Primary cerebrospinal fluid leak complicated with meningitis and parainfectious vasculitis: case report and literature review / Fístula liquórica primária complicada com meningite e vasculite parainfecciosa: relato de caso e revisão de literatura

This paper aims to describe a case of an immunocompetent 60-year-old patient presenting a subarachnoid hemorrhage in the absence of aneurysmal disease. Initial evaluation pointed to vasculitis of the central nervous system secondary to meningeal infection. After initial treatment, a cerebrospinal fluid leak was identified, with no antecedent of trauma, elucidating the origin of infection. Primary cerebrospinal fluid rhinorrhea has nonspecific symptomatology, defying diagnosis, and potentially serious complications. It represents an unusual predisposing factor for meningeal infection and secondary vasculitis. This case report exemplifies a feared complication of spontaneous cerebrospinal fluid leakage.

O estudo objetiva relatar um caso clínico de uma paciente imunocompetente de 60 anos apresentando hemorragia subaracnoide na ausência de doença aneurismática. Avaliação inicial apontou para vasculite de sistema nervoso central secundária à infecção meníngea. Após tratamento inicial, uma fístula liquórica foi identificada, sem antecedente de trauma, elucidando a origem da infecção. Rinorreia liquórica primária possui sintomatologia inespecífica, diagnóstico desafiador e complicações potencialmente graves. Representa um raro fator predisponente para infecção meníngea e vasculite. Este relato de caso exemplifica uma complicação temida da rinorreia liquórica espontânea.

Vasculitis primaria del sistema nervioso central: presentación de caso clínico / Primary vasculitis of the central nervous system: presentation of clinical case / Vasculite primária do sistema nervoso central: apresentação do caso clínico

La vasculitis Primaria del Sistema Nervioso Central (VPSNC) se refiere a un grupo de enfermedades que resultan de la inflamación y destrucción de los vasos sanguíneos de la medula espinal, encéfalo y meninges, tanto en el sector venoso como arterial. La presentación es heterogénea y poco sistematizable. El diagnóstico se establece con un cuadro clínico compatible, una angiografía que evidencie vasculitis y/o biopsia del parénquima encefálico o meninges. Las alteraciones en los estudios de imagen son constantes pero inespecíficas para el diagnóstico y se acompañan habitualmente de alteraciones en el líquido cefalorraquídeo (LCR) y electroencefalograma (EEG) Presentamos un paciente con probable VPSNC basados en un cuadro clínico compatible, hallazgos imagenológicos sugestivos, junto con alteraciones en LCR y EEG. Se realizó tratamiento en base a corticoides e inmunosupresores con mala respuesta y evolución.

The primary central nervous system vasculitis (VPSNC) refers to a group of diseases that result from inflammation and destruction of the blood vessels of the spinal cord, brain and meninges, both in the venous and arterial sector. The presentation is heterogeneous and unsystematized. The diagnosis is made based on compatible symptoms, supported by an angiography showing evidence of vasculitis and/or biopsy of the brain parenchyma or meninges. Alterations in imaging studies are consistent but nonspecific for diagnostic and are usually accompanied by alterations in the electroencephalogram (EEG) and cerebrospinal fluid (CSF). We present a clinical case of probable VPSNC based on clinical presentation and findings on imagenological studies suggestive vasculitis, along with alterations in CSF and EEG. Treatment was based on Corticosteroids and immunosuppressive agents with poor response and evolution.

Vasculite Primária do Sistema Nervoso Central (VPSNC) refere-se a um grupo de doenças que resultam de inflamação e destruição dos vasos sanguíneos na medula espinal, o sector venosa arterial cerebral e meninges, ambos. A apresentação é heterogênea e não muito sistematizável. O diagnóstico é estabelecido com um quadro clínico compatível, uma angiografia que evidencia vasculite e / oubiópsia do parênquima cerebral ou meninges. Alterações nos estudos de imagemsão constantes, mas não específica para o diagnóstico e são normalmente acompanhadas por alterações no líquido cefalorraquidiano (LCR) e eletroencefalograma (EEG) descrevem um paciente com VPSNC provável com base em um quadro clínico, achados de imagem sugestivos compatíveis, em conjunto com alterações no CSF e EEG. O tratamento foi realizado com base em corticosteróides e imunos supressores compouca resposta e evolução.

Central nervous system vasculitis in adults: An update.

Primary central nervous system vasculitis (PCNSV) is a challenging diagnosis due to broad clinical manifestations and variable specificity and sensitivity of laboratory and imaging diagnostic tools. Differential diagnosis includes reversible cerebral vasoconstriction syndrome (RCVS), secondary vasculitis of the CNS and other noninflammatory vasculopathies. Brain biopsy is essential for definitive diagnosis and to exclude mimickers. Recent data show that data large-vessel PCNSV present worse prognosis when compared to small-vessel PCNSV. Herein we review diagnosis and management of PCNSV, secondary vasculitis of CNS and RCVS.

Intracerebral hemorrhage with a favorable outcome in a patient with childhood primary angiitis of the central nervous system.

Childhood primary angiitis of the central nervous system (cPACNS) is a rare inflammatory brain disease of unknown etiology. Of note, brain hemorrhage has been rarely reported in cPACNS patients, generally associated with a delayed clinical diagnosis, or with a diagnosis only at necropsy. We present the case of a boy with cPACNS that previously suffered an ischemic stroke. At the age of 7 years and 10 months, he presented a sudden and severe headache, vomiting and reduction in consciousness level (Glasgow coma scale 7), requiring prompt tracheal intubation. Brain computed tomography demonstrated intraparenchymal hematoma in the right parieto-occipital lobe and a small focus of bleeding in the right frontal lobe, vasogenic edema, herniation of the uncus and a 10mm deviation to the left from the midline. C-reactive protein (9.2mg/dL) and von Willebrand factor (vWF) antigen (202%) were elevated. Decompressive craniotomy was performed and methylprednisolone and cyclophosphamide were administered. One week later, the patient had left hemiparesis without other sequelae. Importantly, motor deficits have been improving progressively. Our case reinforces the inclusion of this vasculitis as a differential diagnosis in children and adolescents with CNS hemorrhage.

Intracerebral hemorrhage with a favorable outcome in a patient with childhood primary angiitis of the central nervous system / Hemorragia intracerebral com evolução favorável em paciente com angiíte primária do sistema nervoso central juvenil

ABSTRACT Childhood primary angiitis of the central nervous system (cPACNS) is a rare inflammatory brain disease of unknown etiology. Of note, brain hemorrhage has been rarely reported in cPACNS patients, generally associated with a delayed clinical diagnosis, or with a diagnosis only at necropsy. We present the case of a boy with cPACNS that previously suffered an ischemic stroke. At the age of 7 years and 10 months, he presented a sudden and severe headache, vomiting and reduction in consciousness level (Glasgow coma scale 7), requiring prompt tracheal intubation. Brain computed tomography demonstrated intraparenchymal hematoma in the right parieto-occipital lobe and a small focus of bleeding in the right frontal lobe, vasogenic edema, herniation of the uncus and a 10 mm deviation to the left from the midline. C-reactive protein (9.2 mg/dL) and von Willebrand factor (vWF) antigen (202%) were elevated. Decompressive craniotomy was performed and methylprednisolone and cyclophosphamide were administered. One week later, the patient had left hemiparesis without other sequelae. Importantly, motor deficits have been improving progressively. Our case reinforces the inclusion of this vasculitis as a differential diagnosis in children and adolescents with CNS hemorrhage.

RESUMO Angiíte primária do sistema nervoso central juvenil (APSNCJ) é uma doença inflamatória cerebral rara e de etiologia desconhecida. Hemorragia cerebral tem sido raramente reportada em pacientes com APSNCJ, geralmente associada com atraso diagnóstico, ou com um diagnóstico somente por necrópsia. Relata-se um caso de um paciente do gênero masculino com APSNCJ e que previamente sofreu um acidente vascular cerebral isquêmico. Aos 7 anos e 10 meses de idade, o menino apresentou subitamente cefaleia intensa, vômitos e redução do nível de consciência (escala de coma de Glasgow 7), requerendo imediata intubação traqueal. Uma tomografia computadorizada cerebral demonstrou hematoma intraparenquimatoso no lobo parieto-occipital direito e um pequeno foco de sangramento no lobo frontal direito, edema vasogênico, herniação do úncus e um desvio de 10 mm da linha média para a esquerda. A proteína C-reativa (9.2 mg/dL) e o fator antígeno de von Willebrand (202%) estavam elevados. Foi realizada uma craniotomia descompressiva, seguida pela administração de metilprednisolona e ciclofosfamida. Transcorrida uma semana, o paciente apresentava hemiparesia esquerda, sem outras sequelas. É digno de nota que o déficit motor tem melhorado progressivamente. Nosso caso reforça a inclusão dessa vasculite como diagnóstico diferencial em crianças e adolescentes com hemorragia do sistema nervoso central.

Diagnostic Yield and Safety of Brain Biopsy for Suspected Primary Central Nervous System Angiitis.

BACKGROUND AND PURPOSE: The utility and safety of brain biopsy for suspected primary angiitis of the central nervous system (PACNS) are uncertain. Factors predictive of a positive biopsy have not been well described. Our aim was to evaluate the diagnostic yield and safety of brain biopsy in suspected PACNS and determine whether any prebiopsy variables are associated with a positive biopsy. METHODS: This is a retrospective study of consecutive patients who underwent diagnostic brain biopsy for PACNS at a single institution. The relationship between biopsy yield and patient demographics, surgical technique, laboratory testing, neuroimaging, biopsy characteristics, and prebiopsy immunosuppressive therapy were examined. RESULTS: PACNS was confirmed in 9 of 79 patients (11%). Biopsy identified alternative diagnoses in 24 patients (30%), with cerebral amyloid angiopathy (8 patients), encephalitis (5 patients), demyelination (3 patients), and CNS lymphoma (3 patients) most commonly found. There was no correlation between a positive biopsy and cerebrospinal fluid results, neuroimaging, surgical technique, biopsy characteristics, or preoperative immunosuppressive therapy. Smaller biopsies (P=0.02) and closed procedures (P=0.013) were less likely to yield a diagnosis. Postoperative complications occurred in 13 patients (16%), 3 (4%) of which were serious. CONCLUSIONS: Brain biopsy leads to pathological confirmation of vasculitis in a minority of suspected PACNS cases but alternative diagnoses are often identified. Importantly, rare but meaningful complications may occur.

An update of neurological manifestations of vasculitides and connective tissue diseases: a literature review / Atualização das manifestações neurológicas das vasculitides e das doenças do tecido conjuntivo: revisão de literatura

Vasculitides comprise a heterogeneous group of autoimmune disorders, occurring as primary or secondary to a broad variety of systemic infectious, malignant or connective tissue diseases. The latter occur more often but their pathogenic mechanisms have not been fully established. Frequent and varied central and peripheral nervous system complications occur in vasculitides and connective tissue diseases. In many cases, the neurological disorders have an atypical clinical course or even an early onset, and the healthcare professionals should be aware of them. The purpose of this brief review was to give an update of the main neurological disorders of common vasculitis and connective tissue diseases, aiming at accurate diagnosis and management, with an emphasis on pathophysiologic mechanisms.

As vasculites são um grupo heterogêneo de doenças autoimunes primárias ou secundárias a uma grande variedade de doenças infecciosas sistêmicas, malignas ou do tecido conjuntivo. Estas últimas são as que ocorrem com mais frequência, porém os mecanismos patogênicos ainda não foram plenamente determinados. Diversas e frequentes complicações do sistema nervoso central e periférico ocorrem nas vasculites e doenças do tecido conjuntivo. Em muitos casos, os distúrbios neurológicos têm evolução clínica atípica ou mesmo início precoce, ao que todos os profissionais de saúde devem estar cientes. O objetivo desta breve revisão foi atualizar os principais distúrbios neurológicos da vasculite comum e das doenças do tecido conjuntivo, visando ao diagnóstico e ao tratamento corretos, com ênfase nos mecanismos fisiopatológicos.

An update of neurological manifestations of vasculitides and connective tissue diseases: a literature review.

Vasculitides comprise a heterogeneous group of autoimmune disorders, occurring as primary or secondary to a broad variety of systemic infectious, malignant or connective tissue diseases. The latter occur more often but their pathogenic mechanisms have not been fully established. Frequent and varied central and peripheral nervous system complications occur in vasculitides and connective tissue diseases. In many cases, the neurological disorders have an atypical clinical course or even an early onset, and the healthcare professionals should be aware of them. The purpose of this brief review was to give an update of the main neurological disorders of common vasculitis and connective tissue diseases, aiming at accurate diagnosis and management, with an emphasis on pathophysiologic mechanisms.

Rheumatologic emergencies.

Rheumatological conditions can sometimes present as emergencies. These can occur due to the disease process or infection; contrary to what many people think, rheumatologic emergencies like a pain, rheumatic crisis, or attack gout do not compromise the patient's life. This article mentioned only true emergencies: catastrophic antiphospholipid syndrome (cAPS), kidney-lung syndrome, central nervous system (CNS) vasculitis, anti-Ro syndrome (neonatal lupus), and macrophage activation syndrome (MAS). The management of above emergencies includes critical care, immunosuppression when indicated, and use of a diagnostic flowchart as well as fast laboratory profile for making decisions. Anticoagulants have to be used in the management of antiphospholipid syndrome. A good understanding of these conditions is of paramount importance for proper management.

Granulomatosis with polyangiitis: recurrence presenting as ependimoplexitis.

A 55-year-old man with granulomatosis with polyangiitis (GPA) developed continuous parietal headache, malaise, nasal crusting and dry cough. Neurological exam revealed only left hand hypoesthesia in 4th and 5th finger. Brain MRI showed enlarged right choroid plexus, hyperintense periventricular white matter, thalami and right side of corpus callosum. The suspected diagnosis was ependimoplexitis due to GPA, the patient received three 500 mg methylprednisolone pulses followed by 1 mg/kg of meprednisone with gradual tapering and was switched to oral cyclophosphamide. He had complete resolution of headache. An MRI following this treatment for relapse revealed only minimal ependimal changes.

Neuro-Behçet's disease, its mimickers and anti-TNF therapy: a case-based review.

When the central nervous system is the primary affected site in an initial attack of Behçet's disease (BD), the differential diagnosis is particularly challenging. Because the specificity of immunobiologic therapy is growing, the specific diagnosis may impact the chosen therapy. For instance, anti-tumour necrosis factor agents are efficacious in BD but may be harmful in multiple sclerosis or systemic lupus erythematosus. We present two cases with similar neurological features but different diagnosis (BD and systemic lupus erythematosus) as a starting point to review diagnostic and therapeutic approaches for neuro-BD and its differential diagnoses.

Isolated rheumatoid arthritis-associated cerebral vasculitis: a diagnostic challenge.

Central nervous system involvement in rheumatoid arthritis is uncommon. In order of frequency, published cases have reported rheumatoid nodules, meningeal vasculitis, and cerebral vasculitis (CV). The frequency of vasculitic cerebral involvement in rheumatoid arthritis is unknown. However, it is known that it is more common in patients with seropositive, long-standing rheumatoid arthritis, and in those with concomitant systemic vasculitis. We report the case of a 47-year-old woman with an 11-year history of seropositive rheumatoid arthritis without any extra-articular manifestations, with the exception of secondary Sjogren's syndrome, presenting with mental status changes and tonic-clonic seizures as symptoms of isolated CV. Magnetic resonance imaging (T2) showed hyperintense white-matter lesions in the frontal and temporal lobes, as well as in the hippocampus and cerebellum. Transcranial Doppler ultrasound findings were consistent with CV. Other differential diagnoses were ruled out. The patient responded favorably to methylprednisolone and intravenous gammaglobulin therapy.

Manifestaciones clínicas, diagnóstico y tratamiento de la vasculitis de Wegener / Wegener vasculitis: clinical manifestations, diagnosis findings and treatment

La granulomatosis de Wegener es una vasculitis sistémica rara y, como consecuencia del compromiso de múltiples órganos, los pacientes afectados pueden consultar con diversos especialistas. En su patogenia se relaciona con un proceso inflamatorio que afecta la pared vascular. El cuadro clásico de la enfermedad muestra compromiso tanto respiratorio como renal, aunque también pueden estar comprometidos otros órganos. Esta revisión describe el valor diagnóstico de los ANCA y la importancia de las imágenes en el diagnóstico y seguimiento de la enfermedad. Se describen los criterios diagnósticos y el tratamiento, así como los efectos adversos de los fármacos utilizados. Además, se enfatiza en la necesidad de un diagnóstico rápido, para lo cual es necesaria la alta presunción diagnóstica de la enfermedad.

Cerebral vasculitis caused by Aspergillus simulating ischemic stroke in an immunocompetent patient.

Although Aspergillus is widespread, clinically significant disease is rare in immunocompetent patients. We present a case of an otherwise healthy individual who developed cerebral vasculitis and stroke symptoms from Aspergillus, to raise awareness of this entity.

Vascular involvement of the central nervous system and systemic diseases: etiologies and MRI findings.

The objective of this study was to review magnetic resonance imaging (MRI) findings in patients with vascular involvement of the central nervous system (CNS) associated with systemic diseases. We reviewed the MRI findings in clinically suspected cases of vascular involvement of the CNS associated with systemic diseases. Vascular CNS involvement was considered in the presence of characteristic clinical, MRI and/or MR angiography findings. In order to be included in the study, patients needed to have a complete clinical and laboratory investigation and a follow-up of a minimum of 6 months. Twenty-four patients (17 women and 7 men), with mean age of 29.5 years had diagnosis of CNS vasculitis and were included. The clinical presentation was variable, but the most common complaints were headache in 18, focal deficits in 9, disturbances of consciousness in 9, and seizures in 8 patients. Underlying causes for CNS vasculitis were identified in all patients and included systemic lupus erythematosus in eight, tuberculosis in three, bacterial meningitis in three, Takayasu arteritis in two, polyarteritis nodosa in two, syphilis in two, drug abuse in two, yellow fever in one and varicella in one patient. Nonspecific high intensity T2WI/FLAIR lesions in white matter were the most common finding, present in ten patients. Eight patients had infarctions in large cerebral arteries territory, associated or not with high intensity T2WI/FLAIR small foci. Vascular involvement of the CNS can be found in a great variety of systemic diseases, including rheumatologic, infectious and drug abuse. Clinical findings are unspecific and MRI/MRA may help to establish the correct diagnosis.

Unusual clinical cases that mimic MS.

Diseases which are associated with relapses and disability accumulation that mimic MS may be effectively treated if they are appropriately diagnosed. Unfortunately, however, it can be easy to assume that a patient is presenting with unusual symptoms of MS rather than establish unequivocally that there is no other cause. This article illustrates five short cases from a selection presented at the MS Forum Interactive Symposium at the LACTRIMS Congress, Brazil, 2004. These cases feature the differential diagnosis of MS and illustrate the importance of early and accurate diagnosis. Each case has clinical features suggestive of MS, together with diagnostic findings that are discordant with this disease. Clinical features that can easily be interpreted as unusual MS presentations, but which indicate the need to undertake additional investigation, are included.