Systemic lupus erythematosus (SLE) is a systemic multiorgan autoimmune disorder of unknown etiology. SLE commonly affects the peripheral or central nervous system. Rosenberg and Lefkowitz from University of California, San Francisco, presented a case of a 21-year-old woman with a recent diagnosis of SLE who presented with fever, generalized tonic-clonic seizures, somnolence, nystagmus, and gait ataxia. Lumbar puncture revealed straw-colored CSF containing 33 white blood cells/mm3 (75% monocytes) and elevated protein of 163 mg/dL. MRI revealed bilateral symmetric poorly marginated lesions, hypointense on T1-weighted images and hyperintense on T2-weighted images involving the superior cerebellum. Lesions also involved the splenium of the corpus callosum. After excluding underlying causes and observing the patient's nearly complete response to immunosuppressive therapy, a diagnosis of lupus cerebellitis was made.
This case illustrates the increasing spectrum of involvement of the brain associated with SLE. Furthermore, in patients with cerebellitis without previous medical illnesses, a work-up for SLE is warranted if no other obvious cause for cerebellitis is identified. Finally, this case shows the role of MRI in diagnosing cerebellitis and guiding further therapy, as previously reviewed in the literature and on Medscape.
Cerebellitis may occur as a primary infectious or postinfectious inflammatory disorder caused by a host of viral agents, such as enteroviruses, herpesviruses, HIV, and rabies, or bacteria such as Borrelia burgdorferi (Lyme disease), Mycoplasma pneumoniae, Legionella, and Coxiella burnetii (Q fever). Cerebellitis may follow immunizations such as hepatitis, smallpox, and measles vaccination, or may occur without an identifiable cause. Although patients with cerebellitis may have a normal MRI, abnormal findings are usually seen, including parenchymal hyperintensities on T2-WI, swelling, and secondary obstructive hydrocephalus. Changes typically involve the cerebellar gray matter more than the white matter. Follow-up studies usually show reversal of lesions and the development of atrophy in some instances. Contrast enhancement may or may not be present.
Medscape Neurology. 2003;5(1) © 2003 Medscape
Cite this: Highlights of the 26th Annual Meeting of the American Society of Neuroimaging - Medscape - Mar 27, 2003.