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Clinical Pathologic Conference | October 15, 2012 ONLINE FIRST

A 62-Year-Old Man With Fluctuating Neurological Deficits and Skin Lesions ONLINE FIRST

John J. Konikkara, MD; Osvaldo H. Perurena, MD; Steven Warach, MD, PhD; Steven C. Bauserman, MD

Arch Neurol. 2012;():1-5. doi:10.1001/archneurol.2013.469.

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ABSTRACT

A 62-year-old man with no significant medical history experienced fatigue, night sweats, hoarseness of voice, and dry cough, which were followed by vision disturbances in his left eye. He lost about 4.5 kg (10 lb) in just over a month. Three weeks later, he had difficulty recollecting his e-mail password and trouble with word finding. The next day, he experienced numbness in his left arm. He also developed a maculopapular and erythematous rash in the groin, genitalia, and buttocks. The results of an initial neurological examination were normal, including his higher mental functions. An initial blood workup revealed normocytic normochromic anemia. The results of cerebrospinal fluid studies were unremarkable. Magnetic resonance imaging of his brain at hospital admission revealed multifocal increased T2 signals in the subcortical white matter. A conventional cerebral angiogram was unremarkable. A biopsy specimen from the right frontal lobe revealed demyelination and perivascular lymphocytic infiltration. A provisional diagnosis of acute disseminated encephalomyelitis was made. In spite of steroid treatment and plasmapheresis, his clinical status deteriorated rapidly. The approach to the diagnosis of a rapidly progressive multifocal brain disorder is discussed.

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Figure 1. Magnetic resonance imaging scans of the brain of a 62-year-old man that show multifocal increased T2 signals in the subcortical white matter (A), restricted diffusion in diffusion-weighted sequences (B), hypointense lesions in the gradient echo sequences (C), and a subtle contrast-enhancing lesion in the left temporal lobe (D). A biopsy of the right frontal lobe shows demyelination (E; Luxol fast blue/PAS stain for myelin with hematoxylin-eosin [original magnification ×10]) and perivascular lymphocytic infiltration (F; hematoxylin-eosin [original magnification ×20]).

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Figure 2. Low-power (A; hematoxylin-eosin [original magnification ×10]) and high-power (B; hematoxylin-eosin [original magnification ×40]) microscopy of the autopsy specimen revealing intravascular lymphoid cells with marked cytological atypia and some endothelial proliferation. Immunohistochemical staining of B lymphocytes in the autopsy specimen for CD20, CD79a (C; original magnification ×40), and PAX5 (D; original magnification ×40) cellular markers is shown. Areas of recent ischemic necrosis, hemorrhage (E; hematoxylin-eosin [original magnification ×10]), and spongiosis (F; hematoxylin-eosin [original magnification ×10]) can be seen in the autopsy specimen.

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Konikkara JJ, Perurena OH, Warach S, Bauserman SC. A 62-Year-Old Man With Fluctuating Neurological Deficits and Skin Lesions. Arch Neurol. 2012;():1-5. doi:10.1001/archneurol.2013.469.

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