Lecture ILO’s Flashcards

Question

Eye movement abnormalities in multiple sclerosis

Answer 1

Eye Movement Abnormalities Lesions affecting the oculomotor (CN III), trochlear (CN IV) or abducens (CN VI) can cause double vision (diplopia) and nystagmus. Oscillopsia refers to the visual sensation of the environment moving and being unable to create a stable image. Internuclear ophthalmoplegia is caused by a lesion in the medial longitudinal fasciculus. The nerve fibres of the medial longitudinal fasciculus connect the cranial nerve nuclei(“internuclear”) that control eye movements (the 3rd, 4th and 6th cranial nerve nuclei). These fibres are responsible for coordinating the eye movements to ensure the eyes move together. It causes impaired adduction on the same side as the lesion (the ipsilateral eye) and nystagmus in the contralateral abducting eye. A lesion in the abducens (CN VI) causes a conjugate lateral gaze disorder. Conjugate means connected. Lateral gaze is where both eyes move to look laterally to the left or right. When looking laterally in the direction of the affected eye, the affected eye will not be able to abduct. For example, in a lesion involving the left eye, when looking to the left, the right eye will adduct (move towards the nose), and the left eye will remain in the middle.

Answer 2

Focal Neurological Symptoms Multiple sclerosis may present with focal weakness, for example: • Incontinence • Horner syndrome • Facial nerve palsy • Limb paralysis Multiple sclerosis may present with focal sensory symptoms, for example: ○ Trigeminal neuralgia ○ Numbness ○ Paraesthesia (pins and needles) ○ Lhermitte’s sign Lhermitte’s sign describes an electric shock sensation that travels down the spine and into the limbs when flexing the neck. It indicates disease in the cervical spinal cord in the dorsal column. It is caused by stretching the demyelinated dorsal column. Transverse myelitis refers to a site of inflammation in the spinal cord, which results in sensory and motor symptoms depending on the location of the lesion.

Answer 3

Ataxia Ataxia is a problem with coordinated movement. It can be sensory or cerebellar. Sensory ataxia is due to loss of proprioception, which is the ability to sense the position of the joint (e.g., is the joint flexed or extended). This results in a positive Romberg’s test (they lose balance when standing with their eyes closed) and can cause pseudoathetosis(involuntary writhing movements). A lesion in the dorsal columns of the spine can cause sensory ataxia. Cerebellar ataxia results from problems with the cerebellum coordinating movement, indicating a cerebellar lesion.

Answer 4

Clinically isolated syndrome describes the first episode of demyelination and neurological signs and symptoms. Patients with clinically isolated syndrome may never have another episode or may go on to develop MS. Lesions on an MRI scan suggest they are more likely to progress to MS.

Answer 5

Relapsing-remitting MS is the most common pattern when first diagnosed. It is characterised by episodes of disease and neurological symptoms followed by recovery. The symptoms tend to occur in different areas with each episode. It can be further classified based on whether the disease is active or worsening: • Active: new symptoms are developing, or new lesions are appearing on the MRI • Not active: no new symptoms or MRI lesions are developing • Worsening: there is an overall worsening of disability over time • Not worsening: there is no worsening of disability over time

Answer 6

Secondary progressive MS is where there was relapsing-remitting disease, but now there is a progressive worsening of symptoms with incomplete remissions. Symptoms become increasingly permanent. Secondary progressive MS can be further classified based on whether the disease is active or progressing.

Answer 7

Primary progressive MS involves worsening disease and neurological symptoms from the point of diagnosis without relapses and remissions. This can be further classified based on whether it is active or progressing.

Answer 8

Diagnosis The diagnosis is made by a neurologist based on the clinical picture and symptoms suggesting lesions that change location over time. Other causes for the symptoms need to be excluded. Investigations can support the diagnosis: • MRI scans can demonstrate typical lesions • Lumbar puncture can detect oligoclonal bands in the cerebrospinal fluid (CSF)

Answer 9

Memory, emotions, language, hearing and speech. Wernickes area (language comprehension) located here.

Answer 10

Visual reception and interpretation

Answer 11

Reception and processing of information and body orientation

Answer 12

Co ordination and control of voluntary movement

Answer 13

Co ordination and control of voluntary movement

Answer 14

Decision making, problem solving, planning, concentration, judgement, inhibitions, personality, emotional traits. Brocas area is here (language production)

Answer 15

Breathing, digestion, heart control, blood vessel control, alertness

Answer 16

• Confusion, altered level of consciousness, and coma. • Headache • Unilateral weakness or paralysis in the face, arm, or leg. • Sensory loss — paraesthesia or numbness. • Ataxia (Co ordination and balance) • Dysphasia. • Dysarthria. • Visual disturbance — homonymous hemianopia, diplopia. • Gaze paresis — this is often horizontal and unidirectional. • Photophobia. • Dizziness, vertigo, or loss of balance — isolated dizziness is not usually a symptom of TIA. • Nausea and/or vomiting. • Specific cranial nerve deficits such as unilateral tongue weakness or Horner’s syndrome (miosis, ptosis, and facial anhidrosi s). • Difficulty with fine motor coordination and gait. • Neck or facial pain (associated with arterial dissection).

Answer 17

Facial movements: • Ask patient to show teeth, is there an unequal smile or grimace? • Note which side does not move well Arm movements: • Lift the patient’s arms together to 90 degrees if sitting, 45degrees if supine and ask them to hold the position for 5 seconds before letting go, does one arm drift down or fall rapidly? • If one arm drifts down or falls, note whether it is the patient’s left or right Speech: • Listen for new disturbance of speech • Listen for slurred speech, get patient to say “British Constitution or Baby Hippopotamus” • Listen for word-finding difficulties with hesitations. This can be confirmed by asking the patient to name objects that may be nearby such as a cup, chair, table, keys, pen • Check with any person who knows the patient, is this normal for them? Time to ring 999

Answer 18

 Stabilise the patient  ABCDE  abbreviated neurological examination  rapidly obtain a brain image, typically a non-contrast head CT, in order to exclude a brain haemorrhage  The ideal time from emergency department arrival to start of CT is 25 minutes  ECG (AF)  IV Cannula inserted x 2  Serum glucose (rule out hypo)  FBC  Electrolytes  Urea and creatinine and LFTs  Partial thromboplastin time, prothrombin times with international normalised ratio  Cardiac enzymes

Answer 19

Recognition of stroke in the emergency room GCS, BP, BM Syncope -1 Seizure -1 All +1 Asymmetrical face weakness Asymmetrical arm weakness Asymmetrical leg weakness Speech disturbance Visual field defect Previous stroke

Answer 20

NIHSS The NIHSS is an internationally accepted tool for the systematic assessment of stroke severity. It comprises a 15-item neurological examination stroke scale used to evaluate the effect of an acute cerebral event on the levels of consciousness, language, neglect, visual-field loss extra-ocular movement, motor strength, ataxia, dysarthria sensory loss.  Used to assess patients prior to thrombolysis  Assesses the impact of the stroke  Score of less than 4 very goodoutcomeif thrombolysed  Score of 25 or more not suitable for thrombolysis as very poor outcome. It can be used by appropriately trained healthcare professionals and takes approximately 10 minutes to administer.

Answer 21

Posterior Circulation stroke may be difficult to diagnose and should be suspected if the person presents with: Symptoms of acute vestibular syndrome — acute, persistent, continuous vertigo or dizziness with nystagmus, nausea or vomiting, head motion intolerance, new gait unsteadiness.

Answer 22

Ischaemic stroke accounts for 85% of all stroke cases Haemorrhagic stroke for 10% Subarachnoid haemorrhage for 5%

Answer 23

Embolism: an embolus originating somewhere else in the body resulting in hypoperfusion to the area of the brain the vessel supplies. Thrombosis: a blood clot forms locally within a cerebral vessel (e.g. due to atherosclerotic plaque rupture). Systemic hypoperfusion: blood supply to the entire brain is reduced secondary to systemic hypotension (e.g. cardiac arrest). Cerebral venous sinus thrombosis: blood clots form in the veins that drain the brain, resulting in venous congestion and tissue hypoxia.

Answer 24

Thrombolysis

Answer 25

Aged 18> with symptoms of acute stroke Onset withing last 4.5 hours prior to rTPA infusion start Measurable significant new deficit on NIHSS Absence of haemorrhage or stroke mimic based on baseline CT

Answer 26

Absolute Exclusion  Systolic BP >185 and /or diastolic >110 despite treatment  Signs and symptoms of acute intracranial/subacrachnoid haemorrhage  history of previous intracranial haemorrhage  Evidence of active bleeding or known bleeding diathesis  Arterial puncture at non- compressible site within 7days or LP within 7 days  There is history of head trauma or prior stroke in the previous 3 months  intracranial/intraspinal surgery < 3 months  Patients with platelets <100  INR >1.7 on warfarin  dose of low-molecular-weight heparin within 48 hours  Patientistakingdirectthrombin inhibitors or direct factor Xa inhibitors within last 48 hours  Symptoms consistent with infective endocarditis

Answer 27

Relative Exclusion criteria  Pregnancy  Stroke with last 3 months  Major surgery or non-head trauma with last two weeks  Brain tumour , cerebral aneurysm or AVM  Gastrointestinal, urinary or gynaecological haemorrhage within the last 21 days  Recent gastro-intestinal or urinary tract haemorrhage within the previous 3 weeks  Age >80, NIHSS >25 and/or early infarct changes >one third of MCA territory

Answer 28

 Antiplatelet therapy  Clopidogrel 75mg OD  Alternatives : Aspirin 75mg OD and dipyridamole 200mg BD  Combined therapy can be used in severe cases Anticoagulants  Only for those with atrial fibrillation or flutter  Anti-hypertensive  may include a thiazide-like diuretic, long-acting calcium channel blocker or angiotensin-converting enzyme inhibitor.  target systolic blood pressure below 130 mmHg (or 140–150 mmHg in people with severe bilateral carotid artery stenosis)

Answer 29

 Frequent complication after ischaemic stroke  Bleeding into the ischaemic tissue occurs after the stroke, often exacerbated by the thrombotic therapy we have given the patient  Can occur 1-2 days post stroke  If GCS decreases, NIHSS increases or patient deteriorates, we will rescan to check for transformation  Statins may increase the risk of HT in acute stroke, so we wait 48 hours before starting a new statin

Answer 30

CT scan shows Haemorrhagic Stroke  Diagnosis confirms haemorrhagic stroke  Surgery - clip the aneurism  Blood pressure control  frequently elevated at presentation and requires treatment when systolic BP (SBP) is >180 mmHg or the mean arterial pressure (MAP) is >130 mmHg  GTN, Labetalol (IV BB) or nicardipine IV  Antipyretics  Anticonvulsants in those with seizure activity  Phenytoin / levetiracetam  DVT prophylaxis  Keep comfortable and monitor closely Control blood pressure target of 140  Stop any exacerbating drugs  Re–bleeding in 54% of ICH patients on anticoagulants occurs up to 7 days post stroke  Warfarin: may need to normalise INR - fresh frozen plasma or prothrombin complex (e.g.Beriplex) +/- vitamin K

Answer 31

 Warfarin : Prothrombin complex (BERIPLEX) & Vitamin K IV  Dabigatran : Idarucizumab (PRAXBIND)  Apixaban, Edoxaban or Rivaroxaban : PCC & discuss with Haematology or new drugs: andexanet alpha

Answer 32

TIA Management > 7 Days  If the person has had a suspected TIA which occurred more than a weekpreviously:  Refer for specialist assessment as soon as possible within 7 days.  Assess for atrial fibrillation and other arrhythmias

Answer 33

TIA Management < 7 days  Suspected TIA within the last week:  Give aspirin 300 mg immediately (with proton pump inhibitor cover where appropriate)  They are taking low-dose aspirin regularly — continue the current dose of aspirin until reviewed by a specialist.  Aspirin is contraindicated — discuss management urgently with the specialist team. and  arrange urgent assessment (within 24 hours) by a specialist stroke physician unless:  They have a bleeding disorder or are taking an anticoagulant as haemorrhage requires exclusion — arrange immediate admission for urgent assessment and imaging.

Answer 34

 Muscle weakness  Movement, incontinence, pressure sores  Dysphagia  Aspiration pneumonia, malnutrition  CNS  Depression, pain, epilepsy, cerebral oedema  Diabetes  DVT or PE  Hypertension UTI  Visual field defects  Speech and language difficulties

Answer 35

MAJOR  Hypertension  Atrial fibrillation  Smoking TIA  Carotid stenosis >50%  Diabetes Mellitus  High cholesterol  Peripheral arterial disease SECONDARY  Lack of exercise  Unhealthy diet  Obesity  Excessive alcohol consumption  Drugs: HRT, oral Contraceptives, COX-2 inhibitors, atypical neuroleptics (e.g. risperidone)  Sickle cell disease Unfortunately some risk factors can’t be changed:  Previous stroke  Age (although 25% of patients are less than 65yrs)  Gender  Race  Family history

Answer 36

HEADACHE RED FLAGS ▪ A headache of sudden onset, reaching maximum intensity by five minutes - Thunderclap (suggestive of subarachnoid haemorrhage) ▪ Fever with a worsening headache, meningeal irritation and change in mental status (viral/bacterial meningitis) ▪ New-onset focal neurological deficit, personality change or cognitive dysfunction (intracranial haemorrhage/ischaemic stroke/space occupying lesion) ▪ Decreased level of consciousness ▪Head trauma (within the last three months) ▪ Headache which is posture dependent (e.g. worse on lying down and coughing with raised ICP). ▪Headache associated with tenderness in the temporal region (unilateral or bilateral) and jaw claudication (temporal arteritis) ▪ Headache associated with severe eye pain/blurred vision/nausea/vomiting/red eye (acute angle closure glaucoma)

Answer 37

NICE RED FLAGS ▪ New severe/unexpected ▪ Sudden onset (maximum intensity within 5 mins) ▪ Drastic change/progressive/persistent ▪ Precipitating factors ▪ Trauma (<3months ago) ▪ Valsalva manoeuvre (cough/sneeze/bending/exertion) ▪ Worsens on posture changes ▪ Comorbidities ▪ Compromised immunity - ?infection/malignancy ▪ Current or past malignancy – esp. those that metastasize to the brain (melanoma) ▪ Current/recent pregnancy

Answer 38

>30 years old Typical features of a primary headache History of similar episodes No abnormal neurological findings No high-risk co-morbidities No new, concerning history or physical examination No change in headaches habits No red flags

Answer 39

HEADACHE HX ▪ Site ▪ Location - Bilateral/unilateral/symmetrical ▪ Onset ▪ Speed of onset ▪ Aura ▪ Precipitating factors – trauma, fatigue/stress, menstr uation, medication change/withdrawal, foods ▪ Character ▪ Sharp/dull/boring/electrical/pressure ▪ Radiation ▪ Face – trigeminal neuralgia ▪ Eyes – glaucoma ▪ Neck – meningitis ▪ Associated symptoms ▪ Autonomic–tearing,dropping,swolleneyelid,painaround one eye, congestion/rhinorrhea (cluster) ▪ Meningism symptoms – fever, neck stiffness, weakness, rash ▪ Temporal - Jaw claudication, scalp tenderness ▪ Glaucoma - Visual problems, red eyes, halos around lights ▪ RaisedICP - Vomiting/nausea,sleepdisturbance ▪ SOL – neurological deficits, weight loss, visual disturbance ▪ Neurological – fit/fall/LOC/dizziness ▪ Timings ▪ Episodic/daily/unremitting ▪ Duration ▪ Exacerbating/relieving factors ▪ Posture, Valsalva, medication, caffeine ▪ Severity ▪ Out of 10 ▪ Getting better or worse

Answer 40

RED FLAGS • PMHx • including compromised immunity, malignancy, systemic illness , current pregnancy • Previous bleeds • Head trauma • DHx–include what (dose/frequency) they have taken for the headache,response,side effects of medications tried before • Anticoagulants/antiplatelets, glucocorticoids, methamphetamines, cocaine, GTN, COCP • Allergies • SHx • Effects on activities • Avoid activity – migraine • Pacing/restless - cluster • Contacts with similar symptoms (CO poisoning)

Answer 41

HEADACHE EXAMINATION ▪ Basic observations (especially temperature and BP) ▪ GCS ▪ Photophobia ▪ Rash ▪ Eyes - pupils, redness ▪ Feel sinuses for tenderness (move head forward) ▪ Neck stiffness: passively turn head side to side and touch ears to shoulder ▪ Brudzinki’s sign (passive flexion of neck causes involuntary flexion of knee and hip) ▪ Kernig’s sign (pain on passive knee extension with hip fully flexed) ▪ Peripheral neruo exam: tone, power, reflexes, gait ▪ Cranial nerve exam ▪ Visual fields ▪ Fundoscopy to look for papilloedema (↑ICP) or haemorrhages (SAH)

Answer 42

Not commonly needed for 1o headaches (clinical dx) ▪ If diagnosis unclear BUT red flags have been ruled out – can use headache diary ▪ If concerned about a 2o/serious headache, refer to hospital urgently (dependent on clinical picture)

Answer 43

• 2S- Systemic symptoms (fever, weight loss, fatigue). Secondary risk factors (HIV, cancer (brain mets), immunosuppressed). • N -Neurologic symptoms/ signs. Pins and needles ect • O- Onset- Thunderclap, abrupt. • O- Older-New in someone 50+. • P- Positional (upright relieves, neck position relieves), Prior Headache (different to previous), Papilloedema

Answer 44

PRIMARY HEADACHES ▪ Generally chronic and episodic in nature ▪ Primary headaches do not have another cause/disorder ▪Primary Headaches ▪ Classic migraine 1A ▪ Atypical/common migraine 2B ▪ Tension headache 2A ▪ Cluster headache 2A ▪ Trigeminal neuralgia* ▪‘Benign’secondary headaches ▪ Medication overuse headache ▪ Sinusitis 1A/B

Answer 45

TENSION (STRESS) HEADACHE ▪ Age – usually 20-50 ▪ Location – usually bilateral and symmetrical – frontal/occipital ▪ Severity – mild-moderate (classically worsen during the day) ▪ Duration – 30mins – 7 days ▪ Quality – pressure, tightness, band-like - NOT pulsatile ▪ Associated symptoms – none ▪ Does not worsen with exercise ▪ Photo/phonophobia/nausea can occur (no more than 1) ▪ Risk factors ▪ Mental tension, stress, fatigue, missing meals ▪ Sternocleidomastoid, trapezius, temporalis commonly tender

Answer 46

TENSION HEADACHE MANAGEMENT ▪ Simple analgesia is effective – NSAIDs/paracetamol/ aspirin ▪ Opioids should never be used – if required revisit diagnosis ▪ If chronic (7-9 “headache days” per month) ▪ Prophylactic low does tricyclic antidepressants (amitriptyline – off label) ▪ If no response discontinue + refer to neurology ▪ If response – attempt withdrawal after 4-6 months ▪ Acupuncture ▪ Relaxation therapy ▪ Patient information – NHS website ▪ Advise the risk of medication overuse headache

Answer 47

MEDICATION OVERUSE HEADACHE ▪ “analgesic rebound” ▪ Due to regular overuse for >3/12 of drugs used to treat headaches ▪ NSAIDs/paracetamol – if taken >15 days a month ▪ Opioids/ergotamine/triptans – if taken >10 days a month ▪ The headache must be present for >15 days a month in a patient with a pre-existing headache disorder ▪ Treatment – medication withdrawal Common agents: ▪ Analgesics ▪ Birth control – usually during inactive days (drop in oestrogen) ▪ Nitrates ▪ CCB ▪ Digoxin ▪ Corticosteroids ▪ HRT ▪ Alcohol/caffeine/barbiturates/narcotics ▪ Withdrawal too

Answer 48

MIGRAINE ▪ Age – 10-40 ▪ Location - usually unilateral (can become bilateral later) ▪ Severity - moderate-severe ▪ Duration - 4hrs-3days ▪ Quality – throbbing, pounding/pulsating ▪ Associated symptoms – prodromal symptoms (30-75% - irritable, cravings, yawning) aura (25%), during headache (N+V, photophobia, phonophobia, allodynia) ▪ Typical migraine = aura (also known as ‘classic’migraine) ▪ Can diagnose after 2 attacks ▪ Atypical migraine = no aura (also known as common migraine) ▪ Can diagnose after 5 attacks ▪ Patients may confuse a migraine headache for sinusitis

Answer 49

Prodrome: Problems concentrating, fatigue, cravings, muscle weakness, nausea, sensitivity to light Aura: ▪ Can last 5mins - 1hr before migraine onset ▪ Fully reversable ▪ Progress gradually over 5 mins ▪ Can vary a huge amount from patient to patient ▪ Visual (usually binocular) – most common ▪ Photopsia (flashes) ▪ Fortification spectra (jagged lines) ▪ Scintillating scotoma (shimmering oddly shaped area of visual deficit) ▪ Hallucinations ▪ Vertigo ▪ Peripheral sensory deficits (e.g. paresthesia) ▪ Focal loss of motor function Headache: Sensitivity to light, odors and noise Nausea, vomiting, GI discomfort Loss of appetite Sweating, chills Dizziness and blurred vision Postdrome ‘Migraine hangover’ Fatigue and depression

Answer 50

▪ Lifestyle ▪ Headache diary – identify triggers ▪Stress management,sleep hygiene, hydration, regular meals, exercise, healthy BMI ▪ Optimisation of other conditions – sleep apnea/insomnia/depression/anxiety ▪ Acute attack ▪ NSAID/paracetamol + triptan (sumatriptan) ▪ Can use monotherapy ▪ Triptans can be oral, nasal or sub cut – contraindicated in cv disease/elderly ▪ Antiemetics (prochlorperazine/metoclopramide*) – even in absence of N+V ▪ Avoid opioids ▪ Acute medication should be taken early while pain is mild. ▪ If they have aura, triptans should be taken at the start of the headache and not at the star t of the aura (unless the aura and ▪ Contraindications ▪ COCP in typical migraines is absolutely contraindicated

Answer 51

MIGRAINE PREVENTION ▪ Aim to reduce the frequency, severity, and duration of migraine attacks, and avoid medication-overuse headache ▪ NOT to cure migraines ▪ Consider if: ▪ Significant impact on quality of life/ADL – frequent/prolonged/severe ▪ Acute management ineffective/contraindicated ▪ Medication overuse headache ruled out ▪ Medications ▪ Propranolol or, ▪ Topiramate (contraindicated in pregnancy — highly effective contraception is required prior to initiation) or, ▪ Amitriptyline (25–75 mg at night) ▪ Start low and titrate – divided doses ▪ Review regularly during titration ▪ May take 4-8 weeks to see benefit ▪ Non-pharmacological ▪ Relaxation therapy/CBT ▪ Acupuncture ▪ Riboflavin 400mg OD vitamin B2

Answer 52

HEMIPLEGIC MIGRAINE ▪ Can mimic stroke ▪ Symptoms can vary ▪ Typical migraine symptoms ▪ Sudden or gradual onset ▪ HEMIPLEGIA ( unilateral weakness of the limbs) ▪ ATAXIA ▪ Changes in consciousness ▪ Analgesic Motor aura - temporary one sided weakness Sensory aura - numbness or pins and needles Visual aura - flashing lights, zigzag lines, visual field loss Aphasia aura - word formation and comprehension affected

Answer 53

HEMICRANIA CONTINUA 3 month headache Similar presentation to migraine although lasting >3months Criteria • All of the following: • 1) unilateral pain • Daily/continuous • Moderate severity with exacerbations • Plus at least one of the following: (ipsilateral) • Conjunctival injection/lacrimation • Nasal congestion/rhinorrhea • Ptosis/miosis Usually completely responsive to indomethacin (NSAID)

Answer 54

“Suicidal headache” CLUSTER HEADACHE ▪ Age – 20-40 ▪ Location – unilateral, peri-orbital pain ▪Severity -severe ▪ Duration – 15-180mins ▪ Quality – sharp, pulsating, boring, burning ▪ Associated symptoms – ipsilateral cranial autonomic symptoms (conjunctival injection, lacrimation, eyelid oedema) ▪ The pain characteristically makes people restless/agitated – they pace ▪ Timings of the attacks are predictable – same time of night/day ▪ Typically 1-2 hours after falling asleep ▪ May have triggers ▪ Alcohol, histamine, exercise, sleep, volatile smells (petrol/perfume)

Answer 55

CLUSTER HEADACHE ▪ Rare ▪ Men > Women ▪ Linked to previous head trauma and smoking ▪ Episodic (80-90%) ▪ Attacks occur in periods typically between 2 weeks and 3 months ▪ Can occur in periods of 7 days to one year ▪ Attacks separated by pain-free periods of at least 1 month ▪ Chronic (10-20%) ▪ Attacks occur for more than one year without remission (or remission lasts less than 1 month) ▪ Can present initially or convert from episodic (10%) ▪ 30% convert to episodic

Answer 56

CLUSTER MANAGEMENT ▪ First presentation ▪ Urgent referral/discuss with neurologist/specialist GP ▪ Requires specialist diagnosis (may need neuroimaging) ▪ Acute ▪ Subcutaneous triptan ▪ 1 injection 6mg sumatriptan ▪ Can have second injection after 1 hour (unless no response) ▪ Maximum 12mg/day ▪ Nasal triptan ▪ 1 nostril 10-20mg sumatriptan ▪ Can have second dose after 2 hours (unless no response) ▪ Maximum 40mg/day ▪ Oxygen therapy ▪ 100% 15L non-rebreather for 15-20mins ▪ Preventative ▪ Verapamil 40mg/day (up to 960mg) – ECG monitoring >120mg/every increase(AV block) ▪ Prednisolone (60-100mg) – short periods (2-3 weeks) - wean off after 2-5 days ▪ Do not use paracetamol/NSAIDs/opioids

Answer 57

TRIGEMINAL NEURALGIA ▪ Not a true primary headache – “painful cranial neuropathy’ ▪ Disorder of 5th cranial nerve (trigeminal) ▪ Most commonly maxillary/mandibular branches involved ▪ Chronic, debilitating ▪ Intense and extreme electric shock pain – unilateral ▪ Lasts seconds to minutes – can have hundreds of attacks per day ▪ Can have remission for weeks/months – shortens gradually shorten between episodes ▪ Can be triggered by – light touch, eating, cold wind, vibrations, brushing teeth ▪ Refer to specialist ▪ May require an MRI to rule out other causes

Answer 58

TRIGEMINAL NEURALGIA MANAGEMENT ▪ Medical ▪ Carbamazepine 1st line ▪ Withdraw gradually after 1 month of remission ▪ Gabapentin ▪ Botox ▪Surgery ▪ Sever trigeminal nerve root ▪ Microvascular decompression

Answer 59

SINUS HEADACHES ▪ Headache due to inflammation of the mucosal lining of the nasal cavity and paranasal sinuses ▪ Tender to palpation over sinuses when leaning head forward ▪ Can be viral or bacterial ▪ Usually 2o to URTI (viral) ▪ If symptoms last >10 days think bacterial infection ▪ Purulent nasal discharge, nasal obstruction, dental pain, or facial pain/pressure/headache are more common with acute bacterial sinusitis

Answer 60

SINUSITIS MANAGEMENT ▪ Viral is self-limiting (usually within 10 days) ▪ NSAIDs/paracetamol/codeine ▪ Decongestants ▪ Nasal corticosteroids ▪ Bacterial ▪ Symptomatic treatment ▪ Watchful waiting (up to 10 days unless immunocompromised) ▪ Antibiotics can be used to shorten symptoms (penicillin / Co amox) ▪ Or if severe/worsening symptoms

Answer 61

• Subarachnoid Haemorrhage 1A • Acute Bacterial Meningitis 1A • TIA/Stroke 1A • Raised intracranial pressure 1B • Accelerated hypertension 1B • Acute Angle Closure Glaucoma 1B • Hypotension 1B • Temporal Arteritis 2A • Pre-eclampsia 2A • Viral Encephalitis 2B • Venous sinus thrombosis 2B • Cavernous sinus thrombosis 2B • Internal carotid dissection 2B • Space occupying lesion • Post-lumbar puncture headache

Answer 62

Subarachnoid haemorrhage • Bleeding into subarachnoid space • 85% caused by aneurysms (non-traumatic) • An emergency–need to identify early • Signs/symptoms • Thunderclap occipital headache • Feels like you’ve been kicked in the back of the head • Worst headache ever • reaching maximum intensity 30-60 seconds- • Vomiting • Neck stiffness • Decreased LOC • Seizures • 30-50% of people have Sentinel headache (warning flags - mini aneurisms) in the days/weeks before (commonly 2 weeks)

Answer 63

Subarachnoid haemorrhage • Polycystic kidney disease increased risk of berry aneurysms too

Answer 64

Risk factors: • Hypertension • Smoking • Alcohol • FHx (3-5x risk) • Genetic conditions (PKD, Ehlers Danlos, sickle cell, Marfan) • Cocaine • Ethnicity (Black) • Age 45-70 • Female • Antiplatelets/anticoagulants increase severity of bleed (not the risk of bleeding in the 1s t place)

Answer 65

SAH Diagnosis • FBC, U+E, Clotting • CT-Head • hyperdense (white) in the basal cisterns, major fissures, and sulci • CT scan- If within 6 hours, 100% sensitivity, 93% at 24 hours, 80% 3 days • Lumbar puncture – 12hrs after symptom onset • If bleeding not seen on CT but still suspicion of SAH • If patient doesn’t present until 24hrs after symptom onset • Look for xanthochromia (faint, yellow tinge) • Angiography (CT/MRI) • Once confirmed to locate source of bleeding

Answer 66

SAH management • Medical • Nimodipine – prevent cerebral ischemia due to vasospasm • Analgesia • Antiemetics • Anticonvulsants • Surgical • Further bleeding biggest risk –occlude the aneurysm • Coiling – endovascular titanium coils inserted into aneurysm • Clipping – direct neurosurgery • Risk of hydrocephalus in the hours/days post SAH is high • Disruption of drainage of CSF • Treat with LP or shunt • Mortality is high - 50% • 25% die before reaching hospital • 1/3 of survivors are dependent for care • 1⁄2 have cognitive impairment

Answer 67

Meningitis • Inflammation of the meninges • Usually due to infection – viral/bacterial/fungal • Can be due to injury/cancer/drugs • Viral • Most common cause of ”aseptic meningitis” • Usually due to enterovirus • Can lead to viral encephalopathy (infection of brain tissue) – new onset confusion • Bacterial • Neisseria meningitidis, Strep Pneumoniae, HiB • Medical emergency • Usually affects extremes of age (<2months and >60years) • Fungal • Progressive, life-threatening • Chronic/subacute • Most commonly caused by cryptococcus

Answer 68

Meningitis • Presenting symptoms- Headache, Neck stiffness, fever, photophobia, confusion, rash. • Also- sore throat, muscle pain, nausea. • Possible Positive examination findings- • Cranial nerve abnormalities • Petechiae/ purpura (neisseria meningitidis) rash

Answer 69

Meningitis Tests/ Investigations • Observations- High temp, sepsis signs • Bloods- Raised WCC, CRP • Lumbar Puncture- • Viral most common • CT scan (NOT required prior to LP unless concerns re: raised ICP/ GCS <12)

Answer 70

Bacterial meningitis antibiotics choice • If in the community • Only give benzylpenicillin if rash is present – priority is transfer to hospital • Start empirical broad-spectrum antibiotics then change based on MC&S • Age≤1month immuno competent: • cefotaxime or ceftriaxone PLUS ampicillin • Between >1month and <50years immuno competent: • cefotaxime or ceftriaxone PLUS vancomycin • Age ≥ 50 years or immuno compromised: • ampicillin PLUS cefotaxime or ceftriaxone PLUS vancomycin. ** If >50, immunocompromised or pregnant – consider Listeria, add Amoxicillin **

Answer 71

Raised ICP • ICP should be between 7-15mmHg Many causes • Mass effect – tumour/haemorrhage/oedema/abscess • Brain swelling (encephalitis) • Venous sinus thrombosis • Obstruction of CSF flow/absorption • Increased CSF production • Idiopathic – common

Answer 72

Raised ICP- signs and symptoms • Headache • Postural changes (lyingdown) • Valsalva/coughing/sneezing will make it worse • Vomiting w/o nausea • Ocular palsies • Altered LOC • Back pain • Papilloedema

Answer 73

Space occupying lesion Secondary headache • SOL • Tumours • Haematoma • Abscess • Signs and symptoms • New onset headache (especially if >50) • Progressive or persistent headache • Dramatic change in headache • Postural changes – headache worse on lying down • New onset neurological changes • Personality, cognition • Papilloedema

Answer 74

Idiopathic intercranial hypertension • IIH may be due to reduced CSF absorption • Predominantly affects obese women of childbearing age • Female overweight, pregnancy • Signs and symptoms • Headache • Neck/back pain • Visual symptoms • 6th CN palsy • Visual field loss – enlargement of blind spot/inferonasal loss • Diplopia • Due to optic nerve atrophy • Papilloedema • Diagnosis • Diagnosis of exclusion • MRI venography (with contrast) & CT • LP - >25mmHg

Answer 75

IIH management • Treatment consists of first eliminating “causal factors” • Drugs • tetracycline, cyclosporine, lithium, nalidixic acid, nitrofurantoin, oral contraceptives, levonorgestrel, danaxol, and tamoxifen • Endocrine abnormalities • corticosteroid withdrawal, anabolic steroids, excessive growth hormone, and thyroid disease • Systemic conditions • pregnancy, menstrual irregularities, polycystic ovarian syndrome, anaemia, and obstructive sleep apnoea • Lifestyle- low-sodium diet. weight-reduction. • Acetazolamide if visual symptoms. • Serial lumbar punctures.

Answer 76

Accelerated Hypertension • Defined as rapid and sudden increased BP • if left untreated can cause end-organ damage • If papilloedema/ end organ damage present – • Hypertensive emergency (‘malignant hypertension’) • Can get hypertensive encephalopathy – neurological symptoms • Refer patients with BP of 180/120 with: • Retinal hemorrhages/papilloedema • Life threatening symptoms – new onset confusion, chest pain, AKI, signs of heart failure • Investigations • Routine bloods • Urinalysis • ECG • CXR • Management • Need to lower BP slowly (over 24hrs) to prevent cerebral infarction (watershed areas) – no more than 25%/24hrs at first • Labetalol 1st line

Answer 77

Acute Angle Closure Glaucoma • Due to reduced drainage of aqueous humor in eye • Leads to accumulation of aqueous humor and raised intraocularpressure–can lead to optic nerve atrophy • Signs and symptoms • Sudden severe pain in/around one eye • Conjunctival injections • Hazy cornea • Dilated pupil • Blurred/reduced vision – haloes seen around lights • N+V • Cupping on fundoscopy • Medical emergency– if suspected send to A&E • Can cause loss of vision

Answer 78

Risk factors • Women • Ethnicity – Inuit,Asian • Far sightedness (hyperopia) • Steroids • Diabetes • Diagnosis – ophthalmologist (Slit lamp, Tonometry, Gonioscope) • Treatment • Acetazolamide (IV/oral) - pressure reducing agent • Topical beta blockers • Miotics - constrict pupil and open angles

Answer 79

Temporal (Giant Cell) Arteritis • Form of vasculitis commonly causing inflamed temporal arteries (however can affect aorta, external carotids) • Affects mostly women+ >50years old • 10% have polymyalgia rheumatica (pain/stiffness/inflammation around axial joints – shoulders/neck/hip) • Signs and symptoms • Frequent severe bilateral headaches • Temporal/occipital pain/tenderness • Scalp tenderness • Jaw claudication • Visual disturbances – diplopia, loss of vision • Fatigue, myalgia • Medical emergency – irreversible blindness can occur

Answer 80

GCA Diagnosis • Examination • Look for palpable, non-pulsatile arteries of the head and face • Bloods • ESR >80 (although 20% have normal ESR) • Male ESR’s

Answer 81

GCA management • Steroids • High dose – 60mg/day prednisolone • Should see improvement in 72hrs • Once GCA resolved taper off (usually 4 weeks) • Bone protection + GI protection • Rheumatology care

Answer 82

• Headaches are common in pregnancy – 90% are migraine/tension • Usually improve in last 6/12 of pregnancy • However, rarer causes of headaches are life threatening • Cerebral venous sinus thrombosis • Blood clot in dural venous sinuses • Prevents venous return • Causes headache/abnormal vision/neurological symptoms • Treat with anticoagulant • Pre-eclampsia (20+ weeks) • HighBP+proteinintheurine • Can lead to eclampsia (seizures) • Medical emergency – need to reduce BP

Answer 83

Head and Neck trauma • Injuries to the head may cause: • bleeding in the spaces between the meninges or within the brain tissue itself - Intracerebral haemorrhage • Other causes in this category include • Oedema or swelling of the brain, not associated with bleeding, may cause pain and a change in mental function • Concussion where head injury occurs without bleeding (headache is one of the hallmarks of post-concussion syndrome) • Whiplash or neck Injury can cause head pain • Post-craniotomy surgery headache • Extra/Epidural haematoma • Young, high-impact, lucid interval • Lemon-shaped CT (lentiform) • Subdural • Older/alcoholic, confused/LOC • banana-shaped CT (crescentic)

Answer 84

Cauda equina syndrome is a surgical emergency where the nerve roots of the cauda equina at the bottom of the spine are compressed. It requires emergency decompression surgery to prevent permanent neurological dysfunction. However, even with immediate decompression, patients may still not regain full function.

Answer 85

Pathophysiology The cauda equina (translated as “horse’s tail”) is a collection of nerve roots that travel through the spinal canal after the spinal cord terminates around L2/L3. The spinal cord tapers down at the end in a section called the conus medullaris. The nerve roots exit either side of the spinal column at their vertebral level (L3, L4, L5, S1, S2, S3, S4, S5 and Co). The nerves of the cauda equina supply: • Sensation to the lower limbs, perineum, bladder and rectum • Motor innervation to the lower limbs and the anal and urethral sphincters • Parasympathetic innervation of the bladder and rectum In cauda equina syndrome, the nerves of the cauda equina are compressed. There are several possible causes of compression, including: ○ Herniated disc (the most common cause) ○ Tumours, particularly metastasis ○ Spondylolisthesis (anterior displacement of a vertebra out of line with the one below) ○ Abscess (infection) ○ Trauma

Answer 86

Red Flags The key red flags to look out for are: • Saddle anaesthesia (loss of sensation in the perineum – around the genitals and anus) • Loss of sensation in the bladder and rectum (not knowing when they are full) • Urinary retention or incontinence • Faecal incontinence • Bilateral sciatica • Bilateral or severe motor weakness in the legs • Reduced anal tone on PR examination TOM TIP: A common way people ask about saddle anaesthesia when taking a history is to ask, “does it feel normal when you wipe after opening your bowels?”

Answer 87

Management Cauda equina is a neurosurgical emergency. It requires: • Immediate hospital admission • Emergency MRI scan to confirm or exclude cauda equina syndrome • Neurosurgical input to consider lumbar decompression surgery Surgery should be performed as soon as possible to increase the chances of regaining function. Even with early surgery, patients can be left with bladder, bowel or sexual dysfunction. Leg weakness and sensory impairment can also persist.

Answer 88

Cauda equina presents with lower motor neuron signs (reduced tone and reduced reflexes). The nerves being compressed are lower motor neurons that have already exited the spinal cord. When the spinal cord is being compressed higher up by metastatic spinal cord compression, upper motor neuron signs (increased tone, brisk reflexes and upping plantar responses) will be seen.

Answer 89

Dura matter Arachnoid Pia matter

Answer 90

Buoyancy - so neurones aren’t in direct contact with the skull Protection Produced in the sub arachnoid meninges

Answer 91

Below L2/3 In-line with the iliac crest Performed this low so it doesn’t damage the spinal chord as anything below L3 is the chorda equina (spinal nerves) rather than the spinal cord

Answer 92

• Post-LP headache (lowpressure) • Local pain • Bleeding/haematoma • Infection • Nerve damage (extremely rare) Herniation of the cerebellum (brain stem) down the spinal cord

Answer 93

• Opening Pressure: 12- 20cm H2O – Must be in supine position (lying down) – Elevated if tense and anxious – higher if overweight (up to 25 cm H2O) • Appearance: Clear and colourless • Microscopy: – WCC: <5 x109/L (and percentage differentiation: polymorphonuclear (PMN) cells e.g. neutrophils, lymphocytes) – RBCs (raised in traumatic tap or SAH) • Protein Count: <0.5 g/L • Glucose (>2/3rds of serum glucose) • Xanthochromia (Bilirubin from heme breakdown) • Oligoclonal bands • Serum glucose and oligoclonal bands • Others: Viral/bacterial PCR (HSV, VZV, Enteroviruses, EBV, CMV, Menningococcal), Cytology, Lactate

Answer 94

Meningitis (viral, bacterial, fungal, TB) Encephalitis (viral)

Answer 95

Neisseria meningitidis (meningococcal) - uni students Streptococcus pneumoniae, or pneumococcus - ear infection, very young and very old patients Listeria - neonatal and pregnant women, immuno compromised, old Fact - increases lymphocytes more so than neutrophils

Answer 96

Anything above 20 H20 Infection Idiopathic intercranial hypertension Venous sinus thrombosis Space occupying lesions ie bleed, tumour, abscess

Answer 97

Neutrophils - bacterial Lymphocytes - viral

Answer 98

Infection (usually bacterial) MS Gullien Barres

Answer 99

Subarachnoid haemorrhage Xanthochromia caused from Bilirubin from heme breakdown LP should be done 12 hours after subarachnoid haemorrhage

Answer 100

MS most commonly Sarcoidosis SLE

Answer 101

Enteroviruses Self limiting

Answer 102

Viral/bacterial PCR HSV - viral encephalitis Enteroviruses - viral meningitis Menningococcal / pneumococcus - bacterial meningitis

Answer 103

– Raised intra-cranial pressure: • Space occupying lesion: tumour, aneurysm, bleed, abscess • Venous sinus thrombosis • Benign intracranial hypertension – Hypercapnia (secondary to type II RF) – Optic neuritis

Answer 104

MR rules out Venus sinus thrombosis LP shows raised intercranial pressure No other significant microbiology (everything normal other than pressure) Diagnosis is idiopathic raised inter cranial pressure

Answer 105

Symptoms: Long lasting headache Causes patient to wake up at night and relieve by sitting up Causes/Associations: - Obesity - Venous sinus thrombosis - Drugs: Tetracycline, Minocycline, Nitrofurantoin, Vitamin A, Isotretinoin Investigations/Diagnosis - MRI (rule out other causes ie space occupying lesion) - Lumbar puncture: Raised opening pressure only

Answer 106

Treatment - Weight loss/manage other contributing factors - Therapeutic lumbar puncture (bring pressure below 20mmHg) - Drugs: Acetazolamide (carbonic anhydrase inhibitor - stops the conversion of carbonic acid to co2 and water), Topiramate; loop diuretics, Prednisolone - Surgery (if above fail or vision worsens): Lumbar- peritoneal shunt (moves CFS from lumbar region to peritoneal cavity), optic nerve sheath fenestration Complications - Visual loss - Reduced quality of life

Answer 107

Eyes 3/4 Movement 6/6 Speech 4/4 Viral encephalitis

Answer 108

Viral encephalitis: WCC - shows viral Protein - high HSV positive

Answer 109

Viral meningitis - conservative management, self limiting Viral encephalitis- unwell patient, reduced GCS, confused, talking weirdly, potential seizures

Answer 110

Aciclovir (anti viral) Specific to HSV virus

Answer 111

Infection of brain parencyhma (cortex, white matter, basal ganglia, brainstem)

Answer 112

Epidemiology/Causes • Herpes simplex (HSV) 1: commonest in Europe- predominantly effects temporal lobes • Herpes simplex 2 usually in immunosuppressed and neonates • CMV, EBV, VZV, HIV (during seroconversion), measles, mumps, rabies, tick- borne, Arbovirus (mosquitos and ticks) • Japanese B encephalitis (East Asia and Australia) • Non-viral: bacterial meningitis, TB, malaria, listeria, Lyme disease, legionella, leptospirosis, aspergillosis

Answer 113

Presentation • Headache • Fever • Focal neurological signs (hemiplegia, aphasia etc.) • Seizures • Reduced consciousness and confusion • Meninngism Acting very weird, not speaking sense and not their usual self

Answer 114

Investigations: • Treat before investigations if suspect • Brain imaging: – Temporal lobe petechiae (small haemorrhages) – MRI more sensitive than CTBS • Lumbar Puncture: – Raised WCC (predominantly lymphocytes, but PMN can predominate- especially in early stages) – Protein may be elevated and glucose usually normal,or mildly low – Send CSF for viral PCR (HSV,VZV,EBV,CMV) • EEG= Periodic slow wave activity Management – Aciclovir- IV – Steroids (Dexamethasone) if raised intracranial pressure – Supportive: anti-convulsants, respiratory support

Answer 115

Septic patient: fluid bolus, blood cultures, urine culture, empirical Abx, critical care involvement, monitor u/o, lactate etc. LP, CT Diagnosis - bacterial meningitis

Answer 116

Bacterial meningitis Pressure high Protein high Glucose low WCC high with neuts

Answer 117

Ceftriaxone - penetrates the blood brain barrier more than amoxicillin Prednisolone - reduced inflammation in the brain

Answer 118

Neisseria meningitis (meningococcal) Gram negative diplococci Streptococcus pneumoniae Gram positive diplococci Group B streptococcus Gram positive cocci in chains New borns Listeria monocytogenes Gram positive rods (bacillus) Pregnancy, <3months, >60years, immunosuppressed Contaminated food

Answer 119

Lower motor neurone lesion Guillian Barre LP very high protein, everything else normal

Answer 120

Incidence/Aetiology • 1-2/100,000 every yr • Usually some preceding trigger (molecular mimircy): infection or immunization within the past few weeks • Recognised infection include: Campylobacter jejuni, CMV, mycoplasma, HZV, HIV (seroconversion), EBV

Answer 121

Presentation • Ascending symmetrical, predominantly motor polyneuropathy • Pain can occur but sensory symptoms less common • Autonomic dysfunction: Sweating, tachycardia, arrythmias, BP changes • Lower motor neurone signs (areflexia, fasiculations, hypotonia) • Ascending from feet→legs→abdo→chest and arms→face) • Progressive up to 4 weeks then recovery • Miller Fisher Variant: Ophthalmoplegia, ataxia and areflexia (affects cranial nerves not peripheral)

Answer 122

Investigations GBS • CSF Analysis: Raised protein count • Nerve Conduction Studies (assess peripheral nerves) : Reduced velocity (de-myelination) • Respiratory Function: FVC/ABG/1 breath count • Anti-ganglioside auto-antibodies (positive in some cases) • ?Investigate Cause: Stool MC+S, Campylobacter/mycoplasma serology, CXR Management • IV Immunoglobulins (5 days) • Plasma exchange • Monitor FVC, assess swallowing • Supportive (if needed): e.g. respiratory support, NBM and NG feed etc. • Physio/OT Advise it will get worse before getting better Lasts different amount of time for everyone

Answer 123

Subarachnoid haemorrhage

Answer 124

Subarachnoid Haemorrhage (SAH) Presentation • Thunderclap occipital headache • Loss of consciousness • Only 1 in 8 with sudden onset severe headache have SAH • Symptoms of meningeal irritation • Focal neurology: Hemiparesis, aphasia, 3rd nerve palsy (compression due to posterior communicating aneurysm), hemiparesis, aphasia

Answer 125

Causes • 85% due to saccular aneurysm, usually in region of circle of Willis • Others due to non-aneurysm haemorrhages, arterio- venous malformations, vertebral artery dissection

Answer 126

Investigations • CTBS • Lumbar Puncture: xanthochromia, 12 hours after onset of headache • MRI arteriogram Management/Prognosis • Mortality of aneurysmal haemorrhage= 30% • Re-bleed rate of 3% annually • Platinum coils into aneurysm or surgical slipping reduces risk of recurrence • Supportive: resp. support, anti-convulsants etc.

Answer 127

B- idiopathic intracranial headache- pressure related headache (worse lying down)

Answer 128

To confirm a clinical diagnosis To rule out something serious and confirm benign diagnosis ie migraine To aid prognosis/ guide treatment VOMIT Victim of modern imaging technology Coincidental findings that aren’t know to cause problems BARF Brainless application of radiologic findings Putting the wrong cause to the symptoms - leads to wrong treatment

Answer 129

CNS Motor cortex White matter tracts Brain stem Spinal cord PNS Anterior horn cell Nerve root Motor nerve (axon/myelin) Neuromuscular junction Muscle

Answer 130

Upper motor neurone lesion - Cervical spine MRI

Answer 131

Left sided middle cerebral artery stroke Urgent CT

Answer 132

Haemorrhage CT scan - very white bleed on the brain

Answer 133

Upper motor neurone signs Central nervous system MS - demyelination of the CNS Confirm with MRI

Answer 134

SPECT Gamma emitting radioisotope Cheap Convenient Poor spatial resolution Nokia quality Eg can diagnose Alzheimer’s but not specific the subtype PET Positron emitting radioisotopes Expensive More effort Good spatial resolution iPhone quality Eg can differentiate types of Alzheimer’s disease, neurotransmitters, pathological proteins (amyloid in Alzheimer’s disease), neuro inflammation

Answer 135

A. Eaton Lambert Syndrome Effects the calcium channels of the neuromuscular junction

Answer 136

• Relapse (episodes of crises) and remitting • Symptoms better in morning and worse later, tend to start in eyes • Ocular (ptosis and diplopia)→Bulbar (dysphagia, dysphonia, dysarthria)→Facial (droopy mouth, less forehead lines)→Limb, Trunk, Respiratory Weakness • Demonstrating fatigability clinically: – look up and observe for worsening ptosis (Simpson’s test) – Repeatedly flap arm for a minute, then test power – Reflexes and sensation usually intact – Muscle wasting can occur if prolonged inactivity

Answer 137

Investigations Myasthenia Gravis • TENSILON test- Edrophonium (acetylcholinesterase inhibitor) + Atropine (may be needed if bradycardia/hypotension)- improvement in symptoms within seconds • Acetylcholine receptor antibodies • EMG- Assess fatigability • Imaging of thymus (CT chest) as it causes enlarged thymus • Spirometry to assess respiratory muscle involvement (FVC or single breath counting)

Answer 138

Management • Pyridostigmine (acetylcholinesterase inhibitor) • Steroids and immunosuppressives (azathioprine, methotrexate) • Thymectomy if hyperplasia of thyoma→better response to treatment and sometimes permanent remission

Answer 139

Lambert-Eaton Myasthenic Syndrome • Antibodies to pre-synaptic voltage gated Calcium channels in the NMJ • Associated with antibodies to voltage-gated calcium channels- especially P/Q type • Weakness in arms and legs but gets better with exercise • Considered a paraneoplastic syndrome as 60% have associated malignancy (commonest= small cell lung cancer)

Answer 140

• Ptosis (levator palpebrae superioris) • Mydriasis (parasympathetic supply via oculomotor nerve→short cilliary nerves) • Eye= down and out (unopposed effect of lateral rectus and inf. oblique) • Enophthalmos Causes- Diabetes, atherosclerosis, posterior communicating artery aneurysm, space occupying lesion, brain infection/inflammation, cavernous sinus thrombosis, multiple sclerosis

Answer 141

There is a classic triad of features in Parkinson’s disease: • Resting tremor (a tremor that is worse at rest) • Rigidity (resisting passive movement) • Bradykinesia (slowness of movement)

Answer 142

Pathophysiology The basal ganglia are a group of structures situated near the centre of the brain. They are responsible for coordinating habitual movements such as walking, controlling voluntary movements and learning specific movement patterns. Dopamine plays an essential role in the basal ganglia function. Patients with Parkinson’s disease have a slow but progressive drop in dopamine production.

Answer 143

Features Tremor in Parkinsons is worse on one side and has a 4-6 hertz frequency, meaning it cycles 4-6 times per second. It is described as a “pill-rolling tremor” due to the appearance of rolling a pill between their fingertips and thumb. It is more noticeable when resting and improves on voluntary movement. It gets worse when the patient is distracted. Performing a task with the other hand (e.g., miming the act of painting a fence) exaggerates the tremor. Rigidity is resistance to the passive movement of a joint. Taking a hand and passively flexing and extending the arm at the elbow demonstrates tension in the arm that gives way to movement in small increments (like little jerks). The jerking resistance to movement is described as “cogwheel” rigidity. Bradykinesia describes the movements getting slower and smaller and presents in several ways: • Handwriting gets smaller and smaller (micrographia) • Small steps when walking (“shuffling” gait) • Rapid frequency of steps to compensate for the small steps and avoid falling (“festinating” gait) • Difficulty initiating movement (e.g., going from standing still to walking) • Difficulty in turning around when standing and having to take lots of little steps to turn • Reduced facial movements and facial expressions (hypomimia) Other features include: ○ Depression ○ Sleep disturbance and insomnia ○ Loss of the sense of smell (anosmia) ○ Postural instability (increasing the risk of falls) ○ Cognitive impairment and memory problems

Answer 144

Parkinson’s Tremor Benign Essential Tremor Asymmetrical Symmetrical 4-6 hertz 6-12 hertz Worse at rest Improves at rest Improves with Worse with intentional movement intentional movement Other No other Parkinson’s features Parkinson’s features No change with alcohol Improves with alcohol

Answer 145

Multiple system atrophy is a rare condition where the neurones of various systems in the brain degenerate, including the basal ganglia. The degeneration of the basal ganglia leads to a Parkinson’s presentation. Degeneration in other areas leads to autonomic dysfunction (causing postural hypotension, constipation, abnormal sweating and sexual dysfunction) and cerebellar dysfunction (causing ataxia).

Answer 146

Dementia with Lewy bodies is a type of dementia associated with features of Parkinsonism. It causes a progressive cognitive decline. There are associated symptoms of visual hallucinations, delusions, REM sleep disorders and fluctuating consciousness.

Answer 147

Multiple system atrophy Dementia with Lewy bodies Progressive supranuclear palsy Corticobasal degeneration

Answer 148

• Levodopa (combined with peripheral decarboxylase inhibitors) • COMT inhibitors • Dopamine agonists • Monoamine oxidase-B inhibitors

Answer 149

Parkinson’s first line treatment Levodopa is synthetic dopamine taken orally. It is usually combined with a peripheral decarboxylase inhibitor (e.g., carbidopa and benserazide), which stops it from being metabolised in the body before it reaches the brain. Levodopa is the most effective treatment for symptoms but becomes less effective over time. It is often reserved for when other treatments are not controlling symptoms. Combination drugs are: ○ Co-beneldopa (levodopa and benserazide), with the trade name Madopa ○ Co-careldopa (levodopa and carbidopa), with the trade name Sinemet The main side effect of levodopa is dyskinesia. Dyskinesia refers to abnormal movements associated with excessive motor activity. Examples are: § Dystonia (where excessive muscle contraction leads to abnormal postures or exaggerated movements) § Chorea (abnormal involuntary movements that can be jerking and random) § Athetosis (involuntary twisting or writhing movements, usually in the fingers, hands or feet) Amantadine is a glutamate antagonist that may be used to manage dyskinesia associated with levodopa.

Answer 150

COMT Inhibitors (e.g., entacapone) are inhibitors of catechol-o-methyltransferase (COMT). The COMT enzyme metabolises levodopa in both the body and brain. Entacapone is taken with levodopa (and a decarboxylase inhibitor) to slow the breakdown of the levodopa in the brain. It extends the effective duration of the levodopa.

Answer 151

Dopamine agonists mimic the action of dopamine in the basal ganglia, stimulating the dopamine receptors. They are less effective than levodopa in reducing symptoms. They are typically used to delay the use of levodopa, then used in combination with levodopa to reduce the required dose. Pulmonary fibrosis is a notable side effect with prolonged use. Examples are: □ Bromocriptine □ Pergolide □ Cabergoline

Answer 152

Monoamine oxidase-B inhibitors block the action of monoamine oxidase-B enzymes, helping to increase the circulating dopamine. Monoamine oxidase enzymes break down neurotransmitters such as dopamine, serotonin and adrenaline. Monoamine oxidase-B is more specific to dopamine. They are typically used to delay the use of levodopa, then in combination with levodopa to reduce the “end of dose” worsening of symptoms. Examples are: ® Selegiline ® Rasagiline

Answer 153

• Loss of dopamine producing cells in the Substantia Nigra. • Dopamine: • the neurotransmitter passing messages to the basal ganglia (area of the brain responsible for balance, walking and control of movement • Dopamine works with acetylcholine to allow smooth coordinated movement. • Up to 90 % dopamine producing cells are lost before signs are reported by patients (2)

Answer 154

• At rest • 3-7Hz • Pin rolling tremor • Asymmetrical • Worse on distraction • Cogwheel rigidity= Tremor on top of rigidity

Answer 155

• ie. lead pipe increased tone – (vs spasticity ie. Clasp-knife rigidity associated with pyramidal weakness) • Asymmetrical • Increased with distraction • Kinnier Wilson manoeuvre • Limbs put also neck and axial muscles • Cogwheel rigidity: Tremor on top of rigidity

Answer 156

Rigidity: Both increased tone Lead pipe like movement Extra pyramidal pathway Movement will always be the same, despite velocity Spasticity: Clasp knife effect Pyramidal pathway Velocity dependent - more velocity, more resistance

Answer 157

• Micrographia – Writing tailors of to small – Spidery • Repetitive movements go from large→small – Asymmetrical • Face: – Featureless – Lack of eye blinking (serpentine stare) • Gait: – Slow hesitant – Stooped posture – Inability to stop or turn – Poor arm swing

Answer 158

• Stooped posture • Gait: Hesitant, festinant gaits • Retropulsion, anteropulsion • Difficulty turning, achieved with several small steps • Continuous blinking with glabellar tap • Speech: – Initially monotonous – As PD worsens becomes tremulous, slurring dysarthria (due to bradykinesia, tremor and rigidity) – Progress further can be lost completely

Answer 159

Levodopa: Madopar® (Co - Beneldopa) -CO- BENELDOPA = Levodopa + Benzerazide (Madopar) Sinemet ® (Co - Careldopa) CO-CARELDOPA=Levodopa+Carbidopa (simenet) Duodopa intestinal gel (in case they can’t swallow)

Answer 160

Domperidone

Answer 161

Side Effects of Dopamine therapy: • N+V (CTZ- area postrema which is OUTSIDE BBB) • Psychiatric SEs: vivid dreams, confusion, hallucinations and delusions, reduced libido, dopamine dysregulation syndrome (self-control) • CVS: Hypotension, cardiac arrhythmias, flushing • Dyskinesias: Typically face and limbs

Answer 162

Second line Parkinson’s disease drugs Selegiline Rasagiline • Can help to reduce dose of drug, evidence to suggest prevents neuronal degeneration • Used to reduce end-of dose deterioration (or as monotherapy in PD)

Answer 163

• Pramipexole • Rotigotine (patch – useful when NBM or struggle to take oral medication) • Ropinirole • Apomorphine (rarely used now) – given subcutaneously

Answer 164

Protein can interfere with Parkinsons medication effectiveness. Advise patients to have their high protein meals in the evening. • If patient is fed via nasogastric tube, work with the dietician (may need the feed stopping 1 hour before or after dose is due). • Ferrous Sulphate can also interfere with absorption – advise to take away from levodopa. Remember constipating effect of this as well.

Answer 165

1. Dehydration 2. Constipation 3. Infection/sepsis 4. Other medical cause Treat the above, then reassess the Parkinsons.

Answer 166

NEVER STOP Parkinson’s Medications: • Patient symptoms will worsen • Risk of neuroleptic malignant syndrome is long-term L-dopa therapy stopped or dose reduced Equivalent dose of L-dopa given transdermal (Rotigotine patch) throughout the day or NG tube

Answer 167

C. Sinemet (Mixture of levodopa and carbidopa)

Answer 168

B. Regular Propranolol (benign essential tremor)

Answer 169

C. IM Procyclidine

Answer 170

• Wilson’s Disease: AR disorder of copper metabolism – Low caeuroplasm; high urinary copper and low serum copper – Copper deposition: Basal ganglia disease; liver cirrhosis; Kayser-Fleischer rings; cardiomyopathy and conduction defects; kidney disease; low IQ – Treatment: Penicillamine (copper chelating agent)

Answer 171

• Drugs- Dopamine antagonist (anti-psychotics, metoclopramide, MPTP) • Dementia pustalgia • Lewy body dementia • Parkinsonism - plus: – Multi-system atrophy – Progressive supranuclear palsy • Wilson’s disease • AIDS

Answer 172

• Progressive Supra-nuclear Palsy: – Parkinsonism, axial rigidity, falls, dementia and inability to move eyes vertically and laterally

Answer 173

• Multi-System Atrophy: – Triad of: autonomic dysfunction, parkinsonism, ataxia

Answer 174

• Anxiety • Hyperthyroidism • Drugs- B2-agonists • Cerebellar disease (intentional) • Essential tremor • Parkinsonism

Answer 175

Benign essential tremor

Answer 176

Essential tremor • Majority have FH- ?Autosomal dominant inheritance in some families • 5-8 Hz tremor • Tremor occurs when try to adopt a posture • Shaking occasionally at rest or on intention • Typically worse in upper limbs • Head and trunk= tremulous • Anxiety makes worse; alcohol improves • Fine movements of hand less effected • Patient only present if effects lifestyle • Commoner in elderly • Treatment: ?Alcohol (small amounts), sleep and stress control, Beta-adrenergic antagonist (Propanolol), Primidone, topiramate – Benzodiazepines and other medications – Stereotactic thalamotomy and thalamic stimulation(deepbrain stimulation)

Answer 177

Intentional Tremour Cerebellar disease= Intentional tremor

Answer 178

• Chorea • Athetosis • Hemiballismus • Myoclonus • Tics • Akithesia • Acute dystonia • Chronic tardive dyskinesia

Answer 179

Ataxia is a term for a group of disorders that affect co-ordination, balance and speech. Any part of the body can be affected, but people with ataxia often have difficulties with: • balance and walking • speaking • swallowing • tasks that require a high degree of control, such as writing and eating • vision

Answer 180

Huntingtons, Wilson’s, Cerebal Palsy, Meningitis, Encephalitis

Answer 181

• Rhythmical, non-purposeful movements • Basal ganglia lesion

Answer 182

Involuntary movements of peripheries (mainly hands) • Slow moving • Typically fingers • Basal ganglia problems • Usually overlap with chorea • Various causes: – Overlap with causes of chorea – Damage to basal ganglia (ischaemia; athetoid cerebral palsy: due to kernicterus- excess bilirubin, associated with dystonias

Answer 183

Hemiballismus • Violent,contorting,continuousmovements (usually rotational) • Unilateral • Secondary to infarction/haemorrhage of contralateral subthalamic nucleus

Answer 184

• Violent, sudden jerks of single or groups of muscles (falling asleep and then jumping when waking up) • Causes: – Nocturnal, paramyoclonus multiplex – Myoclonus associated with epilepsy e.g. Juvenille myoclonic epilepsy (Janz Syndrome) – Progressive myoclonic epilepsies – Static myoclonic encephalopathy (recovery from cerebral anoxia)

Answer 185

• Akathisia – Restless, repetitive and irresistible need to move • Acute dystonia – Acute muscle contractions – Spasmodic torticollis (neck), trismus, oculogyric crisis – After single dose of anti-psychotics and some anti-emetics (metoclopramide and prochlorperazine) – Resolves quickly with IV anti-muscarinics • Chronic tardive dyskinesia – Mouthing and lip smacking, grimaces, eye blinking – After several months of anti-psychotics

Answer 186

• Prolonged muscular contraction (ie. spasm) • Causes: – Primary torsion dystonia – Dopamine responsive dystonia – Drug induced: Metoclopramide, Prochlorperazine, antipsychotics – Symptomatic (post encephalitis lethargica, Wilsons) – Focal Dystonia: Spasmodic torticollis, Writer’s cramp, ordomandibular; blepharospasm, hemiplegic dystonia (e.g. following stroke), Multiple sclerosis

Lecture ILO’s Flashcards

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