Aplasia cutis congenita and arteriovenous fistula. Case report and review.

We describe a child with congenital aplasia cutis congenita of the scalp and an occult giant posterior fossa arteriovenous fistula. Previous case reports of central nervous system malformations associated with aplasia cutis congenita are reviewed. The exact incidence of such malformations is unknown. All patients with aplasia cutis congenita should undergo a neurologic evaluation, and their families should be examined for similar lesions. Early central nervous system imaging and other workup may be required, especially if plastic surgery in the head region is being planned.

Status epilepticus: clinical experience with two special devices for continuous cerebral monitoring.

Continuous cerebral monitoring (CCM) was performed on 34 patients in status epilepticus (SE), using changes in amplitude detected by the cerebral function monitor (CFM) and changes in frequency detected by compressed spectral array (CSA). The EEG was used intermittently to help identify seizure patterns obtained with these methods. Seventeen patients in clinically manifest SE also had non-convulsive seizures. In 17 patients, SE was refractory to conventional anticonvulsants, requiring treatment with pentobarbital or paraldehyde. In these patients, CCM provided dynamic electroencephalographic monitoring of burst-suppression and prompt detection of breakthrough seizures. Patients in SE should undergo CCM to differentiate between non-convulsive seizures and post-ictal state both of which may produce prolonged unresponsiveness following clinical seizures. CCM after data reduction with the two special devices used is a viable and practical alternative to continuous conventional EEG monitoring during SE. However, in order to evaluate the sensitivity and specificity of these methods, it will be necessary to design a study in which both the EEG and the devices using data reduction be used continuously and concurrently.