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Description
An 18-year-old man presented with a 2-day history of intermittent involuntary multifocal movements.
Video 1 in segment 1 shows myoclonic type movements of the right upper limb, facial muscles and paroxysms of irregular breathing. In Segment 2, choreoathetoid movements of the limbs and brief dystonia of the feet were recorded 3 min later. Segment 3 recorded 9 months later shows no involuntary movements.
Segments 1, 2 and 3 showing focal myoclonic jerking, irregular chest wall movements, choreoathetosis in hands and feet, dystonia of feet and normal state after treatment.
The patient's haemoblobin was 6.2 g/dL, white cell count 2.8×103/µL, platelet count 162×103/µL and blood film was normal. Liver and renal functions, treponemal serology, retroviral tests and quantiferon gold were normal. Urinalysis showed proteinuria ++, erythrocyte sedimentation rate was 125 mm/h and C3 and C4 were low. Antinuclear antibody and double-stranded DNA were strongly positive, and extractable nuclear antigen revealed histones and ribosomes++. β2-GP1 IgG and IgM, anticardiolipin IgG, A and M and lupus anticoagulant were markedly elevated at presentation, and 3 months later.
Cerebrospinal fluid protein was elevated and MRI showed cortical and subcortical hyperintensities (figure 1). EEG was normal.
Axial T1 MRI view showing left-sided cortical and bilateral subcortical hyperintensities.
Systemic lupus erythematosus (SLE) with antiphospholipid syndrome (APS) was diagnosed.
Methyl prednisolone 1 g was administered intravenous daily for 3 days, followed by oral prednisolone 30 mg twice daily which caused disappearance of the movements in 1 week. After a tapering dose of steroids over 2 months, mycophenolate mofetil was started in preference to cyclophosphamide following discussions with the patient and his family. Aspirin was also administered as recommended by guidelines.1 Nine months later the patient remains well.
Movement disorders in SLE and APS are rare. Vascular and non-vascular immune-mediated pathogenic mechanisms have been postulated.2 ,3
Learning points
Movement disorders can be caused by systemic lupus erythematosus or antiphospholipid syndrome singly or together.
These include chorea, athethosis, dystonia, myoclonic jerks as shown, but may also include hemiballismus, parkinsonism and tics.
Treatment with immunosuppressants can be effective.
Acknowledgments
The authors thank Drs Shane Karim, Amrit Ramesar, Maria Gosein and Anthony Ameeral for assistance in preparation of this report. The authors thank the patient for allowing his data to be used for this publication to enhance medical knowledge.
Footnotes
Competing interests None.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.