Psychotic Disorders in Behçet’s Disease: Unraveling the Intricacies of Neuropsychiatric Manifestations: A Case Report

This case report describes a 33-year-old patient with Behçet’s disease who presented with chronic delusions and hostility. Despite receiving immunosuppressive treatment for Behçet’s disease, the patient exhibited unsystematized delusions, disorganized speech, and intense psychic dissociation. Neurological examination and MRI showed no abnormalities, suggesting a psychiatric etiology. Treatment with risperidone and lorazepam led to partial improvement in psychiatric symptoms. The case raises the possibility of schizophrenia co-occurring with Behçet’s disease, although psychosis is rare in this condition. Further research is needed to understand the relationship between psychiatric and neurological manifestations of Behçet’s disease.

Introduction

Behçet’s disease is a chronic, multisystem inflammatory disorder characterized by recurrent oral and genital ulcers, skin lesions, and inflammation of the eyes. It can also affect various other organs, including the central nervous system. The disease is believed to involve dysregulation of the immune system, although its exact cause remains unclear.

Behçet’s disease is most prevalent in regions along the ancient Silk Road, including the Middle East, Mediterranean, and East Asia, but it can occur worldwide. The prevalence of neurological manifestations in Behçet’s disease varies among different studies, ranging from 5.3% to 35%, possibly due to recruitment biases related to geographic regions and study types (retrospective vs. prospective). About half of these patients exhibit personality changes, and 5% to 10% develop confirmed psychiatric disorders. The psychiatric manifestations of Behçet’s disease are relatively nonspecific, primarily characterized by depression, although some studies report manic and delusional symptoms.

Clinical Case

We present the case of a young patient who has been diagnosed with Behçet’s disease for six years and presented with chronic delusions on various themes and hostility towards his mother. Given this clinical presentation, we consider two hypotheses: the co-occurrence of schizophrenia and Behçet’s disease versus psychotic disorders induced by systemic illness.

Mr. X, a 33-year-old, only child with a low socioeconomic status, has been managed for Behçet’s disease for six years, receiving immunosuppressive treatment (IMUREL 50 mg/day). He was admitted to a psychiatric hospital due to agitation and hostility persisting for four weeks. On admission, the patient exhibited unsystematized delusions characterized by hallucinatory, interpretive, imaginative, and intuitive mechanisms. The delusions included persecutory and megalomaniacal themes, along with homicidal thoughts towards his mother. He showed intense psychic dissociation, disorganized speech, tangential thinking, and neologisms, hindering communication. Neurological examination revealed no abnormalities. EEG was normal, and blood tests, including complete blood count, electrolytes, glucose, liver and kidney function, and thyroid function, were all within normal limits.

Brain MRI showed reduced blood flow in the left lateral sinus (hyperintensity in T1 and T2) and decreased flow in the superior longitudinal sinus, with no associated parenchymal abnormalities. Ten days after initiating risperidone (4 mg/day) and lorazepam (5 mg/day), incoherence and aggressiveness subsided, and the patient’s contact improved. He was transferred to neurology for further management of Behçet’s disease.

After six months of treatment, psychiatric symptoms showed partial improvement. Antipsychotic therapy alleviated psychomotor excitement, resolved speech disturbances, and reduced the intensity of delusional thinking, although some negative symptoms persisted, including emotional indifference, social withdrawal, and pragmatism. He had no insight.

In light of this case, we consider two hypotheses: schizophrenia associated with Behçet’s disease versus psychotic disorders induced by systemic illness.

Discussion

The psychiatric symptoms exhibited by Mr. X raise the possibility of schizophrenia co-occurring with Behçet’s disease. Notably, this patient had previously experienced a similar episode two years prior to hospitalization, suggesting a recurrent pattern. The course of the current episode further supports this hypothesis, with florid symptoms persisting for several months before transitioning to negative symptoms. This trajectory is consistent with the presentation described by Nakam et al., who reported an acute psychotic disorder associated with Behçet’s disease [1], Another similar case recently described as a case of Behçet’s disease complicated by schizophrenia-like symptoms [2].

However, it is important to acknowledge that psychosis is a rare manifestation of Behçet’s disease, with only isolated cases reported in the literature. For instance, one case involved brain parenchymal lesions [3], while another marked the onset of Behçet’s disease without neurological involvement [4].

Although specific prospective studies are underway to evaluate the prevalence of psychiatric disorders in Behçet’s disease Specific prospective studies are attempting to evaluate the overall neurological and psychiatric complications of Behçet’s disease, reporting 21% of psychiatric disorders in France [5], 5% of acute psychotic episodes in Iraq [6], and 2% in Turkey [7], The rarity of psychosis in Behçet’s disease raises the possibility of a coincidental association, highlighting the primary limitation of isolated case reports.

Moreover, the effectiveness of antipsychotic treatment in alleviating Mr. X’s symptoms suggests a potential benefit of addressing the psychiatric manifestations of Behçet’s disease. However, the partial improvement observed after six months of treatment underscores the complexity of managing psychiatric symptoms in this context. Negative symptoms such as emotional indifference and social withdrawal persist despite pharmacological intervention, emphasizing the need for comprehensive treatment approaches that address both psychiatric and neurological aspects of Behçet’s disease.

Conclusion

While this case suggests a potential association between schizophrenia and Behçet’s disease, further research involving larger cohorts is necessary to confirm this observation. Comprehensive studies are needed to accurately define the psychiatric manifestations of Behçet’s disease and elucidate their relationship with neurological symptoms. Such investigations will not only enhance our understanding of the disease but also inform the development of tailored treatment strategies to address the diverse manifestations of Behçet’s disease.