case report
A fulminant neuropsychiatric crisis was not lupus until labs proved it.
2026-06-23
A fulminant neuropsychiatric crisis was not lupus until labs proved it.
A sudden shift
In June 2025, a 21-year-old woman with an eight-year history of systemic lupus erythematosus (SLE) and hypothyroidism experienced an abrupt change in behavior. In the early morning hours, she became disoriented and aggressive. These psychiatric and behavioral symptoms quickly evolved into somnolence, slow speech (bradylalia), and a slowed thought process (bradypsychia).
The woman had stopped taking all of her medications four months prior to this episode. By the time she reached the emergency department later that morning, she was indifferent to her environment and began vomiting. On admission, she was tachycardic with a heart rate of 116 bpm. Physical examination revealed facial edema and swelling in her legs.
The psychiatric mask
The initial neurological decline was marked by disorientation and behavioral changes, but her body soon followed. She was found to have a Glasgow Coma Scale of 8, mydriatic pupils, and a bilateral Babinski sign. Despite the severity of her presentation, her initial non-contrast head CT was unremarkable, showing no acute lesions, hemorrhages, or ischemic changes.
Rheumatology was consulted in the emergency department and suspected neuro-lupus. They recommended a neurology consultation and a lumbar puncture to rule out infection. Because of her rapid deterioration, she required advanced airway management shortly after arrival. Outwardly, she appeared to be in a state of acute encephalitis.
Normal markers and hidden activity
Physicians ordered a battery of tests to find the cause of the crisis. Surprisingly, the standard markers for lupus activity were normal: her anti-double-stranded DNA (dsDNA) antibodies were negative, and her complement levels (C3 and C4) were within normal ranges. Her cerebrospinal fluid showed only mild pleocytosis with 10 leukocytes/mm3 and no signs of infection.
While her laboratory markers suggested the disease might be inactive, her clinical state continued to spiral. She developed hypotension that required vasopressor support and experienced two generalized seizures that did not respond to benzodiazepines. She was eventually diagnosed with refractory status epilepticus.
The final 72 hours
Seventy-two hours after the first behavioral changes appeared, a second scan revealed the extent of the damage. A cranial CT angiography showed severe cerebral and cerebellar edema and a lack of blood flow in the posterior circulation of the brain. The brain was suffering from malignant ischemic-edematous changes.
Sedation was stopped to assess her neurological response, but no brainstem reflexes were observed. An electroencephalogram (EEG) showed an isoelectric tracing, indicating a total lack of brain activity. Diagnosis of brain death was confirmed within three days of her admission. Physicians concluded that she had likely suffered from fulminant cerebral vasculitis, a rare and catastrophic manifestation of her lupus that progressed too quickly for standard intervention.
The medical picture
anti-double-stranded DNA (dsDNA)
negative
Complement C3
131.9 mg/dL
Complement C4
35.3 mg/dL
Leukocytes (Blood)
16.63×10^3/µL
Cerebrospinal fluid leukocytes
10 leukocytes/mm^3
Glasgow Coma Scale (GCS) on admission
8
Adapted faithfully from the open-access case report: AME Case Reports (PMC12885776). DOI: 10.21037/acr-2025-228. Read the original at https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12885776/.