Electrical status epilepticus during sleep: a case report and review of the literature.

We present the case of a 5-year-old male who other than being six weeks premature at birth had no significant early medical problems, and had normal physical and cognitive development until preschool. It was then that his teacher noticed the child was having learning difficulties, staring spells, and difficulty expressing himself He had a single generalized tonic-clonic seizure (GTCS) in September of 2008. A routine EEG revealed very frequent epileptiform discharges. He was started on levetiracetam and four months later his EEG showed continuous spike wave discharges during sleep. The spike wave discharges occurred in long runs, lasting a minute or more, without clear ictal evolution. The spike wave discharges were seen in a generalized distribution, maximal bifronto-temporally with some asymmetry (right greater than left). When the patient was subsequently awakened, the EEG dramatically improved and the spike wave discharges almost completely resolved. The spike and wave discharges recurred as the patient became drowsy again. Authors have described this as electrographic status during sleep (ESES) which is a typical childhood process of generalization of paroxysmal activity and can be seen with specific childhood epileptic encephalopathies. Treatment with levetiracetam was increased and his EEG and language subsequently returned to normal.

Sudden appearance of new upper extremity motor function while performing neurophysiologic intraoperative monitoring during tethered cord release: a case report.

UNLABELLED: : Tethered cord syndrome occurs when the distal spinal cord or filum adheres to adjacent structures resulting in progressive sensorimotor deficits in the lower extremities, fecal and/or urinary incontinence, and musculoskeletal deformities. Tethering of the distal cord may be idiopathic, may be associated with an intraspinal abnormality such as a lipoma, but most commonly the distal spinal cord remnant is adherent to the area of the original dysraphism repair in patients with myelodysplasia. Surgery to untether the cord is indicated in patients with worsening pain symptoms, progressive limb deformity or spasticity, or before any acute correction of an associated spinal deformity. Neurophysiologic intraoperative monitoring is used to minimize the risk of inadvertent nerve root or spinal cord injury during the untethering procedure and to assess any changes in cord function at the time of an associated spinal deformity correction. We present a patient with a lumbar level myelodysplasia, Chiari II malformation, severe scoliosis, and tethered cord that underwent concurrent scoliosis correction and tethered cord syndrome surgery, who demonstrated immediate intraoperative improvement in neurophysiologic responses in a previously flaccid upper extremity after untethering. These monitoring changes correlated with clinical improvements noted by physicians and family postoperatively. LEVEL OF EVIDENCE: Level IV.