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Year : 2013  |  Volume : 8  |  Issue : 3  |  Page : 262-263

SLE presenting with catatonia in an adolescent girl

1 Department of Psychiatry, Postgraduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Date of Web Publication26-Dec-2013

Correspondence Address:
Sandeep Grover
Department of Psychiatry, Postgraduate Institute of Medical Education and Research, Chandigarh - 160 012
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1817-1745.123715

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How to cite this article:
Grover S, Singh A, Sarkar S, Bhalla A. SLE presenting with catatonia in an adolescent girl. J Pediatr Neurosci 2013;8:262-3

How to cite this URL:
Grover S, Singh A, Sarkar S, Bhalla A. SLE presenting with catatonia in an adolescent girl. J Pediatr Neurosci [serial online] 2013 [cited 2023 Nov 30];8:262-3. Available from: https://www.pediatricneurosciences.com/text.asp?2013/8/3/262/123715

Childhood-onset systemic lupus erythematosus (SLE) is a rare disease with an incidence of 0.3-0.9 per 100,000 children-years and a prevalence of 3.3-8.8 per 100,000 children. [1] Most studies report a median age of onset of childhood onset SLE between 11 and 12 years with disease being quite rare under the age of 5 years. As in adult-onset SLE, approximately 80% of patients with childhood-onset SLE are females. [2],[3] SLE can involve both the central and peripheral nervous systems. In 1999, the American College of Rheumatology (ACR) revised a standard nomenclature and a set of case definitions for 19 neuropsychiatric syndromes of SLE (NPSLE). [4] Up to 65% patients of childhood onset SLE develop NPSLE at any time during the disease course, and up to 85% of these patients will develop NPSLE within the first 2 years from diagnosis. [5] However, catatonia does not figure in the list of NPSLE and has been rarely reported in the published literature of SLE, especially in children and adolescents with SLE. Here we present the case of a young girl presenting with catatonia in association with SLE.

A 15-year-old girl, presented to emergency with catatonia. Exploration of the history revealed that patient was symptomatic since last 4 months. To start with, initially she had low grade fever, lasting for few hours at a stretch, occurring two to three times a day. Fever, would usually respond to taking medications. Fever was not associated with chills or rigors, burning micturation, cough, loose motions, or any specific focus of infection. After about a week of onset of fever, gradually she developed swelling on dorsum of both feet and red itchy rash over both malar areas of face. Additionally she complained of generalized body aches predominantly involving lower limbs and excessive hair fall. Over the period of 4 months, family members consulted different physicians, but there was not much improvement in her symptoms.

A week prior to being brought to the emergency at our center, she was found to have perseveration, muttering to self initially followed by significantly reduced speech, posturing, staring, withdrawal, slowness, negativism, decreased sleep and appetite, and urinary incontinence. There was no history of disorientation, visual hallucinations, thought alienation phenomenon, depressive cognition, manic symptoms, or anxiety symptoms. There was no family history of psychiatric illness.

In the emergency, she was found to have psychomotor retardation, staring, rigidity, perseveration, posturing, and negativism. Her BFCRS score was found to be 16. She also had malar rash and bilateral pedal edema. However, patient was orientated and other cognitive functions were preserved. Investigations revealed strongly positive antinuclear antibody and anti-double stranded-deoxyribonucleic acid (anti-dsDNA) antibody, low hemoglobin levels (7.3 g%), and leukocytosis (16,900/mm 3 ) with neutrophilia (neutrophils 74%). Her platelet counts, serum electrolytes, and renal and hepatic parameters were within normal limits except for low serum albumin levels. Routine urine and cerebrospinal fluid examination did not reveal any abnormality. On the basis of history, findings of physical examination and investigations, a diagnosis of SLE with organic catatonia (F06.1) was considered. She was started on inj. lorazepam 2 mg intravenous (IV) TDS along with supportive management. Injection cyclophosphamide 500 mg was given along with three doses of IV methylprednisolone 1 g over 3 days. Subsequently, she was started on oral steroids. Over the period of 1 week, she showed significant improvement in catatonic symptoms and BFCRS decreased from 16 to 4. She was discharged thereafter.

In a recent review, [6] authors presented the data of catatonia and SLE and concluded that there are only 22 cases of catatonia reported in literature to be associated with SLE and their case being the 23 rd . Of these 23 cases of catatonia in SLE, only eight cases [7],[8],[9],[10],[11],[12],[13],[14],[15] have been reported in patients younger than 18 years of age with youngest patient being aged 13 years. Our case adds to this limited data and suggests that although rare, catatonia can be seen in patients of SLE.

Literature suggests that catatonia may present as the first symptom of SLE [5],[13] or may be associated with lupus relapse [9] or be present in the natural course of illness in children and adolescents. [11] In the index case, symptoms of SLE developed prior to psychiatric manifestations and catatonia developed in the natural course of illness of SLE possibly due to the disease progression.

In the index case catatonia responded to lorazepam, which is similar to some of reported cases. [13] Benzodiazepine in catatonia associated with SLE have been reported to be safe, quick, and effective in relieving catatonia and in prevention of further complications. [16] Available data suggests that in case of failure to respond to lorazepam, the patients may improve with diazepam, [13] electroconvulsive therapy, [17] and plasma exchange. [12] Increase in dose of steroids or addition of cyclophosphamide may also be helpful in managing catatonia. [9],[18] In the index case too, in addition to lorazepam, patient was treated with cyclophosphamide and steroids.

The case demonstrates the association of catatonia with SLE in an adolescent girl and highlights the fact that whenever a patient presents with catatonia thorough evaluation in the form of history, physical examination, and investigations should be done to look for underlying general medical conditions.

   References Top

1.Kamphuis S, Silverman ED. Prevalence and burden of pediatric-onset systemic lupus erythematosus. Nat Rev Rheumatol 2010;6:538-46.  Back to cited text no. 1
2.Hiraki LT, Benseler SM, Tyrrell PN, Hebert D, Harvey E, Silverman ED. Clinical and laboratory characteristics and long-term outcome of pediatric systemic lupus erythematosus: a longitudinal study. J Pediatr 2008;152:550-6.  Back to cited text no. 2
3.Pluchinotta FR, Schiavo B, Vittadello F, Martini G, Perilongo G, Zulian F. Distinctive clinical features of pediatric systemic lupus erythematosus in three different age classes. Lupus 2007;16:550-5.  Back to cited text no. 3
4.The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes. Arthritis Rheum 1999;42:599-608.  Back to cited text no. 4
5.Benseler SM, Silverman ED. Neuropsychiatric involvement in pediatric systemic lupus erythematosus. Lupus 2007;16:564-71.  Back to cited text no. 5
6.Grover S, Parakh P, Sharma A, Rao P, Modi M, Kumar A. Catatonia in systemic lupus erythematosus: A case report and review of literature. Lupus 2013;22:634-8.  Back to cited text no. 6
7.Alao AO, Chlebowski S, Chung C. Neuropsychiatric systemic lupus erythematosus presenting as bipolar I disorder with catatonic features. Psychosomatics 2009;50:543-7.  Back to cited text no. 7
8.Marra D, Amoura Z, Soussan N, Haroche J, Consoli A, Ghillami-Dalbin P, et al. Plasma exchange in patients with stuporous catatonia and systemic lupus erythematosus. Psychother Psychosom 2008;77:195-6.  Back to cited text no. 8
9.Brelinski L, Cottencin O, Guardia D, Anguill JD, Queyrel V, Hatron PY, et al. Catatonia and systemic lupus erythematosus: A clinical study of three cases. Gen Hosp Psychiatry 2009;31:90-2.  Back to cited text no. 9
10.Lanham JG, Brown MM, Hughes GR. Cerebral systemic lupus erythematosus presenting with catatonia. Postgrad Med. J 1985;61:329-30.  Back to cited text no. 10
11.Mon T, L′ecuyer S, Farber NB, White AJ, Baszis KW, Hearn JK, et al. The use of electroconvulsive therapy in a patient with juvenile systemic lupus erythematosus and catatonia. Lupus 2012;21:1575-81.  Back to cited text no. 11
12.Perisse D, Amoura Z, Cohen D, Saintigny P, Mekhloufi F, Mazet P, et al. Case study: Effectiveness of plasma exchange in an adolescent with systemic lupus erythematosus and catatonia. J Am Acad Child Adolesc Psychiatry 2003;42:497-9.  Back to cited text no. 12
13.Wang HY, Huang TL. Benzodiazepines in catatonia associated with systemic lupus erythematosus. Psychiatry Clin Neurosci 2006;60:768-70.  Back to cited text no. 13
14.Daradkeh TK, Nasrallah NS. Lupus catatonia: A case report. Pharmatherapeutica 1987;5:142-4.  Back to cited text no. 14
15.Jørgensen A, Law I, Nielsen S, Jørgensen MB. Fluorodeoxyglucose positron emission tomography in juvenile systemic lupus erythematosus with psychiatric manifestations: Relation to psychopathology and treatment response in two cases. Rheumatology 2012;51:193-5.  Back to cited text no. 15
16.Pustilnik S, Trutia A. Catatonia as the presenting symptom in systemic lupus erythematosus. J. Psychiatr Pract 2011;17:217-21.  Back to cited text no. 16
17.Malur C, Pasol E, Francis A. ECT for prolonged catatonia. J ECT 2001;17:55-9.  Back to cited text no. 17
18.Kronfol Z, Schlesser M, Tsuang MT. Catatonia and systemic lupus erythematosus. Dis Nerv Syst 1977;38:729-31.  Back to cited text no. 18

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