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

Abstract

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.

Discriminating long myelitis of neuromyelitis optica from sarcoidosis

“…It is more common radiologically and pathologically than pial/leptomeningeal enhancement, which is more characteristic of sarcoidosis‐associated meningitis . Long linear (sometimes nodular) intramedullary enhancement adjacent to cord surface reported previously may be similar to what we describe as subpial . Dorsal subpial enhancement accompanying long myelitis may guide initial investigations.…”

Clinical and MRI phenotypes of sarcoidosis-associated myelopathy

“…Indeed, the coexistence of SAM and cervical spondylosis has been previously noted in a number of small case series. [30][31][32] Collectively, we interpret these observations as suggesting that the inflammation of SAM may have a predilection for areas of the spinal cord susceptible to mechanical stress, potentially providing clues to the pathophysiology of this condition. The impact of chronic compression of the spinal cord has been well studied in cervical spondylotic myelopathy, with chronic mechanical pressure inducing localized tissue hypoxia, persistent disruption of the blood- spinal cord barrier (BSCB), secondary inflammation (sometimes manifesting with mild CSF pleocytosis), microglial activation, and infiltration of peripheral immune cells.…”

Imaging findings in spinal sarcoidosis: a report of 18 cases and review of the current literature

“…The clinical presentation of the 13 SS cases was in concordance with the previously reported studies in the literature. [3][4][5][6][7][8][9][10][11][12][13][14] As reported earlier, there was a female dominance and most patients were in the 35-45 year age group. All the patients in the current study experienced neurological symptoms at the time of the initial sarcoidosis diagnosis, and 92% of patients presented with myelopathic symptoms of sensory, motor and sphincter dysfunction including two patients with characteristic 'corset-like' neuropathy presentation.…”

“…Spinal cord sarcoidosis can occur at any spinal level, although cervical (56%) and thoracic segment (37%) involvement was found to be more common compared to lumbosacral involvement, as reported by previous studies. [3][4][5][6][7][8][9][10][11][12][13][14] The average length of the spinal cord involvement by enhancing intramedullary granulomas was found to be long and spanned for 4.2 vertebral segments, which may be an important clue in differentiating SS from other neurological disease mimics. 3,4,6 Furthermore, axial images may be of importance in localizing the intramedullary granulomas and a propensity for the dorsal cord.…”