splenic marginal zone lymphoma ( smzl ) is a well - defined low - grade b - cell non - hodgkin 's lymphoma that primarily affects the white and red pulps of the spleen . patients with this disease are typically elderly men , and this tumor usually involves the bone marrow , liver , and splenic hilar lymph nodes . involvement of the central nervous system ( cns ) in the initial stage is rare in smzl , and only a few cases of meningeal lymphomatosis as the first manifestation of smzl have been reported in the literature [ 2 , 3 ] . we report on the unique case of a patient with smzl presenting with spastic paraplegia as the initial symptom . a 42-year - old male patient with no history of illness was admitted to our hospital because of progressive spastic paraplegia over 4 months . in november 2004 , he noticed paresthesia in the soles of his feet . in february 2005 , he developed paresthesia in both hands , spasticity of his legs , and paresthesia below the t10 level . over the next few months , he noted a progressive worsening of spastic paraplegia and sensory disturbance at the t1 sensory level . on admission , neurologic examinations revealed paraplegia with pyramidal syndrome , hypoesthesia below the t1 level , and anal hypotonia . wbc count was 5,700/dl , with 46% lymphocytes ( 19% of which were atypical ) . the level of a soluble anti - interleukin-2 receptor antibody was elevated ( 3,830 u / ml ) . spinal cord magnetic resonance imaging ( mri ) revealed an extensive high - intensity signal in t2-weighted sequences and swelling involving the thoracic region ( from t1 to t12 ) and conus medullaris ( fig . flow cytometry of peripheral blood and csf showed b - lineage markers ( cd19 + , cd20 + , and cd25 + ; surface immunoglobulin  expression ; igd+ and igm+ , cd5- and cd103- ) . the lymphoid cells exhibited the same phenotypic b - lymphocyte infiltration as in the peripheral blood , csf , and bone marrow . intrathecal cytarabine , methotrexate , hydrocortisone sodium phosphate , systemic fludarabine phosphate , and rituximab were administered . three months after the completion of chemotherapy , the patient was in complete remission , and follow - up spinal mri findings were normal ( fig . five years after the diagnosis , the patient remains in complete remission and his neurologic examination findings are almost normal . we report on the unique case of a patient with smzl with spinal involvement presenting with spastic paraplegia as the initial symptom . in our patient , the rapid diagnosis enabled the immediate initiation of chemotherapy , which led to clinical stability and radiologic improvements . smzl is a rare but distinctive and well - defined low - grade b - cell non - hodgkin 's lymphoma that primarily affects the white and red pulps of the spleen . the clinical symptoms of smzl are nonspecific ; e.g. fatigue , moderate anemia , abdominal symptoms due to splenomegaly and lymphocytosis as incidental findings . cns involvement is a common complication of high - grade lymphoma , but is rare in mature b - cell malignancies such as smzl ; however , it can occur in the advanced stage [ 4 , 5 ] . the cns involvements in smzl , as previously reported , were leptomeningeal involvement [ 2 , 3 , 4 ] and intracerebral tumor . of the four previously reported cases of patients with smzl , only two patients showed neurological symptoms as the initial manifestations [ 2 , 3 ] . these two patients presented with leptomeningeal involvement , and the clinical manifestations of these two cases were polyradiculoneuropathy   and papilledema . our patient showed intramedullary spinal cord involvement as determined by mri . in the four reported cases of smzl with cns involvement , three [ 3 , 4 , 5 ] showed abnormal mri findings . .   showed tumoral infiltration in both the optic nerves and cervicodorsal meninges . that of gotlib et al .   showed multiple meningeal nodules around the posterior fossa . that of thoennissen et al intramedullary spinal cord involvement in smzl is extremely rare , and , to the best of our knowledge , this is the first case of smzl with intramedullary spinal cord involvement associated with clinical and radiologic signs without the involvement of cerebral structures . it is uncertain why clinical cns involvement is rare in b - cell lymphoproliferative disorders . yamazaki et al .   suggested that the low expression level of adhesion receptors of b - lymphocytes might explain the low frequency of meningeal involvement . our patient is still alive after 5 years and imaging studies confirmed him to be in complete remission . smzl is generally considered to be of low grade , with some reported patients having a long - term survival , while others have an aggressive course with death within 1 year of diagnosis . in the reported smzl cases with meningeal involvement [ 3 , 4 ] , the outcomes were not good . further studies on the clinical and morphologic characteristics of smzl are required to prevent recurrence and disease - related death . cns involvement associated with smzl is rare , and symptomatic involvement is even more so . thus , physicians should take into account the possibility of cns invasion in smzl or other mature b - cell proliferative malignancies when patients present with neurologic manifestations .
in this report , we describe the case of a patient with splenic marginal zone lymphoma ( smzl ) who presented with spastic paraplegia as the initial symptom . a 42-year - old male developed progressive spastic paraplegia over 4 months . 
 his neurologic examination revealed paraplegia with pyramidal syndrome , hypoesthesia below the t1 level , and anal hypotonia . 
 magnetic resonance imaging ( mri ) of the spinal cord revealed an extensive high - intensity signal in t2-weighted sequences and swelling involving the thoracic region and conus medullaris . 
 a laboratory test revealed presence of the serum m component . 
 abdominal computed tomography images showed moderate splenomegaly . 
 abnormal lymphocytes of b - cell lineage markers ( cd19 + , cd20 + , and cd25 + ; surface immunoglobulin  expression ; igd+ and igm+ ) were found in the peripheral blood , cerebrospinal fluid , bone marrow and spleen . 
 splenectomy confirmed the smzl diagnosis . 
 after the completion of chemotherapy , the patient was in complete remission , and spinal mri findings were normal . 
 intramedullary spinal cord involvement in smzl is extremely rare , and , to the best of our knowledge , this is the first case of smzl with intramedullary spinal cord involvement associated with clinical and radiologic signs without the involvement of cerebral structures . 
 spastic paraplegia can be the initial presentation of smzl .