Systemic histiocytosis presenting as multiple sclerosis

Abstract

A patient resembling one with progressive multiple sclerosis in clinical presentation and by magnetic resonance imaging was studied in detail. Some features atypical for multiple sclerosis prompted a persistent search for an alternative cause. The diagnosis of a non-Langerhans systemic histiocytosis involving brain and bone was established and showed a partial response to radiation therapy. This patient illustrates the continued importance of a broad approach to the evaluation of possible multiple sclerosis, with particular attention to atypical features.

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

“…Neuro-psychiatric symptoms (depression, apathy, delirium, psychosis) or cognitive symptoms (dementia, amnesia) were present in 14 patients (21%). Cranial nerves which could have been involved in 2 patients with deafness are reported, one with Parinaud syndrome [2], one with trigeminal neuralgia [48], and another with a vestibular syndrom. Visual symptoms such as blindness or hemianopia and sensory disturbances were also described [2,23,44,46,49].…”

“…Myocardial or pericardial involvement, which could sometimes be severe and be responsible for death, was reported; asymptomatic aortic lesions (''coated aorta'' appearance ) were also described [24,47]. Interstitial lung disease [14], pleural thickening or effusion, orchitis [44], mastitis [11], hepatitis [25,48], pancreatitis [30,43], infiltration of the larynx [30], the parathyroid glands, or the adrenal glands [53] were less frequently described.…”

“…And the third one is composite, associating infiltrative and meningeal lesions (19% of patients). Thus, ECD should be considered as a differential diagnosis of multiple sclerosis, gliomas, meningiomas and other neurological diseases [26,45,48]. Cerebral MRI seems to be the most sensitive imaging procedure in ECD.…”

Erdheim-Chester Disease with Spinal Cord Manifestations

“…The differential diagnosis includes granulomatous disorders, such as sarcoidosis, lymphoma, multifocal fibrosis, multiple sclerosis, and other malignant histiocytosis, particularly Hand-Schüller-Christian disease [2,3,5,8]. Some of the features of Erdheim-Chester disease seem to be quite different from those in Hand-Schüller-Christian disease.…”

“…Extra-axial and intra-axial central nervous system lesions have been described with a variety of focal neurologic signs. These include ataxia with hyperintense foci in the dentate nuclei [4], pseudobulbar signs and incoordination with upper brainstem enhanced MRI lesions [8], mental problems and multiple parasagittal and epidural lesions [9], a choroid plexus lesion [8,9] and a spinal dural infiltrate [3,6]. We describe a new case with symptoms of spinal cord compression.…”