Contributed by Ting-Ting Zhang1,2, Yong-Juan Fu1, Yue-Shan Piao1, Guang-Zhi Liu3, Lei-Ming Wang1, Shi-Yun Chen1, De-Hong Lu1, Xu-Guang Gao3
1Department of Neuropathology, Xuanwu Hospital, Capital Medical University, Beijing, China
2Department of Pathology, Chinese People's Liberation Army Fuzhou General Hospital, Fujian, China
3Department of Neurology, Peking University People's Hospital, Beijing, China
A previously healthy 43-year-old woman first suffered from headache with dizziness, vomiting, and nausea beginning 5 months before hospital admission. CT scans showed subarachnoid hemorrhage involving the right temporal and occipital lobes, while MRI revealed abnormal signals in the left thalamus and right temporal-occipital lobes. Five months later she suffered from headache, transient unconsciousness and aggravated left upper-limb paralysis. This time, neurological examinations revealed a decrease in power and deep tendon reflexes of the left upper limb. The activated partial thromboplastin time was notably prolonged at 38.8s, while the prothrombin time was normal. Cerebrospinal fluid (CSF) contained 100 erythrocytes /mm3. Neuroimaging revealed infarcts and hemorrhages in bilateral frontal lobes (Fig. 1a) and slit-like complex softening of the right temporal-occipital lobes (Fig. 1b). MRI showed diffuse enhancement in the dural mater and sulci of bilateral frontal-parietal lobes and tentorium (Fig. 1b).MR venography disclosed occlusions in the right transverse and sigmoid sinus, suggesting thrombosis (Fig. 1c). Five days after admission, the patient died and a restricted cranial autopsy was performed.
Macroscopically, bilateral uncal herniation (right > left) and cerebellar tonsillar herniation were found. On coronal section, the brain tissues (especially on the right) were swollen and dark brown. The midline was shifted to the left, and the ventricles were compressed (right > left) (Fig. 1d). Fresh petechial hemorrhages were present in the right midbrain along the midline and pontine base near the middle cerebellar peduncle, which was called Duret hemorrhages. Thrombi filled in the dural sinuses, especially in the superior sagittal, right transverse and right sigmoid sinuses.
Microscopic observation revealed widespread histiocyte proliferation, with infiltration of varying amounts of lymphocytes, plasma cells, and eosinophils in the subarachnoid space (Fig. 1e), part of the Virchow-Robin space of the parenchyma, cerebral parenchyma and even arachnoid granulations and choroid plexus. Some of the histiocytes contained lymphocytes, plasma cells, and occasionally neutrophils and erythrocytes in their cytoplasm (Fig. 1f). Immunohistochemically, these histiocytes were positive for CD68, MAC387, S-100, CD11c (Fig. 1g), and lysozyme, but negative for CD1α.
Surprisingly, histiocyte proliferation and inflammatory cell infiltration were noted in the inner surface of the sinus lumen as well as in the chordae in the lumen of the dural sinuses (Figs. 1h and 1i). The latter showed severe hyperplasia, particularly in the superior sagittal sinus, torcular herophili, and right transverse sinus (Fig. 1i). Moreover, thrombi filled the lumen of the dural sinus adhering to the chordae. The thrombi in the anterior part of the superior sagittal sinus were relatively old and undergoing organization, while fresh thrombi were noted in the middle and posterior parts of the superior sagittal sinus, torcular herophili, right transverse sinus, and bilateral sigmoid sinuses, suggesting a long course of thrombosis in different stages. Additionally, thrombosis was noted in the veins of the subarachnoid space and brain parenchyma of the bilateral frontal and parietal lobes (Fig. 1j), with vessel wall disruption in some venulae. What is your diagnosis?