CONTINUUM Lifelong Learning in Neurology最新文献

Objective: This article reviews the current understanding of the varied clinical presentations of spontaneous intracranial hypotension and discusses strategies and limitations in diagnosing this complex syndrome, including approaches for medical practitioners outside of specialty centers.

Latest developments: Radiologic algorithms applied to brain MRI (eg, the Bern score) can help assess the probability of an underlying spinal CSF leak, but they do not replace good history-taking and clinical acumen. Brain MRI findings may evolve over time with or without leak-directed treatment. New techniques and improvements in imaging technology have led to better identification and classification of different types of spinal CSF leaks, especially CSF-venous fistulas that were reported for the first time in 2014 and now account for 50% or more of the leaks in patients without spinal extradural fluid collections. Occasionally, spontaneous intracranial hypotension is the result of preexisting intracranial hypertension causing "blowout" leaks.

Essential points: Spontaneous intracranial hypotension is caused by leakage of CSF from the spinal dura and most often presents with orthostatic headache, frequently accompanied by neck pain and stiffness, nausea, vestibulo-auditory distortions, visual changes, and other symptoms. Spontaneous intracranial hypotension can be debilitating, and long diagnostic and treatment delays are common. In rare cases, spontaneous intracranial hypotension can have dangerous sequelae, including superficial siderosis, subdural hematoma, bibrachial amyotrophy, brain sagging dementia, and even death. Early recognition of clinical symptoms and radiologic signs is imperative for best patient outcomes.

Objective: Normal pressure hydrocephalus (NPH) is a clinical syndrome of a gait disorder, cognitive impairment, and urinary incontinence that also has characteristic imaging features. This article provides an overview of the radiographic evaluation and imaging features of NPH.

Latest developments: Ventriculomegaly is an overarching imaging feature of NPH, although it is nonspecific. More specific imaging features have been described in association with NPH subtypes. Patients with idiopathic NPH commonly have features of ventriculomegaly, high-convexity tight sulci, and enlarged sylvian fissures. Patients with delayed-onset congenital NPH have features of marked ventriculomegaly, without extraventricular hydrocephalus, and may have stenosis or obstruction of the cerebral aqueduct. Evaluation of CSF dynamics, patterns of radiotracer uptake on positron emission tomography (PET), and patterns of brain stiffness on MR elastography can help to differentiate idiopathic NPH from secondary NPH or a neurodegenerative process.

Essential points: Imaging features of disproportionately enlarged subarachnoid space hydrocephalus are important to recognize as they are part of the diagnostic criteria for idiopathic NPH and aid in differentiating NPH from a neurodegenerative process. Evaluation of CSF dynamics, patterns of fludeoxyglucose (FDG) uptake, and patterns of brain stiffness may aid in the evaluation of challenging cases that lack typical clinical and structural radiographic features.

Objective: Diagnostic imaging evaluation for patients with suspected spontaneous intracranial hypotension has evolved substantially in recent years. This article describes a practical algorithm for radiographic diagnosis of spontaneous intracranial hypotension and localization of spinal CSF leaks.

Latest developments: Brain and spine MRI is the mainstay of the initial diagnostic workup of patients with spontaneous intracranial hypotension. Recently, sophisticated myelographic techniques to precisely subtype and localize CSF leaks have been developed and refined. These include fluoroscopic techniques, such as digital subtraction myelography, and CT-based techniques, such as hyperdynamic CT myelography. These techniques improve the detection of various types of CSF leaks, thereby enabling targeted therapies.

Essential points: Neurologists should familiarize themselves with the variety of imaging necessary to initially support a diagnosis of spontaneous intracranial hypotension and the myelographic techniques needed to precisely localize spinal CSF leaks.

Objective: The options for treatment of spontaneous spinal CSF leaks and CSF-venous fistulas have expanded in recent years, and this article reviews the current treatments available, factors that affect treatment outcomes, and an algorithmic approach to treatment.

Latest developments: Until recently, treatments for spontaneous intracranial hypotension consisted of supportive care, epidural blood patching, and when the site of CSF leakage was known, surgical repair. CSF-venous fistulas are now known to be common causes of CSF hypotension and are rarely cured by epidural blood patching. Better visualization of CSF-venous fistulas and the novel treatment of CSF-venous fistulas with transvenous embolization have expanded the arsenal of treatment options for spontaneous intracranial hypotension.

Essential points: Clinical outcomes improve with timely treatment of spinal CSF leaks and CSF-venous fistulas. Radiographic features direct treatment, which includes epidural blood patching, transvenous embolization, and surgical correction. CSF-venous fistulas respond poorly to epidural blood patching but can respond favorably to surgical obliteration or transvenous embolization.

Objective: This article reviews the management of idiopathic intracranial hypertension (IIH), which is essential to neurology practice.

Latest developments: The incidence and prevalence of IIH have risen because of the worldwide obesity epidemic. Evidence suggests that IIH has a unique systemic metabolic phenotype. Clinical history, examination, and visual fields remain the cornerstone for monitoring people with IIH. Optical coherence tomography demonstrates structural changes that aid decision-making. The principles of IIH management include protecting vision and escalating to surgical interventions when it is threatened, addressing the role of obesity in the condition, and providing advice on headache therapeutics. There are no labeled therapies for IIH; however, acetazolamide remains the most common medication prescribed. A glucagon-like peptide-1 receptor agonist was recently shown to reduce intracranial pressure independent of weight reduction in a phase II randomized controlled trial in people with IIH, suggesting it could be a promising treatment for IIH. In patients with IIH requiring surgery, there has been an increase in venous sinus stenting.

Essential points: Neurologists are central in the management pathway for people with IIH. Ongoing coordinated care between ophthalmologists and neurologists is essential for optimal care of these patients.