Religiosity is a factor involved in the management of health and diseases/patient longevity. This review article uses comprehensive, evidence-based studies to evaluate the nature of religiosity that ...can be used in clinical studies, thus avoiding contradictory reports which arise from misinterpretation of religiosity. We conclude that religiosity is multidimensional in nature and ultimately associated with inherent protection against diseases and overall better quality of life. However, a number of untouched aspects of religiosity need to be investigated further before we can introduce religiosity in its fully functional form to the realm of health care.
Intracranial epidermoid cysts are exceedingly rare lesions that result from a disorder of gastrulation. They are seen only in the pediatric patient population. We describe a 44-year-old Hispanic ...woman who presented with acute confusion. The family reported two months of progressive headaches and two weeks of fever, blurred central vision, and restricted visual fields. On examination, the patient appeared ill, with a low-grade fever and stiff neck. Neurological testing was limited but grossly non-focal. Computerized tomography (CT) of the head and magnetic resonance imaging (MRI) of the brain showed a large cystic mass arising in the sella, where it displaced the normal pituitary gland. Cerebrospinal fluid (CSF) showed mildly elevated opening pressure with high protein, low glucose, and neutrophilic pleocytosis. Extensive serum and CSF evaluation were negative for infectious agents. The patient was initially started on empiric treatment for presumed infectious meningoencephalitis. As tests for bacterial and viral pathogens were normal, she was switched to fluconazole. The mental status returned to normal and she was discharged home with close follow up. She returned one month later with a recurrent headache, nausea, and stiff neck. The examination showed meningismus but was otherwise non-focal. MRI of the brain showed no change in the parasellar mass. Repeat CSF showed an even higher white blood cell (WBC) count and protein with continued hypo-glycorrhachia. She underwent trans-nasal trans-sphenoidal hypophysectomy and pathology revealed a squamous epithelium-lined keratin-filled cyst suggestive of an epidermoid cyst. The patient responded well to surgery and was discharged on pituitary hormone supplements alone. To our knowledge, this is a first adult case of recurrent chemical meningitis secondary to a ruptured epidermoid cyst in the sella.
Approximately 80% of individuals infected with West Nile virus (WNV) are asymptomatic, and less than 1% suffer from neuroinvasion that can result in permanent neurological deficits or mortality. Our ...institution’s location in southern California predisposes it to a sizable case volume of neuroinvasive WNV. A 2-year retrospective study was performed at the Olive View-UCLA Medical Center to identify patients with confirmed WNV infection with neuroinvasion. Patient demographics, neurological exam findings, and laboratory diagnostics were reviewed. Data were tabulated and are presented as percentage, mean ± standard deviation, or median range. Twenty-two patients (36.4% female, age 50.2 ± 10.6 years) were identified between 20 August 2012 and 24 September 2013. The most common positive findings on review of symptoms included fever (81.8%), nausea/vomiting (81.8%), and headache (68.2%). Thirteen patients (59.1%) presented with fever defined as ≥ 37.8 °C. Motor strength was reduced in nine patients (40.9%) and eight patients (36.4%) were hyporeflexive. Lumbar puncture was performed in all but three patients (cerebrospinal fluid CSF protein 76.8 ± 29.6 mg/dL and glucose 71.0 ± 18.8 mg/dL). Elevated CSF anti-WNV IgM and IgG antibody was detected in 93.8% and 62.5% of the 16 tested cases, respectively. Elevated serum anti-WNV IgM and IgG antibody was detected in 100% and 72.2% of the 18 tested cases, respectively. Encephalitic presentations, with or without focal neurological deficits (e.g., motor weakness, hypotonia), dominated this series. In endemic areas, seasonal presentation of such symptoms should raise suspicion for WNV with neuroinvasion.
Introduction
Identification and treatment of immune‐mediated polyneuropathies may lead to improved strength and function. We studied the clinical and laboratory features, and treatment response, in ...patients with motor‐sensory axonal polyneuropathies who were found to have C5b‐9 complement staining on endoneurial microvessels.
Methods
Retrospective review of 16 consecutive adults with motor‐sensory axonal polyneuropathies who were then found to have C5b‐9 staining of endoneurial microvessels on nerve biopsy, and subsequently treated with intravenous corticosteroids (1 g methylprednisolone for 5 consecutive days, and then weekly). Strength measurements were done using quantitative handheld dynamometry. Nerve biopsy analysis included frozen and fixed tissue.
Results
Patients (mean onset age, 59 ± 4 years; range, 34‐83 years; 12 of 16 were males; 9 of 16 had diabetes) had progressive (median duration, 2 years), asymmetric, distal weakness, in the lower extremities (16 of 16) and/or upper extremities (7 of 16), and panmodal sensory loss. Electrodiagnostic studies showed axon loss. Nerve pathology showed abnormal C5b‐9 staining on endoneurial microvessels. Axon loss was present in all nerves, often varied among fascicles. Inflammation was uncommon. Distal strength usually improved (mean improvement of 34 ± 6% of normal strength; P = .0003) with corticosteroid treatment.
Discussion
Motor‐sensory axonal polyneuropathies having noninflammatory, humoral immune pathology with C5b‐9 staining of endoneurial microvessels (HIEM) frequently manifest progressive asymmetric, distal, lower extremity with or without upper extremity weakness that improves rapidly during corticosteroid treatment. HIEM may represent a new class of noninflammatory‐vasculopathic, treatable axonal motor‐sensory neuropathies.
Objective
Accumulation of misfolded superoxide dismutase‐1 (SOD1) is a pathological hallmark of SOD1‐related amyotrophic lateral sclerosis (ALS) and is observed in sporadic ALS where its role in ...pathogenesis is controversial. Understanding in vivo protein kinetics may clarify how SOD1 influences neurodegeneration and inform optimal dosing for therapies that lower SOD1 transcripts.
Methods
We employed stable isotope labeling paired with mass spectrometry to evaluate in vivo protein kinetics and concentration of soluble SOD1 in cerebrospinal fluid (CSF) of SOD1 mutation carriers, sporadic ALS participants and controls. A deaminated SOD1 peptide, SDGPVKV, that correlates with protein stability was also measured.
Results
In participants with heterozygous SOD1A5V mutations, known to cause rapidly progressive ALS, mutant SOD1 protein exhibited ~twofold faster turnover and ~ 16‐fold lower concentration compared to wild‐type SOD1 protein. SDGPVKV levels were increased in SOD1A5V carriers relative to controls. Thus, SOD1 mutations impact protein kinetics and stability. We applied this approach to sporadic ALS participants and found that SOD1 turnover, concentration, and SDGPVKV levels are not significantly different compared to controls.
Interpretation
These results highlight the ability of stable isotope labeling approaches and peptide deamidation to discern the influence of disease mutations on protein kinetics and stability and support implementation of this method to optimize clinical trial design of gene and molecular therapies for neurological disorders.
Trial Registration
Clinicaltrials.gov: NCT03449212.
Spino-cerebellar ataxia type 10 (SCA10) is an autosomal dominant disorder that is characterized by cerebellar ataxia, seizures and nystagmus with a fragmented pursuit. Schizophrenia has been reported ...with SCAs 1 and 2 yet in SCA 10, psychiatric manifestations are uncommon. We report a Hispanic family involving a father and his four children with SCA10 genetic mutation. Two of his children, a 20-year-old female and a 23-year-old male, presented with gradually progressive spino-cerebellar ataxia and paranoid schizophrenia. Neurological examination revealed ocular dysmetria, dysdiadokinesia, impaired finger-to-nose exam, gait ataxia and hyperreflexia in both the cases. Additionally, they had a history of psychosis with destructive behavior, depression and paranoid delusions with auditory hallucinations. Serology and CSF studies were unremarkable and MRI brain revealed cerebellar volume loss. Ultimately, a test for ATAXIN-10 mutation was positive thus confirming the diagnosis of SCA10 in father and his four children. We now endeavor to investigate the association between schizophrenia and SCA10.
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an autosomal dominant angiopathy caused by a mutation in the notch 3 gene on chromosome 19. ...Clinically, patients may be asymptomatic or can present with recurrent ischemic episodes and strokes leading to dementia, depression, pseudobulbar palsy, and hemi- or quadraplegia. Additional manifestations that have been described include migraine (mostly with aura), psychiatric disturbances, and epileptic seizures. Neuroimaging is essential to the diagnosis of CADASIL. On imaging CADASIL is characterized by symmetric involvement by confluent lesions located subcortically in the frontal and temporal lobes as well as in the insula, periventricularly, in the centrum semiovale, in the internal and external capsule, basal ganglia, and brain stem; with relative sparing of the fronto-orbital and the occipital subcortical regions. We describe a 49 year old male with CADASIL with absence of temporal lobe findings on MRI but predominant lesions within the periventricular white matter, occipital lobes with extension into the subcortical frontal lobes, corpus callosum and cerebellar white matter. Although CADASIL characteristically presents with anterior temporal lobe involvement, these findings may be absent and our case addresses the atypical imaging findings in CADASIL.
Historical perspective of Indian neurology Mishra, Shrikant; Trikamji, Bhavesh; Singh, Sandeep ...
Annals of Indian Academy of Neurology,
10/2013, Letnik:
16, Številka:
4
Journal Article
Recenzirano
Odprti dostop
To chronicle the history of medicine and neurology in India with a focus on its establishment and evolution.
THE HISTORY OF NEUROLOGY IN INDIA IS DIVIDED INTO TWO PERIODS: ancient and modern. The ...ancient period dates back to the mid-second millennium Before Christ (B.C.) during the creation of the Ayurvedic Indian system of Medicine, which detailed descriptions of neurological disorders called Vata Vyadhi. The early 20(th) century witnessed the birth of modern Indian medicine with the onset of formal physician training at the nation's first allopathic medical colleges located in Madras (1835), Calcutta (1835) and Mumbai (1848). Prior to India's independence from Britain in 1947, only 25 medical schools existed in the entire country. Today, there are over 355. In 1951, physicians across the field of neurology and neurosurgery united to create the Neurological Society of India (NSI). Four decades later in 1991, neurologists branched out to establish a separate organization called the Indian Academy of Neurology (IAN).
Information was gathered through literature review using PubMed, MD Consult, OVID, primary texts and research at various academic institutions in India.
Neurological disorders were first described in ancient India under Ayurveda. The transition to modern medicine occurred more recently through formal training at medical schools beginning in the 1930's. Early pioneers and founders of the NSI (1951) include Dr. Jacob Chandy, Dr. B Ramamurthi, Dr. S. T. Narasimhan and Dr. Baldev Singh. Later, Dr. J. S. Chopra, a prominent neurologist and visionary, recognized the need for primary centers of collaboration and subsequently established the IAN (1991). The future of Neurology in India is growing rapidly. Currently, there are 1100 practicing neurologists and more than 150 post-graduate trainees who join the ranks every year. As the number of neurologists rises across India, there is an increase in the amount of basic, clinical and epidemiological research being conducted across the country every day.
The history of neurology in India roots back to its rich culture and tradition. Over time, there has been great structural and organizational evolution and the future of neurology in India appears to be bright. However, the number of neurologists and research in neurology needs to experience a significant growth in the future to ensure the best patient care.
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