Psicosis y alteraciones en la sensopercepción como manifestaciones atípicas de la poliangitis microscópica / Psychosis and alterations in sensory perception as atypical manifestations of microscopic polyangiitis

RESUMEN La poliangitis microscópica es una vasculitis sistémica, asociada a la positividad de anticuerpos anticitoplasma de neutrófilos, caracterizada por el compromiso necrosante de los vasos de pequeño calibre. Las manifestaciones clínicas de la poliangitis microscópica son variadas y las formas más severas se manifiestan con glomerulonefritis rápidamente progresiva y capilaritis pulmonar. El compromiso nervioso afecta principalmente el sistema periférico. La afectación a nivel central no es común, y hallazgos clínicos de psicosis y alteración del comportamiento son bastante infrecuentes en el contexto de estas enfermedades autoinmunes.

A B S T R A C T Microscopic polyangiitis is a systemic anti-neutrophil cytoplasmic antibody-associated vasculitis, and is associated with the necrotising small calibre vessels. Its clinical manifestations are varied, and the most severe forms manifest with rapidly progressive glomerulonephritis and pulmonary capillaritis. In the nervous system, it mainly involves the peripheral system. Involvement of the central nervous system is not common, and clinical findings of psychosis and behaviour alterations are infrequent.

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.

Vasculitis primaria del Sistema Nervioso Central: un desafío diagnóstico / Central Nervous System Primary Vasculitis: a diagnostic challenge

La vasculitis primaria del Sistema Nervioso Central (VPSNC) es una variedad poco frecuente de vasculitis que compromete el encéfalo y la medula espinal exclusivamente. La presentación es heterogénea y poco sistematizable, pudiendo comprometer más de una estructura encefálica al mismo tiempo. El diagnóstico se establece con un cuadro clínico compatible, asociado a una angiografía que evidencie un patrón de vasculitis y/o biopsia de parénquima encefálico o meninges. Las alteraciones documentadas en la Resonancia Nuclear Magnética son constantes pero inespecíficas pudiendo la angiografía no evidenciar lesión sugestiva si el afectado es un pequeño vaso. Presentamos tres casos clínicos de probable VPSNC basado en la presentación clínica, los hallazgos imagenológicos compatibles así como la respuesta terapéutica a ella. En los casos analizados se constataron alteraciones en la resonancia nuclear magnética encefálica que sugerían compromiso de varias estructuras encefálicas, con un líquido cefalorraquídeo inflamatorio y alteraciones en el electroencefalograma. El tratamiento de inducción, se realizó en base a bolos de metil-prednisolona y el de mantenimiento fue con corticoides vía oral. Presentaron una excelente respuesta al tratamiento instituido tanto clínica como imagenológicamente.

The primary central nervous system vasculitis is an uncommon variety of vasculitis that exclusively compromises the encephalon and the bone marrow. It arises as heterogeneous and unsystematized and it could compromise more than one encephalic structure at the same time. The diagnosis is made based on compatible symptoms supported by an angiography showing evidence of a vasculitis pattern or/and an encephalic parenchyma or meninges biopsy. The alterations documented in the Nuclear Magnetic Resonance are frequent but unspecific and it is possible to find no evidence of the injury if only a small vessel is affected. We present three clinical cases of probably PCNSV in which the diagnosis was made based on: the clinical presentation, imaginologicfindingsand therapeutic response. In the cases studied the alterations of the NMR were confirmed, the results suggested that several encephalic structures were compromised with CSF (cerebrospinal fluid) inflammatory and alterations on the EEG. The induction therapy included Methilprednisolone boluses; and the maintenance therapy, locally applied steroids. The response to the treatment was excellent clinically as well as imaginologically.

Polimiosite associada à arterite linfocítica do sistema nervoso central / Polymyositis associated with lymphocytic arteritis of the central nervous system

Complicações do Sistema Nervoso Central (SNC) raramente são descritas em miopatias inflamatórias idiopáticas. Os autores relatam o caso de uma paciente de 48 anos com diagnóstico de polimiosite com autoanticorpo anti-Jo-1 positivo que, após cinco anos de evolução, apresentou extensa lesão desmielinizante do SNC associada à arterite linfocítica.

Central Nervous System (CNS) complications in idiopathic inflammatory myopathies are seldom reported. The authors describe the case of a 48-year old female with polymyositis and positive anti-Jo-1 autoantibody who, after five years of evolution, developed extensive CNS demyelinating injury associated with lymphocytic arteritis.

Differential diagnosis of central nervous system vasculitis and mitochondrial encephalomyopathy / 第二军医大学学报

Objective: To compare the clinical and imaging characteristics between central nervous system vasculitis (CNSV) and mitochondrial encephalomyopathy (ME), so as to analyze the differential diagnosis of the two disorders. Methods: Clinical data on seven patients with CNSV and five with ME were retrospectively analyzed. The clinical manifestations, laboratory parameters, imaging features and histological characteristics were compared to screen for the evidence of their differential diagnosis. Results: The MRI results of both CNSV patients and ME patients (MELAS type) showed a multi-lesion pattern. The symptoms of CNSV patients included headache, limbs weakness, and erythrocyte sedimentation rate (ESR) increase. The symptoms of MELAS patients included epilepsy and increased serum lactic acid. The electroencephalographic manifestations of both diseases were abnormal: CNSV patients mainly had diffused lesions accompanied with limited alterations; ME patients had evidence of epileptic discharge, which was consistent with the clinical symptoms. Conclusion: Clinical manifestations of CNSV and ME patients are more valuable than imaging findings in the diagnosis of the two diseases. CNSV is characterized by vascular disorders and inflammatory reactions; ME is characterized by abnormal energy metabolism and severe damage of gray matter. The final diagnosis should depend on laboratory and histological examinations.

Stroke Caused by Central Nervous System Vasculitis: 49 Cases Report / 中国康复理论与实践

@#Objective To explore the clinical manifestation of stroke caused by central nervous system vasculitis (CNSV). Methods 49 patients who suffered from stroke by CNSV were reviewed retrospectively. Results Among the 49 patients, 5 were intracerebral hemorrhage, 15 were ventricular hemorrhage, 7 were subarachnoid hemorrhage and 22 were cerebral infarction; the mean of age was (26.0±12.7) years old. Conclusion The clinical manifestation of stroke caused by CNSV is variform, and usually happens in young age. CNSV should be considered as the cause of stroke in adolescent.

Differential diagnosis of central nervous system vasculitis and mitochondrial encephalomyopathy / 第二军医大学学报

Objective:To compare the clinical and imaging characteristics between central nervous system vasculitis(CNSV)and mitochondrial encephalomyopathy(ME),so as to analyze the differential diagnosis of the two disorders.Methods:Clinical data on seven patients with CNSV and five with ME were retrospectively analyzed.The clinical manifestations,laboratory parameters,imaging features and histological characteristics were compared to screen for the evidence of their differential diagnosis.Results:The MRI results of both CNSV patients and ME patients(MELAS type)showed a multi-lesion pattern.The symptoms of CNSV patients included headache,limbs weakness,and erythrocyte sedimentation rate(ESR)increase.The symptoms of MELAS patients included epilepsy and increased serum lactic acid.The electroencephalographic manifestations of both diseases were abnormal:CNSV patients mainly had diffused lesions accompanied with limited alterations;ME patients had evidence of epileptic discharge,which was consistent with the clinical symptoms.Conclusion:Clinical manifestations of CNSV and ME patients are more valuable than imaging findings in the diagnosis of the two diseases.CNSV is characterized by vascular disorders and inflammatory reactions;ME is characterized by abnormal energy metabolism and severe damage of gray matter.The final diagnosis should depend on laboratory and histological examinations.