Determinants of Levodopa Responsiveness in Patients with Vascular Parkinsonism.

Introduction: Vascular Parkinsonism (VaP) is characterized by symmetric, predominantly lower limb bradykinesia and rigidity and no significant improvement with levodopa. We aimed to describe the clinical and radiological features of patients with VaP and the factors that determine levodopa responsiveness. Methods: This is a retrospective chart review of patients with VaP. The study included 44 patients (36 men) with VaP. The diagnosis was based on Zijlman's criteria. Demographic and clinical details were recorded from the case files. MRI data were available for all the patients. However, the motor severity scores assessed in the OFF and ON states using the unified Parkinson's disease rating scale (UPDRS) part III were available for 17 patients only. Based on the Magnetic Resonance Imaging (MRI) finds, patients were categorized into isolated periventricular ischemic (PVI) changes, isolated basal ganglia (BG)/thalamic infarcts, and both combined. Results: The mean age at the diagnosis was 65.2 ± 7.4 years. Further, the age at the onset of symptoms was 61.8 ± 8.1 years and the total disease duration was 3.5 ± 2.5 years. Hypertension was the most common risk factor and was observed in 88.6% of patients. Symmetrical lower body parkinsonism was observed in 88.6%. The mean UPDRS part III OFF score was 33.76 ± 12.7 and ON score was 30 ± 13.98. PVI changes were the most common MRI abnormality detected. Patients with isolated BG/thalamic infarcts had better mini-mental status examination scores and better levodopa responsiveness compared to other groups. Conclusions: Hypertension was the most common risk factor seen in patients with VaP. Those with isolated BG/thalamus infarcts demonstrated better levodopa responsiveness.

EEG-based P300 in mesial temporal lobe epilepsy and its correlation with cognitive functions: A case-control study.

OBJECTIVE: P300 is an event-related potential, being explored as an objective tool to assess cognition. This study aimed to investigate the characteristics of auditory and visual P300 in patients with TLE having unilateral HS using electroencephalography (EEG) and to study its correlation with cognition. METHODS: This is a cross-sectional case-control study, where P300 characteristics in thirty patients with unilateral hippocampal sclerosis with refractory epilepsy were compared with fifteen age-, gender-, and years of education-matched healthy controls (M: F-10:5, mean age-28 ±â€¯4.76 years). Among patients, 15 belonged to the right HS group (M: F-9:6, age at onset-12.92 ±â€¯10.22 years, duration of epilepsy-16.67 ±â€¯9.38 years) and 15 to the left HS group (M: F-8:7, age at onset-10.62 ±â€¯7.18 years, duration of epilepsy-15.53 ±â€¯10.14 years). All subjects underwent EEG-based auditory and visual oddball tasks and cognitive assessment. The P300 latencies (in milliseconds) as well as amplitudes (in microvolts) were predicted in EEG and were correlated with cognitive scores. Source localization of P300 was performed with the CLARA algorithm. RESULTS: The auditory P300 latencies in controls, right HS, and left HS were 323.93 ±â€¯40.28, 351.06 ±â€¯47.23, and 328.80 ±â€¯36.03, respectively (p = 0.18) and its amplitudes were 2.3040 ±â€¯1.46, 2.77 ±â€¯1.19, and 2.68 ±â€¯1.78, respectively (p = 0.48). Visual P300 latencies in controls, right HS, and left HS were 365.87 ±â€¯47.37, 359.67 ±â€¯64.45, and 376.00 ±â€¯60.06, respectively (p = 0.51) and its amplitudes were 3.93 ±â€¯2.28, 2.09 ±â€¯1.45, and 3.56 ±â€¯1.74, respectively (p = 0.014). Further, when compared to the control group the cognitive scores were lower in the patient group (p < 0.05). SIGNIFICANCE: In comparison to the controls, patients with right HS recorded lesser amplitude on visual P300 and lower scores on cognitive tests. P300 and cognitive parameters exhibited varied relationship. P300 could be a complementary objective tool to assess cognition in patients with TLE.

P300 in mesial temporal lobe epilepsy and its correlation with cognition - A MEG based prospective case-control study.

PURPOSE: To assess the role of P300 in patients with temporal lobe epilepsy (TLE) with unilateral hippocampal sclerosis (HS) using magnetoencephalography (MEG) based auditory and visual oddball tasks, and to assess its correlation with neuropsychological tests. METHODS: Thirty-patients (M:F-17:13, onset-11.77 ±â€¯8.75 years, duration-16.10 ±â€¯9.61 years) with TLE-HS (Left:15, Right:15) and fifteen-healthy age, gender and years of education matched controls (M:F-10:5, age-28.13 ±â€¯4.76 years) underwent auditory and visual oddball tasks in MEG and cognition assessment using Indian Council of Medical Research (ICMR)-cognitive test battery. Independent component analysis (ICA) was applied to the magnetic evoked field responses for the detection of the P300 component. Source localization of P300 was performed with Classical LORETA Analysis Recursively Applied (CLARA). The latency and amplitude of P300 were estimated and subsequently correlated with cognitive scores. RESULTS: The visual P300 amplitude in the TLE group was lower when compared to the control group. In subgroup comparison (controls vs. right HS vs. left HS), visual P300 amplitudes were lower in the right HS group compared to both left HS and control groups (p-value = 0.014). On the other hand, no significant difference for auditory P300 latency or amplitude was noted between patients and controls as well as between subgroups. A negative correlation found between the MEG visual P300 amplitude and Indian Trial Making Test (TMT)-B duration in the patient group. CONCLUSION: Patients with TLE-HS have decreased visual-P300 amplitude. A significant correlation found between visual P300 amplitude and cognitive tests of visuospatial attention and working memory. Overall, MEG based visual P300 amplitude can be further explored with large sample size studies to establish as a complementary objective test for cognitive assessment in TLE.

Immune-mediated Neuropathies Our Experience over 3 Years.

INTRODUCTION: Immune-mediated peripheral neuropathy is the term applied to a spectrum of peripheral nerve disorders where immune dysregulation plays a role. Therefore, they are treatable. We analyzed the cases seen in the past 3 years by us and evaluated the clinical, laboratory, and outcome parameters in these patients. PATIENTS AND METHODS: Consecutive patients seen by the authors and diagnosed as immune-mediated neuropathy were analyzed for etiology, pathology, and outcome assessed. RESULTS: A total of sixty patients, 31 acute and 29 chronic neuropathies, were identified. Their subtypes treatment and outcome assessed. Males were significantly more in both acute and chronic cases. Miller Fisher 4, AMAN 1, paraplegic type 1, motor dominant type 19, Sensory-motor 1, MADSAM 3, Bifacial 2. Nonsystemic vasculitis was seen in 16 out of 29 chronic neuropathy and HIV, POEMS, and diabetes mellitus one each. DISCUSSION: There is a spectrum of immune-mediated neuropathy which varies in clinical course, response to treatment, etc., Small percentage of uncommon cases are seen. In this group, mortality was nil and morbidity was minimal. CONCLUSION: Immune-mediated neuropathies are treatable and hence should be diagnosed early for good quality outcome.

Pallister-Hall Syndrome.

Polydactyly is a relatively common abnormality in infants. However, it can be a marker of a wide variety of neurological and systemic abnormality. Hence, it is important for pediatrician and physician to have insight into the various association of this apparently innocuous anomaly. In this write-up, we report an extremely rare syndrome associated with polydactyly that is Pallister-Hall syndrome. A 10-month-old male child born by lower segment cesarean section presented with global delay associated with microcephaly, frontal bossing, hypertelorism, flat nose, short philtrum, incomplete cleft in the upper lip and hard palate, polydactyly, and syndactyly. The child presented with repeated vomiting and crying episodes. The patient was investigated which revealed a hypothalamic hamartomas. Pallister-Hall syndrome is a very rare autosomal dominant genetic disorder due to mutation in GLI3 gene in the short arm of chromosome 7 with variable penetrance and expressivity.