Atypical Demyelinating Disorders: MR Imaging Features, Atypical Triggers, and Etiopathogenesis.

Atypical demyelinating lesions (ADLs) can be idiopathic, occurring as isolated and self-limited events, or can appear in different stages of relapsing demyelinating diseases. Not infrequently, ADLs occur in inflammatory syndromes associated with exogenous or endogenous toxic factors, metabolic imbalance, or infectious agents. It is important to recognize imaging patterns that indicate an inflammatory/demyelinating substrate in central nervous system lesions and to investigate potential triggers or complicating factors that might be associated. The prognostic and treatment strategies of ADLs are influenced by the underlying etiopathogenesis.

AQP4-IgG NMOSD, MOGAD, and double-seronegative NMOSD: is it possible to depict the antibody subtype using magnetic resonance imaging?

BACKGROUND: There is clinical and radiological overlap among demyelinating diseases. However, their pathophysiological mechanisms are different and carry distinct prognoses and treatment demands. OBJECTIVE: To investigate magnetic resonance imaging (MRI) features of patients with myelin-oligodendrocyte glycoprotein associated disease (MOGAD), antibody against aquaporin-4(AQP-4)-immunoglobulin G-positive neuromyelitis optica spectrum disorder (AQP4-IgG NMOSD), and double-seronegative patients. METHODS: A cross-sectional retrospective study was performed to analyze the topography and morphology of central nervous system (CNS) lesions. Two neuroradiologists consensually analyzed the brain, orbit, and spinal cord images. RESULTS: In total, 68 patients were enrolled in the study (25 with AQP4-IgG-positive NMOSD, 28 with MOGAD, and 15 double-seronegative patients). There were differences in clinical presentation among the groups. The MOGAD group had less brain involvement (39.2%) than the NMOSD group (p = 0.002), mostly in the subcortical/juxtacortical, the midbrain, the middle cerebellar peduncle, and the cerebellum. Double-seronegative patients had more brain involvement (80%) with larger and tumefactive lesion morphology. In addition, double-seronegative patients showed the longest optic neuritis (p = 0.006), which was more prevalent in the intracranial optic nerve compartment. AQP4-IgG-positive NMOSD optic neuritis had a predominant optic-chiasm location, and brain lesions mainly affected hypothalamic regions and the postrema area (MOGAD versus AQP4-IgG-positive NMOSD, p= 0 .013). Furthermore, this group had more spinal cord lesions (78.3%), and bright spotty lesions were a paramount finding to differentiate it from MOGAD (p = 0.003). CONCLUSION: The pooled analysis of lesion topography, morphology, and signal intensity provides critical information to help clinicians form a timely differential diagnosis.

ANTECEDENTES: Há sobreposição clínica e radiológica entre as doenças desmielinizantes. No entanto, seus mecanismos fisiopatológicos são diferentes e apresentam prognósticos e demandas de tratamento distintos. OBJETIVO: Investigar as características de imagens de RM dos pacientes com doença associada à glicoproteína de oligodendrócito de mielina (MOGAD), a doenças do espectro da neuromielite óptica positivas para antiaquaporina-4 imunoglobulina G (AQP4-IgG NMOSD), e pacientes duplamente soronegativos. MéTODOS: Estudo retrospectivo e transversal para analisar as características e frequência das lesões do sistema nervoso central (SNC). Dois neurorradiologistas avaliaram consensualmente as imagens do cérebro, das órbitas e da medula espinhal. RESULTADOS: Ao todo, foram incluídos 68 pacientes(25 com AQP4-IgG NMOSD, 28 com MOGAD e 15 duplo-soronegativos). Há diferenças na apresentação clínica entre os grupos. O grupo MOGAD demonstrou menor frequência de comprometimento do cérebro (39.2%) comparado com o AQP4-IgG NMOSD (p = 0.002), com predomínio da distribuição das lesões nas regiões subcortical/justacortical, mesencéfalo, pedúnculos cerebelares médios e cerebelo. O grupo duplo-soronegativo demonstrou maior frequência de comprometimento do cérebro (80%), com lesões de maiores dimensões e com morfologia tumefeita, além de neurite óptica com maior extensão (p = 0.006). O grupo AQP4-IgG NMOSD demonstrou neurite óptica com predomínio na região óptico-quiasmática e as lesões encefálicas acometeram predominantemente as regiões hipotalâmica e área postrema (MOGAD versus AQP4-IgG NMOSD p = 0.013). Além disso, foram observadas mais lesões na medula espinhal (78.3%) e a presença da "bright spotty lesion" foi um achado primordial para a sua diferenciação com os pacientes MOGAD (p = 0.003). CONCLUSãO: A análise pormenorizada das características das lesões por RM dos pacientes com doenças desmielinizantes imunomediadas fornece informações fundamentais que auxiliam os médicos no diagnóstico diferencial em um momento oportuno.

AQP4-IgG NMOSD, MOGAD, and double-seronegative NMOSD: is it possible to depict the antibody subtype using magnetic resonance imaging? / NMOSD AQP4-IgG, MOGAD e NMOSD duplo soronegativo: é possível caracterizar o tipo de anticorpo pela ressonância magnética?

Abstract Background There is clinical and radiological overlap among demyelinating diseases. However, their pathophysiological mechanisms are different and carry distinct prognoses and treatment demands. Objective To investigate magnetic resonance imaging (MRI) features of patients with myelin-oligodendrocyte glycoprotein associated disease (MOGAD), antibody against aquaporin-4(AQP-4)-immunoglobulin G-positive neuromyelitis optica spectrum disorder (AQP4-IgG NMOSD), and double-seronegative patients. Methods A cross-sectional retrospective study was performed to analyze the topography and morphology of central nervous system (CNS) lesions. Two neuroradiologists consensually analyzed the brain, orbit, and spinal cord images. Results In total, 68 patients were enrolled in the study (25 with AQP4-IgG-positive NMOSD, 28 with MOGAD, and 15 double-seronegative patients). There were differences in clinical presentation among the groups. The MOGAD group had less brain involvement (39.2%) than the NMOSD group (p = 0.002), mostly in the subcortical/juxtacortical, the midbrain, the middle cerebellar peduncle, and the cerebellum. Double-seronegative patients had more brain involvement (80%) with larger and tumefactive lesion morphology. In addition, double-seronegative patients showed the longest optic neuritis (p = 0.006), which was more prevalent in the intracranial optic nerve compartment. AQP4-IgG-positive NMOSD optic neuritis had a predominant optic-chiasm location, and brain lesions mainly affected hypothalamic regions and the postrema area (MOGAD versus AQP4-IgG-positive NMOSD, p= 0 .013). Furthermore, this group had more spinal cord lesions (78.3%), and bright spotty lesions were a paramount finding to differentiate it from MOGAD (p = 0.003). Conclusion The pooled analysis of lesion topography, morphology, and signal intensity provides critical information to help clinicians form a timely differential diagnosis.

Resumo Antecedentes Há sobreposição clínica e radiológica entre as doenças desmielinizantes. No entanto, seus mecanismos fisiopatológicos são diferentes e apresentam prognósticos e demandas de tratamento distintos. Objetivo Investigar as características de imagens de RM dos pacientes com doença associada à glicoproteína de oligodendrócito de mielina (MOGAD), a doenças do espectro da neuromielite óptica positivas para antiaquaporina-4 imunoglobulina G (AQP4-IgG NMOSD), e pacientes duplamente soronegativos. Métodos Estudo retrospectivo e transversal para analisar as características e frequência das lesões do sistema nervoso central (SNC). Dois neurorradiologistas avaliaram consensualmente as imagens do cérebro, das órbitas e da medula espinhal. Resultados Ao todo, foram incluídos 68 pacientes(25 com AQP4-IgG NMOSD, 28 com MOGAD e 15 duplo-soronegativos). Há diferenças na apresentação clínica entre os grupos. O grupo MOGAD demonstrou menor frequência de comprometimento do cérebro (39.2%) comparado com o AQP4-IgG NMOSD (p = 0.002), com predomínio da distribuição das lesões nas regiões subcortical/justacortical, mesencéfalo, pedúnculos cerebelares médios e cerebelo. O grupo duplo-soronegativo demonstrou maior frequência de comprometimento do cérebro (80%), com lesões de maiores dimensões e com morfologia tumefeita, além de neurite óptica com maior extensão (p = 0.006). O grupo AQP4-IgG NMOSD demonstrou neurite óptica com predomínio na região óptico-quiasmática e as lesões encefálicas acometeram predominantemente as regiões hipotalâmica e área postrema (MOGAD versus AQP4-IgG NMOSD p = 0.013). Além disso, foram observadas mais lesões na medula espinhal (78.3%) e a presença da "bright spotty lesion" foi um achado primordial para a sua diferenciação com os pacientes MOGAD (p = 0.003). Conclusão A análise pormenorizada das características das lesões por RM dos pacientes com doenças desmielinizantes imunomediadas fornece informações fundamentais que auxiliam os médicos no diagnóstico diferencial em um momento oportuno.

Neurological imaging findings in hospitalized COVID-19 patients: a retrospective observational study in two Brazilian reference centers / Achados de neuroimagem em pacientes hospitalizados por COVID-19: um estudo retrospectivo e observacional em dois serviços brasileiros de referência

ABSTRACT Background: A variety of neurological manifestations have been attributed to COVID-19. Objective: To investigate the occurrence of neurological symptoms and neuroimaging findings in patients hospitalized in two Brazilian reference centers. Methods: We performed a retrospective cohort study of patients who had laboratory-confirmed COVID-19 presenting in two hospitals in Brazil between March 4 and July 7, 2020, who underwent brain imaging. Results: We recorded 1,359 patients with laboratory-confirmed COVID-19. Brain imaging was performed in 250 (18.4%) patients with neurological symptoms, and nine of them (3.6%) had acute or subacute ischemic stroke neuroimaging findings. Six of the nine patients initially presented with respiratory symptoms while the other three patients presented to the emergency room with acute stroke signs. Conclusions: We described the neuroimaging findings of patients infected with COVID-19 who presented with neurological symptoms in two major hospitals in Brazil. We reinforce the importance of being aware of cerebrovascular complications, both in severe hospitalized patients and in patients who present to the emergency room with acute neurological symptoms, even in the elderly.

RESUMO Introdução: Diversas manifestações neurológicas têm sido atribuídas à COVID-19. Objetivo: Investigar a ocorrência de sintomas neurológicos e achados de neuroimagem em pacientes internados em dois centros de referência brasileiros. Métodos: Estudo retrospectivo que avaliou pacientes internados em dois hospitais no Brasil entre 4 de março e 7 de julho de 2020, com confirmação laboratorial de infecção pelo COVID-19 e que foram submetidos a tomografia computadorizada ou ressonância magnética do crânio em razão de sintomas neurológicos. Resultados: Obtivemos 1.359 pacientes com confirmação laboratorial de infecção pelo COVID-19. Deles, 250 (18,4%) apresentaram sintomas neurológicos e foram submetidos a exames de imagem do crânio e nove (3,6%) demonstraram achados de neuroimagem compatíveis com acidente vascular cerebral (AVC) isquêmico agudo ou subagudo. Em seis dos nove pacientes, observaram-se inicialmente tosse e dispneia graves, enquanto outros três chegaram ao pronto-socorro com sinais de AVC agudo. Conclusões: Foram descritos os achados de neuroimagem em pacientes infectados com COVID-19 em dois grandes centros de referência brasileiros. Reforçamos a importância de estar atento aos quadros neurológicos, especialmente de AVC, tanto em pacientes graves hospitalizados quanto em pacientes que chegam ao pronto-socorro com sintomas neurológicos agudos, mesmo em idosos.

Neurological imaging findings in hospitalized COVID-19 patients: a retrospective observational study in two Brazilian reference centers.

BACKGROUND: A variety of neurological manifestations have been attributed to COVID-19. OBJECTIVE: To investigate the occurrence of neurological symptoms and neuroimaging findings in patients hospitalized in two Brazilian reference centers. METHODS: We performed a retrospective cohort study of patients who had laboratory-confirmed COVID-19 presenting in two hospitals in Brazil between March 4 and July 7, 2020, who underwent brain imaging. RESULTS: We recorded 1,359 patients with laboratory-confirmed COVID-19. Brain imaging was performed in 250 (18.4%) patients with neurological symptoms, and nine of them (3.6%) had acute or subacute ischemic stroke neuroimaging findings. Six of the nine patients initially presented with respiratory symptoms while the other three patients presented to the emergency room with acute stroke signs. CONCLUSIONS: We described the neuroimaging findings of patients infected with COVID-19 who presented with neurological symptoms in two major hospitals in Brazil. We reinforce the importance of being aware of cerebrovascular complications, both in severe hospitalized patients and in patients who present to the emergency room with acute neurological symptoms, even in the elderly.

COVID-19 as a Cause of Acute Neonatal Encephalitis and Cerebral Cytotoxic Edema.

The majority of coronavirus disease 2019 (COVID-19) have been confirmed in adults, with only a few reported cases in children. In the pediatric population, COVID-19 infection appears to be often unremarkable or associated with mild respiratory symptoms. Little is known about neurologic complications related to COVID-19 in newborns. We present a case of severe encephalitis with cytotoxic brain edema in a newborn with COVID-19.

Improving acute demyelinating lesion detection: which T1-weighted magnetic resonance acquisition is more sensitive to gadolinium enhancement?

OBJECTIVE: Because of the need for a standardized and accurate method for detecting multiple sclerosis (MS) inflammatory activity, different magnetic resonance (MR) acquisitions should be compared in order to choose the most sensitive sequence for clinical routine. To compare the sensitivity of a T1-weighted image to a single dose of gadolinium (Gd) administration both with and without magnetization transfer to detect contrast enhancement in active demyelinating focal lesions. METHODS: A sample of relapsing-remitting MS patients were prospectively examined separately by two neuroradiologists using a 1.5 Tesla scanner. The outcome parameters were focused on Gd-enhancement detection attributed to acute demyelination. All MR examinations with at least one Gd-enhancing lesion were considered positive (MR+) and each lesion was analyzed according to its size and contrast ratio. RESULTS: Thirty-six MR examinations were analyzed with a high inter-observer agreement for MR+ detection (k coefficient > 0.8), which was excellent for the number of Gd-enhancing lesions (0.91 T1 spin-echo (SE), 0.88 T1 magnetization transfer contrast (MTC) sequence and 0.99 magnetization-prepared rapid acquisition with gradient-echo (MPRAGE). Significantly more MR+ were reported on the T1 MTC scans, followed by the T1 SE, and MPRAGE scans. Confidently, the T1 MTC sequence demonstrated higher accuracy in the detection of Gd-enhancing lesions, followed by the T1 SE and MPRAGE sequences. Further comparisons showed that there was a statistically significant increase in the contrast ratio and area of Gd-enhancement on the T1 MTC images when compared with both the SE and MPRAGE images. CONCLUSION: Single-dose Gd T1 MTC sequence was confirmed to be the most sensitive acquisition for predicting inflammatory active lesions using a 1.5 T magnet in this sample of MS patients.

Improving acute demyelinating lesion detection: which T1-weighted magnetic resonance acquisition is more sensitive to gadolinium enhancement? / Aumento da detecção das placas desmielinizantes agudas: qual aquisição de ressonância magnética ponderada em T1 é a mais sensível em demonstrar impregnação pelo gadolínio?

ABSTRACT Because of the need for a standardized and accurate method for detecting multiple sclerosis (MS) inflammatory activity, different magnetic resonance (MR) acquisitions should be compared in order to choose the most sensitive sequence for clinical routine. Objective To compare the sensitivity of a T1-weighted image to a single dose of gadolinium (Gd) administration both with and without magnetization transfer to detect contrast enhancement in active demyelinating focal lesions. Methods A sample of relapsing-remitting MS patients were prospectively examined separately by two neuroradiologists using a 1.5 Tesla scanner. The outcome parameters were focused on Gd-enhancement detection attributed to acute demyelination. All MR examinations with at least one Gd-enhancing lesion were considered positive (MR+) and each lesion was analyzed according to its size and contrast ratio. Results Thirty-six MR examinations were analyzed with a high inter-observer agreement for MR+ detection (k coefficient > 0.8), which was excellent for the number of Gd-enhancing lesions (0.91 T1 spin-echo (SE), 0.88 T1 magnetization transfer contrast (MTC) sequence and 0.99 magnetization-prepared rapid acquisition with gradient-echo (MPRAGE). Significantly more MR+ were reported on the T1 MTC scans, followed by the T1 SE, and MPRAGE scans. Confidently, the T1 MTC sequence demonstrated higher accuracy in the detection of Gd-enhancing lesions, followed by the T1 SE and MPRAGE sequences. Further comparisons showed that there was a statistically significant increase in the contrast ratio and area of Gd-enhancement on the T1 MTC images when compared with both the SE and MPRAGE images. Conclusion Single-dose Gd T1 MTC sequence was confirmed to be the most sensitive acquisition for predicting inflammatory active lesions using a 1.5 T magnet in this sample of MS patients.

RESUMO No que se refere à necessidade de um método preciso e padronizado para a detecção de atividade inflamatória em esclerose múltipla (EM), diferentes aquisições de RM devem ser comparadas com objetivo de escolher a sequência mais sensível para a rotina clínica. Objetivo Comparar a sensibilidade das sequências ponderadas em T1 após a administração endovenosa de uma única dose de gadolínio, com e sem a adição da transferência de magnetização, para detectar a impregnação das lesões desmielinizantes focais agudas. Métodos Uma amostra de pacientes com EM-RR foi prospectivamente avaliada separadamente por dois neurorradiologistas em um equipamento de RM de 1,5 Tesla. Os parâmetros de desfecho foram direcionados para a avaliação da detecção de impregnação pelo Gd atribuída à desmielinização aguda. Todos os exames de RM que demonstraram ao menos uma lesão com impregnação pelo Gd foram considerados positivos (RM+) e cada lesão foi analisada de acordo com suas dimensões e contraste. Resultados Trinta e seis exames de RM foram analisados. Os avaliadores demonstraram elevada concordância para a detecção de RM+ (coeficiente> 0,8), sendo excelente quanto ao número de lesões com impregnação pelo Gd (0,91 SE, 0,88 T1 MTC e 0,99 MPRAGE). A sequência T1 MTC apresentou número significativamente maior de RM+, seguida pelas sequências T1 SE e MPRAGE. De forma análoga, a sequência T1 MTC demonstrou maior acurácia na detecção de lesões com impregnação pelo Gd, seguida pelas sequências T1 SE e MPRAGE. As demais comparações demonstraram aumento estatisticamente significativo na relação de contraste e na área de impregnação pelo Gd nas imagens T1 MTC quando comparadas às imagens SE e MPRAGE. Conclusão A sequência T1 MTC com uma única dose de Gd confirmou ser a sequência mais sensível em demonstrar lesões inflamatórias agudas em equipamento de 1,5 T nessa coorte de pacientes com EM.

Cerebellar Ataxia in Children: A Clinical and MRI Approach to the Differential Diagnosis.

: The cerebellum has long been recognized as a fundamental structure in motor coordination. Structural cerebellar abnormalities and diseases involving the cerebellum are relatively common in children. The not always specific clinical presentation of ataxia, incoordination, and balance impairment can often be a challenge to attain a precise diagnosis. Continuous advances in genetic research and moreover the constant development in neuroimaging modalities, particularly in the field of magnetic resonance imaging, have promoted a better understanding of cerebellar diseases and led to several modifications in their classification in recent years. Thorough clinical and neuroimaging investigation is recommended for proper diagnosis. This review outlines an update of causes of cerebellar disorders that present clinically with ataxia in the pediatric population. These conditions were classified in 2 major groups, namely genetic malformations and acquired or disruptive disorders recognizable by neuroimaging and subsequently according to their features during the prenatal and postnatal periods.

Imaging of Creutzfeldt-Jakob Disease: Imaging Patterns and Their Differential Diagnosis.

Diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD) remains a challenge because of the large variability of the clinical scenario, especially in its early stages, which may mimic several reversible or treatable disorders. The molecular basis of prion disease, as well as its brain propagation and the pathogenesis of the illness, have become better understood in recent decades. Several reports have listed recognizable clinical features and paraclinical tests to supplement the replicable diagnostic criteria in vivo. Nevertheless, we lack specific data about the differential diagnosis of CJD at imaging, mainly regarding those disorders evolving with similar clinical features (mimicking disorders). This review provides an update on the neuroimaging patterns of sCJD, emphasizing the relevance of magnetic resonance (MR) imaging, summarizing the clinical scenario and molecular basis of the disease, and highlighting clinical, genetic, and imaging correlations in different subtypes of prion diseases. A long list of differential diagnoses produces a comprehensive pictorial review, with the aim of enabling radiologists to identify typical and atypical patterns of sCJD. This review reinforces distinguishable imaging findings and confirms diffusion-weighted imaging (DWI) features as pivotal in the diagnostic workup of sCJD, as these findings enable radiologists to reliably recognize this rare but invariably lethal disease. A probable diagnosis is justified when expected MR imaging patterns are demonstrated and CJD-mimicking disorders are confidently ruled out. ©RSNA, 2017.