Contributed by Zuhha Ashraf MD, Nathaniel Todnam MD, John Morgan MD, Amyn M Rojiani MD, PhD
Departments of Pathology, Neurology and Neurosurgery; Medical College of Georgia at Augusta University and Charlie Norwood Veterans Medical Center, Augusta, GA
A 42 year old man with history of asthma, sinusitis and GERD presented to the critical care unit with a 6 day history of worsening headache, nausea, and vomiting. He reported that the symptoms began with a "popping" sensation after a bout of coughing and fever, although he was afebrile on admission. Neurological exam revealed that he was sleepy, but easily arousable and oriented x 3 with a non-focal motor exam. CT scan showed a large right temporal-occipital hyperdense lesion with surrounding edema suggestive of an acute/subacute hemorrhage and a 4-5mm associated midline shift with no evidence of hydrocephalus. On day 2 he developed a 4/5 left sided hemiparesis, dysarthria, and left hemifacial weakness. MRI of brain demonstrated a large right temporal-occipital intraparenchymal hemorrhage, subtle leptomeningeal enhancement, an increased T2 signal of the brainstem especially within the right pons, and a partial thrombosis of the right transverse and sigmoid sinuses. There was no evidence of underlying tumor or vascular malformations. He developed a worsening of the left hemiparesis (2/5) and was started on heparin anticoagulation for a suspected venous infarct, secondary to the venous sinus thrombosis. On hospital day 4 he was noted to be more alert with improvement in his speech. Repeat MRI showed recanalization of the transverse sinus and stable abnormalities in the brainstem and right temporal-occipital lobes. He remained awake, alert, and oriented with some improvement in his hemiparesis. On day 8 he had a sudden neurological decline, became hemodynamically unresponsive and ultimately expired despite aggressive medical management.
There was moderate diffuse cerebral edema, with uncal herniation and bilateral cerebellar tonsillar herniation, worse on the right. There was a right sided hemorrhagic lesion which extended from the level of the right posterior hippocampus to the tip of the right occipital lobe. The lesion consisted of multiple partially connected areas of recent hemorrhage within what appeared to be necrotic tissue measuring approximately 5.0 x 4.0 cm in greatest area. The lesions were predominantly within the white matter although involvement of the gray matter was also noted. A similar hemorrhagic lesion was seen in the pontine base with extension to the tegmentum bilaterally, more prominent on the right and measuring 2.8 x 2.0 cm, involving the entire thickness of the pons (c). There were extensive areas of coalescence of hemorrhagic lesions. The remainder of the uninvolved cerebral cortical ribbon, cerebral white matter, and basal ganglia were unremarkable. Microscopically, the lesions were predominantly within white matter although gray matter extensions were also noted. There were multiple hemorrhages, often with a perivenular, ring and ball pattern (d). Multiple blood vessels, predominantly venules, displayed fibrinoid necrosis accompanied by neutrophilic infiltrates and necrosis of adjacent tissue (e). Myelin stains defined the necrotic and hemorrhagic lesions within white matter with relative preservation of tissue adjacent to the lesions (f). Additionally there was no evidence of hypoxic injury seen in uninvolved areas of the brain. Silver stain (Bielschowsky) defined the areas of white matter destruction and highlighted large numbers of axonal bulbs and spheroids, as well as neuritic fragments (g). What is your diagnosis?