Results 51 to 60 of about 7,776 (199)

Atypical manifestation of human herpes virus-6 encephalitis with extra-limbic lesions: a case report [PDF]

Encephalitis
Human herpesvirus-6 (HHV-6) encephalitis represents a serious opportunistic infection in immunocompromised patients that is associated with substantial mortality and long-term neurological sequelae. The disease most often involves the limbic system, with
Soo Hyun Ahn, Seon-Jae Ahn, Kon Chu
doaj   +1 more source

Severe ADEM‐Like Neuroinflammatory Disease and Cerebrovascular Fragility With Recurrent Pseudoaneurysms and Moyamoya in a Familial Germline CBL Mutation: Expanding the Clinical Phenotype

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Heterozygous germline variants in CBL disrupt its function as a negative regulator of the Ras/MAPK pathway, classically predisposing to Juvenile myelomonocytic leukemia (JMML) and moyamoya. We describe two affected siblings carrying a paternally inherited CBL variant (c.1210 T> C, p.
Michal Bar‐Hakim, Anat Abramovich, Sara Via‐Dorembus, Gali Heimer, Bruria Ben‐Zeev, Etai Adam, Assaf A. Barg, Jonathan Roth, Basheer Sheick‐Yousif, Rony Beeri Berkowiz, Michal Feldon, Haim Bassan, Moran Hausman‐Kedem   +12 more
wiley   +1 more source

Paraneoplastic limbic encephalitis presenting as a neurological emergency: a case report

Journal of Medical Case Reports, 2010
Introduction Paraneoplastic limbic encephalitis remains a challenging clinical diagnosis with poor outcome if it is not recognized and treated early in the course of the disease.
Mehta Brijesh P, Xia Zongqi, Ropper Allan H, Kesari Santosh   +3 more
doaj   +1 more source

Gephyrin Neurological Autoimmunity

Annals of Neurology, EarlyView.
Gephyrin is a postsynaptic scaffold protein essential for inhibitory neurotransmission. Gephyrin‐immunoglobulin G (IgG) was reported, decades ago, in a single case of paraneoplastic stiff‐person‐like syndrome, but its broader clinical relevance remains unknown.
Maria Chiara Pantuliano, Friederike A. Arlt, Naomi Arenson, Irena Dujmovic Basuroski, Divyanshu Dubey, Connie E. Lesnick, John R. Mills, Sean J. Pittock, Anastasia Zekeridou, Michael A. Lane, Andrew McKeon   +10 more
wiley   +1 more source

Anti-Alpha-Amino-3-Hydroxy-5-Methyl-4-Isoxazolepropionic Acid Receptor Encephalitis: A Review

Frontiers in Immunology, 2021
Anti-alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) encephalitis, a rare subtype of autoimmune encephalitis, was first reported by Lai et al.
Tian-Yi Zhang, Meng-Ting Cai, Yang Zheng, Qi-Lun Lai, Chun-Hong Shen, Song Qiao, Yin-Xi Zhang   +6 more
doaj   +1 more source

Diagnosing autoimmune limbic encephalitis [PDF]

Canadian Medical Association Journal, 2019
KEY POINTS Autoimmune limbic encephalitis (ALE) is an inflammatory disease involving the medial temporal lobes; it classically presents with rapid neuropsychiatric decline. Patients with ALE have, and may present with, a diverse array of neuropsychiatric symptoms, which means that they may ...
Adrian, Budhram, Andrew, Leung, Michael W, Nicolle, Jorge G, Burneo   +3 more
openaire   +2 more sources

A guide to neuromodulation in drug‐resistant epilepsy

Epileptic Disorders, EarlyView.
Abstract Neuromodulation is approved for the treatment of drug‐resistant epilepsy. It has been increasingly utilized over the past two decades with the approval of deep brain stimulation (DBS) and responsive neurostimulation (RNS) in addition to vagus nerve stimulation (VNS)—particularly in patients who are not deemed to be good resective surgical ...
Prachi Parikh, Irena Dolezawa, Manu Hegde, Lukas Imbach, Brian N. Lundstrom, Francesca Pizzo, Vikram R. Rao, Jaysingh Singh, Derek Southwell, Satsuke Watanabe, Birgit Frauscher   +10 more
wiley   +1 more source

Localizing value of cutaneous ictal phenomena: A systematic review

Epileptic Disorders, EarlyView.
Abstract Objective Clinical observation of autonomic signs during seizures can aid in localizing the epileptogenic zone (EZ). We performed a systematic review and meta‐analysis to evaluate the localizing value of ictal cutaneous phenomena—piloerection, sweating, pallor, and flushing—in focal epilepsy and their relevance to presurgical evaluation ...
R. Rocamora, L. Vilella, L. Panadés‐De Oliveira, F. Álvarez‐Gómez, T. Ibáñez, A. Celdrán, G. Zimmermann, R. Zucca   +7 more
wiley   +1 more source

Progressive limbic encephalopathy: Problems and prospects

Annals of Indian Academy of Neurology, 2014
Background: It was observed that a good number of patients presenting with psychiatric manifestations when investigated later because of unresponsiveness to treatment or late development of organic features turned out to be treatable limbic syndromes ...
Sadanandavalli Retnaswami Chandra, Roopa Seshadri, Yasha Chikabasaviah, Thomas Gregor Issac   +3 more
doaj   +1 more source

NMDAR‐antibody encephalitis: Seizure semiology and EEG findings

Epileptic Disorders, EarlyView.
Abstract Background N‐methyl‐D‐aspartate receptor antibody encephalitis (NMDAR‐Ab‐E) is an autoantibody‐mediated disorder, characterized by acute development of neuropsychiatric symptoms, seizures, movement disorders, and autonomic instability. Objectives To describe acute seizure semiology and electroencephalogram (EEG) findings in patients with a ...
Maria Emilia C. Andraus, Bernard Liem, Sidra Aurangzeb, Mkael Symmonds, Adam E. Handel, Sarosh R. Irani, Arjune Sen   +6 more
wiley   +1 more source