43 Year Old Woman with Left Arm Paralysis

Clinical Summary

A previously healthy 43‐year‐old woman first presented 5 month history of headache and dizziness. CT scans showed subarachnoid hemorrhage involving the right temporal and occipital lobes. Five months later she suffered from headache, transient unconsciousness and aggravated left upper‐limb paralysis. The left upper limb had decreased power. The activated partial thromboplastin time was notably prolonged at 38.8s, while the prothrombin time was normal. CSF contained 100 erythrocytes /mm³. Neuroimaging revealed infarcts and hemorrhages in bilateral frontal lobes (Figure 1a) and slit‐like complex softening of the right temporal‐occipital lobes (Figure 1b). MRI showed diffuse enhancement in the dural mater and sulci of bilateral frontal‐parietal lobes and tentorium (Figure 1b). MR venography disclosed occlusions in the right transverse and sigmoid sinus (Figure 1c). Five days later, the patient died.

Figure 1.

 

Neuropathological Findings

The brain was edematous with uncal and tonsillar herniation. The ventricles were compressed (Figure 1d). There were Duret hemorrhages. The superior sagittal sinus (SSS), right transverse and sigmoid sinuses were thrombosed.

Microscopy revealed widespread histiocyte proliferation, with some lymphocytes, plasma cells, and eosinophils in the subarachnoid space (Figure 1e), the Virchow‐Robin spaces and the 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 (Figure 1f). The histiocytes were positive for CD68, MAC387, S‐100, CD11c (Figure 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 (Figures 1h and 1i). The latter showed severe hyperplasia, particularly in the SSS, torcular herophili, and right transverse sinus (Figure 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 SSS, torcular herophili, right transverse sinus, and bilateral sigmoid sinuses, suggesting a long course of thrombosis in different stages. There were venous infarcts in the bilateral frontal and parietal lobes (Figure 1i). What is your diagnosis?

Diagnosis

CNS Rosai‐Dorfman disease with extensive meningeal and parenchymal involvement, and thrombosis in the venous sinuses followed by secondary multiple hemorrhagic infarcts.

Discussion

Rosai‐Dorfman disease (RDD) was first described as “sinus histiocytosis with massive lymphadenopathy” by Rosai and Dorfman in 1969.1 The extranodal form occurs in over 40% of patients2 and central nervous system (CNS) involvement is extremely uncommon in these patients.3 Most of the reported cases had solitary or multiple dural‐based lesions.4

Two parts of specialty make this case meaningful. Firstly, in our report the lesions were diffuse and involved not only the dura but also the arachnoid, subarachnoid space, and pia mater. Lesions also developed in the parenchyma of both cerebrum and cerebellum, as well as choroid plexus in the posterior horn of the lateral ventricle. Furthermore, the lesions invaded the venous sinuses, followed by thrombosis and organization, which has not been reported previously.

Secondly, we found fibrous proliferated chordae with inflammatory infiltration in the venous sinuses. It is unclear whether these hyperplastic chordae are congenital abnormal formations or an outcome resulting from the inflammatory cell infiltration caused by RDD. However, we speculate that these abnormally thickened chordae might tremendously decrease the blood flow in the venous sinuses, thus contributing to the thrombosis. CNS RDD may also generate thrombosis, though uncommonly.

In the literature, only one RDD case reported extensive dura and arachnoid mater involvement.5 In that report, the patient presented with increased chronic intracranial pressure and progressive bilateral visual and hearing impairments. MRI showed diffuse dura involvement and dissemination of the orbit and spine. The CSF revealed persistent high lymphocytic pleocytosis, which led to a worse prognosis. Our case also had abnormal CSF results with excessive erythrocytes. Another case reported subarachnoidal involvement with a normal CSF index that later showed stable.4 Therefore, we speculate that the CSF may be used as another auxiliary method for diagnosing and assessing the prognosis of CNS RDD.