Question of the Week

Question :
Posted On : 12 Dec 2006
3.5 yrs girl born of second degree consaguinity with 2 normal older sibs and mother had jaundice in 1st trimester of pregnancy presents with mild developmental delay. She had grade I hepatic encephalopathy with Hepatitis A 6 months back. 3 episodes of para,quadriparesis of mod-severe degree and loss,grossly altered speech lasting for 5-10days over last 15 mths. She once had fever precipitated focal seizures. Workup{KEM-PUNE}-generalised Spikes and wave on EEG. CT brain - normal, MRI-enhancement of right temporal , parietal lobes and choroid plexus with normal myelination. Blood and csf lactate are raised. Liver function tests are normal. Is it MELAS,Leighs?
5
Expert Answer :
No expert answer available.
Answer Discussion :
M
mohannadwaleed
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melas
18 years ago
D
Dilip Deshpande
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MELAS
18 years ago
D
DR DUSHYANT KOTHARI
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Leighs
18 years ago
P
pediatriconcall
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Since CSF lactate is raised and there have been 3 episodes of encephalopahty, one must work up for inborn error. A urine organic acids and urine for dicarboxylic acid should be done. Do a serum ammonia. Also and EMG to look for myopathy. A muscle biopsy may be indicated if strongly suspecting MERRF or MELAS. Specific enzyme analysis will depend on the further workup .
18 years ago
S
saeedelsadek
Profile
herpetic versus other viral encephalop.
18 years ago




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