What Is Your Diagnosis?
NeuroQuiz Section Editor: Lawrence S. Honig, MD, PhD, Columbia University, New York, New York.
A 34-year-old man with no prior medical or neurological difficulties presented with a 4-month history of progressive behavioral change, short-term memory loss, fatigue, and weight loss. Initial symptoms included asking strange questions, often repeated, paranoia about others, disrupted sleep, and episodes of déjà vu; there was some question of complex partial seizures, not documented on electroencephalography. Examination revealed a diffuse encephalopathy with impairments in attention, memory, and language, psychotic symptoms, and some gait instability. Cerebrospinal fluid, human immunodeficiency virus antibody, and other laboratory studies were unrevealing. Initial brain magnetic resonance imaging studies reportedly showed nonenhancing lesions in the left temporal/atrial region and bilateral callosum, still evident 4 months after initial onset of symptoms (Figure 1). Findings on brain magnetic resonance angiographic studies were normal (not shown). The atrial lesion also showed T2 shortening consistent with hemosiderin deposition (Figure 2). After prolonged hospitalization and aggressive treatment with psychotropics, he had substantial recovery. However, at month 12, he had recurrence of neurological impairment, with 3 weeks of increasing gait disorder, left-sided motor dysfacility, slurred speech, confusion, and urinary incontinence. Examination confirmed left-sided ataxia and paresis, dysarthria, and encephalopathy. Repeated magnetic resonance imaging with and without contrast showed substantial resolution of the old left atrial lesion (Figure 3), but new lesions were evident bilaterally, including in the brainstem, basal ganglia, and right frontoparietal white matter, which had restricted diffusion (Figure 4). There was some hemosiderin (not shown) in the dominant lesion and contrast enhancement of some of the lesions (Figure 5). Cerebrospinal fluid and laboratory testing was again unyielding. Because of the need for a definitive diagnosis, brain biopsy was performed.
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Correct Answer: Central Nervous System Lymphoma.
What is your diagnosis?
The syndrome is of a remitting and recurrent encephalopathy in a young man with radiological multifocal brain lesions. The first symptomatic lesion in the left atrial region essentially entirely resolved while the patient was receiving only symptomatic treatments. The remitting nature of the disorder makes glioblastoma, metastatic carcinoma, progressive multifocal leukoencephalopathy, or infectious causes quite unlikely. The principal differential diagnosis was between vasculitis, multiple sclerosis, and lymphoma. The fact that the lesions were almost exclusively in white matter is nondiscriminatory among these 3 disorders. The lack of clear cerebrospinal fluid findings such as oligoclonal bands or immunoglobulin changes makes multiple sclerosis less likely, as does the hemorrhagic component of the lesions. While vasculitis could not be completely excluded, the significant remission and near-complete radiological disappearance of the initial dominant left atrial lesion did make this less likely. Central nervous system lymphoma does frequently remit and relapse, and the multifocal contrast-enhancing lesions made this the most likely diagnosis. Pathological analysis confirmed a B-cell lymphoma. Full staging confirmed that this cell population was confined to the central nervous system. The patient responded well to chemotherapy, with substantial resolution of symptoms and lesions and return to normal activities.