Contributed by Hidehiro Takei, M.D.1,2, J. Clay Goodman, M.D.1,2, Suzanne Z. Powell, M.D.2
Department of Pathology and Laboratory Medicine, 1Baylor College of Medicine and 2The Methodist Hospital, Houston, TX, USA
CLINICAL HISTORY AND NEURORADIOLOGY:
The patient was a 31-year-old African-American female admitted for evaluation and treatment of leg weakness. Neurological examination revealed markedly decreased strength in bilateral hip flexion (right: grade 1/5, left: 2/5) and left knee flexion (grade 3/5) as well as extension (grade 2/5), hyperesthesia in the bilateral ankles and feet, and decreased sensation to light touch on the left leg. Her past medical history was significant for end-stage liver disease secondary to autoimmune hepatitis, status post orthotopic liver transplant in December 2004, fungal meningitis in 2003, and diabetes mellitus (type 2). Approximately one month after liver transplantation, the patient underwent placement of a ventriculoperitoneal (VP) shunt for a diagnosis of acute communicating hydrocephalus.
The spinal magnetic resonance imaging (MRI) with contrast demonstrated diffuse abnormal enhancement throughout the spinal leptomeninges (Figure 1). The spinal cord was compressed both anteriorly and posteriorly (Figure 1), which was the greatest at the level of T12 vertebral body (Figure 1, 2). The patient underwent a thoracic laminectomy and decompression (removal of the intradural mass) at the T11 and 12 levels, and was begun on amphotericin B. Her neurological symptoms improved and she was discharged.
Grossly, the mass consisted of a 3.3x0.7x0.2 cm, roughly elliptical, tan-gray fragment of soft tissue. Microscopically, multiple cross sections, stained with routine hematoxylin-eosin stain, showed multiple epithelioid and giant cell granulomata with central suppuration and necrosis. The background was diffusely fibrogliotic (Figure 3, 4). The granulomata contained multiple large round spherules filled with endospores (Figure 5: Inset: high magnification of a spherule). There are some multinucleated giant cells phagocytizing the microorganisms. The microorganisms were highlighted with Gomori's Methenamine-Silver (GMS) (Figure 6) and PAS stains (Figure 6, Inset).