Transverse myelitis in a patient with primary antiphospholipid syndrome
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/0028-3886.170077
Source of Support: None, Conflict of Interest: None
The manifestations of antiphospholipid antibody syndrome (aPS) are diverse. The neurological manifestations may range from simple headaches to the more devastating phenomena such as stroke. Transverse myelitis (TM) is very rare in aPS, more so in the primary aPS. Further, presently there are no clear guidelines for the management of TM in aPS. The outcome in these patients can be devastating if the treatment is delayed. We present the case of an adult woman, a known case of aPS for 23 years, who developed paraparesis. Her magnetic resonance imaging (MRI) showed features typical of TM. A brief review of literature follows.
A 56-year-old woman, a tourist from Italy, had a significant history of primary aPS syndrome. She presented with progressive asymmetrical weakness that began 7 days prior to her admission. She reported difficulty in walking, numbness in the body below her breasts, and urinary incontinence. Physical examination revealed moderate-to-severe paraparesis with bilateral extensor plantar responses, brisk reflexes in both lower limbs associated with impairment of all sensations and a sensory level at T4-T5. Her magnetic resonance imaging (MRI) revealed a lower cervical and upper thoracic cord lesion that showed enhancement after gadolinium administration, a finding consistent with TM [Figure 1] and [Figure 2].
The patient was managed with methylprednisolone pulse (MP) therapy for 5 days and low molecular weight heparin (LMWH). She improved after treatment with recovery of power in her lower limbs and improvement in sensations. She was discharged on hydroxychloroquine and oral steroids.
TM is the common designation for an acutely evolving inflammatory-demyelinative lesion of the spinal cord characterized by an acute or a subacute motor, sensory, and autonomic (bladder, bowel, and sexual) spinal cord dysfunction. The list of ailments that may be considered in the differential diagnosis of acute TM is fairly long [Table 1].
The patients present with either symmetric or asymmetric paraparesis/plegia, ascending paresthesias, loss of deep sensibility in the feet, a sensory level on the trunk, sphincteric dysfunction, and bilateral Babinski's sign.
aPS is a disorder that manifests clinically as recurrent venous or arterial thrombosis and/or with recurrent abortions. Central nervous system (CNS) involvement is common in patients with antiphospholipid (Hughes) syndrome [Table 2]. In 1983, Hughes, in his original description of the syndrome, stressed on the importance of cerebral features in these patients.
Although the mechanism of neurological involvement in patients with aPS is thought to be thrombotic in origin, many of the central nervous system manifestations cannot be explained solely by the hypercoagulability theory and probably have more complex underlying mechanisms.
TM is a rare complication of aPS syndrome, reported in only 0.4% of the patients. It is particularly rare in primary aPS.,, It was first described by Hughes in 1985 in a 45-year-old woman who developed TM over a 1-year period. Serological tests suggested a lupus-like illness. IgM antibodies to cardiolipin were detected in high titers in her serum. Several of the aPS patients with TM have recurrent episodes. The exact pathogenesis of TM in aPS patients is not known and is thought to involve both ischemic episodes as well as anticardiolipin (aCL) antibody-mediated interactions. A strong association between aCL antibodies and TM in patients of aPS was described by Lavalle et al., who reported 10 out of 12 patients with TM and systemic lupus erythematosus (SLE) having aCL antibodies. Kovacs et al., evaluated 14 patients with SLE and TM and 91 additional cases published in the English and German literature. They found the incidence of TM in aPS to be 2% and TM occurring as the initial manifestation of SLE in a rather high number of patients (39% of the total patient population). In 42% of the total patient population analyzed, TM presented within the first 5 years of the diagnosis of SLE. The prominent presentation of TM in SLE seems to be a sensory level, predominantly in the thoracic area. Of the 64 of a total of 105 patients tested for antiphospholipid antibodies (aPL), 64% patients were found to be positive for them.
As TM in SLE is a very rare clinical manifestation, no definitive treatment guidelines have been instituted. Different treatment options available include methylprednisolone (MP) therapy, intravenous cyclophosphamide, and plasmapheresis. It has been postulated that an aggressive treatment instituted early in the course of the disease is crucial for a favorable response.
Kovacs et al., found that treatment with intravenous MP alone led to a significant recovery. However, the use of intravenous MP followed by intravenous cyclophosphamide pulse (CP) seemed to be more effective than intravenous MP alone. Additional treatment with plasmapheresis in patients who did not improve with intravenous MP and intravenous CP did not seem to improve the outcome any further. Barile et al., found that cyclophosphamide was more effective than MP in the treatment of acute, severe neuropsychiatric manifestations of SLE that were associated with TM.
Mok et al., used mycophenolate mofetil (MMF) in patients with SLE with TM and reported only a modest efficacy. Two of their three patients responded partially to MMF, and their myelopathy relapsed while receiving the maintenance treatment. The authors recommended that adminstration of MMF in lupus myelopathy should be restricted to those patients who are either refractory/intolerant to or are reluctant to receive cyclophosphamide.
TM is a rare manifestation of aPS syndrome, and is even rarer in primary aPS. Early diagnosis and treatment are important for an optimal outcome. The combination of MP with intravenous cyclophosphamide is a better choice than steroids alone.
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[Figure 1], [Figure 2]
[Table 1], [Table 2]