Clinical Neuroscience

[The evaluation of paroxysmal events in neonates and infants]

NAGY Eszter, FARKAS Nelli, HOLLÓDY Katalin

SEPTEMBER 30, 2018

Clinical Neuroscience - 2018;71(09-10)

DOI: https://doi.org/10.18071/isz.71.0313

[Introduction - Differential diagnosis of neonatal and infantile seizures based only on inspection poses a challenge even for specialists. Aims - To investigate the evaluations of neonatal and infantile paroxysmal events based only on inspection. Research question - Is there any difference in the opinion of neonatologists, paediatric neurologists and neurologists about the assessment of common paroxysmal events in infancy? Patients and methods - Video recordings about paroxysmal movements of 15 neonates or infants (aged 2 days- 5 months) were displayed for 47 paediatric neurologists, 35 neonatologists and nurses working in Neonatal or Perinatal Intensive Care Units and 43 neurologists. They had to decide without knowing the past medical history or EEG results whether events presented were epileptic or nonepileptic in nature. Results - Answers of neonatologists and paediatric neurologists were correct in 67% of cases (824/1230), no significant difference was found between their results. The largest uncertainty was in the judgement of discrete hand movements and very rapid clonus with epileptic origin, they were judged correctly by only one third of participants. The result of neurologists was only slightly, but not significantly different from that of paediatric neurologists. Conclusion - In most cases, the correct diagnosis of neonatal and infantile paroxysmal events requires video-EEG recording. No significant difference was revealed between the evaluation of neonatologists and paediatric neurologists about the differential diagnosis of movements. The ongoing cooperation of paediatric neurologists and neurologists going back to several decades facilitates the shaping of a common perspective.]

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Clinical Neuroscience

[Diagnosis of multiple sclerosis: A review of the 2017 revisions of the McDonald criteria]

CSÉPÁNY Tünde

[The revolutionary progress of research in neuroimmu­nology has led to the introduction of disease modifying therapies in multiple sclerosis at the end of the last century. The International Panel on Diagnosis of Multiple Sclerosis originally proposed the 2001 McDonald criteria to facilitate the diagnosis of MS in patients with the first objective neurological symptom(s) suggesting demyelinating event, when magnetic resonance imaging is integrated with clinical and other paraclinical diagnostic methods. New terms have been introduced to substitute clinical information by MRI: dissemination in space - indicating a multifocal central demyelinating process and dissemination in time - indicating the development of new CNS lesions over time. The criteria for diagnosis of Multiple Sclerosis have continuously evolved, they were modified in 2005 and 2010 allowing for an earlier and more accurate diagnosis of MS over time, and they provided the most up-to-date guidance for clinicians and researchers. The last recommended revisions relied entirely on available evidence, and not on expert opinion thereby reducing the risk of the misdiagnosis. The 2017 McDonald criteria continue to apply primarily to patients experiencing a typical, clinically isolated syndrome. In this review, we provide an overview of the recent 2017 revisions to the criteria of dissemination in space and time with the importance of the presence of CSF-specific oligoclonal bands; keeping fully in mind that there is no better explanation for symptoms than diagnosis of MS. In the future, validation of the 2017 McDonald criteria will be needed in diverse populations. Further investigations are required on the value of new MRI approaches, on optic nerve involvement, on evoked potential and optical coherence tomography, in order to assess their possible contribution to diagnostic criteria.]

Clinical Neuroscience

Acute motor and sensory axonal neuropathy associated with Sjögren’s syndrome

ETHEMOGLU Ozlem, KOCATÜRK Özcan, TARINI Zeynep Emine

Sjögren’s syndrome (SS) is an autoimmune disease with mononuclear cell infiltration and destruction of the lacrimal gland and salivary glands, which cause dryness of the eyes and mouth. The most common neurological condition seen in SS is peripheral neuropathy. Initial manifestation of SS as an acute fulminant peripheral neuropathy is extremely rare. We report a 42-year-old patient presenting with acute motor sensory-axonal neuropathy in the presence of SS. She showed partial response to intravenous immunoglobulin but favourable clinical improvement was seen after initiation of corticosteroid treatment.

Clinical Neuroscience

Anterior cerebral artery infarcts; two years follow-up study

LÜTFÜ Hanoglu, ELMIR Khanmammadov, SEMA Demirci, ÜMMÜHAN Altin, DURSUN Kirbaş, TAHA Hanoglu, BURAK Yulug

Objectives – Anterior cerebral infarct (ACA) infarcts are reported very rare that is due to the compensatory collateral circulation provided by the anterior communicating artery. There are very few studies reporting the long-term follow-up results of ACA infarcts regarding their aetiology, clinical features and prognosis. Most studies reported in the literature vary between several months to one year. Patients and methods – A total of 27 patients with ACA infarcts were registered (14 women and 13 men). The mean age of the patients was 68.5 (age range: 45–89 years). Results – Bilateral ACA infarcts were reported in four patients (14.8%), right ACA infarct in 11 (40%) patients and left ACA infarct in 12 patients (44%). During the initial examination 15 patients (55.5%) were found to have apathy, 13 patients (48%) had incontinence, nine patients (33.3%) had primitive reflexes, 11 patients (40.7%) had aphasia, while six patients (22.2%) were found to suffer from neglect. At the end of one-year follow-up, five patients (22.7%) were reported to have apathy, 6 patients (27.2%) had incontinence, one patient (4.5%) had primitive reflexes, while one patient (4.5%) was found to have permanent aphasia, and no patients was found to suffer from neglect. Conclusion – Here we present our clinical data regarding the aetiology, specific clinical characteristics (including the speech disorders) and prognosis of 27 patients with ACA infarcts during a relatively longer follow-up period (3 months – 30 months) in compared to previous literature. We show that there are differences in the etiological factors of ACA infarcts between the Asian and European communities. Regarding speech disorders which are frequently reported during ACA infarcts, our study results are in agreement with other studies suggesting that this clinical picture is more than a real aphasia and associated with general hypokinesia and reduction in psychomotor activity.

Clinical Neuroscience

A multidisciplinary clinical approach to facioscapulohumeral muscular dystrophy

CAKMAK Öztop Özgür, EREN Ilker, ASLANGER Ayca, GÜNERBÜYÜK Caner, KAYSERILI Hülya, OFLAZER Piraye, SAR Cüneyt, DEMIRHAN Mehmet, ÖZDEMIR Gürsoy Yasemin

Background - Impaired shoulder function is the most disabling problem for daily life of Fascioscapulohumeral muscular dystrophy (FSHD) patients. Scapulothoracic arthrodesis can give a high impact to the functionality of patients. Here we report our experience with scapulothoracic arthrodesis and spinal stenosis surgery in FSHD patients. Patients and methods - 32 FSHD patients were collected between 2015-2016. Demographical and clinical features were documented. All the patients were neurologically examined. The Medical Research Council (MRC) and the FSHD evaluation scale was used to assess muscle involvement1. Scapulothoracic arthrodesis and spinal stenosis surgeries were performed in eligible patients. Results - There were 16 male and 16 female (mean age 34.4 years; range 12-73) patients. 6 shoulders of 4 patients aged between 2132 years underwent scapulothoracic arthrodesis (two bilateral, one left and one right sided). Only one 63 years old female patient with severe hyperlordosis had spinal fusion surgery. All of the patients undergoing these corrective surgeries have better functionality in daily life, as well as superior shoulder elevation. Conclusion - Until the emergence and clinical use of novel therapeutics, surgical interventions are indicated in carefully selected patients with FSHD to improve arm movements, the posture and the quality of life of patients in general. Scapulothorosic arthrodesis is a management with good clinical results and patient satisfaction. In selected cases other corrective orthopedic surgeries like spinal fusion may also be considered.

Clinical Neuroscience

Caregiver burden and quality of life in early stages of idiopathic Parkinson’s disease

YUKSEL Burcu, AK Dogan Pelin, SEN Aysu, SARIAHMETOGLU Hande, USLU Celiker Sibel, ATAKLI Dilek

Objectives - The aim of this study was to assess the impact of early stage of idiopathic Parkinson’s disease (IPD) on caregiver burden with disease severity, duration, disability and psychiatric symptoms. Methods - 30 IPD patient (15 female, 15 male) - caregiver (18 female, 12 male) pairs participated in the study. Hoehn and Yahr (H-Y) scale was used to provide the assessment of disease progression and Unified Parkinson’s Disease Rating Scale (UPDRS) was used for assessing disability and impairment. Zarit Caregiver Burden Inventory (ZCBI) was used to ascertain the distress experienced by caregivers. Hospital Anxiety and Depression scale (HADS) was performed on both patient and caregiver groups to evaluate anxiety and depression. Depressive symptoms of both groups were also measured by Beck Depression Inventory (BDI). Patients’ psychotic symptoms were assessed using the part 1- mentation, behavior and mood section of UPDRS. Mini-Mental State Examination (MMSE) was used to evaluate dementia symptoms and Short Form-36 (SF-36) was also used to assess quality of life. Results - We found significant correlation between caregiver burden with disease severity and duration. There was a significant difference between high UPDRS scores and the caregiver’s will for placing her/his patient in a long-term institution. Patients who had depression risk according to BDI had also high UPDRS scores. Patients with off period had higher UPDRS scores and lower SF-36 subdomains of general health, physical functioning, emotional role and social functioning. Conclusion - IPD is a chronic, progressive neuro- degenerative disease and comprises substantial burden on patients, families of patients and caregivers. The disease duration and disability have a remarkable impact on caregiver burden. For the good quality of caregiving, protective therapies should be recommended for caregivers if needed.

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Clinical Neuroscience

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

Effects of valproate, carbamazepine and levetiracetam on Tp-e interval, Tp-e/QT and Tp-e/QTc ratio

YASAR Altun, ERDOGAN Yasar

Aim - To evaluate P-wave dispersion before and after antiepileptic drug (AED) treatment as well as to investigate the risk of ventricular repolarization using the Tpeak-Tend (Tp-e) interval and Tp-e/QT ratio in patients with epileptic disorder. Methods - A total of 63 patients receiving AED therapy and 35 healthy adults were included. ECG recordings were obtained before and 3 months after anti-epileptic treatment among patients with epilepsy. For both groups, Tp-e and Tp-e/QT ratio were measured using a 12-lead ECG device. Results - Tp-e interval, Tpe/QT and Tp-e/QTc ratios were found to be higher in the patient group than in the control group (p<0.05, for all), while QTmax ratio was significantly lower in the patient group. After 3 months of AED therapy, significant increases in QT max, QTc max, QTcd, Tp-e, Tp-e/QT, and Tp-e/QTc were found among the patients (p<0.05). When the arrhythmic effects of the drugs before and after treatment were compared, especially in the valproic acid group, there were significant increases in Tp-e interval, Tp-e/QT and Tp-e/QTc values after three months of treatment (p<0.05). Carbamazepine and levetiracetam groups were not statistically significant in terms of pre- and post-treatment values. Conclusions - It was concluded that an arrhythmogenic environment may be associated with the disease, and patients who received AED monotherapy may need to be followed up more closely for arrhythmia.

Clinical Neuroscience

[Personalised epilepsy treatment]

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[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.]

Lege Artis Medicinae

[Epilepsy in coronavirus pandemic]

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Clinical Neuroscience

[Decisional collisions between evidence and experience based medicine in care of people with epilepsy]

RAJNA Péter

[Background – Based on the literature and his long-term clinical practice the author stresses the main collisions of evidence and experience based medicine in the care of people with epilepsy. Purpose – To see, what are the professional decisions of high responsibility in the epilepsy-care, in whose the relevant clinical research is still lacking or does not give a satisfactory basis. Methods – Following the structure of the Hungarian Guideline the author points the critical situations and decisions. He explains also the causes of the dilemmas: the lack or uncertainty of evidences or the difficulty of scientific investigation of the situation. Results – There are some priorities of experience based medicine in the following areas: definition of epilepsy, classification of seizures, etiology – including genetic background –, role of precipitating and provoking factors. These are able to influence the complex diagnosis. In the pharmacotherapy the choice of the first drug and the optimal algorithm as well as the tasks during the care are also depends on personal experiences sometimes contradictory to the official recommendations. Same can occur in the choice of the non-pharmacological treatments and rehabilitation. Discussion and conclusion – Personal professional experiences (and interests of patients) must be obligatory accessories of evidence based attitude, but for achieving the optimal results, in some situations they replace the official recommendations. Therefore it is very important that the problematic patients do meet experts having necessary experiences and also professional responsibility to help in these decisions. ]