Tehran University Medical Journal

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Background: Creutzfeldt-Jakob disease (C-JD) is a rare disorder characterized with rapidly progressive mental decline, myoclonic jerk and finally death. The transmissible pathogen for this disease is a proteinaceous infectious particle termed prion. The prion protein is encoded by a gene (designated as PRNP) on the short arm chromosome 20.This disorder is diagnosed based on clinical findings, course of disease, EEG, MRI and confirmed with brain biopsy. Case report: A 56- year- old woman presented with confusion, disorientation, hyper somnolence, psychiatric problems such as hallucination, progressive mental deterioration and myoclonic jerks. She had history of several times phlebotomy with traditional and non sterile methods in two past years. She had no past history of other disease. Her illness was diagnosed based on clinical findings, course of her illness, typical MRI, EEG and rule out other dementing disease. She died after one month.
Conclusion: in any patients with psychiatric disorders, rapidly progressive mental deterioration and myoclonic jerks C-JD should be considered as an important diagnosis. Treatable dementing disease should be considered and ruled out at first. The significance of phlebotomy in C-JD has yet to be determined.

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Background: Recent studies have raised the issue of an increased incidence of polycystic ovaries (PCO) and menstrual disturbances in women with epilepsy treated with valproate (VPA). It seems that antiepileptic drugs, especially valproate, may have a functional role in altering the endocrine system of child-bearing women with epilepsy. We conducted this study to investigate the association of VPA and ovarian structural/menstrual disorders in epileptic women.

Methods: In this cross-sectional study, we compared a total of 64 epileptic patients, aged 16-45 years, 32 of whom had been taking VPA alone and 32 were on other antiepileptic drugs for a minimum duration of six months. Ovarian sonography was performed and body mass index (BMI) calculated for all subjects. We also recorded the presence of menstrual disturbances in both groups.

Results: Fifteen (46%) of the VPA subjects had PCO compared to 7 (21.9%) of the other group. In the VPA group, four (12.5%) had oligomenorrhea, one (3.1%) amenorrhea and 13 (40.6%) had irregular menstrual cycles. However, from the other group, two (6.3%) subjects had oligomenorrhea and seven (21.9%) had irregular menstrual cycles amenorrhea was not present in the non-VPA treated patients. Mean BMI was 22.5 kg/m² among the VPA subjects and 20.1 kg/m² in the non-VPA subjects.

Conclusions: This study supports the association of PCO and high BMI with VPA treatment. The frequency of menstrual disturbances did not differ significantly between the two groups.