Challenges and Opportunities in Neurology
Hoost SS
Department of Neurology, Vagelos College of Physicians and Surgeons, Columbia University, and the New York Presbyterian Hospital, New York, NY
Brickman AM & Reyes-Dumeyer D
Taub Institute for Research on Alzheimer’s Disease and the Aging Brain, Vagelos College of Physicians and Surgeons, Columbia University, New York, NY; H. Sergievsky Center, Vagelos College of Physicians and Surgeons, Columbia University, New York, NY; Department of Neurology, Vagelos College of Physicians and Surgeons, Columbia University, and the New York Presbyterian Hospital, New York, NY
Manly JJ, Honig LS & Gu Y
Taub Institute for Research on Alzheimer’s Disease and the Aging Brain, Vagelos College of Physicians and Surgeons, Columbia University, New York, NY; G.H. Sergievsky Center, Vagelos College of Physicians and Surgeons, Columbia University, New York, NY; Department of Neurology, Vagelos College of Physicians and Surgeons, Columbia University, and the New York Presbyterian Hospital, New York, NY
Sanchez D
G.H. Sergievsky Center, Vagelos College of Physicians and Surgeons, Columbia University, New York, NY; Department of Neurology, Vagelos College of Physicians and Surgeons, Columbia University, and the New York Presbyterian Hospital, New York, NY
Lantigua RA
Taub Institute for Research on Alzheimer’s Disease and the Aging Brain, Vagelos College of Physicians and Surgeons, Columbia University, New York, NY; H. Sergievsky Center, Vagelos College of Physicians and Surgeons, Columbia University, New York, NY; Department of Medicine, Vagelos College of Physicians and Surgeons, Columbia University, and the New York Presbyterian Hospital, New York, NY
Kang MS
Taub Institute for Research on Alzheimer’s Disease and the Aging Brain, Vagelos College of Physicians and Surgeons, Columbia University, New York, NY
Dage JL
Stark Neurosciences Research Institute, Indiana University School of Medicine, Indianapolis, IN; Department of Neurology, Indiana University School of Medicine, Indianapolis, IN; Indiana Alzheimer’s Disease Research Center, Indiana University School of Medicine Indianapolis IN
Mayeux R
Taub Institute for Research on Alzheimer’s Disease and the Aging Brain, Vagelos College of Physicians and Surgeons, Columbia University, New York, NY; H. Sergievsky Center, Vagelos College of Physicians and Surgeons, Columbia University, New York, NY; Department of Neurology, Vagelos College of Physicians and Surgeons, Columbia University, and the New York Presbyterian Hospital, New York, NY
Abstract
Background: Comorbidities may influence the levels of blood-based biomarkers for Alzheimer’s disease (AD). We investigated whether differences in risk factors or comorbid conditions might explain the discordance between clinical diagnosis and biomarker classifications in a multi-ethnic cohort of elderly individuals.
Aims: To evaluate the relationship of medical conditions and other characteristics, including body mass index (BMI), vascular risk factors, and head injury, with cognitive impairment and blood-based biomarkers of AD, phosphorylated tau (P-tau 181, P-tau 217), in a multi-ethnic cohort.
Methods: Three-hundred individuals, aged 65 and older, were selected from a prospective community-based cohort for equal representation among three racial/ethnic groups: non-Hispanic White, Hispanic/Latino and African American/Black. Participants were classified into four groups based on absence (Asym) or presence (Sym) of cognitive impairment and low (NEG) or high (POS) P-tau 217 or P-tau 181 levels, determined previously in the same cohort: (Asym/NEG, Asym/POS, Sym/NEG, Sym/POS). We examined differences in individual characteristics across the four groups. We performed post-hoc analysis examining the differences across biomarker and cognitive status.
Results: P-tau 217 or P-tau 181 positive individuals had lower BMI than P-tau negative participants, regardless of symptom status. Symptomatic and asymptomatic participants did not differ in terms of BMI. BMI was not a mediator of the effect of P-tau 217 or P-tau 181 on dementia. Frequencies of other risk factors did not differ between the four groups of individuals.
Conclusions: Participants with higher levels of P-tau 217 or P-tau 181 consistent with AD had lower BMI regardless of whether the individual was symptomatic. These findings suggest that weight loss may change with AD biomarker levels before onset of cognitive decline. They do not support BMI as a confounding variable. Further longitudinal studies could explore the relationship of risk factors with clinical diagnoses and biomarkers.
Hirohisa Ono, Yoji Nishijima, Masaki Sakamoto, Soitirou Kitamura, Yuitiou Naitoh, Keiko Suzuki, Norio Fujii & Ryouta Kikura
Department of Neurosurgery and Neurology, Nishijima Hospital, Ohoka, 2835-7, Numazu- city, Shizuoka-ken 410-0022, Japan
Shigeo Ohta
Department of Neurology Medicine, Juntendo University Graduate School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo 113-8421, Japan
Abstract
Introduction: Molecular hydrogen (H2) has emerged as a therapeutic medical gas that exerts multiple functions. H2 inhalation has been approved as safe, and some clinical studies showed that it effectively improved conditions of patients with a variety of disorders such as stroke, heart infarction, chronic obstructive pulmonary disease, cancer, and COVID-19. Urinary and fecal incontinences are unavoidable symptoms of advanced Alzheimer’s disease (AD) dementia. In particular, fecal incontinence increases the level of confusion for patients and caretakers suffer from increased workloads.
Materials and Methods: To assess the integrity of the neurons related to AD, the bundles of neurons passing through the hippocampus were visualized by modified diffusion tensor imaging (DTI) technology using advanced magnetic resonance imaging (MRI)
Results: A 79-year-old woman with advanced AD continued to inhale 3% H2 gas containing 21% oxygen twice daily for 1 hour and maintained clinical observations for 2 years. After the long period of inhalation of H2 gas, the patient went to the bathroom by herself for adequate excretion. She remained to be able to go to the bathroom for her bowel movements in time, afterwards. After the long-term inhalation of H2 gas, her MRI of the brain to generate DTI images with an anisotropic (FA) value of 0.2 (FA = 0.2) showed improved integrity of the hippocampal neurons along with these clinical improvements.
Conclusion: This case report casts the question on the current understanding that the advanced and severe AD patients will never improve.
Yahia M Lodi, Adam Bowen, Aria Soltani, Varun Reddy, Hanish Polavarapu & Adam Cloud
Neurology, Neurosurgery & Radiology, Upstate Medical University and UHS-Hospitals1, Johnson City, NY
Rohan Arora
Department of Neurology, Zucker School of Medicine at Hofstra/Northwell, New York
Abstract
Background: Cerebral Aneurysms (CA) including dissecting pseudoaneurysms are treated endovascularly through a trans-radial or trans-femoral approach. When these options are not available, a trans-carotid approach via Direct Carotid Artery Cutdown (DCAC) may be used as the last option. However, the safety and feasibility of DCAC is not well studied or defined in these contexts. Our objective is to present our four unique patients who were treated by the DCAC approach for their cerebral aneurysm and/or internal carotid artery dissecting pseudoaneurysm using flow diversion.
Method: This is a report of a case series and retrospective review.
Results: Patient 1; A 75-year-old woman with known left internal carotid artery (ICA) petrocavernous aneurysm that enlarged from 6 mm to 10 mm resulting in double vision and headaches. Trans-femoral approach failed due to the tortuosity in the common carotid artery (CCA). A multidisciplinary team was formed; A vascular surgeon began the surgery followed by FD by a neuroendovascular surgeon. A 6 French sheath was placed on the right common carotid artery via DCAC then brought to right ICA by vascular surgeon, and a neuroendovascular surgeon confirmed the placement with digital subtraction angiography (DSA). The DSA confirmed a large 10 x 8 x 5mm broad-based aneurysm. Flow diversion was performed with pipeline flex measuring 5 x 30mm. Patient was discharged home and achieved baseline modified Rankin Scale (mRS )1 which sustained in 5 years with aneurysm obliteration. Patient 2; A 65- year-old woman with multiple symptomatic left ICA-Para-ophthalmic artery aneurysm measuring 9 mm. Both femoral and radial arteries were occluded and underwent DCAC and flow diversion with pipeline flex of 4×30 mm using the similar technique described above. Patient discharged home in 48 hours with National Institute of Health Stroke Scale (NIHSS) of 0 and achieved her baseline mRS. However, this patient refused to have any further follow-up studies done. Patient 3; A 67-year-old man with aortic arch endograph with stent graft after previous aortic dissection and diagnosed with bilateral internal carotid artery dissecting pseudoaneurysm (ICADP) by computed topographic angiography (CTA). The right ICADP measured 19 x 15 x 20 mm, was multilevel, extending from skull base to the internal carotid artery (ICA) origin. The left ICADP was 16 x 9 x 22 mm with inflow-zone stenosis. The DSA was attempted but failed due to the aortic stent. The right ICADPA was repaired first using Surpass streamline (Stryker Neurovascular, Irving, CA) device measuring 4 x 50 mm x2 and 5 x 40 mm covering the entire dissecting artery. Patient was discharged home in 48 hours. Three months after the first procedure, using similar technique the left ICADPA was treated with a 5 x 50 mm Surpass evolve flow diverter. Patient achieved mRS 0. In 24-months follow up CTA demonstrated complete resolution of left ICDAP, but occlusion of the right ICA without impairing his mRS 0. Patient 4; a 76-year-old-woman with tinnitus, headaches and dizziness; DSA demonstrated RICA dissecting large 16 x 8 mm pseudoaneurysm. Trans-femoral approach failed and underwent DCAC, and flow diversion with a single surpass evolve flow diverter 4.5 x3 0 mm. Patient was discharged with NIHSS 0 and achieved her baseline mRs 0.
Marco Antônio Veloso de Albuquerque, MD, PhD, Kok Fernando, MD, PhD & Umbertina Conti Reed, MD, PhD
Department of Neurology, University of São Paulo, São Paulo/São Paulo, Brazil. Current address: Department of Neurology, University of São Paulo, São Paulo.
Abstract
Background: Limb-girdle muscular dystrophies (LGMD) are a heterogeneous group of genetic muscular dystrophies, involving autosomal recessive (LGMD R) and dominant (LGMD D) subtypes. The recessive forms are far more common than dominant, particularly in children. The clinical course in this group is characterized by progressive proximal weakness, initially in pelvic and after in shoulder-girdle musculature, varying from very mild to severe degree. Significant overlap of clinical phenotypes, with clinical and genetic heterogeneity, constitutes the rule for this group of diseases. Muscle biopsies are useful for histopathologic and immunolabeling studies, and DNA analysis is the gold standard to establish the specific form LGMD.
Objectives: The aim of this study was to characterize the clinical, histological and molecular aspects in children with LGMD who attend a big public neuromuscular centre in our country to determine the frequency of different forms.
Results: Thirty-nine patients were enrolled in this study and 34 were classified as LGMD. The female to male ratio was 3:1. The mean age at onset of the disease was 6 years (2-13 years). The first sign of the disease were related to a proximal muscular involvement in lower limb, including: frequent falls (22 patients), difficulty in climbing stairs (12 patients) and difficulty to rise from the floor (2 patients). In three patients, the first sign was walking on tiptoe. All patients showed normal milestones and were able to walk before 18 months of age. CK level was elevated in all cases. Of the 34 cases defined LGMD, the frequency found were: 15(44%) Sarcoglycanopathies (LGMD Sarcoglycan-related); Five (15%), Calpainopathy (LGMD Calpain3-related); Five (15%) Dysferlinopathy (LGMD Dysferlin-related) and two (6%) LGMD FKRP-related. In 7/34 patients (21%) it was not possible to define the specific subtype, despite the techniques used. Calf hypertrophy, winging of the scapula and scoliosis were the most characteristic signs in Sarcoglycanopathies. In FKRP-related forms, calf hypertrophy was also observed. Atrophy of posterior compartment of thighs is frequent in children with Dysferlinopathy and could suggest the diagnosis. In Calpainopathy winging of the scapulae and contractures in Achilles tendons were the more important findings. Muscle biopsy showed a dystrophic pattern in all cases, more intense in Sarcoglycanopathies and LGMD FKRP-related.
Conclusions: Our study on LGMD in children demonstrates that Sarcoglycanopathy is the most frequent form of LGMD, particularly in most severe forms. Because of clinical findings overlapping and unspecific pattern in muscle biopsy, for a correctly identify of the subtype, mutation screening through a myopathies panel or exome sequencing is essential.
Aynur Ozge, MD, PhD
Department of Neurology, Mersin University School of Medicine, Mersin, Türkiye
Reza Ghouri
Department of Neurology, Brain 360 Integrative Center, İstanbul, Türkiye
Derya Uludüz
Department of Neurology, Brain 360 Integrative Center, İstanbul, Türkiye; Department of Neurology, Cerrahpaşa University Medical Faculty, İstanbul, Türkiye
Abstract
This comprehensive review examines the impact of vascular risk factors on the phenotypic expression of migraine in the elderly population. Migraine, particularly migraine with aura, has been established as a risk factor for ischemic lesions of the brain, stroke, and other cardiovascular diseases. The association between migraine and specific vascular events, such as stroke, myocardial infarction, and angina pectoris, underscores the need for a comprehensive understanding of the interplay between migraine and cardiovascular diseases. The challenges in differentiating migraine from vascular insults, especially in the elderly population, highlight the need for improved diagnostic and treatment strategies to address the complexities of managing migraine in this demographic. Patient education and treatment of modifiable risk factors may decrease future vascular events, emphasizing the importance of addressing vascular risk factors in migraine management. The potential impact of prevention and treatment of unfavorable arterial hemodynamics on neurocognitive outcomes underscores the broader implications of addressing vascular risk factors in migraine management. The clinical and public health relevance of understanding the modifiability of vascular risk factors in elderly migraine patients extends to addressing challenges in cancer survivorship, radiological emergency response, and rational person behavior, emphasizing the diverse applications of addressing vascular risk factors in healthcare and public health. Ultimately, the clinical and public health relevance of understanding the impact of vascular risk factors on the phenotypic expression of elderly migraine underscores the need for continued research and clinical vigilance in addressing the complex interplay between migraine and vascular risk factors in the elderly population.
Shahir Mazaheri
Department of Neurology, School of Medicine, Hamadan University of Medical Sciences, Hamadan, Iran
Yazdan Heshmati
Department of Neurology, School of Medicine, Hamadan University of Medical Sciences, Hamadan, Iran
Mohsen Vazifehdaryazd
Department of Neurology, School of Medicine, Hamadan University of Medical Sciences, Hamadan, Iran
Mojtaba Khazaei
Department of Neurology, School of Medicine, Hamadan University of Medical Sciences, Hamadan, Iran
Masoud Ghiasian
Department of Neurology, School of Medicine, Hamadan University of Medical Sciences, Hamadan, Iran
Ashkan Rasouli-Saravani
Department of Immunology, School of Medicine, Hamadan University of Medical Sciences, Hamadan, Iran
Mehrdad Hajilooi
Department of Immunology, School of Medicine, Hamadan University of Medical Sciences, Hamadan, Iran
Ghasem Solgi
Department of Immunology, School of Medicine, Hamadan University of Medical Sciences, Hamadan, Iran; Psoriasis Research Centre, Hamadan University of Medical Sciences, Hamadan, Iran
Abstract
Backgrounds: Environmental factors such as bacterial infections, as well as genetic factors—in particular the human leukocyte antigen (HLA) alleles—have been implicated in the etiology of multiple sclerosis (MS). This study aims to explore the relationship between Helicobacter pylori infection, HLA alleles, and disease severity in Iranian MS patients.
Methods: The study population comprised 125 MS patients and 153 ethnically matched healthy controls. Stool antigen test was used to detect H. pylori, and the expanded disability status scale (EDSS) scores were assessed in the patients. HLA-DRB1 and DQB1 alleles and haplotypes were determined in both the patients and the controls. The relationships between H pylori infection, HLA alleles, and EDSS were also analyzed.
Results: HLA-DRB1*15 and DQB1*06 alleles families and the DRB1*15~DQB1*06 haplotype were significantly more frequent in MS patients, whereas HLA-DRB1*14 and DRB1*14~DQB1*05 haplotype were less frequent. Of the 125 MS patients, 38 were diagnosed with active H. pylori infection. We found lower frequencies of HLA-DRB1*15 (P = .08) and DRB1*16 (P = .05) alleles and a higher frequency of DQB1*02 (P = .06) in the H. pylori-positive patients. HLA-DRB1*07 was more prevalent in patients with EDSS≤3.0 (P = .06). More severe MS cases (EDSS>3.0) were linked to H pylori positivity (P = .02), disease chronicity (P = .001), receiving non-steroidal anti-inflammatory drugs (P = .02), and female gender (P = .05).
Conclusion: These preliminary findings suggest a link between H. pylori infection and the severity of MS H. pylori-positive patients regardless of the type of HLA carriage.
Lauren Schleicher, PharmD, BCACP
University of Wisconsin (UW) Health, 600 Highland Ave. Madison, WI 53792, United States
Lisa Hawk, PharmD, BCPP
University of Wisconsin (UW) Health, 600 Highland Ave. Madison, WI 53792, United States
Kate Hartkopf, PharmD, BCACP
University of Wisconsin (UW) Health, 600 Highland Ave. Madison, WI 53792, United States
Christopher Hulstein, PharmD, CSP
University of Wisconsin (UW) Health, 600 Highland Ave. Madison, WI 53792, United States
Natasha Frost, MD, MS FAAN
University of Wisconsin (UW) Health, 600 Highland Ave. Madison, WI 53792, United States
Abstract
Background:
Intravenous methylprednisolone is the standard of care for a multiple sclerosis relapse. However, intravenous medications can create several barriers for patients and healthcare organizations. A non-inferiority study by Le Page et al. demonstrated no difference between a patient’s multiple sclerosis disability score when treated with high dose oral versus intravenous methylprednisolone. Implementing an oral methylprednisolone workflow for a multiple sclerosis relapse leads to healthcare cost savings and decreased healthcare utilization.
Methods:
A neurology clinical pharmacist partnered with neurology providers to create and implement a standardized workflow for high dose oral methylprednisolone to treat multiple sclerosis relapses. From October 2018 – April 2022, patients were included in the retrospective analysis if they were prescribed oral methylprednisolone for a 3 or 5 day treatment course for the purpose of an multiple sclerosis relapse by a neurology provider from the same multiple sclerosis clinic. The primary outcome was evaluating healthcare utilization measured by infusion center chair time saved and direct health system cost savings.
Results:
Overall, 290 three-day oral methylprednisolone treatment courses and 50 five-day treatment courses were prescribed. This saved the infusion center 1,680 hours of medication administration time and contributed to an estimated $80,875 in health system cost savings.
Conclusion and Relevance:
Implementing a high dose oral methylprednisolone workflow for a multiple sclerosis relapse at an outpatient neurology clinic did help reduce health system cost and infusion center chair time.
Helin Gosalia
NIHR SLaM Clinical Research Facility @ King’s, SLaM Biomedical Research Centre, King’s College London, London, UK
Diana Y. Wei
NIHR SLaM Clinical Research Facility @ King’s, SLaM Biomedical Research Centre, King’s College London, London, UK; Department of Neurology, King’s College Hospital, London, UK
Peter J. Goadsby
NIHR SLaM Clinical Research Facility @ King’s, SLaM Biomedical Research Centre, King’s College London, London, UK; Department of Neurology, King’s College Hospital, London, UK; Department of Neurology, University of California, Los Angeles, CA, USA
Abstract
Cluster headache is a primary headache disorder and is the most prevalent of the trigeminal autonomic cephalalgias. Cluster headache significantly impacts those affected, necessitating early diagnosis and management. Despite unique clinical features, such as patients experiencing attacks in a circannual pattern and often with a circadian rhythm within bouts, cluster headache patients often are misdiagnosed, mismanaged and have a delay in diagnosis. Preclinical, neuroimaging and clinical studies have advanced our understanding of cluster headache pathophysiology. The trigeminovascular system, the trigeminal autonomic reflex, and the hypothalamus are all involved in the pathophysiology of cluster headache. As our understanding of the pathophysiology of cluster headaches has evolved, new therapeutic options, such as calcitonin gene-related peptide monoclonal antibodies, non-invasive vagal nerve stimulation and sphenopalatine ganglion stimulation, have proved to have efficacy in the treatment of cluster headache. Herein, we aim to review developments to aid readers in their understanding of this debilitating disorder.
Rozita Khalili, Ahmad M. A. Abualhayjaa & Tulio E. Bertorini
Department of Neurology, University of Tennessee Health Science Center, Memphis, TN.
Abstract
Facial-onset sensory and motor neuronopathy (FOSMN) is a rare, progressive neurodegenerative disorder characterized by sensory loss and motor deficits, primarily affecting the face and upper extremities. The condition begins with sensory impairments in the trigeminal nerve distribution, extending to involve the scalp, neck, upper limbs, and trunk. Symptoms include facial weakness, bulbar disturbances (dysphonia, dysarthria, dysphagia, and sialorrhea), and lower motor neuron signs (weakness, atrophy, fasciculation, and cramps) in the limbs. A hallmark of FOSMN is the diminished or absent corneal reflex, indicating trigeminal nerve involvement and leading to potential complications like neurotrophic keratitis (NK), which can cause corneal ulceration and blindness. The etiology of FOSMN remains uncertain, with theories proposing autoimmune or neurodegenerative origins. Diagnosis is challenging due to the overlap with other ocular surface disorders, necessitating careful examination and differential diagnosis. Management focuses on symptomatic relief, including eye protection to prevent corneal complications. Awareness and early detection of ocular symptoms in FOSMN are crucial for preventing severe outcomes like NK and blindness.
Stephen E Nadeau, MD
Neurology Service and the Brain Rehabilitation Research Center, Malcom Randall VA Medical Center and the Department of Neurology, University of Florida College of Medicine.
Richard A Lawhern, PhD
Independent healthcare writer and patient advocate
Abstract
Introduction: The United States currently faces two opioid crises, an evolved crisis currently manifesting as widespread abuse of illicit opioids, and a crisis in pain management largely manufactured by the Centers for Disease Control and Prevention 2016 Guideline. Our goal in this paper is to identify root causes, trace the trajectory of forces unleashed over time, and define potential solutions to these crises.
Methods: Analytic review of the scientific, socioeconomic, and historical literature.
Results: The evolved crisis reflects a socioeconomic rift in American society that began in the 1970s and has resulted in disintegration of lives and rising levels of desperation, particularly among the under-educated, rendering them susceptible to the lure of illicit opioids. Present manifestations of that crisis reflect a complex series of events starting with a consensus in the late1990s that opioids were fully acceptable in the management of chronic noncancer pain. This was followed by vast opportunism by pill mills, drug distributors, and the manufacturers that supplied them; aggressive actions by state governments to rein in the pill mills; and ultimately, the development of an enormous black market in heroin and fentanyl. The manufactured crisis reflects intrusion into the medical care of patients in chronic pain by the Centers for Disease Control that had been politicized decades earlier and that, in 2016, issued a Guideline that reflected serious mis-construal of the causes of the opioid crisis. We trace this history and review the literature on treatment of addiction, including medically assisted therapy, treatment of depression, psychosocial interventions, 12-step programs, programs that seek to address the causes of desperation, supervised injection facilities, decriminalization, legalization, and the impact of the comprehensive approach taken by Portugal. We also analyze the problems affecting the Centers for Disease Control that led to the publication of its ill-advised 2016 Guideline.
Discussion: We conclude that many approaches currently being taken to treat addiction are well supported by scientific evidence but that the overall efficacy of treatment programs is not optimal and only a small fraction of all patients actually enter such programs. We also conclude that the Centers for Disease Control should have no future role in the regulation of patient care.
José Miguel Valdés
Department of Neurology and Psychiatry, Faculty of Medicine, Clínica Alemana-Universidad de Desarrollo, Santiago, Chile.
Fernando Vivanco
Faculty of Medicine, Clínica Alemana-Universidad de Desarrollo, Santiago, Chile.
Marianella Hernández Lorna Galleguillos
Department of Neurology and Psychiatry, Faculty of Medicine, Clínica Alemana-Universidad de Desarrollo, Santiago, Chile.
Abstract
SARS-CoV-2 infection can present with neurological symptoms due to direct or indirect invasion of the central nervous system. There are many hypotheses of why this happens, focusing on systemic inflammation, cytokines and cells, but none of them are completely understood. We present a case of a 66-year-old female that presented with a severe encephalopathy during her COVID-19 infection. We discuss physiopathology, risk factors, work up, possible treatments according to the physiopathological damage and prognosis according to the treatment response.
Subrata Biswas
Department of General Medicine, Burdwan Medical College, and Hospital, Burdwan, West Bengal, India
Ritwik Ghosh
Department of General Medicine, Burdwan Medical College and Hospital, Burdwan, West Bengal, India
Arpan Mandal
Department of General Medicine, Burdwan Medical College and Hospital, Burdwan, West Bengal, India
José Lapeña
Department of Neurology, University Hospital “12 de Octubre”, Madrid, Spain
Dipayan Roy
Department of Biochemistry, All India Institute of Medical Sciences (AIIMS), Jodhpur, Rajasthan, India; Indian Institute of Technology (IIT), Madras, Tamil Nadu, India; School of Humanities, Indira Gandhi National Open University, New Delhi, India
Julián Benito-León
Department of Neurology, University Hospital “12 de Octubre”, Madrid, Spain; Centro de Investigación Biomédica en Red Sobre Enfermedades Neurodegenerativas (CIBERNED), Madrid, Spain; Department of Medicine, Complutense University, Madrid, Spain
Abstract
Background and aim: Organophosphate poisoning is a global health burden due to intentional and occupational exposure, particularly in Asian countries. Patients are usually monitored through serum acetylcholinesterase levels. Still, it is non-specific, does not correlate well with the severity of poisoning, and is not widely available in laboratory settings in developing countries. This study aims to assess serum baseline creatine phosphokinase (CPK) levels as a prognostic biomarker in acute organophosphate poisoning.
Materials and methods: We recruited all patients older than 12 years who were admitted to the wards of the Indoor Medicine Ward in Burdwan Medical College and Hospital in West Bengal (India) because of ingestion or inhalation of organophosphorus compounds within the previous 12 hours between May 1, 2019, and November 1, 2020. Clinical severity was categorized according to Peradeniya organophosphorus poisoning (POP) scale. Serum CPK, pseudocholinesterase levels, and pH were measured. Levels were re-assessed on days three and seven, and patients were followed-up until death or discharge.
Results: 100 patients (68 men and 32 women) were included in the study. Most of them presented with miosis (98%), followed by abdominal pain (96%), diarrhea (78%), and vomiting (52%). In the multivariate analysis, the patients with a higher risk of being intubated were younger. Of the analytical levels, the one that showed a better relationship with the risk of intubation was the pseudocholinesterase level, although without statistical significance. Initial CPK levels, time of admission, or stratification on the POP severity scale offered poor performance after adjustment.
Conclusion: The analytical values of CPK or the POP severity scale at the time the patient presents in the emergency room have limited value to predict the final severity of the picture. The amount of the poison consumed should be collected for future studies to elucidate these differences.
Osman Sinanovic
Medical Faculty, University of Tuzla, Tuzla, Bosnia and Herzegovina; Sarajevo Medical School, Sarajevo School of Science and Technology, Sarajevo, Bosnia and Herzegovina; International Academy of Science and Arts of Bosnia and Herzegovina; Academy of Medical Sciences of Bosnia and Herzegovina, 71 000 Sarajevo
Muhamed Lepuzanović & Edin Bašagić
Department of Neurology, Cantonal Hospital Bihać, 77 000 Bihać, Bosnia and Herzegovina
Mirsad Muftić
Faculty of Health Sciences University of Sarajevo, Sarajevo Medical School, Sarajevo School of Science and Technology 71000 Sarajevo, Bosnia and Herzegovina
Sedjad Kahrić
Institute of occupational and sports medicine, 72 000 Zenica, Bosnia and Herzegovina
Abstract
Background: Corona infection is primarily a respiratory disease, but the SARS-CoV-2 virus also penetrates other organs, causing various symptoms, including olfactory and gustatory dysfunction, which is why we can consider COVID-19 as a multisystem disease.
Aim: To present review of some aspects of the olfactory and gustatory dysfunction in SARS-CoV- infection.
Methods: The article has an analytical character and review of the literature.
Results and Discussion: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has a high similarity with SARS-CoV-1 and uses the same receptors to enter the human body (angiotensin-converting enzyme 2/ACE2). COVID-19 is primarily a disease of the respiratory system, but SARS-CoV-2 also penetrates the other organs including central nervous system (CNS). Patients with SARS-CoV-2 infection can experience a range of clinical manifestations, from no symptoms to critical illness. The entry of the virus into the brain can lead to different neurological and psychiatric manifestations, including loss of smell (anosmia) and the loss of taste (ageusia). The frequency of anosmia and ageusia in patients with COVID-19 varies widely, from 10 to 65%, being the primary symptom in about 12% of patients. For now, the etiopathogenesis of anosmia and ageusia in SARS-CoV-2 infection is still unknown. Most of the analyzed subjects reported olfactory recovery. However, anosmia and ageusia can last several months or even longer. While most patients are expected to recover their sense of smell or taste within the first three months, a major subpopulation of patients might develop long lasting dysfunction. Although a substantial proportion of patients with Covid-19 might develop long lasting of diferent level of ansomia and ageusia it is uncertain what proportion of patients develop persistent dysfunction. Anosmia/ageusia can be as an important risk factor for fog, anxiety, and depression that may show a prolonged and/or delayed impact. However, we do not yet know what long-term effects these disorders may have on the central nervous system and mental health in general.
Conclusion: The COVID-19 is primarily a disease of the respiratory system, but SARS-CoV-2 also penetrates other organs (multisystem disease), causing various symptoms, including olfactory and gustatory dysfunction. The frequency of anosmia and ageusia in patients with COVID-19 is common but according to different papaers varies widely, from 10 to 65%, being the primary symptom in about 12% of patients. Most of the analyzed subjects reported olfactory recovery. However, anosmia and ageusia can last several months or even longer. We do not yet know what long-term consequences these disorders may have on the central nervous system and mental health in general.
A Robert Spitzer
Mackinac Neurology
Abstract
The treatment of sleep disorders is problematic because the diagnoses consist of an array of unrelated terms, there is little knowledge or link to disease processes, and progression from the patient presentation to effective therapy is not systematic. The purpose of this paper is to provide a coherent framework for understanding sleep disorders, based on anatomy and pathophysiology.
A classification of sleep disorders based on classical neurological diagnosis is presented. First, the diagnostic process in classical, clinical neurology is reviewed. In this traditional approach, diagnoses are not inferred directly from symptoms. Rather, symptoms are used to identify putative anatomic localizations, and pathophysiological mechanisms. Diagnoses are inferred as a second step from the localizations and mechanisms of disease. Subsequently, testing may be applied as necessary, using a probabilistic interpretation to guide treatment. Treatment for diseases classified in this manner is more likely to be successful. In addition, be generating alternative hypotheses during this process, if initial treatments are not successful, alternative approaches may be considered.
The anatomy and physiology of sleep disorders is briefly reviewed. The process of diagnosis is then presented, starting with specific symptoms, including insomnia, hypersomnia, limb movement disorders, fatigue and pain syndromes. Groups of symptoms, as syndromes, are considered. By relating the symptoms to the localizations and pathophysiology, a more ordered approach to management is presented. The distinction of etiology from diagnosis is discussed. Etiologies that have resolved are typically not treatable.
Prior research on fibromyalgia is summarized, including possible anatomic and pathophysiological substrates, and underlying sleep disorders. Other forms of fatigue are contrasted, with implications for different treatments.
Joel M Oster, MD & James T Kryzanski, MD
Tufts University School of Medicine, Tufts Medical Center Boston MA Departments of Neurology (JMO) and Neurosurgery (JTK)
Abstract
Objective: The goal of this work is to retrospectively and qualitatively describe a case series of two (2) patients with Occipital Epilepsy Networks treated with Responsive Neurostimulation
Method: A retrospective qualitative review of two (2) clinical cases is presented and clinicopathologic features and quantitative Responsive Neurostimulation datasets and associated variables are analyzed for review
Results: Significant and incremental seizure reduction occurred post RNS placement and this report highlights these 2 cases. One patient exhibited no seizures subsequently 2.5 years post Responsive Neurostimulation placement (Engel class I) and another patient became almost free of seizures except for the occurance of fleeting and occasional visual auras approximately 2 years post Responsive Neurostimulation placement (Engel class II). This report highlights that these 2 cases exhibited good clinical outcome after Responsive Neurostimulation placement for epilepsy localized to onset zones in occipital networks.
Vasilena Petrova & Latchesar Traykov
Military Medical Academy, Clinic of Nerve Diseases, Sofia, Bulgaria University Hospital Aleksandrovska, Clinic of Neurology, Sofia, Bulgaria
Abstract
Background: Fatigue is one of the most frequent complaints presented by multiple sclerosis patients. Fatigue may be multifactorial. Proton magnetic resonance spectroscopy studies have shown significant reductions in N-acetylaspartate/creatine ratios in multiple brain regions among fatigued multiple sclerosis patients in comparison to non-fatigued multiple sclerosis patients, suggesting axonal loss as a contributing factor. Females are twice as likely to develop the disease.
Aim: To evaluate gender variability in fatigue scores in relapsing-remitting multiple sclerosis patients. To explore potential gender differences in metabolite profiles of normal appearing white matter. To correlate metabolite changes distribution with fatigue severity and to evaluate the gender impact.
Methods: We enrolled 50 relapsing-remitting multiple sclerosis patients on disease modifying treatment and 28 healthy controls. All participants underwent proton magnetic resonance spectroscopy of normal appearing white matter corresponding regions and fatigue severity evaluation.
Results: We found higher fatigue scores in the multiple sclerosis group, due to greater severity in female subjects. We found a significant decrease of N-acetylaspartate/creatine ratio with increase in N-acetylaspartate, choline, and creatine levels in multiple sclerosis subjects. N-acetylaspartate and choline levels were significantly higher in the multiple sclerosis males. Female multiple sclerosis patients presented with lower N-acetylaspartate levels than healthy controls and greater increases in Fatigue Severity Scale score. Regression analysis revealed metabolite specific relationships between fatigue against metabolite variables.
Conclusion: Proton magnetic resonance spectroscopy registered differences in metabolite profiles in normal appearing white matter male and female multiple sclerosis subjects. We might presume gender dependent specifiers in metabolite profiles in relapsing- remitting multiple sclerosis. They impact fatigue severity. N- acetyaspartate might be crucial contributor in central fatigue in multiple sclerosis. Bioenergetic role of N-acetylaspartate needs further collaborative research on genetics and electrical properties of neurons to reveal the underlying mechanism of fatigue and conductivity deterioration.
Ritwik Ghosh, Sona Singh Sardar & Srijit Bandyopadhyay
Department of General Medicine, Burdwan Medical College and Hospital, Burdwan, West Bengal, India
Moisés León-Ruiz
Section of Clinical Neurophysiology, Department of Neurology, University Hospital “La Paz,” Madrid, Spain
Dipayan Roy
All India Institute of Medical Sciences (AIIMS), Jodhpur, Rajasthan, India; Indian Institute of Technology (IIT), Madras, Tamil Nadu, India; School of Humanities, Indira Gandhi National Open University, New Delhi, India
Kunal Bole
Department of General Medicine, Burdwan Medical College, and Hospital, Burdwan, West Bengal, India
Souvik Dubey
Department of Neuromedicine, Bangur Institute of Neurosciences, Institute of Post Graduate Medical Education and Research & SSKM Hospital, Kolkata, West Bengal, India
Julián Benito-León
Department of Neurology, University Hospital “12 de Octubre”, Madrid, Spain; Centro de Investigación Biomédica en Red Sobre Enfermedades Neurodegenerativas (CIBERNED), Madrid, Spain; Department of Medicine, Complutense University, Madrid, Spain
Abstract
Background: Snakebite is a preventable yet often-neglected public health hazard with high chronic disability and mortality, mainly faced by rural communities in the tropics/subtropics. Endocrinological disorders following snakebite (especially Russell’s viper in India) are notably underrecognized and can lead to remarkable morbidity, poor quality of life, and cardiovascular mortality. Anterior pituitary insufficiency has been the most common ailment following Russell’s viper envenomation amid those endocrinological dysfunctions. On the contrary, the posterior pituitary and nearby hypothalamus mostly remain unharmed, so central diabetes insipidus is extremely rare following a viperid snakebite envenomation.
Case Presentation: The authors present a patient developing panhypopituitarism with evident spontaneous central diabetes insipidus 29 years after Russell’s viper envenomation. Relevant investigations ruled out other possible etiologies, and he responded well to hormonal replacement therapy.
Conclusions: Panhypopituitarism with concurrent central diabetes insipidus may occur following snakebite (especially in Russell’s viper envenomation). Early recognition and proper management of these complications are quintessential to preventing further misdiagnosis, under-recognition, morbidity, impaired quality of life, and mortality.
