A 30-year-old right-handed white woman presented to the emergency department with a 2-day history of mild aphasia and apraxia. She was seen by a psychiatrist at that time and was sent home after being evaluated for depression. The patient returned to the emergency department 2 days later because of worsening symptoms; her aphasia and apraxia had increased to the point that it was interfering with her occupation as an accountant. She was feeling confused and having trouble saying and remembering words, and she was having difficulty performing tasks at work that she had performed daily for a couple of years. She had become particularly alarmed when she could no longer manage to use text messaging on her cell phone. Her family history was significant for the presence of MS in 3 family members: her father, a paternal uncle, and a maternal cousin.
In addition to the symptoms already noted, the patient also had symptoms of depression. She denied any change in vision, eye pain, sensory loss, focal or generalized weaknesses, or difficulty with gait or balance. Her vital signs at physical examination included a blood pressure of 137/88 mm Hg, a heart rate of 87 beats per minute, a tympanic temperature of 97.9°F, and a respiratory rate of 16 breaths per minute. She was well nourished and in no acute distress. On auscultation, her heart rate and rhythm were regular without an audible murmur, and both lungs were clear. Her abdomen was soft and nontender with normal bowel sounds in the right lower quadrant.
At examination, the patient was alert and oriented to person, place, and time. Her recent and remote memories were intact, and her attention span and concentration were normal. In testing for aphasia, she demonstrated mild to moderate impairment of fluency and comprehension, but she was able to repeat, recall, and name familiar objects.
A neurologic examination revealed that the patient's cranial nerves II through XII were intact bilaterally. Her deep tendon reflex scores on the left side were 2/4 in the upper extremity and 3/4 for the patellar reflex. The patient also had a positive Babinski sign on the left side. In both upper and lower extremities, she exhibited mild muscle weakness (4/5) on the left side and normal muscle strength (5/5) on the right The neurologic examination findings were otherwise unremarkable.
The laboratory tests ordered for hematologic, metabolic, and liver function testing had normal results. The rapid plasma regain test was nonreactive, and results of the antinuclear antibody test and serologic testing for Lyme disease were normal. The patient's vitamin B
12 level and erythrocyte sedimentation rate were also normal. The MRI ordered at initial presentation and performed the same day showed a concentric lesion with alternating hypoand hyperintense rings corresponding to bands of myelinated and demyelinated tissue, respectively. The concentric patterns seen on MRI resemble the rings of a tree and thus give Balò disease it's hallmark appearance. Given the patient's presentation, her family history of MS, and the pathognomic concentric pattern seen on the MRI results
13 (
Figure 1 and
Figure 2A), it was determined that Balò disease was the most likely diagnosis; this diagnosis was later confirmed by physicians at the Johns Hopkins Hospital, where she went for a second opinion.
After the diagnosis of Balò disease, the patient was treated with intravenously (IV) administered methylprednisolone, 2000 mg/d for 3 days followed by 1000 mg/d for another 6 days. After the first 3 days at 2000 mg/day, she showed substantial improvement in her abstract reasoning and aphasia. This improvement was confirmed by a second MRI study at 4 days after admission that showed resolution of the previously enhanced ring (
Figure 2B). At 9 days, the fluency of the patient's speech had returned to normal, she no longer appeared to be aphasic, and her strength on the left side had returned to baseline. She still showed signs of mild apraxia, but her condition was deemed stable enough for her to be discharged. She was discharged on a treatment regimen of oral steroids (prednisone), with tapering doses over 4 weeks starting at 60 mg.
Neuroimaging studies performed during the first year after initial presentation demonstrated no additional lesions. The patient's latest MRI (
Figure 2C) revealed stabilization of the lesion with no signs of enhancement, and the patient reported that she has not experienced additional flares and was able to return to work shortly after her discharge from the hospital. Because of patient noncompliance, the patient did not follow up for almost 1 year after discharge from the hospital. However, at her latest physical examination, her neurologic deficits were nearly completely resolved.