Neuropsychiatric symptoms in a patient with Dyke–Davidoff–Masson syndrome and systemic lupus erythematosus: a case report

Abstract

Background Dyke–Davidoff–Masson syndrome is an uncommon constellation of radiological and clinical findings. Few reports describe co-occurring psychiatric manifestations. Systemic lupus erythematosus is a systemic disease with vascular, neurologic, and psychiatric involvement. To the best of our knowledge, no case reports have been made associating these entities. Case presentation We present the case of a 21-year-old Mexican mestizo woman with a history of systemic lupus erythematosus diagnosed at 4 years of age, who developed focal impaired awareness seizures when she was 8-years old, which became treatment-resistant at age 15. Two years prior to our evaluation, she developed deep vein thrombosis; clinical and laboratory criteria were met to diagnose secondary antiphospholipid syndrome. After being treated with anticonvulsants, glucocorticoids, and immunosuppressants with only a partial response, she developed a severe major depressive episode 1 year prior to our assessment, including two suicide attempts. She was referred to the out-patient clinic of our department for evaluation; intellectual disability, depressive symptoms, and behavioral symptoms were documented. Imaging studies revealed structural abnormalities in the left cerebral hemisphere: cortical atrophy, enlargement of sulci and cisternal spaces, and hyperpneumatization of the frontal sinus. Treatment with an antidepressant was initiated and maintained for 1 year, added to anticonvulsants and immunosuppressants. Depressive and behavioral symptoms diminished and no suicidal ideation has been noted at follow-up. Conclusions Dyke–Davidoff–Masson syndrome was diagnosed, accompanied by clinical symptoms previously reported as epilepsy and intellectual disability. This case report illustrates the complexity of syndrome presentation in an adult female, constituting a diagnostic and therapeutic challenge. This constellation of symptoms and structural brain abnormalities should be kept in mind in patients with neuropsychiatric manifestations and systemic diseases with central nervous system involvement, especially when diagnosed at a young age.

A Challenging Diagnosis of Systemic Lupus Erythematosus with Status Epilepticus

“…SLE could present with other autoimmune disorder as well, which could possibly complicate the treatment. The study conducted by Sordia-Ramírez et al showed the presence of neuropsychiatric symptoms in a patient with Dyke-Davidoff-Masson syndrome [14]. The seizures in this patient presented due to cortical hypotrophy in the left medial temporal lobe.…”

Clinical characteristics and neuroimaging findings of seven patients with Dyke Davidoff Masson syndrome

“…The characterization of neuropsychiatric manifestations of DDMS was one of the main contributions of this study. Very few DDMS cases complicated by psychiatric manifestations in DDMS were reported [ 7 – 12 ]. The involvement psychiatric disorders including schizophrenia, schizoaffective disorder, bipolar I mania, depressive episode and emotional dysregulation have been reported in patients with DDMS [ 7 – 12 ].…”

“…Very few DDMS cases complicated by psychiatric manifestations in DDMS were reported [ 7 – 12 ]. The involvement psychiatric disorders including schizophrenia, schizoaffective disorder, bipolar I mania, depressive episode and emotional dysregulation have been reported in patients with DDMS [ 7 – 12 ]. Intellectual disability, depressive symptoms, suicidal ideation and behavioral symptoms were documented in a 21-year-old woman with a history of systemic lupus erythematosus [ 7 ].…”

“…The involvement psychiatric disorders including schizophrenia, schizoaffective disorder, bipolar I mania, depressive episode and emotional dysregulation have been reported in patients with DDMS [ 7 – 12 ]. Intellectual disability, depressive symptoms, suicidal ideation and behavioral symptoms were documented in a 21-year-old woman with a history of systemic lupus erythematosus [ 7 ]. Although the underlying pathophysiological mechanism had not yet been well clarified, our results added some positive evidence for the association between psychiatric disorder and DDMS [ 7 – 12 ].…”

“…Few DDMS patients with contralaterally crossed cerebellar atrophy have been reported [ 6 ]. However, cases on the correlation between DDMS and neuropsychiatric symptoms is rarely reported [ 7 – 12 ]. To the best of our knowledge, none of the cases till date were presented with ear malformations.…”