Chronic Disease 2 notes PDF

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Notes for Chronic Disease 2
-Stroke
-MSK
-Dermatology ...

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Chronic Disease 2 Stroke A stroke is a sudden interruption in the vascular supply of the brain. • Clinical syndrome consisting of rapidly developing clinical signs of foal (at times global) disturbance of cerebral function, lasting more than 24 hours or leading to death with no apparent cause other than that of vascular origin • In contrast a TIA, the symptoms resolve within 24 hours Since neural tissue is completely dependent on aerobic metabolism, any problem with oxygen supply can lead to irreversible damage. There are two main types: • Ischaemic – blockage in the blood vessel that stops blood flow o Occurs in 85% of cases o Thrombotic stroke – thrombosis from large vessels like the carotid o Embolic stroke – usually a blood clot but fat, air or clumps of bacteria may act as an embolus. AF is an important cause o Risk factors: § Those for cardiovascular disease e.g. age, high BP, DM, hyperlipidaemia, smoking § Those for cardio embolism e.g. AF • Haemorrhagic – blood vessel bursts leading to reduction in blood flow o Occurs in 15% of cases o Intracerebral haemorrhage – bleeding within the brain o Subarachnoid haemorrhage – bleeding on the surface of the brain o Risk factors: § Age § Hypertension § Arteriovenous malformation § Anticoagulation therapy Signs and symptoms: • Motor weakness • Speech problems (dysphasia) • Swallowing problems • Visual field defects (homonymous hemianopia) • Balance problems • Cerebral hemisphere infarcts may also include: o Contralateral hemiplegia: initially flaccid then spastic o Contralateral sensory loss o Homonymous hemianopia o Dysphagia

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Brainstem infarction may result in more severe symptoms i.e. quadriplegia and lockin-syndrome Lacunar infarcts are small infarcts around the basal ganglia, internal capsule, thalamus and pons o This may result in pure motor, pure sensory, mixed motor and sensory signs or ataxia

Most patients that have suffered haemorrhages are more likely to have a decrease in level of consciousness, headache, nausea and vomiting and seizures. Oxford Stroke classification: classifies strokes based on initial symptoms. 1. Unilateral hemiparesis and/or hemisensory loss of the face, arm and leg 2. Homonymous hemianopia 3. Higher cognitive dysfunction e.g. dysphagia Stroke types: • Total anterior circulation infarcts - all 3 of above criteria are present o Involves middle and anterior cerebral arteries • Partial anterior circulation infarcts – 2 of the above criteria are present o Involves smaller arteries of anterior circulation e.g. upper or lower division of middle cerebral artery • Lacunar infarcts – presents with one of the following: o Unilateral weakness (and/or sensory deficit) of face, arm and leg or all three o Pure sensory stroke o Ataxic hemiparesis o Involves perforating arteries around the internal capsule, thalamus and basal ganglia • Posterior circulation infarcts – presents with one of the following: o Cerebellar or brainstem syndromes o Loss of consciousness o Isolated homonymous hemianopia o Involves vertebrobasilar arteries • Lateral medullar syndrome (posterior inferior cerebellar artery) – Wallenberg’s syndrome o Ipsilateral: ataxia, nystagmus, dysphagia, facial numbness, cranial nerve palsy e.g. Horner’s o Contralateral: limb sensory loss • Weber’s syndrome o Ipsilateral III palsy o Contralateral weakness

Investigations - the main two types of CT and MRI • CT head – ischaemic stroke – white in acute or dark grey chronically

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With haemorrhagic stroke, CT with contrast or MRI should be used

Management: • Urgent neuroimaging classifies the stroke as either ischaemic or haemorrhagic. If the stroke is ischaemic then the patient should be offered thrombolysis o Contraindications to thrombolysis: Absolute Relative Concurrent anticoagulation Previous intracranial haemorrhage (INR>1.7) Seizure at onset of stroke Haemorrhagic diathesis Intracranial neoplasm Active diabetic haemorrhagic retinopathy Suspected subarachnoid Suspected intracardiac thrombus haemorrhage Stroke or traumatic brain injury in Major surgery/trauma in preceding preceding 7 days in 2 weeks Lumbar puncture in preceding 3 months Gastrointestinal haemorrhage in preceding 3 weeks Active bleeding Pregnancy Oesophageal varices Uncontrolled hypertension >200/120mmHg • • •

Once haemorrhagic stroke has been excluded patients should be given aspirin 300mg as soon as possible and antiplatelet therapy should be continued If it is a haemorrhagic stroke, anticoagulants and antithrombotic medications should be stopped to minimise further bleeding. If cholesterol is >3.5mmol/l patients should be commenced on a statin

Assessment Stroke can be assessed by ROSIER score: • Loss of consciousness or syncope: -1 point • Seizure activity: - 1 point • Asymmetric facial weakness: +1 point • Asymmetric arm weakness: +1 point • Asymmetric leg weakness: +1 point • Speech disturbance: +1 point • Visual field defect: +1 point A stroke is likely if >0

Transient Ischaemic Attack Management: • Give aspirin 300mg immediately unless: o Patient has a bleeding disorder or is taking an anticoagulant (needs immediate admission for imaging to exclude a haemorrhage) o The patient is already taking low-dose aspirin regularly o Aspirin is contraindicated • Antithrombotic therapy: o Clopidogrel is recommended first-line o Aspirin + dipyridamole should be given to patients who cannot tolerate clopidogrel • Carotid artery endartectomy: o Recommend if patient has suffered stroke or TIA in the carotid territory and are not severely disabled o Should only be considered if carotid stenosis >70% according ECST criteria or >50% according to NASCET criteria

Dementia Progressive global decline in cognitive function, without impairment of consciousness. Causes: • Alzheimer’s o Associated with apo-e4 allele o General atrophy of brain tissue, particularly the frontal and temporal lobes o Excess deposition of beta-amyloid in the brain o Atrophy of cholinergic fibres • Lewy-Body o Presence of Lew bodies in the brainstem and neocortex o Associated with Parkinsonianism o Causes visual hallucinations, depression and sleep disturbance • Frontal lobe o Atrophy in the fronto-temporal region without histology seen in Alzheimer’s o Causes personality changes and impaired social function • Diffuse vascular disease (multi-infarct dementia) o Result of many small infarcts – so may be signs of other vascular problems such as high BP and past strokes o May also be pseudobulbar palsy, and shuffling gait with small steps Symptoms: • Poor memory (normally short-term) • Mild cognitive impairment • Visuo-spatial problems • Emotional disturbance • Loss of normal social behaviour • Poor sleep • Behavioural changes Investigations: • Clinical diagnosis • MMSE (mini=mental state exam) such as the MOCA • Bloods to rule out other differentials such as delirium, dehydration, infection Management: For Alzheimer’s: • Anticholinesterase drugs (donepezil, galantamine, rivastigmine) • NMDA receptor antagonists (memantine) • Antipsychotics typically at night For vascular dementia: • Prevention of vascular risk factors – aspirin/warfarin, controlling BP For Lewy body: • Anticholinesterase drugs (rivastigmine)

Parkinson’s disease Idiopathic Parkinson’s disease: progressive neurodegenerative condition caused by degeneration of dopaminergic neurons in the substantia nigra. More common in men and mean age of onset is 65 years old. Symptoms: • Bradykinesia o Short, shuffling steps with reduced arm swinging • Pill-rolling tremor o Most marked at rest and disappears with deliberate activity o Worse when stressed or tired • Rigidity o Cogwheel lead pipe • Slow speech • Plain face/facial stare – lack of emotion and reduced blinking rate • Depression, dementia and hallucinations can often be seen Investigations: • Clinical diagnosis Management: • L-Dopa Drug-induced parkinsonism normally presents with motor symptoms that are generally rapid onset and bilateral. Rigidity and rest tremor are uncommon. Caused by antipsychotics, metoclopramide.

Wilson’s disease Autosomal recessive disorder characterised by excessive copper deposition in the tissues. Metabolic abnormalities include increased copper absorption from the small intestine and decreased hepatic copper excretion. Age of onset is usually around 10-25. Symptoms: • Liver: hepatitis, cirrhosis • Neurological: basal ganglia degeneration, speech, behavioural and psychiatric problems • Kayser-Fleischer rings (dark rings around iris) • Haemolysis, blue nails, renal tubular acidosis Diagnosis is by reduced serum caeruloplasmin, reduced serum copper and increased 24hr urinary copper excretion. Management: penicillamine (chelates copper)

Common causes of Parkinsonism

Rheumatolo Rheumatology gy Polymyalgia rheumatica Inflammatory rheumatological syndrome especially in older patients. Vasculitis with giant cells that characteristically ‘skip’ certain sections of affected artery. More common in Caucasians and females Core inclusion criteria: • Age >50 years, duration of >2 weeks • Bilateral shoulder or pelvic girdle aching, or both • Morning stiffness duration of >45min • Evidence of an acute phase reaction Symptoms may also include mild polyarthralgia, lethargy, depression, low-grade fever, anorexia, night sweats.

Risk factors: • Giant cell arteritis, age >50 years, female, shoulder/hip girdle stiffness or pain, rapid response to corticosteroids Investigations: • Increased ESR (>40mm/hr), CRP, CK and EMG normal and reduced CD8+ T cells • Bursitis and joint effusions on ultrasound Treatment • Prednisolone 15-20mg OD o Taper down in the next 8 weeks. If they aren’t tapered, they can relapse again o If more than 20mg is used, then it is not PMR and other conditions need to be looked at o Normally used for 1-2 years • Follow up should include FBC, ESR/CRP, U+E and glucose Difference between polymyalgia rheumatica and fibromyalgia: younger, diagnosis of exclusion (blood tests are normal), more chronic and they are fatigued For PMR and GCA when prescribing high dose steroids consider steroid toxicity. Complications can include: • Osteoporosis • Steroid induced diabetes • GI bleed • Increased infection risk due to immunosuppression • Skin fragility

Giant cell arteritis Chronic vasculitis of large and medium vessels that lead to granulomatous inflammation. It primarily affects the branches of external carotid artery particularly the posterior ciliary arteries that supply the optic nerve and the superficial temporal artery. Most common >50yrs, in women and those with polymyalgia rheumatica symptoms Symptoms: • Headache - abrupt-onset, usually unilateral in the temporal area • Scalp pain and tenderness • Jaw claudication o Pain when eating in jaw • Visual symptoms – amaurosis fugax, partial or complete loss of vision • Systemic symptoms of fever, weight loss etc. Examination findings: • Transient or permanent reduction in visual acuity • Relative afferent papillary defect on swing light test • Pale, swollen disc with haemorrhages • Asymmetry of pulses and blood pressure • Bruit may be heard on auscultation if severe • Unpalpable temporal artery Investigations: • Bloods o Raised CRP and ESR (80-100), normocytic normochromic anaemia, abnormal LFTs (particularly ALP) o CXR o Urinalysis – check for protein/blood in glomerulonephritis • Temporal artery biopsy o Ideally a within a week of starting steroids o Shows granulomatous inflammation o Negative results should be interpreted clinically as it could be due to skip lesions • Ultrasound o Shows wall thickening – halo sign, stenosis or occlusion Treatment • High dose glucocorticoids o Uncomplicated GCA (no jaw claudication or visual symptoms): prednisolone 40 – 60mg daily until symptoms resolve or lab results are normal o Complicated (evolving visual loss or amaurosis fugax): IV methylprednisolone 500mg to 1g daily o Established vision loss: at least 60mg of prednisolone OD A CXR or angiography should be done every 2 years to monitor for aortic aneurysm.

Gout An acute, relapsing self-limiting severe inflammatory arthritis. When chronic, is associated with tophus formation and bone and joint destruction. Most commonly affects middle aged males and is associated with obesity, heavy alcohol intake, hypertension, renal impairment and diuretic use. Symptoms: • Pain - rapid-onset and severe o Most first presentations are the MTP joint (big toe) but can be others • Swelling • Erythema Investigations: • Arthrocentesis (joint aspiration) o High WCC, negative birefringent needle-shaped crystals under polarised light • Uric acid level o >416micromol/L (7mg/dL) in men or >360micromol/L (6 mg/dL) in women § Should be done 2 weeks after the attack resolves, as may be falsely normal or low • X-ray o Joint effusion o Well-defined ‘punched-out’ erosions with sclerotic margins o Erosions o No periarticular osteopenia o Soft tissue tophi • Ultrasound Management: Radiological features of gout Acute (decreases the inflammation): • NSAIDs or colchicine as first-line o PPI for gastric protection may be needed o NSAIDS should not be used in patients with renal impairment • Prednisolone tablets – 15mg/day • Intra-articular steroid injection Urate-lowering therapy: • Allopurinol (xanthine oxidase inhibitor) 100mg od and then dose titrated until uric acid levels 38.5 degrees C • Non-weight bearing • Raised ESR • Raised WCC Investigations: • Synovial fluid Gram stain and culture • Synovial fluid WCC • Blood culture • WCC Treatment: • IV broad spectrum antibiotics e.g. vancomycin and clindamycin • Analgesics • Repeat aspiration to dryness

Pseudogout A form of microcrystal synovitis caused by deposition of calcium pyrophosphate dihydrate. Risk factors include hyperparathyroidism, hypothyroidism, haemochromatosis, acromegaly, low magnesium, low phosphate, Wilson’s disease. Symptoms: • Painful and tender joints • Fever and malaise • Triggering factors e.g. trauma, intercurrent illness and surgical operation Investigations: • Arthrocentesis with synovial fluid analysis o Positively birefringent rhomboid-shaped crystals under polarised light Treatment: • Aspirate joint to dryness • Analgesia – oral NSAIDs or colchicine o An intra-articular steroid injection can be given when infection is excluded

Osteoarthritis A degenerative joint disorder that commonly affects the knees, hips, hands, lumbar and cervical spine. It results from the focal destruction of articular changes. Common in elderly and obese patients. Symptoms: • Joint pain and stiffness – improves with rest • Crepitus • Loss of function • Synovitis – causes joint swelling, palpable nodes • Heberden’s nodes (DIP) and Bouchard’s nodes (PIP) Investigations (can be diagnosed clinically): • X-ray o Loss of joint space o Osteophytes o Subchondral cyst formation o Sclerotic changes Management: • Oral analgesia • Intra-articular injections • Joint replacement • Physiotherapy

Psoriasis arthritis An autoimmune, inflammatory arthritis that is seronegative for rheumatoid factor that also has skin changes accompanying it. Often a genetic component (HLA-B27) and/or associated with rheumatoid arthritis and spondyloarthritides. Symptoms: • Oligoarthritis (2-5 joints, usually asymmetrical) • Psoriasis skin lesions - silvery plaques that are common at the knees and elbows • Psoriatic nail dystrophy o Discoloured nails (yellow/brown patchy colour), may also be onycholysis (detachment of the nail from the skin), pitting, ridging or subungual hyperkeratosis Investigations: • Synovial biopsy • X-ray – erosion in DIP joint, osteolysis and ‘pencil-in-cup’ deformity Treatment: • DMARD – methotrexate and leflunomide o Hydrocholoquine is avoided as it exacerbates skin disease

Rheumatoid arthritis A chronic inflammatory arthritis. More common in women around 30-50 years. Symptoms: • Swollen, painful joints in the hands and feet o Most commonly MCP, PIP, MTP • Stiffness that is worse in the morning • In severe arthritis, swan neck deformity, Boutonniere’s deformity, ulnar deviation and rheumatoid nodules can be seen Investigations: • Rheumatoid factor • Anti-CCP antibody • X-rays o Loss of joint space o Erosions o Soft tissue swelling o Soft bones (osteopenia) • Clinical diagnosis (American College of the Rheumatology criteria): o Morning stiffness >1 hour o >3 joints involved o Hand and wrist involvement o Symmetrical o Nodules o Positive RF o Radiological changes o Symptoms present for >6 weeks Management: • DMARDs o Methotrexate – monitoring of FBC and LFT due to risk of myelosuppression and livor cirrhosis o Sulfasalazine o Hydroxychloroquine • TNF-inhibitors • Rituximab Complications: • Respiratory: pulmonary fibrosis, pleural effusion, pulmonary nodules, bronchiolitis obliterans, methotrexate pneumonitis, pleurisy, • Ocular: keratoconjunctivitis sicca (dry eye syndrome), episcleritis (erythema), scleritis (erythema and pain), corneal ulceration, keratitis, steroid-induced cataracts, chloroquine retinopathy

Reactive arthritis An inflammatory arthritis that occurs post 1- 4 weeks exposure to certain GI and GU infections. Associated with HLA-B27. • GI infections include Shigella, Salmonella, Yersinia and Campylobacter • STI infections are normally Chlamydia – more common in men Symptoms: • Classical triad of urethritis, conjunctivitis and arthritis – “can’t see, pee or climb a tree” • Fever • Arthritis – asymmetrical oligoarthritis of large lower limbs • Dactylitis • Symptoms of urethritis • Skin changes – keratinous brown plaques on soles and palms, pustular vesicles Investigations: • Increased ESR and CRP • Positive stool sample and/or positive STI screen Management: • NSAIDs • Corticosteroids • DMARDs – second line when NSAIDs fail or in more chronic presentations Seronegative spondylarthroses are a group of conditions with overlapping clinical signs and association with the HLA-B27 gene. It includes ankylosing spondylitis, psoriatic arthritis, reactive arthritis, IBD. • They tend to mimic rheumatoid conditions but are serologically negative

Ankylosing spondylitis Chronic progressive inflammatory arthopathy. Typically presents in males aged 20-30. Symptoms: • Severe pain and spinal stiffness o Insidious onset, worse in the morning, improves with exercise • Anterior uveitis/iritis Investigations: • Pelvic x-ray – showing sacroiliitis (subchondral erosions and sclerosis), syndesmophytes (bony vertical lesions) and bamboo spine in late presentations Management: • Regular exercise • Physiotherapy • NSAIDs • DMARDs

Behcet’s syndrome Complex multisystem disorder associated with presumed autoimmune mediated inflammation of the arteries and veins. More common in Eastern Mediterranean, men and young adults. Associated with HLA B5. Symptoms: • Oral ulcers • Genital ulcers • Anterior uveitis • Thrombophlebitis • GI symptoms including cramping abdomen pain, diarrhoea or GI ulceration Investigations: • Diagnosis is based on clinical findings • Positive pathergy testing o A SC skin prick is made and a papule or pustule forms in 48 hours Management: • Oral topical corticosteroid for mouth ulcers • Corticosteroids + immunosuppressant such as azathioprine or infliximab for uveitis and GI ulcers

Ehlers-Danlos Syndrome is an autosomal dominant connective tissue disorder that mostly affects type III collagen à tissue being more elastic than normal leading to joint hypermobility and increased elasticity of the skin. • Symptoms: joint hypermobility, joint or spine pain, easy bruising, fatigue • Diagnosis: based on clinical findings normally in childhood • Management: avoid contact sports, pain management, physiotherapy • Complications: aortic regurgitation, mitral valve prolapse, aortic dissection, subarachnoid haemorrhage, angiod retinal streaks – bilateral, narrow, irregular lines deep to the retina

Marfan’s syndrome is an autosomal dominant connective tissue disorder, caused by a defect in the FBN1 gene. • Symptoms: tall stature, high-arched palate, arachnodactyly (long fingers), pectus exacatum, pes planus, scoliosis, dilation of the aortic sinuses (can lead to aortic aneurysm or dissection), repeated pneumothoraces • Management: ...