Hafize Nalan Güneş

Serum glutathione peroxidase, xanthine oxidase, and superoxide dismutase activities and malondialdehyde levels in patients with Parkinson's disease.

Parkinson’s disease (PD) is a progressive neurodegenerative disorder characterized by loss of dopaminergic (DA) neurons in the substantia nigra pars compacta (SNpc). Oxidative stress has been hypothesized to play a major role in the development of PD in various studies. This study assessed to investigate oxidative and anti-oxidative status in PD patients. We evaluated oxidant/antioxidant status by measuring serum malondialdehyde (MDA) levels, xanthine oxidase (XO) activities, and activities of antioxidant enzymes, namely, glutathione peroxidase (GSH-Px) and superoxide dismutase (SOD). The study included 29 patients with PD and 32 healthy subjects as controls. Comparison of oxidative parameters in the patient and control groups revealed significantly higher GSH-Px and XO activities in the patient group. Serum MDA and SOD activities in PD patients were not significantly different from the controls. MDA was negatively correlated with duration of the PD and positively with age of onset. There was a negative correlation between SOD and Hoehn and Yahr (H&Y) stage. According to these results, we suggest that oxidative stress may contribute to the development of PD.

Serum oxidant and antioxidant status of patients with chronic tension-type headache: possible effects of medical treatment

Tension-type headache (TTH) is one of the most common and costly primary types of headache in clinical practice, with an unknown etiology. This study assessed to investigate oxidative and antioxidative status in patients with chronic tension-type headache (CTTH), and to evaluate possible effect of medical treatment. The study included 41 CTTH patients and 19 age- and sex-matched healthy subjects without headache as controls. The CTTH group comprised 20 patients receiving treatment and 21 untreated patients. We evaluated oxidant/antioxidant status by measuring serum malondialdehyde (MDA) levels and activities of antioxidant enzymes, namely glutathione peroxidase (GSH-Px) and catalase (CAT). Comparison of oxidative parameters in the patient and control groups revealed significantly lower CAT activities and higher MDA level and GSH-Px activities in the patient group. In the CTTH group, serum CAT activities were found to be significantly decreased in patient groups, while serum MDA levels and GSH-Px activities were found to be higher in the untreated CTTH patients. These findings suggest that oxidative stress is increased in the patients with CTTH, and medical treatment abolishes the stress in part. It has been concluded that antioxidant support might be helpful for the patients with CTTH to prevent oxidant stress and peroxidation damages further.

Oxidative and nitrosative stress in serum of patients with Parkinson’s disease

Parkinson’s disease (PD) is one of the common neurodegenerative disorders. Oxidative stress is considered as a contributing factor to the development of PD. The present study aims to investigate serum oxidative stress status in patients with PD. Oxidative stress was assessed by measuring serum nitric oxide levels, lipid hydroperoxide concentrations, and nitric oxide synthase activity. In addition, total serum antioxidant capacity (TAC) was evaluated using the serum 2,2-Diphenyl-1-picryl-hydrazyl (DPPH) free-radical scavenging method in 32 patient with Parkinson’s disease and 32 control subjects. Our results indicated that serum nitric oxide and lipid hydroperoxide levels were significantly lower in patients with PD than controls. Moreover, nitric oxide levels were found to be negatively correlated with Unified Parkinson’s Disease Rating Scale (UPDRS). However, no statistical difference was observed in total serum antioxidant capacities and nitric oxide synthase activities between patients and controls. The present study indicates that although antioxidant capacity was not changed, lipid hydroperoxide (LPO) level was found decreased. This might show pre-oxidative process in these patients. In addition, decreased nitric oxide (NO) level and negative correlation observed between NO level and disease rating scale implicated a role for NO in the disease process.

Whole thoracal spinal cord involvement in case of neuro-Behçet's disease.

Behçet’s disease is a chronic inflammatory, multisystem vasculitis. Neurological involvement is one of the most serious manifestations of Behçet’s disease. Although brain stem and diencephelon are the most affected areas in neuro-Behçet’s disease, spinal cord involvement are rarely seen. We report a case of paraplegia caused by completely thoracic cord involvement of Behçet’s disease in a 20-year-old woman.

Clinical associations, biological risk factors and outcomes of cerebral venous sinus thrombosis.

Objective Cerebral venous sinus thrombosis (CVST) is a rare cerebrovascular disease affecting young adults. The majority of the patients are female. The aim of this study is to assess the clinical associations, risk factors and outcomes of the patients with CVST. Methods The data of 75 patients with CVST admitted to our hospital between 2006 and 2016 were reviewed. Demographic and clinical features and the thrombophilic risk factors of the patients were recorded. The localizations of the thrombi were determined and modified Rankin score at the time of onset and discharge were calculated. Results The majority of our patients (78.7%) were female. Median age was 35 years (16–76). The most common symptom was headache (86.7%). In 82.6% of our patients, inherited or acquired risk factors for thrombosis were detected. Transverse sinus was the most common site of thrombosis followed by sigmoid and superior sagittal sinuses. Two thirds of the patients had involvement of multiple sinuses. The patients with the involvement of sagittal sinus had better disability at the time of admittance (p = 0.013) while the number of involved sinuses was correlated worse disability (p = 0.015). The neurologic states in the majority of the patients were improved by the end of the hospitalization period (p = 0.001). There was no significant difference in disability score at discharge between men and women (p = 0.080). No patient with CVST died in the hospitalization period. Conclusions This study is one of the largest cohort studies on CVST in our region. The results of the study disclosed that CVST had wide range of clinical manifestations and non-specific symptoms at the beginning. For that reason, in especially high risk groups for thrombosis, the diagnosis of CVST should be kept in mind.

Attention Deficit/Hyperactivity Disorder in Adults with Multiple Sclerosis

AbstractObjectives: To determine symptoms and their effects of attentiondeficit/ hyperactivity disorder (ADHD) in adult patients with multiple sclerosis. Studies of psychiatric comorbidities in patients with multiple sclerosis (MS) have reported greater rates in MS patients than the general population. Methods: Subjects consisted of 72 relapsing remitting multiple sclerosis patients and 57 controls. Demographic characteristics including age, gender, education level, duration of disease and Expanded Disability Symptom Scale (EDSS) were recorded. ADHD symptoms were assessed by Adult Attention Deficit/HyperactivityDisorder Self-Report Scale (ASRS). Furthermore, Hospital Anxiety and Depression Scale (HADS) and Short Form-36 (SF-36) were also applied. Results: MS patients had statistically significant higher the rate of ADHD mean scores compared to controls. Patients with MS had statistically significant higher rate of clinical elevation in ASRS scores. There were 26 MS patients and 4 controls with ADHD symptoms. ASRS scores in MS patients were associated with SF- 36 mental and physical component scores and depression and anxiety scores. Conclusion: ADHD symptoms may develop in adults MS patients and are associated with lowered quality of life and increased psychosocial morbidity. In the future, more comprehensive investigations could clarify origins of ADHD symptoms in adult MS patients.

New mutations and genotype–phenotype correlation in late-onset Pompe patients

Pompe disease is a glycogen storage disease caused by acid alfa-glucosidase deficiency. Here, we report clinical properties, genetic features of our late-onset Pompe patients. Seven patients were followed during the last 10 years in our institute. The clinical and laboratory findings were reviewed. Neuropsychological evaluation was performed in four patients. Myotonic discharges of paraspinal muscles and denervation potentials were seen in all patients at the diagnosis and were disappeared during follow-up in two. Only one patient, whose MRI showed cerebral atrophy, had attention and executive dysfunction. Compound heterozygous patients with IVS 1-13T>G have a milder disease. One patient who has homozygous IVS 1-13T>G mutation had more severe disease. Two of our patients who had very severe and fatal disease course carry double mutations on both alleles (c.547-39T>G and c.858+5ins7) that previously scored as “unknown” in Erasmus Pompe Center database. Lastly, we found new mutations (c.1209 C>A, 2737dupG) in two patients carrying IVS 1-13T>G in the other allele. Systemic involvements are very rare in late-onset Pompe patients. Similarly, Pompe disease does not cause cognitive impairment in adult population. Homozygous IVS 1-13T>G mutation and c.547-39T>G mutation which are previously noted as “unknown” pathogenicities cause a more severe disease.

Asymmetric sensory nerve action potential amplitudes as an early hint for diagnosing Lewis–Sumner syndrome

Lewis–Sumner syndrome (LSS) is an immune-mediated peripheral nerve disorder characterized by multifocal conduction blocks. LSS is typically associated with distal, asymmetric weakness and sensory impairment, affecting primarily upper limbs [1]. It is still an open question whether LSS and multifocal motor neuropathy (MMN) are a variant of CIDP or whether these conditions represent different entities [2].

Thrombotic Thrombocytopenic Purpura and Ischemic Stroke: A Case Report

Thrombotic Thrombocytopenic Purpura (TTP) characterized by thrombocytopenia, microangiopathic hemolytic anemia, neurological changes, renal failure, and fever is a life threatening, multisystem disease. The first was defined by Moskowitz in 1924. Thrombosis of small arterioles, venules and capillaries lead to cerebral micro-infarct. There are many neurological symptoms such as headache, seizures, changes in consciousness and mental, focal neurological changes. A case which was observed that neurological complications while the patient was monitoring with diagnose of TTP is presented.

POEMS syndrome with peripheral and central nervous system demyelination: case report.

Introduction:POEMS syndrome is a rare, atypical plasma cell proliferative disorder. Predominantly motor chronic demyelinating polyneuropathy and monoclonal plasma cell disease are the major clinical features of POEMS syndrome. Demyelinating lesions of the central nervous system have been reported only in 2 patients with POEMS syndrome up to now. Case Report:A 71-year-old man was investigated for numbness, burning sensation, and weakness of the lower extremities. He was found to have hypertrichosis, white nails, and multiple hemangiomas in the lower extremities. Neurological examination revealed decreased strength of the proximal and distal muscles of the lower extremities and absence of deep tendon reflexes. There was a marked impairment of temperature, position, and vibration senses in the lower extremities. Romberg test was positive. Nerve conduction was markedly reduced in all extremities and needle electromyography showed denervation pattern. Cerebrospinal fluid was clear, colorless, and had no oligoclonal band. IgM kappa monoclonal gammopathy was identified in serum immunofixation electrophoresis. In bone marrow aspirate plasma cells were counted as being <2%, with normal morphology. Brain magnetic resonance imaging revealed hyperintense lesions in the regions of periventricular and subcortical white matter and thalamus. Normal TSH, T4, and T3 levels and elevated levels of antitiroglobulin antibody and antithyroid peroxydase antibody were found in the laboratory tests. Our patient was diagnosed with POEMS syndrome and treated with intermittent courses of melphalan and prednisone. Conclusions:POEMS syndrome should also be considered in differential diagnosis of patients with demyelinating brain lesions, accompanied by peripheral neuropathy. Diagnostic evaluation of patients with demyelinating polyneuropathy should include brain magnetic resonance imaging.