CT and MR Imaging of Progressive Dural Involvement by Nephrogenic Systemic Fibrosis

Abstract

SUMMARY:We present a patient with progressive dural calcifications, thickening, and enhancement presumably related to the development of nephrogenic systemic fibrosis (NSF). Head CT demonstrated progressive dural calcifications, whereas MR imaging demonstrated progressive dural thickening and enhancement during a 3-year period in which the patient received several gadoliniumenhanced MR imaging studies. To the best of our knowledge, dural calcifications are the only described intracranial finding of NSF.

Pathophysiology of gadolinium-associated systemic fibrosis

“…With the use of light microscopy, the dermal collagen is notably increased (27), with bundles haphazardly arranged (56) or "disorganized" (48). Mucin may be present (29,55,61,105), but is not required for the diagnosis (59). The abundant, thin, and spindle-shaped cells often express CD34 and type I (immature) procollagen (22,24,59).…”

Acetazolamide therapy in meningeal involvement of nephrogenic systemic fibrosis

“…Meningeal involvement has been previously reported in two autopsy cases 3,5 . The CT brain scan usually shows dural calcification and MRI demonstrates progressive dural thickening with enhancement 6 . This process of meningeal fibrosis usually evolves over several years.…”

Distinguishing magnetic resonance imaging features between idiopathic hypertrophic pachymeningitis and secondary hypertrophic pachymeningitis

“…Patients commonly present with longstanding headache and/or progressive neurologic deficits, multiple cranial nerve palsies, or blindness [1]. Several etiologies have been recognized including infections (such as syphilis and tuberculosis), autoimmune disorders (such as IgG4-related disease, rheumatoid arthritis), vasculitis (such as Wegener's granulomatosis), granulomatous inflammation (such as sarcoidosis), and neoplasms (such as lymphoma or metastasis) [2][3][4][5]. Blood serology, CSF examination and cytology, and/or dural biopsy for histopathology are required for determining the etiology.…”