Browsing by Author "Konuskan, Bahadir"

Now showing 1 - 4 of 4

Clinical Presentation of Anti-N-Methyl-D-Aspartate Receptor and Anti-Voltage-Gated Potassium Channel Complex Antibodies in Children: A Series Of 24 Cases
(2018) Konuskan, Bahadir; Yildirim, Mirac; Topaloglu, Haluk; Erol, Ilknur; Oztoprak, Ulkuhan; Tan, Huseyin; Gocmen, Rahsan; Anlar, Banu; https://orcid.org/0000-0002-3530-0463; 29153996; AAK-4825-2021
Objective: The symptomatology and paraclinical findings of antibody-mediated encephalitis, a relatively novel disorder, are still being characterized in adults and children. A high index of suspicion is needed in order to identify these cases among children presenting with various neurological symptoms. The aim of this study is to examine the clinical, demographic and laboratory findings and outcome of children with anti-NMDAR and anti-VGKC encephalitis for any typical or distinctive features. Methods: Cases diagnosed with anti-N-Methyl o-aspartate receptor (NMDAR) and anti voltage gated potassium channel (VGKC) antibody-mediated encephalopathy in four major child neurology centers are described. Results: In four years, 16 children with NMDAR and 8 children with VGKC antibody associated disease were identified in the participating centers. The most frequent initial manifestation consisted of generalized seizures and cognitive symptoms in both groups. Movement abnormalities were frequent in anti-NMDAR patients and autonomic symptoms, in anti-VGKC patients. Cerebrospinal fluid (CSF) protein, cell count and IgG index were normal in 9/15 anti-NMDAR and 5/8 anti-VGKC patients tested. EEG and MRI findings were usually nonspecific and non-contributory. The rate and time of recovery was not related to age, sex, acute or subacute onset, antibody type, MRI, EEG or CSF results. Treatment within 3 months of onset was associated with normal neurological outcome. Conclusions: Our results suggest anti-NMDAR and VGKC encephalopathies mostly present with non-focal neurological symptoms longer than 3 weeks. In contrast with adult cases, routine CSF testing, MRI and EEG did not contribute to the diagnosis in this series. (C) 2017 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.
Electroencephalographic Findings in Anti-N-Methyl-D-Aspartate Receptor Encephalitis in Children: A Series Of 12 Patients
(2018) Yildirim, Mirac; Konuskan, Bahadir; Yalnizoglu, Dilek; Topaloglu, Haluk; Erol, Ilknur; https://orcid.org/0000-0002-3530-0463; 29179102; AAK-4825-2021
Objective: Anti-N-methyl-D-aspartate receptor encephalitis (a-NMDARe) is an acute or subacute encephalopathy where electroencephalogram (EEG) is frequently obtained as part of the workup. Although no diagnostic EEG finding has been described so far, the definition of specific or typical patterns might help to distinguish this group among various encephalopathies of childhood. We examined EEG recordings of our patients with a-NMDARe in order to describe the most frequent findings. Methods: Clinical and laboratory data and digital EEG recordings of 12 pediatric patients diagnosed with a-NMDARe in two major child neurology centers are evaluated. Results: We reviewed 43 EEG recordings from 12 children with a-NMDARe and followed their evolution for a median of 6 (range: 1-60) months. Initial EEG was abnormal in 11/12 patients. The most frequent finding was focal or diffuse slowing of the background rhythm. Generalized rhythmic delta activity, brief rhythmic discharges (BRDs), and occipital intermittent rhythmic delta activity (OIRDA) were seen in two patients each. Diffuse excess beta frequency activity was seen in three patients. Extreme delta brushes were observed in 5/12 (41.7%) patients, disappeared in 4-6 months (two patients), or persisted at 10-17 months (two patients). Epileptic activity was seen in seven patients (58%) and lateralized periodic discharges in one. On follow-up EEGs, most epileptic activity disappeared in a median of 8 months. Conclusions: A normal EEG is rare in a-NMDARe. Focal or diffuse slowing, epileptic activity, and extreme delta brush are common findings. Epileptic activity in early EEGs do not persists in most patients. Severe diffuse slowing may predict neurological impairment if confirmed in larger series. (C) 2017 Elsevier Inc. All rights reserved.
Neuromyelitis Optica in Children: A Review of The Literature
(2014) Derle, Eda; Gunes, H. Nalan; Konuskan, Bahadir; Tuncer-Kurne, Asli; https://orcid.org/0000-0003-2122-1016; 26388586; AAI-8830-2021
Neuromyelitis optica (NMO) is a rare and severe inflammatory disease of the central nervous system (CNS), which constitutes up to 5% of pediatric aquired demyelinating diseases. The optic nerves and the spinal cord are the most affected sites. The discovery of an autoantibody called NMO-IgG, which targets aquaporin-4, the main water channel in the CNS, gave a new direction to understanding the underlying immunologic mechanisms. This specific biomarker also helps to distinguish the disease from other demyelinating disorders. Here, we review the clinical and paraclinical features, immunological properties and treatment options of the disease as reported in the literature.
Unfavorable Outcome of Pediatric Onset Multiple Sclerosis: Follow-Up in the Pediatric and Adult Neurology Departments of One Referral Center, in Turkey
(2016) Derle, Eda; Kurne, Asli Tuncer; Konuskan, Bahadir; Karabudak, Rana; Anlar, Banu; 0000-0001-6727-6229; 0000-0003-2122-1016; 27645334; HJH-2490-2023; I-9090-2013; AAI-8830-2021
Background:: The prevalence of MS starting under 18 years of age ranges between 2-10% of the total MS population. Objective:: We aimed to examine the clinical and long term follow-up data of pediatric-onset cases in our institutional MS database. Method:: We evaluated the clinical data from the MS database of the Departments of Neurology and Pediatric Neurology of Hacettepe University Hospital. Results:: The clinical features of 74 patients who had experienced the first attack before age 18 years comprised 3.9% of our MS population. Median age at onset was 15 (3, 5-17, IQR=3.63) years, and female: male ratio was 2.4. The most frequent symptom at onset was brainstemicerebellar dysfunction (32.4%). Seventy two patients (97.3%) initially had relapsing remitting course and in the follow-up, 17 (23%) of them developed secondary progressive (SP) course. The median interval to develop SPMS course was 10 (5-21, IQR=8) years. At the last visit, median disease duration was 6.67 (0.83-25, IQR=9.06) years, 41 (55.4%) of them had EDSS of >= 4. Conclusion:: These findings illustrate the profile of our pediatric MS patients: almost all are relapsing remitting initially; about one fourth become secondarily progressive in 10 years, and about half acquire disability EDSS >= 4 in mean 8 years. (C) 2016 Elsevier B.V. All rights reserved.