P 89 Case presentation – Clinically unrecognized status epilepticus in neuronal ceroid lipofuscinosis (CLN 3 gene)

On the previous day the 12-year-old boy P.-J. had a convulsive spasm (duration about 30s) in the morning and at noon. After that, he recovered and went outside for a walk. Before the evening meal P.-J. cramped again. Since then, he was not really awake, was not responsive, and had repeatedly convulsions (seven times on the day of admission). In between he was always sleepy and unresponsive. Since noon on the previous day, P. Luca did not eat and drink any more and did not take his medication. In the evening, the emergency physician was called, who did not administer any medicines because of stable vital parameters and only a short convulsive spasm when drawing blood from the vein.

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