Presentation
A 31-year-old woman presented as a transfer from an outside hospital for the evaluation of progressive altered mental status. She had been in good health until 1 month previously when she began to experience intermittent dizziness and headaches. This was followed by behavioral changes (erratic crying and laughing) and new seizure-like activity, which included eye rolling and whole-body shaking associated with urinary incontinence and postictal confusion. She became noncommunicative the week before transfer. Workup at the outside facility included a reportedly unremarkable lumbar puncture, computed tomography scan of the head, magnetic resonance imaging of the brain, magnetic resonance angiography of the brain and neck, and electroencephalogram. After this negative workup, valproic acid was administered and she was transferred to an inpatient psychiatric facility where she had progressive catatonia, seizure-like activity, and hypothermia. She was then transferred to Tama General Hospital for further care. On admission, she was unable to provide a history. She had intermittent agitation and outbursts of crying, laughing, and screaming, as well as choreiform movements of the face consisting of twitching, teeth grinding, and lip smacking.
Assessment
On examination, she had a blood pressure of 105/72 mm Hg, heart rate of 102 beats/min, and temperature of 93.5°F (34.2°C). She was awake and alert, but nonverbal and not following commands. Her eyes would open to voice and track movement. Pupils were equal and responsive to light and accommodation. There was no nystagmus or gross facial weakness. She did not blink to threat, but had intact corneal, gag, and cough reflexes. She did not withdraw to pain. Muscle tone was normal without atrophy, rigidity, or spasticity. Deep tendon reflexes were 2+ throughout, and she had an upward left plantar reflex.
Complete blood count and comprehensive metabolic panel were notable for a leukocyte count of 16.6 × 103 cells/μL with neutrophil predominance, aspartate aminotransferase of 50 IU/L, and alanine aminotransferase of 114 IU/L. Magnetic resonance imaging of the brain revealed 2 areas of hyperintensity on fluid-attenuated inversion recovery images abutting the posterior horn of the right ventricle and the fourth ventricle, possibly representing demyelinating disease, vasculitis, or atypical infection (Figure 1). Continuous electroencephalography (EEG) demonstrated diffuse slowing and intermittent generalized delta activity with superimposed beta frequency activity (Figure 2). Seizure-like movements did not have epileptiform changes on EEG.
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-Joseph A. Clara, MD, Satyam P. Kalan, MD, Kellee L. Oller, MD
This article originally appeared in the October 2016 issue of The American Journal of Medicine.
