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 follows-

1. 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 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, time management is very 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 ones own ability, memory and concept.

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

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

Best of luck for PACES.

 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 LTOT

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
Surgery-
o Bulectomy/ Lung volume reduction
o Lung transplant
2. Bronchiectasis

Investigation
o Blood-Differential count, ESR, CRP- to look for signs of infection, Immunoglobin 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 +/- Steroids
o LTOT
o Diuretics and Phosphodiesterase inhibitors for Cor pulmonale
Surgery-
o Lobectomy
o Bronchial artery embolisation for hemoptysis
o Lung transplant for cystic fibrosis

3. Pulmonary Fibrosis/ ILD/Rheumatoid lung

Investigation
a. Chest x ray 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 Steroid for ground glass, Thalidomide, Cyclophosphamide,
o Antifibrotic Pirfenidone for IPF ( UIP Pattern on HRCT)
o Diuretics and Phosphodiesterase inhibitors for Cor pulmonale
Surgery-
o 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 , USG 30ml

Management:
1. Therapeutic Pleural 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

Investigation
o Chest x ray 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 not done now a days
Indication for 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 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 to look for confirmation
o CT thorax to rule out any mass lesion
o Blood- Septic screening -Differential count , ESR, CRP, PCT - to look for signs of infection, Urea/ electrolytes-
CURB 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 for confirmation
o CT thorax to rule out any mass lesion, Enlarged L/N
o Blood- Septic screening -Differential count , ESR, CRP-
Urea/ 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/ 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/ metabolic/drugs)

Investigation

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

Severity-
o Synthetic function PT/INR, Albumin
o Renal function
o Bilirubin
o Serum ammonia
To look for complication
o Full Blood count 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( WILSONS)
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 Wislsons- d penicilamine, Zinc
o PBC- UDCA, Immunosuprressant, cholestyraimine, antihistamine
o NASH- Weight reduction, Control of diabetes
Treat complication
o Varix- ABCDE, 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( WILSONS)
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 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( WILSONS)
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, hemolysis
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, hemolysis
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, 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 with 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 and inflammatory markers
o PBS look abnormal cells
o Bone marrow to look for myelo/ 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 MRCP/ CT depending on USG findings
o Viral markers
o Autoimmune profile (AMA,ANA, ASMA, Anti LKM)
 o PBS look abnormal cells, spherocytes,
o Hemoglobin electrophoresis, DCT to look for hemolytic diseases
o HIV serology
o FOR complication
o Coagulation profile
o OGD for esophageal varices
o Renal parameters
o Septic screen
o Ascitic fluid if any
8. Jaundice and Ascites +/_ hepato/ Hepatospleenoegally ( DCLD, , Malignancy ,Disseminated TB, Cardiac
cirrhosis)
o Confirmation: LFTs
o For etiology
o USG of Abdomen followed MRCP/ CT depending on 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
o 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
Recurrent infection
Uncontrolled hemorrhage
Suspected malignancy
Symptomatic mass effect
Extension of polycystic kidney into transplant site.
o 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, compliance 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

Friedreichs / B12 deficiency

No

Check Sensory- Deficit Spinal Level Spinal Cord lesion

Dissociated sensory loss Syringomyelia/ Ant Spinal AO

No Sensory deficit Peipheral nerve pattern Pheripheral neuropathy + Pyramidal

Fasciculation parasagital meningioma

Syringomyelia

MND Heriditary/Tropical Spastic Spastic Diplegia ( Cerebral Palsy)

Paraparesis
 Lower limb LMN Cerebeller Alcohol, drugs

Sensory Deficit

Yes NO Polio

Small wasted limb

Root Lesion Peripheral nerve Both root and nerve Muscle hypertrophy fasciculation

Cauda Diabetes GBS/ CIDP Muscle disease Multifocal Motor NP

GBS Alcohol MND
CIDP HSMN
Diabetic amyotrphy B6/B12
Paraneoplastic
CIDP
Drugs Affect only LMN
Asymmetric, UL and LL
Prominent fasciculation
Autoimmune origin
Anti GM 1 antibody
Treatment IvIg,
Rituximab and
Immunosupressant

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
o M- MS, MND
o A- Ataxia Freidreichs , Cauda conus
o S- SACD, Syphilitic taboparesis
o S- Spinal shock , stroke with PN
 Foot drop

Ankle Jerk

Brisk Present absent

UMN Sensory loss S1 radiculopathy Sciatic nerve

Lateral thigh and leg compartment Part of leg and No sensory loss
& Dorsum of foot Dorsum of foot Bilateral
Usually unilateral Unilateral
H/O Pain
EHL weakness

L5 radiculopathy Common peroneal nerve Myotonic dystrophy

D/D HSMN / trauma/ diabetes IBM
Paraneoplastic

Cause of Pes Cavus

Unilateral Bilateral

Polio HSMN
Burn mascular dystrophy
Taruma spinal mascular atrophy
Syringomyelia

Palpable nerve

HSMN
Acromegally
Leprosy
CIDP
Amyloidosis
Neurofibromatosis
 Upper limb Weakness

Check LL UMN Unilateral stroke ask permission LL/Face

LMN and UMN Bilateral (D/D- bilateral stroke/MND/ PD/ Freidreichs/ cervical 1-4)

MND

Examine neck any scar, kyphoscoliosis

Check Cerebeller Yes MS, Brainstem , Spino Cerebeller Ataxia, Wilsons

Friedreichs No

Check Sensory- No Sensory deficit Fasciculation Yes MND

No

Deficit

Tremor Yes PD

Spinal Level Dissociated sensory loss Peipheral nerve pattern Multiple sclerosis

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

cervical myelopathy
 Upper limb LMN Cerebeller Alcohol, drugs, Syrings,

Sensory Deficit

Yes NO Polio

Small wasted limb

Root Lesion Peripheral nerve Both root and nerve Muscle hypertrophy fasciculation
Dermatomal loss Gloves pattern both/ variable Proximal Distal/proximal

Brachial Neuritis Diabetes GBS/ CIDP Fatigable Multifocal Motor NP

CIDP HSMN MND
GBS Alcohol Cervical Spon
CIDP
Drugs
B6/B12
Paraneoplastic

Affect only LMN

Asymmetric, UL and LL
Prominent fasciculation
Yes No Autoimmune origin
MG Muscle ds Anti GM 1 antibody
LEMS
Treatment IvIg,
Rituximab and
Immunosupressant
Small Muscle wasting of hand

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

1. Hand-
a. 5 movements of thumb 4 movements of fingers 1 movement of little finger
b. DTR c. Sensory d. Cerebeller e. Lower limb/ cranial

WASTING OF HAND

Unilat stroke

LMN UMN Bilateral MND

Parkinsons Ds
Upper cervical
SENSORY

Median and Ulnar nerve

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

MND Polyneuropathy Pancoast Syringomyelia Friedreichs

MMN Neurofibroma ataxia
Myotonic dystrophy Cervical Rib
Disuse atrophy RA Cervical myelopathy (+UMN)
Cachexia Look relevant Brachial plexopathy
IBM

Spinal Cord
Cervical Spondylosis Look at back
Syringomyelia Test sensation over cap area, look for Horners
Anterior Horn Cell
Motor Neuron Disease Fasiculation of the tongue or other muscles, ?bulbar speech
Polio
Spinal muscular atrophy Examination of lower limbs
Nerve Lesions
Median Alone will not give wasting of all small muscles of the hand
Ulnar Should have a claw hand
Peripheral Neuropathy e.g. CMT absent reflexes, forearms > hands, thickened nerves
Brachial Plexus C8-T1 ? clubbed from lung ca, cervical ribs, Horners
Myopathy
Myotonic Dystrophy Appearance, weak neck muscles
Distal myopathy e.g. inclusion body myositis
 3rd nerve

Medical 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

4, 6, V1
Disc- papiloedema, Retinopathy
Ispilat cerebeller
Contra pyramidal- webers

Associated syndromes

Caverous sinus syndrome- III, IV, V1, V2., VI + symatetic fibers horners
Orbital apex- II, III,IV, V1, VI
Superior orbital syndrome- III, IV, V1, VI

4th nerve

Inspection: affected eye in higher than other in neutral position.

H impairment of the affected eye adducted eye cant 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

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

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 stem
4. 8th CP angle
5. Disc- DN/HTN/OA/papiloedemafalse localizing sign
6. Long tract sign - Brain stem syndrome- Millard gublers syndrome VI+ VII+ contra hemiparesis

Important causes of cranial nerve palsy Relevant

1. Infection- basal meningitis
2. Infarction- stroke N- Nerve 1up and 1 down until normal
3. Inflammation- MS I- Ipsilateral cerebeller
4. Trauma C- Contralateral pyramidal
5. Mononeuritis multiplex E Eye fundus for raised ICT
6. Autoimmune condition
7. Diabetes

7th Nerve

1. UMN- stroke/ tumor/ demyelination/ trauma

2. B/L UMNMND, pseudobulber palsy present jaw jerk, taste sensation preserved
3. LMN- Bells palsy, basal meningitis, Leprosy, sarcoid, Ramsay hunt , Lyme disease, HIV
4. B/L LMN- GBS, b/L bells 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 examination with

corneal reflex,
 otoscopy,
formal audiometry and
examination of upper and lower limbs.

Bulbar and Pseudobulbar palsy

Pseudobulbar Bulbar

UMN LMN
CN V, VII (IX, X,XII) CN IX, X,XII
Facial expression, mastication Diminished gag
Bilateral degeneration of corticobulbar tracts Tongue fasciculation, wasting
Gag reflex, tongue spasticity Jaw jerk normal
Jaw jerk exagerated Unilateral raspy voice
Spastic dysarthria Bilateral nasal speech
Daffy Duck GBS, Stroke, MND
MS, MND

Myotonic Dystophy:

Face Autosomal Dominant ,CTG trinucleotide repeat

Loss of facial and neck musculature sequence in
Frntotemporal balding DM 1=Chromosome 19
Difficulty in opening eyes after firm closure DM2= Chromosome 3
Drooping mouth
Dysarthria Investigation
Cataract Dive bomber potential in EMG
Low IQ CPK mild elevation
Hands Muscle biopsy- variability in fiber size and
Distal wasting and weakness LMN pattern fibrosis
Percussion myotonia FBS and Hba1c, LFTs
NO Sensory loss ECG to look for conduction block, Echo
Lower Limb Genetic testing
Distal wasting and weakness LMN pattern MRI brain brain atrophy
Foot drop and high steppage gait
Others Management
GYnecomastia Patient education and counseling
Cardiomyopathy, conduction block May die prematurely
Esophagial dysmotility Phenytoin may help myotonia
Hypoventilation Advice against General Anaesthesia
Testicular atrophy
Ptosis

Unilateral Bilateral

Without Without
With ophthalmoplegia ophthalmoplegia With ophthalmoplegia ophthalmoplegia

Third nerve Horners syndrome Fatigable Myotonic

palsy Miosis Myasthenia gravis dystrophy
Mydriasis Partial ptosis Senile
Non fatigable Congenital
Myasthenia Mitocondrial B/L Horners
initial stage myopathy CPEO
fatigable Occulopharyngeal
muscular dystrophy
Miller fisher
syndrome
Neurotoxic snake bite

Steps:

1. Inspection 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

Hormers

position of eyeball. down + out

colour of iris Position of eye Normal

heterochromia please close eyes tightly for me please

follow scheme of nerve
palsy

follow scheme
of Horner s Cant close eye tightly Can close tightly but cant open

Myasthenia Gravis Myotonic dystrophy

Horners Syndrome

Central 1st order Demyelination /Syringomyelia No sweating in face, arm and trunk

2nd order Trauma, surgery, pancoast , Lymph node, goiter No sweating in facearm trunk
normal
3rd order cavernous sinus syndrome, carotid dissection 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, nicotin staining
5. Neck- Scar, dressing, cervical rib palpate, Goiter, palpalte thyroid gland
6. Chest- Scar, Dressing, pancoast
7. Lower limb- if time allows

Presentation

I would like to complete my examination by testing sweating

1. Partial ptosis, which is overcome by voluntary upgaze

2. Apparent Enopthalmos
3. Miosis
4. Scar
Myasthenia Gravis

Fetures Investigation
Fatigable ptosis CBC, ESR
Complex ophthalmoplegia CXR to look for mediastinal mass
Nasal speech CT chest for thymoma
Bulbar weaknessNasal speech, poor swallow Anti AChR antibody 85%
Proximal weakness UL>>LL Anti MuSK antibody 15%
Reflex/ sensory normal Vital capacity
Look for sternotomy scar RNST >10% decrement on 3HZ
Look for features of immunosupession Single Fiber EMG (most specific)
o Cushingoid Thyroid profile, CPK
FBS
Single breath count FVC Treatent
Other autoimmune disease Patient education and counseling
Avoidance of precipitators
LEMS Acute:
limb girdle weakness LL>>UL o IvIG/Plasma Exchange
Rarely Ptosis and diplopia Maintenance
Diminished reflexes become brisk after o Acetylcholine esterase inhibitors
exercise o Immunosuppressant
Associated with malignancy- SCLC o Steroid
Anti voltage gated Ca cannel antibody o Azathioprine
Autonomic dysfunction o MMF
EMG shows second wind phenomenon o Cyclophosphamide
Mx-3,4 Diaminopyridine and treatment of
tumor Thymectomy -For all patients even if there is
no thymoma
 Friedreichs ataxia: Pyramidal+ LMN+ Cerebeller+ Dorsal Column

Young adult with Kyphoscoliosis Investigation

Pes cavus NCS- slowing of motor velocities
Pyramidal type of weakness in lower limb Genetic analysis
Planter Bilateral upgoing ECG ECHO
Peripheral neuropathyDiminished / absent Vitamin E level
reflex Functional MRI
Posterior column sign FBC, U/E/ FBS HbA1C
Bilateral Cerebeller signs (Ataxia, Dysarthria, MRI of brain and spinal cord
nystagmus) VEP/ AER
Audiomety
I would like to complete with Management
HOCM MDT Neurologist/ geneticist/ genetic
Hearing difficulty aid counselor/physiotherapist/ speech and
Diabetes language therapist/occupational therapist and
Low IQ social worker
Optic atrophy Hearing aid
High arch palate Symptomatic
Pacemaker Antidepressant
Insulin prick mark 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 parkinsons if no look for cerebeller signs

FSHD (mostly upper limb)

1. Myopathic face Wasting & weakness of facial muscle D/D- (MG/ MD)
2. 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
 HSMN
Electrolyte disturbance( hypokalemia, hypomagnesemia)

Proximal myopathy

D/D- Cushings, 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 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 control-
a. 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 does not improve symptom, does not prevent death. Prolong survival 3 month after 18 month therapy.
 Cardiovascular
Aortic Stenosis
Median sternotomy scar
Cause Metallic valve replacement
Tissue valve replacement
Bicuspid aortic valve
CABG
Corrective surgery of congenital heart
Congenital Disease
Rheumatic fever Heart transplant
Supravalvular aortic stenosis Thymectomy
Degenerative in old people

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
X ray chest to look for post stenotic dialatation of aorta, pulmonary congestion
Echocardiography to look for LV size and ejection function, valve area 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 , hypertension, Aortic dissection, rupture , Aortic root Abscess, IE ,

Chronic: Bicuspid aortic valve, Degeneration, Rheumatic, Marfans , Ehlar danolos syndrome,
Aortitis Syphilic/ Takayasu/RA/ SLE/ 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
X ray chest to look for Cardiomegally, pulmonary congestion
Echocardiography to look for LV size and ejection function, valve area 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)

Asymptomaticpatient undergo replacement if Rule of 50

o LV Ejection fraction <50%

o Aortic root diameter > 50 mm
o Pulse pressure > 100mm Hg

Mixed aortic Valve disease

Stenosis predominant AR predominant

Pulse Low volume, High volume
Character slow rising Collapsing
Apex Undisplaced Displaced
Pulse Pressure Low Wide
(May Ask)

Prosthetic Aortic valve

Metallic click which 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 valvular dysfunction
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
Life expectancy shorter than life expectancy of valve
Advantage: No need for warfarin, Safe in Child bearing age and elderly
Disadvantage: Short life of prosthesis, due degeneration and calcification

Complication

IE
Thromboembolism
Hemolysis
Valve dysfunction Heart failure
Over/under anti coagulation

Mitral Stenosis (D/D- left Arial Myxoma, Left 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 MVA Gradient

Mild >1.5 cm2 <5 mm Hg
Moderate 1-1.5 cm2 5-10 mm Hg
 Severe <1 cm2 >10 mm Hg

Investigation

FBC to look for anemia

Blood cultures for IE
LFTs to look for coagulation profile
Urine dipstick for hematuria
ECG to look for Atrial Fibrillation, left atrial enlargement
X ray chest to look for Double right heart border, Left Atrial enlargement, pulmonary congestion
Echocardiography to look for LV size and ejection function, valve area and gradient,
Coronary Angiography

Management

General- patient education , counseling, vaccination, Rest, IE prophylaxis

Specific include

Medical: management of

o asymptomatic IE prophylaxis and follow up

o Symptomatic Heart failure/ atrial fibrillation and Pulmonary hypertension surgery
Surgical indication : BMV/ CMC/ MVR

o Hemoptysis
o Recurrent thromboembolic event despite anticoagulation
o Hoarseness of voice ( Ortners syndrome)
o Pulmonary hypertension
PBMV- wilkins score Low / minimal MR/ No thrombus in LA

Mitral Regurgitation ( D/D- MR/ TR/ VSD/ ASD)

MR cause
Acute: IE, Papilary muscle dysfunction MI, Trauma
Chronic- MVP, Reumatic fever, Connective tissue disorder dilated LV ( functional MR),Post MI

Severity

Soft S1
S3, S4, Displaced apex,
Systolic thrill
 Diastolic flow murmur
Pulmonary hypertension and congestion

Investigation
FBC to look for anemia
Blood cultures for IE
LFTs to look for coagulation profile
Urine dipstick for hematuria
ECG to look for Atrial Fibrillation, left atrial enlargement, previous infarction
X ray chest to look for Double right heart border, Cardiomegally, pulmonary congestion
Echocardiography (TTE/ TOE)to look for LV size and ejection function, valve area and gradient,
Coronary Angiography

Management

General- patient education , counseling, IE prophylaxis, vaccination, Rest,

Specific include

Medical:

asymptomatic IE prophylaxis and follow up with echo cardiography

Symptomatic Anticogulation for Af or embolic phenomenon

Diuretic , Beta blocker and ACE inhibitors
Percutaneous Mitral clip device for palliation in inoperative cases of MVP

Surgical indication : Valve repair ( preferable) with annuloplasty ring or MVR

Asymptomatic patient LVEF <60% , LVESD > 45 mm

Symptomatic (NYHA III/ IV) despite medical management

Mixed Mitral Disease

Stenosis predominant MR predominant

Pulse Low volume, High volume
Apex Undisplaced Displaced
S1 Loud Soft
Pulse Pressure Low Wide
(May Ask)

Prosthetic Mitral valve

Must comment on Pallor, Valve function, Over anticoagulation, IE status

 Systolic murmur pathological

CC s2 oc flow murmur Target INR- 2.5-3.5

Complication of valve

Operative mortality 3-5%

Late complication
o Thromboembolism 1-2% per year despite warfarin
o Bleeding Fatal 0.6%, major 3%, minor 7% per anum on warfarin
o Valve obstruction from thrombosis
o Dehicence of valve
o BIoprosthetic dysfunction and LVF - usually within 10 yr
o Hemolysis- mechanical in metal valve
o Infective endocarditis-
Early Staph epidermiditis from skin
Late-Sterpto Viridans hematogenous spread
A second valve replacement needed to treat
Mortality of IE in prosthetic valve approx 60%
A fib for MVR

Investigations

FBC to look for anemia

Serial Blood cultures for IE
LFTs and coagulation profile to see adequacy
Urine dipstick for hematuria
ECG to look for Atrial Fibrillation, left atrial enlargement,
X ray chest to look for Double right heart border, Cardiomegally, pulmonary congestion
Echocardiography (TTE/ TOE)to look for LV size and ejection function, valve area and gradient

Infective endocarditis
Dukes criteria

Major Minor

Blood culture- Typical organism in 2 blood culture and or Pyrexia

Predisposed valve- prosthetic
Embolic phenomenon
Immunologic/ vasculitis phenomenon- rash
 Atypical organism in blood c/s
Echocardiography- abscess/ large vegetation/ dehiscence Diagnose IE= 2 major/ 1 moajor+ 3 minor/ 5 minor

Patients at risk

Patients with prosthetic valve, cardiac transplant

Acquired valvular heart disease with stenosis and regurgitation
Structural congenital heart disease
a. Including surgically corrected or palliated structural conditions
b. Excluding isolated ASD, fully repaired VSD/ PDA and closure device considered to be endothelialized
Hypertrophic cardiomyopathy
Previous episode of endocarditis

If patients at risk are undergoing a gastrointestinal or genitourinary procedure at site where infection is
suspected, they should receive appropriate antibacterial therpy that includes cover against that cause
endocarditis.
Patients at risk of IE should be
a. Advised to maintain good oral hygiene
b. Told how to recognize signs of IE and when to seek expert advice

NICE do not advocate prophylaxis even for high risk patients whereas AHA recommends so.

Rheumatic fever Streptococus pyogenes

Ducket Jones criteria 2 major / 1 major + 2 minor

Major Minor
C Carditis Arthalgia
E Erythema marginatum Fever
N nodules Subcutenous High ESR
C Chorea Raised WBC
E eryhtema marginatum Prolong PR
R Previous Rheumatic fever
Treatment General and specific

Rest
High dose aspirin
Penicillin
Prophylaxis-
o Primary Penicilin V or clindamycin for 10 days
o Secondary Pencilin V for 5-10 yrs