Intracranial Arachnoid Cyst in Children: Clinical Presentation and Risk Factors for Surgical Intervention.

INTRODUCTION: Intracranial arachnoid cysts (IAC) in children are a common incidental finding on imaging. Most IACs are asymptomatic and can be monitored; however, a small percentage may enlarge and require surgical intervention. This study aimed to identify clinical risk factors in patients with IAC who underwent surgery versus those who did not. METHODS: We conducted a retrospective chart review from 2009 to 2021 at a free-standing children's hospital. A total of 230 patients diagnosed with an IAC aged 0-21 years of age were included in the study. Data on demographics, imaging, and neurological follow-up were analyzed. RESULTS: Out of 230 patients, 45 (19.6%) underwent surgery. At time of IAC diagnosis, the surgical patients were younger (median age 1.1 years), and their median cyst volume was larger (41.7 cm3), compared to nonsurgical patients (median age 5.9 years, volume 11.8 cm3, respectively). Headache was the most common reason for initial imaging in nonsurgical patients (54/185, 29.2%) while prenatal ultrasound (11/45, 24.4%) and macrocephaly (11/45, 24.4%) were the most common reasons for surgical patients. The majority of both surgical and nonsurgical patients had the IAC incidentally found (41/45, 91.1% and 181/185, 97.8%, respectively). Surgery relieved symptoms in 38/45 (84.4%) patients. Cyst volume and age were predictors of increased odds of having surgery. DISCUSSION/CONCLUSION: Patients who underwent surgery were younger and had larger cyst volumes at time of diagnosis. The majority of the IAC were found incidentally and remained stable over prolonged follow-up. The majority of the patients experienced relief of symptoms postsurgical intervention. There is a greater odds of having surgical treatment with decreased age and greater cyst volume at diagnosis, and therefore these patients should be monitored closely for development of symptoms indicating need for surgical intervention.

Peripheral Nerve Blocks: A Tool for Inpatient Pediatric Status Migrainosus.

BACKGROUND: To assess the efficacy and safety of peripheral nerve blocks in the inpatient settings for pediatric patients presenting with status migrainosus. METHODS: An analysis of a retrospective cohort of patients aged 13 to 18 years, admitted for status migrainosus from 2017 to 2022, was performed. Among the 1805 patients who presented with status migrainosus, 265 required hospital admission. A total of 177 patients failed the first- and second-line intravenous therapy and were treated with either peripheral nerve block (PNB) or dihydroergotamine (DHE) intravenous infusions. The primary outcome of the study was pain score level reduction by 50%, and the secondary outcome was duration of hospital stay. Visual analog scale score was used for pain assessment. RESULTS: Among the 177 patients, 100 patients were treated with DHE and 77 were treated with PNB. Target pain control was achieved in 59 of 100 patients receiving DHE and 38 of 77 patients receiving PNB. The average hospital stay of patients who responded to PNB was significantly lower compared with that of patients receiving DHE (3.6 days vs 4.9 days). Among the 41 patients who were refractory to DHE, 30 patients received PNB, of which 12 responded to nerve blocks. The most common side effects for PNBs were pain at injection site in 39% of patients and nausea/vomiting for DHE in 50% of patients. CONCLUSIONS: PNB can be safely administered in the hospital setting to pediatric patients with status migrainosus. PNB treatment helped achieve target pain control with minimal side effects and reduced the hospital stay duration.

Is Botox Right for Me: When to Assess the Efficacy of the Botox Injection for Chronic Migraine in Pediatric Population.

Botulinum toxin type A (BoNT-A) has shown to be a safe and effective treatment for children with chronic migraines. Our study was to assess the efficacy of the Onabotulinum toxin type A at different intervals after initiation of therapy. We conducted a retrospective and prospective analysis of 34 patients at a children's hospital where children received four rounds of the BoNT-A therapy for the treatment of chronic migraine. Among the 34 patients, 25 patients (age range: 13-21 years), who responded to the BoNT-A therapy, were included in the analysis. Patients received standard 31 injection, 155 unit's protocol. Patients were assessed every 3 months after their initial injection. Reasons for discontinuation of therapy were analyzed. After the first two BoNT-A sessions, significant improvement was observed with a decrease in headache frequency and intensity (p < 0.001). There was further reduction in headache frequency and intensity with the fourth round of BoNT-A therapy, with comparative analysis between the second and fourth round showing a p-value of <0.001. In terms of reduction of emergency room visits and hospitalization, a significant improvement was seen after the third round of BoNT-A therapy (p < 0.01). A significant decrease in the number of abortive and preventive medications was seen after the second round of BoNT-A therapy (p < 0.001). The efficacy of BoNT-A treatment in decreasing headache frequency, intensity, and the number of abortive and preventive medications can be assessed effectively after two treatment sessions. This trend continued to be observed with additional third and fourth sessions.

Generalized Anxiety Disorder: A Predictor for Poor Responsiveness to Botulinum Toxin Type A Therapy for Pediatric Migraine.

BACKGROUND: To assess the efficacy, safety, and predictors for poor responsiveness of botulinum toxin type A (BoNT-A) for chronic migraine in the adolescent and young adult population. METHODS: A retrospective analysis of 56 patients who received BoNT-A for chronic migraine with an age range from 13 to 21 years was performed. Of 56 patients, 34 were enrolled in the study based on the inclusion criterion. Patients who received three dosages of BoNT-A were assessed at nine months from the first injection. Variables including age, body mass index, headache intensity, frequency, character, and side effects were assessed. The patients were divided into two groups based on response to BoNT-A therapy, responders and nonresponders. RESULTS: Overall among the 34 patients enrolled in the study, the average headache frequency decreased from 18.6 of 28 to 9.9 of 28 days, P value, <0.001 from baseline. There was significant decrease in the average headache intensity, 8.1 to 4.3. Of 34 patients, 25 (73%) patients responded to treatment with decrease in headache frequency by ≥ 50% from the baseline. Among the nonresponder patients, a significant number of patients (six of nine, 67%) had generalized anxiety disorder with Generalized Anxiety Disorder-7 score greater than 15 versus the responder group (six of 25, 24%, P value 0.040). CONCLUSIONS: BoNT-A remains a safe and effective therapy for adolescent and young adult patients with chronic migraines at nine months of follow-up. Generalized anxiety disorder with Generalized Anxiety Disorder-7 score greater than 15 can be a major predictor of poor response to this therapy.

Utility of electroencephalogram in the pediatric emergency department.

INTRODUCTION: Electroencephalograms (EEG) can be helpful in evaluating patients presenting to the emergency department (ED). METHODS: We reviewed the charts of patients who had an EEG done in the ED at Dayton Children's Hospital in Dayton, Ohio from 2010 to 2018. We divided the indication for EEG into 6 categories, 1) suspected new onset seizures, 2) recurrent seizures, 3) altered mental status, 4) death, 5) non-convulsive status epilepticus, and 6) psychogenic seizures. We collected data on age, gender, suspected diagnosis, EEG result, outcome of the visit in terms of discharge versus inpatient admission, follow up of the patient with respect to a) diagnosis, b) medication, c) recurrent visit to the ED, and d) clinical outcome over a two-year follow up. We also collected data on the results of the subsequent video EEG in patients who had an EEG in the ED and determined whether the video EEG had the same results and/or added any additional information. RESULTS: We studied 162 patients (mean ± SD age 7.8 ± 5.8 years, 42.6% females) with routine EEG in the ED from 2010 to 2018. In 142 patients (87.7%), the EEG was helpful in confirming or ruling out the suspected diagnosis. For the indications of new onset seizures (n = 90), recurrent seizures (n = 48), acute mental status change (n = 22), and psychogenic seizures (n = 5), the EEG was useful in 91.1%, 81.3%, 81.8%, and 100% respectively. Of the 162 patients, 58 were discharged and 104 were admitted. For the 142 patients in whom the routine EEG was diagnostically useful, 59.9% were admitted, compared to 95.0% of the 20 patients in whom the EEG did not help in clarifying the diagnosis (p = 0.002). In 31 of the admitted patients, a video EEG was done. In the 31 patients who obtained video EEG, it did not add any additional information in 23 patients while in 8 patients (25.8%) the video EEG provided additional information that was useful for diagnosis and management. CONCLUSIONS: EEG done in the ED is a useful diagnostic tool that may prevent admission to the hospital. Video EEGs should be considered in patients where the diagnosis is uncertain despite obtaining a routine EEG in the ED.

Bilateral Ptosis, Zosteriform Rash and Flaccid Bladder in a 10-Year-old boy.

We present a case report of a 10-year-old completely immunized boy presenting with a 2-week history of bilateral eyelid drooping, fatigue followed by bladder and bowel paralysis. This was followed by the appearance of a vesicular painful and itchy rash which directed further diagnosis and treatment as it was consistent with a varicella reactivation rash. This case is a very important addition to the current body of literature on varicella-related neurological complications. It outlines that varicella reactivation can present in completely vaccinated, immunocompetent young children as a neurological syndrome affecting the autonomic nervous system primarily and the rash can occur a few weeks later after presentation of the neurological symptoms.

Pediatric auto-immune encephalitis.

Pediatric Auto-Immune Encephalitis (PAE) is a neuro-inflammatory disorder with a varied presentation. The discovery of the Anti NMDA receptor and other antibodies as the causative agents of PAE, has led to an increased need for guidelines for diagnosis and management of these disorders. PAE remains a challenging group of disorders due to their varying presentations and etiology with a prolonged clinical course. The wide spectrum of clinical symptoms involves altered mental status, movement disorders, acute behavioral changes, psychosis, delirium, seizures, and insomnia. This group of disorders was recently recognized in the children. This review provides clinicians with information on the most common PAE disorders, the spectrum of their clinical presentation, diagnostic tests and treatment protocols based on the current literature.

New diagnostic and therapeutic modalities in neuromuscular disorders in children.

Pediatric neuromuscular disorders are a diverse group of conditions that affect how muscle and nerve function. They involve the motor neurons, nerves, neuromuscular junction and muscles. Pathology of any of these regions leads to the inability to perform voluntary movements. Over time, the natural progression of most of these disorders is followed by significant disability, and at the most extreme, almost complete paralysis and death secondary to complications. Diagnostic measures for pediatric neuromuscular disorders, like that of most medical conditions, relies heavily on clinical presentation, history and a detailed physical examination. Primary additional diagnostic measures have included serum creatine kinase (CK) levels, electromyography (EMG), nerve conduction studies (NCS) and muscle or nerve biopsies, which has historically been the gold standard. In the last several decades less invasive testing has become more common such as muscle magnetic resonance imaging (MRI) and genetic testing.  The advances of molecular genetics, such as next generation sequencing (NGS) which includes whole-exome sequencing (WES) and whole-genome sequencing (WGS), enable clinicians to pinpoint more accurately exact gene mutations. The advent of genetic testing enhances personalized medicine. The field of pediatric neuromuscular disorders is also undergoing a remarkable evolution in therapeutic modalities including novel targeted therapies such as exon skipping/inclusion and gene replacement therapies. This is a review of the initial approach to suspected neuromuscular disorders in children as well as up to date diagnostic and therapeutic modalities for the most common pediatric neuromuscular disorders. As the world enters the new decade, there are encouraging therapeutic results. However, there remain key challenges to these modalities including limitations in its applicability, optimization for delivery of gene replacement therapies and in its effectiveness.

Childhood basilar artery occlusion: a report of 5 cases and review of the literature.

Basilar artery occlusion has poor outcome in adults; little is known regarding outcomes in children. Whether intra-arterial treatments improve adult outcomes is controversial. Safety and efficacy of intra-arterial treatments in children are unknown. We report 5 cases of basilar artery occlusion and review published cases. We estimated National Institute of Health Stroke Scale (NIHSS) and modified Rankin Score (mRS) of published cases, compared scores between non-intra-arterial treatments and intra-arterial treatments groups, and examined the correlation between NIHSS and mRS. Of our cases, 4 had good outcomes and 1 died. Of 63 published cases, 45 had no intra-arterial treatments and 18 had intra-arterial treatments. In the non-intra-arterial treatments group 24 had good outcomes. In the intra-arterial treatments group 13 had good outcomes. There was strong correlation between the NIHSS and the mRS. Children with basilar artery occlusion have better outcomes than adults. Certain children with basilar artery occlusion may be treated conservatively. A registry for childhood basilar artery occlusion is urgently needed.