Abstracts

Rationale: The patient is a 5 year-old M who was admitted with confusion, neck stiffness, drooling, and tongue deviation. Mom reports that he first started having mild neck stiffness, fever, a mild cough/runny nose and vomiting 3 days prior to admission (Tmax 101). He became more drowsy and with drooling. At OSH he was rapid strep positive and was found to have a "shuffling gait” questionable left face droop and tongue deviation with slurred speech. He has a prior history of latent TB and a strong family history of autoimmune disease.extensive wrok up was unremearkable including : infectious, autoimmune, rheumatological and MRI brain and neck WWO. VEEG frequent independent paroxysmal, high-amplitude, spike/spike-and-wave discharges in the left and right occipital area (O1 and O2) and becamemore active and frequent during sleep.complete resolution of symptoms prior to discharge home.The patient was seen in clinic 1 months after discharge. Since discharge mom reported he had another similar episode with intractable vomiting, tongue deviation and drooling for 3 days. No abdominal pain, fever, or headache . He was “out of it”. The episode resolved after 4 days. He was started on Levetiracetam which was changed to Oxcarbazepine due to behavioral side effects. He has had no subsequent episodes.  Methods: N/A Results: N/A Conclusions: There has been no case reported of an association between Panayiotopoulos Syndrome (PS) and isolated cranial nerve (CN) findings. In our case it can be postulated that brainstem dysfunction is a postictal finding. Our patient had two stereotyped episodes with complete resolution after 4 days associated with emesis and EEG findings consistent with PS. PS is known as a benign childhood epilepsy. It is characterized by seizures, often prolonged, with predominantly autonomic symptoms and by an EEG that shows shifting and/or multiple foci, often with occipital predominance. Autonomic seizures consist of  episodes characterized by emetic symptoms usually characterized by other autonomic features ( color change, pallor, pupillary changes, cardiorespiratory and thermoregulatory alterations) and more conventional ictal features such as unilateral eye deviation and convulsions. Peak age 3-6years. PS is often misdiagnosed at onset as atypical migraine, gastroenteritis, syncope, encephalitis, or intoxication especially when occurring during a fever. Children seem more vulnerable to autonomic symptoms than adults independent of seizure focus due to presumed immature brainstem and cortical inhibition. It is likely that an epileptic electrical discharge activates susceptible autonomic centers leading to autonomic seizures and autonomic status epilepticus. This occurs before the generation of clinical manifestations from brain regions that are topographically related to the ictal electrical discharge (occipital, frontal, central, parietal and less often temporal) with seizure thresholds higher than those of the autonomic centers.  Funding: N/A