Clinical Neuroscience - 2021;74(07-08)

Clinical Neuroscience

JULY 30, 2021

[In memoriam Prof. Dr. Ferenc Garzuly (1937-2021)]

ILLÉS Zsolt, HAHN Katalin, KÁLMÁN Bernadette

[Ferenc Garzuly passed away after a long and productive life at the age of 84. He worked for almost 60 years at the Markusovszky University Teaching Hospital, where primarily led the laboratory of neuropathology and the department of neurology, but transferred to the department of pathology after his retirement. By authoring several books on rare diseases, he greatly enriched the case-based tea­ching approach in medicine. He described the Hun­garian type of transthyretin mutation causing the familial me­nin­go­cerebrovascular amyloidosis phenotype. The presentation of a special phenotype of Fabry disease associated with megadolichobasilar anomaly and a novel mutation in the alpha-galactosidase-A gene is also associated with his name. ]

Clinical Neuroscience

JULY 30, 2021

[Personalised epilepsy treatment]

ALTMANN Anna

[Epilepsy is one of the most common chronic neurological disease in childhood. Patients with epilepsy – even with so-called benign epilepsy – need medication for years. During this time, children go through a very big change, not only gaining weight and height, but also changing hormonal and metabolic processes. Maturation processes in different brain areas also take place at different rates depending on age. All of these should be considered when preparing a therapeutic plan. In everyday practice after the diagnosis of epilepsy, the applied drug is most often selected based on the shape and type of seizure. However, a number of other factors need to be considered when designing a therapeutic strategy: 1. efficacy (form of epilepsy, type of seizure), 2. age, gender, 3. pharmacological properties of the drug, 4. adverse drug reaction profile, 5. lifestyle (community), figure (skinny, corpulent, obese), 6. other comorbidities (nutrition, behavioral and learning problems, circulatory disorders, kidney or liver disease), 7. expected interactions with other drugs already used, 8. genetics, 9. other aspects (drug registration and prescription rules). The purpose of this article is to help to decide which antiepileptic drugs are expected to have the least side effects in a particular child with different comorbidities and which medications should be avoided if possible.]

Clinical Neuroscience

JULY 30, 2021

[Dysphagiafelmérések akut stroke-ban]

SZABÓ Pál Tamás, MÛHELYI Viktória, BÉRES-MOLNÁR Katalin Anna, KOVÁCS Andrea, BALOGH Zoltán, FOLYOVICH András

[Stroke associated dysphagia can have serious consequences such as aspiration pneumonia. The Hungarian guideline on nutritional therapy for stroke patients recommends dysphagia assessment, as early screening can optimize disease outcome and hospital cost. Thus far, this may be the first study in Hungarian that has documented a systematic review about the available validated dysphagia assessments of acute stroke. Purpose – The aim of this study was to summarize the instrumentally validated bedside dysphagia screening tools for acute stroke patients, which were published in the last twenty years. Our objective was to describe the characteristics of the validation studies, examine their study design, and sample the sub-tests and the diagnostic accuracy of the assessments. A systematic research was carried out of the literature between 2001 and 2021 in eight scientific databases with search terms appropriate to our objectives. Subjects of the study – 652 articles were found and were reduced to eight. We made a comparative analysis of these. The GUSS test reached a high level of sensitivity compared to the others. In our study sample, the prevalence of instrumentally confirmed dysphagia among acute stroke patients was 56.1%. The focus and the composition of the analyzed studies differed and posed problems such as the ambiguity of the concept of dysphagia, the difference in outcome indicators, or the timing of screening. The GUSS test, which offers domestic management, is a suitable tool for the Hungarian clinical use.]

Clinical Neuroscience

JULY 30, 2021

[Controversies in neurology: Diagnosis, follow up and therapy of multiple sclerosis with pathomechanismal approach]

VÉCSEI László

[The clinical boundaries between the relapsing and progressive course of multiple sclerosis are often indistinct. Despite the variable patterns of evolution, there are no biological reasons for discerning different multiple sclerosis phenotypes. Indeed, both primary progressive and secondary forms of the disease share similar pathological features in respect of the extent of inflammatory infiltrates, axonal damage, and cortical demyelination. The data indicating that primary progressive multiple sclerosis is preceded by an asymptomatic relapsing remitting phase. The proposed definition of secondary progressive multiple slcerosis, the attainment of at least EDSS of 4 is required to mark the transition to the progressive phase. Therefore, the clinical progress can be uncovered in the early phase of the disease. Furthermore, a continuous progression independent of relapsing activity is commonly observed during the relapsing remitting phase. A continuous smouldering process underpins the subtle clinical deterioration, which stands out as an important unmet treatment target. Concerning cognitive dysfunction of the patients pro-inflammatory cytokines have been associated with worse cognition in active multiple sclerosis, and this inflammatory milieu could also contribute to altered mentation during relapses. Therefore, long before people with multiple sclerosis become physically disabled, they have usually acquired hidden disabilities related to cognitive impairment. Silent progression appears during the relapsing remitting phase and it associates with brain atrophy. This suggests that the same process that underlies secondary progressive multiple sclerosis likely begins far earlier than is generally recognized. This supports a unitary view of multiple sclerosis biology. ]

Clinical Neuroscience

JULY 30, 2021

Management of bone metabolism in epilepsy

UÇAN TOKUÇ Ezgi Firdevs , FATMA Genç, ABIDIN Erdal, YASEMIN Biçer Gömceli

Many systemic problems arise due to the side effects of antiepileptic drugs (AEDs) used in epilepsy patients. Among these adverse effects are low bone mineral density and increased fracture risk due to long-term AED use. Although various studies have supported this association with increased risk in recent years, the length of this process has not been precisely defined and there is no clear consensus on bone density scanning, intervals of screening, and the subject of calcium and vitamin D supplementation. In this study, in accordance with the most current recommendations, our applications and data, including the detection of possible bone mineralization disorders, treatment methods, and recommendations to prevent bone mineralization disorders, were evaluated in epilepsy patients who were followed up at our outpatient clinic. It was aimed to draw attention to the significance of management of bone metabolism carried out with appropriate protocols. Epilepsy patients were followed up at the Antalya Training and Research Hospital Department of Neurology, Epilepsy Outpatient Clinic who were at high risk for osteoporosis (use of valproic acid [VPA] and enzyme-inducing drugs, using any AED for over 5 years, and postmenopausal women) and were evaluated using a screening protocol. According to this protocol, a total of 190 patients suspected of osteoporosis risk were retrospectively evaluated. Four patients were excluded from the study due to secondary osteoporosis. Of the 186 patients who were included in the study, 97 (52.2%) were women and 89 (47.8%) were men. Prevalence of low bone mineral density (BMD) was 42%, in which osteoporosis was detected in 11.8% and osteopenia in 30.6% of the patients. Osteoporosis rate was higher at the young age group (18-45) and this difference was statistically significant (p=0.018). There was no significant difference between male and female sexes according to osteoporosis and osteopenia rates. Patients receiving polytherapy had higher osteoporosis rate and lower BMD compared to patients receiving monotherapy. Comparison of separate drug groups according to osteoporosis rate revealed that osteoporosis rate was highest in patient groups using VPA+ carbamazepine (CBZ) (29.4%) and VPA polytherapy (19.4%). Total of osteopenia and osteoporosis, or low BMD, was highest in VPA polytherapy (VPA+ non-enzyme-inducing AED [NEID]) and CBZ polytherapy (CBZ+NEID) groups, with rates of 58.3% and 55.1%, respectively. In addition, there was no significant difference between drug groups according to bone metabolism markers, vitamin D levels, and osteopenia-osteoporosis rates. Assuming bone health will be affected at an early age in epilepsy patients, providing lifestyle and diet recommendations, avoiding polytherapy including VPA and CBZ when possible, and evaluating bone metabolism at regular intervals are actions that should be applied in routine practice.

Clinical Neuroscience

JULY 30, 2021

Restless leg syndrome in adult patients with β-thalassemia major

ERDEM Ramazan , ERDEM Şimşek Nazan, KURTOĞLU Erdal

To investigate the quality of sleep and the presence of Restless Legs Syndrome (RLS) in the Turkish population with β-thalassemia major (TM). The second aim was to assess the risk factors of RLS in TM adults. The study sample comprised of 121 patients at least 18 years old with TM. The patients’ socio-demographic information, body mass indexes (BMI), current medications, laboratory data were recorded. The patients were asked if they had a history of chronic kidney disease, diabetes mellitus (DM), and polyneuropathy. Restless legs syndrome was diagnosed according to the International Restless Legs Syndrome Study Group criteria. The sleep quality of the patients was assessed using the Pittsburgh Sleep Quality Index (PSQI) scale. The Epworth Sleepiness Scale (ESS) was used to assess excessive daytime sleepiness in the patients. The median age of the patients was 25 years (range 18-52). The mean BMI was 21.49±2.5 (R 14-26.5) for all patients. The prevalence of RLS was 5% in TM adult patients. The TM patients with RLS had no major complications of TM. The median PSQI global score of all patients was 3. Twenty-two (18.1%) patients had poor sleep quality. The reason for poor sleep quality was RLS symptoms in four patients (18%). There was no significant association between PSQI total score and blood parameters of the patients. Twelve (9.9%) patients had ESS scores greater than 10, which indicates excessive daytime sleepiness. The prevalence of RLS in TM patients was similar to that of the general Turkish adult population. These results indicate that RLS may occur in patients with TM, although they had a high level of serum ferritin.

Clinical Neuroscience

JULY 30, 2021

Acute transverse myelitis after inactivated COVID-19 vaccine

ERDEM Şimşek Nazan, DEMIRCI Seden, ÖZEL Tuğba , MAMADOVA Khalida, KARAALI Kamil , ÇELIK Tuğba Havva , USLU Ilgen Ferda, ÖZKAYNAK Sibel Sehür

Vaccines against the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) have been rapidly developed to prevent coronavirus disease 2019 (COVID-19) pandemic. There is increasing safety concerns regarding COVID-19 vaccines. We report a 78-year old woman who was presented with tetraparesis, paresthesias of bilateral upper extremities, and urinary retention of one-day duration. Three weeks before these symptoms, she was vaccinated with CoronaVAC vaccine (Sinovac Life Sciences, China). Spine magnetic resonance imaging showed longitudinally extensive transverse myelitis (TM) from the C1 to the T3 spinal cord segment. An extensive diagnostic workup was performed to exclude other possible causes of TM. We suggest that longitudinally extensive TM may be associated with COVID-19 vaccination in this case. To the best of our knowledge, this is the first report of longitudinally extensive TM developing after CoronaVac vaccination. Clinicians should be aware of neurological symptoms after vaccination of COVID-19.

Clinical Neuroscience

JULY 30, 2021

[Encephalitis following COVID-19 disease]

VARANNAI Lajos, MAGYAR Zsuzsa, BARANYAI Beatrix, AJZNER Éva, CZURKÓ Marina

[Shortly after that COVID-19 appeared it became clear, that although the disease mainly characterized by respiratory symptoms, other signs frequently appeared, which showed involvation of other organs. There are several new publications which report about neurological complications. According to data developing of encephalitis could be relatively frequent among these. Its symptoms can mostly be observed concommittantly with respiratory symptoms or during critical state of the disease, and several forms were detected. In our patient symptoms of central nervous system involvement appeared a few weeks after healing of COVID-19 pneumonia. Clinical signs, imaging, electroencephalograpy and cerebrospinal fluid analysis confirmed the diagnosis of encephalitis. Considering the previous SARS-CoV-2 infection and the results of the examinations, we think this case was a postinfectious central nervous system disease. There are only a few data available regarding encephalitis after Covid-19 disease in the literature, yet. ]

Clinical Neuroscience

JULY 30, 2021

A variant of Guillain-Barre syndrome after SARS-CoV-2 vaccination: AMSAN

TUTAR Kaya Nurhan, EYIGÜRBÜZ Tuğba, YILDIRIM Zerrin, KALE Nilufer

Introduction - Coronavirus disease 2019 (COVID-19) is a respiratory infection that has rapidly become a global pandemic and vaccines against SARS-CoV-2 have been developed with great success. In this article, we would like to present a patient who developed Guillain-Barré syndrome (GBS), which is a serious complication after receiving the inactive SARS-CoV-2 vaccine (CoronaVac). Case report – A 76-year-old male patient presented to the emergency department with nine days of progressive limb weakness. Two weeks prior to admission, he received the second dose of CoronaVac vaccine. Motor examination revealed decreased extremity strength with 3/5 in the lower extremities versus 4/5 in the upper extremities. Deep tendon reflexes were absent in all four extremities. Nerve conduction studies showed predominantly reduced amplitude in both motor and sensory nerves, consistent with AMSAN (acute motor and sensory axonal neuropathy). Conclusion - Clinicians should be aware of the neuro­logical complications or other side effects associated with COVID-19 vaccination so that early treatment can be an option.