Introduction Sjögren syndrome (SS) is a chronic autoimmune disorder characterized by inflammatory destruction of the body's exocrine glands and may be considered primary or secondary, in association with other autoimmune disorders, such as collagen vascular diseases. Although SS can present…
Introduction Background IgG4-related disease (IgG4-RD) is a relatively recently recognized fibroinflammatory condition of unknown etiology characterized by elevated serum IgG4, plasmacytic infiltration, multifocal tumefactive fibrosis, and clinical improvement by steroid therapy. The association between autoimmune (sclerosing) pancreatitis and elevated serum…
Introduction Thyroid-associated orbitopathy (TAO), thyroid eye disease (TED), dysthyroid/TAO, and Graves orbitopathy all refer to a poorly understood autoimmune phenomenon resulting in the adipogenesis of orbital fat. An estimated 40% of patients with Graves disease develop TAO. Up to 20%…
Introduction Anomalies arising from the third and fourth branchial cleft remnants are quite rare. In particular, branchial abnormalities related to the pharynx are only a small subset of the already rare collection of branchial cleft lesions. Branchial abnormalities with a…
Introduction There are various forms of orbital infection, including those listed in the Jain-Rubin classification: 1. Periorbital cellulitis 2. Orbital cellulitis with or without intracranial complications 3. Orbital abscess with or without intracranial complications a. Intraorbital abscess, which may arise…
Introduction Deformities along the dorsal aspect of the thoracic spinal cord are occasionally identified on magnetic resonance imaging (MRI), often in patients presenting with myelopathy ( Fig. 36.1 ). A variety of nontumoral etiologies result in deformities of the dorsal…
Introduction Hirayama disease is a rare, slowly progressive, and self-limiting cervical myelopathy related to neck flexion in young adults. It is characterized by a pure focal amyotrophy in the C7-T1 innervated musculature. It presents classically in an adolescent male with…
Introduction Spinal cord astrocytomas are quite uncommon and account for only 3% to 4% of all central nervous system (CNS) astrocytomas. Furthermore, although intracranial glioblastomas are the most common primary brain parenchymal neoplasm, spinal cord glioblastomas are exceedingly rare. The…
Introduction Spinal cord ependymomas are the most common intramedullary tumor in adults and the second most common tumor in children after astrocytomas. Ependymomas typically present within the fourth decade of life and affect men more often than women. A higher…
Introduction Subacute progressive ascending myelopathy (SPAM) is an exceedingly rare and poorly understood complication of spinal cord injury. As the natural history of this process is quite peculiar, it is especially important for the imaging interpreter to be aware of…