Isolated spinal neurosarcoidosis: An enigmatic intramedullary spinal cord pathology-case report and review of the literature

Abstract

Isolated spinal cord neurosarcoidosis (NS) in the absence of systemic disease or intracranial involvement is exceptionally rare. Adjunctive laboratory tests though useful may not be reliable and the absence of any pathognomonic radiological features makes the diagnosis difficult. As spinal cord NS may be a presenting feature of systemic sarcoidosis which may be occult on routine workup, 18F-fluorodeoxyglucose-positron emission tomography (FDG-PET) may be of value in unraveling this systemic involvement avoiding biopsying the spinal cord. A case of truly isolated NS is described with review of literature on this enigmatic pathology. Long segment intramedullary signal changes with focal parenchymal along with dural/meningeal enhancement in the absence of significant cervical stenosis in a young patient of northern European or African-American decent is very suggestive of NS and although may be presumably treated with steroids; there should be a low threshold for spinal cord biopsy especially in the absence of response to steroids to confirm isolated spinal cord NS in a patient with clinical neurological deterioration.

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

“…However, it is also known to affect other organs, including joints, liver, heart, spleen, gastrointestinal tract, nervous system, paranasal sinuses, parotid, breast, tongue, and skeletal muscle, which accounts for its myriad of symptoms and presentation s 2. Nervous system involvement is seen in 5–15% of cases, with 6–8% of these cases having spinal cord involvement; isolated spinal cord involvement is noticed in less than 0.5% of patients 3,4. Postmortem studies found an increasing frequency of neurosarcoidosis cases, only half of which were diagnosed prior to autopsy.…”

“…Many patients have cervical stenosis, leading to a consideration of spondylotic myelopathy, and thus they are subjected to decompression surgery with no improvement 11. Central nervous system lesions may be small, granulomas are not well formed, and biopsy may produce negative results 3…”

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

“…After 12 months of adequate treatment, the patient was clinically stable with persistent sensorimotor paraparesis and the neuroradiological findings partially remitted. 7,24 Another study by Saleh et al found only one biopsyconfirmed case of true isolated spinal cord sarcoidosis, further highlighting the rarity of the disease. 25 Brain MRIs were more frequently reported to be abnormal in SS, and we found brain parenchymal abnormalities in 70% and bony calvarium involvement in 10% of our cohort of patients.…”

“…After 12 months of adequate treatment, the patient was clinically stable with persistent sensorimotor paraparesis and the neuroradiological findings partially remitted. 7,24 Another study by Saleh et al. found only one biopsy-confirmed case of true isolated spinal cord sarcoidosis, further highlighting the rarity of the disease.…”

Neurosarcoidosis: unusual presentations and considerations for diagnosis and management

“…Occasionally, biopsy of the brain, spinal cord or meninges is performed to confirm the diagnosis. 22 Here, we present three cases of neurosarcoidosis and discuss important features in relation to early diagnosis and management. Furthermore, we provide a detailed review of the literature and highlight important clinical points for consideration.…”