Worsening of Hand Function but Significant Improvement of Abilities Measured in PEDI in a Child with Sturge-Weber Syndrome

Background/Purpose: In epilepsies due to hemispheric lesions, hemispherectomy often is the best therapeutic option regarding postoperative seizure outcome and thus better preconditions regarding child development. But one of the functional results of the operation is a complete hemiparesis. In children with no or little preoperative hemiparesis, this means a worsening of motor function. In such cases, it is always a matter of discussion, if the chance for seizure freedom and better developmental potential justifies the motor deficit.

Methods: We present a boy with drug-resistant epilepsy (preoperative: 15 seizures/day) due to Sturge-Weber syndrome. Prior to hemispherectomy (done at the age of 20 months; postoperative seizure free), the child had a clear but incomplete hemiparesis.

Results: There was a significant worsening of hemiparesis and use of the paretic hand (mini-assisting hand assessment: preoperative 20 units; postoperative 9 units; significant change ≥ 5 units). Result of evaluation with PEDI, however, showed a significant improvement in all three measured domains: self-care (pre 21.4 [standard error of measurement, SEM = 3.6], post 41.2 [SEM 1.8]), mobility (pre 20.9 [SEM 3.6], post 46.1 [SEM 2.2]), and social function (pre 3.1 [SEM 3.1], post 37.9 [SEM 1.5]).

Conclusion: The evaluation of this patient showed an objective improvement of abilities in PEDI after hemispherectomy in spite of worsening of hemiparesis and use of the paretic hand. Objective reports like this could be helpful in decision making and preoperative counseling of caregivers.

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