Contributed by Pasquale Donnarumma1, Angelo Pichierri1, Roberto Tarantino1, Andrea Gennaro Ruggeri1
Manila Antonelli2, Roberto Delfini1
1Dept. of Neurological Sciences , Institute of Neurosurgery, University "Sapienza", Rome, Italy
2Dept. of Anatomical pathology, University "Sapienza", Rome, Italy
A 72 year-old right handed woman was referred to the Emergency Room of our Department with drug-resistant headache and unstable gait that had begun 7 days earlier. Neurological examination showed psychomotor slowing, wide-based ataxic gait, left homonymous hemianopsia, left-sided spatial neglect and ideomotor apraxia which emerged while putting on her shoes. In her medical history she mentioned hypothyroidism treated with levothyroxine 100mg, moderate hypertension, well-managed with antihypertensive medication, and a 5-year history of systemic lupus erythematosus (SLE), treated with prednisone 25mg. The complete blood count was normal. Lymphocyte count was 0.82×109/L
CT scan performed on admission revealed a white matter hypodensity involving the right parietal, temporal and occipital lobes. An MRI revealed the presence of crescent-shaped tissue with irregular borders in sagittal sequences involving the right parietal temporal and occipital white matter, and the splenium of the corpus callosum. It appeared hypointense in T1 weighted images (Fig. 1) with no gadolinium enhancement, and was hyperintense in FLAIR images. (Fig. 2) The NMR spectroscopy showed an abnormal increase of the Choline/NAA ratio and no lactate (Fig. 3). We performed a neuronavigation-guided brain biopsy. There weren't any complication in the perioperative period.
The histological examination revealed the presence of oligodendrocytes with nuclear swelling hyperchromatic nuclei and "ground-glass" inclusions, highly positive for p53. Macrophage infiltration (CD68+) and reactive astrocytes were also present (Fig. 4). There was relative sparing of axons by neurofilament stain (Fig. 5). A PCR test was diagnostic. What is the diagnosis?