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Case ID: 1
Created: 13 July 2006

This gentleman has difficulty in walking. Examine his lower limbs neurologically.

MRCP_becker

Discussion:

       An uncommon case in your daily practice but it is suprisingly a popular question in MRCP. Many candidates were asked to examine this case in their PACES before. I have friends who sat thier MRCP in Malaysia ( especially at University Malaya Medical Centre), Singapore ( at National University Hospital) and Hong Kong were asked about this case in their Neurology Station. Obviously if you observe properly, you notice there is hypertrophy of both calf muscles. ( Yes, you are dealing with PSEUDOHYPERTROPHY of calf muscle), further examination reviews that,

arrowThis gentleman has waddling gait.
arrowHis proximal muscles are weaker that his distal muscles.
arrowHis ankle and knee reflexes may be normal and there is no sensory involvement.

After your complete neurological examination, do the following steps,

arrowDemonstrate Gower's sign..
arrowSuggest to examiners that you would examine the upper limbs and look hard of upper limbs involvement as well. Look for pseudohypertrophy of deltoid muscle also.
arrowDemonstrate winging of scapula by asking patient to straighten his elbow and push against resistance.
arrowSuggest to examiners you would ask about family history of similar problem.
arrowLook for any surgical scar to suggest recent muscle biopsy.

Common questions examiners would ask you,

1) Why do you say that it is pseudohypertrophy and not true hypertrophy of calf muscle in this condition?
2) How do you differentiate Duchenne from Becker muscular dystrophy?
3) What is the inheritance pattern in this condition?
4) How do you investigate and manage this condition? ( Remember GENETIC COUNSELLING!)

Conclusion:

This gentleman has proximal myopathy due to Becker Muscular Dystrophy.

Extra points:

1) There are a lot of causes for proximal myopathy, however if you notice pseudohypertrophy of calf or deltoid muscles, it is usually due to Hereditary Muscular Dystrophy.
2) Other common cases of proximal myopathy in MRCP PACES are either polymyositis or dermatomyositis.

Case ID: 2
Created: 13 July 2006

Examine this gentleman lower limbs neurologically.

MRCP_cavus

Discussion:   

  Another popular neurology case in MRCP PACES, there are a few possibilities you are dealing with Pes Cavus in MRCP. You may be asked to talk to a patient who has a Cerebellar ( staccato and scanning speech) speech and find out that he/she has pes cavus with Friedreich's ataxia. Or there is another scenario where you are asked to examine a patient's upper limbs and you find that he/she has small muscles wasting of both hands and 'inverted champagne bottles' lower limbs with pes cavus. Yes , you are dealing with Charcot-Marie-Tooth Disease. The two commonest causes for Pes Cavus in MRCP are Friedreich's ataxia and Charcot-Marie-Tooth Disease. Your further steps of examination depend on your finding, if you suspect Friedreich's ataxia after your lower limbs examination, suggest to examiners that you would like to,
arrowask about family history,
arrowcheck fundoscopy to look for optic atrophy,.
arrowexamine relevant cerebellar signs,.
arrowlook for kyphoscoliosis,

Common questions examiners would ask you,

1) What is the mode of inheritance for Friedreich's ataxia or Charcot Marie Tooth?

2) What is the pathogenesis of pes cavus? (To learn more, click here!)

Conclusion:

This gentleman has pes cavus due to cerebral palsy.

Extra points,

1) In MRCP PACES, if you are asked to examine lower limbs, always look at the upper limbs as well and vice versa.
2) Remember that other causes of Pes Cavus are poliomyelitis and muscular dystrophy..

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