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Table 2 Disease Summary by Case

From: Adolescent Sjogren’s syndrome presenting as psychosis: a case series

 Case 1Case 2Case 3Case 4
Age16161917
GenderFemaleFemaleFemaleFemale
Initial PresentationInsomnia, polyphagia, polydipsia, SI, abnormal behaviorAnxiety, OCD, auditory hallucinationsAbnormal behavior, incoherent speech, insonia, HA, tangential thoughtsSI, paranoia, confusion, emotional lability, intermittent unresponsiveness, nocturnal enuresis
MRI Brain FindingsBilateral volume loss of the parietal lobesL frontal lobe focus of white matter changeWNLWNL
CSF FindingsWNL, no pleiocytosis or increased oligoclonal bandsWNL, no pleiocytosis or increased oligoclonal bandsWNL, no pleiocytosis or increased oligoclonal bandsWNL, no pleiocytosis or increased oligoclonal bands
EEG FindingsWNLOccasional delta range slowing in the left fronto-central-temporal regionWNLWNL
Initial Immunosuppresive txRituximab 1000 mg q 2 weeks × 2 and pulse methylprednisolone 1000 mg × 3 days followed by prednisone 60 mg tapered over 24 weeksRituximab 1000 mg × 1Methylprednisolone 30 mg/kg IV and rituximab 1000 mg q 2 weeks × 2Methylprednisolone (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 Improvement2–5 months1–5 months2 months1 week - 7 months
Length of Follow Up Since Initial tx18 months12 months18 months then lost to follow-up7 months
Total # rounds of anti-CD20 tx12.522
Maintenance TherapyNoneObinutuzumab q 6 monthsRituximab 1000 mg q2 weeks ×  2 every 6 months, mycophenolate mofetil 1500 mg bidRituximab 1000 mg q2 weeks ×  2 every 6 months, hydroxychloroquine 200 mg daily and prednisone 0.65 mg/kg/day
Currently Off Antipsychotics?YesNoYesYes
  1. Key:
  2. OCD Obsessive compulsive disorder, WNL Within normal limits, Tx Treatment, SI Suicidal Ideation, HA Headache