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

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.

Dissociation in SLE: A part of lupus fog?

Introduction Lupus fog is ill-defined. We aimed to study whether lupus fog is the result of dissociation by studying the prevalence of dissociation and dissociative fog in patients with SLE and neuropsychiatric manifestations of inflammatory and non-inflammatory origin. Methods Patients visiting the tertiary referral center for neuropsychiatric systemic lupus erythematosus (NPSLE) of the LUMC between 2007–2019 were included. Patients were classified as having neuropsychiatric symptoms of inflammatory or non-inflammatory origin. Dissociation was studied using the Dissociative Experience Scale-II (DES), in which the presence of 28 dissociative symptoms is rated (0–100% of the time), of which one question assesses the presence of a dissociative fog directly. Average scores are calculated and scores ≥ 25 are considered indicative of a dissociative disorder. A score of ≥ 30 on question 28 (dissociative fog) was considered indicative for the presence of a fog. Summary scores in the general adult population range from 4.4 to 14. Multiple regression analysis (MRA) was performed to study the association between inflammatory neuropsychiatric symptoms and dissociation. DES results are presented as median (range) and MRA as B and 95% confidence interval (CI). Results DES questionnaires were available for 337 patients, of which 69 had an inflammatory NPSLE phenotype (20%). Mean age in the total study population was 43 ± 14 years and the majority was female (87%). The median dissociation score was 7.1 (0–75) and did not differ between patients with neuropsychiatric symptoms of inflammatory or non-inflammatory origin (B: −0.04 (95% CI: −0.17; 0.09)). 35 patients (10%) had a score indicative of a dissociative disorder. The most common type of dissociation was absorption/imagination. 43 patients (13%) reported a dissociative fog. Discussion In most patients with SLE and neuropsychiatric symptoms, dissociative symptoms are within normal range, regardless of underlying etiology. Dissociative fog is present, but uncommon. Lupus fog is most likely not associated with dissociation.

A Schema Therapy Approach to Complex Dissociative Disorder in a Cross-Cultural Setting: A Single Case Study

Complex dissociative disorders (CDD) include dissociative identity disorder (DID) and the most common other specified dissociative disorder (OSDD, type 1). One of the strongest predictors of CDD is antecedent trauma, particularly early childhood trauma. Currently, consensus-based treatment guidelines for CDD are lacking. Schema Therapy (ST) is a psychotherapeutic approach that has been recently proposed as a treatment for CDD given its emphasis on the consequences of early childhood neglect and abuse, and the explanation within the therapeutic model of the patient’s experience of drastic shifts between personality states. The present case study examined the process of individual ST, in a three phases-based approach, with a 38-year-old male Arabic-speaking refugee with OSDD, type 1 (i.e., chronic dissociative disorder with mixed symptoms), posttraumatic stress disorder (PTSD) and borderline personality disorder (BPD). The setting included two therapists (co-therapy) and an interpreter/cultural mediator. We assessed the patient’s change by using self-report assessments of dissociative and PTSD symptoms, cognitive schemas over 2-year and 4-months treatment periods and a 6-months follow-up. We also assessed the therapeutic alliance. Posttreatment and follow-up reliable change analyses showed significant improvements in dissociative and PTSD symptoms as well as in some cognitive schemas. The therapeutic alliance between the patient and the therapists grew in a progressive and balanced way. This case study shows that ST within a phase-oriented approach may be an effective treatment for CDD. Our study also suggests cross-cultural validity of ST and, particularly, of modes construct. More research based on large samples is needed to confirm these assumptions.