Neurology 4 mrcp answers book - part 5 pptx

Q6: Answer: 4 1-True, be careful as there may be a co-existent iron deficiency anemia causing a falsely low or normal HbA2.. 4- False, may be due to severe longstanding anemia causing hi

Q6:

Answer: 4

1-True, be careful as there may be a co-existent iron deficiency anemia causing a falsely low or normal HbA2

2- Together with iron chelation therapy and daily folic acids

3- The usually story, and then confirmed by hemoglobin electrophoresis

4- False, may be due to severe longstanding anemia causing high out put cardiac failure

5-True, and profound dyserythropoiesis

Q7:

Answer: 5

Hemolytic disease of the new born causes mainly extra vascular hemolysis

Q8:

Answer: 4

1- Any neurological syndromes + fever + skin rash =exclude TTP

2- True for unknown reason

3- Purpuric rash or frank ecchymosis

4- False, impaired coagulation systems (PT and aPPT) indicate DIC, these are normal

in pure HUS or TTP

5- To prematurely release platelets from the bone marrow

Q9:

Answer: 3

1- and eczema and other skin diseases

2- and filiariasis, ascariasis

3- False, treatment with steroids causes neutrophilia and eosinopenia

4- usually forgotten

5- PAN and esosinophilic granuloma

Q10:

Answer: 5

1- and bone marrow recovery from radiotherapy

2- and inflammatory bowel disease, SLE

3- and myeolomonocytic leukemia

4- and TB, typhus, brucellosis, SBE, malaria, trypanosomiasis

5- false, causes monocytopenia

Q11:

Answer: 5

1- as in Arabs and blacks

2- like hepatitis A

3- ususally associated with low platelets

4- and other drugs like penicillamine

5- false, causes neutrophilia which is a useful clue to differentiate it from viral

hepatitis

Q12:

Answer: 4

The common ALL type has a good prognosis The T cell type presents with a

mediastinal mass and a very high leukocyte count and has a gloomy prognosis

Also t(9, 22) cytogenetic abnormality has a very poor prognosis (unlike in CML where its presence confers a good prognosis)

Q13:

Answer: 2

Knowing the cytogentics of AML is very important, not only for the prognosis but also to decide the future treatment like bone marrow transplantation in those with poor cytogenetics

M2-t(8,21)

M3-t(15,17)

M4-inv16

Also in ALL: t(1,19) in pre-B ALL, t(8,14) ALL L3 Burkitt’s subtype

Q14:

Answer 4

1- True

2- Retenoic Acid Receptor Alpha

3- True, hence a rapid development of neurtrophilia is seen

4- False, hypergranular

5- True and usually asymptomatic to start with

Q15:

Answer: 4

1- True, but remember; Ph chromosome is found in 5% of childhood ALL, 25% of adult ALL and 1% of adult AML

2- True, due to its inhibitory effect on the BCL-ABL fusion gene product

3- True, a useful clue

4- False, serum vitamin B12 is high due to high vitamin B12 binding protein

5- True, other causes of low LAP score: PNH and some cases of aplastic anemia and MDS

Q16:

Answer: 5

1- and 13q abnormalities, and both portend poor prognosis

2- and warm immune hemolytic anemia and ITP like picture may be seen

3- true

4- as in many lymphoproliferative disorders

5-FALSE, the doubling time is usually long and the cell turnover low

Q17:

Answer: 5

1- as in many hematological malignancies

2- and ESR is high and indicates an active disease

3- and hence it is difficult to know weather these represents lymphomatous masses or not; PET scan may be used to differentiate between them

4- and lymphocytic predominant has good prognosis

5- Fasle, with EBV infection

Q18:

Answer: 5

1- true, myeloma high ESR is responsible only for 3% of cases of ESR>100

2- keep it in mind ,on the other hand there may be no serum paraprotein, but only urinary Bence John’s protein

3- true, infected stem cells may be responsible for the secretion of high level of IL-6 4- true, as there is an overall inhibitory effect on bone osteoblasts

5- false, only in the presence of a fracture

Q19:

Answer: 5

Immune paresis is in favor of MM, as well as bone and renal diseases

Q20:

Answer: 5

1- and in neonates

2- and smoking, COPD and morbid obesity

3- and other high affinity hemoglobins

4- and renal cell carcinoma

5- false, a primary one

Q21:

Answer: 5

Other causes, systemic inflammatory disorders and vasculitis, trauma, infections PRV may have a high platelet count but it is not a reactive process because it is part of the panmyelosis process seen in PRV

Q22:

Answer: 5

1- true, mainly it is a disease of elderlies

2- like hypo granular neutrophils , abnormal neutrophil nuclear lobulation, vacuolated erythroblasts

3- other causes of death are from infection and bleeding

4- and secondary MDS is seen after chemotherapy and radiotherapy

5- false, refractory anemia with excess blasts in transformation

Q23:

Answer: 5

1- indicates the presence of inhibitors in the patient's plasma(unlike hemophilia) 2- bilateral adrenal vein thrombosis

3- may be confused with SLE per se

4- and epilepsy, TIAs, stroke and multi-infarct dementia

5- false, arterial and venous thrombosis, if hemorrage occurs it is either due to over anticoagulation or due to severe thrombocytopenia

Remember: the aPTT is already prolonged making heparin therapy follow up is difficult

Q24:

Answer: 5

1- many cases are mild and discovered later in life

2- becauses it induces further thrombocytopenia, other types have normal platelets count

3- true, the only useful indication of bleeding time application

4- but an abnormal vWf multimers

5- some cases are autosomal recessive and usually the dominant ones have a variable expression

Q25:

Answer: 5

1- hence no family history is present

2- characteristic, and the platelets may be elevated after a bleeding episode like a GIT bleeding or due to recurrent bleedings causing iron deficiency anemia and

thrombosytosis

3- in severe cases should be always the first line IF AVAILABLE

4- if does not correct this indicates the development of inhibitors

5- its activity is between 50-150%

Q26:

Answer: 5

1- and also SLE

2- in contrast to children

3- and 2/3rd responds to splenomegally after failure of steroids

4- its role mainly is to exclude other diseases like ALL in children, and antiplatelets antibodies are not used routinely in the diagnosis

5- false, such a history is usually obtained in children not adults

Q27:

Answer: 5

Treatment with steroids (together with melphalan) helps to stabilize the disease and may produce improvement

Q28:

Answer: 3

HIGH serum Beta 2 microglobulin level is a bad prognostic sign

Q29:

Answer: 5

1- for many reasons, dehydration is common

2- about 55%, IgA 21%, light chain only 22% and others including non secretory about 2%

3- true, hypogammaglobulinemia is common and severe Bone marrow failure is an advanced stage in the course of the disease

4- true, and may indicate the need for plasmapharesis

5- indicates a recent fracture MM per se does not raise serum alkaline phosphatase due to inhibitory cytokines on osteoblastic function and hence radio isotope scan is also normal

Q30:

Answer: 5

1- hence symptoms of infection are common

2- true, relatively has a slow pace

3- can be seen when performing flow cytometry in the diagnosis

4- and to deoxycofermycin

5- false, splenomegally is seen in up to 90% of cases and may be massive, but lymph node enlargement is highly unusual (unlike CLL)

Q31:

Answer: 2

1- many options are there but the treatment is mainly supportive

2- false, chromosomes 5 and 7

3- usually in elderly population, but in theory no age is exempted

4- like following alkylating agents or etoposide

5- with dysplastic, NOT megaloblastic changes

Q32:

Answer: 3

1- true, and all other cases are secondary, like infections, tumors, drugs

2- smudge cells are seen in CLL; the presence of spherocytes per se may be a clue to AIHA but not its cause

3- direct Coomb’s test detects IgG and complement but not IgA or IgE antibodies hence may be falsely NEGATIVE when AIHA is associated with these antibodies 4- true hence those with low tires may be falsely NEGATIVE

5- yes, also seen in ITP

Q33:

Answer: 4

1- these are usually mild More severe cases either indicate a coexistent defect of second different protein or coincidental polymorphism of ALPHA spectrin

2- and indicates the need for spelnectomy

3- as well as growth retardation in children and death of a family member from the disease

4- false, 25% only

5- a must BUT may become positive after many transfusions i.e alloimmunization!!

Q34:

Answer: 5

1- hence during the acute hemolytic episode don’t assess the enzyme level as it may become transiently normal or even high

2- a useful clue in acute attacks

3- so you have to give an enough HISTORY to the hematology lab so as to use this stain

4- especially after receiving the water soluble analogue of vitamin K

5- false, the Caucasian and the Oriental types are the most severe when compared with the African type

Q35:

Answer: 4

1- true, it just provides a clue to them like hypersegmented neutrophils

2- true, but (written in some textbooks) a peripheral neuropathy state may be seen in folate deficiency also

3- true, anorexia ,alcohol and phenytoin can severely depress the serum level in the absence of deficiency state; also a single meal may totally NORMALISE it in a severely deficient patient So Red cell folate is better than serum folate level

4- false, may precipitate severe hypokalemia

5- and reticulocytes percentage may reach 50% with in the first week of treatment

Remember: Pregnancy is the commonest cause of megaloblastosis world-wide and more likely to be seen in the context of multiple pregnancy, and multiparity

Q36:

Answer: 5

Hemosidrinuria, hemoglobinuria, and reduced serum haptoglobin all are seen

Q37:

Answer: 4

1- and levels down to 100 microgram/ L are still suggestive of IDA in chronic

inflammatory illnesses

2- serum iron fluctuates by 30-50% on daily basis and even diurnal basis It is low in acute phase responses

3- and in nephrotic syndrome and malnutrition It is raised in pregnancy and oral CCP

4- false, less than 16 %

5- measured by immunoassay

Q38:

Answer: 5

They (bronchospasm, angiodema and hypotension) predominate the clinical picture

Q39:

Answer: 5

Ensure urine out put of at least 100 ml / minute

Q40:

Answer: 1

Item 1 is wrong because he has a clear cut precipitating factor, besides this is the first episode, so no need to screen

Chapter X / Rheumatology Answers

Q1:

Answer: 3

1-true, is an autoantibody

2-but, we detect only IgM in the slide agglutination test

3-false, does not reflect any thing apart from poor prognosis if found in high titer in

RA

4- like slide tube agglutination, SCAT and Rose Waaler tests

5-true, must be seropositive here

Q2:

Answer: 5

1- as it rises rapidly and falls rapidly and hence reflects the degree of the APRs

2- true, as it rapidly rises from a low to high levels in acute inflammatory process and closely mirrors the degree of inflammatory

3- true, in cases of improvement in the APR, the CRP normalizes rapidly while the ESR LAGS behind for a variable period of time

4- and in polycythemia rubra vera (PRV), so in these cases we measure the plasma viscosity as a measure of APR (remember in PRV there is florid APR; yet the ESR is very low)

5- the CRP is elevated in the presence of infection of trauma in SLE, other wise in acute flare ups it is normal;a useful CLUE!

Q3:

Answer: 4

1-hypochromic microcytic one is seen up to 25 % but does not respond to iron

therapy It is not an iron deficiency states, but there is defects in ferro-kinetics and iron utilization; unfortunately may be complicated by true iron deficiency anemia eg long term treatment with NSAIDS

2- true, useful guide to the overall activity eg in RA

3-true, varieties of pictures, and may be complicated by drug side effects on the blood count and bone marrow ,so keep it in mind

4-leukocytosis and neutrophilia eg in classical PAN

5-true, a common cause of neutrophila

Q4:

Answer: 4

1- eg using rodent organs or human cell lines

2- like rheumatoid factor, low tires may be seen in healthy normal people in a good percentage

3-hence its name

4- FALSE, 100% of cases; a negative titer virtually excludes drug induced lupus (here

it is mainly anti histone H2 A and B subtypes)

5- depends on the antigen preparation used in the test and whether we detect IgG or IgM type; however the tests are unfortunately NOT standardized and liaison with local labs is important

Q5:

Answer: 5

1- with a fall from standing height or less.

2- also radiological evidence of osteoporosis, and premature gonadal failure

3-remmeber, not only the disease may pose a risk but also its treatment like

corticosteroids

4-also, clinical features of osteoporosis, like loss of height and kyphosis

5-false ,BMI less than 19; hence obese persons are usually protected

Remember: polycystic ovarian syndrome patients although may be infertile with irregular or absent cycles, but the high BMI and the good amount of estrogens and androgens protect them from osteoporosis

Q6:

Answer: 5

1- as the isotop is taken by body bones

2- also in primary and secondary bone tumors

3- as the Tc-biphosphanate later localizes to areas of bone remodeling; theses LATE images are usually taken after few hours

4-true, the main indication in general

5-false, it depends on the osteoblatstic activity which is inhibited in multiple

myeloma; so it is not useful here, but only if it was complicated by fractures

Remember: although bone scan has a high sensitivity, it extremely lacks specificity and lacks high anatomical resolution

Q7:

Answer: 5

Other indications: reflex sympathetic dystrophy and hypertrophic osteoarthropathy; and useful in the assessment of the extent of Paget’s disease of the bone In multiple myeloma (MM), it depends on the osteoblatstic activity which is inhibited in MM, so

it is not useful here, only if it was complicated by fractures

Q8:

Answer: 1

1- false, less than 20 and more than 50 years of age

2- and a history of a major trauma

3- and the presence of a painful spinal deformity, severe symmetrical spinal

deformity, saddle anesthesia, progressive neurological signs in the lower limbs, sphincter dysfunction, and a sensory level

4- very important indicators

5- and HIV, malignancy, on long term steroids etc

Q9:

Answer: 5

90% of cases improve after 6 weeks so it has an excellent prognosis Other features: Tendency for recurrent episodes, pain is limited in the back or thigh but NEVER below the knee, and no clear cut root signs

Q10:

Answer: 5

In general, the prognosis is reasonable with 50% recovery at 6 weeks

Q11:

Answer: 4

1- so it is common

2- true associations, also hyperinsulinemia

3- true, at least 4

4- FALSE, there is NO such features which are (with posterior apophyseal joint involvement) indicate spinal spondylosis

5-true, very rarely have symptoms, usually low back pain

Q12:

Answer: 5

1- and from capsular fibrosis and intervening large osteophytes; There is palpable, sometimes, audible coarse crepitus

2- so called "good days and bad days"

3- also may increase upon joint movement

4- usually one or few painful joints (rarely multiple painful areas)

5- false, NO such joint instabilities

Q13:

Answer: 5

1- ogether with trauma; usually are monoarticular

2- like JIA

3- and the so called endemic osteoarthritis

4- and hemachromatosis

5- FALSE, arthropathy is seen up to 50% of cases of acromegally (not in GH

deficiency)

Q14:

Answer: 5

1- true, you should treat appropriately to minimize disability

2- true, they are “safe and cheap”

3- true, with excellent results

4- true, with no significant drug-drug interaction and are often effective at relieving pain

5- false, a common age associated phenomenon, and may precipitate acute

pseudogout attacks

Q15:

Answer: 4

In Caucasians, the prevalence is about 1-1.5 % with female to male ratio of 3:1

Q16:

Answer: 1

HLA DR4 is the major susceptibility haplotype in most ethnic group eg found in 75%

of Caucasian patients with RA, and HLA DR4 positivity is more common in those with severe disease HLA DR1 is more important in Indians and Israelis while HLA DW15 is more important in Japanese

Q17:

Answer: 2

1- true, with infiltrations by lymphocytes, plasma cells and macrophages

2- CD4 positive cells

3- similar granulomatous lesions are seen in the sclera and pericardium, pleura and lungs

4- fibrous or bony ankylosis occurs late

5- the regional lymph nodes draining the actively inflamed joints are frequently hyperplasic

Q18:

Answer: 5

1- and when rupture may simulate DVT, but remember both may coexist together as the patient may be immobile and there is an increased risk of DVT, so be careful!! 2- usually seen in extensor body surfaces, sclera and lung

3- Episcleritis is a benign complication but sclertits is serious

4- seen in up to 30% of nodular seropositive patients, but constrictive pericarditis is rare

5- the CNS is surprisingly SPARED in RA vasculitis

Q19:

Answer: 1

1- false, less than 1 % only

2- and nodular seropositive disease

3- and weight loss, recurrent infections, and sicca syndrome

4- and normochromic anemia, defective T and B cell function and neutropenia 5- usually between 50-70 years of age

Q20:

Answer: 4

1- mouth ulceration and Pemphigus are both rare

2- drug induced lupus and Goodpasture's syndrome are also rare

3- rapidly falling platelet count, mild thrombocytopenia, and proteinuria are

indications to stop the drug and reintroduce it slowly, but if they recur then stop it for good

4- febrile reactions and pancytopenia are absolute indication to stop the drug

IMMEDIATELY and for GOOD

5-and glomerulonephritis

Q21:

Answer: 3

Marrow suppression and aplastic anemia may occur and both carry a significant mortality

Q22:

Answer: 2

1- an association with HLA B27

2- peripheral asymmetrical oligoarthritis affecting the lower limbs more than the upper