Publications by authors named "Banu Anlar"

123 Publications

International Consensus Recommendations for the Treatment of Pediatric NMDAR Antibody Encephalitis.

Neurol Neuroimmunol Neuroinflamm 2021 Jul 22;8(5). Epub 2021 Jul 22.

From the Paediatric Neurology and Neurophysiology Unit (M.N.), Department of Women's and Children's Health, University Hospital of Padova; Neuroimmunology Group (M.N.), Paediatric Research Institute "Città della Speranza," Padova, Italy; Department of Paediatrics (T.T.), Neurology Service, KK Women's and Children's Hospital, Singapore; School of Biomedical Engineering & Imaging Sciences (M.E.), King's College London; Children's Neurosciences (M.E.), Evelina London Children's Hospital at Guy's and St Thomas' NHS Foundation Trust, United Kingdom; Department of Pediatric Neurology (B.A.), Hacettepe University, Ankara, Turkey; Neuroimmunology Program (T.A.), Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Hospital Clínic, Universitat de Barcelona; Pediatric Neuroimmunology Unit (T.A.), Neurology Department, Sant Joan de Déu (SJD) Children's Hospital, University of Barcelona, Spain; Alberta Children's Hospital Research Institute (S.M.B.), Department of Pediatrics, Cumming School of Medicine, University of Calgary; Division of Rheumatology (T.C.), Department of Pediatrics, McMaster University, Hamilton, Ontario, Canada; Assistance Publique-Hôpitaux de Paris (K.D.), Pediatric Neurology Department, University Hospitals Paris Saclay, Bicêtre Hospital, France; French Reference Network of Rare Inflammatory Brain and Spinal Diseases (K.D.), Le Kremlin Bicêtre, France and European Reference Network-RITA; Departments of Neurology and Pediatrics (W.G.), Stanford University and Lucile Packard Children's Hospital, Palo Alto, CA; Division of Pediatric Neurology (G.G.), Department of Pediatrics, Emory University School of Medicine and Children's Healthcare of Atlanta, GA; Department of Neurology (M.P.G.), Boston Children's Hospital, Harvard Medical School, Boston, MA; Department of Neuroinflammation (Y.H.), Queen Square MS Centre, UCL Institute of Neurology, University College London; Department of Paediatric Neurology (Y.H.), Great Ormond Street Hospital for Children, London, United Kingdom; Department of Pediatrics (Y.J.), Peking University First Hospital, Beijing, China; Department of Pediatrics (B.C.L.), Pediatric Clinical Neuroscience Center, Seoul National University Children's Hospital, Seoul National University College of Medicine, South Korea; Department of Pediatrics (E.M.), Section Rheumatology, Co-appointment in the Section of Neurology and Developmental Neuroscience, Texas Children's Hospital, Baylor College of Medicine, Houston; Division of Paediatric Neurology (A.N.), Department of Paediatrics and Child Health, Red Cross War Memorial Children's Hospital, University of Cape Town; Faculty of Health Sciences (A.N.), University of Cape Town Neuroscience Institute, South Africa; Department of Neurology (R.N.), Erasmus Medical Center, Rotterdam, the Netherlands; Department of Pediatric Neurology (K.R.), Children's Hospital Datteln, University Witten/Herdecke, Germany; Department of Brain and Neural Science (H.S.), Tokyo Metropolitan Institute of Medical Science, Japan; Department of Pediatrics (Neurology Division) (S.S.), Lady Hardinge Medical College and Associated Kalawati Saran Children's Hospital, New Delhi, India; Department of Neurology (S.N.T.), National Pediatric Hospital Dr. J. Garrahan, Buenos Aires, Argentina; Department of Pediatrics (H.A.V.M.), Duke University, Durham, NC; Department of Neurology (E.W.), Children's National Medical Center, Washington, DC; Neuropaediatric Unit (R.W.), Karolinska University Hospital, Stockholm, Sweden; Department of Neurology (A.K.Y.), Icahn School of Medicine at Mount Sinai, New York; Oxford Autoimmune Neurology Group (S.R.I.), Nuffield Department of Clinical Neurosciences, University of Oxford, John Radcliffe Hospital; Department of Neurology (S.R.I.), Oxford University Hospitals NHS Foundation Trust, United Kingdom; Neuroimmunology Program (J.D.), Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Hospital Clínic, University of Barcelona, Spain; Department of Neurology (J.D.), University of Pennsylvania, Philadelphia; Institució Catalana de Recerca i Estudis Avançats (ICREA) (J.D.), Barcelona, Spain; Children's Neurosciences (M.L.), Evelina London Children's Hospital at Guy's and St Thomas' NHS Foundation Trust; King's Health Partners Academic Health Science Centre (M.L.); Faculty of Life Sciences and Medicine (M.L.), King's College Hospital, United Kingdom; and Kids Neuroscience Centre (R.C.D.), The Children's Hospital at Westmead, Faculty of Medicine and Health, University of Sydney, NSW, Australia.

Objective: To create an international consensus treatment recommendation for pediatric NMDA receptor antibody encephalitis (NMDARE).

Methods: After selection of a panel of 27 experts with representation from all continents, a 2-step Delphi method was adopted to develop consensus on relevant treatment regimens and statements, along with key definitions in pediatric NMDARE (disease severity, failure to improve, and relapse). Finally, an online face-to-face meeting was held to reach consensus (defined as ≥75% agreement).

Results: Corticosteroids are recommended in all children with NMDARE (pulsed IV preferred), with additional IV immunoglobulin or plasma exchange in severe patients. Prolonged first-line immunotherapy can be offered for up to 3-12 months (oral corticosteroids or monthly IV corticosteroids/immunoglobulin), dependent on disease severity. Second-line treatments are recommended for cases refractory to first-line therapies (rituximab preferred over cyclophosphamide) and should be considered about 2 weeks after first-line initiation. Further immunotherapies for refractory disease 1-3 months after second-line initiation include another second-line treatment (such as cyclophosphamide) and escalation to tocilizumab. Maintenance immune suppression beyond 6 months (such as rituximab redosing or mycophenolate mofetil) is generally not required, except for patients with a more severe course or prolonged impairments and hospitalization. For patients with relapsing disease, second-line and prolonged maintenance therapy should be considered. The treatment of NMDARE following herpes simplex encephalitis should be similar to idiopathic NMDARE. Broad guidance is provided for the total treatment duration (first line, second line, and maintenance), which is dictated by the severity and clinical course (i.e., median 3, 9 and 18 months in the best, average, and worst responders, respectively). Recommendations on the timing of oncologic searches are provided.

Conclusion: These international consensus recommendations for the management of pediatric NMDARE aim to standardize the treatment and provide practical guidance for clinicians, rather than absolute rules. A similar recommendation could be applicable to adult patients.
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http://dx.doi.org/10.1212/NXI.0000000000001052DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8299516PMC
July 2021

Immunization in multiple sclerosis and other childhood immune-mediated disorders of the central nervous system: A review of the literature.

Eur J Paediatr Neurol 2021 Jun 17;33:125-134. Epub 2021 Jun 17.

Hacettepe University, Faculty of Medicine, Department of Pediatric Neurology, 06230, Ankara, Turkey.

Childhood is a period where most vaccines are administered in order to build-up immunological memory, and immunization against vaccine-preventable diseases is an essential part of child care and health. The administration of vaccines to children with inflammatory diseases is a frequent point of concern for parents and physicians. Published information on the relation between vaccines and autoinflammatory diseases of the central nervous system (CNS) consists of case and cohort studies and reviews, in great majority on adult patients. Vaccines do not have any established causative or triggering effects on these diseases. Another issue is the immunization schedule of patients with autoinflammatory CNS diseases, specifically the interactions between the disorder, the clinical status, the treatment and the vaccine. In this review, we summarize the existing information between autoinflammatory disorders of the CNS and vaccines in childhood and underline the points to be considered under various treatment regimens.
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http://dx.doi.org/10.1016/j.ejpn.2021.06.002DOI Listing
June 2021

Impairment of vestibulo-collic reflex and linear vestibulo-ocular reflex in pediatric-onset multiple sclerosis patients.

Clin Neurophysiol 2021 Aug 24;132(8):1813-1819. Epub 2021 May 24.

Hacettepe University, Faculty of Medicine, Department of Pediatric Neurology, Ankara, Turkey.

Objectives: This study aimed to examine the vestibulo-collic reflex (VCR) and linear vestibulo-ocular reflex (lVOR) and their correlation with brain lesions in pediatric-onset multiple sclerosis (POMS).

Methods: The study group consisted of 17 patients (34 ears) with POMS (mean age 18.73 ± 2.02, mean age at disease onset 14.64 ± 1.36 years), and the control group included 11 age-matched healthy subjects (22 ears). Ocular and cervical Vestibular Evoked Myogenic Potentials (oVEMP and cVEMP) were performed to assess IVOR and VCR pathways. Magnetic Resonance Imaging was evaluated in the study group.

Results: In the POMS group, 47.05 % of oVEMPs and 17.64 % of the cVEMPs were abnormal, while all VEMPs were normal in the control group. The oVEMP amplitude was associated with infratentorial lesion volume (r = -0.459, p = 0.01) and total lesion volume of the brainstem and cerebellum (r = -0.450, p = 0.01). The cVEMP asymmetry ratio was correlated with the deep white matter lesion volume (r = 0.683, p < 0.001). The MVEMP scores were found to correlate only with lesion volumes in the cerebellum (r = 0.488, p = 0.04) and infratentorial region (r = 0.573, p = 0.01).

Conclusions: Ocular and cervical VEMP abnormalities confirm that lVOR and VCR pathways may be affected in early POMS.

Significance: Routine use of the VEMP test, especially the oVEMP test is recommended as a useful tool in the follow-up of POMS patients.
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http://dx.doi.org/10.1016/j.clinph.2021.04.014DOI Listing
August 2021

Etiological and Clinical Profile of Acute Nonbacterial Encephalitis in Children: A Single-Center Prospective Study.

Neuropediatrics 2021 Feb 12. Epub 2021 Feb 12.

Department of Pediatric Neurology, Hacettepe University Medical Faculty, Ankara, Turkey.

Encephalitis is a serious neurological syndrome caused by inflammation of the brain. The diagnosis can be challenging and etiology remains unidentified in about half of the pediatric cases. We aimed to investigate demographic, clinical, laboratory, electroencephalographic and neuroimaging findings, and outcome of acute encephalitis of nonbacterial etiology. This prospective study included children hospitalized with the diagnosis of acute encephalitis between 2017 and 2019. Microbiological investigations of the cerebrospinal fluid (CSF) were recorded. All CSF specimens were tested for anti-N methyl D-aspartate receptor (NMDAR) antibodies. In total, 31 children aged 10 months to 17 years (median = 6 years) were included. Pathogens were confirmed in CSF in three patients (9.7%): varicella zoster virus, herpes simplex virus type 1 (HSV-1), and both HSV-1 and NMDAR antibodies. Presenting features included encephalopathy (100%), fever (80.6%), seizure (45.2%), focal neurological signs (29%), and ataxia (19.4%). On clinical follow-up of median 9 (6-24) months, six patients showed neurological deficits: together with two patients who died in hospital, total eight (25.8%) patients were considered to have unfavorable outcome. Need for intubation, receiving immunomodulatory treatment, prolonged hospitalization, and high erythrocyte sedimentation rate at admission were associated with unfavorable outcome. The etiology of encephalitis remains unexplained in the majority of children. HSV-1 is the most frequently detected virus, consistent with the literature. The fact that anti-NMDAR encephalitis was detected in one child suggests autoimmune encephalitis not being rare in our center. The outcome is favorable in the majority while about one-fifth of cases suffer from sequelae.
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http://dx.doi.org/10.1055/s-0041-1723954DOI Listing
February 2021

Comprehensive clinical, biochemical, radiological and genetic analysis of 28 Turkish cases with suspected metachromatic leukodystrophy and their relatives.

Mol Genet Metab Rep 2020 Dec 11;25:100688. Epub 2020 Dec 11.

Department of Medical Biochemistry, Faculty of Medicine, Hacettepe University, 06230 Ankara, Turkey.

Metachromatic leukodystrophy (MLD) is a glycosphingolipid storage disease caused by deficiency of the lysosomal enzyme arylsulfatase A (ASA) or its activator protein saposin B. MLD can affect all age groups in severity varying from a severe fatal form to milder adult onset forms. Diagnosis is usually made by measuring leukocyte ASA activity. However, this test can give false negative or false positive laboratory results due to pseudodeficiency of ASA and saposin B deficiency, respectively. Therefore, we aimed to evaluate patients with suspected MLD in a Turkish population by comprehensive clinical, biochemical, radiological, and genetic analyses for molecular and phenotypic characterization. We analyzed 28 suspected MLD patients and 41 relatives from 24 families. ASA activity was found to be decreased in 21 of 28 patients. Sixteen patients were diagnosed as MLD (11 late infantile, 2 juvenile and 3 adult types), 2 MSD, 2 pseudodeficiency (PD) and the remaining 8 patients were diagnosed as having other leukodystrophies. Enzyme analysis showed that the age of onset of MLD did not correlate with residual ASA activity. Sequence analysis showed 11 mutations in , of which 4 were novel (p.Trp195GlyfsTer5, p.Gly298Asp, p.Arg301Leu, and p.Gly311Asp), and 2 mutations in causing multiple sulfatase deficiency, and confirmed the diagnosis of MLD in 2 presymptomatic relatives. All individuals with confirmed mutations had low ASA activity and urinary sulfatide excretion. Intra- and inter-familial variability was high for the same missense genotypes, indicating the contribution of other factors to disease expression. Imaging findings were evaluated through a modified brain MRI scoring system which indicated patients with protein-truncating mutations had more severe MRI findings and late-infantile disease onset. MRI findings were not specific for the diagnosis. Anti-sulfatide IgM was similar to control subjects, and IgG, elevated in multiple sulfatase deficiency. In conclusion, the knowledge on the biochemical, clinical and genetic basis of MLD was expanded, a modified diagnostic laboratory algorithm for MLD based on integrated evaluation of ASA activity, urinary sulfatide excretion and genetic tests was devised.
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http://dx.doi.org/10.1016/j.ymgmr.2020.100688DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7734308PMC
December 2020

Analysis of plasma protein biomarkers in childhood onset multiple sclerosis.

J Neuroimmunol 2020 11 8;348:577359. Epub 2020 Aug 8.

Hacettepe University, Faculty of Medicine, Department of Pediatric Neurology, 06230 Ankara, Turkey.

Multiple sclerosis (MS) manifesting before age 18 years is defined as pediatric MS (pMS). We analysed plasma proteins in pMS by an untargeted proteomic approach. Patients with pMS (Group pMS, n = 33), patients with demyelinating disease not meeting pMS diagnostic criteria (unclassified demyelinating disease, Group U, n = 4) and age-matched healthy subjects (Group C, n = 40) were included. Plasma proteomic analysis was performed using Q-TOF LC/MS. Proteins having fold change >1.2 and found to be statistically different (p < 0.05) between the groups were identified and discussed with a clinical perspective. Group pMS had higher alpha 1B glycoprotein (A1BG), complement factor B (CFB), plasminogen (PLG), alpha-2-antiplasmin (α-AP, SERPINF2), inter alpha trypsin inhibitor heavy chain H2 (ITIH2), and lower centrosomal protein of 290 (CEP290) and F-box/LRR-repeat protein 17 (FBXL17) concentrations than Group C. Measurements from Group U, whose definite diagnoses were established as pMS (n = 3) and myelin oligodendrocyte glycoprotein antibody-associated disease (n = 1) on follow-up after the study, were statistically close to the results of Group pMS. Plasma protein changes observed in our study were related to the inflammation, coagulation and oxidative stress pathways. If confirmed and validated in larger groups, these results may indicate potential biomarker(s) for demyelinating diseases at proteome level and could encourage studies for the development of novel diagnostic kits.
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http://dx.doi.org/10.1016/j.jneuroim.2020.577359DOI Listing
November 2020

Aprepitant in the Treatment of Subacute Sclerosing Panencephalitis: A Randomized, Double-Blind, Placebo-Controlled Study.

Pediatr Neurol 2020 09 1;110:59-63. Epub 2020 Jun 1.

Department of Pediatric Neurology, Hacettepe University, Ankara, Turkey.

Background: Aprepitant is a neurokinin-1 receptor antagonist approved for the treatment of chemotherapy-induced nausea. We aimed to investigate the safety and efficacy of aprepitant in patients with subacute sclerosing panencephalitis.

Methods: A randomized, double-blind, placebo-controlled study was conducted in patients with subacute sclerosing panencephalitis assigned to receive two courses of aprepitant 250 mg/day orally or placebo for 15 days with an interval of two months between courses. Primary end points were safety and tolerability, and secondary end point was clinical improvement or stabilization assessed by subacute sclerosing panencephalitis scoring system. Electroencephalography (EEG), brain magnetic resonance imaging, and cerebrospinal fluid measles-specific immunoglobulin G index were evaluated before and after treatment.

Results: Sixty-two patients with subacute sclerosing panencephalitis were allocated to aprepitant (n = 31, median age 18 years) or placebo (n = 31, median age 22 years) group. Fifteen patients left the study within the first six months and 12 patients left between six and 12 months. Aprepitant was well tolerated and treatment-associated adverse events were similar to those described in the treatment of nausea. Clinical status at six and 12 months' follow-up did not differ between aprepitant and placebo groups. Post-treatment EEG scores at 12 months were better in the aprepitant group (P = 0.015). Cerebral atrophy on magnetic resonance imaging increased in both groups, whereas measles-specific immunoglobulin G index decreased in the placebo group.

Conclusions: In this first clinical trial of aprepitant treatment in patients with subacute sclerosing panencephalitis, the drug was safe and well tolerated. No clinical effect was observed. A modest improvement in EEG findings might justify trials for longer periods because EEG changes can precede clinical findings in subacute sclerosing panencephalitis.
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http://dx.doi.org/10.1016/j.pediatrneurol.2020.05.009DOI Listing
September 2020

Vestibulo-ocular reflex involvement in childhood-onset multiple sclerosis.

Mult Scler Relat Disord 2020 Sep 22;44:102329. Epub 2020 Jun 22.

Hacettepe University, Faculty of Health Sciences, Department of Audiology, Ankara, Turkey; Hacettepe University, Dizziness and Balance Disorders Research and Application Center, Ankara, Turkey.

Background: Multiple Sclerosis (MS), an autoimmune demyelinating disease of the central nervous system, is an important cause of disability in young adults. The purpose of this cross-sectional study was to evaluate the vestibular system with video Head Impulse Test (vHIT) and determine the impairment of the Vestibulo-ocular Reflex (VOR) in childhood-onset MS.

Methods: The study group, 20 persons with MS (pwMS) with onset before 18 years of age (6 M, 14 F; mean age 19.06 ± 1.66) and the control group, 20 healthy, age- and sex-matched individuals were retrieved from vHIT recordings. The mean age of MS onset in the study group was 14.60 ± 1.53 years. The VOR pathway was evaluated using vHIT.

Results: The median VOR gains of right anterior (1.00), left lateral (0.96) and left posterior (0.91) semicircular canals were significantly lower in the pwMS group than those of the healthy control group (1.05, 1.00, 0.98 respectively, p < 0.05). Four of pwMS (20%) had abnormal VOR gains. The pwMS with dizziness had significantly lower VOR gains (median 0.91) compared with pwMS without dizziness (median 1.01, p < 0.05).

Conclusion: This study demonstrates vestibulo-ocular system can be affected in patients with childhood-onset MS and suggests using vHIT especially in the follow-up of pwMS with dizziness.
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http://dx.doi.org/10.1016/j.msard.2020.102329DOI Listing
September 2020

Pontine Tegmentum Lesions Accompanying Myelitis During an Enterovirus Outbreak: Differential Diagnosis and Outcome.

J Child Neurol 2020 07;35(8):501-508

Department of Pediatric Neurology, Ihsan Doğramaci Children's Hospital, Faculty of Medicine, Hacettepe University, Ankara.

Aim: To investigate etiology and prognostic significance of pontine tegmentum lesions accompanying a cluster of acute flaccid myelitis.

Method: We retrospectively examined patients from 6 centers in Turkey who manifested encephalitis or myelitis associated with dorsal pontine lesions on magnetic resonance imaging (MRI) between July 2018 and February 2019.

Results: Twenty-two patients were evaluated. Ten of 22 (45%) presented with acute paralysis and 12 of 22 (55%) with brainstem symptoms only. Reverse transcription polymerase chain reaction for enterovirus was positive in 2 patients' respiratory tract. Other etiologic factors were detected in 10 cases. On follow-up, patients presenting with symptoms of myelitis developed motor sequalae although spinal cord lesions on MRI resolved in 5 of 9 (55%). Encephalitic symptoms, present in 17 cases, recovered in 13 (76%), and brain MRI showed complete or near-complete resolution in 11 of 14 (78%).

Conclusion: Various etiologic agents can be detected in patients with pontine involvement, even in a series collected during an outbreak of EV-D68. Encephalitis has a fair outcome but clinical recovery is slow and motor sequalae are frequent in spinal involvement, irrespective of follow-up spinal MRI findings.
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http://dx.doi.org/10.1177/0883073820911737DOI Listing
July 2020

Gross motor development of preschool children: effects of socioeconomic status and maternal education.

Turk J Pediatr 2020 ;62(1):10-18

Departments of Pediatric Neurology, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Motor development reflects the general health status of the child and affects other areas of development. It is influenced by biological and family characteristics especially in infancy and early childhood, and by environmental conditions in preschool age. We assessed the effect of several family and environment characteristics on gross motor developmental items included in the Denver-II test on 2,042 healthy children. Increasing maternal age and education were associated with later achievement in several items after age 12 months while socioeconomic status, sex and birth rank did not show a clear effect. Our observations suggest in a relatively homogenous urban population, few external factors affect gross motor development in preschool children.
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http://dx.doi.org/10.24953/turkjped.2020.01.002DOI Listing
January 2020

Acute Cerebellitis or Postinfectious Cerebellar Ataxia? Clinical and Imaging Features in Acute Cerebellitis.

J Child Neurol 2020 05 12;35(6):380-388. Epub 2020 Mar 12.

Department of Pediatric Neurology, Hacettepe University Ihsan Dogramaci Children's Hospital, Ankara, Turkey.

Acute cerebellitis is a rare condition often considered within the group of acute postinfectious cerebellar ataxia despite its distinctive clinical and imaging features. We retrieved clinical, laboratory, and follow-up data of 15 children diagnosed with acute cerebellitis in our department between 2011 and 2019. There were 10 boys and 5 girls aged 3-15 years, median 9.5 years. The most common first symptoms were ataxia, vomiting, and headache. Magnetic resonance imaging (MRI) generally showed bilateral symmetrical T2 hyperintense changes with moderate swelling in the cerebellar cortex. Tonsillar herniation was present in 73.3% and obstructive hydrocephalus in 26.6%. Etiologic workup for infectious pathogens revealed , influenza A virus, cytomegalovirus, and varicella zoster virus in 1 case each. Fourteen of 15 patients were treated with intravenous and/or oral steroids and 8 cases with intravenous immunoglobulin. No patient required surgical decompression. Neurologic examination median 12 months later revealed ataxia and dysmetria in 4 cases (27%), accompanied by memory difficulties, dysarthria or tremor. Follow-up magnetic resonance imaging (MRI; n = 12) showed diffuse cerebellar cortical T2-hyperintense signal changes in 11 cases and cerebellar atrophy in 9. The diagnosis of acute cerebellitis rather than acute postinfectious cerebellar ataxia should be considered when headache and vomiting accompany ataxia in a child. Acute cerebellitis heals with sequelae in about one-third of cases. The absence of fatalities in our series suggests early diagnosis, and steroid treatment can increase the chance of recovery. MRI results were not found to be predictive of outcome.
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http://dx.doi.org/10.1177/0883073820901407DOI Listing
May 2020

Corrigendum to "Subacute sclerosing panencephalitis and immune thrombocytopenia: More than a coincidence?" [Med. Hypotheses 111 (2018) 70-72].

Med Hypotheses 2020 May 20;138:109619. Epub 2020 Feb 20.

Hacettepe University Faculty of Medicine, Department of Pediatric Neurology, Ankara, Turkey.

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http://dx.doi.org/10.1016/j.mehy.2020.109619DOI Listing
May 2020

Cognitive functions in pediatric multiple sclerosis: 2-years follow-up.

Neurol Res 2020 Feb 8;42(2):159-163. Epub 2020 Jan 8.

Department of Pediatric Neurology, Hacettepe University Faculty of Medicine, Ankara, Turkey.

: To assess the neuropsychological status of pediatric multiple sclerosis (MS) patients and its relationship with clinical variables in a longitudinal study.: Patients with MS (n = 46) and age- and gender-matched healthy control subjects (HCs, n = 53) were given tests of non-verbal reasoning, attention/concentration, visuospatial judgement and verbal fluency at baseline visit and after 2 years of follow-up. Cognitive impairment was defined as a failure on at least three of the four tests. Patients were grouped according to the age of disease onset (≤12 years as group 1 and > 12 years as group 2).: Cognitive impairment was detected in 22 of 46 patients at follow-up (47.8%). Patients with cognitive worsening had higher EDSS scores at follow-up compared to cognitively improved/stable group (0.68 ± 1.16 vs 0.04 ± 0.2, p = 0.01). The most affected domains were attention/concentration and non-verbal reasoning. Comparison between baseline and follow-up tests showed impairment in non-verbal reasoning over time in group 1 patients while other functions improved over time in patient and control groups as expected.: Pediatric MS is likely to affect patients' cognition concurrently with their disability levels. This effect is significant in the non-verbal reasoning area in patients with disease onset before age 12 years. A practical method assessing this function should be part of these patients' regular follow-up for optimal treatment, prevention and rehabilitation.
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http://dx.doi.org/10.1080/01616412.2019.1710417DOI Listing
February 2020

Recurrent Demyelinating Episodes as Sole Manifestation of Inherited CD59 Deficiency.

Neuropediatrics 2020 06 21;51(3):206-210. Epub 2019 Nov 21.

Department of Pediatric Neurology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.

Defects in the regulatory components of the complement system can lead to inflammatory diseases. We present a patient who had four episodes of demyelination in the central nervous system as the only manifestation of inherited CD59 deficiency. Relapsing encephalopathy partially responsive to intravenous immunoglobulin and steroid treatments on the background of parental consanguinity suggested an inherited immune dysregulation. Next generation sequencing revealed homozygous mutation in the CD59 gene, confirmed by lack of CD59 expression on flow cytometry. Inherited CD59 deficiency is a rare autosomal recessive condition characterized by chronic hemolysis, recurrent strokes, and relapsing peripheral demyelinating neuropathy mimicking Guillain-Barré syndrome or chronic inflammatory demyelinating polyneuropathy. Recurrent central nervous system demyelinating episodes as the only manifestation has not been reported to date in inherited CD59 deficiency. This entity should be considered in the differential diagnosis of patients with early-onset recurrent neurological diseases with central or peripheral origin.
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http://dx.doi.org/10.1055/s-0039-3399583DOI Listing
June 2020

Elevated quinolinic acid levels in cerebrospinal fluid in subacute sclerosing panencephalitis.

J Neuroimmunol 2020 02 18;339:577088. Epub 2019 Oct 18.

Department of Pediatrics, Yamaguchi University Graduate School of Medicine, Yamaguchi 755-8505, Japan.

Subacute sclerosing panencephalitis (SSPE) is a rare neurodegenerative disorder caused by a persistent infection with aberrant measles virus. Indoleamine-2, 3-dioxygenase (IDO) initiates the increased production of kynurenine pathway (KP) metabolites quinolinic acid (QUIN), which has an excitotoxic effect for neurons. We measured serum IDO activity and cerebrospinal fluid (CSF) levels of QUIN. The CSF QUIN levels were significantly higher in SSPE patients than in controls, and increased according as neurological disability in a patient studied. Elevation of CSF QUIN and progression of SSPE indicate a pathological role of KP metabolism in the inflammatory neurodestruction.
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http://dx.doi.org/10.1016/j.jneuroim.2019.577088DOI Listing
February 2020

Measles.

N Engl J Med 2019 11;381(20):1978

Hacettepe University, Ankara, Turkey

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http://dx.doi.org/10.1056/NEJMc1912468DOI Listing
November 2019

A clinical score to guide in decision making for monogenic type I IFNopathies.

Pediatr Res 2020 03 22;87(4):745-752. Epub 2019 Oct 22.

Division of Rheumatology, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Objective: To develop a set of clinical criteria that identifies patients with a potential autoinflammatory IFNopathy.

Methods: Based on a literature review, a set of clinical criteria identifying genetically confirmed monogenic IFNopathies was selected. For validation, the clinical score was assessed in healthy controls (HCs) and 18 disease controls, including 2 known autoimmune IFNopathies, juvenile systemic lupus erythematosus (JSLE, n = 4) and dermatomyositis (JDM, n = 4); adenosine deaminase 2 deficiency (DADA2, n = 4); and oligoarticular juvenile idiopathic arthritis (oJIA, n = 6). We assessed an IFN score (IRG-S) in whole blood by NanoString using a previously published 28-gene-IRG-S and a reduced 6-gene-IRG-S.

Results: The 12 patients with a possible IFNopathy had higher clinical scores (3-5) than the patients with sJLE, JDM, DADA2, and oJIA and in HCs. Both the 28-IRG-S and 6-IRG-S were significantly higher in the autoinflammatory IFNopathy patients compared to HCs and oJIA and DADA2 patients but not different from patients with JSLE and JDM. Subsequently, genetic analysis revealed mutations in genes previously reported in genes related to the IFN pathway in 9 of the 12 patients.

Conclusion: We developed a clinical score to identify patients with possible autoinflammatory IFNopathies. A clinical score was associated with a high IRG-S and may serve to identify patients with an autoinflammatory IFNopathy.
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http://dx.doi.org/10.1038/s41390-019-0614-2DOI Listing
March 2020

Early neurological complications in children with classical galactosemia and p.gln188arg mutation.

Int J Dev Neurosci 2019 Nov 20;78:92-97. Epub 2019 Jul 20.

Prof. Dr. Specialist in Pediatric Neurology, Department of Pediatrics, Division of Pediatric Neurology, Hacettepe University Children's Hospital, Ankara, Turkey.

Background: Despite implementation of a controlled diet, children with classical galactosemia (CG) may develop a variety of developmental and cognitive problems. In this study, we examined the early developmental status of, as well as the neurological and neuroradiological findings for, children with CG.

Methods: We retrospectively evaluated 46 galactosemia patients who were followed between 2003 and 2017. We included those who exhibited CG and p.gln188arg homozygous mutation without concomitant disease and who had undergone detailed neurological examination, brain magnetic resonance imaging (MRI), and Denver II developmental testing.

Results: The mean ages at the time of the most recent neurological examination and Denver II testing were 48.5 ± 28.5 months and 34.4 ± 18.2 months, respectively. Developmental delay was defined as developmental age ≥ 20% lower than chronological age. The results were normal in 25 patients and delayed ≥ 20% in least in one domain, primarily in language development, in 21 patients. Brain MRI was abnormal in 22 patients.

Conclusions: This analysis of the youngest children with the same genetic mutation reported thus far showed that, despite treatment, developmental delays and abnormalities on brain MRI may begin at an early age.
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http://dx.doi.org/10.1016/j.ijdevneu.2019.07.004DOI Listing
November 2019

Epilepsy in neurofibromatosis type 1: Diffuse cerebral dysfunction?

Epilepsy Behav 2019 09 9;98(Pt A):6-9. Epub 2019 Jul 9.

Hacettepe University, Faculty of Medicine, Department of Child Neurology, Ankara, Turkey. Electronic address:

Introduction: Neurofibromatosis type 1 (NF1) is accompanied by epileptic seizures in 4-7% of patients. We examined clinical, electrophysiological, and radiological features associated with epilepsy in our NF1 series in order to identify risk factors.

Methods: We reviewed data of 641 pediatric patients with NF1 diagnosis according to National Institutes of Health (NIH) criteria in Hacettepe University records from January 2008-August 2018. Demographic features, NF1-related clinical and imaging characteristics, age at onset of epilepsy, seizure semiology, and frequency, electroencephalogram (EEG) findings, and response to treatment were noted.

Results: Twenty-six patients with NF1, 15 male, 11 female, had epilepsy. Age at seizure onset was 6 months to 13 years. Seizure semiology was focal with impaired awareness (n = 9, 34%), focal aware motor (n = 2, 8%), focal to bilateral tonic-clonic (n = 3, 12%), generalized tonic-clonic (n = 7, 28%), absence (n = 3, 12%), infantile spasms (n = 1), and unclassified type (n = 1). None had a history of status epilepticus. The EEG findings were normal for age in ten patients (38%). Others had focal (n = 8, 30%), generalized (n = 7, 27%), or multifocal (n = 1, 4%) discharges. On brain magnetic resonance imaging (MRI) signal intensity changes typical for NF1 (neurofibromatosis bright objects, NBOs) were the most common finding (80%), followed by normal MRI (20%). There was no relation between the localization of NBOs and discharges on EEG. Seventeen patients (65%) were seizure-free at the time of the study; 11 of them still under medication including four on multiple antiepileptic drugs. The rate of learning problems and NBO were significantly higher in patients with NF1 with epilepsy compared to those without.

Discussion: Epilepsy in NF1 is associated with relatively infrequent seizures and good response to treatment. Learning disorders are markedly frequent in this group, irrespective of the severity of epilepsy. The absence of correlation between the localizations of epileptiform discharges and lesions on MRI support the role of cellular or synaptic mechanisms rather than structural causes in the pathogenesis of epilepsy.
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http://dx.doi.org/10.1016/j.yebeh.2019.06.022DOI Listing
September 2019

Two sisters with anti-MuSK-positive myasthenia gravis.

Clin Neurol Neurosurg 2019 07 21;182:17-18. Epub 2019 Apr 21.

Hacettepe University Faculty of Medicine, Department of Pediatric Neurology, Ankara, Turkey.

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http://dx.doi.org/10.1016/j.clineuro.2019.04.011DOI Listing
July 2019

Treatment in childhood central nervous system demyelinating disorders.

Dev Med Child Neurol 2019 11 16;61(11):1281-1288. Epub 2019 Apr 16.

Department of Pediatric Neurology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.

The last two decades witnessed significant advances in the treatment of acquired demyelinating disorders: thirteen new agents have been approved for the treatment of multiple sclerosis in adults by the European Medicines Agency and US Food and Drug Administration in the last twenty years. Although the long-term efficacy and safety profiles of some new drugs are still being assessed in paediatric MS, clinicians may have to use them in the management of paediatric onset MS resistant to first-line medications, based on results obtained in adult-onset disease. This review summarizes the current approach to treatment in children with demyelinating syndromes. WHAT THIS PAPER ADDS: Serological markers affect management in paediatric demyelinating diseases. Antibodies against aquaporin-4 and myelin oligodendrocyte glycoprotein should be tested in children with acute demyelinating disease. New therapeutic agents currently in trial for pediatric disease should be used with close follow-up.
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http://dx.doi.org/10.1111/dmcn.14228DOI Listing
November 2019

Cardiac autonomic function evaluation in pediatric and adult patients with congenital myasthenic syndromes.

Neuromuscul Disord 2019 04 19;29(4):290-295. Epub 2019 Feb 19.

Department of Pediatric Neurology, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Cardiac autonomic dysfunction has been examined in myasthenia gravis but not in congenital myasthenic syndromes (CMS). We aimed to evaluate cardiac autonomic functions in genetically defined CMS. Patients diagnosed with and under treatment for CMS were reviewed for 24-hour cardiac rhythm monitoring. Heart rate variability (HRV) measures were defined as: SDNN, mean of the standard deviations for all R-R intervals; SDNNi, standard deviation of all R-R intervals in successive five-minute epochs; RMSSD, square root of the mean of squared differences between successive R-R intervals. Ten patients with mutations in the epsilon subunit of the acetylcholine receptor (AChRε) and five patients with mutations in the collagen-like tail of asymmetric acetylcholinesterase (ColQ) were included. Median age at evaluation was 17 (2.5-46) years. In the AChRε group, RMSSD values; and in the ColQ group, SDNN, SDNNi and RMSSD values were significantly lower than those of healthy subjects. This first extensive report examining HRV in CMS showed alterations in patients with ColQ mutations and, to a lesser extent, in the group with AChRε mutations. This might indicate an increased risk of cardiac arrhythmias. We suggest cardiological follow-up in CMS, and consideration of any potential cardiovascular effects of therapeutic agents used in management.
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http://dx.doi.org/10.1016/j.nmd.2019.02.004DOI Listing
April 2019

Concurrence of juvenile idiopathic arthritis and primary demyelinating disease in a young child.

Mult Scler Relat Disord 2019 Jan 3;27:20-22. Epub 2018 Oct 3.

Department of Pediatric Neurology, Hacettepe University Ihsan Dogramaci Children's Hospital, Ankara, Turkey.

Case Report: The association of juvenile idiopathic arthritis (JIA) and primary demyelinating disease of central nervous system (CNS) in the same patient is rare. Here we present a 10-year-old girl formerly diagnosed with JIA who presented with acute total vision loss. Magnetic resonance imaging of the brain and spinal cord showed bilateral optic neuritis and T2 hyperintense lesions in the brain, cerebellum and cervical spinal cord, some of them gadolinium-enhancing. Oligoclonal bands were present in the cerebrospinal fluid. Visual evoked potentials were prolonged. Aquaporin-4 antibodies were negative. The patient was treated with methylprednisolone 30 mg/kg daily for five days, resulting in improvement in vision and gait. This first demyelinating event in this patient with JIA with clinical and paraclinical features meeting the 2017 MS diagnostic criteria supports a possible predisposition to autoimmune disorders.

Conclusion: The concurrence of JIA and multiple sclerosis (MS) has been reported in only two adult cases and not in the pediatric population. While JIA and MS are two distinct chronic inflammatory diseases, immunogenetic predisposition and common environmental triggers might be involved.
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http://dx.doi.org/10.1016/j.msard.2018.10.002DOI Listing
January 2019

Electrophysiological Subtypes and Prognostic Factors of Childhood Guillain-Barré Syndrome.

Noro Psikiyatr Ars 2018 Sep 5;55(3):199-204. Epub 2018 Jun 5.

Department of Pediatric Neurology, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Introduction: We assessed the clinical, epidemiologic, electrophysiological and prognostic characteristics of childhood Guillain-Barré Syndrome admitted to 13 pediatric neurology centers in Turkey.

Method: Using a standard data recording form age, sex, duration of symptoms, distribution of weakness at onset, cranial nerve involvement, cerebrospinal fluid findings, electrophysiological findings, duration of hospitalization, requirement of ventilation, treatment and clinical evaluation scale at onset, discharge and 1, 3, 6, and 12 months after discharge were recorded.

Results: Among the 236 children with a median age of 6.8 years there was a male to female ratio of 1.3. Based on the electrophysiological features; 84 patients were classified as acute inflammatory demyelinating polyrediculoneuropathy (AIDP), 61 as acute motor axonal neuropathy (AMAN), 21 as acute motor-sensory axonal neuropathy (AMSAN). The incidence of cranial nerve involvement was 16%, and was related to lower clinical scores at discharge and 6 months after discharge. Clinical scale scores between axonal and demyelinating subgroups did not show statistically significant difference except for admission (p<0.05).

Conclusion: Electrophysiological subtypes are not important in prognosis in our series. However, duration of weakness, duration of hospitalization and ventilation requirement can affect prognosis negatively.
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http://dx.doi.org/10.5152/npa.2017.16996DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6138236PMC
September 2018

Retrospective analysis of children with myelin oligodendrocyte glycoprotein antibody-related disorders.

Mult Scler Relat Disord 2018 Nov 10;26:1-7. Epub 2018 Sep 10.

Department of Pediatric Neurology, Hacettepe University Ihsan Dogramaci Children's Hospital, Ankara, Turkey. Electronic address:

Background: Knowledge has been expanding on myelin oligodendrocyte glycoprotein (MOG) antibody-associated central nervous system disorders. We delineate the clinical and paraclinical findings and outcome of our pediatric patients with MOG antibody seropositive disease.

Methods: We retrospectively analyzed the clinical presentation, cerebrospinal fluid findings, magnetic resonance imaging (MRI) studies, course and outcome of children seropositive for anti-MOG IgG.

Results: Total 20 children with neurological symptoms and serum anti-MOG IgG were identified from six centers in Turkey. Median age at onset was 9 years (mean 8.8 ± 5.0 years, range: 1.5-16.5 years). Final diagnoses were acute disseminated encephalomyelitis (ADEM) (n = 5), ADEM + optic neuritis (n = 4), neuromyelitis optica spectrum disorder (NMOSD) (n = 3), myelitis (n = 2), relapsing optic neuritis (n = 2), multiphasic DEM (n = 3), and unclassified relapsing demyelinating disease (n = 1). Seven/20 (35%) children experienced a single episode while 13/20 (65%) had a least one relapse during follow-up. On MRI, subcortical white matter, brainstem, and corpus callosum were preferentially involved regions. Full recovery was observed in 15/20 (75%) children.

Conclusion: MOG autoimmunity in children has a wide clinical spectrum, tendency to relapse, and a favourable outcome compared with other relapsing demyelinating diseases.
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http://dx.doi.org/10.1016/j.msard.2018.07.022DOI Listing
November 2018

Heightened CXCR4 and CXCL12 expression in NF1-associated neurofibromas.

Childs Nerv Syst 2018 05 17;34(5):877-882. Epub 2018 Feb 17.

Department of Medical Biology and Genetics, Faculty of Medicine, TOBB University of Economics and Technology, Ankara, Turkey.

Background: Neurofibromatosis type 1 (NF1) is a common autosomal dominantly inherited disorder that affects both the skin and the nervous system. NF1 occurs due to the mutations in the NF1 gene. Neurofibromas are the most common Schwann cell-based tumors in NF1 patients, which are mainly categorized into dermal and plexiform neurofibromas. Studies on different tumor types demonstrate that CXCR4 expression increases in tumor tissues and is linked to metastasis and cancer progression.

Purpose: In the present study, we aimed to analyze the gene expression of CXCR4, and its ligand CXCL12, in human neurofibromas.

Methods: Eight NF1 patients aged between 5 and 37 (2 males, 6 females) were selected. The patient group comprised 1 plexiform neurofibroma, 1 pheochromocytoma, and 6 dermal neurofibromas. Following pathological examination and diagnosis, tumors were co-stained with antibodies against Schwann cell marker S100 and target molecule CXCR4. CXCR4 expression in Schwann cell-based tumors was detected at the protein level. RNA isolated from the same tumors was used for RT-PCR-based studies to measure the quantitative expression of CXCR4 and CXCL12.

Results: CXCR4 gene expression increased 3- to 120-fold and CXCL12 gene expression increased 33- to 512-fold in all human Schwann cell-based tumors.

Conclusion: In order to validate the role of CXCR4 and its relationship with CXCL12 in NF1, future studies should be performed with additional tumors and different tumor types.
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http://dx.doi.org/10.1007/s00381-018-3745-6DOI Listing
May 2018

Neurologic Involvement in Primary Immunodeficiency Disorders.

J Child Neurol 2018 04 8;33(5):320-328. Epub 2018 Feb 8.

1 Department of Pediatric Neurology, Hacettepe University Ihsan Dogramaci Children's Hospital, Ankara, Turkey.

The nervous system may be affected in primary immune deficiency (PID) syndromes through infectious, autoimmune, neoplastic mechanisms, or as a primary feature of the syndrome. However certain neurologic problems do not conform to these etiopathogenetic groups. We retrospectively examined PID patients seen in neurology consultation between 2014 and 2017 in order to determine the spectrum of nervous system involvement. Among patients with confirmed neurologic problems (n = 35), common manifestations were encephalopathy and global developmental/cognitive delay. In 13 (37%) instances, the neurologic signs had no apparent relation with a treatment-related, infectious, or vascular complication and were considered as primary findings: acquired microcephaly, central nervous system malformation, or peripheral neuropathy. The diagnosis of PID was made after, and based on, the neurologic manifestation in 6 of 35 (17%) patients. Neurologic presentation may constitute the initial manifestation in some types of primary immune deficiency.
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http://dx.doi.org/10.1177/0883073817754176DOI Listing
April 2018

Disease Course and Treatment Responses in Children With Relapsing Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease.

JAMA Neurol 2018 04;75(4):478-487

Children's Neurosciences, Evelina London Children's Hospital at Guy's and St Thomas' National Health Service Foundation Trust, King's Health Partners Academic Health Science Centre, London, United Kingdom.

Importance: Myelin oligodendrocyte glycoprotein antibodies (MOG-Abs) are consistently identified in a range of demyelinating disorders in adults and children. Current therapeutic strategies are largely center specific, and no treatments have been formally evaluated.

Objective: To examine the clinical phenotypes, treatment responses, and outcomes of children with relapsing MOG-Ab-associated disease.

Design, Setting, And Participants: This study prospectively collected demographic, clinical, and radiologic data from 102 patients from 8 countries of the EU Paediatric Demyelinating Disease Consortium from January 1, 2014, through December 31, 2016. Patients were treated according to local protocols.

Main Outcomes And Measures: Annualized relapse rates (ARRs) and Expanded Disability Status Scale (EDSS) scores before and during treatment with disease-modifying drugs (DMDs).

Results: A total of 102 children were identified (median [range] age, 7.0 [1.5-7.9] years; male to female ratio, 1.0:1.8; white to other race/ethnicity ratio, 3.6:1.0). Original diagnoses were neuromyelitis optica spectrum disorder (44 patients [43.1%]), acute disseminated encephalomyelitis followed by optic neuritis (20 [19.6%]), multiphasic disseminated encephalomyelitis (20 [19.6%]), and relapsing optic neuritis (18 [17.6%]). In all, 464 demyelinating events were reported. Treated patients had more relapses (median, 3.0; range, 1.0-17.0) than untreated patients (median, 1.0; range 1.0-7.0) (P = .009) and higher EDSS scores (median, 1.5; interquartile range, 0-2.5) than untreated patients (median, 1.0; interquartile range, 0-1.5) (P < .001). Fifty-two children (51.0%) received DMDs: 28 (53.8%) were treated with 1 DMD, 17 (32.7%) with 2, and 7 (13.5%) with 3 or more sequential DMDs. Patients relapsed during all treatments, with a total of 127 relapses on treatment reported. No changes in median ARR and EDSS score were observed between the preinitiation and postinitiation phases of interferon beta and glatiramer acetate treatment (n = 11). The median ARR was reduced from 1.84 to 1.0 with azathioprine (n = 20, P < .001), 1.79 to 0.52 with mycophenolate mofetil (n = 15, P = .003), and 2.12 to 0.67 with rituximab (n = 9, P < .001), although the median EDSS score remained unchanged. An improvement in ARR (from 2.16 to 0.51, P < .001) and EDSS score (from 2.2 to 1.2, P = .01) was observed in the 12 patients treated with regular intravenous immunoglobulins.

Conclusions And Relevance: Although commonly used to treat patients with multiple sclerosis, DMDs were not associated with clinical improvement in children with MOG-Ab-associated disease, whereas azathioprine, mycophenolate mofetil, rituximab, and particularly intravenous immunoglobulins were associated with a reduction in relapse frequency. A correct diagnosis of relapsing MOG-Ab-associated disorders is therefore important to optimize immune treatment.
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http://dx.doi.org/10.1001/jamaneurol.2017.4601DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5885190PMC
April 2018

Changes of primary headache related white matter lesions in pediatric patients.

Turk J Pediatr 2018 ;60(4):380-384

Division of Pediatric Neurology, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Bayram E, Yiş U, Paketçi C, Okur D, Polat İ, Çakmakcı H, Hız S, Anlar B. Changes of primary headache related white matter lesions in pediatric patients. Turk J Pediatr 2018; 60: 380-384. We aimed to describe the long-term prognosis of white matter lesions detected on magnetic resonance imaging in children with primary headache. Children who were admitted with the complaint of headache and had nonspecific white matter lesions on magnetic resonance imaging were included in the study. The clinical findings of the patients were reinvestigated using the same magnetic resonance imaging scanner and acquisition protocol after at least a two year period. Magnetic resonance imaging results of the patients were documented in detail. Findings of the baseline and follow-up studies were compared with each other by the same radiologist. Among the 11 patients, 8 ( 72.7%) were male and 3 (27.3%) were female. Mean age of patients at the time of second imaging was 12.9±2.3 years. Eight (72.7%) had migraine without aura, 1 (9.1%) had tension-type headache and 2 (18.2%) had migraine with aura. The mean clinical follow-up period of the patients was 4.31±1.31 years. All patients had low headache frequency on the last control visit when compared to the first clinical findings. The follow-up magnetic resonance imaging studies showed two newly developed white matter lesions in two patients who had migraine without aura and the white matter lesions disappeared in the patient who had tension-type headache, compared to the baseline neuroimaging. Findings of the baseline and long-term follow-up magnetic resonance imaging studies of the patients with primary headache showed no significant changes in terms of the location, size and laterality. Repeated neuro-imaging studies are not warranted in the absence of the progression in clinical findings.
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http://dx.doi.org/10.24953/turkjped.2018.04.004DOI Listing
May 2019

Multiple Sclerosis with Onset Younger Than 10 Years in Turkey.

Neuropediatrics 2018 02 28;49(1):51-58. Epub 2017 Nov 28.

Department of Pediatric Neurology, Hacettepe University, Faculty of Medicine, Ankara, Turkey.

Objective: To identify the demographics, clinical characteristics, disease course, treatment patterns, and disability levels of multiple sclerosis (MS) patients with onset under the age of 10 years (early onset multiple sclerosis, EOMS).

Methods: EOMS patients were reviewed retrospectively in detailed records from 27 child neurology centers. Patients with preschool (≤7 years) and school age (>7 years) onset were compared.

Results: There were 30 children (16 girls, 14 boys) who have disease onset between 4 and 10 (mean8.1 ± 1.8) years. MS was relapsing-remitting in 29 (96.7%) and primary progressive in one (3.3%) of the patients. In patients with onset ≤7 years, motor symptoms (54.5%) and encephalopathy (45.5%) predominated, while in those with onset >7 years brainstem (42.1%), sensory (26.3%), and optic nerve (26.3%) involvement were the most frequent presentations.

Conclusions: MS starting ≤7 years differs from the 7-10-year-old group by the higher rate of motor symptoms and more attacks in the first year: the latter suggests a more inflammatory character for EOMS.
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http://dx.doi.org/10.1055/s-0037-1608776DOI Listing
February 2018
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