Harlequin syndrome: using clinical features and autonomic testing to unmask the disorder.

Purpose: Harlequin syndrome is a rare autonomic disorder characterized by unilateral facial flushing and contralateral anhidrosis. We sought to delineate underlying causes, clinical presentations, and autonomic testing profiles of patients with Harlequin syndrome.

Methods: Retrospective chart review was performed of the Mayo Clinic electronic health record for patients with a Harlequin syndrome diagnosis from 1998 to 2024.

Multiple Synchronous CSF-Venous Fistulas in Spontaneous Intracranial Hypotension: A Multi-Institutional Case Series.

CSF-venous fistulas are a common cause of spontaneous intracranial hypotension. Due to the more routine use of decubitus myelography and advancements in various imaging techniques, recognition of CSF-venous fistulas has increased in recent years. Most commonly, patients harbor only one fistula at the time of myelography (although additional de novo fistulas can arise after treatment).

Application of Convolutional Neural Network Denoising to Improve Cone Beam CT Myelographic Images.

Cone beam CT is an imaging modality that provides high-resolution, cross-sectional imaging in the fluoroscopy suite. In neuroradiology, cone beam CT has been used for various applications including temporal bone imaging and during spinal and cerebral angiography. Furthermore, cone beam CT has been shown to improve imaging of spinal CSF leaks during myelography.

Evaluating longitudinal changes of gait parameters following shunt placement in patients with idiopathic normal pressure hydrocephalus.

Objective: Idiopathic normal pressure hydrocephalus (iNPH) is characterized by gait deterioration, cognitive disturbances, and urinary incontinence. Gait deficits pose safety hazards due to frequent falls, yet objective investigations of gait improvement following ventriculoperitoneal shunt (VPS) placement are limited. Therefore, the aim of this study was to assess objective improvement in gait parameters for patients with iNPH after VPS placement.

Management of Normal Pressure Hydrocephalus.

Objective: This article reviews the preoperative considerations, postoperative complications, and ongoing management of shunts for normal pressure hydrocephalus.

Latest Developments: The radiographic pattern of disproportionately enlarged subarachnoid space hydrocephalus (DESH) predicts a positive response to permanent CSF diversion. MRI-safe programmable shunt valves allow for fluid drainage adjustment based on patients' symptoms and radiographic images.

Clinical Features and Diagnosis of Spontaneous Intracranial Hypotension.

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.

Reversal of Coma With Trendelenburg Position in Spontaneous Intracranial Hypotension.

Spontaneous intracranial hypotension (SIH) is a clinical and radiologic syndrome caused by spinal leakage of cerebrospinal fluid due to a dural tear, leaking meningeal diverticulum, or cerebrospinal fluid-venous fistula. Whereas the hallmark clinical feature of SIH is orthostatic headache, in rare instances, life-threatening complications may include altered consciousness and even coma as a result of extreme downward displacement of the midbrain and brainstem. We describe the clinical features, neuroimaging findings, management strategies, and short-term outcomes of 2 unique cases of severe SIH and the role of Trendelenburg position to reverse coma.

Optimization of Photon-Counting CT Myelography for the Detection of CSF-Venous Fistulas Using Convolutional Neural Network Denoising: A Comparative Analysis of Reconstruction Techniques.

Background And Purpose: Photon-counting detector CT myelography (PCD-CTM) is a recently described technique used for detecting spinal CSF leaks, including CSF-venous fistulas. Various image reconstruction techniques, including smoother-versus-sharper kernels and virtual monoenergetic images, are available with photon-counting CT. Moreover, denoising algorithms have shown promise in improving sharp kernel images.

CSF-Venous Fistulas Arising Intraosseously within Bone Remodeled by Meningeal Diverticula.

CSF-venous fistulas (CVFs) are a common and increasingly recognized type of spinal CSF leak. Most of these fistulas occur in the setting of spontaneous intracranial hypotension, though nonspontaneous cases have been described as well. In most instances, CVFs arise from the dome or neck of nerve root sleeve diverticula (also called meningeal diverticula).

Education Research: Trends of Neurology Clerkships in the United States Amidst a Global Pandemic.

Background And Objectives: To report a 2022 survey of US medical school neurology clerkship directors (CDs) and to compare the results with those of similar surveys conducted in 2005, 2012, and 2017.

Methods: An American Academy of Neurology (AAN) Consortium of Neurology Clerkship Directors (CNCD) workgroup developed the survey sent to all neurology CDs listed in the AAN CNCD database. Comparisons were made with 2005, 2012, and 2017 surveys.

Curriculum Innovations: A Novel Neurology Clinician-Educator Program.

Background And Objectives: Neurology residents serve as frontline teachers for junior trainees but often lack formal training in medical education. We developed a novel longitudinal curriculum to enhance the teaching skills and educational leadership of residents interested in pursuing careers as clinician-educators.

Methods And Curriculum Description: We developed and piloted a Neurology Clinician-Educator Program (NCEP) with the following goals: (1) improve resident satisfaction with opportunities to develop teaching skills, (2) improve resident satisfaction with opportunities to transition into a clinician-educator role after training, and (3) enhance resident teaching skills using evidence-based strategies.

Real-Time 2D Phase-Contrast MRI to Assess Cardiac- and Respiratory-Driven CSF Movement in Normal Pressure Hydrocephalus.

Background And Purpose: In idiopathic normal pressure hydrocephalus (iNPH) patients, cerebrospinal fluid (CSF) flow is typically evaluated with a cardiac-gated two-dimensional (2D) phase-contrast (PC) MRI through the cerebral aqueduct. This approach is limited by the evaluation of a single location and does not account for respiration effects on flow. In this study, we quantified the cardiac and respiratory contributions to CSF movement at multiple intracranial locations using a real-time 2D PC-MRI and evaluated the diagnostic value of CSF dynamics biomarkers in classifying iNPH patients.

Quantifying CSF Dynamics disruption in idiopathic normal pressure hydrocephalus using phase lag between transmantle pressure and volumetric flow rate.

Background And Purpose: Idiopathic normal pressure hydrocephalus (iNPH) is a cerebrospinal fluid (CSF) dynamics disorder as evidenced by the delayed ascent of radiotracers over the cerebral convexity on radionuclide cisternography. However, the exact mechanism causing this disruption remains unclear. Elucidating the pathophysiology of iNPH is crucial, as it is a treatable cause of dementia.

Myelographic Techniques for the Localization of Cerebrospinal Fluid Leaks.

Spinal cerebrospinal fluid (CSF) leaks require advanced myelographic techniques for precise localization, which is in turn necessary for optimal treatment. Here, we will discuss the various myelographic techniques that have become available in recent years for CSF leak localization. Each of these can be used to detect many different types of spinal CSF leaks, although each modality has unique advantages and disadvantages, which will be outlined here.

Causes of Intracranial Hypotension: Spontaneous, Traumatic, and Iatrogenic Cerebrospinal Fluid Leaks.

Cerebrospinal fluid leaks are important to recognize because they can cause debilitating symptoms for patients and have life-threatening complications. Leakage of cerebrospinal fluid (CSF) from the subarachnoid space can occur at the cranial or spinal level, with distinct clinical presentations, diagnostic evaluations, and treatment modalities depending on the type and location of the leak. Spontaneous, traumatic, and iatrogenic spinal CSF leaks cause reduced intracranial CSF volume and the clinicoradiologic syndrome commonly called "intracranial hypotension".

Additional Diagnostic Value of Conebeam CT Myelography Performed after Digital Subtraction Myelography for Detecting CSF-Venous Fistulas.

Background And Purpose: CSF-venous fistulas (CVFs) are a common cause of spontaneous intracranial hypotension. The diagnosis and precise localization of these fistulas hinges on specialized myelographic techniques, which mainly include decubitus digital subtraction myelography and decubitus CT myelography (by using either energy-integrating or photon-counting detector CT). A previous case series showed that conebeam CT myelography (CB-CTM), performed as an adjunctive tool with digital subtraction myelography, increased the detection of CVFs.

CSF-venous fistulas associated with traumatic spinal pseudomeningoceles.

CSF-venous fistulas (CVFs) are a common cause of spontaneous intracranial hypotension. These fistulas usually occur without any preceding major trauma, surgery, or other iatrogenic cause. Occasionally, patients have a history of minor trauma, though such cases are usually still considered spontaneous.

Diagnosis and Treatment of Spontaneous Intracranial Hypotension: Role of Epidural Blood Patching.

Purpose Of Review: This review focuses on the challenges of diagnosing and treating spontaneous intracranial hypotension (SIH), a condition caused by spinal CSF leakage. It emphasizes the need for increased awareness and advocates for early and thoughtful use of empirical epidural blood patches (EBPs) in suspected cases.

Recent Findings: SIH diagnosis is hindered by variable symptoms and inconsistent imaging results, including normal brain MRI and unreliable spinal opening pressures.