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Antiparkinsonian drugs especially dopamine agonists have many side effects. Dopamine agonists have been linked with compulsive inappropriate behaviours such as gambling, compulsive shopping, hobbyism, other repetitive or purposeless behaviours such as punding, compulsive eating and hypersexuality. However pathological hypersexuality has rarely surfaced in routine Parkinson’s disease practice. We present clinical findings of a seventy-three year old male Parkinson’s disease patient with diagnosis of hypersexuality as a side effect of pramipexole. After pramipexole was withdrawn, dopamine agonist was changed to ropinirole and full remission was observed. Prevalence of psychiatric disorders in patients suffering from Parkinson’s disease varies from 12 to 90%. Hypersexuality is a common impulse control disorder seen in Parkinson’s disease and noted in these patients taking a variety of dopaminergic agents; including levodopa, dopamine agonists or MAO inhibitors. In our patient, pramipexole caused hypersexuality unexpectedly. Since this case constitutes interesting findings in two different points, one is hypersexuality with pramipexole, the other is no hypersexuality with use of ropinirole after pramipexole withdrawal, it is chosen to be presented here.

Dysphagia is one of the most common and important complications of stroke. It is an independent marker of poor outcome after acute stroke and may become chronic after the acute period and continues to affect all aspects of the patient's life. Patients with stroke may encounter any of the medical branches in the emergency room or outpatient clinic, and as in our country, there may not be specialists specific for dysphagia, such as speech-language pathologists (SLP), in every hospital. This study aimed to raise awareness and create a common opinion of medical specialists for stroke patients with dysphagia. This recommendation paper has been written by a multidisciplinary team and offers 45 recommendations for stroke patients with dysphagia. It was created using the eight-step Delphi round via e-mail. This study is mostly specific to Turkey. However, since it contains detailed recommendations from the perspective of various disciplines associated with stroke, this consensus-based recommendation paper is not only a useful guide to address clinical questions in practice for the clinical management of dysphagia in terms of management, diagnosis, and follow-up, but also includes detailed comments for these topics.

Dysphagia is one of the most common and important complications of stroke. It is an independent marker of poor outcome following acute stroke and it continues to be effective for many years. This consensus-based guideline is not only a good address to clinical questions in practice for the clinical management of dysphagia including management, diagnosis, follow-up, and rehabilitation methods, but also includes detailed algorithms for these topics. The recommendation paper has been written by a multidisciplinary team and offers 117 recommendations for stroke patients with dysphagia. While focusing on management principles, diagnosis, and follow-up in the 1st part (45 items), rehabilitation details were evaluated in the 2nd part (72 items).

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that affects both upper and lower motor neurons and its etiology is not fully understood. The incidence of ALS is 2-3/100,000 people in the world. Although ALS occurs sporadically in most patients, 5-10% of patients are thought to have genetic inheritance. The most common gene mutations are C9orf72, superoxide dismutase 1 (SOD1), TDP43, FUS, and ubiquilin 2. In our study, within the light of the literature, we wanted to represent three patients with familial ALS who had SOD1 and C9orf72 gene mutations, who were observed in detail in our clinic in terms of clinical, electromyographic, and genetic findings.

Paraneoplastic neurologic syndromes (PNS) are rare disorders, which are remote effects of cancer that are not caused by the tumor, its metastasis or side effects of treatment. We had nine patients with PNS; two of our patients had limbic encephalitis, but one had autoimmune limbic encephalitis with no malignancy; two patients had subacute cerebellar degeneration; three had Stiff-person syndrome; one had Lambert-Eaton myasthenic syndrome; and the remaining patient had sensory neuronopathy. In most patients, the neurologic disorder develops before the cancer becomes clinically overt and the patient is referred to a neurologist. Five of our patients’ malignancies had been diagnosed in our clinic after their neurologic symptoms became overt. PNS are more common than expected and neurologists should be aware of the variety of the clinical presentations of these syndromes. When physicians suspect PNS, cancer screening should be conducted. The screening must continue even if the results are negative

INTRODUCTION: Acute ischemic stroke is an emergency which can be treated by intravenous thrombolytic treatment in the first 4.5 hours. The most important complication effecting prognosis of intravenous thrombolysis is the symptomatic intracerebral hemorrhage. The aim of our study is to determine the risk factors of intracerebral hemorrhage due to intravenous thrombolytic treatment. METHODS: In our study, 12 patients with intracerebral hemorrhage due to intravenous thrombolytic therapy hospitalized in Neurological Intensive Care Unit between 2009-2014 were revised retrospectively. RESULTS: Twelve patients who had intracerebral hemorrhage due to thrombolytic treatment were included. Nine of the cases (75.0%) had asymptomatic intracerebral hemorrhage, whilst 3 (% 25.0) had symptomatic intracerebral hemorrhage. DISCUSSION AND CONCLUSION: There were many studies aiming to put forth the risk of intracerebral hemorrhage associated with intravenous thrombolytic therapy. Advanced age, body weight > 95 kg, the history of diabetes mellitus and hypertension, the use of antiaggregant agents, symptom-treatment interval, hyperglycemia at the time of arrival, high value of the National Institutes of Health Stroke Scale, the presence of dense artery in first cranial computerized tomography, the size of the ischemic infarct were determined as risk factors. Also, our results showed that hyperglycemia and high blood pressure was associated with parenchymal hemorrhage.

Summary Objective To investigate electrophysiological parameters of swallowing in all stages of Alzheimer's disease. Methods Forty Alzheimer's disease patients, 20 age-matched normal controls and 20 young normal controls were included. Dysphagia limit (DL) and sequential water swallowing (SWS) tests were performed. Cardiac rhythm, respiration and sympathetic skin responses were concomitantly recorded. Results Dysphagia was found in 30/40 (75%) of Alzheimer's disease patients. Mean volume at the DL test was significantly reduced (16.5 ± 1.0 mL) in the Alzheimer's disease group. Swallowing and apnea times in the SWS test were significantly prolonged in elderly controls, but even longer in Alzheimer's disease patients. Conclusions Alzheimer's disease patients had electrophysiological features of dysphagia, even in the early period of disease. The cortical involvement and severity of cognitive disorder can increase swallowing problems, but subclinical signs of dysphagia may be observed even in patients with mild or moderate Alzheimer's disease.

The reliability and diagnostic value of Lhermitte's sign in multiple sclerosis (MS) has not been fully established. The purpose of this study was to determine the clinical, neurophysiological and neuroradiological correlations of Lhermitte's sign in a cohort of MS patients and reassess the relevance of this phenomenon in the clinical history of the disease. A prospective study of 694 patients with MS and 110 age-matched healthy adults was evaluated by a structured questionnaire that included basic demographic data, age of onset, clinical characteristics of the disease, and the inquiry of Lhermitte's sign. Cranial and spinal magnetic resonance imagings (MRI) and median and tibial somatosensory evoked potentials (SSEP) were performed at the same time. One hundred and twelve (16 %) patients were reported to have Lhermitte's sign; 582 (84 %) patients did not experience Lhermitte's sign during their disease duration (P < 0.026). No correlation was found between Lhermitte's sign and age, gender, EDSS, and disease duration; 88 % of patients with Lhermitte's sign had a demyelinating lesion on the cervical MRI. In negative Lhermitte's sign group, 64 % patients had a positive MRI. SSEP conductions were delayed in 92 % of patients with positive Lhermitte's sign and in 70 % of patients with negative Lhermitte's sign. Regarding the data, a significant correlation was found between MRI lesion and Lhermitte's sign (P < 0.001), and between SSEP abnormality and Lhermitte's sign as well (P < 0.001). This study underlines the relevance of this phenomenon with neuroradiological and neurophysiological abnormalities.

Objective Neurogenic dysphagia (ND) is a prevalent condition that accounts for significant mortality and morbidity worldwide. Screening and follow-up are critical for early diagnosis and management which can mitigate its complications and be cost-saving. The aims of this study are to provide a comprehensive investigation of the dysphagia limit (DL) in a large diverse cohort and to provide a longitudinal assessment of dysphagia in a subset of subjects. Methods We developed a quantitative and noninvasive method for objective assessment of dysphagia by using laryngeal sensor and submental electromyography. DL is the volume at which second or more swallows become necessary to swallow the whole amount of bolus. This study represents 17 years experience with the DL approach in assessing ND in a cohort of 1278 adult subjects consisting of 292 healthy controls, 784 patients with dysphagia, and 202 patients without dysphagia. A total of 192 of all patients were also reevaluated longitudinally over a period of 1–19 months. Results DL has 92% sensitivity, 91% specificity, 94% positive predictive value, and 88% negative predictive value with an accuracy of 0.92. Patients with ALS, stroke, and movement disorders have the highest sensitivity (85–97%) and positive predictive value (90–99%). The clinical severity of dysphagia has significant negative correlation with DL ( r = −0.67, p Conclusions We propose the DL as a reliable, quick, noninvasive, quantitative test to detect and follow both clinical and subclinical dysphagia and it can be performed in an EMG laboratory. Significance Our study provides specific quantitative features of DL test that can be readily utilized by the neurologic community and nominates DL as an objective and robust method to evaluate dysphagia in a wide range of neurologic conditions.

Neuroleptic malignant syndrome (NMS) is a rare but life-threatening clinical manifestation induced by neuroleptic medication. Although NMS is regarded as a psychiatric diagnosis, its treatment requires a systematic approach and thus intensive care follow-up. In this paper, we report nine cases with NMS followed up in our Neurology Intensive Care Unit over the last three years.

Patients with Guillain-Barré syndrome (GBS), especially severe cases that require treatment in intensive care units, often experience swallowing difficulties. However, the oropharyngeal function of patients with GBS not treated in intensive care units is not typically evaluated using neurophysiological techniques.Electrophysiological techniques were used to determine dysphagia limit and sequential water swallowing values in an electromyography laboratory.This study assessed 18 patients with GBS who were not treated in the intensive care unit between 4 and 45 days after their hospital admission; 18 healthy volunteers were used as a control group. Of the 18 patients with GBS, 7 exhibited the clinical involvement of either a single cranial nerve or a combination of cranial nerves while 11 did not show any lower cranial nerve involvement. Clinical dysphagia was observed in seven patients and six of these cases involved a lower cranial nerve while five patients without cranial nerve involvement had silent dysphagia according to the dysphagia limit test. In addition, the duration of sequential swallowing was significantly prolonged in all patients with GBS compared with the control subjects.The present findings demonstrated that neurophysiological techniques are useful and easily applicable for patients with GBS and that there were no complications. Furthermore, cranial nerve involvement in patients with GBS likely increased the incidence of oropharyngeal dysphagia, and subclinical dysphagia may be present in this population as well. Therefore, neurophysiological techniques can be initially used and then repeated during follow-up visits for all types of patients with GBS.

Introduction Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiological entity characterized by headache, altered mental status, epileptic seizures, visual disturbances and typically transient changes in posterior cerebral circulation areas. In this article, we present a case of alcohol withdrawal accompanied by PRES. Case presentation A 53-year-old male patient presented to the emergency department with visual hallucinations and meaningless speech. History from his relatives revealed that he has been consuming alcohol for about 35 years and the last consumption was 3 days before the admission. On neurological examination, there was limited cooperation and disorientation was evident to person, place and time. The speech was incoherent. No localizing sign was observed. Cranial magnetic resonance imaging (MRI) revealed bilateral hyperintense areas in medial occipital cortices and in subcortical white matter extending partly into parietal region. Treatment for alcohol withdrawal was started. Signs and symptoms regressed on the 7th day of the treatment as well as the lesions on MRI. Discussion The clinical presentation, characteristic MRI features together with the reversible nature of the syndrome suggest the diagnosis of PRES. The precise pathophysiological mechanism of PRES still remains unclear. Hypertension, clinical conditions that are associated with impaired cerebral auto-regulation as well as alcohol use which increases the levels of reactive oxygen species and nitric oxide may lead to the disruption of endothelial cells and blood-brain barrier breakdown. Overall, in our case, we think chronic alcoholism and alcohol withdrawal might have caused endothelial dysfunction leading to PRES.

Background Sequential water swallowing (SWS) was mostly investigated by the videofluoroscopic and endoscopic methods. However some physiological features of SWS was rarely evaluated by neurophysiological techniques. Our aim was to investigate some neural and muscular changes on sequences of SWS using electromyography (EMG) methods. Methods Fifty-eight normal adults were investigated. SWS was initiated voluntarily with 50 ml and 100 ml water volumes from a cup. Submental EMG, respiratory signals, heart rate, and sympathetic skin responses (SSR) were measured during SWS. Key results All parameters were increased significantly during the 100 ml SWS. During swallowing apnea period, compensatory respiration cycles occurred in 24% and 48% of participants in the 50 ml and 100 ml SWS, respectively. Heart rate increased during swallowing apnea. SSR were evoked just before and just after the SWS in more than halves of participants. A foreburst EMG in SM muscles at the initiation of SWS was recorded in 86% of normal participants. Older age was associated with a prolonged duration of the apnea period. Conclusions All parameters of the SWS could be recorded numerically and objectively using electrphysiological methods. These are similar to those obtained by videofluoroscopic and similar methods. The foreburst activity of the initiation of SWS may represents preparatory activity from the activation of the fast cortical descending motor pathway. Increasing heart rate and the prolonged apnea urged that older people and patients could be carefully tested for respiratory and cardiac rhythm disorders.

Summary Study aims Autonomic changes, especially those of sympathetic skin responses (SSR), during sequential water swallowing (SWS) have not been systematically investigated. This study aims to electrophysiologically examine these autonomic changes (SSR and heart rate) that occur during 50 ml sequential water swallowing from a cup. Materials and methods Fifty-eight normal healthy adults were included in the study. Their submental muscle activity, respiratory activity, heart rate changes, and sympathetic skin responses were recorded during 50 ml water swallowing. In addition, we requested subjects to imagine drinking water as they did just before. The same recordings were performed during this imagination period. Results SSR appeared at the beginning and at the end of SWS in 52% of subjects. A first sympathetic skin response was evoked at the onset of SWS, and a second one appeared 8.6 ± 1.7 seconds after the first one and at the end of swallowing. Similar double SSRs were also obtained during imagination in most investigated subjects (33 out of 35 of selected subjects in a total group of 58 subjects). Swallowing tachycardia was observed during the SWS-associated apnea period, but not during the imagination period. Heart rate significantly increased during the SWS-associated apnea period. Conclusion The first SSR that appeared at the onset of swallowing is likely related to arousal. The appearance of a second response is a novel finding, which is probably related to the activity of subtil corticosubcortical networks. While discrete/single swallows can be used to evoke SSRs, SWS is unlikely to be clinically useful in its current form. In contrast, swallowing tachycardia could be a useful tool to examine dysphagic patients.

Background. Hereditary inclusion body myopathy is caused by biallelic defects in the GNE gene located on chromosome 9p13. It generally affects adults older than 20 years of age.Methods and Results. In this study, we present two Turkish sisters with progressive myopathy and describe a novel mutation in the GNE gene. Both sisters had slightly higher levels of creatine kinase (CK) and muscle weakness. The older sister presented at 38 years of age with an inability to climb steps, weakness, and a steppage gait. Her younger sister was 36 years old and had similar symptoms. The first symptoms of the disorder were seen when the sisters were 30 and 34 years old, respectively. The muscle biopsy showed primary myopathic features and presence of rimmed vacuoles. DNA analysis demonstrated the presence of previously unknown homozygous mutations [c.2152 G>A (p.A718T)] in the GNE genes.Conclusion. Based on our literature survey, we believe that ours is the first confirmed case of primary GNE myopathy with a novel missense mutation in Turkey. These patients illustrate that the muscle biopsy is still an important method for the differential diagnosis of vacuolar myopathies in that the detection of inclusions is required for the definitive diagnosis.

Intraoral trigeminal afferents elicit EMG activity from the lower facial muscle, orbicularis oris (OR) during swallowing. The upper facial muscles and especially orbicularis oculi (OC) were not previously known to be associated with deglutitional events. Nevertheless, given the large area of intraoral mucosa and teeth innervated by the trigeminal nerve afferents, a connection between OC motoneurons and deglutition may theoretically be expected, which we sought to evaluate in this study. Healthy controls were investigated for the possible synchronization of orbicularis OC and OR muscles during deglutition by the following methods: EMG activities were recorded during voluntary dry, 3-, 10-, 20-ml discrete wet swallowing, and sequential swallowing from a cup, concurrent with respiratory recording. A polygraphic recording was obtained from these muscles to determine whether they were synchronously activated during spontaneous swallowing. The polygraphic recording during spontaneous swallowing demonstrated that the OC and OR muscles were synchronously activated in all subjects. This synchronous activation was less prominent in voluntary discrete swallowing. It is proposed that this might be based on trigemino-solitarii-facial pathways with weaker connection to OC muscles. The synchronization of OC muscle activity with deglutition may be an evolutionary process that should be rudimentary in higher mammals including humans. The swallowing-induced cranial muscle activities could potentially explain some movement disorders, such as craniofacial dystonias.

highlights The prolongation of cauda equina motor conduction time was statistically significant in the lumbar abstract Objective: Lumbar spinal stenosis (LSS) is a chronic degenerative disease with pain in the back, buttocks and legs aggrevated by walking and relieved after rest without associated vascular disease of lower extremities observed in patients between 50 and 60 years. Several studies, using different methods indi- cated an association between slowing or blocking of root-nerve conduction and LSS. None of the previous research had applied the more conceivable methods such as recording the cauda equina potentials from the lumbar level or stimulating the spinal roots within the canal using either leg nerves or muscles. In this study, electrical lumbar laminar stimulation was used to demonstrate prolongation of cauda equina motor conduction time in lumbar spinal stenosis. Methods: Twenty-one LSS patients and age matched 15 normal control subjects were included in the study. Lumbar laminar electrical stimulation from L1 and L5 vertebra levels were applied by needle elec- trodes. Compound muscle action potential (CMAP) from gastrocnemius muscles were recorded bilater- ally. Latency difference of CMAPs obtained from L1 and L5 spine levels were accepted as the cauda equina motor conduction time (CEMCT). Results: CEMCT was significantly longer in patient group when compared to normal controls. Mean latency difference was 3.59 ± 1.07 msec on the right side, 3.49 ± 1.07 msec on the left side in LSS group, it was 1.45 ± 0.65 msec on the right side, 1.35 ± 0.68 msec on the left side on normal control group (p < 0.0001). Conclusions: The prolongation of CEMCT was statistically and individually significant in patient group. This may indicate that lower lumbosacral motor roots were locally and chronically compressed due to lumbar spinal stenosis. Lumbar spinal stenosis may have induced local demyelination at the cauda equina level. Significance: Since the prolongation of CEMCT was found only in patients with LSS, the method of laminar stimulation can be chosen for patients with uncertain diagnosis of LSS.

Background Acute centrum ovale (CO) infarction confined to the territory of perforating long medullary arteries is rarely reported. This study was designed to investigate the full spectrum of clinical findings, topography, and pathogenesis of patients with acute CO infarct. Methods A total of 64 patients with CO infarct were recorded. Risk factors such as age, hypertension, diabetes mellitus, hypercholesterolemia, smoking, and cardiopathy were recorded. Results The average age of the patients was 64.2 years. There were 46 patients (71.8%) in large-infarct group and 18 patients (28.1%) in small-infarct group. The most frequent risk factor was hypertension (89.1%). Other risk factors included smoking (39%), diabetes mellitus (29.7%), hyperlipidemia (28.1%), transient ischemic attack (15%), carotid stenosis (9%), atrial fibrillation (21%), other potential cardiac source of embolism (39%), and myocardial infarct (4%). The main clinical feature was faciobrachiocrural paralysis associated dysarthria (51.6%). Other clinical characteristics were facial, upper and lower extremity paralysis with sensorial deficits and/or dysarthria (26.6%), hemiparesis including face (12.5%), and either facial and upper or upper and lower extremity paralysis with hemisensorial deficit (9.4%). The baseline Barthel index was found to be less than 50 in 22 patients (34.4%) and greater than 50 in 42 patients (65.6%) for the entire group whereas 72% of patients were independent at discharge. Conclusion In this article, risk factors, clinical spectrum, and features of CO infarcts were correlated in a large patient group. The majority of CO infarcts were large and had a poorer outcome compared to previous reports.

Migraine attacks are characterized by headaches associated with neurological, gastrointestinal, and autonomic symptoms. A relationship between migraine and hypertension or hypotension is controversial. In this study, we aimed to determine if blood pressure changes were related to migraine attacks. From the outpatient clinic of our neurology department, 62 normotensive migraine patients with and without aura were chosen for study in accordance with the International Headache Society 2004 criteria. A questionnaire including general and specific questions was given to the patients to be filled out during 6 consequent migraine attacks. The patients received a fully automatic digital brachial upper arm sphygmomanometer (Omron M 4-1) to measure the changes in their blood pressure during attacks. The patients were asked to record their blood pressure changes 3 times: (1) just before or very early, (2) during (when headache peaks), and (3) 1 hour after the attack. Twenty-three of the 62 patients (57 women, 5 men) had migraine with aura (22 women and 1 man), and 39 of them did not have aura (35 women and 4 men). There was no statistically significant difference between systolic and diastolic values obtained before or very early, during the peak level, and 1 hour after the end of the attacks (P > 0.05). Although diastolic hypotensive values were not different statistically between groups, when all the patients were considered, diastolic hypotensive values were detected in a considerable number of patients (a total of 115 measurements). In this normotensive migrainous population, we observed that diastolic hypotension before or very early, during, and after migraine attack was the most significant result (5.1%). Although it was not statistically significant, the total number of hypotensive values was remarkable.

INTRODUCTION: The frequency of dementia is increasing all over the world. The aim of this study is to determine the prevalence of dementia mild cognitive impairment in the target nursing home population. MATERIAL-METOD: The diagnosis of dementia was made clinically. Imaging and biochemical tests were requested for differential diagnosis. RESULTS: Of the 102 patients who participated in the study, 57 were males and 45 were females. The average age of the total group was 74,81 years, the average age of demented group was 77,96 years and the average age of non-demented group was 72,62 years. The prevalence of dementia was 22.5% and the prevalence of mild cognitive impairment was 25.5%. Dementia was detected in 17.5% of males and 28.9% of females. In the dementia group was 25% who did not attend school, primary school-secondary school was 22,6%, high school and college graduate was 18,8%. CONCLUSION: As the elderly population in Turkey has increased rapidly in recent years, an increase in the prevalence of dementia is expected. As in many other countries, this is a significant public health problem. Future epidemiological studies should aim to increase the quality of life of patients through diagnosis in the early stages and the necessary precautions should be taken.

Lateral spreading and synkinetic responses of blink reflex are a sign of ephaptic transmission in idiopathic hemifacial spasm (HFS). The aim of this study was to evaluate the effect of botulinum toxin A (Btx A) on ephaptic transmission in idiopathic HFS.Thirty-three patients with idiopathic HFS were investigated. Btx A was injected only into the affected orbicularis oculi (OC) muscle. Electrophysiological studies were performed before and three weeks after the Btx A injection.After Btx A, the latencies of motor response and blink reflexes elicited from the OC muscle were significantly increased. The lateral spreading was not obtained in the OC muscle, while the orbicularis oris muscle response was not changed. There were no significant differences in the synkinetic responses of blink reflex. During needle EMG examination, positive sharp waves and fibrilation potentials were observed due to chemodenervation only in the OC muscle.Btx A affects only the neuromuscular junctions of the injected muscle and has no effect upon ephaptic transmission.

Oropharyngeal dysphagia is not rare in older children before the adult age, especially the patients with cerebral palsy. Non-invasive simple tests are needed for the evaluation of children with neurogenic dysphagia including the patients with cerebral palsy. So we aimed to evaluate non-invasive ways to screen for dysphagia in children and the usefulness of this almost new electrophysiologic method for the detection of dysphagia in children with cerebral palsy. Twenty-eight healthy children and 12 patients with cerebral palsy were investigated for the applicability of this method. The movement of the larynx was monitored using a simple piezoelectric wafer sensor and submental surface EMG activity was recorded by bipolar silver-chloride electrodes taped under the chin over the submental muscle complex. The onset and duration of pharyngeal swallowing was recorded from submental-suprahyoid muscles such as the mylohyoid-genitohyoid-anterior digastric complex. By this method, the maximal water volume capacity was measured in single swallows with progressively increasing water volumes, this was called 'dysphagia limit'. The healthy control children revealed to swallow the bolus at once maximally 11.2+/-0.4 and 2.5 ml in average. Dysphagia limit varied from 7 to above 20 ml water volume from age 5-16 years old. Patients with cerebral palsy had the dysphagia limit of 7.7+/-1.8 and 6.4 ml in average. The dysphagia limit was significantly reduced in patients with cerebral palsy (p0.05). Dysphagia limit seemed to be less sensitive in demonstrating the oropharyngeal swallowing disorders in childhood period (90% in the adult dysphagic patients). But the majority of patients with cerebral palsy (58%) showed abnormality. This electrophysiologic method is completely non-invasive, devoid from any hazard and applicable to children above 5 years. It may be candidate as a screening test before selection of dysphagic children.

Multiple Sclerosis is known to cause autonomic and sexual dysfunction. However, genitourinary and sexual problems in female multiple sclerosis patients are difficult to analyse. Therefore, it is an understudied field. As an attempt to fill this gap, we evaluated genital region autonomic dysfunction of female multiple sclerosis patients by using genital sympathetic skin response. Forty female patients with definite multiple sclerosis and twenty healthy female controls were included in the study. We examined hand, foot and genital sympathetic skin responses. Some genitourinary parameters were questioned by and results were compared with genital sympathetic skin response results. Among multiple sclerosis patients 42.5% of them had genitourinary and 75% had sexual problems clinically. Sympathetic skin response pathologies were distributed as following: 22.5% in hand, 20% in the foot, and 50% in genital region in the patient group. A statistically significant correlation between sexual dysfunction and genital sympathetic skin response could not been found. It is found that patients with multiple sclerosis have higher genital sympathetic skin response abnormalities than foot and hand sympathetic skin response abnormalities. This might be the early sign of autonomic dysfuntion in multiple sclerosis patients. Genital sympathetic skin response may be an easy and objective method to apply and helpful to evaluate genitourinary dysfunction in women in conjunction with female sexual dysfuntion index. Multiple Sclerosis 2007; 13: 742-748. http://msj.sagepub.com

A patient is described with some dystonic features related to the preswallowing period of oropharyngeal swallowing in the muscles of deglutition sequentially. This case may be called a "preswallowing dystonia."

Recently it has been proposed that corticobulbar innervation of the lower facial muscles is bilateral, that is from both right and left sides of the motor cortex. The objectives of this study were, i) to evaluate the corticonuclear descending fibers to the perioral muscles and, ii) to determine how central facial palsy (CFP) occurs and often recovers rapidly following a stroke. Eighteen healthy volunteers and 28 patients with a previous history of a stroke and CFP (mean ages: 51 and 61 years) were investigated by TMS (transcranial magnetic stimulation) with a figure of eight coil. Intracranial facial nerve and cortical motor evoked potentials (MEPs) were recorded from the perioral muscles. The periorbital MEPs were also studied. The absence of MEPs in both perioral muscles with TMS of the affected hemisphere was the most obvious abnormality. Also, central conduction time was significantly prolonged in the remaining patients. The mean amplitude of the affected hemisphere MEPs was diminished. The amplitudes of the unaffected hemisphere MEPs recorded from the intact side were enhanced especially in the first week following the stroke. During TMS, only the blink reflexes were elicited from the periorbital muscles due to stimulus spreading to trigeminal afferent nerve fibers. It is concluded that perioral muscles are innervated by the corticobulbar tract bilaterally. CFP caused by a stroke is generally incomplete and mild because of the ipsilateral cortical and multiple innervations out of the infarction area, and recovers fast through cortical reorganisation.

The nature of the oropharyngeal dysphagia in polymyositis/dermatomyositis (PM/DM) has been investigated by EMG methods. Nineteen patients with PM/DM were studied. The oropharyngeal phase of swallowing was evaluated by the electrophysiological methods measuring the laryngeal relocation time, pharyngeal transit time and the triggering of the pharyngeal phase of swallowing reflex. The EMG of cricopharyngeal muscle of the upper esophageal sphincter was also recorded in 10 patients. The patients have been compared with a group of 22 healthy controls matched with age and gender. Dysphagia limit was also measured for all patients and control subjects. Fourteen out of 19 patients could not swallow 20 ml or less amount of water at one go and divided the bolus into two or more pieces (piecemeal deglutition) in comparison to normal subjects. In PM/DM patients, the triggering of the swallowing reflex for the voluntarily initiated swallow was normal while the pharyngeal phase of swallowing was significantly prolonged. The cricopharyngeal sphincter muscle EMG demonstrated severe abnormalities in halves of the patients investigated. These findings demonstrated the weakness of the striated oropharyngeal muscles. Cricopharyngeal sphincter muscle was affected less frequently and showed either hyperreflexic or hyporeflexic states during swallowing. It is concluded that the pharyngeal stage of oropharyngeal swallowing is mainly involved in patients with PM/DM.

Objectives – Pseudotumor cerebri (PC) is a clinical condition characterized by signs and symptoms of increased intracranial pressure, such as headache and papilledema. Material and method – A total of 62 patients diagnosed with PC, who were on follow-up for a period ranging from 4 to 60 months, were investigated retrograde from 1990 to 1998 and then anterograde from 1998 to 2001 to find out the etiological factors, symptoms and signs and the prognosis in the western part of Turkey. Results – There were 47 (76%) women and 15 (24%) men. The age of onset of symptoms was 32.7 ± 9.9 (range 18–56) years. Obesity was found in only 17 (30%) of them. There were eight patients (13%) with venous sinus thrombosis causing PC. Five patients (8%) had Behcet's disease. The most common symptom was headache, recorded in 93% of the patients, which was followed by transient visual obscurations (60%). Snellen visual acuity was disturbed in 17 patients (27%) at the initial visit. Visual loss determined by automated perimetry was present in 71% of the cases. Three patients (3%) became blind in both eyes. Of the 62 patients, 41 were on follow-up during the study. Twenty-one (51%) showed regression of the visual field grade, nine patients (22%) worsened and 11 (27%) were stationary. Conclusion – Obesity was not as frequent as reported in western countries but Behcet's disease was found to be a frequent cause. Perimetry was the most reliable method to follow-up the patients.

Swallowing mechanism and neurogenic dysphagia in MS have been rarely studied by electromyographical (EMG) methods. This study aims to evaluate the presence of subclinical dysphagia in patients with mild multiple sclerosis (MS) using electrophysiological methods. A prospective study of 51 patients with relapsing remitting multiple sclerosis and 18 age-matched healthy adults was investigated. We used electromyography to measure the activity of the submental muscles during swallowing. Electrophysiological recordings of patients were obtained during relapse, after relapse, and at any time in remission period. Clinical dysphagia was found in 12 % of MS patients, while electrophysiological swallowing abnormalities were encountered in 33 % of patients. Subclinical dysphagia was determined in 35 % of patients during an MS relapse, in 20 % of patients after a relapse, and in 25 % of all 51 patients in the remission period based on EMG findings. Duration of swallowing signal of submental muscles in all MS patients was found to be longer than in normal subjects (p = 0.001). During swallowing of 50 ml of sequential water, the compensatory respiratory cycles occurred more often in MS patients than normal subjects, especially during a relapse (p = 0.005). This is the first study investigating swallowing abnormalities and subclinical dysphagia from the electrophysiological aspect in MS patients with mild disability. The electrophysiological tests described in this study are useful to uncover subclinical dysphagia since they have the advantage of being rapid, easy to apply, non-invasive, and without risk for the patients.

Objective In this study, it was aimed to describe the clinical, histopathological and genetic features of 20 patients with gamma sarcoglycanopathy confirmed by muscle biopsies and genetic analysis. Material and method We retrospectively reviewed 20 patients from whom muscle biopsy specimens were obtained between 2007 and 2012. All patients were clinically diagnosed as muscular dystrophy and biopsy materials were collected from five different centers of neurological disorders. All DNAs were extracted from muscle tissues or blood samples of patients and genetic tests (mutation analyses for gamma sarcoglycan gene and deletion-duplication analyses for all 4 sarcoglycan genes) were performed. Results The mean age of the patients was 7.6 years (2 -21 years). Only one case (5%) was older than 14 years. The mean CPK level was 10311 U/L (1311 - 35000 U∕L). There were 4 siblings in these series. Expression defects of gamma sarcoglycan staining were determined in (15 males, and 5 females) all patients with muscle biopsy specimens. But only in 9 of them, disease-causing defects could be determined with genetic analyses. Conclusion The present study has demonstrated that both examination of muscle biopsy specimens and DNA analysis remain important methods in the differential diagnosis of muscular dystrophies. Because dystrophinopathies and sarcoglycanopathies have similar clinical manifestation.

Objective : Cricopharyngeal (CP) muscle of the upper oesophageal sphincter (UES) has a significant role in the pharyngo-esophageal phase of deglutition. The linkage between the CP muscle of UES and the motor cortex has not been previously studied electrophysiologically in healthy humans and in patients with neurogenic dysphagia. Methods : Needle recordings of EMG responses were carried out from the CP sphincter muscle following transcranial magnetic stimulation (TMS) over the vertex around the Cz electrode position (cortical MEP), and on the parieto-occipital skull and the occiput ipsilaterally (peripheral MEP) in 14 healthy control subjects and in 26 patients with and without neurogenic dysphagia. Needle recordings obtained from the cricothyroid muscle of the larynx were also evaluated in six healthy subjects. Results : The cortical motor latency of CP sphincter muscle was 10.7±0.5 ms with an amplitude of 0.8±0.2 mV in healthy subjects. Both the latency and amplitude of CP-MEP were facilitated during swallowing. The peripheral MEP of the CP muscle was very stable in all normal subjects (5.1±0.3 ms; 1.3±0.3 mV) and swallowing did not influence these parameters. The cortically elicited CP-MEP was significantly longer than the cortical MEPs obtained from the cricothyroid muscle of the larynx. In 10 dysphagic patients with corticobulbar tract involvement (6 ALS and 4 pseudobulbar palsy) and with pathologic and hyperreflexic EMG of the CP-sphincter muscle, the cortical MEP of CP muscle of the upper esophageal sphincter could not be elicited, although the peripheral CP-MEPs were obtained. TMS never produced a swallowing movement in neither healthy subjects nor patients. Conclusion : The CP muscle of the upper esophageal sphincter can produce MEPs by cortical TMS and by stimulation at the root/nerve levels of vagus nerve. The MEP latency values and central motor delay suggest that there is an oligosynaptic corticobulbar pathway to the motoneurons of CP muscles. When the pathway is affected by a pathology (i.e. ALS or pseudobulbar palsy) the CP sphincter becomes hyperreflexic due to disinhibition and the cortical MEP of the CP muscle disappears due to degeneration of the corticobulbar pathway. These mechanisms appear to be responsible for the pathogenesis of dysphagia.

A patient is presented with acute onset of partial oculomotor paresis on one side and upper eyelid retraction on the other due to a vascular insult. An electromyographic study revealed frontalis mu...

We report a 68-year-old-man who suddenly developed transient, bilateral cortical blindness after coronary angiography, that appeared to be an adverse reaction to contrast agent. Vision and neuroradiological findings returned to normal within 24 hours.

Spontaneous and reflex movements have been described in brain death and these unusual movements might cause uncertainties in diagnosis. In this study we evaluated the presence of spinal reflexes in patients who fulfilled the criteria for brain death. Thirty-two (22 %) of 144 patients presented unexpected motor movements spontaneously or during examinations. These patients exhibited the following signs: undulating toe, increased deep tendon reflexes, plantar responses, Lazarus sign, flexion-withdrawal reflex, facial myokymia, neck-arm flexion, finger jerks and fasciculations. In comparison, there were no significant differences in age, sex, etiology of brain death and hemodynamic laboratory findings in patients with and without reflex motor movement. Spinal reflexes should be well recognized by physicians and it should be born in mind that brain death can be determined in the presence of spinal reflexes.