BMC Neurology最新文献

Background: 1,2-dichloroethane (DCE) induced toxic encephalopathy, a rare toxic disease of the central nervous system, is mainly reported in developing countries. Although clinicians have got some understanding about the clinical and neuroimaging features of 1,2-DCE-induced toxic encephalopathy, abnormality along the cortico-medullary junction on diffusion-weighted image (DWI) mimicking neuronal intranuclear inclusion disease (NIID) has not yet been described in this entity.

Case presentation: We reported a patient with 1,2-DCE-induced toxic encephalopathy who was admitted to our department due to a 7-day history of nausea, vomiting, and cognitive decline. Brain magnetic resonance imaging (MRI) showed symmetrical hyperintensities in bilateral subcortical white matte on T2-weghted and Fluid-attenuated inversion recovery (FLAIR) images. In addition, abnormal signal intensity could also be found in the cortico-medullary junction on DWI, mimicking NIID. After treated with glucocorticoid, dehydrating agents, neuroprotective agents, and hyperbaric oxygen, our patient received a partial recovery.

Conclusion: Our case highlights a special MRI finding-abnormalities along the cortico-medullary junction-that can be seen in 1,2-DCE-induced toxic encephalopathy. When confronted with patients with lesion located in the cortico-medullary junction and neuropsychiatric symptoms, our clinicians should not neglect the detailed inquiry of history of toxic exposure.

Background: This case report presents the case of a patient with P369S and R408Q variants in the MEFV gene who exhibited clinical features of Kikuchi disease and Mollaret meningitis. Furthermore, it discusses colchicine as a new potential treatment option for Kikuchi disease-associated meningitis.

Case presentation: A 41-year-old Japanese woman presented with fever and headache. She had nuchal rigidity and bilateral cervical lymphadenopathies. Her past medical history included multiple episodes of aseptic meningitis and cervical lymphadenopathy for more than twenty years. Lumbar puncture showed increased lymphocytes and IL-6 level and pathognomonic Mollaret cells. Excisional lymph node biopsy revealed histiocytic necrotizing lymphadenitis, confirming the diagnosis of Kikuchi disease. Subsequently, her recurrent Kikuchi disease and meningitis were successfully treated with colchicine. Furthermore, genetic analysis of the MEFV gene revealed heterozygous P369S/R408Q variants in exon 3.

Conclusion: Mollaret meningitis can be associated with Kikuchi disease, and recurrence of both conditions may be suppressed by colchicine when these two coexist.

Background: Dynamic spasticity (DS) is a common complication post-spinal cord injury (SCI), marked by intermittent increases in muscle tone during postural transitions or movement. Despite its prevalence, high-quality research on DS incidence, risk factors, and underlying mechanisms in SCI patients remains limited. With the growing application of spinal cord stimulation (SCS) for spasticity control, the role of proprioception in DS development has garnered attention. Additionally, advances in diffusion tensor imaging (DTI) allow for the analysis of the reticulospinal tract's (RST) role in postural control among SCI patients. However, the impact of microstructural changes in RST and proprioception on DS following SCI remains unclear.

Objective: To investigate the relationship between microstructural changes in the RST and proprioceptive circuits in SCI patients, exploring their association with DS and potential predictive factors that influence recovery.

Methods: This is a 12-month prospective cohort study. The neurophysiological and global functional status of the participants will be assessed. Primary outcomes include DTI indices of RST and proprioception, and spasticity assessment. Secondary outcomes include auditory startle reflex (ASR), H-reflex, shear wave velocity (SWV), overall functional status including spinal cord independence measure, gait analysis, modified Barthel Index, and the 36-item short form health survey. Data will be collected at 1, 3, 6, and 12 months after enrolment. Matched healthy controls will be measured during the same period after recruitment.

Discussion: This study is the first to explore the role of microstructural changes in the RST and proprioception in the assessment and prediction of DS following SCI. The findings aim to enhance theoretical understanding of SCS in spasticity management and to establish pre-treatment criteria for SCS interventions targeting motor function recovery in SCI.

Trial registration number: ChiCTR2400090724.

Background: Central Asia is known to face various ecological challenges that constitutes major risk factors for Parkinson's disease (PD). This study examines the burden of PD in Central Asia, a region where data on neurological disorders is notably sparse.

Methods: Building on the latest Global Burden of Disease Study (GBD 2021), this study investigates the Years of Life Lost (YLLs), Years Lived with Disability (YLDs), and Disability-Adjusted Life Years (DALYs) associated with PD in Central Asia and its countries from 1990 to 2021. The authors calculated average annual percent change (AAPC) to analyze trends, and compared individual country estimates to global figures. Additionally, incorporating data from the World Bank, both Bayesian hierarchical and non-hierarchical frequentist regression models were employed to assess their impact on DALYs.

Results: The DALYs varied across the study period, primarily driven by YLLs. While YLLs showed a uniform trend, YLDs were mostly incremental. Kazakhstan had the highest estimates across all metrics and was the only country aligned with global patterns. Age- and sex-specific estimates revealed substantial variations, with notably high figures found in male subjects from Tajikistan. The YLLs, YLDs, and DALYs for Kazakhstan, Uzbekistan, and Turkmenistan saw a significant increase in AAPCs. In contrast, Kyrgyzstan and Tajikistan saw declines, likely attributable to civic conflict and inter-country differences in population structure. Further comparison of DALY trends revealed significant deviations for all countries from the global pattern.

Conclusion: This study showed an overall increase in PD burden from 1990 to 2021. These findings underscore the need for targeted strategies to reduce PD burden, with a particular focus on Kazakhstan. Integrating historical information is crucial for discussing the plausible mechanisms in studies sourced from the GBD.

Background: Autoimmune encephalitis (AE) is a disease caused by an abnormal reaction between the body's autoimmunity and the central nervous system, in which the abnormal immune response targets antigenic components within or on the surface of neuronal cells. The main manifestations are mental and behavioural changes, cognitive impairment, impaired consciousness, seizures, movement disorders, etc. Most cell surface antibodies respond well to immunotherapy, intracellular antibodies, on the other hand, are usually associated with more tumours and are relatively difficult to treat with a poor prognosis. In recent years, autoimmune encephalitis that is positive for multiple anti-neuronal antibodies has been gradually recognized in the clinic, with complex and varied clinical manifestations, especially in combination with malignant tumours, which have worse treatment and prognosis. Current clinical studies on the coexistence of multiple anti-neuronal antibodies in patients with AE are mainly disseminated case reports. Patients with AE in which four anti-neuronal antibodies coexist are even rarer.

Case presentation: We report a patient who initially presented with an irritating dry cough and hyponatraemia and a chest CT suspicious for malignancy, followed by progressive deterioration of persistent status epilepticus, consciousness and cognitive deficits, and psycho-behavioural abnormalities. Serum and cerebrospinal fluid antibodies against neuronal surface or intracellular antigens were detected using a cell-based assay (CBA) method. Serum and cerebrospinal fluid were found to be positive for anti-GABABR, GAD65, SOX1 and Ma2 antibodies. And a definitive diagnosis of small cell lung cancer was made by immunohistochemistry. He eventually received gammaglobulin, steroid pulsed therapy and tumour chemotherapy.

Conclusions: The coexistence or overlap of multiple anti-neuronal surface antibodies with anti-neuronal intracellular antibodies is rare and increases the likelihood of underlying malignancy. Elucidating the impact of individualized immunotherapy and coexisting antibodies on the clinical presentation of patients has the potential to improve long-term prognosis.

Background: Up to 25% of patients suffering from an acute stroke are diagnosed with delirium during the hospital stay, with older age increasing the risk. Generalized slowing in the electroencephalogram (EEG) supports the diagnosis of delirium. We examined the potential of single-channel EEG (DeltaScan^®) as an easy-to-use device on intensive care units for detecting delirium. Our aim was to investigate characteristics of bihemispheric EEG recordings and single-channel EEG in patients suffering from strokes with and without delirium and to analyze the diagnostic accuracy of EEG-based diagnoses.

Methods: Within the first five days after stroke onset, patients received single-channel EEG DeltaScan^® and a routine 21-channel EEG. The DeltaScan^® analyzes right sided fronto-parietal EEG using a proprietary algorithm focusing on polymorphic delta activity (PDA). In routine EEG the power spectral density (PSD) in predefined frequency bands was analyzed based on 2-minute eyes-closed resting state segments. EEG-analyses were conducted in MNE (v1.3.1) in Python (3.10) and RStudio (v4.2.1).

Results: In 9 of 53 patients (52-90 years) delirium was diagnosed according to DSM-V criteria. Sensitivity of DeltaScan^® was 44% (95% CI = 15.3-77.3%), while specificity was 71% (95% CI = 57-83%). We found patients with right hemispheric stroke having a higher probability to be false positive in DeltaScan^® (p = 0.01). The 21-channel EEG based power analysis revealed significant differences in frontal delta and theta power between patients with and without delirium (p < 0.05).

Conclusions: When EEG is used in clinical practice to support a delirium diagnosis in stroke patients, bihemispheric recordings are likely preferable over unilateral recordings. Slowing in the delta- or theta-frequency spectrum over the site of stroke may lead to false-positive results in single channel EEG based delirium scoring.

Background: Primary skull base chondrosarcoma (SBC) is a rare malignant central nervous system tumor, often involving the cavernous sinus. Complete excision of tumors invading this region is exceptionally challenging due to the presence of the internal carotid artery and numerous nerves within the cavernous sinus, particularly in cases with substantial tumor volume.

Case presentation: This report describes a rare case of a massive primary SBC pushing the lateral wall of the cavernous sinus, measuring approximately 6.6 cm × 4.5 cm × 4.4 cm. Utilizing neurophysiological monitoring and intraoperative navigation, we successfully achieved complete tumor resection along the membranous structure via a left modified pterional approach (pterional-zygomatic arch-subdural-infratemporal approach), employing tools such as a cavitron ultrasonic surgical aspirator (CUSA) and piezosurgery. During the excision, localized rupture and bleeding of the internal carotid artery occurred, but prompt repair and anastomosis were performed. Postoperatively, the patient's symptoms markedly improved, and good reperfusion of the internal carotid artery was observed without new severe complications. The postoperative pathological diagnosis, according to the World Health Organization classification, was Grade 1 chondrosarcoma; therefore, radiotherapy was not administered. Magnetic resonance imaging at the 8-month follow-up showed no residual tumor or recurrence.

Conclusions: This case highlights that surgical complete excision of large intracavernous SBCs, while preserving vital neurovascular functions, is feasible and paramount for achieving favorable outcomes, particularly for Grade 1 and 2 SBCs, which comprise 82.4% of all subtypes. The use of a modified left pterional approach, intra-capsular tumor resection techniques, alongside CUSA and piezosurgery, provides valuable insights and serves as a reference for achieving complete excision of SBCs within the cavernous sinus.

Background: Syringomyelia is a rare disease with diverse etiologies, and the syrinx is typically confined to certain segments of the spinal cord. Case of syringomyelia affecting the whole cord due to tethered cord is extremely rare, and the underlying pathophysiological mechanisms remain poorly understood.

Case presentation: We described an 18-year-old male patient who presented with progressive weakness in both lower extremities and bladder dysfunction over the past four years. Magnetic resonance imaging (MRI) of the entire spine revealed a tethered spinal cord with a large syrinx extending from C1 to L5. Common causes of syrinx such as Chiari malformation, intramedullary tumors and spinal cord injury were systematically ruled out, leading to a strong suspicion that the tethered cord was the primary etiology of the extensive syringomyelia. After undergoing un-tethering surgery, the patient experienced significant symptomatic improvement, and the subsequent follow-up MRI examinations demonstrated a remarkable reduction and eventual resolution of the large syrinx.

Conclusions: Although rare, tethered cord syndrome can serve as the sole etiology for extensive syringomyelia. For such patients, performing un-tethering surgery can lead to complete resolution of the syrinx and achieve a satisfactory clinical outcome.