Mehwish Farrukh, Adil Ramzan.
A Case of Lupus Cerebritis in Dilemma: An Early Presentation of Overlap Syndrome or Mixed Connective Tissue Disease.
Ann Abbasi Shaheed Hosp Karachi Med Dent Coll Jan ;21(4):245-8.

We describe the unusual case of a teenage girl who was presented to a tertiary care center with unexplained acute encephalopathy preceded by long-standing severe headaches, irritability, cognitive impairment and ultimately altered level of consciousness. Her extensive workup was exceptional for abnormal Computerized Tomography (CT) scan suggestive of small and medium-vessel vasculitis, persistently elevated protein in the cerebrospinal fluid and steadily high titers of Anti Nuclear Antibody (ANA), anti-ribonuclear protein and anti-double stranded DNA antibody. Lupus cerebritis is a debilitating neurological complication confronted in rare patients of mixed connective tissue disease. We discuss a case of lupus cerebritis successfully diagnosed and treated. The coexistence of signs, symptoms and immunological features of 4 defined autoimmune diseases: systemic lupus erythematosus (SLE), systemic sclerosis (SS), polymyositis (PM) and rheumatoid arthritis (RA). The patient showed unpretentious response to intravenous high-dose methylprednisolone, azathioprine and hydroxychloroquine. Debate exists as to the best approach for treatment. Newer combination therapies based on anecdotal facts are suggested. We proposed that the patient's neurologic disease is secondary to immune-mediated central nervous system vasculitis, possibly as an initial manifestation of mixed connective tissue disease. Prompt diagnosis of lupus cerebritis is tremendously difficult because there is no single radiological confirmatory test and laboratory parameter. Assessment of clinical features, antibody detection and cerebrospinal fluid analysis lead to the diagnosis. Keywords: Anti-double stranded DNA, antinuclear antibody, lupus cerebritis, polymyositis, antiribonuclear protein, systemic lupus erythematosus.

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