Arquivos de neuro-psiquiatria最新文献

Status epilepticus (SE) is the most severe presentation of epilepsy. Currently, SE is defined according to 2 sequential time frames: time 1, after which it is unlikely that the seizure will resolve spontaneously, therefore requiring the initiation of therapy; and time 2, when long-term consequences become more likely. For convulsive SE, these time frames are well defined: 5 minutes for time 1 and 30 minutes for time 2. "Time is brain" in the treatment of SE, as delays in diagnosis and treatment are associated with worse outcomes. After clinical stabilization, the first step is the administration of intravenous (IV) benzodiazepines. Rapid initiation of treatment and use of appropriate dosing are more important than the selection of a specific benzodiazepine. Following this, treatment continues with the use of an IV antiseizure medication (ASM). In Brazil, the recommended options available are phenytoin and levetiracetam. Status epilepticus is considered refractory to treatment if seizures persist after the administration of benzodiazepines and IV ASM. The cornerstone of this stage is the induction of therapeutic coma using IV anesthetic drugs (IVADs), although evidence is limited regarding the choice among midazolam, propofol, or barbiturates. Super-refractory SE is defined when seizures persist despite continuous infusion of IVADs or recur after these drugs are tapered. There is very limited data regarding the treatment of super-refractory SE. In the absence of randomized controlled trials, treatment should be guided by the physician's experience, clinical judgment, and established therapeutic options from previous reports.

Erdheim-Chester disease (ECD) is a rare histiocytic disorder that poses diagnostic and therapeutic challenges. Neurological manifestations are characterized by involvement of the meninges, brainstem, and/or cerebellum, and the differential diagnoses include sarcoidosis, IgG4 related disorders, autoimmune encephalitis, and high-risk syndromes. While present in a significant proportion of cases, neurological involvement is a predictor of mortality and may be the sole manifestation of the disease. In this paper, we discuss recent updates in histiocytic disorders and complementary diagnostic approaches, including positron-emission tomography-computed tomography (PET-CT), as guidance for biopsy in patients with neurological symptoms. Additionally, we explore how clinicians can interpret biopsy findings in conjunction with immunohistochemistry to guide targeted therapies, such as vemurafenib, for BRAF V600E mutation.

Background:  The movement disorder known as hemifacial spasm is characterized by involuntary contractions of the muscles that are innervated by the facial nerve. The treatment of choice for this condition is botulinum toxin injections.

Objective:  To analyze the botulinum toxin dosage in patients undergoing treatment for hemifacial spasm during a 14-year period.

Methods:  A retrospective study of medical records from patients treated at the Neurology Service of Hospital Universitário Onofre Lopes, Universidade Federal do Rio Grande do Norte, from 2010 to 2024, was performed.

Results:  A total of 151 patients met the inclusion criteria. The dose of botulinum toxin revealed a statistically significant increase during the first 3.46 years of follow-up. In the long-term, a trend toward dose stabilization was identified. The median latency for the onset of effect was 4 days, while the median duration of effect was 3 months. All side effects were temporary, with the most common being hemifacial weakness (17.9%) and palpebral ptosis (3.3%). Most patients presented primary hemifacial spasm (88.1%), with a neurovascular conflict identified in 24.1% of cases.

Conclusion:  The increase in botulinum toxin dosage during the first years may be explained by dosage adjustment to control hemifacial spasm with the lowest possible doses. A prolonged interval between applications may also be associated with this increase. Dose stabilization tends to be achieved over time, indicating disease control.

Alzheimer's disease (AD), diabetic cognitive impairment (DCI), and vascular dementia (VD) are considered the most common causes of severe cognitive impairment in clinical practice. Numerous factors can influence their progression, and many studies have recently revealed that metabolic disorders play crucial roles in the progression of cognitive impairment. Mounting evidence indicate that the regulation of lipid metabolism is a major factor in maintaining brain homeostasis. Generally, abnormalities in lipid metabolism can affect amyloid-beta (Aβ) deposition, tau hyperphosphorylation, and insulin resistance through lipid metabolic signaling cascades; affect the neuronal membrane structure, neurotransmitter synthesis and release; and promote synapse growth, which can impact neural signal transmission and exacerbate disease progression in individuals with cognitive impairment, including AD, DCI, and VD. Moreover, apolipoprotein E (APOE), a key protein in lipid transport, is involved in the occurrence and development of the aforementioned diseases by regulating lipid metabolism. The present article mainly discusses how lipid metabolic disorders in the brain microenvironment are involved in regulating the progression of cognitive impairment, and it explores the regulatory effects of targeting the key lipid transport protein APOE in the context of the role of lipid metabolism in the common pathogenesis of three diseases-Aβ deposition, tau hyperphosphorylation, and insulin resistance-which will help elucidate the potential of targeting lipid metabolism for the treatment of cognitive impairment.

Background:  Temporal lobe epilepsy (TLE) predisposes individuals to cognitive difficulties and psychosocial consequences. Evaluating its impact on quality of life (QOL) is essential for patient care.

Objective:  To identify factors influencing QOL in low-income patients with TLE.

Methods:  An institution-based cross-sectional study was conducted on 40 patients with TLE during neurological consultations at a day clinic in Agadir, Morocco. The Quality of Life in Epilepsy Inventory-31 (QOLIE-31) was used to measure QOL. Multivariate linear regression analysis was performed to assess the associations between QOL and demographic, clinical, psychiatric, social, and cognitive variables. Results were considered statistically significant at a p-value < 0.05.

Results:  The mean overall QOL score was 48.14 ± 22.02. Among the seven scales of the QOLIE-31, the Seizure Worry scale had the lowest mean score. Cognitive function, social support, and self-esteem were positively associated with QOL. In contrast, memory complaints, seizure duration, seizure frequency, anxiety, and depression were negatively associated with QOL.

Conclusion:  While current interventions primarily target seizure control, our findings emphasize the need for holistic approaches that address both cognitive and psychosocial challenges to optimize QOL.

Background:  Parkinson's disease (PD) is a degenerative, progressive, chronic disease that mainly affects the central nervous system, caused by dopamine deficiency. One of the ways to evaluate the central nervous system is with auditory evoked potentials (AEP).

Objective:  To characterize the audiometric responses, and the auditory brainstem response (ABR), and cortical auditory evoked potentials (CAEP) in individuals with PD.

Methods:  Thirty-two patients aged between 40 and 81 of both sexes were assessed, 16 with PD (study group [SG]) and 16 without PD (control group [CG]) matched for sex and age. The subjects were assessed using pure tone audiometry, ABR with click stimuli, and CAEP using the oddball paradigm with tone burst and speech stimuli. The results were compared between the groups using a repeated measures analysis of variance (ANOVA) test.

Results:  In pure-tone audiometry, significantly higher hearing thresholds were found in the SG at 6 and 8 kHz. For the ABR, no differences were observed between groups. The CAEP analysis did not find statistical differences in the latencies between the groups, however, the SG presented smaller amplitudes of P1-N1, P2-N2, and N2-P3 than the CG.

Conclusion:  The results of this study showed a significantly higher threshold in higher frequencies in PD. Although no differences were observed at the brainstem level, the decrease in amplitude of all components in patients with PD in the CAEP suggests a deficit in both automatic and attentional cortical processing of acoustic stimuli.