Sjogren's syndrome: a neurological perspective.

Sjogren's syndrome (SS) is a complex autoimmune disease characterized by lymphocytic infiltration of salivary and lacrimal glands, resulting in sicca symptoms. Additionally, SS presents with neurological manifestations that significantly impact the nervous system. This review aims to provide a comprehensive overview of the neurological aspects of SSj, covering both the peripheral and central nervous system involvement, while emphasizing diagnosis, treatment, and prognosis.

A síndrome de Sjogren (SS) é uma doença autoimune complexa caracterizada pela infiltração linfocítica das glândulas salivares e lacrimais, resultando em sintomas sicca. Além disso, a SS apresenta manifestações neurológicas que afetam significativamente o sistema nervoso. Esta revisão tem como objetivo fornecer uma visão abrangente dos aspectos neurológicos da SSj, abordando tanto o envolvimento do sistema nervoso periférico quanto do central, com ênfase no diagnóstico, tratamento e prognóstico.

Sjogren's syndrome: a neurological perspective / Síndrome de Sjogren: uma perspectiva neurológica

Abstract Sjogren's syndrome (SS) is a complex autoimmune disease characterized by lymphocytic infiltration of salivary and lacrimal glands, resulting in sicca symptoms. Additionally, SS presents with neurological manifestations that significantly impact the nervous system. This review aims to provide a comprehensive overview of the neurological aspects of SSj, covering both the peripheral and central nervous system involvement, while emphasizing diagnosis, treatment, and prognosis.

Resumo A síndrome de Sjogren (SS) é uma doença autoimune complexa caracterizada pela infiltração linfocítica das glândulas salivares e lacrimais, resultando em sintomas sicca. Além disso, a SS apresenta manifestações neurológicas que afetam significativamente o sistema nervoso. Esta revisão tem como objetivo fornecer uma visão abrangente dos aspectos neurológicos da SSj, abordando tanto o envolvimento do sistema nervoso periférico quanto do central, com ênfase no diagnóstico, tratamento e prognóstico.

Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids (SLIPPERS)-Does it Really Exist?

Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids (SLIPPERS) is a rare variant of the CLIPPERS spectrum with less than ten reports published so far. There is ongoing discussion regarding whether SLIPPERS is a disease entity on its own or just an acronym encompassing many underlying diagnoses, such as sarcoidosis, vasculitis and anti-glial fibrillary acidic protein (GFAP)-associated disease. A 40-year-old woman presented with episodes of language and attention impairment. Magnetic resonance imaging (MRI) revealed T2/FLAIR hyperintense lesions in the subcortical white matter associated with a micronodular, curvilinear perivascular contrast-enhancement. Alternative diagnoses were excluded. There was a remarkable response to steroids. A relapse occurred after six years, and the biopsy showed perivascular T-cell lymphocytic infiltrate, without granulomas, vasculitis, or neoplasia. There was complete resolution of the relapse after steroids. This case represents the longest reported follow-up of a patient diagnosed with SLIPPERS, and brain biopsy after 6 years did not suggest alternative diagnoses. This report contributes to the discussion regarding the possibility that exclusive supratentorial CLIPPERS-like pathology might be an isolated disease entity, but more biopsy-proven cases with a longer follow-up are needed to support this hypothesis. Recently, GFAP astrocytopathy has been characterized and might correspond to a significant number of cases previously diagnosed as CLIPPERS or SLIPPERS.

The Intersection Between the Oculomotor Nerve and the Internal Carotid Artery to Distinguish Extracavernous and Intracavernous Paraclinoid Aneurysms Using Anatomic Dissections-A New 3T Magnetic Resonance Imaging Protocol Confirmed by Three-Dimensionally Printed Biomodels.

OBJECTIVE: To evaluate the relationship between the oculomotor nerve (CNIII) and the internal carotid artery (ICA) as a new anatomic-radiologic landmark for distinguishing the exact location of a paraclinoid intracranial aneurysm (IA). METHODS: Microanatomic dissections were performed in 20 cavernous sinuses to evaluate the ICA paraclinoid region. Based on anatomic observations, a new magnetic resonance (MRI) protocol to classify paraclinoid aneurysms was proposed. MRI of 42 IAs from 34 patients was independently analyzed and classified as intracavernous, extracavernous, or transitional by 2 neuroradiologists. To validate the proposed MRI protocol, each IA was classified by a three-dimensionally (3D) printed biomodel and agreement with the radiologic classifications was evaluated. Of 42 IAs, 23 undergoing microsurgeries were also classified by direct visualization. RESULTS: We observed that the true cavernous sinus roof is defined by the carotid-oculomotor membrane, which has an intimate relationship with the intersection between the superior limit of the CNIII and the ICA. Based on this intersection, all 42 IAs were radiologically classified and agreement with the 3D printed biomodels was observed in 95% IAs. Concordance tests showed a statistically significant (P < 0.05) agreement between the classifications. All 23 IAs treated had the radiologic and 3D biomodel classification confirmed. CONCLUSIONS: The intersection between the ICA and the CNIII, which crosses it transversely in its entire diameter, is a reliable anatomic-radiologic landmark to correctly classify paraclinoid aneurysms. Through a new MRI protocol, it is possible to radiologically identify this intersection and to easily distinguish the intracavernous and extracavernous ICA paraclinoid aneurysms.

Case Report: Rapid Desensitization to Ocrelizumab for Multiple Sclerosis Is Effective and Safe.

Monoclonal antibodies have become a mainstay of treatment for many inflammatory diseases and malignancies. Multiple sclerosis is a chronic inflammatory, demyelinating, and neurodegenerative disease of the central nervous system and a common cause of disability in young adults. Ocrelizumab is a recombinant humanized monoclonal antibody that targets CD20-positive B cells and has been approved in the treatment of multiple sclerosis. Although considered safe, more than 30% of patients treated with Ocrelizumab developed infusion-related reactions, mostly regarded as mild. When severe, they can lead to a definite suspension of that drug. We present a case report of Ocrelizumab desensitization in a female patient who presented an immediate hypersensitivity reaction (urticaria and angioedema) during the first Ocrelizumab infusion. Although mechanisms involved in the response were not elucidated, the procedure occurred uneventfully and permitted first-line multiple sclerosis treatment maintenances. Desensitization should be considered a safe therapeutic option in patients with immediate hypersensitivity reactions to Ocrelizumab.

Myxoid glioneuronal tumour - report of three cases of a new tumour in a typical location and review of literature.

Formerly called dysembryoplastic neuroepithelial tumour (DNET) of the septum pellucidum, myxoid glioneuronal tumour (MGT) was recently recognized as a distinct entity. We report three cases of presumed MGT with typical location and image features.

Hemorragia cerebral durante fase ativa de infecção por SARS-CoV-2 em paciente com angiopatia amiloide: relato de caso / Cerebral hemorrhage during the active phase of SARS-CoV-2 infection in a patient with amyloid angiopathy: case report

RESUMO As alterações neurológicas associadas à COVID-19 têm sido frequentemente descritas, principalmente nos casos de maior severidade, e estão relacionadas a causas multifatoriais, como a disfunção endotelial, a liberação de mediadores inflamatórios (cytokine storm), a disfunção endotelial e a hipoxemia. Relatamos o caso de uma paciente do sexo feminino, 88 anos, com quadro de hemorragia cerebral associada à angiopatia amiloide, no contexto de infecção por SARS-CoV-2.

ABSTRACT The neurological changes associated with COVID-19 have been frequently described, especially in cases of greater severity, and are related to multifactorial causes, such as endothelial dysfunction, inflammatory mediator release (cytokine storm), endothelial dysfunction and hypoxemia. We report the case of a female patient, 88 years old, with cerebral hemorrhage associated with amyloid angiopathy in the context of SARS-CoV-2 infection.

Neurovascular and perfusion imaging findings in coronavirus disease 2019: Case report and literature review.

Central nervous system involvement in severe acute respiratory syndrome caused by coronavirus disease 2019 (COVID-19) has increasingly been recognised in the literature, and possible mechanisms of neuroinvasion, neurotropism and neurovirulence have been described. Neurological signs have been described in 84% of COVID-19 intensive care unit patients, and haemostatic abnormalities in such patients may play an important role, with a broad spectrum of neuroimaging findings. This report describes the magnetic resonance imaging neurovascular findings in an acutely ill patient with COVID-19, including perfusion abnormalities depicted in the arterial spin labelling technique.

Cerebral hemorrhage during the active phase of SARS-CoV-2 infection in a patient with amyloid angiopathy: case report. / Hemorragia cerebral durante fase ativa de infecção por SARS-CoV-2 em paciente com angiopatia amiloide: relato de caso.

The neurological changes associated with COVID-19 have been frequently described, especially in cases of greater severity, and are related to multifactorial causes, such as endothelial dysfunction, inflammatory mediator release (cytokine storm), endothelial dysfunction and hypoxemia. We report the case of a female patient, 88 years old, with cerebral hemorrhage associated with amyloid angiopathy in the context of SARS-CoV-2 infection.

As alterações neurológicas associadas à COVID-19 têm sido frequentemente descritas, principalmente nos casos de maior severidade, e estão relacionadas a causas multifatoriais, como a disfunção endotelial, a liberação de mediadores inflamatórios (cytokine storm), a disfunção endotelial e a hipoxemia. Relatamos o caso de uma paciente do sexo feminino, 88 anos, com quadro de hemorragia cerebral associada à angiopatia amiloide, no contexto de infecção por SARS-CoV-2.

Neuroimaging of Emergent and Reemergent Infections.

Infectious diseases emerge and reemerge over the years, and many of them can cause neurologic disease. Several factors contribute to the emergence and reemergence of these conditions, including human population growth, an increase in international travel, the geographic expansion of recognized pathogens to areas where they were previously nonendemic, and greater contact with wild animal reservoirs. The antivaccination social movement has played an important role in the reemergence of infectious diseases, especially some viral conditions. The authors review different viral (arboviruses such as dengue, chikungunya, and Zika virus; enterovirus 71; measles; and influenza), bacterial (syphilis, Lyme disease, and listeriosis), and parasitic (Chagas disease) diseases, focusing primarily on their neurologic complications. Although there are several additional infectious diseases with central nervous system manifestations that could be classified as emergent or reemergent, those listed here are the most relevant from an epidemiologic standpoint and are representative of important public health issues on all continents. The infections caused by these pathogens often show a variety of neuroimaging patterns that can be identified at CT and MRI, and radiology is central to the diagnosis and follow-up of such conditions. Given the increasing relevance of emerging and reemerging infections in clinical practice and public health scenarios, radiologists should be familiar with these infections. Online supplemental material is available for this article. ©RSNA, 2019.

SPG7 with parkinsonism responsive to levodopa and dopaminergic deficit.

We report a 55-year-old woman with a long history of a gait disturbance that was followed by dysarthria and urinary incontinence. She underwent brain MRI, SPECT with TRODAT imaging and whole-exome sequencing, revealing the diagnosis of SPG7. She developed parkinsonism responsive to levodopa, expanding the phenotype of complex SPG7.