Teaching NeuroImages: Rosai-Dorfman disease presenting with progressive early-onset cerebellar ataxia

A 13-year-old girl presented with a 3-year history of progressive gait abnormality. She recently had a self-limited cervical lymphadenopathy. Neurologic examination showed brisk tendon reflexes and moderate ataxia. Brain MRI disclosed hyperintense lesions in the cerebellum and pons (figure, A). Spin...

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Veröffentlicht in:Neurology 2013-07, Vol.81 (5), p.e27-e28
Hauptverfasser: Candeias da Silva, Carolina, Pedroso, José Luiz, Moraes, Fabiano Moulin de, Rivero, René Leandro M, Callegari, Fabiano Mesquita, Araujo, Francisco, Toso, Fabio Fieni, Stávale, João Norberto, Barsottini, Orlando Graziani Povoas
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container_end_page e28
container_issue 5
container_start_page e27
container_title Neurology
container_volume 81
creator Candeias da Silva, Carolina
Pedroso, José Luiz
Moraes, Fabiano Moulin de
Rivero, René Leandro M
Callegari, Fabiano Mesquita
Araujo, Francisco
Toso, Fabio Fieni
Stávale, João Norberto
Barsottini, Orlando Graziani Povoas
description A 13-year-old girl presented with a 3-year history of progressive gait abnormality. She recently had a self-limited cervical lymphadenopathy. Neurologic examination showed brisk tendon reflexes and moderate ataxia. Brain MRI disclosed hyperintense lesions in the cerebellum and pons (figure, A). Spine MRI showed a heterogeneous lesion in the T12 vertebra (figure, B). Histopathology of the cervical lymph node confirmed Rosai-Dorfman disease (RDD) by showing emperipolesis (figure, C and D). The patient will be followed up in order to determine disease progression and therapy. RDD is a rare autoimmune histiocytic proliferative disorder first recognized in 1969. The CNS is involved in 5% of cases and generally mimics meningiomas. Bone erosion can be detected in the spine. Herein, we describe a rare CNS manifestation of RDD resembling a neurodegenerative ataxia.
doi_str_mv 10.1212/WNL.0b013e31829d85d2
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She recently had a self-limited cervical lymphadenopathy. Neurologic examination showed brisk tendon reflexes and moderate ataxia. Brain MRI disclosed hyperintense lesions in the cerebellum and pons (figure, A). Spine MRI showed a heterogeneous lesion in the T12 vertebra (figure, B). Histopathology of the cervical lymph node confirmed Rosai-Dorfman disease (RDD) by showing emperipolesis (figure, C and D). The patient will be followed up in order to determine disease progression and therapy. RDD is a rare autoimmune histiocytic proliferative disorder first recognized in 1969. The CNS is involved in 5% of cases and generally mimics meningiomas. Bone erosion can be detected in the spine. 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subjects Adolescent
Diagnosis, Differential
Disease Progression
Female
Histiocytosis, Sinus - complications
Histiocytosis, Sinus - diagnosis
Humans
Spinocerebellar Degenerations - diagnosis
Spinocerebellar Degenerations - etiology
title Teaching NeuroImages: Rosai-Dorfman disease presenting with progressive early-onset cerebellar ataxia
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