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.

Understanding the importance of cerebrovascular involvement in Kawasaki disease / 소아과

Kawasaki disease (KD) is a systemic vasculitis in infants and young children. However, its natural history has not been fully elucidated because the first case was reported in the late 1960s and patients who have recovered are just now entering middle age. Nevertheless, much evidence has raised concerns regarding the subclinical vascular changes that occur in post-KD patients. KD research has focused on coronary artery aneurysms because they are directly associated with fatality. However, aneurysms have been reported in other extracardiac muscular arteries and their fate seems to resemble that of coronary artery aneurysms. Arterial strokes in KD cases are rarely reported. Asymptomatic ischemic lesions were observed in a prospective study of brain vascular lesions in KD patients with coronary artery aneurysms. The findings of a study of single-photon emission computed tomography suggested that asymptomatic cerebral vasculitis is more common than we believed. Some authors assumed that the need to consider the possibility of brain vascular lesions in severe cases of KD regardless of presence or absence of neurological symptoms. These findings suggest that KD is related with cerebrovascular lesions in children and young adults. Considering the fatal consequences of cerebral vascular involvement in KD patients, increased attention is required. Here we review our understanding of brain vascular involvement in KD.

A Case of Primary Central Nervous System Vasculitis That Worsened Despite Early Corticosteroid Therapy

Primary central nervous system vasculitis (PCNSV) is rare, and the diagnosis is difficult to make because of its variable radiologic expressions. Early corticosteroid therapy often is effective. Herein we report the case of a 56-year-old man who had a well-enhanced cystic mass with severe edema in the right frontal lobe, which was initially felt to be a malignancy. Histologic examination of tissue removed at craniotomy revealed that it was a PCNSV. Despite early administration of corticosteroids, a new lesion developed within 3 days. The lesions responded to treatment with cyclophosphamide and corticosteroid.

Vasculitis primaria del sistema nervioso central en población pediátrica: reporte de caso y revisión de la literatura / Childhood primary vasculitis of the central nervous system: Case report and literature review

RESUMEN La vasculitis primaria del sistema nervioso central es una enfermedad infrecuente que puede afectar a adultos y población pediátrica. Puede comprometer los vasos sanguíneos cerebrales tanto de mediano o gran calibre como los de pequeño calibre y estar asociada a procesos inflamatorios, infecciosos, tumorales o ser de origen idiopático. Describimos el caso de un adolescente con deterioro neurológico focal dado por hemiplejía derecha en quien se descartaron otras causas más frecuentes de isquemia cerebral, con diagnóstico final de vasculitis primaria del sistema nervioso central asociada a infección por virus de Epstein Barr.

ABSTRACT Primary angiitis of the central nervous system is an uncommon disease that may affect adults and the paediatric population. It can involve both the medium-large and small sized cerebral blood vessels, and can be associated with inflammatory, infectious, tumour processes, or of idiopathic origin. The case is presented of an adolescent with focal neurological impairment due to right hemiplegia, in whom other more frequent causes of cerebral ischaemia were ruled out. The final diagnosis was Epstein Barr virus associated with primary angiitis of the central nervous system.

Cognitive and psychiatric changes as first clinical presentation in Sneddon Syndrome / Alterações cognitivas e psiquiátricas como primeira apresentação clínica na Síndrome de Sneddon

ABSTRACT Sneddon syndrome (SS) is a rare progressive non-inflammatory thrombotic vasculopathy affecting small/medium-sized blood vessels of unknown origin. It is strongly associated with the presence of antiphospholipid antibodies (AA). The presence of livedo reticularis and cerebrovascular disease are hallmark features. The condition is far more common in young women. We report a case of SS in a 43 year-old male with a two-year history of progressive cognitive impairment consistent with dementia syndrome, and major personality changes, besides livedo reticularis and cerebral angiographic pattern of vasculitis. AA were borderline. The recognition of skin blemishes that precede strokes should raise the hypothesis of SS. AA are elevated in more than half of cases, but their role in the pathogenesis or association of positive antibodies and SS remains unclear. Dementia syndrome in young patients should be extensively investigated to rule out reversible situations. Typical skin findings, MRI and angiography may aid diagnosis.

RESUMO A síndrome de Sneddon (SS) é uma vasculopatia trombótica não inflamatória progressiva rara que afeta os vasos sanguíneos de pequeno e médio tamanho com origem desconhecida. Está fortemente associada à presença de anticorpos antifosfolipídios (AA). A presença de livedo reticularis e doença cerebrovascular são a marca registrada. É muito mais comum em mulheres jovens. Relatamos um caso de SS em um homem de 43 anos de idade com dois anos de história de comprometimento cognitivo progressivo compatível com síndrome demencial e mudanças graves de personalidade, além de livedo reticular e padrão angiográfico cerebral de vasculite. AA eram limítrofes. O reconhecimento das manchas da pele que precedem eventos isquêmicos cerebrovasculares deve reforçar a hipótese de SS. Os AA são elevados em mais da metade dos casos, mas seu papel na patogênese ou associação de anticorpos positivos e SS permanece obscuro. A síndrome demencial em pacientes jovens deve ser amplamente investigada para se descartarem situações reversíveis. Achados típicos da pele, ressonância magnética e angiografia podem ajudar no diagnóstico.

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.

Eosinophilic Granulomatosis with Polyangiitis Presented as Acute Polyneuropathy and Cerebral Vasculitis

Eosinophilic granulomatosis with polyangiitis (EGPA) is an immune related systemic disease that is caused by vasculitis affecting multiple organ systems. It is characterized by asthma, fever, eosinophilia, cardiac problems, renal injury, and peripheral neuropathy. In this report, we describe a patient with EGPA with concurrent cerebral infarction and acute polyneuropathy mimicking a Guillain-Barre syndrome (GBS). A 46-year-old man presented with rapidly progressing gait disturbance, muscular weakness, and tingling sensation in all four limbs. A nerve conduction study revealed sensorimotor polyneuropathy in all four limbs, and a test of the cerebrospinal fluid showed an albumin-cytologic dissociation. In addition, brain magnetic resonance imaging (MRI) using fluid-attenuated inversion recovery and diffusion weighted MRI revealed high signal intensity lesions with gadolinium enhancement on T1-weighted MRI in the right caudate nucleus. After performing laboratory tests, paranasal sinus computed tomography, and a nasal smear, the patient was diagnosed with EGPA and treated with high dose glucocorticoid and oral cyclophosphamide. In conclusion, our findings indicate that a diagnosis of EGPA should be considered when a patient presents with rapidly progressing polyneuropathy mimicking a GBS along with unusual systemic symptoms or brain lesions.

Degos-Like Lesions Associated with Systemic Lupus Erythematosus

Degos disease, also referred to as malignant atrophic papulosis, was first described in 1941 by Köhlmeier and was independently described by Degos in 1942. Degos disease is characterized by diffuse, papular skin eruptions with porcelain-white centers and slightly raised erythematous telangiectatic rims associated with bowel infarction. Although the etiology of Degos disease is unknown, autoimmune diseases, coagulation disorders, and vasculitis have all been considered as underlying pathogenic mechanisms. Approximately 15% of Degos disease have a benign course limited to the skin and no history of gastrointestinal or central nervous system (CNS) involvement. A 29-year-old female with history of systemic lupus erythematosus (SLE) presented with a 2-year history of asymptomatic lesions on the dorsum of all fingers and both knees. The patient had only skin lesions and no gastrointestinal or CNS vasculitis symptoms. Her skin lesions were umbilicated, atrophic porcelain-white lesions with a rim of erythema. On the basis of clinical, histologic, and laboratory findings, a diagnosis of Degos-like lesions associated with SLE was made. The patient had been treated for SLE for 7 years. Her treatment regimen was maintained over a 2 month follow-up period, and the skin lesions improved slightly with no development of new lesions.

Primary Central Nervous System Vasculitis Mimicking a Cortical Brain Tumor: A Case Report

We report a case of primary central nervous system vasculitis (PCNSV) mimicking a cortical brain tumor. A 25-year-old woman presented with a 2-week history of headache and transient right hemiparesis. Brain magnetic resonance imaging (MRI) revealed a cortical-involving lesion on the left frontal lobe. The 6-cm sized lesion showed low signal intensity on T1-weighted images and high signal intensity on T2-weighted images. The lesion had continual linear enhancement on the subcortical white matter and leptomeninges. There was no evidence of hemorrhage on susceptibility-weighted images and no diffusion restriction on diffusion-weighted images. The regional cerebral blood volume was decreased on the MR perfusion images, and spectroscopy showed increased lactate and lipid peaks. The symptoms were aggravated by fever and seizures. Biopsy was performed to rule out tumorous or inflammatory lesions. Pathologically, lymphocytes were infiltrated on the vessels, and the arachnoid membrane was thickened with inflammatory cells. The patient did not have any underlying diseases, including immune disorders. After high-dose steroid administration, her symptoms improved. Two months later, brain MRI showed a reduction in the infiltration of the T2 hyperintensity lesion with subtle subcortical enhancement. We present a case of PCNSV involving the left frontal lobe, showing vasogenic edema, mass effect, and subcortical linear contrast enhancement without hemorrhage or infarction.

Afectación cerebral en la granulomatosis de Wegener. Reporte de caso / Brain involvement in Wegener's granulomatosis. Case report

La granulomatosis con poliangeítis (GPA) ­antes denominada granulomatosis de Wegener­ es una enfermedad autoinmunitaria multisistémica poco frecuente, de etiología desconocida. En esta enfermedad el compromiso del sistema nervioso central es muy raro aunque tiene mucha importancia porque se relaciona con períodos refractarios. El diagnóstico diferencial de las manifestaciones cerebrales de la GPA es difícil porque no hay signos radiográficos excluyentes de otras lesiones intra o extra-axiales. Este artículo presenta el caso de un paciente con granulomatosis de Wegener de larga evolución, que debuta con crisis convulsivas y focalidad neurológica secundarias a una lesión intraaxial parietal izquierda. Las características sintomáticas, histopatológicas y radiográficas se describen cuidadosamente y se discute el papel de la cirugía para esclarecer el diagnóstico en este paciente. (AU)

The granulomatosis with polyangiitis (GPA), known before as Wegener's granulomatosis, is an autoimmune multisystemic disease, not so common, of unknown etiology. The compromise of this particular disease to the neurological central system is very rare although it's very important because of its relationship with refractory disease periods. The differential diagnosis of the manifestations of the GPA at cerebral level it's not easy to see because there are not that allows the exclusion of other lesions intra or extra axials. We present the case of the patient with Wegener granulomatosis of long evolution that begins with seizures and motor deficit secondary to a left parietal intra axial mass. The clinical characteristics histopathological and radiological are careful described and the role of surgery in management is discussed. (AU)

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.

Neurological Manifestations of Acute Posterior Multifocal Placoid Pigment Epitheliopathy

BACKGROUND AND PURPOSE: Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is an immune-mediated chorioretinal disease that causes acute visual symptoms with characteristic ophthalmoscopic findings. Neurological complications are rarely reported in the literature. Here we report two new cases of APMPPE that presented with neurological manifestations, one of which was associated with peripheral neuropathy, which has not been described before. METHODS: A retrospective database review of all patients with a diagnosis of APMPPE was performed. Clinical, ophthalmological, and neurological data were analyzed, and only cases of APMPPE with neurological complications were included. A literature review of several databases was also performed, and previous case reports were reviewed and analyzed in detail. RESULTS: In total, 56 cases of APMPPE-associated neurological complications were included in the analyses: 54 from the literature and 2 from our own practice. The most common complication was cerebral vasculitis, which affected 28 patients (50%), followed by headaches in 15 patients (26.8%). The other complications include sixth-cranial-nerve palsy, transient hearing loss, meningoencephalitis, cavernous sinus thrombosis, and viral meningitis. CONCLUSIONS: This report adds to the literature of a novel association of APMPPE with peripheral neuropathy, and comprehensively reviews the neurological manifestations of this disease. A high level of suspicion should be applied when dealing with a case of APMPPE. We recommend applying detailed clinical neurological examinations and magnetic resonance imaging to APMPPE patients, and then early steroid treatment if the examination is positive or even suspicious. Early treatment with steroids and long-term treatment with immunosuppressive azathioprine with interval neurological evaluations will contribute positively to the outcomes and avoid fatal complications, namely strokes.

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.

Review of giant cell arteritis

Giant-cell arteritis [GCA] is a systemic autoimmune disease affecting primarily the elderly. Giant cell arteritis can cause sudden and potentially bilateral sequential vision loss in the elderly. Therefore, it is considered a medical emergency in ophthalmology and a significant cause of morbidity in an increasingly aging population. Ophthalmologists need to be able to recognize the classic symptoms and signs of this disease, and then be able to work-up and treat these patients in an efficient manner. An in-depth review of GCA from the literature as well as personal clinical experience follows

Relapsed Brucellosis Presenting as Neurobrucellosis with Cerebral Vasculitis in a Patient Previously Diagnosed with Brucellar Spondylitis: A Case Report / 감염과화학요법

Brucellosis is a multisystem disease with various clinical symptoms. Neurobrucellosis is a rare but serious manifestation of brucellosis. A 60-year-old man with a previous diagnosis of brucellar spondylitis presented with sudden onset of aphasia and numbness of the right upper extremity. Cerebral angiography showed diffuse narrowing and dilatation on the distal branches of both the anterior cerebral artery (ACA) and the left middle cerebral artery (MCA) which indicated cerebral vasculitis, and the patient's Brucella agglutinin titer was 1:1280. After combined antimicrobial and steroid therapy was started, the patient's condition improved significantly, and he was discharged after 1 month. Antimicrobial therapy was continued for 16 months on an outpatient basis, and the last Brucella agglutinin titer was 1:40. To our knowledge, this is the first case of relapsed neurobrucellosis with vasculitis in Korea to have been treated successfully.

Síndrome da angústia respiratória aguda associada à varicela em paciente adulto: exemplo de suporte respiratório extracorpóreo em doenças endêmicas brasileiras / Varicella associated acute respiratory distress syndrome in an adult patient: an example for extracorporeal respiratory support in Brazilian endemic diseases

Descreveu-se aqui o caso de um homem de 30 anos de idade com quadro de varicela grave, hipoxemia refratária, vasculite do sistema nervoso central e insuficiência renal anúrica. Foi necessário transporte por ambulância com suporte respiratório extracorpóreo veno-venoso, sendo este utilizado até a recuperação do paciente. Discute-se o potencial uso de oxigenação por membrana extracorpórea em países em desenvolvimento para o controle de doenças comuns nestas áreas.

A case of a 30 year-old man presenting with severe systemic chickenpox with refractory hypoxemia, central nervous system vasculitis and anuric renal failure is described. Ambulance transportation and support using veno-venous extracorporeal membrane oxygenation were necessary until the patient recovered. Ultimately, the potential use of extracorporeal membrane oxygenation support in low-middle income countries to manage common diseases is discussed.

Role of High-Resolution Magnetic Resonance Imaging in the Diagnosis of Primary Angiitis of the Central Nervous System

BACKGROUND: Primary angiitis of the central nervous system (PACNS) is a rare disorder and is often difficult to diagnose due to the lack of a confirmatory test. PACNS can generally be diagnosed based on typical angiographic findings. We describe herein a patient diagnosed with PACNS despite the presence of normal findings on conventional angiography. CASE REPORT: A 44-year-old man with a recent history of ischemic stroke in the right posterior cerebral artery territory developed acute-onset vertigo. Diffusion-weighted imaging revealed an acute infarction within the left posterior inferior cerebellar artery. His medical history was unremarkable except for hyperlipidemia; the initial examination revealed mild gait imbalance. During the 10 days of hospital admission, the patient experienced four recurrent ischemic strokes within the posterior circulation territory (occipital lobe, pons, and cerebellum). He was diagnosed with recurrent cerebral infarctions due to PACNS. The basilar artery exhibited no demonstrable luminal stenosis, but there were direct imaging signs of central nervous system angiitis including wall thickening and contrast enhancement. High-dose intravenous steroid therapy followed by oral prednisolone was administered. There was no further stroke recurrence and follow-up imaging of the arterial walls showed normalization of their characteristics. CONCLUSIONS: The present case emphasizes the importance of wall imaging in the diagnosis and treatment of PACNS.

Rapidly progressive dementia due to neurosarcoidosis / Demência rapidamente progressiva causada por neurosarcoidose

Rapidly progressive dementia (RPD) is typically defined as a cognitive decline progressing to severe impairment in less than 1-2 years, typically within weeks or months. Accurate and prompt diagnosis is important because many conditions causing RPD are treatable. Neurosarcoidosis is often cited as an unusual reversible cause of RPD. METHODS: We report two cases of neurosarcoidosis presenting as RPD. RESULTS: Case 1: A 61-year-old woman developed a RPD associated with visual loss. In seven months she was dependent for self-care. Magnetic resonance imaging (MRI) revealed temporal and suprasellar brain lesions. Treatment with high-dose intravenous prednisolone was associated with partial improvement. Case 2: A 43-year-old woman who was being treated for diabetes insipidus developed a severe episodic amnesia one year after onset of cognitive symptoms. Previous MRI had shown a hypothalamic lesion and she had been treated with oral prednisone and cyclophosphamide. There was reduction of the MRI findings but no improvement in the cognitive deficits. Brain biopsy disclosed noncaseous granulomas and granulomatous angiitis; treatment was changed to high-dose intravenous methylprednisolone, with poor symptomatic response. CONCLUSION: The diagnosis of RPD due to neurosarcoidosis can be challenging when the disease is restricted to the nervous system. In these cases, clinical presentation of RPD associated with neuroendocrine and visual dysfunction, imaging findings showing hypothalamic lesions and, in some cases, brain biopsy, are the key to a correct diagnosis. It is possible that earlier diagnoses and treatment could have led to a better outcome in these patients.

Demência rapidamente progressiva (DRP) é tipicamente definida como um declínio cognitivo que progride para prejuízo funcional severo em menos de 1-2 anos, geralmente em semanas a meses. O diagnóstico rápido e acurado é fundamental, já que muitas condições que levam a DRP são reversíveis. MÉTODOS: Relatamos dois casos de neurosarcoidose que se apresentaram como DRP. RESULTADOS: Caso 1: Uma mulher de 61 anos desenvolveu uma DRP associada a perda de acuidade visual. Em sete meses evoluiu com dependência para auto-cuidado. A ressonância magnética (RM) revelou lesões encefálicas temporais e supraselares. Evoluiu com melhora parcial após tratamento com metilprednisolona intravenosa em altas doses. Caso 2: Uma mulher de 43 anos que estava em tratamento para diabetes insipidus desenvolveu uma amnésia episódica severa um ano após o início dos sintomas cognitivos. A RM anterior mostrava uma lesão hipotalâmica, e ela recebeu tratamento oral com prednisona e ciclofosfamida. Houve redução dos achados da RM, porem sem melhora dos déficits cognitivos. A biópsia cerebral mostrou granulomas não caseosos e angeíte granulomatosa; o tratamento foi modificado para metilprednisolona intravenosa em altas doses, com resposta sintomática pobre. CONCLUSÃO: O diagnóstico de DRP por neurosarcoidose pode ser desafiador quando a doença está restrita ao sistema nervoso central. Nestes casos, a apresentação clínica da DRP associada a disfunção neuroendócrina e visual, exames de imagem com lesões hipotalâmicas e, em alguns casos, a biópsia cerebral são fundamentais para um diagnóstico correto. é possível que o diagnóstico e tratamento precoces poderiam ter trazido melhores resultados nesses pacientes.