case study by Shama Khan , MD 1 ; Uzair Khan , MD 2 ; Darrell R . Over , MD , MSc , FAAFP 1
UAMS South Central Family Medicine Residency , Pine Bluff , Ark .
Oak Hill Hospital , Tampa , Fla .
Primary Psychiatric Disorder Masking the Diagnosis of
Neuropsychiatric Syndrome in SLE
Systemic lupus erythematosus ( SLE ) is a widespread , chronic inflammatory autoimmune disorder affecting various organ systems in the body . Neuropsychiatric syndrome of systemic lupus erythematosus ( NPSLE ) refers to the nervous system involvement of SLE manifesting as neurologic or neuropsychiatric symptoms . 1 Use of older terminology such as “ lupus cerebritis ” and “ lupus sclerosis ” is no longer recommended for this condition . NPSLE has a wide range of presentation such as cognitive dysfunction , seizure disorders , movement disorder , psychosis , acute confusional state , myelopathy , or with focal symptoms such as cerebrovascular accident or transient ischemic attacks . NPSLE can present actively during an SLE flare or even precede the SLE flare , but not all cases present during the active disease course . 1 Prompt identification and diagnosis of NPSLE is crucial so that appropriate treatment can be administered and not delayed . The diagnosis of NPSLE remains challenging as there are no confirmatory laboratory or radiologic findings ; thus , this condition is a diagnosis of exclusion after ruling out other causes such as infections , electrolyte or metabolic disorders , mass lesions , and primary psychiatric disorders . 2
This report discusses a young , female patient without a previous diagnosis of SLE who initially presented with acute confusion , new onset seizures , and acute psychosis in addition to septic shock , bacteremia , severe metabolic acidosis , and acute renal failure requiring hemodialysis . Notably , she had a past medical history of bipolar disorder , IV drug abuse , and multiple previous admissions to the psychiatric unit for acute psychosis , suicide ideation , and aggressive behavior . Her previous psychiatric history made the diagnosis of NPSLE difficult ; however , once identified and treatment with IV methylprednisolone commenced , her neurologic status improved within days .
A 27-year old African American female with past medical history bipolar disorder ( on lithium , risperidone and Depakote ) and IV drug abuse presented to the emergency room ( ER ) with altered mental status , confusion , and bilateral lower extremity edema . Her initial vitals were : blood pressure 100 / 70 mm Hg , respiratory rate of 25 / min , heart rate of 105 / min , and an oral temperature of 100.1 oF . The Glascow Coma Score was 11 ( E = 3 V = 3 M = 5 ). She appeared older than her age , disheveled , cachectic , confused , with slurred speech , and was a poor historian . On general observation , she had bilateral conjunctivitis with green / yellow discharge and discoid rash was noted on her forehead extending to and around the bilateral eyes and there was diffuse thinning of hair and patches of hair loss on scalp . She had bilateral mastitis , with no abscess or nipple discharge . Examination of the chest revealed a normal S1S2 without murmur and decreased breath sounds in bilateral lung fields . She had 3 + bilateral lower extremity edema with palpable pulses . No IV track marks were noted on her body .
Admission laboratories showed a complete blood count with a white cell count of 19,000 mm , 3 hemoglobin of 4.9 gm / dl , hematocrit of 16.3 % with red cell indices showing a mean corpuscular volume of 93.1 fL , and mean corpuscular hemoglobin concentration of 30.1 gm / dl . Serum chemistries revealed potassium 7.6 mmol / L , bicarbonate 4 mmol / L , blood urea nitrogen 108 , creatinine 18.3 , lactic acid 2.4 mmol / L , and the glomerular filtration rate ( GFR ) 3 ml / min . A lithium level of 2.6 mmol / L was also noted . The urinalysis showed hyaline casts , white blood cell ( WBC ) clumps , bacteria , red and white blood cells , and proteinuria ; a urine drug screen was positive for cannabinoids , barbiturates , and amphetamines . An electrocardiogram ( EKG ) showed sinus tachycardia , and the chest x-ray demonstrated cardiomegaly and bilateral pleural effusions . A computed tomogram ( CT ) of the chest and abdomen revealed cardiomegaly with pericardial effusion , bilateral axillary lymphadenopathy , and body wall edema and free fluid in the pelvis . The CT head was unremarkable . An echocardiogram showed the left ventricular ejection fraction 20 %, with pericardial effusion but no tamponade . Considering her presentation , examination findings , and laboratory and radiologic results in the context of her previous history of IV drug use , an infectious etiology – bacterial vs . viral – was suspected and cultures obtained , and empiric therapy begun . The patient was admitted to the intensive care unit for initial management with fluid resuscitation , broad spectrum antibiotics , and urgent hemodialysis . Cerebrospinal fluid showed no cells or elevated protein . An erythrocyte sedimentation rate was elevated > 150mm / hr and the C reactive protein level was 1.5 mg / dL . Her condition clinically improved and she was transferred from the ICU after 24 hours , but on hospital day four , she had multiple generalized , tonic-clonic seizures and was re-admitted to the ICU and Neurology consultation was obtained . A CT head showed diffuse edema of bilateral cerebral hemispheres , bilateral basal ganglia , bilateral cerebellar hemispheres and brainstem , without hemorrhage or hematoma and no skull fractures . The EEG suggested mild encephalopathy and anti-epileptics were added to her treatment regimen .
The rapid decline in her neurologic status with new onset seizures , deteriorating sen-
130 • The Journal of the Arkansas Medical Society www . ArkMed . org