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A rare association of schizophrenia and Mayer-Rokitansky-Küster-Hauser syndrome

Identifieur interne : 000080 ( Pmc/Curation ); précédent : 000079; suivant : 000081

A rare association of schizophrenia and Mayer-Rokitansky-Küster-Hauser syndrome

Auteurs : Roshan Bhad [Inde] ; Rakesh Chadda [Inde] ; Nand Kumar [Inde] ; Prashant Goyal

Source :

RBID : PMC:4623660
Url:
DOI: 10.4103/0019-5545.166636
PubMed: 26600595
PubMed Central: 4623660

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<pmc-dir>properties open_access</pmc-dir>
<front>
<journal-meta>
<journal-id journal-id-type="nlm-ta">Indian J Psychiatry</journal-id>
<journal-id journal-id-type="iso-abbrev">Indian J Psychiatry</journal-id>
<journal-id journal-id-type="publisher-id">IJPsy</journal-id>
<journal-title-group>
<journal-title>Indian Journal of Psychiatry</journal-title>
</journal-title-group>
<issn pub-type="ppub">0019-5545</issn>
<issn pub-type="epub">1998-3794</issn>
<publisher>
<publisher-name>Medknow Publications & Media Pvt Ltd</publisher-name>
<publisher-loc>India</publisher-loc>
</publisher>
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<article-id pub-id-type="pmid">26600595</article-id>
<article-id pub-id-type="pmc">4623660</article-id>
<article-id pub-id-type="publisher-id">IJPsy-57-324</article-id>
<article-id pub-id-type="doi">10.4103/0019-5545.166636</article-id>
<article-categories>
<subj-group subj-group-type="heading">
<subject>Letters to Editor</subject>
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<title-group>
<article-title>A rare association of schizophrenia and Mayer-Rokitansky-Küster-Hauser syndrome</article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname>Bhad</surname>
<given-names>Roshan</given-names>
</name>
<xref ref-type="aff" rid="aff1"></xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Chadda</surname>
<given-names>Rakesh</given-names>
</name>
<xref ref-type="aff" rid="aff1"></xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Kumar</surname>
<given-names>Nand</given-names>
</name>
<xref ref-type="aff" rid="aff1"></xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Goyal</surname>
<given-names>Prashant</given-names>
</name>
<xref ref-type="aff" rid="aff2">1</xref>
</contrib>
</contrib-group>
<aff id="aff1">Department of Psychiatry, All India Institute of Medical Sciences, New Delhi, India</aff>
<aff id="aff2">
<label>1</label>
Department of Psychiatry, Max Hospital, New Delhi, India. E-mail:
<email xlink:href="drroshansindia@gmail.com">drroshansindia@gmail.com</email>
</aff>
<pub-date pub-type="ppub">
<season>Jul-Sep</season>
<year>2015</year>
</pub-date>
<volume>57</volume>
<issue>3</issue>
<fpage>324</fpage>
<lpage>325</lpage>
<permissions>
<copyright-statement>Copyright: © Indian Journal of Psychiatry</copyright-statement>
<copyright-year>2015</copyright-year>
<license license-type="open-access" xlink:href="http://creativecommons.org/licenses/by-nc-sa/3.0">
<license-p>This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.</license-p>
</license>
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<body>
<p>Sir,</p>
<p>Schizophrenia is known to be associated with the various congenital disorders such as craniofacial anomalies, DiGeorge syndrome but its association with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome has rarely been reported.[
<xref rid="ref1" ref-type="bibr">1</xref>
<xref rid="ref2" ref-type="bibr">2</xref>
] MRKH syndrome is characterized by congenital aplasia of the uterus and the upper part. (2/3) of the vagina in women showing the normal development of secondary sexual characteristics and a normal 46, XX karyotype.[
<xref rid="ref3" ref-type="bibr">3</xref>
] To the best of our knowledge, till date, there has only been one published case report of the coexistence of these two disorders.[
<xref rid="ref4" ref-type="bibr">4</xref>
] We report another case of a 24-year-old unmarried woman Ms. A, with MRKH syndrome and schizophrenia for its interesting phenomenology and management. She presented with worries related to primary amenorrhea and infertility since adolescence, though detailed evaluation had not been done before. This was followed after 3 years by suspiciousness, the delusion of control and impulsive behavior. She believed that a certain unseen man staying abroad was controlling her mind and wants to marry her. She would hear voices of that man and converse with him. She would experience sensations in her vaginal area that she would attribute to sexual intercourse with that man, possible through some scientific technology devised by him. In addition, she would get distressed due to hearing voices of some other unknown persons, discussing about her and commanding her to do certain activities. Those unknown people would know her thoughts and control her actions through a transmitter device fitted in her ear. Ms. A would become irritable, aggressive, and violent toward family members whenever confronted regarding her beliefs. On psychiatric assessment, the psychopathology was confirmed. She had hallucinatory behavior, fearful affect, thought broadcast, the delusion of control, second and third person auditory hallucinations, a haptic hallucination of sexual content, impaired judgment with absent insight on mental status examination.</p>
<p>She received a diagnosis of schizophrenia, paranoid type with MRKH syndrome. Diagnosis of MRKH was established by the presence of primary amenorrhea, normal development of secondary sexual characteristics, external genitalia, and ultrasonography finding of absence of the uterine structure between the bladder and rectum. The patient was started on oral olanzapine 10 mg/day, which was later, changed to oral quetiapine up to 600 mg/day with a satisfactory response. She was counseled for the vaginal atresia and vaginoplasty was recommended just before marriage after the consultation with the gynecologist. Future treatment options such as
<italic>in vitro</italic>
fertilization and embryo transfer was also discussed with the patient and family members.</p>
<p>MRKH syndrome is associated with a number of neuropsychiatric manifestations such as distortions of body image, anxiety, depression, suicide, inter-personal sensitivity, and high psychological distress.[
<xref rid="ref3" ref-type="bibr">3</xref>
<xref rid="ref5" ref-type="bibr">5</xref>
<xref rid="ref6" ref-type="bibr">6</xref>
<xref rid="ref7" ref-type="bibr">7</xref>
] Recent genetic research shows the deletion 17q12 is a recurrent,. pathogenic copy number variants that confers a very high risk for schizophrenia,[
<xref rid="ref8" ref-type="bibr">8</xref>
] similarly 17q12 complex micro deletion is also found to be associated with the MRKH syndrome[
<xref rid="ref9" ref-type="bibr">9</xref>
<xref rid="ref10" ref-type="bibr">10</xref>
] suggestive of common genetic link between the two disorders. Both, psychiatrists and gynecologists need to be aware of this association and more research is required to test the link between these two disorders.</p>
<sec disp-level="2" id="sec2-1">
<title>Financial support and sponsorship</title>
<p>Nil.</p>
</sec>
<sec disp-level="2" id="sec2-2">
<title>Conflicts of interest</title>
<p>There are no conflicts of interest.</p>
</sec>
</body>
<back>
<ack>
<title>Acknowledgement</title>
<p>We acknowledge the help of the department of obstetrics and gynecology in helping us to manage the patient.</p>
</ack>
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