some important cases for paces

common cases in PACES

DIABETIC RETINOPATHY(41) microaneurysms, blot haemorrhages, hard exudates (Background), circinates of hard exudate near macula, poor acuity (Macular oedema), cotton wool spots, flame haemmorhages, leashes of new vessels, photocoagulation scars (proliferative), vitreous haemorrhage/scars, retinal detachment (advanced diabetic eye dx). may be bp, cateract. <indications for referral< all except background, refer background involving macula. indications for photocoagulation: pre-proliferative (cotton wool spots, multiple blots, venous beeding) or proliferative retinopathy. <LI> RHEUMATOID ARTHRITIS (31) symetrical deforming arthropathy, spindling of fingers. PIP, MCP swelling, DIP spared, muscle wasting, deformity, pain. nodules at elbow/dorsum hand, ulnar deviation, nail fold infarcts, arteric lesions. warmth and swelling sugests active inflam. swan neck, boutonnière deformity, Z thumb, palmer erythema, anaemia, episcleritis,

CVS/RESP signs. causes of anaemia: chronic dx, nsaids, myelosupressive drugs, feltys, assoc pernicious anemia. treatment: general (education, excercise, physio - thermal, splinting, diet), NSAIDs, DMARDs (1st line: methotrexate, sulphasalazine, gold, penicillamine, (hydroxy)chloroquin, 2nd line: azathioprine, cyclophosphamide, chlorambucil), corticosteroids (7.5 mg/day with calcium, bisphophanates), biological agents. <LI> EXOPTHALMOS (26) (protrusion of eye revealing sclera above lower lid on forward gaze). bilateral asymetrical swelling of medial caruncle, congestion of lateral canthus, and exopthalmos. look for pretibial myxoedema, thyoid acropachy. graves malignant exopthalmos: may be eu/hypo/hyperthyroid with severe exopthalmos, chemosis, exposure keratitis, corneal ulceration, painful opthalmoplegia (lateral, upward and convergent). due to muscle infiltration, oedema, fibrosis. sight threatening due to optic nerve pressure. Rx: steroids, tarsoraphy (scar), orbital decompression, radiotherapy. Causes: hyperthyroid graves dx, euthyroid graves dx, hypothyroid graves. asymetrical: cavernous sinus thrombosis, carticocavernous fistula (pusatile), unilat: retroorbital tumour, orbital celulitis (Hertel exopthalmometer) Treatment: lubricant eye drops, avoid smoking, wrap around glasses,

optimize thyroid status. radioiodine may worsen exopthalmos. <LI> ACROMEGALLY (24) prominent supraobital ridges, large lower jaw, exagerated wrinkles, full lips, malocclusion/prognathism, interdental spaces, nose/tongue/ears enlarged, kyphosis, large doughy spade hands, thick skin, carpal tunnel syndrome, excesive sweating, 1/3 hersuit, husky, cavernous voice, bitemporal hemianopia. Others: shoe/hat size, bowed legs, rolling gait, gynaecomatia, gallactorhoea, large/small testes, acne, greasy skin, skin tags (colonic polyps), acanthosis nigricans, osteoarthrosis, prox muscle weakness, cardiomegally (bp, cardiomyopathy), CN3 palsy. Associations: DM, bp, hyercaliuria, hypercalcemia (if MEN1=pipa=pituitry, islet cell, parathyroid, adrenal), urolithiasis, hyperTG, diabetes insipidus, hypopituitrism. Inv: IGF1, GTT absent GH supression, MRI pituitry, visual fields, old photos, SXR, ant pit tests (short synACTHen, ITT, TSH, T4, oestadiol, LH/FSH, testosterone, prolactin). Rx: trnasphenoidal/transfrontal hypophysectomy, external irradiation, gold/yttrium implants, bromocriptine (no shrinkage), somatostatin analogues (shink). cause:99% pit adenoma, 1% gangliocytoma <LI> CHRONIC LIVER DISEASE (21) icterus, pigmentation, clubbing, leuconychia, palmer

erythema, Dupytrens, spider naevi, flapping tremor, scratch marks, purpura, gynaecomastia, scanty body hair, small testes, hepatomegally, splenomegally, ascites, ankle oedema, distended abdo veins flow away +/- cyanosis from pulm-venous shunts. consider transplantation. causes alcohol, viral hepatitis, autoimmune CAH, haemochromtosis, cryptogenic. other: cardiac failure, constrictive pericarditis, Budd Chiari, 2ry billiary cirrhsis, drugs (methotrexate, mehydopa, isoniazid, CCl4, amiodarone, aspirin, phenytoin, propylthiouracil, sulphonamides), Wilsons dx, alpha1antitrypsin def, metabolic (galactosemia, tyrosinemia, glycogenosis4) <LI> FIBROSING ALVEOLITIS (21) sob, clubbing, steroid purpura, reduced symetrical expansion, fine inspiratory crackles (+/- cyanosis, dullnes lung base, LTOT) causes cryptogenic, rheumatoid, conn tissue disorders (systemic sclerosis, SLE, polymyositis, dermatomyositis, Sjorgens), ankylosing spondylitis, extrinsic allergic alveolitis, asbestosis, silicosis, drugs (bleomycin,buslphan, nitrofurantoin, amiodarone), radiation fibrosis, chemicals (Berylium, mercury), poisons (paraquet), ARDS. <LI> PROSTHETIC VALVES (17) Mitral valve prosthesis midline sternotomy, S1 prosthetic click, diastolic opening click

+/- mid diastolic flow murmur, pansystolic murmur/failure suggests leak. Aortic valve replacement midline sternotomy, normal S1, systolic opening click, prosthetic S2 +/- systolic flow murmur, early diastolic murmur/collapsing pulse suggests leak Complictions thromboemolism, haemorhage, endocarditis, valve leak, dehiscence, Starr-Edwards ball embolus, valve obstruction (thrombus/fibrosis), haemolysis. <LI> HEPATOSPLENOMEGALLY (17) spleen enlarged..cm below costal margin. Liver palpable ... cm below right costal margin. Check for anemia, lymphadenopathy, chronic liver disease and renal enlargement. Causes: myeloproliferative,lymphoproliferative (CLL, lymphoma), liver cirrhosis with portal hyperension, infections (hepB/C, EBV, CMV), zoonoses (brucella,weils, toxplasma), megaloblastic anemia, storge dx (gauchers, glycogen storage), amyloidosis, sarcoidosis,budd chiari=hep vein thrombosis, infantile PKD. worldwide causes: malaria, kala azar, schistosomiasis, TB. Bruit suggests hepatoma. <LI> PNEUMONECTOMY (16) chest wall deformity, with flattened L side associated ith L thoracotomy scar, L tracheal deviation, L expansion reduced, percussion dull, breath sounds diminished. Bronchial breathing L upper zone due to tracheal deviation. L

lower lobctomy deformity chest, L lower ribs pulled in, L thoracotomy scar, trachea central, apex displaced L, percussion dull L lower zone, breath sounds diminished. Indications for pnumonectomy 25% non small cell ca (2-6% mort), solitary pulm nodule of uncertain cause, treatment unresponsive bronchiectasis, TB in preantibioic era. <LI> RETINITIS PIGMENTOSA (16) widespread scattering of black pigment resembling bone spicules, the macula spared. There is tunnel vision and night blindness. Associaions: cateracts, deaf mutism, mental deficiency. Other causes of pigmentary degeneration Laurence Moon Biedl, Refsums dx, hereditary ataxia, familial nuropathy, neuronal lipidoses (ceroid lipofuscinosis). <LI> SYSTEMIC SCLEROSIS (15) middle aged female, skin smooth, shiny, tight fingers and face. Raynaulds, atrophy of finger tips, telangectasias face, pigmentation, nodules of calcinosis palpable on some fingers. other: skin ulcers, vitiligo, Sjorgens, diffuse intersitial fibrosis (leading cause of death) Rx vasoilators. other systems: CREST, renal (bp, failure), CVS (cardiomyopathy, effusion), muscle

(myositis, arthritis), intestinal hypermotility, PBC. Treatment: general (education, exercise, lubricants), Raynauds (calcium ch blockers, ACEi, proscycline, calcitonin GRP), oesophagus (PPI, prokinetics=cisapride), malabsoption (low residue diet, supplements, rotational abx), renal (ACEi, steroids), pulm vasc dx (vasodilators),fibrosis (dpenicillamine, corticosteroids). <LI> POLYCYSTIC KIDNEY DISEASE (14) bilateral flank masses, bilaterally ballotable, can get above them, percussion resonant, abdominal scars from CAPD/cyst aspiration, AV fistula, uremic facies, hypertension, renal transplant, parathyroidectomy. Complications: flank pain, bleeding, uti, nephrolithiasis, obstrucive uropathy. Other features: berry aneurysm, mitral valve prolapse, renal cell carcinoma. Genetics: PKD1 (Ch16, 85%, renal failure), PKD2 (Chr4, 15%), PKD3 rare. Liver cysts: rare in adult AD-PKD, common in infantile AR-PKD. Screening: U/S and genetic testing at 20 years (inconclusive before 20), earlier if FH aneurysm, signs renal dx. Bilateral renal enlargement: polycystic kidney dx, bilateral hydronephrosis, amyliodosis, tuberous sclerosis, von Hippel Lindau disease.

<LI> MITRAL REGURGITATION (13) pulse regular, JVP not raised, no ankle/sacral oedem unless cardiac failure, thrusting apex suggestng volume overload in 6 ICS, ant axilliary lne, possible systolic thrill, left parasternal heave, Soft S1, S3 suggests severe MR. Loud pansystolic murmur at apex radiates to axilla. MR with pulm bp. Causes: degenerative, rheumatic heart dx, severe LV dilatation, MV prolapse, papillairy muscle dysfunction, previous valvotomy for mitral stenosis, infective endocarditis, annular calcification, hypertrophic cardiomyopathy, chodrae rupture, congenital (marfans, erhler danlos, pseudxanthoma elasticum), endocardial fibrosis. Indications surgry: SOBOE despite ACEI,/diuretics, increased end systolic volume (> 30 ml/m2), serial echo progressive LV dilatation, valvuloplasty +/- annuloplasty preferable due to dec mort/no anticoagulation. Mitral vave prolapse: post leaflet LSE, ant eaflet over spine. <LI> SPLENOMEGALLY (13) mass left hypochondrium, cnnot get above it, there is a notch. It moves diagonally cross abdomen, percussion is dull over left lower chest and mass. I think this is a spleen enlarged at..cm. Causes: v.large:

CML (Ph chr t9;22), myelofibrosis, makaria/kala arar. Large (4-8cm):myeloproliferaive (CML, myelofibrosis), lymphoproliferative (lymphoma, CLL), cirrhosis with portal hypertension. Small (2-4 cm): myeloproliferative, lymphoproliferative, cirrhosis with portal hypertension, infections (EBV, hepatitis,SBE) <LI> NEUROFIBROMATOSIS (13) multiple (5+) neurofibromas, sessile pedunculated fibromata some soft others firm, some single others lobulated, some mobile subcutaneously lumps others nodules along peripheral nerves, cafe au lait spots, 2/3 axilliary freckling, Lisch nodules=iris harmatomas, retinal harmatomas, rib nochiing, mental retardation, epilepsy, renal art stenosis. complications: kyphoscoliosis, pressure effect (acoustic neuromas CN5-8, cerebellar signs), CN5 neuroma, spinal nerve root, sarcomatous change, lung cysts (honeycomb lung), pseudoarthrosis, plexiform neuroma. Can also have: glioma, meningioma, medulloblastoma. Genetics: aut dominant , NFT1=von Ricklenhausen (chr 17), NFT2 (chr 22) bilateral acoustic neuroma <LI> BRONCHIECTASIS (12) underweight, cyanosed, sob, finger clubbing, frequent productive cough, sputum pot, inspiratory ? heard with unaided

ear, localised crepitations, may be widespread ronchi.. causes: childhood infections (whooping cough, measels, TB), cystic fibrosis, bronchial obstruction (foreign body, carcinoma, sarcoid, lymphadenopathy and fibrosis), hypogammaglobulibemiam, allergic bronchpulmonary aspergillosis, Marfans, yellow nail, congenital (defomity, Kartageners) , smoking related copd (common) <LI> PSORIASIS (12) (11) Asymetrical arthropathy involving mainly TIP, pitting of fingernails, onycholysis, some nail plates thickened, hypetkeratosis under them.Patches of psoriasis over bony prominemces particularly elbows, knees, trunk, scalp, intragluteal cleft, behind ears, in navel. Plaques circular, well difined edges, red, silvery scaly surface. Other arthropathy: arthritis mutilans, Rheumatoid-like, oligo/mono artthropathy, ank spond treatment local sunlight, uv light, coal tar, dithranol, local steroids, calipotriol, PUVA (psoralen and uv light). Treatment systemic: acitretin (retinoid), antimetabolites (sulphasalazine, methotrexate, azathioprine, hydroxyurea). Analgesic anti inflam for arthropathy, intraarticular steroids. Chloroquin contraindicated worsens skin. Incidence 5% Caucasian, NW europe, USA. Uncommon in Japanese, N

American Indians, Japanese. <LI> OSLER WEBER RENDU (11) (heridatary haemmorhagic telangectasias) Telangectasias on face, around mouth, on lips, tongue, buccal and nasal mucosa and fingers. Anemia but no features of systemic sclerosis .complications: GI haemorhhage, epistaxis, hemoptysis 2ry to pulm AV aneurysms, conjunctival telagactesias (bloody tears), retinal detachment/haemorrhage, cirrhosis (telangectasias/transfusion), massive intrahepatic shunting Treatment: chronic oral Fe therapy, oestrogens to induce squamous metaplasia of nasal mucosa, low dose finbrinolytic agents (aminocaproic acid), pulse dye laser NOT cautery. Genetics: Autosomal dominant, several gene defects possible.. non contractile capillaries cause prologed bleeding.

<LI> PLEURAL EFFUSION (10) Pulse regular, JVP normal, trachea usually central,expansion normal, percussion stony dull at R/L base, diminished TVF, VR and diminished breath sounds. May be bronchial breathing over effusion. Causes Exudate

(>30 g/l, LDH >200) bronchial carcinoma, secondary malignancy, PE with infarction, pneumonia, TB, mesothelioma, rheumatoid arthritis, SLE, lymphoma. Transudate (<30 g/l) cardiac failure, nephrotic syndrome, cirrhosis. Other causes of dullness at lung bases raised hemidiaphragm, basal collapse, collapse/consolidation totally occluding distal airways, pleural thickening. (TB, asbestos, empeyema) Pleural bx: Abram's needle for tb/malignancy place in normal saline. Thoracoscopy for either diagnosis and pleurodesis <LI> PERIPHERAL NEUROPATHY (9) impaired light touch, vibration, joint position and pinprick in a stocking +/- glove distribution. causes: DM, carcinomatous neuropathy, vitB12 def, vit B def (alcoholics, poor nutrition, dialysis), drugs (isoniazid, vincristine, nitrofurantoin, gold, ethanbutol, phenytoin, hydralazine, metronidazole, amiodarone, chloramphenicol, cyclosporin), idiopathic, leprosy, other (guillin barre, PAN, amyloidosis, AIDS), rare (myxoedema, acromegaly, sarcoidosis, uremia, Lyme dx, tetanus, botulism, paraprotein, hereditary ataxia, Refsum's dx, arsenic posoning, POEM syndrome) Motor causes: carcinomatous, lead, porphyria, diptheria, Charcot Marrie Tooth. <LI> MIXED AORTIC VALVE DISEASE (9) Predominant AS

Regular slow rising pulse (possibly bisferens). JVP not elevated. Apex palpable 1 cm left of MCL, forcefull sustained heave (pressure overload). Systolic thrill apex, aortic area and carotid. Harsh ejection systolic murmur aortic area radiating to neck, soft A2, early diastolic murmur LSE loudest on leaning forward, in inspiration. BP low with low pulse pressure. Predominant AR regular pulse, large volume and collapsing (possibly bisferens). JVP not raised. Apex in ant ax line 6ICS thrusting (volume overload). Harsh systolic murmur aortic area radiating to neck, early diastolic murmur LSE loudest on leaning forward, in inspiration. BP. Management: Cardiac cath is gold standard, gradient on echo is unreliable in severe AR. Best treatment AV repair, surgery indicated for stenotic symptoms, lv compromise in regurgitation. Ex testing can be used except for severe AS.

<LI> MIXED MITRAL VALVE DISEASE (9) Predominant MS malar flush, left thoracotomy scar. Pulse irregularly irregular, JVP not raised. Tapping Apex, not displaced. Left parasternal heave. Loud S1, pansystolic murmur radiating axilla, loud P2, opening snap, mid dialstolic rumbling localised to apex. Predominant MR with pulm hypertension

left thoracotomy scar, pulse irregular. Apex thrusting (volume pverload), apex 6 IC ant ax line, soft S1loud pansystolic murmur LSE and or apex. Loud P3, in left lateral posn mid diastolic low rumble followed by opening snap. Management: Cardiac catheter study gold standard. <LI> TRANSPLANTED KIDNEY (9) Usually assoc APKD. Fullness in flanks, swelling under scar RIF. Bilateral flank masses bimanually ballotable (or 1 mass and 1 nephrectomy scar). Palpable rounded mass under the scar in RIF feels like kidney. Diseases leading to transplantation: DM nephropathy, hypertensive renal dx, glomerulonephritis. Difficult to transplant: HUS (recurrence possible, esp if on cyclosporin), sickle cell (increased crisis), systemic sclerosis (chornic vasular/gi problems), focal glomerulosclerosis (recurrence in graft common), oxalosis (severe recurrence of stone dx), cystinosis and Farby's dx (continued dx activity). 5, 10 yr outcome: HLA identicle living (88, 73), other living (74, 56), HLA matched cadaver (70, 56). <LI> GRAVES DISEASE (9) M:5F, hyperthyroid

graves usually female, thin, sweaty palms, fine tremor of outstretched hands, tachycardia, fidgety and nervous, small diffuse goitre with bruit, exophalmos (?chemosis, opthalmoplegia, lateral tasoraphy), lid lag. Euthyroid Graves: exopthalmos, thyroid acropachy, pretibial myxoedema, pulse regular with normal rate, palms not sweaty, no hand tremor, no lid lag. Previous thyroidectomy scar. Hypothyroid Graves: exopthalmos, goitre, thyroidectomy scar, pretibial myxoedema, thyroid acropachy, hypothyroid facies, hoarse voice, slow pulse, slowly reacting reflexes.. It is likely that she has hyperthyroidism treated in the past (?thyroidectomy or radioactive iodine) now on inadequate throxine replacement alternatively Graves occasionally develops autoimmune hypthyroidism. Ask some questions: heat intolerence, weight loss, increased apetite, diarhhoea, exertional dyspnoea, fatiguability, “can't keep still”, irritability, nervousness, menstrual symptoms., other signs: fever, systolic hypertension, vasodilation, flow murmur, proximal myopathy, hyperactive reflexes, childhood choreoathetosis, fine thin hair with temporal recession, onycholysis (Plummer's nails esp 4th

finger), palmer erythema, spider naevi, minimal hepatosplenomegally, axillary lymph nodes, osteoperosis, regardless of thyroid statud pulse may be regular or irregular. Graves can occur without eye signs and in elderly males. Graves exopthalmos due to retrorbital fat, enlarged intraorbital muscles, lymphocytic infiltration inc water and mucopolysacharide. Can develop without hyperthyroidism , course not linked to thyroid status. Pretib mxyoedema develops usually after hyperthyroidosm treated esp with radioiodine. Investigations: thyrotrophin receptor Abs, even radioiodine uptake with exception of nodules, new bone formation (acropachy) on xray = soap bubbles, coarse spicules cf HPOA(=linear new bone formation). Organ specific autoimmune dx thyroid, pernicious anemia, atrophic gastritis, DM, Addisons, hypoparathyroidism, premature ovarian failure, renal tubular acidosis, fibrosing alveolitis, chronic active hepatitis, primary biliary cirrhosis. Female propenderence assoc premature hair greying, alopecia areata, vitiligo. Autoimmune thyroiditis also assoc with Sjorgens, myasthenia gravis, systemic sclerosis, mixed conn tissue dx, cranial arteritis, polymyalgia rheumatica. Autoimmune thyroid dx's: Grave's, Hashimotos thyroiditis, atrophic myxoedema, usually FH of

other 2. all 3 fall under “hashitoxicosis”. <LI> RHEUMATOID LUNG (8) possibly cyanosis and dyspnoea. Fine inspiratory crackles aka crepitations on auscultation both bases. In view of rheumatoid changes in the hands the likely diagnosis is fibrosing alveolitis associated wirg rheumatoid disease. Comments: Develops overtly in 2% RA, subclinical in 50%, poor prognosis, progessing to honeycomb CXR, bronchiectasis, chronic cough, progressive dyspnoea. PFT reduced diffusion capacity and compliance, restrictive pattern. Gold can induce same interstitial lung dx but reverses on discontinuing drug. Pulm Rheumatoid dx: pleural disease (exudate, Rh F +ve, low glucose, WCC <5), intrapulmonary nodules (can become infected/cavitate/haemoptysis/rupture -> pneumothorax, massive confluent nodules in assoc with pneumoconiosis=Caplan syndrome), obliterative bronchiolitis (classucally dyspnoea, high pitched wheeze=squawk, hyperinflation), pulmonary arteritis, apical fibrobulous dx. <LI> MIXED MITRAL AND AORTIC DISEASE (8) If 1 murmur found LOOK FOR 2ND MURMUR e.g .Mixed aortic and mitral valve disease Left thoracotomy scar, malar flush, pulse irregularly irregular, slow rising, jvp not elevated, apex is heaving/lifting, etc in the abt ax line, parasternal heave, systolic thrill at apex, aortic area and

neck. S1 loud, harsh ejection systolic murmuraortic area radiating to neck, pansystolic murmur LLSE radiating to apex and axilla, early diastoli mumur just audible in aortic area and down LSE with patient sitting forward in expiration and opening snap followed by mid diastolic rumbing murmur localised to apex. Which lesion is dominant? Slow rising pulse suggests AS is the dominant aortic valve lesion, it is not possible to determine major mitral valve lesion. Further investigation with echo leading to TOE +/- cardiac catheterisation with LV angiography would be required to assess heamodynamic signifance of each lesion. <LI> MYOTONIC DYSTROPHY (8) Myopathic facies (drooping mouth, long lean, lifeless, sad, sleepy expression), male frontal balding, ptosis (may be unilateral), wasting of facial muscles (temporalis, masseter, sternomastoid, shoulder girdle, quadreiceps. When making a fist unable to open it quickly especially repeatively. Worsen in cold and excitement, difficulty opening eyes after firm closure. When shaking hands difficulty releasing grip (feature of myotonia). dimples/depressions induced by percussion fill slowly (percussion myotonia of tongue/thenar eminence). Genetics: Aut dom, expanded trinucleotine repeats Chr 19 myotonin protein kinas gene, shows anticipation (ancestors presenile cateracts), diagnosis by genetic test, M>F. Other features:

cardiomyopathy, intellect/personality deterioration, slurred speech combined with tongue/pharyngeal myotonia, testicula atrophy, diabetes mellitus (end organ unresponsiveness to insulin). myotonia congenita (Rhomsens's dx) myotonia without other features of dyotonic dystrophy. Normal refkexes, possible Herculean appearance ?involuntary isometric exercise. Aut dom/recessive ion channelopathies. <LI> CHRONIC OBSTRUCTIVE PULMONARY DISEASE (8) Emphysema thin man “pink puffer” appearance, nicotine staining of fingers. Tachypnoeic at rest, lip pursing, prolonged expiration, suprasternal notch to cricoid distance reduced suggesting hyperinflation (normally >3 finger breaths). Chest hyperinflated, expansion mainly verticle, tracheal tug. Accessory muscles respiration, indrawing of lower ribs on inspiration due to flattened diaphragm. Percussion hypersonant obliterating cardiac and hepatic dullness, breath sounds quiet in classical emphyema, wheezes are often heard if associated bronchial dx. SOMETIMES decreased breath sounds over the upper/middle/lower zoneof R/L lung raises possibility of emphysematous bulla. Chronic Bronchitis male smoker, foggy city, dust/fumes, frequent respiratory infection, classical “blue bloater”. Nicotine staining, stocky, centrally cyanosed, suffused conjuntivae. Hyperinflated ches evidenced by use of accessory muscles on inspiration and tracheal

tug, pulse 80 bpm, venous pressure not elevated (ankle oedema, hepatomegally if cor pulmanal present), trachea central, suprasternal notch to cricoid distance reduced. Expansion equal but reduced to 2 cm, percussion note resonant, auscultation: expiratory phase prolonged, widespread expiratory ronchi (and may be coarse crepitations). Forced expiratory time 8 seconds, no flapping tremor (unless severe hypercapnoea in which case fundi – papilloedema). Possibly cor pulmanale if ankle oedema. Definitions: Emphysema = pathological diagnosis, chronic bronchitis = cough productive of sputum on 3 months of the year in 2 consequtive years. COPD = obstructive spirometry. Causes of empysema: smoking (assoc with chronic bronchitis mixed centrilobular and panacinar), alpha1atitrypsin deficiency (young, lower zone, panacinar ?iceterus, hepatomegally), coal dust (centrilobular emphysema – simple coal dust pneumoconiosis – only minor abnormalities of gas exchange), Macloed's syndrome (Swyer-James rare unilateral emphysema following childhood bronchiolitis and subsequent impaired alveolar growth. <LI> CARCINOMA BRONCHUS (0,8) LOOK FOR RADIOTHERAPY TATOES Carcinoma bronchus with pleural effusion: Clubbing, nicotine staining, hard lymph node in R/L supraclaviculare fossa, pulse regular, JVP not elevated, trachea central, chest

expansion normal, persussion stony dull at R/L base, tactile fremitus, vocal resonance and breath sounds all diminished over area of dullness. Carcinoma of bronchus with radiation therapy: cachectic, radiation burn R/L upper chest, clubbing, noctine staining, pulse regular, JVP nor elevated, no lymph nodes. Trachea deviated to R/L, expansion diminished R/L upper chest, TVF and resonance increased over upper chest, dull percussion note, area of bronchial breathing. Likely radiotherapy for carcinoma bronchus causing collapse and consolidation of /L upper lung. Pancoast's syndrome: radiation burn to chest, lymph nodes palpable in R/L axilla, trachea central, chest signs normal, wasting of small muscles of R/L hand, sensory loss +/- pain over T1 dermatome (can be C8-T2), R/L Horner's syndrome (ptosis, myosis, anhydrosis, enopthalmos). This is pancoast's syndrome due to apical carcinoma involving the lower brachial plexus and cervical sympathetic nerves. Lobectomy: R/L thoracotomy scar, trachea deviated R/L/ R/L chest expansion diminished, percussion resonant, breath sounds harsher. R/L lobectomy for removal of tumour, resistant lung abscess or localised bronchiectasis. Complications : local effects:

SVC obstruction (?oeema face/upper extremities, suffusion eyes, fixed engorgement of neck veins, dilataio superficial veins), stridor (SVC obstruction, dysphagia). Metastases and their effects (pain, ?hepatomegally, neurological signs). Non metastatic effects: HPOA wrists/ankles, peripheral neuropathy, cerebellar degeneration, encephalopathy, priximal myopathy, polymyosistis, dermatomyositis, Eaton Lambert syndrome), SIADH, ectopic ACTH, PTH,PTHrP, carcinoid), gynaecomastia (?HCG secreting tumour?), thrombophlebitia migrans, non bacterual thrombotic endocarditis, anemia (usually normoblastic, occasionally leucoerythroblastic), pruritis, Herpes zoster, acanthosis nigricans, arythema gyratum repens (irregular wavy bands with marginal desquamation on trunk, neck and extremities). <LI> HYPERTENSIVE RETINOPATHY (0, 8) Narrow retinal arterioles (normal AV ratio 1:1), may be tortuous, varying calibre, increased light reflex (copper/silver wiring), AV nipping (all occur in aging, arteriosclerosis, hypertension). Flame haemmorhages and blot haemorrhage , cotton wool exudates (this constitutes grade 3 retinopathy and a diagnosis of malignant (accelerated) hypertension even without papilloedema), there is papilloedema (cerebral oedema or malignant hypertension – haemorrhages and exudates not necessary). This is grade 4 retinopathy. Causes

of hypertension: essential (94%), renal (4% - ?RAS, acute nephritis, PKD), endocrine 1% (cushing's, conn's, phaechromocytoma, acromegaly, hyperPTH, hypothyroidism, OCP), miscellaneous <1% (caorctation, polycythemia, acute porphyria, pre-eclampsia). cerebral tumour or raised ICP from any cause can cause 2ry hypertension (Cushing's reflex). If phaeochromocytoma screen for MEN2 and von Hippel-Lindau in patient AND families. <LI> AORTIC STENOSIS (7) Aortic stenosis: Pulse regular, small volume and slow rising, JVP not raised (unless cardiac failure), apex 1cm left of MCL 5ICS (i.e. Apex Normal or Slightly displaced unless cardiac failure) with forcefull sustained heave (pressure overload). Sysolic thrill aortic area, carotids +/- apices. Harsh ejection systolic murmur aortic area radiating to neck., soft A2 (or absent). Associated ejection click if valve bicuspid). Blood pressure low normal with decreased pulse pressure. Murmur quietens as dx progresse/cardiac failure. Look careflully for MS as both conditions obscure eachother. (MS missed AS severity unerestimated) need echo. Aortic sclerosis: normal pulse, apex undiplaced, barely palpable, no thrills, ejection systolic murmur not harsh or loud, only radiates faintly to neck. A2 normal +/- ejection click. This is aortic sclerosis (assoc with 50%

inc death CVS risk, no significant gradient on echo). Causes: 1. Rheumatic heart disease (often associated mitral valve dx or aortic regurg), 2. Bicuspid aortic valve (M 60's), 3. Degenerative calicification (in elderly, stenosis usually mild), 4. Congenital (worsen during childhood and adolescence due to calification). Indications for surgery: symptoms, pressure gradient 50-60 mmHg lower if EF low, may need simultaneous CABG, , consider valvotomy in young. Beta blockers as temporising measure. Av block in calcific aortic stenosis/post aortic valve operation/pacemaker insertion..Other causes of short systolic murmur: prolapsed mitral valve, trivial MR, hypertrophic cardiomyopathy. <LI> OCCULAR PALSY (3, 7) 6th nerve palsy: convjivergent strabismus, impairment lateral movement R/L eye, diplopia worse on looking R/L. (outermost image comes from affected eye). Causes mononeuritis multiplex, demyleinating dx, raised ICP false localising sign due to long course, neoplasm (assoc ipsilateral facial palsy if pontine tumour, myasthenia gravis, vascular lesions, compression by aneurysm (ectatic basilar artery – uncommon), subacute meningitis (carcinomatous, lymphomatous, fungal (AIDS), TB,

meningovascular syphilis) 3rd nerve palsy.: ptosis, lifting lids reveals divergent strabismus, dilated pupil, eye fixed in down and out position (and there is angulated dipliopia. This is complete (NB often partial) causes unruptured aneurysm of Post comm art (or int carotid) artery painful, mononeuritis multiplex, vascular lesion (contralateral hemiplegia = Webers synddrome, opthalmoplegia predominant=vascular if ptosis/pupil changes prominent extriinsic compression), 4. midbrain demyelinating lesion (intranuclear opthalmoplegia commoner), myasthenia gravis, other (sabacute meningitis, opthalmoplegic migraine, paraseller neoplasm, sphenoidal wing meningioma, carcinomatous lesions skull base) nerve palsy adducted eye cannoot look downwards causing “one above the other” diplopia, angulated diplopia on looking down and out, worse on reading or going down stairs, SKEW deviation (sometimes also seen in brainstem lesions). Cavernous sinus (Sup orb fiss) total or subtotal opthalmoplegia often painful, sensory loss in CN5i (absent corneal reflex) due to tumor compressing 3, 4, 5, 6 as travel from cavernous sinus to SOF. Causes of Mononeuritis multiplex: DM, PAN, Churg Strauss, Rheumatoid, SLE, Wegeners, sarcoidosis, carcinoma, amyloidosis, leprosy, Sjorgens, Lyme dx).

Other causes ocular palsy1. intranuclear opthalmoplegia (abduction impaired bilaterally, normal adduction or vice versa, no strabismus, ataxic nystagmus distguishes from bilat CN6 palsy), 2. Exopthalmic opthalmoplegia (upward and outward gaze most often reduced), 3. Myasthenia gravis (?ptosis, variable strabismus, facial weakness, snarling smile, proximal muscle weakness, weak nasal voice, WORSENS ON REPITITION, FATIGUABILITY test eye movements wuth eyes held 1 position between movements, count to 50 – may superficially resemble 3rd of 6th nerve palsy.,4. Cavernous sunus syndrome, 5. 4th n palsy, 6. Ocular myopathy <LI> MITRAL STENOSIS (7) Malar flush, left thoracotomy scar, irregularly irregular pulse in rate and volume OR sinus rythm small volume pulse. JVP not raised, no ankle or sacral oedema (unless in heart failure). Tapping cardiac impulse (S1), apex indisplaced, left parasternal heave, loud S1, loud P2, opening snap, mid-diastolic low rumbling murmur (with presystolic accentuation if NSR), localised to the apex heard loudest in left lateral position, diagnosis is mitrak stenosis with previous valvotomy. There are signs of pulmonary hypertension. Opening snap: nearer S2 = tighter stenosis, absent=calcified mitral

valve, s1 will be soft) Other signs: giant v waves (tricuspid incompitence), Graham Steele murmur (rare 2ry pulmary incompetence. Brief high pitched early diastolic whiff). Indications for surgery/ valvuloplasty:symptoms limiting normal activity, pulmonary iedena without cause, recurrent emboli (anticoagulate most MS's even if sinus rhythm), pulm oedema in pregnancy (emergency valvotomy), deterioration due to AF unresponsive to med rx), haemoptysis. valvotomy criteria: mobile valve: louds S1, opening snap absence of calcium in submitral apparatus on TOE, absence of MR. Esp in young female desiring later pregnancy. MS accentuated by exercise. <LI> GOITRE (7) 1) multinodlar goitre: multinodular goitre, R/L lobe more enlrged cf L/R. No lymph nodes, no retrosternal extension, no bruit, clinically euthyroid (check pulse, palms, tremor lid lag, tendon reflexes). Diagnosis in middle aged/elderlyy patient is likely to be simple multinodular goitre which is due to relative iodine deficiency in suscetible person. Multinodular suggests

longstanding. If no recent change asymptomatic observe 6-12 months otherwise FNAC.. Diffuse goitre firm, diffusely enlarged goitre, without retrosternal extension (check for bruit, feel pulse to assess thyroid status) Causes of diffuse goitre: 1. simple goitre (euthyroid, no bruit, relative iodine deficiency, esp females ?puberty, pregnancy), 2. treated Grave's dx (?exopthalmos +/- bruit, eu or hypothyroid), 3. hyperthyroid Graves (?bruit, tachycardia, exopthalmos, tremor, sweating, 3. Hashimito's dx (usually micronodular, symetrical, ?hypthyroid facies, bradycardia, jerks, 4. de Quervain's viral throiditis (tender, contitutional upset, absent radioiodine uptake , inc T4, supressed TSH), 5. goitrogens (e.g. Lithium, iodine xs, phenylbutazoned, para-aminosalysilic acid), 6. dyshormonogenesis (6 congenital enzyme defects all rare) solitary thyroid nodule check for lymphadenopathy. Causes solitary nodule: 1. palpable nodule in multinodular goitre, 2. thyroid adenoma (variable radioiodine uptake), 3. toxic adenoma (hot nodule, tacy, sweaty palms, lid lag), 4. thyroid cysts, 5. thyroid carcinoma (?hard, lymph nodes, recent change, cold on scan). check TFT's, FNAC, radioiodine scan (cold = possible malignancy), possible subtotal lobectomy. thyroid carcinoma: 1. papilliary carcinoma (commonest, children/middle aged. Regional lymph node spread, often resectable, good prognosis, often TSH

dependent so responds to thyroxine. 2. follicular carconimoa (2nd commonest, later life, blood borne mets, rx surgery, supressive thyroxine, fair prognosis, take up/respond to radioiodine therapy. 3. anaplastic carcinoma (elderly, highly malignant), 4. medullary ca (rare, young adults, secretes calcitonin, sometimes ACTH, usually good prognosis), 5. usually previous Hashimoto's, large rapidly expanding mass. MEN2a (sipple's) med ca thyroid, phaeochromocytoma, parathyroid hyperlasia (50%) MEN 2b(aka 3) med ca thyroid , phaeochromocyto,a, nuro abnormalties (mucosal neuromas, marfanoid, pigmentation, proximal myopathy, megacolon, intestinal ganglioneuromatosis. Both aut dom, RET oncogene, genetic screening – prophylactic thyroidectomy or annual calcitonin check, urine catecholamines, serum calcium) <LI> DIABETIC FOOT/CHARCOT JOINT (7) Diabetic foot ulcer on sole R/L foot (most commonly under head of 1st metatarsal), 2 toes previously amputated. Thick callous over pressure points of feet, normal concavity of transverse arch at head of metatarsals is lost. Loss of sensation to light touch, vibration and pinprick in glove and stocking distribution. Feet cold, foot pulses not

palpable, loss of hair lower legs which are shiny. Peripheral neuropathy, neuropathic ulcer, evidence periph vasc dx, it is likely underlying diabetes (?fundi). Charcot joint: relevent above plus ankle joint greatly deformed and swollen, loud crepitus accompanying movement which is of abnormal range. Charcot joint (neuropathic arthropathy – gross osteoarthrosis and new bone formation from repeated minor trauma without normal protective pain response causing painless joint destruction). Contributing factors to DM foot: injury, neuropathy (calluses), small vessel dx, large vessel dx (ischemia, gangrene), inc susceptibility to infection, maldistributed pressure and foot deformity. causes of neuropathic ulcers: tabes dorsalis, leprosy, porphyria, amyloidosis, progressive sensory neuropathy (familial, cryptogenic), rare late manifestation of Charcot-Marie-Tooth dx Causes of Charcot joint: DM, tabes dorsalis, syringomyelia, leprosy (worldwide), other (yaws, progressive sensory neuropathy, hereditory neuropathy, neurofibromatosis) <LI> OLD TUBERCULOSIS (6) Thoracoplasty: Trachea

deviated R/L. R/L upper chest shows deformity with decreased expansion, dull percussion note. Bronchial breathing and crepitations. Apex beat may be displaced to R/L. Thoracotomy scar posteriorly with evidence of rib resections. This patient has had R/L thoracoplasty for Rx of TB in pre-chemotherapy era. Apical TB trachea deviated R/L. Diminished expansion, crackles at R/L apex suggesting R/L apical fibrosis. Old TB is likely cause. Phrenic crush: Expansion diminished on R/L, dullness, reduced/absent breath sounds at R/L base. R/L supraclavicular scar (+/- crepitations). Patient has had phrenic crush for TB before the days of chemotherapy. <LI> HEMIPLEGIA (6) R/L UMN facial weakness, R/L arm and leg weak (without wasting), increased tone, hyperreflexia. R/L plantar is extensor, abdominal reflex diminisghed on R/L side. This is R/L hemiplegia. May be also R/L hemisensory loss, visual field testing may reveal R/L homonymous hemianopia. causes:-CVA (thrombosis, haemorrhage - check bp, embolism – AF, murmurs, bruits), Brain tomour (?insidious onset, papiklloedema, headache, ?evidence of 1ry e.g. Clubbing). posterior circulation nystagmus, ocular palsy, dysphagia, cerebellar signs. Parietal lobe signs:

agnosia (inattention) e.g. tactile (astereognosis=cant recognise by feel), visual, auditory (only recognises voice if can see/touch), autotopognosia (parts of own body), apraxia (cannot execute despite normal power/coord) e.g. dressing apraxia (usually r parietooccipital|), gait apraxia, trunk, facial movements.( Corpus collosum, parietal, premotor) Constructional apraxia (hepatic encephalopathy), dyslexia (reading), dysgraphia (writing), dyscalculia (calculating) – post parietal lobe. <LI> CEREBELLAR SYNDROME (6) unilateral cerebellar lesion: Nystagmus to R/L, ataxia with eyes open evidenced by rapid alternating movements (dysdiadokinesia). Finger-nose test impaired on R/L.with past-pointing, intention tremor (increased on approaching target), heel-shin tetst impaired on R/L, gait ataxic with tendency to fall R/L. Ataxic dysarthria with explosive staccato speech. Patient has a R/L cerebellar lesion. Other cerebellar sugns: ipsilateral hypotonia, reduced power, pedular jerks, skew deviation eyes, pendular arm drift rebound. Cerebellar vermis lesion: widebased cerebellar ataxia and robergism same eyes open and closed (cf sensory ataxia which is wirse eyes closed). Little or no abnormality in limbs when tested on bed. Causes: 1. mutiple sclerosis (?internuclear opthalmoplegia, optic neuritis,

atrophy, etc), 2. brainstem vascular lesion, 3. posterior fossa SOL (papilloedema e.g. tumor, abscess e.g. 2Ry to otitis media), 4. paraneoplastic cerebellar syndrome (clubbing, cachexia, 5. alcoholic cerebellar degeneration (nutritional), Freidrich's ataxia (?scoliosis, pes cavus, pyrimadal and dorsal colum signs, absent ankle jerks). Other causes of cerebellar ataxia: hypothyroidism (?facies, pulse, reflexes), anticonvulsant toxicity (esp phenytoin nystagmus), ataxia telangactasia (recessive progressive ataxia, choreoathetosis, ocular apraxia, telangectasia conjunctivae, face, skin, low IgA infections, lymphoreticular malignancy). <LI> TOPHACEOUS GOUT (6) Asymetrical swelling small joints of hands and feet, tophi formation in perarticular tissues. Occasional severe deformity. Tophi on helix of ear and some of the tendon sheaths (esp ulnar surface forearm, olecranon bursa, achiles tendon and pressure points). treatment: avoid aspirin causes uric acid retention unless v high doses. Rx 1st line NSAIDs (except if CRF, GI ulcer, severe heart failure), colchicine (SE: nausea, diarrhoea, abdo pain), 2nd line: intrarticular/systemic steroids. Allopurinol or uricosuric (probenecid, sulphinpyrazone) drugs started 2-3 weeks after acute episode (otherwise prolong acute attack, trigger further episides). Indictations for prophylaxis if: recurrent, tophi, chronic arthritis, renal dx, young with hyperuricemia FH renal/heart dx, normo-uricaemia

not achieved by lifestyle – i.e. wt loss, decease alcohol, food and high purines). Significance of tophi Tophus proportional severity and duration. Paradoxically tophi indicate dec frequency, severity cf non tophacouus. Large tophi may have area of necrosis exuding pasty/chalky monosodium urate crystals. Sinuses possible.Complications: renal dx, carpal tunnel syndrome. Associations: obesity, hyperlipidemia, hypertension these cause an association with DM and IHD.causes of 2ry uricemia: drugs (esp thiazides, also ethanbutol, nicotinic acid, cyclosporin), myeloproliferative and lymphoproliferative disorders (cell tunover preformed purines), chronic renal failure, alcoholism, obesity. <LI> RASH OF UNCERTAIN CAUSE (6) Rash description routine. Visual survey, distribution, surrounding skin (scratch marks, radiotherapy field marks, paper thin skin). Adequate exposure. Monomorphic or pleomorphic. 6 features. 1. colour (erythema, pigment), 2. size, 3. shape (oval, circular, annulaar), 4. surface (scaling or eroded), 5. character (papule, macule, vesicle, etc), 6. secondary features (lichenification, crusting, etc). Discussion: DD, how you would differentiate, mention drug hx. Emolients, abx for infection. Consider biopsy.

Dermatology terms: macule=flat curcumscribed, not raised, papule=raised, circumscribed, <1cm, nodule=larger papules, weals=circumscibed elevations itchy, tingly, vesicles=small well defined fluid collections (bullae=large vesicles), scales, crusts , scars, pustules (contain puss),

 ulcers have: shape, edge, floor, base and secretion. <LI> HYPOTHYROIDISM (6) myxoedema Overweight, myxoedematous facies (thickened and coarse facial features, periorbital puffiness and pallor). Skin rough, dry and inelastic with distinct yellowish tint (carotenemia), generalised non pitting swelling subcutaneous tissues, hoarse voice, croaking, hard of hearing, slow movements, thinning of hair which is dry and brittle, may be loss of outer third of eyebrow (not reliable). Slow pulse (give rate), no palpable goitre, relaxation of ankle jerks (and other reflexes) is delayed and slow. Patient has myxoedema (look for assoc autoimmune dx). Other symptoms: CNS disorders: peripheral neuropathy, cerebellar ataxia, pseudodementia, drop attacks, epipepsy. myxoedema=accumulation hyaluronic acid binding water in dermis Hasimoto's thyroiditis. as above + symmetrical firm, fine micornodular goitre, likely diagnosis is Hasimotos thyroiditis (assoc autoimmine dx). Hypothyroid

Grave's dx as above + exopthalmos, patient likely to have Grave's dx treated with radioiodine or thyroidectomy (scar) and is now hypthyroid (occasionally Grave's sponteously progresses to hypothroidism). ask patient some questions: deafness and hoarse voice, cold intolerence, tiredness and depression, constipation (occasionally presents to surgeons), angina unmasked once rx started, menhorhagia (middle aged), 1ry/2ry amennorhea (younger). Other feature: anemia (normochromic Fe deficient due to atrophic gastritis, magaloblastic – pernicious anemia, mild form in siimple hypothyroidism), carpal tunnel, preipheral cyanosis (+/- malar flush), Raynaud's phenomenon, hypertension, accident prone, hypothermia, Hoffman's syndrome (pain, ache, swellin muscles after exertion and myotonia), pychosis (myxoedema madness), hypothyroid coma. Assoc autoimmune dx: pernicious anemia (?spleen, SACDC), Addisons dx (?buccal + scar pigmentation), vitiligo, Rheumatoid arthritis (?hands, nodules), Sjorgens (?dry eyes and mouth), ulcerative colitis, idiopathoc (presumed autoimmune ?CAH with icterus etc), SLE (?rash), haemolytic anemia, diabetes mellitus (?fundi), Grave's dx, hypoparathyroidism, premature ovarian failure. Comments: should check B12, fasting sugar. Autoimmune thyroiditis presents as

case 1 or 2. <LI> OPTIC ATROPHY (6) Disc pale, clearly delineated, pupil reacts consensually to light but not directly. Field testing with head of hat pin reveals central scotoma. Well defined disc edge suggests not 2ry to papilloedema (yellow grey disc blurred margins). V early optic neruritis direct pupil reflex sluggish not absent – check Marcus Gunn phenomenon (diret reflex, then rapidly alternate pupil and will slowly dilate). Causes: 1. mutiple sclerosis (temporal pallor only ?nystagmus, scanning speech, cerebellar ataxia, etc), 2. compression of optic nerve by a) tumour (pituitry ?bitemporal heminaopsia)) b) aneurysm. 3. Glaucoma (?pathological cupping), 4. Optic atrophy in DM assoc with DID-MOAD (Diabetes insipidus, DM, deafness rare recessive inheritence), 5. Other causes: ischemic optic atrophy (abrupt visual loss in elderly +/- pain; thrombosis or embolus of post ciliary art; temporal arteritis), Lebers optic atrophy (6M:1F), retinal artery occlusion, toxic ambylopia (lead, methyl alcohol, arsenic, insecticides, quinine), nutritional ambylopia (famine, etc, tobacco-alcohol ambylopia, B12 deficiency, DM), Freidrichs ataxia, tabes dorsalis, Pagets dx , consecutive optic atrophy (2ry to ganglion cell destruction) <LI> AORTIC REGURGITATION (5) Regular pulse, large volume, collapsing in character. JVP not raised, vigourous arterial pulsations in neck (Corrigan's sign), apex thrusting (volume overload) in ant axilliary line, 6 ICS, high pitched early diastolic murmur audible down LSE and aortic area,

louder on expiration, sitting forward. Wide pulse pressure e.g. 250-300/30-50). Look for cause: high arched palate, aryl robertson pupils, arthropathy/ank spond, if none then Rheumatic/IE likely. Other signs: de Mussett sign (head nods), Quinkes signs (visible cap pulsation), Duroziez's sign (fem art auscultated when compressed distally, diastolic murmur = retrograde flow). Austin flint murmur=severe aortic incompetence (regurgitant jet interefes with mitral valve). Causes: Rheumatic fever, Infective endocarditis, longstanding hypertension (aortic dilatation, aneurysm), Marfans syndrome (tall extermities, arachnodactyly, high arched pakate), Ank spond (male, fixed kyphosis, question mark), Rheumatoid arthritis, coarctation of aorta (inn assoc with biscupid aortic valve), associated with mermemranous VSD, syphilitic aortitis (argyll robertson pupils, asc aortic anerysm), Hurlers syndrome. Indication surgery: aim to replace valve before serious LV dysfunction occurs. Serial echoes, radionuclear angiography, mild/moderate dx: ACEinhibitors, calcium antagonists decrease rate of deterioration. Replace valve urgently for infective endocarditis. <LI> HEPATOMEGALLY (5) Liver palpable at x cm below right costal margin (?icterus, ascites, signs of cirrhosis esp gynaecomastia, pigmentation, lymph

nodes). Common causes: cirrhosis – usually alcohol (?spide naevi, gynaecomastia, etc.), secondary carcinoma (?hard nobly, cachexia, evidence of primary), congestive cardiac failure (?raised JVP, ankle oedema, S3/murmur, tender pulsatille liver wiith giant v waves in tricuspid incompetence) Causes of hard, nobbly hepatomegally Malignancy – 1ry or 2ry, polycstic kidneydx (?kidneys?), macronodular cirrhosis (after hep B with widespread necrosis), hydatid cysts (eosinophylia, rupture -> anaphylaxis), syphilitic gummas (late benign syphilis usually hepatosplenomeggaly and anemia, rapid response to penicillin). Other causes of hepatomegally: infection (hep A/B, glandular fever, Weils dx), primary tumours (hepatoma, adenoma), lymphoproliferative (?lymph nodes), PBC (?middle aged female, scratch marks, xanthelasma), haemochromatosis (male, slate grey), sarcoidosis (?erythema nodosum, lupus pernio, chest signs), amyloidosis (?rheumatoid artritis or other chronic dx), hydatid cyst (?welsh connection – Nb patients name), amoebic abscess (?tropical connection ?name ?appeaance), budd chiari syndrome (?icterus, ascites, tenser hepatomegally), Reidels lobe, emphysema (apparent hepatomegally). <LI> CHARCOT MARIE TOOTH DISEASE (5) Distal wasting of lower limbs, relatively preserved thighs. Pes cavus, clawing of toes, weakness of extensors of toes and feet. Ankle jerks are absent, plantars no response. Only slight distal sensory sensoty loss, lateral popliteal ?and ulnar nerves palpable

(in some families only), steppage gait due to bilateral foot drop. May be wasting of small muscles of hand. Commentry: Variable inheritance. deformity>>disability. Rarely: Talipes eqionovarus, toe retraction, fasciculation. Mainly motor nerves, also dorsal roots/columns, pyramidal tracts slightly effected. Condition becomes arrested in midlife. FH: formes fruste (minor changes e,g, pes cavus, absent ankle jerks only). Inverted champagne botle legs is more neuromythology.There is slow progression up the limbs. <LI> MOTOR NEURONE DISEASE (5) Weakness, wasting, fasciculation of hand, arms, shoulder, but upper limb reflexes are exagerated (Progressive muscular atrophy – minimal pyramidal signs can be inc, dec, absent). There is upper motor neurone spastic weakness with exagerated reflexes in the legs. (amyptrophic lateral sclerosis). Ankle clonus, bilateral extensor plantars, may have indistinct nasal speech, fasciculating tongue and palatal paralysis (progressive bulbar palsy). There are no sensory signs. Other causes of fasciculations: cervical spondylosis, syringomyelia (dissoc sensory loss), Charcot Marie Tooth (champagne bottles, pes cavus, palpable nerves), acute stages of poliomyelitis rarely in old polio, neuralgic amyotrophy (eg. Following viral infection), thyrotoxic myopathy, syphilitic amyotrophy, chronic symetrical spinal muscular atrophy, after exercise in fit adults, benign giant fasciculations. DD of MND:

cervical cord compression (Cx MRI), syphilitic myopathy, occasionally old polio, spinal muscular atrophy juvenile onset type 3 (Kugelberg Welander dx) survival motor neurone gene defect. <LI> ULNAR PALSY (5) Generalised muscle wasting of hands (dorsal guttering, loss hypothenar eminence), weakness sparing the t

some important cases for paces

Documents