Subacute myelopathy as the presenting manifestation of sarcoidosis

Bogousslavsky, Julien; Hungerbühler, J P; Régli, F; Graf, Hansjörg

doi:10.1007/bf01405845

Cited by 41 publications

(18 citation statements)

References 18 publications

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“…Although the clinical phenotype varies depending on the geographical region and ethnicity, both sexes and all ethnicities and ages are affected globally. In comparison to that observed in Western countries, Japanese sarcoidosis has been 2042 ment is an extremely rare manifestation of sarcoidosis, occurring in 0.34% of cases of systemic sarcoidosis (3). In Japan, the incidence of nervous system involvement has been reported to be 7.2% in patients with sarcoidosis (1); therefore, making the initial diagnosis of spinal cord sarcoidosis is challenging due to the rarity of the disease, particularly when systemic findings of the disease are unclear (4).…”

Section: Introductionsupporting

confidence: 41%

Spinal Magnetic Resonance Imaging Manifestations at Neurological Onset in Japanese Patients with Spinal Cord Sarcoidosis

2013

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Objective We retrospectively investigated spinal magnetic resonance imaging (MRI) manifestations at neurological onset in Japanese patients with spinal cord sarcoidosis. Methods Between July 2000 and April 2012, we reviewed our database and recruited patients with spinal cord sarcoidosis. On spinal MRI performed at neurological onset, the following items were evaluated: the vertebral-segment distribution and length of intramedullay T2-elongated lesions, abnormal enhancement patterns and distributions and the concomitant presence of spondylosis and associated extraspinal lesions. If available, brain MRI scans were concomitantly assessed. Results Nine patients were enrolled (four men and five women; median, 49 years). Reflecting Japanese epidemiological backgrounds, a predilection for occurrence was observed in young men and middle-aged women. Intramedullary T2-elongated lesions were present in eight patients, peaking at the C5 level, with a mean length of 3.7±2.6 vertebral segments. Spondylosis coexisted in the middle-aged patients. Abnormal intramedullary enhancement with concomitantly involved the nerve roots was observed in six patients, comprised of two types reflecting the disease progression: linear-and/or nodular enhancement along the surface of the spinal cord and intramedullary enhancement consisting of patchy, broad-based enhancement adjacent to the cord surface. Five patients had associated extraspinal lesions, including lymphadenopathy in four patients and brain involvement in four patients. Conclusion Spinal cord sarcoidosis exhibits a predilection for young men and middle-aged women among Japanese individuals and is characterized by intramedullary T2-elongated lesions spreading more than three vertebral segments peaking at the C5 level, two types of abnormal intramedullary enhancement reflecting disease progression, frequent nerve root involvement and lymphadenopathy.

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Section: Introductionsupporting

confidence: 41%

Spinal Magnetic Resonance Imaging Manifestations at Neurological Onset in Japanese Patients with Spinal Cord Sarcoidosis

2013

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“…However, as shown in this study, the effects of laminoplasty are not predictable in the treatment of spinal cord sarcoidosis even when patients have spondylotic changes and/or a constitutionally narrowing cervical spinal canal. It has been reported that a long interval from the onset of disease to the initiation of corticosteroid therapy contributes to poor functional recovery [1]; however, poor results were achieved in all our three patients even with the rapid administration of corticosteroid therapy. Thus, laminoplasty is not an effective intervention for the treatment of spinal cord sarcoidosis with or without spondylotic changes.…”

Section: Discussionmentioning

confidence: 52%

“…About 6-8% of cases with central nervous system involvement show spinal cord lesions [10,23]. Spinal cord involvement is rare, occurring in only 0.3-0.4% of patients with systemic sarcoidosis [1,26]. Thus, intramedullary neurosarcoidosis is a rare neurological manifestation, especially as an initial presentation [10].…”

Section: Introductionmentioning

confidence: 99%

Is extensive cervical laminoplasty an effective treatment for spinal cord sarcoidosis combined with cervical spondylosis?

Doita

Miyata

et al. 2009

Eur Spine J

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Patients with neurosarcoidosis are usually initially treated with steroid administration even when they have concomitant cord compression on magnetic resonance imaging (MRI). Operative intervention may be indicated in patients with spinal cord sarcoidosis requiring either tissue biopsy for diagnosis or associated with progressive neurologic symptoms. However, there have been no previous reports describing clinical outcomes of laminoplasty for spinal cord sarcoidosis. The objectives of this study are to investigate whether extensive cervical laminoplasty is an effective treatment for spinal cord sarcoidosis combined with spondylotic changes and/or cervical spinal canal stenosis. Open-door laminoplasty was performed in three patients with spinal cord sarcoidosis. All patients received intensive corticosteroid therapy after the operation MRI imaging was performed in all patients before and after the operation. Operative outcomes were not satisfactory and the clinical courses of the patients fluctuated after corticosteroid therapy. Daily life activities were not significantly improved after treatments in any of the three patients, and in the long-term follow-up period the clinical course of one patient was one of inexorable deterioration to a state of quadriplegia. The possibility of spinal cord sarcoidosis should be included in the differential diagnosis, when a distinct high signal intensity area is observed within the spinal cord on T2-weighted MR images in patients with spondylotic changes. Laminoplasty is not an effective intervention for the treatment of spinal cord sarcoidosis even when patients have spondylotic changes and/or a constitutionally narrowing cervical spinal canal. Patients with neurosarcoidosis should be treated first with steroid administration even when they have concomitant cord compression on MRI.

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“…Spinal cord involvement is extremely rare, occurring in only 0.3-0.4% of patients with systemic sarcoidosis [9][10][11] . Moreover, most spinal sarcoid lesions are intramedullary, only a few cases of extramedullary sarcoid lesions have been reported [12][13][14][15] .…”

mentioning

confidence: 44%