Psychiatric Annals

Case Report 

A Case of Lupus Cerebritis

Rasna Patel, MD; Amit Mistry, MD

Abstract

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by multisystem organ involvement in which the body's immune system attacks its own self. Clinical presentation and course are variable; common symptoms at presentation may include fever, fatigue, malaise, joint pain, abdominal pain, and stereotypical malar rash.1 In severe cases, it can lead to lupus cerebritis, a neuropsychiatric manifestation of SLE that presents clinically with confusion, lethargy, seizures, coma, mood changes, and even psychosis. It is a serious condition that is difficult to diagnose but it is potentially treatable with early intervention.2,3 The patient in this case not only developed lupus cerebritis, but also catatonia secondary to lupus. There are not many cases reporting on the neuropsychiatric manifestation of SLE.4

The patient was a 16-year-old girl in ninth grade who resided with her mother, grandmother, and two siblings. She had no known psychiatric history and her past medical history was significant for chronic anemia and benign heart murmur. The patient presented with fluctuating mentation, fatigue, psychomotor retardation, anorexia (with 16 lbs of weight loss), dysarthria with word-finding difficulty, slow speech, and unsteady gait. She had no significant recent stressors, head trauma, or travel. Laboratory tests were remarkable for elevated thyroid hormone, mild anemia, leukopenia, elevated lipase, elevated erythrocyte sedimentation rate (ESR), and hematuria on urinary analysis. A chest X-ray and computed tomography scan of her head were unremarkable. Physical examination revealed slow speech and cognition, tachycardia with systolic murmur, nontender cervical adenopathy, splenomegaly, slight tremor noted in left hand, and decreased strength in both upper limbs. The patient was transferred for continuation of care and admitted to Hospital Medicine, who consulted Neurology. At the time, her mentation had improved and no focal findings were noted. Neurology recommended continuation of infectious and metabolic investigations, with consultation of Psychiatry for possible psychogenic etiology.

During the initial evaluation by Psychiatry, the patient was able to maintain adequate eye contact and engage in conversation with soft and hypophonic speech. Her thought process was linear and logical and she denied active suicidal or homicidal ideations and reported no psychosis. Her cognition was grossly intact with no significant findings. Although the patient was vulnerable to mental illness in relation to her significant history of verbal abuse inflicted on her, witness to domestic abuse in the home as a child, and significant family history of psychiatric disease, her current symptoms could not solely be explained by a primary psychiatric condition. Psychiatry recommended continuation of testing for organic cause in relation to her physical examination findings (tachycardia, fever, nontender cervical adenopathy, and splenomegaly).

The patient's hospital course was further complicated when her sympathetic nervous system became overactive, with heart rate of approximately 150 beats per minute and respiration rate averaging 25 breaths per minute with uncontrolled blood pressure. Cardiology was consulted to help manage this complication and recommended imaging of her abdomen for possible pheochromocytoma and renal artery stenosis. Infectious Disease (consulted for the patient's fever of unknown origin) found no abnormalities in blood and urine cultures; other tests revealed normocytic anemia, elevated ESR, normal C-reactive protein, and continued hematuria. As a result of these laboratory findings, Rheumatology was consulted for concern of an autoimmune disease.

The suspicion for SLE was high due to the multisystem involvement (ie, hematological, nervous, renal, pulmonary, and cardiac systems). Further physical examination showed a dermatological finding of punctate palmar erythema, lipstick fingertip staining, and ulceration erythema on her hard palate. Additional tests were positive for antinuclear antibodies (ANA) and anti-DNA antibodies, elevated myoglobin, and low complement 3 and 4. Her urinary analysis was positive for moderate blood and small proteins (Table 1).…

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by multisystem organ involvement in which the body's immune system attacks its own self. Clinical presentation and course are variable; common symptoms at presentation may include fever, fatigue, malaise, joint pain, abdominal pain, and stereotypical malar rash.1 In severe cases, it can lead to lupus cerebritis, a neuropsychiatric manifestation of SLE that presents clinically with confusion, lethargy, seizures, coma, mood changes, and even psychosis. It is a serious condition that is difficult to diagnose but it is potentially treatable with early intervention.2,3 The patient in this case not only developed lupus cerebritis, but also catatonia secondary to lupus. There are not many cases reporting on the neuropsychiatric manifestation of SLE.4

Case Presentation

The patient was a 16-year-old girl in ninth grade who resided with her mother, grandmother, and two siblings. She had no known psychiatric history and her past medical history was significant for chronic anemia and benign heart murmur. The patient presented with fluctuating mentation, fatigue, psychomotor retardation, anorexia (with 16 lbs of weight loss), dysarthria with word-finding difficulty, slow speech, and unsteady gait. She had no significant recent stressors, head trauma, or travel. Laboratory tests were remarkable for elevated thyroid hormone, mild anemia, leukopenia, elevated lipase, elevated erythrocyte sedimentation rate (ESR), and hematuria on urinary analysis. A chest X-ray and computed tomography scan of her head were unremarkable. Physical examination revealed slow speech and cognition, tachycardia with systolic murmur, nontender cervical adenopathy, splenomegaly, slight tremor noted in left hand, and decreased strength in both upper limbs. The patient was transferred for continuation of care and admitted to Hospital Medicine, who consulted Neurology. At the time, her mentation had improved and no focal findings were noted. Neurology recommended continuation of infectious and metabolic investigations, with consultation of Psychiatry for possible psychogenic etiology.

During the initial evaluation by Psychiatry, the patient was able to maintain adequate eye contact and engage in conversation with soft and hypophonic speech. Her thought process was linear and logical and she denied active suicidal or homicidal ideations and reported no psychosis. Her cognition was grossly intact with no significant findings. Although the patient was vulnerable to mental illness in relation to her significant history of verbal abuse inflicted on her, witness to domestic abuse in the home as a child, and significant family history of psychiatric disease, her current symptoms could not solely be explained by a primary psychiatric condition. Psychiatry recommended continuation of testing for organic cause in relation to her physical examination findings (tachycardia, fever, nontender cervical adenopathy, and splenomegaly).

The patient's hospital course was further complicated when her sympathetic nervous system became overactive, with heart rate of approximately 150 beats per minute and respiration rate averaging 25 breaths per minute with uncontrolled blood pressure. Cardiology was consulted to help manage this complication and recommended imaging of her abdomen for possible pheochromocytoma and renal artery stenosis. Infectious Disease (consulted for the patient's fever of unknown origin) found no abnormalities in blood and urine cultures; other tests revealed normocytic anemia, elevated ESR, normal C-reactive protein, and continued hematuria. As a result of these laboratory findings, Rheumatology was consulted for concern of an autoimmune disease.

The suspicion for SLE was high due to the multisystem involvement (ie, hematological, nervous, renal, pulmonary, and cardiac systems). Further physical examination showed a dermatological finding of punctate palmar erythema, lipstick fingertip staining, and ulceration erythema on her hard palate. Additional tests were positive for antinuclear antibodies (ANA) and anti-DNA antibodies, elevated myoglobin, and low complement 3 and 4. Her urinary analysis was positive for moderate blood and small proteins (Table 1). The patient met criteria for a diagnosis of SLE (Table 2), so she was started on high-dose steroids with plans to discharge in a few days; however, the patient's mentation deteriorated and she was unable to follow commands and carry on conversation. Her airway became compromised and she was transferred to the pediatric intensive care unit for her advancing medical needs. Concern for seizure activity prompted an electroencephalogram and she was given levetiracetam. The patient continued to decompensate so a nasogastric tube was placed to aid in her dysphagia. Additional supportive measures were taken to improve functionality via occupational, physical, and respiratory therapy. The patient had severe neuropsychiatric systemic lupus with ongoing encephalopathy; serological improvement was noted but there was no clinical response to treatment.

Summary of the Patient's Significant Laboratory Results

Table 1:

Summary of the Patient's Significant Laboratory Results

Systemic Lupus International Collaborating Clinics Classification Criteria for Systemic Lupus Erythematosusa

Table 2:

Systemic Lupus International Collaborating Clinics Classification Criteria for Systemic Lupus Erythematosus

Her hospitalization was further complicated when she became catatonic. On examination, the patient presented as nonverbal, unable to follow commands, and exhibiting hypokinesis, catalepsy, waxy flexibility, negativism, echopraxia. There was no evidence of mannerisms, stereotypical movements, agitation, or grimacing.10 Psychiatry was reconsulted and recommended lorazepam, but the patient showed only minimal improvement until the dosage was increased. Electroconvulsive therapy was discussed with the patient's mother, who refused the treatment. The patient slowly improved over the course of 2 months and eventually was discharged with appropriate follow-up and medications.

Discussion

This case exemplifies many of the challenges of diagnosing and treating SLE and its complications. This case was unique in that the patient presented with sudden onset of symptoms. Her course of disease rapidly manifested itself with neuropsychiatric symptoms, which became a challenge as she did not have a prior diagnosis of SLE. However, once SLE was included in the differential diagnosis, the diagnosis was made quickly via laboratory tests measuring autoantibodies.5

Aggressive steps to manage her systemic lupus were taken. She was given intravenous cyclophosphamide and immunoglobins, and had three rounds of plasma exchange.

This case also illustrates the importance of first ruling out organic causes of symptoms of a present illness before suggesting psychiatric disorders as the etiology. Psychiatry is often the first to be consulted when a patient presents with central nervous system (CNS) symptoms, but if a primary organic condition can be identified and adequately treated, then CNS symptoms may be mitigated, if not entirely alleviated, with the correct treatment.6

In this patient, the correct diagnosis was made and she was able to receive aggressive treatment. However, due to the severity of her acute presentation, the insult to her brain was so significant that her condition progressed to catatonia. Catatonia is a medical condition characterized by abnormality in movement and behavior. There are multiple etiologies for catatonia, including SLE. The severity of catatonia can be rated by using the Bush-Francis rating scale, which can also be used for monitoring improvement.7,8

References

  1. Tsokos GC. Systemic lupus erythematosus. N Engl J Med. 2011;365:2110–2121. doi:. doi:10.1056/NEJMra1100359 [CrossRef]
  2. Calabrese L, Stern T. Neuropsychiatric manifestation of systemic lupus erythematosus. Psychosomatics. 1995;36:344–359. doi:10.1016/S0033-3182(95)71644-9 [CrossRef]
  3. Stojanovich L, Zandman-Goddard G, Pavlovich S, Sikanich N. Psychiatric manifestation in systemic lupus erythematosus. Autoimmun Rev. 2007;(6):421–426. doi:. doi:10.1016/j.autrev.2007.02.007 [CrossRef]
  4. Ali A, Taj A, Uz-Zehra M. Lupus catatonia in a young girl who presented with fever and altered sensorium. Pak J Med Sci. 2014;30(2):446–448.
  5. Zandman-Goddard G, Chapman J, Shoenfeld Y. Autoantibodies involved in neuropsychiatric SLE and antiphospholipid syndrome. Semin Arthritis Rheum. 2007;36(5):297–315. doi:. doi:10.1016/j.semarthrit.2006.11.003 [CrossRef]
  6. Bronheim HE, Fulop G, Kunkel EJ, et al. The Academy of Psychosomatic Medicine practice guidelines for psychiatric consultation in the general medical setting. The Academy of Psychosomatic Medicine. Psychosomatics.1998;39(4):S8–3S0. doi:10.1016/S0033-3182(98)71317-9 [CrossRef]
  7. Walther S, Strik W. Catatonia. CNS Spectr. 2016;21:341–348. doi:. doi:10.1017/S1092852916000274 [CrossRef]
  8. Sienaert P, Dhossche DM, Vancampfort D, De Hert M, Gazdag G. A clinical review of the treatment of catatonia. Front Psychiatry. 2014;5:181. doi:. doi:10.3389/fpsyt.2014.00181 [CrossRef]
  9. Kuhn A, Bonsmann G, Anders H, Herzer P, Tenbrock K, Schneider M. The diagnosis and treatment of systemic lupus erythematosus. Dtsch Arztebl Int. 2015;112(25):423–432. doi:10.3238/arztebl.2015.0423 [CrossRef].
  10. Bush G, Fink M, Petrides G, Dowling F, Francis A. Catatonia. I. Rating scale and standardized examination. Acta Psychiatr Scand.1996;93(2):129–136. doi:. doi:10.1111/j.1600-0447.1996.tb09814.x [CrossRef]

Summary of the Patient's Significant Laboratory Results

Laboratory Test Result
Antinuclear antibodies Positive
Anti-DNA antibodies >300 IU/mL
Complement 3 and 4 43 and 7 mg/dL
Anti-Smith antibodies <0.2
White blood cell count 2.43/mcL
Red blood cell count 2.76/mcL
Hemoglobin 8 g/dL
Hematocrit 26.8%
Erythrocyte sedimentation rate 40 mm/h

Systemic Lupus International Collaborating Clinics Classification Criteria for Systemic Lupus Erythematosusa

Clinical Criteria Laboratory Criteria
Acute cutaneous lupus Antinuclear antibodies
Chronic cutaneous lupus Anti-DNA
Oral or nasal ulcers Anti-Smith antibodies
Nonscarring alopecia Antiphospholipid antibodies
Arthritis Low complement (C3, C4, CH50)
Serositis Direct Coombs' test absence in the presence of hemolytic anemia
Renal clinical symptoms
Neurologic clinical symptoms
Hemolytic anemia
Leukopenia
Thrombocytopenia
Authors

Rasna Patel, MD, is a Year-2 Fellow, Child and Adolescent Psychiatry, Yale University School of Medicine. Amit Mistry, MD, is a Postgraduate Year-4 Resident, Department of Psychiatry, University of Oklahoma Health Sciences Center.

Address correspondence to Rasna Patel, MD, Child Study Center, Yale University School of Medicine, 230 S. Frontage Road, New Haven, CT 06519; email: Rasna.patel@yale.edu.

Disclosure: The authors have no relevant financial relationships to disclose.

10.3928/00485713-20180525-01

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