Gulcin Benbir

Istanbul University, İstanbul, Istanbul, Turkey

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Publications (53)81.53 Total impact

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    ABSTRACT: Unlabelled: Fucosidosis is a rare lysosomal storage disease with clinical presentation of developmental retardation, coarse facial features, hepatosplenomegaly, dysostosis multiplex, and angiokeratomas. Here, a 7-year-old female patient with progressive dystonic movement disorder and loss of acquired motor skills is presented. Coarse facial feature and abnormal globuspallidus signaling in brain magnetic resonance imaging (MRI) led the patient to be investigated in terms of fucosidosis despite absence of hepatosplenomegaly, dysostosis multiplex, and angiokeratomas. Markedly decreased enzyme activity of alpha-fucosidosis led to the correct diagnosis. Conclusion: Various neurological findings have recently been reported in fucosidosis. However, neuroimaging findings have not been studied in detail except a few studies. It is critically important to discuss the wide neuroradiological spectrum of the disease and to highlight fucosidosis in differential diagnosis of bilateral pallidalhypointensity on T2-weighted images in brain MRI. In addition, description of atypical clinical findings of fucosidosis should avoid clinicians from diagnostic delay.
    No preview · Article · Oct 2015 · Annals of Indian Academy of Neurology
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    ABSTRACT: Objective: Accompanying neurological signs are not expected in primary cervical dystonia (PCD), characterized by involuntary muscle contractions resulting in abnormal posture of the neck. We aimed to assess the associated arm movements during walking in patients with PCD in compared to age-, sex-, weight- and height-matched controls. Methods: We examined 16 consecutive de novo patients with PCD and controls in a quantitative trial, and calculated the mean gait speed, mean number of steps, mean frequencies of right and left arms, mean frequencies of right and left legs, and mean arm/leg frequencies for right and left sides. Results: Eleven patients (68,8%) had right-sided cervical dystonia, 5 patients (31,2%) had left-sided cervical dystonia. The mean speed of gait and the mean number of steps were similar between patients and controls. The mean frequencies of bilateral upper extremities and arm/ leg frequencies were significantly slower in patients with PCD (p<0,05). In patient group, the mean armswing frequency was significantly lower in symptomatic side versus asymptomatic side (p=0,038). The disease duration was positively associated with the mean arm/leg frequency (p=0,042). Discussion: Our results imply that the reduced armswing in cervical dystonia might be a feature of primary cervical dystonia.
    Full-text · Article · Jun 2015
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    ABSTRACT: Willis-Ekbom disease/restless legs syndrome (WED/RLS) is the most common sleep-related movement disorder in pregnancy. We designed a prospective longitudinal study to investigate the correlates of WED/RLS during and after pregnancy. A total of 138 pregnant women with WED/RLS and a control group of 251 age-matched pregnant women were enrolled prospectively. A questionnaire was administered during a face-to-face interview at first evaluation during pregnancy and three months after delivery. Among all women in the first trimester, 15.6% were diagnosed with WED/RLS, whereas 32.8% of those in the second trimester and 38.8% of those in the third trimester were diagnosed with WED/RLS (p = 0.032). In regression analysis, later gestational age [p < 0.001; odds ratio (OR) 1.054] and previous history of WED/RLS (p = 0.001; OR 2.795) were positively correlated with the presence of WED/RLS, while ferritin levels (p = 0.001; OR 0.956) were negatively correlated with the presence of WED/RLS. Ferritin levels were also negatively correlated with the International RLS Study Group severity index (p = 0.041). Forty-eight patients (34.8%) experienced WED/RLS symptomatology after delivery. The ferritin levels were lower, and the mean number of pregnancies was higher, in women with residual WED/RLS (p = 0.008). Our survey showed that WED/RLS was more common in the second and third trimesters. Emergence of WED/RLS during the second trimester was strongly associated with residual WED/RLS. Lower ferritin levels were associated with both WED/RLS in pregnancy and residual WED/RLS after delivery. A higher number of pregnancies were also associated with a greater likelihood of having residual WED/RLS after delivery. Copyright © 2015 The Authors. Published by Elsevier B.V. All rights reserved.
    Full-text · Article · May 2015 · Sleep Medicine
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    ABSTRACT: The differentiation of narcolepsy without cataplexy from idiopathic hypersomnia is based on the number of sleep-onset rapid eye movement periods (SOREMPs) observed by multiple sleep latency test (MSLT) and nocturnal polysomnography. The main aim of this study was to investigate the utility of SOREMP in differential diagnosis of central hypersomnias. The authors retrospectively evaluated consecutive 101 patients with a normal polysomnography other than the presence of SOREMP and/or REM without atonia and a latency of ≤8 minutes in MSLT. The authors classified patients as follows: 52 patients had at least 2 SOREMPs (narcolepsy group), 23 had no SOREMPs (idiopathic hypersomnia group), and 26 patients had only 1 SOREMP (intermediate group). In polysomnographic recordings, both mean sleep latency and REM latency were significantly shorter in the narcolepsy (P = 0.012, P < 0.001, respectively) and intermediate groups (P = 0.005 and P = 0.035, respectively) compared with the idiopathic hypersomnia group. In MSLT recordings, sleep latency was 2.7 ± 2.2 minutes in the narcolepsy group, 3.6 ± 1.4 minutes in the intermediate group, and 5.2 ± 2.7 minutes in the idiopathic hypersomnia group (P < 0.001). The mean REM latency and sleep stages SOREMPs arised from were similar between the narcolepsy and intermediate groups. To date, SOREMPs in MSLT and polysomnography remain the sole electrodiagnostic feature that discriminates narcolepsy without cataplexy from idiopathic hypersomnia. Different parameters or combined criteria are being increasingly investigated to increase the sensitivity and specificity of MSLT. The findings showed an altered instability of REM sleep not only in patients with 2 or more SOREMPs in MSLT but also in patients with one SOREMP.
    No preview · Article · May 2015 · Journal of Clinical Neurophysiology
  • Gulcin Benbir · Sakir Delil · Derya Karadeniz · Naz Yeni
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    ABSTRACT: Recurrent hypersomnias are very rare with two subtypes as Kleine-Levin syndrome and menstruation-related hypersomnia, which is very rarely encountered worldwide. Here, we report a young girl with menstruation-related recurrent hypersomnia, who was misdiagnosed as epilepsy due to co-existing generalized epileptic discharges. The importance of this comorbidity in terms of differential diagnosis of the attacks is discussed.
    No preview · Article · Apr 2015 · Journal of Pediatric Neurosciences
  • Sinem Yazici · Gulcin Benbir · Birsen Ince
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    ABSTRACT: Ulcerative colitis (UC) is characterized by an inflammatory disorder of the gastrointestinal tract. Immune-mediated extraintestinal manifestations of UC have increasingly attracted attention in the literature recently, for which UC is now considered as a systemic disease. Neurologic involvement associated with UC is probably under-reported because of the unawareness of many physicians, although early recognition and treatment are crucial in preventing major morbidity and sequel. In this case report is presented a patient newly diagnosed as UC, who developed both sensorineural hearing loss and intractable status epilepticus that we suggest to have resulted from immune-mediated mechanisms.
    No preview · Article · Jan 2015
  • Gulcin Benbir

    No preview · Article · Jan 2015 · Agri: Agri (Algoloji) Dernegi'nin Yayin organidir = The journal of the Turkish Society of Algology

  • No preview · Article · Dec 2014 · La Presse Médicale
  • Gulcin Benbir · Meral E Kiziltan
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    ABSTRACT: The somatosensory-evoked blink reflex (SBR) is one of the release phenomena of blink reflex, possibly resulting from increased excitability of brainstem reticular formation. The authors investigated trigeminal blink responses and SBR in 26 patients with postparalytic facial syndrome (PFS) with synkinesia, 18 patients with essential blepharospasm, and 36 healthy volunteers (control participants). Trigeminal blink reflex responses were elicited in all participants, whereas SBRs were elicited in 44.4% of control participants, 38.9% of patients with essential blepharospasm, and 65.4% of patients with PFS. The mean R2 amplitude and duration and the mean amplitude and duration of SBR were highest in patients with essential blepharospasm. The mean latency of SBR was shorter on the symptomatic side of patients with PFS when compared with the asymptomatic side. The mean R2 duration on the symptomatic side of the patients with PFS was longer than the control participants. These results showed that somatosensory stimulation could be used as an alternative method to demonstrate increased excitability in facial motor neuron in patients with PFS and essential blepharospasm. Disease states relating to different peripheral and/or suprasegmental structures could also influence blink reflex and change its basal excitability and manner in which the reflex responds to modulatory factors.
    No preview · Article · Dec 2014 · Journal of clinical neurophysiology: official publication of the American Electroencephalographic Society
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    ABSTRACT: Creutzfeld-Jacob disease (CJD) is a rare, progressive disease that has a vast clinical manifestation range. Cranial magnetic resonance imaging (MRI), electroencephalography (EEG), and measurement of 14-3-3 in cerebrospinal fluid (CSF) may offer a pragmatic approach in the diagnosis of CJD as an alternative to histopathological confirmation. To present the symptoms and signs of the CJD patients in regard to radiological and neurophysiological findings. We collected all cases with the diagnosis of probable CJD admitted to our neurology department between June 2010 and June 2014. The medical records and laboratory data, clinical features, results of MRI (including diffusion weighted images), EEG and CSF evaluations, and other laboratory data to exclude other possible diagnoses were recorded. None of the patients underwent biopsy or autopsy for histological diagnosis. Of 20 patients, 11 (55%) were men and nine (45%) were women. The mean age at disease onset was 60.0 ± 9.5 years (age range, 47-80 years). All patients without exception had characteristic abnormalities in DWI and/or FLAIR on admission, about 4 months after the initial symptom. Periodic complexes on EEGs characteristic for CJD were detected only in 10 patients (50%) on admission and in 13 patients (65%) during disease course. Out of 14 patients who underwent CSF examination, 11 (78.5%) were positive for 14-3-3 protein. Although the definite diagnosis of CJD is made histopathologically, we aimed to discuss the value of magnetic resonance imaging in the diagnosis of CJD in respect to EEG findings and protein 14-3-3 levels in CSF.
    Full-text · Article · Nov 2014
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    ABSTRACT: AimThe prevalence of insomnia is influenced by environmental factors. This study aimed to investigate the prevalence of insomnia and its sociodemographic and clinical correlates in a general population-based survey in Turkey.Methods This population-based study included 4758 subjects among 5021, who participated in TAPES (Turkish Adult Population Epidemiology of Sleep Disorders) study. Questionnaire items evaluating insomnia were adapted from International Classification of Sleep Disorders (ICSD)-II and DSM–IV–TR. Subjects with restless legs syndrome were excluded.ResultsInsomnia was found to be associated with older age (18-24 years: 9.8%, 25-44 years: 11.7%, 45-64 years: 13.8%, 65 years or older: 13.9%), lower income level (<500 USD: 16.5%), time spent watching TV (6-8 hour or more: 18.4%), tea consumption in the evening (≥6 glasses: 14.5%) and smoking status (current and ex-smoker both: 14.2%) in multiple logistic regression analysis. In respect to other medical disorders, insomnia was significantly associated with the presence of hypertension, diabetes and heart diseases after the adjustment for relevant risk factors for each disease, across all age and gender groups.Conclusions Insomnia is a major health problem in our population, affecting subjects in working age group and lower socioeconomic status. It should especially be screened in patients with chronic diseases. Relatively low proportion of insomnia diagnosed as a sleep disorder suggests the possibility of under-recognition of this condition and its clinical correlates.
    Full-text · Article · Nov 2014 · Psychiatry and Clinical Neurosciences
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    G. Benbir · O. Arpat · O. Kizilkilic · C. Islak · B. Ince

    Full-text · Article · Oct 2014 · International Journal of Stroke
  • F. Bolukbasi · G. Benbir · B. Ince

    No preview · Article · Oct 2014 · International Journal of Stroke

  • No preview · Article · Oct 2014 · International Journal of Stroke
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    ABSTRACT: Cerebellum is highly vulnerable in the prenatal period. Increasing experience with fetal imaging studies has demonstrated that unilateral cerebellar hypoplasia (UCH) is mainly prenatally acquired, representing disruption rather than a true malformation. Here, we report the case of a 17-month-old boy presented with a sudden onset of abnormal eye movements, who was diagnosed during routine fetal screening with UCH and brain stem hypoplasia and suffered from cerebral palsy; however, no posterior arterial system pathology was detected on cranial magnetic resonance images at that time. Following this acute event, diagnostic neuroradiological interventions revealed a dissecting aneurysm with a saccular component in midbasilar arterial segment and hypoplastic left posterior cerebral artery, which may support the ischemic disruptive mechanism in the development of prenatally detected UCH in this child. The pathogenetic mechanisms for cerebellar disruption are certainly multifactorial in origin, although ischemic arterial etiologies were often undervalued.
    No preview · Article · Aug 2014 · Neuropediatrics
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    Hulya Apaydin · Gulcin Benbir

    Full-text · Article · Jul 2014
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    ABSTRACT: Background: The diversity of clinical presentation and neuroimaging findings of CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) from different regions of the world has not yet been studied in depth. Here we investigated the variability of clinical, radiological and genetic data of 48 patients analyzed for NOTCH3 mutation in Turkey. Methods: Clinical evaluation was made according to a preformed questionnaire. Cranial neuroimaging findings were determined on the basis of T1, T2, FLAIR and proton-density magnetic resonance scans. For genetic analysis, polymerase chain reaction was performed with primers flanking exons 2-6 and 11 of NOTCH3 gene. Results: Twenty-five patients (52.1%) were diagnosed as CADASIL with NOTCH3 mutation, while 23 patients (47.9%) had no mutation (NOTCH3-negative patients). The mean age and age at stroke onset were lower in male CADASIL patients (p < 0.03). A family history of migraine (p = 0.012), stroke (p < 0.001), recurrent strokes (p = 0.020) and dementia (p = 0.012) was more common in CADASIL patients. Temporal pole involvement was more common in CADASIL patients (p = 0.004). Conclusion: It is of clinical importance to identify the heterogeneity of CADASIL from different countries due to a low correlation of clinical and radiological data with respect to NOTCH3 mutation.
    Full-text · Article · Jul 2014 · European Neurology
  • Birsen Ince · Gulcin Benbir · Gokcen Gozubatik-Celik
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    ABSTRACT: Many efforts have been made to develop decision-support tools and bleeding prediction schemes to start or resume anticoagulation after intracerebral hemorrhage, related with anticoagulation use or not, such as CHA2DS2-VASc or HAS-BLED scoring. HAS-BLED is a validated scoring system to predict the risk of major bleeding in a patient with atrial fibrillation; some current scientific guidelines suggest its use in ‘risk–benefit’ reasoning when deciding whether to start long-term oral anticoagulation. Here the authors present a patient with atrial fibrillation and intracerebral hemorrhage, and aim to discuss the use of HAS-BLED, suggesting that some revisions may help better management of these patients for major bleeding risk.
    No preview · Article · Jul 2014 · Expert Review of Cardiovascular Therapy
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    Hulya Apaydin · Gulcin Benbir

    Full-text · Article · Jul 2014 · The Journal of Neuropsychiatry and Clinical Neurosciences
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    ABSTRACT: A 37-year-old male, previously diagnosed with GAPO syndrome, was admitted to our hospital complaining of recurrent episodes of transient weakness and numbness in his left arm for 3 months, and severe headache with progressive dysphagia for 15 days. His cranial magnetic resonance (MR) images showed multiple ischemic foci in the bilateral periventricular and supraventricular white matter. Cerebral MR-angiography showed total occlusion of the right internal carotid artery and moderate stenosis in the left internal carotid. We also detected chronic thrombotic changes in the distal left sigmoid sinus, proximal right sigmoid sinus, and bilateral jugular veins on cerebral MR-venography. He was diagnosed with dilated cardiomyopathy at age 31 years, which was reported as a novel association; and later he had a myocardial infarction at age 34 years. To the best of our knowledge, this is the first patient with GAPO syndrome and arterial atherosclerosis in cerebral-as well as coronary-arteries and intracranial venous thrombosis. We report the evolution of the disease in this patient, who died at age 38 years due to respiratory failure secondary to lower respiratory tract infection. © 2014 Wiley Periodicals, Inc.
    No preview · Article · May 2014 · American Journal of Medical Genetics Part A