P300 Wave Alterations and Cognitive Impairment in Cerebellum Lesions.

Patients with cognitive deficits have a prolonged latency and reduced amplitude of the P300 wave. However, no study has correlated P300 wave alterations with the cognitive performance of patients with cerebellar lesions. We aimed to determine if the cognitive status of these patients was associated with P300 wave alterations. We recruited 30 patients with cerebellar lesions from the wards of the N.R.S. Medical College, Kolkata, in West Bengal (India). The Kolkata Cognitive Screening Battery tasks and the Frontal Assessment Battery (FAB) were used to assess the cognitive status and the International Cooperative Ataxia Rating Scale (ICARS) for cerebellar signs. We compared the results with the normative data of the Indian population. Patients had P300 wave alterations with a significant increase in latency and a non-significant trend in amplitude. In a multivariate model, P300 wave latency was positively associated with the ICARS kinetic subscale (p = 0.005) and age (p = 0.009), regardless of sex and years of education. In the model that included cognitive variables, P300 wave latency was negatively associated with performance in phonemic fluency (p = 0.035) and construction (p = 0.009). Furthermore, P300 wave amplitude was positively associated with the FAB total score (p < 0.001). In closing, patients with cerebellar lesions had an increase in latency and a decrease in the amplitude of the P300 wave. These P300 wave alterations were also associated with worse cognitive performance and some of the subscales of the ICARS, reinforcing that the cerebellum has motor, cognitive, and affective functions.

A Study on Clinical Profile of Hemifacial Spasm in India and the Therapeutic Response to Botulinum Toxin Type A Injection as well as Pre and Postinjection Quality of Life.

BACKGROUND: Hemifacial spasm (HFS) is a distressing, involuntary, irregular tonic-clonic contraction of the facial muscles innervated by the seventh cranial nerve. It affects the quality of life. Botulinum toxin is a preferred symptomatic treatment option for the condition. However, there is a lack of study in the Indian scenario. Therefore, we observed the demographic profile, clinical spectrum, therapeutic response, and adverse effects of botulinum toxin and assessed the quality of life in the pre and postinjection phases in our subjects with HFS. MATERIALS AND METHODS: The study design is a prospective open-label observational study. Consecutive cases of HFS were selected from the general neurology outpatient department (OPD) and movement disorder clinic of a medical college hospital in Eastern India. Clinical and relevant neuroimaging studies excluded mimickers and secondary causes of HFS. Institutional Ethics Committee's permission was obtained. Informed consent was taken from patients before botulinum toxin injection. The pre and postinjection assessment tools were spasm rate for a specific period of time, quantification of facial asymmetry, widening palpebral fissure by visual analog scale, Jankovic disability rating scale, HFS-7 scale, and videography. RESULTS: A total of 250 cases of HFS (F:M = 138:112) were studied. The mean age of presentation was 47 years. The mean dose of botulinum toxin injection was 24.2 units per patient. The mean duration of improvement was 4 months. The spasm frequency was decreased by 90%, and the facial asymmetry was improved by 86%. The improvement in quality of life was 86%. Local adverse effects are seen in 10.4% of cases, and all were reversible. CONCLUSION: This is one of the largest studies on the effects of botulinum toxin in subjects with HFS in the Indian population. Periodic injection of botulinum toxin is a safe and effective therapy for subjects with HFS. There is a significant improvement in the quality of life following botulinum toxin therapy in subjects with HFS. Adverse effects were local, mild, well-tolerated, and reversible.

Deciphering the Interregional and Interhemisphere Proteome of the Human Brain in the Context of the Human Proteome Project.

This study, which performs an extensive mass spectrometry-based analysis of 19 brain regions from both left and right hemispheres, presents the first draft of the human brain interhemispheric proteome. This high-resolution proteomics data provides comprehensive coverage of 3300 experimentally measured (nonhypothetical) proteins across multiple regions, allowing the characterization of protein-centric interhemispheric differences and synapse biology, and portrays the regional mapping of specific regions for brain disorder biomarkers. In the context of the Human Proteome Project (HPP), the interhemispheric proteome data reveal specific markers like chimerin 2 (CHN2) in the cerebellar vermis, olfactory marker protein (OMP) in the olfactory bulb, and ankyrin repeat domain 63 (ANKRD63) in basal ganglia, in line with regional brain transcriptomes mapped in the Human Protein Atlas (HPA). In addition, an in silico analysis pipeline was used to predict the structure and function of the uncharacterized uPE1 protein ANKRD63, and parallel reaction monitoring (PRM) was applied to validate its region-specific expression. Finally, we have built the Interhemispheric Brain Proteome Map (IBPM) Portal (www.brainprot.org) to stimulate the scientific community's interest in the brain molecular landscape and accelerate and support research in neuroproteomics. Data are available via ProteomeXchange with identifier PXD019936.

Retinal nerve fiber layer thinning found in amyotrophic lateral sclerosis - Correlation with disease duration and severity.

Purpose: The retinal involvement of amyotrophic lateral sclerosis (ALS) is a novel idea about a possible correlation between retinal nerve fiber layer (RNFL) thickness in different spectra of ALS patients. Finding the association of RNFL with disease duration and severity will help identify a novel noninvasive biomarker. Methods: The study was designed as a cross-sectional study and was conducted with a suitable proforma. We included the ALS cases based on the revised El Escorial criteria. Healthy controls were age and gender matched. We used the revised ALS functional rating scale (ALSFRS-R) to assess the operational status of the patients. We measured RNFL thickness in the four quadrants with spectral-domain optical coherence tomography (OCT) and analyzed it. Results: We included 30 cases (60 eyes) and 10 healthy controls (20 eyes) having a mean (standard deviation [SD]) age of 49.5 (11.1) years with a median of 50 years, and a majority of them (65%) were middle aged (between 41 and 60 years). We found statistically significant differences in RNFL thicknesses between ALS patients and healthy controls. On segmental analysis, the right eye superior and nasal quadrants and the left eye superior, inferior, and nasal quadrants were significantly affected, along with a gross asymmetry found between the left and right eyes among ALS patients. There was a significant decrease in average RNFL thickness in definite ALS patients than probable ALS patients, with significantly reduced average RNFL thickness in moderate to severe ALS patients. On correlation analysis, disease duration showed a good negative correlation with bilateral average RNFL thickness, and the ALSFRS-R score demonstrated a good positive correlation with bilateral average RNFL thickness, which was statistically significant. Thus, a reduced bilateral RNFL thickness is associated with a decreased ALSFRS-R score. Conclusion: The retinal changes can serve as a marker for diagnosing and monitoring patients with ALS.