Erdheim–Chester disease presenting with an intramedullary spinal cord lesion
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Erdheim–Chester disease (ECD) is a rare form of non-Langerhans histiocytosis characterized by multi-systemic xanthogranulomatous infiltration. The disease commonly affects the skeleton, but may also involve the skin, viscera including the cardiovascular system and lungs, as well as the central nervous system [1, 2, 3, 4]. Diagnosis is based on histopathology typically showing infiltration by histiocytes expressing CD68, but not CD1a . In the nervous system, ECD commonly affects the neurohypophysis resulting in diabetes insipidus. Extrahypophyseal nervous system involvement is seen in about a third of ECD patients and includes intra-axial infiltrative lesions, meningioma-like masses, and periarterial infiltration [3, 5, 6]. Spinal cord involvement may occur due to extramedullary masses [3, 7, 8] but intra-axial cord lesions are rare and only a single patient has been described . We report a case of ECD presenting with progressive spastic paraparesis caused by an...
KeywordsDiabetes Insipidus Multinucleated Giant Cell Spastic Paraparesis Cerebellar White Matter Intramedullary Spinal Cord
Conflicts of interest
The authors declare that they have no competing interests.
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