Jorge Vidaurre

Complex Febrile Seizures: A Practical Guide to Evaluation and Treatment

Febrile seizures are the most common type of childhood seizures, affecting 2% to 5% of children. A complex febrile seizure is one with focal onset, one that occurs more than once during a febrile illness, or one that lasts more than 10 to 15 minutes. Confusion still exists on the proper evaluation of a child presenting with a complex febrile seizure. There are ongoing research attempts to determine the link between complex febrile seizures and epilepsy. Further clarification and understanding of this disorder would be of great benefit to primary care providers and child neurologists.

Unique Characteristics of the Photoparoxysmal Response in Patients With Neuronal Ceroid Lipofuscinosis Type 2 Can EEG Be a Biomarker

Objective: The objective was to identify unique features of the photoparoxysmal response seen in patients with neuronal ceroid lipofuscinosis type 2 as compared to patients with a photoparoxysmal response associated with other epilepsy syndromes. Methods: Electroencephalograms from patients with neuronal ceroid lipofuscinosis type 2 seen at the authors’ institution in the past 10 years as well as electroencephalograms (EEGs) reported to have a photoparoxysmal response during a single year were reviewed. Results: A photoparoxysmal response was seen in 60% of the patients with neuronal ceroid lipofuscinosis type 2. This was most commonly seen with low frequency intermittent photic stimulation (76%) which often occurred in a time-locked fashion (63%) and was seen on the patient’s initial EEG (78%). A unique pattern the authors called “sentinel” discharge was identified in 30% of EEGs in patients with neuronal ceroid lipofuscinosis. Conclusions: Photoparoxysmal responses in patients with neuronal ceroid lipofuscinosis type 2 have features which are distinguishing from photoparoxysmal responses seen in other epilepsies.

Antiepileptic Drugs and Liver Disease

Acute, symptomatic seizures or epilepsy may complicate the course of hepatic disease. Choosing the most appropriate antiepileptic drug in this setting represents a difficult challenge, as most medications are metabolized by the liver. This article focuses on the acute and chronic treatment of seizures in patients with advanced liver disease and reviews the hepatotoxic potential of specific antiepileptic drugs. Newer antiepileptic drugs without, or with minimal, hepatic metabolism, such as levetiracetam, lacosamide, topiramate, gabapentin, and pregabalin should be used as first-line therapy. Medications undergoing extensive hepatic metabolism, such as valproic acid, phenytoin, and felbamate should be used as drugs of last resort. In special circumstances, as in patients affected by acute intermittent porphyria, exposure to most antiepileptic drugs could precipitate attacks. In this clinical scenario, bromides, levetiracetam, gabapentin, and vigabatrin constitute safe choices. For the treatment of status epilepticus, levetiracetam and lacosamide, available in intravenous preparations, are good second-line therapies after benzodiazepines fail to control seizures. Hepatotoxicity is also a rare and unexpected side effect of some antiepileptic drugs. Drugs such as valproic acid, phenytoin, and felbamate, have a well-recognized association with liver toxicity. Other antiepileptic drugs, including phenobarbital, benzodiazepines, ethosuximide, and the newer generations of antiepileptic drugs, have only rarely been linked to hepatotoxicity. Thus physicians should be mindful of the pharmacokinetic profile and the hepatotoxic potential of the different antiepileptic drugs available to treat patients affected by liver disease.

Healthcare Utilization Characteristics for Intranasal Midazolam Versus Rectal Diazepam

To investigate connections between patient demographics, health care utilization, prescription use, and refills for patients using intranasal midazolam, per rectum diazepam, or both. A retrospective cohort contained patients with epilepsy prescribed intranasal midazolam, per rectum diazepam, or both. We analyzed number of emergency department visits, ambulance services, urgent care visits, and unplanned hospitalizations. A total of 5458 patients were identified. Patients on intranasal midazolam had on average 1.53 fewer emergency department visits (95% confidence interval 1.16-1.89, P < .0001), 0.29 fewer uses of ambulance services (95% confidence interval 0.17-0.41, P < .0001), and 0.60 fewer urgent care visits (95% confidence interval 0.36-0.83, P < .0001) compared to patients in the per rectum diazepam group. Patients with commercial insurance were more likely to have intranasal midazolam prescription (odds ratio = 1.73, 95% confidence interval 1.42-2.11, P < .0001). The results substantiate the cost-effective benefits of prescribing intranasal midazolam compared to per rectum diazepam because several aspects of health care utilization were decreased in those using intranasal midazolam.

Multifocal Brain Lesions With Extensive Perilesional Edema in a Young Boy Returning From Kenya

We report the case of an 8-year-old boy who presented with headaches, optic disc edema, and left eye esotropia. He had recently returned to the United States after living in Kenya for approximately 3 years. His brain magnetic resonance imaging showed multifocal lesions with significant perilesional edema seemingly out of proportion to his neurologic examination findings. With appropriate therapy there was remarkable improvement in his symptoms with reduction in size of the lesions and surrounding edema.

Atypical Presentation of a Progressive and Treatable Encephalopathy in an Older Child With Gelastic and Dacrystic Seizures

We discuss an unusual case of a teenage boy who presented with waxing and waning cognitive decline and gelastic-dacrystic seizures, evolving later into a rapidly progressive encephalopathy with status epilepticus. Extensive genetic and metabolic testing did not lead to a specific diagnosis. Cerebrospinal fluid studies performed during admission to the intensive care unit provided the information needed to establish a diagnosis. After implementation of specific treatment, his seizures stopped and his background electroencephalogram returned to normal. He has remained largely seizure-free experiencing a significant cognitive recovery. This case illustrates the importance of performing cerebrospinal fluid analysis in patient with refractory seizures and cognitive decline of unknown etiology.

Attention Deficit Hyperactivity Disorder and Associated Cognitive Dysfunction in Pediatric Epilepsy

Attention deficit hyperactivity disorder (ADHD) is the most common neuropsychiatric comorbidity associated with childhood epilepsy, affecting about a third of children with epilepsy. In contrast, ADHD in the general population occurs in 4%-12% of school-aged children. The cause of this association remains unclear. It is likely that common mechanisms underlie the vulnerability for both executive deficits and epileptogenesis. There are characteristics unique to children with ADHD and epilepsy. The inattentive type of ADHD is more prevalent than the combined presentation in children with epilepsy, while the combined type is more common in the general population. Interestingly, there is an equal sex distribution of ADHD in patients with epilepsy, while in the general population, ADHD is 3-7 times more prevalent in boys. Specific features of ADHD seen in different epilepsy syndromes are frequently associated with executive deficits. Early screening of ADHD symptoms in children with epilepsy is essential, as timely interventions can improve academic and social function and outcomes. The mainstays of therapy include behavioral interventions and pharmacotherapy, with evidence demonstrating that stimulants are both safe and effective in children with ADHD and epilepsy.