A case of sarcoidosis presenting as a non-specific intramedullary lesion

European Journal of Neurology 2007, 14: 346–349

doi:10.1111/j.1468-1331.2006.01527.x

SHORT COMMUNICATION

A case of sarcoidosis presenting as a non-specific intramedullary lesion D. Caneparoa, C. Lucettia, A. Nutib, G. Ciprianib, C. Tessac, P. Fazzid and U. Bonuccellia a

Section of Neurology, Department of Neuroscience, University of Pisa, Pisa, Italy; bNeurology Unit, and cRadiology Unit, USL of

Viareggio, Italy; and dPneumology Section, Cardiothoracic Department, University of Pisa, Pisa, Italy

Keywords:

intramedullary lesions, neurosarcoidosis Received 20 December 2005 Accepted 1 March 2006

Neurosarcoidosis occurs in 5–15% of sarcoidosis cases. Approximately 50% of patients with neurosarcoidosis present with a neurological disease at the time sarcoidosis is first diagnosed. Spinal sarcoidosis is rare. We report the case of a 61-year-old man with a highly aspecific intramedullary lesion as the first manifestation of sarcoidosis. One year after the onset of neurological symptoms, the high levels of angiotensinconverting enzyme and the results of a total body gallium scan and bronchoalveolar lavage supported the diagnosis of sarcoidosis. Isolated single reports indicate that spinal neurosarcoidosis may be the initial manifestation of sarcoidosis. In our case, magnetic resonance imaging of the dorsal spine showed a largely aspecific lesion. Neurosarcoidosis should be considered in the differential diagnosis of intramedullary cord lesion with leptomeningeal enhancement; a systematic search for evidence of sarcoidosis should be mandatory in all cases for a correct diagnosis and early treatment.

Introduction

Case report

Sarcoidosis is a multisystem granulomatous disease of unknown origin, affecting multiple organs; the pathological hallmark is non-caseating epithelioid granulomas. The prevalence of neurological involvement is estimated to be about 5–15% of sarcoidosis cases [1,2]. The diagnosis of neurosarcoidosis is often difficult, especially in patients without any systemic manifestation. Neurosarcoidosis may manifest itself in many different ways, and can appear in an acute fashion or as a slow, progressive disease. Furthermore, neurosarcoidosis can affect any part of the nervous system and almost any neurological symptom could by caused by neurosarcoidosis [3]. Patients with neurosarcoidosis have neurological manifestations at the time sarcoidosis is first diagnosed in the 50% of cases. In the remainder of patients, the neurological manifestations appear within 2 years of the diagnosis of sarcoidosis [4]. Spinal sarcoidosis is relatively uncommon, occurring in 3.5 in the BAL provides a diagnosis of sarcoidosis with a specificity of 94% [15]. The results of the Ga-67 scan confirmed the diagnosis of sarcoidosis [16–18]. Histological confirmation is needed for a diagnosis of definite neurosarcoidosis [8], but our patient, considering the high risk of the surgical procedure, refused a spinal cord biopsy. Single case reports of patients undergoing spinal cord biopsy in intramedullary neurosarcoidosis suggest high morbidity and mortality [5,19]; moreover, spinal cord biopsy may prove to be negative in intramedullary neurosarcoidosis [10] as spinal cord granulomas may be poorly formed, smaller than the systemic granulomas and have fewer giant cells [20,21]. Clinical and laboratory investigation of a large group of patients with neurosarcoidosis followed up for at least 5 years showed that neurological involvement at onset is associated with a poor outcome and a less favourable course of the disease [22]. However, in spinal sarcoidosis, steroid therapy may be associated with a favourable outcome [10]. Even in the absence of systematic symptoms of sarcoidosis, a high index of diagnostic suspicion is required in patients with an intramedullary spinal lesion to start early steroid therapy. In our case, prednisone treatment led to a significant amelioration of clinical and neuroradiological findings. Our report indicates that in cases of intramedullary spinal lesion with leptomeningeal

Ó 2007 EFNS European Journal of Neurology 14, 346–349

Intramedullary sarcoidosis

involvement, a systematic search for evidence of sarcoidosis should be mandatory in all cases for a correct diagnosis and early treatment.

12. 13.

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