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A case of febrile infection-related epilepsy syndrome (FIRES) in young adult: still a diagnostic and therapeutic challenge
We report a case of acute encephalopathy with refractory seizures after a febrile illness (FIRES) in a young adult with complete remission of symptoms as well as dramatic improvement of EEG abnormalities following intravenous immunoglobulin and proper antiepileptic medications. We con ducted an extensive workup including lumbar puncture, blood tests, EEG serial monitoring, MRI brain, total body CT scan, and PET brain with FDG to shed light on the etiology of the disease.
Source: Neurological Sciences - May 4, 2022 Category: Neurology Source Type: research

Thrombotic Thrombocytopenic Purpura Without Schistocytes: Beware of Misdiagnosis
A 43-year-old woman was admitted for acute unusual headache, vertigo, and left upper limb weakness. She had been complaining of asthenia for the past few weeks. Her medical history included uterine cervix cancer treated with conisation and 2 pregnancies without serious complications. No allergy or recent drug introduction was noted. After ruling out current pregnancy, magnetic resonance imaging (MRI) was immediately performed and showed recent ischemic strokes in multiple territories. Doppler ultrasound imaging showed no carotid or vertebral arteries lesion. Electrocardiogram showed no ST elevation or atrial fibrillation, ...
Source: Neurology Clinical Practice - October 11, 2021 Category: Neurology Authors: Decker, P., Moulinet, T., Revuz, S., Perez, P., Jaussaud, R. Tags: Autoimmune diseases, Hematologic, All Cerebrovascular disease/Stroke Case Source Type: research

Anti ‐plexin D1 antibody–mediated neuropathic pain
AbstractNeuropathic pain (NeP) is an intractable pain caused by a lesion or disease of the somatosensory nervous system. NeP is often challenging to manage because most of the mechanisms remain to be elucidated. Recent investigations in the field of autoimmune neurology have demonstrated that specific autoantibodies against antigens in the somatosensory pathway can cause NeP. Detection of pathogenic autoantibodies in NeP adds to the understanding of the mechanism of pain, which might aid in the development of novel immunotherapies. Therefore, it is necessary to explore novel NeP ‐related autoantibodies to improve the man...
Source: Clinical and Experimental Neuroimmunology - March 31, 2020 Category: Neurology Authors: Takayuki Fujii, Ryo Yamasaki, Yukino Miyachi, Kyoko Iinuma, Jun ‐ichi Kira Tags: REVIEW ARTICLE Source Type: research

Ketogenic Diet as a Treatment for Super-Refractory Status Epilepticus in Febrile Infection-Related Epilepsy Syndrome
Conclusions: The characteristic EEG pattern in the acute phase promoted timely diagnosis of FIRES. Our data suggest that KD may be a safe and promising therapy for FIRES with SRSE, and that early initiation of KD produces a favorable prognosis. Therefore, KD should be applied earlier in the course of FIRES. Intravenous KD can be an effective alternative route of administration for patients who may not take KD enterally. Introduction Febrile infection-related epilepsy syndrome (FIRES) is a rare epileptic encephalopathy of unknown etiology which occurs in patients without active epilepsy or underlying neurological diso...
Source: Frontiers in Neurology - April 25, 2019 Category: Neurology Source Type: research

Neurological Involvement in Primary Systemic Vasculitis
Conclusion Neurological involvement is a common complication of PSV (Table 1), and neurologists play an important role in the identification and diagnosis of PSV patients with otherwise unexplained neurological symptoms as their chief complaint. This article summarizes the neurological manifestations of PSV and hopes to improve neuroscientists' understanding of this broad range of diseases. TABLE 1 Table 1. Common CNS and PNS involvements of primary systemic vasculitis. Author Contributions SZ conceived the article and wrote the manuscript. DY and GT reviewed and edited the manuscript. All authors ...
Source: Frontiers in Neurology - April 25, 2019 Category: Neurology Source Type: research

Mucosal tolerance therapy in humans: Past and future
AbstractMucosal tolerance refers to the physiological process by which exposure of proteins to the mucosal surface preferentially induces tolerance. The route of mucosal administration can determine the type of regulatory T (Treg) cells induced, with the oral route inducing transforming growth factor ‐β‐producing Treg cells and the nasal route inducing interleukin‐10‐secreting Treg cells. Previous studies of oral tolerance to autoantigens in humans, including patients with multiple sclerosis, rheumatoid arthritis and diabetes, led to mixed results and negative phase III trials, althoug h oral immunotherapy for fo...
Source: Clinical and Experimental Neuroimmunology - March 13, 2019 Category: Neurology Authors: Rafael M. Rezende, Laura M. Cox, Howard L. Weiner Tags: Review Article Source Type: research

Allergy in patients with anti-N-methyl-d-aspartate receptor encephalitis
Conclusions Patients with anti-NMDAR encephalitis show higher allergic rate than those with virus encephalitis. Patients with allergy show higher CSF antibody titers and greater illness severity. However, the final outcome of anti-NMDAR encephalitis was not influenced.
Source: Epilepsy and Behavior - January 5, 2018 Category: Neurology Source Type: research

Allergy in patients with anti-N-methyl-d-aspartate receptor encephalitis.
CONCLUSIONS: Patients with anti-NMDAR encephalitis show higher allergic rate than those with virus encephalitis. Patients with allergy show higher CSF antibody titers and greater illness severity. However, the final outcome of anti-NMDAR encephalitis was not influenced. PMID: 29306850 [PubMed - as supplied by publisher]
Source: Epilepsy and Behaviour - January 4, 2018 Category: Neurology Authors: Jiang XY, Zhang L, Jiang X, Abdulaziz ATA, Wang YH, Li JM, Zhou D Tags: Epilepsy Behav Source Type: research

Comorbidity between central disorders of hypersomnolence and immune-based disorders
Conclusions: NT1 is not associated with increased risk of comorbid immune disorders, in favor of a potentially unique pathophysiology. Conversely, compared with controls, the frequency of autoimmune diseases was higher in adults with NT2, whereas allergies and autoinflammatory disorders were more common in adults with IH, suggesting an immune dysregulation mechanism in these conditions.
Source: Neurology - December 25, 2016 Category: Neurology Authors: Barateau, L., Lopez, R., Arnulf, I., Lecendreux, M., Franco, P., Drouot, X., Leu-Semenescu, S., Jaussent, I., Dauvilliers, Y. Tags: Autoimmune diseases, Narcolepsy, Other hypersomnias, Case control studies ARTICLE Source Type: research

Immunoregulation of multiple sclerosis by gut environmental factors
Abstract Microbiota exist symbiotically in the human gut to aid the digestion of nutrients. However, gut microbiota also have immunoregulatory properties. The hygiene hypothesis describes the idea that environmental factors, such as diet, pollution, antibiotic use, vitamin D insufficiency and increased sanitation/hygiene, can alter the natural development of the immune system, thus making individuals more susceptible to allergy or autoimmune disease. Therefore, factors that are more prevalent in the current environment and living a “modern” lifestyle have depleted important microbiota in the gut that “shape” the de...
Source: Clinical and Experimental Neuroimmunology - August 1, 2015 Category: Neurology Authors: J. Ludovic Croxford, Sachiko Miyake Tags: Review Article Source Type: research

Immune mediated conditions in autism spectrum disorders
Publication date: Available online 11 February 2015 Source:Brain, Behavior, and Immunity Author(s): Ousseny Zerbo , Albin Leong , Lisa Barcellos , Pilar Bernal , Bruce Fireman , Lisa A. Croen We conducted a case-control study among members of Kaiser Permanente Northern California (KPNC) born between 1980 and 2003 to determine the prevalence of immune-mediated conditions in individuals with autism, investigate whether these conditions occur more often than expected, and explore the timing of onset relative to autism diagnosis. Cases were children and young adults with at least two autism diagnoses recorded in outpatient r...
Source: Brain, Behavior, and Immunity - February 16, 2015 Category: Neurology Source Type: research

Immune Mediated Conditions in Autism Spectrum Disorders.
Abstract We conducted a case-control study among members of Kaiser Permanente Northern California (KPNC) born between 1980 and 2003 to determine the prevalence of immune-mediated conditions in individuals with autism, investigate whether these conditions occur more often than expected, and explore the timing of onset relative to autism diagnosis. Cases were children and young adults with at least two autism diagnoses recorded in outpatient records (n=5,565). Controls were children without autism randomly sampled at a ratio of 5 to 1, matched to cases on birth year, sex, and length of KPNC membership (n=27,825). Th...
Source: Brain, Behavior, and Immunity - February 11, 2015 Category: Neurology Authors: Zerbo O, Leong A, Barcellos L, Bernal P, Fireman B, Croen LA Tags: Brain Behav Immun Source Type: research