Spinal Cord Sarcoidosis Research Articles

“Trident Sign” in Spinal Cord Lymphoma (P7-4.003)

<h3>Objective:</h3> N/A <h3>Background:</h3> Chronic, progressive myelopathies with longitudinally-extensive T2-hyperintense lesions and specific gadolinium enhancement patterns, such as the axial “trident sign” of enhancement, have shown to be strongly suggestive of spinal cord sarcoidosis. However, the sensitivity and specificity of such enhancement patterns are not known. <h3>Design/Methods:</h3> Case report with description of history, examination, laboratory values, and imaging findings. <h3>Results:</h3> A 50-year-old woman presented with progressive left spastic hemiparesis over three weeks. MRI of the cervical spine revealed a longitudinally-extensive T2-hyperintense lesion with linear dorsal subpial enhancement and an axial trident pattern of enhancement. Cerebrospinal fluid analysis revealed a pleocytosis with a white blood cell count 20 cells/mm³ (89% lymphocytes), protein of 50 mg/dL, and glucose of 59 mg/dL with negative oligoclonal bands, cytology, and flow cytometry. Aquaporin-4-IgG and Myelin Oligodendrocyte Glycoprotein-IgG in serum were negative. Whole-body FDG-PET revealed increased uptake in the cervical cord but no other areas of systemic hypermetabolism. She was treated with three courses of IV methylprednisolone but her weakness continued to progress. Repeat MRI two months after her initial presentation showed persistent enhancement. Her neurologic examination revealed left spastic hemiplegia and moderate weakness of her right upper extremity. Given the chronic, progressive myelopathy, persistent trident enhancement pattern suggestive of spinal cord sarcoidosis, and the potential risks involved with a cervical cord biopsy, a course of infliximab was trialed empirically. Despite this, she continued to worsen neurologically and developed hypercarbic respiratory failure. Spinal cord biopsy was pursued, ultimately revealing infiltration by diffuse large B-cell lymphoma. <h3>Conclusions:</h3> Spinal cord lymphoma should be considered in patients presenting with chronic, progressive myelopathy and can present with similar enhancement patterns as spinal cord sarcoidosis. Atypical clinical features, such as rapid and severe neurologic worsening despite immunosuppression, should raise suspicion for diagnoses other than spinal cord sarcoidosis to allow for earlier detection and expedited disease-directed treatment. <b>Disclosure:</b> Dr. Griffin has nothing to disclose. Dr. Toledano has nothing to disclose. The institution of Dr. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon Therapeutics. Dr. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Pharmacy times. The institution of Dr. Flanagan has received research support from Viela Bio. Dr. Flanagan has received research support from UCB. Dr. Flanagan has received publishing royalties from a publication relating to health care. Dr. Flanagan has a non-compensated relationship as a Member of medical Advisory Board with The MOG Project that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial board member with Journal of The Neurologic Sciences that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial board member with Neuroimmunology Reports that is relevant to AAN interests or activities. Dr. Mustafa has nothing to disclose.

Minor Salivary Gland Biopsy for the Diagnosis of Neurosarcoidosis

Introduction: The definite diagnosis of neurosarcoidosis is challenging since it requires a compatible histology of the nervous system. When neurosarcoidosis is suspected, other systemic manifestations are investigated to confirm the diagnosis. A minor salivary gland biopsy (MSGB) is often performed since it is minimally invasive. The objective of the present study was to assess its performance for the diagnosis of neurosarcoidosis. Methods: A retrospective single-center study included patients who underwent a MSGB in a tertiary neurological university hospital (Lyon, France) between 2015 and 2018. Clinical presentations unlikely to be compatible with neurosarcoidosis were excluded. Positive cases of neurosarcoidosis were defined as definite, probable, and possible cases, according to the latest international neurosarcoidosis diagnostic criteria from the Neurosarcoidosis Consortium Consensus Group. Results: A total of 529 patients underwent a MSGB for clinical manifestations compatible with neurosarcoidosis. Among the 13 who fulfilled the criteria for neurosarcoidosis, only one had a positive MSGB. The sensitivity of MSGB was 7.7% (95% CI [0.2–36.0%]) and the specificity was 100.0% (95% CI [99.3–100%]). Conclusion: Considering the low sensitivity of MSGB for the diagnosis of NS, MSGB should be performed in selected indications, including a suspicion of spinal cord sarcoidosis, or when there is a strong clinical, laboratory, and radiological suspicion of NS. MSGB should rather not be performed when the chest CT-scan does not show signs of pulmonary or lymph node sarcoidosis.

A Clinical Case of Generalized Sarcoidosis with a Predominant Lesion of the Spinal Cord

Sarcoidosis, as systemic epithelioid cell granulomatosis, can be accompanied by damage not only to the intrathoracic lymph nodes and lungs, but also to other organs, in particular, the central nervous system and peripheral lymph nodes. In the spectrum of extrathoracic lesions, spinal cord sarcoidosis occurs only in 6-8 % of cases of all brain lesions. The presented clinical example illustrates the lesion of the spinal cord at the level of the thoracic region, although the literature more often describes the lesion of the cervical region. The disease was accompanied by sarcoidosis of the intrathoracic lymph nodes with rapid spontaneous regression and sarcoidosis of the supraclavicular lymph node. The diagnosis was confirmed after a peripheral lymph node biopsy. Spinal cord sarcoidosis in this patient was characterized by rapid regression against the background of parenteral administration of dexamethasone for 14 days, followed by transfer to tablet forms of prednisone. The positive dynamics of spinal cord sarcoidosis refuted the assumption of the presence of a sarcoid reaction in the lymph nodes against the background of a spinal cord tumor. The use of a course of rehabilitation techniques contributed to the recovery of working capacity.

Prognostic factors and treatment efficacy in spinal cord sarcoidosis：An observational cohort with long-term follow-up.

Prognostic factors and treatment efficacy in spinal cord sarcoidosis：An observational cohort with long-term follow-up.

"String of Pearls" in Spinal Cord Sarcoidosis.

"String of Pearls" in Spinal Cord Sarcoidosis.

Spinal cord sarcoidosis in Japan: utility of cerebrospinal fluid examination and nerve conduction study for diagnosis and prognosis prediction.

Spinal cord sarcoidosis (SCS) is rare, and its diagnosis is challenging. We examined clinical, laboratory, and imaging features in patients with SCS to obtain useful clues for diagnosis and prognosis. Eleven consecutive patients (four males, seven females) at a single Japanese institution were investigated. Median age at onset was 66years old. The most frequent site affected, other than the nervous system, was the respiratory system. While histological confirmation of non-caseating granulomas was often found there, no patient had respiratory symptoms. Peripheral nerve involvement was detected in 64% of patients. Soluble IL-2 receptor (sIL-2R) levels in serum and cerebrospinal fluid (CSF) were elevated in 64% and 45% of patients, respectively, and this finding was more common than elevation of angiotensin-converting enzyme (ACE). 18F-fluorodeoxyglucose (FDG) positron emission tomography showed abnormally high uptake in spinal lesions of all examined patients. Although corticosteroids were administrated to all patients, and immuno-suppressants were prescribed to six (55%), the modified Rankin Scale was unchanged or worsened in four (36%) patients during the follow-up period. Neurological exacerbation of myelopathy was seen in four (36%) patients. Complete response rate was only seen in 9%. High levels of cell count, protein, ACE, and sIL-2R in CSF were significantly more frequent in patients with a marked improvement after immunotherapy than in the other patients. These results suggest that high serum and CSF sIL-2R, high uptake of FDG, and peripheral nerve involvement are indicative of SCS. Given that SCS is commonly intractable, CSF abnormalities may predict efficacy of immunotherapies.

Spinal Cord Sarcoidosis

Spinal cord sarcoidosis is rare, but often appears as a diagnostic challenge in myelopathy of unknown origin. Although definite diagnosis requires histological confirmation of non-caseating epithelioid granuloma in the spinal cord, it is hardly obtained due to the invasiveness of the biopsy procedure. Hence, extensive searches for involvement of other organs involvement should be prompted, including biopsy of the most "promising" and "easy touch" lesions identified in individual patients. Spinal sarcoidosis is generally treated with high-dose corticosteroids, immunosuppressants, or both, but it is often refractory to treatment and requires a long treatment period. Therefore, extensive examinations to evaluate the likelihood of sarcoidosis should be carried out before the initiation of immunotherapy, especially in cases without histological confirmation.

STABBED IN THE BACK! A CASE OF NEUROSARCOIDOSIS WITH SPINAL CORD INVOLVEMENT

SESSION TITLE: Tuesday Fellows Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Sarcoidosis is a systemic disease characterized by the presence of non-caseating granulomas. While the lungs are the primary site of involvement, multiple other organ systems can be affected1. Sarcoidosis involving the central nervous system (CNS) is a rare manifestation seen in roughly 5% of sarcoid cases2. Clinical manifestations of neurosarcoidosis depend on the CNS site affected and can be quite variable leading to diagnostic challenges and delays. Neurosarcoidosis with spinal cord involvement (NSSI) is a rarely encountered variant of CNS involvement and can pose serious diagnostic challenges due to the high morbidity and potential mortality associated with direct tissue biopsy from spinal cord structures. However, a majority of cases also have pulmonary involvement3. In such cases; a thorough evaluation for concurrent pulmonary involvement may provide diagnostic confirmation despite absence of overt pulmonary symptoms. CASE PRESENTATION: A 66-year-old nonsmoker with a history of rheumatoid arthritis and hypertension was found to have bilateral FDG avid mediastinal lymphadenopathy in the setting of a recent working diagnosis of neuromyelitis optica (Figure 1). Aside from bilateral lower extremity ascending paresthesias, the patient was otherwise asymptomatic with normal pulmonary function tests within the past five years. On physical exam, there was evidence of bilateral lower extremity spasticity with decreased proprioception and temperature sensation. Cardio-pulmonary exam was otherwise normal. Neuromyelitis optica (NMO) autoantibodies were negative and neuroimaging showed extensive longitudinal central T2 cord signal with subpial enhancement,‘Trident sign’ (Figure 2). Bronchoscopy with endobronchial ultrasound guided transbronchial biopsy (EBUS TBNA) found non-caseating granulomatous inflammation. Diagnosis of NSSI was made and patient was started on high dose prednisone with transition the infliximab. DISCUSSION: NSSI is a rarely encountered variant of sarcoidosis. Patients with this form of disease commonly present with clinical findings of abnormal proprioception, spasticity, paresthesias and urinary dysfunction2. Additionally the ‘trident sign’ comprised of linear dorsal subpial enhancement within the spinal cord is a well described pattern on MRI associated with NSSI3. Recognizing the common symptoms and imaging findings associated with NSSI is important as up to eighty percent of patients lack pulmonary symptoms or impairment4. Once diagnosis is suspected, a thorough workup to identify pulmonary and other non-CNS sites of sarcoid involvement should be undertaken. It is important to remember that up to seventy percent of patients with neurosarcoidosis have some pulmonary manifestations of sarcoidosis. CONCLUSIONS: This case underscores the importance of pulmonary evaluation when NSSI is suspected, as EBUS TBNA can aid in clinching the diagnosis as demonstrated in this case. Reference #1: Baughman RP, Culver DA, Judson MA. A concise review of pulmonary sarcoidosis. Am J Respir Crit Care Med. 2011;183: 573-581 Reference #2: Zalewski NL, Krecke KN, Weinshenker BG, Aksamit AJ, Conway BL, McKeon A, Flanagan EP. Central canal enhancement and the trident sign in spinal cord sarcoidosis. Neurology. 2016;87(7): 743-744 Reference #3: Kingah P, Alam M, Chugh K, Kamholz J, Samavati L. Role of pulmonary evaluation in diagnosis of neurosarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis. 2016;33: 209-215 DISCLOSURES: No relevant relationships by Vivek Iyer, source=Web Response No relevant relationships by Mark Norton, source=Web Response

Management of Cervical Spondylosis by Neurologists

Neurologists have to manage patients with cervical spondylosis, a common neurological disease in individuals over the age of 50 years. The diagnosis of cervical spondylosis requires a detailed neurological examination that takes into account cervical levels. To prevent misdiagnosis, it is important to integrate the levels of the lesions revealed by imaging with the clinical findings. Differential diagnoses for cervical spondylosis include a member of neurological diseases, including but not limited to cerebrovascular disease, amyotrophic lateral sclerosis, peripheral nerve disease, and spinal cord sarcoidosis. Though the course of the disease and the ultimate prognosis for patients with cervical spondylosis are highly variable, many patients experience a relatively benign form of the disease.

Frequency of Aquaporin-4 Immunoglobulin G in Longitudinally Extensive Transverse Myelitis With Antiphospholipid Antibodies

Antiphospholipid (aPL) antibodies have historically been postulated to cause a poorly understood inflammatory myelitis. Neuromyelitis optica spectrum disorder (NMOSD) causes an inflammatory longitudinally extensive transverse myelitis (LETM). In 2004, aquaporin-4 immunoglobulin G (AQP4-IgG) was first reported as a highly specific (>99%) serum diagnostic biomarker of NMOSD, distinguishing it from other disorders (eg, multiple sclerosis). We sought to assess the frequency of AQP4-IgG (and thus NMOSD diagnosis) in LETM with aPL antibodies. We searched Mayo Clinic records (from January 1, 1996, through December 31, 2014) for patients with (1) LETM and (2) aPL or β2-glycoprotein I antibodies and (3) a serum sample available. AQP4-IgG was evaluated in the 24 included patients and in 20 controls with aPL antibodies but without myelitis. Seropositivity for AQP4-IgG was confirmed in 11 of 24 patients with LETM (46%), confirming an AQP4-IgG–seropositive NMOSD diagnosis rather than aPL-associated LETM. Six of 11 AQP4-IgG–seropositive patients (54%) were initially diagnosed as having aPL/lupus-associated myelitis. Recurrent LETM was exclusive to AQP4-IgG–seropositive patients (P=.003). Alternative diagnoses assigned to the remaining 13 AQP4-IgG–seronegative patients included idiopathic transverse myelitis (n=5), seronegative NMOSD (n=2), spinal cord infarct attributed to aPL antibodies (n=2), spinal cord sarcoidosis (n=1), varicella-zoster virus myelitis (n=1), postinfectious myelitis (n=1), and multiple sclerosis (n=1). All 20 controls were seronegative for AQP4-IgG. Clotting disorders occurred in 36% of patients (4 of 11) with LETM with both aPL antibodies and AQP4-IgG. AQP4-IgG should be tested in all patients with LETM and aPL antibodies because AQP4-IgG–seropositive NMOSD accounts for almost half of all cases. Clotting disorders are common in patients with LETM with dual positivity for AQP4-IgG and aPL antibodies.

Characteristics and Long-Term Outcome of Neurosarcoidosis: A Population-Based Study from 1976-2013

Background/Aims: Neurosarcoidosis is a rare condition with serious health consequences. However, little is known about clinical characteristics and outcome of neurosarcoidosis in the community setting. Methods: Patients with neurosarcoidosis were identified from a previously described cohort of patients with incident sarcoidosis from Olmsted County, Minnesota, United States from 1976 to 2013 using individual medical record review. Data on clinical characteristics, treatment, and outcome were collected. Results: Neurological involvement by sarcoidosis occurred in 11 patients (3% of all patients with sarcoidosis). Cranial neuropathy was the most common type of neurological disease (5 patients; 45%) followed by peripheral neuropathy (3 patients; 27%), and meningitis (3 patients; 27%). Cerebrospinal fluid (CSF) pleocytosis and elevated CSF protein levels were observed in patients with meningitis, intramedullary spinal cord sarcoidosis, intracranial mass lesion and some patients with cranial neuropathy but were normal in patients with peripheral neuropathy. All patients received high-dose glucocorticoids as initial treatment and almost all responded to this therapy. Relapse after glucocorticoid dose reduction necessitated subsequent treatment with steroid-sparing agents in 4 patients. Conclusion: Neurosarcoidosis is an uncommon manifestation of sarcoidosis. Neurosarcoidosis manifestations generally responded well to high-dose glucocorticoids in the majority of patients, but relapse was common.

Central canal enhancement and the trident sign in spinal cord sarcoidosis.

We report an enhancement pattern in 9 patients with spinal cord sarcoidosis (SCS) with subacute onset (<4 weeks) myelitis as the initial manifestation of sarcoidosis. Presenting symptoms included numbness in 9, urinary complaints in 7, and weakness in 6. Examination revealed abnormal proprioception in 8, spastic quadriparesis/paraparesis in 6, and sensory level in 6. MRI showed central canal enhancement alone (11%) or in combination with dorsal-subpial enhancement1 (89%) (figure 1), often resembling a trident head on axial sequences (figure 2). Lung biopsies in 7 patients confirmed sarcoidosis; both patients without biopsies had hilar adenopathy. Central canal enhancement and the trident sign in subacute myelitis should raise suspicion for SCS.

Clinical features and prognostic factors of spinal cord sarcoidosis: a multicenter observational study of 20 BIOPSY-PROVEN patients.

Sarcoidosis of the spinal cord is a rare disease. The aims of this study are to describe the features of spinal cord sarcoidosis (SCS) and identify prognostic markers. We analyzed 20 patients over a 20-year period in 8 French hospitals. There were 12 men (60%), mostly Caucasian (75%). The median ages at diagnosis of sarcoidosis and myelitis were 34.5 and 37years, respectively. SCS revealed sarcoidosis in 12 patients (60%). Eleven patients presented with motor deficit (55%) and 9 had sphincter dysfunction (45%). The median initial Edmus Grading Scale (EGS) score was 2.5. The cerebrospinal fluid (CSF) showed elevated protein level (median: 1.00g/L, interquartile range (IQR) 0.72-1.97), low glucose level (median 2.84mmol/L, IQR 1.42-3.45), and elevated white cell count (median 22/mm(3), IQR 6-45). The cervical and thoracic cords were most often affected (90%). All patients received steroids and an immunosuppressive drug was added in 10 cases (50%). After a mean follow-up of 52.1months (range 8-43), 18 patients had partial response (90%), 7 displayed functional impairment (35%), and the median final EGS score was 1. Six patients experienced relapse (30%). There was an association between the initial and the final EGS scores (p=0.006). High CSF protein level showed a trend toward an association with relapse (p=0.076). The spinal cord lesion was often the presenting feature of sarcoidosis. Most patients experienced clinical improvement with corticosteroids and/or immunosuppressive treatment. The long-term functional prognosis was correlated with the initial severity.

Discriminating long myelitis of neuromyelitis optica from sarcoidosis.

To compare longitudinally extensive myelitis in neuromyelitis optica spectrum disorders (NMOSD) and spinal cord sarcoidosis (SCS). We identified adult patients evaluated between 1996 and 2015 with SCS or NMOSD whose first myelitis episode was accompanied by a spinal cord lesion spanning ≥3 vertebral segments. All NMOSD patients were positive for aquaporin-4-immunoglobulin G, and all sarcoidosis cases were pathologically confirmed. Clinical characteristics were evaluated. Spine magnetic resonance imaging was reviewed by 2 neuroradiologists. We studied 71 patients (NMOSD, 37; SCS, 34). Sixteen (47%) SCS cases were initially diagnosed as NMOSD or idiopathic transverse myelitis. Median delay to diagnosis was longer for SCS than NMOSD (5 vs 1.5 months, p < 0.01). NMOSD myelitis patients were more commonly women, had concurrent or prior optic neuritis or intractable vomiting episodes more frequently, had shorter time to maximum deficit, and had systemic autoimmunity more often than SCS (p < 0.05). SCS patients had constitutional symptoms, cerebrospinal fluid (CSF) pleocytosis, and hilar adenopathy more frequently than NMOSD (p < 0.05); CSF hypoglycorrhachia (11%, p = 0.25) and elevated angiotensin-converting enzyme (18%, p = 0.30) were exclusive to SCS. Dorsal cord subpial gadolinium enhancement extending ≥2 vertebral segments and persistent enhancement >2 months favored SCS, and ringlike enhancement favored NMOSD (p < 0.05). Maximum disability was similar in both disorders. SCS is an under-recognized cause of longitudinally extensive myelitis that commonly mimics NMOSD. We identified clinical, laboratory, systemic, and radiologic features that, taken together, help discriminate SCS from NMOSD.

Chapter 19 - Autoimmune myelopathies

Autoimmune myelopathies are a heterogeneous group of immune-mediated spinal cord disorders with a broad differential diagnosis. They encompass myelopathies with an immune attack on the spinal cord (e.g., aquaporin-4-IgG (AQP4-IgG) seropositive neuromyelitis optica (NMO) and its spectrum disorders (NMOSD)), myelopathies occurring with systemic autoimmune disorders (which may also be due to coexisting NMO/NMOSD), paraneoplastic autoimmune myelopathies, postinfectious autoimmune myelopathies (e.g., acute disseminated encephalomyelitis), and myelopathies thought to be immune-related (e.g., multiple sclerosis and spinal cord sarcoidosis). Spine magnetic resonance imaging is extremely useful in the evaluation of autoimmune myelopathies as the location of signal change, length of the lesion, gadolinium enhancement pattern, and evolution over time narrow the differential diagnosis considerably. The recent discovery of multiple novel neural-specific autoantibodies accompanying autoimmune myelopathies has improved their classification. These autoantibodies may be pathogenic (e.g., AQP4-IgG) or nonpathogenic and more reflective of a cytotoxic T-cell-mediated autoimmune response (collapsin response mediator protein-5(CRMP5)-IgG). The presence of an autoantibody may help guide cancer search, assist treatment decisions, and predict outcome/relapse. With paraneoplastic myelopathies the initial goal is detection and treatment of the underlying cancer. The aim of immunotherapy in all autoimmune myelopathies is to maximize reversibility, maintain benefits (while preventing relapse), and minimize side effects.

Longitudinal ultra-extensive transverse myelitis as a manifestation of neurosarcoidosis

ObjectiveTo analyze the clinical characteristics and outcome of patients with neurosarcoidosis manifesting as longitudinal transverse myelitis spanning 6 or more spinal segments. MethodRetrospective analysis of 7 cases from a single institution. ResultsFour males and 5 African–American were included. The mean onset age for neurological symptoms was 49.1years old. Only 1 patient had a prior diagnosis of sarcoidosis. In all patients, spinal MRI showed contiguous cervical and/or thoracic cord lesions predominantly in a central or centrodorsal location, associated with parenchymal or leptomeningeal gadolinium enhancement. Cerebral spinal fluid (CSF) pleocytosis was present in all and hypoglycorrhachia in 3 patients. Angiotensin-converting enzyme (ACE) level was elevated in the serum of 1 patient while being normal in the CSF of all 4 cases tested. Chest imaging facilitated the diagnosis of sarcoidosis in all cases. The use of corticosteroid and immunosuppressive agents including infliximab and methotrexate led to improved outcome. ConclusionsNeurosarcoidosis should be considered in the differential diagnosis of longitudinal ultra-extensive myelitis, even in the absence of previously diagnosed sarcoidosis. Timely usage of corticosteroid and immunosuppressive agents improves the clinical outcome of patients with ultra-extensive spinal cord sarcoidosis.

Nick Kitson

<h3>Objectives</h3> Neurosarcoidosis may present with longitudinally extensive transverse myelitis (LETM) posing a diagnostic challenge. We describe the clinical features, radiology and management of five patients with spinal neurosarcoidosis (SNS). <h3>Methods</h3>...

A CHALLENGING CASE: SPINAL CORD SARCOIDOSIS

A 54-year old man with severe ankylosing spondylitis presented with rapidly progressing bilateral lower limb weakness and altered sensation, associated with urinary retention. Examination showed flaccid paraparesis with lower limb areflexia and a sensory level to T10. His upper limbs were normal. MR scan of whole spine showed marked ankylosing spondylitis changes, including canal narrowing, and extensive, discontinuous signal change within the spinal cord at C7–T1, T3–T4 and T7–T11. There was no leptomeningeal enhancement. Severe ankylosing spondylitis meant we were unable to obtain CSF, MR scan of head or do a leptomeningeal biopsy (anaesthetic risk). CT scan of thorax however showed extensive mediastinal and hilar lymphadenopathy. We could therefore obtain a tissue diagnosis from lymph node biopsy, which showed sarcoid-type, non-caseating granulomas. We diagnosed neurosarcoidosis and prescribed immunosuppression. His paraplegia is unchanged, but his neurology is stable. Spinal cord sarcoidosis is rare (

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

We retrospectively investigated spinal magnetic resonance imaging (MRI) manifestations at neurological onset in Japanese patients with spinal cord sarcoidosis. 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. 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. 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.

Surgical treatment for cervical spondylotic myelopathy with spinal cord sarcoidosis

Surgical treatment for cervical spondylotic myelopathy with spinal cord sarcoidosis