Delayed onset cerebral vasculitis from chronic Schistosoma mansoni infection in Myanmar: A case report

@#Neuroschistosomiasis is an infection of the central nervous system caused by Schistosoma species and constitute a severe manifestation of the disease. Schistosoma japonicum is well-known cause of cerebral schistosomiasis and Schistosoma mansoni for spinal schistosomiasis. Although neuroschistosomiasis is not rare, reports on cerebral vasculitis associated with Schistosoma mansoni infection is scarce. With regards to schistosomiasis in Asia, most of the published literature has been on Schistosoma japonicum and the prevalence of Schistosoma mansoni in Asia is not well established. We report here a 54-yearold Rakhine woman with history of diabetes mellitus for more than ten years presenting with three recurrent stroke occurring over 2 months, twelve months after returning to Yangon from Rakhine, an endemic area for Schistosoma mansoni infection. Cerebral MRA revealed beaded appearance along left ACA and segmental narrowing beyond terminal ICA both sides, and contrast enhancement was noted at left frontal lobe with restricted diffusion on MRI brain. Serum serology testing for Schistosoma mansoni infection was positive. With oral praziquantel and high dose corticosteroid, aphasia and swallowing improved. This is the first report of delayed onset cerebral vasculitis associated with chronic Schistosoma mansoni infection in Asia.

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.

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)

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.

A Case of Henoch-Schonlein Purpura Nephritis Complicating Encephalopathy Accompanied by Hypertension and Cerebral Vasculitis

Henoch-Shonlein purpura(HSP) is a systemic small-vessel vasculitis that primarily affects the skin, gastrointestinal tract, joints, and kidneys. The nervous system may be involved, less commonly than other organs. When the central nervous system(CNS) was involved, headache, changes in mental status, seizures, and focal neurologic deficits have been reported. Hypertension, uremic encephalopathy, metabolic abnomalities, electrolyte abnormalities, or cerebral vasculitis were suggested as possible causes of the neurologic manifestation. Diagnosis of vasculitic involvement of CNS is difficult. Magnetic resonance imaging of the brain is the modality of choice for the evaluation of the CNS disease. Steroid or plasmapheresis are used in treatment of cerebral vasculitis. We experienced a case of 9-year-old boy who had presented with Henoch-Schonlein purpura nephritis complicating encephalopathy accompanied by hypertension and cerebral vasculitis. Brain MRI showed multiple small nodular-linear pattern enhancing lesions in whole cerebral hemispheres and focal increased T2 signal in the right basal ganglia. We used intravenous immunoglobulin in treatment of cerebral vasculitis. We report this case with a brief review of related literature.

A Case of Cerebral Vasculitis in Henoch-Schonlein Purpura / 대한소아신경학회지

Henoch-Schonlein purpura(HSP), also known as anaphylactoid purpura, is a vasculitis of small vessels. Skin, gastrointestinal tract, joints, and kidney are usual location of the acute small vascular damage. Neurologic complications are rare during course of HSP. Hypertensive encephalopathy, uremic encephalopathy, metabolic abnormalities, electrolyte abnormalities or cerebral vasculitis are possible causes of the neurological manifestations of HSP. A 6-year-old girl with HSP presented generalized tonic seizure with drowsy mentality. Electroencephalography(EEG) revealed frequently or nearly continuously diffuse high voltage arrhythmic delta activities and polysharp waves on right hemisphere and prominent polysharp waves on right parieto-occipital areas. Brain magnetic resonance imaging(MRI), showed multifocal high signal intensities in right frontoparietal and left parietal areas involving cortex and subcortical white matter on T2 weighted and fluid attenuated inversion recovery(FLAIR) images. These lesions were compatible with manifestations of vasculitis related HSP encephalopathy. She received oral prednisolone and anticonvulsant therapy. Multifocal T2 hyperintensities had been resolved completely 4 month later with clinical improvement.

A Case of Cerebral Vasculitis in Henoch-Sh nlein Purpura

Henoch-Sh nlein Purpura(HSP) is an immuologically mediated systemic vasculitis of small blood vessels affecting predominantly the skin, gastrointestinal tract, joints, and kidneys. Clinical neurological manifestations such as headaches, behavioral changes, mental changes, seizures, and visual loss are described, but neurological complication are rare during the course of HSP. We experienced a case of an 8 year-old male with HSP who presented with seizures. Magnetic resonance imaging(MRI) showed multiple high signal intensity in both cortical and subcortical areas of frontal and parieto-occipital lobes and magnetic resonance(MR) angiogram showed stenosis of cerebral arteries, compatible with MRI and MR angiogram findings of cerebral vasculitis. We report this case with related literature.