To report early neurodevelopmental outcomes in a child with Tuberous Sclerosis Complex (TSC) treated with everolimus and vigabatrin. The authors report a five-year-old girl with Tuberous Sclerosis Complex who was initiated on vigabatrin at seven weeks old to treat infantile spasms and on everolimus at two months of age to treat a subependymal giant cell astrocytoma. Neuropsychological assessments were conducted in this patient at 12, 24, and 36 months using the Bayley-III and Vineland-II. On the Bayley-III, the patient had a high average cognitive composite score at 12 months and a high average language composite score at 36 months. Bayley-III assessments at 12, 24, and 36 months, yielded average cognitive, language, and motor composite scores. On the Vineland-II at 12 months, her standard score fell in the moderately low range on daily living skills and overall adaptive behavior. Vineland-II assessments at 12, 24, and 36 months yielded average range standard scores in the areas of communication, socialization, daily living skills, overall adaptive behavior, and motor skills. This case highlights early neurodevelopmental outcomes in one TSC child treated with everolimus and vigabatrin in infancy. Continued assessment and longitudinal follow-up is required to understand the broader implications of early treatment with mTOR inhibitors and vigabatrin in TSC patients in childhood.

Several clinical trials have shown the benefit of endovascular thrombectomy (EVT) in patients with large ischemic core infarction. However, the imaging selection modalities used for patient selection have differed across studies. This study aimed to assess the efficacy, safety, and prognostic factors of EVT in patients with large ischemic core selected only on the basis of Diffusion-Weighted Imaging Alberta Stroke Program Early CT Score (DWI-ASPECTS). This single-center study, conducted from 2019 to 2024, included patients with anterior circulation acute large vessel occlusion and stratified them into three groups according to DWI-ASPECTS: non-large ischemic core (≥ 6) treated with EVT (n = 77), large ischemic core (3-5) treated with EVT (n = 91), and large ischemic core (3-5) treated with medical management alone (n = 70). The primary outcome was functional independence at 90days, defined as a modified Rankin Scale (mRS) score of 0-2. Secondary endpoints included symptomatic intracranial hemorrhage (sICH) within 48h and mortality within 90days. Multivariate binary logistic regression was performed to identify factors associated with functional independence in the large-ischemic-core EVT group. Patients with large ischemic core treated with EVT had a significantly higher rate of 90-day functional independence than those who received medical management (53.8% vs 28.6%, P = 0.001). No significant differences in sICH or mortality were observed between the large-ischemic-core EVT and medical management groups. However, compared with patients with non-large ischemic core treated with EVT, those with large ischemic core treated with EVT had a lower rate of functional independence (53.8% vs 70.1%, P = 0.039). In the large ischemic core EVT group, intravenous thrombolysis (OR 0.164, P = 0.018) and parenchymal hematoma type 2 (PH2) hemorrhage (OR 25.641, P = 0.012) were independent predictors of 90-day outcomes. In this cohort, EVT was associated with improved 90-day functional outcomes in patients with large ischemic core (DWI-ASPECTS 3-5) compared with medical management alone, without a statistically significant increase in sICH or mortality. Intravenous thrombolysis and PH2 hemorrhage were identified as independent predictors of outcome. These results require further confirmation in larger and adequately powered studies.

Levodopa-induced dyskinesia (LID) is a common motor complication of Parkinson's disease (PD), yet peripheral biological factors associated with dyskinesia severity remain insufficiently characterized. Systemic inflammation and iron dysregulation have been implicated in PD, but their relationship with LID expression is unclear. We investigated the association between serum ferritin, neutrophil-to-lymphocyte ratio (NLR), lymphocyte-to-monocyte ratio (LMR), and the presence and severity of LID. In this study, 302 patients with idiopathic PD receiving stable levodopa therapy for at least 24 months were evaluated. Dyskinesia severity was assessed in the medication "ON" state using the Unified Dyskinesia Rating Scale (UDysRS). Serum ferritin and complete blood counts were obtained from fasting samples, and NLR and LMR were calculated. Multivariable regression analyses were performed adjusting for age, sex, weight, disease duration, levodopa equivalent daily dose, Hoehn and Yahr stage, motor severity, C-reactive protein, and amantadine use. LID was present in 158 patients (52.3%). Patients with dyskinesia had higher median ferritin levels (152 vs. 104 ng/mL, p < 0.001) and NLR (2.8 vs. 2.0, p < 0.001), and lower LMR (3.5 vs. 4.3, p < 0.001) compared with those without dyskinesia. In adjusted analyses, the highest ferritin quartile was associated with greater UDysRS scores (β = 5.9, p < 0.001), as were the highest NLR quartile (β = 4.2, p = 0.002). Conversely, higher LMR was independently associated with lower dyskinesia severity (β=-3.4, p = 0.017). Elevated serum ferritin and altered leukocyte-derived ratios (NLR, LMR) were independently associated with increased levodopa-induced dyskinesia severity in Parkinson's disease. These biomarkers may reflect systemic systemic processes linked to dyskinesia burden and warrant further evaluation in longitudinal studies.

Depression after a stroke is the most frequent and burdensome neuropsychiatric post-stroke complication. This study aimed to determine the relationship between post-stroke depression (PSD) and the levels of homocysteine (HCY) in patients with spontaneous intracerebral hemorrhage (SICH). We collected data from patients with hemorrhagic stroke (HS) admitted to the hospital and recorded their demographic and clinical characteristics. We also searched for information regarding HAM-D17 (Hamilton Depression) scores and HCY levels at 3m, the use of antidepressant medications and folic acid during the follow-up period in the group of patients diagnosed with PSD and hyperhomocysteinemia (HHcy) in the acute phase. A total of 1,852 patients were included. 642 (34.7%) patients with PSD and 598 (32.3%) patients with HHcy, 364 (19.7%) patients with both conditions. A link between depression and low HCY level has similarly been found in patients with HS (OR, 1.549; 95% CI, 1.358-1.768). Additionally, left-sided stroke, anterior circulatory stroke, intraventricular hemorrhage, symptoms of paralysis or dysarthria, and higher NIHSS scores occurred more often in the PSD group. Compared to the antidepressant medications (ADM) group, HAM-D17 scores decreased significantly in the ADM plus folic acid group at the end of 3m (P = 0.036). On the basis of our data, PSD was significantly more frequent in patients with HHcy in patients with SICH at the acute phase. The location of hemorrhage and the severity of the disease are significantly correlated with the incidence of PSD. Oral doses of folic acid and ADM showed significant improvements in the HAM-D17 scores for the patients with comorbid PSD and HHcy.

Bilateral facial palsy variant Guillain-Barré syndrome (GBS) is a distinct clinical subtype. As an acute immune-mediated polyradiculoneuropathy often triggered by infections, varicella-zoster virus (VZV) is a rare yet significant precipitating factor for this variant. Anti-ganglioside antibodies, particularly anti-GD3, are involved in the pathogenesis of GBS, but their significance in VZV-associated bilateral facial palsy subtype remains incompletely elucidated. We report a 46-year-old female who developed acute bilateral facial palsy and limb sensory deficits following VZV infection. Serum anti-ganglioside testing confirmed GD3 IgG positivity, and cerebrospinal fluid analysis revealed albuminocytological dissociation. Nerve conduction studies supported a demyelinating polyneuropathy, and the patient showed significant improvement after intravenous immunoglobulin therapy. This case suggests that VZV may act as a potential trigger for GD3 antibody-positive variant GBS. Early immunotherapy may improve outcomes, emphasizing the need for systematic antibody testing in atypical presentations of this variant GBS. Further studies are required to clarify the pathophysiological role of GD3 antibodies in VZV-associated variant GBS.

The coexistence of autoimmune encephalitis (AE) and autoimmune gastritis (AIG) is exceedingly rare, and their simultaneous presentation often manifests as refractory hyponatremia, which can be easily overlooked. This report describes a case of anti-leucine-rich glioma-inactivated 1 (LGI1) antibody-associated AE coexisting with AIG, presenting initially as refractory hyponatremia, aiming to enhance awareness of multisystem autoimmune involvement. A 77-year-old man presented with a two-month history of poor appetite, fatigue, and drowsiness, which had worsened over the preceding week. He had an 8-year history of psoriasis. Laboratory evaluation revealed profound hyponatremia (serum sodium nadir 109 mmol/L), macrocytic anemia (hemoglobin 88g/L, mean corpuscular volume 118 fL), and deficiencies of vitamin B12 and iron. Gastroscopy with histopathological examination confirmed the diagnosis of autoimmune gastritis. Due to poor response of hyponatremia to conventional sodium supplementation and the presence of left limb spasms, further diagnostic work-up was pursued. Cerebrospinal fluid analysis tested positive for anti-LGI1 antibodies (titer 1:64), and brain magnetic resonance imaging revealed bilateral hippocampal T2-FLAIR hyperintensity and a T2 hyperintense signal in the pituitary gland, leading to the diagnosis of anti-LGI1 antibody-associated AE complicated by central adrenal insufficiency. Following methylprednisolone pulse therapy, the patient's serum sodium normalized, left limb spasms resolved, and mental status improved. At the 8-month follow-up, the patient remained clinically stable. This case highlights that in patients presenting with refractory hyponatremia accompanied by multisystem manifestations, the possibility of coexisting autoimmune diseases such as AE and AIG should be considered. Comprehensive autoimmune antibody screening and neuroimaging are crucial for early diagnosis and timely intervention.

Pure agraphia represents a rare neuropsychological syndrome in which writing is selectively impaired while other language modalities remain intact, providing a unique window into the specialized cognitive and neural mechanisms that support written language production. Drawing on the dual-route cognitive model that posits separable lexical and sublexical spelling mechanisms (Patterson, Acquired disorders of spelling, 1988; Rapp and Caramazza, J Exp Psychol: Human Perception and Performance 23:1130-152, 1997; Rapp et al., Cogn Neuropsychology 19:1-29, 2002), whereas network models emphasize distributed interactions, this case offers a multilevel analysis of the deficits observed in pure agraphia due to a focal lesion in Exner's area in the left frontal lobe (Chen et al., Sci Rep 9:55129, 2019; Hickok and Poeppel, Nat Rev Neurosci 8:393-402, 2007; Stevens et al., J Neurosci 37:5288-297, 2017; Vigneau et al. NeuroImage 30:1414-432,2006). Exner's area-located in the posterior portion of the left middle frontal gyrus, immediately anterior to the hand region of the primary motor cortex and anterior and inferior to the left Frontal Eye Field- has been implicated in the interface between linguistic and motor aspects of writing. The patient was a 47-year-old right-handed male with 14years of formal education and no prior history of language or writing deficits. Four weeks before admission, his wife reported progressive deterioration in writing, characterized by incorrect word selection and spelling errors. Mild drooling from the right corner of the mouth and food retention on the right side of the tongue subsequently appeared. CT and MRI revealed a left frontotemporal mass lesion with partial haemorrhagic transformation confined to Exner's area in the frontal lobe. Comprehensive neuropsychological and neurolinguistic assessment, including the Aachen Aphasia Test and the Lexicon Model Oriented battery, revealed intact oral language, reading, repetition, copying, and general cognition, in striking contrast to a profound writing impairment. The patient exhibited selective difficulties with irregular words, loanwords, and pseudowords, with writing accuracy declining to 40% for pseudowords and 25% for loanwords, despite 100% reading accuracy. Neuropsychological testing showed only mild deficits in verbal working memory. These findings indicate a mixed central agraphia affecting both lexical and sublexical spelling mechanisms, consistent with Exner's area functioning as a supramodal graphemic convergence hub. This case provides lesion-based evidence for the theoretical dissociation of central writing mechanisms and supports the crucial role of Exner's area acting as a critical integration hub linking phonological, orthographic, and motor processes.