Neurology (Lecture Materials)

Neurology • • • • • • • • • • • • Headache Stroke Cerebrovascular insufficiency Subarachnoid Haemorrhage Meningitis / Encephalitis Epilepsy (& Syncope) Parkinson’s disease Dementia Multiple Sclerosis Muscular Dystrophies Peripheral Neuropathy Cerebral palsy 2 5 8 10 13 16 21 23 27 29 30 32 • • • Traumatic brain injury Spinal cord injury Spinal cord compression 34 37 40 Upper motor neurone Signs Positive Sx Increased tone Brisk reflexes Up going plantar Clonus Spasm Lower motor neurone Signs Decreased tone Poor / absent reflexes Fasiculations Wasting Negative Sx Loss of dexterity Weakness Loss of muscle control Neuro 1 Headache Mechanical = stretching of meninges Chemical = 5HT / histamine Sensation carried by CN V & IX • Outline the features of common types of acute headache including that associated with subarachnoid haemorrhage, meningitis, encephalitis and migraine. Meningitis Raised Intracranial pressure à meningism Headache all over (Meningism head ache) Photophobia Irritable à drowsy + vomiting à fits à Decreasing consciousness à Coma Stiff neck Kernig’s sign + (pain & resistance on passive knee extension with hips fully flexed) Brudzinski’s sign + (pain with hip flexion while bending neck / trunk forward) Opisthotonus (head & heels back, body arched forward Irregular respiration (if coning) Papilloedema Sepsis Decreased pulse, raised BP, Increased RR Rash (if meningococcus) Fever Encephalitis Fever, odd behaviour, fits, decreased consciousness Subarachnoid Haemorrhage Hit over head by brick Migraine With aura (see later for more info) Cluster Headache Nightly pain, around 1 eye lasting for approx 8 weeks Eye can become very bloodshot & watery with lid swelling Can be associated with lacrimation, fascial flush, rhinorrhoea Miosis and ptosis can persist Can be triggered by alcohol Glaucoma Red eyes with fixed large oval pupil Decreased visual acuity Usually in elderly / long sighted Constant dull ache in periorbital & forehead areas Can be assoc with nausea & vomiting Can be precipitated by sitting in the dark • Outline the features of chronic types of headache associated with temporal arteritis, raised intracranial pressure, analgesic-induced and tension headache. Temporal (giant cell arteritis) Usually in over 50s Tender scalp, with decreased visual acuity Tender, thickened, pulse less temporal artery Jaw claudication during eating Tension Headache Tight band around head Linked to increased stress & low mood Neuro 2 Raised ICP Worse on waking, pain can cause to wake from sleep Raised BP, decreased HR Focal signs, mental change, papilloedema, vomiting, epilepsy, Analgesic induced headache Rebound on stopping long term analgesics Seen in overuse of analgesic for migraine • Describe what is meant by migraine. Classical form Initial visual disturbance (aura - flickering f.b. blind spot) – only 20% of cases 15-30 minutes later = throbbing headache, nausea, vomiting, photophobia & exhaustion Can last 1-3 days Onsets teens / 20s F>M Can be triggered by food, stress, menstrual Often strong FH • Outline the pathophysiology of migraine Vascular Initial cerebral vascular v/c (aura) à Extra cerebral v/d Questionable as only 20% experience aura Brain hypothesis 1o due to spreading cortical depression (similar to concussion) Neural inhibition à high K+ & aura, but not headache Sensory nerve hypothesis Increase activation of sensory nerves (V) in meninges + extracranial blood vessels with resulting pain & inflammation Most probably combination of the 3 • Describe drug treatment of migraine. Treat acute attack Aspirin like (NSAID) / paracetamol / ibuprofen / AVOID OPIATES Prochlorperazine OTC (only if diagnosed) Tolfenamic acid (nsaid esp for migraine) Triptans (e.g. sumatriptan) à v/c + inhibit CGRP à reverse attack (can à chest pain so not used in IHD) Ergotamine – many SE Stimulate 5HT1D receptors à contrict large arteries à inhibit trigeminal activity Prophylactic 1st line - Beta-blockers & amitriptyline 2nd line – Na Valproate & Topiramate 3rd line – Methysergide Also try Headache diary Sleep & food hygiene advice Relaxation techniques Herbal / Vitamin supplements Neuro 3 5HT Vascular endothelium Released mediators NO Dilatation of extracerebral vessels Sensitisation of nerve endings Inflammation SENSORY NERVE ACTIVITY Neuropeptide release Peripheral Central Pain sensitisation PAIN ??? Spreading depression Neuro 4 Stroke • Define what a stroke is and outline the three major causes ‘a clinical syndrome, of presumed vascular origin, typified by rapidly developing signs of focal or global disturbance of cerebral functions lasting more than 24 hours or leading to death' “Transient” = complete recovery within 24 hrs = TIA “Completed” deficit persists but is stable “Evolving” deficit persists & worsens after 6 hrs Can be Infarction (80%) or Haemorrhage (20%) o Infarct § Risk factors – HT, DM, Smoking, AF, High Cholesterol, Clotting disorders, infective endocarditis, alcohol, IVDU o Haemorrhage § Can be intracranial haemorrhage (10%) or sub-arachnoid haemorrhage (10%) § Both present as strokes, but are managed differently to infarcts. • Describe the importance of stroke o Single biggest cause of severe disability o One of the leading causes of mortality and morbidity in the UK o Incidence = 2/1000. o NB: not only affect the elderly – 8% under 55, 1% under 30 o 20% of all acute strokes will die within 1 month, ½ of survivors with have physical disability o Huge impact on individuals and families; It can dramatically change an individual's function, role and lifestyle from one day to the next. à require carer / care home o Stroke is associated with high levels of anxiety and depression. o Stroke is preventable – Control of risk factors reduces incidence • Outline the major factors that predispose to stroke disease Non-Modifiable § Increasing age § Male § FH § PMH of stroke / embolism § Heart disease (HF, AF, endocarditis) § High fibrinogen Modifiable § High BP - promote health lifestyle, diet etc, medicate Aim for < 140/90, <130/85 if renal impairment, <130/80 if DM § Heart disease – reduce risk factors, anticoagulate if required § Atrial Fibrillation – verify, convert to sinus if possible, warfarin (INR 2-3) or aspirin if warfarin contraindicated or <65yrs § Carotid stenosis – antiplatelet, endarterectomy § Smoking – support for quitting § Poor diet – advise § Obesity – advise BMI 18-25 § Excess alcohol – avoid excess / binge § Hyperlipidaemia – statin / diet § DM – aim for HbA1C < 7% § Others - Polycythaemia, Oral contraceptive Neuro 5 • Describe the morphology and pathological consequences of haemorrhagic and ischaemic stroke. Ischaemic Occlusion of cerebral artery or its branch Anastomosis required to maintain perfusion Other mechanisms help to maintain homeostasis Failure of these mechanisms à ischaemia à neurones fail Temporary period where recovery is possible if flow is restored If flow is not restored à cell death Can take several hours for cell damage to complete & full effects to be seen Haemorrhagic Rupture of blood vessels à Immediate cessation of function due to structural disruption (damage to white cell tracts) If severe can à increased ICP à coning à instant death Can occur into previous infarcted / TIA regions due to endothelial damage • Describe the Classification of Strokes and the features of each type Bamford stroke classification TACS – Total Anterior Circulation Syndrome Blockage of proximal stem of Middle cerebral artery (by thrombus/embolus) Often associated with internal carotid occlusion ALL patients present with Hemiplegia and/or Sensory loss on contralateral side Hemianopia opposite side to lesion Disturbance of higher cerebral function (speech, neglect, inattention, reading, writing) PACS – Partial Anterior Circulatory Syndrome Restricted infarcts of upper or lower divisions of middle cerebral artery Same symptoms of PACS but fewer LACS – Lacunar Syndrome Single vessel damage, deep within the brain (e.g. internal capsule) = Maximal damage from a single vascular event Partial motor / sensory loss Can be ataxic if in BG or pons NO higher function impairment NO visual field deficit No sign if brainstem disturbance POCS – Posterior Circulatory Syndrome Infarcts of the brain stem, cerebellum or occipital lobes Isolated hemianopia / cortical blindness CN palsy same side, with contralateral motor/sensor loss Cerebellar Ataxia Disorders of eye movement (Diplopia, Nystagmus) • State the differentials for stroke (stroke syndromes) and how to recognise them o o o o Subdural Haematoma – may be more gradual onset, S&S fluctuate, drowsiness. Consider if Hx of fall/trauma/aspirin/warfarin, Diagnose by CT Epilepsy – post ictal weakness = Todds Paresis. Obtain collateral history, recovery <24hrs Hypoglycaemia – poorly controlled DM, ALWAYS CHECK BM Brain Tumour – more insidious onset (days-weeks), stuttering / slow progressive course, vomiting & headache, seen on CT Neuro 6 o o o • Describe the initial investigations for a patient presenting with a stroke including the indications for performing a CT/MRI scan. o o o o o • Migraine – transient weakness, rapidly improves, Hx of headache Encephalitis – uncommon, features of infection, fits common, CT/MRI may help, EEG most use Cerebral Abscess – uncommon, important to diagnose as surgery required, signs of infection, long prodrome, headache, reduced conciousness, CT scan diagnostic FBC, CRP, Glucose, Renal function, LFT, Cardiac Enzymes, cholesterol, TFTs, ECG, Head CT (for most) § Indications – suspicion of SOL / SAH / Meningitis / need to distinguish between haemorrhage & infarct, possible cerebellar haematoma § Contraindications – so likely to die that result unlikely to help CXR Cardiac Echo 24hr tape Describe the initial and long-term management of a patient with an established stroke including rehabilitation and measures for secondary prevention in appropriate cases. Initial Management o Thrombolysis / drainage if appropriate o Aspirin / Warfarin (only if AF) / TEDs o Good hydration + O2 (reduces ischaemic damage) o Check blood glucose – avoid lactic acid build up à avoids further damage o Arrange Carotid Doppler – endarterectomy if > 70% stenosis & symptoms 2o complication of stroke o Aspiration pneumonia Involve SALT team o Malnutrition Check SALT, involve dietician if required o Pressure sores Move position regularly, mobilise early o Depression Set realistic goals, medicate if required o Incontinence Catheterise? Can à decreased bladder size o Joint damage early physio o Shoulder pain o Neurological pain o Contractures / spacsiticy Good physiotherapy & botox / baclofen o DVT from immobility Exclude haemorrhagic stroke and anticoagulate Neuro 7 Cerebrovascular Insufficiency Sudden loss of neurological function Ischaemic is more common Haemorrhage can be subarachnoid or intracerebral • Define and differentiate the following: Amaurosis fugax Transient, sudden loss of vision in one eye Due to emboli in retinal aa / TIA / Migraine Transient ischaemic attack (TIA) Focal neurological deficit lasting less than 24 hours & with complete recovery 80% microemboli (usually from thrombus in great vessels) 20% decreased cerebral perfusion (dysrhythmias, decreased BP, atheroma) Symptoms peak rapidly and have a short duration Anterior circulation (carotid system) – more serious prognosis Amaurosis fugax Aphasia Hemiparesis Hemisensory loss Hemianopia Posterior circulation (Vertebrobasillar) Diplopia, hemianopia Choking, dysarthria Vertigo, vomiting Ataxia Hemisensory loss Transient amnesia Tetraparesis LOC (rare) Diagnosis is usually based on description (history) Prognosis 40% CVA, 25% die within 5 years NB: RIND (reversible ischaemic neurological deficit – resolves in a few days) Cerebrovascular accident (CVA) Persistent deficit Completed (usually within 6 hours) Stroke in evolution (changing signs) Minor stroke (recovery without significant deficit within 7 days) • Outline methods of evaluating patients with carotid stenosis. Carotid Doppler & duplex Carotid angiography & MRA Assess if surgical treatment is possible Not done if >75 as high risk & poor yield. • Describe the symptoms of carotid territory cerebrovascular disease and list the differential diagnosis for a TIA. Anterior cerebral (front and mid cerebrum) Weak & numb contralateral leg +/- arm Akinetic + mute + paraplegia if bilateral Middle cerebral (lateral cerebrum) Contralateral hemiplegia (flaccid à spastic (arm & face)) Sensory loss (arm & face) Neuro 8 Dysphasia, dyslexia, dysgraphia, dyscalculia Visuospatial disturbance Contralateral hemianopia Lenticulostriate arteries (BG) Death Differentials Peripheral nerve conditions Focal epilepsy (jerking limbs, gradual onset) Migraine & focal prodrome (headache, aura, symptoms spread & intensify) Malignant hypertension (rare) Hypoglycaemia (rare) MS (rare) Paeochromocytoma (rare) • Outline a management plan for evaluating a patient with TIA. Urinalysis + BM Hb & Platelets WCC ESR & CRP Serology CXR ECG Blood cultures Antibodies Diabetes Polycythaemia Increased = ? infection Arteritis Syphilis Neoplasm Recent infarct, dysrhythmias Endocarditis SLE in younger patients Assess Cardiac rhythm & heart sounds BP Peripheral pulses Carotid, subclavian & femoral bruits Advise on Weight Exercise Smoking • Outline medical and surgical management for TIA & carotid stenosis including the medical management of recognised risk factors and the indications for carotid endarterectomy. Medical management Decrease risk factors (smoking, alcohol, improve DM control, increase exercise) Statin if high cholesterol (+MI/angina) Antihypertensives – BP must be lowered slowly – beware falls Antiplatelet – low dose aspirin (75mg o.d) Anticoagulation – if AF / dsrhythmia / valve lesions / cardiomyopathy Treat AF Endarterectomy If 80% stenosis in internal carotid artery Complications Stroke – due to embolism / thrombosis (less than untreated risk) MI – BP should be closely controlled Cranial nerve palsy (7.5%) – hypoglossal, vagal, recurrent laryngeal Wound haematoma Neuro 9 Subarachnoid Haemorrhage Dramatic onset spontaneous (non traumatic) arterial bleeding into subarachnoid space 1 in 10,000 ¾ are < 65yrs Risk factors Smoking Hypertension Alcohol • F > M (4:1) RR 2.2 RR 2.5 RR 2.1 Describe the pathology of SAH and the main causes. Saccular (berry) aneurysms (85%) Rare before 20yrs Increased risk if 10 relatived or if polycystic kidneys or collagen defects Locations 30% anterior communicating artery 25% posterior communicating Ant Comm artery 20% middle cerebral artery 15% basilar artery Post Comm Arteriovenous malformations (5%) Developmental origin Usually within hemisphere Can cause epilepsy Rare Bleeding disorders Mycotic aneurysm (endocarditis) Acute bacterial meningitis Brain tumour Arteritis (e.g. SLE) Spinal subarachnoid (spinal AV malformation) Co-arctation of aorta Marfan’s syndrome, ehlers-danlos syndrome Polycystic kidneys – ALL get screened bec high risk of berry aneurysm • Outline the typical presentation with sudden onset of severe occipital headache with sudden collapse and loss of consciousness in severe cases. Devastating “thunderclap” headache “hit over head with brick” (often occipital) Can be ppt by exercise / straining / sexual arousal à vomiting à collapse & seizures Can à LOC & possible Coma • Describe clinical features of neck stiffness, photophobia, with or without focal neurology or reduced level of consciousness. Distressed & irritable Photophobia Neck stiffness (may take hours to develop) Kernig’s sign (pain & resistance on passive knee extension with hips fully flexed) Papilloedema (with possible retinal haemorrhage) Can be CN III palsy due to local pressure Neuro 10 NB: amount of blood leaked from aneurysm is often small But blood à irritation of meninges à symptoms Often have high BP (can be cause or effect (i.e. temporary cushing response) Often high pulse – due to pain Often mild (12-14) leukocytosis • Outline the investigation of suspected SAH including the relative timing of CT scanning and lumbar puncture (LP). Emergency CT as soon as stable Blood / Xanthochromia Negative (10% of SAH) (= Bilirubin from Hb breakdown) Shows SAH Refer to neurosurgeons Resuscitate Give Nimodipine • CSF After 12 hours Traumatic LP doesn’t à xanthochromia as soon as stable If CT & CSF are –ve at 12 hrs patient has not has SAH Outline the medical management & further investigation of a patient with a confirmed SAH. Management post diagnosis If GCS > 13 then supportive management Bed rest - to improve cerebral perfusion BP fluctuations may risk rebleed Nimodipine - reduces stroke by 33% and poor outcome by 40% 3 litres IVI per day - dehydration risks vasospasm & fluid lost by vomiting 0.9% saline - hyponatraemia common Analgesia/antiemetics - codeine/paracetamol or tramadol Regular neuro obs - to detect complications Further investigation CT angio / MRA to identify site of bleed & options for surgery If GCS < 13 or deteriorating Consider: Hydrocephalus Intracerebral haematoma Subdural haematoma à CT angiography ASAP to find cause Identify cause à clip aneurysm (open) or coil it (endovacsular) Risks of Cliping / Coiling Stroke if deployed in wrong place Aneurysm rupture Introduction of infection • Describe the immediate and long-term consequences of subarachnoid haemorrhage Immediate mortality = 30% 75% of all SAH deaths happen in the first 3 days Important complications Rebleed 40% of survivors (mostly in first 4 weeks but increased lifetime risk) Neuro 11 Vasospasm requires fluids and nimodipine Manage with hypertension, hypervolaemia & haemodilution Hydrocephalus <10% need long term shunt Stroke spasm or complication Hyponatraemia & Dehydration Arrhythmias, Pulmonary oedema, LRTI, VTE Residual effects Neurological & cognitive deficits Epilepsy Neuro 12 Meningitis/Encephalitis NB: Bacterial meningitis is a notifiable disease • Outline the morphology and pathological consequences of leptomeningitis. Leptomeningitis is inflammation of the subarachnoid space = organisms in the CSF Bacteria source can be blood, middle ear, mastoid, nasal sinus or dural venous sinus Can be divided into 3 groups: Acute purulent meningitis – bacterial Subarachnoid space contains creamy inflammatory exudates rich in neurophils. Severe inflammation à 2o thrombosis of superficial vessels & cerebral ischaemic damage Can à obstruction of CSF drainage à hydrocephalus Lymphocytic meningitis – viral Clinical malaise & meningism. Usually self limiting Chronic & granulomatous meningitis – TB Chronic inflammation and fibrosis Thickening and opacity of meninges with lymphocytic infiltrates à CSF drainage obstruction, vascular lesions (infarction) à cranial nerve and spinal root palsies • Describe the common bacterial and viral organisms causing meningitis in adult life. NB: viral meningitis is more common that bacterial and is usually self limiting Neonate – E.coli, B haemolytic strep, Strep Pyogenes, S. Aureus, Listeria Infant – Grp B Strep, Listeria, E.coli Children – H. Influenza, Meningococcus, S.pneumoniae, S.aureus, TB Young Adults – Meningococcus, Neisseria meningitidis Older Adults – Strep pneumoniae Immunocompromised – Pneumococcus, listeria, Gr –ve orgs, cryptococcus • Outline the clinical presentation of bacterial meningitis including headache, photophobia and neck stiffness. Pathology Pia is congested & infiltrated with inflammatory cells Layer of pus forms à adhesions à affects CSF flow à raised pressure Raised Intracranial pressure à meningism Headache all over (Meningism head ache) Photophobia Irritable à drowsy + vomiting à fits à Decreasing consciousness à Coma Stiff neck Kernig’s sign + (pain & resistance on passive knee extension with hips fully flexed) Brudzinski’s sign + (pain with hip flexion while bending neck / trunk forward) Opisthotonus (head & heels back, body arched forward Irregular respiration (if coning) Papilloedema Sepsis Decreased pulse, raised BP, Increased RR Rash (petechial if meningococcus) Fever Neuro 13 • Describe the appearance of the typical rash of meningococcal septicaemia. Non blanching Petechia à purpura • Discuss the investigation of a patient with suspected meningitis FBC U&E Glucose Cultures Nasal Swabs & Urine Culture CT (if any signs of raised ICP) or focal neurology Lumbar puncture Contraindicated if – any sign of raised ICP, depressed consciousness or focal neurological signs (until CT exludes hydrocephalus & SOL) Puncture at L3-4 or L4-5 (Between iliac crests) – NB lower in children Appearance Cells Protein Glucose • Normal Clear Viral Clear / Turbid Bacterial Turbid <5/ml Mononuclear 0.2 - 0.4g >2/3 of plasma 10-100/ml Mononuclear 0.4 - 0.8g normal 200-300 /ml Polymorphs 0.5 – 5g <2/3 of plasma TB Turbid / Fibrinous 10-300 /ml Mononucl + poly 0.5 – 5g < 2/3 of plasma Discuss an appropriate antibiotic regimen for treatment of bacterial meningitis. Until results through Adult – i.v. ceftriaxone 2mg bd Child – i.v cefotaxime (+ gentamycin in neonate) Then take advice from lab • Outline the long-term complications of bacterial meningitis. Can develop within days to weeks post infection Infarction and venous thrombosis Cerebral abscess formation Hydrocephalus Cerebral oedema (à death) Seizures Residual effects Memory loss Dyspraxia Hearing / balance problems Weakness / Paralysis (in some cases cerebral palsy) Speech problems Vision changes • Outline the clinical features of encephalitis. Diffuse inflammation of the brain is caused by viruses (herpes simplex, polio and rabies), rickettsial and certain bacterial (listeria, treponema and borrelia) infections Hyperaemia of meninges, petechial haemorrhages on the brain and brain oedema. Can also be focal necrosis (esp with herpes) Neuro 14 Due to diffuse involvement à myelopathy (motor and sensory) and encephalopathy (confusion and decreased level of consciousness) • Discuss the aetiology, diagnosis and management of common types of viral encephalitis. Presentation Acute onset headache, fever, focal neurological signs (aphasia / hemiplegia) and seizures Disturbed consciousness (ranging from drowsy to deep coma) NB: if animal bite Hx, photo & hydrophobia and delusions / hallucinations then consider RABIES Investigations CT before LP (as focal neurology) CSF – can show lymphocytes (or polymorphonuclear cells if early). Send for PCR EEG – slow wave abnormality usually in temporal lobes Management Anticonvulsant treatment Dexamethasone to control ICP Neuro 15 Epilepsy Seizure Sudden event with Impairment of motor function / tone and/or consciousness • Describe the classical features of a grand mal seizure including possible aura, tonic and clonic phases, tongue biting, incontinence and post ictal confusion. Aura May be preceeded by a partial seizure (the aura) Tonic Patient goes rigid and becomes unconscious (can fall down à injury) Respiratory arrest (may à central cyanosis) Clonic phase Clonic jerks Some patients have a “flaccid” phase of deep coma instead) At any stage Urinary incontience Tongue biting Post ictal Confusion Severe headache Drowsiness & desire to sleep Possible trigger factors (for any seizure) Sleep deprivation Alcohol (inc withdrawal) Recreational drug misuse Physical and mental exhaustion Flickering light (primary generalised epilepsy only) Intercurrent infections & metabolic disturbances • Describe the differential diagnosis of epilepsy / seizure Epilepsy Syncope (see below) Hyperventilation/panic attacks Difficulty breathing, sense of doom Non-epileptic attacks = pseudoseizures Subconscious attacks Expression of past psychological trauma Unresponsive to anti-epileptic drugs Usually gradual onset, side-to-side motion with back arching and bite front of tongue. Can be tired for days post ictal Migraine Convulsions rare, severe headache Metabolic causes Alcohol, hypoglycaemia, hyponatraemia, drugs TIA • Outline a classification of epilepsy including simple or complex partial seizures, generalised tonic-clonic or absent seizures and myoclonic jerks. Epilepsy is A tendency to have seizures Affects 0.5% of population Onsets rapidly, relatively short duration Neuro 16 Generalised Involves the whole brain, immediate unconsciousness Tonic clonic (Grand Mal) Initial strong contraction Loss of respiratory & body function (1min) Violent synchronous jerks (3-4 min) Then unconscious with gradual recovery EEG high frequency then intermittent 1st choice for Rx = valproate / carbemazepine / lamotragine, 2nd levetircetam Absence seizures (petit mal) Abrupt loss of consciousness – lasts approx 10 secs Less dramatic, more frequent Vacant – stop activity & speech Tone usually not affected Full immediate recovery but unaware of episode EEG – characteristic synchronous discharge (3Hz spike & wave) 1st choice Rx ethosuxemide, 2nd Valproate / lamotragine Atonic “Drop attacks” Complete loss of conscious & muscle tone (may be localised in body) Usually complete recovery although may have injuries from the fall Myoclonic Very brief single jerks “shock like” Fast (immediate) recovery Loss of consciousness but too short to be appreciated Infantile Spasm Begin between 3 & 12 months Single jerks which cluster together EEG shows hypsarrhythmia Development slows, stops and can regress à severe mental retardation Status Epilepticus Partial Discharge begins & remains local Simple symptoms Simple Partial No loss of consciousness Same as “aura” Focal neurological discharge Motor = jacksonian march Sensory = tingling / burning Psychic = deja vue / hallucinations Special sense = metallic taste, flashing lights Autonomic = epigastric rising sensation Complex partial (psychomotor) Aura – may initially be fully consciousness Altered consciousness – but partially responsive Automatisms – lip-smacking, teeth grinding, wandering • Outline the main causes of epilepsy Neurobiology Dysfunction of GABA inhibition / Glutamate excitation Decreased GABA, Increased Glutamate Causes Idiopathic = childhood epilepsy (absence / myoclonic / tonic-clonic) Genetic – metabolism or storage errors Hydrocephalus Neuro 17 Cerebral anoxia – Stroke / Head injury Drugs / withdrawal (inc alcohol) Toxins – heavy metals (tin, lead) and organophosphates (sarin) Metabolic disease – Hypo Ca / Na / Mg / glucose, Renal / Liver failure Infective – meningitis, post infectious encephalopathy Inflammatory – MS, SLE Degerative – Alzheimers, CJD Tumours (partial) • Outline the immediate 'first aid' treatment of a patient having a generalised seizure plus the drugs used to control an acute seizure. Prevent self-harm Post ictal – ensure airway open • Outline the commonly used anti-epileptic drugs. Treatment Seek to identify cause (if any e.g. tumour, drug) Give drug treatment 75% à complete control 10% à 1 seizure a month or less MANY drugs cause leukopenia so monitoring important Enhancement of GABA Barbiturates (phenobarbitone) limited use now Benzodiazepines (clonazepam) Opens Cl- channels Only used in an emergency as a sedative Vigabatrin Inhibits GABA transaminase à increased GABA availability Valproate Used for tonic clonic / absence Weak GABA transaminase inhibitor ? also Na+ channel blockers SE include weight gain, sedation, tremor, birth defects, hair loss Can cause hepatotoxicity – monitor INR Can cause thrombocytopenia – monitor FBC Gabapentin Inhibits GABA metabolism Tiagabine GABA analogue with ability to penetrate BBB Inhibits GABA reuptake Reduce Glutamate function Phenytoin Used for tonic-clonic Dependent inhibition (block the most active cells) – NB: if all blocked à death! & blocks Na+ channels SE include gingival hyperplasia, Nystagmus, birth defects Causes enzyme induction & has unusual pharmacokinetics (can saturate metabolism à sudden increase in plasma conc so monitoring required) Glutamate receptor antagonists In development Various Actions Carbemazepine Use dependent Na Channel blocker Neuro 18 NB: variable bioavailability between brands SE include rash, dizzy, diplopia, birth defects, enzyme inducer Monitor INR Lamotrigine Useful for bipolar disorder Na+ channel blocker + inhibits glutamate release SE include risk of marrow toxicity = Aplastic anaemia (à rash & flu like illness) SAFEST in pregnancy Felbamate Acts on glutamate function Rx for Lennox Gestaut Side effects can be severe Ethosuxemide Inhibits Ca2+ channels Rx for absence seizures Can cause nausea & anorexia • Outline the management of status epilepticus. Rapidly repeated seizures without regaining consciousness Ensure airway – intubate if required O2 100% +/- suction IV access & bloods Thiamine if malnourished / alcoholic IV glucose unless known to be normal Correct low BP with iv fluids IV lorazepam / rectal diazepam OR slow phenytoin infusion OR GA à paralysis + ventilate • Guidelines for epileptic patients General advice Do not operate machinery etc. Bath / Shower with bathroom door unlocked and someone else in the house. Activities close to water (swimming) should be with someone aware of the condition and capable of rescuing the patient Driving - 1 year seizure free or 3 years (having seizures only at night) Withdrawing drugs – 2-4 years seizure free, withdraw gradually, no driving during withdrawal • Outline the features that distinguish seizures from syncope Facial Colour Tongue biting Nausea Aura Reorientation Paralysis Setting Seizure Cyanotic Common Uncommon Sometimes Minutes Sometimes Any Attacks Age CK Repeated <45 raised Neuro 19 Syncope Pallor Rare Common No Seconds No Rare when recumbent Infrequent Any Normal • Outline the clinical types of syncope (e.g. vasovagal, micturition, carotid sinus, and cough). Presyncope – lightheadedness due to decreased cerebral perfusion Syncope – temporary impaired consciousness, lasts 1-2 minutes Vasovagal Triggered by prolonged standing, big meals, heat, pain, emotion Contraction of underfilled ventricles à parasympathetic activation à decreased HR & v/d à nausea, sweating, pallor, decreased visual fields, muscle jerks Cardiac Arrhythmias, postural hypotension, hypersensitive cardiac sinus, obstructed cardiac / vascular disease Situational micturition @ night, sinus sensitivity on shaving / turning head, weakness after coughing • Describe appropriate investigations for a patient suspected of syncope. History & full examination (inc laid & stood BP) Bloods – FBC, U&E, Ca, Mg, CK, Creatinine, glucose ABG ECG – heart block, arrhythmias, long QT Echo Carotid Doppler Holter monitor (24hr ECG) Tilt table test EEG / sleep study Neuro 20 Parkinson’s Disease • Outline the pathological basis of Parkinsonian syndromes in terms of dopamine depletion in the basal ganglia and outline the pathology of classical Parkinson’s disease. Neurodegeneration of the extrapyramidal system (substantia nigra) o Nigrostriatal pathway lost (remaining cells have lewby bodies) Motor symptoms o Decreased peptides in the striatum (CCK8, enkephalins) o Loss of dopamine in the mesolimbic part of the brain Psychiatric symptoms o Decreased cortical NA & ACh Endocrine symptoms o Decreased hypothalamic amines (DA, NA) Causes of cell loss o Oxidative stress o Genetic (rare) – parkin gene o Drug induced (MPTP) o Drug induced symptoms (DA antagonists) o Viral (post encephalitis parkinsons) • Outline the evidence that oxidative stress and free radical abnormalities may be involved in Parkinson's disease. Decreased glutathione (GSH = antioxidant) so reduced protection Increased free Fe à ROS production Increased free radicals Increased lipid peroxidation (due to oxidative stress) Inceased superoxide dimutase activity (compensator mechanism) Impaired mitochondrial function and DNA damage • All found in parkinsons barins Outline the genetics involved in parkinsons Lewy bodies – cellular inclusions within neurones assoc with neurodegeneration. Contain protein called synuclin Familial PD – if mutation in synuclin gene à increased susceptibility Parkin gene – ubiquitin system & degeneration of protein. If fails à increased no of lewy bodies • Outline the common clinical features including tremor at rest, rigidity and bradykinesia. Tremor Initially intermittent, showing when tired Usually more marked in one hand & same leg 3-6Hz – more marked at rest “pill rolling” Rigidity Resistance to passive movement (increased tone) Can be smooth thoughout = lead piping When combined with tremor = cog wheel Bradykinesia Difficulty initating movements Small spidery handwriting Shuffling “festinant” gait, no arm swing Stoops as walk Decreased blinking Others Mask like expression Abnormal swallowing à saliva in mouth (can dribble) Neuro 21 Constipation & urinary retention Depression Dementia Postural abnormality Endocrine problems • Outline commonly used drugs to treat Parkinson's disease including L-dopa, plus dopa decarboxylase inhibitors, dopamine agonists and their common adverse effects. L-dopa + Dopa-decarboxylase inhibitor (e.g. carbidopa) Dopamine is ionised at pH7.4 (cant cross BBB) and metabolised by MAO (found on cells of BBB) L-dopa (precursor) is unionised at 7.4 so can cross BBB but is converted to DA in periphery Dopa-decarboxylase Inhibitors prevent conversion peripherally & cannot cross BBB as ionised Restores function in corpus striatum & benefits hypokinesias Side Effects After 2 years can develop on/off (bec short ½ life) and choreic movements Nausea & anorexia (due to peripheral effect) Hypotension Psychotic effects Eventually à dyskinesias & loss of control Dopamine agonist e.g. bromocriptine D2 receptors = inhibitory (mainly in BG) D1 receptors = excitatory (few in BG) Post synaptic DA receptors not affected by disease so use a D2 agonists to treat symptoms Side effects Used alone less SE than levodopa treatments But more neuropsychiatric Ses Can cause excessive daytime sleepiness and fibrotic reactions (pulmonary, retroperitoneal and pericardial MAO & COMT enzyme inhibitors MAO inhibitor (selegeline) & COMT inhibitors prevent breakdown of DA à prolong effects Amantidine Increases DA release Indirect DA enhancement Adenosine A2A receptor antagonist In Future Fetal cell transplants (stem cells) Surgical – stimulate subthalamic nuclei Prevent neurodegeneration • Describe the causes of Parkinsonism including drugs (neuroleptics, anti-emetics) and the Parkinson-plus syndromes. Parkinsonism can be due to Stroke Encephalitis Meningitis Antipsychotics (haloperidol, metoclopromide, phenothiazine) MPTP (synthetic heroine) Overdose of narcotics, anaesthesia, toxins, carbon monoxide Neuro 22 Dementia A syndrome of acquired global impairment of higher cerebral function, gradually affecting memory, intellect, comprehension, personality, emotion & behaviour & not to be confused with the syndrome of delirium/acute confusional state. Consciousness is not impaired Affects occupation & social functioning Must be > 6 months onset & duration • List the main clinical features of dementia including memory loss and global intellectual deterioration and relate these to different lobes of the cerebral hemisphere. Memory (first & most prominent – recent memory worse affected) Mild = forgetfulness in daily life Later à long term with all aspects of memory affected Impaired abstract thinking (e.g. proverbs) Dysphasia – central language control – affects read, write, understand. Memory related Dyspraxia – sequencing problem – also memory related Judgement Changes in personality and behaviour – due to frontal lobe involvement Impaired judgement – loss of inhibitions due to frontal lobe involvement Accentuation of personality traits (e.g. aggression, paranoia) – frontal lobe Behavioural & Psychological Symptoms Some get depressed, challenging behaviour, apathy, agitation & aggression, disinhibition, wandering Psychosis (delusions & Hallucinations (may develop in moderate severity, but usually short-lived)) Often Die from bronchopneumonia due to dysphagia NB: Avoid antipsychotics for as long as possible & only use with full assessment NB: Check if its delirium on mild dementia (if so its treatable to restore to prev. state) • List the main non-reversible causes of dementia and outline the pathology seen, including Alzheimer’s disease, vascular disease, dementia with Lewy bodies, and frontotemporal dementias. Incidence 5% prevalence 65+ population Rises with age, higher in low socio-economic class (education is protective) 20% of 80 +’s 25% of 85 +’s 30% of 90 +’s 20% in care, 80% at home (Membrane proteins assoc with ER. If wrong can’t support brain Increased risk if +ve FH architecture. Not AR/AD but APP gene on Chr21 multifactorial) Presenilin 1 on Chr 14 Presenilin 2 on Chr 1 Apo E type E4 (ApoE should carry cholesterol, E4=increased risk, E2 = protective) Causes in >65s 60% 15-20% 15-20% >5% Alzheimer’s disease = DEGERATIVE Vascular dementia (inc multiinfarct) – usual vascular risk factors DLB (Parkinsons = in substantial nigra, dementia = widespread) (30-70% of parkinsons patients develop dementia) Alcohol-related ‘Reversible’ (mostly young people) Neuro 23 Causes in <65s 1st Alzheimer’ s 2nd Frontotemporal dementia (FTD) Other causes:Huntington’s disease Prion disease (CJD) – very rapid progression Infective – e.g. HIV Dementia, Herpes simplex encephalitis Toxic/metabolic causes (E.g. Copper) Picks Disease Neoplastic – mets cause infarct, short life expectancy Trauma – e.g. boxing à increased plaques and tangles Alzheimer’s disease Gradual progressive decline in cognition and ability to function; may be brief plateaus; usually presents loss of memory, especially new learning & then later higher cortical functions (eg. language, praxis, executive (planning & decisions) function) affected and behavioural & psychiatric disturbance may be seen. Physiology Cortical atrophy, cerebellum less affected Neurofibrillary tangles seen in the grey matter & hippocampus Hyperphosphorylated tau proteins are insoluble à form paired helical filaments à disrupts axon transport & cells die NB: tangles occur in normal brain (increase with age), also parkinsons, post meningitis, SSPE and head injury (e.g. boxing) Senile plaques with “halo” are also seen in alzheimers Amyloid Precursor Protein (APP) from Chr 21 should be broken down by Bsecretase. If G-secretase à Beta Amyloid à Insoluble & disrupts function forming plaques & tangles. Alzheimers more common in Downs Syn bec extra APP from extra Chr21 Genetic links are seen, but most cases are sporadic Disease modifying drugs are now available Management Cholinesterase inhibitors (slows progression, not cure) cholinergic receptors very important for memory. Act to increase Ach, no effect on tangles/plaques NB: Also useful in other types of dementia Donepezil (aricept) – licenced for warly-mod, improves memory NICE said NOT cost effective Requires: Diagnosis of Alzheimer’s (exclusion of others) MSE > 10 Carer available to administer drug No complex medical history Rivastigmine (exelon) Galantamine (reminyl) Glutamatergic modulator memantine (ebixa) Other tech. Approaches Neuroprotective tx’s, nerve growth factors Vit.e & antioxidants (help preserve cognitive function) NSAIDs (for vascular dementia) Hormone replacement therapy (rarely used) Neuro 24 ? Gene therapy – antibody to knock out G-secretase (success in mice, made humans very ill) ? Stem cells Vascular dementia Due to brain blood vessel disease; can present after acute vascular event (e.g. stroke) or sub-acutely with accumulating events or more gradually with small vessel disease, presenting with more patchy deficits, perhaps attentional & executive/planning problems, gait disturbance and apraxia; fluctuation, neurological signs and behaviours that challenge are common Saw tooth progression with improvement between but never recovery to initial level Dementia with lewy bodies (DLB) Presents with recurrent visual hallucinations, fluctuating cognitive disturbance and motor features of Parkinsonism (these following/proceeding the cog. imp. within 1 year); often associated are falls, disturbed consciousness and autonomic dysfunction Lewy Bodies (containing ubiquitin & A-synuclein) are in the substantia nigra of Parkinson’s patients BUT found everywhere inc cerebral cortex of DLB NB: important to diagnose as parkinsonian symptoms worsen if given antipsychotics for hallicinations Frontotemporal dementia Syndrome with various causes (e.g. MND, AD & Pick’s), usually presents with language disturbance and/or behavioural difficulties (either disinhibition or apathy), more prominently impaired function than cognition; mainly “presenile” & relatively uncommon in older people; frontal type may be associated with overspending, poor hygiene, criminal or sexual behaviour, poor eating habits or repeated odd stereotyped behaviour Picks disease – affects frontal & temporal lobes Controls social skills à shoplifting, sexual dis-inhibitions Rapidly progressive with hyperaurality & no satiety Can occur in younger patients Pick bodies seen in grey matter • List the causes of a dementia syndrome that might be reversible on treatment Space occupying lesion – e.g. tumour / subdural haematoma. (Focal picture) Normal Pressure Hydrocephalus – unusual gait, “heavy feet”, early urinary incontinence, atypical dementia. Due to increased pressure but brain atrophies (ventricles enlarge) à normal pressure. Manage with CSF shunt Vit B12 deficiency Severe hypothyroidism (often very ill if severe enough to affect brain) Neurosyphillis (increasing in incidence due to STDs) • Outline an investigation plan to exclude a treatable cause of dementia. FBC - Infection, autoimmune Chemistry - Metabolic encephalopathy Serum: B12, folate - Deficiency Endocrine (T4,TSH) - Hypothyroid Other (HIV, syphilis) CT, MRI - Mass, infarcts, NPH, atrophy, white matter disease EEG - Diffuse slowing / Focal slowing / Spike-and-wave CJD / Triphasic waves / PLEDs Neuro 25 • Discuss the long-term management of a patient with dementia including the support of their family, social services and possible long-term supervised care. Assess the patient’s social situation – are carers needed etc Treat any identifiable risk factors (vascular etc) Direct treatment of condition as appropriate (e.g. aricept) • Outline the clinical features that should lead to consideration of Creutzfeldt Jakob disease Young adults Rapidly progressive Cognitive impairment Motor signs - Myoclonus, spasticity, asymmetrical Cerebellar signs Eye signs – diplopia, field defects, hallucinations, cortical blindness Neuro 26 Multiple Sclerosis Is an autoimmune neuro-degenerative disease (confined to the brain & spinal cord) leading to impaired function of the CNS 120/100,000 prevalence. F:M = 2:1 - 15% 1st degree relative, 35% twin-twin Peak onset 40s • List the common sites of CNS involvement Plaques of demyelination 2-10mm on: Optic nerves Periventricular region Brain stem and cerebellar connections Cervical spinal cord (corticospinal and posterior colums) Usually no focal muscle wasting (anterior horn is intact) • Outline the pathology of demyelination and clinical implications of secondary axonal loss in MS T lymphocytes cross BBB & recognise myelin antigens à proliferation à cytokine release & macrophage destruction of oligodendrocytes (1o demyelination) à plaques (patches) of demyelination (of white matter) but axons remain in tact à Unable to transmit impulses (no K+ channels where myelin used to be) à oedema à compromised conduction in neighbouring fibres à plaques replaced by glial scar tissue & axon degenerates NEVER affects peripheral nerves • Outline the common symptoms & signs of MS Visual loss Blurring unilaterally à sight loss for hours / days Blindness is rare If lesion à optic neuritis à disc swelling à loss of acuity No residual symptoms Brainstem demyelination Acute can à diplopia, vertigo, facial numbness, weakness & dysphagia Spinal cord lesions Spastic paraparesis (over days / weeks) due to plaques in cervical & thoracic cord) Difficulty walking = sensory ataxia (broad, stamping gait & look down) Sensory disturbance Can be urinary symptoms End stage Spastic paraparesis Ataxia (with shaking movements) Optic atrophy Nystagmus Brainstem signs (opthalamoplegia) Pseudobulbar palsy (eating, swallowing, talking all affected, spastic tongue, speech like Donald duck, increased jaw jerk) Urinary incontinence Dementia Death – due to uraemia or bronchopneumonia • Discuss the natural history of relapsing-remitting and progressive forms of MS Relapsing-Remitting (70%) 2 recurrences of dysfunction over 3 preceeding years without evidence of progression Neuro 27 Full functional recovery between Remissions eventually incomplete à progressive disability Chronic Progressive (30%) Steady progression from outset No remission • Outline the typical examination findings associated with MS Fatigue, Depression Motor – weakness & spasticity Gait – spastic, ataxic (broad based stamping) Altered sensation – numbness / pins & needles Pain – trigeminal neuralgia, dysaesthesia Bladder – frequency, urgency, incontinence Bowel – constipation Sex – impotence Dysphagia Eye – uhthoff’s sign (defects of exercise / increased temp), optic neuritis (pain on eye movement), diplopia, nystagmus Cerebellum – ataxia Poor cognition, memory, dementia signs Vertigo • Outline the diagnostic usefulness of MRI, evoked potentials and CSF examination MRI – can be used to demonstrate the lesions CSF – Protein electrophoresis to look for oligoclonal bands (show inflammation). Also look at cell count Evoked potentials - to demonstrate sites of involvement To exclude other conditions: CXR Serum Angiotensin converting enzyme (sarcoidosis) Serum B12 Antinuclear Ab – SLE Antiphospholipid Ab • Understand the principles of treatment of: o o Immunosupression Short term – pulsed steroids for acute exacerbations that threaten function. No evidence for long term steroid use Long term – Azothioprine, Interferon Symptomatic management and rehab of spasticity, bladder problems, pain, paroxysmal symptoms, weakness, fatigue and depression Spasticity Physiotherapy Baclofen / Botox Chemical neuronectomy Ataxia Isoniazid / Clonazepam Dysaesthesia Carbemazepine / Gabapentin / Phenytoin / Amitryptyline Bladder symptoms Anticholinergics and intermittent self catheterisation Fatigue Amantadine / Modafinil / Amitryptline Impotence Sidenafil Neuro 28 Muscular Dystrophies • Describe the nature of primary disease of muscle distinguishing between myositis, myopathy and dystrophy. Myositis – inflammation of voluntary muscle Myopathy – any disorder of muscle Dystrophy – disorder arising from defective / faulty nutrition, metabolism or genetics • Outline the pathology and causation of the main muscular disease to include myotonic dystrophy, Duchenne dystrophy, Polymyositis and Inclusion body myositis Muscular dystrophies – diseases characterised by progressive degeneration and loss of muscle fibres without nervous system disorder Duchenne MD X-linked recessive (1/3 are spontaneous mutations) 1/3000 male infants Absence of dystrophin Usually obvious by 4 yrs old and death by 20 Difficulty standing (+ve gowers sign) Proximal limb weakness & calf pseudohypertrophy Myocardial involvement Severe disability by age 10 Increased Creatinine phosphate & characteristic muscle biopsy Physiotherapy reduces impact but no cure available Becker MD Milder form – also X-linked recessive Onset late childhood, early adulthood Myotonic dystrophy AD – becomes evident 20-50yrs Progressive distal muscle weakness, ptosis, weak & thinning face & SCM Myotonia (increased contraction, poor/slow relaxation) Cateracts, frontal baldness, cognitive impairment, dysphagia, cardiomyopathy, small pituitary, Hypogonadism, glucose intolerance Polymyositis Idiopathic inflammatory myopathy 5:1 black : white 2:1 female : male Rare in under 20s Symmetrical proximal muscle weakness Dysphagia & aspiration if pharyngeal involvement Arthralgia Difficult kneeling, climbing stairs, standing from seated Muscle tenderness on palpation Muscle atrophy Inclusion body Myositis Idiopathic inflammatory myopathy Slow relentless progression Muscle weakness à difficulty walking à frequent falls Dysphagia and risk of aspiration pneumonia Onsets 50-60yrs Asymmetry common, often in knee extensors, ankle dorsiflexors, wrist & finger flexors Neuro 29 Peripheral neuropathy • Define neuralgia and neuritis Neuralgia – paroxysmal pain along the course of a nerve Neuritis – inflammation of a nerve, with pain & tenderness over it, anaesthesia and paraesthesia over its area and paralysis, wasting and loss of reflexes • List the common causes of peripheral neuropathy including DM, toxic (alcohol & drugs), autoimmune, other metabolic and inherited causes Infection – HZV, HIV, leprosy, Lyme disease, syphilis Post Infection – (Guillain Barre Syn) – diptheria, campylobacter, CMV Chronic demyelinating polyradiculoneuropathy – immune mediated repeated de and remyelination à hypertrophied nerves Malignancy – paraneoplastic syndrome = auto Ab and cross reaction Metabolic – DM, RF, hypothyroid, hypoglycaemia, mitochondrial disorders Mechanical trauma – laceration (distal degeneration), entrapment & compression (CTS) Vasculitis – polyarteritis nodosa, RA, wegeners granulomatosis Vitamins – B12 / B1 / B6 / Folate deficiency, B6 excess Athero / arteriolosclerosis – ischaemic damage Hereditary – carcot-marie-tooth syndrome, porphyria, fabry’s disease, leukodystrophy Drugs & Toxins – chronic alcohol abuse (direct and vit def), arsenic, lead, mercuty, acrylamide, isoniazid (TB), nitroflurantoin (UTI), Vincristine (cytotoxic for leukaemia), amiodarone, chloroquine, cisplatin, dapsone, metronidazole, sulphonamides, thalidomide • Classify peripheral neuropathy into demyelinating, axonal and mixed types Demyelinating – slowed conduction +/- conduction block Focal – entrapment Multifocal – diptheria, leprosy Generalised – Guillian Barre, Hereditary, Lymphoma Axonal – reduced or absent action potentials, normal conduction velocity Focal – severe entrapment Multifocal – Diabetes, Vasculitis, Lyme disease Generalised – Alcohol, Drugs, Vitamin deficiencies • Outline the main clinical patterns of peripheral neuropathy, and describe the typical findings on examination Mononeuropathy – single nerve Multifocal mononeuropathy – multiple isolated single nerves Polyneuropathy – diffuse, bilaterally symmetrical Often get Loss of reflexes Distal weakness Glove and stocking sensory loss • Discuss the diagnosis of the likely cause of peripheral neuropathy using the relevant aspects of history, examination, electrophysiological and laboratory investigations First line FBC, ESR, Serum B12 U&E, Creatinine, calcium, LFTs, Blood glucose & HbA1c, TFTs, Plasma protein electrophoresis Neuro 30 VDRL (sexual infection screen), Serum autoantibodies Nerve conduction / EMG Second line Serum lipids, lipoproteins, toxic & drug screen, PSA, Porphyrin screen, Bence-jones proteins, Faecal occult blood Antiganglioside Ab, Antineuronal Ab Genetic screening, CXR, CT, Mammogram, Abdo imaging Occasionally useful Vitamin assays, nerve biopsy • Discuss the clinical presentation, diagnosis and management of Guillain Barre syndrome Immune mediated 2-4 weeks post infection with Diptheria, Campylobacter, CMV Damage to schwann cells à disrupts myelin à slows conduction à lymphocyte and macrophage infiltration à distal numbness & weakness, can get respiratory failure, recovery after 3-4 months Rx with support and immunoglobulin • Discuss the clinical features and management of shingles Reactivation of herpes zoster (assoc with decline in cell mediated immunity) In DRG (usually CN V & VII) Can à corneal scarring Can persist as postherpetic neuralgia Rx with acyclovir / valacyclovir Neuro 31 Cerebral palsy 2/1000 – commonest cause of handicap in children Cerebral pathology which is permanent and non progressive --> disordered movement & posture Occurs from conception to 5yrs Causes Congenital malformation – cysts, fusion defects, failure of migration of grey matter, aplasia During fetal life – infection, hypoxic ischaemia Birth – trauma, hypoxia Post natal – severe illness (meningitis, encephalitis), NIA, RTA, Near drowning, Cardiac arrest, toxins / poisons, kernicterus, hypoglycaemia Classification Spastic (70%) Damage to motor cortex or its connections --> clasp knife hypotonia, abnormal brisk tendon reflexes, ankle clonus. Can be Hemiparesis (one side, Arm > leg), quadriparesis (all, arms > legs) or diplegia (both legs > arms) Dystonic (athetoid) (10%) Irregular involuntary movements – usually slow purposeless muscle movements with extensor spasms May be continuous / only when voluntary movements Ataxic (10%) Hypotonic, weak, uncoordinated with intention tremor Mixed (10%) Mixture of different forms Clinical features Neonatal Poor suckling ability Increased / decreased muscle tone Abnormal reflexes Irritable / drowsy Convulsions Infant Development of hand preference before 1 yr Persistence of primitive reflexes (grasp, moro, stepping) Dystonic Floppy, delayed motor function, not evident until > 1yr Ataxic Hypotonic & delayed motor development +/- tremor Management Paediatrician Local nerve blocks Permanent phenol injection Botulinum toxin Baclofen - GABA agonist , Oral (poor BBB penetration), continous spinal infusion Physiotherapist Encourages normal muscle development Inhibits abnormal muscle development Prevents contractures Neuro 32 Orthopaedic surgeon Aims: Correct Deformity, Balance Muscle Power, Stabilise Joints Procedures: tendon lengthening, capsulotomies, fasciotomies, osteotomies, tendon tenotomy, muscle lengthening, tendon transfers, muscle rerouting, neurectomy Neurosurgery Longitudinal Myelectomy (spinal cord) Cortex Ablation (brain) Electrical stimulational (cerebellum and dorsal spinal cord) Selective dorsal rhizotomy - Section a portion of sensory nerve roots in the lumbosacral plexus thus altering anterior horn cell control in the spinal cord and reducing spasticity Occupational therapist Orthotics to brace weakened muscles & prevent contractures Mobility aids Learning & communication aids Speech and language therapist Social worker Teachers and play specialists Other problems Learning disorder – 60%. Rare in dystonic group Epilepsy – 30% Visual impairment – 20% (refraction errors, optic atrophy, cortical damage) Squint – 30% Hearing loss – 20% (often sensorineural, higher prevalence in dystonic group) Speech disorders Poor school performance Behavioural disorders Orthopaedic deformities – due to muscle weakness & spacsticity. Can get hip dislocations, equines deformity (requiring tendon release). Can get painful OA Neuro 33 Traumatic brain injury • Discuss the general management of the unconscious traumatised patient Immediate management ABC – ALWAYS assume ALL unconscious patients with head injuries have a spinal injury Assess GCS – intubate if < 8 – consider intubation if < 10 (contact anaesthetist) DEFG – Don’t ever forget glucose Arrange CT scan Specific management Anticonvulsant for seizures – phenytoin (try to avoid diazepam as depresses breathing) Send to ITU – cerebral swelling will be worst during 24-72 hrs Arrange ICP monitoring Nurse patient in 15o head up Aim to keep CO2 low (hyperventilate) Keep normo / hypothermic • Describe the Glasgow coma scale and discuss its value in neurological assessment. Eyes Open spontaneously To speech To pain None 4 3 2 1 Orientated Disorientated Inappropriate words Incomprehensible sounds None 5 4 3 2 1 Verbal Motor Obey command Localise pain Flexion to pain Flexion abnormal Extension None 6 5 4 3 2 1 Other useful definitions Coma – GCS < 8 Vegetative state – sleep wake cycles and emotions but unaware of surrounding. Brain damage but some consciousness recovered Akinetic mutism – due to frontal pathology. Aware and fully conscious but wont move – lost all motivation Locked in Syndrome – brain stem damage but still aware. Need ventilating. Can communicate using CN 3,4 & 6 Brain stem death – coma and absence of brain stem functions (pupils, caloric responses). NB: must exclude ALL reversible causes Psychogenic unresponsiveness – pretends to be in a coma. Test by letting arm fall onto face. Often respond if threaten with a large needle! • To appreciate the incidence and prevalence of traumatic brain injury in the UK 20% of all A&E attendances Peak age 20-25 M:F = 2:1 (most prevalent in younger age groups) High association with alcohol Causes Neuro 34 RTAs (50%) Domestic accidents (ladders, stairs) Sport Assault Industrial accidents Risk factors Previous head injury (with remaining impairments) Psychiatic disorder Epilepsy Lower socio-economic group Drug & alcohol abuse Young males Elderly prone to falls Poor prgnostic factors: Older age Longer duration of post traumatic amnesia Low GCS in acute stage Longer duration of Amnesia Other injuries Complications Alcohol / neurotoxins at time of injury Seizures • Describe the mechanisms of damage involved in TBI including primary and secondary insults Primary injury – due to acceleration-decelleration injury Diffuse axonal sheering injury damaging axons throughout the brain, inc CN roots Focal brain damage / laceration from sheering over bony prominences (natural or #) Secondary injuries Intracranial Haemorrhage – focal as haematoma or diffuse as contusion General oedema & raised ICP Infection Local toxin release – free radicals, excitatory amino acids Extracranial factors Hypercapnia – Normal range 3.5 - 5KPA. Increased levels à cerebral vasodilatation à increase in cerebral blood volume à increased intracranial pressure Hypoxia – Normal range 11 - 12KPA on air. Hypoxaemia à rapid lactic acidosis à neuronal damage. Severe hypoxia (<50%) à cerebral vasodilaton à further brain swelling occurs. Hypotension – Normally autoregulation maintains constant cerebral blood flow despite wide changes in blood pressure. After a head injury auto regulation is paralysed and the flow in the brain depends upon blood pressure. Therefore vital to maintain the patient's blood pressure to prevent secondary insults. Septicaemia – can affect blood flow and oxygen usage. Can also à oedema • Describe the main causes, pathophysiological mechanisms and effects of increased intracranial pressure. 5-15 mmHg = normal Neuro 35 > 30mmHg for > 5 minutes = worrying NB: Cerebral perfusion pressure = MAP – ICP So aim to keep ICP low, but if cant then raise MAP Clinical signs of raising ICP Decreasing consciousness Progressive dilatation of pupil on ipsilateral side (due to pressure on CN3) Hypertension with Bradycardia = Cushing reflex (due to pressure on medulla) Papilloedema doesn’t occur in acute head injuries Management Place catheter to measure pressure Mannitol for raised ICP – from haematomas or contusions. NB – Catheterise as à diuresis! Dexamethasone if raised ICP due to tumour oedema • Common injuries and their managements Common intracranial haematomas after a head injury are: Extradural – usually in young patients due to sporting injuries. Lucid interval. Usually due to pterion # à laceration to the middle meningeal artery Subdural – due to severe injury so worse prognosis. High incidence of epilepsy & brain damage Intracerebral (contusions / bruising) – due to coup & contrecoup injuries. Most often frontal and temporal lobes Compound (open) depressed skull fractures Usually due to missile injuries Dura may be torn (intracranial air) à increased infection risk (& epilepsy) Require exploration within 12 hours CSF leakage Usually dural tear with skull base # Most commonly from anterior cranial fossa à leakage down nose ALL have high risk of meningitis • To be able to explain what post traumatic amnesia (PTA) is Characteristic state in severe head injury cases Disorientation to variable degree – unable to form new memories • List at least three important cognitive difficulties after closed head injury Short term memory impairment Attention and concentration issues Speed of information processing Executive functioning (planning and organisational skills) • To list at least three important behavioural features after closed head injury Poor anger control Emotional lability Impulsivity, Disinhibition Depression, Anxiety Lack of insight, Apathy – towards rehab, Lack of spontaneity Neuro 36 Spinal cord injury • Describe the demography of spinal cord injury Mainly due to falls, RTAs, Sports, assault Injury mechanisms include flexion, hyperextension, rotation, compression and penetration • Outline the impairments at different levels of spinal cord injury Classification Complete injury – transverse ischaemic necrosis occurs --> permanent loss of movement & sensation below the level of the lesion Incomplete injury – some function remains – determined by which part of the cord is affected Anterior cord syndrome – commonly assoc with flexion injuries (due to injury / compression of anterior spinal artery --> loss of motor function & impairment of pain & temperature below the lesion. Posterior column functions are maintained Posterior cord syndrome – assoc with hyperextension injuries. Impairment of light touch, proprioception & vibration sense Brown-Sequard Syndrome – physiological hemisection of the cord (penetration or rotational injuries) --> ipsilateral loss of motor function, sense of vibration and proprioception AND contralateral loss of pain and temperature sensations Central cord syndrome – cervical spondylosis / cervical stenosis --> motor / sensory loss greater in upper limb than lower limb Autonomic dysreflexia – uncontrolled sympathetic output as an autonomic response to noxious stimulus below the injury – due to Injury at T6 or above -> Initial v/c --> HT --> sympathetic response --> v/d & bradycardia above lesion Symptoms – severe HT, bradycardia, pounding headache, flushed appearance above the injury level & pallor below, profuse sweating above the lesion, nasal congestion, goose flesh Common causes – distended bladder / blocked catheter, distended bowel, pressure sores, UTI / renal calculi, pain, pregnancy / labour, severe anxiety / distress, ingrowing toenails Management – sit patient upright / head up, loosen clothing, remove stimulus, BP at 5 minute intervals, GTN / Captopril if not resolving • State specific complications of spinal cord injury & interventions to prevent them Pathological process Initial minutes / hrs Release of vaso-active agents & cellular enzymes --> changes to microvessels in central grey matter & multifocal haemorrhages Infiltration of the site by neutrophils & macrophages --> postcapillary venule distension Increase in intracellular Ca --> damage to endovascular endothelium & leakage of RBCs into perivascular space Increasing extracellular K --> depolarisation of cells --> conduction block 4-8hrs Hypoxia induced catecholamines release & further haemorrhage & necrosis Elevated Ca --> appearance of aneurysms & vessel rupture in lateral columns of the spinal cord 24hrs Thrombi formation in capillaries 3-4 weeks Traumatised cord replaced by acellular scar tissue Neuro 37 Spinal Shock Transient condition with loss of all neurological activity below the level of the injury due to loss of supraspinal control. Caused by massive release of catecholamines at the time of the injury Begins 30-60 minutes after injury Can last 48hrs to 6 weeks Can --> Vasomotor and visceral paralysis --> urine retention Absence of muscular reflexes (flaccid paralysis) Disruptions to the sympathetic nervous system --> hypotension & bradycardia Disturbed temperature regulation --> Polkothermia Respiratory complications Main cause of mortality – dependent on the level of injury Impaired cough reflex --> partial obstruction and risk of pneumonia Inability to produce forced inspiration / expiration Chest infection Hypoventilation / Respiratory failure Assessment - Blood gases, Pulse Ox, Spirometry, Regular resp exam Haemodynamic complications SCIs above T6 are prone to bradycardia & AV block – worsened by procedures causing vagal stimulation (suctioning, position change) Hypotension – due to peripheral vasodilatation, penous pooling & decreased venous return Prolonged hypotension --> ischaemia --> MODS Gastrointestinal complications Paralytic ileus is common --> abdominal distension. Consider NG tube & H2 blockers to prevent ulcers • Specify the principles of acute management of the patient with spine or spinal cord injury. Objectives of acute management: Prevent death from assoc side effects of injury Prevent neurological deterioration Management of assoc injuries Avoidance of long term complications ABC with C-Spine immobilisation Arrange XRs of C-Spine (AP, Lat & AP Peg view), Chest and Pelvis (+ any ? # limbs) • Understand the pathological process, identification, management and prevention of pressure sores Pressure sore – an area of localised damage to the skin (can involve the underlying structures Pathology Direct unrelieved pressure – if P > capillary pressure à hypoxia Friction Shearing Risk factors Immobility / sensory impairment – spinal cord injury, post op, depression, pain, sedatives, coma, diabetic Neuro 38 Concurrent disease – anaemia, DM, ischaemia, jaundice, malignancy Poor nutrition Poor blood supply – hypotension, shock, dehydration Moisture – incontinence Common sites Heel & lateral malleolus Knee Ischium, greater trochanter, perineum & sacrum Elbow, scapula Occiput, ear Classification Stage 1 – redness that doesn’t subside after pressure is relieved. Epidermis is intact Stage 2 – damage to epidermis but no further Stage 3 – Full thickness of skin. Very difficult to heal Stage 4 – extends into muscle, tendon or bone Factors affecting healing Nutritional status Skin perfusion Age Concurrent illness Medication Wound environment, temperature & pH Infection & contamination Prevention measures Ideally move / turn patient every 2 hours Remove source of irritation Frequent visual checks Bladder & bowel management Pressure reducing mattress, chair etc Adequate nutrition & hydration Wound care & treatment of any infections Neuro 39 Spinal Cord & Nerve Root Compression • Define cauda equina syndrome, its presentation and management Compression of the nerve roots in the lower portion of the spinal canal Most commonly caused by a posterior disc herniation, then tumours Symptoms Faecal incontinence Urinary incontinence Sensory deficit over perineum Investigation Laxity of anal sphincter Urgent MRI Management Urgent surgical decompression (within 6 hrs of onset to avoid permanent damage) • Discuss the pathology, effects, presentation and management of herniated intervertebral discs Pathology Discs desiccate and degenerate with age --> increasing likelihood of annulus fibrosis tearing Most commonly occurs at L4-5 or L5-S1 discs THEREFORE LMN signs Causes / Precipitating events Injury Improper lifting Sx of posterolateral herniation Back pain – aggravated by activity, bending forwards, coughing & prolonged sitting. Muscle spasm – paraspinal mm due to pain --> loss of lumbar lordosis Sciatica - sharp and shooting pain down leg Paresthesia – pin prick reduction of Outer shin, dorsum of foot (L5) and/or side of foot (S1) Motor weakness --> foot drop (Tib Ant = L5) Loss of reflexes – ankle jerk (L5, S1) reduced NB: true posterior protrusion --> cauda equina syndrome Wasting – will only be there if very severe Fasiculation – is VERY rare Rx Anagesia – paracetamol --> NSAID --> opiate --> muscle relaxant / Antidepressant Physiotherapy Decompression – weakness should recover, reflexes wont • Discuss the cause, presentation and management of spinal stenosis Narrowing of the spinal canal due to degenerative disease of the spine leading to a decrease in diameter of the spinal canal and/or foramina (from facet joint and ligamentum flavin hypertrophy as well as disk Neuro 40 disease).Narrowing is most pronounced with the lumbar spine in extension, resulting in compression of spinal nerve roots. Usually 60s & 70s, M>F Most common site is L4-5, and L3-4 Manifestations Aching in back and both legs, most often posteriorly Occasion bilateral leg numbness / weakness / stiffness, or paresthesias Variable walking (pseudoclaudication) Sx present with standing and walking and relieved promptly by bending forward and supporting weight with arms or by sitting down Reflexes may be mildly decreased to absent Strength and sensation are most often normal Arterial pulses are intact unless arteriosclerosis is also present Rx: Exercise to restore spinal flexability and strenghten muscles Medication - analgesia Surgery - for disabling pain or progressive disease --> laminectomy • Discuss the cause, presentation and management of sponylosis Progressive degeneration of the spine – usually in the C-Spine. Most commonly C5-7 Disc degeneration with age --> loss of cushioning --> end plate reaction -> osteophyte Present in many people. Only Sx if also some degree of stenosis Can affect neural canal or intervertebral foramen Radiological (& pathological) features Loss of disc height Disc fibrosis Annular weakening Extra mobility may result in osetephyte formation – best seen on oblique view Symptoms Can --> brachial neuraligia (pain / numbness / weakness in arm) if affects intervertebral foramen OR … Cervical Myelopathy if affects spinal canal Subacute onset (usually > 6months) with progressive symptoms LMN lesion at level of lesion --> Numbness & clumsy fingers, reduced reflexes UMN lesion below lesion --> Difficult with walking (due to spacticity) = myelopathic gait, increased tone & increased reflexes LOOK FOR: Hoffman’s sign, ankle clonus Rx Attempt to stabilise joints • Discuss the cause, presentation and management of spondylolithesis Slippage of the vertebral bodies due to defect in pars interarticularis Neuro 41 Most commonly L4-5 or L5-S1 disc levels Sx Pain in lower back, thighs and/or legs Muscle spasms Weakness Exaggerated lumbar lordosis Waddling gait Causes Congenital, degenerative, traumatic, pathological and idiopathic Sx common between 7 & 20 due to sports (gymnasts, football linemen, soldiers with back packs) Severity Graded by the degree of slip Management Physio to strengthen the spinal & abdominal muscles Limitation of stressing activities Decompression and fusion of vertebra = last resort Neuro 42