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Moyamoya disease (MMD) is a chronic, occlusive cerebrovasculopathy typified by progressive steno-occlusive disease of the intracranial internal carotid arteries (ICAs) and their proximal branches. Moyamoya syndrome (MMS) categorizes patients with characteristic MMD plus associated conditions. As such, the most usual presentations are those that occur with cerebral ischemia, specifically transient ischemic attack, acute ischemic stroke, and seizures. Hemorrhagic stroke, headaches, and migraines can also occur secondary to the compensatory growth of fragile collateral vessels propagated by chronic cerebral ischemia. While the pathophysiology of MMD is unknown, there remain numerous clinical associations including radiation therapy to the brain, inherited genetic syndromes, hematologic disorders, and autoimmune conditions. We describe the case of a 31-year-old woman who presented with recurrent ischemic cerebral infarcts secondary to rapidly progressive, bilateral MMD despite undergoing early unilateral surgical revascularization with direct arterial bypass. She had numerous metabolic conditions and rapidly decompensated, ultimately passing away despite intensive and aggressive interventions. The present case highlights that progression of moyamoya disease to bilateral involvement can occur very rapidly, within a mere 6 weeks, a phenomenon which has not been documented in the literature to our knowledge.
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BACKGROUND: Intraparenchymal hemorrhage (IPH), possibly due to reperfusion, after evacuation of a cranial chronic subdural hematoma (cSDH) is a known phenomenon. However, it is sparingly reported and not well understood. METHODS: An illustrative case series is presented. A literature review was conducted according to Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines to identify all previously reported cases. RESULTS: A total of 48 cases were analyzed. Males were 85.4% of the population, and the mean age was 67.5 years. Pre-existing head trauma and hypertension were the most common comorbidities. Headache was a presenting symptom in 60.4% of cases. Midline shift was explicitly stated in 54.2% of cases. Initial burr hole alone was performed 75.0% of the time, whereas craniotomy alone was performed in 16.7% of cases. Any initial craniotomy patients were associated with a modified Rankin Scale score of 5 (P = 0.03). The IPH was located in the cerebral hemisphere in 62.5% of cases and more likely to occur ipsilateral to a unilateral cSDH (P = 0.02). The IPH occurred a mean 1.9 days after surgery, and 50.0% occurred within 24 hours of initial intervention. The median modified Rankin Scale at discharge was 2. The mortality rate was 25%. Lastly, a multifactorial reperfusion pathophysiology was proposed. CONCLUSION: IPH after cSDH evacuation is associated with significant morbidity and mortality. Prompt recognition, regulating blood pressure, controlling the amount and rate of extra-axial fluid drained, and a meticulous surgical technique are critical to optimize the care of patients with cSDH and reduce the rate of postoperative IPH.
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Hematoma Subdural Crônico/cirurgia , Hemorragias Intracranianas/etiologia , Procedimentos Neurocirúrgicos/efeitos adversos , Hemorragia Pós-Operatória/etiologia , Idoso , Feminino , Humanos , Hemorragias Intracranianas/cirurgia , Masculino , Pessoa de Meia-Idade , Hemorragia Pós-Operatória/cirurgia , Resultado do TratamentoRESUMO
Skull base osteomyelitis (SBO) is a complex and fatal clinical entity that is often misdiagnosed for malignancy. SBO is commonly a direct complication of otogenic, sinogenic, odontogenic, and rhinogenic infections and can present as central, atypical, or pediatric clival SBO. This review describes the clinical profile, investigational approach, and management techniques for these variants. A comprehensive literature review was performed in PubMed, MEDLINE, Research Gate, EMBASE, Wiley Online Library, and various Neurosurgical and Neurology journals with the keywords including: SBO, central or atypical SBO, fungal osteomyelitis, malignant otitis externa, temporal bone osteomyelitis, and clival osteomyelitis. Each manuscript's reference list was reviewed for potentially relevant articles. The search yielded a total of 153 articles. It was found that with early and aggressive culture guided long-term intravenous broad-spectrum antibiotic therapy decreases post-infection complications. In cases of widespread soft tissue involvement, an early aggressive surgical removal of infectious sequestra with preferentially Hyperbaric Oxygen (HBO) therapy is associated with better prognosis of disease, less neurologic sequelae and mortality rate. Complete resolution of the SBO cases may take several months. Since early treatment can improve mortality rates, it is paramount that the reporting radiologists and treating clinicians are aware of the cardinal diagnostic signs to improve clinical outcomes of the disease. It will decrease delayed diagnosis and under treatment of the condition. However, due to rarity of the condition, complete prognostic factors have not fully been analyzed and discussed in the literature.
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Pseudohypoxic brain swelling (or the more recent term, postoperative intracranial hypotension-associated venous congestion) is a rare and potentially deadly complication that can occur after routine spine or brain surgery. The mechanism of this injury has been described as a rapid cerebral spinal fluid drainage leading to venous cerebral congestion. The clinical and radiographic findings mimic those found in a patient who has suffered an anoxic brain injury. We present the third reported case of postoperative intracranial hypotension-associated venous congestion following spinal surgery.
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INTRODUCTION: Brain is suspended in cerebrospinal fluid (CSF)-filled subarachnoid space by subarachnoid trabeculae (SAT), which are collagen-reinforced columns stretching between the arachnoid and pia maters. Much neuroanatomic research has been focused on the subarachnoid cisterns and arachnoid matter but reported data on the SAT are limited. This study provides a comprehensive review of subarachnoid trabeculae, including their embryology, histology, morphologic variations, and surgical significance. METHODS: A literature search was conducted with no date restrictions in PubMed, Medline, EMBASE, Wiley Online Library, Cochrane, and Research Gate. Terms for the search included but were not limited to subarachnoid trabeculae, subarachnoid trabecular membrane, arachnoid mater, subarachnoid trabeculae embryology, subarachnoid trabeculae histology, and morphology. Articles with a high likelihood of bias, any study published in nonpopular journals (not indexed in PubMed or MEDLINE), and studies with conflicting data were excluded. RESULTS: A total of 1113 articles were retrieved. Of these, 110 articles including 19 book chapters, 58 original articles, 31 review articles, and 2 case reports met our inclusion criteria. CONCLUSIONS: SAT provide mechanical support to neurovascular structures through cell-to-cell interconnections and specific junctions between the pia and arachnoid maters. They vary widely in appearance and configuration among different parts of the brain. The complex network of SAT is inhomogeneous and mainly located in the vicinity of blood vessels. Microsurgical procedures should be performed with great care, and sharp rather than blunt trabecular dissection is recommended because of the close relationship to neurovascular structures. The significance of SAT for cerebrospinal fluid flow and hydrocephalus is to be determined.
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Espaço Subaracnóideo/anatomia & histologia , Tecido Conjuntivo , HumanosRESUMO
Recent studies have identified the rs75932628 (R47H) variant in TREM2 as an Alzheimer's disease risk factor with estimated odds ratio ranging from 2.9 to 5.1. The Cache County Memory Study is a large, population-based sample designed for the study of memory and aging. We genotyped R47H in 2974 samples (427 cases and 2540 control subjects) from the Cache County study using a custom TaqMan assay. We observed 7 heterozygous cases and 12 heterozygous control subjects with an odds ratio of 3.5 (95% confidence interval, 1.3-8.8; p = 0.0076). The minor allele frequency and population attributable fraction for R47H were 0.0029 and 0.004, respectively. This study replicates the association between R47H and Alzheimer's disease risk in a large, population-based sample, and estimates the population frequency and attributable risk of this rare variant.