Neuroimaging Clinics of North America

Primary central nervous system vasculitis (PCNSV) is a vasculitis limited to the brain and spinal cord. Induction therapy often consists of steroids and cyclophosphamide. Maintenance therapy includes a prednisone taper and may be combined with medications such as azathioprine or mycophenolate mofetil. Relapse is common in PCNSV and an increased dose of steroids is often given, sometimes with a change in therapy. Medications such as rituximab and mycophenolate mofetil may be good alternatives in those who do not respond to initial treatment or who have relapse of disease...

Cerebral amyloid angiopathy (CAA) is a cerebrovascular disorder marked by the accumulation of amyloid-beta peptide (Aβ) within the leptomeninges and smaller blood vessels of the brain. CAA can be both noninflammatory and inflammatory, and the inflammatory version includes Aβ-related angiitis (ABRA). ABRA is a vasculitis of the central nervous system related to an inflammatory response to Aβ in the vascular walls, which necessitates differentiating ABRA from noninflammatory CAA, as ABRA may require immunosuppressive treatment...

Childhood cerebral vasculitis is a condition that affects the blood vessels in the brain of children and is rare but life-threatening. Imaging plays a crucial role in the diagnosis and monitoring of the disease. This article describes the classification, diagnostic algorithm, and various imaging modalities used in the evaluation of childhood cerebral vasculitis and the imaging findings associated with primary and secondary vasculitis. Understanding the imaging features of this condition can assist in early diagnosis, effective treatment, and improve outcomes...

Stroke is a complication of many central nervous system (CNS) infections, but only a few present with stroke without other symptoms or signs of CNS infection. Chief among these are varicella zoster virus (VZV) and syphilis. Delayed cerebral vasculopathy after successful treatment of bacterial meningitis, most commonly pneumococcal, is an emerging entity with uncertain pathogenesis.

PRES and RCVS are increasingly recognized due to the wider use of brain MRI and increasing clinical awareness. Imaging plays a crucial role in confirming the diagnosis and guiding clinical management for PRES and RCVS. Imaging also has a pivotal role in determining the temporal progression of these entities, detecting complications, and predicting prognosis. In this review, we aim to describe PRES and RCVS, discuss their possible pathophysiological mechanisms, and discuss imaging methods that are useful in the diagnosis, management, and follow-up of patients...

Illicit and recreational drugs, such as cocaine, heroin, amphetamines, and marijuana, can result in drug-related vasculitis or vasculopathy. Similarly, the use of certain antithyroid, oncologic, and immunosuppressive medications for therapeutic purposes can lead to vasculopathy. This in turn may result in significant complications in the central nervous system, including intracranial hemorrhage and stroke. Cocaine abuse can also lead to midline destructive lesions of the sinonasal complex. MR imaging, Vessel Wall imaging, and CT/CTA are valuable imaging tools for the evaluation of patients with suspected drug-induced vasculopathy or vasculitis...

Vasculitis is characterized by the inflammation of blood vessels. Vasculitides refers to the different forms of vasculitis, often classified according to the size of the blood vessel that is involved. Vasculitis may occur as a primary process or secondary to many systemic diseases. This topic provides an overview of the clinical features, diagnosis, and classification of the different forms of vasculitides.

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Most primary orbital pathology in children is due to bacterial infection. Radiologists typically encounter these cases to evaluate for clinically suspected postseptal orbital involvement. Contrast-enhanced cross-sectional imaging is important for the detection and early management of orbital infection and associated subperiosteal/orbital abscess, venous thrombosis, and intracranial spread of infection. Benign mass-like inflammatory processes involving the pediatric orbit are rare, have overlapping imaging features, and must be distinguished from orbital malignancies...

Odontogenic and sinogenic infections are frequently encountered in the pediatric population. Although the diagnosis is often suspected clinically, imaging can play a significant role in localizing the site of infection, assessing for involvement of deep neck spaces, detection of abscess and other potentially life-threatening complications, and providing valuable information to help with treatment planning. This article reviews the general imaging considerations and anatomy relevant to odontogenic and paranasal sinus infections and describes the salient clinical and imaging features of infectious diseases of the dentition and sinuss...

Neck infections are common in children, though the clinical presentation is often vague and nonspecific. Therefore, imaging plays a key role in identifying the site and extent of infections, evaluating for potentially drainable collections, and assessing for airway and vascular complications. This review focuses on imaging features associated with common and characteristic neck infections in children to include tonsillar, retropharyngeal, and otomastoid infections; suppurative adenopathy; superimposed inflammation or infection of congenital cystic lesions; and Lemierre syndrome...

In this article, we will describe relevant anatomy and imaging findings of extraocular and orbital rim pathologic conditions. We will highlight important clinical and imaging pearls that help in differentiating these lesions from one another, and provide a few practical tips for challenging cases.

In this article, we will discuss the essential MR imaging protocol required for the assessment of ocular abnormalities including malignancies. Then we will describe relevant anatomy, ocular embryogenesis, and genetics to establish a profound understanding of pathophysiology of the congenital ocular malformations. Finally, we will discuss pediatric ocular malignancies, benign mimics, and the most common congenital ocular malformations with case examples and illustrations and give tips on how to distinguish these entities on neuroimaging...

Neck masses are frequent in the pediatric population and are usually divided into congenital, inflammatory, and neoplastic. Many of these lesions are cystic and are often benign. Solid masses and vascular lesions are relatively less common, and the imaging appearances can be similar. This article reviews the clinical presentation and imaging patterns of pediatric solid and vascular neck masses.

Congenital cystic masses are commonly encountered when imaging a patient presenting with a neck mass. Congenital cysts are present at birth; however, these cysts may not present until later in life with some growing slowly and others rapidly increasing in size due to hemorrhage of infection. A neonatal presentation is rare but when present may allow a narrower differential diagnosis. Imaging plays a significant role in defining a lesion as cystic, assessing location, and directing the next step in evaluation and or intervention...

There is a wide variety of disease entities in children, which can present with cervical adenopathy. The spectrum of pathology and imaging appearance differs in many cases from that seen in adults. This review aims to compare the strengths and limitations of the various imaging modalities available to image pediatric patients presenting with cervical adenopathy, provide guidance on when to image, and highlight the imaging appearance of both common and uncommon disorders affecting the cervical nodes in children to aid the radiologist in their clinical practice...

Pattern recognition of specific temporal bone radiological phenotypes, in association with abnormalities in other organ systems, is critical in the diagnosis and management of syndromic causes of hearing loss. Several recent publications have demonstrated the presence of specific radiological appearances, allowing precise genetic and/or syndromic diagnosis, in the right clinical context. This review article aims to provide an extensive but practical guide to the radiologist dealing with syndromic causes of hearing loss...

A variety of congenital and acquired disorders result in pediatric conductive hearing loss. Malformations of the external auditory canal are invariably associated with malformations of the middle ear space and ossicles. Isolated ossicular malformations are uncommon. Syndromes associated with external and middle ear malformations are frequently associated with abnormal development of first and second pharyngeal arch derivatives. Chronic inflammatory disorders include cholesteatoma, cholesterol granuloma, and tympanosclerosis...

Pediatric hearing loss is common with significant consequences in terms of language, communication, social and emotional development, and academic advancement. Radiological imaging provides useful information regarding hearing loss etiology, prognosis, therapeutic options, and potential surgical pitfalls. This review provides an overview of temporal bone imaging protocols, an outline of the classification of inner ear anomalies associated with sensorineural hearing loss and illustrates some of the more frequently encountered and/or important causes of non-syndromic hearing loss...