Juan Piantino

Nonconvulsive seizures are common in children treated with extracorporeal cardiac life support.

Objectives: The prevalence of electrographic seizures or nonconvulsive status epilepticus and the effect of such seizures in children treated with extracorporeal cardiac life support are not known. We investigated the occurrence of electrographic abnormalities, including asymmetries in amplitude or frequency of the background rhythm and interictal activity in children undergoing extracorporeal cardiac life support and their association with seizures. We compared mortality and radiologic evidence of neurologic injury between patients with seizures and those without seizures. Design: Retrospective review of medical records and the Extracorporeal Life Support Organization database. Setting: PICU at a single institution. Patients: All pediatric patients up to 18 years old, who had extracorporeal cardiac life support and continuous electroencephalography monitoring between the years 2006 and 2011. Interventions: None. Measurements and Main Results: Nineteen patients treated with extracorporeal cardiac life support underwent continuous electroencephalography monitoring. Seizures occurred in four patients (21%) and were exclusively nonconvulsive in three patients. Two of these four patients had nonconvulsive status epilepticus. Interictal discharges on electroencephalography were associated with seizures (odds ratio, 19.5 [95% CI, 1.29–292.75]; p = 0.03). Only 50% of the seizures were detected in the first hour of monitoring, whereas all seizures were detected within 24 hours. All patients with seizures had structural abnormalities seen on neuroimaging. Seizures were not significantly associated with increased mortality. To evaluate for ascertainment bias, we compared outcomes between patients who underwent extracorporeal cardiac life support and received continuous electroencephalography monitoring and those patients who underwent extracorporeal cardiac life support during the study period but did not receive electroencephalography (n = 30). Conclusions: Seizures are common in children during extracorporeal cardiac life support, and most seizures are nonconvulsive. In patients undergoing extracorporeal cardiac life support, clinical features are unreliable indicators of the presence of seizures. The presence of seizures is suggestive of CNS injury. This study is limited by the exclusion of neonates, a feature of the clinical use of electroencephalography at our institution. Although seizures were not associated with increased mortality, further prospective studies in larger populations are needed to assess the long-term morbidity associated with seizures during extracorporeal cardiac life support.

Pediatric neurocritical care in the 21st century: From empiricism to evidence

Purpose of reviewApproximately one in five children admitted to a pediatric ICU have a new central nervous system injury or a neurological complication of their critical illness. The spectrum of neurologic insults in children is diverse and clinical practice is largely empirical, as few randomized, controlled trials have been reported. This lack of data poses a substantial challenge to the practice of pediatric neurocritical care (PNCC). PNCC has emerged as a novel subspecialty, and its presence is expanding within tertiary care centers. This review highlights the recent advances in the field, with a focus on traumatic brain injury (TBI), cardiac arrest, and stroke as disease models. Recent findingsVariable approaches to the structure of a PNCC service have been reported, comprising multidisciplinary teams from neurology, critical care, neurosurgery, neuroradiology, and anesthesia. Neurologic morbidity is substantial in critically ill children and the increased use of continuous electroencephalography monitoring has highlighted this burden. Therapeutic hypothermia has not proven effective for treatment of children with severe TBI or out-of-hospital cardiac arrest. However, results of studies of severe TBI suggest that multidisciplinary care in the ICU and adherence to guidelines for care can reduce mortality and improve outcome. SummaryThere is an unmet need for clinicians with expertise in the practice of brain-directed critical care for children. Although much of the practice of PNCC may remain empiric, a focus on the regionalization of care, creating defined training paths, practice within multidisciplinary teams, protocol-directed care, and improved measures of long-term outcome to quantify the impact of such care can provide evidence to direct the maturation of this field.

If You Build It, They Will Come: Initial Experience with a Multi-Disciplinary Pediatric Neurocritical Care Follow-Up Clinic

Pediatric Neurocritical Care diagnoses account for a large proportion of intensive care admissions. Critical care survivors suffer high rates of long-term morbidity, including physical disability, cognitive impairment, and psychosocial dysfunction. To address these morbidities in Pediatric Neurocritical Care survivors, collaboration between Pediatric Neurology and Pediatric Critical Care created a multidisciplinary follow-up clinic providing specialized evaluations after discharge. Clinic referrals apply to all Pediatric Neurocritical Care patients regardless of admission severity of illness. Here, we report an initial case series, which revealed a population that is heterogenous in age, ranging from 1 month to 18 years, and in diagnoses. Traumatic brain injuries of varying severity as well as neuroinfectious and inflammatory diseases accounted for the majority of referrals. Most patients (87%) seen in the clinic had morbidities identified, requiring ongoing evaluation and expansion of the clinic. Cognitive and psychological disturbance were seen in over half of patients at the initial clinic follow-up. Sleep disturbances, daytime fatigue, headache or chronic pain, and vision or hearing concerns were also common at initial follow-up. Data from this initial population of clinic patients reiterates the need for specialized follow-up care, but also highlights the difficulties related to providing this comprehensive care and evaluating interventions to improve outcomes.

A treatable metabolic cause of encephalopathy: Cobalamin C deficiency in an 8-year-old male

Neurologic regression in a previously healthy child may be caused by metabolic or neurodegenerative disorders, many of which have no definitive treatment. We report a case of a previously healthy 8-year-old boy who presented with a month-long history of waxing and waning encephalopathy and acute regression, followed by seizures. Evaluation for a metabolic disorder revealed methylmalonic acidemia and hyperhomocysteinemia of the cobalamin C type due to a single, presumed homozygous pathogenic c.394 C>T mutation in the MMACHC gene. With the appropriate diet restrictions and vitamin replacement, he improved significantly and returned to his premorbid level of behavior. This case illustrates an unusual presentation of a treatable metabolic disorder and highlights the need to consider cobalamin defects in the differential diagnosis of healthy children with neurologic regression.

Venous stroke and status epilepticus due to milk-induced anemia in a child.

The risk factors for cerebral sinus venous thrombosis include dehydration, infection, and anemia. The clinical presentation in children of venous strokes associated with cerebral venous thrombosis is variable and may include seizures. Acute management should focus on the treatment of the primary cause and anticoagulation or antiplatelet therapy if needed. Early recognition and targeted treatment is important because survivors are at increased risk for long-term neurologic complications. We report a case of a 4-year-old girl who presented with status epilepticus and was subsequently found to have a cerebral venous sinus thrombosis in the transverse and sigmoid sinus, with venous infarction in the temporal lobe. Laboratory results were significant for a microcytic anemia caused by excessive milk intake. Although iron deficiency anemia is a common pediatric disorder, this uncommon presentation demonstrates the potential for neurologic complications secondary to anemia, as well as the need for a high index of suspicion in order to identify venous stroke as a cause in children who present to the emergency department with seizures.

Pediatric Hypothermic Submersion Injury and Protective Factors Associated with Optimal Outcome: A Case Report and Literature Review

Drowning is the 3rd leading cause of unintentional injury death worldwide, with the highest rates of fatality among young children. Submersion injuries with cardiac arrest can lead to long-term neurologic morbidity. Severe hypothermic submersion injuries have complex treatment courses and survivors have variable neurocognitive outcomes. We describe the course of a hypothermic submersion injury in a 6-year-old previously healthy boy. The description includes premorbid and post-injury neurocognitive functioning. A review of the literature of pediatric cold-water submersion injury was performed. Despite prolonged cardiopulmonary resuscitation (>100 min) and water temperature well above freezing, our patient had an optimal neurocognitive outcome following hypothermic submersion injury. Available literature is limited but suggests that increased submersion time, increased duration of resuscitation, and higher water temperatures are associated with worse outcomes. Care guidelines have been created, but outcomes related to these guidelines have not been studied. Our case highlights potential important determinants of outcome after drowning. Incident specific characteristics and therapeutic interventions should be considered when evaluating this population. Treatment guidelines based on currently available literature may fail to incorporate all potential variables, and consideration should be given to prolonged resuscitative efforts based on individual case characteristics until further data is available.

Baseline and outcome assessment in pediatric status epilepticus

PURPOSE To summarize different aspects of short and long-term outcomes associated with SE, including mortality, recurrence, subsequent epilepsy, neurocognitive dysfunction, imaging abnormalities, and health-related quality of life. METHODS We searched MEDLINE for studies that assessed the short-term and long-term outcome of status epilepticus in pediatric population, including mortality, recurrence of seizure and status epilepticus, neurological, cognitive, or behavioral impairment, and health-related quality of life. We excluded studies that exclusively assessed the adult population. RESULTS Mortality in pediatric SE is relatively low, while morbidity poses more challenges. The underlying cause of SE has been shown to be a major determinant in the outcome after SE. However, it is difficult to establish the net effect of SE on outcome due to the heterogeneity of the studies. Notably, this review highlights that health-related quality of life, an important aspect of long-term outcome in pediatric SE, is under-addressed and merits further investigation. CONCLUSION There is a need to acquire high-quality long-term data evaluating QoL, neuroimaging, use of continuous infusions, and cognitive and behavioral outcome of children who experience SE.

Optimizing Neurocritical Care Follow-up through the Integration of Neuropsychology

BACKGROUND Pediatric critical care survivors often suffer persisting multisystem health problems and are left with treatment needs that go unmet due to limits in current care models. We proposed that integration of neuropsychology into neurocritical care follow-up provides incremental benefit to the identification and treatment of persisting complications and reduction in co-morbidities. BASIC PROCEDURES The aims of this study were three-fold. First, we described pilot programs at two pediatric hospitals as models for implementing systematic follow-up care with interdisciplinary clinic teams consisting of critical care, neurology, and neuropsychology. Second, we described working models specific to neuropsychological service delivery in these programs. Third, we presented preliminary data from the first six months of one of the pilot programs in order to examine incremental benefit of neuropsychology in improving patient care and parent satisfaction. MAIN FINDINGS A total of 16 patients (age range three to 17 years) were seen by neuropsychology within the first six months of the program. Results showed that integration of neuropsychology into follow-up care resulted in recommendations being made for services or concerns not already addressed in 81% of cases. Parents reported high satisfaction, endorsing the highest possible rating on 96% of all items. Parents reported that neuropsychological consultation improved their understanding and communication with their child, and helped them know what to expect from their child during postacute recovery. CONCLUSIONS The results of this pilot study suggest that integration of neuropsychology into neurocritical care follow-up programs contributes to parent satisfaction and may provide incremental benefit to patient care.

Lacosamide in Pediatric Status Epilepticus

Investigators from Baylor College of Medicine studied the efficacy of lacosamide in pediatric status epilepticus (SE).

Transcranial Doppler Identification of Neurologic Injury during Pediatric Extracorporeal Membrane Oxygenation Therapy

BACKGROUND We used transcranial Doppler to examine changes in cerebral blood flow velocity in children treated with extracorporeal membrane oxygenation. We examined the association between those changes and radiologic, electroencephalographic, and clinical evidence of neurologic injury. METHODS This was a retrospective review and prospective observational study of patients 18 years old and younger at a single university childrens hospital. Transcranial Doppler studies were obtained every other day during the first 7 days of extracorporeal membrane oxygenation, and 1 additional study following decannulation, in conjunction with serial neurologic examinations, brain imaging, and 6- to 12-month follow-up. RESULTS The study included 27 patients, the majority (26) receiving veno-arterial extracorporeal membrane oxygenation. Transcranial Doppler velocities during extracorporeal membrane oxygenation were significantly lower than published values for age-matched healthy and critically ill children across different cerebral arteries. Neonates younger than 10 days had higher velocities than expected. Blood flow velocity increased after extracorporeal membrane oxygenation decannulation and was comparable with age-matched critically ill children. There was no significant association between velocity measurements of individual arteries and acute neurologic injury as defined by either abnormal neurologic examination, seizures during admission, or poor pediatric cerebral performance category. However, case analysis identified several patients with regional and global increases in velocities that corresponded to neurologic injury including stroke and seizures. CONCLUSIONS Cerebral blood flow velocities during extracorporeal membrane oxygenation deviate from age-specific normal values in all major cerebral vessels and across different age groups. Global or regional elevations and asymmetries in flow velocity may suggest impending neurologic injury.