Clinical characteristics and course of spinal cord involvement in Behçet's disease

Parenchymal neurological involvement in Behçet's disease (p-NBD) usually presents with a brainstem syndrome; occasionally spinal cord may also be involved. Files of patients with Behçet's disease and spinal cord involvement were reviewed retrospectively, in comparison with other types of p-NBD. Amongst 216 patients with p-NBD, 24 had spinal cord involvement (11%). Most commonly patients presented with sensory-motor symptoms, sphincter and/or sexual dysfunction evolving over days. Four of 10 patients showed single or multiple cervical and/or dorsal lesions on spinal MRI's and one showed dorsal atrophy. Although the clinical picture was variable, it tended to be severe; seven cases had primary progressive course, 11 cases had a secondary progressive course after initial attack(s), four had attacks with severe residual sequela and two had improvement after attacks. After a median follow-up period of 67 months, eight were independent and 14 were dead or dependent, whereas amongst the remaining patients with p-NBD, 113 patients were independent and 56 patients were dead or dependent ( P  < 0.05). Our study suggests that spinal cord involvement has even worse prognosis compared with other types of p-NBD. Therefore, recognition of spinal cord involvement in Behçet's patients should prompt early vigorous treatment.     

Neurolymphomatosis,a rare manifestation of peripheral nerve involvement in lymphomas: Suggestive features and diagnostic challenges

Neurolymphomatosis, the infiltration of the peripheral nervous system from lymphoid cells, represents an uncommon manifestation of lymphomas. We describe the challenging diagnostic work‐up in a patient with neurolymphomatosis. A 58‐year‐old woman with previous breast diffuse large B‐cell lymphoma treated with chemo‐ and radiation‐therapy, presented with dysesthesias, neuropathic pain at left abdomen and thigh, and weakness at left lower limb 9 years after disease remission. Neurophysiology revealed left T10‐L4 radiculo‐plexopathy with no abnormalities at cerebrospinal fluid (CSF), nerve ultrasound, and ¹⁸fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT). MR‐neurography disclosed left rectus abdominis muscle atrophy, neurogenic edema, and denervation. Radiation‐induced damage, paraneoplastic, infectious radiculo‐plexopathies, and atypical chronic inflammatory demyelinating polyradiculoneuropathy were ruled out. Neurolymphomatosis was suspected, and the patient treated with rituximab with improvement. Despite treatment, the radiculo‐plexopathy eventually extended to the right side and sacral roots. Later in the disease course, sural nerve biopsy confirmed the diagnosis. Maintenance therapy was continued, until cutaneous localizations occurred, requiring salvage therapy and autologous stem cell transplant. Although rare, neurolymphomatosis should be considered in all patients with lymphomas and unexplained peripheral nervous system involvement. Hematological, CSF, and neuroimaging findings may be unremarkable, and a high index of suspicion required in order to achieve the diagnosis.     

Meningeal Ewing Sarcoma/Peripheral PNET: Clinicopathological,Immunohistochemical and FISH study of four cases

Meningeal Ewing Sarcoma (ES)/peripheral primitive neuroectodermal tumor (pPNET) is a rare diagnostically challenging small round cell tumor in the CNS. This study investigates the clinical pathological features of four cases of this tumor from archives of 6 years in our hospital. Patients were within the median age of 21.5 years and male to female ratio was 1:1. The tumors distributed at the supra‐tentorial location, posterior fossa and lumbar vertebral canal, usually presenting as the dura‐sited nodule or having close connection with the meninges within the cranium or vertebral canal. Histopathologically, small round undifferentiated tumor cells with hypercellularities, scant cytoplasm and inconspicuous nucleoli were observed, although some components such as atypical larger vesicular nuclei, prominent nucleoli of tumor cells, necrotic foci and mesenchymal collagen proliferation forming the lobular structure, were also appreciated. Immunohistochemally, tumor cells displayed membranous positivity of CD99 (4/4), nuclear positivity of FLI‐1 (4/4) and NKX2.2 (4/4), negativity of EMA, GFAP and synaptophysin expression. The histochemical PAS staining showed weak positivity in one case. Fluorescence in situ hybridization (FISH) test using EWSR1 (22q12) dual color break apart rearrangement probe showed positive results in two cases. Results suggest that using a panel of immunohistochemical markers, including NKX2.2, CD99, FLI‐1, EMA, GFAP and synaptophysin, combined with the supplementary EWSR1 FISH test, helps to define the diagnosis of meningeal ES/pPNET of CNS.     

Incidence and etiology of ischemic stroke in Persian young adults

BACKGROUND: Stroke in young adults causes morbidity in this socioeconomically active age group. The etiologic frequency of ischemic stroke in young adults differs around the world. METHODS: The study population consisted of 314,000 'young adult' residents in Southern Khorasan province in Iran. All patients with stroke are routinely admitted to the Valie Asr tertiary care hospital. Data on patients demographics, clinical presentation and investigations of consecutive patients aged 15-45 years with ischemic stroke are registered in Southern Khorasan stroke data bank for the period March 2000 to March 2005. All patients underwent a standard battery of diagnostic investigations by a stroke neurologist. The etiologic classification of stroke in the patients was made based on the trial of ORG 10172 in acute stroke treatment (TOAST) criteria. RESULTS: One hundred and twenty-four patients (60 female, 64 male) were prospectively investigated during a 5-year period. The incidence of ischemic stroke in young adults was eight cases per 100,000 people per year. Cardioembolic mechanism comprised 54% of stroke etiology in young adults. Rheumatic valvular disease was present in 32% of the patients and caused 2.5 preventable stroke cases per 100,000 'young adults' per year. CONCLUSION: Rheumatic valvular disease is the most common cause and a preventable etiology of stroke in Persian young adults.     

Incidence of social phobia and identification of its risk indicators: a model for prevention

Objective: This study seeks to examine the incidence of social phobia in the general population and to establish a number of risk indicators. Method: Data were derived from the Netherlands Mental Health Survey and Incidence Study (NEMESIS) which is a population based prospective study (n = 7076). A sample of adults aged 18–64 years (n = 5618) were re‐interviewed 1 year later using Composite International Diagnostic Interview (CIDI). Results: The 12‐month incidence of DSM‐III‐R social phobia was 1.0%. Low education, low mastery, low self‐esteem, emotional neglect in childhood and ongoing difficulties were found to be risk indicators. After including other mental disorders as risk indicators in the model, the incidence was found to be more common among those with low mastery, major depression, subthreshold social phobia, emotional neglect, negative life events, and low education. Conclusion: The incidence of social phobia can be predicted relatively well with psychosocial variables and comorbidity.     

Lifestyle factors and the course of depression in older adults: A NESDO study

Objectives

To investigate whether lifestyle indicators including physical exercise, sleep duration, alcohol use, body mass index, smoking status, and a composite lifestyle index are associated with the depression course in older adults.

Methods

Data of 283 older adults were used from the Netherlands Study of Depression in Older Persons. Depressive disorders at baseline were assessed with the Composite International Diagnostic Interview. The depression course at 2‐year follow‐up was assessed with the Inventory of Depressive Symptoms (IDS, score 0–84) every 6 months; physical exercise with the International Physical Activity Questionnaire; alcohol use with the Alcohol Use Disorders Identification Test; body mass index by anthropometry; and sleep duration and smoking status by interview questions. A composite lifestyle index was calculated by summing scores assigned to each lifestyle factor, with a higher score indicating healthier behavior.

Results

Of all participants, 61.1% had chronic depression (all IDS scores 14–84), 20.1% had intermittent depression (1 IDS score ≤ 14), and 18.7% remitted depression (last 2 IDS scores ≤14). None of the investigated lifestyle indicators, nor the composite lifestyle index was associated with depression course, after adjustment for covariates.

Conclusions

Lifestyle factors do not predict the course of depression at 2‐year follow‐up in older adults.     

Cognitive, emotional, physical and social effects of growth hormone treatment in adults with Prader–Willi syndrome

Background Prader–Willi syndrome (PWS) is a multisystem genetic disorder characterized by short stature, muscular hypotonia, hyperphagia, obesity, maladaptive behaviour, hypogonadism and partial growth hormone (GH) deficiency (GHD). Severe GHD of other aetiologies has been shown to affect mood and quality of life negatively, and there are reports of improvements with GH replacement. We have studied cognitive, emotional, physical and social parameters in PWS adults at baseline, during and after GH treatment. Patients and methods Nineteen patients, 9 females and 10 males, median age 25 years, mean BMI 35 kg/m² participated in this study. Approximately half of the group had GHD. All patients fulfilled the clinical criteria for PWS and 13 had a positive genotype. The patients were randomized to 6 months of treatment with either GH [1.6 IU/day (0.53 mg/day)] or placebo, followed by 12 months of active GH treatment. Treatment was then stopped, and the patients were followed for an additional period of 6 months. A test battery for general cognitive evaluation and a computer-based measurement of reaction time, motor speed and fluency were employed at baseline, after 6 months and at the end of GH treatment. At the same time intervals, a self-evaluation questionnaire was answered at the end of each test session. Other questionnaires reflecting the patients’ cognitive, emotional, physical and social status were answered by relatives/caretakers at baseline and at 3 and 6 months following cessation of GH treatment. Results Baseline cognitive level was estimated to be moderately to mildly impaired; IQ range was 40–90. The results from some of the cognitive and the motor performance tests improved significantly after 6 and 18 months of GH treatment. According to the questionnaires, both the patients and the relatives/caretakers evaluated physical status rather negatively at baseline, but still, impairments in both physical and social status and overall functioning were observed when GH treatment was discontinued. The self-evaluation did not change in any aspect during GH treatment. Conclusions In this pilot study of an adult PWS cohort, we were able to document beneficial effects in mental speed and flexibility and in motor performance during GH treatment. Impairment was seen in physical and social status as well as overall functioning, when GH treatment stopped. Studies of larger cohorts are needed to further elucidate the role of GH treatment in this group of patients.     

成人烟雾病治疗的相关研究进展

烟雾病（moyamoya disease,MMD）是一种慢性进行性脑血管疾病,涉及颈内动脉末端和/或其近端分支狭窄闭塞,导致侧支血管网形成。这些变化引起脑实质慢性缺血,随后发生严重的脑血管意外。成人MMD患者在未经治疗的情况下会逐渐累及认知功能,且病死率是儿童MMD患者的2倍。由于MMD发展病因的复杂性和后果的严重性,该病的治疗尤为棘手且迫切。外科血管重建术作为MMD治疗的基石,主要分为直接血管重建术、间接血管重建术和联合血管重建术三类。考虑到间接血管重建术不能降低围手术期脑卒中的发生率,直接血管重建术通常是缺血型MMD患者治疗的首选术式。若为了预防出血型MMD患者再发出血,选择直接血管重建术或联合血管重建术能够更容易建立侧支循环,促进血运重建,达到治疗的目的。当患者术后出现新发的缺血脑卒中,则优先考虑间接血管重建术。对于血流动力学不稳定的成人MMD患者,直接血管重建术或联合血管重建术则是首要选择。由于目前无逆转MMD病情进展的特效药,内科治疗仅局限于对症治疗和围手术期的管理。临床上通常使用阿司匹林抗血小板聚集,促红细胞生成素和他汀类药物等促进侧支血管发育和地塞米松促进新生血管的形成等。远隔缺血适应训练改善MMD患者的后遗症的疗效已经得到了业界的肯定。发病机制研究的深入为MMD的诊疗手段提供更多的可能性。甲硫氨酸循环异常参与了MMD的发病,提示甲硫氨酸循环相关风险评分对烟雾病风险具有良好的预测能力。此外,内皮祖细胞移植可能成为临床上治疗MMD的新策略。 [国际神经病学神经外科学杂志, 2023, 50(3): 78-83]     

Impact of a brief behavioral treatment for insomnia (BBTi) on metacognition in older adults

IntroductionBrief (≤4 sessions) behavioral treatment for insomnia (BBTi) improves insomnia symptoms in older adults. Findings for BBTi-related improvements in objective cognition are mixed, with our recent trial reporting no effects. Metacognition (appraisal of one's own performance) has not been examined. This study examined the effects of BBTi on metacognition in older adults with insomnia.MethodsOlder adults with insomnia [N = 62, Mage = 69.45 (SD = 7.71)] were randomized to 4-weeks of BBTi (n = 32; psychoeducation, sleep hygiene, stimulus control, sleep restriction, relaxation, review/maintenance) or self-monitoring control (SMC; n = 30; social conversations). Throughout the study (2 week baseline, 4 week treatment, 2 week post-treament, 2 week 3-month followup), participants completed daily paper/pencil cognitive tasks (measuring verbal memory, attention, processing speed and reasoning) and provided daily metacognition ratings of their performance in four areas: quality, satisfaction, compared to same age peers, compared to own ability. Two-week averages of metacognitive ratings were calculated for baseline, treatment-first half, treatment-second half, post-treatment, and 3-month follow-up. Multilevel Modeling examined treatment effects (BBTi/SMC) over time on metacognition, controlling for age and sex.ResultsA significant group by time interaction (p = 0.05) revealed consistent improvements over time in better metacognitive ratings relative to same age peers for BBTi. Specifically, baseline ratings [mean (M) = 51.21, standard error (SE) = 3.15] improved at first half of treatment (M = 56.65, SE = 3.15, p < 0.001), maintained improvement at second-half of treatment (p = 0.18), showed additional improvement at post-treatment (M = 60.79, SE = 3.15, p = 0.02), and maintained improvement at follow-up (M = 62.30, SE = 3.15; p = 0.02). SMC prompted inconsistent and smaller improvements between baseline (M = 53.24, SE = 3.29) and first-half of treatment (M = 56.62, SE = 3.28; p = 0.004), with additional improvement at second-half of treatment (M = 59.39, SE = 3.28; p = 0.02) that was maintained at post-treatment (p = 0.73) and returned to levels observed at first-half of treatment (M = 57.78, SE = 3.21; p = 0.55). Significant main effects of time (all ps < 0.001) for other metacognition variables (Quality, Satisfaction, Compared to own ability) indicated general improvements over time for both groups.DiscussionMetacognition generally improved over time regardless of treatment. BBTi selectively improved ratings of performance relative to same age peers. Repeated objective testing alone may improve metacognition in older adults with insomnia. Better understanding of metacognition and how to improve it has important implications for older adults as metacognitive complaints have been associated with mild cognitive impairment.     

Incidence, course, and predictors of apathy in Huntington's disease: a two-year prospective study

This study examined the incidence and course of apathy in subjects with Huntington's disease (HD). Our results showed that at follow-up 14% of the subjects free of apathy at baseline had developed apathy. In these subjects, a lower baseline Mini-Mental State Exam score predicted incidence of apathy. Of the 34 subjects with apathy at baseline, 14 subjects were no longer apathetic at follow-up. Twenty subjects had persistent apathy, with a low baseline Symbol Digits Modalities Test as the only predictor. These results showed that apathy in HD is most closely linked to global and executive cognitive performance.     

Perspectives on lithium treatment of bipolar disorder: action, efficacy, effect on suicidal behavior

Objective: To review studies of (A) whether lithium has a prophylactic action in bipolar disorder, (B) the efficacy of prophylactic lithium treatment in comparison with the efficacy of treatment with anticonvulsant drugs, and (C) the effect of lithium treatment on suicidal behavior.
Methods: Analysis of all relevant publications.
Results: (A) The claim that a prophylactic action of lithium has never been satisfactorily demonstrated is based on wrong assumptions, biased selection of references, and unjustified generalizations. (B) In typical bipolar disorder lithium is significantly more efficacious than carbamazepine; in atypical bipolar disorder there is a non-significant trend for carbamazepine to be better than lithium. Valproate has not been proven prophylactically efficacious in typical bipolar disorder; in atypical bipolar disorder it may have an effect, but it has not been compared with that of lithium. (C) A significant association has been found between prophylactic lithium treatment, on the one hand, and reduced mortality and suicidal behavior, on the other. No such association has been reported for prophylactic treatment with other mood stabilizers.
Conclusion: In bipolar disorder the choice of prophylactic drug must be based on a weighing of efficacy against tolerability, interactions, ease of management, use during pregnancy and lactation, and expense. Lithium should be the preferred prophylactic drug in patients with typical bipolar disorder and in patients who are at high risk of committing suicide, that is, patients with severe depressions or depressions combined with persistent suicidal ideas or with suicide attempts in the past.     

Incidence and lifetime risk of motor neuron disease in the United Kingdom: a population-based study

Background and purpose:  To estimate the incidence and lifetime risk of motor neuron disease (MND) in a population-based sample in the United Kingdom.
Methods:  We identified new cases of MND during the period 1990–2005 in the General Practice Research Database, which includes clinical information from more than 3 million Britons enrolled with selected general practitioners. Individuals with a first medical diagnosis of MND recorded in the database were considered incident cases of the disease. The positive predictive value of the computer-based diagnosis was estimated through review of a sample of medical records from potential MND cases.
Results:  In the period 1990–2005, 830 new cases of MND were identified. Age-standardized incidence of MND was 2.6 per 100 000 persons per year in women (95% CI: 2.3, 2.8) and 3.9 in men (95% CI: 3.6, 4.3). Incidence for both sexes peaked between 75 and 79 years. The rate of MND in men was 54% higher than in women (95% CI: 33%, 77%). The lifetime risk of MND, adjusting for competing causes of death, was 1 in 472 (2.1 per 1000) in women and 1 in 350 (2.9 per 1000) in men. No increase in MND incidence over time was apparent.
Conclusion:  In this population-based database, we found that MND incidence is higher in men than women, peaking in both sexes between 75 and 79 years.     

Hip fractures in people with idiopathic Parkinson's disease: Incidence and outcomes

The incidence of hip fracture and outcomes from hip surgery for people with Parkinson's disease (PD) are thought to be poorer than for people without PD. The aim of this audit of a prospective hip‐fracture database was to establish the incidence of, and outcomes from, hip fracture in people with and without PD living in North East England. The number of people with PD living in the study area was estimated using data from two previous prevalence studies in the same geographical area. Using data collected prospectively for the National Hip Fracture Database for Northumbria Healthcare National Health Service Foundation Trust in the UK, the annual incidence of hip fracture in people with and without PD was calculated. Type of fracture, time to surgery, time to discharge, and 30‐day outcomes from surgery were compared. Annual incidence of hip fracture was significantly higher in people with PD across all age bands. In those 60 years of age and over, it was 2,171 (95% confidence interval [CI]: 2,082–2,264) per 100,000 in people with PD and 551 (95% CI: 506–598) in people without PD. The experience of PD and non‐PD patients within hospital was remarkably similar. However, PD patients had poorer mobility before hip fracture, took longer to be discharged to the community, and were less mobile postsurgery. Specific guidelines for managing people with PD who sustain a hip fracture may help to improve awareness of the potential complications of the condition and improve outcomes. © 2012 Movement Disorder Society     

Incidence, types and characteristics of aggressive behaviour in treatment facilities for adults with mild intellectual disability and severe challenging behaviour

Background Inpatient aggression in treatment facilities for persons with intellectual disability (ID) can have aversive consequences, for co‐clients and staff, but also for the aggressors themselves. To manage and eventually prevent inpatient aggressive incidents, more knowledge about their types and characteristics is necessary. Method In four facilities, totalling 150 beds, specialized in the treatment of adults with mild ID or severe challenging behaviour, aggressive incidents were registered during 20 weeks using the Staff Observation Aggression Scale‐Revised. Characteristics of auto‐aggressive and outwardly directed incidents and differences in their incidence in male and female clients in these facilities were compared. Results During the observation period of 20 weeks, 639 aggressive incidents were documented. Most of these (71%) were outwardly directed, predominantly towards staff, while most of the remaining incidents were of an auto‐aggressive nature. Of the 185 clients present during the observation period, 44% were involved in outwardly directed incidents (range per client 1–34), and 12% in auto‐aggressive incidents (range per client 1–92). Auto‐aggressive and outwardly directed incidents differed regarding source of provocation, means used during the incident, consequences of the incident and measures taken to stop the incident. The proportion of men and women involved in each type of incident was comparable, as well as the majority of the characteristics of outwardly directed incidents caused by men and women. Conclusions Although approximately half of all clients were involved in aggressive incidents, a small minority of clients were responsible for the majority of incidents. Therefore, better management and prevention of aggressive incidents for only a small group of clients could result in a considerable overall reduction of aggressive incidents in treatment facilities. Comparability of aggressive behaviour in these facilities shown by men and women and differences in characteristics of auto‐aggressive and outwardly directed incidents are discussed.     

Incidence and predictors of seizures in patients with Alzheimer's disease

PURPOSE: To determine cumulative incidence and predictors of new-onset seizures in mild Alzheimer's disease (AD) with a cohort followed prospectively. Limited information is available on the incidence of seizures, and no reports exist of seizure predictors in AD patients. METHODS: Mild AD patients were prospectively followed at 6-month intervals to estimate incidence of unprovoked seizures, compare age-specific risk of unprovoked seizures with population norms, and identify characteristics at baseline (demographics, duration and severity of AD, physical and diagnostic test findings, and comorbid medical and psychiatric conditions) influencing unprovoked seizure risk. Review of study charts and medical records supplemented coded end-point data. RESULTS: The cumulative incidence of unprovoked seizures at 7 years was nearly 8%. In all age groups, risk was increased compared with a standard population, with an 87-fold increase in the youngest group (age 50-59 years) and more than a threefold increase in the oldest group (age 85+ years). In multivariate modeling, independent predictors of unprovoked seizures were younger age [relative risk (RR), 0.89 per year increase in age; 95% confidence interval (CI), 0.82-0.97], African-American ethnic background (RR, 7.35; 95% CI, 1.42-37.98), more-severe dementia (RR, 4.15; 95% CI, 1.06-16.27), and focal epileptiform findings on electroencephalogram (EEG) (RR, 73.36; 95% CI, 1.75-3075.25). CONCLUSIONS: Seizure incidence is increased in people starting with mild-to-moderate AD. Younger individuals, African Americans, and those with more-severe disease or focal epileptiform findings on EEG were more likely to have unprovoked seizures.