Minimally Invasive Epilepsy Surgery.

Surgery remains a critical and often necessary intervention for a subset of patients with epilepsy. The overarching objective of surgical treatment has consistently been to enhance the quality of life for these individuals, either by achieving seizure freedom or by eliminating debilitating seizure types. This review specifically examines minimally invasive surgical approaches for epilepsy. Contemporary advancements have introduced a range of treatments that offer increased safety and efficacy compared to traditional open resective epilepsy surgeries. This manuscript provides a comprehensive review of these techniques and technologies.

Portable Headband Electroencephalogram in the Detection of Absence Epilepsy.

The accuracy of headband electroencephalogram (EEG) was compared to traditional EEG in pediatric patients with absence epilepsy. This study enrolled 10 patients with previously diagnosed absence epilepsy and examined the concordance of headband EEG and traditional EEG in the follow-up EEG of treated absence epilepsy. The study found a concordant result in 80% of cases providing a signal that absence epilepsy is an effective target for headband EEG. The study showcases a need for further research in headband EEG technology and continued improvements in technology.

Radiologic Classification of Hippocampal Sclerosis in Epilepsy.

Temporal lobe epilepsy is a common form of epilepsy that is often associated with hippocampal sclerosis (HS). Although HS is commonly considered a binary assessment in radiologic evaluation, it is known that histopathologic changes occur in distinct clusters. Some subtypes of HS only affect certain subfields, resulting in minimal changes to the overall volume of the hippocampus. This is likely a major reason why whole hippocampal volumetrics have underperformed versus expert readers in the diagnosis of HS. With recent advancements in MRI technology, it is now possible to characterize the substructure of the hippocampus more accurately. However, this is not consistently addressed in radiographic evaluations. The histologic subtype of HS is critical for prognosis and treatment decision-making, necessitating improved radiologic classification of HS. The International League Against Epilepsy (ILAE) has issued a consensus classification scheme for subtyping HS histopathologic changes. This review aims to explore how the ILAE subtypes of HS correlate with radiographic findings, introduce a grading system that integrates radiologic and pathologic reporting in HS, and outline an approach to detecting HS subtypes by using MRI. This framework will not only benefit current clinical evaluations, but also enhance future studies involving high-resolution MRI in temporal lobe epilepsy.

Pediatric epilepsy syndromes with associated developmental impairment.

In 2022, the International League Against Epilepsy revised their classification of epilepsy syndromes for clinicians to better understand the relationships between different epilepsy syndromes, their underlying causes, and their associated developmental and behavioral features. This review highlights portions of the current classification with an emphasis on epilepsy syndromes that readily present with developmental challenges and provides a unique framework, based on electroencephalography, to easily identify and understand these syndromes. Included in this review are a helpful categorization scheme with visual aid, descriptions of updated epilepsy syndromes, figures of relevant identifiers of syndrome and information regarding future directions toward treatment and research. Covered syndromes include developmental and epileptic encephalopathy, Dravet syndrome, Rasmussen syndrome, and infantile epileptic spasm syndrome, among others. WHAT THIS PAPER ADDS: The revised epilepsy syndrome classification by the International League Against Epilepsy aims to improve the outcomes for children with epilepsy. The electroencephalography features of epilepsy syndromes are grouped based on a categorization model. This model allows clinicians to understand overlapping phenotypes and aid with both identification and diagnosis.

Residency Training: A Practical Guide for Medical Students who are Planning a Future in Child Neurology.

At the time of graduation from medical school, medical students have been exposed primarily to adult neurology and have limited exposure to child neurology. Child neurology is a unique field that encompasses caring for children with neurological conditions ranging from routine to rare. There are many opportunities for a variety of unique careers in child neurology including both in the inpatient and outpatient setting. This article aims to provide practical advice for the medical student interested in child neurology to best prepare for a successful match and rewarding career.

When in doubt, return to fundamentals (seizure semiology).

This case of drug resistant focal epilepsy highlights several invaluable teaching points. Hypotheses grounded in seizure semiology provide the best framework to ensure accurate interpretation of diagnostic testing. Without a hypothesis, information gathered from tests can be difficult to linearly piece together and can lead to poor patient outcomes. The case also provides a real-world conundrum of discordant test results that were ultimately rectified by re-visiting the initial hypothesis and cross-testing. Perinatal stroke continues to be a common etiology of drug resistant epilepsy. Patients can achieve seizure freedom and good quality of life with appropriate workup and in appropriate cases, surgery.

Prevalence of hearing protection use and subjective auditory symptoms among Spanish classical orchestral musicians.

INTRODUCTION: Sensorineural hearing loss caused by exposure to noise is one of the most frequent causes of deafness. Professional musicians have significant occupational exposure to high levels of noise. Use of hearing protection among musicians could substantially prevent hearing damage, though the rate of use is quite low. METHODS AND MATERIAL: A questionnaire on the use of protective hearing devices, hearing care, and subjective judgments of hearing difficulties was completed by a group of classical musicians from Spain. We analysed the frequency of device use by instrument based on contingency tables analysed by χ2 tests. RESULTS: One hundred and ninety-four Spanish classical orchestral musicians voluntarily completed the questionnaire. The percentage of musicians who reported using hearing protection in our survey was very low and varied with the type of instrument played. However, we found a high prevalence of subjective auditory disorders within this group. CONCLUSION: Few Spanish musicians use hearing protection. Training on hearing-loss prevention in this field and better protective devices could increase device use and improve the auditory health of this group.

The devil is in the details: Understanding how misinformation regarding epilepsy manifests in TikTok videos.

OBJECTIVE: This study sought to understand the amount of misinformation surrounding epilepsy on the social media platform, TikTok. METHODS: Videos from TikTok were searched using keyword "epilepsy." In all, 109 videos were categorized as personal experience videos, event videos, or educational videos. Misinformation surrounding each video was analyzed by two independent reviewers. The number of videos and views of each were tracked. RESULTS: Event videos garnered the most attention (80% of total views). Of the event videos, 26/47 contained misinformation in the form of calling a nonepileptic event an epileptic seizure (55%). Videos of nonepileptic events labeled as epileptic seizures amassed 109 956 400 views. SIGNIFICANCE: This novel study quantifies the amount of misinformation regarding epilepsy on the social media platform, TikTok. The results showcase that misinformation is very prevalent on the application. Most of the misinformation consists of the mislabeling of nonepileptic events as epileptic seizures. Physicians should be aware of cardinal features of nonepileptic events to better treat patients. The medical community must understand the type of misinformation people are exposed to in order to educate patients appropriately.

Core Features Differentiate Dravet Syndrome from Febrile Seizures.

An 11-month-old girl with febrile seizures and first unprovoked seizures was evaluated in the hospital. Relevant history included developmental delay and strong family history of febrile seizures and migraines. A routine electroencephalogram was performed and was abnormal due to the presence of a slowed posterior dominant rhythm, generalized spike-wave discharges, and multifocal sharp waves. The findings were concerning for a developmental and epileptic encephalopathy. Given the concern for a developmental and epileptic encephalopathy, a next generation sequence epilepsy gene panel was ordered which identified a pathogenic variant in SCN1A. The clinical history, electroencephalogram, and pathogenic variant were compatible with a diagnosis of Dravet syndrome. This Grand Rounds manuscript highlights the thought process, evaluation, differential diagnosis, treatment, and prognosis in Dravet syndrome.

Validation of a Spanish chronic obstructive sialadenitis quality of life questionnaire (CSOC).

BACKGROUND AND OBJECTIVES: Interest in chronic obstructive sialadenitis is increasing with the introduction of sialendoscopy. A self-administered instrument to assess quality of life in patients with chronic obstructive sialadenitis is needed to improve clinical management and support research. The objectives of this study are to design a Spanish questionnaire to assess quality of life in chronic obstructive sialadenitis, named CSOC and assess its reliability, validity and feasibility. MATERIAL AND METHODS: A prospective, multicentre, observational study was conducted. Patients with diagnosis of chronic obstructive sialadenitis were included in the study. The item generation process included a review of published data as well as interviews with patients. An expert panel then tested the content validity of the instrument, and the construct validity was tested in 120 patients and 100 controls. Patients completed a self-administered CSOC questionnaire, a Short Form-36 and a Visual Analogue Scale. Feasibility, reliability, internal consistency, construct validity and responsiveness were assessed. RESULTS: All the patients found the instrument understandable. Cronbach α coefficient was high (0.85). The time required to fill out was 5.7 and 4.5min for pre and postsialendoscopy CSOC respectively. Cronbach α coefficient was very high for both pre and postsialendoscopy CSOC (0.90 and 0.94 respectively). The correlation with the SF-36 dimensions was negative and positive with the VAS. The mean score of CSOC was 28.63 and 8.33 for pre and postsialendoscopy. In the control group the mean score of CSOC was 1.31. CONCLUSIONS: The CSOC questionnaire is understandable, feasible, reliable and representative of quality of life in chronic obstructive sialadenitis.

A Favorable Treatment Outcome in RANBP2 and Influenza Associated Acute Necrotizing Encephalitis.

In current literature, there is uncertainty in the pathophysiology and management of influenza-associated Acute Necrotizing Encephalitis. Because of this and the rarity of the disease, no clear treatment guidelines exist. It is thought that treatment after 24 h of symptom onset or known brainstem involvement are poor predictors of outcome. Here, we present a case that provides support for aggressive management of the inflammatory cascade with combination high-dose steroid, immunoglobulin, and anti-viral therapy with oseltamivir despite initiation after 24 h from symptom onset, brainstem involvement, and a pathogenic RANBP2 gene mutation which mechanistically increases oxidative stress, cytokine effects, and possibly viral invasion into brain tissue and vasculature.

A Novel Mutation in Lafora Disease and Update on Pathophysiology and Future Treatments.

Lafora disease is a rare refractory epilepsy that results in death. This report highlights two cases of lafora disease and introduces a novel mutation in the patients. A review of the pathophysiology and future therapies is reviewed.

Sialolithiasis: mineralogical composition, crystalline structure, calculus site, and epidemiological features.

The purpose of this paper was to describe the characteristics of salivary calculi and their relationship to epidemiological factors, through a cross-sectional study. We analysed 100 calculi obtained in 2017-2021. Patient data including age, time since onset of symptoms, gland involved, and site of location in the salivary system were studied. The calculi were studied to determine their morphological features using scanning electron microscopy and energy dispersive plain radiographic analysis. Most of the calculi had formed in the submandibular gland (SG) (82%). The mean age of patients at onset was 45.83 years; patients presenting parotid gland (PG) stones were somewhat older (p = 0.031). The mean time since the onset of symptoms was longer in PG calculi (p = 0.038). The most common lithiasis site was the main duct (74%), followed by the hilum (22%). Hilar stones were the largest (p < 0.05) and heaviest (p = 0.028). Octacalcium phosphate (OCP) was the most common crystalline phase (Cp) founded, followed by hydroxyapatite (HA) and whitlockite (WH). Specifically, OCP had a higher presence in PG calculi (p = 0.029) and WH was the most common phase in SG calculi (p = 0.017). The most prevalent site of lithiasis was the main duct, and the largest and heaviest calculi were found in the SG. PG stones were associated with a longer history of symptoms and older age. OCP was the most frequent Cp of the calculi studied, and the main Cp in PG stones. WH was the predominant Cp in SG stones. The Cp of the calculi was not influenced by location, patient age, or time of symptoms.

Epidemiologic, radiologic, and sialendoscopic aspects in chronic obstructive sialadenitis.

PURPOSE: Chronic obstructive sialadenitis (COS) is a recurring inflammation of the salivary gland. To date, there are no known predisposing factors for COS. Given the advances seen in radiology and sialendoscopy, we must update our knowledge of COS, analyzing factors that can favor its development. METHODS: We prospectively analyzed 333 patients who underwent sialendoscopy between 2012 and 2021. Epidemiologic, radiologic, and sialendoscopy-related factors were correlated. Suspected diagnosis was established based on the clinical and radiologic data. The final diagnosis was determined on the basis of sialendoscopic findings. RESULTS: The most common etiology of COS was stricture (40.8%). Lack of papilla distensibility (LPD) was also described as an etiology. COS was related to patient gender and age. Submandibular gland involvement was significantly more associated with lithiasis and LPD, while COS of the parotid gland was most frequently caused by stricture. Radioiodine sialadenitis and Sjögren's syndrome were significantly associated with stricture. MR sialography (MR-Si) showed the best overall sensitivity and specificity. CONCLUSION: In our series, stricture was the most common cause of COS. We describe LPD as a frequent cause of COS in this series; ours is the first study to report this finding. There was a significant association between the salivary gland involved, patient sex and age, and the cause of COS. MR-Si showed the greatest diagnostic yield.

Super-refractory status epilepticus during blinatumomab initiation for B-cell acute lymphoblastic leukemia.

Seizures have been reported as an adverse effect of blinatumomab, a bispecific T-cell engager monoclonal antibody, which is mainly used for the treatment of pediatric relapsed/refractory leukemia. Here, we present the first reported case of super-refractory status epilepticus in an 11-year-old boy with B-cell acute lymphoblastic leukemia (B-ALL) while receiving blinatumomab. Our patient had a complete return to baseline despite enduring encephalopathy, refractory subclinical seizures requiring prolonged therapeutic burst suppression and MRI signal changes. This case demonstrates that super-refractory status epilepticus is a possible neurotoxic adverse effect of blinatumomab treatment, which responds well to conventional protocols for acute refractory seizures.

Seizures are a known side effect of blinatumomab, a relatively new immunotherapy drug, which is mainly used for the treatment of relapsed leukemia in children. Here, we present the first reported case of seizure continuing for more than 24 h despite appropriate antiseizure treatment while also receiving blinatumomab. Despite an extended period of altered mental status, new abnormalities on imaging of the brain and a medication-induced coma to treat unrelenting seizures, our patient returned completely to his healthy brain function. This case demonstrates that seizures, which are especially difficult to treat, can be associated with blinatumomab immunotherapy for pediatric refractory B-ALL; however, standard-tiered seizure treatments can be effective.

Infantile Hypotonia: A Case of Spinal Muscular Atrophy With Respiratory Distress Type 1 Presenting As Infant Botulism.

Spinal muscular atrophy with respiratory distress type 1 (SMARD 1) is a rare autosomal recessive disease characterized by distal muscular atrophy and respiratory distress. It presents between six weeks and six months of age, with an eventual requirement of respiratory support. To date, no curative treatment to attenuate or stop the clinical deterioration has been found; therefore, supportive treatment is the corner stone of management. We report a 12-week-old infant with SMARD1 initially diagnosed and managed as a case of infant botulism secondary to a history of significant exposure to honey. SMARD1 and infant botulism all share characteristic clinical features, namely, respiratory distress, hypotonia, and autonomic dysfunction with typical onset of less than one year of age. This case report illustrates that SMARD1, SMA Type 1, and infant botulism share common clinical features. It is important to maintain a broad differential when evaluating an infant with hypotonia, especially when there is a lack of clinical response to conventional medical interventions directed toward the working diagnosis.