Assuntos
Síndrome do Cromossomo X Frágil , Paraplegia Espástica Hereditária , Humanos , Paraplegia Espástica Hereditária/diagnóstico , Paraplegia Espástica Hereditária/genética , Síndrome do Cromossomo X Frágil/genética , Expansão das Repetições de Trinucleotídeos , Proteína do X Frágil da Deficiência Intelectual/genéticaRESUMO
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.
Assuntos
Doenças do Sistema Nervoso , Síndrome de Sjogren , Humanos , Síndrome de Sjogren/complicações , Doenças do Sistema Nervoso/etiologiaRESUMO
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.
RESUMO
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.
RESUMO
Introduction: Developmental venous anomalies (DVAs) are considered variants of normal transmedullary veins. Their association with cavernous malformations is reported to increase the risk of hemorrhage. Expert consensus recommends meticulous planning with MR imaging, use of anatomical "safe zones", intraoperative monitoring of long tracts and cranial nerve nuclei, and preservation of the DVA as key to avoiding complications in brainstem cavernoma microsurgery. Symptomatic outflow restriction of DVA is rare, with the few reported cases in the literature restricted to DVAs in the supratentorial compartment. Case: We present a case report of the resection of a pontine cavernoma complicated by delayed outflow obstruction of the associated DVA. A female patient in her 20's presented with progressive left-sided hemisensory disturbance and mild hemiparesis. MRI revealed two pontine cavernomas associated with interconnected DVA and hematoma. The symptomatic cavernoma was resected via the infrafacial corridor. Despite the preservation of the DVA, the patient developed delayed deterioration secondary to venous hemorrhagic infarction. We discuss the imaging and surgical anatomy pertinent to brainstem cavernoma surgery, as well as the literature exploring the management of symptomatic infratentorial DVA occlusion. Conclusion: Delayed symptomatic pontine venous congestive edema is extremely rare following cavernoma surgery. DVA outflow restriction from a post-operative cavity, intraoperative manipulation, and intrinsic hypercoagulability from COVID-10 infection are potential pathophysiological factors. Improved knowledge of DVAs, brainstem venous anatomy, and "safe entry zones" will further elucidate the etiology of and the efficacious treatment for this complication.
RESUMO
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.