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MULTICENTER STUDY
Praveen Panicker, Dileep R, Abdul V Gafoor, Prasanth S R, Thomas Iype, James Jose, Antony Stanley
BACKGROUND: Guillain-Barre syndrome (GBS) is one of the most common neurological manifestations associated with severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection. Although data for a strong causal association is lacking, anecdotal reports, case series and systematic reviews linking the two have emerged in the literature. This prompted us to compare the clinical features, electrophysiology, and outcomes of GBS cases presenting during the pandemic with cases reported during a similar time period prior to the pandemic...
September 2023: Journal of the Association of Physicians of India
#2
JOURNAL ARTICLE
Wai Yan Yau, Catherine Ashton, Eoin Mulroy, Thomas Foltynie, Patricia Limousin, Jana Vandrovcova, Kunal P Verma, Rick Stell, Mark Davis, Phillipa Lamont
While biallelic POLR3A loss-of-function variants are traditionally linked to hypomyelinating leukodystrophy, patients with a specific splice variant c.1909+22G>A manifest as adolescent-onset spastic ataxia without overt leukodystrophy. In this study, we reported eight new cases, POLR3A-related disorder with c.1909+22 variant. One of these patients showed expanded phenotypic spectrum of generalised dystonia and her sister remained asymptomatic except for hypodontia. Two patients with dystonic arm tremor responded to deep brain stimulation...
May 3, 2024: Annals of Clinical and Translational Neurology
#3
JOURNAL ARTICLE
Kyra D Zens, Ekkehardt Altpeter, Monica N Wymann, Annora Mack, Nora B Baer, Sarah R Haile, Robert Steffen, Jan S Fehr, Phung Lang
BackgroundTick-borne encephalitis (TBE) is a severe, vaccine-preventable viral infection of the central nervous system. Symptoms are generally milder in children and adolescents than in adults, though severe disease does occur. A better understanding of the disease burden and duration of vaccine-mediated protection is important for vaccination recommendations.AimTo estimate TBE vaccination coverage, disease severity and vaccine effectiveness (VE) among individuals aged 0-17 years in Switzerland.MethodsVaccination coverage between 2005 and 2022 was estimated using the Swiss National Vaccination Coverage Survey (SNVCS), a nationwide, repeated cross-sectional study assessing vaccine uptake...
May 2024: Euro Surveillance
#4
Makoto Watanabe, Nobuhito Atagi, Yosuke Makino, Kunihiko Kooguchi, Bon Ohta
BACKGROUND: Marchiafava-Bignami disease is a rare neurological disease characterized by acquired lesions of the corpus callosum. Although the major causative etiology is chronic alcoholism, a case caused by acute alcohol intoxication has not yet been reported. CASE PRESENTATION: A 19-year-old female with no known medical history or a history of chronic alcohol consumption was brought to the emergency department in a coma after binge alcohol consumption. Even after an overnight observation, she remained comatose...
2024: Acute Medicine & Surgery
#5
COMPARATIVE STUDY
Anna Drynda, Agnieszka Padjas, Krzysztof Wójcik, Radosław Dziedzic, Grzegorz Biedroń, Katarzyna Wawrzycka-Adamczyk, Anna Włudarczyk, Joanna Wilańska, Jacek Musiał, Zbigniew Zdrojewski, Zenobia Czuszyńska, Anna Masiak, Maria Majdan, Radosław Jeleniewicz, Hanna Augustyniak-Bartosik, Katarzyna Jakuszko, Magdalena Krajewska, Alicja Dębska-Ślizień, Hanna Storoniak, Barbara Bułło-Piontecka, Witold Tłustochowicz, Joanna Kur-Zalewska, Małgorzata Wisłowska, Piotr Głuszko, Marta Madej, Ewa Jassem, Iwona Damps-Konstańska, Eugeniusz Kucharz, Marek Brzosko, Marcin Milchert, Anna Hawrot-Kawecka, Joanna Miłkowska-Dymanowska, Paweł Górski, Anna Lewandowska-Polak, Joanna Makowska, Joanna Zalewska, Lech Zaręba, Stanisława Bazan-Socha
OBJECTIVE: To characterize the eosinophilic granulomatosis with polyangiitis (EGPA) population from the POLVAS registry depending on ANCA status and diagnosis onset, including their comparison with the granulomatosis with polyangiitis (GPA) subset with elevated blood eosinophilia (min. 400/ μ l) (GPA HE) to develop a differentiating strategy. METHODS: A retrospective analysis of the POLVAS registry. RESULTS: The EGPA group comprised 111 patients...
2024: Journal of Immunology Research
#6
Guilherme Salazar Serrano, Alexandre Dias de Oliveira, Ramsés Miotto, Katia Lin, Guilherme Loureiro Fialho
Epilepsy is one of the most common chronical neurological conditions affecting over 50 million people worldwide. In addition to the stigma and discrimination, individuals with epilepsy suffer from a nearly three-fold increased risk of premature death compared to the general population. Although these premature deaths occur due to multiple causes, sudden unexpected death in epilepsy (SUDEP) still challenges neurologists and clinicians dealing with individuals with epilepsy. Recently, an increased interest in cardiac outcomes related to acute seizures and chronic epilepsy resulted in the groundbreaking development of the "epileptic heart" concept, and sudden cardiac death in individuals with epilepsy, which is 4...
2024: Epilepsy & behavior reports
#7
Eslam Shosha, Colleen Connolly, Adrian Budhram
Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a recently emerging autoimmune disease of the central nervous system (CNS); GFAP astrocytopathy is characterized by optic neuritis and meningoencephalomyelitis. We report the case of a 55-year-old man, otherwise healthy, who presented with isolated headaches for three months, without other features of meningoencephalitis or myelitis. His neurological examination and fundoscopy were unremarkable. Gadolinium-enhanced brain MRI demonstrated increased T2 hyperintensity within the right sub-lenticular basal ganglia, with additional leptomeningeal enhancement along the bilateral perisylvian regions and mesial temporal lobes...
2024: Frontiers in Neurology
#8
Zhengxue Zhang, Xiang Zhang, Mingming Dai, Yingying Wu, Yong You
Even though long-term immunosuppressant drugs (ISD) are employed to inhibit immune system activity, enhancing graft functionality and patient survival in solid organ transplantation (SOT), these transplants often lead to immune complications, with post-transplant autoimmune diseases of the central nervous system (CNS) being uncommon. Here, we detail the case of a 66-year-old woman who underwent a renal transplantation 8 months prior, who was admitted with subacute onset of encephalomyelitis, accompanied by headaches, paraplegia, weakness, vomiting, and abdominal pain, with a positive COVID-19 nasopharyngeal swab test 1 month before admission...
2024: Frontiers in Neurology
#9
REVIEW
Benjamin Dejakum, Stefan Kiechl, Michael Knoflach, Lukas Mayer-Suess
INTRODUCTION: This study aimed to emphasize the importance of cranial nerve (CN) palsies in spontaneous cervical artery dissection (sCeAD). METHODS: A search term-based literature review was conducted on "cervical artery dissection" and "cranial nerve palsy." English and German articles published until October 2023 were considered. RESULTS: Cranial nerve (CN) palsy in sCeAD is evident in approximately 10% of cases. In the literature, isolated palsies of CN II, III, VII, IX, X, and XII have been reported, while CN XI palsy only occurs in combination with other lower cranial nerve palsies...
2024: Frontiers in Neurology
#10
Dapeng Cai, Haohao Wu, Baogang Huang, Weiwei Xiao, Kang Du
This study reported a case of early-onset parkinsonism associated with a novel variant of the PLA2G6 gene. The boy first started showing symptoms at the age of 11, with gait instability and frequent falls. As the disease progressed, his gait instability worsened, and he developed difficulties with swallowing and speaking, although there was no apparent decline in cognitive function. An MRI of the head revealed significant atrophy of the cerebellum. The initial diagnosis for the boy was early-onset parkinsonism, classified as Hoehn-Yahr grade 5...
2024: Frontiers in Neurology
#11
Zakaria Saied, Rania Zouari, Amine Rachdi, Fatma Nabli, Dina Ben Mohamed, Samia Ben Sassi
BACKGROUND: Primary Sjogren's syndrome (pSS) is an autoimmune exocrinopathy in which extraglandular signs of pSS are determinant for the prognosis. Involvement of both peripheral and central nervous system (CNS) are known to be among the sites of high systemic activity in pSS. CASE PRESENTATION: We, herein, report a case of a 57-year-old female patient with pSS presenting with typical Guillan-Barré syndrome (GBS), shortly followed by acute headaches accompanied by cortical blindness...
May 15, 2024: Heliyon
#12
JOURNAL ARTICLE
Ángel Golimstok, María José García Basalo, Mariana Majul, Waleska Berrios, Marcelo Rugiero, Maria Cecilia Fernández, Roni Eichel
OBJECTIVES: Past reports have suggested that attention-deficit/hyperactivity disorder (ADHD) may be a risk factor for Lewy body disease (LBD). To confirm this relationship, we conducted the present study. DESIGN: A prospective observational cohort study with a follow-up to 15 years. SETTING: The subjects were recruited from cognitive neurology clinics, where they attended for a cognitive complaint or health check-up. PARTICIPANTS: Two groups of subjects: ADHD adults and healthy subjects...
April 10, 2024: American Journal of Geriatric Psychiatry
#13
JOURNAL ARTICLE
Wala Ben Kridis, Issaad Nefzi, Basma Souissi, Kheireddine Ben Mahfoudh, Mariem Dammak, Jamel Daoud, Afef Khanfir
Intramedullary spinal cord metastasis (IMSC) from solid tumors is rare. In this report, we describe the case of a patient treated at our center for breast cancer with intramedullary spinal cord metastases without bone and brain metastases or meningitis. Management of the disease remains challenging even with recent advances in the treatment of metastatic breast cancer. Treatment options include surgery, radiotherapy and chemotherapy. The prognosis of these patients still very poor.
May 2, 2024: BMJ Supportive & Palliative Care
#14
REVIEW
Julian K Lo, Lawrence R Robinson
Piriformis syndrome is a condition that is proposed to result from compression of the sciatic nerve, either in whole or in part, in the deep gluteal space by the piriformis muscle. The prevalence of piriformis syndrome depends upon the diagnostic criteria being used and the population studied but is estimated by some to be 5%-6% in all cases of low back, buttock, and leg pain and up to 17% of patients with chronic low back pain. While the sciatic nerve may pierce the piriformis muscle in about 16% of healthy individuals, this frequency is no different in those with the syndrome; thus, the relationship to this anatomic finding is unclear...
2024: Handbook of Clinical Neurology
#15
JOURNAL ARTICLE
Vijayakumar Karthik, Kiren George Koshy, Arsha Asok, Selvarajan Chettiar
Subacute sclerosing panencephalitis (SSPE) is a fatal disorder that occurs as a rare complication of childhood measles. Symptoms typically manifest between the ages of 5 and 15. While the incidence of SSPE is declining globally, it is still prevalent in regions where measles remains common and vaccination rates are low due to poverty and lack of health education. Diagnosing SSPE can be challenging, particularly when patients exhibit unusual symptoms. A thorough clinical evaluation, including vaccination history, physical examination, electroencephalogram (EEG) and Cerebrospinal fluid (CSF) analysis, can help in making a diagnosis...
May 2, 2024: BMJ Case Reports
#16
JOURNAL ARTICLE
Jeremias Motte, Melissa Sgodzai, Christiane Schneider-Gold, Nina Steckel, Thomas Mika, Tobias Hegelmaier, Dominic Borie, Aiden Haghikia, Dimitrios Mougiakakos, Roland Schroers, Ralf Gold
Myasthenia gravis (MG) and Lambert-Eaton myasthenic syndrome (LEMS) are autoimmune disorders affecting neuromuscular transmission. Their combined occurrence is rare, and treatment remains challenging. Two women diagnosed with concomitant MG/LEMS experienced severe, increasing disease activity despite multiple immunotherapies. Anti-CD19 chimeric antigen receptor (CAR) T cells have shown promise for treating autoimmune diseases. This report details the safe application of anti-CD19 CAR T cells for treating concomitant MG/LEMS...
April 25, 2024: Neuron
#17
Putu Anda Tusta Adiputra
INTRODUCTION: Injuries inflicted by needlefish resemble stab wounds, resulting from the rapid and forceful jumping of needlefish jaws from the water's surface. Needlefish impalement on the neck and face are often fatal compared to body areas or extremities. This case report investigates a rare incident where a 50-year-old male tourist sustained a cervical injury from needlefish impalement during an inter-island cruise. CASE PRESENTATION: A 50-year-old male experienced right neck pain due to accidental impalement by a needlefish...
April 27, 2024: International Journal of Surgery Case Reports
#18
Talal A Altuwaijri
INTRODUCTION AND IMPORTANCE: Arteriovenous malformation (AVM) is a rare condition affecting less than 5/10,000 people, with high misdiagnosis due to its heterogeneity, inconsistent nomenclature, and diverse diagnostic criteria. Understanding its prevalence in this age group is crucial for effective treatment. Here we present a case report of a patient with AVM. IRB approval and patient consent were obtained. This study was done based on SCARE criteria. CASE PRESENTATION: A 53-year-old female patient was diagnosed with a right hip vascular mass suggestive of AVM...
April 30, 2024: International Journal of Surgery Case Reports
#19
JOURNAL ARTICLE
Stephanie B Syc-Mazurek, Hannah Zhao-Fleming, Yong Guo, Nanthaya Tisavipat, John J Chen, Anastasia Zekeridou, Ioannis Kournoutas, Jacob J Orme, Matthew S Block, Claudia F Lucchinetti, Rafid Mustafa, Eoin P Flanagan
OBJECTIVES: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an autoimmune demyelinating disease rarely associated with malignancy. We report the clinical, MRI, immunopathology, and treatment response in a person with MOGAD and melanoma. METHODS: This is a case report of a person with a multidisciplinary evaluation at a tertiary referral center. RESULTS: A 52-year-old man presented with progressive encephalomyelitis that led to identification of metastatic melanoma...
July 2024: Neurology® Neuroimmunology & Neuroinflammation
#20
Naoto Jingami, Kosai Cho, Takayuki Nitta, Miwa Takatani, Katsuya Kobayashi, Ryo Takenaka, Naoko Sugita, Shigeru Ohtsuru
Delayed post-hypoxic leukoencephalopathy (DPHL) is a poorly recognized syndrome characterized by neuropsychiatric symptoms following recovery from an acute hypoxic episode. Although most cases are related to carbon monoxide poisoning, some have been linked to excessive opioid use. Opioid intoxication has recently become known for manifesting the characteristic imaging findings involving cerebellar, hippocampal, and basal nuclei transient edema with restricted diffusion (CHANTER) syndrome. Herein, we present a patient with severe disturbances in consciousness who was initially diagnosed with CO poisoning but was later found to have taken excessive tramadol...
2024: Frontiers in Medicine
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