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Abstract Details

Bilateral Confluent Leukoencephalopathy and Parkinsonism as a Manifestation of Systemic Lupus Erythematosus: A Case Report
Neurotoxicology
P06 - (-)
216
BACKGROUND: A 48 year-old woman with a 20-year history of SLE presented with a 6-month history of progressive hand clumsiness and gait dysfunction. She also described a progressive decline in her mood and memory.
DESIGN/METHODS: On examination, she was bradyphrenic, hypophonic, and hypomimic with symmetric bradykinesia, rigidity, intention tremor, stooped posture and a shuffling gait. Her handwriting was micrographic. Brain MRI showed extensive bilateral confluent leukoencephalopathy affecting hemispheres and cerebellum and sparing the basal ganglia and dentate nuclei.
RESULTS: In the context of the clinical and MRI findings, diagnostic considerations included adult leukodystrophy versus atypical, acquired forms of demyelinating disease due to SLE/aPLs, and secondary Sjogren's syndrome (SS). Genetic testing for adult-onset Gaucher disease, Alexander disease, and cerebrotendinous xanthomatosis. Cerebrospinal fluid examination showed an elevated myelin basic protein and a faint gamma band on electrophoresis. CSF angiotensin converting enzyme was normal. The serum ANA titer was highly elevated, while SSA and SSB titers were normal. Elevated serum anticardiolipin antibody and positive lupus anticoagulant indicated aPLs. Urinalysis showed an elevated protein and a kidney biopsy revealed class IV lupus nephritis. She experienced significant clinical improvement with partial resolution of MRI findings after 6 months of treatment with high-dose steroids and cyclophosphamide followed by mycophenylate, as well as controlled-release carbidopa/levodopa.
CONCLUSIONS: We conclude that parkinsonism and confluent white matter injury may be immune-mediated and can be an atypical manifestation of aPLs and SLE. Importantly, it may respond to immunosuppressive therapy.
Authors/Disclosures
Roople K. Unia, MD (Halifax Infirmary)
PRESENTER 		Dr. Unia has nothing to disclose.
Thomas G. Brott, MD, FAAN (Mayo Clinic) Dr. Brott has nothing to disclose.
Eric L. Logigian, MD, FAAN (University of Rochester Medical Center) Dr. Logigian has nothing to disclose.
Karen Odrzywolski, MD (CMC VAMC) Dr. Odrzywolski has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Empire State Medical, Scientific and 好色先生al Foundation.