Bilateral claw hand: An uncommon presentation of regional Guillain-Barré syndrome

We present an uncommon case of a 38-year-old man presented with bilateral subacute weakness of intrinsic hand muscles, manifesting as bilateral claw-hand, without sensory deficits and absent tendon reflexes in upper arms. Nerve conduction studies showed findings consistent with demyelinating GBS. During the fourth day of hospitalization the patient presented symmetrical distal leg weakness and was treated with intravenous immunoglobulin. © 2013 Elsevier B.V

Extracranial venous hemodynamics in multiple sclerosis A case-control study

Objectives: A chronic state of impaired cerebral and cervical venous drainage, termed chronic cerebrospinal venous insufficiency (CCSVI), has recently been implicated in the pathogenesis of multiple sclerosis (MS). We performed a color-coded Doppler sonography case-control study to externally validate the CCSVI criteria. Methods: We prospectively evaluated consecutive patients with clinically definite MS and healthy volunteers using extracranial and transcranial color-coded Doppler sonography. The recently developed neurosonology criteria for CCSVI detection were used for interpretation of ultrasound assessments. The presence of venous reflux in cervical veins was assessed both in the sitting and upright position during a short period of apnea and after Valsalva maneuver. Results: We recruited 42 patients with MS (mean age 39 +/- 11 years, 17 men) and 43 control individuals (mean age 38 +/- 12 years, 16 men). Very good/excellent intrarater and interrater agreement (kappa values 0.82-1.00) was documented in 3 out of 5 CCSVI criteria. There was no evidence of stenosis or nondetectable Doppler flow in cervical veins in patients and controls. Reflux in internal jugular vein (IJV) was documented in 1 patient (2%) and 1 control subject (2%), both in sitting and supine posture during apnea. After performing Valsalva maneuver, we documented the presence of IJV valve incompetence in 3 patients with MS (7%) and 4 healthy volunteers (9%; p > 0.999). Conclusions: With established reproducibility of venous ultrasound testing, our data argue against CCSVI as the underlying mechanism of MS. Without further independent validation of CCSVI, potentially dangerous endovascular procedures, proposed as novel therapy for MS, should not be performed outside controlled clinical trials. Neurology (R) 2011; 77: 1241-124

Influence of sleep disturbance on quality of life of patients with epilepsy

SummaryThe frequency of sleep disturbances in patients with epilepsy and their impact on quality of life (QoL) have been documented in a few reports, and the results are conflicting. We identified 124 consecutive epilepsy out-patients who visited the epilepsy out-patient clinics at the University Hospital of Alexandroupolis, the AHEPA Hospital in Thessaloniki and the Aeginitio Hospital in Athens. We measured excessive daytime sleepiness (EDS) with the Epworth Sleepiness Scale (ESS), obstructive sleep apnea (OSA) with the Sleep Apnea scale of the Sleep Disorders Questionnaire (SA-SDQ), and insomnia with the Athens Insomnia Scale (AIS). We evaluated quality of life by the Quality of Life in Epilepsy Inventory (QOLIE-31). EDS was found in 16.9% (21/124) of epileptic patients, OSA in 28.2% (35/124), and insomnia in 24.6% (30/122). In multivariate analysis, we found that insomnia was an independent negative factor for Total score (p<0.001), Overall QoL (p=0.002), Emotional well-being (p<0.001), Energy/fatigue (p<0.001), Cognitive functioning (p=0.04) and Social functioning (p=0.03), and OSA only for Cognitive functioning (p=0.01). According to our findings, EDS, OSA, and insomnia are frequent in epileptic patients. Epileptic patients with sleep disturbance, mainly insomnia, have significant QoL impairment

Click Evoked Neurogenic Vestibular Potentials (NVESTEPs): A Method of Assessing the Function of the Vestibular System

To obtain neurogenic vestibular evoked potentials (NVESTEPs) with surface scalp recording using high intensity auditory clicks. The same stimulus is used in myogenic vestibular evoked potentials which has been shown to evoke potentials in the vestibular division of the vestibulocochlear nerve. A whole head recording with surface EEG electrodes was performed using high intensity clicks in one normal volunteer to determine the best recording position for vestibular evoked potentials. The results were compared to responses at moderate click intensities used for brainstem auditory evoked potentials (BAEPs). The difference in the location of the two responses on the scalp was assumed to be from the vestibular system. Responses specific to the high intensity clicks were best obtained in the parietal areas, with no reproducible responses obtained in the same area with moderate intensity clicks normally used in BAEPs. Recordings in neurologically normal volunteers showed a consistent response with a negative polarity at around 3 ms, which we therefore called N3. Two case studies are presented. The first case is a patient with unilateral sensorineural hearing loss with NVESTEPs present, suggesting that NVESTEPs is not a cochlear response. The second case is a patient with multiple sclerosis with demyelinating lesions in the pons and an unobtainable NVESTEP response. NVESTEPs is a possible new diagnostic technique that may be specific for the vestibular pathway. It has potential use in patients with symptoms of dizziness, subclinical symptoms in multiple sclerosis, and in disorders specific for the vestibular nerve

Safety of transcranial doppler &apos;bubble study&apos; for identification of right to left shunts: An international multicentre study

Background and purpose: A recent retrospective study using an online list service established by the American Academy of Neurology has suggested that ischaemic cerebrovascular events may occur in patients who undergo &apos;bubble studies&apos; (BS) with either transcranial Doppler (TCD) or transoesophageal echocardiography (TOE). The safety of TCD-BS for right to left shunt (RLS) identification was evaluated prospectively in an international multicentre study. Methods: Consecutive patients with cerebral ischaemia (ischaemic stroke or transient ischaemic attack (TIA)) were screened for potential ischaemic cerebrovascular events following injection of microbubbles during TCD-BS for identification of RLS at three tertiary care stroke centres. TCD-BS was performed according to the standardised International Consensus Protocol. TOE-BS was performed in selected cases for confirmation of TCD-BS. Results: 508 patients hospitalised with acute cerebral ischaemia (mean age 46±12 years, 59% men; 63% ischaemic stroke, 37% TIA) were investigated with TCDBS within 1 week of ictus. RLS was identified in 151 cases (30%). TOE-BS was performed in 101 out of 151 patients with RLS identified on TCD-BS (67%). It was positive in 99 patients (98%). The rate of ischaemic cerebrovascular complications during or after TCD-BS was 0% (95% CI by the adjusted Wald method: 0-0.6%). Structural cardiac abnormalities were identified in 38 patients, including atrial septal aneurysm (n=23), tetralogy of Fallot (n=1), intracardiac thrombus (n=2), ventricular septal defect (n=3) and atrial myxoma (n=1). Conclusion: TCD-BS is a safe screening test for identification of RLS, independent of the presence of cardiac structural abnormalities

Prevalence of Symptomatic Intracranial Atherosclerosis in Caucasians: A Prospective, Multicenter, Transcranial Doppler Study

BACKGROUND: There are limited data available regarding symptomatic intracranial atherosclerosis (SIA) prevalence in Caucasians. We sought to investigate SIA prevalence among Caucasian patients hospitalized with acute cerebral ischemia (ACI) in a prospective, multicenter Transcranial Doppler sonography (TCD) study. METHODS: Consecutive patients with ACI were prospectively evaluated with TCD over a 24-month-period. The previously validated criteria of SONIA trial were used for detection of &gt;50% intracranial stenosis with TCD. Brain angiography was performed to confirm the diagnosis in cases with abnormal TCD findings. SIA was diagnosed when there was evidence of a cerebral infarction in the territory of the stenotic artery (identified by TCD and confirmed by Magnetic resonance angiography [MRA]/Computed tomography angiography [CTA]). RESULTS: A total of 467 consecutive patients with ACI (60.4% men, mean age 58 ± 14 years) were evaluated. SIA was documented in 43 patients (9.2%; 95%CI: 6.9%-12.2%). The most common SIA location was M1MCA (34.9%) followed by TICA (18.8%). Diabetes mellitus (OR: 4.25, 95%CI: 2.18-8.26; P &lt; .001) and hypertension (OR: 2.41, 95%CI: 1.02-5.67; P = .045) were independently associated with SIA on multivariate models adjusting for potential confounders. CONCLUSIONS: SIA was identified in almost 10% of patients admitted with symptoms of ACI. These preliminary findings support further collaborative initiatives among stroke physicians to increase the yield of SIA detection in Caucasian patients with ACI. © 2012 by the American Society of Neuroimaging