A case of sarcoidosis presenting as a non-specific intramedullary lesion
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European Journal of Neurology 2007, 14: 346–349
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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
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.
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 . 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 . Spinal sarcoidosis is relatively uncommon, occurring in 3.5 in the BAL provides a diagnosis of sarcoidosis with a speciﬁcity of 94% . The results of the Ga-67 scan conﬁrmed the diagnosis of sarcoidosis [16–18]. Histological conﬁrmation is needed for a diagnosis of deﬁnite neurosarcoidosis , 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  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 . However, in spinal sarcoidosis, steroid therapy may be associated with a favourable outcome . 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
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involvement, a systematic search for evidence of sarcoidosis should be mandatory in all cases for a correct diagnosis and early treatment.
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