Paces Notes Sc 2017

PACES NOTES INVESTIGATION AND MANAGEMENT

Subhankar Chatterjee, Kolkata 5/1/2017

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 the case. The usual questions which they ask are as follows1. Tell me the positive clinical finings- You should take no more than 30-40 sec for this 2. What are the differential diagnosis- It may take some time as you may be interrupted if going in wrong direction. But never > 1min. 3. Which investigations would you do for this patient?- 40 sec 4. How do you manage the case? –  Again  Again 40-60 sec. If you have answered all of the above in correct manner, you may have further questions which will fetch you towards  full marks. So, So, time management is very important. important. 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, you have small time to 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 notes which might be more effective if made according to one’s own ability, memory and concept.  At the end I have tried to simplify some Neurology scenarios in a schematic pattern, which helps in recognition during examination. Obviously this note carries contribution from all of my teachers, mentors, books and notes targeting PACES. Best of luck for PACES.

pattern

Respiratory system

1. COPD Sir, my INVESTIGATIONs include : 

Blood for FBC to look for Polycythemia



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



Chest x ray /HRCT to look for hyperinflation and Bulla



Pulmonary function test to look for Obstructive pattern with reduced DLCO



ECG and Echocardiography to rule out Cor pulmonale



Sputum microscopy and C/S to isolate any organism



6min walking test for functional assessment



Arterial blood Gas to decide about L TOT

My TREATMENT plan include: 





General measures o

Stop smoking

o

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

o

Vaccination

Specific medical o

Bronchodilator- inhaled B agonist/ ICS/ LABA/LAMA

o

Antibiotic and systemic steroid for acute exacerbations

o

Mucolytics

o

LTOT

o

Diuretics and Phosphodiesterase inhibitors for Cor pulmonale

Surgeryo

Bulectomy/ Lung volume reduction

o

Lung transplant

2. Bronchiectasis 

Investigation o

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

o

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

o

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

o

Pulmonary function test to look for Obstructive pattern

o

ECG and Echocardiography to rule out Cor pulmonale

o

6min walking test for functional assessment

o

Sweat test if suspect cystic fibrosis



Management



General measures

o

Stop smoking

o

Pulmonary rehabilitation ( Pt education, Nutrition, Psychological support, Physiotherapy, POSTURAL DRAINAGE)

o 



Vaccination

Specific medical o

Mucolytics

o

Bronchodilator- inhaled B agonist/ ICS/ LABA/LAMA

o

Antibiotic- systemic / inhalational inhalational +/- Steroids

o

LTOT

o

Diuretics and Phosphodiesterase inhibitors for Cor pulmonale

Surgeryo

Lobectomy

o

Bronchial artery embolisation for hemoptysis

o

Lung transplant for cystic fibrosis

3. Pulmonary Fibrosis/ ILD/Rheumatoid lung Investigation a. Chest x ray – ray –to to look for bilateral interstitial reticulo nodular pattern. Loss of lung volume b. HRCT to look for subpleural reticulation and traction broncheitasis, basal honeycombing , ground glass patterns c.

Pulmonary function test to look for Restrictive pattern with reduced DLCO

d. Blood-Differential count for eosinophilia, ESR, CRP- to look for signs of infection e. Markers for Autoimmune profile, ACE level, Ca, f.

Brocnchoscopy and BAL /Sputum microscopy and C/S to rule out any organism

g.

ECG and Echocardiography to rule out Pulmonary HTN

h. 6min walking test for functional assessment i.

ABG to decide about LTOT

Management 





General measures o

Stop smoking and toxic or allergic substances

o

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

o

Vaccination

o

LTOT

Specific medical o

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

o

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

o

Diuretics and Phosphodiesterase inhibitors for Cor pulmonale

Surgeryo

Single or Double lung transplantation

4. Pleural effusion Investigation a. Chest x ray to look for blunt costophrenic and cardiophrenic angles b. CT thorax to look for any MASS c.

USG to rule out multisepted/ loculated effusion

d. Pleural fluid assessment for cellularity, protein, sugar, cultures, malignant cell, special tests. e. Blood-Differential count , ESR, CRP- to look for signs of infection, Albumin, Urea/ electrolytes to rule out any renal disease, LFT to rule out any liver disease f.

ECG and Echocardiography to rule out Pulmonary HTN/ cardiac decompensation

g.

Markers for Autoimmune profile

h. Pleural biopsy in refractory cases i.

Other Special tests as required

Diagnosis of plerual effusion Clinically 500ml, x ray PA 300ml, x ray lat decubitus-180ml d ecubitus-180ml , USG 30ml Management: 1. Therapeutic Pleural drainage drainage if Symptomatic, infective ( empyema) 2. Treatment of the cause 3. Decortication/intrapleuarl thrombolytics for multiloculated effusion 4. Pleurodesis for refractory malignant effusion

5. Lobectomy / Pneumonectomy Pneumonectomy Investigation o

Chest x ray – ray –to to look for confirmation

o

CT thorax to look for underlying cause

o

Pulmonary function test to look for Obstructive

o

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

o

ECG and Echocardiography to rule out Pulmonary HTN

o

Other Special tests as required

Management:





o

Supportive

o

Management of the original disease

Indication of Lobectomy o

Localized broncheictasis with massive hemoptysis

o

Lung abscess

o

Solitary pulmonary nodule

o

Trauma

o

Aspergiloma

o

NSCLC T3AN0M0

o

TB – TB – not  not done now a days

Indication for Pneumonectomy Pneumonectomy



o

Massive Broncheictasis

o

Multiple lung abscess

o

Fungal infection Malignancy

o

Trauma

o

Bronchial obstruction with destroyed lung

o

Congenital lung disease

o

Malignant mesothelioma/ disseminated thymoma

Indication of lung transplant o

COPD

o

IPF

o

Cystic fibrosis

o

Alpha 1 antitrypsin deficiency

o

Primary pulmonary hypertension

6. OLD TB- Fibrothorax Investigation o

Chest x ray – ray –to to look for confirmation

o

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

o

Pulmonary function test to look for volume loss

o

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

o

ECG and Echocardiography to rule out Pulmonary HTN

o

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

Management: 1. General 

Smoking cessation/ Pulmonary rehabilitation

Specific 

Bronchodilator



Early antibiotic therapy for suspected infection

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

Chest x ray – ray –to to look for confirmation

o

CT thorax to rule out any mass lesion lesion

o

Blood- Septic screening -Differential count , ESR, CRP, PCT - to look for signs of infection, Urea/ electrolytesCURB 65,

o

ABG to look for hypoxemia/ hypercapnoea

o

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

o

ECG and ECHO to rule out pulmonary embolism

o

Bronchoscopy with BAL for microbiology, cytology

o

If septic screen and embolism negative- send autoimmune profile

Management 



General measures o

Stop smoking and toxic or allergic substances

o

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

o

Vaccination

Specific medical o

Pneumonia

Antibiotics/ Bronchodilator/ Mucolytics

o

Infarction

LMWH/ warfarin/ modification of risk factor

o

Mass

Chemo/ radiotherapy/ surgery

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

Chest x ray – ray – for  for confirmation

o

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

o

Blood- Septic screening -Differential count , ESR, CRPUrea/ electrolytes/ LFT / ACE level

o

ABG to look for hypoxemia

o

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

o

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

Management 



General measures o

Physiotherapy

o

Positive pressure ventilation

Specific medical o

Mucus plug

Bronchodilator/ Mucolytics/ Mucolytics/ Bronchoscopy and lavage

o

Malignancy

Chemo/ radiotherapy/ Bronchoscopy may need stenting

o

Others

Bronchoscopy and treatment of the cause , may need stenting

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

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

o

Blood- FBC/ Urea/ electrolytes/ LFT

o

ECG /ECHO

o

CT Angiography / venography

o

Brochoscopy with BAL for microbiology, cytology , Biposy ,

Management 



General measures o

Elevation of head,

o

Positive pressure ventilation

Specific medical o

Steroids ( Dexamathasone) and diuretics

o

Radiotherapy/ chemothapy

o

SVC Stenting

Abdomen 1. CLD ( D/D Alcohol/ viral/autoimmune/ viral/autoimmune/ metabolic/drugs) metabolic/drugs) Investigation 

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



Severity-





o

Synthetic function

o

Renal function

o

Bilirubin

o

Serum ammonia

PT/INR, Albumin

To look for complication o

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

o

USG/ AFP to rule out HCC

o

OGD

to look for varices

To look for etiology o

Alcohol and drug history

o

Metabolic profile ( NASH)

o

Viral markers

o

Cerulopasmin and urinary copper study( WILSON’S)

o

Ferritin (HH)

o

Liver biopsy( NASH, PBC, PSC)

o

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

o

Alpha 1 antitrypsin

Management 

MDT



Treat underlying cause





o

Alcohol: abstinence

o

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

o

HH- venesection, iron chelation

o

Wislson’sWislson’s- d penicilamine, Zinc

o

PBC- UDCA, Immunosuprressant, cholestyraimine, antihistamine

o

NASH- Weight reduction, Control of diabetes

Treat complication o

Varix- ABCDE, Ligation, Ligation, B blocker

o

Encephalopathy- rifaximin, treat precipitants

o

Ascites- Diuretics, paracentesis

o

SBP- Antibiotic, drainage

o

Hepatorenal- Albumin, Terlipressin

o

HCC- resection, embolization, transplant

Definitive management- LIVER Transplant

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

USG abdomen for confirmation of diagnosis



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



To look for etiology o

Alcohol and drug history

o

Metabolic profile ( NASH)

o

Viral markers

o

Cerulopasmin and urinary copper study( WILSON’S)

o

Ferritin (HH)

o

Liver biopsy( NASH, PBC, PSC)

o

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

o

Alpha 1 antitrypsin

o

AFP for HCC

o

Renal parameters and urinary protein for Nephrotic syndrome

o

ECHO for CCF/ CP/ RCM

o

Thrombophilia workup and CECT abdomen for Veno-occusive disease

Management: 

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



Medical- Diuretics- Spironolactone, Furosemide



Treatment of SBP



Therapeutic paracentesis

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

Investigation 1. confirmation of diagnosis- USG abdomen 2. Look for etiology o

Alcohol and drug history

o

Metabolic profile ( NASH)

o

Viral markers

o

Cerulopasmin and urinary copper study( WILSON’S)

o

Ferritin (HH)

o

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

o

Alpha 1 antitrypsin

o

AFP for HCC

o

ECHO for CCF/ CP/ RCM

o

Liver biopsy ( NASH, PBC, PSC)

3. Look for complications o

LFTs

o

PT, INR, Albumin

o

FBC with inflammatory markers

o

CECT ABDOMEN

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

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

Confirmation: USG of Abdomen

o

Doppler study to look for portal vein thrombosis

o

FBC and inflammatory markers

o

PBS look for malaria, kala azar, he molysis

o

Bone marrow to look for myeloprolifearative disorders

o

JAK 2 mutation, Philadelphia chromosome

o

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

o

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

o

HIV

o

CXR for mediastinal enlargement

Management General: Patient education Specific : treat underlying cause. Spleenectomy for severe hyperspleenism. (Influenza/ Hemophilus/ pneumococcus, meningococcus vaccine needed after spleenectomy) Prophylactic Penicilline for at least 2 yrs.

5. Hepatospleenomagally Investigations: o

Confirmation: USG of Abdomen

o

Doppler study to look for portal vein thrombosis

o

FBC and inflammatory markers

o

PBS look for malaria, kala azar, he molysis

o

Bone marrow to look for myeloprolifearative mye loprolifearative disorders

o

JAK 2 mutation, Philadelphia chromosome

o

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

o

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

o

HIV

o

CXR for mediastinal enlargement, ACE level for sarcoidosis

Management General: Patient education Specific : treat underlying cause. Spleenectomy for severe hyperspleenism. (Influenza/ Hemophilus/ pneumococcus, meningococcus vaccine needed after spleenectomy) Prophylactic Penicilline for at least 2 yrs.

6. Heptospleenomegally Heptospleenomegally with Lymphadenopathy Lymphadenopathy ( d/d Lymphoma, leukemia in blast crisis, disseminated TB, SLE, sarcoidosis, Infectious Mononeucleosis syndrome, HIV) Investigations: o

Confirmation: USG of Abdomen

o

Doppler study to look for portal vein thrombosis

o

FBC to see leucocyte count count and inflammatory markers

o

PBS look abnormal cells

o

Bone marrow to look for myelo/ mye lo/ Lympho prolifearative disorders

o

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

o

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

o

HIV serology

o

CXR for mediastinal enlargement, ACE level for sarcoidosis

o

LFTs, Renal parameters,

Management General: Patient education Specific : treat underlying cause

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

Confirmation: LFTs

o

For etiology o

USG of Abdomen followed followed MRCP/ CT depending on USG USG findings

o

Viral markers

o

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

o

o

PBS look abnormal cells, spherocytes,

o

Hemoglobin electrophoresis, DCT to look for hemolytic diseases

o

HIV serology

FOR complication

8.

o

Coagulation profile

o

OGD for esophageal varices

o

Renal parameters

o

Septic screen

o

Ascitic fluid if any

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

o

Confirmation: LFTs

o

For etiology

o

o

USG of Abdomen followed followed MRCP/ CT depending on USG USG findings

o

Viral markers

o

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

o

Ascitic fluid for Cellularity, SAAG, Malignant cell, Culture

o

Laparoscopy and peritoneal biopsy

o

Echocardiography

For complication o

Coagulation profile

o

OGD for esophageal varices

o

Renal parameters

o

Septic screen

Management General: Patient education Specific : treat underlying cause

9. Polycystic Kidney Investigation 

USG abdomen for Confirmation and measuring the number of cysts



CBC to look for anemia/ polycythemia



Renal function, Calcium and phosphate to look for secondary hyperparatyroidism



Chest x ray to look for fluid overload



Urine dipstick to look for hematuria/ protein



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



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



Genetic study for potential donors with no cysts on US



Echocardiography to look for MVP, AR

Management:





General o

Patient education and counseling about course of disease and complication

o

Attempt 3lit fluid intake per day to suppress ADH secretion

o

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

o

Avoid contact sports/risk of trauma to abdomen

Specific medical o

ACEI for control of hypertension

o

Avoid nephrotoxic drugs

o

Antibiotics for UTI/ cyst infection

o

Renal replacement therapy

o

Indication of Nephrectomy

o



Recurrent infection



Uncontrolled hemorrhage



Suspected malignancy



Symptomatic mass effect



Extension of polycystic kidney into transplant site.

Management of Hematuria- bed rest, hydration and analgesia

10. Transplanted Kidney Investigations 

USG Doppler to confirm diagnosis and assess graft



To check that the graft is working



o

FBC

o

Renal Function

o

Calcium and Phosphate

o

CXR for volume overload

o

ABG for metabolic acidosis

To look for complication of immunosupression o

Septic screen for patients with fever

o

Renal function/ LFT ( Cyclosporin )

o

Fasting lipid/ sugar ( tacrolimus)

o

Screen for opportunistic infection

o

Biopsy from transplanted kidney to look for Graft failure

Management 

MDT



Patient education and counseling, c ompliance to immunosuppressant



Monitoring for toxicity of immunosuppressant



o

FBC, Renal parameters, LFT

o

Septic screen

o

Dermatology surveillance for PTLD

Treat acute presentation of sepsis with antibiotic/ Antiviral/ antifungal



Renal replacement therapy if graft function deteriorates despite adequate immunosupression o

Uremic encephalopathy

o

Pericarditis

o

Refractor fluid overload

o

Decompensated metabolic acidosis and hyperkalemia

Lower limb Weakness

Quadruplegia

Bilateral

UMN   Unilateral  stroke ask permission UL/Face

Examine neck Lower cranial Nv cerebreller Paraparesis/ paraplegia

Check Cerebeller   Yes  MS, Brainstem , Spino Cerebeller Ataxia Friedreich’s / B12 deficiency No

Check Sensory- Deficit  Spinal Level Spinal Cord lesion Dissociated sensory loss Syringomyelia/ Ant Spinal AO No Sensory deficit

Fasciculation

Peipheral nerve pattern  Pheripheral neuropathy + Pyramidal Pyram idal

parasagital meningioma Syringomyelia

MND

Heriditary/Tropical Spastic Paraparesis

Spastic Diplegia ( Cerebral Palsy)

Lower limb LMN LMN  Cerebeller Alcohol, drugs

Sensory Deficit

Yes

NO 

Polio Small wasted limb

Root Lesion

Peripheral nerve

Both root and nerve

Muscle hypertrophy

fasciculation

Cauda GBS CIDP Diabetic amyotrphy

Diabetes Alcohol HSMN B6/B12 Paraneoplastic CIDP Drugs

GBS/ CIDP

Muscle disease

Multifocal Motor NP MND

Both UMN and LMN Lower limb    

No Sensory Loss- ALS Dissociated Sensory Loss- Syrings Absent KJ+ brisk AJ, Planter ↑= Conus lesion Absent ankle jerk + planter ↑↑ M- MS, MND o A- Ataxia Freidreich’s Freidreich’s , Cauda conus o S- SACD, Syphilitic taboparesis o o S- Spinal shock , stroke with PN

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

Foot drop

Ankle Jerk

Brisk

UMN

Lateral thigh and leg compartment & Dorsum of foot Usually unilateral H/O Pain EHL weakness

L5 radiculopathy

Present

Sensory loss

absent

S1 radiculopathy

Part of leg and Dorsum of foot Unilateral

No sensory loss Bilateral

Common peroneal nerve D/D HSMN / trauma/ diabetes Paraneoplastic

Myotonic dystrophy IBM

Cause of Pes Cavus

Unilateral

Bilateral

Polio

HSMN

Burn

mascular dystrophy

Taruma

spinal mascular atrophy Syringomyelia

Palpable nerve      

HSMN Acromegally Leprosy CIDP Amyloidosis Neurofibromatosis

Sciatic nerve

Upper limb Weakness

Check LL

LMN and UMN

UMN   Unilateral  stroke ask permission LL/Face

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

MND Examine neck any scar, kyphoscoliosis

Check Cerebeller  Yes  MS, Brainstem , Spino Cerebeller Ataxia, Wilson’s

Friedreich’s Friedreich’s

No

Check Sensory- No Sensory deficit

Fasciculation 

Yes

MND

No Deficit Tremor

Spinal Level

Spinal Cord lesion

cervical myelopathy

Dissociated sensory loss

Peipheral nerve pattern

Multiple sclerosis

Syringomyelia/ Ant Spinal AO

Pheripheral neuropathy + Pyramidal

Yes PD

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

Sensory Deficit

Yes

NO 

Polio

Small wasted limb

Root Lesion Dermatomal loss

Peripheral nerve Gloves pattern

Both root and nerve both/ variable

Muscle hypertrophy fasciculation Proximal Distal/proximal

Brachial Neuritis CIDP GBS

Diabetes HSMN Alcohol CIDP Drugs B6/B12 Paraneoplastic

GBS/ CIDP

Fatigable

Yes MG LEMS

No Muscle ds

Multifocal Motor NP MND Cervical Spon

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

Small Muscle wasting of hand D/DMND, Cervical myelopathy, Syrings, combined median and ulnar nerve damage,Peripheral neuropathy( CMT) , C8T1 lesion ( cervical r ib, Pancoast tumor, trauma) 1. Handa. 5 movements of thumb 4 movements of fingers b. DTR c. Sensory d. Cerebeller

1 movement of little finger e. Lower limb/ cranial

WASTING OF HAND

Unilat stroke LMN

UMN

Bilateral  MND Parkinson’s Ds Upper cervical

SENSORY Median and Ulnar nerve

No sensory loss

gloves pattern

Dermatomal pattern C8-T1

MND Polyneuropathy MMN Myotonic dystrophy Disuse atrophy RA Cachexia Look relevant IBM Spinal Cord Cervical Spondylosis Syringomyelia Syringomyelia Anterior Horn Cell Motor Neuron Disease Polio Spinal muscular atrophy Nerve Lesions Median Ulnar Peripheral Neuropathy Brachial Plexus C8-T1 Myopathy Myotonic Dystrophy Distal myopathy

Dissociated

P ancoast Syringomyelia Neurofibroma Cervical Rib Cervical myelopathy (+UMN) Brachial plexopathy

Look at back Test sensation over cap area, look for Horners Fasiculation of the tongue or other muscles, ?bulbar speech Examination of lower limbs Alone will not give wasting of all small muscles of the hand Should have a claw hand e.g. CMT –  CMT –  absent  absent reflexes, forearms > hands, thickened nerves ? clubbed from lung ca, cervical ribs, Horner’s Appearance, weak neck muscles e.g. inclusion body myositis

cerebeller+ dorsal

Friedreich’s ataxia

3rd nerve Medical – Medical – DM,  DM, MS, Basal meningitis, Vasculitis, GCA Surgical- Trauma, PCA aneurysm, tumor

Inspection- Complete ptosis, Lift the eyelid  see eyeball down and out  see pupil miosis/ mydriasis H Diplopia maximal on contalateral superior gaze Check – Check – 

4, 6, V1



Disc- papiloedema, Retinopathy



Ispilat cerebeller



Contra pyramidal- weber’s

Associated syndromes 

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



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



Superior orbital syndrome- III, IV, V1, VI

4th nerve Inspection: affected eye in higher than o ther in neutral position. H impairment of the affected eye adducted eye can’t look down. Diplopia maximum at looking down and away from the affected side and cover test that outer image from affected side.

INO

6th  Nerve meningitis, Vasculitis, False localizing sign, sign, CP angle Mass Cause- DM, MS, Basal meningitis,

Approach inspection : Convergent strabismus H impaired abduction of affected eye  Diplopia cover test ask which image disappear? Outer/ inner. Relavant 1. Associated 3rd , 4th – search Gaze palsy 2. Associated V1- Cavernous 3. 7th – Brain  – Brain stem 4. 8th – CP  – CP angle 5. Disc- DN/HTN/OA/papiloedema— DN/HTN/OA/papiloedema—false localizing sign 6. Long tract sign - Brain stem stem syndrome- Millard gubler’s syndrome VI+ VII+ contra hemiparesis

Important causes of cranial nerve palsy 1. Infection- basal meningitis 2. Infarction- stroke 3. Inflammation- MS 4. Trauma 5. Mononeuritis multiplex 6. Autoimmune condition 7. Diabetes

Relevant N- Nerve 1up and 1 down until normal I- Ipsilateral cerebeller C- Contralateral pyramidal E – Eye fundus for raised ICT

7th Nerve 1. UMN- stroke/ tumor/ demyelination/ trauma 2. B/L UMN— UMN—MND, pseudobulber palsy  present jaw jerk, taste sensation preserved 3. LMN- Bell’s palsy, basal meningitis, Leprosy, sarcoid, Ramsay hunt , Lyme disease disease,, HIV 4. B/L LMN- GBS, b/L bell’s palsy 5. Muscle ds- MG, Muscular dystrophy, FSHD, Mitochondrial myopathy Approch a.

Parotid scar previous surgery

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

Ipsilateral sixth , contralat hemiparesis medial pons

d. Ear herpes/ pox Ramsay Hunt e. Mastoiditis Base of skull f.

UMN with hemiparesis- MCA stroke

I would like to complete my e xamination with 

corneal reflex,



otoscopy,



formal audiometry and



examination of upper and lower limbs.

Bulbar and Pseudobulbar palsy Pseudobulbar         

UMN CN V, VII (IX, X,XII) Facial expression, mastication Bilateral degeneration of corticobulbar tracts Gag reflex, tongue spasticity Jaw jerk exagerated Spastic dysarthria “Daffy Duck” MS, MND

Bulbar        

LMN CN IX, X,XII Diminished gag Tongue fasciculation, wasting Jaw jerk normal Unilateral – Unilateral – raspy  raspy voice Bilateral – Bilateral – nasal  nasal speech GBS, Stroke, MND

Myotonic Dystophy: Face       

Loss of facial and neck musculature Frntotemporal balding Difficulty in opening opening eyes after firm closure Drooping mouth Dysarthria Cataract Low IQ

Hands Distal wasting and weakness LMN pattern Percussion myotonia  NO Sensory loss  Lower Limb Distal wasting and weakness LMN pattern  Foot drop and high steppage gait  Others GYnecomastia  Cardiomyopathy, conduction block  Esophagial dysmotility   Hypoventilation Testicular atrophy  

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

Investigation Dive bomber potential in EMG   CPK mild elevation Muscle biopsy- variability in fiber size and  fibrosis FBS and Hba1c, LFTs  ECG to look for conduction block, Echo  Genetic testing  MRI brain brain atrophy  Management Patient education and counseling  May die prematurely  Phenytoin may help myotonia myotonia  Advice against General Anaesthesia 

Ptosis Unilateral

With ophthalmoplegia 



Third nerve palsy Mydriasis

Bilateral

Without ophthalmoplegia

Without ophthalmoplegia

With ophthalmoplegia

Horner’s syndrome Miosis Partial ptosis

Fatigable Myasthenia gravis 





Non fatigable Mitocondrial  myopathy CPEO Occulopharyngeal  muscular dystrophy Miller fisher  syndrome Neurotoxic snake bite 

Myasthenia initial stage fatigable

 

Myotonic dystrophy Senile Congenital B/L Horner’s

Steps: 1. Inspection – Inspection – one  one eye/ both eye, 2. Come near to the patient without thouching the patient  complete / partial 3. Lift the eyelid see Pupil.

Miosis

Normal

Dilated

Hormer’s position of eyeball. down + out colour of iris

heterochromia

Position of eye Normal

please close eyes tightly for me please follow scheme of nerve palsy

follow scheme of Horner’ s

Cant close eye tightly

Myasthenia Gravis

Can close tightly but can’ can’t open

Myotonic dystrophy

Horner’s Syndrome

Central 1st order

Demyelination /Syringomyelia

No sweating in face, arm and trunk

2nd order

Trauma, surgery, pancoast , Lymph node, goiter

3rd order

cavernous sinus syndrome, carotid dissection

No sweating in facearm trunk normal Sweating normal

Sequence 1. Partial Ptosis ask to look up. 2. Lift the eyelid colour of iris ( congenital heterochromia), Ask to dim the light for pupil, light reflex 3. Eye movement H 4. Hands- Samll muscle wasting, clubbing, clubbing, nicotin staining staining 5. Neck- Scar, dressing, cervical rib palpate, Goiter, palpalte palpalte thyroid gland 6. Chest- Scar, Dressing, pancoast 7. Lower limb- if time allows Presentation I would like to complete my e xamination by testing sweating 1. Partial ptosis, which is overcome by voluntary upgaze 2. Apparent Enopthalmos 3. Miosis 4. Scar

Myasthenia Gravis Fetures        

 

Fatigable ptosis Complex ophthalmoplegia Nasal speech Bulbar weakness— weakness—Nasal speech, poor swallow Proximal weakness UL>>LL Reflex/ sensory normal Look for sternotomy scar Look for features of immunosupession Cushingoid o Single breath count – count – FVC  FVC Other autoimmune disease

LEMS   

    

limb girdle weakness LL>>UL Rarely Ptosis and diplopia Diminished reflexes become brisk after exercise Associated with malignancy- SCLC Anti voltage gated Ca cannel antibody Autonomic dysfunction EMG shows “second wind” phenomenon Mx-3,4 Diaminopyridine and treatment of tumor

Investigation CBC, ESR  CXR to look for mediastinal mass  CT chest for thymoma  Anti AChR antibody 85%  Anti MuSK antibody 15%   Vital capacity RNST >10% decrement on 3HZ  Single Fiber EMG (most specific)  Thyroid profile, CPK  FBS  Treatent Patient education and counseling  Avoidance of precipitators   Acute: IvIG/Plasma Exchange o Maintenance  Acetylcholine esterase inhibitors o Immunosuppressant o Steroid o Azathioprine o MMF o Cyclophosphamide o 

patients even if there is Thymectomy -For all patients no thymoma

Friedreich’s ataxia: ataxia: Pyramidal+ LMN+ Cerebeller+ Cerebeller+ Dorsal Column     

 

Young adult adult with Kyphoscoliosis Kyphoscoliosis Pes cavus Pyramidal type of weakness in lower limb Planter Bilateral upgoing Peripheral neuropathyDiminished / absent reflex Posterior column sign Bilateral Cerebeller signs (Ataxia, Dysarthria, Dysarthria, nystagmus)

I would like to complete with HOCM   Hearing difficulty aid Diabetes  Low IQ  Optic atrophy  High arch palate  Pacemaker  Insulin prick mark 

Investigation NCS- slowing of motor velocities  Genetic analysis  ECG ECHO  Vitamin E level  Functional MRI   FBC, U/E/ FBS HbA1C MRI of brain and spinal cord  VEP/ AER  Audiomety  Management MDT – MDT – Neurologist/  Neurologist/ geneticist/ genetic  counselor/physiotherapist/ speech and language therapist/occupational therapist and social worker Hearing aid  Symptomatic  Antidepressant  Pacemaker 

Autosomal recessive GAA repeat Frataxin gene

Exam possibility= Spstic paraparesis / Imballance If asked Lower limb= Start from gait

Wasting+ pes cavus+ UMN + post column+ + absent jerk +cerebeller

If asked upper limb= See tremor in hand to rule out parkinson’s

if no

look for cerebeller signs

FSHD (mostly upper upper limb) limb) 1. Myopathic face Wasting & weakness of facial muscle  D/D2. No Ptosis and eye movements are normal 3. Proximal muscle weakness and wasting 4. Superior margin of scapula are visible from front 5. Winging of scapula with positive Beevor sign 6. Foot drop 7. Hearing aid

Fasciculation cause 

MND



Syringomyelia



Cervical myelopathy



Thyrotoxic myopathy

(MG/ MD)



HSMN



Electrolyte disturbance( hypokalemia, hypomagnesemia)

Proximal myopathy D/D- Cushing’s, Acromegally, Osteomalacia, Thyroid ds, PMR, PM, DM, MG, electrolytes, Drugs, Muscular dystrophies Station 3- MG, Muscular dystrophies

Station 5- others

Inspection: 

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



Neck – Neck – thyroid,  thyroid, Scar,



Hands- clubbing



Legs- pretibial myxedema



Chest- thymectomy scar



Rash- DM



Gait- myopathic gait



Finish UL/ LL as per scheme in 5 min 1 min for relevant

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

Symptom controla. Baclofen for spasticity b. Analgesia for pain c.

Anticholinergic for drooling of saliva

d. Magnesium and Vitamin E for Fasciculation e. SSRI for depression 

NIV



PEG



Riluzol – Riluzol – does  does not improve symptom, does not prevent death. Prolong survival 3 month after 18 month therapy.

Cardiovascular Aortic Stenosis Cause 

Median sternotomy scar 

Bicuspid aortic valve



  



Congenital



Rheumatic fever





Supravalvular aortic stenosis





Degenerative in old people

Metallic valve replacement Tissue valve replacement CABG Corrective surgery of congenital heart Disease Heart transplant Thymectomy

Severity 1. Slow rising pulse 2. Narrow pulse pressure 3. Systolic thrill 4. Heaving apex 5. Soft S2 6. S4 7. Late peaking of long duration murmur 8. Pulmonary hypertension and congestion 9. Reverse spilt Investigation 

FBC to look for anemia



Blood cultures for IE



LFTs to look for coagulation profile



Urine dipstick for hematuria



ECG to look for LV strain pattern pattern



X ray chest to look l ook for post stenotic dialatation of aorta, pulmonary congestion



Echocardiography Echocardiography to look for LV size and ejection function, valve area and and gradient



Coronary Angiography



Echo criteria for severe AS • Aortic valve area: < 1cm2 • Jet Velocity: > 4.0m/sec • Mean transvalvular pressure: > 40mmHg

Management General- patient education , counseling, vaccination, Rest Symptomatic AVR if syncope/ angina/ Af Asymptomatic-



IE prophylaxis for Dental, Genitourinary and Colonic procedure  prophylaxis



Moderate/ severe stenosis undergoing other cardiac surgery



Gradient of > 40 mm Hg with any of the following o

LVEF < 50%

o

Abnormanl BP response on ETT

o

LVH > 15mm

o

VT

o

Valve area < 0.6 cm2

Aortic regurgitation

Cause Acute: Trauma Acute: Trauma , hypertension, Aortic dissection, rupture , Aortic root Abscess, IE , Chronic: Bicuspid aortic valve, Degeneration, Rheumatic, Marfan’s , Ehlar danolos syndrome, Aortitis – Aortitis – Syphilic/ Takayasu/RA/ Takayasu/RA/ SLE/ Ank Ank Spondylosis

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

Wide pulse Pressure > 100



Displaced apex



Thrusting apex



Long duration murmur



Austin flint murmur diastolic murmur at apex without opening snap



LVF

Investigation 

FBC to look for anemia



Blood cultures for IE



LFTs to look for coagulation profile



Urine dipstick for hematuria



ECG to look for LV strain pattern pattern



X ray chest to look l ook for Cardiomegally, pulmonary congestion



Echocardiography Echocardiography to look for LV size and ejection function, valve area and and gradient, aortic root size and dilatation



Coronary Angiography

Management General- patient education , counseling, vaccination, Rest, IE prophylaxis Specific include Medical: management of 

Hypertension



Heart failure



PAH



Associated pathology

Surgical AVR for patient who are Symptomatic (angina/ Heart failure) Asymptomatic— Asymptomatic—patient undergo replacement if “Rule of 50” o

LV Ejection fraction 50 mm

o

Pulse pressure > 100mm Hg

Mixed aortic Valve disease

Pulse Character Apex Pulse Pressure (May Ask)

Stenosis predominant Low volume, slow rising Undisplaced Low

AR predominant High volume Collapsing Displaced Wide

Prosthetic Aortic valve 

Metallic click which coincides coincides with S2



No peripheral stigmata of IE



No sign of fluid overload



No sign of over anti coagulation Must mention if any



ESM  flow murmur



Diastolic murmur murmur valvular dysfunction



Anemia Anemia blood loss due to anti coagulation, hemolysis, IE

Prosthesis function normal or not? 

Diastolic murmur is due to malfunctioning Aortic prosthesis



Decreased intensity of closing click ( clot or vegetation)

Bioprosthetic 

Age >70 yrs



Anticoagulation contraindicated contraindicated



Life expectancy shorter than life expectancy of valve



Advantage: No need need for warfarin, Safe in Child bearing age age and elderly



Disadvantage: Short life of prosthesis, due degeneration and calcification calcification

Complication 

IE



Thromboembolism



Hemolysis



Valve dysfunction Heart failure



Over/under anti coagulation

Mitral Stenosis (D/D- left Arial Myxoma, Left Atrial Atrial thrombus, Austin flint Murmur) Cause 

Rheumatic fever



Congenital



RA

 

SLE Carcinoid

Severity 

Low pulse pressure



Early opening snap, Low S2 OS gap



Longer duration murmur



Pulmonary hypertension



Graham Steel Murmur

Echo criteria Grade Mild Moderate

MVA >1.5 cm2 1-1.5 cm2

Gradient