A 25-year-old male was found wandering naked in his room with the shower running after failing to come to work on a Monday morning. When found, he was able to talk and follow some commands but was confused about what was happening. He had experienced a right periorbital headache with nausea and vomiting for several days before admission. A computed tomography (CT) scan of the head at an outside hospital was negative.
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The patient had no history of tick or insect bites, skin rash, chest pain, shortness of breath, trauma, or illicit drug or alcohol use. He smoked a half pack of cigarettes per day. The patient had spent time in the military in the Middle East and North Africa 3 years earlier and had 3 tattoos. Over the past few months, he had been noted to be more aggressive, including having gotten into a bar fight. His past medical history was significant only for documented hearing loss in the right ear per reports from the air base.
On examination, the patient’s temperature was 97.3°F, heart rate 47 bpm, respirations 20 breathes/min, and blood pressure 97/60 mm Hg. His neck was supple, and the remainder of the general examination was normal. The neurologic examination revealed the patient to be awake and alert but apathetic, irritable, and refusing to talk after a few minutes. He was slow to respond, spoke loudly, and had a poor attention span. The patient was disoriented to time and place and remembered 0/5 objects at 5 minutes.
Blood tests revealed 12,500/μL white blood cell (WBC) count (increased mononuclear cells, 13.7%); hemoglobin, 14.6 g/dL; platelet count 194,000/μL; and hematocrit, 43.6%. Electrolytes, general chemistries, vitamin B12, thyroid-stimulating hormone, copper, erythrocyte sedimentation rate, and urinalysis were all normal. The CT head scan was normal, and the urine drug screen and alcohol levels were negative.
The initial audiologic evaluation revealed absent acoustic reflexes bilaterally at 500 Hz, 1 KHz, 2 KHz, and 4 KHz. The brain stem auditory evoked potentials showed no replicable waveforms from the right ear and a wave I present in the left ear with no other replicable waveforms.
A very broad differential diagnosis was considered at this point (Table 1). Lumbar puncture was performed with an opening pressure of 17.5 cm H2O. There were 10 WBC/μL with 12% segmented polymorphonuclear cells and 83% lymphocytes, 30 red blood cells/μL, glucose of 71 mg/dL, and protein of 221 mg/dL. The Venereal Disease Research Laboratory Test was nonreactive; cryptococcal antigen, acid-fast stain, and bacterial and fungal cultures were negative. The electroencephalogram (EEG) showed mild diffuse slowing with frontal intermittent rhythmic delta activity. The magnetic resonance imaging (MRI) was significant for leptomeningeal and pachymeningeal enhancement with a small area of restricted diffusion in the splenium of the corpus callosum (Figure 1). Other cerebral spinal fluid and serum studies were negative or nonreactive (Table 2).
The patient completed a 2-week course of ceftriaxone 1 gram q 12 hours, vancomycin 1,000 mg q 12 hours, acyclovir 700 mg q 8 hours, and doxycycline 100 mg bid without any notable clinical change. A repeat lumbar puncture was acellular and had a protein of 254 mg/dL.
Two days later he worsened, becoming more withdrawn, unable to speak, irritable, and unwilling to be examined. He refused to get out of bed even with his family members present. A repeat MRI at this time showed continued meningeal enhancement, enlargement of the previously seen corpus callosal lesion, a new white matter lesion in the right parietal region, and a dark hole in the corpus callosum on the sagittal T1 image (Figures 2A and 2B). Audiometric testing showed profound hearing loss at low and high frequencies, with severe loss at middle frequencies in both ears.
- What is your diagnosis?
- How would you treat this patient?
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