sarcoidosis is a systemic granulomatous disease of unknown origin , characterized by the presence of noncaseating granuloma in affected organs ( 1 ) .
lesions are commonly seen in the lungs , lymphatic system , eyes , skin , liver , spleen , salivary glands , heart , nervous system , muscles , and bones ( 1 , 2 ) .
although neurosarcoidosis is a rare manifestation of sarcoidosis , the clinical symptoms can be devastating and occasionally life - threatening .
the diagnosis of neurosarcoidosis can be challenging because the disease can present with a lot of symptoms and diverse radiologic findings ( 1 - 5 ) . among them , spinal sarcoidosis is a very rare entity , occurring in < 1% , and can be manifested as intramedullary , intradural extramedullary , intraspinal epidural spaces and in vertebral bodies ( 6 ) . in korea ,
only a few cases of neurosarcoidosis involving brain , spinal nerve root , peripheral nerve and spinal dura , have been reported with or without histological confirmation in the literature .
patients with spinal sarcoidosis are considered to be at high risk for severe neurological sequelae ( 7 ) .
the information available about spinal sarcoidosis management and diagnosis comes from a few case reports , small series and expert opinions ( 8 - 10 ) .
however , the documents gave conflicting conclusions regarding the treatment , including corticosteroids and alternative immunosuppressants ( 6 , 7 ) .
we recently experienced a patient with isolated spinal cord sarcoidosis , which was confirmed by tissue biopsy and well responsive to high - dose corticosteroid and immunosuppressant .
a 54-yr - old woman , with no significant past medical history , presented with progressive 4-extremities weakness and sensory changes , followed by urinary difficulty since 1 month ago and was admitted to our hospital in october 2007 .
physical examination revealed increased deep tendon reflex , positive babinski sign and decreased motor power with medical research council ( mrc ) grade 2 strength in the right side and mrc grade 4 strength in the left side .
initial magnetic resonance imaging ( mri ) revealed increased t2 signal from c4 to c6 level , edematous expansion of the cord and intense nodular enhancement ( fig .
1a ) . based on the mri , spinal cord tumor , demyelinating disease involving multiple sclerosis ,
serological studies for systemic autoimmunity , including rheumatoid factor and antinuclear , anti - dsdna , anti - ssa / ssb , and antiphospholipid antibodies , showed no abnormality .
the cerebrospinal fluid ( csf ) analysis including biochemistry , igg index and oligoclonal band was normal .
one month later , the follow - up spinal mri showed the more extended lesion relative to previous mri , and which highly suggested the spinal cord tumor such as intramedullary astrocytoma ( fig .
based on the suspicion of intramedullary astrocytoma , the laminectomy and tissue biopsy of 2 l , which showed yellowish color , were performed at the central portion of dorsal column in affected cervical cord lesion .
postoperatively , patient 's neurologic deficits were not aggravated and , unexpectedly , the histology of biopsy revealed non - caseating granuloma without malignant cell ( fig .
angiotesin converting enzyme ( ace ) was mildly elevated to 56 ( u / l ; normal ,
, there was no disease activity on other organs with using brain mri , chest and abdominal computed tomography and nerve conduction study ( ncs ) .
the patient was treated with high - dose corticosteroid ( 60 mg / day ) followed by methotrexate ( 10 mg / week ) for over 2 yr .
two years later , cervical spinal cord lesion was much improved in the spinal mri ( fig .
1d ) , however clinical symptoms of weakness and sensory change were not definitely improved .
recently , the patient had to stop corticosteroid medication because of the cellulitis in the left leg . instead , we tried thalidomide 100 mg daily for 1 month with the goal of titrating up to 400 mg per day , which had been reported to be effective in the refractory neurosarcoidosis ( 11 ) .
however the patient refused high - dose thalidomide due to severe fatigue and high cost , and just treated with low - dose thalidomide ( 100 mg / day ) and treatment did not show distinct effect in our patient .
the spinal cord sarcoidosis is very rare and the diagnosis of this entity is difficult , as there are no pathognomic diagnostic study for neurosarcoidosis ( 3 , 12 , 13 ) . in general , this disease has been diagnosed clinically using mri , lumbar puncture and attendant laboratory tests , and the diagnosis is possible when systemic sarcoidosis is detected in other organs involving lung , kidney , eye and skin .
spinal cord sarcoidosis may present as an idiopathic inflammatory demyelinating disease both clinically and radiologically ( 5 ) .
only a positive biopsy of suspicious lesions in the central nervous system is considered to be definitive confirmation of the diagnosis of isolated neurosarcoidosis .
zajicek and colleagues established a diagnostic classification system for neurosarcoidosis that distinguished ' definite ' , ' probable ' and ' possible ' neurosarcoidosis based on tissue evidence of non - caseating granuloma and supportive evidence of sarcoid pathology in laboratory and imaging studies ( 13 ) .
however , biopsy should be cautiously considered if possible because of the risk involved in approaching the spinal cord .
our patient was initially suspected to have intramedullary astrocytoma or multiple sclerosis based on neurologic symptoms and mri results .
of course , the possibility of multiple sclerosis was not high because the clinical symptoms and cervical lesion were gradually progressive for months .
the spinal cord biopsy was challenging procedure , nevertheless the cord biopsy and laminectomy were performed for diagnostic confirmation in the consent of patient , and the histology of which was unexpectedly consistent with sarcoidosis .
the histology revealed multinucleated giant cells , lymphocytes and aggregated histiocytes within granulomatous inflammation , consistent with non - caseating granuloma seen in sarcoidosis .
a few cases with neurosarcoidosis have been reported , however most cases suggested only clinical manifestation without histology , or demonstrated indirect biopsy in the brain , meninges and lymph node ( 6 - 10 ) . with an initial treatment , we tried high dose corticosteroid and immunosuppressant .
systemic corticosteroids and immunosuppressive therapy are the treatment of choice for neurosarcoidosis , however which shows partial response especially in the spinal cord neurosarcoidosis ( 4 , 6 - 10 ) .
unfortunately , 25% of neurosarcoidosis still have a refractory course with steroid treatment and , and 20%-40% of those refractory patients will not respond to current conventional immunosuppressant ( 3 ) . in our case , the clinical symptoms were initially severe and rapidly progressive , and therefore the combined therapy of high - dose corticosteroid and immunosuppressant was necessary .
combination with immunosuppressant was useful to improve symptom control and reduce corticosteroid - related side effects in some cases .
therefore , making a diagnosis of neurosarcoidosis was therapeutically essential , since corticosteroid and immunosuppressant treatment must be started and continued for years in order to prevent progression and permanent disability .
a few reports have documented that the high - dose thalidomide ( 400 mg / day ) was effective in the refractory neurosarcoidosis ( 11 ) , and so we tried low - dose thalidomide ( 100 mg / day ) for one month in our patient .
however there was no clear evidence that thalidomide was effective in our case of neurosarcoidosis , although there was limitation on the dosage and duration of treatment compared with other cases .
our case indicates that diagnosis of spinal cord sarcoidosis is not easy and may require histological examination , and high - dose corticosteroid and immunosuppressant will be a good choice in the treatment of spinal cord sarcoidosis , and the thalidomide has to be debated in the treatment of spinal cord sarcoidosis .
this is the first korean case , to our knowledge , which shows an isolated spinal cord sarcoidosis confirmed by direct tissue biopsy and good response to corticosteroid and immunosuppressant .
 we report a case of 54-yr - old woman who presented with 4-extremities weakness and sensory changes , followed by cervical spinal cord lesion in magnetic resonance imaging . based on the suspicion of spinal tumor 
 , spinal cord biopsy was performed , and the histology revealed multinucleated giant cells , lymphocytes and aggregated histiocytes within granulomatous inflammation , consistent with non - caseating granuloma seen in sarcoidosis . 
 the patient was treated with corticosteroid , immunosuppressant and thalidomide for years . 
 our case indicates that diagnosis of spinal cord sarcoidosis is challenging and may require histological examination , and high - dose corticosteroid and immunosuppressant will be a good choice in the treatment of spinal cord sarcoidosis , and the thalidomide has to be debated in the spinal cord sarcoidosis . 
INTRODUCTION
CASE DESCRIPTION
DISCUSSION