Differential diagnosis and prognosis for longitudinally extensive XXXX in Buenos Aires, Argentina.

@entity1 @entity1652 @entity5 @entity1651 @entity15 @entity4685
INTRODUCTION: Longitudinally extensive @entity4685 (LETM) has classically been grouped with the full or limited @entity1652 spectrum disorders (NMOSD). However, differential diagnosis reveals a wide range of aetiologies. OBJECTIVE: To report on differential diagnosis and prognosis for LETM observed in a group of @entity1 in Buenos Aires, Argentina. @entity1 AND METHODS: Cross-sectional and retrospective multicentre study in two hospitals in Buenos Aires from June 2008 to June 2014. INCLUSION CRITERIA: medullary syndrome associated with spinal cord lesion extending for 3 or more contiguous spinal segments in magnetic resonance imaging (MRI). Clinical, radiological, and biochemical data were collected and subjects were rated on the Hughes functional disability scale (WHFDS) at 3 months. RESULTS: We evaluated 27 @entity1 , 74% of whom were @entity1 ; mean age was 35.22 years. The NMO-IgG antibody test was performed in 66.6% and oligoclonal band testing in 71%. NMO-IgG seropositivity was found exclusively in NMOSD @entity1 (75%). Brain MRI was normal in 59.2% and revealed a mean of 7.9 affected spinal segments. Differential diagnoses revealed NMOSD (37%), idiopathic LETM (22.2%), lupus (11.1%), @entity5 (11.1%), dural fistula (7.4%), acute disseminated @entity1651 (7.4%), and a single case of @entity15 (3.7%). @entity1 with lesions to >=  7 spinal segments showed poor recovery at 3 months (P<.001); these cases were associated with neoplastic, vascular, idiopathic, and lupus-related aetiologies. CONCLUSIONS: The most frequent causes of LETM in our cohort were NMOSD followed by idiopathic cases. Neoplastic, vascular, lupus-related, and idiopathic LETM may constitute a critical group with a distinct prognosis and other treatment needs.
