UMS Medical Short Cases Records 1st Edition

Cheo, Jun, Lee, Shoban Medical Short Cases Record
Medical Short Cases Record

Cheo Seng Wee (Chief Editor)
Tan Yi Jun, Lee Hui Juin, Shoban Raj

1. Vulvular Heart Disease
2. Prosthetic Heart Valve
3. Bronchiectasis
4. Pleural Effusion
5. Thalassaemia
6. Chronic liver disease
7. Graves disease
8. Rheumatoid arthritis
9. Ankylosing spondylitis
10. Scleroderma
11. Parkinson Disease
12. Stroke

Publish Date : 11
th
April, 2012


Case 1 : Valvular Heart Diseases
Presentation :
Sir, I would like to complete my examination with checking the peripheral signs of Aortic Regurgitation, check
the temperature chart, check fundus for Roth Spots, urine for hematuria. I would also like to measure the
blood pressure for her and I would expect a wide pulse pressure.
On general examination, this is a young lady who appears to be well and not in any forms of distress or pain.
She has no stigmata of infective endocarditis such as Janeway lesion, Osler nodes and splinter hemorrhages.
She has a regular pulse rate of 80 beats/minutes and collapsing in nature. No radio-radio, no radiofemoral
disease. She has no signs of anemia and not cyanotic. She has no signs to suggest heart failure as well, JVP is
not raised.
In the precodrium, there is no scar, no deformity. She has a displaced apex beat at left 6
th
intercoastal space at
anterior axillary line. No parasternal heave, no thrills. On auscultation, first and second heart sound can be
heard. There is a grade 4 harsh pan-systolic murmur at mitral area radiating to axilla, accentuated by
expiration suggestive of Mitral Regurgitation. There is a grade 3 early diastolic murmur, best heart over the left
lower sternal edge that suggestive of Aortic Regurgitation. No loud P2 or gallop rhythm. Lung is clear. No
hepatomegaly.
In summary, this is a young lady with mitral regurgitation and aortic regurgitation most likely secondary to
chronic rheumatic heart disease and clinically not in heart failure. No signs of pulmonary hypertension or
infective endocarditis.
What are your differential diagnosis for multiple valvular heart lesions?
Chronic rheumatic heart disease, Infective endocarditis
Name other causes of collapsing pulse.
Pregnancy
Patent Ductus Arteriosus (PDA)
Pagets Disease
Anemia
Thyrotoxicosis
What are the peripheral signs of Aortic Regurgitation?
Corrigans: visible vigorous neck pulsation
Quinckes: nail bed capillary pulsation
De Mussets: head nodding
Duroziezs: diastolic murmur proximal to femoral artery compression
Traubes: pistol shot sound over the femoral arteries
Mullers sign systolic pulsations of the uvula


What are investigations, would you like to do ?
ECG
- MR: p-mitrale, atrial fibrillation and previous MI (Q wave)
- AR: Lateral T wave inversion
CXR
- MR: Cardiomegaly, enlargement of the left atrium and pulmonary edema.
- AR: Cardiomegaly, widened mediastinum and pulmonary edema
ECHO
- MR :
a. Severity: size/density of MR jet, LV dilatation and reduced EF
b. Cause: prolapse, vegetations, ruptured papillae and infarction
- AR:
a. Severity: LV ejection fraction and dimensions, root dimensions
b. Cause: intimal dissection flap or vegetation
How would you manage a patient with chronic Mitral regurgitation?
Control rate if fast AF
Anticoagulants are not indicated unless there is: a history of systemic embolism; a prosthetic mitral
valve, either xenograft or mechanical; additional mitral stenosis with a low output or AF.
Diuretics are needed to reduce pulmonary venous congestion and LV preload.
Afterload reduction with intravenous nitrates or nitroprusside is indicated in acute MR by helping to
reduce the regurgitant fraction and increase forward stroke volume. Afterload reduction in acute MR is
less successful than in aortic regurgitation. ACE inhibitors are used routinely but with little evidence of
their long-term benefit.
In acute MR with chordal rupture and pulmonary oedema, a continuous positive airway pressure
(CPAP) mask or artifi cial ventilation and full monitoring as in cardiogenic shock may be necessary
Infective endocarditis prophylaxis should be considered
How would you follow up a patient with aortic regurgitation and name the indication for aortic valve
replacement in such patient.
All patients need antibiotic cover for dental or surgical procedures
Long-acting nifedipine has been shown to delay the development of LV dysfunction in chronic aortic
regurgitation
Indication for surgery
- Aortic valve should be replaced before LV dysfunction develops
- The indications are:
a. Symptoms of increasing dyspnea and LVF
b. Enlarging heart in CXR or ECHO
c. Infective endocarditis that does not responds to treatment


Case 2 : Prosthetic Valve
Presentation :
Sir, I would like to complete my examination with checking the peripheral signs of Aortic Regurgitation, check
the temperature chart, check fundus for Roth Spots and urine for hematuria. I would also like to measure the
blood pressure for him as well.
My patient is middle aged man lying comfortably propped up in bed at 45 degree supported by one pillow. He
is not cachexic, not pale, not jaundiced and not cyanosed. I can appreciate audible metallic click. Purpura
noted over the extremities (evidence of over warfarinization).
On peripheral examination, there is no evidences of infective endocarditis such as Osler nodes, Janeway lesion,
splinter hemorrhage, no clubbing, no needle marks. He has a regular pulse of 80beats/min, not collapsing in
nature, no radio-radio, no radio-femoral delay. JVP is not raised and no pedal edema.
In the precordium, there is a midline sternotomy scar measuring 15 cm. No chest wall deformity. Visible
pulsation appreciated all over the precordium. He is a displaced apex beat at left 6
th
intercostal space, anterior
axillary line. No thrills, parasternal heave or palpable P2.
Upon auscultation, metallic first heart sound is heard and normal second heart sound. Metalic first heart
sound is best heard at the mitral area. No murmur. (Sometimes ESM heard normal)
No basal crepitations. No hepatomegaly or splenomegaly.
In summary, this patient has metallic prosthetic mitral valve and clinically not in heart failure. He has signs to
suggest overwarfarinization. No evidence of IE.
What are the types of prosthetic valve
a. Mechanical valves
Caged ball
Tilting disk
The valves are very durable, but have a higher thromboembolism rate than xenografts. Very occasionally a
patient or his or her partner may be disturbed by the audible valve clicks. The double tilting disc valves have
much better flow profiles than the Caged ball valve and have largely superseded it.

b. Bioprostheses
Heterograft
Homograft
Biological valves do not have as good long-term durability as mechanical valves and may need replacing at
about 810 years (mitral) or 1015 years (aortic).


Common complications of prosthetic valve

Valve dysfunction leakage, dehiscence, obstruction
Systemic thromboembolism stroke
Bleeding - warfarin
Bacterial endocarditis
Hemolysis

What are the considerations involved in choosing mechanical or bioprosthetic valve?

A bioprosthesis is preferred in older patients and in patients in whom lifetime anticoagulation poses
important risks. This includes persons with clotting disorders, and gastrointestinal problems with the
potential for bleeding and persons who may not be able to comply with required anticoagulant
medication and follow-up testing.
The major disadvantage of biologic prostheses is primary valve failure as a result of leaflet
degeneration, which limits their functional life span.
Mechanical heart valves, which have greater durability than bioprosthetic valves, are usually preferred
in patients younger than 65 years without contraindications to long-term anticoagulation.

What are the signs suggestive of prosthetic valve infection?

Change in their valve sounds
New symptom, however vague: dyspnoea, night sweats, myalgia, anorexia.
In mechanical valves the opening and closing sounds of either ball or disc should be clear and sharp,
not muffled. Vegetations may restrict ball or disc movement and muffle the relevant prosthetic sounds.
Check ECG for PR interval prolongation (septal abscess).


Case 3 : Bronchiectasis
Presentation :
Sir, I would like to complete my examination by checking the temperature chart and sputum cup for this
patient. I would also like to ask for history of tuberculosis in the past.
On general examination, this is a middle aged man who appears to be well and not in respiratory distress
(Patients in exam are usually not in respiratory distress). He does not appears to be cachexic or in pain. He has
a respiratory rate of 16breaths/min.
In the periphery, he has grade 3 clubbing and nicotine stain. No evidence of HPOA. No evidence of CO2
retention as there is no palmar erythema, no flapping tremor, no bounding pulse. His pulse is regular. His JVP
is not raised, no conjunctiva pallor. No lower limb edema. No palpable cervical lymph nodes.
In the precodium, he has no evidence of mediastinal shifting as trachea is centra and apex beat is not deviated.
No chest deformity or scar noted. His chest expansion, vocal fremitus, vocal resonance appears to be equal on
both side. Percussion note is resonance in all lung field.
On auscultation, vesicular breath sound can be heard. There is presence of bilateral end-inspiratory coarse
crepitations. No prolonged expiratory phase/rhonchi. No loud P2.
In summary, this is a middle aged man with bronchiectasis and clinically not in respiratory distress. Most likely
etiology is pulmonary tuberculosis.

What are your differential diagnosis?
Bronchiectasis
Lung fibrosis
Bronchogenic Carcinoma
Chronic Lung abscess
What is Bronchiectasis ?
Chronic suppurative inflammation of the bronchi that results in permanent dilatation of the airways

What are the investigations you would like to do?
Sputum for culture and cytology
CXR tramlines and ring shadows
High Resolution CT Thorax - signet ring sign, thickened dilated bronchi larger than the adjacent
vascular bundle.


Causes of Bronchiectasis
Congenital: Kartageners and cystic fibrosis
Mechanical: bronchial carcinoma (suspect if localized bronchiectasis)
Childhood infection: measles and TB
Immune OVER activity: allergic bronchopulmonary aspergillosis (ABPA);
Immune UNDER activity: hypogammaglobulinaemia
Aspiration: chronic alcoholics and GORD

Treatment :
Mainstay physiotherapy
A Antibiotic
B Bronchiodilator
C Corticosteroid
D Drainage ( Postural Drainage)
S Surgery ( occasionally for localized disease)

Complication of bronchiectasis :
Hemoptysis
Cor pulmonale
Secondary amyloidosis ( Dip urine for protein)
Pneumonia
Cerebral abscess

Management of hemoptysis in bronchiectasis :
Medically : Transnexamic acid & mefenamic acid (mild)
Bronchoscopy : Balloon tamponade, iced saline lavage, topical medications, laser therapy, and
electrocautery.
If failed bronchoscopy, proceed to arteriographic embolization of bleeding sites (typically from a
bronchial artery) by an interventional radiology service.
Lastly, surgical treatment by open lung surgery to resects it.


Case 4 : Pleural Effusion
Introduction :
A pleural fluid is fluid in the pleural space. There are 5 types of pleural effusions exudates, transudates,
empyema, haemothorax, chylous thorax. 500 ml of fluid is needed for clinical detection.
Presentation :
Sir, this is a young man who is lying on the bed with 45 degree propped up. He is in respiratory distress as
evidence by usage of accessory muscles while breathing and is tachypnic with respiratory rate of 24 breaths
per minutes. He is on nasal prong with pulse oxymeter monitoring. Otherwise, his body build is moderate and
is pink.
On examination, he has no clubbing, no cyanosis, no nicotine staining, and no signs of CO2 retention such as
bounding pulse, palmar erythema and flapping tremor. He has no signs to suggest Horners syndrome, good
oral hygiene, no raised JVP and no ankle oedema.
In the chest has no scars, no deformity, no visible pulsation. He has evidence of mediastinal shifting with
trachea deviated to right. He has evidence of massive left sided pleural effusion as evidence by reduced chest
expansion, reduced tactile fremitus, stony dull on percussion, reduced air entry and reduced vocal resonance
over the left upper, middle and lower zone of the left lung.
No cervical lymphadenopathy and hepatomegaly noted. I would like to complete my examination by checking
the fever chart, sputum pot and do a bedside peak expiratory flow rate.
Besides, I would like to look for the underlying cause of this pleural effusion like cardiac failure, chronic liver
disease, hypothyroidism, rheumatoid arthritis and butterfly rash for SLE.
What is your provisional diagnosis?
Left sided massive pleural effusion most probably due to ?? and is SOB at rest.
What are your differentials for dullness over the left base of the lung?
Pleural thickening
Consolidation and collapse of lung
Elevated diaphragm
How would you like to investigate this patient?
I would order an erect PA CXR to look for obliteration of costophrenic angle and meniscus sign. 175ml
of pleural fluid is needed for it to detect PE in CXR.
Pleural tap can be done and send for pleural analysis like gross appearance, cytology, clinical chemistry
like protein, glucose, pH, LDH, Amylase.
If glucose <3.3mmol/L, pH<7.2, LDH (pleural:serum >0.6), it is suggestive of TB, malignancy, RA, SLE,
Empyema.


In autoimmune disorders pleural fluid for RF, ANA and complement factors can be send. If pleural fluid
analysis is inconclusive, I would consider parietal pleural biopsy with Abrams needle for
histopathological examination and mycobacterial culture.
What are the causes of pleural effusion?
Transudate Exudate
Increase venous pressure cardiac failure,
constrictive pericarditis, fluid overload
Hypoproteinaemia cirrhosis, nephrotic
syndrome, malabsorption
Hypothyroidism
Meigs syndrome (Ovarian fibroma and right
pleural effusion)

Inflammatory SLE, RA
Malignancy bronchogenic carcinoma,
malignant metastasis, lymphoma,
mesothelioma, lymphangitis carcinomatosis
Infection TB, pneumonia, pulmonary
infarction

How you differentiate between transudate and exudates?
If Protein <25 g/L, it is transudate.
If protein >35 g/L, it is exudates.
If protein between 25 35 g/L, Lights criteria is used.
Lights criteria for an exudates are applied:
The ratio of pleural fluid to serum protein >0.5
Ratio of pleural fluid to serum LDH >0.6
Pleural fluid of serum LDH is 2/3 the upper normal limit for blood LDH levels.

How would you manage this patient?
Treat the underlying cause.
Drainage removed <2L/24hours
Pleurodesis by tetracycline, talc poweder, bleomycin
Surgery if persistent collections and increase pleural thickness.
What are the conditions associated with bloody pleural fluid?
Malignancy, pulmonary embolism, tuberculosis, and trauma to the chest
What are the causes of exudates with negative cytological findings and pleural fluid lymphocytosis?
TB, malignancy, collagen vascular diseases.
What causes increase amylase of pleural fluid?
Pancreatitis, carcinoma, bacterial pneumonia, oesophageal ruptures.


Case 5 : Thalassaemia (Sabah very own cystic fibrosis)
Introduction :
The thalassaemias are a heterogeneous group of genetic disorders with defective synthesis of one or more
globin chains. In Malaysia, the most common types are the and thalassaemias. The thalassaemias
together with its heterozygous interaction with HbE disease constitute the bulk of the patients load.
The East Malaysian state of Sabah had the most number of registered patients standing at 1,272 with the
Kadazan-Dusun ethnic group contributing almost half of these affected individuals.
History :
1. Pre and post transfusion drop rate?
2. Dx as thallassaemia where? When? By who? How? First presentation?
3. Blood transufusion when start? How frequent? Usually where? Far from home- why? Recently
increase in frequency? Any acute reaction?
4. Start on desfo? When? Why- ferritin increase? How? Compliance? Follow up for desfo toxicity? Any
complication? Electricity prob?
5. Iron overload complication growth retard, Bronze Diabetes, bone pain? When Dx? How? Mx?
6. Follow up(ANE & outpatient attendance) echo? Bone scan? HIV, hep B Hep C screening?
7. Detail Family hx? Screen?
8. Social financial problem? Transport problem? Child care problem?
9. Drugs Vit C? compliance?
10. DIET changes? Increase calcium intake, decrease iron intake?
11. Bone marrow HLA compability

Presentation :
Sir, Muhammad is a 10-year-old boy with pallor and slate gray skin. He has evidence of extramedullary
hemopoesis as evidenced by frontal bossing and maxillary overgrowth. His height and weight appears to be
small for his age and I would like to confirm it by plotting on the growth chart.

On peripheral examination, he has no evidence of chronic liver disease such as leuconychia, palmar erythema,
flapping tremor or bruising. He has conjunctival pallor and tinge of jaundice noted. His oral hygiene appears to
be appropriate.

On inspection of the abdomen, there are multiple injection sites. There is fullness over the right and left
hypochondrium region too. Otherwise, abdomen moves with respiration, umbilicus centrally located and flat.
No visible peristalsis, no dilated veins, no surgical scars, hernia orifices are intact. Abdominal palpation
revealed that there is hepatosplenomegaly with and 8cm liver below left subcostal margin. The liver moves
with repiration, smooth surface, non tender, non pulsatile, well defined margin, firm in consistency, dull on
percussion and no bruit heard. He has a 10cm spleen below left subcostal margin, which move inferior
medially with respiration, cannot get above it, with a splenic notch felt, dull on percussion and splenic rubs


heard. No ballotable kidney and no free fluid in the abdomen. Bowel sounds present. There are no cervical or
inguinal lymphadenopathies.

I would like to complete my examination by checking the cardiovascular system for gallop rhythm and lung
basal crackles, do a Tanner staging, check his plasma glucose level and ask for family history of
hemoglobinopathies.

What is your provisional diagnosis?
These findings are in keeping with a diagnosis of chronic hemolytic anemia most likely Beta Thalassaemia
major, currently anaemic and complicated with iron overload.

Differential for hepatosplenomegaly
Liver Cirrhosis with portal hypertension
Hematological disorders myelo/lymphoproliferative disorders
Infection infectious mononucleosis, acute viral hepatitis, CMV
Connective tissue disease
Infiltrative amyloidosis

How do you approach a patient present with you with anaemia?


What are the investigations you would like to do for her?
First, I would like to confirm the diagnosis first, by doing FBC to look for microcytic hypochromic
anaemia, PBF to look for marked anisocytosis, poikilocytosis (including fragments and tear-drop
poikilocytes), hypochromia and microcytosis. Basophilic stippling, Pappenheimer bodies and target
cells.
Circulating nucleated red cells showing defective haemoglobinisation and dyserythropoietic features
are present. The total white cell count and the neutrophil count might be increased. If hypersplenism
develops, there is leucopaenia, neutropaenia and thrombocytopaenia.
I would also like to do Hemoglobin electrophoresis. A HbF >90% and complete absence of HbA will
confirm him as Thalasaemia major. This must be done before initiation of blood transfusion.

How would you differentiate between iron deficiency anaemia and Thallasaemia from the result of FBC?
I would like to use Mentzer index, which is product of MCV divided by RBC. If less than 13, it is
suggestive of Thalasaemia. If more than 13, it is suggestive of IDA.
In iron deficiency, the marrow cannot produce as many RBCs and they are small (microcytic), so the
RBC count will be low along with the MCV, and as a result, Mentzer's index is not as low, >13
Comparatively, in thalassemia, which is a disorder of globin synthesis, RBC production is preserved, but
the cells are smaller and more fragile. Therefore, the RBC count is normal with a low MCV and the
Mentzer's index is <13.

What are the HbA2 level in beta Thallasaemia trait?
In heterozygosity or severe thallasaemia trait, HbA2 will be 4 9% while mild thallasaemia trait, the HbA2
would be 3.6% to 4.2 %.

How you manage this case before first transfusion?


How you going to manage during routine admission for blood transfusion?
First, I would like to measure his height, weight, liver and spleen size.
Pretransfusion Hb, platelet count, post transfusion Hb (half an hour after transfusion) is done.
Aim pre transfusion Hb 9 -10 g/dL and post transfusion Hb 12-12.5 g/dL.
3 to 6 monthly check the serum ferritin, liver function test and evaluate growth and development.
Every year or more frequent, endocrine assessment RBS, T4/TSH, Ca PO4, Vit D, PTH level
Pubertal and sexual development from 10 years onwards,
Tanner staging for breast and genitalia
Check the FSH, LH, oestradiol or testosterone level.
Infection screen HIV, Hepatitis B, Hepatitis C, VDRL.
Calculate transfusion indices volume of pure RBD transfused/ median weight).
Evaluate iron balance liver iron assessment.
Bone osteoporosis and skeletal abnormalities.
Cardiac assessment at variable intervals especially after 10 years of age yearly ECG, annual cardiac
echography and cardiac T2*MRI

What are the long term complications of blood transfusion?

In chronic iron overload, endocrine puberty delay, growth retardation, hypothyroidism,
hypoparathyroidism, diabetes mellitus.
Cardiac arrhythmias, pericarditis, cardiac failure.
Liver liver cirrhosis.


What will you advice to the patient regarding his daily activities?
I would advise him to avoid contact sports like football that may injure his spleen, causing splenic rupture.

When will you consider splenectomy and what are the anticipate complication?
I would opt for splenectomy if the transfusion rate has increased to 200-250 ml/kg/year (normally 180
ml/kg/year), evidence of hypersplenism, or massive spleen causing discomfort, risk of infarct and splenic
rupture due to trauma.

Complication would be sepsis (OPSI- overwhelming post splenectomy infection), especially by encapsulated
bacteria like Streptococcal pneumonia, Hemophilus Influenza and Neisseria Gonorrhea. Immunoprophylaxis
and chemoprophylaxis can be given.
Besides, Thromboembolic phenomenon is common more in Thallasaemia media and antithrombotic agent can
be given.
Post-splenectomy thrombocytosis is a known complication and the use of low dose aspirin or dypyridamole if
platelet count is more than 800 x 10^9/L may be considered.
When would you start chelation therapy?
It is started when there is >10 units of blood transfusion or ferritin level has increased up to 1000 ng/mL for
more than two occasions two weeks apart.
What are the dietary advice for this patient?
I will give him folate at minimum 1mg OD, low dose Vitamin C and Vitamin E. I would ask him to avoid iron rich
food such as red meat and iron fortified cereals and milk. Avoid tea as it may decrease intenstinal iron
absorption.
Would you recommend him for bone marrow transplant?
NO. It depends on the 3 risk groups, hepatomegaly, iron chelation status and liver fibrosis. This patient has at
least 2 risks including hepatomegaly and bad iron chelation status and hence has less success rate in BMT.
What are the non-invasive ways of estimating tissue iron overload?
Cardiac MRI T2*, Liver MRI R2/ferriscan
How will you advice your female thalassaemia patient who plan to get pregnant?
With advances in hypertransfusion and iron chelation, some women with beta thalassemia major have had
favorable pregnancy outcomes. However, such pregnancies are recommended only in those with normal
cardiac function and adequate hypertransfusion and iron chelation regimens.
Genetic counseling is strongly advised, since these mothers will be transmitting a thalassemic gene to all of
their offspring, and partnership with a male with beta thalassemia may lead to beta thalassemia major in their
offspring.


Who should receive Hematopoietic cell transplantation (HCT) transplant?
In preparation for possible HCT, all children with BTM receive treatment with a hypertransfusion protocol
along with iron chelation therapy (Grade 1A).
For a child with BTM who has an HLA-matched sibling or HLA-matched unrelated donor and who has
undergone rigorous medical therapy (ie, transfusion plus high-quality iron chelation therapy), we recommend
that HCT be performed as soon as is reasonably practical (Grade 1A).
For those without an HLA-matched donor, children with prior poorly-controlled chelation therapy, and adults,
transplant-related mortality can be as high as 35 to 50 percent. The risks and benefits of undergoing either
curative HCT (with its high incidence of transplant-related mortality), or continuing with non-curative medical
therapy (life-long transfusions and chelation therapy) are very patient specific, and a decision favoring one
over the other should be made on a case-by-case basis, depending upon the values and preferences of the
patient/family.
What is the PBF picture of a thalassemia major patient?
Anisocytosis, poikilocytosis, hypochromia, microcytosis, target cells.
What is the PBF picture of a post splenectomy patient?
Howell-Jolly bodies, thrombocytosis, pappenheimer bodies.


What are the difference types of chelation therapy?


Case 6 : Chronic liver disease
Introduction:
Cirrhosis is defined pathologically as fibrosis and abnormal regenerating nodule of liver
Presentation:
Sir, I would like to complete my examination by checking the external genitalia of this patient, do a per rectal
examination and take a good history from this patient.
This is a middle age gentlemen who appears to be well with average built and obvious tattoo mark over both
arm. There are signs of chronic liver disease such as palmar erythema, jaundice, loss of axillary hair and spider
naevi, otherwise there is no clubbing, no duputryen contracture, no hepatic flap, no injection mark, no parotid
enlargement, and no gynaecomastia.
On inspection of the abdomen, there is no scar, no dilated vein. Abdomen is flat and umbilicus is centrally
located and move with respiration. On palpation, there is no hepatomegally but traubes space is dull
suggestive of splenomagaly. There is also evidence of ascites with presence of shifting dullness and fluid thrill.
Bowel sound are heard with no bruit.
In summary, this gentlemen has chronic liver disease most probably due to hepatitis infection and in
decompensated state with presence of ascites but no encephalopathy.
Causes of chronic liver disease:
1. Chronic alcohol ingestion
2. Viral hepatitis
3. Drug: methotrexate, methyldopa
4. Autoimmune hepatitis
5. Wilson disease
6. Primary biliary cirrhosis
7. Alfa 1-antitrypsin deficiency
8. Hemachromatosis
Investigation:
I would like to check full blood count because patient is prone to anemia due to gastrointestinal bleeding,
folate deficiency, or hypersplenism. Liver function test and coagulation profile to check synthetic function of
liver (albumin and APTT). Investigation for the causes of chronic liver disease: hepatitis screening (HbsAg,
HbeAg, anti core antibody, anti-HCV), autoimmune hepatitis screening (antinuclear antibody, anti liver-kidney
microsomal antibody, anti smooth muscle antibody), antimitochondria antibody (for primary biliary cirrhosis),
Wilson disease ( low serum ceruloplasmin and increase 24 hours urine copper secretion, serum protein
electrophoresis for alfa-antitrypsin, serum alfa-feto protein and hepatobiliary system ultrasound. Ascitic fluid
tapping and send for analysis.


Management:
Treat the complication and causes. Treatment for variceal bleeding: resuscitate patient, wide bore needle
insertion and cross match blood. Blood transfusion or use fluid resuscitation while awaiting blood, intravenous
proton pump inhibitor, early endoscopy to indentify bleeding site and treat with endoscopic sclerotherapy
with octreotide or ligation. If severe bleeding may use sangstaken blackmoore tube for temporary stopping
bleeding. Surgery by mean of transjugular intrahepatic portosystemic stent shunt can temporarily reduce the
portal pressure. Definitive is liver transplant.
Complication of chronic liver disease :
Portal hypertension (hepatorenl syndrome, variceal bleeding), hepatoma, ascites, infection eg spontaneous
bacterial peritonitis, hepatic encephalopathy, coagulopathy
Precipitating factor of hepatic encephalopathy :
Hemorrhage, electrolyte imbalance, protein diet, alcohol, trauma, infection, constipation, surgery (HEPATICS)
How to grade encephalopathy?


How to assess severity of cirrhosis?


Case 7 : Graves disease :
Introduction:
Graves disease is autoimmune disease with presence of thyroid stimulating antibodies which bind and
stimulate the receptor. This antibody is found in 90% of patient. Patient will present with hyperthyroid, goiter,
eyes sig, thyroid acropachy and pretibia myxoedema. Commoner in females (9:1). Associated with other
autoimmune disease.
Presentation:
This is a young lady who sits comfortably on chair appears well with appropriate attire, thin and not irritable.
There is a diffuse thyroid swelling which move with deglutition and not with protrusion of tongue. The swelling
is butterfly in shape measuring 10 x 15cm, smooth surface, well define margin, soft in consistency with lower
border can be appreciated. The mass is non tender, no increase temperature, no bruit, and not attach to
overlying or underlying structure. Otherwise, tracheal is not deviated, carotid pulse can be felt and no cervical
lymphadenopathy. There is no evidence of retrosternal exapansion.
On peripheral examination, the pulse rate is 100 beats per minute regularly regular. There is thyroid acropathy,
sweaty warm palm with tremor, exophthalmos, and brisk deep tendon reflex. Otherwise no opthalmoplegia,
no lids lag or lids retraction, no diplopia, no proximal muscle weakness, and no pretibial myxoedema.
I would like to complete my examination by checking the blood pressure, cardiovascular examination and
respiratory examination.
This lady has diffuse thyroid swelling most probably due to Graves disease and currently in hyperthyroid state.
Differential diagnosis for diffuse goiter :
1. Simple diffuse thyroid
2. Hashimoto thyroiditis
3. Subacute thyroiditis

Investigation:
I would like to check thyroid function test where I expect the TSH will be low and free T4 will be high. Thyroid
stimulating antibodies will be presence in 90% of patient. Neck ultrasound to determine solid or cystic
component of the mass.


Management:
There is three modality of treatment: medical, radioiodine and surgical. The antithyroid drugs are carbimazole
and propythiouracil which decrease thyroid peroxidase. Beta blocker eg: propranolol to control adrenergic
symptom and decrease peripheral conversion of T4-T3. Radiotherapy is treatment of choice when fail medical
treatment. Surgery is subtotal or total thyroidectomy.
WHO grading of goiter :
Grade0: no palpable or visible thyroid
Grade 1: palpable goiter(A), palpable and visible with neck extension (B)
Grade 2: goiter visible at normal position
Grade 3: large goiter visible from a distance
Indication for surgery :
Patient preferences, large goiter, fail medical treatment after trial of 2 years, non compliance, recurrent with
no radioiodine facility.
What are the complications of thyroidectomy?
Immediate: hemorrhage, respiratory obstruction, hoarseness of voice due to recurrent laryngeal nerve
injury
Early: infection
Late: hypothyroidism, hyperthyroidism, hypoparathyroidism
How to manage thyroid storm?
Precipitating factors: infection, surgery, trauma, myocardiac infarction
Presentation: agitation, fever, tachycardia, atrial fibrilation, hepatic dysfunction
Treatment: high mortality thus needs to admit to ICU. Give antithyroid drug, propythiouracil is
preferable because decrease peripheral conversion of T3-T4. Sodium iodine or lugols iodine to
decrease hormone releases, which give 1 hour after initial dose of antithyroid drug. Give IV
dexamethasone to decrease hormone release and peripheral conversion. Give IV propanolol if no
heart failure; oxygen, diuretic and digoxin if heart failure presence. Well hydration, tepid sponge with
antipyrexia. Remove or treat the underlying cause.


Case 8 : Rheumatoid Arthritis
Introduction :
RA is the most common inflammatory arthritis in women. The typical clinical phenotype of RA is a symmetrical,
deforming, small and large joint polyarthritis, often associated with systemic disturbance and extra-articular
disease.
Before the age of 45, the female : male ratio is 6:1. Prevalence increases with age, with 5% of women and 2%
of men over 55 years being affected.
Examination steps :
1. Greet but dont shake the hand. Offer pillow to allow patients hand to put on it in prone position.
2. General cushingoid facies, Weight, Eyes, Obvious joint deformity, Well
3. Look - Describe from distal to proximal or vice versa.
a. Nail psoriatic nails, vasculitic changes, splinter hemorrhage (SLE), periungual telangiectasiae,
Raynauds phenomenon, pulp atrophy
b. PID, DIP - skin changes, swelling, subluxation, deformity Swan neck and boutonniere deformity of
fingers, OA changes Heberdens nodes, Bouchards nodes,
Psoriatic plagues, Gottrons papules, calcinosis
Thumb squaring of thumb or Z deformity
c. MCP skin changes, swelling, deformity ulnar deviation, volar subluxation
d. Wrists skin, scars, redness, atrophy, rash,
Tight shiny skin, prominent styloid
e. Palm muscle wasting, skin - palmar erythema, scars, vasculitic changes, palmar creases for
anaemia, Raynauds phenomenon

4. Feel
a. Temperature? Tenderness?
b. Wrist CRES sign! Crepitation, Range of movements, Effusions, Synovitis,
+ulnar styloid tenderness
c. MJP, PIP, DIP CRES + subluxation
d. Palmar tendon crepitus


e. Carpal tunnel syndrome Tinels sign
5. Active movement
a. Wrist flexion and extension
b. Abduction and adduction of thumb
c. Opposition of fingers
d. Prayers sign
e. Test for trigger finger ask patient flex and extend finger
6. Hand function
a. Practical button, key grip, scratch back
7. Complete
a. PULSE!! one important part of the hand
b. Elbow psoriatic plagues, rheumatoid nodules
c. Face
Eyes (episcleritis), scleromalacia, keratoconjunctivitis sicca, cataract (steroid)
Dry mouth,
TMJ crepitus,
Scalp psoriasis over hairline or back of ear
Neck ask patient flex neck and ask if presence of electrical shock wave suggestive of atlanto-axial
deformity
Precordium Aortic/mitral regurgitation, Loud P2, pericardial rub
Lungs Pleural rubs, Basal crackles interstitial fibrosis, pneumonia
Abdomen splenomegaly
Legs ruptured bakers cyst, ulcers (vasculitis), mononeuritis multiplex

Presentation:
Sir, this is a middle-aged lady sitting on the chair comfortably. She has Cushingnoid features as evidenced by
fullness over the supraclavicular fat pad and round face.
The patient has bilateral symmetrical deforming polyarthropathy affecting the metacarpal and interphalangeal
joints sparing the DIP and wrist joint. I noted volatile subluxation and ulnar deviation over MCP joint. The
significant deformities are swan neck deformities over the (which finger) and boutonnires deformity of
(fingers). I also see Z deformity over right thumb. There is skin thinning and I do not notice any rash or nail
changes such as pitting or ridging.
Over the palmar surface, there is thenar wasting and erythema of the palms. No vasculitic ulcers or raynauds
phenomenon noticed. No scars.
On palpation, there is no raise in temperature and tenderness. No crepitus, no swelling, no effusions over the
all wrist, MJP, PIP, DIP joints.
Active movements limited as patient unable to do prayers sign and opposition of fingers.
In terms of hand function, it is impaired as patient unable to button her clothes and do key grip.


I would like to complete my examination by (Refer above)

Provisional diagnosis:
She has bilateral symmetrical deforming polyarthritis due to rheumatoid arthritis, currently in remission and
functional status is impaired.

Differential Diagnosis:
Lupus arthritis
Infective cause : dengue, lyme, rheumatic fever and other viral infection.
Psoriatic arthritis
Reactive arthritis
Osteoarthritis
Jaccoud arthropathy

How do you diagnose RA?
I would like to use the latest 2010 ARA/EULAR classification for RA. A score of >6/10 needed to classify definite
RA.

How do you investigate this patient?
For diagnosis purpose, I would like to check the rheumatoid factors, ACPA and acute phase reactants like ESR
and CRP.
On top of it, I would like to do FBC to look for anaemia, renal function test, liver function test, imaging of the
joints to look for progression of destruction of the joints, soft tissue swelling, juxta-articular osteopenia and
reduced joint spaces. USG (detect early stage) and MRI can identify synovitis more accurately.


What are the other causes that can cause RF to be positive?
Rheumatic diseases scleroderma, SLE, Sjogrens, disease, MCTD
Infections bacterial endocarditis, syphilis, TB, hepatitis
Lung Interstitial fibrosis, chronic bronchitis, silicosis
Liver cirrhosis, sarcoidosis
Healthy people 1-2% increase with age

How would you manage this case?
I would treat early disease aggressively as there is a window opportunity. My goals would be to relieve
symptoms, prevention of symptoms, preserve function, maintain lifestyles. There are two principles
nonpharmacological and pharmacological.
For the non-pharmacological part, I would opt for multidisciplinary approach and refer her to
physiotherapist, social workers, surgeon and occupation therapist.
For the pharmacological part, I would relief her pain by giving her NSAIDS or COX 2 if severe GI
symptoms. However, I would be watch out for renal impairment, cardiac failure and uncontrolled
hypertension.
Early DMARDS is helpful in stabilizing the disability but not reverse it. Examples include
hydrochloroquine, methotrexate, Sulphasalazine, Leflunomide.
They take 6-12 weeks for symptomatic benefit.
While waiting for DMARDS to work, steroid up to 7.5 mg can be given s=and stop gradually after 3 to 4
months. Intraarticular steroid can be used as adjunct too.
If patient is resistant, TNF alpha inhibitors like etanercept, adalimumab or infliximab can be given.
However, before initiation of this, TB work out need to be done as it can reactivate TB. If she is positive,
treat her as latent TB by giving isoniazide and start biological agent after 1 month of isoniazide.
In seropositive arthritis, never stop biological agent once it is started.

What are the common side effects of DMARDS?
Myelosuppresion leads to pancytopenia regular FBC monitoring required.
Methotrexate pneumonitis(need baseline chest xray), oral ulcers, hepatotoxicity (give Folic acids as
supplement)
Hydrochloroquine irreversible retinopathy
Sulfasalazine Rash, reduce sperm count, oral ulcers
Leflunomide teratogenicity

How would you suggest this lady about the medication DMARDS if she wants to get pregnant?
If she is on methtrexate, stop for 3 cycles before she conceived.
If she is on Leflunomide, stop for 2 years.
Other option would be ingestion of cholestryramine to wash out the drugs from blood.
RA gets better with pregnancy and flare up after delivery.


What are the causes of anaemia in RA?
RA can cause all 3 types of anemia :
Microcytic (PUD 2 steroid/NASAIDS),
Macrocytic (associated with pernicious anemia, autoimmune; folate deficiency secondary to
methotrexate)
Normocytic (chronic disease, hypersplenism in felty, aplastic anemia 2 gold/penicilamine).

What are the pulmonary manifestations of RA?
The respiratory complications are listed from proximal to distal airway :
Cricoarytenitis
Bronchiolitis obliterans & organizing pneumonia (BOOP)
Pulmonary fibrosis (MTX) / Pulmonary hypertension.
Caplan nodules ( coal dust exposure).
Pleural effusion.

What are ophthalmic manifestations in RA?
Extraocular muscle (Mononeuritis multiplex, myasthenia 2 penicillamine)
Sclera (Episcleritis, scleritis, scleromalacia perforans)
Conjunctiva (Pallor, keratoconjunctivitis sicca 2 Sjogren syndrome)
Lens & vitreous (Cataract & glaucoma 2 prolonged steroid)
Fundus (Vasculitis, chloriquine Bulls eye maculopathy, gold retinopathy)

What are the neurological manifestations in RA?
Peripheral neuropathy glove-and- stocking sensory loss
Mononeuritis multiplex
Entrapment neuropathy eg. Carpal tunnel syndrome
Cervical disease or atlanto-axial subluxation causing cervical myelopathy

What are the dermatological manifestations in RA?
Vasculitis (Small vessels Palpable purpura, nail fold/digital infarct; Larger vessel skin ulcer, digital
gangrene).
Palmar erythema
Rheumatoid nodules (20% patient @ olecranon process)
Raynauds phenomenon


Case 9 : Ankylosing Spondylitis :
Introduction :
Ankylosing spondylitis is one of the favourite exam cases in rheumatology. It is one of the four seronegative
arthritis. It is common in male with the ratio of 9 : 1. It is associated with HLA B27 antigen.
Examination steps :
1. Greet, seek permission, chaperone and expose the upper body of the patient.
2. Ask the patient to stand and walk from one end to the other end. Look for stoop posture and stiffness
when he walks.
3. Ask the patient lean against the wall. Measure the occiput to wall distance. Look for question mark
posture and lost of lumbar spine lordosis.
4. Ask the patient take a step forward. Do Schobers test. Examine cervical and lumbar spine mobility
including flexion, extension, lateral flexion and lateral rotation. Feel for spinal tenderness.
5. Ask the patient sit down. Measure the chest expansion by measuring tape.
6. Inspect for red eye, ausculate the lung and heart. Look for psoriatic plaque to exclude psoriatic
arthropathy. Look for Achilles tendinitis and plantar fasciitis.
7. Lie the patient down. Examine for sacroiliac spine tenderness by doing Patricks test.
Presentation :
Sir, this is a middle age gentleman who appears to be well. He does not appear to be in respiratory distress or
in pain. He has a stoop posture and appears to be stiff when he walks.
He has a question mark posture and a positive occiput-to-wall test of 4cm. He is noted to have lost of lumbar
lordosis and his Scobers test is positive.
In term of mobility, he has restricted flexion and extension of cervical and lumbar extension of approximately
0-10 degree. His lateral flexion and lateral rotation are fairly satisfactory. Otherwise, he has no spinal
tenderness. Patricks test is negative as well.
His chest expansion is restricted with 1cm expansion. Otherwise, he has no red eye to suggest anterior uveitis
or psoriatic plaque to suggest psoriatic arthropathy. Per auscultation, lung is clear and first and second heart
sound with no murmur heard. He also has no evidence of Achilles tendinitis and plantar fasciitis.
I would like to complete my examination by abdomen examination to look for hepatosplenomegaly, examine
the peripheral joints and genitalia to exclude possibility of Reiters disease. I would also like to ask the patient
for any history of chronic diarrhea or PR bleeding.
In summary, this is a middle age gentlemen with ankylosing spondylitis and functionally he has restricted
mobility of spine. There is no features to suggestive complications such as pulmonary fibrosis or aortic
regurgitation.


Differential diagnosis :
1. Psoriatic arthropathy
2. Reactive arthritis
3. Osteoarthritis of the spine
4. Enteropathic arthropathy
Investigation :
In term of investigation, I would like to do HLA B27 testing, ESR/CRP, spine x-ray and MRI spine if indicated.
HLA 27 is positive in 95% of the cases. However, HLA B27 is not diagnostic of AS. CRP or ESR level is important
in determining inflammatory activity in the patient. In the spine x-ray, I would look for sacroiliac joint
erosion/sclerosis, squaring of vertebral, ossifications of anterior longitudinal ligament, marginal
syndesmophytes and bamboo spine. MRI will be helpful if there is no changes on x-ray.
Management :
The primary goal of management for patients with AS is to maximize long term health-related quality of life.
The treatment aims are to get adequate pain relief, restoration of function, prevention and treatment of
complications.
The treatment options include non-pharmacological and pharmacological. Non-pharmacological treatment is
mainly rehabilitation to prevent joint stiffness and strengthening of the muscles. Pharmacological options
includes paracetamol and NSAIDs for pain relief, glucocorticoids to reduce inflammatory activity, DMARDS and
biological agents. Choices of DMARDS includes methotrexate and sulfasalazine whereas biological agents
includes infliximab, adalimumab, etanercepts.
What are the seronegative arthritis?
Ankylosing spondylitis, reactive arthritis, psoriatic arthritis and arthritis of inflammatory bowel disease.
What are the common features of seronegative arthritis?
They have oligoarthritis presentations, more commonly involve the lower limb and sacroiliac joints. There is
familial tendencies and RF is negative.
What are the complications of AS?
7 As of AS : Apical lung fibrosis, Aortic regurgitation, Achilles tendinitis, Anterior Uveitis, Amyloidosis,
Autoimmune bowel disease, Atlantoaxial subluxation.
How do you diagnose AS?
Most cases of AS can be diagnosed with history taking, clinical examination, simple blood tests and appropriate
X-rays. Currently, there is no universal criteria to diagnose AS. There is criterias to diagnose
spondyloarthropathy which is Assessment of SpondyloArthritis International Society (ASAS) criteria.
What are the features suggestive of inflammatory back pain?


What is the natural history of AS?
About 40% go on to develop severe spinal restriction, about 20% have significant disability, early peripheral
joint disease, particularly of the hip indicates a poor prognosis.
How do you evaluate the patient of AS?
Patient self assessment of pain (1-10)
Bath Ankylosing Spondylitis Disease Activity index (BASDAI)
Daily activities that are limited by disease
Physician's global assessment on a scale of 1 (mild) to 4 (severe)
ESR or CRP


Case 10 : Scleroderma :
Introduction :
Scleroderma encompasses a spectrum of related disorders, most of which share a characteristic clinical feature
of skin thickening due to an excess of collagen fibers.
Exam Stems :
In the exam, the common stem given is Do a general examination and proceed.
Examination Steps :
1. Start with hands. Look for pulp atrophy, Raynauds phenomenon, sclerodactyly, finger tip ulcer,
calcinosis, vasculitis. Examine the hand for joint pain, swelling and movement.
2. Look for telangiectasia. Pinch the skin up and below elbow joint.
3. Look for evidence of proximal myopathy.
4. Look at the face. Demonstrate microstomia by insertion of 3 fingers. Look for perioral furrowing, bird
beak nose, pursed lips
5. Auscultate the lung and heart.
6. Look for proximal myopathy in leg and leg ulcers.
Presentation :
Sir, this is middle-aged lady who appears to be well, not in respiratory distress or in pain. She has typical
features of scleroderma with shinny skin, perioral furrowing and microstomia.
In the hand, she has finger pulp atrophy, sclerodactyly and telangietasia. However, there is no evidence of
Raynauds phenomenon, calcinosis, finger tip ulcer and vasculitis. Skin tightness extends above the elbow.
Besides, she also has fixed flexion deformity of her fingers but no joint pain or swelling noted. Functionally, she
has restricted movement of her hands.
She has no evidence of proximal myopathy with full muscle power of both upper and lower limb proximal
muscle. There is no leg ulcers seen as well.
Besides, she has evidence of pulmonary fibrosis with crepitations heard over the lung and evidence of
pulmonary hypertension with a loud P2 heard.
I would like to complete my examination by abdominal examination, check the blood pressure and check the
stool and urine to look for proteinuria and steatorrhea.
In summary, this is a middle-aged lady who has diffuse systemic sclerosis and functionally has restricted hand
movements. She also has evidences to suggest pulmonary fibrosis and pulmonary hypertension.


Investigations :
To confirm my diagnosis, I would like to do blood tests such as anti-topoisomerase and anti-centromere
antibodies. I would expect anti-topoisomerase antibody to be positive in diffuse systemic sclerosis and anti-
centromere antibodies to be positive in limited systemic sclerosis. Other investigations are ANA, urinalysis,
renal profile, ECG, chest x-ray, CT thorax to look for other complications of the disease.
Management :
The principle of treatment of scleroderma is mainly conservative and identify and treat possible complications.
The treatment is mainly organ-based.
- Raynauds phenomenon : avoid cold, stress and B-blocker. Calcium channel blocker or sildenafil can be
given for vasodilatory effects. Bosentan and ilioprost are also effective in treating Raynauds.
- Renal hypertensive crisis : avoid steroid. ACE inhibitor is effective in treating this.
- GERD : PPI.
- Malabsorption syndrome : oral antibiotics.
- Pulmonary hypertension : bosentan, ilioprost. Lung transplant is another options.
- Arthralgia : NSAIDS, paracetamol, low dose steroids
What are the main types of scleroderma?
Diffuse and limited systemic sclerosis.
What is the diagnostic criteria for scleroderma?
- Major criteria : proximal scleroderma (to MCP and MTP joint)
- Minor criteria : sclerodactyly, fingertip pitting or atropy, bibasal pulmonary fibrosis.
At least the major or 2 minor criteria must be fulfilled to diagnose scleroderma.
What are the antibody that will be positive in scleroderma?
Anti-topoisomerase for diffuse systemic sclerosis and anti-centromere for limited systemic sclerosis.
What are the possible causes of Raynauds phenomenon?
- Cold
- Connective tissue diseases such as SLE, RA, Scleroderma
- B-blocker
- Polycythemia vera rubra
- Thoracic outlet syndrome
- Buergers disease
- Occupational vibrating tools
- Cryoglobulinemia


What are the systemic complications of scleroderma?
System Complications
Heart Myocardial fibrosis, pericarditis, myocarditis, arthymia, conduction block,
pericardial effusion
Lung Lung fibrosis, pulmonary hypertension
GIT Dysphagia, GERD, constipation, diarrhea, watermelon stomach, small bowel
bacteria overgrowth
Renal Renal hypertensive crisis
Neurological Peripheral neuropathy, myopathy, myositis, erectile dysfunction
Joint Arthralgia
Skin Telangiectasia, vasculitis, pulp atrophy
Endocrine Hypothyroidism

What is mixed connective tissue disease?
Scleroderma + SLE + Myositis
Causes of anemia in Scleroderma :
IDA, anemia of chronic disease, megaloblastic anemia (bacterial overgrowth)
How would you manage renal hypertensive crisis?
ACE inhibitor is the 1
st
line of treatment. If there is contraindication to ACE inhibitor, ARB can be tried even
though the effectiveness is still not well known. Steroids must be avoided as it will worsen the hypertensive
crisis. Creatinine level should be monitored to look for renal failure.


Case 11 : Parkinsons disease
Introduction :
Parkinsonism is a syndrome characterized by termor, rigidity and bradykinesia. Causes of Parkinsonism include
idiopathic Parkinsons disease and Parkinsons Plus syndrome.
Exam Stems :
Do a general examination and proceed.
Examination Steps :
1. Look for expressionless face, pill-rolling tremor.
2. Elicit cogwheel rigidity and bradykinesia.
3. Ask the patient to write. At the same time, look for scar in the head or chest.
4. Do a glabellar tap and assess the speech.
5. Assess the eye movement. Look for vertical gaze palsy.
6. Ask the patient to walk and examine the gait.
7. Do a retropulsion test if time permits.
Presentation :
Sir, my patient is a elderly gentlemen who appears to be well, not in respiratory distress or in pain. He has an
expressionless face and pil-rolling tremor at rest.
In the hands, I could appreciate cogwheel rigidity on the left arm which present throughout the whole range of
movement and exaggerated by distraction. He also has evidence of bradykinesia and micrographia.
He has a positive glabellar tap but the speech appears to be fairly good. He has no evidence to suggest
progressive supranuclear palsy as there is no vertical gaze palsy.
He walks with a fenestating gait and his arm swings are very much reduced. He also has difficulty in turning.
Retropulsion test is negative.
I would like to complete my examination by checking the supine and standing blood pressure. I would also like
to ask for history of neurological disorder in the family and any history of neuroleptics consumption.
In summary, this is a elderly gentlemen who has Parkinsonism most likely idiopathy Parkinsons disease and
functionally he is disabled by bradykinesia.
How would you diagnose Parkinsons disease?
Parkinson disease is a clinical diagnosis. There is no investigations that can confirm Parkinsons disease.


What investigation would you like to do?
I would like to do a MRI of the brain to exclude possible Parkinsons plus syndrome. Otherwise, there is no
single test that can confirm the diagnosis of idiopathic Parkinson disease.
How would you manage the patient?
The principle of treatment is mainly pharmacological treatment. The group of drugs that can be employed are :
- Dopamine : madopar
- Dopamine agonist : bromocriptine, carbegoline, pramipexole, ropinirole
- Anticholinergics : benzhexol
- MAO inhibitor : Selegiline
- COMT inhibitor : Entacapone
If oral drugs fail to improve, I would like to consider subcutaneous apomorphine infusion. If this fails, I would
consider surgical intervention.
What are the Parkinsons Plus syndrome?
Progressive supranuclear palsy, multisystem atrophy, cortico-basalganglionic degeneration, diffuse lewy body
disease
What are the surgery that can be done for Parkinson?
Deep brain stimulation, pallidotomy, thalamotomy
What is the staging system used for Parkinson?
Hoehn and Yahr Classification :
Stage Features
I Unilateral involvement only, minimal or no functional impairment
II Bilateral involvement, without impairment of balance
III First sign of impaired righting reflexes. Functionally restricted but physically capable, mild-
moderate disability
IV Fully developed, severely disabling disease, still able to walk and stand unassisted but markedly
incapacitated
V Confined to bed or wheelchair unless aided

What are the non-motor complications of Parkinsons disease?
Depression, restless leg syndrome, constipation, sexual dysfunction
What is progressive supranuclear palsy?
akinesia, dementia, vertical gaze palsy, axial rigidity
What is the first sign of Parkinson disease?
Reduced arm swing


Case 12 : Stroke
Introduction:
Clinical syndrome characterized by acute onset of focal neurological deficit lasting >24 hours due to
cerebrovascular accident (embolic, thrombotic or hypoperfussion).
Presentation:
Sir, I would like to complete my neurological examination by checking the upper limb, spine, anal tone, cranial
nerve and higher cortical function. I also want examine the cardiovascular system.
This is a middle aged gentlemen with average built and appears to be well. He has a hemiplegic gait with fisting
and pronation of right arm. Neurological examination show that there is upper neuron sign over the right
lower limb with muscle wasting with no contracture, hypertonia, brisk deep tendon reflex, positive Babinski
sign but no clonus. Muscle power is 3/5. The proprioception and coordination test is normal.
In summary, this gentleman has right sided hemiplegia most probably due to left corticospinal lesion.
Differential diagnosis:
1. Metabolic or toxic encephalopathy eg: hypoglycaemia, non ketotic hyperglycaemia
2. Post ictal todd paralysis
3. Structural intracranial lesion: tumor, hematoma, AVM, abcess
Investigation:
First, I would like to take CT brain to exclude or diagnose hemorrhagic stroke or intracranial lesion. Then I
would like to check full blood count to rule out coagulopathy, renal function profile, blood sugar profile to
exclude hypoglycaemia, fasting lipid profile, electrocardiogram to look for arrhythmia, chest x-ray,
echocardiogram to rule out cardiac emboli, carotid Doppler and MRI angiography. Thrombophila and
connective tissue screen in young stroke.
Management:
Early detection, stabilize patient and admit to stroke unit with vital sign monitoring. Maintenance of
electrolyte and glucose level, prevent hyperthermia, give oxygen supplement, wide bore needle insertion with
intraveneous fluid and nil by mouth. Acute ischemic stroke patient who present within 4.5 hours after onset
can be thrombolysed with intravenous recombinant tissue plasminogen activator. Give aspirin within 48 hours
of ischemic stroke shown to reduce risk of death and recurrent stroke. Refer to physiotherapy and
occupational therapy to prevent contracture, muscle wasting and improve outcome. Turn patients position
every 2 hourly to prevent pressure sore. Deep vein thrombosis prophylaxis. Control risk factor: stop smoking,
control blood pressure and diabetes, treat hyperlipidaemia. Give antihypertension drug eg labetolol or
nicardipine if systolic >220mmHg and diastolic >120mmHg, otherwise just monitor the blood pressure. Reduce
blood pressure slowly with target of about 160-180 mmHg systolic and 100-110mmHg diastolic.


How to classify stroke?
Bamford clinical classification of stroke:

Common site of hypertensive hemorrhagic stroke :
Rupture of charcot-bouchard aneurysm. Common site: basal ganglia (putamen, caudate nucleus), cerebellum,
internal capsule, external capsule, hypothalamus
Contraindication for thrombolysis :
Stroke or head trauma or myocardial infarction in the past 3 month.

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