Treatment and long-term prognosis of myoclonic-astatic epilepsy of early childhood

PURPOSE: We retrospectively studied patients with myoclonic-astatic epilepsy of early childhood (MAE) to investigate the most effective treatment and long-term seizure and intellectual prognosis. SUBJECTS: Eighty-one patients with MAE were recruited from among 3600 patients with childhood epilepsy according to the ILAE criteria of MAE. METHODS: We retrospectively investigated the clinical characteristics and ultimate prognosis of the patients with MAE from the medical records. The effects of various antiepileptic drugs, ketogenic diet and ACTH treatments on myoclonic-astatic seizures (MS/AS), apparently a hallmark of this unique epileptic syndrome, were also studied. RESULTS: MS/AS in 89 % of the patients disappeared within 1 to 3 years despite initial resistance, but generalized tonic-clonic or clonic seizures [G(T)CS] tended to continue. The most effective treatment for the MS/AS was ketogenic diet, followed by ACTH and ESM. At the last follow-up, 55 patients or 68 % of all the patients had remission of epilepsy, 11 patients or 14 % experienced a recurrence of GTCS after a long remission period but easily regained control, and the remaining 15 patients or 18 % continued to have seizures and intellectual outcomes were poor. In one half of these patients with poor outcomes, repeated minor epileptic status and nocturnal generalized tonic seizures persisted. A family history of epilepsy and a combination of minor epileptic status are risk factors for poor outcomes. CONCLUSION: MAE is considered to form a clinical spectrum ranging in its main seizure type from myoclonic to atonic, and in seizure and intellectual outcomes from benign to malignant. The overall prognosis, despite initial resistance to treatment, appears to be much better than originally thought when ILAE definitions excluding SME are followed.     

Five-year prognosis for dysthymic disorder in old age

The 5-year prognosis for 199 elderly patients (60 yr+) suffering from dysthymic disorder is described. Twenty-nine per cent of the men and 39% of the women recovered, 26% of both were suffering from depression after 5 years, 9% of both had a possible dementia, 32% of the men and 22% of the women had died and 3% of the men and 5% of the women could not be contacted. The outcome did not differ between the sexes or age groups. A poor prognosis among men was related to a low educational level, a non-independent previous occupation, the occurrence of depression previously, a high number of hobbies and the following depressive symptoms: initial and delayed insomnia, retardation and psychomotor agitation. A poor prognosis was also found for those whose functional capacities, hobbies or emotional relations with their wives had declined or who had experienced a pulmonary disease or few or no social growth events during the follow-up. A poor prognosis among women was related to low self-perceived health, high sum score of the Hamilton Rating Scale for Depression, thyroid disorder and the following depressive symptoms: loss of libido and diurnal variation of symptoms. A poor prognosis was also found for those whose physical health, functional capacities, social participation rate or hobbies had declined or who had experienced a serious disease during the follow-up. A favourable prognosis was found for women who moved to live alone or became more active in taking physical exercise.     

行血管内介入手术治疗非急性期眼段症状性ICAO患者预后不良的危险因素

目的 探讨行血管内介入手术治疗非急性期眼段症状性颈内动脉闭塞(Internal carotid artery occlusion,ICAO)患者预后不良的危险因素。方法 回顾性分析本院2010年1月-2018年12月收治并行血管内介入手术治疗非急性期眼段症状性ICAO患者共216例的临床资料,根据随访2年预后情况分组,采用Logistic回归模型评价预后不良的独立危险因素。结果 入选且接受血管内介入手术患者出院当天实现血管开通208例,开通成功率为96.30%(208/216),预后不良率为19.23%(40/208); 同时出院当天改良Rankin 量表(Modified Rankin scale,mRS)评分和美国国立卫生院神经功能缺损量表(Natinal Institute of health strke scale,NIHSS)评分均显著少于入院当天(P<0.05); 2组年龄、合并高血压病、2型糖尿病(Type 2 diabetes,T2DM)及冠心病的比例均有明显差异(P<0.05); Logistic回归模型多因素分析显示,合并高血压病和T2DM是行血管内介入手术治疗非急性期眼段症状性ICAO患者预后不良的独立危险因素(P<0.05)。结论 行血管内介入手术治疗非急性期眼段症状性ICAO患者预后不良与合并高血压病和T2DM关系密切。     

The temporal aspects of prognosis in epilepsy.

A major reason for the conflicting views concerning prognosis in epilepsy has been the failure to account for the temporal aspects of seizure relapse and remission. In this paper prognostic studies in a variety of areas are reviewed to emphasise this point. Most traditional studies (including studies of withdrawal of medication) show a generally poor prognosis for epilepsy. These have been hospital-based and, as such, inevitably over-represent patients with chronic epilepsy. Investigations (both hospital and community based) of patients from the onset of seizures show a much better prognosis, and most patients suffer only a small number of seizures in total over a relatively short period of time and then remit. The longer the epilepsy is active the less likely is eventual remission, but once remission is achieved it is usually permanent. The traditional view of epilepsy as a chronic condition with a continuing tendency to relapse is therefore not tenable for the majority of patients. The patterns of epilepsy are established relatively early in its course in most cases, and long term prognosis might therefore be predictable within a few years of the onset of seizures in most patients. Prior to the introduction of effective treatment, it was said that epilepsy rarely remitted, and it is possible that early treatment may actually improve long term prognosis.     

Incidence of central nervous system involvement in chronic lymphocytic leukemia and outcome to treatment

Leptomeningeal involvement in patients with CLL is relatively rare and the prognosis is usually considered to be poor. The authors reviewed all CLL patients treated in a tertiary referral center to assess the incidence and outcome of leptomeningeal involvement (LI) in CLL. They found an incidence of 1-2% of LI. Most of the patients with LI had a longterm survival, despite failure to clear the cerebrospinal fluid from tumor cells.     

Intracranial large artery disease in Alzheimer’s disease and vascular dementia among ethnic Asians

Background and purpose: An underlying vascular etiology underpins vascular dementia (VaD) and possibly Alzheimer’s disease (AD). Intracranial large artery disease (ICLAD) is a common site of disease among ethnic Asians, and carries a poor prognosis. We studied the prevalence of ICLAD among ethnic Asian patients with AD and VaD. Methods: We recruited patients with AD and VaD from a retrospective review of consecutive ethnic Asian patients presenting to our dementia clinic. ICLAD was evaluated by visual inspection of brain magnetic resonance angiography by two observers in consensus, and defined as >50% luminal narrowing. Results: There were 56 patients with probable AD and 47 with probable VaD. ICLAD was prevalent among 53% of VaD patients and 18% of AD patients. Conclusions: There is a relatively high burden of ICLAD among AD and VaD patients of Asian ethnicity. We suggest that ethnic Asian dementia patients are a potential group to investigate if ICLAD is associated with clinical symptoms or prognosis and if treatment strategies targeted at ICLAD retard the progression of cognitive impairment.     

Catatonia: prediction of outcome

Of 250 patients diagnosed as having catatonia, 40% of the 214 for whom follow-up information was available had at some time recovered completely. Although certain features of history and mental status significantly predicted recovery, or its absence, many factors differed from those used in studies of mixed subtypes of schizophrenia. Most motor symptoms correlated poorly with outcome: in particular, those symptoms classically associated with catatonia (catalepsy, posturing, staring, mutism, rigidity, grimacing, negativism) proved poor predictors of outcome. A seven-factor scale used in previous studies allowed refinement of prognosis, but the majority of cases were bunched in the middle of the scale, leaving relatively few cases in the good- and poor-prognosis groups. Duration of illness from onset of prodromal symptoms to hospitalization correlated with outcome as strongly as did the seven-factor scale and provided a more useful distribution of cases among the prognostic groups. Catatonia, a syndrome that has long been associated with schizophrenia, has a prognosis generally better than other subtypes of schizophrenia. The presence of catatonic symptoms should be grounds for separately reporting these patients in studies of treatment and outcome.     

Actual situation and social prognosis of women seeking psychiatric care at the emergency hostel of Tokyo Metropolitan Women's Counseling Center]

The emergency hostel of Tokyo Metropolitan Women's Counseling Center, established in 1957, provides protection and care for about 600 Japanese or foreign women per year. The women housed there need social support for various reasons such as prostitution, poverty, somatic or mental diseases, or domestic violence (DV). We investigated the sociodemographic characteristics, psychiatric diagnoses and social prognoses of 2667 women who consulted the psychiatric clinic in the emergency hostel between 1961 and 1997. Seventy-four women consulted the psychiatric clinic per year, on average. Most were aged between 20 and 49. During the study period, there was a gradual decline in the number referred through the prostitution prevention law. Psychiatric diagnoses at the first visit varied widely. Annual comparison showed a gradual decrease in schizophrenia and manic-depressive illness, but an increase in substance abuse, psychogenic reaction, and personality disorder. Sociodemographically, most subjects appeared to have been children from underprivileged backgrounds. The social prognoses of 930 cases judged in March 1998 were good in 25%, moderate in 23% and poor in 48%. The poor prognosis group contained significantly more women with schizophrenia and personality disorder than the other two groups. The poor prognosis group tended to include more cases of substance abuse, while the good prognosis group contained more cases of depression and mental retardation. Women in the DV group tended to have more children than those in the non-DV group, and to have a higher prevalence of psychogenic reaction and a lower prevalence of schizophrenia. The DV group also tended to include more subjects with a moderate social prognosis and fewer subjects with a poor social prognosis. Specialized treatment should be provided for women after emergency admission to the hostel and this treatment needs to be aimed at improving social adaptation of the hostel residents, especially those with schizophrenia, personality disorders and substance abuse. Victims of DV should receive more conscious attention. In particular, prevention of mental disorders should be aimed not only at the residents but also their children.     

256例重型、特重型颅脑外伤后行大骨瓣减压术患者的回顾性分析

目的探讨256例重型、特重型颅脑外伤（TBI）患者行大骨瓣减压的远期治疗效果。 方法单中心回顾性队列研究纳入自2012年7月至2016年12月济南军区总医院神经外科收治256例的因重型、特重型TBI急诊行大骨瓣减压的患者,其中重型（GCS评分6~8分）166例、特重型（GCS评分3~5分）90例,采用标准大骨瓣开颅术,减压窗面积约为12 cm×15 cm左右。术后随访18个月,预后分为良好恢复、中残、重残、植物状态、死亡;其中,良好恢复、中度病残为预后良好者,严重病残和植物状态为预后较差者。 结果恢复良好/中残患者104例（40.61%）,但其规范评估行为、心理表现与心理健康的负担未纳入此次临床评估。重残54例、植物状态38例、死亡60例,共计152例患者预后较差（59.37%）,可判断59.37%的患者存在预后不良。 结论标准外伤大骨瓣减压术是治疗重型TBI脑疝形成的有效方法。发生重型、特重型TBI的患者其临床预后差,表现为死亡、植物生存、功能损伤,需要医学界探索新的治疗策略。     

入院NLR与外伤性脑内血肿扩大及病人预后的关系

目的 探讨入院中性粒细胞/淋巴细胞比值（NLR）与外伤性脑出血（t ICH）血肿扩大及病人预后的相关性。方法 2019年7月至2021年8月前瞻性收集t ICH病人共115例。根据入院血常规计算NLR。伤后3个月,根据改良Rankin量表评分评估预后,0～2分为预后良好。血肿扩大定义为二次CT扫描与基线CT扫描相比体积增加33%或>5 ml。结果 115例中,13例（11.30%）发生血肿扩大。伤后3个月,41例（35.65%）预后不良,74例（64.35%）预后良好。多因素logistic回归分析显示,血肿扩大以及入院NLR水平增高为t ICH病人预后不良的独立危险因素（P<0.05）,入院NLR水平增高是血肿扩大的独立危险因素（P<0.001）。ROC曲线分析显示,入院NLR水平评估血肿扩大的AUC为0.896(95%CI 0.821～0.971;P<0.001),入院NLR水平评估t ICH病人伤后3个月预后不良的曲线下面积为0.709(95%CI 0.615～0.802;P<0.05)。结论 t ICH血肿扩大影响病人预后,神经炎症参与t ICH进...     

脑小血管病影像学标志物与自发性高血压脑出血的相关性研究进展

临床中脑小血管病患者发生自发性高血压脑出血的现象多见,且很多患者合并脑小血管病的影像学标志物,本综述对二者临床上的相关性进行总结。脑白质高信号的严重程度与自发性高血压脑出血复发的风险呈正相关,增加死亡率和不良预后;总微出血数量与血肿扩大显著相关,可预测患者的不良预后;血管周围间隙扩大或腔隙性梗死合并自发性高血压脑出血的临床研究较少,有待于进一步阐明;多个影像学特征的脑小血管病患者,其脑小血管病总体负荷评分越高,临床不良功能预后及脑出血复发风险越高。     

Devic's optic neuromyelitis. 4 cases

We report 4 cases of neuromyelitis optic (Devic's disease), with 3 to 7 year-follow-up for 3 of them. Most of the data agreed with the previously reported cases: subacute onset, association of optic neuritis and myelitis within several weeks or months, absence of relapse, good prognosis for paraplegia but poor prognosis for visual loss, and effectiveness of corticosteroids on spinal cord involvement in 4 cases. Moreover these 4 cases have revealed the following rare features: unilateral visual disturbance (1 case), total recovery of visual signs (1 case) with only a short period of follow-up, onset during childhood (1 case), onset following an acute lymphocytic meningitis (1 case), and dependency from corticosteroid therapy (2 cases). None of the patients had obvious immunological disturbance. N.M.R. imaging revealed in 2 cases, disseminated demyelinated areas which confirmed neuropathological data from the literature, showing that anatomical lesions could be wider than expected on clinical signs. The poor visual prognosis, the serum inflammatory signs, the epidemiologic data, the high cell and protein levels in C.S.F., and the absence of relapse suggested, in our cases and in most of the previously reported ones, that neuromyelitis optic must be differentiated from multiple sclerosis.     

Eye closure sensitivity in juvenile myoclonic epilepsy and its effect on prognosis

PurposeTo investigate eye closure sensitivity (ECS) in the EEGs of patients diagnosed with juvenile myoclonic epilepsy (JME) and its relationship to prognosis.MethodsWe included 76 JME patients with a minimum follow-up of one year and evaluated a total of 254 EEGs to obtain evidence of ECS. The patients were grouped according to their response to treatment, and these subgroups were compared in relation to ECS and other clinical and EEG features.ResultsThere were 12 patients (15.8%) with poor prognosis who showed resistance to appropriate anti-epileptic drug treatment, 15 (19.7%) patients with pseudo-resistance, and 49 (64.5%) patients with good prognosis. The EEGs of only four of the patients displayed pure ECS (5.3%), and only one of these exhibited poor prognosis. Furthermore, 11 patients (14.5%) had both ECS and photosensitivity, and two of these patients exhibited poor prognosis. Thus, neither pure ECS nor ECS with photosensitivity correlated with poor prognosis. A family history of epilepsy and focal findings on the EEG was correlated with poorer prognosis.ConclusionsECS is a rare EEG finding in JME and does not appear to be a marker for poor prognosis.     

Deep vein thrombosis following ischemic stroke among Asians

BACKGROUND: Deep vein thrombosis (DVT) is perceived as uncommon among Asian stroke patients. However, there is a paucity of published data, and thus, we studied the frequency, characteristics and prognosis of DVT following ischemic stroke in Asian patients with lower limb paresis. METHODS: Doppler ultrasound scans of the lower limbs were performed at days 7-10 and 25-30 after stroke onset. The functional status of patients was assessed at 6 months using the modified Rankin scale. RESULTS: DVT was detected in 30% of patients at days 7-10 and in 45% of patients at days 25-30. Most thromboses were distal. There were significant associations of age and degree of weakness with the presence of DVT at days 25-30, but not at days 7-10. DVT in the first month after stroke was associated with poorer outcome at 6 months. CONCLUSIONS: DVT following ischemic stroke among Asians is common and associated with poor functional outcome.     

Massive intraventricular haemorrhage from aneurysmal rupture: patient proportions and eligibility for intraventricular fibrinolysis

Massive intraventricular haemorrhage (IVH) complicating aneurysmal subarachnoid haemorrhage (SAH) is associated with a poor prognosis. Small observational studies suggest favourable results from fibrinolysis of the intraventricular blood. We performed an observational study on IVH in a large series of patients with SAH to assess the proportion of patients that may benefit from fibrinolytic treatment. From our prospective database we retrieved patients with aneurysmal SAH admitted between January 2000 and January 2005. We calculated the proportion of patients with massive IVH and the proportion of patients that are eligible for fibrinolysis on basis of clinical and CT-scan characteristics and assessed neurological outcome in a treatment strategy without fibrinolysis. Poor neurological condition was defined as World Federation of Neurological Surgeons scale 4 and 5, poor outcome as death or dependence 3 months after SAH. Of the 573 patients admitted with aneurysmal SAH, 59 (10%; 95% confidence interval CI 8–13%) had massive IVH, of which 55 were in poor clinical condition. For these 55 patients, the case-fatality rate was 78% (95% CI 66–88%) and the proportion with poor outcome 91% (95% CI 81–97%). Of the 55 patients, 31 (56%, and 5% of all patients SAH within the study period) fulfilled our eligibility criteria and were considered suitable for intraventricular fibrinolysis. At 3 months, 30 of these 31 eligible patients (97%; 95% CI 85–100%) had a poor outcome. Massive IVH occurs in 10% of patients with aneurysmal SAH. Half of these patients may benefit from intraventricular fibrinolysis. Without fibrinolysis outcome is almost invariably poor in these patients.     

WFNS分级Ⅳ~Ⅴ级颅内动脉瘤的血管内栓塞治疗预后影响因素分析

目的 探讨WFNS分级Ⅳ~Ⅴ级颅内动脉瘤的血管内栓塞治疗预后影响因素。方法 回顾性分析2015年1月到2019年8月血管内栓塞治疗的102例WFNS分级Ⅳ~Ⅴ级颅内动脉瘤的临床资料。随访至少6个月,采用改良Rankin量表（mRS）评分评估预后,其中0~2分为预后良好,3~6分为预后不良。结果 102例中,预后良好47例（46.1%）,预后不良55例（53.9%）;死亡40例。多因素logistic回归分析结果显示,入院WFNS分级Ⅴ级、术前脑疝、载瘤动脉痉挛是预后不良的独立风险因素（P<0.05）。结论 对于WFNS分级Ⅳ~Ⅴ级颅内动脉瘤,预后影响因素很多,早期治疗有助于改善病人预后。     

双侧椎动脉/基底动脉重度狭窄或闭塞的急性后循环梗死患者临床特点及预后分析

目的总结分析双侧椎动脉/基底动脉重度狭窄或闭塞的急性后循环梗死患者的临床特点和预后。方法收集急性后循环梗死且伴有双侧椎动脉/基底动脉重度狭窄或闭塞的住院患者资料,分析其血管危险因素、起始症状、治疗及病情演变情况,并随访90 d预后,以mRS2分定义为预后不良。结果共纳入28例患者,最常见的起始症状为孤立性头晕或眩晕(57.1%)。53.6%的患者出现病情加重,加重≥2次9例(32.1%),预后不良15例(53.6%)。预后不良组高血压3级、病情加重、加重≥2次比例明显高于预后良好组(P0.05)。病情加重≥2次组高血压3级、高脂血症、孤立性头晕或眩晕起病、预后不良和死亡比例明显高于加重2次组(P0.05)。基底动脉不显影组死亡率(75%)明显高于显影组(4.2%)(P0.05)。结论双侧椎动脉/基底动脉重度狭窄或闭塞的急性后循环梗死患者多以孤立性头晕或眩晕起病;孤立性头晕或眩晕起病和高血压3级患者病情容易加重;病情反复加重患者预后不良比例高,死亡率高;基底动脉不显影患者死亡率高。     

Improved outcome after atherosclerotic stroke in male smoker

BACKGROUND: Smoking is a well-known risk factor for ischaemic stroke or transient ischaemic attack. Paradoxically, smokers have been reported to have better prognosis after myocardial infarction when compared to nonsmokers. This study examined the independent effect of smoking status on long-term prognosis after ischaemic stroke in male patients. METHODS: A total 476 male patients with acute cerebral infarction within the middle cerebral artery territory were reviewed. Baseline characteristics and long-term prognosis were compared among smokers, ex-smokers, and nonsmokers. RESULTS: Although the baseline severity of stroke did not differ among the groups, poor long-term outcome (Barthel index<60 or modified Rankin score>3) at 6 months after ischaemic stroke was more frequently observed in nonsmokers than in smokers (P=0.013); the outcome for ex-smokers was intermediate. After adjustment for age and other variables, current smoking was negatively correlated to poor long-term outcome (odds ratio, 0.286; 95% confidence interval, 0.119-0.686; P=0.005). On subgroup analysis, the impact of smoking on stroke prognosis was significant only in younger patients (<65 years of age) and those with atherosclerotic stroke. CONCLUSIONS: There was a strong independent correlation between smoking status and long-term outcome in patients with ischaemic stroke. Further studies about the impact of smoking habit on stroke outcome depending on the characteristics of patients (ie. age and stroke subtype) are needed.     

Signet-ring cell carcinoma of the colon with leptomeningeal involvement

Primary signet-ring cell carcinoma of the colon is rare. Most patients with this type of cancer have a poor prognosis. We describe a patient with signet-ring cell carcinoma of the colon, for whom leptomeningeal metastasis presented a variety of clinical symptoms.