The term rhombencephalitis refers to inflammatory diseases affecting the hindbrain (brainstem and cerebellum). Rhombencephalitis has a wide variety of etiologies, including infections, autoimmune diseases, and paraneoplastic syndromes. Infection with bacteria of the genus Listeria is the most common cause of rhombencephalitis. Primary rhombencephalitis caused by infection with Listeria spp. occurs in healthy young adults. It usually has a biphasic time course with a flu-like syndrome, followed by brainstem dysfunction; 75% of patients have cerebrospinal fluid pleocytosis, and nearly 100% have an abnormal brain magnetic resonance imaging scan. However, other possible causes of rhombencephalitis must be borne in mind. In addition to the clinical aspects, the patterns seen in magnetic resonance imaging can be helpful in defining the possible cause. Some of the reported causes of rhombencephalitis are potentially severe and life threatening; therefore, an accurate initial diagnostic approach is important to establishing a proper early treatment regimen. This pictorial essay reviews the various causes of rhombencephalitis and the corresponding magnetic resonance imaging findings, by describing illustrative confirmed cases.

Keywords: Encephalitis; Rhombencephalon; Magnetic resonance imaging.

Fungal infections of the central nervous system (CNS) are rare. However, because of the increase in the number of immunocompromised individuals, they have been gaining prominence in the differential diagnosis of CNS infections. Imaging techniques are sensitive for detecting and localizing an abnormality, in many cases allowing the origin of a lesion to be categorized as infectious, inflammatory, neoplastic, or vascular. This essay illustrates the magnetic resonance imaging and computed tomography findings of the most common fungal infections of the CNS, based on the experience of the Radiology Department of the Hospital de Clínicas de Porto Alegre, in the city of Porto Alegre, RS, Brazil.

Keywords: Central nervous system fungal infections/diagnostic imaging; Mycoses/diagnosis; Brain abscess/diagnostic imaging; Tomography, X-ray computed; Magnetic resonance imaging.

Cerebral venous thrombosis (CVT) is an uncommon condition that is potentially reversible if properly diagnosed and promptly treated. Although CVT can occur at any age, it most commonly affects neonates and young adults. Clinical diagnosis is difficult because the clinical manifestations of CVT are nonspecific, including headache, seizures, decreased level of consciousness, and focal neurologic deficits. Therefore, imaging is crucial for the diagnosis. Radiologists should be able to identify the findings of CVT and to recognize potential imaging pitfalls that may lead to misdiagnosis. Thus, the appropriate treatment (anticoagulation therapy) can be started early, thereby avoiding complications and unfavorable outcomes.

Keywords: Intracranial venous thrombosis/diagnostic imaging; Tomography, X-ray computed; Magnetic resonance imaging.

Von Hippel-Lindau (VHL) disease is a rare, autosomal dominant inherited syndrome that affects the germline of the VHL gene, a tumor suppressor gene. VHL disease is characterized by the multisystemic development of a variety of benign and malignant tumors, especially in the central nervous system (CNS). Such tumors include retinal and CNS hemangioblastomas, as well as endolymphatic sac tumors. The various tumor sites are responsible for the diversity of signs and symptoms related to the disease. The mean age at symptom onset is 33 years. Despite medical advances, the average life expectancy of patients with VHL disease is 49 years. Imaging plays a pivotal role in the clinical diagnosis and is essential to the follow-up of patients with VHL disease. This pictorial essay describes characteristic CNS manifestations of VHL disease-related tumors that all radiology residents should be aware of.

Keywords: von Hippel-Lindau disease; Hemangioblastoma; Endolymphatic sac/pathology; Magnetic resonance imaging; Tomography, X-ray computed.

Idiopathic intracranial hypertension is characterized by increased intracranial pressure, headache, and visual perturbations. Although the pathophysiology of idiopathic intracranial hypertension is obscure, several mechanisms have been proposed, such as increased cerebral blood volume, excessive cerebrospinal fluid volume (due to high production or impaired resorption), and inflammatory mechanisms as a likely cause of or contributor to impaired cerebrospinal fluid circulation. It predominantly affects women of reproductive age who are overweight or obese. The most common symptoms are daily headache, synchronous pulsatile tinnitus, transient visual perturbations, and papilledema with visual loss. The main neuroimaging findings are a partially empty sella turcica; flattening of the posterior sclera; transverse sinus stenosis (bilateral or in the dominant sinus); a prominent perioptic subarachnoid space, with or without optic nerve tortuosity; and intraocular protrusion of the optic nerve head. The main complication of idiopathic intracranial hypertension is visual loss. Within this context, neuroimaging is a crucial diagnostic tool, because the pathology can be reversed if properly recognized and treated early.

Keywords: Pseudotumor cerebri; Neuroimaging; Papilledema.

OBJECTIVE: This study aimed to assess whether the evaluation of the central vein sign (CVS) and paramagnetic rim lesions (PRLs) using susceptibility-weighted magnetic resonance imaging (MRI) can distinguish multiple sclerosis (MS) from migraine.
MATERIALS AND METHODS: In this single-center observational study, we conducted a cross-sectional analysis of the CVS, determining the proportion of CVS-positive lesions per individual and absolute counts, using thresholds of 3 lesions (select3*) and 6 lesions (select6*), and of PRLs in participants with MS and in those with migraine, from 3.0-T MRI brain scans.
RESULTS: The study included 20 participants with MS, 20 with migraine, and 20 included as healthy controls. The proportion of participants with CVS-positive lesions was higher in the MS group than in the migraine group (61.8% vs. 10.4%), and PRLs were observed exclusively in the MS group. The presence of at least one PRL and the select6* criterion demonstrated the highest diagnostic accuracy within the study sample.
CONCLUSION: The detection of the CVS and of a PRL on 3.0-T MRI scans may serve as a reliable biomarker to differentiate MS from migraine.

Keywords: Cerebral veins/diagnostic imaging; Multiple sclerosis/diagnosis; Migraine disorders/diagnosis; Neuroimaging/methods; Magnetic resonance imaging/methods; Biomarkers/analysis.