Catatonia – An unusual presenting clinical manifestation of systemic lupus erythematosus

doi:10.1016/j.reuma.2016.03.013

Reumatología Clínica

Volume 13, Issue 4, July–August 2017, Pages 224-226

https://doi.org/10.1016/j.reuma.2016.03.013 Get rights and content

Abstract

A 24-year-old female presented with catatonia and symptoms suggestive of Depressive Disorder. She also gave history of undocumented low grade irregular fever. The patient was worked up to rule out any organic cause or psychiatric illness. However, further investigations revealed immunological profile diagnostic of Systemic Lupus Erythematosus (SLE) with CNS involvement (CNS lupus). The diagnosis of SLE in this patient presenting with catatonia was of practical importance because catatonia as one of the manifestations of SLE or as a standalone presenting symptom is extremely rare. Hence, clinicians should be aware of this rarity so that diagnosis of Neuropsychiatric SLE (NPSLE) or catatonia as a presenting feature of SLE is never missed.

Resumen

Mujer de 24 años que se presentó con catatonía y síntomas indicativos de trastorno depresivo. También presentó historia de febrícula discontinua no registrada. Se llevó a cabo evaluación diagnóstica para descartar cualquier causa orgánica o enfermedad psiquiátrica. Sin embargo, exploraciones complementarias posteriores revelaron un perfil inmunológico diagnóstico de lupus eritematoso sistémico (LES) con implicación del SNC (lupus del SNC). El diagnóstico de LES en esta paciente que se presenta con catatonía era de significado práctico ya que la catatonía, como una de las manifestaciones del LES o como un síntoma que se presenta de forma independiente, es extremadamente rara. Por ello, los médicos deben ser conscientes de esta rareza y no deben olvidar nunca el diagnóstico de LES neuropsiquiátrico (LESNP) o catatonía como rasgo presente en el LES.

Introduction

SLE is a chronic autoimmune disease of unknown etiology, typically diagnosed on the basis of the SLICC criteria.¹ Its clinical presentation is variable with arthralgia, photosensitivity and rash being the most common symptoms. Although Central Nervous System (CNS) involvement in SLE varies from 14% to over 80%,2, 3 initial presentation with neurological features is considered rare, seen only in approximately 3% of the patients.⁴ In the absence of typical manifestations, patients presenting solely with neuropsychiatric symptoms of SLE can pose a diagnostic dilemma to the clinician. We report a case of catatonia as the presenting manifestation of SLE in a young female.

Section snippets

Case report

A 24-year-old young female patient presented with the complaints of behavioral abnormalities like decreased oral intake, speech output and interaction with family members, inability to recognize relatives or communicate via eye contact, immobility, abnormal posturing, vacant staring, grimacing, repetitive utterances, poor self-care, loss of bowel and bladder control and low grade undocumented fever since last one month.

General physical examination revealed disoriented patient with stable

Discussion

NPSLE can present with various neurologic and psychiatric manifestations. The etiology behind NPSLE is postulated to be multifactorial involving up-regulation of the expression of adhesion proteins on endothelial cells due to production of proinflammatory cytokines and autoantibodies, facilitating lymphocyte entry into the central nervous system.³ Cataonia is defined by DSM-V⁵ and as a presentation of NPSLE has been described very rarely in literature; however, it is not clearly understood why

Conclusion

This case report gives a strong insight that patients of SLE like ours may not have any of the typical features of SLE and present with highly unusual neuropsychiatric symptoms like catatonia. This can be misleading to the clinician leading to erroneous diagnosis of a condition that is amenable to treatment. Catatonia with SLE is completely reversible with steroid and/or cytotoxic drug therapy. Hence it is highly essential to differentiate catatonia as a result of NPSLE from other causes of

Protection of human and animal subjects

The authors declare that the procedures followed were in accordance with the regulations of the relevant clinical research ethics committee and with those of the Code of Ethics of the World Medical Association (Declaration of Helsinki).

Confidentiality of data

The authors declare that they have followed the protocols of their work center on the publication of patient data.

Right to privacy and informed consent

The authors must have obtained the informed consent of the patients and/or subjects mentioned in the article. The author for correspondence must be

Conflict of interest

The authors declare that they had no conflicts of interest.

References (8)

E. Muscal et al.
Neurologic manifestations of systemic lupus erythematosus in children and adults
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R. Tandon et al.
Catatonia in DSM-5
Schizophr Res
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R.L. Brey et al.
Neuropsychiatric syndromes in lupus: prevalence using standardized definitions
Neurology
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H. Ainiala et al.
The prevalence of neuropsychiatric syndromes in systemic lupus erythematosus
Neurology
(2001)

There are more references available in the full text version of this article.

Cited by (4)

Molecular and immunological origins of catatonia
2024, Schizophrenia Research
Catatonia occurs secondary to both primary psychiatric and neuromedical etiologies. Emerging evidence suggests possible linkages between causes of catatonia and neuroinflammation. These include obvious infectious and inflammatory etiologies, common neuromedical illnesses such as delirium, and psychiatric entities such as depression and autism-spectrum disorders. Symptoms of sickness behavior, thought to be a downstream effect of the cytokine response, are common in many of these etiologies and overlap significantly with symptoms of catatonia. Furthermore, there are syndromes that overlap with catatonia that some would consider variants, including neuroleptic malignant syndrome (NMS) and akinetic mutism, which may also have neuroinflammatory underpinnings. Low serum iron, a common finding in NMS and malignant catatonia, may be caused by the acute phase response. Cellular hits involving either pathogen-associated molecular patterns (PAMP) danger signals or the damage-associated molecular patterns (DAMP) danger signals of severe psychosocial stress may set the stage for a common pathway immunoactivation state that could lower the threshold for a catatonic state in susceptible individuals. Immunoactivation leading to dysfunction in the anterior cingulate cortex (ACC)/mid-cingulate cortex (MCC)/medial prefrontal cortex (mPFC)/paralimbic cortico-striato-thalamo-cortical (CSTC) circuit, involved in motivation and movement, may be particularly important in generating the motor and behavioral symptoms of catatonia.
Catatonia Associated With Systemic Lupus Erythematosus (SLE): A Report of Two Cases and a Review of the Literature
2018, Psychosomatics
Citation Excerpt :
Four articles contained insufficient clinical details to confirm a diagnosis of catatonia using DSM-5 criteria. The remaining 26 articles described a total of 35 patients (Table 1).14–39 All of the patients described were female, and their ages ranged from 13 to 47.
Systemic lupus erythematosus (SLE) is known to cause neuropsychiatric symptoms (NPSLE). While not formally recognized as a syndrome associated with NPSLE, catatonia has frequently been reported.
It is important for clinicians to recognize and treat catatonia as a potential manifestation of NPSLE. We present 2 cases of SLE with catatonia and review the cases reported in the literature.
We performed a PubMed search for reported cases of catatonia in SLE. Case reports that met Diagnostic and Statistical Manual of Mental Disorders-5th ed. diagnostic criteria for catatonia were summarized to assess common diagnostic tests and treatments.
Twenty-six articles describing a total of 35 patients (all female), in addition to our 2 patients, were included in the report. All but one of the patients received immunosuppressive therapy for treatment of SLE. To treat catatonia symptoms, 81% of the patients received benzodiazepines, and 38% received electroconvulsive therapy.
Catatonia can be a manifestation of NPSLE, particularly in the presence of serologies and symptoms indicative of an active lupus flare. Management of catatonia involves management of the underlying condition, in this case immunomodulatory treatments for NPSLE; avoidance of treatments, such as antipsychotics, which can worsen catatonia; and symptomatic treatments for catatonia, for which benzodiazepines are a first-line treatment, and electroconvulsive therapy when catatonia is refractory to benzodiazepines.
Catatonia in systemic lupus erythematosus: case based review
2022, Rheumatology International
Malignant catatonia as the presenting manifestation of systemic lupus erythematosus
2020, Bulletin of the Hospital for Joint Diseases

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Case reportCatatonia – An unusual presenting clinical manifestation of systemic lupus erythematosusCatatonía-manifestación clínica de presentación infrecuente del lupus eritematoso sistémico

Abstract

Resumen

Introduction

Section snippets

Case report

Discussion

Conclusion

Protection of human and animal subjects

Confidentiality of data

Right to privacy and informed consent

Conflict of interest

Neurol Clin

Schizophr Res

Neuropsychiatric syndromes in lupus: prevalence using standardized definitions

Neurology

The prevalence of neuropsychiatric syndromes in systemic lupus erythematosus

Neurology

Case report
Catatonia – An unusual presenting clinical manifestation of systemic lupus erythematosusCatatonía-manifestación clínica de presentación infrecuente del lupus eritematoso sistémico