Catatonia and Cotard's Syndrome in a Patient With Systemic Lupus Erythematosus: A Case Report

Abstract

Introduction

Systemic lupus erythematosus (SLE) is an autoimmune disease with multiorgan involvement, being the development of neuropsychiatric (NP) symptoms variable, in which the presentation of catatonia and Cotard syndrome (CoS) is rare.

Case presentation

We report the case of a 29-year-old woman who was diagnosed with SLE in 2018 based on the presence of articular, serosal and, hematological manifestations and immunological abnormalities. During her hospitalization, inappropriate behaviors including repetitive and incoherent speech, visual and olfactory hallucinations developed, so NP involvement was considered, and quetiapine and methylprednisolone pulses were administered with good response. The patient was discharged to the care of her family, being stuporous, bradypsychic, with thought blockages and nihilistic delusions; in consequence, she was diagnosed with Cotard syndrome (CoS). Moreover, she presented symptoms of catatonia including mutism, catalepsy and rigidity. Treatment consisted of diazepam, aripiprazole 30 mg/day, fluoxetine 20 mg/day and prednisone 60 mg/day.

Conclusion

Catatonia can be a manifestation of NPSLE in active SLE. Catatonic symptoms and CoS in NPSLE have never been described. This is the first report of a SLE patient exhibiting CoS and catatonia.

Resumen

Introducción

El lupus eritematoso sistémico (LES) es una enfermedad autoinmune con afectación multiorgánica. Es variable el desarrollo de síntomas neuropsiquiátricos (NP), en los que es rara la presentación de catatonía y síndrome de Cotard (SdC).

Presentación del caso

Reportamos el caso de una mujer de 29 años que fue diagnosticada de LES en 2018 con base en la presencia de manifestaciones articulares, serosas, hematológicas y anormalidades inmunológicas. Durante su hospitalización, se desarrollaron conductas inapropiadas, que incluían habla repetitiva e incoherente, alucinaciones visuales y olfativas, por lo que se consideró compromiso NP y se administraron pulsos de quetiapina y metilprednisolona, con buena respuesta. La paciente fue dada de alta al cuidado de su familia, encontrándose estuporosa, bradipsíquica, con bloqueos de pensamiento y delirios nihilistas; en consecuencia, fue diagnosticada de síndrome de Cotard (SdC). Además, presentaba síntomas de catatonía que incluían mutismo, catalepsia y rigidez. El tratamiento consistió en diazepam, aripiprazol (30 mg/día), fluoxetina (20 mg/día) y prednisona (60 mg/día).

Conclusiones

La catatonía puede ser una manifestación de NP en el LES activo. Nunca se han descrito síntomas catatónicos y de SdC en el LES con compromiso NP. Este es el primer informe de una paciente con LES que presenta CdS y catatonía.

Introduction

Systemic lupus erythematosus (SLE) is a chronic disease in which the immune system reacts against host antigens, resulting in multi organ/system involvement. The symptoms are often insidious in onset and non-specific in nature, making its diagnosis difficult.¹ SLE is known to cause neuropsychiatric (NP) symptoms, although their prevalence is highly variable (12–95%).²

Catatonia is a cluster of motor features. The classic signs are mutism, a rigid posture, fixed staring, stereotypic movements and stupor.³ It is a transnosographic syndrome presenting along psychiatric and somatic disorders; it has been described in epilepsy, dementia, porphyria, encephalopathies, and others.⁴ However, catatonia is an infrequent manifestation of SLE.⁵

Cotard's syndrome (CoS) is a rare psychiatric condition, initially described by Jules Cotard in 1880.⁶ CoS was classified for the first time by Berrios and Luque⁷ in 1995; they described three CoS types, important for therapeutic decisions. The first form denominated “psychotic depression factor” consists of anxiety, melancholia, delusions of guilt, and auditory hallucinations as its most prominent features. The second form, called CoS type I is associated with hypochondriac and nihilistic delusions and absence of a depressive episode. The third form, or CoS type II, is characterized by anxiety, depression, auditory hallucinations, delusions of immortality, nihilistic delusions and suicidal behavior.

A negation or nihilist delusion in which patients deny the existence of some parts of their bodies, or even of their totality, getting to finally deny the existence of the world surrounding them, is considered as a synonym of Cotard's syndrome.⁸ Catatonic symptoms are unusual in patients with CoS as well as in those with SLE.⁹ We report for the first time a SLE patient who at presentation exhibited both, CoS and catatonia.

Case report

A previously healthy 29-year-old woman with no significant past medical history presented in April 2018 for evaluation of articular (symmetrical polyarthritis), serosal (pericardial effusion), hematological (lymphopenia), and immunological (low C4, positive ANA 1:10,000 – homogeneous pattern by indirect immunofluorescence technique and anti-dsDNA) manifestations. Antiphospholipid antibodies were negative. She had an important family history: her father had type 2 diabetes and schizophrenia, her brother had an undefined intellectual disability and pulmonary tuberculosis, and a first-degree cousin had SLE.

The patient was admitted to the hospital and diagnosed with SLE based on her clinical presentation. During her hospitalization, inappropriate behaviors including repetitive and incoherent speech, visual and olfactory hallucinations ensued, so NP involvement was considered. A brain computerized tomography scan showed no significant abnormalities. A lumbar puncture revealed a clear cerebrospinal fluid (CSF) with a glucose of 50 mg/dl, protein: 13.59 mg/dl, white blood cell count: 2 cells/mm³, all mononuclear; Indian ink, Ziehl–Neelsen and Gram stains were all negative. Administrative issues prevented performing a brain magnetic resonance imaging (MRI) at that time. The patient was evaluated by psychiatry was diagnosed with a psychotic disorder and quetiapine 25 mg/day and prednisone 1 mg/kg/day were started, this one was indicated by rheumatology. However, the patient persisted with psychotic symptoms so after medical board, the rheumatologists prescribed methylprednisolone pulses 1 g/day (April 25th–27th) and a cyclophosphamide pulse (1 g) on April 28th were administered with the patient exhibiting a good response. Also, hydroxychloroquine 200 mg/day was added. The patient was discharged to the care of her family; after two days, her husband reported his wife's abrupt onset of incoherent speech, repetitive phrases, insomnia and visual and auditory hallucinations. She stated that “her body was already dead”. For this reason, she was hospitalized again.

The patient was stuporous, bradypsychic, with thought blockages and nihilistic delusions (“my body is dead, I no longer exist, I am dead”). In view of the delusions of denial and the absence of depressive episodes, she was diagnosed with CoS type I. Moreover, she presented mutism, catalepsy and rigidity, so she was diagnosed with catatonia. The Bush-Francis Catatonia Rating Scale (BFCRS) was scored at 21. The Clinical Global Impressions (CGI) Scale was scored at 4. Treatment consisted of diazepam 30 mg/day, aripiprazole 30 mg/day, fluoxetine 20 mg/day and prednisone 60 mg/day. The patient's hospitalization lasted 27 days; gradual improvement of her symptoms occurred: delusions of denial disappeared after one week of treatment; catatonic and psychotic symptoms remitted after two weeks of treatment.

Discussion

SLE has been described as the “thousand faces disease”.¹⁰ The heterogeneity of SLE clinical presentation makes its diagnosis difficult. NP symptoms of SLE includes central and peripheral nervous system manifestations; the first ones are cerebrovascular disease, cognitive dysfunction, seizures, and psychosis; the second ones include autonomic disorder, myasthenia gravis and neuropathies.¹¹ The occurrence of psychotic symptoms at disease onset is unusual; they have been described in 3.6% of SLE patients¹² and they seem to occur most often in the early phases of the disease.¹³ Catatonia has not been formally recognized as a presentation of NP SLE but it has been reported during its course by Boeke et al.¹⁴ who identified 37 cases in the published literature.

CoS can be summarized as “belief that one is dead”. Successful treatment of CoS includes fluoxetine, paroxetine, and lithium as well as combination approaches, such as haloperidol and clomipramine. Cases with melancholia or psychotic depression are perhaps best managed by electroconvulsive (ECT) therapy, which seems to be the most frequently reported successful treatment, especially in combination with post-ECT pharmacological maintenance.¹⁵ Also, benzodiazepines and ECT are the most recommended modalities in current usage for catatonia.¹⁶

Catatonic symptoms in CoS have been classically described as rare. However, according to Simpson et al.¹⁷ their coexistence could be more frequent. This could be explained, in part, because catatonic symptoms are commonly underdiagnosed since they are rarely explored, which could lead, in the worst-case scenario, to an ineffective treatment; this, in turn, may place patients’ lives at risk of complications including premature death from immobility, dehydration and malnutrition.

There are few reports of CoS associated with catatonia, but none of these cases were associated with SLE; thus, this is the first time this association is being reported. Sometimes, the diagnosis of SLE is a challenge. As to our patient, she initially seemed to respond favorable to antipsychotics. However, the NP symptoms reappeared quickly, so, in the absence of evidence of other pathologies to attribute the patient's clinical manifestations, she was treated with high-dose steroids as NP involvement in SLE, obtaining the prompt remission. of her symptoms.

There are still uncertainties as to the neurophysiological mechanisms causing NPSLE symptoms, including catatonia. Their etiopathogenesis are likely to be multifactorial and several mechanisms have been implicated. For example, the antiphospholipid syndrome (APS) can contribute to NP symptoms either through large vessel ischemia or microvascular disease.¹⁴ In addition, the CSF of NPSLE patients has shown the presence of many inflammatory cytokines, including a proliferation-inducing ligand (APRIL), tumor necrosis factor (TNF), interleukin-6 (IL-6), IL-1, B cell-activating factor (BAFF), and interferon alpha (IFN-α). These molecules may trigger inflammation, but pathological and physiological mechanisms still need to be further studied.¹⁸

We should consider CoS as a severe condition, since these patients, due to their affective and delusive symptoms, are at risk of harming themselves; therefore, hospitalization and close observation must be done.¹⁵ The treatment is very diverse and focuses mainly on the management of the basic clinical condition, e.g., in patients with depressive disorders and psychotic symptoms, antidepressants combined with antipsychotics have been found to be useful.¹⁷ Some authors propose the use of mood stabilizers when the affective symptomatology is very intense.¹⁹ Finally, rheumatologists and psychiatrists should work together to improve the care of rheumatic disease patients, identifying the symptoms that uniquely reflect mental health problems, so the patients’ quality of life can be substantially improved.²⁰

Conclusion

Catatonia can be a manifestation of NPSLE, especially when there are symptoms and serologies consistent with active SLE. The appearance of catatonic symptoms and CoS in NPSLE has never been described. Clinicians should keep in mind that catatonic symptoms can be part of SLE and treat them accordingly if the consequences of not treating these patients adequately are to be avoided.

Conflicts of interest

The authors have no conflicts of interest.

Acknowledgments

We are grateful to Manuel F. Ugarte-Gil, MD, MSc and Graciela S. Alarcón, MD, MPH, MACR, for providing expert assistance in the review of earlier versions of this manuscript.