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EMQs in Clinical Medicine.pdf - Peshawar Medical College

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98 Neurology<br />

A test dose of an antichol<strong>in</strong>esterase, e.g. edrophonium, improves muscle<br />

power and can also be used to aid diagnosis.<br />

Myasthenia gravis can be treated with the use of antichol<strong>in</strong>esterases (oral<br />

pyridostigm<strong>in</strong>e is a popular drug) and oral corticosteroids (for immunosuppression).<br />

Be aware of Eaton–Lambert syndrome (a manifestation of bronchial<br />

small cell carc<strong>in</strong>oma) where there is defective release of acetylchol<strong>in</strong>e at<br />

neuromuscular junctions giv<strong>in</strong>g rise to a myasthenic syndrome. In myasthenic<br />

syndrome the muscle strength <strong>in</strong>creases and reflexes return with<br />

repeated contraction, unlike MG where repeated contraction gives rise to<br />

more weakness and a reduction of reflexes.<br />

D<br />

A 15-year-old boy is found to have several coffee-coloured patches on<br />

his body. Slit-lamp exam<strong>in</strong>ation reveals the presence of Lisch nodules.<br />

This is a presentation of type I neurofibromatosis (also known as von<br />

Reckl<strong>in</strong>ghausen’s disease). It is an autosomal dom<strong>in</strong>ant condition characterized<br />

by decreased production of neurofibrom<strong>in</strong>.<br />

Diagnosis requires two of seven diagnostic criteria.<br />

(i) six or more café-au-lait spots 5 mm (<strong>in</strong> children 10) or 15 mm<br />

<strong>in</strong> adults<br />

(ii) axillary/<strong>in</strong>gu<strong>in</strong>al freckl<strong>in</strong>g<br />

(iii) two or more neurofibromas or even one plexiform neurofibroma<br />

(iv) optic glioma<br />

(v) two or more Lisch nodules (iris hamartoma detected with slit-lamp<br />

exam<strong>in</strong>ation)<br />

(vi) osseous lesion such as sphenoid dysplasia/pseudoarthrosis<br />

(vii) family history <strong>in</strong> first-degree relative.<br />

36 Gait<br />

Answers: F D L G B<br />

F<br />

D<br />

A 65-year-old man with a fest<strong>in</strong>ant, shuffl<strong>in</strong>g gait.<br />

There is a characteristic fixed stoop with reduced arm sw<strong>in</strong>g while walk<strong>in</strong>g.<br />

As a result of the impairment of right<strong>in</strong>g reflexes, sufferers are<br />

sometimes observed to fall stiffly ‘like a tree’ without putt<strong>in</strong>g their arms<br />

out to break the fall.<br />

A 60-year-old woman walks with a drop foot and high-stepp<strong>in</strong>g gait<br />

shortly after hip replacement.<br />

Sciatic nerve is derived from the anterior primary rami of L4, L5, S1, S2<br />

and S3, and divides <strong>in</strong>to the common peroneal and tibial nerves about<br />

two-thirds of the way down the thigh.<br />

Sciatic nerve <strong>in</strong>jury is an important postoperative complication of hip<br />

replacement and can result from direct trauma or traction on the nerve.

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