Asra Akbar

Background: Chronic daily headaches are the cause of significant morbidity in adolescents. Many patients with migraine show minimal response to daily preventive medications and often have significant side effects. Calcitonin gene–related peptide (CGRP) inhibitors have been found to be effective for managing migraines in the adult population. However, limited data are available for their use in pediatrics. Methods: Single-center retrospective case series of all consecutive patients (<18 years) treated at the outpatient pediatric neurology clinic at Children's Hospital of Illinois/Illinois Neurological Institute between 2020 and 2023. Data extracted from the electronic medical record included demographics, comorbidities, headache characteristics, including the Headache Impact Test–6 (HIT-6) score, and Pediatric Migraine Disability Assessment (PedMIDAS) score. The study was approved by the Institutional Review Board, and informed consent from all patients was obtained. Results: Details of 8 patients treated with CGRP inhibitors are provided. Median age of the cohort was 17.2 years, and 62.5% were female. The median number of 6.5 medications were tried before CGRP inhibitors. Median Headache Impact Test–6 and Pediatric Migraine Disability Assessment scores were 76 and 40, respectively, and they decreased to a median of 36 and 5, by the last reported visit of each patient. All patients responded to the therapy, with 7 of 8 responding within 3 months (50% decrease of MIDAS score). One patient was co-treated with Botox injections and also with as-needed use of CGRP receptor blockers. No significant adverse events were reported by any patient. Conclusion: Once a month, anti-CGRP injections are effective and well tolerated in the pediatric population. CGRP inhibitor therapy may be a reasonable therapeutic option for managing chronic daily headaches in patients who have failed other routine prophylactic medication management.

Morbid obesity is a systemic disease which can result in chronic complications, including hypertension, diabetes mellitus, depression, osteoarthritis and low self-esteem in the adolescent population. Bariatric surgery can be indicated to treat more severe forms of obesity, but these procedures are not without long-term risks. Therefore, adequate preoperative and postoperative care, which includes preoperative psychosocial evaluation for compliance, ongoing nutrition counselling and vitamin and micronutrient supplementation, is required for all patients, especially adolescent patients, who generally may not comply with medical therapies and/or be able to developmentally fully appreciate or comprehend the health consequences of their behaviours, prior to as well as after bariatric surgery to prevent complications. Thiamine pyrophosphate, an active form of thiamine (also known as vitamin B1, a water-soluble vitamin), which functions as a coenzyme in glucose and energy metabolism, is one such vitamin that requires supplementation postoperatively. It is mandatory for glucose to be administered concomitantly with thiamine, as glucose alone can precipitate Wernicke’s encephalopathy (WE) in thiamine-deficient individuals. WE is a medical emergency, with a mortality rate of up to 20%. WE is best understood as a classic triad of mental confusion, gait ataxia and eye movement abnormalities, and atypical WE or Wernicke’s syndrome (WS) is seen when the classic triad is not present. Cases that meet some, but do not necessarily meet all three criteria, are referred to as atypical WE or WS which can lead to delayed diagnosis. Atypical WE has an incidence of 19% which can lead to misdiagnosis of a preventable medical emergency with fatal complications. The following case reviews the consequences of post-bariatric thiamine supplementation therapy non-adherence and resulting in a deficiency in an adolescent patient.

Acute ischemic stroke is an uncommon presentation in the pediatric population as compared to the elderly population. COVID-19 infection is associated with several neurological manifestations, with ischemic strokes being underrecognized. Cerebrovascular events associated with COVID-19 may be due to systemic inflammation and hypercoagulable state. Neurofibromatosis type 1 (NF1) is an inherited multisystem disorder caused by dominant loss-of-function mutations of the tumor-suppressor gene neurofibromin 1, which is located at 17q11.2.1. NF1 is associated with multiple cerebrovascular abnormalities, including internal carotid artery occlusion. A review of the current literature on manifestations of COVID-19 in the pediatric population, including stroke and seizures, is also provided in this case report. A brief review of the literature on neurofibromatosis and the risk of stroke as well as other clinical manifestations is also included as a part of this case report. This case illustrates the importance of recognizing acute and rare complications of neurofibromatosis. Cerebral vasculopathy is an important but underrecognized complication of NF1. Children with neurofibromatosis and hypertension require a thorough and complete neurologic evaluation. This case describes a young infant with a delayed clinical diagnosis of NF1 who was presented with viral manifestations of COVID-19 infection and was diagnosed with a large middle cerebral artery stroke.

Background: Chronic migraine in children has been a challenging condition to treat, prompting the investigation of alternative therapies. This retrospective single-center chart review aimed to evaluate the efficacy and safety of Botox injections for managing chronic migraine in children. Methods: The study included children with chronic daily headaches and chronic migraine who were medically refractory to previous treatments at OSF Healthcare/Illinois Neurological Institute, Peoria, between 2015 and 2021. Botox injections were administered quarterly following a specific protocol. Data were obtained from electronic medical records by manual review. Results: Twenty-four patients met the inclusion criteria (median age 15.4 years, 87% female). Comorbidities included depression (41.6%) and sleep disturbances (33.2%). Prior to Botox treatment, patients had been tried on a median number of 5 (interquartile range [IQR] 4, 7) medications. Botox injections resulted in a significant reduction in headache frequency, with a mean difference (6 months vs pretreatment) in the Headache Impact Test (HIT 3) scores of −19.6 (95% CI −24.8, −14.3), P < .001, and mean difference in the Migraine Disability Assessment (MIDAS) scores of −50.8 (95% CI −62.6, −39.0, P < .001). Subjective improvements included mood enhancement (13/24, 54.2%) and improved concentration (12/24, 50%). Treatment-related side effects were reported by 5/24 (20%) of patients and were mostly mild to moderate. Conclusions: Botox injections offer a promising therapeutic option for managing chronic migraine in children who have not responded to traditional medications. Future controlled trials and long-term follow-up studies are needed to further evaluate Botox treatment's benefits and adverse effects in children with chronic migraine.

Loeys-Dietz syndrome (LDS) is a rare, autosomal dominant multisystem disorder that is caused by mutations of transforming growth factor-β receptors. Mutations in SMAD3 and TGFB3 have been recently reported. LDS is characterised by the triad of arterial tortuosity, hypertelorism and a bifid uvula or cleft palate among other cardiovascular, craniofacial and orthopaedic manifestations. Patients with LDS show clinical and genetic variability and there is a significant risk of reduced life expectancy due to widespread arterial involvement, aortic root dilation, aneurysms and an aggressive vascular course. Thus early genetic testing is warranted if clinical signs and history are suggestive of this potentially catastrophic disorder. LDS predisposes patients to aortic aneurysms and early death due to vascular malformations, but neurological emergencies, such as seizures and febrile status epilepticus, have not been reported. Febrile status epilepticus is the most common neurological emergency in childhood. Neurological manifestations of COVID-19 in the paediatric population are not as well described in medical literature. To the best of our knowledge, this is the first reported case of febrile status epilepticus with COVID-19 infection in an infant with LDS. Our patient had focal epileptiform activity emanating over the left posterior hemisphere, which evolved into an electrographic seizure on video EEG. Such patients have a heightened risk of epilepsy in the future, and this occurrence is consistent with a diagnosis of focal epilepsy. Neurological complications such as epilepsy and status epilepticus in a patient with LDS have never been reported before. A brief review of literature is also given here.

Neurological manifestations of COVID-19 in the pediatric population are not as well described as those in the adult population. We describe a case of myelin oligodendrocyte glycoprotein immunoglobulin G (MOG-IgG)–associated disorder in a 9-year-old girl, who experienced complete recovery. This rare disorder is a demyelinating disease that often relapses and has the potential to cause severe morbidity. The case highlights the need for early recognition of asymptomatic and subacute presentations of demyelinating disorders and testing for MOG-IgG antibodies, as the management of presumed monophasic demyelinating disorders vs MOG-IgG–positive demyelinating disorder is different.

Status epilepticus (SE) is a life-threatening medical emergency which is frequently encountered in the critical care setting and can be refractory to treatment. Refractory status epilepticus (RSE) is defined as SE that has failed to respond to adequately used first-line and second-line antiepileptic medications. Super refractory status epilepticus is defined as SE that persists for 24 hours or more after the use of an anaesthetic agent or recurs after its withdrawal. If SE persists beyond a period of 7 days it is referred to as prolonged, refractory status epilepticus (PRSE). There are limited data guiding treatment of RSE in the paediatric population. Lacosamide (LCM) is licensed as an adjunctive treatment for partial-onset seizures. Evidence for the efficacy of LCM in paediatric SE is scarce. This case report may suggest a synergistic effect of LCM on slow-activation sodium channels in conjunction with medications such as phenytoin that causes fast inactivation of sodium channels. The dual fast and slow inactivation of sodium channels may enhance the effectiveness in treatment of RSE. This is the first case report of PRSE in an infant, successfully treated with LCM. A brief review of literature is also a part of this report.

Coronavirus disease 2019 (COVID-19) infection is caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). This infection usually presents with upper respiratory symptoms; however, it can also present with a wide variety of other multisystem and neurological symptoms, including seizures. There are several proposed mechanisms by which COVID-19 can cause systemic signs of infections, including neurological complications and seizures. This case report describes a pediatric patient without a previously documented history of epilepsy who was admitted for new-onset focal seizures with impaired consciousness. No other cause and triggers of seizures were found, and the child was tested positive for COVID-19 infection. The patient had six electroclinical seizures during EEG. Video EEG findings showed atypical features of onset of intermittent rhythmic delta activity (IRDA) slowing over the left hemisphere with evolution/generalization of rhythmic delta/theta activity and without clear typical generalized epileptiform discharges. These EEG findings correlated with a clinical change of behavior arrest, staring, and yawning. Similar spells were reported multiple times a day prior to the admission, and past EEG was normal. A review of current literature on COVID-19 and neurological manifestations in children, including new seizures and prior diagnosis of epilepsy, is also provided in this case report. The clinical experience in children with newly diagnosed or chronic epilepsy suggests that exacerbation of seizures, especially from systemic effects such as those caused by severe COVID-19 infection, will be a major concern.

Mild encephalopathy with reversible splenial lesion (MERS) is a disorder characterised by prodromal symptoms such as fever, cough, vomiting or diarrhoea, followed by mild encephalopathy 1–7 days later with a documented reversible corpus callosum splenial lesion. MERS is mostly reported in children in the region of East Asia. To the best of our knowledge, this is the first reported case of MERS associated with mycoplasma infection in a child in North America. A brief review of literature is also a part of this report.

We report a 41-year-old woman who developed histology- and colonoscopy-proven ischemic colitis with the use of naratriptan not exceeding the maximum 2 doses a day and 3 days per week and without a known medical or cardiovascular history. By exclusion of other causes of colonic ischemia, naratriptan was considered the sole causal agent. Discontinuation of naratriptan resulted in a complete clinical recovery. To date, our patient is the youngest known patient to develop ischemic colitis on isolated naratriptan in the setting of no known medical risk factors or predisposing medical condition. Even though triptans are commonly used for the abortive treatment of migraine headaches, such a reported side effect is rare; however, careful assessment and individual patient-based treatment is advised.