Table 2 Disease Summary by Case
From: Adolescent Sjogren’s syndrome presenting as psychosis: a case series
Case 1 | Case 2 | Case 3 | Case 4 | |
|---|---|---|---|---|
Age | 16 | 16 | 19 | 17 |
Gender | Female | Female | Female | Female |
Initial Presentation | Insomnia, polyphagia, polydipsia, SI, abnormal behavior | Anxiety, OCD, auditory hallucinations | Abnormal behavior, incoherent speech, insonia, HA, tangential thoughts | SI, paranoia, confusion, emotional lability, intermittent unresponsiveness, nocturnal enuresis |
MRI Brain Findings | Bilateral volume loss of the parietal lobes | L frontal lobe focus of white matter change | WNL | WNL |
CSF Findings | WNL, no pleiocytosis or increased oligoclonal bands | WNL, no pleiocytosis or increased oligoclonal bands | WNL, no pleiocytosis or increased oligoclonal bands | WNL, no pleiocytosis or increased oligoclonal bands |
EEG Findings | WNL | Occasional delta range slowing in the left fronto-central-temporal region | WNL | WNL |
Initial Immunosuppresive tx | Rituximab 1000 mg q 2 weeks × 2 and pulse methylprednisolone 1000 mg × 3 days followed by prednisone 60 mg tapered over 24 weeks | Rituximab 1000 mg × 1 | Methylprednisolone 30 mg/kg IV and rituximab 1000 mg q 2 weeks × 2 | Methylprednisolone (8 mg/kg/day IV) followed by oral prednisone (1.3 mg/kg/day) and IVIG 2 g/kg; then rituximab 1000 mg and pulse methylprednisolone; then IV plasmapharesis, rituximab 1000 mg and IV cyclophosphamide 750 mg/m2 |
Time to Improvement | 2–5 months | 1–5 months | 2 months | 1 week - 7 months |
Length of Follow Up Since Initial tx | 18 months | 12 months | 18 months then lost to follow-up | 7 months |
Total # rounds of anti-CD20 tx | 1 | 2.5 | 2 | 2 |
Maintenance Therapy | None | Obinutuzumab q 6 months | Rituximab 1000 mg q2 weeks × 2 every 6 months, mycophenolate mofetil 1500 mg bid | Rituximab 1000 mg q2 weeks × 2 every 6 months, hydroxychloroquine 200 mg daily and prednisone 0.65 mg/kg/day |
Currently Off Antipsychotics? | Yes | No | Yes | Yes |