Clinical management of infectious cerebral vasculitides.

A wide range of infections (virus, bacteria, parasite and fungi) may cause cerebral vasculitides. Headache, seizures, encephalopathy and stroke are common forms of presentation. Infection and inflammation of intracranial vessels may cause pathological vascular remodelling, vascular occlusion and ischemia. Vasculitis in chronic meningitis may cause ischemic infarctions, and is associated with poor outcome. Appropriate neuroimaging (CT-angiography, MR-angiography, conventional 4-vessel angiography) and laboratory testing (specific antibodies in blood and CSF, CSF culture and microscopy) and even brain biopsy are needed to quickly establish the aetiology. Enhancement of contrast, wall thickening and lumen narrowing are radiological signs pointing to an infectious vasculitis origin. Although corticosteroids and prophylactic antiplatelet therapy have been used in infectious cerebral vasculitis, there are no randomized clinical trials that have evaluated their efficacy and safety. Stable mycotic aneurysms can be treated with specific antimicrobial therapy. Endovascular therapy and intracranial surgery are reserved for ruptured aneurysms or enlarging unruptured aneurysms.

Stroke subtypes and comorbidity among ischemic stroke patients in Brasilia and Cuenca: a Brazilian-Spanish cross-cultural study.

BACKGROUND: With the increase in life expectancy worldwide, changes in stroke subtypes and burden of stroke population are expected in both developing and developed countries. Prevalence of stroke subtypes and comorbidity in ischemic stroke patients was assessed in Brasilia, Brazil, and Cuenca, Spain. METHODS: This was an international (Brazilian-Spanish) cross-sectional study. Stroke subtypes were assessed by means of Trial of ORG 10172 in Acute Stroke Treatment (TOAST) classification. Modified Rankin scale was used to measure functional recovery and the Cumulative Illness Rating Scale for Geriatrics (CIRS-G) was used to assess comorbidity. RESULTS: A total of 500 patients (mean age 66.2 ± 16.4 years; 48% female; 48.2% Spanish) were included in the study. Spanish patients were significantly older than Brazilian ones (76.4 ± 11.2 versus 56.7 ± 14.6 years; P < .0001). Prevalence of ischemic cardiopathy (20.3% versus 6.2%) and atrial fibrillation (25.7% versus 6.6%) was significantly higher in Spanish stroke patients, whereas they less frequently used tobacco (28.3% versus 52.9%); P less than .0001. Prevalence of stroke subtypes in Spanish and Brazilian stroke patients was: stroke of undetermined etiology (58.1% versus 32.4%), cardioembolism (24.5% versus 11.6%), lacunar infarct (11.6% versus 25.5%), atherothrombotic (3.7% versus 19.7%), and other causes (2.1% versus 10.8%); P less than .0001. The Spanish sample had a significantly higher frequency of comorbidities. The CIRS-G total score and CIRS-G mean number of affected organs significantly increased with age, and correlated with the level of functional dependence as measured by Rankin scale (rS = 0.50; P = .0005). CONCLUSION: Spanish stroke people had a higher frequency of comorbid conditions, atrial fibrillation, and cardioembolism and these facts were associated with age. Atherothrombotic and lacunar strokes were more common in the younger Brazilian stroke population.

Cerebral and spinal schistosomiasis.

Cerebral schistosomiasis and spinal schistosomiasis are severe underrecognized complications of Schistosoma sp. infection, and can occur at any time during the parasitic infection. Neuroschistosomiasis has been increasingly reported not only in endemic areas but also in Western countries owing to immigration and international travel. Immunogenic interaction between schistosome egg deposition and the delayed hypersensitivity reaction of the host are the main neuropathogenic mechanisms involved. Eggs induce a periovular granulomatous reaction in the tissues. In some cases, schistosome adult worms may aberrantly migrate to the central nervous system via the vertebral venous plexus and place the ova at an ectopic site. Headache and seizures are common in cerebral schistosomiasis, and intracranial hypertension and hydrocephalus may occur in tumour-like and cerebellar schistosomiasis. Spinal schistosomiasis may manifest itself as acute myelitis and/or myeloradiculopathy. Recognition of neuroschistosomiasis is important so that early treatment with praziquantel and steroids can be started in an attempt to prevent severe disability.

Stroke in asymptomatic Trypanosoma cruzi-infected patients.

BACKGROUND: The knowledge about the natural history of stroke in Chagas disease is incomplete. METHODS: Vascular risk factors and stroke subtypes of asymptomatic Trypanosoma cruzi-infected patients with no clinical evidence of heart failure were assessed. They were compared with chronic chagasic cardiomyopathy patients who suffered a stroke and with a control group of 60 T. cruzi-noninfected stroke patients. Eighty-six consecutive chagasic stroke patients (mean age: 57.4 years; 64% females) were studied. RESULTS: 38.4% of chagasic stroke patients had asymptomatic T. cruzi infection. Smoking was more frequent in asymptomatic chagasic stroke patients (21.2 vs. 5.7%; p = 0.04). Prevalence of hypertension, diabetes and prior stroke was similar in both groups. Small-vessel infarction (15.6 vs. 3.8%) and large-vessel atherosclerosis (9.4 vs. 3.8%) were significantly more frequent in asymptomatic than in symptomatic T. cruzi-infected stroke patients (p = 0.001). Nevertheless, their frequency was even higher in T. cruzi-noninfected stroke patients (36.7 and 13.3%, respectively). Apical aneurysm (27.3%), left atrial dilatation (12.1%), left ventricle hypokinesia (9.4%) and right bundle branch block (36.4%) were also detected in asymptomatic T. cruzi-infected stroke patients. CONCLUSIONS: Ischemic stroke may be the first manifestation of Chagas disease in asymptomatic patients with mild left ventricle dysfunction. Other noncardioembolic stroke subtypes can occur in asymptomatic T. cruzi-infected patients.

Neuroschistosomiasis.

Neuroschistosomiasis, the infection of the CNS by Schistosoma spp., is a neglected and under-recognized complication of schistosomiasis. Cerebral and spinal neuroschistosomiasis can provoke severe disability. Neurological symptoms occur as a consequence of the immune reaction around the eggs deposited in the CNS. Cerebral neuroschistosomiasis may present with altered sensorium, headache, seizures and focal neurological deficit. Pseudotumoral and cerebellar neuroschistosomiasis may provoke intracranial hypertension and hydrocephalus. Brain-enhancing lesions with associated mass effect can be observed on MRI. Transverse myelitis and myeloradiculopathy affecting the conus medullaris and/or cauda equina are the most common spinal cord syndromes. Transverse myelitis can present as flaccid arreflexic paraplegia with sensory level and sphincter dysfunction. Praziquantel and corticoids have been successfully used to treat neuroschistosomiasis. Ventricle-peritoneal shunt may be necessary to treat hydrocephalus associated with tumor-like brain and/or cerebellar schistosomiasis.

Chronic spinal epidural abscess caused by Scedosporium prolificans in an immunocompetent patient.

STUDY DESIGN: Case report and clinical discussion. OBJECTIVE: To describe a rare case of chronic spinal epidural abscess caused by Scedosporium prolificans in an immunocompetent patient. SUMMARY OF BACKGROUND DATA: S. prolificans is an emerging multiresistant fungal pathogen that may cause a wide variety of infections, whose severity depends of the patient's immune status. METHODS: A 35-year-old Brazilian man developed a 5-year chronic progressive paresis in lower limbs followed by sphincter dysfunction. Spinal magnetic resonance imaging revealed a thoracic-lumbar epidural mass that heterogeneously enhanced gadolinium. Spinal biopsy showed a chronic inflammatory process localized in epidural thoracic spine. S. prolificans was isolated in a specific culture. RESULTS: He was treated with endovenous voriconazole for 2 weeks followed by oral voriconazole for 90 days. Three months later, a T9-T10 epidural mass was detected on a new magnetic resonance imaging. The abscess was drained, and the culture of the material confirmed the persistence of infection by S. prolificans. A progressive paraparesis due to chronic meningo-myelitis was observed at a 6-month follow-up. Although patient was treated with caspofungin, terbinafine, and voriconazole, no improvement was observed. CONCLUSION: S. prolificans can cause chronic epidural abscess and spine infection in immunocompetent patients. The potential resistance of S. prolificans to antifungal agents should be borne in mind.

Neurological complications of Schistosoma infection.

Schistosomiasis is a parasitic disease caused by blood flukes of the genus Schistosoma. Currently more than 200 million people worldwide are affected. Neuroschistosomiasis constitutes a severe presentation of the disease. Neurological symptoms result from the inflammatory response of the host to egg deposition in the brain and spinal cord. Neurological complications of cerebral schistosomiasis include delirium, loss of consciousness, seizures, dysphasia, visual field impairment, focal motor deficits and ataxia. Cerebral and cerebellar tumour-like neuroschistosomiasis can present with increased intracranial pressure, headache, nausea and vomiting, and seizures. Myelopathy (acute transverse myelitis and subacute myeloradiculopathy) is the most common neurological complication of Schistosoma mansoni infection. Schistosomal myelopathy tends to occur early after infection and is more likely to be symptomatic than cerebral schistosomiasis. The conus medullaris and cauda equina are the most common sites of involvement. Severe schistosomal myelopathy can provoke a complete flaccid paraplegia with areflexia, sphincter dysfunction and sensory disturbances. Schistosomicidal drugs, steroids and surgery are the currently available treatments for neuroschistosomiasis. Rehabilitation and multidisciplinary team care are needed in severely disabled patients.

Spectrum of HSPG2 (Perlecan) mutations in patients with Schwartz-Jampel syndrome.

Schwartz-Jampel syndrome (SJS) is a rare autosomal recessive condition defined by the association of myotonia with chondrodysplasia. SJS results from mutations in the HSPG2 gene, which encodes perlecan, a major component of basement membranes. Only eight HSPG2 mutations have been reported in six SJS families. Here, we describe the molecular findings in 23 families (35 patients) with SJS, being one-third of the SJS cases reported in the medical literature. We identified 22 new HSPG2 mutations and unreported polymorphisms. Mutations included nine deletion or insertion (41%), six splice site (27%), five missense (23%), and two nonsense mutations (9%). All but four mutations were private, and we found no evidence for a founder effect. Analyses of HSPG2 messenger RNA (mRNA) and perlecan immunostaining on patients' cells revealed a hypomorphic effect of the studied mutations. They also demonstrated distinct consequences of truncating and missense mutations on perlecan expression as truncating mutations resulted in instability of HSPG2 mRNA through nonsense mRNA-mediated decay, whereas missense mutations involving cysteine residues led to intracellular retention of perlecan, probably due to quality control pathways. Our analyses strengthen the idea that SJS results from hypomorphic mutations of the HSPG2 gene. They also propose tools for its molecular diagnosis and provide new clues for the understanding of its pathophysiology.

Distonia psicogênica: relato de dois casos / Psychogenic dystonia: report of 2 cases

Desordens de movimento raramente podem ser devidas a distúrbios psiquiátricos. A distonia psicogênica caracteriza-se pela inconsistência dos achados, presença de fatores precipitantes, manifestar-se inicialmente nos membros inferiores, associar-se a dor, a outros movimentos anormais incaracterísticos e a somatizações múltiplas. Descrevemos duas pacientes com diagnóstico de distonia psicogênica clinicamente estabelecida. Paciente 1, feminina, apresentou episódio súbito de perda de força dos quatro membros, evoluiu com distonia nos pés, laterocolo alternante, tremor generalizado, irregular, e hipertonia dos membros inferiores que desapareciam a distração; a avaliação psicológica evidenciou depressão, hipocondria, transtorno obsessivo. Paciente 2, feminina, há nove anos começou a ter tremor irregular nos membros inferiores, que desaparecia com a distração, e distonia no pé esquerdo associada a dor; progressivamente perdeu a marcha; a avaliação psicológica revelou comportamento infantilizado, com baixa tolerância a frustração, impulsividade e auto-agressão. Os exames complementares de ambas não mostraram alterações e a resposta ao tratamento farmacológico foi nula. Distonia raramente é de origem psicogênica. A inconstância e a incongruência com o quadro clássico, associadas a outras somatizações ou a distúrbios psiquiátricos, sugerem o diagnóstico.