Myelography Using Energy-Integrating Detector CT Versus Photon-Counting Detector CT for Detection of CSF-Venous Fistulas in Patients With Spontaneous Intracranial Hypotension.

BACKGROUND. CSF-venous fistulas (CVFs), which are an increasingly recognized cause of spontaneous intracranial hypotension (SIH), are often diminutive in size and exceedingly difficult to detect by conventional imaging. OBJECTIVE. This purpose of this study was to compare energy-integrating detector (EID) CT myelography and photon-counting detector (PCD) CT myelography in terms of image quality and diagnostic performance for detecting CVFs in patients with SIH. METHODS. This retrospective study included 38 patients (15 men and 23 women; mean age, 55 ± 10 [SD] years) with SIH who underwent both clinically indicated EID CT myelography (slice thickness, 0.625 mm) and PCD CT myelography (slice thickness, 0.2 mm; performed in ultrahigh-resolution mode) to assess for CSF leak. Three blinded radiologists reviewed examinations in random order, assessing image noise, discernibility of spinal nerve root sleeves, and overall image quality (each assessed using a scale of 0-100, with 100 denoting highest quality) and recording locations of the CVFs. Definite CVFs were defined as CVFs described in CT myelography reports using unequivocal language and having an attenuation value greater than 70 HU. RESULTS. For all readers, PCD CT myelography, in comparison with EID CT myelography, showed higher mean image noise (reader 1: 69.9 ± 18.5 [SD] vs 37.6 ± 15.2; reader 2: 59.5 ± 8.7 vs 49.3 ± 12.7; and reader 3: 57.6 ± 13.2 vs 42.1 ± 16.6), higher mean nerve root sleeve discernibility (reader 1: 81.6 ± 21.7 [SD] vs 30.4 ± 13.6; reader 2: 83.6 ± 10 vs 70.1 ± 18.9; and reader 3: 59.6 ± 13.5 vs 50.5 ± 14.4), and higher mean overall image quality (reader 1: 83.2 ± 20.0 [SD] vs 38.1 ± 13.5; reader 2: 80.1 ± 10.1 vs 72.4 ± 19.8; and reader 3: 57.8 ± 11.2 vs 51.9 ± 13.6) (all p < .05). Eleven patients had a definite CVF. Sensitivity and specificity of EID CT myelography and PCD CT myelography for the detection of definite CVF were 45% and 96% versus 64% and 85%, respectively, for reader 1; 36% and 100% versus 55% and 96%, respectively, for reader 2; and 57% and 100% versus 55% and 93%, respectively, for reader 3. The sensitivity was significantly higher for PCD CT myelography than for EID CT myelography for reader 1 and reader 2 (both p < .05) and was not significantly different between the two techniques for reader 3 (p = .45); for all three readers, specificity was not significantly different between the two modalities (all p > .05). CONCLUSION. In comparison with EID CT myelography, PCD CT myelography yielded significantly improved image quality with significantly higher sensitivity for CVFs (for two of three readers), without significant loss of specificity. CLINICAL IMPACT. The findings support a potential role for PCD CT myelography in facilitating earlier diagnosis and targeted treatment of SIH, avoiding high morbidity during potentially prolonged diagnostic workups.

Patient experience of spontaneous intracranial hypotension (SIH): qualitative interviews for concept elicitation.

BACKGROUND & OBJECTIVES: Spontaneous intracranial hypotension (SIH) is an underdiagnosed and debilitating condition caused by a spinal cerebrospinal fluid (CSF) leak. Although SIH can lead to substantial morbidity and disability, little data exists about patients' perspectives. Without hearing directly from patients, our understanding of the full experience of having SIH is limited, as is our ability to identify and use appropriate patient-reported outcome measures (PROMs) within clinical care and research. The purpose of this study was to conduct qualitative interviews with confirmed SIH patients to fully describe their experiences and identify relevant concepts to measure. METHODS: Patients were recruited from an SIH specialty clinic at a large, U.S.-based healthcare center. Patients undergoing an initial consultation who were ≥ 18 years old, English-speaking, met the International Classification of Headache Disorders-3 criteria for SIH, and had a brain MRI with contrast that was positive for SIH were eligible to participate. During semi-structured qualitative interviews with a trained facilitator, participants were asked to describe their current SIH symptoms, how their experiences with SIH had changed over time, and the aspects of SIH that they found most bothersome. Analysts reviewed the data, created text summaries, and wrote analytic reports. RESULTS: Fifteen participants completed interviews. Common symptoms reported by patients included headache, tinnitus, ear fullness/pressure/pain, and neck or interscapular pain. Patients reported that their symptoms worsened over the course of their day and with activity. The most bothersome aspect of SIH was disruption to daily activities and limits to physical activities/exercise, which were severe. With regard to symptoms, the most bothersome and impactful included physical pain and discomfort (including headache), as well as fatigue. CONCLUSIONS: Patients reported a diverse set of symptoms that were attributed to SIH, with devastating impacts on functioning and high levels of disability. Researchers considering use of PROMs for SIH should consider inclusion of both symptom scales and aspects of functioning, and future work should focus on evaluating the validity of existing measures for this patient population using rigorous qualitative and quantitative methods in diverse samples. Additionally, these data can be used to assist clinicians in understanding the impacts of SIH on patients.

Dual energy CT for the identification of CSF-Venous Fistulas and CSF leaks in spontaneous intracranial hypotension: Report of four cases.

Spontaneous intracranial hypotension (SIH) is a debilitating condition caused by spinal CSF leaks or CSF-venous fistulas (CVFs). Localizing the causative CSF leak or CVF is critical for definitive treatment but can be difficult using conventional myelographic techniques because these lesions are often low contrast compared to background, diminutive, and in some cases may be mistaken for calcified structures. Dual energy CT (DECT) can increase the conspicuity of iodinated contrast compared to background and can provide the ability to distinguish materials based on differing anatomic properties, making it well suited to address the shortcomings of conventional myelography in SIH. The purpose of this report is to illustrate the potential benefits of using DECT as an adjunct to traditional myelographic techniques in order to increase the conspicuity of these often-subtle CVFs and CSF leaks. This retrospective case series included 4 adult patients with SIH who demonstrated findings equivocal for either CVF or CSF leak using our institution's standard initial CT myelogram and in whom subsequent evaluation with DECT ultimately helped to identify the CVF or CSF leak. DECT demonstrated utility by increasing the conspicuity of two subtle CVFs compared to background and also helped to differentiate between calcified osteophytes and extradural contrast in 2 CSF leaks, confirming their presence and identifying the causative pathology. Our observations demonstrate the benefit of DECT as a problem-solving tool in the accurate diagnosis and localization of CVFs and CSF leaks.

Differentiation of Chiari malformation type 1 and spontaneous intracranial hypotension using objective measurements of midbrain sagging.

OBJECTIVE: Chiari malformation type 1 (CM-1) and spontaneous intracranial hypotension (SIH) are causes of headache in which cerebellar tonsillar ectopia (TE) may be present. An accurate method for differentiating these conditions on imaging is needed to avoid diagnostic confusion. Here, the authors sought to determine whether objective measurements of midbrain morphology could distinguish CM-1 from SIH on brain MRI. METHODS: This is a retrospective case-control series comparing neuroimaging in consecutive adult subjects with CM-1 and SIH. Measurements obtained from brain MRI included previously reported measures of brain sagging: TE, slope of the third ventricular floor (3VF), pontomesencephalic angle (PMA), mamillopontine distance, lateral ventricular angle, internal cerebral vein-vein of Galen angle, and displacement of iter (DOI). Clivus length (CL), an indicator of posterior fossa size, was also measured. Measurements for the CM-1 group were compared to those for the entire SIH population (SIHall) as well as a subgroup of SIH patients with > 5 mm of TE (SIHTE subgroup). RESULTS: Highly significant differences were observed between SIHall and CM-1 groups in the following measures: TE (mean ± standard deviation, 3.1 ± 5.7 vs 9.3 ± 3.5 mm), 3VF (-16.8° ± 11.2° vs -2.1° ± 4.6°), PMA (44.8° ± 13.1° vs 62.7° ± 9.8°), DOI (0.2 ± 4.1 vs 3.8 ± 1.6 mm), and CL (38.3 ± 4.5 vs 44.0 ± 3.3 mm; all p < 0.0001). Eight (16%) of 50 SIH subjects had TE > 5 mm; in this subgroup (SIHTE), a cutoff value of < -15° for 3VF and < 45° for PMA perfectly discriminated SIH from CM-1 (sensitivity and specificity = 1.0). DOI showed perfect specificity (1.0) in detecting SIH among both groups. No subjects with SIH had isolated TE without other concurrent findings of midbrain sagging. CONCLUSIONS: Measures of midbrain sagging, including cutoff values for 3VF and PMA, discriminate CM-1 from SIH and may help to prevent misdiagnosis and unnecessary surgery.

CSF-Venous Fistulas: Anatomy and Diagnostic Imaging.

CSF-venous fistulas (CVFs), first described in 2014, are an important cause of spontaneous intracranial hypotension. CVFs can be challenging to detect on conventional anatomic imaging because, unlike other types of spinal CSF leak, they do not typically result in pooling of fluid in the epidural space, and imaging signs of CVF may be subtle. Specialized myelographic techniques have been developed to help with CVF identification, but these techniques are not yet widely disseminated. This article reviews the current understanding of CVFs, emphasizing correlations between venous anatomy and imaging findings as well as potential mechanisms for pathogenesis, and describes current imaging techniques used for CVF diagnosis and localization. These techniques are broadly classified into fluoroscopy-based methods, including digital subtraction myelography and dynamic myelography, and cross-sectional methods, including decubitus CT myelography and MR myelography with intrathecal injection of gadolinium. Knowledge of these various options, including their relative advantages and disadvantages, is critical in the care of patients with spontaneous intracranial hypotension. Investigation is ongoing, and continued advances in knowledge about CVFs as well as in optimal imaging detection are anticipated.

Magnetic Resonance Imaging Abnormalities of the Optic Nerve Sheath and Intracranial Internal Carotid Artery in Giant Cell Arteritis.

BACKGROUND: Giant cell arteritis (GCA) is an important diagnostic consideration in elderly patients with vision changes. Superficial temporal artery biopsy (TAB) has long been considered the gold standard diagnostic approach for GCA, but MRI has gained interest as an alternative diagnostic modality. Although most of the literature has focused on imaging abnormalities of branches of the external carotid artery, there have been a few reports of GCA-related inflammatory involvement of the orbit and internal carotid arteries (ICAs) on MRI. METHODS: This was a retrospective cross-sectional study of patients undergoing TAB at a single tertiary referral center over a 5-year period. Patients who had undergone contrast-enhanced MRI of the brain and orbits within 1 month of biopsy were included. Fifty-four TAB-positive and 78 TAB-negative patients were reviewed, with the MRI studies of 7 TAB-positive and 6 TAB-negative patients deemed adequate for interpretation. MRI studies were reviewed by 2 masked neuroradiologists, and the findings were correlated with biopsy results and clinical findings. RESULTS: Intracranial ICA vessel wall enhancement was identified in 6 of 7 TAB-positive patients (sensitivity 86%), compared with 2 of 6 TAB-negative patients (specificity 67%). Optic nerve sheath enhancement was identified in 5 of 7 TAB-positive patients (sensitivity 71%) and in 2 of 6 TAB-negative patients (specificity 67%), bilateral in all such cases. The combination of both abnormal imaging findings was observed in 5 of 7 TAB-positive patients (sensitivity 71%) and in none of the 6 TAB-negative patients (specificity 100%). CONCLUSIONS: Intracranial ICA and optic nerve sheath enhancement were observed in a majority of patients with TAB-proven GCA, and the combination of these findings was highly specific for GCA. Identification of these abnormalities on MRI should raise concern for GCA and prompt a thorough review of systems, laboratory testing, and consideration of TAB in patients with ocular complaints potentially consistent with ischemia.

Spontaneous Intracranial Hypotension: Pathogenesis, Diagnosis, and Treatment.

Spontaneous intracranial hypotension (SIH) is a clinical syndrome that is increasingly recognized as an important and treatable secondary cause of headaches. Insight into the condition has evolved significantly over the past decade, resulting in a greater understanding of the underlying pathophysiology, development of new diagnostic imaging tools, and a broadening array of targeted treatment options. This article reviews the clinical presentation and pathogenesis of SIH, discusses the important role of imaging in diagnosis, and describes how imaging guides treatment.

Intraspecific variation in semicircular canal morphology-A missing element in adaptive scenarios?

OBJECTIVES: Recent evidence suggests that the amount of intraspecific variation in semicircular canal morphology may, itself, be evidence for varying levels of selection related to locomotor demands. To determine the extent of this phenomenon across taxa, we expand upon previous work by examining intraspecific variation in canal radii and canal orthogonality in a broad sample of strepsirrhine and platyrrhine primates. Patterns of interspecific variation are re-examined in light of intraspecific variation to better understand the resolution at which locomotion can be reconstructed from single individuals. MATERIALS AND METHODS: Data was collected from high-resolution CT scans of 14 size-matched, related species. Six of these taxa have existing data on rotational head speeds. RESULTS: The level of intraspecific variation was found to differ in strepsirrhine and in platyrrhine species pairs, with larger ranges of variation generally observed for the slower moving taxon than the faster moving one. Taxa that are classified as relatively agile can to some extent be separated from those who are slower-moving, but only when comparing similarly sized, closely related species with more extreme forms of locomotion. DISCUSSION: Our findings agree with previous research showing that canal intraspecific variation can fluctuate according to species-specific locomotor behavior and extends this further by identifying behaviors that may be under unusual selective pressure. It also demonstrates the complexity of interpreting inner ear morphology in the context of broadly applicable locomotor "categories" of the kind commonly used in behavioral studies. We suspect that simplified models predicting vestibular sensitivity may be unable to differentiate behaviors when only a single specimen is available.

Approach to Imaging in Patients with Spontaneous Intracranial Hemorrhage.

Spontaneous intracranial hemorrhage is a commonly encountered neurologic emergency. Imaging plays important roles in both guiding the emergent stabilization of patients with ICH and in elucidating the etiology of the hemorrhage to prevent rebleeding. A thorough understanding of the factors that have an impact on immediate management, the causes of hemorrhage, and the strengths of various imaging techniques in addressing these 2 concerns is vital to crafting a patient-centered approach to this condition.

Update on the Diagnosis and Treatment of Spontaneous Intracranial Hypotension.

PURPOSE OF REVIEW: The purpose of this study is to provide an update on recent developments in the understanding, diagnosis, and treatment of spontaneous intracranial hypotension (SIH). RECENT FINDINGS: SIH is an important cause of headaches caused by spinal cerebrospinal fluid (CSF) leaks, with an increasingly broad spectrum of clinical presentations and diagnostic findings. A simple conception of the condition as being defined by the presence of low CSF pressure is no longer sufficient or accurate. A number of etiologies for spinal CSF leaks have been identified, including the recent discovery of CSF-venous fistulas, and these various etiologies may require different diagnostic and therapeutic pathways in order to affect a cure. Familiarity with the spectrum of presentations and causes of SIH is critical to accurate and timely diagnosis and management. Challenges exist in both diagnosis and treatment, and require understanding of the underlying pathogenesis of the condition in order to appropriately select testing and treatment. Prospective studies are needed going forward in order to inform workup and guide treatment decisions.

Locomotor head movements and semicircular canal morphology in primates.

Animal locomotion causes head rotations, which are detected by the semicircular canals of the inner ear. Morphologic features of the canals influence rotational sensitivity, and so it is hypothesized that locomotion and canal morphology are functionally related. Most prior research has compared subjective assessments of animal "agility" with a single determinant of rotational sensitivity: the mean canal radius of curvature (R). In fact, the paired variables of R and body mass are correlated with agility and have been used to infer locomotion in extinct species. To refine models of canal functional morphology and to improve locomotor inferences for extinct species, we compare 3D vector measurements of head rotation during locomotion with 3D vector measures of canal sensitivity. Contrary to the predictions of conventional models that are based upon R, we find that axes of rapid head rotation are not aligned with axes of either high or low sensitivity. Instead, animals with fast head rotations have similar sensitivities in all directions, which they achieve by orienting the three canals of each ear orthogonally (i.e., along planes at 90° angles to one another). The extent to which the canal configuration approaches orthogonality is correlated with rotational head speed independent of body mass and phylogeny, whereas R is not.