke</li><li>Aortic aneurysms</li></ul>
How can giant cell arteritis result in permanent monocular blindness?
<ul><li>Anterior ischaemic optic neuropathy-> occlusion of posterior ciliary artery-> ischaemia of optic nerve head</li></ul>
How can giant cell arteritis cause diplopia?
<ul><li>Involvement of any paart of oculomotor system(e.g. cranial nerves)</li></ul>
What is Bell’s palsy?
<ul><li>Acute, unilateral, idiopathic facial nerve paralysis</li></ul>
Describe the presentation of Bell’s palsy
<div><ul><li>Acute(not sudden) onset of unilateral LMN facial weakness with NO foreheard sparing</li><li>Post auriicular otalgia(may precede paralysis)</li><li>Hyperacusis</li><li>Nervus intermedius symptoms: altered taste, dry eyes/mouth</li></ul></div>
Name some differential diagnoses for bell’s palsy
<ul><li>Ramsay Hunt syndrome</li><li>Stroke-forehead sparing</li><li>Guillain Barre</li></ul>
What investigations might be done in a patient presenting with suspected Bell’s palsy?
<ul><li>Clinical: rule out other causes/assess extent of damage</li><li>FBC/ESR/CRP/viral serology/lyme serology/otoscopy/EMG/MRI/CT</li></ul>
Describe the management of Bell’s palsy
<ul><li>50mg oral pred OD for 10 days then taper</li><li>Aciclovir in certain patients (e.g. for Ramsay Hunt)</li><li>Supportive: artificial tears/ocular lubricants/eye tape</li></ul>
Describe the prognosis of Bell’s palsy
<ul><li>Complete recovery: 70-80% in weeks-months</li><li>If untreated: 15% have permanent moderate/severe weakness</li></ul>
Name some poor prognostic factors for Bell’s palsy
<ul><li>Older age</li><li>More severe initial facial weakness</li></ul>
What is essential tremore?
<ul><li>Common movement disorder characterised by a rhythmic postural or kinetic tremor primarily affecting the upper extremities</li></ul>
Describe the epidemiology of essential tremor
<ul><li>Commmon</li><li>Increased age: peak 40-50 years</li><li>F:M:1:1</li><li>Family history</li></ul>
Describe the pathophysiology of essential tremor
<ul><li>GABA-ergic dysfunction-> increased activity in cerebellar-thalamic cortical circuit</li></ul>
Describe the features of essential tremor
<ul><li>Postural/kinetic tremor that predominantly affects the upper limbs distally</li><li>Usually bilatera: high frequency: 6-12Hz</li><li>Exacerbated by intentional movements, absent on rest</li><li>Increasing amplitude over time</li><li>Relieved by alcohol</li><li>Exacerbated by anxiety, excitement etc</li><li>Can affect head, lower limbs, voice, tongue, face and trunk</li></ul>
What additional features of essential tremor might prompt further investigation to look for differentials?
<ul><li>Difficulty with tandem gait</li><li>Mild cognitive impairment</li><li>Slight resting tremor alongside action tremor</li></ul>
How is essential tremor diagnosed?
<ul><li>CLinical diagnosis</li><li>bilateral uppper limb action tremor lasting >3 years with no other tremor/neurological signs</li></ul>
Name some differential diagnoses for essential tremor
<ul><li>Parkinson's</li><li>Hyperthyroidism</li><li>Dystonic tremor</li><li>Spasmodic dysphonia</li></ul>
Describe the management of essential tremor
Pharmacological:<br></br><ul><li>Propanolol</li><li>Primidone</li><li>2nd line: gabapentin, topirimate, nimodipine</li></ul><div>Surgical: </div><div><ul><li>DBS</li><li>Botulinum toxin type A injections</li></ul></div>
Describe the prognosis of essential tremor
<ul><li>Typically worsens with increasing age</li><li>Can remain isolated or can spread e..g. to head or voice over years</li><li>Cna cause major disability</li></ul>
What is myasthenia gravis?
<ul><li>Autoimmune disease-> AChR(nicotinic ACH receptor antibodies)</li></ul>
Describe the epidemiology of myasthenia gravis
<ul><li>M:F 1:1</li><li>Bimodal distribution</li><li>Peak incidence in F<40 years and M>60 years</li></ul>
Describe the pathophysiology of myasthenia gravis
85%:<br></br><ul><li>AChR antibodies-> lower ability of ACh to trigger muscle contractioons-> weakness</li><li>Also MuSK(muscle-specific kinase) and LLRP4(low density lipoprotein receptor related protein)-> creation and organisation of AChR-> inadequate AChR</li></ul>
Describe the presentation of a patient with myasethnia gravis
<ul><li>Muscle weakness that worsens with repetitive activity and weakens with rest(typically ocular/bulbar and limb muscles)</li><li>Ptosis</li><li>Diplopia</li><li>Dysarthria</li><li>Dysphagia</li><li>Proximal limb weakness</li><li>EExacerbated by beta blockers, lithium, phenytoin and certain abx</li></ul>
How is myasthenia gravis investigated/diagnosed?
<ul><li>Bedside: ice pack test</li><li>Serology for AChR antibodies, MuSK and LRP4 antibodies</li><li>CT/MRI chest to look for thymomas/thymic hyperplasia</li><li>Edrophonium test</li></ul>
What is the ice pack test for myasthenia gravis?
<div><ul><li>Measure degree of ptosis</li><li>Apply ice pack for a few minutes</li><li>Measure degreee of ptosis again</li><li>Positive if >2mm improvement</li></ul></div>
What is the edrophonium test for myasthenia gravis?
<ul><li>Administer small amount of edrophonium <span>chloride </span><span>(Tensilon)</span></li><li><span>See effects </span></li><li><span>If rapid, transient increase in muscle strenght-> indicative of diagnosis</span></li></ul>
Describe the management of myasthenia gravis
<ul><li>ACh inhibitors: pyridostigmine, neostigmine</li><li>Immunosuppression-> steroids, azathioprine</li><li>Thymectomy</li><li>Rituximab</li><li>Plasma exchange and IVIG</li></ul>
Name some prognostic factors for myasthenia gravis
<ul><li>Age of onset</li><li>Antibody subtype</li><li>Thymus histology</li><li>Response to treatment</li></ul>
Name one complication of myasthenia gravis
<ul><li>Myasthenic crisis</li></ul>
What is a myasthenic crisis?
<ul><li>Life threatening acute worsening of symptoms, often triggered by another illness like a URTI-> can result in respiratory muscle failure</li></ul>
How is a myasthenic crisis treated?
<ul><li>Usual meds</li><li>IVIG and plasmapharesis</li><li>If FVC<=15mL/kg: mechanical ventilation</li></ul>
What is chronic fatigue syndrome?
<ul><li>Chronic disabling condition characterised by profound fatigue and impariment following minimal physical/cognitive effort</li></ul>
Describe the symptoms of chronic fatigue syndrome
<ul><li>Extreme fatigue</li><li>Post-exertional malaise</li><li>Sleep disturbances and unrefreshing sleep</li><li>Cognitive impairment</li><li>Orthostatic intolerance</li><li>Immune/neurological/autonomi/psychiatric manifestations</li></ul>
Name some differential diagnoses for chronic fatigue syndrome
<ul><li>Fibromyalgia</li><li>Depression</li><li>Hypothyroidism</li><li>AI disorders</li></ul>
How is chronic fatigue syndrome diagnosed?
<ul><li>Most clinical-rule out other causes</li></ul>
<div>TFT's, bloods: inflammation, infection, blood cell abnormalities</div>
<div><br></br></div>
<div>Has to last >3 months and significantly decrease ability to engage in activities</div>
Describe the managment of chronic fatigue syndrome
<ul><li>Refere to specialist CSF service if >3 months</li><li>Energy management</li><li>Graded exercise therapy no longer recommended</li><li>Symptoms control-treat other conditions, pain and sleep management</li><li>CBT</li></ul>
What is an acoustic neuroma also known as?
<ul><li>Vestibular schwannoma</li></ul>
What is an acoustic neuroma?
<ul><li>Benign subarachnoid tumour that exerts local pressure on cranial nerve 8</li></ul>
Describe the epidmiology of acoustic neuromas
<ul><li>Rare in UK</li><li>Adults aged 40-60 years</li></ul>
Describe the presentation of acoustic neuroma
<ul><li>Asymmetric/unilateral hearing loss</li><li>Progressive ipsilateral tinnitus</li><li>Sensorineural deaffness</li><li>Larger tumours: raised ICP like focal neurology: CN5/67/8</li><li>Dizziness, headaches, disequilibrium</li></ul>
Name some differential diagnoses for acoustic neuroma
<ul><li>Meniere's disease</li><li>Labyrinthitis</li><li>BPPV</li></ul>
What investigations should be done in a patient with a suspected acoustic neuroma?
<ul><li>Audiometry</li><li>MRI scan of cerebellopontine angle</li></ul>
Describe the management of an acoustic neuroma
<ul><li>Urgetn referral to ENT</li><li>>40mm: surgery</li><li><40mm: 6 monthly annual surveillance scans via MRI</li></ul>
What focal neurology might be seen in a patient with an acoustic neuroma?
<ul><li>CN8: vertigo, unilateral sensorineural hearing loss, unilateral tinnitus</li><li>CN5: absent corneal reflex</li><li>CN7: facial palsy</li></ul>
What is Meniere’s disease?
<ul><li>Inner ear disorder caused by increased fluid pressure in the endolymphatic spaces of the membranous labyrinth</li></ul>
Describe the epidemiology of Meniere’s disease?
<ul><li>30-60 years</li><li>Predominantly unilateral</li></ul>
Describe the presentation of meniere’s disease
<ul><li>Sudden attacks of paroxysmal vertigo</li><li>Attacks last minutes to hours</li><li>Associated deafness</li><li>Tinnitus</li><li>Attacks often occur in clusters with period of remission where funciton is recovered</li><li>Can cause nystagmus and positive Romberg's test</li><li>Can be very disabling-> bedbound with nausea, vomiting and fluctuating hearing</li></ul>
Name some differential diagnoses for meniere’s disease
<ul><li>Vestibular neuritis</li><li>Labyrinthitis</li><li>BPPV</li></ul>
What investigations are used to diagnose meniere’s disease?
<ul><li>Clinical evaluation</li><li>Audiometric testing</li><li>Imaging/other tests may be used ot rule out other potential causes of symptoms</li></ul>
Describe the management of meniere’s disease
<ul><li>ENT assessment</li><li>Prophylactic use of betahistine to reduce frequenct</li><li>Acute: prochlorperazine</li><li>Diuretics-> reduce endolymphatic fluid(only prescribed by specialists)</li><li>Low salt diets can help rpevent attacks</li><li>DVLA: no driving until good control of sx</li></ul>
Describe the natural history of Meniere’s disease
<ul><li>Sympotms resolve in most patients after 10-15 years</li><li>Majority of patients left with a degree of hearing loss</li><li>Psychological distress common</li></ul>
What is trigeminal neuralgia?
<ul><li>Chronic pain condition characterised by severe sudden and brief bouts of shooting/stabbing pain that follow the distribution of one or more divisions of the trigeminal nerve, affecting the patients facial region</li></ul>
Describe the epidemiology of trigeminal neuralgia
<ul><li>>50yrs</li><li>F>M</li></ul>
Describe the presentation of a patient with trigeminal neuralgia
<ul><li>Unilateral facial pain that is sudden, severe and brief</li><li>Pain is shooting/stabbing</li><li>Triggered by lightly touching affected side of face, eating, or wind blowing</li><li>Neuro exam is typically normal</li></ul>
Name some differential diagnoses for trigeminal neuralgia
<ul><li>Post herpetic neuralgia</li><li>Temperomandibular joint disorders</li><li>Giant cell arteritis</li><li>Cluster headache</li></ul>
How is trigeminal neuralgia diagnosed?
<ul><li>Mostly clinical</li><li>MRI or other neuroimaging to rule out secondary causes</li></ul>
Describe the management of trigeminal neuralgia
Medical:<br></br><ol><li>Carbamazepine</li><li>Phenytoin</li><li>Lamotrigine</li><li>Gabapentin</li></ol><div>Surgical:</div><div><ul><li>Microvascular decompression-remove/relocate vessels</li><li>Treat underlying cause like AVM/tumour</li><li>Alcohol/glycerol injections</li></ul><div><br></br></div></div><div>Failure to respond to treatment/atypical: refer to neuro</div>
Name some red flag symptoms for trigeminal neuralgia
<ul><li>Sensory changes</li><li>Deafness</li><li>Pain only in ophthalmic division(eye socket, forehead and nose) or bilaterally</li><li>Optic neuritis</li><li>FHx of MS</li><li><40 yrs</li></ul>
What is bulbar palsy?
<ul><li>Subtype of LMN lesion impacting the 9/10/12th cranial nerves</li></ul>
Name the major causes of bulbar palsy
<ul><li>Motor neurone disease-mc</li><li>Myasthenia gravis</li><li>Guillain-barre</li><li>Brainstem stroke-Wallenberg's/lateral medullary syndrome</li><li>Syringobulbia</li></ul>
Describe the signs and symptoms of bulbar palsy
<ul><li>Dysarthria and dysphagia</li><li>Absent/normal jaw jerk reflex</li><li>Absent gag reflex</li><li>Flaccid, fasciculating tongue</li><li>Nasal speech, often described as 'quiet'</li><li>Additional signs suggestive of underlying cause(e.g. limb fasciculations: MND)</li></ul>
Name some differentials for bulbar palsy
<ul><li>Pseudobulbar palsy</li><li>Brainstem tumour</li><li>MS</li><li>Polymyositis and dermatomyositis</li></ul>
What investigations should be done in a patient with bulbar palsy?
<ul><li>Neuro exam</li><li>EMG and nerve conduction studies:MND/mysthenia gravis</li><li>Bloods: FBC, electrolytes, CK, autoantibody screening</li><li>MRI-lesions in brainstem</li><li>LP: rule out infections/AI causes</li></ul>
How is bulbar palsy managed?
<ul><li>Speech and swallowing therapy-manage dysarthria and dysphagia</li><li>Nutritional support</li><li>Treat underlying cause</li></ul>
How can Horner’s syndrome be characterised?
<ul><li>Pre-ganglionic</li><li>Post-ganglionic</li><li>Central</li></ul>
<div>Dependent on locatoin of sympathetic nerve interruption</div>
Name some causes of Horner’s syndrome
<ul><li>Pancoast tumour: pre-ganglionic</li><li>Stroke-central</li><li>Carotid artery dissection: post-ganglionic</li><li>Trauma, tumours, surgery-central</li></ul>
What is a pancoast tumour and how can it case Horner’s syndrome?
<ul><li>Non-small cell lung carcinoma</li><li>Located at superior sulcus of lung affects lower roots of brachial plexus anf sympathetic chain</li></ul>
How do patients with Horner’s syndrome typically present?
<ul><li>Ptosis-dropping of upper eyelid</li><li>Miosis-constriction of pupil</li><li>Anhidrosis</li><li>Enophthlamos-eye may appear sunken</li><li>Heterochromia-eye colour may change, more common in congenital Horner's syndrome</li><li><img></img><br></br></li></ul>
Name some differential diagnoses for Horner’s syndrome
<ul><li>Oculomotor nerve palsy-will also have ophthalmoplegia</li><li>Myasthenia gravis</li><li>Bell's palsy</li></ul>
What investigations might be done in a patient presenting with Horner’s syndrome?
<ul><li>Imaging: MRI/CT head neck and chest to ID structural causes</li><li>Bloods: assess for diabetes or AI disorders</li></ul>
<div>Pharmacological pupil testing to confirm diagnosis and differentiate between pre- and post-ganglionic lesions</div>
Describe the management of Horner’s syndrome
<ul><li>Treat underlying cause</li><li>If no casue identified, observatoin and regular follow up</li><li>Cosemtic interventions for ptosis or miosis may be considered</li></ul>
What is Guillain Barre syndrome?
<div><ul><li>Ascending inflammatory demyelinating polyneuropathy-> acute onset of bilateral and roughly symmetric limb weakness</li></ul></div>
How do patients with Guillan Barre present?
<ul><li>Progressive ascending symmetrical limb weakness-> lower limbs first</li><li>Low back pain from radiculopathy</li><li>Paraesthesia</li><li>LMN signs in lower limbs: hypotonia, flaccid paralysis, areflexia</li><li>Cranial nerve signs: opthlamoplegia, facial nerve palsy, bulbar palsy</li><li>Potential autonomic dysfunction</li><li>Potential respiratory muscle involvement</li></ul>
Name some variants of Guillaim Barre syndrome
<ul><li>Parapetic</li><li>Miller-Fischer</li><li>Pure motor</li><li>Bilateral facial palsy with paraesthesias</li><li>Pharyngeal brachial cervical weakness</li><li>Bickerstaff's brainstem encephalitis</li></ul>
Name some differential diagnoses for Guillain Barre
<ul><li>Vascular: strokes</li><li>Infective/inflammatory: polio, lyme, CMV, TB, HIV, CIDP, myastehnia gravis</li><li>Spinal cord compression</li><li>Metabolic-porphyria, electrolyte abnormalitis</li></ul>
What investigations might be done in a patient with suspected Guillain Barre?
<ul><li>Monitoring of FVC for respiratory muscle involvement</li><li>Serological: anti ganglioside antibodies</li><li>LP: albuminocytological dissociation</li><li>Nerve conduction studies: prolongation or loss of the F wave</li><li>ID underlying cuase: stool cultures, serology, CSF virology</li></ul>
What might be seen on an LP of a patient with Guillain barre?
<ul><li>albuminocytological dissociation</li><li>Increased albumin without corresponding increase in white blood cells</li></ul>
How is Guillain Barre managed?
<ul><li>Regular FVC monitoring-can rapidly deteriorate</li><li>VTW prophylaxis: TEDS and LMWH</li><li>Analgesia if needed</li><li>Manage complications: arrhythmias, autonomic dysfunction</li><li>Enteral feeding if unsafe swallow</li></ul>
<div>If significant disability:</div>
<div><ul><li>IVIG for 5 days</li><li>Plasmapharesis(more side effects that IVIG)</li></ul></div>
What is the prognosis of Guillain Barre syndrome
<ul><li>Mostly full recovery</li><li>Can have residual weakness or fatigue</li></ul>
Give some prognostic factors for Guillain Barre
<ul><li>Speed of onset</li><li>Severity</li><li>Age</li><li>Presence of preceding diarrhoeal illness</li></ul>
