Test 20  (systemic diseases and ophthalmology) 

 

Refer to this picture for question 1.

1. The following can precipitate or result from the condition shown in 
     the picture above:

a. 

b. 

c. 

d. 

e.

Answera.T   b.T  c.T  d.F e.T
The referring picture shows Kayser-Fleischer's 
ring seen in Wilson's disease and is the result of 
abnormal copper metabolism leading to copper toxicity. The condition is autosomal recessive.
a. shows intrauterine coils used for contraception.
The coils contain copper. Women with Wilson's 
disease who use the coils can exacerbate the condition.
b shows hepatic cirrhosis in a patient with Wilson's 
disease. Copper deposition in the liver causes hepatic damage leading to cirrhosis and failure.
c. shows degeneration of the basal ganglia which is a feature in this condition.
d. shows pigmentary retinopathy which is not a feature of Wilson's disease.
e. is a patient with facies and upper limbs typical of 
untreated Wilson's disease. The patient was one of
the original patients of Dr. Samuel A. K. Wilson and the picture first appeared in an article published in 1912 issue of Brain. In Wilson's disease, the neurological signs can include tremor, chorea, rigidity and spasticity

 
 
 

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Refer to these picture for question 2.  The figures show the same patient  before and after an intravenous injection.

2. The following can be associated with the pictures above:

a. 

b. 

c. 

d. 

e. 

Answera.T   b.T   c.T   d.T  e.T
The pictures show a patient with positive Tensilon's test.
a. shows X-ray of a hand with rheumatoid arthritis. Patient who are treated with penicillamine for rheumatoid arthritis can develop myasthenia gravis.
b. is EMG showing decrement of response to repetitive nervous stimulation.
c. is Calabar bean from which the anti-cholinesterase 
physostigmine is extracted. This is used to treat myasthenia gravis.
 d. is a thymoma which can occur in myasthenia gravis.
e. is Aristotle Onasiss perhaps the most well known celebrity to suffer from myasthenia gravis.

 
 

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Refer to this picture  for question 3.

a

3. The following are associated with the sign shown above:

a. 

b. 

c. 

d. 

e. 

Answera.T   b.T  c.T  d.T  e.F
The referring picture shows the presence of a pigmented lesion in the lateral aspect of the right eye. The 
pigmentation is situated in the sclera close to the insertion of the lateral rectus. This is a typical sign for ochronosis.
Ochronosis or alkaptonuria is a genetic disease caused by the absence of an enzyme homogentisic acid oxidase
(HGAO). This causes the accumulation of homogentisic acid; some of the acid is eliminated in the urine and the rest is deposited in the body tissue. The two main effects of such depositions are:
i. blue-black discoloration of the sclera, cartilage and bone
ii. arthropathy.
a. shows a family tree suggestive of an autosomal recessive condition. Ochronosis like most enzyme defects is an autosomal recessive condition.
b. shows degeneration of the spine and sacral iliac joints.
c. shows blue-black discoloration of the pinna.
d. shows discoloration of the urine on standing. The urine turns black when left to stand in the air or if alkali such as sodium hydroxide is added. This changes is caused by oxidation of the homogentisic acid.
e. shows dark patches over the left and to a smaller extent the right upper face. Although ochronosis can affect the skin, the pigmentation tends to be symmetrical. This patient has naevus of Ota.

e

d

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Refer to this picture for question 4.

4. Systemic association include:

a. 

b. 

c. 

d. 

e. 

Answera.T  b.T  c.T  d.T  e.T
The referring picture shows angioid streaks.
a. shows the blood film of a patient with sickle cell disease. Note the sickle cells in the film. Angioid streaks occur in sickle cell disease.
b. shows dissecting aneurysm of the aorta. Patient with 
pseudoxanthoma elasticum is at risk of aortic aneurysm.
c. is a gross specimen of the tibia and fibula showing 
abnormal expansion of the whole tibia. The specimen is taken from a patient with Paget's disease.
d. shows joint hyperextensibility in Ehler-Danlos's syndrome.
e. shows multiple punch out lesions in the skull of multiple myeloma.

 
 

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Refer to the picture above for question 5.

5. The child may have the following additional signs:

a. 

b. 

c. 

d. 

e. 

Answera.T   b.T  c.T   d.F  e.T
The referring picture shows interstitial keratitis in congenital syphilis.
a. is the dark field microscopy showing the spirochetes which cause syphilis.
b. shows perforation of the hard palate and c. shows 
Hutchinson's teeth. Both are features of congenital syphilis.
d. shows periostitis of the third metacarpal bone. 
Periostitis is a feature of congenital syphilis.
e. shows disorganized ankle joint ie. Charcot's joint. This occurs in acquired tertiary syphilis.

 
 

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