Comprehensive Outcome Assessment and Quality of Life Following Epilepsy Surgery.

BACKGROUND: Seizure freedom without deficits is the primary goal for epilepsy surgery. However, patients with medically refractory epilepsy commonly suffer from many co-morbidities related to mood, cognition, and sleep as well as social problems and resultant stigma. While epilepsy surgery literature does describe quality of life (QOL) and neuropsychological outcomes, there is a paucity of information on various common non-seizure outcomes, especially pertaining to mood, sleep, cognition, and social aspects. The objective of this study was to evaluate the role of various non-seizure parameters on post-epilepsy surgery QOL. METHODS: Consecutive adult patients operated for refractory epilepsy at least 1 year prior to initiation of this study were included and classified as seizure-free (group 1) or non-seizure-free (group 2). QOL was assessed using the QOLIE-31 instrument; patients with a T score less than 40 were categorized as "poor QOL." Non-seizure parameters assessed were cognition, mood disturbances, social improvement, social stigma, and sleep disturbances. Categorization into "good" and "poor" outcome subgroups on each item was carried out by dichotomization of scores. RESULTS: Thirty-seven patients (16 F) [mean age 23.5 ± 5.6 years] were evaluated; 26 were seizure-free (group 1). In this group, impaired memory, lower language scores, depression, not having been employed, not receiving education prior to surgery, and experiencing social stigma were factors significantly associated with poor QOL. In group 2, all patients had poor QOL scores. CONCLUSION: Non-seizure factors related to common epilepsy co-morbidities and social issues are highly prevalent among seizure-free patients reporting poor QOL after epilepsy surgery.

Classification and comparative analysis of psychogenic nonepileptic seizures (PNES) semiology based on video-electroencephalography (VEEG).

BACKGROUND: Multiple classification systems for psychogenic nonepileptic seizures (PNES) based on semiological features have been described. We sought to compare the efficiency of four PNES classification systems. METHODS: We retrospectively analyzed medical and video-electroencephalography (VEEG) records of patients with PNES with at least one typical event recorded on VEEG. Semiology of PNES events was stringently classified using Hubsch, Dhiman, Wadwekar, and Asadi-Pooya's classification systems. RESULTS: We studied 248 patients with PNES (78% females, mean age 23.1 ±â€¯10.3 years) and reviewed 498 PNES events. Using Hubsch's scheme, we classified events into: dystonic attacks with primitive gestural activity (5.2%), paucikinetic attacks with preserved responsiveness (9.7%), pseudosyncope (59.8%), hyperkinetic prolonged attacks (16.2%) and axial dystonic prolonged attacks (1.6%), and unclassified (7.5%). Using Dhiman's classification, events were: abnormal motor (hypermotor [10.4%]/ partial motor [12.7%]), dialeptic type (58.6%), mixed patterns (17.3%), and unclassified (1%). Using Wadwekar's classification: dystonic attacks with primitive gestural activity (5.2%), paucikinetic attacks with preserved responsiveness (9.6%), pseudosyncope with/without hyperventilation (65.1%), hyperkinetic prolonged attacks involving limbs ±â€¯trunk (18.5%), and axial dystonic prolonged attacks (1.6%). Using Asadi-Pooya's classification, events were: hypermotor (30.1%), non-motor (62.9%), and mixed (7.0%). All events could be classified via Wadwekar and Asadi-Pooya systems. CONCLUSION: In our study, pseudosyncope/dialeptic/non-motor semiology emerged as most frequent. Most of our patients with PNES had stereotyped semiology. All events could be classified using the schemes by Asadi-Pooya and Wadweker et al. Dhiman et al. scheme could classify 99% and 7.5% remained unclassified using Hubsch et al. scheme.

Educating Caregivers to Reduce Complications and Improve Outcomes of Stroke Patients (ECCOS) - A Cluster-Randomized Trial.

OBJECTIVES: Stroke constitutes a significant public health problem in developing countries. Caregivers provide an important support system for patient care but usually lack knowledge and skill to attend their stroke patients. We assessed whether a caregiver-directed educational intervention would reduce hospital-acquired complications and improve stroke patients' outcomes. MATERIALS AND METHODS: We randomly assigned two Neurology inpatient wards to receive either standard care or an educational intervention. The coprimary outcomes included incidence of hospital-acquired complications and in-hospital mortality. Secondary outcomes included the modified Rankin Scale and mortality at three months. RESULTS: Among 164 patients recruited, 82 received intervention, and standard care each. The mean (Standard deviation) Glasgow coma scale of patients was 11.01 (3.4), and National Institute of Health Stroke Scale was 19.17 (8.54). The incidence of complications (72 in the intervention versus 81 in the control group; p=0.56) was not different. Ten patients (12.2%) in the intervention group and 16 (19.5%) in the control group (p=0.20) died in-hospital. Twenty patients (27.8%) in the intervention and twelve (18.2%) in the control group attained modified Rankin Scale 0-2 at three months (p=0.12). The mortality at three months (20 [24.4%] in the intervention versus 25 [30.5%] in the control group) was not different (p=0.38). The intervention group had fewer complications (42 versus 68 in the control group; p=0.01) during the initial ten days of hospital stay, but adjusted analysis revealed no difference. CONCLUSION: A structured educational intervention did not reduce the incidence of hospital-acquired complications, mortality, or morbidity. However, there was a trend towards fewer complications in the initial days of hospital stay. Extended hospital stay, caregiver fatigue, and dilution of the intervention over time might be reasons for the apparent lack of effect. CLINICAL TRIAL REGISTRATION-URL: http://www.ctri.nic.in. Unique identifier: CTRI/2018/11/016312.

Development and implementation of acute stroke care pathway in a tertiary care hospital in India: A cluster-randomized study.

BACKGROUND: In-hospital care of stroke patients can reduce the risk of death and disability. There is an emerging evidence for the routine use of care pathways (CPs) for acute stroke management. Our aim was to develop evidence-based CPs and test the hypothesis that acute stroke management by CPs is superior to that provided by conventional care. MATERIALS AND METHODS: An acute stroke CP was designed in accordance with the recent American Stroke Association (ASA)/American Heart Association (AHA) stroke guidelines and the Indian Academy of Neurology (IAN) stroke management guidelines in India. A total of 162 acute stroke patients, who were managed within 72 hours of onset of their symptoms, were enrolled prospectively in two groups-the stroke care pathway (CP) arm (n = 77) and the conventional care (CC) arm (n = 85). The incidence of aspiration pneumonia, complications during the hospital stay, and death or dependency at discharge and at 90 days were among the main outcomes of our study. RESULTS: The CP arm had a lower incidence of aspiration pneumonia (AP) in comparison with the CC arm (6.5% vs. 15.3%, risk ratio [RR] = 0.42, 95% confidence interval [CI] = 0.16-1.14, P = 0.062). The CP group had a decreased risk of requirement of mechanical ventilation (7.8% vs. 17.6%, odds ratio [OR] = 0.39, 95% CI = 0.14-1.07, P = 0.05). Barthel and modified Rankin Scale scores were similar in both the groups, but death at 90 days was significantly lesser in the CP arm (7.8% vs. 20%, P = 0.022). This benefit was most prominent in the Glasgow Coma Scale (GCS) subgroup scoring 9-15 (RR = 0.16, 95% CI = 0.03-0.71). There were no significant differences in the other outcome measures. CONCLUSION: Stroke CPs reduce the incidence of aspiration pneumonia, the need for mechanical ventilation, and the risk of death, when assessed at a follow-up of 90 days.

Predictors of Ischemic Stroke in Rheumatic Heart Disease.

BACKGROUND: Studies on predictors of ischemic strokes caused by rheumatic heart disease (RHD) are sparse and extremely important for identifying high-risk cases to direct future therapeutic trials for prevention of ischemic stroke in this population. OBJECTIVE: The aim of the present study was to study the predictors of ischemic stroke in patients with RHD and to observe outcome of patients with ischemic stroke at 3 months' follow-up using modified Rankin scale. METHODS: We conducted a case-control study comparing the clinical profile of 40 adult patients with acute ischemic stroke caused by RHD with equal numbers of matched controls comprising patients with RHD without any prior history of stroke. We also observed the functional outcome of ischemic strokes in these patients. RESULTS: The presence of left atrial spontaneous echo contrast (odds ratio = 39.9; 95% confidence interval, 3.16-501.9; P = .004) and atrial fibrillation (AF) (odds ratio = 3.2; 95% confidence interval, 1.6-6.7; P = .002) was significantly associated with stroke occurrence in RHD populations. The outcome of patients was good with low mortality and significant improvement of modified Rankin scale at 3 months' follow-up. CONCLUSIONS: Presence of AF and left atrial spontaneous echo contrast are significant risk factors for ischemic stroke in patients with RHD. There is high percentage of subclinical AF in this population. Future large clinical trials for oral anticoagulation/antiplatelet agents are needed for stroke prevention in high-risk RHD patients identified by a detailed workup.

Seasonal differences and circadian variation in stroke occurrence and stroke subtypes.

BACKGROUND: India is a subtropical country with clear seasonal variations in weather conditions. Seasonal and circadian variation in occurrence of subtypes of cerebrovascular disease has been of interest in several studies from different countries and climate zones, but discrepant results have made the conclusions unclear. The aim of the present study was to observe the seasonal and circadian variation in the occurrence of stroke and its subtypes among our population. METHODS: This was a cross-sectional observational study based on new cases and past cases of stroke on follow-up, conducted between January 2011 and December 2012 in the Department of Neurology, at the All India Institute of Medical Sciences, New Delhi, India. The date and time of onset of the stroke was recorded. The categorization of months into season was in accordance with the Indian Meteorological Department guidelines. The time of onset was distributed into 6 hourly intervals. Statistical calculations were performed using Stata version 12.1 and SPSS version 20. RESULTS: A total of 583 patients were included for the study. The rate of occurrence of stroke was highest in the late morning 0600-1159 hours (P value <.001) compared with other times of the day, regardless of gender or age for both ischemic and hemorrhagic strokes. It was lowest in late evening (1800-2359 hours) quadrant compared with other quadrants. Although there was no significant difference found by dichotomizing the groups into two 6-month periods, there was an increasing trend in number of patients with stroke during the months November-February. There was no difference in stroke occurrence between the types of stroke or within each type among different seasons with different temperatures. Trial of Org 10172 in Acute Stroke Treatment (TOAST) classification of ischemic strokes also did not show any association with season or circadian rhythm. CONCLUSIONS: There is a significant increase in occurrence of strokes between 0600 and 1159 hours and lowest between 1800-2359 hours. No significant variation in stroke occurrence or subtype for any of the seasons was observed.

Safety and efficacy of N-acetylcysteine (NAC) as an adjunct to standard treatment in patients with acute ischemic stroke: a randomized controlled pilot trial (NACTLYS).

There is a pressing clinical need for thrombolytic agents that can effectively disaggregate arterial thrombi in acute ischemic stroke without significantly increasing the risk of bleeding. This pilot study aimed to investigate the safety and efficacy of N-acetylcysteine (NAC) as an adjunctive therapy to intravenous recombinant tissue plasminogen activator (rtPA or alteplase). A randomized, open-label, blinded assessor pilot study was conducted. Patients presenting with an acute ischemic stroke within 4.5 h from onset were randomized into two groups: intravenous NAC and rtPA or rtPA alone. Primary outcomes included intracerebral hemorrhage, symptomatic intracerebral hemorrhage, extracranial bleeding, and adverse reactions. Secondary outcomes comprised major neurological improvement assessed by (National Institute of Health Stroke Scale) NIHSS at 24 h, recanalization on first run of angiography in patients who underwent thrombectomy or on repeat vascular imaging at 24 h, modified Rankin scale, and three-month mortality. Forty patients were enrolled, with 21 receiving only rtPA and 19 receiving NAC with rtPA. Baseline characteristics were comparable among groups. No significant differences were observed in adverse events (p = 0.99), intracranial hemorrhage (p = 0.21), symptomatic intracerebral hemorrhage (p = 0.47), or extracranial bleeding (p = 0.21). Median NIHSS at 24 h was significantly lower in the intervention group (p = 0.03). Functional outcomes and three-month mortality were similar between groups (p = 0.85 and p = 0.99 respectively). The co-administration of N-acetylcysteine with alteplase did not significantly alter safety profiles, morbidity, or mortality at 3 months. While no substantial differences were noted, a slightly improved early neurological outcome was observed in the intervention arm. The study's findings were constrained by a small sample size, emphasizing the necessity for future large-scale trials to comprehensively evaluate the safety and efficacy of N-acetylcysteine as a thrombolytic agent in acute ischemic stroke.Trial Registration Clinical Trials Registry India-CTRI/2019/05/019305.

Prevalence of symptomatic and asymptomatic coronary artery disease in patients with stroke.

Background. Studies have shown that myocardial infarction is a leading cause of death in patients recovering from stroke or transient ischaemic attacks. We aimed to study the prevalence of symptomatic and asymptomatic coronary artery disease (CAD) in patients with stroke. Methods. Eighty-six patients with stroke were evaluated for risk factors and presence of CAD. Patients without a previous diagnosis of CAD underwent stress-rest gated technetium-99m (Tc99m) tetrofosmin myocardial perfusion SPECT (MPS) scan to estimate the presence or absence of a reversible perfusion deficit. Results. Thirty-three patients (clinically asymptomatic for CAD) did not consent for the MPS scan. Among the remaining 53 patients, 13 patients had been previously diagnosed to have CAD, 8 patients were suspected to have underlying CAD and 32 patients were asymptomatic. Among the patients with suspected CAD, 2 had abnormal MPS scans and one had triple-vessel disease on coronary angiography. Of the asymptomatic patients, 6 had CAD. The overall proportion of CAD among patients with stroke was 41.5% (22/53) and that of asymptomatic CAD 18.8% (6/32). Conclusion. A considerable number of patients with stroke may have associated CAD. An optimal management strategy in stroke patients who have silent CAD may improve clinical outcomes.

A prospective study of in-hospital mortality and discharge outcome in spontaneous intracerebral hemorrhage.

BACKGROUND: Intracerebral hemorrhage (ICH) is associated with high mortality and morbidity. Various clinical and imaging predictors of mortality have been observed in previous studies. AIMS: To study factors associated with in-hospital mortality in patients with ICH and observe the disability status of patients [assessed by modified Rankin scale (mRS)] at the time of discharge. DESIGN: Prospective observational study. MATERIALS AND METHODS: All consecutive patients with acute hypertensive ICH admitted during the study period were enrolled. Data recorded included: Demographics, clinical, biochemical and cranial computed tomography (CT) findings. Primary outcome was defined as either death or survival within the hospital. mRS was used to assess outcome at discharge. RESULTS: Of the total 214 patients with ICH (193 supratentorial and 21 infratentorial), 70 (32.7%) patients died during the hospital stay. On bivariate analysis, low Glasgow Coma Scale (GCS) score, ventilatory assistance, higher hematoma volume, midline shift, hydrocephalus and intraventricular hematoma (IVH) were associated with mortality. ICH grading scale (ICH-GS) and ICH scores were higher in patients who died (P < 0.0001). Ninety-five (44.6%) patients underwent a neurosurgical intervention; 66 (45.8%) patients among the survivors compared with 29 (41.4%) among those who died (P = 0.54, Odds Ratio (OR) 0.83, 95% Confidence Interval (CI) 0.46-1.48). Independent predictors of mortality included a higher baseline hematoma volume ( P = 0.04 OR 1.01, 95% CI 1.00-1.02), lower GCS ( P = 0.01 OR 2.57, 95%CI 1.25-5.29), intraventricular extension of hematoma ( P = 0.007 OR 2.66, 95% CI 1.26-5.56) and ventilatory requirement (P < 0.0001 OR 8.34, 95%CI 2.75-25.38). Among survivors (n = 144), most were disabled [mRS 0-3, 7 (4.8%) and mRS 4-5, 137 (95.13%)] at discharge. CONCLUSIONS: Low GCS, higher baseline ICH volume, presence of IVH and need for ventilatory assistance are independent predictors of mortality. Most of the patients at discharge were disabled. Surgery did not improve mortality or outcome.

Randomized double-blind, placebo-controlled study of oral gabapentin for prevention of neuropathy in patients receiving paclitaxel.

BACKGROUND: Peripheral neuropathy is a common dose-limiting side effect of paclitaxel. To date, there is no effective strategy to prevent paclitaxel-induced peripheral neuropathy. A recent small phase II study demonstrated the potential role of oral gabapentin in this setting. This phase III study is aimed to assess the efficacy of oral gabapentin in preventing paclitaxel-induced neuropathy. OBJECTIVE: To compare the efficacy of oral gabapentin with placebo in preventing clinically significant peripheral neuropathy (NCI CTCAEv5.0 grade 2 or higher) in patients receiving paclitaxel. METHODS: This is a randomized, placebo-controlled, double-blind, parallel-group superiority trial. The primary outcome is the development of grade 2 or higher chemotherapy-induced peripheral neuropathy. Secondary outcomes include any grade neuropathy, the percentage change in sensory nerve conduction velocities in peripheral nerves, time to development of any grade neuropathy, paclitaxel dose reductions and delays due to peripheral neuropathy, patient-reported outcomes, adverse events, and adherence to oral therapy. A total of 136 patients receiving paclitaxel will be randomly allocated (stratified by weekly vs. non-weekly administration) to receive either oral gabapentin or placebo till three weeks after the last dose of chemotherapy or occurrence of the primary outcome. CONCLUSION: This study aims to find if oral gabapentin reduces the incidence of grade 2 or higher chemotherapy-induced peripheral neuropathy in patients receiving paclitaxel. TRIAL REGISTRATION: The trial is registered prospectively with the Clinical Trials Registry of India (CTRI/2022/02/040030) on April 4, 2022.

A Real-World Experience of Azathioprine Versus First-Line Disease-Modifying Therapy in Relapsing-Remitting Multiple Sclerosis-A Prospective Cohort Study.

Azathioprine (AZA) has demonstrated efficacy in multiple randomized control trials (RCTs) for Relapsing-Remitting Multiple Sclerosis (RRMS). However, we still need comparative real-world data with other first-line disease-modifying therapies (DMTs). We aimed to assess AZA's effectiveness regarding relapses, disability progression, time to the first relapse, magnetic resonance imaging (MRI) activity, and safety compared with other approved first-line DMTs in an Indian population in a real-world setting. We conducted a single-center prospective study of treatment-naive RRMS patients between 2017 and 2019. We evaluated the effects of AZA and other approved DMTs on clinical and radiological measures. Among 192 eligible patients (F:M ratio 2.84:1), 68 patients (35.4%) were on AZA, 68 patients (35.4%) were on dimethyl fumarate (DMF), 32 patients (16.7%) on interferon (IFN beta-1a), and 16 patients (8.3%) on teriflunomide (TFL). Four treatment groups were comparable: AZA v/s DMF v/s TFL v/s IFN beta-1a. In primary outcomes, there was no significant difference between the groups in terms of change in the Expanded Disability Status Scale (EDSS) score at three months (p-value = 0.169), six months (p-value = 0.303), 12 months (p-value = 0.082), and 24 months (p-value = 0.639), the number of relapses (p-value = 0.229), and time to the first relapse (p-value > 0.05 in all groups). In the secondary outcome, there was no significant difference between the treatment groups on serial MRI parameters used according to "Magnetic Resonance Imaging in Multiple Sclerosis" (MAGNIMS) 2016 criteria (p-value > 0.05). In safety outcomes, leukopenia was significantly more common in the AZA group (p-value = 0.025), flu-like symptoms (p-value = 0.0001), and injection site reactions (p-value = 0.035) were significantly more common in the IFN beta-1a group. Our study suggests AZA is as effective as other approved DMTs and a good alternative as a first-line treatment for multiple sclerosis's clinical and radiological activity in real-world settings on short follow-up. Based on these results, more randomized controlled trials of AZA v/s DMF or other DMTs are needed for more robust outcomes.

Social Cognition in Parkinson's Disease: A Case-Control Study.

Background: Social cognition is the study of how people make sense of themselves and others. Impairment in several domains of social cognition is increasingly being recognized in Parkinson's disease (PD). Objectives: We aimed to study multiple domains of social cognition in Indian PD patients using a culturally appropriate, validated instrument. Methods: We recruited 52 individuals with PD and 31 healthy volunteers (HV) and used the Social Cognition Rating Tools in Indian Setting (SOCRATIS) tool to assess theory of mind (ToM), attributional biases and social cue perception. Quality of life (QoL) was assessed using the PDQOL scale. Results: Baseline characteristics were comparable between PD and HV. The mean (SD) FOT index (first order ToM index) was 0.86(0.18) in PD and 0.99(0.07) in HV [P < 0.001]. The PD group showed higher Externalizing Bias [EB, 4.42(3.91)], compared to HV [1.58(3.22), P = 0.001]. The mean (SD) Faux Pas Composite Index (FPCI ALT) was 0.69(0.09) in PD and 0.78(0.13) in HV [P < 0.001]. Social cognition indices were not associated with QoL in PD. Clinical parameters-age, gender, HAM-D, MOCA, education, levodopa equivalent daily dose of medication, number of PD drugs and trihexyphenidyl use did not predict social cognition. Conclusion: PD patients were less successful than age, gender matched controls in understanding social situations and other's thought processes and had higher tendency to attribute undesirable events to external causes. Deficits in social cognition did not impair the quality of life.

Biosimilar Tenecteplase Versus Alteplase in Acute Ischemic Stroke: A Real World Study.

Background and Purpose: There is an unmet need for a more effective thrombolytic agent in acute ischemic stroke (AIS) management. Various studies and meta-analysis suggest tenecteplase (TNK) as non-inferior over alteplase (rTPA). The present single-center study compares biosimilar TNK and rTPA in a tertiary care setting. Methods: Data of patients who presented with AIS and underwent intravenous thrombolysis (IVT) were recruited retrospectively from January 2018 to July 2021. Primary efficacy outcome was a modified Rankin score (mRS) at 90 days dichotomized at < = 2. Qualitative and quantitative variables were assessed using Chi-square test and Student's t-test, respectively. Results: A total of 160 patients, 103 in the rTPA and 57 in TNK group, were analyzed. The baseline characteristics were well matched apart from hypertension. Large artery atherosclerosis was the most frequent subtype of stroke among the two groups. Good functional outcome was seen in 47.92% of patients TNK and 64.77% of patients in rTPA group (p = 0.069). No difference was seen in the rates of any ICH (p = 0.29) and mortality at 3 months (p = 0.32) among the two groups. Conclusion: This present study observed no difference in the efficacy and safety between biosimilar TNK and rTPA. Our findings are in concordance with published trials showing equivalence between the two molecules.

Escalate: Linezolid as an add on treatment in the intensive phase of tubercular meningitis. A randomized controlled pilot trial.

Most drugs used in the treatment of Tuberculous Meningitis have limited CNS penetration thereby limiting efficacy. CSF penetration of linezolid is 80-100%.The study was a prospective, randomized, open label with blinded outcome assessment pilot trial carried out in patients with TBM. Patients were randomized in a 1:1 ratio into two treatment groups either to receive standard ATT alone or add on oral 600 mg BD Linezolid for 4 weeks along with standard four drug ATT [HRZE/S]. Primary outcome was safety and mortality at the end of one and three months measured by intention to treat analysis. 29 patients were recruited and 27 completed three months of follow up. There was no significant difference in terms of mortality with Odds ratio (95% CI) of 2 (0.161-24.87; p = 1) at one month and 0.385 (0.058-2.538; p = 0.39) at three months. There was a significant improvement in GCS in Linezolid group at one month and mRS within the Linezolid group at one and three months. No major safety concerns were observed. The sample size is underpowered to draw any definitive conclusions but improvement in mRS and GCS as well as mortality change make a case for a large sample size trial.

Telemedicine for Individuals with epilepsy: Recommendations from the International League Against Epilepsy Telemedicine Task Force.

Worldwide, People with Epilepsy (PWE) are confronted with several barriers to face-to-face consultations. These obstacles hamper appropriate clinical follow-up and also increase the treatment gap for Epilepsy. Telemedicine holds the potential to enhance management as follow-up visits for PWE are focused on more on clinical history and counselling rather than physical examination. Besides consultation, telemedicine can also be used for remote EEG diagnostics and tele-neuropsychology assessments. In this article, the Telemedicine Task Force of the International League Against Epilepsy (ILAE) outlines recommendations regarding optimal practice in utilizing in the management of individuals with epilepsy. We formulated recommendations for minimum technical requirements, preparing for the first tele-consultation and the specificities for follow-up consultations. Special considerations are necessary for specific populations, including paediatric patients, patients who are not conversant with tele-medicine and those with intellectual disability. Telemedicine for individuals with epilepsy should be vigorously promoted with the aim of improving the quality of care and ultimately reduce the wide clinician access related treatment gap across several regions of the globe.

Neuropathic Tremor in Guillain-Barré Syndrome.

Background: Neuropathic Tremor (NT) is a postural/kinetic tremor of the upper extremity, often encountered in patients with chronic neuropathies such as paraprotein-associated and hereditary neuropathies. Objectives: To describe the clinical and electrophysiological features of NT in a previously underrecognized setting- during recovery from Guillain-Barré Syndrome (GBS). Methods: Patients with a documented diagnosis of GBS in the past, presenting with tremor were identified from review of clinical records. Participants underwent structured, videotaped neurological examination, and electrophysiological analysis using tri-axial accelerometry-surface electromyography. Tremor severity was assessed using the Fahn-Tolosa-Marin Tremor Rating Scale. Results: We describe the clinical and electrophysiological features of 5 patients with GBS associated NT. Our cohort had a fine, fast, and slightly jerky postural tremor of frequency ranging from 8 to 10 Hz. Dystonic posturing and overflow movements were noted in 4/5 patients. Tremor appeared 3 months-5 years after the onset of GBS, when patients had regained near normal muscle strength and deep tendon jerks were well elicitable. Electrophysiological analysis of tremor strongly suggested the presence of a central oscillator in all patients. Conclusion: NT is not limited to chronic inflammatory or hereditary neuropathies and may occur in the recovery phase of GBS. The tremor is characterized by a high frequency, jerky postural tremor with dystonic posturing. Electrophysiological evaluation suggests the presence of a central oscillator, hypothetically the cerebellum driven by impaired sensorimotor feedback.

Modulation of epileptiform EEG discharges in juvenile myoclonic epilepsy: an investigation of reflex epileptic traits.

PURPOSE: Previous studies have suggested that cognitive tasks modulate (provoke or inhibit) the epileptiform electroencephalography (EEG) discharges (EDs) in patients with juvenile myoclonic epilepsy (JME). Their inhibitory effect was found to be especially frequent (64-90%). These studies arbitrarily defined modulation as a >100% increase or >50% decrease of the EDs compared with baseline, which may not sufficiently distinguish from spontaneous fluctuations. The aim of our study was to assess the modulation of EDs and the precipitation of myoclonic seizures by cognitive tasks and by conventional provocation methods, taking into account also the spontaneous fluctuation of EDs. METHOD: Sixty patients with JME underwent video-EEG recordings including 50-min baseline, sleep, hyperventilation, intermittent photic stimulation (IPS), and cognitive tasks. To account for spontaneous fluctuations of the EDs we divided the baseline period into 5-min epochs and calculated the 95% confidence interval for the baseline EDs in each patient. Modulation was assumed when the number of EDs during any 5-min test period was outside the 95% confidence interval. KEY FINDINGS: Using the arbitrary method, our results were similar to previous publications: Cognitive tasks seemed to inhibit the EDs in 94% of the patients, and to provoke them in 22%. However, when the spontaneous fluctuations were accounted for, inhibition was found in only 29% of the patients and provocation in 18%. A nonspecific effect of any cognitive task seemed to account for the observed significant inhibition in two-thirds of the cases, but was observed in only one of the patients with significant provocation. Photoparoxysmal response was observed in 23% of the patients. When accounting for the spontaneous occurrence of EDs, IPS had provocative effect in 10% of the patients. Hyperventilation and sleep had provocative effect on EDs to an extent similar to the cognitive tasks (hyperventilation: 22%; sleep: 18%). The conventional provocation methods tended to be more efficient in patients who were not seizure free. Myoclonia were recorded most often during the cognitive tasks (10 patients). SIGNIFICANCE: Spontaneous fluctuations of EDs account for most of the previously described inhibitory effect of the cognitive tasks. The provocative effect of the cognitive tasks is task-specific, whereas the inhibitory effect seems to be related to cognitive activation in general.