Figure 1
Deoxyglucose positron emission tomography shows bilaterally increased metabolic activity (putamen and caudate), more prominent on the left side.
Video 1.
Choreiform movements involving both upper and lower limb, predominantly on the right side.
Table 1
Reported Cases of Chorea with Increased Antiphospholipid Antibody and Normal Lupus Anticoagulant Titers in Absence of Antiphospholipid Antibody Syndrome
| Reference | N | Age (years) | Chorea Description | Other Symptoms | aPL Antibodies | MRI Findings | Treatment | Outcome |
|---|---|---|---|---|---|---|---|---|
| Kiechl-Kohlendorfer et al.29 | 1 | 5 | Isolated left-side hemichorea | No | LAC – | Normal | None | Chorea improved, aCL normalized |
| aCL IgM 28 | ||||||||
| aCL IgG 18 | ||||||||
| Usugi et al.30 | 1 | 6 | Chorea started at age 4 months. At age 3 years, continuous bilateral arrhythmic rapid purposeless jerks of the arms and legs were noted | Myoclonic seizures developed at age 2 years | aCL (IgG) aCL +(99) | Infarction in left MCA distribution at 3 months. Atrophy of basal ganglia, at age 3 years and additional atrophy of cerebellum at after 5 years | Methylprednisolone sodium succinate (10 mg/kg) for 3 days caused temporary improvement | Chorea and general condition gradually worsened |
| IgM –, LAC – | ||||||||
| IgG level was decreased to 17 at age 6 years | ||||||||
| Biernacka-Zielinska et al.31 | 1 | 16 | Left upper limb chorea | ? | Marked increase in aCL and aβ2GPI | Not reported | Acetylsalicylic acid (75 mg/day) and risperidone | Chorea disappeared after 9 months |
| LAC first absent but present a year later | ||||||||
| Shimomura et al.32 | 1 | 21 | Onset at age 17, in the left hand, progressed to both hands, mouth/tongue | Unsteady gait and wild gyrations of the arms on walking | LAC – | MRI and CT NL | Not reported | Not reported |
| aCL + | ||||||||
| Sundén-Cullberg et al.33 | 1 | 20 | Chorea started in right side of the body and face, later evolved into generalized chorea | Inability to eat and dress and dramatically impaired speech | LAC – | MRI NL | Pimozide and then after DC of pimozide, IV methylprednisolone | Deterioration after pimozide discontinued. Chorea ceased after IV methylprednisolone |
| aCL + (>100) | ||||||||
| ANA – | PET scan increased metabolism bilaterally more prominent in the lentiform nucleus and caudate and more on the left side. Repeat PET scan was normal after chorea subsided. | |||||||
| ANCA – | ||||||||
| Orzechowski et al.34 | 10 | N/A | Mild six, moderate two, severe two | One generalized ataxia + seizures | 10 patients hadLAC – and aCL + | MRI was normal in all patients | Four patients received tetrabenazine-carbidopa/levodopa,respiridone-fluphenazine/quitiapen | Partial response in seven and a complete response in three |
| Bilateral six | One epilepsy | |||||||
| Four patients: more than one episode | Two dysarthria + seizures | 71% of the non-APS patients had positive IgM aCL. | ||||||
| One dementia | IgM aCL in all patients with definite APS: all – | Three received warfarin/heparin and four treated with aspirin. Three had immunosuppression | ||||||
| Two dystonia |
[i] Abbreviations: aβ2GPI, anti-β2 glycoprotein-I antibody; aCL, Anticardiolipin; ANA, Antinuclear Antibody; ANCA, Antineutrophilic Cytoplasmic Antibodies; aPL, Antiphospholipid; Ig, Immunoglobulin; IV, Intravenous; LAC, Lupus Anticoagulant; MRI, Magnetic Resonance Imaging; NL, normal; PET, Positron Emission Tomography. DC, Discontinuation.