P57 Shapiro syndrome in prepubertal period

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Abstract

Introduction

Shapiro syndrome is an extremely rare disorder in the worldwide and it had observed only in 50/60 patients.Main symptoms are: paroxysmal sweating, sponton hypothermia. Agenesis of the corpus callosum is observed in some patients.But there are some patients they don’t have agenesis of the corpus callosum.

Introduction

It may starts in different age and the duration and frequency of the episodes are different.We want to present a topic about the patient who doesn’t have Agenesis of the corpus callosum during prepubertal period and she had good results with valproat treatment and excat regression after menstrual cycle.

Introduction

We related this syndrome with the chemical changes occurs in hypotalamus during this period.

Method

Patient: 10 years old girl

Method

Weight: 28 kg

Method

Height: 146

Method

Born and raised in a timely manner, parents are not realative.Patient never had infection diseases.She has got all vaccins.Appetite is normal. Intelligence is suitable for age, generalised sweating attacks is observed every day 8–10 times and lasts for 45 min. there is no connexion of sweating with physical activity.It happen sudden and temperature of body drops to 34.5.

Method

Patient examined.Full blood count, hormones: FSH, LH, TSH normal,biochemical analyses of blood, slow waves appeared during ECG. Epileptiform activity was not observed.There was no detectable pathology during MRT examination. sinus rhythm followed in the ECG.30 mq/kg depakin was prescribed to patient and the patient was controlled.After 3 weeks the number of sweating attacks were decreased and then were cut off (after 6 week)

Method

During 1 year the patient was taken the medicine and then menstrual cycle was started.

Method

Then we decided to stop taking the medicine.After medicine cut off the patient was under control for 2 years and no attacks were determined.

Method

Currently patient is 13 years old and healthy.The complete regression of the symptoms during cycle is related with the changes that happen during prepubertal period.

Conclusion

The changes happen during prepubertal period should be noted and must be under control.Sometime even if it takes a year but after puberty happen the complete regressions shows that we help to patient to use few medicine by controlling.

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