Brain disorders (Amsterdam, Netherlands)最新文献

Objectives

The location of the brain tumor in the frontal or temporal lobes has been reported as a predictor for seizure occurrence in patients with a brain tumor but the predictive value of other locations is uncertain. We aimed to ascertain the pooled prevalence of pre-operative seizures in relation to the brain tumor locations, by employing a systematic review and meta-analysis.

Methods

The search was conducted up to 1st May 2023, in Pubmed, Embase, and Web of Science and references were exported and managed using EndNote 20. Articles were included if they reported a prevalence or incidence of the seizure at the tumor location. MetaXL by Epigear was used to generate the meta-analysis.

Results

The pooled prevalence for preoperative seizures in gliomas was 51–63% in most locations (frontal, temporal, parietal, and insula) but lowest in the occipital lobe (28%). Subgroup analysis on low-grade gliomas showed a high pre-operative seizure prevalence in all locations: frontal lobe [75% (95% CI: 68–81%)], temporal lobe [73% (95% CI: 63–82%)], parietal lobe [79% (95% CI: 57–82%)], occipital lobe [46% (95% CI: 0–95%)], and insular [76% (95% CI: 65–77%)]. In the astrocytoma and meningioma subanalysis, the pooled prevalence is ≥ 40% in most cortical location. In brain metastases, the pooled prevalence was similar in most locations (18–39%), lowest in the temporal lobe.

Conclusion

Tumor location is not the main influencing factor for pre-operative seizure independent from tumor type. Extensive seizure screening should be considered irrespective of tumor location, especially in those with low-grade gliomas.

Neuroleptic Malignant Syndrome (NMS) and Serotonin Syndrome (SS) are life-threatening conditions caused by adverse reactions to neuroleptics and antidepressant medications. The overlap of both syndromes is rare but has been described previously. Differentiating between both entities based on the clinical presentation can be difficult. We present a case of a young male patient with history of schizophrenia, admitted to the Medical Intensive Care Unit (MICU) for septic shock, who developed NMS and SS with oculomotor dysfunction following the abrupt cessation of clozapine while still being on an antidepressant.

Catamenial epilepsy is a condition marked by an increased occurrence of seizures that coincide with the menstrual cycle in women. Our study explored the role of neurotransmitter/neuromodulator agmatine in progesterone withdrawal induced increased seizure susceptibility that is one of the causes of catamenial epilepsy. Additionally, it investigates potential interactions between agmatine and the central neurosteroid system. The experiments involved the use of female Sprague-Dawley rats. To mime the seizure susceptibility as seen in catamenial epilepsy, rats were treated with PMSG and b-HCG which cause a higher release of progesterone and later injected with Finasteride on the 11th day of post-human chorionic gonadotropin administration to cause a sudden drop of progesterone. On the 12th day, seizure susceptibility was assessed through a low-dose (35 mg/kg) pentylenetetrazol (PTZ) injection. The results revealed that agmatine treatment showed a protective effect against seizures. Furthermore, this study explored the impact of neurosteroids such as allopregnanolone or progesterone (a precursor of allopregnanolone) and neurosteroidogenic drugs like FGIN 1–27 or metyrapone (an 11β-hydroxylase inhibitor) on the anticonvulsant effect of agmatine. The findings indicated that prior administration of these substances significantly enhanced the anticonvulsant effect of agmatine. In contrast, inhibiting neurosteroid production with drugs like trilostane (a 3β-hydroxysteroid dehydrogenase inhibitor) or indomethacin (a 3α-hydroxysteroid dehydrogenase inhibitor) completely nullified agmatine's effect. Seizures were associated with elevated progesterone levels and reduced allopregnanolone levels, which were normalized by agmatine treatment. Notably, agmatine exhibited seizure protection even in ovariectomized rats, affirming the involvement of neurosteroids in its anti-seizure effects in progesterone withdrawal increased seizure susceptibility. In conclusion, these findings emphasize central neurosteroid involvement in agmatine's pharmacological effects against seizure susceptibility in progesterone withdrawal in female rats.

Immune-mediated peripheral neuropathies are heterogeneous group of disorders due to the present of autoantibodies against peripheral nerve molecules located in node of Ranvier such as gangliosides and cell adhesion proteins or myelin components of peripheral nerves. Although the exact aetiology and pathophysiological mechanisms involved are not fully understood, both humoral and cellular immunity are likely playing a role in their pathogenesis. A proportion of patients present with clinical phenotype of rapidly progressive neuropathy refractory to conventional therapies but are lacking in identifiable or detectable antibodies. This makes diagnosis and treatment decision challenging.

We illustrate a patient with rapidly progressive seronegative immune-mediated neuropathy resembling autoimmune nodopathy (AN) associated with atypical features (prominent ataxia) and cranial involvement (facial and oropharyngeal weakness, dysgeusia), refractory to conventional therapies (IV immunoglobulin and corticosteroids) but responded to ‘remove and suppress’ treatment regime using therapeutic plasma exchange (TPE), followed by long-term B-cell suppressive therapy.

Introduction

Bruxism is a repetitive masticatory muscle activity that may cause substantial morbidity and reduce the quality of life in children with profound intellectual and multiple disabilities. Assessment methods most commonly used were caregiver reporting and dental examination, This systematic review with meta-analysis aims to determine the prevalence of bruxism in children with profound intellectual and multiple disabilities and to describe the currently used assessment methods for bruxism in this population.

Methods

We conducted a systematic review and meta-analysis using a multi-component search strategy. We used a random effects model to calculate the prevalence and 95 % confidence intervals for each study, for all studies combined, and specifically for Rett syndrome (RS), cerebral palsy (CP), Down syndrome (DS), and “other disorders (primarily Angelman syndrome and Prader–Willi syndrome).”

Results

The prevalence for the entire group based on a random effects model was found to be 49 % (95 %CI 41–57 %) with high heterogeneity (I² = 93 %, p < 0.01), for RS 74 % (95 %CI 53–88 %, I² = 84 %, p < 0.01), CP 48 % (95 %CI 38–57 %, I² = 86 %, p < 0.01), DS 40 % (95 %CI 33–47 %, I² = 60 %, p < 0.01) and “other disorders” 40 % (95 %CI 18–67 %, I² = 98 %, p < 0.01). The group prevalences were not equal, indicating a significant difference (P-value = 0.03), with a notably higher likelihood of RS.

Conclusion

We observed a five-fold increased likelihood of bruxism in children with profound intellectual and multiple disabilities. The disorder with the highest prevalence was Rett syndrome, with a seven-fold increased likelihood of bruxism. The increased likelihood of bruxism in this vulnerable group of children demands clinicians pay heed to this substantial morbidity.

AD is the main contributor to dementia and one of the largest healthcare concerns of the twenty-first century. The principal components of plaques and tangles, respectively, amyloid-β (Aβ) and tau, have made molecular pathogenetic processes accessible, little is known about the disease's etiology and there are no proven treatments. Minerals known as trace elements or trace metals in very small amounts were found in living tissues. Although some metallic ions, such as iron and copper, are involved in oxidation–reduction processes in energy metabolism, trace elements often function as enzyme system catalysts. The major pathological markers of AD are Aβ plaques (_40–42) & NF-tangles are associated with this disease. By examining various clinical & preclinical studies have proven that Al causes memory impairment as well as oxidative stress that results in mitochondrial dysfunction via the nucleus and mitochondria dysfunction (complex-I, II, IV), which leads to a mechanistic understanding of aging and the etiology of neurodegenerative illnesses. Multi-targeted pharmaceutical evidence-based therapy may need to be combined with non-pharmacological approaches and/or lifestyle modifications to stop the pandemic of neurological disease in the elderly. Other markers like aging, illnesses, and apoptosis have a greater focus in the field of research on NDs in the future.

Background: Acute hepatic porphyrias are heme metabolism disorders presenting with a broad clinical spectrum, including neurovisceral manifestations. Peripheral neuropathy is one of the most frequent complications in porphyrias and can correspond about 40 % of the neurovisceral manifestations, and it is mainly attributed to the ALA and PBG accumulation neurotoxicity. The hallmark of an acute porphyric attack is an acute motor axonal neuropathy accompanied by autonomic dysfunction. Sensory impairment is infrequent, and when present, it is classically described as the stocking-glove pattern or, more rarely, a “bathing suit” proximal distribution pattern. Objective: To report a variegate porphyria case in a young patient with unusual sensory manifestations. Methods: A 27-year-old man, previously healthy, present in context of possible arbovirus infeccion with severe abdominal pain, followed by dysautonomia due to orthostatic hypotension, change in urine color and, the emergence of sensory impairment in the trunk and proximal region of the four limbs about twenty days from onset. Dysestesia was severe and marked. Results: Positive clinical features and a urinary exposure test to sunlight corroborate in request genetic testing for acute hepatic porphyrias, with a positive mutation of the PPOX gene in heterozygosis. Discussion: Clinical manifestations with initial presentation of severe dysautonomia followed by sensory changes demarcate neurovisceral involvement of variegate porphyria, adopting an "old-bathing-suit costume" sensory pattern throughout its evolution.

Polyglutamine (polyQ) diseases are devastating neurological disorders that cannot be effectively treated. Repeat-associated non-AUG (RAN) translation has been documented in transcripts in polyQ diseases. RAN products include proteins with polyleucine (polyL) tracts. Similar to polyQ, polyL tends to aggregate, which is toxic to cells and mice. Irradiation with a free electron laser (FEL) tuned at mid-infrared wavelengths can dissociate polyQ aggregates in cultured cells. However, whether FEL dissociates the polyL is unclear. It is also unclear whether brain dysfunction caused by polyL aggregates in mice can be ameliorated by FEL irradiated polyL. Here, we show that FEL at approximately 6 μm can destroy polyL aggregates, as evidenced by scanning electron microscopy, atomic force microscopy, and dot blot analyses. Although polyL aggregates induced low viability and aberrant morphology of cultured astrocytes, FEL irradiated polyL exhibited mild defects. Likewise, the toxicity of polyL-containing microglia in vitro was ameliorated by FEL irradiation. In vivo, mice administered polyL aggregates in the cerebellum induced loss of Purkinje cells, which was ameliorated when FEL irradiated polyL was injected. These results justify the clearing of aggregates by approaches using molecular chaperones, laser irradiation, and ultrasound as a general therapeutic strategy to correct brain dysfunction by the RAN products.

The current study was aimed to explore the effect of light and traffic noise pollution exposure on behavioral and neurochemical deficits in adult male Wistar rats. We hypothesized that exposure to these pollutants alone or in combination may cause behavioral deficits in rats. Animals were allocated into different groups and were exposed to either varying light cycles (150 lx-5 lx, 14:10 h) or traffic noise with sound level 100 dBA for 6 h/day or both for 28 days. Concurrently, Curcumin (100 mg/kg; per oral; 28 days) was administered in varying light cycles and noise exposed animal groups to study the preventive effect. After the exposure time, the Morris water maze test, elevated plus maze test, novel object recognition test, locomotor activity test, and forced swimming test were conducted. The neurobehavioral results suggested that only combined exposure of light and noise pollution markedly impaired the learning as well as spatial and reference memory in rats. Furthermore, we found significant anxiety (less no. of open arm entries in EPM) and depressive-like behavior (increased immobility time) in light or/and traffic noise pollution exposed group animals in comparison to normal control groups. Curcumin treatment significantly prevented the behavioral anomalies in combined light and noise exposed rats. Furthermore, our results revealed augmentation of acetylcholinesterase, oxidative stress markers and interleukin (IL)-1β in the hippocampal region of noise and light-exposed groups which were markedly ameliorated by curcumin. In conclusion, our results suggest that curcumin significantly modulates light and noise pollution exposed behavioral and neurochemical anomalies in rats.

Background and Purpose

No consensus exists on the ideal duration of rehabilitation in patients with intracerebral hemorrhage (ICH). The aim of this study is to identify demographic and clinical characteristics associated with rehabilitation duration (RD) in patients with ICH during hospitalization.

Methods

This retrospective study followed consecutive patients admitted to a tertiary care center between 2016 and 2019 with primary diagnosis of ICH. The primary outcome, RD was calculated by adding the total number of therapy (speech, occupational, and physical) minutes received during admission, divided by the hospital length of stay. Variables abstracted included demographic and clinical characteristics (ICH score, neurosurgical procedure). Multiple linear regression models were used to measure adjusted association between select predictors and mean RD.

Results

A total of 316 ICH patients (62.5 ± 15.9 years, 49 % White) were included in the final analysis. Compared to Whites, other racial minorities (β= -9.017, p = 0.002) received rehabilitation therapy for a significantly shorter duration in the adjusted model. Age was significantly associated with having higher RD (β=0.320, p < 0.001). Patients with lobar ICH (β= -7.486, p = 0.014) had significantly shorter RD compared to deep. ICH score was significantly associated with having a lower RD (β= -8.624, p < 0.001).

Conclusion

Age, race, ICH score, and location were significantly associated with RD. Non-White patients had significantly shorter RD, indicating a potential racial disparity in the rehabilitation of patients with ICH.