A 27 year-old African American female with a history of bipolar disorder, cocaine abuse, Mixed Connective Tissue Disease with Raynaud’s syndrome presented to the emergency department with acute onset of altered mental status. Her family reported that she exhibited bizarre behavior for three days. She was found undressed at home with pressured and unintelligible speech. They noted that she would intermittently become unresponsive and have roving eye movements. In the ED, her temperature was 101.5F, pupils were pinpoint and she was speaking incoherently and very rapidly. Motor exam revealed normal reflexes. Kernig and Brudzinski’s signs were negative.
Urine drug screen was negative and CT head showed mild cerebral swelling. She developed status epilepticus which required treatment with levatiracetam, lorazepam, and phenytoin. Lumbar puncture revealed acellular cerebrospinal fluid with increased protein of 412 mg/dl. Blood cultures and testing of the CSF for viral, bacterial, and fungal organisms was negative. Thyroid stimulating hormone and electrolytes were within normal limits. Anti-nuclear antibodies were positive at 1:1280, dsDNA antibody of 1:40 and Anti-Smith antibody of 35. These findings were consistent with neuropsychiatric lupus. Treatment was initiated with high dose methylprednisone. Hypertonic saline was administered to decrease cerebral edema. Her mental status improved significantly and she was transitioned to cyclophosphamide. The patient had a complete recovery.
Neuropsychiatric SLE (formerly known as Lupus cerebritis) refers to the various cognitive, neurologic and psychiatric symptoms that may result from a variety of organic pathologic processes that affect the CNS. It may present with a wide range of symptoms including cognitive dysfunction, seizures, strokes, neuropathy, and psychosis. The most common presentation of neuropsychiatric SLE is cognitive dysfunction which can manifest with impairments in memory, abstract thinking, and judgment. It may be difficult to differentiate these symptoms from co-existing psychopathology or side effects from steroid treatment. Psychiatric symptoms in SLE are most often functional and in response to coping with a chronic illness. Psychosis occurs in about five percent of patients with SLE. Clues to an organic etiology are fever, seizure-like activity, or other focal neurologic signs.
While the underlying etiology is unclear, potential mechanisms may include anti-neuronal antibodies, inflammation-induced neuronal dysfunction, an increase in intrathecal cytokine production, accelerated atherosclerosis, and thrombosis. In most cases NPSLE is a diagnosis of exclusion with a differential diagnosis of acute or chronic meningitis, mass lesions, arterial emboli and primary psychiatric disorders. Sequential diagnostic testing often involves CNS imaging (CT or MRI), lumbar puncture, EEG, and psychometric testing. Patients with neuropsychiatric SLE should be treated with pulse dose steroids and in more severe cases a trial of cyclophosphamide may be beneficial.
Psychosis and mania are rare presentations of neuropsychiatric SLE. Therefore, diagnosis and treatment are often delayed. Diagnosis requires excluding other causes, most importantly infection. Steroids are the mainstay of treatment in the acute care setting.
To cite this abstract:Deep K, Jesinger M, Wingerter K. It’s Never Lupus…?. Abstract published at Hospital Medicine 2015, March 29-April 1, National Harbor, Md. Abstract 502. Journal of Hospital Medicine. 2015; 10 (suppl 2). https://www.shmabstracts.com/abstract/its-never-lupus/. Accessed April 7, 2020.