Bilateral transverse venous sinus stenosis (TVSS) or stenosis of a dominant venous sinus has been found to be very sensitive radiological findings in patients with intracranial hypertension (IH), yet there is still an ongoing debate whether they constitute reversible or permanent phenomena. Thus, the purpose of this study was to investigate the reversibility of TVSS in patients with IH, including conservatively treated patients with signs of IH as defined by the presence of papilledema.

This was a retrospective chart review of all patients diagnosed with IH between 2016 and 2022, assessed from 2 tertiary university-affiliated neuro-ophthalmology practices. Inclusion criteria were the presence of papilledema, as quantified by optical coherence tomography, and bilateral TVSS, which is considered typical of IH on neuroimaging. During follow-up, included patients must have had confirmation of papilledema resolution as well as subsequent neuroimaging after conservative treatment or cerebrospinal fluid flow diversion. Patients with dural sinus vein thrombosis or intrinsic stenosis from sinus trabeculations or significant arachnoid granulations were excluded from the study. Either CT venography or MRI/MR venography was reviewed by a fellowship-trained neuroradiologist, and the degree of stenosis was scored through the combined conduit score (CCS), as described by Farb et al. The primary outcome was to assess TVSS changes after resolution or improvement of papilledema.

From 435 patients, we identified a subset of 10 who satisfied all inclusion criteria. Our cohort comprised entirely women with a median age of 29.5 years and a median BMI of 32.5 kg/m 2 . Treatment consisted of acetazolamide in 7 patients, of which 1 had additional topiramate and 2 underwent cerebrospinal fluid flow diversion. Furthermore, 6 patients demonstrated significant weight loss during follow-up. For the primary outcome, 5 of 10 patients exhibited no appreciable TVSS change, and 5 patients demonstrated significant improvement in TVSS, of which 4 received conservative treatment only. Papilledema resolution or improvement was statistically significantly associated with increasing average CCS, TVSS diameter, and grade.

We were able to demonstrate that TVSS can be both irreversible and reversible in patients with resolved papilledema. The finding of TVSS reversibility from conservative treatment alone is novel and has important implications to optimize patient care. Future studies should work to identify factors associated with irreversible TVSS for subsequent targeted intervention and prevention.

Neuroimaging is a paramount element for the diagnosis of idiopathic intracranial hypertension, a condition characterized by signs and symptoms of raised intracranial pressure without the identification of a mass or hydrocephalus being recognized. The primary purpose of this review is to deliver an overview of the spectrum and the specific role of the various imaging findings associated with the condition while providing imaging examples and educational concepts. Clinical perspectives and insights into the disease, including treatment options, will also be discussed.

Transverse sinus stenosis (TSS) is an abnormality in the cerebral venous system in which the narrowing of the transverse sinus of the brain leads to obstructed cerebral venous outflow. It is an infrequent, incidental radiological finding. However, it is not uncommon among patients with chronic headaches of unclear cause, particularly those that remain unexplained after initial evaluation or those that are refractory to medical treatment. Its diagnosis frequently eludes the initial workup, and a high degree of suspicion should be maintained since its identification can lead to potentially curative treatment. This report describes the case of a 36-year-old female with a history of chronic headache who was found to have TSS. This paper discusses its etiology, pathophysiology, clinical presentation, radiological findings, and management.

The surgical treatment of Chiari malformation type I (CM-I) frequently involves dural incision at the posterior cranial fossa. In cases of persistent patent occipital sinus (OS), the sinus is usually obliterated and divided. However, there are some patients whose OS is prominent and requires crucial modification of the operative planning to avoid potentially life-threatening massive hemorrhage and disturbance of cerebral venous circulation. In the present study, the anatomical variations of the dominant OS in patients with CM-I were analyzed and the authors attempted to develop treatment recommendations for patients with CM-I with dominant OS.

The study included 213 patients with CM-I who underwent MR venography (MRV) prior to surgical treatment. OS dominance was assessed using 2D time-of-flight MRV or 3D phase-contrast MRV. Particular attention was paid to the pattern of venous outflow channels. The characteristics of the patients with dominant OS and the surgical outcomes were retrospectively reviewed.

Dominant OS was identified in 7 patients (3.3%). The age in those with dominant OS was significantly younger than in those without (p = 0.0202). The incidence of concurrent scoliosis in the patients with dominant OS was significantly higher than in those without (p = 0.0366). All the dominant OSs were found to be of the oblique type. Unilateral oblique OS (OOS) with normal ipsilateral transverse sinus (TS) and hypoplastic contralateral TS was found in 2 patients (0.9%). The authors found 1 patient each (0.5%) who had unilateral OOS with hypoplastic ipsilateral TS and normal contralateral TS, unilateral OOS with bilateral hypoplastic TSs, and bilateral OOSs with bilateral normal TSs. Bilateral OOSs with bilateral hypoplastic TSs were found in 2 patients (0.9%). All these patients had syringomyelia. Instead of performing Y-shaped dural incision and duraplasty, surgical procedures were modified depending on the types of the OOSs to preserve their venous drainage routes. Although massive bleeding from the dominant OS during dural incision occurred in 1 patient, none suffered neurological deterioration. The syrinx volume decreased in all but 1 of the patients postoperatively.

Assessment of the venous drainage pattern using MRV is indispensable for safe surgical treatment in patients with CM-I. The surgical procedure should be modified based on the type of dominant OS to minimize the surgical risks.

Some patients with idiopathic intracranial hypertension (IIH) have cerebellar tonsillar herniation ≥ 5 mm mimicking Chiari malformation I (CMI), which can result in misdiagnosis and unjustified treatment. Our purpose was to identify IIH patients with tonsillar herniation ≥ 5 mm (IIH_TH) and compare with CMI patients to assess imaging findings that could distinguish the two conditions.

Ninety-eight patients with IIH, 81 patients with CMI, and 99 controls were retrospectively assessed. Two neuroradiologists blindly reviewed MR images. IIH_TH patients were compared with CMI patients and controls regarding the extent of tonsillar herniation (ETH), bilateral transverse sinus stenosis (BTSS), hypophysis-sella ratio (HSR), and bilateral tortuosity of optic nerve (BTON).

13/98 (13.2%) IIH patients had tonsillar herniation ≥ 5 mm (IIH_TH) and were significantly younger and had higher BMI compared with CMI patients and controls. ETH was significantly less in the IIH_TH than CMI (6.5 ± 2.4 mm vs. 10.9 ± 4.4 mm; p < 0.001). BTSS and HSR < 0.5 were more common in IIH_TH than CMI (p < 0.001 and p = 0.003, respectively). No differences were seen between CMI and controls. BTON was significantly more common in IIH_TH compared to control (p = 0.01) but not to the CMI (p = 0.36). Sensitivity and specificity to differentiate IIH_TH from CMI were 69.2% and 96.1% for BTSS and 69.2% and 75.3% for HSR < 0.5.

The presence of BTSS and/or HSR < 0.5 in patients with ETH ≥ 5 mm should suggest further evaluation to exclude IIH before considering CMI surgery.

The etiology of idiopathic intracranial hypertension (IIH) is uncertain. Studies suggest the fundamental cause of the Chiari 1 malformation, a congenitally hypoplastic posterior fossa, may explain the genesis of IIH in some patients.

To assess the hypothesis that linear and volumetric measurements of the posterior fossa (PF) can be used as predictors of IIH.

A retrospective analysis of magnetic resonance imaging (MRI) studies on 27 patients with IIH and 14 matched controls was performed. A volumetric sagittal magnetization prepared rapid acquisition gradient echo sequence was used to derive 10 linear cephalometric measurements. Total intracranial and bony posterior fossa volumes (PFVs) were derived by manual segmentation. The ratio of PFV to total intracranial volume was calculated.

In total, 41 participants were included, all women. Participants with IIH had higher median body mass index (BMI). No significant differences in linear cephalometric measurements, total intracranial volumes, and PFVs between the groups were identified. Linear measurements were not predictive of volumetric measurements. However, on multivariate logistic regression analysis, the likelihood of IIH decreased significantly per unit increase in relative PFV (odds ratio [OR]=3.66 × 10^-50; 95% confidence interval [CI]=1.39 × 10^-108 to 1.22 × 10^-5; P = 0.04). Conversely, the likelihood of IIH increased per unit BMI increase (OR=1.19; 95% CI=1.04-1.47; P = 0.02).

MRI-based volumetric measurements imply that PF alterations may be partly responsible for the development of IIH and Chiari 1 malformations. Symptoms of IIH may arise due to an interplay between these and metabolic, hormonal, or other factors.

Morphological variations of falx cerebri and tentorium cerebelli are extremely rare. The authors report an extremely rare type of combined variation of falx cerebri and dural venous sinuses in the tentorium cerebelli. During the removal of the brain from cranial cavity, it was noted that the anterior part of the falx cerebri had fenestrations and it looked like a mesh. There was no associated variations of medial surface of cerebrum. Further, there were unusual sinuses within the tentorium cerebelli. Two of them were present in the right half of the tentorium cerebelli and one in the left half of the tentorium cerebelli. There variations could be of importance to radiologists and neurosurgeons. The fenestrations of falx cerebri might lead to misinterpretations in cases of head injuries and the additional sinuses in the tentorium cerebelli might cause unexpected bleeding during surgeries of posterior cranial fossa.

Symptomatic Chiari Type I Malformation (CM) is treated with posterior fossa decompression with or without duroplasty. We have noticed some cases with concomitant severe cerebellar ataxia due to cerebellar atrophy. The aim of this study is to review the literature of CM associated with severe cerebellar atrophy and discuss its potential physiopathology.

A systematic literature review in the Pubmed Database was performed using the following key-terms: "cerebellar atrophy Chiari", and "cerebellar degeneration Chiari". Articles reporting the presence of cerebellar degeneration/atrophy associated with CM were included.

We found only six studies directly discussing the association of cerebellar atrophy with CM, with a total of seven cases. We added one case of our own practice for additional discussion. Only speculative causes were described to justify cerebellar atrophy. The potential causes of cerebellar atrophy were diffuse cerebellar ischemia from chronic compression of small vessels (the most mentioned speculative cause), chronic raised intracranial pressure due to CSF block, chronic venous hypertension, and association with platybasia with ventral compression of the brainstem resulting in injury of the inferior olivary nuclei leading to mutual trophic effects in the cerebellum. Additionally, it is not impossible to rule out a degenerative cause for cerebellar atrophy without a causative reason.

Severe cerebellar atrophy is found in some patients with CM. Although chronic ischemia due to compression is the most presumed cause, other etiologies were proposed. The real reasons for cerebellar degeneration are not known. Further studies are necessary.

Intracranial pressure (ICP) is the pressure inside the bony calvarium and can be affected by a variety of processes, such as intracranial masses and edema, obstruction or leakage of cerebrospinal fluid, and obstruction of venous outflow. This review focuses on the imaging of 2 important but less well understood ICP disorders: idiopathic intracranial hypertension and spontaneous intracranial hypotension. Both of these ICP disorders have salient imaging findings that are important to recognize to help prevent their misdiagnosis from other common neurological disorders.

Chiari type 1 malformation (CM1) rarely causes papilloedema, which is indicative of high intracranial pressure with or without ventricular dilatation. Furthermore, concomitant brain parenchymal abnormalities have not been reported to date. In this paper, the authors report on a young woman of CM1-induced intracranial hypertension (ICH) with diffuse brain edema with a focus on venous sinus assessment, and discuss the surgical strategy. A 24-year-old woman presented to Nagoya University Hospital complaining of 4-year history of severe occipital headache and blurry vision with slowly progressive worsening. Head and whole spine MRI showed a CM1 with diffuse white matter hyperintensities (WMH) on T2-weighted imaging and narrowed brain sulci without hydrocephalus. Lumbar puncture revealed extremely high opening pressure. Detailed blood examination and other radiographical imaging studies denied the presence of tumor, collagen disease, encephalitis and other entities. Head magnetic resonance venography and angiography demonstrated severe transverse sinus stenosis on both sides. Foramen magnum decompression was performed to alleviate the ICH by restoration of cerebrospinal fluid (CSF) stagnation at the foramen magnum with successful outcome. The patient completely recovered from preoperative symptoms immediately after surgery. The diffuse WMH and narrowing brain sulci have been resolving. The most feasible explanation for this complicated pathophysiology was ICH induced by CM1 led to transverse sinus collapse, resulting in diffuse WMH as a result of venous hypertension. This case report is the first illustration of successful surgical treatment of CM1 with diffuse brain edema with a focus on venous sinus assessment.

The primary role of brain imaging in idiopathic intracranial hypertension (IIH) is to exclude other pathologies causing intracranial hypertension. However, subtle radiologic findings suggestive of IIH have emerged with modern neuroimaging. This review provides a detailed description of the imaging findings reported in IIH and discusses their possible roles in the pathophysiology and the diagnosis of IIH.

References were identified by searches of PubMed from 1955 to January 2015, with the terms "idiopathic intracranial hypertension," "pseudotumor cerebri," "intracranial hypertension," "benign intracranial hypertension," "magnetic resonance imaging," "magnetic resonance venography," "computed tomography (CT)," "CT venography," "imaging," and "cerebrospinal fluid (CSF) leak." Additional references were identified by hand search of relevant articles. When possible, we extracted the number of patients and control subjects from each study for each radiological finding. When at least 2 studies used the same criteria to define a radiological finding, all patients from these studies were pooled to obtain a mean sensitivity and specificity with 95% confidence interval.

Specific neuroimaging findings may suggest long-standing IIH, including empty sella, flattening of the posterior globes, optic nerve head protrusion, distention of the optic nerve sheaths, tortuosity of the optic nerve, cerebellar tonsillar herniation, meningoceles, CSF leaks, and transverse venous sinus stenosis.

Although IIH remains a diagnosis of exclusion, the most recently proposed diagnostic criteria have included neuroimaging findings to suggest IIH when major diagnostic criteria are not fulfilled. However, these findings are not diagnostic of IIH, and their presence is not required for the diagnosis of definite IIH. Their incidental discovery on brain imaging should not prompt invasive procedures, unless other signs of IIH, such as papilledema, are present.

OBJECTIVE Cerebral venous pressure gradient (CVPG) from dural venous sinus stenosis is implicated in headache syndromes such as idiopathic intracranial hypertension (IIH). The incidence of CVPG in headache patients has not been reported. METHODS The authors reviewed all cerebral venograms with manometry performed for headache between January 2008 and May 2015. Patient demographics, headache etiology, intracranial pressure (ICP) measurements, and radiographic and manometric results were recorded. CVPG was defined as a difference ≥ 8 mm Hg by venographic manometry. RESULTS One hundred sixty-four venograms were performed in 155 patients. There were no procedural complications. Ninety-six procedures (58.5%) were for patients with IIH. The overall incidence of CVPG was 25.6% (42 of 164 procedures): 35.4% (34 of 96 procedures) in IIH patients and 11.8% (8 of 68 procedures) in non-IIH patients. Sixty procedures (36.6%) were performed in patients with preexisting shunts. Seventy-seven patients (49.7%) had procedures preceded by an ICP measurement within 4 weeks of venography, and in 66 (85.7%) of these patients, the ICP had been found to be elevated. CVPG was seen in 8.3% (n = 5) of the procedures in the 60 patients with a preexisting shunt and in 0% (n = 0) of the 11 procedures in the 77 patients with normal ICP (p < 0.001 for both). Noninvasive imaging (MR venography, CT venography) was assessed prior to venography in 112 (68.3%) of 164 cases, and dural venous sinus abnormalities were demonstrated in 73 (65.2%) of these cases; there was a trend toward CVPG (p = 0.07). Multivariate analysis demonstrated an increased likelihood of CVPG in patients with IIH (OR 4.97, 95% CI 1.71-14.47) and a decreased likelihood in patients with a preexisting shunt (OR 0.09, 95% CI 0.02-0.44). CONCLUSIONS CVPG is uncommon in IIH patients, rare in those with preexisting shunts, and absent in those with normal ICP.