scholarly journals Two cases of possible neuro-Sweet disease with meningoencephalitis as the initial manifestation

2012 ◽  
Vol 4 (1) ◽  
pp. 5 ◽  
Author(s):  
Go Makimoto ◽  
Yasuhiro Manabe ◽  
Chizuru Yamakawa ◽  
Daiki Fujii ◽  
Yasuko Ikeda-Sakai ◽  
...  
Keyword(s):  
High Intensity ◽  
Skin Lesions ◽  
Pulse Therapy ◽  
Initial Manifestation ◽  
Diffusion Weighted Images ◽  
Fluid Analysis ◽  
Fluid Attenuated Inversion Recovery ◽  
Left Caudate ◽  
Magnetic Resonance Imaging Mri ◽  
The Right

We report 2 cases that were considered to be neuro-Sweet disease. They initially manifested with meningoencephalitis and no skin lesions, and rapidly improved with corticosteroid therapy. In both cases, patients complained of meningitic symptoms such as fever and headache, and HLA-B54 and -Cw1 turned out to be positive over the clinical course. Cerebrospinal fluid analysis showed increased levels of lymphocytes and protein. In case #1, fluid-attenuated inversion recovery (FLAIR), magnetic resonance imaging (MRI) and diffusion-weighted images (DWI) showed high-intensity signals in the right dorsal medulla oblongata, bilateral dorsal midbrain, and left thalamus. In case #2, FLAIR and DWI showed high-intensity signals in the bilateral cerebellar cortex and left caudate nucleus. Symptoms and MRI images were markedly improved in both cases after corticosteroid pulse therapy. According to published diagnostic criteria, these 2 cases were considered possible neuro-Sweet disease. These cases suggest that the combination of meningoencephalitis and HLA specificity is important to consider the possibility of neuro-Sweet disease, even without skin lesions.

A Septuagenarian With Progressive Hemiparesis

2021 ◽  
pp. 48-50
Author(s):  
Roman Kassa ◽  
B. Mark Keegan
Keyword(s):  
Multiple Sclerosis ◽  
Late Onset ◽  
Progressive Multiple Sclerosis ◽  
Oligoclonal Bands ◽  
Hyperintense Lesion ◽  
Fluid Analysis ◽  
Hemiparetic Gait ◽  
Magnetic Resonance Imaging Mri ◽  
Disease Modifying Agents ◽  
The Right

A 78-year-old man with no pertinent medical history sought care for an 18-month history of progressive right lower extremity weakness, gait impairment, and falls. On neurologic examination, he had a hemiparetic gait. He had normal higher cognitive function and cranial nerve function. Motor examination showed decreased bulk over the right hand with no fasciculations, mild spasticity over the right leg, and right hemiparesis with an upper motor neuron pattern. Deep tendon reflexes were brisk throughout his limbs, and he had an extensor plantar reflex on the right side. He had impaired vibratory sense at the toes, with otherwise normal sensory and coordination examinations. Magnetic resonance imaging (MRI) of the brain showed ovoid periventricular and punctate subcortical and deep white matter T2 hyperintense foci. Some of these had corresponding T1 hypointensity. MRI of the cervical spine showed 1 eccentrically located T2 hyperintense lesion over the right lateral aspect of C2. Cerebrospinal fluid analysis showed no pleocytosis, an increased protein concentration of 66 mg/dL, and 4 unique oligoclonal bands. A diagnosis of primary progressive multiple sclerosis, very late onset, was made. With any diagnosis of late-onset multiple sclerosis, a decision about whether multiple sclerosis disease-modifying agents are indicated should be carefully considered. Our older patient had a progressive disease course, and neuroimaging studies did not reveal evidence of active disease. Based on this, a decision was made to monitor him clinically and radiologically. Management of spasticity with regular daily stretching exercises was discussed with him. A first clinical manifestation of multiple sclerosis can occur at a later-than-typical age. Most studies consider an onset at age 50 years or older to be late-onset multiple sclerosis, whereas first symptoms occurring at age 60 years or older are commonly referred to as very late–onset MS.

scholarly journals Overlapping syndrome mimicking infectious meningoencephalitis in a patient with MOG and GFAP IgG

2020 ◽  
Author(s):  
Su-qiong Ji ◽  
Chen-chen Liu ◽  
Bi-tao Bu
Keyword(s):  
White Matter ◽  
Oral Prednisolone ◽  
Pulse Therapy ◽  
Tuberculosis Treatment ◽  
Early Screening ◽  
Steroid Pulse ◽  
Csf Analysis ◽  
Magnetic Resonance Imaging Mri ◽  
The Right

Abstract Background The presence of CNS overlapping autoimmune syndrome is not uncommon, but only one case of overlapping syndrome with coexistence of MOG-IgG and GFAP-IgG had been reported. This is the first reported case of these double antibodies positive presenting as clinical meningoencephalitis. Case presentation: A 23-year-old woman presented with transient convulsions,loss of consciousness, persistent fever, headache and vomiting. The cerebrospinal fluid (CSF) analysis revealed elevated cellularity, Magnetic resonance imaging (MRI) showed diffuse leptomeningeal enhancement. She remained fever and headache with antiviral and antibiotic treatment for two weeks, then was treated with empirical anti-tuberculosis treatment and oral prednisolone therapy. She followed up at 3 months from presentation with symptoms improved and normal CSF analysis. 3-month follow-up MRI performed asymmetric lesions in the cerebellum, corona radiata, and white matter with enhancement. Anti-tuberculosis treatment was continued and steroid was discontinued. After She stopped taking the prednisolone, interrupted headache gradually appeared. MRI at 4 months after presentation revealed partial reduced extent of lesions, but enlarged areas in left cerebellum and right parietal white matter, as well as a new lesion in the region of the right ependyma with linearly enhancement. Screening for anti-myelin oligodendrocyte glycoprotein (MOG) antibody and anti-glial fibrillary acidic protein (GFAP) antibody were positive in CSF by transfected cell-based assay. She was diagnosed with overlapping syndrome of MOG‑IgG‑associated disease and GFAP astrocytopathy and received steroid pulse therapy (methylprednisolone 1 g for 5 days) followed by a gradual tapering of oral prednisolone, as well as addition of immunosuppressant (tacrolimus, 3 mg per day). 6 months after the patient’s initial presentation, no symptom was found, MRI showed the lesions had obviously diminished and no enhancement was found. Conclusions To our knowledge, this is the first reported case of overlapping syndrome with coexistence of MOG-IgG and GFAP-IgG presenting as clinical meningoencephalitis. The early screening of autoantibodies against CNS antigens was of great importance for the patient suspected of intracranial infection to make the definite diagnosis.

scholarly journals Case Report: Anti-NMDAR Encephalitis With Anti-MOG CNS Demyelination After Recurrent CNS Demyelination

2021 ◽  
Vol 12 ◽  
Author(s):  
Bing-Yan Ren ◽  
Yi Guo ◽  
Jing Han ◽  
Qian Wang ◽  
Zai-Wang Li
Keyword(s):  
Case Report ◽  
Brain Mri ◽  
Clinical Manifestations ◽  
Epileptic Seizures ◽  
Autoimmune Disorder ◽  
Pulse Therapy ◽  
Nmdar Encephalitis ◽  
Cns Demyelination ◽  
Magnetic Resonance Imaging Mri ◽  
The Right

Introduction: Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis, a serious neurological autoimmune disorder caused by autoantibodies with diverse clinical manifestations, may simultaneously onset with antimyelin oligodendrocyte glycoprotein (MOG) demyelination after recurrent central nervous system (CNS) demyelination.Case Report: We present a case of anti-NMDAR encephalitis combining with anti-MOG CNS demyelination following recurrent CNS demyelination. A 38-year-old man admitted to hospital developed epileptic seizures following recurrent episodes of cross-sensory disturbance and dizziness. Magnetic resonance imaging (MRI) showed a demyelinating lesion in the right brainstem initially. Despite a good response to methylprednisolone pulse therapy at the beginning, the patient still had relapses and progression after corticosteroid reduction or withdrawal. Then brain MRI discovered new serpentine lesions involving extensive cerebral cortex on his second relapse. Repeat autoantibodies test indicated cerebrospinal fluid (CSF) NMDAR antibodies coexisted with MOG-Abs simultaneously, suggesting the diagnosis of anti-NMDAR encephalitis with anti-MOG CNS demyelination.Results: After a definite diagnosis, the patient was treated with mycophenolate mofetil (MMF) and corticosteroid. He was discharged after his symptoms ameliorated. No neurological sequels remained, and there were no effects on his activities of daily living after 6 months of immunoregulatory therapy of MMF and corticosteroid.Conclusion: For individuals with recurrent CNS demyelination, especially combining with cortical encephalitis, repeated detection of autoantibodies against AE, and demyelination in CSF/serum can be helpful to enable a definite early diagnosis. For patients who suffer from anti-NMDAR encephalitis combining with anti-MOG CNS demyelination, second-line immunotherapy is recommended when first-line treatment such as steroids, intravenous immunoglobulin G (IVIG) and plasma exchange has been proven ineffective to prevent the relapse of disease.

scholarly journals Overlapping syndrome mimicking infectious meningoencephalitis in a patient with MOG and GFAP IgG

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Suqiong Ji ◽  
Chenchen Liu ◽  
Zhuajin Bi ◽  
Huajie Gao ◽  
Jian Sun ◽  
...  
Keyword(s):  
White Matter ◽  
Oral Prednisolone ◽  
Pulse Therapy ◽  
Tuberculosis Treatment ◽  
Case Presentation ◽  
Steroid Pulse ◽  
Csf Analysis ◽  
Magnetic Resonance Imaging Mri ◽  
The Right

Abstract Background Central nervous system overlapping autoimmune syndromes are uncommon, especially with the coexistence of MOG-IgG and GFAP-IgG. Case presentation A 23-year-old woman presented with transient convulsions, a loss of consciousness, persistent fever, headache, and vomiting. Cerebrospinal fluid (CSF) analysis revealed elevated cellularity, and magnetic resonance imaging (MRI) showed diffuse leptomeningeal enhancement. She had fever and headache with antiviral and antibiotic treatment for 2 weeks, and she had empirical anti-tuberculosis treatment and oral prednisolone therapy. She was followed for 3 months after presentation with improved symptoms and normal CSF analysis. A 3-month follow-up MRI showed asymmetric lesions in the cerebellum, corona radiata, and white matter with enhancement. The anti-tuberculosis treatment was continued, and steroid therapy was discontinued. After she stopped taking prednisolone, an interrupted headache gradually appeared. MRI at 4 months after presentation revealed a partial reduction in lesions but enlarged areas in the left cerebellum and right parietal white matter and a new lesion in the region of the right ependyma with linear enhancement. Her CSF was positive for anti-myelin oligodendrocyte glycoprotein (MOG) and anti-glial fibrillary acidic protein (GFAP) antibodies using a transfected cell-based assay. She was diagnosed with overlapping syndrome of MOG‑IgG‑associated disease and GFAP astrocytopathy. She received steroid pulse therapy (methylprednisolone, 1 g for 5 days), followed by a gradual tapering of oral prednisolone and the addition of an immunosuppressant (tacrolimus, 3 mg per day). Six months after the initial presentation, she had no symptoms. An MRI showed that the lesions had diminished, and no enhancement was found. Conclusions We report a case that was positive for double antibodies, which was initially misdiagnosed as infectious meningoencephalitis. This case broadens the clinical and phenotypic presentation of the overlapping syndrome spectrum.

Repeated Cerebral Infarction Immediately after Bypass Graft Angiography: A Case Report

2021 ◽  
Author(s):  
Masaki Monden ◽  
Nobuhiro Murata ◽  
Kurara Takahashi ◽  
Daisuke Fukamachi ◽  
Yasuo Okumura
Keyword(s):  
Cerebral Infarction ◽  
High Intensity ◽  
Bypass Graft ◽  
Occipital Lobe ◽  
Common Complication ◽  
Resonance Imaging ◽  
Left Thalamus ◽  
Noninvasive Assessment ◽  
Magnetic Resonance Imaging Mri ◽  
The Right

AbstractA cerebral infarction (CI) is a common complication of coronary angiography (CAG); however, repeated CIs in the immediate period after a CAG have not been reported yet. The patient in the present study experienced left upper quadrant blindness immediately after internal thoracic arteriography, and magnetic resonance imaging (MRI) showed a high-intensity area in the right occipital lobe. Despite the administration of antithrombotic therapy, the patient became transiently comatose for 6 hours. MRI showed a new high-intensity area in the left thalamus. A noninvasive assessment should be considered before internal thoracic arteriography to prevent the risk of complications.

scholarly journals Diffusion-Weighted Imaging Findings of Ischemic Spinal Injury in a Chondrodystrophic Dog With Fibrocartilaginous Embolism

2020 ◽  
Vol 7 ◽  
Author(s):  
Taesik Yun ◽  
Kang-Il Lee ◽  
Yoonhoi Koo ◽  
Hakhyun Kim ◽  
Dongwoo Chang ◽  
...  
Keyword(s):  
Spinal Injury ◽  
Mri Findings ◽  
Intact Male ◽  
Post Contrast ◽  
Adc Value ◽  
Fluid Analysis ◽  
Fibrocartilaginous Embolism ◽  
Apparent Diffusion ◽  
Fluid Attenuated Inversion Recovery ◽  
Magnetic Resonance Imaging Mri

A 9-year-old, intact male Shih Tzu dog presented with systemic weakness and peracute onset of tetraplegia. Tetraplegia with lower motor neuron signs was noted upon neurological examination. Diseases that cause acute flaccid tetraparesis, such as acute fulminating myasthenia gravis, polyradiculoneuritis, tick paralysis, and botulism, were ruled out based on the medical history, normal electrophysiological tests, and non-response to the neostigmine challenging test. Initial 0.3-Tesla (T) magnetic resonance imaging (MRI) findings included sharply demarcated intramedullary lesions at the C3-C6 level, mainly involving gray matter, which appeared hypo- to iso- intense on T1-weighted images (WIs), and hyperintense on T2-WIs and fluid-attenuated inversion recovery images. There was no enhancement on post-contrast T1-WIs. Neutrophilic pleocytosis was observed in the cerebrospinal fluid analysis. No clinical responses were observed for the treatment of non-infectious myelitis with an immunosuppressive dosage of prednisolone. A follow-up 3-T MRI 6 days later demonstrated hyperintensity on diffusion-WI (DWI) and a decreased apparent diffusion coefficient (ADC) value (0.54 × 10−3 mm2/s) of the spinal lesions. Through histological examination, a fibrocartilaginous embolism was definitively confirmed. This is the first report to describe an ischemic spinal injury visualized by DWI and ADC mapping with high-field MRI in a chondrodystrophic dog diagnosed with a fibrocartilaginous embolism.

scholarly journals Neuroimaging Insights Into Early Stages of HIV-Progressive Multifocal Leukoencephalopathy: A Case Report

2020 ◽  
Vol 12 ◽  
pp. 117957352093933
Author(s):  
Natalia Gonzalez Caldito ◽  
J Scott Loeb ◽  
Darin T Okuda
Keyword(s):  
Progressive Multifocal Leukoencephalopathy ◽  
Early Recognition ◽  
Brain Mri ◽  
Disease Evolution ◽  
Early Stages ◽  
Mri Features ◽  
Fluid Attenuated Inversion Recovery ◽  
Magnetic Resonance Imaging Mri ◽  
Punctate Pattern ◽  
The Right

This report aims to enhance the understanding of early longitudinal neuroimaging features of progressive multifocal leukoencephalopathy (PML) in human immunodeficiency virus (HIV). Neuroimaging has become crucial in the diagnosis and early recognition of PML. Recognition of magnetic resonance imaging (MRI) features in the early stages of PML is paramount to avoid misdiagnosis and facilitate the delivery of treatments aimed at reducing disease progression. A 49-year-old white man with HIV presented with 4-month progressive left-sided weakness. Neurological examination revealed mild cognitive impairment, left-sided hemiparesis, and somatosense impairment to all modalities. Brain MRI revealed a punctate pattern with innumerable T2-FLAIR (fluid attenuated inversion recovery) hyperintensities in the cortex, brainstem, cerebellum, subcortical, and periventricular areas. Susceptibility-weighted imaging (SWI) revealed hypointensities involving subcortical U-fibers and cortical architecture. A comprehensive diagnostic evaluation was inconclusive. John Cunningham virus (JCV) PCR in cerebrospinal fluid (CSF) was indeterminate. He was started on antiretroviral therapy. Repeat brain MRI performed 1.5 months later, in the setting of further neurological decline, demonstrated progression of the T2-hyperintensities into a large confluent white matter lesion in the right frontoparietal lobe. Despite an indeterminate JCV PCR, the appearance and characteristic progression of the lesions in successive imaging in the setting of severe immunosuppression, with extensive negative infectious workup, was indicative of PML. This clinical experience illustrates unique neuroimaging features of HIV-PML in early stages and its progression over time. It especially highlights the relevance of the SWI sequence in the diagnosis and features observed with disease evolution. Short-term imaging follow-up may assist with the recognition of MRI features consistent with the biology of the infection.

scholarly journals Removal of evidential motion-contaminated and poorly fitted image data improves IVIM diffusion MRI parameter scan–rescan reproducibility

2018 ◽  
Vol 59 (10) ◽  
pp. 1157-1167 ◽  
Author(s):  
Olivier Chevallier ◽  
Nan Zhou ◽  
Jian He ◽  
Romaric Loffroy ◽  
Yì Xiáng J Wáng
Keyword(s):  
Coefficient Of Variation ◽  
Image Data ◽  
Liver Parenchyma ◽  
B Value ◽  
Least Square ◽  
Diffusion Weighted Images ◽  
Perfusion Fraction ◽  
Reproducibility Study ◽  
Magnetic Resonance Imaging Mri ◽  
The Right

Background It has been reported that intravoxel incoherent motion (IVIM) diffusion magnetic resonance imaging (MRI) scan–rescan reproducibility is unsatisfactory. Purpose To study IVIM MRI parameter reproducibility for liver parenchyma after the removal of motion-contaminated and/or poorly fitted image data. Material and Methods Eighteen healthy volunteers had liver scans twice in the same session to assess scan–rescan repeatability, and again in another session after an average interval of 13 days to assess reproducibility. Diffusion-weighted images were acquired with a 3-T scanner using respiratory-triggered echo-planar sequence and 16 b-values (0–800 s/mm2). Measurement was performed on the right liver with segment-unconstrained least square fitting. Image series with evidential anatomical mismatch, apparent artifacts, and poorly fitted signal intensity vs. b-value curve were excluded. A minimum of three slices was deemed necessary for IVIM parameter estimation. Results With a total 54 examinations, six did not satisfy inclusion criteria, leading to a success rate of 89%, and 14 volunteers were finally included for the repeatability/reproducibility study. A total of 3–10 slices per examination (mean = 5.3 slices, median = 5 slices) were utilized for analysis. Using threshold b-value = 80 s/mm2, the coefficient of variation and within-subject coefficient of variation for repeatability were 2.86% and 3.36% for Dslow, 3.81% and 4.24% for perfusion fraction (PF), 18.16% and 24.88% for Dfast; and those for reproducibility were 2.48% and 3.24% for Dslow, 4.91% and 5.38% for PF, and 21.18% and 30.89% for Dfast. Conclusion Removal of motion-contaminated and/or poorly fitted image data improves IVIM parameter reproducibility.

scholarly journals Early Motor Fluctuations in a Patient with Striatonigral Degeneration

2016 ◽  
Vol 8 (3) ◽  
pp. 243-250
Author(s):  
Fumihito Yoshii ◽  
Yusuke Moriya ◽  
Tomohide Ohnuki ◽  
Wakoh Takahashi ◽  
Masafuchi Ryo
Keyword(s):  
Marked Decrease ◽  
Emission Tomography ◽  
Resonance Imaging ◽  
Striatonigral Degeneration ◽  
Recovery Sequence ◽  
Positron Emission ◽  
Fluid Attenuated Inversion Recovery ◽  
Wearing Off ◽  
Magnetic Resonance Imaging Mri ◽  
The Right

We report a 44-year-old female with striatonigral degeneration (SND) who showed wearing-off oscillations after 4 months of levodopa treatment. The patient presented with asymmetric left-side dominant rigidity, and levodopa was effective at first. However, she began to show wearing-off oscillations of motor symptoms, which gradually worsened thereafter. Fluid-attenuated inversion recovery sequence magnetic resonance imaging (MRI) showed linear lateral putamen hyperintensities, and positron emission tomography (PET) studies using 18F-fluorodopa (FD) and 11C-N-methylspiperon (NMSP) showed a marked decrease of radioactivity in the right putamen, especially in the posterior putamen. The results of MRI and 2 PET studies with FD and NMSP were well consistent with the diagnosis of SND.

scholarly journals Bilateral lateral ventricular subependymoma with extensive multiplicity presenting with hemorrhage

2017 ◽  
Vol 31 (1) ◽  
pp. 27-31
Author(s):  
FM Moinuddin ◽  
Novita Ikbar Khairunnisa ◽  
Hirofumi Hirano ◽  
Tomoko Hanada ◽  
Tsubasa Hiraki ◽  
...  
Keyword(s):  
Medial Temporal Lobe ◽  
Isocitrate Dehydrogenase 1 ◽  
Generalized Seizures ◽  
Hemosiderin Deposition ◽  
Thyroid Lobectomy ◽  
Fluid Attenuated Inversion Recovery ◽  
Magnetic Resonance Imaging Mri ◽  
The Right ◽  
The Masses ◽  
Hemosiderin Deposits

This 48-year-old-man who had undergone right thyroid lobectomy for undifferentiated thyroid carcinoma nine years earlier developed generalized seizures. His cerebrospinal fluid was xanthochromic with elevation of total protein. Computed tomography (CT) showed mixed-density bilateral ventricular masses. Magnetic resonance imaging (MRI) revealed multiple nodules in both lateral ventricles; they were heterogeneously enhanced by gadolinium. Diffuse hyperintensity in the right medial temporal lobe and bilateral subependymal area was noted on fluid-attenuated inversion recovery images. Susceptibility-weighted imaging showed low intensity in the masses and cerebellar sulci suggesting hemorrhage and hemosiderin deposition. The preoperative diagnosis was disseminated malignant tumor with recurring hemorrhage. Histological examination of biopsy specimens showed clusters of cells with small uniform nuclei embedded in a dense fibrillary matrix of glial cells and microcystic degeneration. Pseudo-rosettes indicating ependymoma were absent. Microhemorrhages and hemosiderin deposits were noted. Immunohistochemically, the background fibrillary matrix and neoplastic cells were positive for glial fibrillary acidic protein. Mutated isocitrate dehydrogenase-1 was negative. The MIB-1 index was 1.5%. The tumor was pathologically diagnosed as subependymoma containing microhemorrhages and hemosiderin deposits. The extensive multiplicity and hemorrhage encountered in this case have rarely been reported in patients with subependymoma.

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