We report a case of pathology-proven acute disseminated encephalomyelitis (ADEM) in which the patient’s symptoms were solely cognitive. Although cognitive dysfunction is a well-recognized symptom in adults with multiple sclerosis, cognitive assessment of adults with ADEM has rarely been reported. A 35-year-old woman was referred to our center for evaluation of cognitive disturbance and demyelinating lesions seen on brain magnetic resonance imaging (MRI). We performed a neurologic examination, full neuropsychological assessment, brain MRI, blood and cerebrospinal fluid analyses, visual evoked potentials, and brain biopsy. The patient’s Mini-Mental State Examination score was 26/30. Cognitive assessment revealed multiple severe dysfunctions, mainly in executive and attention tasks. She scored below the normal range on the Digit Span Forward and Backward Test and the Trail Making Test Part B. The Frontal Assessment Battery showed deficits in mental flexibility, motor programming, and inhibitory control. She also scored in the impaired range on tests of verbal fluency and memory. The brain MRI and biopsy confirmed a diagnosis of ADEM. This case report points to the limitations of relying on clinical presentation, neuroimaging, and current controversial diagnostic criteria in diagnosing ADEM in adults, and highlights the essential role of pathologic evaluation.
*Department of Neurology, University Hospital Center Zagreb, Referral Center for Demyelinating Diseases of the Central Nervous System
†Department of Neurology, University Hospital Center Zagreb, Referral Center for Cognitive Neurology and Neurophysiology
‡School of Medicine, University of Zagreb
Departments of §Pathology
∥Radiology, University Hospital Center Zagreb, Zagreb, Croatia
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Authors’ contributions: Study concept and design: I.A. and M.H. Data acquisition, analysis, and interpretation: all authors. Drafting of manuscript: M.H. Critical revision of manuscript content: all authors. Administrative, technical, and material support: all authors.
The authors declare no conflicts of interest.
Reprints: Mario Habek, MD, PhD, Department of Neurology, University Hospital Center, Kišpatićeva 12, HR-10000 Zagreb, Croatia (e-mail: firstname.lastname@example.org).
Received March 18, 2012
Accepted July 3, 2012