Case Report
ERA’S JOURNAL OF MEDICAL RESEARCH |
VOL.4 NO.1 |
DOI:10.24041/ejmr2017.7
Deeptanshu Agarwal, A.Q. Jilani, Priyadarshi Srivastava, S. B. Gupta, Anju Agarwal, Ajay Kohli
Department of Psychiatry
Era's Lucknow Medical College & Hospital Sarfarazganj, Lucknow, U.P.,
ABSTRACT
The
Received on :
Accepted on :
Address for correspondence
Dr. A.Q. Jilani
Assistant Professor,
Department of Psychiatry, Era's Lucknow Medical College
&Hospital,
Contact no. :
KEYWORD: MRKH syndrome, seizure, disorganised behaviour, Mental Retardation
INTRODUCTION
A 26 year old Unmarried female, presented with complaints of withdrawn behavior since past 6 years, and episodes of excessive fearfulness, suspiciousness, muttering to self and episodes of self harm e.g. biting herself and scratching her body parts since past 3 years. The frequency of such episodes would be
the presence of primary amenorrhea, normal development of secondary sexual characteristics including external genitalia, and ultrasonography finding of absence of the uterine structure between the bladder and rectum. Her CT scan head and EEG came out to be within normal limits. Psychological assessment revealed I.Q. level of 65. Negative history revealed absence of episodic tonic clonic movements with loss of consciousness, persistent sadness of mood, grandiose ideas/big plans etc. The patient provisionally diagnosed as Mayer Rokitansky Kuster Hauser Syndrome with boderline mental retardation with behavioral disturbance (?? Psychosis). The patient was put on Risperidone
DISCUSSION
MRKH syndrome affects reproductive system of females1. It is congenital abnormality characterized by the absence of vagina, cervix and the uterus. Due to incomplete development of the mullerian duct. Mullerian agenesis occurs in every 1 out of 4000- 10,000
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primary amenorrhea in adolescence age groups with normal physical growth and secondary sexual development. Affected individuals have a female chromosome pattern (46,XX) and normal functioning ovaries(4). Although women with this condition are usually unable to carry a pregnancy, they may be able to have children through assisted reproduction. This diagnosis of mullerian agenesis has been an incidental finding in our patient who actually presented with borderline intelligence with behavioral abnormalities. Often a specific type of physical abnormality or deficiency may have chromosomal or neuro- developmental etiology which itself may play as a common etiological factor for the psychological disorder as well as the physical deformity. Therefore, keeping in mind this possible dual association of a major physical/anatomical abnormality and psychological disorder in young adolescent girl, the above case has been presented here after taking valid consent from the family members of the patient. The psychiatric
Hence, on the basis of this case report, authors emphasize need of awareness among psychiatrist and the Gynaecologist about the MRKH and its premature psychiatric manifestations.
CONCLUSION
Hence, with this case report we want to urge that, both psychiatrist and the Gynaecologist should be aware
about the MRKH. A proper physical examination, personal history to be done of each psychiatric case. Both, psychiatrists and gynecologists need to be aware of this association of MRKH with psychological disturbance and should seek psychiatrist advice if needed. Proper psychoeducation of patient and the family members about the illness, so that they can cope up well.
REFERENCES
1.Bernardini L, Gimelli S, Gervasini C, Carella M, Baban A, Frontino G, Barbano G, Divizia MT, Fedele L, Novelli A, Bena F, Lalatta F, Miozzo M, Dallapiccola B: Recurrent microdeletion at 17q12 as a cause of
2.Cheroki C,
Brenner V, Kim CA, Otto PA, osenberg C: Genomic imbalances associated with mullerian aplasia. J Med Genet 2008,
3. Ledig S, Schippert C, Strick , Beckmann MW,
Oppelt PG, Wieacker P: ecurrent aberrations identified by
4.Duncan PA, Shapiro LR, Stangel JJ, Klein RM, Addonizio JC: The MURCS association: ullerian duct aplasia, renal aplasia, and cervicothoracic somite dysplasia. J Pediatr 1979,
5.Gupta M, Kharb V,
6.Bhad R, Chadda R, Kumar N, Goyal P. A rare association of schizophrenia and Mayer-
7.Kamal N., Bhanita B., Subrata N. A Rare Case of
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How to cite this article : Agarwal D., Jilani A.Q., Srivastava P., Gupta S.B., Agarwal A., Kohli A., Mayer
(MRKH) Syndrome WithBehavioural Disturbance And Mental Retardation - A Case Report. Era's Journal of Medical
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