Intracranial Arachnoiditis and Hydrocephalus.

A 62-year-old man presented a diffuse and predominantly cisternal acute nonaneurysmal subarachnoid hemorrhage associated with hydrocephalus. An external ventricular drain was placed, followed by clinical deterioration after its removal. At this point, a heavily T2-weighted high-resolution sequence of a brain magnetic resonance imaging showed acute hydrocephalus recrudescence and multiple arachnoid adhesions in the supravermian and interpeduncular cisterns, creating a loculated/cystic appearance. The diagnosis of intracranial arachnoiditis was made. Intracranial arachnoiditis results from meningeal inflammation. Fibrosis and adhesions at the subarachnoid spaces may follow, restricting cerebrospinal fluid circulation, particularly at the cranial base. Hydrocephalus probably resulted from the combination of subarachnoid hemorrhage and extensive scaring at the basal cisterns, precluding transdural and transvenous cerebrospinal fluid efflux. Heavily T2-weighted high-resolution magnetic resonance imaging sequences allow an exquisite depiction of arachnoiditis, displaying obstructive cisternal membranes, and contribute to better etiologic assessment and management of hydrocephalus.

Predicting Outcome in a Cohort of Isolated and Combined Dystonia within Probabilistic Brain Mapping.

BACKGROUND: Probabilistic brain mapping is a promising tool to estimate the expected benefit of pallidal deep brain stimulation (GPi-DBS) in patients with isolated dystonia (IsoD). OBJECTIVES: To investigate the role of probabilistic mapping in combined dystonia (ComD). METHODS: We rendered the pallidal atlas and the volume of tissue activated (VTA) for a cohort of patients with IsoD (n = 20) and ComD (n = 10) that underwent GPi-DBS. The VTA was correlated with clinical improvement. Afterwards, each VTA was applied on the previously published probabilistic model (Reich et al., 2019). The correlation between predicted and observed clinical benefit was studied in a linear regression model. RESULTS: A good correlation between observed and predicted outcome was found for both patients with IsoD (n = 14) and ComD (n = 7) (r2 = 0.32; P < 0.05). In ComD, 42% of the variance in DBS response is explained by VTA-based outcome map. CONCLUSION: A probabilistic model would be helpful in clinical practice to circumvent unpredictable and less impressive motor results often found in ComD.

Vanishing Pseudotumoral White Matter Lesions Presenting as Aphasia and Altered Mental Status in a 71-Year-Old Male.

Acute disseminated encephalomyelitis (ADEM) is a demyelinating disorder that usually affects the central nervous system (CNS) after an infection and/or vaccination. It is more common in infancy. Here we present a case of late onset ADEM. A 71-year-old male was admitted to the emergency department due to speech difficulty and somnolence. Upon neurological examination he had a mixed aphasia. He performed a brain computed tomography which showed multiple white matter hypodense lesions. After admission to the neurology ward, he performed a lumbar puncture which showed a mildly inflammatory cerebrospinal fluid, negative serological testing and negative oligoclonal bands. Brain magnetic resonance imaging (MRI) confirmed the presence of multiple T2 hyperintense lesions, extensive bilateral frontoparietal lesions with abundant perilesional edema, four with gadolinium enhancement in an open-ring pattern and no mass effect. Anti-aquaporin 4 antibody, virologic and bacteriologic blood testing, screening of autoimmune disorders and occult neoplasm were all unremarkable. He was treated with intravenous methylprednisolone (1 gr) during five days and started to recover, maintaining a slight verbal fluency deficiency. Post-treatment brain MRI showed reduction of previous lesions, corroborating the probable inflammatory/demyelinating etiology. After discharge he maintained follow-up at the neurology outpatient clinic and he is currently asymptomatic with no new lesions and further reduction of the previous ones on follow-up MR scan. Both clinical follow-up of the patient, revealing a monophasic course with complete recovery, and temporal evolution of his brain lesions were essential to establish a diagnosis of ADEM in a septuagenarian patient, in whom other diagnoses have to be considered.

Endoscopic Third Ventriculostomy for the Treatment of Hydrocephalus in a Pediatric Population with Myelomeningocele.

BACKGROUND: Hydrocephalus develops in up to 90% of patients born with myelomeningocele. Although endoscopic third ventriculostomy (ETV) is currently considered the preferred treatment for obstructive hydrocephalus, its results have been inconsistent in patients with myelomeningocele. This study focuses on clinical and radiologic outcomes of ETV in children with hydrocephalus related to myelomeningocele. METHODS: Medical records of 18 pediatric patients with myelomeningocele treated with ETV from 1998 to 2015 at the Centro Hospitalar São João (Porto, Portugal) were reviewed retrospectively. Patients' caregivers were contacted to evaluate their clinical manifestations before and after surgery regarding signs and symptoms of hydrocephalus and Chiari malformation. Control neuroradiologic imaging of 9 patients was obtained and analyzed. Success of ETV was defined by clinical resolution and radiologic confirmation. RESULTS: ETV was successful in 8 of 18 cases (44.4%). Groups of patients were compared according to age at the time of surgery, with a 40% (2/5) success rate in newborns and a 50% success rate (3/6) in children older than 1 year. Eight patients underwent ETV as a first option, with a 37.5% success rate. Ten patients underwent the procedure after previous ventriculoperitoneal shunt (VPS), 5 for malfunction and 5 for VPS infection with 60% and 40% success rates, respectively. Early postoperative complications occurred in 2 patients. CONCLUSIONS: ETV can be performed in patients with myelomeningocele and hydrocephalus with success rates of almost 50%. Prior VPS or VPS malfunction or infection do not contraindicate ETV. If possible, the procedure should be delayed until the patient is at least 1 month old.

Behavior Changes and Gait Unsteadiness: The Value of Imaging and Prompt Neurosurgical Intervention.

Cavernous angiomas are central nervous system malformations. Most common manifestations are seizures and acute focal neurological deficits. We present a case report of a seventy-one year-old man with a two-month history of behavior changes, attention deficit and indifference followed by gait unsteadiness. Neuropsychological evaluation showed severe cognitive impairment and executive dysfunction. Head computed tomography depicted a supraventricular hydrocephaly. Magnetic resonance imaging revealed a small hemorrhage, contiguous to a mesencephalic cavernous angioma, obstructing the Sylvius aqueduct, causing secondary hydrocephalus. Four months after endoscopic ventriculocisternostomy, neuropsychological evaluation showed improvement and the patient regained autonomy. Parenchyma cavernous angiomas causing direct hemorrhage and subsequent obstruction of the Sylvian aqueduct are uncommon. Sub-acute behavior and mental state abnormalities are rare first manifestations of cavernous angioma and requires high clinical suspicion for its correct diagnosis. Magnetic resonance imaging evaluation is crucial in the detection of such patients as prompt neurosurgical intervention may substantially improve cognitive function.

Os angiomas cavernosos são malformações do sistema nervoso central cujas manifestações mais comuns são crises epiléticas e défices neurológicos agudos. Apresentamos o caso clínico de um doente de 71 anos com uma história com dois meses de evolução de alteração do comportamento, défice de atenção e apatia, sucedidas por marcada instabilidade da marcha. A avaliação neuropsicológica revelava alterações cognitivas e disfunção executiva marcadas. Na Tomografia Computorizada Cerebral observava-se hidrocefalia supraventricular enquanto na Ressonância Magnética cerebral era possível observar uma pequena hemorragia que obstruía o aqueduto de Sylvius localizada contiguamente a um angioma cavernoso mesencefálico. Quatro meses após o tratamento com ventriculocisternostomia endoscópica, o doente apresentava melhoria significativa na avaliação neuropsicológica tendo voltado a adquirir a autonomia prévia. A apresentação de um angioma cavernoso parenquimatoso como uma obstrução do aqueduto de Sylvius secundária a hemorragia é invulgar. Rara é também a sua apresentação como alterações do comportamento e do estado mental subagudas. Uma avaliação neuroimagiológica e uma intervenção neurocirúrgica precoces foram essenciais para a melhoria cognitiva observada.

Clinical and Radiological Characterization of Progressive Multifocal Leukoencephalopathy in HIV-Infected Patients: A Retrospective Analysis and Review of the Literature.

INTRODUCTION: Progressive multifocal leukoencephalopathy is a demyelinating disease of the central nervous system caused by John Cunningham virus, mostly associated with immunodeficiency conditions, such as the human immunodeficiency virus infection. Progressive multifocal leukoencephalopathy can have multiple clinical features and usually presents a typical lesion pattern on brain magnetic resonance imaging. Its course may be rapidly progressive, although immunological responsiveness can be associated with an improved prognosis. MATERIAL AND METHODS: We performed a retrospective analysis of the clinical and radiological data from patients admitted in our institution between January 2005 and April 2014 with the diagnosis of definitive progressive multifocal leukoencephalopathy (ICD10:A81.2) in the setting of human immunodeficiency virus infection. RESULTS: Twenty-one patients were included in our study, mostly men (n = 20, 95.2%). Mean age at diagnosis was 39 years. Motor deficits were the most common clinical finding. John Cunningham virus-DNA was detected in the cerebral spinal fluid in 20 patients (95.2%). Brain imaging studies most commonly disclosed bilateral supratentorial, asymmetric lesions. Four (19%) patients developed immune reconstitution inflammatory syndrome in the follow-up. Therapeutic approach included initiation and continuation/optimization of antiretroviral therapy, with adjunctive therapy with corticosteroids in four patients. Seventeen (81%) patients died during the study period; median survival time following progressive multifocal leukoencephalopathy diagnosis was 3 months (range 1 - 13). DISCUSSION: The results of our study are in accordance with the data previously published on progressive multifocal leukoencephalopathy in human immunodeficiency virus patients. Progressive multifocal leukoencephalopathy is predominantly associated with severe immunosuppression, particularly in patients who are not under anti-retroviral therapy, and usually presents with motor and cognitive symptoms and signs. A typical bilateral asymmetric pattern in conventional magnetic resonance imaging is present in the majority of the patients. There is no specific therapy for progressive multifocal leukoencephalopathy and it is usually fatal, although outcomes can improve with highly active anti-retroviral therapy. Immune reconstitution inflammatory syndrome is also an important complication related with progressive multifocal leukoencephalopathy, usually associated with anti-retroviral therapy. Progressive multifocal leukoencephalopathy-immune reconstitution inflammatory syndrome presents with different imaging characteristics from progressive multifocal leukoencephalopathy and treatment with steroids can improve survival. CONCLUSION: The mortality rate and long-term neurological morbidity associated with progressive multifocal leukoencephalopathy are quite high. These data should increase clinician awareness to the occurrence of progressive multifocal leukoencephalopathy among human immunodeficiency virus patients and highlight the important role of magnetic resonance imaging, as early diagnosis may beassociated with better outcome.

Introdução: A leucoencefalopatia multifocal progressiva é uma patologia desmielinizante causada pelo vírus John Cunningham, geralmente associada a estados de imunodepressão, em particular a infeção pelo vírus da imunodeficiência humana. Pode apresentar múltiplas manifestações clínicas e tem habitualmente um padrão imagiológico típico. A evolução clínica é geralmente progressiva, podendo ocorrer uma melhoria do prognóstico associada à recuperação imunológica.Material e Métodos: Foi conduzida uma análise retrospetiva dos dados clínicos e imagiológicos de doentes admitidos no nosso Hospital entre janeiro de 2005 e abril de 2014 com o diagnóstico de leucoencefalopatia multifocal progressiva (ICD10:A81.2) associado a infeção por vírus da imunodeficiência humana.Resultados: Vinte e um doentes foram incluídos, sendo 20 do sexo masculino (95,2%). A idade média na altura do diagnóstico foi 39 anos. A forma de apresentação mais frequente foi défice motor. O vírus John Cunningham foi identificado no líquido cefalorraquidiano em 20 doentes (95,2%). Nos estudos de imagem verificou-se um predomínio de lesões supratentoriais, assimétricas e bilaterais. Quatro doentes (19%) desenvolveram síndrome inflamatória de resposta imunológica. A abordagem terapêutica incluiu início ou otimização de terapêutica anti-retrovirica, associada a corticoterapia em quatro casos. Dezassete (81%) doentes morreram no período do estudo, sendo a sobrevida mediana após diagnóstico de três meses (intervalo 1 a13).Discussão: Os resultados do nosso estudo são concordantes com os dados previamente publicados relativamente à leucoencefalopatia multifocal progressiva, evidenciando a sua associação à infecção pelo vírus da imunodeficiência humana, particularmente nos doentes com imunossupressão grave, o predomínio de sinais e sintomas motores e cognitivos, e a existência de um atingimento bilateral e assimétrico evidente nas sequências convencionais dos estudos de ressonância magnética. Não existe terapêutica específica e esta patologia é normalmente fatal, apesar de se verificar um aumento da sobrevida associado à terapêutica anti-retrovírica. A síndrome inflamatória de reconstituição imunológica é uma complicação associada a leucoencefalopatia multifocal progressiva, habitualmente ocorrendo após início de terapêutica anti-retrovírica, apresentando caraterísticas imagiológicas diferentes da leucoencefalopatia multifocal progressiva. A instituição de corticoterapia pode melhorar o prognóstico dos doentes que desenvolvem síndrome inflamatória de reconstituição imunológica associada a leucoencefalopatia multifocal progressiva.Conclusão: A mortalidade e morbilidade associadas à leucoencefalopatia multifocal progressiva são elevadas, com sequelas neurológicas importantes a longo-prazo. Os dados apresentados deverão alertar os clínicos para a ocorrência desta patologia nos doentes com infecção vírus da imunodeficiência humana, evidenciando o papel cada vez mais determinante da Ressonância Magnética no diagnóstico precoce da leucoencefalopatia multifocal progressiva e consequente melhoria do prognóstico.

Candida albicans brain abscesses in an injection drug user patient: a case report.

BACKGROUND: Fungal brain abscess is an uncommon disease, mostly associated with immunocompromised states and poorly controlled diabetes. Its incidence, however, is rising as a result of the increasing use of immunosuppressive agents, corticosteroids and broad-spectrum antimicrobial therapy. Candida species have emerged as the most prevalent etiologic agents of brain abscesses in autopsy studies. CASE PRESENTATION: A 46-year-old male with a history of injection drug abuse, chronic hepatitis C and diabetes mellitus presented to the Emergency Department of our hospital following a generalized tonic-clonic seizure without recovery of mental status. On admission, the patient was in coma, febrile, with severe acidemia with respiratory and metabolic acidosis, requiring invasive mechanical ventilation. Brain imaging revealed multiple ring-enhancing lesions with oedema and mass effect. Microbiologic studies, including cerebrospinal fluid, blood, sputum and urine cultures, were all negative. A stereotactic brain biopsy was performed and culture of brain specimens revealed Candida albicans. The patient was successfully treated with fluconazole therapy for 48 weeks presenting a good clinical response and a complete radiological resolution of brain abscesses. CONCLUSION: Despite advances in diagnostic and therapeutic procedures, fungal brain abscess remains a life-threatening disease with a poor outcome. Successful treatment requires an early diagnosis and usually a combined medical and surgical approach. A long-term antibiotic regimen is a cornerstone of fungal brain abscesses treatment, with the endpoint determined by clinical and neuroimaging response. The authors report an uncommon case of successfully treated Candida albicans brain abscesses with anti-fungal therapy consisting of fluconazole alone. This case illustrates the importance of early recognition of predisposing factors and multidisciplinary approach in timely therapeutic intervention, in order to prevent neurologic sequelae and improve the outcome of the patients with this severe and challenging form of central nervous system infection.

Long-term mortality analysis in Parkinson's disease treated with deep brain stimulation.

Background. Few data have been published regarding long-term mortality in patients with Parkinson's disease treated with DBS. Methods. This study analyzed long-term mortality rates, causes, and correlates in PD patients treated with DBS. Results. 184 consecutive patients were included; mean follow-up was 50 months. Fifteen deaths occurred (total 8.15%, annual mortality rate 1.94%). Mean age at disease onset and at surgery was 48 ± 2.4 and 63 ± 1.6 years, respectively. Mean disease duration until death was 21 ± 7.8 years. Most deaths related to stroke, myocardial infarction, other vascular/heart disorders, or severe infection; one suicide was recorded. Deceased PD patients were mostly male and had lower motor benefit after DBS, but univariate analysis failed to show significant differences regarding gender and motor benefit. Survival was 99% and 94% at 3 and 5 years. Conclusions. Long-term survival is to be expected in PD patients treated with DBS, possibly higher than previously expected. Death usually supervenes due to vascular events or infection.

Spinal intramedullary ependymal cyst--current concepts for diagnosis and surgical management.

We describe a case of a 48-year-old woman who presented with neck pain and progressive left arm weakness. Magnetic Resonance Imaging showed a cervical intramedullary cystic lesion. The lesion was removed and neuropathological analysis revealed an ependymal cyst.

Lung cancer: atypical brain metastases mimicking neurocysticercosis.

The authors describe a case of a 47-year-old male smoker with a 3-month history of hearing loss, tinnitus and dizziness. Physical examination revealed neurosensory hearing loss. Small rounded hypodensities without mass effect were evident in a computed tomography scan of the head, confirmed by brain magnetic resonance imaging as multiple cystic lesions in both cerebral and cerebellar hemispheres, without perilesional edema or gadolinium enhancement, suggestive of neurocysticercosis. Extraparenchymal involvement was also noted. Albendazole and dexamethasone were started. As a chest radiograph showed a bilateral reticulonodular pattern, a bronchoscopy was performed showing normal results. However, transbronchial biopsy revealed lung adenocarcinoma. Thoracoabdominopelvic computed tomography scan showed secondary lung and bone lesions. Since brain lesions were not suggestive of secondary tumor lesions, a brain biopsy was performed confirming metastatic disease. This case illustrates some peculiar imagiological features of brain metastases in lung cancer, indicating that sometimes invasive procedures are required to establish a definitive diagnosis.