1318 - Decreased Efficacy of Intrathecal Baclofen Related to Lupus Cerebritis

Case Diagnosis: Lupus Cerebritis in the setting of Intrathecal Baclofen Therapy

Case Description:

A 24-year-old female with a history of anxiety, spastic diplegic cerebral palsy (GMFCS III) with an intrathecal Baclofen pump (ITB), presented with 1 week of altered mental status, hallucinations, staring, and rigidity. Two months prior, the patient developed fluctuating spasticity requiring frequent ITB dose adjustments. A diagnostic workup was unremarkable for ITB malfunction. One week prior to presentation, her outpatient psychiatrist diagnosed catatonia. Initially, the catatonia was responsive to Ativan, however, her symptoms progressed prompting hospital admission. Initial imaging and lab work up showed no infectious, or metabolic etiology. Cerebrospinal fluid revealed elevated gamma globulin. Serum findings were significant for positive ANA, anti-dsDNA, and anti-RNP. Of note, she was also found to have significant proteinuria on UA. Rheumatology recommended starting aggressive management for SLE due to renal and cerebral involvement. She was started on Prednisone, Plaquenil, and Methotrexate.She demonstrated rapid clinical improvement in catatonia, spasticity, and hallucination symptoms.

Discussions: Lupus Cerebritis is diagnostically challenging and should be considered in women with family history of autoimmune conditions presenting with new onset psychiatric symptoms in conjunction with worsening spasticity. CSF auto-antibodies and cerebral inflammation may have contributed to ITB resistance in this case.

Conclusions: It is important to investigate all potential underlying etiologies for reduced ITB efficacy in patients previously well controlled.