Mehmet Ufuk Aluçlu

Melkersson-Rosenthal syndrome with partial oculomotor nerve palsy.

Ann Saudi Med 28(2) March-April 2008 www.saudiannals.net 135 Melkersson-Rosenthal syndrome (MRS) is a rare granulomatous disease characterized by orofacial edema, facial nerve palsy, and furrowed tongue (lingua plicata).1,2 This is the classic triad which defines the syndrome, although it is accepted that the presence of two manifestations or one with a granulomatous cheilitis in the eyelid biopsy, is sufficient to make the diagnosis.3 Facial paralysis, most commonly unilateral, may also occur as well as a congenital fissuring of the tongue. It was first described in 1928 by Melkersson as a syndrome of recurrent facial palsy and edema, and Rosenthal later added the third feature of the syndrome, furrowing of the tongue, in 1931.4 The etiology of MRS is unknown, although both genetic and acquired factors have been implicated.2,5 Isolated eyelid edema, particularly bilateral involvement, is also rare, and as such it is usually misdiagnosed as orbital Melkersson-Rosenthal syndrome with partial oculomotor nerve palsy

The effects of intravenous immunoglobulin on cerebral ischemia in rats: An experimental study.

Stroke is one of the major reasons of death in the United States and related to adult disability. Despite aggressive research, the treatment approaches of stroke still remains a major clinical problem. Intravenous immunoglobulin (IVIg) is a polyspecific Ig G preparation obtained from plasma of several thousand healthy people (donors). IVIg is an important treatment approach and used for several disorders. The aim of this study was to investigate the potentially beneficial effects of IVIg therapy in experimentally induced ischemia in middle cerebral artery occlusion (MCAo) models of rats. A total of 30 adult male Sprague Dawley rats were used. The rats were divided into two equal groups, each consisting of 15 randomly selected rats: control group (n = 15) and IVIg group (n = 15). Intraluminal filament method was used for establishment of cerebral ischemia. Intraluminal filament was withdrawn after 2 h of MCAo and reperfusion started again and passed to therapeutic stages for all the groups. Physiologic saline solution of 0.5 ml/kg was administered to the control group and 400 mg/kg IVIg was given to the IVIg group rats intravenously. In neurological evaluation, the worst score was determined as 3 and the best score as 0. After routine process, the brain tissue was prepared histopathological investigation. The IVIg group showed significantly better recovery with respect to the control group by neurological examination. The observation of specimens obtained from IVIg groups showed that findings correlate with grade 1 and -2 histopathologically. Nevertheless, ischemic amendments were observed to comply with grade 3 in ischemic areas in control group. IVIg therapy can be used in the treatment of ischemic stroke patients.

The Effects of Valproic Acid on Sciatic Nerve of Fetal Rats and Protective Effects of Folic Acid and Vitamin E

El objetivo fue investigar los posibles efectos perjudiciales del acido valproico (AVP) materno sobre el nervio ciatico en fetos y los efectos protectores de la vitamina E (Vit E) y acido folico (AF) en fetos de ratas. Se administraron a ratas acido valproico (400mg/kg), acido folico (400mg/kg) y vitamina E (250 mg/kg) en cada uno de los dias de gestacion 8-10. Todos los fetos fueron recogidos a los 20 dias de gestacion. Finas secciones de biopsias obtenidas de los nervios ciaticos de fetos fueron tenidos con acetato de uranilo y examinados bajo microscopio electronico de transmision. Los fetos (n: 36) fueron divididos en cinco grupos: control, avp, avp+af, avp+vit e y avp+fa+vit e. En cada grupo, se realizaron los procedimiento farmacologicos, quirurgicos y los metodos histologicos. Los pesos y longitudes de los fetos de cada grupo fueron comparados y analizados usando la prueba One-Way Anova. La administracion de dosis unicas de acido valproico (400 mg / kg) resulto en la perdida del peso la longitud entre el control y el grupo apv. Sin embargo, las diferencias en la longitud y el peso entre los otros grupos no fueron significativas. Los hallazgos histopatologicos del grupo control fueron normales. En el grupo avp, se mostro especialmente cambios degenerativos en la mielina que envuelve al nervio perifericamente. Ademas, predominatemente se encontro en las muestras de este grupo fibras colagenas condensadas y zonas ampliamente desmielinizadas, mientras que las zonas moderadamente afectadas eran relativamente normales. Ambos grupos avp+fa y avp+vit e exhibieron cambios ultraestructurales similares, lo que supone un minimo o moderado cambio degenerativo. El grupo avp+fa+vit e tuvo casi una estructura normal. La administracion de dosis unicas de acido valproico (400 mg / kg) produjo un efecto sobre el deterioro del nervio ciatico a nivel ultraestructural. La administracion de la AF y vitamina E tienen un efecto protector, en cierta medida, para evitar la cambios degenerativos. La combinacion de AF y vitamina E, junto a la ulterior administracion de AVP tienen un efecto protector mas potente. El objetivo del presente estudio fue analizar los cambios histopatologicos que pueden ocurrir en un modelo experimental de alto riesgo despues de la administracion de acido valproico. Ademas, fueron evaluadas las funciones de proteccion de la administracion de acido folico y la vitamina E.

A database for screening and registering late onset Pompe disease in Turkey

The aim of this study was to search for the frequency of late onset Pompe disease (LOPD) among patients who had a myopathy with unknown diagnosis registered in the pre-diagnostic part of a novel registry for LOPD within a collaborative study of neurologists working throughout Turkey. Included in the study were 350 patients older than 18 years who have a myopathic syndrome without a proven diagnosis by serum creatine kinase (CK) levels, electrodiagnostic studies, and/or muscle pathology, and/or genetic tests for myopathies other than LOPD. Acid alpha glucosidase (GAA) in dried blood spot was measured in each patient at two different university laboratories. LOPD was confirmed by mutation analysis in patients with decreased GAA levels from either both or one of the laboratories. Pre-diagnostic data, recorded by 45 investigators from 32 centers on 350 patients revealed low GAA levels in a total of 21 patients; from both laboratories in 6 and from either one of the laboratories in 15. Among them, genetic testing proved LOPD in 3 of 6 patients and 1 of 15 patients with decreased GAA levels from both or one of the laboratories respectively. Registry was transferred to Turkish Neurological Association after completion of the study for possible future use and development. Our collaborative study enabled collection of a considerable amount of data on the registry in a short time. GAA levels by dried blood spot even from two different laboratories in the same patient may not prove LOPD. LOPD seemed to be rarer in Turkey than in Europe.

Facial diplegia: etiology, clinical manifestations, and diagnostic evaluation.

OBJECTIVE Facial diplegia (FD) is a rare neurological manifestation with diverse causes. This article aims to systematically evaluate the etiology, diagnostic evaluation and treatment of FD. METHOD The study was performed retrospectively and included 17 patients with a diagnosis of FD. RESULTS Patients were diagnosed with Guillain-Barré syndrome (GBS) (11), Bickerstaffs brainstem encephalitis (1), neurosarcoidosis (1), non-Hodgkins Lymphoma (1), tuberculous meningitis (1) herpes simplex reactivation (1) and idiopathic (1). In addition, two patients had developed FD during pregnancy. CONCLUSION Facial diplegia is an ominous symptom with widely varying causes that requires careful investigation.

Balo’s concentric sclerosis in a patient with spontaneous remission based on magnetic resonance imaging: A case report and review of literature

Balo’s concentric sclerosis (BCS) is a rare monophasic demyelinating disease known as multiple sclerosis subtype and seen as a round lesion with variable hyper and hypo-detoxification layers. Characteristic appearance can be seen as “bulb eye” or “onion bulb”. The initial terminology for this neurological disorder was leukoencephalitis periaxialis concentrica; this is defined as a disease in which the white matter of the brain is destroyed in concentric layers in such a way as to leave the axial cylinders intact. This report presents a case of BCS with spontaneous healing of the patient and a mass lesion with concentric rings adjacent to the left lateral ventricle and the posterior portion of the corpus callosum with peripheral vasogenic edema. The neurological lesion of the patient was similar to the magnetic resonance imaging and clinical findings of the BCS.

Recurrence of atretic parietal cephalocele in adult: a case report and review of literature

Common presentation of atretic parietal cephalocele (APC) is mostly seen in infants and young children. And, it is a palpable midline parietal soft tissue mass which is thought to represent involuted true cephalocele (meningocele or encephalocele)connected to dura mater via a fibrous stalk. The incidence of intracranial anomalies have increased with APC. Parietal cephaloceles comprise about 1% of all cerebrospinal congenital malformations and 10% of cephaloceles. We report here the case of an atretic parietal cephalocele with no associated brain malformations in adult.

Protective effects of L-glutamine against toxicity of deltamethrin in the cerebral tissue

Background Deltamethrin (DLM) is a broad-spectrum synthetic dibromo-pyrethroid pesticide that is widely used for agricultural and veterinary purposes. However, human exposure to the pesticide leads to neurotoxicity. Glutamine is one of the principal, free intracellular amino acids and may also be an antioxidant. This study was undertaken in order to examine the neuroprotective and antioxidant potential of l-glutamine against DLM toxicity in female Wistar albino rats. Materials and methods The rats were divided into the following groups (n=10): Group I: control (distilled water; 10 mL/kg, po one dose), Group II: l-glutamine (1.5 g/kg, po one dose), Group III: DLM (35 mg/kg, po one dose), and Group IV: DLM (35 mg/kg, po one dose) and l-glutamine (1.5 g/kg, po one dose after 4 hours). Total oxidant status (TOS), total antioxidant status (TAS), tumor necrosis factor-α, interleukin (IL)-1β, and IL-6 levels and apoptosis were evaluated in brain tissue. Results DLM-treated animals had a significant increase in brain biochemical parameters, as well as TOS and TAS. Furthermore, the histopathological examination showed neuronal cell degeneration in the cerebral tissue. l-Glutamine treatment decreased the elevated brain levels of TOS and neuronal cell degeneration. There was no difference in tumor necrosis factor-α, IL-1β, and IL-6 levels between the groups. Conclusion l-Glutamine may reduce the toxic effects of DLM in the cerebral tissue through antioxidant properties.

Carotid Artery Stenting: Retrospective Evaluation of Experience of an Invasive Tertiary Center

Objective: Carotid artery stenting (CAS) is being applied more frequently as an alternative mode of treatment to carotid endarterectomy. We aimed to present the short-term clinical outcomes of the patients admitted to our clinic with a diagnosis of carotid artery disease and revascularized by CAS. Methods: The study was retrospective and a single-centered study. Between June 2013-January 2016 the patients with an indication for carotid artery intervention and accepted CAS procedure were included in the study. Clinical characteristics and procedural data of the patients were obtained by scanning patient files. After discharge, hospital records were scanned and patients were called to learn whether or not patients were alive and that they have suffered a recent stroke. Results: We included 82 patients that meet the inclusion criteria in the study. 59% of patients were male with a mean age of 68±9 years. 56% of patients were symptomatic. In all patients, stents were used. 85% of patients distal embolic protection devices and 15% MOMA were used. 64 right, 18 left, and two bilateral carotid arteries were stented with a total of 82 patient of 84 successful CAS. Due to residual stenosis a second stent was implanted only in one patient. One patient experienced a partial muscle weakening in upper extremity due to an air embolism and 2 patients received opac material which recovered spontaneously in 24 hours. Conclusion: CAS is being successfully applied with a very low risk of complications in experienced centers. Short-term clinical results of CAS are quite satisfactory. Key words: stroke, carotid artery stenosis, carotid artery stenting, clinical outcomes