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PACES NOTES

PACES NOTES

INVESTIGATION AND MANAGEMENT

INVESTIGATION AND MANAGEMENT

Subhankar Chatterjee, Kolkata Subhankar Chatterjee, Kolkata 5/1/2017

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From my experience it was obvious that in Station 1 and 3 examiners want to hear quick and sensible investigation From my experience it was obvious that in Station 1 and 3 examiners want to hear quick and sensible investigation and management plan, which most of us fail to utter in timely and systematic fashion. They have 4mins after we finish and management plan, which most of us fail to utter in timely and systematic fashion. They have 4mins after we finish the case. The usual questions which they ask are as

the case. The usual questions which they ask are as follows-1.

1. Tell me the positive clinical finings- You should take no more than 30-40 sec for thisTell me the positive clinical finings- You should take no more than 30-40 sec for this 2.

2. What are the differential diagnosis- It may take some time as you may be interrupted if going in wrongWhat are the differential diagnosis- It may take some time as you may be interrupted if going in wrong direction. But never > 1min.

direction. But never > 1min. 3.

3. Which investigations would you do for this Which investigations would you do for this patient?- 40 secpatient?- 40 sec 4.

4. How do you manage the case?How do you manage the case? – –  Again 40-60 sec. Again 40-60 sec. If you have answered all

If you have answered all of the above in of the above in correct manner, you may have further questions which will fetch you correct manner, you may have further questions which will fetch you towardstowards  full marks. S

 full marks. So, time management o, time management is very imporis very important.tant.

I have made this note for my third attempt of PACES, which I passed. During exam felt quite confident as I have I have made this note for my third attempt of PACES, which I passed. During exam felt quite confident as I have rehearsed it many times with my friends, juniors and even before mirror. It might seem inadequate, but believe me, rehearsed it many times with my friends, juniors and even before mirror. It might seem inadequate, but believe me, you have small time to

you have small time to answer each question.answer each question.

This is a template only made for me and not all inclusive. I request all future examinees to prepare their own such This is a template only made for me and not all inclusive. I request all future examinees to prepare their own such notes which might be

notes which might be more effective if made according to one’s own ability, more effective if made according to one’s own ability, memory and concept.memory and concept.  At

 At the the end end I I have have tried tried to to simplify simplify some some Neurology Neurology scenarios scenarios in in a a schematic schematic pattern, pattern, which which helps helps in in patternpattern recognition during examination.

recognition during examination.

Obviously this note carries contribution from all of

Obviously this note carries contribution from all of my teachers, mentors, books and notes targeting my teachers, mentors, books and notes targeting PACES.PACES. Best of luck for PACES.

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Respiratory system Respiratory system

1.

1. COPDCOPD

Sir, my INVESTIGATIONs include : Sir, my INVESTIGATIONs include :

 Blood for FBC to look for Blood for FBC to look for PolycythemiaPolycythemia 

 Differential count, ESR, CRP- to Differential count, ESR, CRP- to look for signs of infection,look for signs of infection, 

 Chest x ray /HRCT Chest x ray /HRCT to look for hyperinflation to look for hyperinflation and Bullaand Bulla 

 Pulmonary function test to look for Obstructive pattern with Pulmonary function test to look for Obstructive pattern with reduced DLCOreduced DLCO 

 ECG and Echocardiography to rule out Cor ECG and Echocardiography to rule out Cor pulmonalepulmonale 

 Sputum microscopy and C/S to isolate any organismSputum microscopy and C/S to isolate any organism 

 6min walking test for functional assessment6min walking test for functional assessment 

 Arterial blood Gas to decide about LArterial blood Gas to decide about LTOTTOT

My TREATMENT plan include: My TREATMENT plan include:

 General measuresGeneral measures

o

o Stop smokingStop smoking o

o Pulmonary rehabilitation ( Pt education, Nutrition, Pulmonary rehabilitation ( Pt education, Nutrition, Psychological support, Physiotherapy)Psychological support, Physiotherapy) o

o VaccinationVaccination

 Specific medicalSpecific medical

o

o Bronchodilator- inhaled B agonist/ ICS/ LABA/LAMABronchodilator- inhaled B agonist/ ICS/ LABA/LAMA o

o Antibiotic and systemic steroid for acute exacerbationsAntibiotic and systemic steroid for acute exacerbations o

o MucolyticsMucolytics o

o LTOTLTOT o

o Diuretics and Phosphodiesterase inhibitors for Diuretics and Phosphodiesterase inhibitors for Cor pulmonaleCor pulmonale

 Surgery-

Surgery-o

o Bulectomy/ Lung volume reductionBulectomy/ Lung volume reduction o

o Lung transplantLung transplant

2.

2. BronchiectasisBronchiectasis

 InvestigationInvestigation

o

o Blood-Differential count, ESR, CRP- to look for signs of infection, ImmBlood-Differential count, ESR, CRP- to look for signs of infection, Imm unoglobin levelsunoglobin levels o

o Chest x ray /HRCT Chest x ray /HRCT to look to look for Tram for Tram line appearance/Signet line appearance/Signet ringring o

o Sputum microscopy and C/S to isolate any organism, particularly PseudomonasSputum microscopy and C/S to isolate any organism, particularly Pseudomonas o

o Pulmonary function test to look for Obstructive patternPulmonary function test to look for Obstructive pattern o

o ECG and Echocardiography to rule out Cor pulmonaleECG and Echocardiography to rule out Cor pulmonale o

o 6min walking test for functional assessment6min walking test for functional assessment o

o Sweat test if suspect cystic fibrosisSweat test if suspect cystic fibrosis

 ManagementManagement 

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o

o Stop smokingStop smoking o

o Pulmonary rehabilitation ( Pt education, Nutrition, Pulmonary rehabilitation ( Pt education, Nutrition, Psychological support, Physiotherapy, POSTURALPsychological support, Physiotherapy, POSTURAL

DRAINAGE) DRAINAGE)

o

o VaccinationVaccination

 Specific medicalSpecific medical

o

o MucolyticsMucolytics o

o Bronchodilator- inhaled B agonist/ ICS/ LABA/LAMABronchodilator- inhaled B agonist/ ICS/ LABA/LAMA o

o Antibiotic- systemic Antibiotic- systemic / inh/ inhalational alational +/- Steroids+/- Steroids o

o LTOTLTOT o

o Diuretics and Phosphodiesterase inhibitors for Cor Diuretics and Phosphodiesterase inhibitors for Cor pulmonalepulmonale

 Surgery-

Surgery-o

o LobectomyLobectomy o

o Bronchial artery embolisation for hemoptysisBronchial artery embolisation for hemoptysis o

o Lung transplant for cystic fibrosisLung transplant for cystic fibrosis

3.

3. Pulmonary Fibrosis/ ILD/Rheumatoid lungPulmonary Fibrosis/ ILD/Rheumatoid lung Investigation

Investigation a.

a. Chest x rayChest x ray – –to look for bilateral interstitial reticulo nodular pattern. Loss of lung volumeto look for bilateral interstitial reticulo nodular pattern. Loss of lung volume b.

b. HRCT to look for subpleural reticulation and traction broncheitasis, basal honeycombing , ground glassHRCT to look for subpleural reticulation and traction broncheitasis, basal honeycombing , ground glass patterns

patterns c.

c. Pulmonary function test to Pulmonary function test to look for Restrictive look for Restrictive pattern with reduced DLCOpattern with reduced DLCO d.

d. Blood-Differential count for eosinophilia, ESR, CRP- to look for signs of infectionBlood-Differential count for eosinophilia, ESR, CRP- to look for signs of infection e.

e. Markers for Autoimmune profile, ACE level, Ca,Markers for Autoimmune profile, ACE level, Ca, f.

f. Brocnchoscopy and BAL /Sputum Brocnchoscopy and BAL /Sputum microscopy and C/S to rule out microscopy and C/S to rule out any organismany organism g.

g. ECG and Echocardiography to rule out Pulmonary HTNECG and Echocardiography to rule out Pulmonary HTN h.

h. 6min walking test for functional assessment6min walking test for functional assessment i.

i. ABG to decide about LTOTABG to decide about LTOT Management

Management

 General measuresGeneral measures

o

o Stop smoking and toxic or allergic substancesStop smoking and toxic or allergic substances o

o Pulmonary rehabilitation ( Pt education, Nutrition, Pulmonary rehabilitation ( Pt education, Nutrition, Psychological support, Physiotherapy)Psychological support, Physiotherapy) o

o VaccinationVaccination o

o LTOTLTOT

 Specific medicalSpecific medical

o

o Immunosupressive –Immunosupressive – Steroid for  Steroid for ground glass, Thalidomide, Cyclophosphamide,ground glass, Thalidomide, Cyclophosphamide, o

o Antifibrotic –Antifibrotic – Pirfenidone for IPF ( UIP P Pirfenidone for IPF ( UIP Pattern on HRCT)attern on HRCT) o

o Diuretics and Phosphodiesterase inhibitors for Diuretics and Phosphodiesterase inhibitors for Cor pulmonaleCor pulmonale

 Surgery-

Surgery-o

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4.

4. Pleural effusionPleural effusion Investigation Investigation

a.

a. Chest x ray to Chest x ray to look for blunt costophrenic and cardiophrenic angleslook for blunt costophrenic and cardiophrenic angles b.

b. CT thorax CT thorax to look to look for any for any MASSMASS c.

c. USG to rule out multisepted/ loculated effusionUSG to rule out multisepted/ loculated effusion d.

d. Pleural fluid assessment for cellularity, protein, sugar, cultures, malignant cell, Pleural fluid assessment for cellularity, protein, sugar, cultures, malignant cell, special tests.special tests. e.

e. Blood-Differential count , ESR, CRP- to look Blood-Differential count , ESR, CRP- to look for signs of infection,for signs of infection, Albumin, Urea/ electrolytes to rule out

Albumin, Urea/ electrolytes to rule out any renal disease, LFT to rule out any any renal disease, LFT to rule out any liver diseaseliver disease f.

f. ECG and Echocardiography to rule out Pulmonary HTN/ cardiac decompensationECG and Echocardiography to rule out Pulmonary HTN/ cardiac decompensation g.

g. Markers for Autoimmune profileMarkers for Autoimmune profile h.

h. Pleural biopsy in refractory casesPleural biopsy in refractory cases i.

i. Other Special tests as requiredOther Special tests as required

Diagnosis of plerual effusion Clinically 500ml, x ray PA 300ml,

Diagnosis of plerual effusion Clinically 500ml, x ray PA 300ml, x ray lat dx ray lat decubitus-180ml , USG 30mlecubitus-180ml , USG 30ml Management:

Management: 1.

1. Therapeutic Pleural draiTherapeutic Pleural drainage if nage if Symptomatic, infective Symptomatic, infective ( empyema)( empyema) 2.

2. Treatment of the causeTreatment of the cause 3.

3. Decortication/intrapleuarl Decortication/intrapleuarl thrombolytics thrombolytics for for multiloculated effusionmultiloculated effusion 4.

4. Pleurodesis for refractory malignant effusionPleurodesis for refractory malignant effusion

5.

5. Lobectomy / Lobectomy / PneumonectomPneumonectomyy Investigation

Investigation

o

o Chest x rayChest x ray – –to look for confirmationto look for confirmation o

o CT thorax CT thorax to look for to look for underlying causeunderlying cause o

o Pulmonary function test to look for ObstructivePulmonary function test to look for Obstructive o

o Blood-Differential count , ESR, CRP- to look Blood-Differential count , ESR, CRP- to look for signs of infection,for signs of infection, o

o ECG and Echocardiography to rule out Pulmonary HTNECG and Echocardiography to rule out Pulmonary HTN o

o Other Special tests as requiredOther Special tests as required

Management: Management:

o

o SupportiveSupportive o

o Management of the original diseaseManagement of the original disease

 Indication of LobectomyIndication of Lobectomy

o

o Localized broncheictasis with massive hemoptysisLocalized broncheictasis with massive hemoptysis o

o Lung abscessLung abscess o

o Solitary pulmonary noduleSolitary pulmonary nodule o o TraumaTrauma o o AspergilomaAspergiloma o o NSCLC TNSCLC T3A3ANN00MM00 o

o TBTB – – not done now a days not done now a days

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o

o Massive BroncheictasisMassive Broncheictasis o

o Multiple lung abscessMultiple lung abscess o

o Fungal infection MalignancyFungal infection Malignancy o

o TraumaTrauma o

o Bronchial obstruction Bronchial obstruction with with destroyed lungdestroyed lung o

o Congenital lung diseaseCongenital lung disease o

o Malignant mesothelioma/ disseminated thymomaMalignant mesothelioma/ disseminated thymoma

 Indication of lung transplantIndication of lung transplant

o

o COPDCOPD o

o IPFIPF o

o Cystic fibrosisCystic fibrosis o

o Alpha 1 antitrypsin deficiencyAlpha 1 antitrypsin deficiency o

o Primary pulmonary hypertensionPrimary pulmonary hypertension

6.

6. OLD TB- FibrothoraxOLD TB- Fibrothorax Investigation

Investigation

o

o Chest x rayChest x ray – –to look for confirmationto look for confirmation o

o CT thorax CT thorax to look for underlying to look for underlying cause and look for cause and look for other lung pathologiesother lung pathologies o

o Pulmonary function test to look for volume lossPulmonary function test to look for volume loss o

o Blood-Differential count , ESR, CRP- to look Blood-Differential count , ESR, CRP- to look for signs of infection,for signs of infection, o

o ECG and Echocardiography to rule out Pulmonary HTNECG and Echocardiography to rule out Pulmonary HTN o

o Sputum gram stain and culture to look Sputum gram stain and culture to look for infection, AFB and NAAT to rule for infection, AFB and NAAT to rule out TBout TB

Management: Management:

1.

1. GeneralGeneral

 Smoking cessation/ Pulmonary rehabilitationSmoking cessation/ Pulmonary rehabilitation

Specific Specific

 BronchodilatorBronchodilator 

 Early antibiotic therapy for suspected infectionEarly antibiotic therapy for suspected infection

7.

7. Lung Consolidation ( d/d- Lung Consolidation ( d/d- Pneumonia/ Infarction/ mass)Pneumonia/ Infarction/ mass) Investigation

Investigation

o

o Chest x rayChest x ray – –to look for confirmationto look for confirmation o

o CT thorax CT thorax to rule out to rule out any mass lany mass lesionesion o

o Blood- Septic screening -Differential count , ESR, Blood- Septic screening -Differential count , ESR, CRP, PCT - to CRP, PCT - to look for signs of infection, Urea/ electrolytes-look for signs of infection, Urea/

electrolytes-CURB 65, CURB 65,

o

o ABG to look for hypoxemia/ hypercapnoeaABG to look for hypoxemia/ hypercapnoea o

o Sputum gram stain and culture to look Sputum gram stain and culture to look for infection, cytology for malignant cellsfor infection, cytology for malignant cells o

o ECG and ECHO to rule out pulmonary embolismECG and ECHO to rule out pulmonary embolism o

o Bronchoscopy with BAL for microbiology, cytologyBronchoscopy with BAL for microbiology, cytology o

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Management Management

 General measuresGeneral measures

o

o Stop smoking and toxic or allergic substancesStop smoking and toxic or allergic substances o

o Pulmonary rehabilitation ( Pt education, Nutrition, Pulmonary rehabilitation ( Pt education, Nutrition, Psychological support, Physiotherapy)Psychological support, Physiotherapy) o

o VaccinationVaccination

 Specific medicalSpecific medical

o

o Pneumonia Pneumonia Antibiotics/ Antibiotics/ Bronchodilator/ Bronchodilator/ MucolyticsMucolytics o

o Infarction Infarction LMWH/ LMWH/ warfarin/ warfarin/ modification modification of of risk risk factorfactor o

o Mass Mass Chemo/ Chemo/ radiotherapy/ radiotherapy/ surgerysurgery

8.

8. Lung collapse ( d/d Malignancy/ TB/Hilar L/N / Lung collapse ( d/d Malignancy/ TB/Hilar L/N / Mucus plug)Mucus plug)

o

o Chest x rayChest x ray – – for confirmation for confirmation o

o CT thorax CT thorax to rule out any mass to rule out any mass lesion, Enlarged L/Nlesion, Enlarged L/N o

o Blood- Septic screening -Differential count , ESR, CRP-Blood- Septic screening -Differential count , ESR,

CRP-Urea/ electrolytes/ LFT / ACE level Urea/ electrolytes/ LFT / ACE level

o

o ABG to look for hypoxemiaABG to look for hypoxemia o

o Sputum gram stain and culture to look Sputum gram stain and culture to look for infection, cytology for malignant cellsfor infection, cytology for malignant cells o

o Bronchoscopy to rule out any endobronchial growth, Bronchoscopy to rule out any endobronchial growth, BAL for microbiology, cytology , Biposy , NAATBAL for microbiology, cytology , Biposy , NAAT

Management Management

 General measuresGeneral measures

o

o PhysiotherapyPhysiotherapy o

o Positive pressure ventilationPositive pressure ventilation

 Specific medicalSpecific medical

o

o Mucus plug Mucus plug Bronchodilator/ Mucolytics/ Bronchodilator/ Mucolytics/ Bronchoscopy Bronchoscopy and and lavagelavage o

o Malignancy Malignancy Chemo/ Chemo/ radiotherapy/ radiotherapy/ Bronchoscopy Bronchoscopy may may need need stentingstenting o

o Others Others Bronchoscopy Bronchoscopy and and treatment treatment of of the the cause cause , , may may need need stentingstenting

9.

9. SVCO ( D/D-Malignancy/ mediastinal goiter/ thoracic aorta aneurysm/ mediastinal fibrosis)SVCO ( D/D-Malignancy/ mediastinal goiter/ thoracic aorta aneurysm/ mediastinal fibrosis) Investigations

Investigations

o

o Chest x ray /CT thorax Chest x ray /CT thorax to rule out any mass to rule out any mass lesion, Enlarged L/Nlesion, Enlarged L/N o

o Blood- Blood- FBC/ Urea/ FBC/ Urea/ electrolytes/ LFTelectrolytes/ LFT o

o ECG /ECHOECG /ECHO o

o CT Angiography / venographyCT Angiography / venography o

o Brochoscopy with BAL for microbiology, cytology , Biposy ,Brochoscopy with BAL for microbiology, cytology , Biposy ,

Management Management

 General measuresGeneral measures

o

o Elevation of head,Elevation of head, o

o Positive pressure ventilationPositive pressure ventilation

 Specific medicalSpecific medical

o

o Steroids ( Dexamathasone) Steroids ( Dexamathasone) and diureticsand diuretics o

o Radiotherapy/ chemothapyRadiotherapy/ chemothapy o

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Abdomen

Abdomen

1.

1. CLD CLD ( D/D Alcohol/ viral/a( D/D Alcohol/ viral/autoimmune/ metaboutoimmune/ metabolic/drugs)lic/drugs) Investigation

Investigation

 USG abdomen for confirmation of diagnosisUSG abdomen for confirmation of diagnosis may proceed to triphasic CT of Liver may proceed to triphasic CT of Liver 

 Severity-

Severity-o

o Synthetic function Synthetic function PT/INR, PT/INR, AlbuminAlbumin o

o Renal functionRenal function o

o BilirubinBilirubin o

o Serum ammoniaSerum ammonia

 To look for complicationTo look for complication

o

o Full Blood count –Full Blood count – anemia, thrombocytopenia( hyperspleenism) anemia, thrombocytopenia( hyperspleenism) o

o USG/ AFP to rule out HCCUSG/ AFP to rule out HCC o

o OGD OGD to to look look for for varicesvarices

 To look for etiologyTo look for etiology

o

o Alcohol and drug historyAlcohol and drug history o

o Metabolic profile ( NASH)Metabolic profile ( NASH) o

o Viral markersViral markers o

o Cerulopasmin and urinary copper study( WILSON’S)Cerulopasmin and urinary copper study( WILSON’S) o

o Ferritin (HH)Ferritin (HH) o

o Liver biopsy( NASH, PBC, PSC)Liver biopsy( NASH, PBC, PSC) o

o AMA ( PBC) , AMA ( PBC) , ASMA, , ASMA, , IgG, Anti IgG, Anti LKM ( LKM ( AIH) AIH) ANCA (PSC)ANCA (PSC) o

o Alpha 1 antitrypsinAlpha 1 antitrypsin

Management Management

 MDTMDT 

 Treat underlying causeTreat underlying cause

o

o Alcohol: abstinenceAlcohol: abstinence o

o Viral hep- Anti virals ( B-Lamivutin, Entacavir, C- Sofosbuvir, Telapravir, Bocepravir)Viral hep- Anti virals ( B-Lamivutin, Entacavir, C- Sofosbuvir, Telapravir, Bocepravir) o

o HH- venesection, iron chelationHH- venesection, iron chelation o

o Wislson’s- d penicilamine, ZincWislson’s- d penicilamine, Zinc o

o PBC- UDCA, Immunosuprressant, cholestyraimine, antihistaminePBC- UDCA, Immunosuprressant, cholestyraimine, antihistamine o

o NASH- Weight reduction, NASH- Weight reduction, Control of Control of diabetesdiabetes

 Treat complicationTreat complication

o

o Varix- ABCDE, LigatiVarix- ABCDE, Ligation, B on, B blockerblocker o

o Encephalopathy- rifaximin, treat precipitantsEncephalopathy- rifaximin, treat precipitants o

o Ascites- Diuretics, paracentesisAscites- Diuretics, paracentesis o

o SBP- Antibiotic, drainageSBP- Antibiotic, drainage o

o Hepatorenal- Albumin, TerlipressinHepatorenal- Albumin, Terlipressin o

o HCC- resection, embolization, transplantHCC- resection, embolization, transplant

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2.

2. Ascites ( CLD, Malignancy, TB, Fluid overload, Vascular, Ascites ( CLD, Malignancy, TB, Fluid overload, Vascular, Peritoneal dialysis peritonitis)Peritoneal dialysis peritonitis) Investigations

Investigations

 USG abdomen for confirmation of diagnosisUSG abdomen for confirmation of diagnosis 

 Ascetic fluid for SAAG, Cell count, type, , Sugar, Protein, Malignant cell, CultureAscetic fluid for SAAG, Cell count, type, , Sugar, Protein, Malignant cell, Culture 

 To look for etiologyTo look for etiology

o

o Alcohol and drug historyAlcohol and drug history o

o Metabolic profile ( NASH)Metabolic profile ( NASH) o

o Viral markersViral markers o

o Cerulopasmin and urinary copper study( WILSON’S)Cerulopasmin and urinary copper study( WILSON’S) o

o Ferritin (HH)Ferritin (HH) o

o Liver biopsy( NASH, PBC, PSC)Liver biopsy( NASH, PBC, PSC) o

o Auto antibodies: AMA ( Auto antibodies: AMA ( PBC) , PBC) , ASMA, , IgG, Anti ASMA, , IgG, Anti LKM ( AIH) LKM ( AIH) ANCA (PSC)ANCA (PSC) o

o Alpha 1 antitrypsinAlpha 1 antitrypsin o

o AFP for HCCAFP for HCC o

o Renal parameters and urinary protein for Nephrotic syndromeRenal parameters and urinary protein for Nephrotic syndrome o

o ECHO for CCF/ CP/ RCMECHO for CCF/ CP/ RCM o

o Thrombophilia workup and CECT abdomen for Veno-occusive diseaseThrombophilia workup and CECT abdomen for Veno-occusive disease

Management: Management:

 General –General – Patient education / salt and fluid restriction Patient education / salt and fluid restriction 

 Medical- Diuretics- Spironolactone, FurosemideMedical- Diuretics- Spironolactone, Furosemide 

 Treatment of SBPTreatment of SBP 

 Therapeutic paracentesisTherapeutic paracentesis

3.

3. Heptomegally ( D/D- Cirrhosis, Cancer, Cardiac, Cyst, Infection, Infiltration, Inflammation)Heptomegally ( D/D- Cirrhosis, Cancer, Cardiac, Cyst, Infection, Infiltration, Inflammation) Investigation

Investigation 1.

1. confirmation of diagnosis- USG abdomenconfirmation of diagnosis- USG abdomen 2.

2. Look for etiologyLook for etiology

o

o Alcohol and drug historyAlcohol and drug history o

o Metabolic profile ( NASH)Metabolic profile ( NASH) o

o Viral markersViral markers o

o Cerulopasmin and urinary copper study( WILSON’S)Cerulopasmin and urinary copper study( WILSON’S) o

o Ferritin (HH)Ferritin (HH) o

o Auto antibodies: AMA ( Auto antibodies: AMA ( PBC) , PBC) , ASMA, , IgG, Anti ASMA, , IgG, Anti LKM ( AIH) LKM ( AIH) ANCA (PSC)ANCA (PSC) o

o Alpha 1 antitrypsinAlpha 1 antitrypsin o

o AFP for HCCAFP for HCC o

o ECHO for CCF/ CP/ RCMECHO for CCF/ CP/ RCM o

o Liver biopsy ( NASH, PBC, PSC)Liver biopsy ( NASH, PBC, PSC)

3.

3. Look for complicationsLook for complications

o

o LFTsLFTs o

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o

o FBC with inflammatory markersFBC with inflammatory markers o

o CECT ABDOMENCECT ABDOMEN

Management: Management:

General-patient education , counseling, dietary modification, weight reduction General-patient education , counseling, dietary modification, weight reduction Specific- treat the underlying cause

Specific- treat the underlying cause 4.

4. Spleenomegally ( D/D Infection/ hematological/ congestive/ inflammatory/ neoplastic/ infiltrative)Spleenomegally ( D/D Infection/ hematological/ congestive/ inflammatory/ neoplastic/ infiltrative) Investigations:

Investigations:

o

o Confirmation: USG of AbdomenConfirmation: USG of Abdomen o

o Doppler study to look for portal vein thrombosisDoppler study to look for portal vein thrombosis o

o FBC and inflammatory markersFBC and inflammatory markers o

o PBS look for malaria, kala azar, hePBS look for malaria, kala azar, he molysismolysis o

o Bone marrow to look for Bone marrow to look for myeloprolifearative disordersmyeloprolifearative disorders o

o JAK 2 mutation, JAK 2 mutation, Philadelphia chromosomePhiladelphia chromosome o

o Auto immune screen for RA, SLE- (RF, Anti CCP, ANA)Auto immune screen for RA, SLE- (RF, Anti CCP, ANA) o

o Hemolysis screen- LDH, DCT, Bilirubin, haptoglobin, Hb electrophoresisHemolysis screen- LDH, DCT, Bilirubin, haptoglobin, Hb electrophoresis o

o HIVHIV o

o CXR for mediastinal enlargementCXR for mediastinal enlargement

Management Management

General: Patient education General: Patient education

Specific : treat underlying cause. Spleenectomy

Specific : treat underlying cause. Spleenectomy for severe hyperspleenism.for severe hyperspleenism.

(Influenza/ Hemophilus/ pneumococcus, meningococcus vaccine needed after spleenectomy) (Influenza/ Hemophilus/ pneumococcus, meningococcus vaccine needed after spleenectomy)

Prophylactic Penicilline for at least 2 yrs. Prophylactic Penicilline for at least 2 yrs.

5.

5. HepatospleenomagallyHepatospleenomagally Investigations:

Investigations:

o

o Confirmation: USG of AbdomenConfirmation: USG of Abdomen o

o Doppler study to look for portal vein thrombosisDoppler study to look for portal vein thrombosis o

o FBC and inflammatory markersFBC and inflammatory markers o

o PBS look for malaria, kala azar, hePBS look for malaria, kala azar, he molysismolysis o

o Bone marrow to look for myeBone marrow to look for myeloprolifearative disordersloprolifearative disorders o

o JAK 2 mutation, JAK 2 mutation, Philadelphia chromosomePhiladelphia chromosome o

o Auto immune screen for RA, SLE- (RF, Anti CCP, ANA)Auto immune screen for RA, SLE- (RF, Anti CCP, ANA) o

o Hemolysis screen- LDH, DCT, Bilirubin, haptoglobin, Hb electrophoresisHemolysis screen- LDH, DCT, Bilirubin, haptoglobin, Hb electrophoresis o

o HIVHIV o

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Management Management

General: Patient education General: Patient education

Specific : treat underlying cause. Spleenectomy

Specific : treat underlying cause. Spleenectomy for severe hyperspleenism.for severe hyperspleenism.

(Influenza/ Hemophilus/ pneumococcus, meningococcus vaccine needed after spleenectomy) (Influenza/ Hemophilus/ pneumococcus, meningococcus vaccine needed after spleenectomy)

Prophylactic Penicilline for at least 2 yrs. Prophylactic Penicilline for at least 2 yrs.

6.

6. HeptospleenomHeptospleenomegally egally with with LymphadenopLymphadenopathyathy

( d/d Lymphoma, leukemia in blast crisis, disseminated TB, SLE, sarcoidosis,

( d/d Lymphoma, leukemia in blast crisis, disseminated TB, SLE, sarcoidosis, Infectious MononeucleosisInfectious Mononeucleosis syndrome, HIV)

syndrome, HIV) Investigations:

Investigations:

o

o Confirmation: USG of AbdomenConfirmation: USG of Abdomen o

o Doppler study to look for portal vein thrombosisDoppler study to look for portal vein thrombosis o

o FBC to see leucocyte counFBC to see leucocyte count t and inflammatory and inflammatory markersmarkers o

o PBS look abnormal cellsPBS look abnormal cells o

o Bone marrow to look for myeBone marrow to look for myelo/ Lympho prolifearative disorderslo/ Lympho prolifearative disorders o

o Auto immune screen for RA, SLE- (RF, Anti CCP, ANA)Auto immune screen for RA, SLE- (RF, Anti CCP, ANA) o

o Lymph node biopsy if > 1month, > 1 region, > Lymph node biopsy if > 1month, > 1 region, > 1cm and not draining any infective focus1cm and not draining any infective focus o

o HIV serologyHIV serology o

o CXR for mediastinal enlargement, ACE level CXR for mediastinal enlargement, ACE level for sarcoidosisfor sarcoidosis o

o LFTs, Renal parameters,LFTs, Renal parameters,

Management Management

General: Patient education General: Patient education Specific : treat underlying cause Specific : treat underlying cause

7.

7. Jaundice +/_ hepato/ Hepatospleenoegally (DCLD, Hepatitis, Hemolytic disease, Jaundice +/_ hepato/ Hepatospleenoegally (DCLD, Hepatitis, Hemolytic disease, malignancy, infections)malignancy, infections) Investigations:

Investigations:

o

o Confirmation: LFTsConfirmation: LFTs o

o For etiologyFor etiology o

o USG of Abdomen folloUSG of Abdomen followed wed MRCP/ CT depending on UMRCP/ CT depending on USG findingsSG findings o

o Viral markersViral markers o

(12)

o

o PBS look abnormal cells, spherocytes,PBS look abnormal cells, spherocytes, o

o Hemoglobin electrophoresis, DCT to look for hemolytic diseasesHemoglobin electrophoresis, DCT to look for hemolytic diseases o

o HIV serologyHIV serology o

o FOR complicationFOR complication o

o Coagulation profileCoagulation profile o

o OGD for esophageal varicesOGD for esophageal varices o

o Renal parametersRenal parameters o

o Septic screenSeptic screen o

o Ascitic fluid if anyAscitic fluid if any

8.

8. Jaundice and Ascites +/_ hepato/ Hepatospleenoegally ( DCLD, , Malignancy ,Disseminated TB, CardiacJaundice and Ascites +/_ hepato/ Hepatospleenoegally ( DCLD, , Malignancy ,Disseminated TB, Cardiac cirrhosis)

cirrhosis)

o

o Confirmation: LFTsConfirmation: LFTs o

o For etiologyFor etiology o

o USG of Abdomen folloUSG of Abdomen followed wed MRCP/ CT depending on UMRCP/ CT depending on USG findingsSG findings o

o Viral markersViral markers o

o Autoimmune profile (AMA,ANA, ASMA, Anti LKM)Autoimmune profile (AMA,ANA, ASMA, Anti LKM) o

o Ascitic fluid for Cellularity, SAAG, Malignant cell, CultureAscitic fluid for Cellularity, SAAG, Malignant cell, Culture o

o Laparoscopy and peritoneal biopsyLaparoscopy and peritoneal biopsy o

o EchocardiographyEchocardiography o

o For complicationFor complication o

o Coagulation profileCoagulation profile o

o OGD for esophageal varicesOGD for esophageal varices o

o Renal parametersRenal parameters o

o Septic screenSeptic screen

Management Management

General: Patient education General: Patient education Specific : treat underlying cause Specific : treat underlying cause

9.

9. Polycystic KidneyPolycystic Kidney Investigation

Investigation

 USG abdomen USG abdomen for Confirmation and for Confirmation and measuring the number of cystsmeasuring the number of cysts 

 CBC to look for anemia/ polycythemiaCBC to look for anemia/ polycythemia 

 Renal function, Calcium and phosphate to look for secondary hyperparatyroidismRenal function, Calcium and phosphate to look for secondary hyperparatyroidism 

 Chest x ray to Chest x ray to look for fluid overloadlook for fluid overload 

 Urine dipstick to look for hematuria/ proteinUrine dipstick to look for hematuria/ protein 

 LFTs to look for deranged liver LFTs to look for deranged liver function as there might be cysts in liverfunction as there might be cysts in liver 

 Screening for cerebral aneurysm Screening for cerebral aneurysm with patients having family h/o SAH @5yrs.with patients having family h/o SAH @5yrs. 

 Genetic study Genetic study for potential for potential donors with donors with no cysts no cysts on USon US 

 Echocardiography to look for MVP, AREchocardiography to look for MVP, AR

Management: Management:

(13)

 GeneralGeneral

o

o Patient education and counseling about course of disease and complicationPatient education and counseling about course of disease and complication o

o Attempt 3lit fluid intake per day to Attempt 3lit fluid intake per day to suppress ADH secretionsuppress ADH secretion o

o Screening of 1Screening of 1stst degree relatives from the age f 20yr at least 3 cyst uni/ bilateral degree relatives from the age f 20yr at least 3 cyst uni/ bilateral o

o Avoid contact sports/risk of trauma to abdomenAvoid contact sports/risk of trauma to abdomen

 Specific medicalSpecific medical

o

o ACEI for control of hypertensionACEI for control of hypertension o

o Avoid nephrotoxic drugsAvoid nephrotoxic drugs o

o Antibiotics for UTI/ cyst infectionAntibiotics for UTI/ cyst infection o

o Renal replacement therapyRenal replacement therapy o

o Indication of NephrectomyIndication of Nephrectomy

 Recurrent infectionRecurrent infection

 Uncontrolled hemorrhageUncontrolled hemorrhage  Suspected malignancySuspected malignancy  Symptomatic mass effectSymptomatic mass effect

 Extension of polycystic kidney into transplant site.Extension of polycystic kidney into transplant site.

o

o Management of Hematuria- bed rest, hydration and analgesiaManagement of Hematuria- bed rest, hydration and analgesia

10.

10. Transplanted KidneyTransplanted Kidney Investigations

Investigations

 USG Doppler to confirm diagnosis and assess graftUSG Doppler to confirm diagnosis and assess graft 

 To check that the graft is workingTo check that the graft is working

o

o FBCFBC o

o Renal FunctionRenal Function o

o Calcium and PhosphateCalcium and Phosphate o

o CXR for volume overloadCXR for volume overload o

o ABG for metabolic acidosisABG for metabolic acidosis

 To look for To look for complication of immunosupressioncomplication of immunosupression

o

o Septic screen for patients with feverSeptic screen for patients with fever o

o Renal function/ LFT ( Cyclosporin )Renal function/ LFT ( Cyclosporin ) o

o Fasting lipid/ sugar ( tacrolimus)Fasting lipid/ sugar ( tacrolimus) o

o Screen for opportunistic infectionScreen for opportunistic infection o

o Biopsy from transplanted kidney to look for Graft failureBiopsy from transplanted kidney to look for Graft failure

Management Management

 MDTMDT 

 Patient education and counseling, cPatient education and counseling, c ompliance to immunosuppressantompliance to immunosuppressant 

 Monitoring for toxicity of Monitoring for toxicity of immunosuppressantimmunosuppressant

o

o FBC, Renal parameters, LFTFBC, Renal parameters, LFT o

o Septic screenSeptic screen o

o Dermatology surveillance for PTLDDermatology surveillance for PTLD

(14)

 Renal replacement therapy if graft Renal replacement therapy if graft function deteriorates despite adequate immunosupressionfunction deteriorates despite adequate immunosupression

o

o Uremic encephalopathyUremic encephalopathy o

o PericarditisPericarditis o

o Refractor fluid overloadRefractor fluid overload o

(15)

Lower limb Weakness Lower limb Weakness

Quadruplegia Bilateral

Quadruplegia Bilateral UMNUMN   Unilateral  Unilateral  stroke stroke ask permission UL/Face ask permission UL/Face Examine neck Examine neck Lower cranial Nv Lower cranial Nv cerebreller cerebreller Paraparesis/ paraplegia Paraparesis/ paraplegia Check Cerebeller

Check Cerebeller   Yes  Yes  MS, Brainstem , Spino Cerebeller Ataxia MS, Brainstem , Spino Cerebeller Ataxia Friedreich’s / B12 deficiency

Friedreich’s / B12 deficiency No

No

Check

Sensory-Check Sensory- Deficit Deficit Spinal Level Spinal Level Spinal Cord lesion Spinal Cord lesion Dissociated sensory loss

Dissociated sensory loss Syringomyelia/ Ant Spinal AOSyringomyelia/ Ant Spinal AO No

No Sensory Sensory deficit deficit Peipheral Peipheral nerve nerve patternpattern Pheripheral neuropathy + Pyram Pheripheral neuropathy + Pyramidalidal

Fasciculation

Fasciculation parasagital parasagital meningiomameningioma Syringomyelia

Syringomyelia MND

MND Heriditary/Tropical Spastic Heriditary/Tropical Spastic Spastic Spastic Diplegia Diplegia ( ( Cerebral Cerebral Palsy)Palsy) Paraparesis

(16)

Lower limb LMN

Lower limb LMN Cerebeller Cerebeller Alcohol, drugs Alcohol, drugs

Sensory Deficit Sensory Deficit

Yes NO

Yes NO   PolioPolio

Small wasted limb Small wasted limb

Root

Root Lesion Lesion Peripheral Peripheral nerve nerve Both Both root root and and nerve nerve Muscle Muscle hypertrophy hypertrophy fasciculationfasciculation Cauda

Cauda Diabetes Diabetes GBS/ GBS/ CIDP CIDP Muscle Muscle disease disease Multifocal Multifocal Motor Motor NPNP GBS

GBS Alcohol Alcohol MNDMND

CIDP HSMN

CIDP HSMN

Diabetic

Diabetic amyotrphy amyotrphy B6/B12B6/B12

Paraneoplastic Paraneoplastic CIDP

CIDP Drugs

Drugs Affect Affect only only LMNLMN

Asymmetric, UL and LL Asymmetric, UL and LL Prominent fasciculation Prominent fasciculation Autoimmune origin Autoimmune origin Anti GM 1 antibody Anti GM 1 antibody Treatment IvIg, Treatment IvIg, Rituximab and Rituximab and Immunosupressant Immunosupressant Both UMN and LMN

Both UMN and LMN Lower limbLower limb

 No Sensory Loss- ALSNo Sensory Loss- ALS 

 Dissociated Sensory Loss- SyringsDissociated Sensory Loss- Syrings 

 Absent KJ+ brisk AJ, PlanterAbsent KJ+ brisk AJ, Planter ↑↑= Conus lesion= Conus lesion 

 Absent ankle jerk + planterAbsent ankle jerk + planter ↑↑↑↑

o

o M- MS, MNDM- MS, MND o

o A- Ataxia Freidreich’s , Cauda conusA- Ataxia Freidreich’s , Cauda conus o

o S- SACD, Syphilitic taboparesisS- SACD, Syphilitic taboparesis o

(17)

Foot drop Foot drop

Ankle Jerk Ankle Jerk

Brisk

Brisk Present Present absentabsent

UMN

UMN Sensory Sensory loss loss S1 S1 radiculopathy radiculopathy Sciatic Sciatic nervenerve

Lateral

Lateral thigh thigh and and leg leg compartment compartment Part Part of of leg leg and and No No sensory sensory lossloss &

& Dorsum Dorsum of of foot foot Dorsum Dorsum of of foot foot BilateralBilateral Usually

Usually unilateral unilateral UnilateralUnilateral H/O Pain

H/O Pain EHL weakness EHL weakness

L5

L5 radiculopathy radiculopathy Common Common peroneal peroneal nerve nerve Myotonic Myotonic dystrophydystrophy D/D

D/D HSMN HSMN / / trauma/ trauma/ diabetes diabetes IBMIBM Paraneoplastic

Paraneoplastic

Cause of Pes Cavus Cause of Pes Cavus

Unilateral Bilateral

Unilateral Bilateral

Polio HSMN

Polio HSMN

Burn

Burn mascular mascular dystrophydystrophy

Taruma

Taruma spinal spinal mascular mascular atrophyatrophy Syringomyelia Syringomyelia Palpable nerve Palpable nerve   HSMNHSMN   AcromegallyAcromegally   LeprosyLeprosy   CIDPCIDP   AmyloidosisAmyloidosis   NeurofibromatosisNeurofibromatosis

(18)

Upper limb Weakness Upper limb Weakness

Check LL

Check LL UMNUMN   Unilateral  Unilateral  stroke stroke ask permission LL/Face ask permission LL/Face

LMN

LMN and and UMN UMN Bilateral Bilateral ((D/D-D/D- bilateral stroke/MND/ PD/ bilateral stroke/MND/ PD/ Freidreich’sFreidreich’s/ / cervical cervical 1-4)1-4)

MND MND

Examine neck

Examine neck any scar, kyphoscoliosis any scar, kyphoscoliosis

Check

CheckCerebellerCerebeller  Yes Yes  MS, Brainstem , Spino Cerebeller Ataxia, Wilson’sMS, Brainstem , Spino Cerebeller Ataxia, Wilson’s

Friedreich

Friedreich’s’s NoNo

Check Sensory

Check Sensory-- No No Sensory Sensory deficit deficit FasciculationFasciculation   YesYes MNDMND No No Deficit Deficit Tremor Yes Tremor Yes PD PD Spinal

Spinal Level Level Dissociated Dissociated sensory sensory loss loss Peipheral nerve Peipheral nerve pattern pattern Multiple Multiple sclerosissclerosis

Spinal

Spinal Cord Cord lesion lesion Syringomyelia/ Syringomyelia/ Ant Ant Spinal Spinal AO AO Pheripheral Pheripheral neuropathy neuropathy + + PyramidalPyramidal

cervical myelopathy cervical myelopathy

(19)

Upper

Upper limb limb LMNLMN Cerebeller Cerebeller Alcohol, drugs, Syrings, Alcohol, drugs, Syrings,

Sensory Deficit Sensory Deficit

Yes NO

Yes NO   PolioPolio

Small wasted limb Small wasted limb

Root

Root Lesion Lesion Peripheral Peripheral nerve nerve Both Both root root and and nerve nerve Muscle Muscle hypertrophy hypertrophy fasciculationfasciculation Dermatomal

Dermatomal loss loss Gloves Gloves pattern pattern both/ both/ variable variable Proximal Proximal Distal/proximalDistal/proximal

Brachial

Brachial Neuritis Neuritis Diabetes Diabetes GBS/ GBS/ CIDP CIDP Fatigable Fatigable Multifocal Multifocal Motor Motor NPNP CIDP

CIDP HSMN HSMN MNDMND

GBS

GBS Alcohol Alcohol Cervical Cervical SponSpon

CIDP CIDP Drugs Drugs B6/B12 B6/B12 Paraneoplastic Paraneoplastic Affect only LMN Affect only LMN Asymmetric, UL and LL Asymmetric, UL and LL Prominent fasciculation Prominent fasciculation Yes No

Yes No Autoimmune Autoimmune originorigin

MG

MG Muscle Muscle ds ds Anti Anti GM GM 1 1 antibodyantibody LEMS LEMS Treatment IvIg, Treatment IvIg, Rituximab and Rituximab and Immunosupressant Immunosupressant

(20)

Small Muscle wasting of hand Small Muscle wasting of hand

D/D-

D/D- MND, MND, Cervical Cervical myelopathy, myelopathy, Syrings, Syrings, combined combined median median and and ulnar ulnar nerve nerve damage,Peripheral damage,Peripheral neuropathy(neuropathy( CMT) , C8T1 lesion ( cervical r

CMT) , C8T1 lesion ( cervical rib, Pancoast tumor, trauma)ib, Pancoast tumor, trauma) 1.

1. Hand- Hand-a.

a. 5 5 movements movements of of thumb thumb 4 4 movements movements of of fingers fingers 1 1 movement movement of of little little fingerfinger b.

b. DTR DTR c. c. Sensory Sensory d. d. Cerebeller Cerebeller e. e. Lower Lower limb/ limb/ cranialcranial WASTING OF HAND

WASTING OF HAND

Unilat

Unilat stroke stroke LMN

LMN UMN UMN BilateralBilateral  MND  MND

Parkinson’s Ds Parkinson’s Ds Upper cervical Upper cervical SENSORY SENSORY

Median and Ulnar nerve Median and Ulnar nerve

No

No sensory sensory loss loss gloves gloves pattern pattern Dermatomal Dermatomal pattern pattern C8-T1 C8-T1 Dissociated Dissociated cerebeller+ dorsalcerebeller+ dorsal

MND

MND Polyneuropathy Polyneuropathy PP ancoast ancoast SyringomyeliaSyringomyelia Friedreich’sFriedreich’s MMN

MMN Neurofibroma Neurofibroma ataxiaataxia

Myotonic

Myotonic dystrophy dystrophy Cervical Cervical RibRib Disuse atrophy

Disuse atrophy RA RA Cervical myelopathy Cervical myelopathy (+UMN)(+UMN) Cachexia

Cachexia Look Look relevant relevant Brachial Brachial plexopathyplexopathy IBM

IBM

Spinal Cord Spinal Cord Cervical

Cervical Spondylosis Spondylosis Look Look at at backback Syringomyeli

Syringomyelia a Test Test sensation sensation over over cap cap area, area, look look for for HornersHorners Anterior Horn Cell

Anterior Horn Cell Motor

Motor Neuron Neuron Disease Disease Fasiculation Fasiculation of of the the tongue tongue or or other other muscles, muscles, ?bulbar ?bulbar speechspeech Polio

Polio Spinal

Spinal muscular muscular atrophy atrophy Examination Examination of of lower lower limbslimbs Nerve Lesions

Nerve Lesions Median

Median Alone will Alone will not not give give wasting wasting of of all all small small muscles muscles of of the the handhand Ulnar

Ulnar Should have Should have a a claw claw handhand Peripheral

Peripheral Neuropathy Neuropathy e.g. e.g. CMTCMT –  –  absent reflexes, forearms > hands, thickened nerves absent reflexes, forearms > hands, thickened nerves Brachial Plexus C8-T1

Brachial Plexus C8-T1 ? clubbed from lung ca, cervical ribs, Horner’s? clubbed from lung ca, cervical ribs, Horner’s Myopathy

Myopathy Myotonic

Myotonic Dystrophy Dystrophy Appearance, Appearance, weak weak neck neck musclesmuscles Distal

(21)

3

3rdrd nerve nerve

Medical

Medical – – DM, MS, Basal meningitis, Vasculitis, GCA DM, MS, Basal meningitis, Vasculitis, GCA Surgical- Trauma, PCA aneurysm, tumor

Surgical- Trauma, PCA aneurysm, tumor Inspection

Inspection- Complete ptosis, Lift the eyelid- Complete ptosis, Lift the eyelid see eyeball see eyeball down and out down and out see pupil see pupil miosis/ mydriasis miosis/ mydriasis H

H Diplopia maximal on contalateral superior gaze Diplopia maximal on contalateral superior gaze Check

Check – –

 4, 6, V14, 6, V1 

 Disc- papiloedema, RetinopathyDisc- papiloedema, Retinopathy 

 Ispilat cerebellerIspilat cerebeller 

 Contra pyramidal- weber’sContra pyramidal-weber’s

Associated syndromes Associated syndromes

 Caverous sinus syndrome- Caverous sinus syndrome- III, IV, V1, V2., VI + symatetic fibersIII, IV, V1, V2., VI + symatetic fibers horner’shorner’s 

 Orbital apex- Orbital apex- II, III,IV, II, III,IV, V1, VIV1, VI 

 Superior orbital syndrome- III, IV, V1, VISuperior orbital syndrome- III, IV, V1, VI

4

4thth nerve nerve

Inspection: affected eye in higher than o

Inspection: affected eye in higher than o ther in neutral position.ther in neutral position. H

H impairment of the impairment of the affected eye adducted eye can’t look affected eye adducted eye can’t look down. Diplopia maximum at looking down anddown. Diplopia maximum at looking down and away from the affected side and cover test that outer image from affected side.

away from the affected side and cover test that outer image from affected side.

INO INO

(22)

6

6thth  Nerve  Nerve

Cause-Cause- DM, MS, Basal meninDM, MS, Basal meningitis, Vasculitis, gitis, Vasculitis, False localizing signFalse localizing sign, CP angle Mass, CP angle Mass Approach

Approach

inspection : Convergent strabismus inspection : Convergent strabismus H

H impaired abduction of affected eye impaired abduction of affected eye Diplopia Diplopia cover test cover test ask which image disappear? Outer/ inner.ask which image disappear? Outer/ inner. Relavant

Relavant 1.

1. Associated 3Associated 3rdrd , 4 , 4thth – – search search Gaze palsyGaze palsy

2.

2. Associated V1- CavernousAssociated V1- Cavernous 3.

3. 77thth – – Brain stem Brain stem

4.

4. 88thth – – CP angle CP angle

5.

5. Disc- DN/HTN/OA/papiloedemaDisc- DN/HTN/OA/papiloedema——false localizing signfalse localizing sign 6.

6. Long tract sign Long tract sign - Brain s- Brain stem syndrome-tem syndrome- Millard gubler’s syndromeMillard gubler’s syndrome VI+ VII+ contra hemiparesisVI+ VII+ contra hemiparesis

7

7thth Nerve Nerve

1.

1. UMN- UMN- stroke/ stroke/ tumor/ tumor/ demyelination/ demyelination/ traumatrauma 2.

2. B/L UMNB/L UMN——MND, pseudobulber palsyMND, pseudobulber palsy present jaw jerk, taste sensation preserved present jaw jerk, taste sensation preserved 3.

3. LMN-LMN- Bell’s palsy, basal Bell’s palsy, basal meningitis, Leprosy, sarcoid, meningitis, Leprosy, sarcoid, Ramsay hunt , Lyme diseaRamsay hunt , Lyme diseasese, HIV, HIV 4.

4. B/L LMN-B/L LMN- GBS, b/L bell’s palsyGBS, b/L bell’s palsy 5.

5. Muscle ds- MG, Muscular Muscle ds- MG, Muscular dystrophy, FSHD, Mitochondrial myopathydystrophy, FSHD, Mitochondrial myopathy Approch

Approch a.

a. Parotid scarParotid scar previous surgery previous surgery b.

b. Hearing loss ( VIII), ipsilateral loss of facial sensation( V)Hearing loss ( VIII), ipsilateral loss of facial sensation( V)  CP Angle CP Angle c.

c. Ipsilateral sixth , contralat hemiparesisIpsilateral sixth , contralat hemiparesis medial pons medial pons d.

d. Ear herpes/ poxEar herpes/ pox Ramsay Hunt Ramsay Hunt e.

e. MastoiditisMastoiditis Base of skull Base of skull f.

f. UMN with hemiparesis- MCA strokeUMN with hemiparesis- MCA stroke I would like to complete my e

I would like to complete my examination withxamination with

 corneal reflex,corneal reflex,

Important causes of cranial nerve palsy Important causes of cranial nerve palsy

1.

1. Infection- basal meningitisInfection- basal meningitis 2.

2. Infarction- strokeInfarction- stroke 3.

3. Inflammation- MSInflammation- MS 4.

4. TraumaTrauma 5.

5. Mononeuritis multiplexMononeuritis multiplex 6.

6. Autoimmune conditionAutoimmune condition 7.

7. DiabetesDiabetes

Relevant Relevant

N-N- Nerve Nerve 1up and 1 1up and 1 down until down until normalnormal

I-I- Ipsilateral cerebellerIpsilateral cerebeller

C-C- Contralateral pyramidalContralateral pyramidal E

(23)

 otoscopy,otoscopy, 

 formal audiometry andformal audiometry and 

 examination of upper and lower limbs.examination of upper and lower limbs.

Bulbar and Pseudobulbar palsy Bulbar and Pseudobulbar palsy

Pseudobulbar Bulbar

Pseudobulbar Bulbar

 UMNUMN 

 CN V, CN V, VII VII (IX, (IX, X,XII)X,XII) 

 Facial expression, masticationFacial expression, mastication 

 Bilateral degeneration of corticobulbar tractsBilateral degeneration of corticobulbar tracts 

 Gag reflex, tongue spasticityGag reflex, tongue spasticity 

 Jaw jerk exageratedJaw jerk exagerated 

 Spastic dysarthriaSpastic dysarthria 

 “Daffy Duck”“Daffy Duck” 

 MS, MNDMS, MND

 LMNLMN 

 CN CN IX, IX, X,XIIX,XII 

 Diminished gagDiminished gag 

 Tongue fasciculation, wastingTongue fasciculation, wasting 

 Jaw jerk normalJaw jerk normal 

 Unilateral –Unilateral – raspy voice raspy voice 

 Bilateral –Bilateral – nasal speech nasal speech   GBS, Stroke, MNDGBS, Stroke, MND Myotonic Dystophy: Myotonic Dystophy: Face Face 

 Loss of facial and neck musculatureLoss of facial and neck musculature 

 Frntotemporal baldingFrntotemporal balding 

 Difficulty in opDifficulty in opening eyes ening eyes after firm closureafter firm closure 

 Drooping mouthDrooping mouth   DysarthriaDysarthria   CataractCataract   Low IQLow IQ Hands Hands 

 Distal wasting and weakness LMN patternDistal wasting and weakness LMN pattern 

 Percussion myotoniaPercussion myotonia 

 NO Sensory lossNO Sensory loss

Lower Limb Lower Limb

 Distal wasting and weakness LMN patternDistal wasting and weakness LMN pattern 

 Foot drop and high steppage gaitFoot drop and high steppage gait

Others Others

 GYnecomastiaGYnecomastia 

 Cardiomyopathy, conduction blockCardiomyopathy, conduction block 

 Esophagial dysmotilityEsophagial dysmotility 

 HypoventilationHypoventilation 

 Testicular atrophyTesticular atrophy

Autosomal Dominant ,CTG trinucleotide repeat Autosomal Dominant ,CTG trinucleotide repeat sequence in sequence in DM 1=Chromosome 19 DM 1=Chromosome 19 DM2= Chromosome 3 DM2= Chromosome 3 Investigation Investigation 

 Dive bomber potential in EMGDive bomber potential in EMG 

 CPK mild elevationCPK mild elevation 

 Muscle biopsy- variability in fiber size andMuscle biopsy- variability in fiber size and

fibrosis fibrosis

 FBS and Hba1c, LFTsFBS and Hba1c, LFTs 

 ECG to look for conduction block, EchoECG to look for conduction block, Echo 

 Genetic testingGenetic testing 

 MRI brain brain atrophyMRI brain brain atrophy

Management Management

 Patient education and counselingPatient education and counseling 

 May die prematurelyMay die prematurely 

 Phenytoin may Phenytoin may help mhelp myotoniayotonia 

(24)

Ptosis Ptosis Unilateral Bilateral Unilateral Bilateral With ophthalmoplegia With ophthalmoplegia Without Without ophthalmoplegia

ophthalmoplegia With With ophthalmoplegiaophthalmoplegia

Without Without

ophthalmoplegia ophthalmoplegia

 Third nerveThird nerve

palsy palsy Mydriasis Mydriasis   MyastheniaMyasthenia initial stage initial stage fatigable fatigable Horner’s syndrome Horner’s syndrome Miosis Miosis Partial ptosis Partial ptosis Fatigable Fatigable 

 Myasthenia gravisMyasthenia gravis

Non fatigable Non fatigable   MitocondrialMitocondrial myopathy CPEO myopathy CPEO   OcculopharyngealOcculopharyngeal muscular dystrophy muscular dystrophy 

 Miller fisherMiller fisher

syndrome syndrome

 Neurotoxic snake biteNeurotoxic snake bite

  MyotonicMyotonic dystrophy dystrophy   SenileSenile   CongenitalCongenital   B/L Horner’sB/L Horner’s Steps: Steps: 1.

1. InspectionInspection – – one eye/ both eye, one eye/ both eye, 2.

2. Come near to the Come near to the patient without thouching the patientpatient without thouching the patient complete / partial complete / partial 3.

3. Lift the eyelidLift the eyelid see Pupil. see Pupil.

Miosis

Miosis Normal Normal DilatedDilated

Hormer’s Hormer’s

position of eyeball. down + out position of eyeball. down + out colour

colour of of iris iris Position Position of of eye eye NormalNormal

heterochromia

heterochromia please please close close eyes eyes tightly tightly for for me me pleaseplease

follow scheme of nerve follow scheme of nerve palsy

palsy follow scheme

follow scheme of Horner’ s

of Horner’ s Cant Cant close close eye eye tightly tightly Can Can close close tightly tightly but but cancan’’t opent open

Myasthenia

(25)

Horner’s

Horner’s SyndromeSyndrome

Central 1

Central 1ststorder order Demyelination Demyelination /Syringomyelia /Syringomyelia No No sweating sweating in in face, face, arm arm and and trunktrunk

2

2ndndorder order Trauma, Trauma, surgery, surgery, pancoast pancoast , , Lymph Lymph node, node, goiter goiter No No sweating sweating in in facefacearm trunkarm trunk

normal normal 3

3rdrdorder order cavernous cavernous sinus sinus syndrome, syndrome, carotid carotid dissection dissection Sweating Sweating normalnormal

Sequence Sequence

1.

1. Partial PtosisPartial Ptosis ask to look up. ask to look up. 2.

2. Lift the eyelidLift the eyelid colour of iris ( congenital heterochromia), Ask to dim the light  colour of iris ( congenital heterochromia), Ask to dim the light for pupil, light reflexfor pupil, light reflex 3.

3. Eye movement HEye movement H 4.

4. Hands- Hands- Samll muscle wasting, clubSamll muscle wasting, clubbing, nicotin staininbing, nicotin stainingg 5.

5. Neck- Scar, dressing, Neck- Scar, dressing, cervical rib palpate, Goiter, palpcervical rib palpate, Goiter, palpalte thyroid glandalte thyroid gland 6.

6. Chest- Scar, Dressing, pancoastChest- Scar, Dressing, pancoast 7.

7. Lower limb- if time allowsLower limb- if time allows Presentation

Presentation

I would like to complete my e

I would like to complete my examination by testing sweatingxamination by testing sweating 1.

1. Partial ptosis,Partial ptosis,which is overcome by voluntary upgazewhich is overcome by voluntary upgaze

2.

2. Apparent EnopthalmosApparent Enopthalmos 3.

3. MiosisMiosis 4.

(26)

Myasthenia Gravis Myasthenia Gravis

Fetures Fetures

 Fatigable ptosisFatigable ptosis 

 Complex ophthalmoplegiaComplex ophthalmoplegia 

 Nasal speechNasal speech 

 Bulbar weakness—Bulbar weakness—Nasal speech, poor swallowNasal speech, poor swallow 

 Proximal weakness UL>>LLProximal weakness UL>>LL 

 Reflex/ sensory normalReflex/ sensory normal 

 Look for sternotomy scarLook for sternotomy scar 

 Look for features of immunosupessionLook for features of immunosupession

o

o CushingoidCushingoid

 Single breath count –Single breath count – FVC FVC 

 Other autoimmune diseaseOther autoimmune disease

LEMS LEMS

 limb girdle limb girdle weakness weakness LL>>ULLL>>UL 

 Rarely Ptosis and diplopiaRarely Ptosis and diplopia 

 Diminished reflexes become brisk afterDiminished reflexes become brisk after

exercise exercise

 Associated with malignancy- SCLCAssociated with malignancy- SCLC 

 Anti voltage gated Ca cannel antibodyAnti voltage gated Ca cannel antibody 

 Autonomic dysfunctionAutonomic dysfunction 

 EMG shows “second wind” phenomenonEMG shows “second wind” phenomenon 

 Mx-3,4 Diaminopyridine and treatment ofMx-3,4 Diaminopyridine and treatment of

tumor tumor Investigation Investigation   CBC, ESRCBC, ESR 

 CXR to look for mediastinal massCXR to look for mediastinal mass 

 CT chest for thymomaCT chest for thymoma 

 Anti AChR antibody 85%Anti AChR antibody 85% 

 Anti MuSK antibody 15%Anti MuSK antibody 15% 

 Vital capacityVital capacity 

 RNST >10% decrement on 3HZRNST >10% decrement on 3HZ 

 Single Fiber EMG (most specific)Single Fiber EMG (most specific) 

 Thyroid profile, CPKThyroid profile, CPK 

 FBSFBS

Treatent Treatent

 Patient education and counselingPatient education and counseling 

 Avoidance of precipitatorsAvoidance of precipitators 

 Acute:Acute:

o

o IvIG/Plasma ExchangeIvIG/Plasma Exchange

 MaintenanceMaintenance

o

o Acetylcholine esterase inhibitorsAcetylcholine esterase inhibitors o o ImmunosuppressantImmunosuppressant o o SteroidSteroid o o AzathioprineAzathioprine o o MMFMMF o o CyclophosphamideCyclophosphamide 

 ThymectomyThymectomy -For all pa-For all patients tients even if there iseven if there is

no thymoma no thymoma

(27)

Friedreich’s ataxia

Friedreich’s ataxia: : Pyramidal+ Pyramidal+ LMN+ CeLMN+ Cerebeller+ rebeller+ Dorsal Dorsal ColumnColumn

 Young adult Young adult with Kyphoswith Kyphoscoliosiscoliosis 

 PPes cavuses cavus 

 PPyramidal type of weakness in lower limbyramidal type of weakness in lower limb 

 PPlanter Bilateral upgoinglanter Bilateral upgoing 

 PPeripheral neuropathyeripheral neuropathyDiminished / absentDiminished / absent

reflex reflex

 PPosterior column signosterior column sign 

 Bilateral Cerebeller signs Bilateral Cerebeller signs (Ataxia, Dysarthri(Ataxia, Dysarthria,a,

nystagmus) nystagmus)

I would like to complete with I would like to complete with

 HOCMHOCM 

 Hearing difficultyHearing difficulty aidaid 

 DiabetesDiabetes 

 Low IQLow IQ 

 Optic atrophyOptic atrophy 

 High arch palateHigh arch palate 

 PacemakerPacemaker 

 Insulin prick markInsulin prick mark

Autosomal recessive GAA repeat Frataxin gene Autosomal recessive GAA repeat Frataxin gene

Investigation Investigation

 NCS- slowing of motor velocitiesNCS- slowing of motor velocities 

 Genetic analysisGenetic analysis 

 ECG ECHOECG ECHO 

 Vitamin E levelVitamin E level 

 Functional MRIFunctional MRI 

 FBC, U/E/ FBS HbA1CFBC, U/E/ FBS HbA1C 

 MRI of brain and spinal cordMRI of brain and spinal cord 

 VEP/ AERVEP/ AER 

 AudiometyAudiomety

Management Management

 MDT –MDT – Neurologist/ geneticist/ genetic Neurologist/ geneticist/ genetic

counselor/physiotherapist/ speech and counselor/physiotherapist/ speech and

language therapist/occupational therapist and language therapist/occupational therapist and social worker

social worker

 Hearing aidHearing aid   SymptomaticSymptomatic   AntidepressantAntidepressant   PacemakerPacemaker

Exam possibility= Spstic paraparesis / Imballance Exam possibility= Spstic paraparesis / Imballance If asked

If asked Lower Lower limb= limb= Start Start from from gaitgait Wasting+ pes cavus+ UMN + post column+ + absent jerk Wasting+ pes cavus+ UMN + post column+ + absent jerk +cerebeller+cerebeller If asked upper limb= See tremor in

If asked upper limb= See tremor in hand to rule out parkinson’shand to rule out parkinson’s if noif no look for cerebeller signslook for cerebeller signs FSHD

FSHD (mostly u(mostly upper limbpper limb)) 1. Myopathic face

1. Myopathic faceWasting & weakness of facial muscleWasting & weakness of facial muscle D/D- D/D- (MG/ (MG/ MD)MD) 2. No Ptosis and eye movements are

2. No Ptosis and eye movements are normalnormal 3. Proximal muscle weakness and wasting 3. Proximal muscle weakness and wasting 4. Superior margin of scapula are visible from 4. Superior margin of scapula are visible from frontfront 5. Winging of scapula with positive Beevor sign 5. Winging of scapula with positive Beevor sign 6. Foot drop 6. Foot drop 7. Hearing aid 7. Hearing aid Fasciculation cause Fasciculation cause   MNDMND   SyringomyeliaSyringomyelia 

 Cervical myelopathyCervical myelopathy 

(28)

 HSMNHSMN 

 Electrolyte disturbance( hypokalemia, hypomagnesemia)Electrolyte disturbance( hypokalemia, hypomagnesemia)

Proximal myopathy Proximal myopathy

D/D-D/D- Cushing’s, Acromegally, Osteomalacia,Cushing’s, Acromegally, Osteomalacia, Thyroid ds, PMR, PM, Thyroid ds, PMR, PM, DM, MG, electrolytes, Drugs, Muscular dystrophiesDM, MG, electrolytes, Drugs, Muscular dystrophies Station

Station 3- 3- MG, MG, Muscular Muscular dystrophies dystrophies Station Station 5- 5- othersothers Inspection:

Inspection:

 Face- cushingoid/ acromegally/ myopathic face/ eye- Face- cushingoid/ acromegally/ myopathic face/ eye- for Gravesfor Graves 

 NeckNeck – – thyroid, Scar, thyroid, Scar, 

 Hands- clubbingHands- clubbing 

 Legs- pretibial myxedemaLegs- pretibial myxedema 

 Chest- thymectomy scarChest- thymectomy scar 

 Rash- DMRash- DM 

 Gait- myopathic gaitGait- myopathic gait 

(29)

MND MND

Management Management

General: Patient education, counseling, MDT, Patient autonomy for

General: Patient education, counseling, MDT, Patient autonomy for future choice, early involvement of palliative carefuture choice, early involvement of palliative care Specific:

Specific:

 Symptom control-Symptom

control-a.

a. Baclofen for spasticityBaclofen for spasticity b.

b. Analgesia for painAnalgesia for pain c.

c. Anticholinergic for drooling of salivaAnticholinergic for drooling of saliva d.

d. Magnesium and Vitamin E for FasciculationMagnesium and Vitamin E for Fasciculation e.

e. SSRI for depressionSSRI for depression

 NIVNIV 

 PEGPEG 

(30)

Cardiovascular

Cardiovascular

Aortic Stenosis Aortic Stenosis Cause Cause 

 Bicuspid aortic valveBicuspid aortic valve 

 

 CongenitalCongenital 

 Rheumatic feverRheumatic fever 

 Supravalvular aortic stenosisSupravalvular aortic stenosis 

 Degenerative in old peopleDegenerative in old people

Severity Severity

1.

1. Slow rising pulseSlow rising pulse 2.

2. Narrow pulse pressureNarrow pulse pressure 3.

3. Systolic thrillSystolic thrill 4.

4. Heaving apexHeaving apex 5.

5. Soft S2Soft S2 6.

6. S4S4 7.

7. Late peaking of long duration murmurLate peaking of long duration murmur 8.

8. Pulmonary hypertension and congestionPulmonary hypertension and congestion 9.

9. Reverse spiltReverse spilt Investigation

Investigation

 FBC to look for anemiaFBC to look for anemia 

 Blood cultures for IEBlood cultures for IE 

 LFTs to look for coagulation profileLFTs to look for coagulation profile 

 Urine dipstick for hematuriaUrine dipstick for hematuria 

 ECG to look ECG to look for LV for LV strain patterstrain patternn 

 X ray chest to lX ray chest to look for post stenotic dialatation of aorta, pulmonary congestionook for post stenotic dialatation of aorta, pulmonary congestion 

 EchocardiographEchocardiography to look for LV y to look for LV size and ejection function, valve area ansize and ejection function, valve area and gradientd gradient 

 Coronary AngiographyCoronary Angiography 

 Echo criteria for severe ASEcho criteria for severe AS

• Aortic valve area: < 1cm2

• Aortic valve area: < 1cm2 • Jet Velocity: > 4.0m/sec • Mean • Jet Velocity: > 4.0m/sec • Mean transvalvular pressure: > 40mmHgtransvalvular pressure: > 40mmHg

Management Management General-

General- patient education , patient education , counseling, vaccination, counseling, vaccination, RestRest Symptomatic AVR if syncope/ angina/ Af

Symptomatic AVR if syncope/ angina/ Af

Asymptomatic-Median sternotomy scar Median sternotomy scar

 Metallic valve replacementMetallic valve replacement 

 Tissue valve replacementTissue valve replacement 

 CABGCABG 

 Corrective surgery of congenital heartCorrective surgery of congenital heart

Disease Disease

 Heart transplantHeart transplant 

(31)

 IE prophylaxis for Dental, IE prophylaxis for Dental, Genitourinary and Colonic procedureGenitourinary and Colonic procedure  prophylaxis prophylaxis 

 Moderate/ severe stenosis undergoing other Moderate/ severe stenosis undergoing other cardiac surgerycardiac surgery 

 Gradient of > 40 mm Hg with any of the followingGradient of > 40 mm Hg with any of the following

o

o LVEF < 50%LVEF < 50% o

o Abnormanl BP response on ETTAbnormanl BP response on ETT o

o LVH > 15mmLVH > 15mm o

o VTVT o

o Valve area < 0.6 cmValve area < 0.6 cm22

Aortic regurgitation Aortic regurgitation

Cause Cause

Acute:

Acute: Trauma , hypertension, Aortic dissection, rupture , Aortic root Abscess, IE  Trauma , hypertension, Aortic dissection, rupture , Aortic root Abscess, IE ,, Chronic:

Chronic: Bicuspid Bicuspid aorticaortic valve, Degeneration, Rheumatic, Marfan’s , valve, Degeneration, Rheumatic, Marfan’s , Ehlar danolos syndrome,Ehlar danolos syndrome, Aortitis

Aortitis – – Syphilic/ TakayasSyphilic/ Takayasu/RA/ SLE/ Au/RA/ SLE/ Ank Spondylosisnk Spondylosis

D/D-D/D- Pulmonary regurgitation, Mitral Stenosis, Tricuspid stenosis,Pulmonary regurgitation, Mitral Stenosis, Tricuspid stenosis, Severity

Severity

 Wide pulse Pressure > 100Wide pulse Pressure > 100 

 Displaced apexDisplaced apex 

 Thrusting apexThrusting apex 

 Long duration murmurLong duration murmur 

 Austin flint murmurAustin flint murmur diastolic murmur at apex without opening snapdiastolic murmur at apex without opening snap 

 LVFLVF

Investigation Investigation

 FBC to look for anemiaFBC to look for anemia 

 Blood cultures for IEBlood cultures for IE 

 LFTs to look for coagulation profileLFTs to look for coagulation profile 

 Urine dipstick for hematuriaUrine dipstick for hematuria 

 ECG to look ECG to look for LV for LV strain patterstrain patternn 

 X ray chest to lX ray chest to look for Cardiomegally, pulmonary congestionook for Cardiomegally, pulmonary congestion 

 EchocardiographEchocardiography to look for LV y to look for LV size and ejection function, valve area ansize and ejection function, valve area and gradient, aortic root sized gradient, aortic root size

and dilatation and dilatation

References

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