Dissociative disorder in Mayer Rokitansky Küster Hauser syndrome with pulmonary agenesis: a case report

Abstract Background The Mayer Rokitansky Küster Hauser (MRKH) syndrome is a rare congenital disorder characterized by the absence of uterus and vagina in a patient who is phenotypically a female, with 46 XX karyotypes. It affects 1 in 4000 to 5000 female new-borns. Pulmonary agenesis is a rare assoc...

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Main Authors: Sulochana Joshi, Anup Raj Bhandari, Prashant Shrestha, Rabi Shakya
Format: Article
Language:English
Published: SpringerOpen 2022-01-01
Series:The Egyptian Journal of Neurology, Psychiatry and Neurosurgery
Subjects:
Online Access:https://doi.org/10.1186/s41983-021-00440-y
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author Sulochana Joshi
Anup Raj Bhandari
Prashant Shrestha
Rabi Shakya
author_facet Sulochana Joshi
Anup Raj Bhandari
Prashant Shrestha
Rabi Shakya
author_sort Sulochana Joshi
collection DOAJ
description Abstract Background The Mayer Rokitansky Küster Hauser (MRKH) syndrome is a rare congenital disorder characterized by the absence of uterus and vagina in a patient who is phenotypically a female, with 46 XX karyotypes. It affects 1 in 4000 to 5000 female new-borns. Pulmonary agenesis is a rare association in this MRKH syndrome. Females with MRKH face various mental health issues and psychological disturbances, including dissociative disorder which is a stress-related psychiatric disorder. Dissociative disorder in MRKH syndrome is under-recognized and under-treated. Case presentation A 23-year-old unmarried woman presented to the emergency forabnormal behaviour attack. Recurrent episodes of dissociative convulsions were present most of the time with MRKH syndrome as the pertinent stressor. Both antidepressants and psychotherapy helped to decrease the frequency of dissociative convulsions and come interms with the syndrome. Conclusion This case describes dissociative disorder as the presentation and comorbid condition of MRKH syndrome and the impact of MRKH syndrome on the patient. We attempt to explain the occurrence of dissociative disorder in MRKH syndrome and the results of under-recognition and under-treatment of the same. We aim to highlight the presence of commonly treatable conditions associated with a rare syndrome and its effect when untreated and unrecognized.
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spelling doaj.art-fbca5fceea01475e88064e60319af1ec2022-12-22T04:04:03ZengSpringerOpenThe Egyptian Journal of Neurology, Psychiatry and Neurosurgery1687-83292022-01-015811410.1186/s41983-021-00440-yDissociative disorder in Mayer Rokitansky Küster Hauser syndrome with pulmonary agenesis: a case reportSulochana Joshi0Anup Raj Bhandari1Prashant Shrestha2Rabi Shakya3Department of Psychiatry, Patan Academy of Health SciencesDepartment of Psychiatry, Patan Academy of Health SciencesDepartment of Gynaecology and Obstetrics, Patan Academy of Health SciencesDepartment of Psychiatry, Patan Academy of Health SciencesAbstract Background The Mayer Rokitansky Küster Hauser (MRKH) syndrome is a rare congenital disorder characterized by the absence of uterus and vagina in a patient who is phenotypically a female, with 46 XX karyotypes. It affects 1 in 4000 to 5000 female new-borns. Pulmonary agenesis is a rare association in this MRKH syndrome. Females with MRKH face various mental health issues and psychological disturbances, including dissociative disorder which is a stress-related psychiatric disorder. Dissociative disorder in MRKH syndrome is under-recognized and under-treated. Case presentation A 23-year-old unmarried woman presented to the emergency forabnormal behaviour attack. Recurrent episodes of dissociative convulsions were present most of the time with MRKH syndrome as the pertinent stressor. Both antidepressants and psychotherapy helped to decrease the frequency of dissociative convulsions and come interms with the syndrome. Conclusion This case describes dissociative disorder as the presentation and comorbid condition of MRKH syndrome and the impact of MRKH syndrome on the patient. We attempt to explain the occurrence of dissociative disorder in MRKH syndrome and the results of under-recognition and under-treatment of the same. We aim to highlight the presence of commonly treatable conditions associated with a rare syndrome and its effect when untreated and unrecognized.https://doi.org/10.1186/s41983-021-00440-yDissociative disorderMayer Rokitansky Küster Hauser syndromePsychological disturbanceTreatmentCase report
spellingShingle Sulochana Joshi
Anup Raj Bhandari
Prashant Shrestha
Rabi Shakya
Dissociative disorder in Mayer Rokitansky Küster Hauser syndrome with pulmonary agenesis: a case report
The Egyptian Journal of Neurology, Psychiatry and Neurosurgery
Dissociative disorder
Mayer Rokitansky Küster Hauser syndrome
Psychological disturbance
Treatment
Case report
title Dissociative disorder in Mayer Rokitansky Küster Hauser syndrome with pulmonary agenesis: a case report
title_full Dissociative disorder in Mayer Rokitansky Küster Hauser syndrome with pulmonary agenesis: a case report
title_fullStr Dissociative disorder in Mayer Rokitansky Küster Hauser syndrome with pulmonary agenesis: a case report
title_full_unstemmed Dissociative disorder in Mayer Rokitansky Küster Hauser syndrome with pulmonary agenesis: a case report
title_short Dissociative disorder in Mayer Rokitansky Küster Hauser syndrome with pulmonary agenesis: a case report
title_sort dissociative disorder in mayer rokitansky kuster hauser syndrome with pulmonary agenesis a case report
topic Dissociative disorder
Mayer Rokitansky Küster Hauser syndrome
Psychological disturbance
Treatment
Case report
url https://doi.org/10.1186/s41983-021-00440-y
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AT anuprajbhandari dissociativedisorderinmayerrokitanskykusterhausersyndromewithpulmonaryagenesisacasereport
AT prashantshrestha dissociativedisorderinmayerrokitanskykusterhausersyndromewithpulmonaryagenesisacasereport
AT rabishakya dissociativedisorderinmayerrokitanskykusterhausersyndromewithpulmonaryagenesisacasereport