Reduced motor cortex plasticity following inhibitory rTMS in older adults

ObjectiveAgeing is accompanied by diminished practice-dependent plasticity. We investigated the effect of age on another plasticity inducing paradigm, repetitive transcranial magnetic stimulation (rTMS).MethodsHealthy young (n = 15; 25 ± 4 years) and old (n = 15; 67 ± 5 years) adults participated in two experiments. Motor evoked potentials (MEPs) were measured in the target muscle (first dorsal interosseus, FDI) and a remote muscle (abductor digiti minimi) during a set of single stimuli. Subjects then received real or sham inhibitory rTMS (intermittent subthreshold trains of 6 Hz stimulation for 10 min). MEPs were measured for 30 min after rTMS.ResultsIn young adults, MEPs in the target FDI muscle were ∼15% smaller in the real rTMS experiment than in the sham rTMS experiment (P < 0.026). In old adults, FDI MEP size did not differ between experiments.ConclusionsAdvancing age is associated with reduced efficacy of inhibitory rTMS.SignificanceThis work has important implications for the potential therapeutic use of rTMS in stroke and neurological disease.     

Tendencia de la hospitalización por ictus isquémico en adultos jóvenes de la Región de Murcia durante el periodo 2006-2014

IntroductionRecent studies conducted in Europe and the United States suggest upward trends in both incidence and hospitalisation rates for ischaemic stroke in young adults; however, data for Spain are scarce. This study analyses the trend in hospitalisation due to ischaemic stroke in adults aged under 50 years in the region of Murcia between 2006 and 2014.MethodWe performed a retrospective study of patients discharged after hospitalisation due to cerebrovascular disease (CVD); data were obtained from the regional registry of the Minimum Basic Data Set. Standardised rates were calculated, disaggregated by age and CVD subtype. Time trends were analysed using joinpoint regression to obtain the annual calculated standardised rate and the annual percentage of change (APC).ResultsA total of 27 064 patients with CVD were discharged during the 9-year study period. Ischaemic stroke was the most frequent subtype (61.0%). In patients aged 18 to 49 years, the annual number of admissions due to ischaemic stroke increased by 26%, and rates by 29.2%; however, the joinpoint regression analysis showed no significant changes in the trend (APC = 2.74%, P≥.05). By contrast, a downward trend was identified in individuals older than 49 (APC = –1.24%, P<.05).ConclusionsNo significant changes were observed in the rate of hospitalisation due to ischaemic stroke among young adults, despite the decline observed in older adults. Identifying the causes of these disparate trends may be beneficial to the development of specific measures targeting younger adults.     

Is off-label thrombolysis safe and effective in a real-life primary stroke center? A retrospective analysis of data from a 5-year prospective database

BackgroundIntravenous thrombolysis (IVT) use for acute ischemic stroke (AIS) varies among countries, partly due to guidelines and product labeling changes. The study aim was to identify the characteristics of patients with AIS treated with off-label IVT and to determine its safety when performed in a primary stroke center (PSC).MethodsThis observational, single-center study included all consecutive patients admitted to Perpignan PSC for AIS and treated with IVT and patients transferred for EVT, between January 1, 2015 and December 31, 2019. Data of patients treated with IVT according to (“in-label group”) or outside (“off-label”) the initial guidelines and manufacturer's product specification were compared. Safety was assessed using symptomatic intracerebral hemorrhage (SIH) as the main adverse event.ResultsAmong the 892 patients in the database (834 screened by MRI, 93.5%), 746 were treated by IVT: 185 (24.8%) “in-label” and 561 (75.2%) “off-label”. In the “off-label” group, 316 (42.4% of the cohort) had a single criterion for “off-label” use, 197 (26.4%) had two, and 48 (6.4%) had three or more criteria, without any difference in IVT safety pattern among them. SIH rates were comparable between the “off-label” and “in-label” groups (2.7% vs. 1.1%, P = 0.21); early neurological deterioration and systematic adverse event due to IVT treatment were similar in the 2 groups. “Off-label” patients had higher in-hospital (8.7% vs. 3.8%, P = 0.05) and 3-month mortality rates (12.1% vs 5.4%, P < 0.01), but this is explained by confounding factors as they were older (76 vs 67 years, P < 0.0001) and more dependent (median modified Rankin scale score 0.4 vs 0.1, P < 0.0001) at admission.Conclusions“Off-label” thrombolysis for AIS seems to be safe and effective in the routine setting of a primary stroke center.     

Endovascular treatment for acute ischemic stroke at a primary stroke center: First results of the Perpignan center

IntroductionConverting a high-volume primary stroke center (PSC) into a stroke center that can perform emergency endovascular treatment (EVT) could reduce the time to thrombectomy. We report the first results of a newly established EVT facility at the Perpignan PSC and their comparison with the targets defined by the established guidelines.Patients and methodFor this comprehensive observational study, data of patients with acute ischemic stroke (AIS) due to proximal large vessel occlusion (LVO) and treated by EVT at the Perpignan PSC from December 5, 2019 to September 15, 2020 were extracted from an ongoing prospective database.ResultsDuring the study period, 37 patients underwent EVT at the Perpignan PSC. The median (range) symptom-onset to recanalization time was 262 min (100–485 min). The median (range) intra-hospital times were: 20 min (2–58 min) for door-to-imaging, 57 min (30–155 min) for imaging-to-puncture, 55 min (15–180 min) for puncture-to-recanalization, and 137 min (59–319 min) for door-to-recanalization. At 3 months post-AIS, the favorable outcome (modified Ranking Score: 0–2) rate was 50% and the mortality rate was 19.4%. These results are comparable to those of previous clinical trials, and meet the targets defined by the current consensus statements for EVT.Discussion and conclusionOur results show the feasibility and safety of EVT in a PSC for patients with AIS due to LVO. The implementation of this strategy may be important for shortening the time to thrombectomy.     

Stroke after adult-onset epilepsy: A population-based retrospective cohort study

BackgroundEarlier studies indicate a higher risk of subsequent stroke in PWE aged ≥60. However, little is known of the incidence of subsequent stroke in people with epilepsy (PWE) aged 35 through 60. We determined the risk factors that increase the incidence of stroke following adult-onset epilepsy in a large statewide population over a 10-year period.MethodsSouth Carolina hospital discharge and emergency department (ED) data from 2000 to 2011 were used. The study was limited to persons aged ≥ 35 years without prior stroke. Cases included patients diagnosed with epilepsy who were hospitalized or visited the ED. Controls were people with an isolated fracture of the lower extremity without any history of epilepsy or seizure disorders, presumed to approximate the health status of the general population. Epilepsy, fracture, stroke, and comorbid conditions were ascertained by diagnostic codes from health-care encounters. Only persons having stroke occurring ≥ 6 months after the onset of epilepsy or after the first clinical encounter for controls were included. Cox proportional hazards modeling was performed to determine the risk of stroke.ResultsThere were 21,035 cases with epilepsy and 16,638 controls who met the inclusion criteria. Stroke incidence was 2.5 times higher following adult-onset epilepsy (6.3%) compared with controls (2.5%). After adjusting for comorbidities and other factors, cases with epilepsy showed a 60% higher risk of stroke (HR = 1.6; 95% CI: 1.42–1.80) compared with controls. Nearly half of the strokes in cases with epilepsy occurred in those with first diagnosis between ages 35 and 55. Somatic comorbidities associated with increased risk of stroke were more prevalent in cases with epilepsy than controls yet similar in both groups with stroke. Risk of stroke increased with increasing age in both groups. However, the risk of stroke in cases with epilepsy increased faster and was similar to that in controls who were ≥ 10 years older.ConclusionAdult-onset epilepsy at age 35 and older warrants consideration for occult cerebrovascular disease as an etiology of the epilepsy, which may also increase the risk of subsequent stroke. Somatic comorbidities frequently associated with epilepsy include comorbid conditions that share the same underlying pathology with stroke (i.e., hypertension, hyperlipidemia, myocardial infarction, diabetes, and arteriosclerosis). This increased risk of stroke in patients with adult-onset epilepsy should dictate the evaluation and management of stroke risk factors to prevent stroke.     

Symptomatic Moyamoya disease: Clinical features and outcome after indirect bypass surgery in four French adults

BackgroundThere are a few reports of moyamoya disease (MMD) in the European Caucasian adult population. We present the clinical manifestations, the neuroradiological aspects, the treatment, and the outcome after surgical revascularization of four French patients with MMD.Patients and methodsWe identified four adults (age >18 years; three women and one man) with MMD who underwent digital subtraction catheter angiography at our institution from 1997 through 2006. The median age at symptom onset was 35 years (range, 22 to 41 years). The initial clinical presentation was intracerebral hemorrhage in three patients and ischemic stroke in one patient.ResultsThree patients underwent bilateral surgical revascularization and one patient underwent unilateral surgical revascularization. All patients underwent the same surgical revascularization procedure (encephalo-duro-arterio-myo-synangiosis). The mean (+/? SD) period of follow-up after diagnosis of MMD was 6 years and 9 months (+/? 3 years and 5 months). No patient experienced any recurrent hemorrhagic or ischemic stroke. No perioperative stroke occurred. No patient was severely disabled or unable to walk. Three patients out of four were employed.ConclusionOur data suggest safety and a potential benefit of surgical revascularization (indirect bypass surgery) in European adult patients with symptomatic MMD. Further long-term prospective multicenter studies are needed. The establishment of a registry would be useful in order to accumulate data in large numbers of European patients with this uncommon disease.     

Psychiatric disorders in low birthweight young adults. Prevalence and association with assessments at 11 years

ObjectiveTo compare mental health of 136 young adults without neurosensory handicaps born with low birthweight (LBW, birthweight less than 2000 g) with 132 adults with normal birthweight (NBW).MethodA cohort of moderate LBW and NBW young adults were assessed with the Mini-International Neuropsychiatric Interview (MINI) at 19 years and the Children Assessment Schedule (CAS) at 11 years of age.ResultsAt 19 years of age, 44 out of 136 (32%) LBW young adults were diagnosed with a psychiatric disorder compared to 10% NBW (OR: 2.8; 95% CI: 1.1, 4.5, P = 0.02). Among the LBW young adults, affective-, anxiety-, ADHD- and antisocial personality disorders were most common, and nine subjects (20%) had more than one diagnosis. Of 97 LBW subjects examined both at 11 and 19 years of age, 54 (56%) were mentally healthy though out adolescence. This was half as many as for controls (OR: 0.6; 95% CI: 0.3 to 0.9).ConclusionModerate LBW was associated with an increased risk of psychiatric disorders in young adulthood. Only half of LBW young adults stayed healthy throughout adolescence.     

Clinical utility of a personalized and long-term monitoring device for Parkinson's disease in a real clinical practice setting: An expert opinion survey on STAT-ON™

BackgroundSTAT-ON™ is an objective tool that registers ON-OFF fluctuations making possible to know the state of the patient at every moment of the day in normal life. Our aim was to analyze the opinion of different Parkinson's disease experts about the STAT-ON™ tool after using the device in a real clinical practice setting (RCPS).MethodsSTAT-ON™ was provided by the Company Sense4Care to Spanish neurologists for using it in a RCPS. Each neurologist had the device for at least three months and could use it in PD patients at his/her own discretion. In February 2020, a survey with 30 questions was sent to all participants.ResultsTwo thirds of neurologists (53.8% females; mean age 44.9 ± 9 years old) worked in a Movement Disorders Unit, the average experience in PD was 16 ± 6.9 years, and 40.7% of them had previously used other devices. A total of 119 evaluations were performed in 114 patients (range 2–9 by neurologist; mean 4.5 ± 2.3). STAT-ON™ was considered “quite” to “very useful” by 74% of the neurologists with an overall opinion of 6.9 ± 1.7 (0, worst; 10, best). STAT-ON™ was considered better than diaries by 70.3% of neurologists and a useful tool for the identification of patients with advanced PD by 81.5%. Proper identification of freezing of gait episodes and falls were frequent limitations reported.ConclusionSTAT-ON™ could be a useful device for using in PD patients in clinical practice.     

Continuity and discontinuity of trouble sleeping behaviors from early childhood to young adulthood in a large Australian community-based-birth cohort study

ObjectiveTo examine the continuity and discontinuity of trouble sleeping behaviors (TSB) from childhood to adolescence and young adulthood in a community-based prospective birth cohort study.MethodsThe original study comprised 7223 mother–offspring pairs who were followed prospectively at pregnancy, 6 months, 5, 14 and 21 years post-delivery. Participant numbers differ by follow-up stages. There were 3184 offspring for whom we have consistently collected information on TSB retrospectively at 2–4 years, and prospectively at 14 and 21 years of age.ResultsThese comprised maternal-reported offspring TSB at 2–4 years and 14 years, and offspring-reported trouble sleeping at 14 and 21 years. One in two children had persistent trouble sleeping from 2–4 to 14 years and two-thirds from 14 to 21 years. In the adjusted analysis, compared with 2–4-years-old children with no trouble sleeping, those who experienced trouble sleeping were 1.20 (95% CI: 1.00, 1.44) times more likely to have trouble sleeping at 21 years. Similarly, adolescents who experienced trouble sleeping were 1.94 (95% CI: 1.66, 2.27) times more likely to experience trouble sleeping at 21 years.ConclusionsThere is a continuity of TSB from early childhood through adolescence and young adulthood, although the persistence of TSB is strongest from adolescence to young adulthood. Interventions improving sleep in young children may prevent longer term difficulties in adolescents and young adults.     

Socio-demographic and psychosocial correlates of erectile dysfunction among hypertensive patients

ObjectiveThe main aim of the study was to estimate the prevalence of ED and the associated socio-demographic and psychological correlates among hypertensive patients from a rural multiethnic community in Malaysia.MethodsA cross-sectional study was conducted among hypertensive patients attending rural primary care clinics. The socio-demographic, health characteristics, erectile function and levels of depression, anxiety and stress were recorded and analysed. The International Index of ErectileFunction-5 (IIEF-5) questionnaire and the Depression, Anxiety and Stress Scale (DASS-21) were used to assess erectile function and the levels of depression, anxiety and stress, respectively.ResultsA total of 253 hypertensive patients comprising 178 (70.4%) Malays, 56 (22.1%) Chinese and 18 (7.5%) Indians participated. The mean age of participants was 59.8 ± 10.62 years. Overall, the prevalence rate of ED was 62%: 90 (35%) with moderate and 69 (27%) with severe ED. The prevalence rate of ED among those aged 65 years or older (83.1%) was significantly higher than those less than 65 years (51.8%), (p < 0.001). Higher prevalence rates were also noted among the Chinese (78.6%) compared to Malays (59.6%) and Indians (50%) (p = 0.021); lower education level (69.1%) (p = 0.026), among hypertensive patients with concomitant diabetes mellitus (70.6%) (p = 0.026) and WHR ≥0.9 (31.3%) (p = 0.021). However, no significant association was found between depression, anxiety and stress scores with IIEF-5 score.ConclusionThe prevalence rate of ED among Malaysian hypertensive patients is high. The rate increases significantly with age, Chinese ethnicity, concomitant diabetes mellitus, lower education level, WHR and the number of pack years of smoking. ED should be properly recognized and managed in hypertensive patients.     

Effectiveness of a digital application to improve stroke knowledge for kids

IntroductionStroke is highly prevalent in Latin America and one of the leading causes of morbidity and mortality in the world. Educating children about stroke has been established as an effective method to detect symptoms early, reduce hospital visits, and raise awareness among adults.ObjectiveTo analyze the effectiveness of a mobile application to improve knowledge and understanding of stroke among children.MethodWe conducted a focus group session including 12 children in order to analyze the behavior of 6 questions previously validated by expert neurologists. Subsequently, 105 primary school students between the ages of 7 and 12 completed a questionnaire on stroke symptoms and how to contact the emergency services before and after using an application on stroke symptoms. Qualitative analyses and the Student t test were used to verify the existence of differences between pre- and post-intervention test results.ResultsAfter a single 40-min working session with the application, between 50% and 67% of the children were able to identify the signs of stroke, and 96.2% knew the national emergency services telephone number. Statistical analysis revealed statistically significant differences before and after the intervention with the digital application (t = 19.54; p < 0.001) and intragroup differences in the post-intervention test results (t = 40.71; p < 0.001).ConclusionPrimary school children who used our digital application increased their knowledge, understanding, and learning of stroke symptoms.     

Sudden unexpected death in epilepsy: Knowledge and experience among U.S. and Canadian neurologists

ImportanceSudden unexpected death in epilepsy (SUDEP) is a common cause of mortality in patients with the disease, but it is unknown how neurologists disclose this risk when counseling patients.ObjectiveThis study aimed at examining SUDEP discussion practices of neurologists in the U.S. and Canada.DesignAn electronic, web-based survey was sent to 17,558 neurologists in the U.S. and Canada. Survey questions included frequency of SUDEP discussion, reasons for discussing/not discussing SUDEP, timing of SUDEP discussions, and perceived patient reactions. We examined factors that influence the frequency of SUDEP discussion and perceived patient response using multivariate logistic regression.ParticipantsThe participants of this study were neurologists who completed postgraduate training and devoted > 5% of their time to patient care.ResultsThere was a response rate of 9.3%; 1200 respondents met eligibility criteria and completed surveys. Only 6.8% of the respondents discussed SUDEP with nearly all (> 90% of the time) of their patients with epilepsy/caregivers, while 11.6% never discussed it. Factors that independently predicted whether SUDEP was discussed nearly all of the time were the following: number of patients with epilepsy seen annually (OR = 2.01, 95% CI = 1.20–3.37, p < 0.01) and if the respondent had a SUDEP case in the past 24 months (OR = 2.27, 95% CI = 1.37–3.66, p < 0.01). A majority of respondents (59.5%) reported that negative reactions were the most common response to a discussion of SUDEP. Having additional epilepsy/neurophysiology training was associated with an increased risk of a perceived negative response (OR = 1.36, 95% CI = 1.02–1.82, p = 0.038), while years in practice (OR = 0.85, 95% CI = 0.77–0.95, p < 0.005) and seeing both adults and children were associated with a decreased likelihood of negative response (OR = 0.15, 95% CI = 0.032–0.74, p = 0.02).ConclusionsU.S. and Canadian neurologists rarely discuss SUDEP with all patients with epilepsy/caregivers though discussions are more likely among neurologists who frequently see patients with epilepsy or had a recent SUDEP in their practice. Perceived negative reactions to SUDEP discussions are common but not universal; more experienced neurologists may be less likely to encounter negative reactions, suggesting that there may be ways to frame the discussion that minimizes patient/caregiver distress.     

Large artery intracranial stenosis in young adults with ischaemic stroke

Large artery intracranial stenosis (ICS) is a common finding in stroke patients, but is much less prevalent in Western countries than in Asia and in young adults than in the elderly. We investigated the prevalence and causes of ICS among French young adults with ischaemic stroke. Clinical and radiological data of patients aged 18–54 years treated consecutively for acute ischaemic stroke in the anterior circulation at a tertiary stroke centre were analysed retrospectively. Patients with > 50% ICS were identified. ICS was evaluated using TOF-MRA, vessel wall-MRI, digital subtraction angiography and CT-angiography. A total of 316 patients were included. ICS was diagnosed in 29 patients, resulting in a prevalence of 9.2% (95% CI, 6.2 to 13.3). The leading cause of ICS was atherosclerosis (n = 13), ahead of moyamoya disease (n = 4), dissection (n = 2), vasculitis (n = 2), and reversible cerebral vasoconstriction syndrome (n = 1). The cause of ICAS could not be determined in 7 patients. ICS was found in nearly one in 10 ischaemic strokes among French young adults. Atherosclerosis was the leading cause of ICS. The cause of ICS could not be determined in almost a quarter of the patients.     

First-ever population-based study on status epilepticus in French Island of La Reunion (France) – Incidence and fatality

PurposeWe aimed to determine the incidence and case-fatality of first-ever status epilepticus (SE) among the general population living in La Reunion Island, a French overseas territory in the Indian Ocean near Madagascar.MethodsWe recruited cases (1st July 2004–30th June 2005) in a population-based manner using neurology, neurosurgery, electroencephalogram, emergency, paediatric and neuroradiology services; emergency medical aid service; emergency and admission service of private and public clinics; neurologists (public and private); private paediatricians and practitioners of various rural hospitals. All cases had an electroencephalogram (EEG) and were assessed by an epileptologist. Standard definition and classification schemes were used. Those with known epilepsy were not part of this analysis.ResultsSixty-five cases (males: n = 41, 63.1%) had epileptologist-confirmed SE, with 38.5% (n = 25) being >60 years of age. Global incidence rate was 8.52/100 000 (95% confidence interval 6.5–10.5). A bimodal age distribution with high frequency and incidence among young (<10 years age) (frequency: 12.3%; incidence 6.6/100,000) and aged (>60 years) (frequency: 40.0%; incidence 35.0/100,000) was observed. We found that 60%, 32.3%, 6.7% had convulsive, partial and non-convulsive SE respectively (1% remained unclassified). Of the cases identified, 44.6%, 38.5%, 16.9% had unprovoked, provoked or cryptogenic seizures respectively. The most important aetiological factors identified included: stroke (27.7%), alcoholism/toxicity (18.5%), cryptogenic (16.9%), infections (10.8%). Mortality was 18.5%.ConclusionThe incidence of SE incidence in La Reunion Island was lower than that described elsewhere. The status type was found to be dependent on aetiology and age. The study confirms that SE is more frequent in men and in older adults and is associated with significant short-term case mortality.     

How predictable is the erectile function of patients with epilepsy?

IntroductionErectile dysfunction (ED) is often reported by patients with epilepsy and may be related to endocrine system abnormalities, side effects of antiepileptic drugs, psychiatric comorbidities, and family or social difficulties.AimsThis study aimed to identify independent predictor factors for ED in patients with epilepsy.Main outcome measuresthe five-question form of the International Index of Erectile Function (IIEF-5).MethodsIndependent predictive factors for ED evaluated by the IIEF-5 questionnaire in 36 patients (mean age: 39 years) with focal epilepsy (mean: 6 seizures/month) were identified by multiple linear regression analysis.ResultsEight (21.1%) patients were asymptomatic. Among the symptomatic patients, 11 (28.9%) had mild dysfunction, 10 (26.3%) had moderate dysfunction, and 9 (23.7%) showed severe ED. The multiple linear regression model including family income (B = 0.005; p = 0.05), education levels in years (B = 0.54; p = 0.03), depressive symptoms determined by HADS depression subscale (B = − 0.49; p = 0.03), and prolactin levels (B = − 0.45; p = 0.07) showed a moderate association (r = 0.64) with the IIEF questionnaire and explained 41% (r² = 0.41) of its variation.ConclusionsErectile dysfunction is highly prevalent in patients with focal epilepsies. Education, depressive symptoms, and prolactin levels can predict erectile dysfunction in up to 41% of patients with epilepsy. This preliminary report justifies further efforts to make a large sample size study to identify independent biomarkers and therapeutic targets for ED treatment in patients with epilepsy.     

Medical outcome in epilepsy patients of young adulthood—A 5-year follow-up study

The appearance of new anti-epileptic drugs (AED) during the last decade has provided neurologists and their patients with a greater choice, but the proof for their superiority over traditional AEDs is sparse, especially their use in adolescence and young adulthood. We studied a group of young adults (18–27 years) with epilepsy and compared their situation in 2004 with those 5 years earlier.Materials and methodsThe participants (n = 97) answered questionnaires regarding seizure-frequency, AED, side-effects and quality-of-life. Information was also taken from medical records.ResultsThe use of new generation AEDs increased during the 5-year study period, particularly among women. However seizure frequency had not changed significantly over time, and compared to men the effectiveness in controlling seizures was lower in women. The participants reported normal quality-of-life (QOL), which may indicate that the increase in number of AEDs to choose from actually improved the situation for these young adults with epilepsy. Frequency of seizures and cognitive side-effects of AEDs were associated with a lower QOL.ConclusionsMore women than men seem to be treated with new AEDs, and that the increase in use of new AEDs does not reduce seizure frequency in young adulthood. The effectiveness in controlling seizures seems to be lower in women in the age group studied. Further studies are required to better understand how epilepsy related factors interact.     

Intracerebral hemorrhage in young from a tertiary neurology center in North India

ObjectiveThere is paucity of information on the etiology and predictors of outcome of intracerebral hemorrhage (ICH) in young which may have regional and ethnic differences. In this study, we report the etiology and predictors of outcome of ICH in young patients from North India.Methods404 patients with ICH in young (16–50 years) were retrospectively reviewed who were admitted in neurology service of a tertiary care teaching hospital in North India. The data were retrieved from the computerized hospital information service. The information about the demography, risk factors, clinical status, laboratory findings, CT/MRI features and angiography (CT, MRI or digital substraction) were noted. The etiology of ICH was ascertained based on clinical, laboratory and radiological findings. Outcome at 1 month was assessed using Glasgow Outcome Scale (GOS).ResultsThe mean age of the patients was 41.6 years and 23.8% were females. Hypertension (57.2%), hypocholesterolemia (33.7%), alcohol (15.8%) and anticoagulant (3.5%) were the important risk factors. The etiology of ICH was hypertension in 320 (79.2%), vascular malformation in 17 (4.2%), coagulopathy in 16 (4%), cerebral venous sinus thrombosis (CVST) in 9 (2.2%), thrombocytopenia in 3 (0.7%), vasculitis in 2 (0.5%) and cryptogenic in 37 (9.2%) patients. The patients with cryptogenic ICH were younger, had better Glasgow coma scale (GCS) on admission and good outcome compared those with known etiology. The most common location of ICH was basal ganglion and thalamus (71.3%). 102 (25%) patients died, 161 (39.9%) had poor and 141 (34.9%) had good outcome. Hypertensive ICH patients had frequent death or disability (P < 0.001). On multivariate analysis, low GCS score (P < 0.001), large ICH (P = 0.01) and high leukocyte count on admission (P = 0.03) were significantly related to the 1 month mortality.ConclusionHypertension is the commonest cause of ICH in young Indian adults and its outcome is related to volume of ICH, GCS score and admission leukocyte count.     

Clinical characteristics and prognosis of mah-jong-induced epilepsy: A cohort review of 56 patients

ObjectiveThe objective of this study was to investigate the clinical characteristics of reflex epilepsy elicited by playing mah-jong in Western China.MethodsConsecutive patients with reflex epilepsy triggered by mah-jong from Jan 2011 to April 2014 were recruited at West China Hospital. Each patient underwent EEG recording and brain MRI scan. All clinical data were reviewed. Each patient had at least 12 months of follow-up.ResultsFifty-six patients were included, with majority of them being male (52/56, 92.9%). Mean age was 43.9 ± 10.0 years. Mean age at seizure onset was 35.1 ± 16.3 years. Mean duration of reflex epilepsy was 4.0 ± 2.2 years. The mean follow-up time was 32.0 ± 13.2 months. Seventeen patients (30.4%) took antiepileptic drugs. Regardless of the medication, all 14 patients who avoided playing mah-jong reached seizure freedom, and 22 of the 42 patients (52.4%) who still played mah-jong continued to have seizures (p < 0.05).ConclusionsReflex epilepsy induced by mah-jong is a unique type of epilepsy in the Chinese population and usually occurs in adults after a long time lag of playing mah-jong. Avoiding the triggers is the most effective treatment for epilepsy.     

Leukoencephalopathy with calcifications and cysts (LCC): 5 cases and literature review

IntroductionLeukoencephalopathy with calcifications and cysts (LCC) is a rare autosomal recessive cerebral angiomatous-like microangiopathy characterized by diffuse and asymmetric white-matter lesions associated with multiple calcifications and cysts. The disease is caused by SNORD118 mutations. The entire clinical spectrum of LCC is not yet fully determined.Material and methodsTo define the clinical spectrum of LCC, we analyzed data from recently diagnosed cases and from the litterature. Both clinical and imaging features from our five LCC cases harboring compound heterozygous SNORD118 mutations were presented and all cases reported in the litterature reviewed.ResultsNinety-two LCC cases including our five patients were identified. Consanguinity was rare (4%), and 97% of cases were symptomatic. Mean age of first clinical manifestations was 16.1 ± 16.1 years (range 1 month–71 years) and was earlier in men (10.3 ± 14.3 years) than in women (20.2 ± 22.8 years) (P = 0.02). The main inaugural symptoms were seizures (36%; mean age at onset: 5.2 ± 9.5 years) and progressive neurological symptoms including ataxia, dystonia and spasticity (26%; 27.8 ± 23.6 years). Intracranial hypertension was less frequently observed (14%), mostly in adults (mean age 31.5 ± 13.2 years). Ischemic or hemorrhagic strokes were inaugural symptoms in two adults (2%). During follow-up, most patients developed progressive extrapyramidal, cerebellar and pyramidal signs (83%), cognitive decline (56%), seizures (37%), intracranial hypertension (30%) or stroke (2%).ConclusionIn LCC, the clinical spectrum is largely heterogeneous and the course of the disease appears highly variable in contrast to other hereditary cerebral small vessel diseases.