Best Practice Recommendations for Stroke Patients with Dysphagia: A Delphi-Based Consensus Study of Experts in Turkey-Part I: Management, Diagnosis, and Follow-up.

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.

Best Practice Recommendations for Stroke Patients with Dysphagia: A Delphi-Based Consensus Study of Experts in Turkey-Part II: Rehabilitation.

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).

[Posterior Reversible Encephalopathy Syndrome Triggerred By Alcohol Withdrawal]. / Alkol Yoksunlugu ile Tetiklenen Posterior Reversibl Ensefalopati Sendromu.

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.

Guillain-Barré Syndrome and Swallowing Dysfunction.

PURPOSE: 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. METHODS: Electrophysiological techniques were used to determine dysphagia limit and sequential water swallowing values in an electromyography laboratory. RESULTS: 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. CONCLUSIONS: 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.

GNE Myopathy in Turkish Sisters with a Novel Homozygous Mutation.

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.

Dysphagia in Alzheimer's disease.

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±1.0mL) 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.

Reassessment of Lhermitte's sign in multiple sclerosis.

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.

Electrophysiological Evaluation of Dysphagia in the Mild or Moderate Patients with Multiple Sclerosis: A Concept of Subclinical Dysphagia.

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.

Diagnostic value of "dysphagia limit" for neurogenic dysphagia: 17 years of experience in 1278 adults.

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<0.0001). 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.

Histopathological and genetic features of patients with limb girdle muscular dystrophy type 2C.

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.

Spinal reflexes in brain death.

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.

Intracranial hemorrhages related with warfarin use and comparison of warfarin and acetylsalicylic acid.

BACKGROUND: Acetylsalicylic acid (ASA) and warfarin are used to prevent ischemic cerebrovascular events. They have serious complications including intracranial hemorrhages (ICHs). Warfarin-related intracerebral hemorrhage (ich) incidence is .2%-5% in population that accounts for 10%-12% of all ichs. In this article, we investigated the profile of ASA and warfarin-related spontaneous ICHs in comparison with ICHs without any drug use (WADU) with their clinical, radiological, and biochemical properties. METHODS: In all, 486 patients aged 18-101 years with spontaneous ICHs were included. Patients constituted 4 separate groups: users of warfarin, ASA, ASA + warfarin, and WADU. Clinical, neurological, etiological, and radiological data of these patients were compared. RESULTS: There were 32 patients in warfarin, 58 patients in ASA, and 7 in warfarin + ASA group. Most of the patients were in no drug group (389 patients). The most frequent type of hemorrhage was supratentorial intraparenchymal hemorrhage. The most common accompanying disease was hypertension. The number of female patients was statistically significant in the warfarin group. Glasgow Coma Scale (GCS), accompanying diseases, opening of the hematoma to the ventricle, localization of the hemorrhage, age of the patient, and activated partial thromboplastin time level are all related to the outcome of patients. Warfarin users had worst mortality rate. CONCLUSIONS: Use of warfarin, low GCS score, opening to ventricle, older age, accompanying diabetes, and/or hypertension were worse prognostic factors. It is possible that patients with these unfavorable prognostic factors cannot survive.

Orbicularis oculi muscle activation during swallowing in humans.

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.

Diagnostic value of lumbar root stimulation at the early stage of Guillain-Barré syndrome.

OBJECTIVE: The aim of this study is to investigate diagnostic value of electrical lumbar root stimulation (RS) at the laminar level in the early stage of Guillain-Barré syndrome (GBS). METHODS: Fifteen patients (30 sides) and nine controls (17 sides) were included in the study. Conventional nerve conduction studies, needle electromyography, F responses and electrical lumbar RS were obtained from both groups. The needle electrical stimulation was performed at the L2-3 intervertebral level. Vastus lateralis, tibialis anterior and soleus muscles were investigated bilaterally and simultaneously in the first and fourth weeks. RESULTS: In all patients, the amplitudes elicited by lumbar RS were significantly attenuated while the conventional electrophysiological findings were normal and/or not diagnostic in 6 of 15 patients (40%) within the first week. Motor latencies by the lumbar RS were prolonged in the patients, compared to the controls, but the results were not statistically significant. CONCLUSIONS: M-responses elicited by lumbar RS appear to be helpful in disclosing proximal conduction abnormalities of GBS early in the course. SIGNIFICANCE: Lumbar RS seems to be a useful method in making the diagnosis of GBS early and there is no considerable side effect of this particular method.

Diagnostic value of cauda equina motor conduction time in lumbar spinal stenosis.

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 indicated 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 electrodes. Compound muscle action potential (CMAP) from gastrocnemius muscles were recorded bilaterally. 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.

Fasciculations in brain death.

OBJECTIVES: Brain death is the colloquial name for human death determined by tests showing irreversible cessation of the clinical functions of the brain. Spontaneous and reflex movements have been described in brain death. The aim of this report is to describe a brain-dead patient with unusual motor movements. DESIGN AND SETTING: The patient was followed and her motor movements were videotaped. PATIENT: We report the presence of extensive and long-lasting fasciculations in a patient who fulfilled the criteria for brain death. MEASUREMENTS AND MAIN RESULTS: We describe and show on videotape a brain-dead patient with rare motor movements. CONCLUSION: We suggest that fasciculations outlined in this study has to be accepted as motor symptoms in brain death patients.

Clinical and radiologic correlations of small and large centrum ovale infarcts.

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.