Slovo úvodem

prof. MUDr. Ivan Rektor, CSc., FCMA, FANA, FEAN

Neurol. praxi. 2024;25(3):167  

Status epilepticus - úvod

prof. MUDr. Petr Marusič, Ph.D., MUDr. David Krýsl, Ph.D.

Neurol. praxi. 2024;25(3):173-174  

Convulsive status epilepticus and its early treatment

doc. MUDr. Irena Doležalová, Ph.D., prof. MUDr. Petr Marusič, Ph.D.

Neurol. praxi. 2024;25(3):177-181 | DOI: 10.36290/neu.2024.038  

Convulsive status epilepticus is the form of a tonic-clonic seizure lasting longer than 5 minutes, or in the case of repeated tonic-clonic seizures within this time period. Initially is treated with benzodiazepines - diazepam i. v., midazolam i. m. or buccal, or diazepam administered rectally. If benzodiazepine therapy fails, intravenous administration of anti-seizure drugs is indicated - levetiracetam at 60 mg/kg (maximum 4500 mg), valproate at 40 mg/kg (maximum 3000 mg), or phenytoin at 20 mg/kg (maximum 1500 mg). In this article, we present maintenance doses of the drugs and discuss their important pharmacological properties and possible adverse...

Nonconvulsive status epilepticus

MUDr. David Krýsl, Ph.D., MUDr. Hana Krijtová, prof. MUDr. Petr Marusič, Ph.D.

Neurol. praxi. 2024;25(3):182-191 | DOI: 10.36290/neu.2024.022  

Non-convulsive status epilepticus (NCSE) is an important cause of morbidity and mortality, especially in patients with acute cerebral insults and those in need of neurointensive care. NCSE is a clinically heterogeneous group of conditions and the clinical manifestations, EEG findings, treatment and prognosis vary widely according to the clinical context in which NCSE occurs (age, presence of epilepsy, comorbidities, comedication, presence of an acute structural brain lesion or acute systemic disorder). EEG is essential for the diagnosis of NCSE. However, especially in patients with acute symptomatic NCSE, a wide variety of EEG findings occur that do...

Refractory and super­‑refractory status epilepticus

doc. MUDr. Zdeněk Vojtěch, Ph.D., MBA, MUDr. Michaela Nová

Neurol. praxi. 2024;25(3):192-196 | DOI: 10.36290/neu.2024.021  

In this article, we give definitions of refractory and super-refractory status epilepticus (SE). Evidence-based or consensual treatment strategies are not known even for refractory SE. In super-refractory SE, the treatment options are even more ambiguous. Therefore, we also present the results of methods published in small case series or case reports. Potentially, this information may contribute to the development of in­‑house treatment protocols.

New­‑onset refractory status epilepticus

MUDr. Michaela Nová, doc. MUDr. Zdeněk Vojtěch, Ph.D., MBA

Neurol. praxi. 2024;25(3):198-202 | DOI: 10.36290/neu.2024.026  

New-onset refraktory status epilepticus (NORSE) is a rare severe condition with possible fatal consequences. Etiology of the syndrome often remains unclear. The term NORSE describes the clinical condition according to patient's symptoms and history but has no connection with a specific etiology. Achievement of the control of seizures is our main priority in management of every status epilepticus (SE), however finding the etiology of SE has both therapeutic and prognostic implications. Aim of this article is to suggest diagnostic and therapeutic processes based on the considered/confirmed underlying cause of NORSE.

Specifics of status epilepticus in children

MUDr. Ondřej Horák, MUDr. Klára Brožová, MUDr. Martin Kudr, Ph.D.

Neurol. praxi. 2024;25(3):203-208 | DOI: 10.36290/neu.2024.031  

In comparison to adults, status epilepticus (SE) in childhood is characterized by a higher incidence, different causes, and a lower mortality. Most cases of SE in children occur without a prior history of epileptic seizures. The etiology of SE in children is distinct, with febrile SE being the most common in this age group. Specific to childhood are SE cases in certain epileptic syndromes, such as Dravet syndrome and Lennox-Gastaut syndrome. The treatment of SE in children employs similar algorithms as in adults, but with certain specifics, such as the use of vitamin B6 and a preference for midazolam or thiopental over propofol in younger patients....

Emerging recommended practices for the diagnosis and treatment of Alzheimer's disease and other cognitive disorders: an interdisciplinary consensus

prof. MUDr. Robert Rusina, Ph.D., MUDr. Vanda Franková, prof. MUDr. Aleš Bartoš, Ph.D., doc. MUDr. Svatopluk Býma, CSc., MUDr. Ing. Klára Knápková, MUDr. Astrid Matějková, MUDr. Zuzana Šnajdrová, MUDr. Hana Vaňková, Ph.D.

Neurol. praxi. 2024;25(3):210-214 | DOI: 10.36290/neu.2024.029  

Care for patients with Alzheimer's disease (AD) and other cognitive disorders has been fragmented in the Czech Republic and unevenly and inadequately accessible in different regions. Neurologists, psychiatrists, geriatricians and general practitioners are involved in treatment. The aim of the implementation of the National Action Plan for AD and Similar Diseases 2020-2030 (NAPAN), approved by the Government of the Czech Republic, is to establish a single recommended approach for the diagnosis and treatment of patients with AD and similar diseases, so that the patient always receives good comprehensive care, regardless of the consulted specialist and...

Dravet syndrome in adult population

doc. MUDr. Irena Doležalová, Ph.D., prof. MUDr. Milan Brázdil, Ph.D.

Neurol. praxi. 2024;25(3):216-223 | DOI: 10.36290/neu.2024.043  

Dravet syndrome (DS) is predominantly caused by a mutation in the sodium channel subunit (SCN1A). It begins in childhood but is a lifelong condition. In this article, we focus on the characteristics of adult patients with DS. From an epileptological perspective, the occurrence of epileptic seizures is crucial. In adulthood, there are mainly clonic seizures (generalized tonic-clonic seizures, tonic seizures or focal seizures to bilateral tonic-clonic seizures). They can be provoked by increased body temperature or by emotions. However, parents/caregivers of patients with DS often report other manifestations of the disease as very significant, often...

New treatment options for Neuromyelitis optica spectrum disorders (NMOSD)

prof. MUDr. Jarmila Szilasiová, PhD.

Neurol. praxi. 2024;25(3):224-229 | DOI: 10.36290/neu.2024.025  

The article provides an overview of the current state of knowledge about neuromyelitis optica spectrum disorder (NMOSD) immunotherapy. Therapy focuses on the relapses that determine disability in NMOSD. Autoantibodies against aquaporin 4 (AQP4-IgG) are a diagnostic biomarker of the disease and have an important role in the pathogenesis of damage to astrocytes. Recent advances in the understanding of NMOSD have led to the development of new therapies and validation of their effectiveness in randomized controlled trials. New immunotherapies have been approved for patients with positive AQP4-igG antibodies, with the potential to reduce the number of relapses,...

Spastic paraparesis as a clinical manifestation of severe X-linked adrenoleukodystrophy

RNDr. Anna Uhrová Mészárosová, Ph.D., MUDr. Jana Laštůvková, MUDr. Filip Cihlář, Ph.D., doc. MUDr. Alena Šantavá, CSc., MUDr. Radim Mazanec, Ph.D.

Neurol. praxi. 2024;25(3):231-238 | DOI: 10.36290/neu.2023.037  

Pathogenic variants in the ABCD1 gene are the genetic cause of X-linked adrenoleukodystrophy (X-ALD). X-ALD can manifest as severe fatal adrenoleukodystrophy (ALD), milder adrenomyeloneuropathy (AMN), or adrenal insufficiency (Addison's disease), but it can present clinically only as mild spastic paraparesis, particularly in female carriers. During several years of genetically diagnosing hereditary spastic paraparesis (HSP), four Czech families were diagnosed with a pathogenic variant in the ABCD1 gene when one of the family members was referred for testing of genes associated with hereditary spastic paraparesis that was the only clinical...

Adherence to treatment in multiple sclerosis: case reports of three female patients on high-efficacy therapy with ocrelizumab

MUDr. Jana Pavlíčková, Ph.D.

Neurol. praxi. 2024;25(3):240-245 | DOI: 10.36290/neu.2024.019  

Multiple sclerosis (MS) is a chronic and incurable autoimmune disease of the central nervous system (CNS). The goals of disease-modifying therapy (DMT) are to slow down disease progression, prevent relapses, and increase the overall quality of life of the patient. Adherence refers to the degree to which a patient complies with prescribed treatment and as such is required for the treatment to be effective. Non-adherence is associated with a risk of disability progression and increased healthcare costs. The aim of the article is to present three case reports of female patients on high-efficacy therapy (HET). Patient 1 has been treated with HET since...