Novel presentation of Rosai-Dorfman histiocytosis with a prolonged course of cranial and peripheral neuropathies

Publication date: Available online 8 March 2017
Source:Pediatric Neurology
Author(s): Richa Tripathi, Fatema Serajee, H. Jiang, A.H.M.M. Huq
IntroductionRosai-Dorfman disease is a form of histiocytosis affecting the systemic lymph nodes. Intracranial Rosai-Dorfman disease is very rare and presents with extra or intraparenchymal proliferative mass lesions. Cranial neuropathy except mass effect by surrounding lesion has not been reported in Rosai-Dorfman disease.MethodsWe describe a case of Rosai-Dorfman Disease presenting with peripheral as well as multiple cranial neuropathies. Detailed clinical, immunological, neurophysiology, imaging and genetic studies were performed.ResultThe patient had a prolonged course but recovered fully after immune therapies. She had elevated titers of striated muscle and smooth muscle antibodies. Imaging studies revealed contrast enhancement of cranial nerves and striated muscles. Demyelination was evident in the nerve twigs from muscle biopsy. Exome sequencing did not reveal any genetic mutations.ConclusionMost patients with Rosai-Dorfman disease have a benign course. But severe neurological complication with bulbar involvement and cranial and peripheral neuropathy may occur. Treatment with Immunoglobulin and steroids may be of benefit.



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