Elisabeth Andreadou

Serum uric acid levels in patients with Parkinson's disease: their relationship to treatment and disease duration.

UNLABELLED There is evidence to support that oxidative stress is increased in Parkinsons disease (PD) and contributes to degeneration of dopaminergic neurons. Uric acid (UA), a natural antioxidant in blood and brain tissue, scavenging superoxide, peroxynitrite and hydroxyl radical, was found reduced in the serum of PD patients. In addition low plasma uric acid (UA) levels have been associated with an increased risk of PD. OBJECTIVES The aim of our study was to investigate serum UA levels in PD patients compared with age-matched healthy controls and their possible relationship with several clinical parameters of PD and pharmaceutical treatment. PATIENTS AND METHODS We measured serum UA levels in 43 PD patients and 47 healthy volunteers, age and sex-matched. UA levels were correlated with disease duration, severity and treatment. RESULTS Low UA levels were observed in PD patients compared with controls (p=0.009). Age, Body Mass Index (BMI) and UPDRS III score did not significantly affect serum UA concentrations, whereas gender was found to contribute significantly to UA level (p<0.000). Strong and significant inverse correlations of UA with disease duration (R(s)=-0.397, p=0.009) and daily levodopa dosage (R(p)=-0.498, p=0.026) were observed. These associations were significant for men (R(s)=-0.441, p=0.04 and R(s)=-0.717, p=0.03 respectively), but not for women (R(s)=-0.221, p=0.337 and R(s)=-0.17, p=0.966 respectively). CONCLUSION Our results suggest that there may be increased consumption of UA as a scavenger in PD, possibly heightened by dopaminergic drug treatment. Given the antioxidant properties of UA, manipulation of its concentrations should be investigated for potential therapeutic strategies of the disease.

Frontal lobe dysfunction in amyotrophic lateral sclerosis

The aim of the present study was to investigate the involvement of frontal lobe dysfunction in amyotrophic lateral sclerosis (ALS) using ocular motor paradigms and neuropsychological testing. Fifty-one patients with ALS participated in the following ocular motor tasks: (1) a three-choice task and (2) a remembered saccade task. The patients underwent a clinical and neuropsychological evaluation. One-third of ALS patients presented with signs of frontal dysfunction, as determined by their high distractibility factors (DF) in the three-choice task and their performances in both the Wisconsin and Stroop tests. ALS patients exhibited longer latencies to eye movement than controls in the performance of the remembered saccade task, specifically in performance of both remembered and delayed saccades, but saccade accuracy was not impaired. Finally, performance indices of the ocular motor tasks, in particular the DF, was correlated only with the degree of dysarthria.

Demyelinating Disease following Anti-TNFa Treatment: A Causal or Coincidental Association? Report of Four Cases and Review of the Literature.

Tumor necrosis factor antagonists (anti-TNFa) are an established therapeutic option for several autoimmune and inflammatory bowel diseases. Despite their clinical effectiveness, neurological adverse events have been reported and literature data suggest a potential role of anti-TNFa in the induction of demyelination of the CNS. We present four patients treated with anti-TNFa who developed symptoms suggestive of CNS demyelination. The first patient, a 17-year-old male who received etanercept for psoriatic arthritis for eight months, presented with dysesthesias up to T4 level. The second patient, a 30-year-old male treated with adalimumab for three years due to ankylosing spondylitis, presented with right unilateral tinnitus. The third case, a 47-year-old female, received etanercept for four years because of psoriatic arthritis and developed persistent headache and left-sided face and head numbness. Finally, the fourth patient, a 57-years-old female treated with etanercept for six years due to ankylosing spondylitis, presented with difficulty in speech, swallowing, and ptosis of the right corner of the mouth. In all cases, brain MRI showed lesions suggestive of demyelination, while positive oligoclonal bands were detected in the CSF. Anti-TNFa treatments were discontinued and patients showed clinical improvement with pulsed intravenous corticosteroid therapy. CNS demyelination following anti-TNFa treatment represents a relatively rare but potential serious complication. Close follow-up and MRI monitoring of these patients is mandatory to elucidate whether the clinical manifestations represent adverse events occurring during anti-TNFa therapy or a first demyelinating episode.

Lack of association between vitamin D levels and bone mineral density in patients with multiple sclerosis

BACKGROUND There is conflicting evidence regarding the association of vitamin D status with bone mineral density (BMD) in adult patients with multiple sclerosis (MS). We evaluated cross-sectionaly the determinants (including vitamin D levels) of low BMD in patients with relapsing-remitting MS (RRMS). METHODS The BMD at lumbar level (L2-L4) and femoral neck was measured in consecutive adult, ambulatory, RRMS patients by dual-energy X-ray absorptiometry. Blood samples were collected for total serum calcium, phosphorus, magnesium, 25-hydroxyvitamin D(3) and parathormone. Osteopenia and osteoporosis were defined according to the World Health Organization operational BMD definition. MS severity was assessed using the EDSS-score. Cross-sectional associations were evaluated using Spearmans correlation-coefficient and multiple linear regression models. RESULTS A total of 119 patients were evaluated (mean age 39.2 ± 10.4 years; 40% men). Osteopenia at lumbar spine (L2-L4) and femoral neck was present in 26% (95%CI: 18%-35%) and 50% (95%CI: 41%-60%) of the patients respectively. Osteoporosis was documented at lumbar spine and femoral neck of 3% (95%CI: 0%-8%) and 11% (95%CI: 6%-18%) of the study population respectively. There was no correlation (p>0.1) of 25-hydroxyvitamin D3 levels with any of BMD measurements (including Z- and T-scores) both in lumbar spine and in femoral neck. Increasing MS duration and increasing dosage of intravenous corticosteroids were independently and negatively associated with both lumbar spine and femoral neck BMD. CONCLUSIONS We documented no correlation between vitamin D levels and decreased BMD at femoral neck and lumbar spine in RRMS patients. Vitamin D insufficiency appears not to be the underlying cause of secondary osteoporosis in MS.

The impact of comorbidity and other clinical and sociodemographic factors on health-related quality of life in Greek patients with Parkinson's disease

Objectives: This study was designed to evaluate the impact of other common self-reported comorbid disorders (hypertension, dyslipidemia, ischemic heart disease, diabetes mellitus, minor stroke, arthritis, low back pain or osteoporosis and depression) on health-related Quality of Life (HRQoL) of Parkinsons disease (PD) patients and to explore the association of their HRQoL with various sociodemographic and clinical factors. Methods: Data about age, gender, education, occupation, income, marital and residential status, social relations, disease duration, functional status, treatment and concomitant diseases were collected of 139 Greek patients (68 men and 71 women) with PD. Patients were consecutively recruited from the outpatient clinic of the first Neurology Department of Athens National University at Aeginition Hospital. Disease severity was assessed using the unified Parkinsons disease rating scale including Hoehn and Yahr and Schwab and England (S&E) scales. HRQoL was measured by the specific Parkinsons disease questionnaire (PDQ-39). A multivariate multiple regression model with normal errors was used for the statistical analysis. Results: The main determinants of HRQoL were low degree of independence measured by the S&E scale (F = 35.942, p < 0.001), social isolation (F = 20.508, p < 0.001), disease duration (F = 14.983, p < 0.001), sleep (F = 6.507, p = 0.013) and gastrointestinal disturbances (F = 4.643, p = 0.035) and the presence of depression (F = 6.022, p = 0.017). Conclusion: Among the other chronic comorbidities only depression was associated with a poor HRQoL in PD patients. Functional dependence and social isolation contributed most to worse HRQoL. Our findings suggest that adequate social support and management of depression, sleep and gastrointestinal disturbances could reduce the distress and improve HRQoL in patients with PD.

Familial asymmetric distal upper limb amyotrophy (Hirayama disease): report of a Greek family.

Introduction:Hirayama disease is a rare nonprogressive, predominantly unilateral, juvenile distal upper limb amyotrophy that involves C7, C8, and Th1 innervated muscles. The etiology and pathogenesis of this focal amyotrophy is presently unknown. There is a debate as to whether Hirayama disease is an unusual neck flexion induced cervical myelopathy or an intrinsic motor neuron disease. Despite being a sporadic disorder, familial forms have been occasionally described, with either autosomal recessive or dominant inheritance. Case Series:We describe a 3-generation Greek family, with 4 members affected by a benign distal upper limb amyotrophy of long duration, reminiscent of Hirayama disease, suggesting an autosomal dominant inheritance pattern. Hypothesizing that this familial amyotrophy might be related to autosomal dominant distal spinal muscular atrophy type V(dSMA-V) that is characterized by prominent involvement of the distal upper extremities, we tested the index case for glycyl tRNA synthetase and Berardinelli-Seip congenital lipodystrophy (BSCL2) N88S and S90L gene mutations (by direct sequencing) that are involved in the development of dSMA-V phenotype. Despite the phenotypical similarity of this familial amyotrophy to dSMA-V, no missense mutation in the genes presently associated with it was detected. Conclusion:The reported family is the first in the literature with occurrence of Hirayama amyotrophy in 3 generations of a family. Considering that familial forms of Hirayama amyotrophy are uncommon, it could be assumed that they might represent a different subtype of the same disease having the same clinical features but different pathogenesis.

Benign Raeder Syndrome Evolving Into Indomethacin-Responsive Hemicranial Headache

Benign Raeder syndrome is characterized by a self‐limiting unilateral continuous headache associated with ipsilateral ptosis, miosis, and frequently, facial hypohydrosis. Hemicrania continua is a chronic, strictly unilateral continuous headache associated with ipsilateral cranial autonomic symptoms. We report a 50‐year‐old man who presented with benign Raeder syndrome, which evolved into an indomethacin‐responsive hemicranial headache that resembled hemicrania continua.

Prevalence of cerebrospinal fluid oligoclonal IgG bands in Greek patients with clinically isolated syndrome and multiple sclerosis

UNLABELLED Lower prevalence of cerebrospinal fluid oligoclonal IgG bands (IgG-OCBs) has been reported in multiple sclerosis (MS) patients from Southern Europe compared to other western countries. OBJECTIVES We aimed to determine the prevalence of CSF OCBs in Greek MS patients and to examine their relation with some selected clinical and demographical features. METHODS Included patients fulfilled the 2005 McDonald criteria for definite MS (CDMS) or clinically isolated syndrome (CIS) and had a spinal tap performed between 2006 and 2010. Paired CSF and plasma samples were analyzed using isoelectric focusing followed by IgG-specific immunofixation. A pattern of two or more bands present only in the CSF was defined as positive. OCB status was correlated with age at disease onset, initial symptomatology, relapse rate, disease subtype, disease duration, medication, EDSS score and MSSS. RESULTS Of the 231 included patients (53.2% with CDMS and 48.6% with CIS) 67.5% had OCBs. The prevalence of positive patterns did not differ between CIS and CDMS patients (67.6% vs. 67.5%, respectively). OCB-positive patients were younger than OCB-negative patients (35.2±10.3 vs. 38.7±11.8 years respectively, p=0.022) and had more frequently cervical spinal cord lesions (x2=7.08, p=0.008). No difference was observed between the two subgroups in the other studied disease parameters. CONCLUSION Despite the lower frequency of positive IgG-OCB patterns in our patients, both subgroups were mostly similar with regard to their clinical and demographic characteristics suggesting that the OCB status lacks prognostic significance in MS.

Auditory P300 event-related potentials in patients with type 2 diabetes mellitus

Abstract Background: There is increasing interest in the impact of diabetes mellitus on cognitive functioning. Several studies found evidence of decreased cognitive performance in type 2 diabetics (T2DM). Since the P300

Stiff person syndrome: avoiding misdiagnosis

Stiff person syndrome (SPS) is a rare neurological disorder characterised by muscular rigidity and superimposed spasms of the trunk and limbs that may be precipitated by voluntary movements and unexpected tactile, auditory or emotional stimulation. The high prevalence of autoantibodies against glutamic acid decarboxylase (antiGAD) in both serum and cerebrospinal fluid, as well as the frequent association of SPS with other autoimmune disorders, suggest an autoimmune pathogenesis. SPS is frequently misdiagnosed as axial dystonia or psychogenic movement disorder. We report a patient with SPS in order to emphasise the reasons for this common misdiagnosis.