Review
Medicine, General & Internal
Yazeed S. Alolayan, Kelly McKinley, Ritwik Bhatia, Ayham Alkhachroum
Summary: RSE is defined as continuous status epilepticus that persists despite administering at least two appropriately dosed parenteral medications, while SRSE is status epilepticus that persists at least 24 h after adding at least one appropriately dosed continuous anesthetic. Other therapeutic interventions include immunotherapy, neuromodulation, ketogenic diet, or even surgical intervention, with continuous EEG monitoring being essential for diagnosis and treatment.
JOURNAL OF CLINICAL MEDICINE
(2021)
Review
Clinical Neurology
Siew-Na Lim, Tony Wu, Wei-En Johnny Tseng, Hsing- Chiang, Mei-Yun Cheng, Wey-Ran Lin, Chia-Ni Lin
Summary: Perampanel shows effectiveness and satisfactory safety profile in the emergency treatment of established refractory and super-refractory status epilepticus. Patients with unique types and causes of SE, particularly nonconvulsive status epilepticus, may have a better response to perampanel treatment.
JOURNAL OF NEUROLOGY
(2021)
Article
Clinical Neurology
Simona Lattanzi, Giada Giovannini, Niccolo Orlandi, Francesco Brigo, Eugen Trinka, Stefano Meletti
Summary: This study aimed to evaluate whether differences in clinical outcomes exist according to treatments received and seizure activity resolution in patients with refractory status epilepticus (RSE). The study found that different types of RSE are associated with higher mortality rates and functional deterioration at discharge.
JOURNAL OF NEUROLOGY
(2023)
Article
Veterinary Sciences
Patrick Roynard, Ann Bilderback, Curtis Wells Dewey
Summary: Ketamine 5 mg/kg IV bolus may be successful in treating RSE in dogs, but its efficacy in handling CS is limited. The incidence of adverse effects is low, but timing of administration should be carefully considered.
FRONTIERS IN VETERINARY SCIENCE
(2021)
Review
Clinical Neurology
Juan G. Ochoa, Michelle Dougherty, Alex Papanastassiou, Barry Gidal, Ismail Mohamed, David G. Vossler
Summary: There is insufficient evidence to support the effectiveness of any antiseizure medications, inhalational anesthetics, ketogenic diet, acute vagus nerve stimulation, brain surgery, and therapeutic hypothermia for the treatment of super-refractory status epilepticus (SRSE). Data supporting the use of these treatments for SRSE are scarce and mainly based on small case series and case reports with confounding factors such as differences in patient populations and comedications.
Review
Biochemistry & Molecular Biology
Tzu-Hsin Huang, Ming-Chi Lai, Yu-Shiue Chen, Chin-Wei Huang
Summary: Status epilepticus (SE) is a neurological emergency with high mortality rate. The downregulation of GABAA receptors and upregulation of NMDA and AMPA receptors are the most widely accepted hypothesis underlying BZD resistance.
Article
Clinical Neurology
Esra Kochan Kizilkilic, Rumeysa Unkun, Ugur Uygunoglu, Sakir Delil, Cigdem Ozkara
Summary: COVID-19 infection can lead to neurological complications, including epileptic seizures and status epilepticus. IL-6 plays an important role in the severity of symptoms, and tocilizumab, as an IL-6 inhibitor, can be used for the treatment of these complications.
EUROPEAN JOURNAL OF NEUROLOGY
(2022)
Review
Clinical Neurology
Camilla Dyremose Cornwall, Thomas Kroigard, Joachim Sejr Skovbo Kristensen, Henriette Edemann Callesen, Christoph Patrick Beier
Summary: Super-refractory status epilepticus (SRSE) is a condition where status epilepticus (SE) continues or recurs after 24 hours or more of anesthesia, or on the reduction/withdrawal of anesthesia. This systematic review and meta-analysis assessed the clinical characteristics, causes, outcomes, prognostic factors, and treatment approaches for patients with SRSE. The study identified a total of 1200 patients with nonanoxic SRSE and found that long-term treatment was associated with lower mortality but increased risk of moderate to severe disability. Rating: 7/10.
Review
Clinical Neurology
Albert Aboseif, Kaitlyn Palmer, Aaron W. Abrams, Deepak Lachhwani, Elia Margarita Pestana Knight, Ahsan Moosa Naduvil Valappil, Andrew Zeft
Summary: Paraneoplastic neurological disorders in children are rare, and paraneoplastic cerebellar degeneration (PCD) is considered highly atypical. This case report describes a 13-year-old girl with progressive neurobehavioral regression, cerebellar ataxia, and intractable epilepsy, who was diagnosed with probable autoimmune encephalitis (AE). Surgical resection of an adrenal mass led to significant clinical improvement, and pathology revealed a benign ganglioneuroma. This report expands the understanding of paraneoplastic manifestations of ganglioneuroma and highlights important clinical considerations in the management of paraneoplastic neurological syndromes.
FRONTIERS IN NEUROLOGY
(2023)
Review
Clinical Neurology
Roberta Roberti, Morena Rocca, Luigi Francesco Iannone, Sara Gasparini, Angelo Pascarella, Sabrina Neri, Vittoria Cianci, Leonilda Bilo, Emilio Russo, Paola Quaresima, Umberto Aguglia, Costantino Di Carlo, Edoardo Ferlazzo
Summary: Status epilepticus (SE) in pregnancy is a life-threatening emergency for both mother and fetus. Literature data on SE management during pregnancy are limited. Prompt treatment and a multidisciplinary team are crucial. Benzodiazepines are the first-line drugs, followed by levetiracetam and phenytoin. Valproic acid should be used only as a last resort. Anesthetic drugs may be needed for refractory SE, with propofol and midazolam as preferred drugs. Magnesium sulfate is the first-line treatment for SE in eclampsia. Termination of pregnancy is recommended in case of failure of general anesthetics. Further research is needed to establish a standardized treatment protocol.
EXPERT REVIEW OF NEUROTHERAPEUTICS
(2022)
Article
Neurosciences
Julia E. Morgan, Sara C. Wilson, Benjamin J. Travis, Kathryn H. Bagri, Kathleen T. Pagarigan, Hannah M. Belski, Cecelia Jackson, Kevin M. Bounader, Jessica M. Coppola, Eden N. Hornung, James E. Johnson, Hilary S. McCarren
Summary: Nerve agent-induced status epilepticus poses challenges in treatment, with current guidelines showing limited effectiveness. Aggressive treatment with a three-line approach is necessary for positive outcomes in NA poisoning-related SE patients.
FRONTIERS IN NEUROSCIENCE
(2021)
Review
Clinical Neurology
Alicia Gonzalez-Martinez, Sonia Quintas, Nuria Redondo, Laura Casado-Fernandez, Jose Vivancos
Summary: This study reviewed the relationship between NCSE and sCJD, specifically focusing on cases where NCSE was the presenting symptom of sCJD. A case of an 82-year-old woman with sCJD initially diagnosed as NCSE was described, highlighting the importance of considering sCJD in the differential diagnosis of rapid cognitive decline with EEG changes consistent with NCSE. It was also suggested that immunotherapy may be beneficial in cases of NCSE with underlying autoimmune disorders like NORSE.
EUROPEAN JOURNAL OF NEUROLOGY
(2021)
Review
Clinical Neurology
Thahesh Tharmaraja, Jamie Sin Ying Ho, Aidan Neligan, Sanjeev Rajakulendran
Summary: New-onset refractory status epilepticus (NORSE) is a devastating neurological condition with limited research. This review aimed to summarize the etiology and establish the mortality rate of NORSE. The study analyzed relevant literature from January 2005 to April 2021 and found that cryptogenic and autoimmune causes were the most common etiologies of NORSE, with an overall mortality rate of 22%. Treatments included antiseizure medications, general anesthesia, and immunotherapy.
Article
Clinical Neurology
Marin Jacobwitz, Caitlyn Mulvihill, Michael C. Kaufman, Alexander K. Gonzalez, Karla Resendiz, Jennifer M. MacDonald, Conall Francoeur, Ingo Helbig, Alexis A. Topjian, Nicholas S. Abend
Summary: This retrospective study analyzed the use of ketamine infusion for refractory status epilepticus (RSE) in pediatric patients. The results showed that ketamine was effective in reducing seizure frequency and had few adverse events.
Article
Clinical Neurology
Rima Nabbout, Sara Matricardi, Paola De Liso, Olivier Dulac, Mehdi Oualha
Summary: This study retrospectively analyzed medical records of 16 children with super-refractory status epilepticus (SRSE) who received ketogenic diet (KD) treatment. The study found that KD can be effective in patients with NORSE, but it takes longer to achieve resolution of status epilepticus in cases with cryptogenic NORSE.
FRONTIERS IN NEUROLOGY
(2023)
Article
Clinical Neurology
Ani Gok, Seha Kamil Saygili, Sebuh Kurugoglu, Sema Saltik, Nur Canpolat
Summary: This study examines the occurrence of nephrolithiasis in children with type 1 SMA and identifies hypercalciuria and high urine specific gravity as common risk factors. The study suggests that children with SMA type 1 may have an increased risk of nephrolithiasis compared to the general population.
PEDIATRIC NEUROLOGY
(2024)
Article
Clinical Neurology
Glenn S. Rivera, Jesse A. Stokum, Janet Dean, Cristina L. Sadowsky, Allan J. Belzberg, Matthew J. Elrick
Summary: This study evaluated the surgical efficacy of nerve transfer surgery in patients with acute flaccid myelitis (AFM) and found that preoperative clinical and neurophysiological data play a valuable role in preoperative planning and patient selection. The postoperative outcomes demonstrated that nerve transfer surgery is an effective strategy to restore strength.
PEDIATRIC NEUROLOGY
(2024)
Review
Clinical Neurology
Tristan T. Sands, Jennifer N. Gelinas
Summary: This review explores the concept of epileptic encephalopathy (EE) in pediatric epilepsy, discussing the potential impact of epileptic activity on normal brain development and mechanisms underlying cognitive and behavioral impairments. The findings highlight the threat of epileptic activity to normal brain physiology and development.
PEDIATRIC NEUROLOGY
(2024)
Editorial Material
Clinical Neurology
Grace Gombolay, Melissa A. Walker
PEDIATRIC NEUROLOGY
(2024)
Article
Clinical Neurology
Salman Rashid, Monisha Goyal, Kathryn Lalor, Khaled Al-Robaidi, Vivek Shukla, Fazlur Rahman, Manimaran Ramani
Summary: This study investigated the seizure risk in asymptomatic preterm infants with high-grade intraventricular hemorrhage who underwent long-term video electroencephalographic monitoring. The findings showed that approximately 16% of these infants had seizures, and those with seizures were younger and more likely to be remonitored.
PEDIATRIC NEUROLOGY
(2024)
Letter
Clinical Neurology
Bernadeta Zabielska, Natalia Rzewuska, Sergiusz Jozwiak
PEDIATRIC NEUROLOGY
(2024)
Article
Clinical Neurology
Kelly C. Burk, Maki Kaneko, Catherine Quindipan, My H. Vu, Maritza Feliz Cepin, Jonathan D. Santoro, Michele Van Hirtum-Das, Deborah Holder, Gordana Raca
Summary: This study compared the diagnostic yields of chromosomal microarray (CMA) and epilepsy-genes sequencing (EGS) for genetic epilepsy etiologies. It found that EGS had a significantly higher diagnostic yield than CMA, especially for patients with infantile seizure onset and additional neurological findings.
PEDIATRIC NEUROLOGY
(2024)
Article
Clinical Neurology
Ursula Thome, Larissa A. Batista, Renata P. Rocha, Vera C. Terra, Ana Paula A. Hamad, Americo C. Sakamoto, Antonio C. Santos, Marcelo Santos, Helio R. Machado
Summary: Rasmussen encephalitis (RE) is a characterized by pharmacoresistant epilepsy and progressive neurological deficits. Surgical disconnection of the affected hemisphere is considered the treatment of choice, although the precise etiology is still unknown. This study reports the clinical, electrographic, and neuroimaging features of children and adolescents who underwent hemispheric surgery (HS) for the treatment of RE.
PEDIATRIC NEUROLOGY
(2024)
Article
Clinical Neurology
Michal Tzadok, Rotem Gur-Pollack, Hadar Florh, Yael Michaeli, Tal Gilboa, Mirit Lezinger, Eli Heyman, Veronika Chernuha, Irina Gudis, Andreea Nissenkorn, Tally Lerman-Sagie, Bruria Ben Zeev, Shimrit Uliel-Sibony
Summary: Purified CBD is well-tolerated and effective in reducing seizure frequency in children and young adults with drug-resistant epilepsy.
PEDIATRIC NEUROLOGY
(2024)
Editorial Material
Clinical Neurology
Zimeng Ye, Sufang Lin, Xia Zhao, Mathew Wallis, Xinyi Gao, Li Sun, Jiarui Wu, Jing Duan, Yi Yao, Lin Li, Li Chen, Dezhi Cao, Zhanqi Hu, Victor W. Zhang, Samuel F. Berkovic, Ingrid E. Scheffer, Jianxiang Liao, Michael S. Hildebrand
PEDIATRIC NEUROLOGY
(2024)
Article
Clinical Neurology
Nicolas J. Abreu, Madeline Chiujdea, Shanshan Liu, Bo Zhang, Sarah J. Spence
Summary: This study found a low completion rate of chromosomal microarray (CMA) testing for patients with autism spectrum disorder (ASD). Provider recommendation and more follow-up visits were associated with CMA completion. Age, intellectual disability or global developmental delay, first-degree relative with ASD, and public insurance were all related to CMA completion. Parental concern and cost/insurance coverage were the most frequently documented barriers.
PEDIATRIC NEUROLOGY
(2024)
Article
Clinical Neurology
Wafa Bouchaala, Sirine Laroussi, Yosra Mzid, Imen Maaloul, Olfa Jallouli, Salma Zouari, Sihem Ben Nsir, Zeineb Mnif, Fatma Kammoun, Chahnez Triki
Summary: Moyamoya angiopathy is a rare but serious cerebrovascular disease in children, which may result in arterial ischemic stroke. The clinical and radiological presentations can vary and are often overlooked. Early diagnosis and treatment are crucial.
PEDIATRIC NEUROLOGY
(2024)
Letter
Clinical Neurology
Eline M. C. Hamilton, Pinar Topaloglu, Jigyasha Sinha, Francesco Nicita, Genevieve Bernard, S. Ali Fatemi, Marjo S. van der Knaap
PEDIATRIC NEUROLOGY
(2024)
Article
Clinical Neurology
Muhamad Azamin Anuar, Muhammad'Adil Zainal Abidin, Soo Hong Tan, Cai Fong Yeap, Nor Azni Yahaya
Summary: This study conducted in Malaysia found that most infants with epilepsy can achieve seizure remission. However, poor seizure control and developmental delay are associated with abnormal EEG background and neuroimaging abnormalities.
PEDIATRIC NEUROLOGY
(2024)