Swallow-induced syncope in 5 patients: Electrophysiologic evaluation during swallowing.

BACKGROUND: We sought to characterize a cohort of participants with swallow-induced syncope (SIS) with clinical and electrophysiologic evaluations. METHODS: Using electrocardiographic monitoring and neurophysiologic methods of swallowing, we evaluated a cohort of 5 patients with SIS, 4 of whom had longitudinal follow-up. RESULTS: We determined electrophysiologically that the duration between the onset of swallow and a bradyarrhythmia or asystole is extremely short (2-3 seconds) in SIS. Most participants with SIS do not have a neurologic or esophageal disorder. SIS can occur with different food types, in sitting or standing position, and has varying frequency in different participants. Permanent pacemaker placement is a curative measure in SIS. CONCLUSIONS: Our findings suggest that SIS is elicited by reflex afferent pathways originating in the oropharynx, rather than an esophageal origin, as previously proposed. Our longitudinally followed cohort with detailed clinical and electrophysiologic characterization should aid the clinician in the diagnosis and treatment of this potentially life-threatening condition.

A non-alcoholic Wernicke's encephalopathy case with atypical MRI findings: Clinic versus radiology.

Wernicke's Encephalopathy is an acute neuropsychiatric syndrome that can be seen in both alcohol abusers and non-alcoholic population as a result of thiamine deficiency. We herein report a non-alcoholic patient with typical clinical presentations but atypical MRI findings. Early clinical suspicion of WE is especially important in non-alcoholic patients with atypical MRI findings. The diagnosis of this group is more difficult to make and the prognosis of the syndrome mostly depends on early initiation of thiamine supplementation.

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.

Impact of poststroke seizures on neurological deficits: magnetic resonance diffusion-weighted imaging study.

BACKGROUND: The impact of poststroke seizures on the neurological deficits related to ischemic stroke is not well known. It has been reported that following poststroke epilepsy, transient or long-lasting worsening of the poststroke sequelae may develop, but the underlying mechanism of deficit worsening has not been systematically studied by magnetic resonance diffusion-weighted imaging (MRI-DWI). METHODS: From 2008 to 2009, 1,010 ischemic stroke patients were admitted to our stroke unit at the Ege University Hospital with first-time strokes. Of these, 76 (7.5%) patients developed delayed seizures in a follow-up period of 18 months. We extracted the clinical and imaging data of the patients from our Stroke Registry databases and other medical records, and evaluated brain MRI, including spin-echo DWI with apparent diffusion coefficient (ADC) maps, FLAIR and T2-weighted images. RESULTS: There were 15 (20%) patients who had long-lasting worsening of the previous neurological sequelae, which we called long-lasting neurological worsening (LLW); 38 (50%) had transient neurological worsening (TNW) and 23 (30%) were without neurological worsening (WNW) after poststroke seizures. DWI findings were present in 3/23 (13%) patients with simple partial-type seizure, in 4/17 (29%) patients with complex partial-type seizure, and in 7/13 (54%) patients with generalized toni-clonic type seizure (p = 0.002). Patients with LLW showed more frequent changes on DWI than those with TNW (53 vs. 16%; p = 0.009). Forty percent of patients with LLW and 5% of those with TNW had ADC decrease (p = 0.004). Patients with LLW had DWI changes in the occipital region more frequently than those with TNW (57 vs. 18%; p = 0.05). Correlation analysis found a significant association between LLW and DWI changes, multiple DWI lesions, ADC decrease, and recurrent seizures. In the control MRI-DWI 1 month after the poststroke seizure, no signal abnormalities were detected in neuroimaging studies of all patients. Despite no functional outcome differences between the groups before the seizure, the functional scales 1 week after the seizure (National Institutes of Health Stroke Scale, Modified Rankin Scale and Barthel Index) showed significantly worse neurological functional statements in the patients with LLW than those with TNW and WNW (p = 0.001). CONCLUSIONS: Poststroke seizures may affect poststroke sequelae transiently, which we see more often, but some seizure types may prolong the duration of deficits. Multiple DWI changes and LLW following recurrent and longer poststroke seizures were strongly associated, and this may be due to the effect of seizures causing additional metabolical changes.

Myoclonic status epilepticus in six patients without epilepsy.

Myoclonic status epilepticus (MSE) is defined as prolonged period of myoclonic jerks that are correlated with epileptiform discharges on EEG. We here describe clinical features and video-EEG records of six adult patients with MSE who did not have a prior diagnosis of epilepsy. In four out of six patients, MSE was precipitated by drugs. Two out of four patients had chronic renal disease and received beta lactam group antibiotics. Two other patients, who described chronic pain, developed MSE while taking pregabalin. One patient who had dementia and family history of juvenile myoclonic epilepsy (JME) developed MSE one month after quetiapine was introduced. Another patient, who had a recent ischemic stroke, developed MSE due to an unknown reason. In these last two patients, an immediate triggering factor was not evident. Myoclonic status epilepticus ceased in five out of six patients after withdrawal of the drugs and/or intravenous antiepileptic treatment. Myoclonic status epilepticus is a rare event in patients without epilepsy. A correct diagnosis and prompt drug discontinuation may reverse this severe and life-threatening condition.

Hyposchematia without spatial neglect in 2 patients with lesions involving the insula.

BACKGROUND: : Hyposchematia is a rare variant of aschematia in which patients underestimate the size of part or all of their body. The term also describes an abnormality in drawing tasks, in which patients underestimate the size of 1 side of an image and draw it too small. Little is known about the neuroanatomy of the syndrome. CASE REPORTS: : We report 2 patients who developed contralateral hyposchematia without spatial neglect after suffering an ischemic lesion involving the right insula. Both patients felt that the left side of their face and their left arm and leg were disproportionately smaller than their right. On a drawing task, both patients drew the left sides of objects smaller than the right; they perseverated on the left sides of the images, for example, adding extra left-sided petals to a daisy. CONCLUSIONS: : In 2 reported patients, the cause of hyposchematia may be a lesion involving multiple insular circuits that affect the perception of extrapersonal space and self-related systems.