Annals of Indian Academy of Neurology最新文献

Background and objectives: Acute transverse myelitis (ATM) is a rare inflammatory spinal cord disorder causing significant motor, sensory, and autonomic dysfunction in children. Although advances in diagnosis and therapy have improved outcomes, data on early treatment response and associated factors remain limited, particularly in Southeast Asia. This study aimed to identify clinical, radiological, and immunological factors associated with early in-hospital response in children with ATM in Vietnam.

Methods: This retrospective case-control study included 56 children under 18 years of age diagnosed with ATM between 2018 and 2023. Participants were divided into a case group (poor early response, n = 29) and a control group (good early response, n = 27) based on their functional status at discharge, as assessed by the Paine and Byers scale. Clinical, cerebrospinal fluid (CSF), and magnetic resonance imaging (MRI) findings, as well as myelin oligodendrocyte glycoprotein (MOG) and aquaporin-4 (AQP4) antibody results, were analyzed. Logistic regression was performed to identify factors associated with poor early response.

Results: Severe lower limb motor weakness (Medical Research Council [MRC] muscle strength scale ≤ 1) at admission was independently associated with poor early response (OR 14.7, 95% CI: 3.0-73.2, p < 0.05). Respiratory muscle weakness, the need for mechanical ventilation, and the absence of MOG antibodies were also linked to poor early outcomes. Radiological features, including longitudinally extensive transverse myelitis (LETM), cervical involvement, and gadolinium enhancement, were not significantly associated with early response.

Conclusions: Severe motor and respiratory involvement at presentation were key indicators of poor early treatment response in pediatric ATM. Recognizing these factors promptly may guide risk stratification and early management. Longitudinal studies are needed to evaluate their predictive value for long-term recovery.

Background and objectives: Cognitive impairments in the executive and visuospatial domains are common after ischemic stroke and significantly affect a patient's independence. These impairments can affect problem-solving abilities, planning, decision-making, and spatial awareness, which are critical for functional recovery. Understanding the trajectory of recovery and its association with functional outcomes is essential for improving the quality of life of stroke survivors. The Objectives of the study are to evaluate the recovery patterns of executive and visuospatial functions 90 days after ischemic stroke and to examine their relationship with functional outcomes.

Methods: In this longitudinal study, 401 patients with neuroimaging-confirmed ischemic stroke were assessed at admission and on Days 7, 30, and 90 post-stroke. Executive function was measured using the alternate Trail Making Test, and visuospatial function was assessed using the cube-copying and clock-drawing tests. Functional outcomes were evaluated using the modified Rankin Scale (mRS) and Barthel Index. Repeated measures ANOVA (analysis of variance) was employed to assess cognitive recovery over time, and Spearman's correlation was used to examine the relationship between cognitive and functional outcomes.

Results: Significant improvements in executive and visuospatial functions were observed over the 90-day period, with the greatest gains noted within the first 30 days. A moderate-to-strong negative correlation between mRS and cognitive function was identified at all time points, peaking on Day 7.

Conclusions: Executive and visuospatial functions show dynamic recovery in the first 90 days post-stroke, particularly during the acute and subacute phases. Functional disability is closely associated with cognitive performance, underscoring the importance of early cognitive assessment.

Ictal bruxism (IB) is a rare oro-alimentary automatism in focal seizures. We report a series of six patients with IB in drug-resistant temporal lobe epilepsy (TLE) treated subsequently with temporal lobectomy. All patients had ictal teeth grinding with semiological features, suggestive of temporal lobe origin. Ictal electroencephalogram (EEG) onset showed artifacts corresponding to bruxism; one patient had a "checkerboard" pattern of EEG artifacts corresponding with lateral jaw movements. Five patients had a lesion in the mesial temporal lobe, while one patient had a neocortical temporal lobe lesion. Right temporal localization was observed in five, while one patient had left temporal localization. At the latest post-surgery follow-up of 3 to 14 years (mean 8.83 ± 3.53 years), all patients were seizure-free. IB is an under-recognized ictal phenomenon of TLE representing oro-mandibular automatisms involving the limbic networks. It is a sign of ictal localization to the temporal lobe but not a lateralizing sign.

Atypical parkinsonian disorders (APD), such as progressive supranuclear palsy (PSP), multiple system atrophy (MSA), corticobasal syndrome (CBS), and Lewy body dementia (LBD) are frequently misdiagnosed, commonly as Parkinson's disease (PD). The study aims to assess the utility of the interpeduncular angle (IPA) in distinguishing APD from PD across age ranges. This retrospective study was conducted with 225 patients (75 with APD, 75 with PD, and 75 healthy controls). Patients were categorized into three age groups: (51-60, 61-70, and 71-80 years). Two independent raters measured the IPA from T1-weighted axial brain magnetic resonance images (MRIs) at a level below the mammillary bodies using standardized measurement techniques. The APD group included 51 (68%) with PSP, 16 (21.33%) with MSA, 5 (6.67%) with LBD, and 3 (4%) with CBS. Bland-Altman analysis for angle measurement suggested good to excellent agreement between raters ( P < 0.001). IPA measurements among the different diagnostic groups showed that PSP was higher than controls ( P < 0.001) and PD ( P < 0.001), and MSA was higher than controls ( P < 0.001) and PD ( P = 0.003). There was no significant association between IPA and age in the APD phenotypes. With increasing age, the significance between APD and IPD groups decreased ( P < 0.001 in 51-60 years to P = 0.686 in 71-80 years). Receiver Operating Characteristic (ROC) analysis revealed increasing IPA thresholds for PSP versus PD (67.66° in 51-60 years to 75.71° in 71-80 years). IPA is not reliable in differentiating APD, particularly PSP and MSA, from PD and controls.

Background and objectives: Isolated dystonia generally occurs due to genetic causes, and the pattern and distribution may change over time. Botulinum toxin is the first-line treatment in those with isolated focal and segmental dystonia. We aimed to describe the clinical profile and evolution of adult patients with isolated focal and segmental dystonia presenting for treatment in our botulinum neurotoxin (BoNT) clinic, and the response to treatment, over 20 years.

Methods: We retrospectively reviewed the medical records of patients with isolated focal and segmental dystonia who had at least two visits in our BoNT clinic. The clinical features at presentation and during follow-up, muscles injected for each type of dystonia, the self-reported benefit with BoNT, and adverse effects were analyzed.

Results: Five hundred seventy-eight patients with isolated dystonia were injected and had at least one additional follow-up. Five hundred seventeen (89.4%) had focal dystonia, and 61 (10.6%) had segmental dystonia. Cervical dystonia was the most common type. Spread to other regions was seen in 81 (14%) of patients. Those with blepharospasm as initial presentation tended to have the highest occurrence (27.3%) of spread to other parts. The mean response to BoNT was around 68%. The presence of a sensory trick and the absence of tremor were found to be predictors of good outcomes with BoNT.

Conclusions: Isolated focal and segmental dystonia in adults has a good prognosis, with the majority responding well to BoNT and the dystonia remaining confined to the initially affected body part. Our study provides evidence for the response to BoNT therapy in a real-world scenario, outside of a clinical trial setting.