Tuesday, January 16, 2018

A Wong-Kisiel potpourri

English, S. W., Ho, M. L., Tollefson, M. M., & Wong-Kisiel, L. C. (2017). Focal Epilepsy in a Teenager With Facial Atrophy and Hair Loss. Seminars in Pediatric Neurology. https://doi.org/10.1016/j.spen.2017.03.009

There is increasing evidence to demonstrate that Parry-Romberg syndrome and linear scleroderma en coup de sabre are both forms of linear scleroderma, representing localized autoimmune conditions affecting the skin, eyes, brain, and surrounding tissues. We present a case highlighting the clinical presentation of a 12-year-old boy with focal seizures and physical examination findings of facial atrophy and hair loss. This article reviews the literature related to the presentation, epidemiology, diagnosis, and treatment of Parry-Romberg syndrome and linear scleroderma en coupe de sabre with focus on the significant correlation with neurologic disease, particularly seizures.

Lapalme-Remis S, Witte RJ, Wong-Kisiel LC. Anteroinferior Temporal
Encephalocele: A Surgically Treatable Cause of Pharmacoresistant Epilepsy.
Pediatr Neurol. 2017 Dec;77:89-90.

No abstract.  From the article.

This 14-year-old boy with pharmacoresistant epilepsy was referred for presurgical evaluation. Seizure semiology consisted of staring, swallowing, and left hand fumbling. An earlier brain magnetic resonance imaging (MRI) had been described as nonlesional. Electroencephalography showed epileptiform discharges from the left temporal region and two seizures arising from the left frontotemporal area. His brain MRI showed a left temporal anteroinferior encephalocele, confirmed by high-resolution skull-base computed tomography. Fluorodeoxyglucose positron emission tomography demonstrated diminished left temporal uptake. He experienced a greater than 90% reduction in seizure frequency following resection of the encephalocele and temporal pole...

At one center, temporal encephaloceles were identified in 1.9% of surgical epilepsy referrals. Clinical characteristics and electroencephalography findings were typical of temporal lobe epilepsy.  High seizure-free rates following surgery are reported.  Simple resection of the encephalocele and temporal pole, or temporal lobectomy with amygdalohippocampectomy, may be considered.

No abstract.  From the article.

Feyissa AM, Patterson MC, Wong-Kisiel LC. Too Old for a Diaper! A Child With
Diaper Changing-Induced Seizures. Pediatr Neurol. 2016 Jan;54:91-2.

This 3-year-old boy with developmental delay presented at age 7 months with seizures characterized by sudden brief whole-body jerking. Seizures almost exclusively occurred during diaper changing and coincided with the perianal wiping phase of the act. Neurological examination and brain magnetic resonance imaging were normal. Interictal electroencephalography (EEG) revealed midline and left central epileptiform discharges and a moderate degree of slowing in these same regions. Ictal EEG documented a midline-central fast activity followed by a 1-2 Hz polyspike-wave discharge over the midline and left parietocentral areas.  Based on EEG findings and clinical observation of the seizures, the diagnosis of focal-onset epilepsy with seizures provoked by diaper changing was made. Treatment with oxcarbazepine resulted in a significant initial reduction in seizure frequency. However, a month after starting therapy the seizure frequency increased again and the dose of oxcarbazepine was escalated, resulting in further reduction of seizure frequency.

Here we describe the first example of diaper changing–induced seizures. In our patient, seizures did not occur soon after wet diapers and micturition reflex epilepsy is less likely. Seizures related to diaper changing may be provoked by the tactile stimulation of the perianal area and, therefore, may be a variant of sensory reflex seizure. Although cranial magnetic resonance imaging did not show a focal lesion to explain the diaper changing focal seizures, his interictal and ictal EEG findings are suggest a midline or frontal seizure focus. Reflex epilepsy without an identifiable lesion on neuroimaging is not unprecedented.

Avoidance of provocative stimuli is important in maintaining seizure control in reflex epilepsies, but stimulus avoidance in this boy was not practical. For the treatment of reflex seizures, conventional antiepileptic drugs as well as vagus nerve stimulation have been tried with varying success.  Currently, there is no standard antiepileptic drug regimen that is effective for all reflex seizures. However, previous reports in children appear to suggest that oxcarbazepine and carbamazepine could be superior to other antiepileptic drugs for focal reflex epilepsies.  In our patient, oxcarbazepine significantly reduced seizures but did not result in seizure freedom. For selected individuals with reflex epilepsy due to an identifiable focal lesion, surgical resection as well as chronic subthreshold cortical stimulation are sometimes effective.  

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