Successful Reduction in Electrode-Related Pressure Ulcers During EEG Monitoring in Critically Ill Neonates.

BACKGROUND: Neonates are at a high risk for pressure ulcers (PU) due to skin immaturity and exposure to various medical devices. The prevalence of PU in the neonatal intensive care unit is estimated to be 23%, with 80% of those being related to medical devices, including electroencephalographic (EEG) electrodes. Proposed mechanisms involve prolonged pressure to the electrodes and chemical reactions to conductive agents. PURPOSE: The object of this quality improvement project was to reduce PU in neonates during continuous EEG (cEEG) monitoring by 50% within 12 months and 75% within 18 months. A secondary objective was to eliminate electrode-related infections by 12 months. Balancing measures included gestational age at the time of monitoring, integrity of the EEG setup, and cost effectiveness. The process measure was adherence to the skin-monitoring tool kit. METHODS: A multiple Plan-Do-Study-Act cycle method was used. All neonates monitored with cEEG were included. The monitoring tool kit was used to document the condition of scalp and EEG electrodes before, during, and after cEEG. RESULTS: In the preproject period, 8.5% (9/106) of monitored patients developed PU, and 22.2% (2/9) of those developed infections. During the project period, 3.5% (7/198) of monitored patients developed PU and no infections were observed. During monitoring, 21 patients showed skin irritation, and timely intervention resulted in resolution in more than 90% of the cases and prevented progression into PU. Silver/silver chloride-plated electrodes, when exposed to external heat sources, can cause burns, resembling PU. IMPLICATIONS FOR PRACTICE: Intervention at the electrode level together with skin inspection successfully reduces PU in neonates. Silver/silver chloride-plated electrodes should be avoided in neonates. IMPLICATIONS FOR RESEARCH: Further research is needed to identify the optimal electrode for neonatal EEG.

Standardized Treatment of Neonatal Status Epilepticus Improves Outcome.

We aimed to decrease practice variation in treatment of neonatal status epilepticus by implementing a standardized protocol. Our primary goal was to achieve 80% adherence to the algorithm within 12 months. Secondary outcome measures included serum phenobarbital concentrations, number of patients progressing from seizures to status epilepticus, and length of hospital stay. Data collection occurred for 6 months prior and 12 months following protocol implementation. Adherence of 80% within 12 months was partially achieved in patients diagnosed in our hospital; in pretreated patients, adherence was not achieved. Maximum phenobarbital concentrations were decreased (56.8 vs 41.0 µg/mL), fewer patients progressed from seizures to status epilepticus (46% vs 36%), and hospital length of stay decreased by 9.7 days in survivors. In conclusion, standardized, protocol-driven treatment of neonatal status epilepticus improves consistency and short-term outcome.

The phenotypic spectrum of SCN8A encephalopathy.

OBJECTIVE: SCN8A encodes the sodium channel voltage-gated α8-subunit (Nav1.6). SCN8A mutations have recently been associated with epilepsy and neurodevelopmental disorders. We aimed to delineate the phenotype associated with SCN8A mutations. METHODS: We used high-throughput sequence analysis of the SCN8A gene in 683 patients with a range of epileptic encephalopathies. In addition, we ascertained cases with SCN8A mutations from other centers. A detailed clinical history was obtained together with a review of EEG and imaging data. RESULTS: Seventeen patients with de novo heterozygous mutations of SCN8A were studied. Seizure onset occurred at a mean age of 5 months (range: 1 day to 18 months); in general, seizures were not triggered by fever. Fifteen of 17 patients had multiple seizure types including focal, tonic, clonic, myoclonic and absence seizures, and epileptic spasms; seizures were refractory to antiepileptic therapy. Development was normal in 12 patients and slowed after seizure onset, often with regression; 5 patients had delayed development from birth. All patients developed intellectual disability, ranging from mild to severe. Motor manifestations were prominent including hypotonia, dystonia, hyperreflexia, and ataxia. EEG findings comprised moderate to severe background slowing with focal or multifocal epileptiform discharges. CONCLUSION: SCN8A encephalopathy presents in infancy with multiple seizure types including focal seizures and spasms in some cases. Outcome is often poor and includes hypotonia and movement disorders. The majority of mutations arise de novo, although we observed a single case of somatic mosaicism in an unaffected parent.

Is it ADEM, POLG, or both?

OBJECTIVE: To describe a child with apparent brain biopsy-confirmed acute disseminated encephalomyelitis (ADEM) but genetic confirmation of compound heterozygosity for DNA mutations of the polymerase gamma (POLG) gene. DESIGN: Case report. SETTING: Tertiary referral center. PATIENT: A 4-year-old boy presented with ataxia and encephalopathy. RESULTS: Magnetic resonance imaging demonstrated multiple focal areas of T2 prolongation. The patient's family refused steroid treatment. His symptoms improved then progressed. Magnetic resonance imaging findings also progressed. A cerebrospinal fluid specimen revealed myelin basic protein and oligoclonal bands. A brain biopsy specimen demonstrated demyelination, suggesting progression of ADEM. However, polymerase chain reaction amplification and sequencing revealed 2 heterozygous mutations of the POLG gene, suggesting mitochondrial disease. The patient died 9 months after his initial presentation. CONCLUSIONS: This case raises interesting questions about whether ADEM triggered severe neurologic degeneration in a patient with mitochondrial disease, whether mitochondrial disease predisposed to a pathologic immune response, or whether mitochondrial disease can mimic an autoimmune disease. Mitochondrial disease-causing mutations may help explain the poor outcome in some cases of apparent autoimmune central nervous system disease.

Intractable seizures, developmental delay, and the ketogenic diet.

Glucose transporter type 1 (GLUT1) deficiency syndrome is a rare, treatable cause of developmental delay and seizures. It must be considered in the differential diagnosis of infants with intractable seizures. The finding of a low glucose level in the cerebrospinal fluid with normal level in the blood in the absence of pleocytosis or other cerebrospinal fluid abnormalities identifies the condition. Genetic analysis for confirmation is available. Treatment with antiepileptic medications often is unsuccessful, and the ketogenic diet is the favored treatment for seizure control. Early identification and initiation of treatment may prevent or lessen the severity of developmental delay.