Erdheim-Chester Disease with Spinal Cord Manifestations

Pego-Reigosa, Robustiano; Brañas-Fernández, Francisco; Martínez-Vázquez, F; Rivas-Bande, María José; Sanjuanbenito, L; García-Villanueva, Mercedes; Cortés-Laíño, J A

doi:10.1159/000008166

Cited by 13 publications

(6 citation statements)

References 7 publications

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“…These meningeal lesions could be compressive on adjacent neurological structures such as the brain stem. Six patients presented exclusive spinal cord involvement: in these cases, imaging revealed extramedullary intradural masses which compressed and displaced the spinal cord [3,4,9,25,29,40]. One patient developed cerebral venous thrombosis [42].…”

Section: Resultsmentioning

confidence: 99%

Neurological manifestations and neuroradiological presentation of Erdheim-Chester disease: report of 6 cases and systematic review of the literature

et al. 2006

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Erdheim-Chester disease (ECD) is a rare, non-Langerhans form of histiocytosis of unknown etiology that affects multiple organs. We report 6 cases of ECD with neurological involvement and neuroradiological abnormalities on brain MRI. A literature review revealed 60 other cases of ECD with neurological involvement. We therefore analyzed 66 ECD patients with neurological involvement. Cerebellar and pyramidal syndromes were the most frequent clinical manifestations (41% and 45% of cases), but seizures, headaches, neuropsychiatric or cognitive troubles, sensory disturbances, cranial nerve paralysis or asymptomatic lesions were also reported. Neurological manifestations were always associated with other organ involvement, especially of bones (at least 86%) and diabetes insipidus (47%). Neurological involvement was responsible for severe functional handicaps in almost all patients and was responsible for the death of 6 of the 66 patients (9%). Neuroradiological findings could be separated into three patterns: the infiltrative pattern (44%), with widespread lesions, nodules or intracerebral masses, the meningeal pattern (37%), with either thickening of the dura mater or meningioma-like tumors, and the composite pattern (19%), with both infiltrative and meningeal lesions.

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Section: Resultsmentioning

confidence: 99%

Neurological manifestations and neuroradiological presentation of Erdheim-Chester disease: report of 6 cases and systematic review of the literature

et al. 2006

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“…Radiographic abnormalities are distinctive and always bilateral. The limbs are predominantly involved with diffuse symmetric sclerotic lesions of the diaphyseal and metaphyseal parts of the major long bones, and sparing of the epiphyses (although involvement of the epiphysis has been described in a few cases) [2,4,5,9,10]. Additional lesions can be found in the ribs, sacrum, craniofacial bones and lumbar vertebrae.…”

Section: Discussionmentioning

confidence: 99%

“…Mixed sclerotic and lytic lesions have been observed in approximately one third of the patients [2,4,7]. The extra-skeletal sites reported to be involved are mediastinum or pericardium, heart, lungs, gastrointestinal tract, kidneys, retroperitoneum, central nervous system, skin and orbits [9]. The lung involvement shows a constellation of findings strongly suggesting ECD: smooth interlobular septal thickening, centrilobular nodular opacities, multifocal regions of ground-glass attenuation, fissural thickening and pleural effusions [11].…”

Section: Discussionmentioning

confidence: 99%

“…ECD can range from a focal asymptomatic process to a fatal multisystemic condition [8,9]. It often has a prolonged indolent behaviour.…”

Section: Discussionmentioning

confidence: 99%

“…The literature offers few therapeutic guidelines and there is no established effective treatment for ECD [2,9,14]. By analogy with the treatments used in LCH systemic corticotherapy, vinblastine, doxorubicin, cyclophosphamide, interferon, indomethacin, cyclosporine and localised bone radiotherapy have been attempted; outcomes in the reported cases have been variable.…”

Section: Discussionmentioning

confidence: 99%

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