Zeynep Öztürk

Multiple Sclerosis with Onset Younger Than 10 Years in Turkey

Abstract 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.

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

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.

Internal carotid artery dissection without intracranial infarct following a minor shoulder trauma: The second pediatric case and review of the literature

INTRODUCTION Carotid artery dissections may occur in severe trauma such as motor vehicle accidents or may also develop due to minor trauma. We aimed to present a case with internal carotid artery dissection that referred to the pediatric neurology department due to speech impairment after minor shoulder trauma. CASE A previously healthy 10-year-old male patient was admitted to the pediatric emergency clinic due to headache, vomiting and speech impairment. In his story, we learned that he had bumped shoulder to shoulder with his friend about 6 h ago. He did not fall or hit his head. On his admission he could not speak and had right central facial paralysis. There was no infarct or diffusion limitation in MRI but MR angiography showed thinning in left internal carotid artery calibration. Fat-suppressed, non-contrast T1-weighted MRI showed that the left carotid artery had ring-shaped pathological signal changes. Low-molecular-weight heparin therapy was initiated with the diagnosis of carotid artery dissection (CAD). No hemiparesis or hemiplejia occurred in the follow-up of the patient. Within a few days, his speech improved. At the end of the first month, facial paralysis completely recovered. CONCLUSION In carotid artery dissections, prodromal symptoms such as transient ischemic attack, like in our patient, are rarely present in children. For good long term outcomes, it is very important to suspect, diagnose and initiate appropriate treatment in a rapid manner in carotid artery dissection before severe neurological findings such as acute ischemic stroke develops.

Reply to “Childhood Peripheral Facial Palsy”

Dear Editor: We would like to thank you for the opportunity to respond to the issues raised in Dr. Calık’s letter and to clarify their concerns. We would also like to thank Dr. Calık and his colleague at Harran University for their interest in our paper and for their valuable input to express their concerns. In our study, we evaluated 144 childhood peripheral facial palsy patients by their sex, family history, grading, and disease outcomes in both idiopathic (Bell’s palsy) and cause-defined facial palsy. At the end of the first year, our recovery rates were 98.3%. In the Bell’s palsy group, no significant difference in recovery outcome was detected between the patients who were treated with or without steroid treatment [1]. We agree that the number of the patients without steroid treatment was lower than that of the patients with steroid treatment in our study; however, this discrepancy did not create a statistical error in comparison analysis. Statistical analysis performed in our study was carefully designed and checked before publication. The main agents used for the treatment of Bell’s palsy are steroids. In the literature, studies with adults support the usage of steroid treatment especially in the early period (i.e., within 72 h) [2–4]. Despite the conclusive evidence of benefits from steroids in adults with Bell’s palsy, childhood studies with the use of steroids to treat Bell’s palsy were not placebo-controlled trials [5, 6]. There was no evidence that supports the usage of steroid treatment in childhood Bell’s palsy [6, 7]. Unüvar et al. randomized trial of steroid use in children with Bell’s palsy from Turkey found no difference at the end of the 12-month follow-up in both treated and non-treated groups [8]. Medical conditions often manifest differently in childhood who also have different responses to treatment when compared to adults; therefore, the findings of adult studies using steroids to treat Bell’s palsy are difficult to extrapolate to children. Indeed, the primary objective of our study was to compare the outcomes of the patients with Bell’s palsy to the patients with definitive etiology in childhood peripheral facial palsy. Our study had many informative results for the literature. This study had one of the largest numbers of childhood peripheral palsy patients. One of the strongest findings of our study was evaluating patients for a long follow-up period and determining the childhood peripheral palsy recurrent attack periods and its prognosis. In our study, we did not mention about the steroid treatment effects in the patients who have recurrent attacks. If we tried to give the results of steroid treatment effects for the patients with recurrent attacks, that could cause a statistical error due to the small number of this group. Also, patients with recurrent attacks consist of both the patients with definitive etiology and Bell’s palsy, which produces heterogeneous groups for the comparison of steroid treatment. In conclusion, usage of steroid treatment in childhood Bell’s palsy is still controversial. Large multicenter randomized trials are needed to define the efficacy of prednisolone treatment when compared to placebo in childhood Bell’s palsy. We hope that an ongoing multicenter, placebo-controlled randomized trial that is conducted by Babl et al. will enlighten our questions [9]. * Zeynep Selen Karalok selen_z@yahoo.com