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Introduction:Tuberous Sclerosis Complex (TSC) is a genetic inherited disease characterized by hamartomatous growths in several organs as brain, skin, kidneys, hearth and eyes. The estimated incidence is approximately 1:6000 live births. The diagnosis is made clinically. Seizures are present in 87% of patients. Psychiatric comorbidity has been reported.Objectives:We report the clinical course of two patients with previous diagnosis of TSC. Psychiatric symptoms start in the adulthood without seizures history and absence of Subependimal Giant Cells Tumor (SGCT). The evolution and clinical features are described.Methods:Patient 1Married 33-years-old woman with two children affected with TSC. She was diagnosed after headache presentation in 2011. Initial MRI showed periventricular glioneuronal hamartomas. In January 2013 start with self-injurious (swallowing of objects) and autistic behaviours as well as several hospital urgency room visits. In addition, the patient presented with dull mood, emotional indifference and intellectual impairment, with no response to medication.Patient 2Married 43-years-old woman with a daughter affected with TSC. Diagnosis was made in 1999 and psychotic symptoms (delusional beliefs and auditory hallucinations) started in 2011 without previous psychiatric history. The MRI in 2013 shown subependymal nodules. Treatment with risperidone was effective.Results:Psychiatric symptoms are very often associated to the physical findings on TSC, even in adulthood diagnoses.Conclusions:Psychiatric comorbidities are well described in literature. about 10-20% adult patients with TSC present clinically significant behavioral problems as self-injuries, frequently associated with SGCT. The European Expert Panel recommended regular assessment of cognitive development and behaviour and symptomatic treatment.

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Introduction:S100B is a calcium-binding protein produced by the astrocytes that has been used as a biomarker of brain inflammation. S100B has been involved in the schizophrenia pathophysiology, being considered a marker of state and prognosis.Objectives:Studying the relationship between serum S100B levels and psychopathology in first-episode psychosis (FEP).Methods:At admission and discharge, serum S100B levels were measured in 20 never-medicated FEP in-patients and 20 healthy controls. Psychopathology was assessed with the PANSS (Positive and Negative Syndrome Scale). The total, positive, negative and general psychopathology scores were assessed. Results are presented as mean±sd. and S100B levels in pg./ml.Results:At admission, patients had significantly higher serum S100B concentrations than healthy subjects (39.2±6.4 vs. 33.3±0.98, p<0.02). S100B levels increased from admission to discharge (39.2±6.4 vs. 40.0±6.8, p=0.285) but they do not reach statistical significance. There were no correlations between PANSS (total, positive, negative and general) scores and S100B at admission and discharge. Individual item by item PANSS correlations with S100B elicited a positive correlation with P5 (grandiosity) (r=0.486, p=0.030) and G5 (mannerisms/posturing) (r=0.514; p=0.02) at discharge. There also was a positive trend with G7 (motor retardation) (r=0.409; p=0.073) at discharge.Conclusions:FEP in-patients have significantly increased serum levels of S100B proteins, suggesting an activation of glial cells that may be associated with a neurodegenerative/inflammatory process. Apart from the study of total scale scores, the analysis of individual item is also recommended. The long-term treatment effect (one year or more) may be relevant to see their relationship to S100B levels.