Annals of Indian Academy of Neurology最新文献

Background: Primary central nervous system lymphoma (PCNSL) is an aggressive form of non-Hodgkin's lymphoma primarily restricted to the brain, spinal cord, eyes, and leptomeninges without any evidence of systemic spread. It is often challenging to diagnose PCNSL due to its clinical and radiological overlap with various central nervous system (CNS) inflammatory conditions and other brain tumors. Cytokines and chemokines in cerebrospinal fluid (CSF) have shown potential as biomarkers for PCNSL, but the conclusion on their diagnostic utility is unavailable. This study intended to assess the role of CSF cytokines and chemokines in differentiating PCNSL from other CNS disorders.

Methods: We searched electronic databases extensively to find studies that examined the sensitivity and specificity of CSF cytokine or chemokine levels in PCNSL-synthesizing results involved standardizing the units of measurement where necessary and verifying data accuracy. Pooled effect estimates were computed by meta-analyses utilizing random-effects models.

Results: A total of 12 studies were included. The increased levels of CSF interleukin (IL)-10 and C-X-C motif Chemokine Ligant 13 (CXCL 13) were significantly associated with a positive diagnosis of PCNSL. Diagnostic accuracy measures demonstrated the promising discriminatory power of CSF IL-10 and IL-10:IL-6 ratio.

Conclusion: Our findings revealed considerable diversity in the diagnostic accuracy of individual biomarkers. Notably, CSF IL-10 and CSF IL-10:IL-6 ratios exhibited the highest pooled specificity and sensitivity, suggesting their promising potential as a diagnostic marker for PCNSL.

Background and objectives: Wilson's disease (WD) is a rare autosomal recessive disorder due to abnormal hepatic copper transport, leading to copper accumulation in the liver, brain, and other tissues. Although conventional magnetic resonance imaging (MRI) features are valuable for diagnosis, the role of diffusion-weighted imaging (DWI) remains underexplored in WD. The study aims to assess the prevalence and clinical correlates of diffusion restriction on MRI in WD.

Methods: A single-center, retrospective study was conducted. Clinicodemographic and MRI findings of patients diagnosed with WD based on a modified Leipzig score cut-off of 4 were analyzed. Characteristics of patients with diffusion restriction (WD DR+ ) and without diffusion restriction (WD DR- ) were compared using statistical tests.

Results: Of 91 patients with WD, 17 (18.7%) demonstrated MRI diffusion restriction. WD DR+ were noted to have a lower median age at symptom onset (14 [9-17] versus 15 [14-23] years; P = 0.020) and presentation (15 [13-20] years versus 19 [15-25.5] years; P = 0.022) compared to WD DR- . WD DR+ had a higher modified Leipzig score compared to WD DR- (5 [4-5] versus 7 [5-8]; P ≤ 0.001). They also had significantly higher Global Assessment Scale (GAS) for WD (32.5 [30-37]) compared to WD DR- (19 [13-22]) ( P < 0.001). WD DR+ had significantly higher proportions of patients with dysphagia (10/17, 58.8% versus 6/74, 8.1%; P < 0.001) and portal hypertension (8/17, 47.1% versus 6/74, 8.1%; P < 0.001). WD DR+ also experienced significantly higher rates of early neurological deterioration (END) (23.5% versus 6.6%, P = 0.04).

Conclusions: Diffusion restriction may hence serve as a pointer toward more severe neurological and hepatic involvement in WD, indicating the need for close supervision in this group of patients.

Hypernatremia is a condition that can lead to severe neurological symptoms, including flaccid paralysis, cognitive impairment, encephalopathy, and coma. Osmotic demyelination syndrome (ODS), which encompasses central pontine myelinolysis and extrapontine myelinolysis (EPM), is a known complication of the rapid correction of sodium levels. Limited data exist on the occurrence of ODS in post-partum women, particularly those with hypernatremia. Here, we present a case of a post-partum lady who presented in an unconscious and agitated state with hypernatremia and was found to have imaging characteristics consistent with hypernatremic demyelination. Despite a moderately elevated sodium level, she exhibited typical symptoms and imaging findings of EPM. While previously reported cases often involve patients with significantly higher sodium levels, this case highlights the importance of maintaining a low threshold for suspicion of ODS, particularly in post-partum women with hypernatremia, to prevent catastrophic consequences.

Background and objectives: This study aims to determine the effect of nonpharmacological treatments, such as vibration, electric stimulation, acupressure, massage therapy, cryotherapy, and exercise, on the restless legs syndrome (RLS) severity score.

Methods: Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines were followed for selecting studies to be included in the meta-analysis. The International Prospective Register of Systematic Reviews (PROSPERO) registered this study. Many databases, including Google Scholar, Embase, PubMed, and Scopus, were utilized to locate randomized controlled trials (RCTs). The analysis employed Cochrane's risk of bias tool 2.0 to evaluate the quality of the incorporated RCTs. Standardized mean difference (SMD) figures were employed for all outcomes to assess the impact of every nonpharmacological intervention. The aggregate effect of each was expressed using forest plots. A random-effects model was used for pooling. The heterogeneity of the included studies was evaluated using I2 values. All relevant statistical calculations were carried out using R Studi version 4.3.0.

Results: A total of 17 studies were selected for meta-analysis. There was a significant reduction in RLS severity after vibration/stimulation (SMD -1.09, 95% CI -2.45 to -0.27, P = 0.014), and a non-significant trend for acupressure (SMD -0.87, 95% CI -2.21 to 0.47, P = 0.203), massage therapy (SMD -3.49, 95% CI -10.81 to 3.83, P = 0.350), cryotherapy (SMD -1.27, 95% CI -3.72 to 1.18, P = 0.309), and exercise (SMD -2.05, 95% CI -23.95 to 19.84, P = 0.856). There was an improvement in sleep quality measured using the pooled Pittsburgh Sleep Quality Index after the nonpharmacological intervention (SMD: -0.51, 95% CI: -0.73 to -0.28). However, this improvement was not found to be significant ( P = 0.73).

Conclusions: The study demonstrated significant reductions in RLS severity with nonpharmacological interventions, offering viable alternatives to medication. However, the improvement in sleep quality was not statistically significant. Further research is needed to optimize these treatments.

Background and objectives: Accurate identification of the seizure type and prompt initiation of appropriate treatment are crucial in reducing the morbidity and mortality associated with epilepsy. In this observational study, our objective was to develop a questionnaire-based tool to assist in recognizing seizure types based on the International League Against Epilepsy (ILAE) 2017 classification system. Additionally, the study assessed the level of agreement between pediatric neurologists (PNs) and this tool.

Methods: Six additional questions were added to the existing, validated AIIMS modified International Clinical Epidemiology Network diagnostic instrument for epilepsy. These questions were designed to encompass all seizure types outlined in the ILAE 2017 classification scheme. Two pediatric postgraduate residents (PGs) and four undergraduate interns were trained to classify seizures using the tool. The classification of seizure semiology by PGs and undergraduate interns was performed verbatim using the tool. PNs used their clinical experience for seizure classification and served as the gold standard for comparison. The seizure classification by the tool was compared with the classification of three pediatric PNs using kappa agreement.

Results: A total of 144 children (97 males) with confirmed epilepsy were enrolled. The kappa agreement between PGs and PNs was 0.52 at the first level (broad categorization of seizure type as per the ILAE classification) and 0.28 at the second level (detailed categorization of seizure type as per the ILAE classification). Between interns and PNs, the kappa agreement was 0.45 at the first level and 0.30 at the second level.

Conclusions: The tool is appropriate for the broad classification of seizures into generalized, focal, multiple, and unclassified seizure types. However, for detailed seizure classification as per the ILAE 2017 classification, more extensive training may be required.

The neurocutaneous syndrome of infantile B12 deficiency, also called the "infantile tremor syndrome," is reported mainly from India. Typically, the child of a vegetarian mother who breastfeeds presents with developmental delay, regression of milestones, and tremors. The baby typically appears chubby, has sparse hypopigmented hair, and hyperpigmentation on the dorsum of the hands, feet, and other parts of the body. We share our observations in five children with akinesis and the typical features of severe infantile vitamin B12 deficiency. After initiating therapy with injectable vitamin B12, the children showed rapid recovery of movements in the first two weeks. The recovery went through a phase of generalized rigidity in two children. We postulate that the state of akinesis, or pseudoparalysis, is secondary to energy depletion of the basal ganglia. The basal ganglia, one of the regions with high metabolic rates, appear to be selectively vulnerable to vitamin B12 deficiency.