scholarly journals Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease Presenting as Recurrent and Migrating Focal Cortical Encephalitis

2020 ◽  
Vol 7 ◽  
pp. 2329048X2096617
Author(s):  
Xinran Maria Xiang ◽  
Rachel Evans ◽  
Jesus Lovera ◽  
Rashmi Rao
Keyword(s):  
Brain Mri ◽  
Occipital Cortex ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
New Right ◽  
High Dose ◽  
Antibody Testing ◽  
Leptomeningeal Enhancement ◽  
The Right ◽  
Work Up ◽  
New Onset

Although pediatric myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease is increasingly well-recognized, its full clinical spectrum is still being defined. Cortical encephalitis is emerging as a distinct clinico-radiologic syndrome of adult MOG antibody-associated disease. We describe a 12-year-old girl who presented with new onset seizures and left-sided hemiparesis. Brain MRI showed edema of the right temporal-parietal-occipital cortex with associated focal leptomeningeal enhancement. Patient received high-dose corticosteroids and 21 days of acyclovir despite negative infectious work-up due to the focal nature of encephalitis. Patient remained seizure-free for 20 months before presenting with new right hemiclonic seizures with right-sided hemiparesis and edema of the left temporal-parietal cortex with associated leptomeningeal enhancement. Patient’s MOG antibody titer was 1:40. She completed high-dose corticosteroids and intravenous immunoglobulin. Our patient highlights the importance of MOG antibody testing in pediatric focal cortical encephalitis to avoid unnecessary anti-viral agents and provide more appropriate immunotherapy and a more informed prognosis.

scholarly journals Autonomic Dysregulation, Cognitive Impairment, and Symptoms of Psychosis as an Unusual Presentation in an Anti-Aquaporin 4-Positive Patient

2017 ◽  
Vol 9 (1) ◽  
pp. 12-16 ◽  
Author(s):  
Annabel M. Ruiter ◽  
Jan F. Meilof ◽  
Rosemarijn R.J. Somanje-Bolweg ◽  
Erik van Gorsel ◽  
Nynke F. Kalkers
Keyword(s):  
Cognitive Impairment ◽  
Psychiatric Symptoms ◽  
Brain Mri ◽  
Aquaporin 4 ◽  
High Dose ◽  
Autonomic Dysregulation ◽  
Hyperintense Signal ◽  
High Dose Methylprednisolone ◽  
The Right ◽  
A Minor

We present the unusual case of a patient with an aquaporin 4 antibody-seropositive neuromyelitis optica spectrum disorder who presented with autonomic dysregulation, cognitive impairment, and symptoms of psychosis. Only a few previous cases have been described with similar psychiatric symptoms. Brain MRI showed an abnormal hyperintense T2 signal of the hypothalamus and, to a lesser extent, a minor hyperintense signal of the right optic nerve. Her symptoms and MR abnormalities improved after high-dose methylprednisolone.

scholarly journals Recurrent Kawasaki Disease: A Case Report of Three Separate Episodes at >4-Year Intervals

Children ◽  
2018 ◽  
Vol 5 (11) ◽  
pp. 155 ◽  
Author(s):  
Nikita Goswami ◽  
Katherine Marzan ◽  
Elizabeth De Oliveira ◽  
Sharon Wagner-Lees ◽  
Jacqueline Szmuszkovicz
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Systemic Vasculitis ◽  
High Dose ◽  
Z Score ◽  
Coronary Aneurysms ◽  
Increased Risk ◽  
The Right ◽  
Work Up ◽  
High Dose Aspirin

Kawasaki disease (KD) is a self-limited systemic vasculitis, most often occurring in children 1–5 years old. It has a 2% recurrence rate and is associated with coronary aneurysms (CA), which can develop within two weeks of onset. A 25% increased risk is noted in patients who are recalcitrant to treatment. We describe a patient with recurrence of KD three times, approximately four years apart. A 10-year-old female with two previous episodes of KD, at 11 months and five years of age), in which she met five out of five criteria for KD and had no coronary involvement, presented with 15 days of fever, conjunctivitis and mucocutaneous changes. Infectious work-up was negative, and she was diagnosed with incomplete KD meeting three out of five criteria. An echocardiogram (ECHO) on day 12 revealed dilation of the right coronary artery (RCA) and left coronary artery (LCA). Treatment with intravenous immunoglobulin (IVIG) and high-dose aspirin was started at an outside hospital. After transfer, serial ECHOs showed evolving coronary aneurysms, left anterior descending (LAD) z-score + 8.2 and RCA z-score + 4.0. She received 10 mg/kg infliximab (day 18) and began clopidogrel. A cardiac MRI (day 20) demonstrated progression of the LAD aneurysm, with a z-score + 13, and warfarin was started. To our knowledge, this is the first report of recurrent KD occurring three times at ~5 year intervals.

scholarly journals КЛІНІЧНИЙ ВИПАДОК МОЖЛИВОЇ МІГРЕНІ З АУРОЮ, УСКЛАДНЕНОЇ МІГРЕНОЗНИМ ІНФАРКТОМ

2021 ◽  
Vol 25 (5-6) ◽  
pp. 28-31
Author(s):  
О.С. Нікіфорова ◽  
О.В. Саник ◽  
М.Ю. Дельва
Keyword(s):  
Valproic Acid ◽  
Ischemic Stroke ◽  
Sleep Deprivation ◽  
Migraine Attack ◽  
Clinical Case ◽  
Brain Mri ◽  
Right Visual Field ◽  
Temporal Area ◽  
The Right ◽  
New Onset

We present a clinical case of ischemic stroke in a man with a new-onset migraine attack. Clinical case. A 25-year-old man was admitted to the acute cerebrovascular department due to severe throbbing headache in the left temporal area, vomiting, impaired vision on the right and numbness of the right leg. Complaints appeared abruptly in the form of scotoma in the right visual field. 20 minutes later, severe throbbing headache, nausea and numbness of the right leg developed. The event was preceded by sleep deprivation for 2 days. The patient’s mother has migraine with aura. Previously, the patient never had such attacks. On examination: right-sided homonymous upper-quadrant hemianopsia, hypoesthesia of the right leg. Brain MRI - focal hyperintensity at T2 and DWI in the left occipital region (acute ischemic stroke). The patient took aspirin at a dose of 300 mg for the first day, then 100 mg per day and valproic acid at a dose of 1200 mg per day. Two days after hospitalization, the patient experienced recovery of sensitivity in the right leg and regression of right-sided homonymous upper quadrant hemianopsia to small scotoma. The patient was discharged on the 5th day with a small right scotoma. It is recommended to continue taking aspirin 100 mg/day and valproic acid 1200 mg/day for the secondary prevention of migraine attacks and stroke. Discussion. The peculiarities of this case include the lack of history of migraine (at least 5 or more migrainous headache attacks) and the development of severe migraine attack with prolonged aura symptoms, which was triggered by sleep deprivation and eventually evolved into ischemic stroke. Conclusions. It is necessary to consider the possibility of ischemic stroke even in the cases of a new-onset migraine attack. If migrainous aura duration exceeds 60 minutes, it is necessary to perform a brain MRI to detect the early signs of cerebral ischemia.

scholarly journals Myelin Oligodendrocyte Glycoprotein Antibody Associated Cerebral Cortical Encephalitis: Case Reports and Review of Literature

2022 ◽  
Vol 15 ◽  
Author(s):  
Hang Shu ◽  
Manqiu Ding ◽  
Pei Shang ◽  
Jia Song ◽  
Yue Lang ◽  
...  
Keyword(s):  
Systematic Review ◽  
Demyelinating Disease ◽  
Age Of Onset ◽  
Clinical Phenotype ◽  
Brain Mri ◽  
Clinical Manifestations ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Lessons Learned ◽  
Population Characteristics ◽  
High Dose

Myelin oligodendrocyte glycoprotein antibody-associated disease is an immune-mediated demyelinating disease of the central nervous system that is present in both adults and children. The most common clinical manifestations are optic neuritis, myelitis, acute disseminated encephalomyelitis, and brainstem syndrome. Cerebral cortical encephalitis (CCE) is a rare clinical phenotype of myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD), which usually begins with seizures, headaches, and fever, and may be misdiagnosed as viral encephalitis in the early stages. Herein, we report two typical MOG antibody (MOG-Ab)-positive patients presenting with CCE, both of whom presented with headache, fever, seizures, and who recovered completely after immunotherapy. In addition, we performed a systematic review of the present literature from the perspectives of population characteristics, clinical symptoms, MRI abnormalities, treatments, and prognosis. Among the patients reported in 25 articles, 33 met our inclusion criteria, with the age of onset ranging from 4 to 52 years. Most of the patients had seizures, headache, fever, and unilateral cortical lesions on brain MRI. For acute CCE, 30 patients were treated with high-dose intravenous methylprednisolone, and the symptoms of most patients were completely relieved after immunotherapy. This study reported our experience and lessons learned in the diagnosis and treatment of MOG-Ab-positive CCE and provides a systematic review of the literature to analyse this rare clinical phenotype.

Myelin Oligodendrocyte Glycoprotein Antibody-Associated Optic Neuritis in Canada

2020 ◽  
pp. 1-6
Author(s):  
Anubhav Garg ◽  
Edward Margolin ◽  
Jonathan A. Micieli
Keyword(s):  
Optic Neuritis ◽  
Clinical Characteristics ◽  
Vision Loss ◽  
Brain Mri ◽  
Visual Fields ◽  
Case Series ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Mean Deviation ◽  
High Dose ◽  
Rnfl Thickness

ABSTRACT: Objective: To describe clinical characteristics of Canadian patients with myelin-oligodendrocyte glycoprotein immunoglobulin-G optic neuritis (MOG-IgG ON). Methods: Retrospective observational case series of MOG-IgG seropositive patients with ON referred to tertiary neuro-ophthalmology practices. Outcome measures included clinical characteristics, radiologic findings, and visual outcomes. Results: Forty-six eyes of 30 patients were included. Twenty-three (76.7%) were women, mean onset age was 40.7 years (range 16–77), and most were Caucasian. Seventeen (56.7%) presented with their first ON episode. Sixteen (53.3%) had bilateral eye involvement. Isolated ON without associated neurological symptoms occurred in 90.0%. In 22 patients with acute ON (seen within 1 month of onset), presenting mean visual acuity (VA) was 20/258 (logMAR 1.11), mean deviation (MD) on Humphrey visual fields was −16.90 ± 10.83 dB, and peripapillary retinal nerve fiber layer (RNFL) thickness on ocular coherence tomography (OCT) was 164.23 ± 46.53 um. Orbital magnetic resonance imaging (MRI) within 1 month of symptom onset for 19 patients demonstrated orbital optic nerve enhancement in 11 (57.9%) and perineural enhancement in 11 (57.9%). Brain MRI was normal in 28 (93.3%) patients. Twenty out of 22 patients with acute presentation were treated with high-dose glucocorticoids and 5 with plasma exchange in addition to corticosteroids. Long-term immunosuppression was utilized in 9 (30%) out of all 30 patients. Final VA was 20/30 (logMAR 0.18), MD was −7.17 ± 8.85 dB, and RNFL thickness was 72.15 ± 20.16 um. Conclusion: MOG-IgG ON in Canada has a variable presentation with most patients having substantial initial vision loss with good recovery. This is the largest characterization of the disease in Canada to date.

scholarly journals Leptomeningeal Enhancement due to Neurosarcoidosis Mimicking Malignancy

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Sahara N. Saltijeral ◽  
Horiana B. Grosu ◽  
Henriette De La Garza ◽  
Barbara O’Brien ◽  
Gloria Iliescu
Keyword(s):  
Present Report ◽  
Brain Magnetic Resonance Imaging ◽  
Granulomatous Inflammation ◽  
Needle Aspiration ◽  
High Dose ◽  
Lower Extremity Weakness ◽  
Leptomeningeal Enhancement ◽  
Positron Emission ◽  
Endobronchial Ultrasonography ◽  
The Right

The present report describes the case of a 56-year-old African American man experiencing progressive disequilibrium, lower extremity weakness, difficulty walking, and hearing loss. Brain magnetic resonance imaging showed leptomeningeal enhancement. Initial differential diagnosis was broad, including malignant, infectious, and inflammatory etiologies. The cerebrospinal fluid analyses demonstrated lymphocytic pleocytosis, hypoglycorrhachia, and hyperproteinorrachia but no other abnormalities. An extensive infectious disease workup was negative. Positron emission tomography revealed hypermetabolic lymph nodes in the right mediastinum and right hilum, correlating with findings on endobronchial ultrasonography. Subsequently, image-guided fine-needle aspiration of the right upper paratracheal lymph node was performed, and biopsy studies showed noncaseating granulomatous inflammation. Based on the clinical picture, the diagnosis of neurosarcoidosis was made, and high-dose steroids were started and resulted in significant improvement.

scholarly journals MOG-antibody Related Unilateral Cerebral Cortical Encephalitis With Refractory Cephalalgia: a Case Report

2021 ◽  
Author(s):  
Ning Ren ◽  
Jing Lei ◽  
Haibao Zhu ◽  
Zilong Zhu ◽  
Jie Qin ◽  
...  
Keyword(s):  
Brain Mri ◽  
Occipital Cortex ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
High Signal Intensity ◽  
Clinical Feature ◽  
High Signal ◽  
Fundus Examination ◽  
Intravenous Methylprednisolone ◽  
Refractory Headache

Abstract Background: Myelin oligodendrocyte glycoprotein (MOG) antibody positive utilateral cerebral cortical encephalitis (UCCE) comprises a new entity with heterogeneity which usually present as epilepsy. Cases with cephalalgia as the only clinical feature were rare reported. Here, We report a case with MOG antibody positive UCCE who only presented with refractory cephalalgia and had a good response to glucocorticoid. MOG antibody-related UCCE should be identified when patients present with refractory headache and it may represent benign cortical encephalitis.Case Presentation: The case of a 41-year-old woman with MOG antibody positive UCCE presented with refractory cephalalgia. Neurological examination is normal, and fundus examination suggested bilateral optic nerve head edema. Brain MRI discovered Flair hyperintense lesions in the sulci of right temporoparietal occipital cortex. MOG antibody was positive both in the serum and cerebrospinal fluid. When the patient was treated with intravenous methylprednisolone, her headache completely disappeared and brain MRI showed partly recovery. At the follow-up of 6 months after discharge, she had no relapse, serum MOG antibody was negative and the high signal intensity on Flair had completely disappeared.Conclusions: MOG antibody-positive UCCE with headache as the single clinical symptom may represent benign cortical encephalitis.

scholarly journals Neural Substrates Associated With Fatigue Symptom In Fibromyalgia

2022 ◽  
Author(s):  
Hung-Yu Liu ◽  
Pei-Lin Lee ◽  
Kun-Hsien Chou ◽  
Yen-Feng Wang ◽  
Shih-Pin Chen ◽  
...  
Keyword(s):  
Brain Mri ◽  
Temporal Cortex ◽  
Occipital Cortex ◽  
Neural Substrates ◽  
Inferior Temporal Cortex ◽  
Morphometry Analysis ◽  
Fatigue Severity ◽  
Subcortical Regions ◽  
The Right ◽  
Neural Signatures

Abstract Many patients with fibromyalgia (FM) experience fatigue, but the associated biological mechanisms have not been delineated. We aimed to investigate the neural signatures associated with fatigue severity in patients with FM using MRI. We consecutively recruited 138 patients with FM and collected their clinical profiles and brain-MRI data. We categorized the patients into 3 groups based on their fatigue severity. Using voxel-based morphometry analysis and trend analysis, we first identified neural structures showing volumetric changes associated with fatigue severity, and further explored their seed-to-voxel structural covariance networks (SCNs). Results showed decreased bilateral thalamic volumes were associated with higher severity of fatigue. There was a more widespread distribution of the thalamic SCNs to the frontal, parietal, subcortical, and limbic regions in patients with higher fatigue severity. In addition, increased right inferior temporal cortex volumes were associated with higher severity of fatigue. The right inferior temporal seed showed more SCNs distributions over the temporal cortex and a higher strength of SCNs to the bilateral occipital cortex in patients with higher fatigue severity. The thalamus and the right inferior temporal cortex as well as their altered interactions with cortical and subcortical regions comprise the neural signatures of fatigue in FM.

Eosinophilic vasculitis: an inhabitual and resistant manifestation of a vasculitis

VASA ◽  
2010 ◽  
Vol 39 (4) ◽  
pp. 344-348 ◽  
Author(s):  
Jandus ◽  
Bianda ◽  
Alerci ◽  
Gallino ◽  
Marone
Keyword(s):  
Skin Biopsy ◽  
Intravenous Iron ◽  
Small Vessel Vasculitis ◽  
High Dose ◽  
Raynaud Phenomenon ◽  
The Third ◽  
Left Elbow ◽  
Right Hand ◽  
The Right ◽  
Trunk Skin

A 55-year-old woman was referred because of diffuse pruritic erythematous lesions and an ischemic process of the third finger of her right hand. She was known to have anaemia secondary to hypermenorrhea. She presented six months before admission with a cutaneous infiltration on the left cubital cavity after a paravenous leakage of intravenous iron substitution. She then reported a progressive pruritic erythematous swelling of her left arm and lower extremities and trunk. Skin biopsy of a lesion on the right leg revealed a fibrillar, small-vessel vasculitis containing many eosinophils.Two months later she reported Raynaud symptoms in both hands, with a persistent violaceous coloration of the skin and cold sensation of her third digit of the right hand. A round 1.5 cm well-delimited swelling on the medial site of the left elbow was noted. The third digit of her right hand was cold and of violet colour. Eosinophilia (19 % of total leucocytes) was present. Doppler-duplex arterial examination of the upper extremities showed an occlusion of the cubital artery down to the palmar arcade on the right arm. Selective angiography of the right subclavian and brachial arteries showed diffuse alteration of the blood flow in the cubital artery and hand, with fine collateral circulation in the carpal region. Neither secondary causes of hypereosinophilia nor a myeloproliferative process was found. Considering the skin biopsy results and having excluded other causes of eosinophilia, we assumed the diagnosis of an eosinophilic vasculitis. Treatment with tacrolimus and high dose steroids was started, the latter tapered within 12 months and then stopped, but a dramatic flare-up of the vasculitis with Raynaud phenomenon occurred. A new immunosupressive approach with steroids and methotrexate was then introduced. This case of aggressive eosinophilic vasculitis is difficult to classify into the usual forms of vasculitis and constitutes a therapeutic challenge given the resistance to current immunosuppressive regimens.

Phosphene perceptions and safety of chronic visual cortex stimulation in a blind subject

2020 ◽  
Vol 132 (6) ◽  
pp. 2000-2007 ◽  
Author(s):  
Soroush Niketeghad ◽  
Abirami Muralidharan ◽  
Uday Patel ◽  
Jessy D. Dorn ◽  
Laura Bonelli ◽  
...  
Keyword(s):  
Visual Cortex ◽  
Adverse Events ◽  
Clinical Intervention ◽  
Occipital Cortex ◽  
Neuronal Population ◽  
Serious Adverse Events ◽  
Stimulation Parameters ◽  
The Right ◽  
Visual Cortical ◽  
Stimulation Of

Stimulation of primary visual cortices has the potential to restore some degree of vision to blind individuals. Developing safe and reliable visual cortical prostheses requires assessment of the long-term stability, feasibility, and safety of generating stimulation-evoked perceptions.A NeuroPace responsive neurostimulation system was implanted in a blind individual with an 8-year history of bare light perception, and stimulation-evoked phosphenes were evaluated over 19 months (41 test sessions). Electrical stimulation was delivered via two four-contact subdural electrode strips implanted over the right medial occipital cortex. Current and charge thresholds for eliciting visual perception (phosphenes) were measured, as were the shape, size, location, and intensity of the phosphenes. Adverse events were also assessed.Stimulation of all contacts resulted in phosphene perception. Phosphenes appeared completely or partially in the left hemifield. Stimulation of the electrodes below the calcarine sulcus elicited phosphenes in the superior hemifield and vice versa. Changing the stimulation parameters of frequency, pulse width, and burst duration affected current thresholds for eliciting phosphenes, and increasing the amplitude or frequency of stimulation resulted in brighter perceptions. While stimulation thresholds decreased between an average of 5% and 12% after 19 months, spatial mapping of phosphenes remained consistent over time. Although no serious adverse events were observed, the subject experienced mild headaches and dizziness in three instances, symptoms that did not persist for more than a few hours and for which no clinical intervention was required.Using an off-the-shelf neurostimulator, the authors were able to reliably generate phosphenes in different areas of the visual field over 19 months with no serious adverse events, providing preliminary proof of feasibility and safety to proceed with visual epicortical prosthetic clinical trials. Moreover, they systematically explored the relationship between stimulation parameters and phosphene thresholds and discovered the direct relation of perception thresholds based on primary visual cortex (V1) neuronal population excitation thresholds.

Sign in / Sign up

Export Citation Format

Share Document