2018, Vol 18, No 3
Custom-made database enabling a quick search for patients fulfilling specific criteria. A simple and effective tool for clinical data collection for scientific purposes
Aktualn Neurol 2018, 18 (3), p. 113–116
DOI: 10.15557/AN.2018.0015

Introduction: Every evidence-based scientific work requires precise and reliable data. In clinical medicine, this means searching for patients fulfilling specific criteria. Unfortunately, most hospital-operated information systems are designed to process data concerning treatment of specific patients, mainly in its medical and administrative aspects, while a crosssectional search for patients with a specific pathology at a specific location is difficult if not impossible. Therefore, most of these databases are essentially useless for scientific purposes. Material and method: Based on medical records of all patients treated at our facility, we have created a simple database enabling a quick search for patients fulfilling specific criteria. We used Microsoft® Office Access® software installed on a standard PC. Structure and the use of database are described in detail. Results: Our database contains basic information about 16,126 patients treated over 38 years and occupies 5.19 MB. To date, 48 queries have been performed, providing reliable scientific material. Conclusion: This is an effective and comprehensive tool for the search of patients or items fulfilling specific criteria. To the best of our knowledge, such a tool for the management of clinical data has not been described to-date. After introducing appropriate modifications, a similar database will be very useful in every clinical centre.

Keywords: medical records, database, search criteria
An introduction to gender diff erences in neurological treatment
Aktualn Neurol 2018, 18 (3), p. 117–122
DOI: 10.15557/AN.2018.0016

As in other medical fields, the efficacy of neuropharmacotherapy depends on both psychosocial and biological characteristics of the patient. Patient’s gender is one of the latter. Although women are more likely to experience adverse drug effects, they still represent the minority in the first two phases of clinical trials. This results in fragmentary knowledge of potential dosage modifications in women. The paper presents an outline of sex differences that need to be considered in neuropharmacotherapy. The reported differences in pharmacokinetics are based on, among other things, different absorption due to lower gastric pH in women, longer gastrointestinal transit and lower P-glycoprotein expression. Drug distribution, which varies between men and women as a result of the different body fat percentage, also has an impact on the pharmacological response. Furthermore, there are also differences in the activity of drug metabolising enzymes, probably due to oestrogens. Additionally, a lower excretion rate may lead to insufficient drug elimination and subsequent side effects. The paper also presents examples of pharmacodynamic disparities. Neglecting gender differences in neurological pharmacotherapy may result in avoidable adverse effects or poor therapeutic outcome. Understanding the source of pharmacotherapeutic differences promotes therapy individualisation, and thus improved treatment outcomes and reduced adverse events, both in women and men.

Keywords: pharmacokinetics, pharmacodynamics, gender differences
The use of immunomodulatory therapy in pregnant patients with multiple sclerosis
Aktualn Neurol 2018, 18 (3), p. 123–131
DOI: 10.15557/AN.2018.0017

Multiple sclerosis is a chronic autoimmune disease which mainly affects young adults – women are 2–3 times more likely to be affected than men. The highest incidence in the procreative age entails the issue of the coexistence of the disease with pregnancy. The treatment of multiple sclerosis in pregnant women is an important problem, as, so far, none of the drugs modifying the course of the disease has been considered completely safe for the foetus. In this article, on the basis of the analysis of relevant literature, information was collected on the mechanisms of action of drugs modifying the course of multiple sclerosis: beta interferon, glatiramer acetate, natalizumab, fingolimod, dimethyl fumarate, teriflunomide, alemtuzumab, mitoxantrone, cladribine, and ocrelizumab, as well as medicines used in relapse: methylprednisolone, immunoglobulins. The safety profile of drugs used in pregnancy is discussed, as well as the indications for interruption or continuation of a therapy in pregnant patients. Data on bridging therapy before planning the offspring were also summed up. Glatiramer acetate is the only drug modifying the course of the disease which is not contraindicated for use during pregnancy as part of the National Health Fund’s drug program. The continuation of interferon beta, natalizumab, dimethyl fumarate, alemtuzumab and ocrelizumab therapy can be considered when the risk of high disease activity outweighs the risk to the foetus. In the case of drugs with the highest toxicity and long half-life in the body (teriflunomide, mitoxantrone, cladribine), an interval between drug withdrawal and pregnancy planning adequate for each of the substances should be maintained. The treatment of multiple sclerosis relapse in pregnant women is no different from the one used in non-pregnant patients – methylprednisolone crosses the placental barrier but is metabolised to inactive forms. Used in the first trimester, it increases the risk of cleft lip and low birth weight, while remaining relatively safe in the second and third trimester. As part of bridging therapy, it is recommended during pregnancy planning to consider the use of glatiramer acetate and interferon beta.

Keywords: multiple sclerosis, immunomodulatory treatment, pregnancy, breastfeeding
Epilepsy in women – selected issues
Aktualn Neurol 2018, 18 (3), p. 132–137
DOI: 10.15557/AN.2018.0018

Epilepsy is one of the most frequent diseases of the central nervous system and affects about 50 million people in the world. It is a heterogeneous disease entity associated with various clinical manifestations and coexisting symptoms (such as neurodevelopmental disorders), resulting in different response to medication and different prognosis. Clinical evidence points to the existence of epilepsy and epilepsy syndromes dependent on sex. Female and male sex hormones affect the brain convulsion threshold. Catamenial epilepsy is a well-known example here, in which the neurohormonal mechanisms dependent on the menstrual cycle affect the occurrence of convulsions. Progesterone is an anticonvulsant hormone, while oestrogens can be both pro- and anticonvulsant, depending on the physiological state. Unlike in the case of progesterone, the influence of the potential convulsion threshold modulation of testosterone is complex. In animal models, but also in clinical trials, testosterone intensifies the occurrence of convulsions through its metabolism to oestrogens. The paper presents the effects of antiepileptic drugs on the offspring of mothers with epilepsy (congenital malformations, lower birth weight, and neurodevelopmental disorders). Aggregate data indicate the highest teratogenicity of the exposure to valproic acid and other antiepileptics used in polytherapy. Other factors may also influence foetal development: severity of epilepsy itself, the type of seizures, mother’s intelligence quotient and socioeconomic status as well as the exposure to toxic agents (including alcohol and other stimulants). The optimal selection of antiepileptic drugs should be sought even before the planned pregnancy in the care of women at childbearing age suffering from epilepsy.

Keywords: epilepsy, sex hormones, pregnancy
Thrombectomy – a new hope in the treatment of ischaemic stroke
Aktualn Neurol 2018, 18 (3), p. 138–143
DOI: 10.15557/AN.2018.0019

First reports from randomised trials presenting positive outcomes of invasive treatment of ischaemic stroke, i.e. thrombectomy, were published a few years ago. The procedure is performed in patients with documented embolism, usually in the proximal anterior cerebral circulation. Studies have also shown positive outcomes of patency restoration in the distal segment of the middle cerebral artery, basilar artery, and even vertebral arteries. No differences were demonstrated between anterior and posterior cerebral circulation thrombectomy. There is a need for the development of methods and further adjustment of tools for thrombectomy in posterior circulation. Currently, the time window is 6 hours from the onset of symptoms. As a result of DEFUSE 3 and DAWN studies in carefully selected patients, this time may be extended to 16 hours (class IA recommendation) or 24 hours (class IIA recommendation) following an assessment of the ratio between infarct volume and penumbra. There is a large amount of data to compare combined therapy, i.e. thrombolytic treatment plus thrombectomy, with thrombectomy alone. One of meta-analyses, which included 13 studies, showed that combined therapy is associated with improved scores in Rankin scale, lower mortality, higher recanalisation rates and reduced duration of the procedure itself. Other publications suggest an increased risk of haemorrhagic complications and prolonged time to thrombectomy in combined treatment. Currently, there is an ongoing SWIFT DIRECT study comparing these two modes of treatment. Haemorrhagic complications, such as intracranial and subarachnoid haemorrhages, are the most common complications. Other complications include vessel rupture, vasoconstriction, clotting in a different location, inguinal haematoma, retroperitoneal haematoma, and femoral artery pseudoaneurysm. Nevertheless, the procedure is safe (low rate of adverse effects) and allows for effective causative treatment of ischaemic stroke.

Keywords: mechanical thrombectomy, ischaemic stroke, thrombolysis
Late onset distal hereditary motor neuropathy type IIB (dHMN IIB) – case reports
Aktualn Neurol 2018, 18 (3), p. 144–147
DOI: 10.15557/AN.2018.0020

Distal hereditary motor neuropathies are a heterogeneous group of rare, genetically determined neuromuscular disorders. Distal hereditary motor neuropathy type IIB is an autosomal dominant disorder, and the onset of symptoms is observed in adulthood. Mutation refers to heat shock protein 27, also known as heat shock protein beta-1. The main symptoms of distal hereditary motor neuropathy type IIB are muscular atrophy and paresis of distal limb muscles. In this article, we present the first Polish case of familial late onset distal hereditary neuropathy type IIB with a T151I mutation (p.Thr151Ile) in one allele of the heat shock protein 27 gene. The first symptoms of the disease in our patients began around the age of 60.

Keywords: dHMN, late onset dHMN IIB, HSP27 gene, T151I mutation