[Rosai-Dorfman disease accompanying hypertrophic cranial pachymeningitis with an early symptom of right peripheral facial palsy].

We experienced a rare case of sinus histiocytosis with massive lymphoadenopathy (Rosai-Dorfman disease) accompanying hypertrophic cranial pachymeningitis. The patient is a 64-year-old woman with an early symptom of rt. peripheral facial palsy. She had a 4-month history of headache with a 5-week history of numbness in the rt. supraorbital nerve area, and lost her weight by 10 kg in 2 months. She developed rt. trochlea nerve palsy and numbness in the lt. mandibular nerve area. Laboratory findings showed that ALP, LDH and CRP were higher than normal. Of CT, MRI and MRA, the images of her head were normal. However, the Gd-enhanced MRI only showed a diffuse pachymeningeal enhancement. After about 3 months from the onset of rt. peripheral facial palsy, she died of DIC of unknown etiology. As a result of examinations in anatomical pathology, she was diagnosed as having sinus histiocytosis with massive lymphoadenopathy (Rosai-Dorfman disease). There were a large number of histiocytes on the pachymeninx. These findings suggest that hypertrophic cranial pachymeningitis caused multiple cranial neuropathy. We emphasize that use of Gd-enhanced MRI in the early stage is important for diagnosis.