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

Background 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. Methods 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. Diagnosis of IIH according to ICHD-3 criteria admitted to and followed in our clinic before receiving any treatment between 2008 and 2018 were reviewed retrospectively after obtaining approval from the Ethics Committee. Results There was no statistically significant difference between both groups' ages (P: 0.494) while differences in lumbar puncture (LP) opening pressure and VAS were found to be statistically significant (p = 0.034, 0.001, respectively). In our VEP investigation, it was seen that latencies in the group without papilledema were seen to be closer to those in the control group (P latency: 0.706, P amplitude: 0.080). Increase in latency and decrease in amplitude were seen in the group with papilledema compared to the group without papilledema (p < 0.001). Conclusion In conclusion, alterations in the optic nerve may be detected with VEP investigation before the detection of papilledema through ophthalmoscopic examination in these patients. Thus, VEP investigation may be considered to have predictive value. VEP examination may be recommended in diagnosis and treatment and during follow-up periods.

Highlights Clinical imaging (cranial MRI-MR-angiography-venography) and VEP analyses were evaluated comparatively with detail in the patients with and without papilledema. In the literature, there are studies on VEP analysis. They have been done on acute and chronic stages of idiopathic intracranial hypertension. In the present study, the patients with and without papilledema were compared in contrast to other studies. Increased VEP latency was seen in both groups being especially more prominent in the group with papilledema.

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. RESULTS: In two patients spontaneous nystagmus beating towards the unaffected side was present. In one patient spontaneous nystagmus changed direction on looking to the affected side. In the fourth gaze evoked nystagmus was present without any spontaneous nystagmus. In all, HIT was positive to the affected side. In three cVEMPs was studied revealing delayed latency, reduced amplitude p13/n23 potentials on the lesioned side in two of them. Initial MRIs including diffusion-weighted images disclosed acute infarction in the area of the vestibular nuclei in two patients, with normal results in the other two. Follow-up MRI's performed 48 hours later revealed vestibular nuclear infarction. CONCLUSION: It is not always easy to differentiate small lesions restricted to central vestibular structures from peripheral vestibular lesions both on clinical and radiological grounds. Follow-up cranial MRI is necessary in patients with known vascular risk factors.

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. MATERIALS AND METHODS: Thirty patients who were diagnosed as having severe OSAS in the neurology-sleep outpatient clinic and 30 healthy control subjects were included in the study. Ophthalmic examinations were performed prior to (month 0) and after (month 6) PAP treatment, and pVEP (peak time [PT] and amplitude) and OCT parameters (peripapillary retinal-macular layers) were compared. RESULTS: In the comparison between the severe OSAS (before treatment) and control groups, thinning was found in pRNFL (average, nasal, inferior) and in the macular layers (external and internal superior quadrants) (p < 0.05). pVEP investigation revealed increased PT in P100 and N145 waves and decreased amplitude of N75-P100 waves. In the comparisons before and after PAP treatment, a decrease in PT of N75 and P100 waves and increase in N75-P100 amplitudes were found. In the pRNFL, significant thickening was found in the layers with thinning before treatment, whereas no significant thickening was found in macular layers, except for the fovea. DISCUSSION: It was shown that PAP treatment in patients with severe OSAS prevents hypoxia without causing alterations in intraocular pressure and thus reduces inflammation and causes thickening in the pRNFL and macular layers.

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. In our study, the subjects were divided into three groups as impaired fasting glucose (IFG), impaired glucose tolerance (IGT) and normal patients. They also underwent physical, ophthalmological and VEP examination. Three main components of VEP obtained from these groups were N75, P100, and N145 latency and N75-P100 amplitude. RESULTS: The study participants consisted of the IFG group (n: 30, female/male ratio: 21/9; mean age: 49.17 ± 10.52 years), the IGT group (n: 30, female/male ratio: 23/7; mean age: 47.00 ± 11.09 years), and the Control Group (n: 40, female/male ratio: 30/10; mean age: 48.03 ± 10.96 years). Difference in sex and age between the study groups (p > 0.05). P100 latency was found to increase significantly in comparison between the IGT and Control Group for both eyes (p right: 0.003, p left: 0.001) whereas it did not increase significantly in the comparison between the IFG and the Control Group (p right: 0.065, p left: 0.116). CONCLUSION: It was observed that VEP may be a parameter of predictive value that might be used in evaluating prediabetic cases in terms of retinopathies similar to DM.

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. We wanted to present our patient who developed clinical course of myasthenia gravis following discontinuation of medications due to complications of corticosteroid for Behçet's disease. It was observed that clinical findings of myasthenia gravis recovered following restarting steroid treatment and he did not experience attacks of both conditions. Although Myasthenia gravis and Behçet's disease are distinct entities clinically as well as in terms of pathogenesis, they share common physiopathological features and their treatment is based on their common features.

Clinical, Demographic, and Radiological Characteristics of Patients Demonstrating Antibodies Against Myelin Oligodendrocyte Glycoprotein

Background: Optic neuritis, myelitis, and neuromyelitis optica spectrum disorder (NMOSD) have been associated with antibodies against myelin oligodendrocyte glycoprotein-immunoglobulin G (anti-MOG-IgG). Furthermore, patients with radiological and demographic features atypical for multiple sclerosis (MS) with optic neuritis and myelitis also demonstrate antibodies against aquaporin-4 and anti-MOG-IgG. However, data on the diagnosis, treatment, follow-up, and prognosis in patients with anti-MOG-IgG are limited. Aims: To evaluate the clinical, radiological, and demographic characteristics of patients with anti-MOG-IgG. Study Design: Multicenter, retrospective, observational study. Methods: Patients with blood samples demonstrating anti-MOG-IgG that had been evaluated at the Neuroimmunology laboratory at Ondokuz Mayis University's Faculty of Medicine were included in the study. Results: Of the 104 patients with anti-MOG-IgG, 56.7% were women and 43.3% were men. Approximately 2.4% of the patients were diagnosed with MS, 15.8% with acute disseminated encephalomyelitis (ADEM), 39.4% with NMOSD, 31.3% with isolated optic neuritis, and 11.1% with isolated myelitis. Approximately 53.1% of patients with spinal involvement at clinical onset demonstrated a clinical course of NMOSD. Thereafter, 8.8% of these patients demonstrated a clinical course similar to MS and ADEM, and 28.1% demonstrated a clinical course of isolated myelitis. The response to acute attack treatment was lower and the disability was higher in patients aged > 40 years than patients aged < 40 years at clinical onset. Oligoclonal band was detected in 15.5% of the patients. Conclusion: For patients with NMOSD and without anti-NMO antibodies, the diagnosis is supported by the presence of anti-MOG-IgG. Furthermore, advanced age at clinical onset, Expanded Disability Status Scale (EDSS) score at clinical onset, spinal cord involvement, and number of attacks may be negative prognostic factors in patients with anti-MOG-IgG.

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. Demographic, clinical, laboratory features and treatment strategies that can be associated with generalization and time to generalization were evaluated. Of the 139 patients with OMG, 54 (39%) showed generalization with a mean time of 10.3 (range 2-24) months. GMG-B and GMG-E were diagnosed in 31 (22.3%) and 23 patients (16.5%), respectively. Seropositivity for acetylcholine receptor and muscle-specific tyrosine kinase antibodies, abnormal single-fiber electromyography (SFEMG), and the presence of thymic abnormalities (thymoma and hyperplasia) were factors associated with generalization on multivariate analysis without a significant difference between the GMG-B and GMG-E groups. In addition, an abnormal repetitive nerve stimulation test was related to a shortened time to generalization. Bilateral ptosis at onset was found as a risk factor for generalization. In a neuro-ophthalmology clinic, bilateral ptosis as an initial feature of OMG must be approached cautiously because it may be the first sign of impending GMG.

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. RESULTS: Mean age at onset age was 32.4 years, BMI was 311 kg/m² and median LP-OP was 380 mm H2O. All patients were treated with acetazolamide with a median dose of 1500 mg. The mean follow-up period was 44.8 months. AODO, BMI, LP-OP were not correlated with any of the examination parameters (VA, ODA, VF) at the first or last visit. The correlation between the VA and VF both at the first and last visit was not very powerful. VA of the last visit was fairly correlated with the VA of the first visit. However, the correlation between the last and first visit VF was very good. A very significant improvement in both VA and VF was recorded after treatment. Recurrences were noted in 23%. Demographic and clinical features of the recurring and non-recurring patients were not significantly different in terms of AODO, BMI, LP-OP, VA, VF or ODA. CONCLUSIONS: The patients with IIH respond to treatment with acetazolamide. First visit VF is the main determinant of the final visual outcome. Recurrences cannot be predicted by the demographic or clinical features at presentation.

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. Two patients with CADASIL syndrome, whose diagnosis was made based on clinical course, age of onset, imaging findings, and genetic assays in the patients and family members, are presented here because of new familial polymorphisms. The first patient, with cerebellar and psychotic findings, had widespread non-confluent hyperintense lesions as well as moderate cerebellar atrophy in cranial magnetic resonance scanning. The other patient, with headache, dizziness, and forgetfulness, had gliotic lesions in both cerebral hemispheres. CADASIL gene studies revealed a new polymorphism in exon 33 in the first patient. In the other patient, the NOTCH3 gene was identified as a new variant of p.H243P (c.728A > C heterozygous). By reporting a family presenting with various clinical symptoms in the presence of new polymorphisms, we emphasize that CADASIL syndrome may present with various clinical courses and should be considered in differential diagnoses.

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.

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. It was planned to determine normal or pathological conditions in the form of increased latency or decreased amplitude of VEP and to evaluate possible pathologies in the visual and retinal pathways at early stages and at months 3 and 6 of follow-up in the patients with RLS (with or without iron deficiency anemia), in those without RLS (at the time of diagnosis prior to any medical therapy) without any visual symptoms. It was observed that latency of VEP improved but didn't return to normal limits following treatment with dopamin agonists, iron, or combination of both and that there was no significant difference between the post-treatment data and those of the control group. These results in combination with the fact that the latencies and amplitudes didn't return to normal levels despite the 6-month-treatment but showed a progressive course with partial regeneration suggests that there was incomplete remyelination. It should be kept in mind that this syndrome is likely to be a part of neurodegenerative process.

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. The results were as follows: 1. P300 latencies were significantly longer in the intractable and partial groups when compared with the control subjects. 2. The duration of epilepsy, seizure frequency, cerebral imaging pathologies were not significantly correlated with delayed P300 latencies. 3. The results of the visual and auditory number assays test showed significant abnormalities when each of the three groups were compared with the normal controls. 4. Though some subgroups of the neuropsychological tests were correlated with the P300 latencies, an overall significant correlation was not present between them. So, we suggest that neuropsychological tests are more convenient for the assessment of cognitive functions in children with epilepsy than the P300 recordings.

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. P300 latency values and neuropsychological tests were not significantly different from the normal controls in BD patients without neurological manifestations. In neurologically symptomatic patients attention and memory deficits and delayed P300 potentials were found.

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. METHODS: We studied 60 patients with lesions involving SCA territory proved by DWI, which have been selected from 3,800 patients with first ischemic stroke consecutively admitted to our stroke unit over a period of 5 years. RESULTS: There are 7 distinctive SCA lesion patterns: (1) a lesion was found in the medial (m) branch territory of SCA (mSCA) in 14 patients; (2) a lesion in the lateral (l) branch territory of SCA (lSCA) was seen in 9 patients; (3) a coexisting lesion involving mSCA and lSCA was found in 9 patients; (4) a lesion in cortical borderzones between SCA and m/l branches of the posterior inferior cerebellar artery (PICA) was observed in 6 patients; (5) a lesion in deep borderzones between lSCA and mSCA, and lPICA and mPICA was present in 8 patients; (6) a lesion involving the medial rostral cerebellum between the right and left SCA was found in 4 patients; (7) multiple lesions involving SCA and other vertebrobasilar artery territories were present in 10 patients. The main cause was possible artery-to-artery embolism from atherosclerotic vertebrobasilar arteries to distal branches of SCA in 20 patients (33%). Fourteen patients had a source of cardioembolism (23%), and 6 patients (8%) had concomitant atherosclerotic vertebrobasilar artery disease and a source of cardioembolism. CONCLUSIONS: An acute ischemic lesion in the SCA territory is mainly multiple. The lSCA territory was the most involved area. Small territorial infarcts were frequently associated with large territorial SCA infarcts. Borderzone SCA infarcts occurred in one third of the patients with transient benign symptoms. Mass effects are unusual despite the large amount of SCA involvement. Our results supported the fact that embolism is the predominant stroke mechanism in the SCA territory infarction.

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. RESULTS: We found six subgroups of PICA territory infarcts according clinico-topographical relationship: (1) 9 patients with lesion in the territory lateral branch of PICA; (2) 23 patients with an infarct in the territory of medial branch of PICA; (3) 9 patients with a lesion involving both medial and lateral branches of the PICA; (4) 9 patients with cortical infarcts at the boundary zones either between medial and lateral branches of the PICA or between PICA and m/l superior cerebellar artery (SCA); (5) 10 patients with a lesion at the deep boundary zones either between medial and lateral PICA, or between PICA and medial/lateral SCA; (6)14 patients with concomitant multiple lesions in the PICA and in other vertebrobasilar artery territories. The main cause of PICA infarcts was extracranial large-artery disease in 30 patients (41%) patients, cardioembolism and in situ branch disease in 15 patients (20%) each. CONCLUSIONS: Multiple PICA territory lesions on DWI were not uncommon and could be caused by multiple emboli originating from break-up of atherosclerotic plaque in the subclavian/innominate-vertebral arterial system. DWI findings of single or multiple small lesions could account for some cases with transient and subtle cerebellar symptoms which have been considered before as 'vertebrobasilar insufficiency' without morphologic lesion. Different clinical-DWI correlations allow us to determine better definition of the topographical and etiological spectrum of acute PICA territory lesions, which was previously defined by pathological and conventional MRI studies.