Case presentation
Sixteen years old female was referred to rheumatology with a history of
progressive forgetfulness and episodes of jerky movements that were
associated with loss of consciousness, upward rotation of both eyeballs,
tongue bite, and frothy secretions from the mouth, which persisted for 3
to 10 minutes and followed by an incoherent talk. She had complained of
a headache for the last 12 months. There was no history of cough,
vomiting, blurred vision, head injury, anorexia, weight loss, or travel
history to a malaria area. She is nonsmoker nor an alcoholic. She is
allergic to penicillin. Her family history of autoimmune diseases and
encephalitis is unremarkable. She was seen in ER in another hospital and
then was labeled as a case of suspected viral encephalitis associated
with fever; a lumbar puncture was done, then started on antiviral for 14
days and discharged. After that, she still noticed behavioral changes
like depression, increased forgetfulness, and recurrent attacks of
seizures with agitation for one month. Later on, she presented to our
clinic and was diagnosed to have systemic lupus erythematosus based on
her clinical presentation, which included neurological manifestations
and malar rash, and lab results which showed positive ANA and dsDNA.On initial examination, the patient was found agitated but
oriented. Her vital signs were, her pulse rate was 87 beats/minute, her
blood pressure was 166/91 mm Hg, her Respiratory rate was 17/minute, her
temperature: was 36°C, and her weight was: 102 Kg. She had a
photosensitive malar rash, alopecia, lymphadenopathy, and thyroid
nodules. Other neurological examination findings were normal, and
examination of other systems also revealed no other abnormalities.Investigations done were , complete blood count (CBC) showed
decrease in white blood cells (WBC) = 2700 /μL (4.1 – 11.6 ), increase
in monocytes = 14.4% (3.8 – 11.2), normal red blood cells (RBCs)
count, and normal hemoglobin (HGB) level. The liver function tests (LFT)
showed mild elevation of alanine aminotransferase (ALT) = 44 (0 – 40),
normal albumin level, and other findings were normal. Kidney function
test (KFT) showed creatinine level = 33 μmol/L (44 – 80), urea level =
1.95mmol/L (2.1 – 7.1), and other findings were normal. Urine analysis
showed negative blood, negative protein, negative glucose, and other
findings were normal. Erythrocyte sedimentation rate (ESR) was 07
mm/hour (0 – 20). C-reactive protein (CRP) was positive, and rheumatoid
factor (RF) and Anti-cyclic citrullinated peptide (ANTI-CCP) were
negative. Anti-dsDNA antibodies was positive 81.7 IU/mL (positive
> 35). C3 complement was 90 mg/dL (90 – 180) and C4
complement was 12 mg/dL (12 – 40). A thyroid function test (TFT)
revealed a mild decrease in free thyroid hormone 3 (FT3) with normal
thyroid-stimulating hormone (TSH) and free thyroid hormone 4 (TF4)
levels. ECG shows sinus tachycardia. EEG was done and showed normal
brain activity. MRI for the brain revealed prominent sulci, mainly
Sylvian fissure, corpus callosum atrophy, and bilaterally prominent
temporal horns with relatively symmetrical increased FLAIR signal
intensity of both hippocampal formations (figures
1a, 1b,
1c, and 1d ). Ultrasound
for thyroid shows multiple bilateral nodules with tiny calcific foci. An
echocardiogram revealed trace aortic and mitral regurgitation, impaired
relaxation by tissue Doppler, no pericardial effusion, and normal
ventricular dimension. CT of the chest, abdomen, and pelvis was done,
and their results were unremarkable.