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

Abstract

Spinal sarcoidosis, referring to involvement of the spine in sarcoidosis, is relatively rare and may mimic other neurological disease affecting the spine. The authors present a clinic radiological review of 18 spinal sarcoidosis patients who presented to a tertiary hospital, with emphasis on initial imaging and radiological response to treatment. Materials and methods: We retrospectively reviewed our departmental imaging archives over a 15-year period and found 49 cases of neurosarcoidosis out of which 18 patients had spinal magnetic resonance imaging. Results: Approximately 72% (13/18) of the neurosarcoidosis patients showed some form of spinal involvement. The clinical, epidemiological and imaging data were reviewed for these 13 patients at presentation and follow-up. The findings on magnetic resonance imaging included leptomeningeal enhancement (61%), pachymeningeal (23%), intramedullary enhancing lesions (38%) and bony involvement (15%). The cervical segment was most frequently involved followed by the thoracic segment. Involvement was often long segment (4.2 spinal segments) with proclivity for the dorsal cord. Mean follow-up was 23.2 months. A complete or near-complete radiological response occurred in 66% while partial response was seen in 25% patients. Four patients had isolated central nervous system involvement including one with isolated spinal cord involvement. On diffusion-weighted imaging, the apparent diffusion coefficient of intramedullary lesions was increased compared to normal-appearing cord on baseline and subsequent follow-up scans. Conclusions: Spinal sarcoidosis was previously considered uncommon but is being increasingly recognized with widespread use of magnetic resonance imaging. Proclivity for dorsal surface involvement is characteristic, although not necessarily pathognomonic. Also, quantitative diffusion studies may serve as a biomarker for the disease activity and parenchymal injury.

<p>Intramedullary spinal cord involvement: a rare presentation of sarcoidosis</p>

“…Our patient had stage 2 and 3 disease with multifocal patchy enhancement and cord edema. Diagnosis is challenging when spinal sarcoidosis presents without overt systemic complaints, as observed in our patient 10. Patients with neurosarcoidosis also have a high frequency of pulmonary involvement, and hence chest X-rays can be taken as part of the initial work-up 4.…”

“…Most common is leptomeningeal enhancement, followed by pachymeningeal and then intramedullary enhancing lesions 9. MRI findings in spinal sarcoidosis can be divided into four stages: 1) leptomeningeal linear enhancement; 2) intramedullary enhancing lesions with cord enlargement due to spread of inflammation to parenchyma through the perivascular spaces; 3) normal size cord with focal or multifocal enhancement; and 4) spinal atrophy and no enhancement 10. Our patient had stage 2 and 3 disease with multifocal patchy enhancement and cord edema.…”

Neurosarcoidosis

“… 4 , 27 Characteristic imaging findings include nodular and linear leptomeningeal contrast enhancement associated with intraparenchymal T2 hyperintensity. 30 , 31 Longitudinally extensive myelitis (LETM; ≥3 vertebral segments) from sarcoidosis is relatively common (75% of NS myelitis in 1 series) 30 and must be distinguished from other causes of LETM. MRI clues that favor a diagnosis of sarcoidosis myelitis include a dorsal cord subpial pattern of gadolinium enhancement ≥2 spinal segments and persistence of enhancement for >2 months despite treatment.…”

Thoracic intramedullary neurosarcoidosis with thoracic disc herniation: Diagnostic importance of intramedullary contrast enhancement

“…The occurrence of spinal neurosarcoidosis is uncommon, with the cervical spine being the most common location followed by the thoracic spine. [ 10 , 11 ] Similarly, intramedullary manifestation of the disease is also uncommon with the disease usually spanning >3–4 intramedullary levels. [ 10 , 11 ] As seen in this case, the presence of T2 signal change out of proportion to the disc size/compression as well as the rapid and significant improvement in the neurological examination following steroid administration prompted investigation of another etiology before pursuing surgical intervention.…”