Objective: To study the clinical course, imaging, diagnosis and management of acute onset of generalized chorea with suspected Systemic lupus Erythematosus (SLE).

Background: Neuropsychiatric symptoms of SLE (NPSLE) comprise of 19 syndromes, including movement disorders¹.  Chorea, a hyperkinetic movement disorder characterised by involuntary, dance-like, and unpredictable movements, is rare in SLE, with a cumulative incidence of 0.6%.²

Method: A 17 year old boy, first-born child of healthy and non-consanguineous parents, presented with generalized chorea, difficulty in speaking, writing and walking for last 1 months, without history of dystonia, tremor, feeding difficulty and psychiatric or cognitive impairment. There is no relevant past or family history, including neuroleptic ingestion. Further, his mother informed of intermittent fever, dry cough, back pain, joint pain and leg pain with pedal oedema since last 3 years, with multiple admissions and symptomatic management.

On physical examination, he was conscious, oriented and following commands, with normal cranial nerve, motor, sensory and cerebellar examination. He has generalized chorea involving all four limbs, face and tongue.

Results: Renal and Liver function tests were normal. ASO Titer [47 IU/ml (normal<200IU/ml)] was normal. Significant derangements were Hypochromic Microcytic Anemia [Hb 9.5g/dl, MCV 73.9fl, MCH 23.2pg] with proteinuria (176 mg/dl) and positive APLA, Direct Coomb’s Test and ANA. Further evaluation showed positive dsDNA antibody (187.85 IU/ml), low C3 level [51 mg/dl] (Normal 88-165)  and low C4 Level [<8 mg/dl] (Normal 14-44)]. 2D Echo and MRI Brain were normal.

He was started on immunosuppressive therapy – methylprednisolone 1g/day for three days, followed by prednisone 1mg/kg/day and mycophenolate mofetil, up to a dose of 2g/day and Tab. Tetrabenazine 75mg/day and Tab. Risperidone 2mg/day without any response. Hence, Inj. Rituximab 1gm was administered with favourable response with completely resolution of chorea.

Conclusion: Our case highlights the crucial importance of a well-conducted interview and clinical observation, and the richness of a thorough differential diagnosis, underlining the importance of maintaining a high index of suspicion. This case illustrates the relevance of SLE in the differential diagnosis of acute-onset movement disorders in young patients.

References: 1. The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes. Arthritis Rheum. 1999, 42:4-599.
2. Bertsias GK, Boumpas DT: Pathogenesis, diagnosis and management of neuropsychiatric SLE manifestations. Nat Rev Rheumatol. 2010, 6:358-67.

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