Risk for generalization in ocular onset myasthenia gravis: experience from a neuro-ophthalmology clinic.

Conversion to generalized myasthenia gravis (GMG) within the first 2 years has been reported in 18-85% of patients with ocular myasthenia gravis (OMG). The aim of the study was to investigate the risk factors for generalization in patients with OMG admitted to a neuro-ophthalmology clinic and to determine if there were differences between patients with GMG with predominant bulbar (GMG-B) or extremity muscle (GMG-E) involvement according to the 6th and 24th-month Myasthenia Gravis Foundation of America classification ranks. Patients with OMG who were followed-up for at least 24 months were retrospectively analyzed.

Vestibular nuclear infarction: Case series and review of the literature.

Background And Purpose: Acute vestibular syndrome (AVS) is a common cause of emergency admittance and has very rarely been reported due to a vestibular nucleus infarction. Initial magnetic resonance imaging studies (MRIs) including diffusion-weighted images may reveal normal results and even bedside examination tests like HINTS battery which involves head impulse test (HIT), nystagmus and test of skew can be challenging in differing a peripheral vestibulopathy from a central lesion.

Methods: Four patients seen in the emergency department with AVS and evaluated with HINTS battery, cervical vestibular-evoked myogenic potentials (cVEMP) and cranial MRI revealing infarcts restricted to vestibular nuclei were evaluated.

Evaluation of effects of positive airway pressure treatment on retinal fiber thickness and visual pathways using optic coherence tomography and visual evoked potentials in the patients with severe obstructive sleep apnea syndrome.

Introduction: Hypoxia during sleep in obstructive sleep apnea syndrome (OSAS) increases intracranial pressure, decreases cerebral perfusion pressure, and alters vascular supply to the optic nerve. Pattern visual evoked potential (pVEP) has revealed that it causes alterations in the optic nerve, and optic coherence tomography has shown that it causes alterations in the retinal and macular layers.

Objectives: To detect and compare possible alterations in macula and peripapillary retinal nerve fiber thickness (pRNFL) using OCT and in the optic nerve pathways using pVEP before and after positive airway pressure (PAP) in the patients with severe OSAS.

Evaluation of the patients diagnosed as idiopathic intracranial hypertension with and without papilledema visual pathways by analysis of visual evoked potential.

Idiopathic intracranial hypertension (IIH) is a clinical syndrome characterized by increased intracranial pressure in the absence of clinical, laboratory, or radiological findings of space-occupying lesion in the cranium. Papilledema is found in majority of the patients with IIH while it is absent in only about 5-6% of the patients. Our primary objective was to evaluate the patients with IIH with (n: 45) and without (n: 15) papilledema using cranial MRI and VEP analyses and to compare the obtained results.

Idiopathic intracranial hypertension: Are there predictors for visual outcome or recurrences?

Objective: To find out the predictors of final visual outcome and recurrences in idiopathic intracranial hypertension (IIH).

Patients And Methods: Medical records of 75 patients with IIH were analyzed retrospectively. Gender, age of disease onset (AODO), body mass index (BMI), lumbar puncture opening pressure (LP-OP), visual acuity (VA) in logMAR, optical disc appearance (ODA), visual field (VF) mean deviation (MD), treatment results and recurrence rates were considered.

CADASIL with Atypical Clinical Symptoms, Magnetic Resonance Imaging, and Novel Mutations: Two Case Reports and a Review of the Literature.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary microangiopathy with adult onset caused by a missense mutation in the NOTCH3 gene in chromosome 19p13. It presents with autosomal dominant arteriopathy, subcortical infarctions, and leukoencephalopathy. Its common clinical presentations are seen as recurrent strokes, migraine or migraine-like headaches, progressive dementia, pseudobulbar paralysis, and psychiatric conditions.

Evaluation of visual pathways using visual evoked potentials in the patients with impaired fasting glucose and impaired glucose tolerance.

Purpose: There are many studies on degeneration of the ganglion cells using visual evoked potential (VEP) in Diabetes mellitus (DM). The present study intended to investigate whether the retinopathy findings would be helpful for detecting the degeneration to develop or not in retinal ganglion cells with the VEP test before being detectable in ophthalmoscopic examination on prediabetic patients.

Materials And Methods: The present study was conducted prospectively after obtaining approval from the Ethics Committee.

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.

Association of myasthenia gravis and Behçet's disease: A case report.

Myasthenia gravis is a disease of neuromuscular junction due to auto-immune destruction of the acetylcholine receptors. Behçet's disease, on the other hand, is a multisystemic vascular-inflammatory disease. Both conditions are not common in the general population although their association has not been reported in the literature.

Evaluation of neurodegeneration through visual evoked potentials in restless legs syndrome.

Restless legs syndrome (RLS) is a disease characterized by some type of dysesthesia, an indescribable abnormal sensation in the extremities. Our objective was to determine whether the visual evoked potentials (VEP) can be used as a quantitative monitoring method to evaluate demyelination-remyelination and neurodegeneration in the patients with RLS. The present study was carried out prospectively.

Spectrum of the posterior inferior cerebellar artery territory infarcts. Clinical-diffusion-weighted imaging correlates.

Background And Purpose: The clinical, etiological and stroke mechanisms are defined well before but the detailed clinical and etiologic mechanisms regarding to all clinical spectrum of posterior inferior cerebellar artery (PICA) infarcts were not systematically studied by diffusion-weighted imaging (DWI).

Methods: Seventy-four patients with PICA territory ischemic lesion proved by DWI with decreased apparent diffusion coefficient and FLAIR (fluid attenuation inversion recovery) included in our Registry, corresponding to 2% of 3,650 patients with ischemic stroke, were studied. The presence of steno-occlusive lesions in the posterior circulation were sought by magnetic resonance angiography, and reviewed with a three-dimensional rotating cineangiographic method.

Evaluating cognitive functions with visual and auditory number assays and P300 in children with epilepsy.

This study was planned to evaluate cognitive functions, especially attention and immediate recall, in children with epilepsy by using P300 and neuropsychological tests, which included visual and auditory number assays. Fifty five patients with partial seizures, 45 patients with generalized seizures and 20 patients with intractable seizures were enrolled in the study. Twenty five healthy children were taken as the control group.

Lesion patterns and etiology of ischemia in superior cerebellar artery territory infarcts.

Background And Purpose: Infarcts in the territory of superior cerebellar artery (SCA) are uncommon. The clinical, and etiological mechanisms of different infarct patterns of SCA are not well known. Diffusion-weighted imaging (DWI) is superior to conventional magnetic resonance imaging for detecting acute small and multiple ischemic lesions.

P300 and neuropsychological evaluation in Behçet's disease with and without neurological manifestations.

We evaluated cognitive dysfunction in Behçet's disease (BD) with and without neurological manifestations by P300 and neuropsychological tests. Thirty-four BD patients without neurological manifestations, eighteen BD patients with neurological manifestations and 15 controls were studied. P300 potentials and neuropsychological tests were performed.