Parkinsonism as unusual neurological complication in childhood systemic lupus erythematosus

Abstract

Parkinsonism complicating systemic lupus erythematosus (SLE) is extremely rare. We report two girls with SLE who developed extrapyramidal parkinsonian features after an initial stormy course. One patient presented with generalized tonic clonic seizure and was then noted to have akinetic mutism and masked face. MRI brain revealed abnormal signals in bilateral basal ganglia and single photon emission computed tomography (SPECT) showed hypoperfusion in the same area. EEG background was slow and disorganized. Symptoms persisted despite high dose intravenous methylprednisolone and cyclophosphamide. Intravenous immunoglobulin (IVIG) was prescribed empirically and was followed by complete recovery. Both EEG and MRI brain were normal on follow-up. The second patient was found unconscious and then developed bradykinesia, mutism and shuffling gait. MRI and SPECT both detected abnormalities in basal ganglia. EEG was slow. Intravenous immunoglobulin was given after methylprednisolone and cyclophosphamide. This was followed by clinical improvement. The pathogenesis of basal ganglia injury in SLE, along with the management of cerebral lupus and the mechanisms of action of IVIG, are discussed.

Keywords

systemic lupus erythematosus children Parkinsonism intravenous immunoglobulin neurological complications

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References

1 Yancey CL , Doughty RA , Athreya BH . Central nervous system involvement in childhood systemic lupus erythematosus . Arthritis Rheum 1981; 24: 1389-1395 .

2 Steinlin MI , Blaster SI , Gilday DL et al. Neurologic manifestaitons of systemic lupus erythematosus . Pediatr Neurol 1995; 13: 191-197 .

3 Parikh S , Swaiman KF , Kim Y . Neurologic characteristics of childhood lupus erythematosus . Pediatr Neurol 1995; 13: 198-201 .

4 Shahar E , Goshen E , Tauber Z et al. Parkinsonian syndrome complicating systemic lupus erythematosus . Pediatr Neurol 1998; 18: 456-458 .

5 Ellis SG , Verity MA . Central nervous system involvement in systemic lupus erythematosus: a review of neuropathologic findings in 57 cases, 1955-1977 . Semin Arthritis Rheum 1979; 8: 212-221 .

6 West SG . Lupus and the nervous system . Curr Opin Rheumatol 1996; 8: 408-414 .

7 Jacobs L , Kinkel PR , Costello PB et al. Central nervous system lupus erythematosus: the value of magnetic resonance imaging . J Rheumatol 1988; 15: 601-606 .

8 Reiff A , Miller J , Shaham B et al. Childhood central nervous system lupus; longitudinal assessment using single photon emission computed tomograhy . J Rheumatol 1997; 24: 2461-2465 .

9 Sherer Y , Levy Y , Langevitz P et al. Successful treatment of systemic lupus erythematosus cerebritis with intravenous immunoglobulin . Clin Rheumatol 1999; 18: 170-173 .

10.

10 Klippel JH . Treatment. In: Schumacher HR (ed). Primer on the Rheumatic Disease. 10th edn. Arthritis Foundation: Atlanta , 1993, pp 112-116.