Narcolepsy is a sleep disorder characterized by excessive daytime sleepiness. It can be either primary or symptomatic due to other neurologic disorders. Neuromyelitis optica (NMO) is an inflammatory demyelinating disorder in which symptomatic narcolepsy is being described as one of the core clinical features. Here, we report a patient with NMO who presented with narcolepsy. Signal changes on magnetic resonance imaging in hypothalamus and other periventricular regions of high aquaporin-4 expression should prompt considering NMO as diagnosis. Serum anti-aquaporin-4 IgG antibody testing should be done in such cases, and appropriate treatment should be initiated to prevent further neurological worsening and relapses.

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Background: Parkinson's disease (PD) is one of the most common neurodegenerative movement disorders and its incidence is increasing worldwide along with population aging. Previous clinical and histologic studies suggest that the neurodegenerative process, which affects the brain, may also affect the retina of PD patients. Objective: The objective of this study was to determine the thickness changes of retina nerve fibers and macular volume with optical coherence tomography (OCT) in PD patients. Materials and Methods: The spectral domain OCT was used to assess the thickness of retinal nerve fiber layer (RNFL) and macular volume from 34 PD patients and 50 healthy age-matched controls. Results: Compared with healthy age-matched controls, the RNFL thickness of PD patients was much thinner (P < 0.05) in all retinal quadrants, with temporal thinning being more than nasal thinning. Macular volumes were diminished in both perifoveal and outer macular regions in all sectors (P < 0.05) with preserved foveal volume. The degree of tissue loss corroborated with the severity of disease as objectively assessed by standardized rating scales (UPDRS). Conclusion: There is generalized retinal nerve degeneration in patients of PD and the degree of loss correlated with the severity and duration of disease.

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Background: Cognitive impairment is increasingly being recognized as a major cause of morbidity and increased dependence over the caregivers in Parkinson's disease (PD) patients. Objective: The present study aimed to compare the cognition testing in young- and late-onset PD patient. Methods: Sixty PD patients (20 young onset and 40 late onset) fulfilling UKPDS Brain Bank diagnostic criteria were enrolled in the study. Patients were assessed clinically and using scales for cognition testing such as Scales for Outcomes in PDCognition (SCOPA-COG), Unified Parkinson's Disease Rating scale (motor part), and Hoehn and Yahr staging. Results: Young-onset group comprised 20 (33.3%) and late-onset group comprised 40 (66.7%) patients. Most of the young- and late-onset patients, 15 (75%) and 21 (52.5%), had SCOPA-COG score in the range of 30–39, respectively. On comparison between young- and late-onset groups, SCOPA-COG score's mean ± standard deviation (SD) for young and late onset was 32.60 ± 2.52 and 30.30 ± 3.65, respectively, with statistical significance (P = 0.01). SCOPA-COG score's mean ± SD for mild, moderate, and severely impaired PD patients was 31.48 ± 3.19, 30.60 ± 3.24, and 23.50 ± 3.53, respectively, which on group comparisons (ANOVA) were statistically significant (P = 0.004). However, the SCOPA-COG score was statistically insignificant with respect to disease duration. Conclusion: There was statistically significant difference in SCOPA-COG score between young- and late-onset PD patients and in patients with more severe motor impairment.

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Background: Neuromyelitis optica (NMO) is an inflammatory demyelinating disease that selectively affects the optic nerves and spinal cord and generally follows a relapsing course. Therapeutic plasma exchange (TPE) appears to be effective in patients with central nervous system inflammatory demyelinating disease who do not respond to first-line corticosteroid treatment. Objective: We represent a retrospective review of the use of TPE in the treatment of an acute attack of NMO in five patients who failed to respond to initial immunomodulatory treatment. Materials and Methods: We evaluated the effect of TPE on the degree of recovery from NMO. It was performed using a single volume plasma exchange with intermittent cell separator (Hemonetics Mobile Collection System plus) by femoral or central line access and scheduled preferably on alternate-day intervals from 8 to 10 days. Both subjective and objective clinical response to TPE was estimated, and final assessment of response was made at the time of the last TPE in the series. Results: All patients were severely disabled before the initiation of TPE and they were female; with the mean age of these patients was 52.5 years (range = 36-69 years), the median age of NMO diagnosis was 49.4 years (range = 35–65 years), and the median duration of disease was 2.6 years (range = 0–5 years). Out of five patients, three had a history of bilateral optic neuritis, and all patients were anti-against protein aquaporin-4antibody positive. Totally 24 TPE procedures were performed on five patients, the mean time of start of TPE in the acute attack was 18.6 days. Patients were severely disabled at the initiation of TPE (range = expanded disability status scale 6.5–9), and improvement was observed early in the course of TPE treatment in most patients. Conclusion: The present study provides clinical support for the importance of TPE in refractory acute attack in NMO. However, with new diagnostic technologies and increasing clinical awareness, we may see a more improved ways of TPE in these patients in the future; hence, TPE is more effective modality of treatment as it also removed the antibodies.

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Introduction: Retinal ganglion cell (RGC) degeneration was histopathologically proved previously in Alzheimer's disease (AD) patients. In this study, we aimed to determine RGC degeneration in vivo using optical coherence tomography (OCT) in AD. Methods: Twenty-one mild-to-moderate AD patients and 25 cognitively healthy age-matched controls were enrolled in this case–control prospective study. All participants underwent OCT examination to assess peripapillary retinal nerve fiber layer (RNFL) thickness, macular volume, and thickness. Results: Foveal thickness and volume were significantly higher in AD patients than controls (P = 0.023 and P = 0.024, respectively). Compared to controls, peripapillary RNFL and other macular region measurements of AD patients were not statistically different (for all P > 0.05). Discussion: Increased foveal thickness and volume can be associated with the pathological changes in the early stages of degeneration These results differ from previous studies, but still confirm retinal degeneration in AD. Conclusion: With further OCT studies on large populations, OCT will be in clinical use for early diagnosis of AD.

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Objectives: Recent studies have shown that Apelin 13 may have a neuroprotective property. Therefore it can be used as a biomarker for multiple sclerosis. Our purpose to assess serum apelin-13 levels in adult patients with multiple sclerosis and healthy controls. Patients and Methods: Subjects consisted of 42 relapsing remitting multiple sclerosis patients and 41 controls. Demographic characteristics including age, gender, duration of disease and Expanded Disability Symptom Scale (EDSS) were recorded. In serum samples obtained from the patients and controls, serum apelin-13 levels were measured with Enzyme Linked Immunosorbent Assay (ELISA) method. Results: Serum apelin-13 levels were significantly higher in the patients groups than the healthy controls (P = 0.003). Pearson analysis did not show any significant correlation between EDSS, disease duration and apelin-13 levels. Conclusion: The results of our study have been showed statistically significant higher levels of serum apelin-13 in multiple sclerosis patients compared to controls. Further studies with larger patients populations and healthy controls should be done to clarify to use serum apelin levels as a biomarker for multiple sclerosis.

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The objective of this analysis is to study the life of Hans Gustav Wilhelm Steinert and his role in identifying several neurologic disorders including myotonic dystrophy (DM). DM type 1 (DM1) is a commonly inherited adult muscle disorder. In 1909, its characteristics were first described by Hans Steinert (1875–1911), a German neurologist. Born in Dresden, Germany, Steinert studied philosophy and medicine at the Universities of Leipzig, Berlin, Freiberg, and Kiel. There, under the supervision of Heinrich Curschmann, he accomplished his own works on aphthongia, cerebral muscular atrophy, and cerebral hemiplegia. In 1909, he published his study on six patients exhibiting characteristic myotonia. With autopsy findings of muscular fibrosis, Steinert identified this independent symptom complex as “Dystrophien Myotoniker” (DM). Overall, Steinert's accurate clinical characterization, coupled with the first ever autopsy finding of this condition, laid the foundations of our current understanding about DM1. This review serves as a tribute to the achievements of Hans Steinert and provides an opportunity to understand the historical perspective of DM1. Information for this analysis was gathered from PubMed and libraries at University of Southern California and University of California, Los Angeles. In addition, personal communications with Professor Benedikt Schoser at The University of Munich, Professor Tiemo Grimm at The University of Wuerzburg, and Professor Peter Harper at The University of Wales are acknowledged.

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Background and Purpose: Mild cognitive impairment (MCI) is a focus of considerable research. The present study aimed to test the utility of a logistic regression-derived classifier, combining specific quantitative multimodal magnetic resonance imaging (MRI) data for the early objective phenotyping of MCI in the clinic, over structural MRI data. Methods: Thirty-three participants with cognitively stable amnestic MCI; 15 MCI converters to early Alzheimer's disease (AD; diseased controls) and 20 healthy controls underwent high-resolution T1-weighted volumetric MRI, diffusion tensor imaging (DTI), and proton magnetic resonance spectroscopy (¹H MR spectroscopy). The regional volumes were obtained from T1-weighted MRI. The fractional anisotropy and mean diffusivity maps were derived from DTI over multiple white matter regions. The ¹H MRS voxels were placed over posterior cingulate gyri, and N-acetyl aspartate (NAA)/creatine (Cr), choline (Cho)/Cr, myoinositol (mI/Cr), and NAA/mI ratios were obtained. A multimodal classifier comprising MR volumetry, DTI, and MRS was prepared. A cutoff point was arrived based on receiver operator characteristics analysis. Results were considered significant, if P < 0.05. Results: The most sensitive individual marker to discriminate MCI from controls was DTI (90.9%), with a specificity of 50%. For classifying MCI from AD, the best individual modality was DTI (72.7%), with a high specificity of 87.9%. The multimodal classifier approach for MCI control classification achieved an area under curve (AUC) (AUC = 0.89; P < 0.001), with 93.9% sensitivity and 70% specificity. The combined classifier for MCI-AD achieved a highest AUC (AUC = 0.93; P < 0.001), with 93% sensitivity and 85.6% specificity. Conclusions: The combined method of gray matter atrophy, white matter tract changes, and metabolite variation achieved a better performance at classifying MCI compared to the application of individual MRI biomarkers.

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Association of dengue fever with transverse myelitis is a rare phenomenon; involvement of a long segment is even rarer. We describe a middle-aged female who presented with weakness of bilateral lower limbs and urinary retention 4 days after recovery from dengue fever. She, in addition, had a sensory level up to the level of nipples. Magnetic resonance imaging confirmed the diagnosis of longitudinally extensive transverse myelitis. Besides, the patient had spontaneous subarachnoid hemorrhage (SAH) in the absence of dengue hemorrhagic fever. The patient was started on steroids along with rehabilitation. Our case highlights the extensive involvement of spinal cord in the postinfectious phase of dengue and inclusion of this arboviral disease in the differential diagnoses of myelitis as well as an etiology of SAH.

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Neuroimaging in dementia has advanced several folds in the past decade. It has evolved from diagnosing secondary causes of dementia to the current use in identifying primary dementia and aid in clinically perplexing situations. There has been a leap in the imaging technology that can virtually dissect the brain with a high degree of radiopathological correlation. The neuroimaging in dementia is classified into structural, functional, and molecular imaging. Structural imaging includes voxel-based morphometry and diffusion tensor imaging. Functional imaging includes 18F-fluorodeoxy glucose positron emission tomography imaging, ^99mTc hexamethylpropyleneamineoxime single photon emission computed tomography imaging, and functional magnetic resonance imaging studies. Molecular imaging includes amyloid imaging, tau imaging, and translocated protein imaging. These advancements have led to using neuroimaging as a biomarker in assessing the progression and also in deciphering prognosis of the disease. In this article, we discuss the current clinical relevance of these neurological advancements.

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Multiple sclerosis (MS) is a chronic neurological disease which often leads to disability. The complex etiology and progressive nature pose challenges in the management of patients with MS, particularly in developing countries like India. Lack of data on prevalence further complicates estimation of the magnitude of MS in India. There are various other challenges associated with management of patients with MS due to which the therapy is utilized by only a small segment of population in India. This article encapsulates the gaps and challenges in the management of patients with MS and presents suggestions and recommendations of the members of advisory boards held to discuss these challenges. The advisory board members suggested that an early diagnosis of MS and an early initiation of treatment are essential to achieve better results for tackling MS-related challenges. In addition, awareness and education about MS among people, regular training to physicians, emphasis on the use of revised 2010 McDonald criteria, and utilization of advanced diagnostic modalities in magnetic resonance imaging would help to achieve desirable as well as effective therapeutic outcomes. Further, access to an easy-to-use therapy delivery system could also be beneficial in attaining an adequate treatment adherence and related health benefits.

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Toxoplasma is an obligate intracellular parasite that remains asymptomatic in humans but, at times, can cause devastating disease. Here, we describe an autopsy study of a young immunocompetent gentleman with no comorbidities whose presentation was acute transverse myelitis. Magnetic resonance imaging spine showed longitudinally extensive spinal cord lesion (LESCL) that mimicked neuromyelitis optica with normal brain imaging at presentation. Investigations showed albuminocytological dissociation which prompted a course of parenteral steroid. However, the lesion relentlessly progressed to involve the brain stem and cerebrum leading to toxoplasmic encephalitis that terminated fatally. This report highlights that toxoplasma can present as LESCL and needs to be considered in the differential diagnosis of atypical myelitis.

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This sign is observed in atypical parkinsonism mainly in Progressive Supranuclear Palsy. The exact mechanism of this sign is not clear but the contraction of the Corrugator Supercilii muscle is the most important factor for the generation of vertical forehead creases. Though the wrinkling of forehead is a complex phenomenon involving multiple muscles but the 'Corrugator sign' would be a better term to describe this phenomenon. Despite the controversies regarding the mechanism and nomenclature, the 'Procerus sign' remains an important clinical clue for early Progressive Supranuclear palsy.

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Introduction: Approximately 5%–11% of neurologically normal population has extensor plantar response (EPR). Method: This study is aimed to identify differentiating features of EPR between physiological and pathological population. Results: A total of 43 patients with pyramidal lesions and 113 normal controls were recruited for this study. The pathological EPRs were more reproducible, with 89.4% having at least two positive Babinski responses and 91.5% having two positive Chaddock responses (vs. 14.3% and 4.8% in controls, P < 0.001). The pathological EPR was more sensitive to stimulation, in which 89.1% were elicited when the stimulation reached mid-lateral sole (vs. 11.9% in controls, P < 0.001). Most (93.6%) pathological cases had sustained big toe extension throughout stimulation (vs. 73.8% in controls, P < 0.001). As compared to those with brain lesion, the plantar responses in those with spinal lesion are less likely to have ankle dorsiflexion (5.3% vs. 25%, P < 0.05) more likely to have sustained extensor response with Babinski (94.7% vs. 71.4%, P < 0.05), Chaddock (89.5% vs. 64.3%, P < 0.05), and Schaefer (26.3% vs. 3.6%, P < 0.05) methods. A scoring system was computed using four variables, i.e., two consecutive positive Babinski or Chaddock responses, extensor response at mid-lateral sole, and sustained extension throughout stimulation. A score ≥3 is predictive of pathological origin, with sensitivity and specificity of 78.7% and 95.2%, respectively. Conclusion: The pathological EPR is more reproducible, sensitive to stimulation, and sustainable compared to physiological extensor response.

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