scholarly journals Clinical and Radiological Features of Adult Onset Bilateral Medial Frontal Cerebral Cortical Encephalitis With Anti-myelin Oligodendrocyte Glycoprotein Antibody

2020 ◽  
Vol 11 ◽  
Author(s):  
Juichi Fujimori ◽  
Masashi Nakamura ◽  
Takahito Yagihashi ◽  
Ichiro Nakashima
Keyword(s):  
Basic Protein ◽  
Clinical Symptoms ◽  
Brain Mri ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Cortical Lesions ◽  
Adult Onset ◽  
Mri Findings ◽  
Radiological Features ◽  
Csf Pleocytosis ◽  
Memory Disturbance

Objective: To clarify the clinical and radiological features of adult onset anti-myelin oligodendrocyte glycoprotein (MOG) antibody-associated bilateral medial frontal cerebral cortical encephalitis (BFCCE).Methods: We systematically reviewed the literature for patients with anti-MOG antibody-associated BFCCE. Patients who were also positive for other encephalitis-related autoantibodies were excluded from the study. The frequency of several characteristic neurological symptoms and lesion distributions were analyzed.Results: We identified six patients with anti-MOG antibody-associated BFCCE. Among them, 6/6 had headache, 4/6 had fever, 3/6 had seizure, 2/6 had paraparesis, 2/6 had lethargy, and 2/6 had memory disturbance. CSF pleocytosis was observed in 5/6 patients, while CSF myelin basic protein was not elevated in any of the six patients. On brain MRI, 6/6 had bilateral medial frontal cortical lesions, 3/6 had corpus callosum lesions, and 3/6 had leptomeningeal enhancements. Most of the lesions were distributed in the territory of the anterior cerebral artery (ACA).Conclusion: Our results indicate that anti-MOG antibody-associated BFCCE presents with characteristic clinical symptoms and MRI findings, which might reflect lesion formation in the ACA territory.

A patient with adult-onset type II citrullinemia on long-term hemodialysis: Reversal of clinical symptoms and brain MRI findings

2002 ◽  
Vol 39 (1) ◽  
pp. 189-192 ◽  
Author(s):  
Saori Oshiro ◽  
Tutomu Kochinda ◽  
Takeshi Tana ◽  
Masanobu Yamazato ◽  
Keiko Kobayashi ◽  
...  
Keyword(s):  
Clinical Symptoms ◽  
Brain Mri ◽  
Type Ii ◽  
Adult Onset ◽  
Mri Findings ◽  
Onset Type

Myelin basic protein and myelin oligodendrocyte glycoprotein T-cell repertoire in childhood and juvenile multiple sclerosis

2006 ◽  
Vol 12 (4) ◽  
pp. 412-420 ◽  
Author(s):  
Jorge Correale ◽  
Silvia N Tenembaum
Keyword(s):  
Multiple Sclerosis ◽  
T Cell ◽  
Myelin Basic Protein ◽  
Basic Protein ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
T Cell Repertoire ◽  
Adult Onset ◽  
Cell Repertoire ◽  
Juvenile Onset ◽  
Exon 2

Multiple sclerosis (MS) is usually a disease of young adulthood, its clinical onset occurring between 20 and 40 years of age; however, today there is general consensus that MS can also occur in children, adolescents and even in infants. In order to gain further insight into the T-cell repertoire present in this particular group of patients myelin basic protein (MBP)-, MBP exon-2- and myelin oligodendrocyte glycoprotein (MOG)Igd-specific T-cell lines (TCLs) were isolated from 18 patients whose symptoms had started before the age of 16. Epitope specificity was established by measuring proliferative responses, and interferon-g (IFN-g) secretion by using a panel of overlapping synthetic peptides. For MOGIgd, the T-cell response was focused on three main immunodominant epitopes comprising residues 1-26, 36-60 and 63-87. For MBP the predominant immune responses were directed against peptides 83-102, 139-153 and 146-162. When compared to those observed in adult-onset MS patients, anti-MOGIgd specificity and anti-MBP responses showed similar results. Moreover, the number of MBP exon-2 TCLs isolated, and the magnitude of the specific IFN-g secretion induced were similar, both in childhood/juvenile-onset and adult-onset MS patients. Thus, despite differences in the clinical and neuroimaging manifestations of MS, these results would seem to indicate that both the spectrum of MBP found, as well as the MOGIgd epitopes recognized by peripheral blood T cells in MS, appear to be similar for childhood/juvenile-onset and adult-onset patients.

scholarly journals Is Brain MRI Needed in Diagnostic Evaluation of Mild Intellectual Disability?

2020 ◽  
Author(s):  
Miro Pekka Jussila ◽  
Päivi Olsén ◽  
Jaakko Niinimäki ◽  
Maria Suo-Palosaari
Keyword(s):  
Intellectual Disability ◽  
Clinical Symptoms ◽  
Brain Mri ◽  
Neurological Symptoms ◽  
Diagnostic Evaluation ◽  
Mild Intellectual Disability ◽  
Mri Findings ◽  
Patient Reports ◽  
Epilepsy Diagnosis ◽  
Abnormal Head

Abstract Aim The purpose of our study was to suggest an imaging strategy and guidelines for the selection of the children with mild intellectual disability (ID) for magnetic resonance imaging (MRI), to avoid unnecessary imaging. Methods The brain MRIs and patient reports of 471 children were reviewed for the imaging findings and ID severity. The correlation between the clinical and brain MRI findings was analyzed in the 305 children with mild ID. Results Thirty-eight (12.5%) of the children with mild ID had significant abnormal brain MRI findings. Thirty-five of these had other neurological symptoms or diseases in addition to ID, which were an indication for brain MRI. In the logistic regression analysis, seizures (in patients without an epilepsy diagnosis), epilepsy, movement disorders, dysmorphia, encephalitis, traumatic brain injury, and abnormal head size were statistically significant symptoms or comorbidities associated with abnormal MRI findings. Only three children (1.0%) with mild ID had a significant MRI finding without any other clinical symptoms or disease. Conclusion Routine MRI in children with mild ID without specific neurological symptoms, dysmorphic features, or related diseases is not suggested for revealing an etiology of mild ID. Since children with ID usually need to be sedated for MRI, routine imaging in the diagnostic evaluation of mild ID should be carefully considered. Clinical examination, other symptoms, and related diseases should be carefully assessed to decide the need for MRI.

Abstract WP483: Grouped Cortical Micro Bleeds a Unique Radiological Marker in Inflammatory Cerebral Amyloid Angiopathy

Stroke ◽  
2020 ◽  
Vol 51 (Suppl_1) ◽  
Author(s):  
Sanjay Mittal ◽  
Karan Tarasaria
Keyword(s):  
Cognitive Decline ◽  
Cerebral Amyloid Angiopathy ◽  
Neurological Examination ◽  
Brain Mri ◽  
Case Series ◽  
Amyloid Angiopathy ◽  
Gadolinium Enhancement ◽  
Mri Findings ◽  
Radiological Features ◽  
Cerebral Amyloid

Background: Inflammatory Cerebral Amyloid Angiopathy (iCAA) is a rare cause of rapidly progressive cognitive decline. Prior studies have proposed radiological diagnostic criteria and demonstrated evidence of inflammation related to Aβ. Though lacking any therapeutic trials, various authors have suggested a single pulse IV Methyl Prednisolone (MPS) therapy as curative in most patients. Methods: We studied clinical, radiological and neuropathological features of 2 of biopsy proven iCAA cases over the past 5 years correlating their neuropathological and radiological features. We also reviewed published literature for radiological findings of proven cases of ICAA Clinical features Case 1: 75/female presented with visual hallucinations, her neurological examination was un-remarkable >Case 2: 84/female presented with language and memory difficulties her neurological examination was remarkable for word finding and object naming difficulties Radiological Features: Gadolinium enhanced brain MRI with fluid attenuated inversion recovery (FLAIR) and susceptibility weighted imaging (SWI) were obtained Patient 1: SWI artifact s in bilateral cerebral hemispheres basal ganglia and cerebellum suggestive of cortical micro-bleeds (CMBs ) there was a cluster of CMBs in the right parietal region associated with FLAIR changes and gadolinium enhancement Patient 2 numerous scattered cortical micro-bleeds (CMBs). There was a large cluster of CMBs in left temporal lobe along with FLAIR changes and gadolinium enhancement Biopsy: B oth patient underwent lesional biopsy corresponding to the area of clustered CMBs Neuropathology: demonstrated histopathological and electron microscopic evidence of perivascular inflammation and amyloid deposition consistent with iCAA Review of literature : In a total of 7 brain MRI s in 3 recently published case series 4 had reported CMBs of which 3 patients were identified representing MRI findings consistent with Grouped CMBs associated with FLAIR changes Conclusion: iCAA is a rare and treatable cause of subacute cognitive decline. We suggest grouped cortical micro bleeds (GCMBs) associated with FLAIR changes as a unique radiological feature of this disorder.

scholarly journals Myelin oligodendrocyte glycoprotein-antibody (MOG-IgG) associated disease with centrally located long spinal cord lesion in a 14-month old child

2020 ◽  
Vol 12 ◽  
pp. 117957352095500
Author(s):  
Mohamad Syafeeq Faeez Md Noh
Keyword(s):  
Spinal Cord ◽  
Acute Disseminated Encephalomyelitis ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Spinal Cord Lesion ◽  
Igg Antibodies ◽  
Mri Findings ◽  
Radiological Features ◽  
Spinal Mri ◽  
Children And Young Adults ◽  
Cord Lesion

Myelin oligodendrocyte glycoprotein-antibodies (MOG-IgG) are associated with acquired inflammatory demyelinating syndromes, seen predominantly in children and young adults. The overlapping clinical and radiological features of the heterogenous spectrum of demyelinating central nervous system (CNS) diseases makes the detection of MOG-IgG antibodies important for prognosis and treatment decisions. Herein, we describe the occurrence of MOG-IgG associated disease presenting as acute disseminated encephalomyelitis (ADEM), with spinal MRI findings of centrally located long cord lesion in a 14-month old child.

Headache among CADASIL patients with R544C mutation: Prevalence, characteristics, and associations

Cephalalgia ◽  
2013 ◽  
Vol 34 (1) ◽  
pp. 22-28 ◽  
Author(s):  
Jay Chol Choi ◽  
Sook-Keun Song ◽  
Jung Seok Lee ◽  
Sa-Yoon Kang ◽  
Ji-Hoon Kang
Keyword(s):  
Clinical Features ◽  
Clinical Symptoms ◽  
Brain Mri ◽  
Brain Magnetic Resonance Imaging ◽  
Cross Sectional Study ◽  
Tension Type Headache ◽  
Mri Findings ◽  
Cross Sectional ◽  
Asian Patients ◽  
Type Headache

Background and aim There are variations of migraine prevalence in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) among different regions of the world. Previous studies on Asian CADASIL patients have not provided detailed descriptions of the characteristics of their headaches. The aims of this study were to determine prevalence and characteristics of headaches and to investigate associations between headache and other clinical symptoms or brain magnetic resonance imaging (MRI) findings among homogenous group of patients having the same R544C mutation. Methods In this cross-sectional study, we enrolled 53 CADASIL patients with the R544C mutation between May 2010 and April 2011. We obtained the history of headache using a structured questionnaire and detailed interview. Other clinical features and brain MRI findings were also assessed for potential associations. Results Overall headache prevalence was 45.3% (24 patients). Among them, 21 patients (87.5%) were classified as having tension-type headache, followed by migraine (two patients) and unclassifiable headaches (one patient). Except for alcohol consumption, no significant associations were observed between the headaches and other clinical features or brain MRI findings. Conclusion Migraine was found in only 3.8% of CADASIL patients with the R544C mutation. Such a low prevalence of migraine may hinder clinical detection of CADASIL among ethnically Asian patients.

Persisting myelin oligodendrocyte glycoprotein antibodies in aquaporin-4 antibody negative pediatric neuromyelitis optica

2012 ◽  
Vol 19 (8) ◽  
pp. 1052-1059 ◽  
Author(s):  
K Rostásy ◽  
S Mader ◽  
EM Hennes ◽  
K Schanda ◽  
V Gredler ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Pediatric Patients ◽  
Temporal Dynamics ◽  
Clinical Symptoms ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Demyelinating Diseases ◽  
Aquaporin 4 ◽  
Igg Antibodies ◽  
Mri Findings ◽  
Distinct Subgroup

Background: Recently we showed that antibodies to myelin oligodendrocyte glycoprotein (MOG) can be found in aquaporin-4 (AQP4)-immunoglobulin (IgG) seronegative pediatric and adult patients with definite and high-risk neuromyelitis optica (NMO). Objective: The purpose of this study was to describe the clinical characteristics and temporal dynamics of MOG-IgG in AQP4-IgG seronegative pediatric patients presenting with definite NMO. Methods: Children with definite NMO who were referred for further testing of serum antibodies for AQP4 and MOG with a cell-based assay were included in this study. Clinical disease course, cerebrospinal fluid and magnetic resonance imaging (MRI) studies of these patients were reviewed. Results: Between 2008 and 2012 eight children who fulfilled the diagnostic criteria of definite NMO were recruited. Two children with definite NMO tested positive for AQP4-IgG but were negative for MOG-IgG antibodies. Three children had an absence of AQP4-IgG and MOG-IgG antibodies. Three children with definite NMO had high titers of serum MOG-IgG antibodies (≥1: 160), but no AQP4-directed humoral immune response. Longitudinal analysis of serum samples of the latter three children showed persisting high MOG-IgG titers over time. Conclusion: Pediatric patients presenting with clinical symptoms and MRI findings highly suggestive of NMO but with high and persisting MOG-IgG antibody titers are most likely to represent a distinct subgroup of acute demyelinating diseases with important clinical and therapeutic implications.

scholarly journals Misdiagnosis: CNS Erdheim–Chester disease mimicking CLIPPERS

2017 ◽  
Vol 31 (4) ◽  
pp. 399-402 ◽  
Author(s):  
Jillian Berkman ◽  
Caleb Ford ◽  
Emily Johnson ◽  
Beth A Malow ◽  
Joseph M Aulino
Keyword(s):  
Case Report ◽  
Clinical Symptoms ◽  
Brain Mri ◽  
Subcortical White Matter ◽  
Mri Findings ◽  
Erdheim Chester Disease ◽  
Benign Process ◽  
Magnetic Resonance Imaging Mri ◽  
Erdheim Chester ◽  
Chester Disease

Introduction Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory central nervous system (CNS) disorder with particular involvement of the pons. Diagnostic criteria include a range of clinical symptoms related to the underlying brainstem pathology, visible with magnetic resonance imaging (MRI). MRI findings include the appearance of punctuate and curvilinear gadolinium enhancement ‘peppering’ the pons. We discuss a patient presenting with clinical and radiographic characteristics of CLIPPERS who was diagnosed with Erdheim–Chester disease (ECD). Case report A 52-year-old male presented with 2 years of progressive spasticity, dysarthria, and gait instability. Initially, he was diagnosed with Parkinson’s disease at an outside hospital, based on tremor, rigidity, and gait instability; however, he failed to improve with a trial of levodopa. Brain MRI showed small enhancing parenchymal nodules coalescing in the central pons, but also affecting the cerebellum and cerebellar peduncles, with more punctate enhancing lesions in the cerebral lobar subcortical white matter. When the patient’s response to steroids was inadequate, further imaging was done, revealing perinephric processes. Subsequent biopsy revealed ECD. Conclusions A review of the literature for cases of CLIPPERS demonstrated a subset of patients later found to have various malignancies involving the CNS. This case report uses the patient’s unique radiographic and clinical presentation to demonstrate the importance of the exclusion criteria within the CLIPPERS diagnostic requirements and stresses red flags suggestive of alternative diagnoses. This distinction is of high importance when differentiating a relatively benign process such as CLIPPERS from more malignant diseases.

Brain MRI-findings in Ph - negative myeloproliferative disorders

2019 ◽  
Vol 91 (7) ◽  
pp. 29-34 ◽  
Author(s):  
M M Tanashyan ◽  
A L Melikyan ◽  
P I Kuznetsova ◽  
A A Raskurazhev ◽  
A A Shabalina ◽  
...  
Keyword(s):  
Cerebrovascular Disease ◽  
Sinus Thrombosis ◽  
Cerebral Arteries ◽  
Brain Mri ◽  
Myeloproliferative Disorders ◽  
Cerebral Venous Sinus Thrombosis ◽  
Mri Findings ◽  
Cerebral Lesions ◽  
Cerebrovascular Pathology ◽  
Underlying Mechanisms

Myeloproliferative disorders (MPD) are accompanied by a high proportion of thrombotic complications, which may lead to cerebrovascular disease (CVD). Aim. To describe MRI-findings in patients with Ph - negative MPD and evaluate any cerebrovascular disease. Materials and methods. We included 104 patients with Ph - negative MPD (age varied between 20 and 58) with clinical correlates of cerebrovascular pathology. Results. Brain MRI showed post - stroke lesions in 20% of patients (7 hemispheric infarcts due to thrombotic occlusion of one of the large cerebral arteries, 14 - cortical infarcts). 37 patients (36%) had vascular cerebral lesions. Cerebral venous sinus thrombosis occurred in 5 patients - in 7% (n=3) of patients with polycythemia vera and 5% (n=2) - in patients with essential thrombocythemia. The incidence of vascular cerebral lesions was associated with higher levels of the following: erythrocyte, platelet count, fibrinogen, and with the decrease in fibrinolytic activity, as well. Conclusion. The pioneering results of the study include the description and analysis of brain MRI-findings in patients with Ph - negative MPD. The underlying mechanisms of cerebrovascular pathology in these patients are associated with certain blood alterations (particularly, hemorheology) which present a major risk factor.

scholarly journals Comparisons of clinical characteristics, brain MRI findings, and responses to epidural blood patch between spontaneous intracranial hypotension and post-dural puncture headache: retrospective study

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Gha-Hyun Lee ◽  
Jiyoung Kim ◽  
Hyun-Woo Kim ◽  
Jae Wook Cho
Keyword(s):  
Intracranial Hypotension ◽  
Dural Puncture ◽  
Epidural Blood Patch ◽  
Brain Mri ◽  
Spontaneous Intracranial Hypotension ◽  
Vein Of Galen ◽  
Mri Findings ◽  
Post Dural Puncture Headache ◽  
Straight Sinus ◽  
Blood Patch

Abstract Background Spontaneous intracranial hypotension and post-dural puncture headache are both caused by a loss of cerebrospinal fluid but present with different pathogeneses. We compared these two conditions concerning their clinical characteristics, brain imaging findings, and responses to epidural blood patch treatment. Methods We retrospectively reviewed the records of patients with intracranial hypotension admitted to the Neurology ward of the Pusan National University Hospital between January 1, 2011, and December 31, 2019, and collected information regarding age, sex, disease duration, hospital course, headache intensity, time to the appearance of a headache after sitting, associated phenomena (nausea, vomiting, auditory symptoms, dizziness), number of epidural blood patch treatments, and prognosis. The brain MRI signs of intracranial hypotension were recorded, including three qualitative signs (diffuse pachymeningeal enhancement, venous distention of the lateral sinus, subdural fluid collection), and six quantitative signs (pituitary height, suprasellar cistern, prepontine cistern, mamillopontine distance, the midbrain-pons angle, and the angle between the vein of Galen and the straight sinus). Results A total of 105 patients (61 spontaneous intracranial hypotension patients and 44 post-dural puncture headache patients) who met the inclusion criteria were reviewed. More patients with spontaneous intracranial hypotension required epidural blood patch treatment than those with post-dural puncture headache (70.5% (43/61) vs. 45.5% (20/44); p = 0.01) and the spontaneous intracranial hypotension group included a higher proportion of patients who underwent epidural blood patch treatment more than once (37.7% (23/61) vs. 13.6% (6/44); p = 0.007). Brain MRI showed signs of intracranial hypotension in both groups, although the angle between the vein of Galen and the straight sinus was greater in the post-dural puncture headache group (median [95% Confidence Interval]: 85° [68°-79°] vs. 74° [76°-96°], p = 0.02). Conclusions Patients with spontaneous intracranial hypotension received more epidural blood patch treatments and more often needed multiple epidural blood patch treatments. Although both groups showed similar brain MRI findings, the angle between the vein of Galen and the straight sinus differed significantly between the groups.

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