Cyclic alternating pattern analysis in REM sleep behavior disorder.

OBJECTIVE: Rapid eye movement (REM) sleep behavior disorder (RBD) is a type of REM parasomnia characterized by complex motor activity during REM sleep. In this study, cyclic alternating pattern (CAP) in patients with idiopathic RBD was analyzed to evaluate the expression of arousal instability of NREM sleep. METHODS: A total of 31 idiopathic RBD patients and age- and gender-matched 21 control subjects were consecutively recruited. Conventional sleep polysomnographic recording parameters and CAP parameters were compared between RBD and the control group. RESULTS: The number of CAP cycles (120.13 ± 113.56, p = 0.007), CAP sequences (20.9 ± 18.15, p < 0.001), CAP index (25.14 ± 24.44, p = 0.017), and CAP rate (24.07 ± 13.22, p = 0.016) were all significantly higher in RBD patients compared to the control group. The increase in CAP sequences was observed in phase A2 and A3 subtypes while phase A1 subtype was significantly lower in RBD patients. A significant positive correlation was observed between disease duration with total CAP time (r = 0.289, p = 0.042) and A3 index (r = 0.32, p = 0.024). There was a negative correlation between the age and A1 index (r = -0.4491, p = 0.0001). CONCLUSION: To our knowledge this is the first polysomnographic clinical study which evaluated CAP parameters in RBD. Increased CAP rate found may be considered as a sign showing that NREM sleep may also be affected in RBD patients. Therefore, CAP analysis may be important to enlighten the pathogenesis of parasomnias.

Treatment with enhanced external counterpulsation improves cognitive functions in chronic heart failure patients.

OBJECTIVES: Chronic heart failure (CHF) has been associated with an increased risk of poorer cognitive performance in older adults. Reversibility of cognitive impairment after medical treatment has been reported, although the restorative effects of enhanced external counterpulsation (EECP) on cognitive performance have not been studied. We investigated the effect of EECP on cognitive functions in CHF patients. STUDY DESIGN: Thirty-six individuals (mean age: 66±8 years) who were diagnosed with CHF and were New York Heart Association (NYHA) Class II-III and Canadian Cardiovascular Society (CCS) Class II-III participated in this study. Neuropsychological assessment was performed in these patients. RESULTS: Patients in the EECP treatment group showed a statistically significant improvement in spontaneous naming (p=0.011) and forward row score of the attention subset among domains of cognition (p=0.020) and interference time of executive function (p=0.012). CONCLUSION: Enhanced external counter pulsation resulted in improvement in all domains of cognitive functions except verbal and visual memory tests.

Evaluation of MR-Tractography Findings in Hemifacial Spasm Patients Injected with Botulinum Neurotoxin.

Background and Introduction: Botulinum neurotoxin (BoNT) is a potent biological toxin extracted from Clostridium Botulinum bacteria. BoNT injection is mainly used for medical purposes; it is frequently used for cosmetic purposes as well. The hypothesis that frequent application of this treatment modality may also affect the central nervous system constitutes the subject of our study. Objective: We aimed to demonstrate the possible central effects of BoNT in hemifacial spasm patients. Methods and Materials: Diffusion tensor imaging was used for this study. Patients were divided into two groups, and the measured values for each determined bilateral neuroanatomic region were compared within the relevant group. Results: Fractional anisotropy (FA) and apparent diffusion coefficient (ADC) values were found to be closer to the pathological values in the right motor cortex and in the left internal capsule areas of the patients who were injected with BoNT into the left side, in the left motor cortex area of the patients who were injected with BoNT into the right side. No significant changes were detected in other regions. Conclusion: Botulinum neurotoxin administration in patients with hemifacial spasms may cause some changes in the central nervous system as well as peripheral effects. In the case of similar studies supporting pathological changes, BoNT treatment modalities or appropriate indications may be reviewed, and regulation on excessive cosmetic use may be in question.

Fahr's disease presenting with paroxysmal non-kinesigenic dyskinesia: a case report.

Fahr's disease is characterized by presence of abnormal calcifications in certain areas of the brain. We report on 23-year-old man admitted to us with the episodes of paroxysmal non-kinesigenic dyskinesia. He was detected to have symmetrical intracerebral calcifications in basal ganglia, thalamus and cerebellar hemispheres, and diagnosed as sporadic Fahr's disease. Paroxysmal dyskinesia was well responded to oxcarbazepine (600 mg/day) treatment.

Route learning impairment associated with encephalomalasia secondary to traumatic brain injury: a case report.

Topographical disorientation is marked by difficulty finding one's way in familiar or new environments. The present case study reports findings from a 30-year-old male with encephalomalasia of the left parahippocampal region secondary to brain trauma with subsequent difficulty in learning of new routes. His navigation in premorbidly known (familiar) surroundings was intact. Magnetic resonance images revealed left parahippocampal and bilateral occipital encephalomalasia. Neuropsychological screening showed impairment in structuring a representation of the spatial relationships among landmarks with relatively preserved ability to learn visual and verbal information of these landmarks. Decreased visual perception and inappropriate visual inputs due to cervical dystonia and right homonymous hemianopsia also appear to play a role in his disability. The current knowledge about the neuronal systems involved in visual cognition and topographical orientation also are addressed in this report.

Levetiracetam-responding paroxysmal nonkinesigenic dyskinesia.

We report on a patient with 7-year history of short-lasting paroxysmal nonkinesigenic dyskinesia. The episodes occurred 100 to 125 times per day, lasted from 5 seconds to 3 minutes, and were not suppressed with sleeping, underlining the heterogeneity of phenomenology in paroxysmal dyskinesias. Neuroimaging studies showed calcifications in the basal ganglia, thalamus, brain stem, and subcortical and cerebellar regions. He was diagnosed with idiopathic hypoparathyroidism. After failure of valproate, he responded well to levetiracetam (1000 mg/d). This report revealed that intracerebral calcifications secondary to hypoparathyroidism could present as paroxysmal nonkinesigenic dyskinesia, and levetiracetam could be effective in this particular entity.

Magnetization transfer ratio in early period of Parkinson disease.

RATIONALE AND OBJECTIVES: The aim of this study is to determine the role of magnetization transfer ratio (MTR) in the early period of Parkinson disease (PD). MATERIALS AND METHODS: Clinically diagnosed 33 patients with PD in the first year of diagnosis, and 30 healthy volunteers were assessed. Magnetic resonance imaging (MRI) was performed without and with magnetization transfer (MT) imaging. Signal intensity measurements were obtained from 15 anatomic regions (substantia nigra pars compacta [SNPC], substantia nigra pars reticulate [SNPR], red nucleus, dentate nucleus, cerebellum, pons, globus pallidus, putamen, caudate nucleus, thalamus, internal capsule posterior horn, forceps major, forceps minor, and genu and splenium of corpus callosum) and MTR was calculated. Comparisons of the findings between each anatomic location of the patients with PD and normal subjects were performed. RESULTS: Most prominent decrease of MTR was found in SNPC (p < 0.001). A significant decrease of MTR was also found in the SNPR (p = 0.006), red nucleus (p = 0.037), and pons (p = 0.046). The other regions lack significance. CONCLUSION: MTR analysis is a useful technique for initial PD assessment. Even in the first year of diagnosis, significant reduction of MTR is found in substantia nigra, red nucleus, and pons compared with that of the control group.

Psychiatric approach in the treatment of reflex sympathetic dystrophy in an adolescent girl: a case report.

Reflex sympathetic dystrophy (RSD) is an unusual diagnosis in the pediatric age group. It is a syndrome characterized by pain in one or more extremities with a significant morbidity in childhood. Patients with RSD have frequently undergone many unnecessary investigations such that the diagnosis and treatment may be considerably delayed. The pathophysiology remains unclear; however, a number of psychological problems were frequently suggested to play a role in this disorder. We describe a 13-year-old girl diagnosed as pediatric RSD who was admitted to a child and adolescent psychiatry unit with a history of severe pain in the right hand, increasing disability and symptoms of nervousness and withdrawal from social activities. In this report, we discuss psychogenic factors underlying the disorder of an adolescent girl and psychiatric approach as a part of a multimodal treatment of pediatric RSD.

Isolated abducens nerve palsy associated with retinoic acid therapy: a case report.

Isolated abducens nerve palsy is a rare complication of treatment with various drugs. Here, the authors report the case of a 23-year-old female with isolated left abducens nerve palsy after long-term retinoic acid therapy. The association is based on the temporal relationship and the exclusion of other possible etiologic factors following extensive laboratory and imaging diagnostics. The authors suggest that isolated abducens nerve palsy may be a presenting sign of a toxic neuropathy associated with retinoic acid therapy. After the exclusion of other organic lesions, especially idiopathic intracranial hypertension, and an assessment of the risk-benefit ratio, discontinuation of treatment must be considered in such cases.

Fibrous dysplasia of the cranial bones: a case report and review of the literature.

Fibrous dysplasia (FD) is a relatively uncommon disorder that affects primarily the cranial region; its occurrence in the cranial base in combination with hindbrain herniation and aneurysmal bone cyst (ABC) constitutes an extremely rare condition. We report a case of polyostotic fibrous dysplasia with progressive occipital, temporal, and clival involvement. Clinical findings and differential diagnosis with special emphasis on the imaging features were discussed. A small posterior fossa volume has been thought to lead to hind brain herniation. The resultant obstruction to the CSF pathways at the level of the foramen magnum has been implicated in the development and subsequent progression of syringobulbia.

Linking a compound-heterozygous Parkin mutant (Q311R and A371T) to Parkinson's disease by using proteomic and molecular approaches.

Parkin is an E3-protein ubiquitin ligase, which plays an important role as a scavenger in cell metabolism. Since the discovery of the link between Parkin and Parkinson's disease, Parkin was placed in the center of Parkinson's disease research. Previously, we isolated a mutant form of the Parkin protein (Q311R and A371T) from a Parkinson's disease patient. In this study, we aimed at characterizing this mutant Parkin protein by using biochemical and proteomic approaches. We used neuroblastoma cells (SH-SY5Y) as our model and created two inducible cell lines that expressed the wild type and the mutant Parkin proteins. We first investigated the effect of expressing both the wild type and the mutant Parkin proteins on the overall proteome by using 2D-DIGE approach. The experiments yielded the identification of 22 differentially regulated proteins, of which 13 were regulated in the mutant Parkin expressing cells. Classification of the identified proteins based on biological process and molecular function revealed that the majority of the regulated proteins belonged to protein folding and energy metabolism. Ingenuity Pathway Analysis predicted the presence of a link between the regulated proteins of the mutant Parkin expressing cells and Parkinson's disease. We also performed biochemical characterization studies on the wild type and the mutant Parkin proteins to make sense out of the differences observed at the proteome level. Both proteins displayed biological activity, had similar stabilities and localized similarly to the cytoplasm and the nucleus in SH-SY5Y cells. The mutant protein, however, was cut by a protease and subjected to a post-translational modification. The observed differences at the proteome level might be due to the differences in processing of the mutant Parkin protein. Overall, we were able to create a possible link between a pair of Parkin mutations to its pertinent disease by using 2D-DIGE in combination with biochemical and molecular approaches.

The sociodemographic findings, beliefs and behaviours of the patients admitted to Kocaeli University, Faculty of Medicine, Epilepsy Section.

Using a standard questionnaire, 121 consequent epileptic patients have been evaluated sociodemographically and according to their beliefs and behaviours about their illness. Monthly income per person was between 26 and 62.5 U.S. dollars (USD) for 3/4 of the patients. About half of the patients were concealing their illness. About one third of the patients' education was hindered because of their illness. When the patients who concealed their illness were compared with the ones who did not, it became clear that the education of the ones who did not conceal the illness was far more hindered. "Carrying an amulet" ratio was greater among the patients who had no social security when compared to the patients with social security. We concluded that prejudice against epileptic patients and superstitions in Turkish population are still valid. Physician should consider the expense of antiepileptics for both patients and the country when prescribing them. All parts of the society should be informed about epilepsy but this again depends on the country's budget.

Treatment of unilateral masseter hypertrophy with botulinum toxin in two cases.

Botulinum toxin is a new method for treating masseteric hypertrophy, which offers many advantages over conventional surgical treatment. We describe the successful outpatient medical management of two patients with masseteric hypertrophy using botulinum toxin type A. No significant side effects occurred and the patients were satisfied cosmetically six months after the treatment.

The effect of etanercept and methylprednisolone on functional recovery of the facial nerve after crush injury.

OBJECTIVE: To establish whether etanercept, a TNF-α antagonist, is an alternative and effective treatment on facial nerve after crush injury. METHOD: Fifty-four rats underwent exposure of the left main trunk of the facial nerve followed by a standard crush injury. Animals were randomly divided into 3 groups: control group, methylprednisolone-treated group, and etanercept-treated group. All these groups were divided into 2 subgroups; animals were sacrificed on the 4th day after facial crush injury in the first subgroup and on the 28th day in the second subgroup. Functional recovery of vibrissae movement, eye blink reflex, and vibrissae orientation was measured on a 3-point scale (1 = no recovery, 2 = partial recovery, and 3 = complete recovery) during the recovery process. Facial nerve, from the main trunk at the stylomastoid foramen to the zygomatic, buccal, and marginal branches, were dissected and postfixed in the same fixative. The paraffin sections were studied with macrophage marker, GAP-43 and T Cell Marker. RESULTS: Animals receiving etanercept demonstrated significantly better functional recovery compared with control and methylprednisolone-treated animals. The etanercept-treated group showed highest GAP-43 immunoreactivity in the nerves. After the macrophage marker and T cell marker staining, the etanercept and methylprednisolone groups demonstrated statistically significant difference compared with the control group (p < 0.001). CONCLUSION: The present study demonstrates accelerated functional recovery associated with etanercept treatment after facial nerve crush injury in rats.

The effect of pulse methylprednisolone plus theophylline treatment on clinical, pulmonary and inflammatory markers in relapses of multiple sclerosis.

OBJECTIVE: The aim of this study was to evaluate whether there was a relationship between severity of multiple sclerosis (MS) exacerbation and pulmonary function test (PFT) and to determine the effect of theophylline, which was added to intravenous methylprednisolone, on serum Tumor Necrosis Factor (TNF)-alpha levels and clinical scores in MS relapses. STUDY DESIGN: Double blind randomized controlled trial. MATERIAL AND METHODS: The baseline Expanded Disability Status Scale (EDSS) score was determined, PFT was performed and blood was taken for analysis of TNF- alpha in patients with MS exacerbation. Patients were randomly divided into two groups; group 1 received intravenous methylprednisolone+IV theophylline and group 2 intravenous methylprednisolone+placebo for 5 days. PFT and EDSS score were repeated and blood was taken for TNF-alpha on the 5(th) and 30(th) days of the treatment. RESULTS: Twenty-four patients (14 female, 10 male) were included in the study. Mean age was 32.6±9, duration of disease was 5.4±4.2 years, number of exacerbations was 5±2. There was a significant correlation between the number of exacerbations and EDSS score (p=0.000, r=1). Restrictive PFT findings were detected in 8 and decrease in carbon monoxide diffusing capacity (DLCO) in 3 cases. In within-group analysis, EDSS score was found to be decreased on day 5 and still low on day 30 in the theophylline group (baseline 3±1.3; 5(th) day 2.4±1.6; 30(th) day 2±1.7). There was no statistically significant difference in the EDSS score of the placebo group (3±1.6; 2.8±1.7; 2.4±1.9 respectively). While serum TNF-alpha level was not changed in the placebo group, there was a non-significant decrease on day 5 and increase on day 30 in the theophylline group. There was no correlation between the clinical parameters, PFT and TNF-alpha level. CONCLUSION: There was no correlation between severity of MS and PFT findings. It is suggested that theophylline might be effective in MS exacerbations since it caused decreases in clinical scores; studies with longer treatment duration are needed to clarify its possible anti-inflammatory effect.

Ischemic stroke history predicts increased cardiovascular mortality in chronic heart failure.

OBJECTIVE: To investigate comorbidities that predict cardiac mortality and re-hospitalization in chronic heart failure (CHF) patients. METHODS: Five hundred eighty patients (mean age 63 ± 13 years, 373 male, 207 female, mean ejection fraction (EF) 26 ± 9%) with mild, moderate or severe CHF [NYHA class II-IV] were included in this prospective observational study. We evaluated all comorbidities such as history of ischemic stroke, coronary artery disease, peripheral arterial disease, chronic obstructive lung disease, hypertension, diabetes mellitus and chronic kidney disease in CHF patients who were hospitalized due to decompensated heart failure in Kocaeli University, Faculty of Medicine's Hospital between January 2003 and July 2009. Cox regression and Kaplan-Meier survival analyses were used to establish predictors of unfavorable outcomes. RESULTS: Of 580 patients 207 (36%) patients died due to cardiovascular reasons. In multivariable Cox regression analysis age (HR-1.06, 95% CI 1.04-1.08, p<0.001), NYHA functional class (HR-3.20 95% CI, 1.90-5.41, p<0.001), history of ischemic stroke (HR-2.48, 95% CI 1.14-5.37, p=0.022), high-sensitive C-reactive protein (HR-1.09, 95% CI, 1.04-1.15, p=0.001), brain natriuretic peptide (HR-1.00, 95% CI 1.00-1.00, p=0.01) and hemoglobin (HR-0.90, 95% CI 0.81-0.99, p=0.038) were independent predictors of cardiac death in the present study. History of ischemic stroke was demonstrated as an important comorbidity that predicts cardiovascular mortality beyond other co-morbidities in CHF patients. NYHA functional class (HR-2.85, 95% CI 1.80-4.65, p<0.001), left ventricular EF [(HR-0.98, 95% CI 0.95-0.99, p=0.039) and ischemic stroke history (HR-2.41, 95% CI 1.15-5.05, p=0.019) were independent predictors for recurrence hospitalization. The stroke history was only predictor showing recurrent hospitalization at least in one year among the other comorbid conditions, which were evaluated during study. CONCLUSION: History of ischemic stroke may be an important risk factor for increased cardiac mortality and recurrence hospitalization in CHF patients.

The effect of memantine in harmaline-induced tremor and neurodegeneration.

Essential tremor (ET) is one of the most common and most disabling movement disorders among adults. The drug treatment of ET remains unsatisfactory. Additional therapies are required for patients with inadequate response or intolerable side effects. The current study aims to investigate the anti-tremogenic and neuroprotective effects of memantine (NMDA receptor antagonist) on the harmaline model of transient action tremor. The effects of memantine were further compared with ethanol. Three separate groups of male Wistar rats were injected either with saline, ethanol (1.5 gr/kg), or memantine (5 mg/kg) 15 min prior to a single intraperitoneal injection of harmaline (20 mg/kg). Tremor and locomotion were evaluated by a custom-built tremor and locomotion analysis system. After 24 h of harmaline injection, cellular viability, and apoptosis were assessed using crystal violet staining, and caspase-3 immunostaining, respectively. Harmaline caused neuronal cell loss and caspase-3 mediated apoptosis in cerebellar granular and purkinje cells as well as the inferior olivary neurons. Despite a reduction in tremor intensity and duration with ethanol, this compound resulted in cell loss in cerebellum and olivary nucleus. Memantine exhibited neuroprotective efficacy on cerebellar and inferior olivary neurons albeit weaker anti-tremor effect compared to ethanol. In conclusion, anti-tremogenic and neuroprotective effects do not necessarily overlap. Memantine is a potential treatment for ET particularly given its neuroprotective efficacy.

Correlation between retinal morphological and functional findings and clinical severity in Parkinson's disease.

BACKGROUND: To measure the parapapillary retinal nerve fiber layer and macular thickness and macular volume in vivo and to evaluate whether retinal structural changes and visual cortical responses may be related to the clinical severity of the PD. METHODS: We included 17 patients with Parkinson's disease and 11 healthy subjects of a similar age. Unified Parkinson's disease rating scale scores in Parkinson's disease and control subjects were assessed for clinical evaluation. The retinal nerve fiber layer thickness, macular thickness and volume were measured by commercially available optical coherence tomography Model 3000 unit. Peak latencies of P100 component were measured by pattern visual evoked potential examination. RESULTS: The mean retinal nerve fiber layer average thickness was significantly reduced in Parkinson's disease patients (98.76 +/- 10.90 microm) when compared with those of control subjects (114.54 +/- 5.72) (P < 0.05). The retinal thickness reduction was statistically significant in superior inner macula; temporal, nasal and inferior quadrants of outer macula (P < 0.05)(Table 2). The mean total macular volume of Parkinson's disease patients (6.82 +/- 0.32 mm(3)) was significantly reduced when compared with those of control subjects (7.09 +/- 0.23 mm(3)). Highly significant inverse correlation between foveal retinal thickness and total and motor subscores of Unified Parkinson's disease rating scale was observed in Parkinson's disease patients, respectively (r = -0.660; P = 0.004), (r = -0.625, P = 0.007). There was a moderate nearly significant inverse correlation between total macular volume and P100 latency in PD (r = -0.328; P = 0.058). CONCLUSIONS: In Parkinson's disease patients there is a reduction of retinal nerve fiber layer thickness, macular thickness and volume evaluated in vivo by optical coherence tomography. Reduced foveal thickness which is not found to be statistically different from normal is correlated to the severity of disease.

Posttraumatic stress disorder and major depressive disorder is common in parents of children with epilepsy.

OBJECTIVE: The purpose of the study was to determine the prevalence of posttraumatic stress disorder (PTSD), posttraumatic stress symptoms (PTSS), and major depressive disorder (MDD) in parents of children with epilepsy. METHODS: Parents (77 mothers and 3 fathers) of children with epilepsy were administered the Structured Clinical Interview for DSM-IV (SCID), PTSD and MDD modules, and the General Health Questionnaire (GHQ). RESULTS: The prevalence of both PTSD and MDD was 31.5%. Fifty-six percent (n = 14) of the participants with PTSD had a diagnosis of MDD. PTSD symptom clusters were very prevalent in the parents of children with epilepsy. Reexperiencing and arousal symptom clusters were more frequent (88.8 and 80% respectively) than the avoidance and numbing symptom cluster (32.5%). CONCLUSION: These findings suggest that a significant proportion of parents of children with epilepsy experience PTSD and MDD. Increased awareness of the clinical presentations of these disorders may help clinicians to develop preventive and intervention strategies for parents of children with epilepsy.