Seizure semiology reflects spread from frontal to temporal lobe: evolution of hyperkinetic to automotor seizures as documented by invasive EEG video recordings

This patient report demonstrates the importance of seizure evolution in the localising value of seizure semiology. Spread of epileptic activity from frontal to temporal lobe, as demonstrated by invasive recordings, was reflected by change from hyperkinetic movements to arrest of activity with mild oral and manual automatisms

The comparative effectiveness of fingolimod, natalizumab, and ocrelizumab in relapsing-remitting multiple sclerosis

© 2023, Fondazione Società Italiana di Neurologia.Background: Fingolimod, natalizumab, and ocrelizumab are commonly used in the second-line treatment of relapsing-remitting multiple sclerosis (RRMS). However, these have only been compared in observational studies, not in controlled trials, with limited and inconclusive results being reported. A comparison of their effect on relapse and disability in a real-world setting is therefore needed. Objectives: The objective of this study was to compare the efficacy of fingolimod, natalizumab, and ocrelizumab in reducing disease activity in RRMS. Methods: This multicenter, retrospective observational study was carried out with prospectively collected data from 16 centers. All consecutive RRMS patients treated with fingolimod, natalizumab, and ocrelizumab were included. Data for relapses, Expanded Disability Status Scale (EDSS) scores, and brain magnetic resonance imaging (MRI) scans were collected. Patients were matched using propensity scores. Annualized relapse rates (ARR), time to first relapse, and disability accumulation were compared. Results: Propensity score matching retained 736 patients in the fingolimod versus 370 in the natalizumab groups, 762 in the fingolimod versus 434 in the ocrelizumab groups, and 310 in the natalizumab versus 310 in the ocrelizumab groups for final analyses. Mean ARR decreased markedly from baseline after treatment in all three treatment groups. Mean on-treatment ARR was lower in natalizumab-treated patients (0.09, 95% confidence interval (CI), 0.07–0.12) than in those treated with fingolimod (0.17, 0.15–0.19, p<0.001), ocrelizumab (0.08, 0.06–0.11), and fingolimod (0.14, 0.12–0.16, p=0.001). No significant difference was observed in mean on-treatment ARR between patients treated with natalizumab (0.08, 0.06–0.11) and ocrelizumab (0.09, 0.07–0.12, p=0.54). Compared to fingolimod, the natalizumab and ocrelizumab groups exhibited a higher percentage of relapse-free patients and a lower percentage of MRI-active patients at year 1. No significance differences in disability accumulation were determined between the therapies. Conclusion: Natalizumab and ocrelizumab exhibited similar effects on relapse control, and both were associated with better relapse control than fingolimod. The effects of the three therapies on disability outcomes were similar

A case of spontaneous arm levitation in progressive supranuclear palsy

Progressive supranuclear palsy is one of the parkinsonial syndromes causing atypical parkinsonism. In recent reports, other than subcortical involvement, also cortical structures have been shown to be involved in progressive supranuclear palsy patients. One of the clinical presentations of this involvement is spontaneous arm levitation which is a component of alien limb syndrome. Here we report a clinically diagnosed progressive supranuclear palsy patient with spontaneous arm levitation. Clinically spontaneous levitation of one arm without denial of ownership suggests the presence of spontaneous arm levitation. Spontaneous arm levitation can occur in the setting of progressive supranuclear palsy and it possibly demonstrates the cortical involvement in this disorder

Morning headache in sleep apnoea: clinical and polysomnographic evaluation and response to nasal continuous positive airway pressure

Morning headache is accepted as part of clinical findings of obstructive sleep apnoea syndrome (OSAS). The prevalence of morning headache is at variable levels from 18% to 74% in patients with OSAS. However, there is controversy over the association of morning headache and OSAS. We studied morning headache prevalance and characteristics in 101 controls with apnoea-hypnoea index (AHI) = 5. Morning headache was reported by only nine (8.9%) subjects in a control group compared with 156 (33.6%) of OSAS patients (P < 0.01). Morning headache prevalance was significantly higher in severe and moderate OSAS groups. AHI was significantly higher in OSAS patients with morning headache compared with patients without morning headaches. Oxygen saturation nadir during rapid eye movement and non-rapid eye movement sleep as well as mean oxygen saturation value during total sleep time were also found to be significantly lower in morning headache group. However, none of the sleep parameters was found to be determinants of morning headache. Morning headache was more frequently reported by patients of female gender and with primary headache history. Morning headache was totally resolved in 90% of patients treated with nasal continuous positive airway pressure. The history of OSAS should be considered in the differential diagnosis of morning headache

The effect of piracetam on ataxia: clinical observations in a group of autosomal dominant cerebellar ataxia patients

Objectives: Autosomal dominant cerebellar ataxias are clinically and genetically heterogeneous neurodegenerative disorders. There is no known treatment to prevent neuronal cell death in these disorders. Current treatment is purely symptomatic; ataxia is one of the most disabling symptoms and represents the main therapeutic challenge. A previous case report suggesting benefit from administration of high dose piracetam inspired the present study of the efficacy of this agent in patients with cerebellar ataxia. Piracetam is a low molecular weight derivative of gamma-aminobutyric acid. Although little is known of its mode of action, its efficacy has been documented in a wide range of clinical indications, such as cognitive disorders, dementia, vertigo and dyslexia, as well as cortical myoclonus. The present report investigated the role of high dose piracetam in patients with cerebellar ataxia. Methods: Eight patients with autosomal dominant cerebellar ataxia were given intravenous piracetam 60 g/day by a structured protocol for 14 days. The baseline and end-of-the study evaluations were based on the International Cooperative Ataxia Rating Scale. Results: Statistical analysis demonstrated a significant improvement in the patients' total score (P = 0.018) and a subscale analysis showed statistical significance for only the posture and gait disturbances item (P = 0.018). Conclusion: This study is providing good clinical observation in favour of high dose piracetam infusion to reduce the disability of the patients by improving their gait ataxia

Electroencephalographic response to intravenous diazepam during status epilepticus

To determine the electroencephalographic (EEG) response to intravenous bolus administration of diazepam during status epilepticus (SE), we retrospectively evaluated the time to the disappearance of epileptiform activity in EEG recordings after 10 mg intravenous bolus administration of diazepam, and examined the relationship of this response time to the duration, etiology, and outcome of SE. Patients with SE who responded positively to diazepam administration (n = 53; 37 women, 16 men), aged 17-88 years were recruited from our SE registry. According to their response time to intravenous administration of diazepam, patients were divided into four subgroups: Group I response times ranged from 20 to 60 s, group II from 61 to 120 s, group III from 121-180 s, and group IV from 181 to 360 s. The duration of SE was 10.76 +/- 3.46 h in the first group and 27.00 +/- 12.57 h in the last group. According to the etiology, patients with central nervous system tumors and metabolic disorders were the fastest responders, whereas those with cerebrovascular diseases and withdrawal of antiepileptic drugs were the slowest responders. This study revealed a positive correlation between the response time to diazepam administration and seizure duration during status epilepticus. Response time may have a role in predicting outcome of status epilepticus treatment, in particular, the effects of diazepam. Thus, longer-duration EEGs are indicated. (C) 2011 Elsevier GmbH. All rights reserved

Episodes of status epilepticus in young adults: Etiologic factors, subtypes, and outcomes

The aim of this study was to evaluate the type, duration, etiology, treatment, and outcome of status epilepticus (SE) episodes, among patients aged 16-50 years. A total of 101 SE episodes in 88 young adult patients fulfilled our criteria. The mean age was 32 years. Status epilepticus episodes were most frequently observed in patients 21-30 years of age. A total of 53% of the patients were male, and 57% had pre-existing epilepsy. Seventy of the 101 episodes were convulsive SE. The most common etiology was withdrawal of or change in antiepileptic drugs (AEDs), seen in 31% of the SE episodes. This study included treatment of SE with traditional AEDs. Sixty-six episodes were treated successfully with intravenous infusion of 18-mg/kg phenytoin, and six episodes were treated with 10-mg/kg phenytoin. A total of 28% of the SE episodes remained refractory to first-line treatment, which was related to the duration of SE and mortality. The outcome was death in 14% of the patients due to underlying etiologies in the hospital. (C) 2013 Elsevier Inc. All rights reserved

Predictors of refractoriness in a Turkish status epilepticus data bank

Refractory status epilepticus (RSE) is known to constitute approximately 10-50% of all cases of status epilepticus (SE) and is associated with significant morbidity and mortality. In the present study, data from a prospectively collected SE database were analyzed. Patients with RSE (defined as a SE episode requiring a second line of intravenous treatment following intravenous phenytoin) were compared with patients with nonrefractory SE (NRSE); 290 episodes of SE were identified, of which 108 (38%) were defined as RSE. Univariate analysis revealed that age, female gender, SE type, SE duration, and acute etiology were associated with refractoriness, whereas electroencephalographic patterns were not. Nonconvulsive SE, which is probably associated with delays in treatment initiation, was a predictor of RSE, although it was not retained as a predictor in multivariate analysis. In the latter analysis, female gender (odds ratio: 1.815, 95% Cl: 1.053-3.126) and acute etiology (odds ratio: 0.619, 95% Cl: 0.429-0.894) were shown to be the only significant independent predictors of refractoriness. (C) 2009 Elsevier Inc. All rights reserved

Speech-induced primary lingual dystonia: a rare focal dystonia

Lingual dystonia, a type of focal dystonia that may be primary or secondary, is related to brain damage, neuroleptic use, neurodegenerative, metabolic, and neurodevelopmental disorders, varicella infection, and so on. However, primary lingual dystonia induced by speaking is a rare type of focal dystonia that is usually idiopathic in origin and is characterized by increased tonus of the tongue, which causes protrusion only during speaking. This report describes a 55-year-old male patient with lingual dystonia during speech. One interesting clinical feature of this case was that the speech disturbance improved while the patient vocalized a praise-like hymn in a manner that resembled singing