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AIM: To evaluate the possibility of implementing a new scheme of rescue treatment after relapse or progression of high-grade glioma (HGG) treated at the first-line with bevacizumab and irinotecan (BVZ+CPT11), evaluating the response and toxicity of associating BVZ and fractionated stereotactic radiotherapy (BVZ+FSRT). MATERIALS AND METHODS: We retrospectively analysed data from 59 patients with relapse of HGG. Nine patients with HGG relapse after treatment using the Stupp protocol that were treated with BVZ+CPT11 for progression between July 2007 and August 2012, after which the response was assessed according to the Revised Assessment in Neuro-Oncology (RANO) criteria. BVZ was administered at a dose of 10 mg/kg and FSRT up to a prescribed dose of 30 Gy, 500 cGy per fraction, three days a week. The median follow-up was 38 months. RESULTS: The treatment was well-tolerated by all patients. The response after nuclear magnetic resonance imaging (MRI) at 3-6 months was progression in two patients, stable disease in four, and three patients had a partial response. The median overall survival (OS) from diagnosis until death or the last control was 36.8 months. The median progression-free survival (PFS) was 10.8 months. The results from tumour sub-group analysis indicated that the PFS was not statistically significant although it seemed that it was higher in grade-III. The OS was higher in grade-III gliomas. CONCLUSIONS: The combination of BVZ+FSRT as a second-line HGG relapse rescue treatment is well-tolerated and seems to offer promising results. We believe that multi-centre prospective studies are needed to determine the long-term efficacy and toxicity of this therapeutic approach.
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Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare chronic central-nervous-system inflammatory disorder that became known only recently, and the pathogenesis of CLIPPERS remains poorly understood. This report presents clinical and radiological features of a rare case: a young female patient who rapidly died of suspected CLIPPERS. Helpful multiparametric MRI diagnostic criteria are proposed that can help discriminate CLIPPERS from non-CLIPPERS pathologies. We reviewed clinical history, symptoms, quantitative data from brain multiparametric MRI before and after treatment, and histopathological data. Perfusion-weighted imaging revealed a decrease in regional cerebral blood flow by 31% and in cerebral blood volume by 64%, with a moderate increase in transit time and in time to peak by up to 23% in affected pontine and cerebral white matter. As estimated by diffusion tensor imaging, there was elevated density of tracts (n/mm2) and a decrease of fraction anisotropy (×10-3 mm/s2) in the patient's pons as compared to a healthy control: density of tracts = 13.5 vs 12.4 and fraction anisotropy = 0.32 vs 0.45, respectively. Macromolecular proton fraction values proved to be reduced (15.8% and 14.5% in the control, respectively) in the patient's cerebral peduncles by 3% and in the pons by 4.1% and in a periventricular white matter lesion by 6.4% (11.3% in the normal-looking contralateral hemisphere). Based on our findings, we argue that quantitative MRI techniques may be a valuable source of biomarkers and reliable diagnostic criteria and can shed light on the pathogenesis and exact nosological position of this disorder.
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Introduction: Craniopharyngiomas are benign tumours mainly confined to the cranial cavity in the suprasellar region. Research Question and Case Description: We present a rare case of an aggressive papillary craniopharyngioma with disseminated spinal intradural disease. A 67-year-old woman presented with a 4-month history of headache, visual disturbance, acute confusion and radicular leg pain. Previous history of breast carcinoma (ER â+ âPR â+ âHER2-) was noted. The importance of histological diagnosis prior to treatment of sellar or suprasellar lesions with atypical or aggressive features is explored. Materials and methods: MRI demonstrated a partly solid and partly cystic pituitary mass lesion in the sellar and suprasellar region with chiasmal compression and hypothalamic involvement. The sella was mildly enlarged and there were no calcifications. Whole neuraxis MRI revealed intradural deposits involving the ventricular system, spinal cord and conus. Within a month, the lesion rapidly increased in size. The patient underwent a craniotomy and transventricular resection of the sellar and suprasellar mass. Cranial lesion histology favoured papillary craniopharyngioma, confirmed by BRAF V600 mutation. Lumbar puncture CSF cytology confirmed craniopharyngioma with BRAF mutation and no evidence of metastatic breast cancer. Results: The patient remained confused postoperatively without focal neurological deficit and underwent palliative whole brain radiotherapy. She died 4 months later. A review of the literature identified 29 reports of ruptured craniopharyngioma. Discussion and Conclusion: Ruptured craniopharyngioma presents with a suprasellar mass and drop lesions in the spinal canal, characteristics radiologically indistinguishable from metastatic disease. The importance of histological diagnoses in directing the management of these cases is highlighted.
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Introduction: Dengue is a common febrile illness caused by Dengue virus and spread by Aedes mosquitoes. The neurological complications like encephalopathy or encephalitis or immune-mediated neurological syndromes are uncommon though. Discrete neuroimaging findings in this setting are even rarer. We report a case of dengue encephalitis with uncommon MRI features in a young female. Case presentation: The patient presented with complains of fever, vomiting, weakness in all limbs and difficulty in speech. Neurological examination revealed bilateral horizontal gaze palsy with impaired oculo-cephalic reflex, bulbar dysarthria and quadriplegia with bilateral planters up-going. Laboratory reported anemia, thrombocytopenia and positive NS1 antigen while excluding other tropical and immunological diseases. Brain MRI revealed extensive thalamic involvement as unique "double-doughnut" sign along with lesions in brainstem. The patient received supportive treatment in intensive unit and was discharged following improvement in clinical condition and laboratory reports. Clinical discussion: Dengue can infect the central nervous system directly as encephalitis or can have neurological consequences following multi-organ dysfunction and shock as encephalopathy or post-infection immunological syndromes as Guillain-Barré Syndrome or cerebrovascular complications or dengue muscle dysfunction. The MRI appearance of "double-doughnut" sign points towards dengue encephalitis in appropriate setting. Conclusion: A high index of suspicion is required to make a diagnosis of dengue encephalitis. The "double-doughnut" sign in MRI sequences has the potential to become a diagnostic marker for dengue encephalitis.
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Staphylococcus aureus infectious endocarditis has a high mortality, major causes of death being cardiac failure, systemic embolism, and sepsis. Pseudoaneurysms, a rare complication of this infection, are not invariably fatal with appropriate treatment. A previously healthy 32-year-old man was found to have multiple cerebral infarctions, and infectious endocarditis with mitral valve vegetation was diagnosed by echocardiography. Because methicillin-resistant Staphylococcus aureus (MRSA) was identified from blood cultures, vancomycin was administered. Massive intracerebral hemorrhage in the left temporo-occipital lobe occurred in the patient on the 3rd day after admission, and the hematoma was completely removed surgically. Another hemorrhage was identified in the right occipital region on the 7th hospital day, which led the patient deep coma. Blood cultures on the 10th day were negative for MRSA; however, imaging studies revealed pseudoaneurysms in the superior mesenteric, hepatic, and left popliteal arteries 3 weeks after admission. No surgical indication was applied to these pseudoaneurysms because the patient remained comatose. On the 78th day after admission, the patient's blood pressure suddenly dropped and he died. Autopsy demonstrated massive bleeding in the abdominal cavity caused by rupture of the superior mesenteric artery pseudoaneurysm. Our patient's clinical course was fulminant, his endocarditis being complicated by cerebral infarctions, intracranial hemorrhages, and multiple pseudoaneurysms within 3 weeks of admission. In retrospect, he may have survived if emergency resection of the mitral valve vegetation had been performed on the first or second day of admission; however, the in-hospital mortality rate after such surgery is high.
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Rasmussen's encephalitis (RE) is a relatively rare chronic inflammatory neurological disease that usually only affects one hemisphere of the brain. It primarily affects children under the age of 10, although it can also affect teens and adults, causing drug-resistant seizures, progressive hemiparesis, and dementia. RE presents as a challenging diagnosis with MRI as the cornerstone of the evaluation and nuclear imaging as a complementary tool. We'd like to present a case of a 12-year-old girl who was diagnosed with RE after an MRI. In this study, we examine the diagnostic criteria, differential diagnoses, and issues that underpin the diagnostic challenge in great detail.
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Background: Although embolic stroke accounts for most cerebral infarction, examinations to identify the embolic source have been insufficient. Nonobstructive general angioscopy (NOGA) has developed to allow the detailed observation of atheromatous changes of the aorta. Objectives: The purpose of this study was to clarify the importance of the aortogenic mechanism in the development of ischemic stroke. Methods: We examined 114 consecutive patients whose aorta was observed by NOGA and who subsequently underwent brain magnetic resonance imaging to detect ischemic stroke lesions. In the evaluation of the aorta, the presence and location of spontaneously ruptured aortic plaque (SRAP) were determined. The aorta was observed from the origin to the arch (proximal aorta [PAo]) and the proximal descending aorta. Results: Forty-nine of 114 patients had SRAP observed by NOGA. Among these, 24 had SRAP in the PAo, and 43 had SRAP in the descending aorta. Thirty-three patients had ischemic stroke lesions, including 6 with a clinical neurologic deficit. The frequency at which SRAP was detected in these patients was significantly higher in comparison to 81 patients without ischemic stroke (69% vs 33%; P < 0.01). The sensitivity and specificity of the presence of SRAP for ischemic stroke were 0.70 and 0.68, respectively. The presence of SRAP in PAo was significantly correlated with ischemic stroke (odds ratio: 14.3; P < 0.001). Conclusions: In the treatment of ischemic stroke, attention should be paid to SRAP, especially that in the PAo. (STROKE-NOGA [SponTaneously Ruptured aOrtic plaques as a potential cause of embolic stroKEs visualized by Non-Obstructive General Angioscopy] Study; UMIN000034588).
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Middle cerebral artery stenosis is the leading and the most frequent cause of stroke due to intracranial stenosis in Asia. Magnetic resonance imaging (MRI) is more sensitive than computed tomography of the head for detecting acute brain ischemia. We are reporting a case of a 28-year-old female with recurrent left hemiparesis. After the last attack, an improvement in motor function was seen in less than 24 hours. Though the restoration of motor functions is not complete yet, an MRI scan that was done two weeks later appeared normal. Ischemic stroke in middle cerebral artery stenosis is associated with hemodynamic stroke due to hypoperfusion or lack of blood flow to brain tissue. Recurrent strokes can be prevented by better medical management in patients through regulation and management of risk factors.
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Aphasic status epilepticus (SE) is a rare manifestation of non-convulsive SE (NCSE) and may occasionally be under-recognized. We report a 69-year-old male patient with a pre-existing left parietal oligodendroglioma WHO III after two resections and radio-chemotherapy. The patient was left with some word finding difficulties but had no history of overt seizures. He developed aphasic NCSE, which was only detected by long-term electroencephalography (EEG) monitoring. The 24-hour EEG revealed paroxysmal rhythmic theta-delta activity in left posterior regions that propagated to left temporo-parietal areas. Rhythmic activity appeared every 15-30 min and lasted for 10-110 s. Aphasia was continuously present with superimposed short-lasting clinical deteriorations during the day. Magnetic resonance imaging showed peri-ictal edema on diffusion-weighted images in the insula and fronto-parietal cortex, which supported the diagnosis of SE. NCSE persisted for seven months. The patient recovered upon addition of intravenous phenytoin. One should not only consider aphasic SE when language impairment is episodic, but also when there are prolonged manifestations, especially when the typical differential diagnoses have been excluded. Intravenous therapy may be required to terminate NCSE. With this report, we would like to draw attention to aphasic SE as a rare phenomenon that may be difficult to diagnose and delay management in clinical practice.
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In this case report, we describe a 60-year-old man who presented with headaches for 1 year and mild confusion for 3 weeks and was initially diagnosed as having a cerebral tumor on the basis of finding a round lesion in the right lenticular nucleus with ring enhancement on gadolinium-enhanced T1-weighted brain magnetic resonance imaging. However, the discovery of positive serology for Treponema pallidum infection on routine tests on admission prompted analysis of cerebrospinal fluid, which was also positive on Treponema pallidum hemagglutination (TPHA), rapid plasma reagin (RPR), and treponemal antibody absorption (FTA-ABS) tests. Thus, he was diagnosed as having an intracranial syphilitic gumma. After commencing treatment with penicillin G, the lesion temporarily increased in size, but subsequently resolved completely with continuing antibiotic treatment. In the present era of increasing prevalence of syphilitic infection and because they are eminently treatable, syphilitic gummas should be included in the differential diagnosis of apparent brain tumors. Additionally, temporary enlargement of a probable gumma after instituting antibiotic treatment should not prompt cessation or change of the antibiotics.
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Primary dural lymphoma is a rare subtype of primary central nervous system lymphoma. Primary dural lymphoma may be radiologically misdiagnosed as it shares similar imaging characteristics with several pathologies, including meningiomas and subdural or epidural hematomas. We present a patient who was originally diagnosed with a subdural hematoma following a syncopal episode on computed tomography. Follow-up magnetic resonance imaging of the brain demonstrated heterogeneously enhancing dural-based mass overlying the left frontoparietal convexity associated with bidirectional dural tails, suggestive of a malignant meningioma. Neurosurgical histopathology revealed marginal zone B-cell lymphoma. This case represents the potential difficulty in diagnosing primary dural lymphoma, especially in the setting of uncertain clinical history and obscured imaging features.
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We report the case of a 19-month-old girl with late-onset ornithine transcarbamylase (OTC) deficiency initially referred to gastroenterology for intermittent vomiting lasting a year and abnormal liver enzymes (AST 730 U/L [reference range 26-55 U/L]; ALT 1213 U/L [reference range 11-30 U/L]) without hepatomegaly. While the patient was hospitalized for liver biopsy, intermittent tremors of the upper extremities with varying severity were noted. The patient was presumed to have hyperammonemia secondary to acute liver failure and was discharged after 5 days; follow-up monitoring led to readmission 7 days later. A brain MRI showed nonspecific bilateral pericallosal and bifrontal white matter FLAIR hyperintensities. These findings raised suspicion for a metabolic disease and prompted a genetics consultation. After inconclusive biochemical testing and worsening clinical status, rapid whole genome sequencing results were obtained identifying a novel, de novo, likely pathogenic, variant c.608C > T (p.Ser203Phe) in the OTC gene. The patient was promptly started on an oral nitrogen scavenger, citrulline supplementation, and protein restriction. Ammonia and glutamine levels normalized within 1 month of treatment and have stayed within the goal ranges with continued tailoring of treatment. Her parents noted resolution of vomiting and improved mood stability. Liver enzymes normalized after 2 months of treatment. The tremor, identified as asterixis, improved and a repeat brain MRI 3 months after the initial imaging showed near-complete resolution of previous white matter hyperintensities.
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Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome presents with the features of herpes simplex encephalitis (HSE), which is rare and has been described in only a few case reports. Our case describes a 17-year-old female with no significant previous medical history presenting with an acute onset of fever, headache, and epilepsy, similar to HSE. Computed tomography of the brain showed bilateral basal ganglia calcification. Magnetic resonance imaging demonstrated gyriform restricted diffusion with T2-weighted images prolongation. Further investigation showed elevated blood lactate concentration at rest. Hence, MELAS was suspected and the diagnosis was confirmed by the presence of a nucleotide 3243 AâG mutation in the mitochondrial DNA. The clinical presentation and imaging studies of MELAS are variable and may mimic those of HSE. Infection may have also precipitated MELAS manifestation in this patient. Laboratory features, such as elevated lactate, basal ganglia calcification, and gyriform restricted diffusion may be helpful in identifying patients with MELAS.
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Aim: To investigate the clinical relevance of the radiotherapy (RT) dose bath in patients treated for lower grade glioma (LGG). Methods: Patients (n = 17) treated with RT for LGG were assessed with neurocognitive function (NCF) tests and structural Magnetic Resonance Imaging (MRI) and categorized in subgroups based on tumour lateralisation. RT dose, volumetric results and cerebral microbleed (CMB) number were extracted for contralateral cerebrum, contralateral hippocampus, and cerebellum. The RT clinical target volume (CTV) was included in the analysis as a surrogate for focal tumour and other treatment effects. The relationships between RT dose, CTV, NCF and radiological outcome were analysed per subgroup. Results: The subgroup with left-sided tumours (n = 10) performed significantly lower on verbal tests. The RT dose to the right cerebrum, as well as CTV, were related to poorer performance on tests for processing speed, attention, and visuospatial abilities, and more CMB.In the subgroup with right-sided tumours (n = 7), RT dose in the left cerebrum was related to lower verbal memory performance, (immediate and delayed recall, r = -0.821, p = 0.023 and r = -0.937, p = 0.002, respectively), and RT dose to the left hippocampus was related to hippocampal volume (r = -0.857, p = 0.014), without correlation between CTV and NCF. Conclusion: By using a novel approach, we were able to investigate the clinical relevance of the RT dose bath in patients with LGG more specifically. We used combined MRI-derived and NCF outcome measures to assess radiation-induced brain damage, and observed potential RT effects on the left-sided brain resulting in lower verbal memory performance and hippocampus volume.
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We describe a case of a 52 year-old woman who was hospitalized with rhombencephalitis caused by Borrelia burgdorferi sensu lato. The patient presented with intermittent fever, dry cough, fatigue, global headache, night sweats, unintentional weight loss, and neurological symptoms like diplopia, tremor, paresthesia and ataxia. Examination of serum and cerebrospinal fluid (CSF) revealed positive Borrelia burgdorferi-specific antibody index and presence of CSF oligoclonal IgG bands, indicating intrathecal synthesis of Borrelia-specific antibodies. The clinical and biochemical picture thus suggested neuroborreliosis. Unexpectedly a magnetic resonance imaging (MRI) scan demonstrated inflammation in rhombencephalon that are extremely rare in patients with neuroborreliosis. The patient was treated with intravenous ceftriaxone with rapid improvement of her symptoms. The MRI findings were in regress six weeks after onset of antibiotic treatment, and normalized after about seven months.
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Coronavirus disease is caused by the SARS-CoV-2 virus. The virus first appeared in Wuhan (China) in December 2019 and has spread globally. Till now, it affected 269 million people with 5.3 million deaths in 224 countries and territories. With the emergence of variants like Omicron, the COVID-19 cases grew exponentially, with thousands of deaths. The general symptoms of COVID-19 include fever, sore throat, cough, lung infections, and, in severe cases, acute respiratory distress syndrome, sepsis, and death. SARS-CoV-2 predominantly affects the lung, but it can also affect other organs such as the brain, heart, and gastrointestinal system. It is observed that 75 % of hospitalized COVID-19 patients have at least one COVID-19 associated comorbidity. The most common reported comorbidities are hypertension, NDs, diabetes, cancer, endothelial dysfunction, and CVDs. Moreover, older and pre-existing polypharmacy patients have worsened COVID-19 associated complications. SARS-CoV-2 also results in the hypercoagulability issues like gangrene, stroke, pulmonary embolism, and other associated complications. This review aims to provide the latest information on the impact of the COVID-19 on pre-existing comorbidities such as CVDs, NDs, COPD, and other complications. This review will help us to understand the current scenario of COVID-19 and comorbidities; thus, it will play an important role in the management and decision-making efforts to tackle such complications.
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BACKGROUND: Cocaine use is associated with an increased risk of cerebrovascular accidents. Small vessel pathology has been linked to the risk of stroke in cocaine users, but can be challenging to detect on conventional magnetic resonance (MR) scans. Fluid-attenuated inversion recovery (FLAIR) scans permit better resolution of small vessel lesions. OBJECTIVES: FLAIR scans are currently only acquired based on the subjective judgement of abnormalities on MR scans at face value. We sought to evaluate this practice and the added value of FLAIR scans for patients with cocaine use disorder (CUD), by comparing microbleeds detected by MR and FLAIR scans. We hypothesised that microbleeds are more pronounced in CUD patients, particularly so in participants who had been selected for a FLAIR scan by radiographers. METHODS: Sixty-four patients with CUD and 60 control participants underwent a brain scan. The MR of 20 CUD patients and 16 control participants showed indicators of cerebral infarction at face value and were followed up by a FLAIR scan. We determined the volume of microbleeds in both MR and FLAIR scans and examined associations with various risk factors. RESULTS: While MR lesion volumes were significantly increased in CUD patients, no significant differences in lesion volume were found in the subgroup of individuals who received a FLAIR. CONCLUSION: The current practice of subjectively evaluating MR scans as a basis for the follow-up FLAIR scans to detect vascular pathology may miss vulnerable individuals. Hence, FLAIR scans should be included as a routine part of research studies.
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In the midst of the national opioid crisis, it is necessary for emergency physicians and radiologists to be familiar with presentations of opioid-related complications. We describe a case report of a 51-year-old female who developed bilateral cerebellar hemorrhages following opioid and benzodiazepine overdose. Malignant cerebellar edema is a rare but recognized complication following opiate overdose in children or chronic heroin toxicity. However, acute cerebellar involvement is rarely reported in adults. We feel that clinicians and radiologists should keep in mind the possibility of opioid toxic encephalopathy in their differential for adults with acute bilateral cerebellar infarcts and/or hemorrhages.
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The diagnosis of leptomeningeal metastases is sometimes difficult when the cytology of cerebrospinal fluid is negative. We report a rare case of leptomeningeal metastases that required differentiation from paraneoplastic limbic encephalitis. A 67-year-old man with extensive-stage small cell lung cancer was admitted for a sudden decrease in the level of consciousness. He suffered memory disturbances that began the day before admission. Diffusion-weighted and fluid-attenuated inversion recovery images of brain magnetic resonance imaging (MRI) showed bilateral symmetric areas of hyperintensity in the hippocampus, amygdala, insular cortex, and medial temporal lobe; contrast enhancement was positive. Cytology of the cerebrospinal fluid (CSF) was negative. Anti-N-methyl-d-aspartate receptor antibody and herpes simplex virus DNA were not detected in the CSF. Paraneoplastic Limbic encephalitis was suspected due to his symptoms and brain MRI scan. The patient developed generalized seizures after admission. High-dose methylprednisolone and intravenous immune globulin were administered, but his condition did not improve. Uncontrollable seizures persisted and he died in the hospital at day 13. Autopsy revealed leptomeningeal metastasis and invasion of cancer cells into the limbic system. Contrast-enhanced MRI should be performed even if limbic encephalitis is suspected, and leptomeningeal metastases should be suspected if the lesions are enhanced.
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BACKGROUND: Although silent brain infarction is an independent risk factor for subsequent symptomatic stroke and dementia in patients with nonvalvular atrial fibrillation, little is known regarding differences in risk factors for silent brain infarction between patients with paroxysmal and persistent nonvalvular atrial fibrillation. METHODS: This study population consisted of 190 neurologically asymptomatic patients (mean age, 64 ± 11 years) with nonvalvular atrial fibrillation (119 paroxysmal, 71 persistent) who were scheduled for catheter ablation. All patients underwent brain magnetic resonance imaging to screen for silent brain infarction prior to ablation. Transthoracic and transesophageal echocardiography was performed to screen for left atrial abnormalities (left atrial enlargement, spontaneous echo contrast, or left atrial appendage emptying velocity) and complex plaques in the aortic arch. RESULTS: Silent brain infarction was detected in 50 patients (26%) [26 patients (22%) in paroxysmal vs. 24 patients (34%) in persistent, p = 0.09]. Multiple logistic regression analysis indicated that age and diabetes mellitus or chronic kidney disease (estimated glomerular filtration rate < 60 mL/min/1.73 m2) were associated with silent brain infarction in patients with paroxysmal nonvalvular atrial fibrillation (p < 0.05), whereas no modifiable risk factors of silent brain infarction were observed in patients with persistent nonvalvular atrial fibrillation. CONCLUSIONS: These findings suggest that intensive intervention for diabetes mellitus and renal impairment from the paroxysmal stage or ablation therapy at the time of paroxysmal stage to prevent progression to persistent nonvalvular atrial fibrillation may prevent silent brain infarction and consequently reduce the risk of future symptomatic stroke.