Approach

The goals of treatment are to restore neurological function lost during the acute TM event; to initiate preventive strategies for, and symptomatic relief of, TM complications; and, when applicable, to start therapies aimed at preventing disease relapse.

Acute neurological deficits

After exclusion of compressive lesions, acute infections, and other non-TM disorders, first-line treatment of the acute neurological deficits related to TM is with intravenous methylprednisolone, typically administered daily for 3 to 5 consecutive days.[23] There are no controlled trials of corticosteroids for TM but the approach is extrapolated from treatment of acute multiple sclerosis (MS) attacks.

Patients with severe deficits that do not respond to corticosteroid therapy, or whose deficits worsen despite treatment, are candidates for rescue therapy with plasma exchange (plasmapheresis).[23][76][77]

Intravenous immunoglobulin may be considered but there are no randomised controlled trials that have assessed its efficacy.[23][76][8]

Supportive therapy and acute rehabilitation

Supportive therapy for individual symptoms such as respiratory distress, spasticity, and urinary retention may be added to the treatment regimen as required.

  • Respiratory failure: in a minority of patients with cervical TM, the lesion extends into the medulla and may cause neurogenic respiratory failure.[4] Close observation of respiratory parameters, including measurement of maximal respiratory pressures and forced vital capacity, and involvement of a skilled critical care team are recommended in cases of ascending cervical myelitis.

  • Spasticity: managed by stretching exercises, anti-spasticity drugs (e.g., baclofen, tizanidine), therapeutic botulinum toxin injections.[78] 

  • Acute urinary retention: may be managed by bladder catheterisation. Residual neurogenic bladder symptoms may include urge incontinence, retention, or a mixed disorder, each of which requires specific treatment.[78]

  • Deep venous thrombosis (DVT) prevention: immobilised patients are at increased risk. Extrapolation of data from general medical and orthopaedic-surgery patients indicates that subcutaneous heparin or enoxaparin plus use of lower extremity compression stockings or devices reduces the risk of DVT.

Acute rehabilitation consists of passive and active therapy to maintain the range of motion of limbs, reduce spasms and the risk of contractures, and to reduce risk of decubitus ulceration.

Preventive therapy for patients at risk of developing MS or recurrent TM

If the patient is found to have idiopathic TM, no preventive therapy is required.

MS disease-modifying therapy, aimed at reducing the risk of MS relapses and disability progression, may be indicated for patients with acute partial TM who are deemed to be at risk for development of MS due to the presence of typical demyelinating lesions on magnetic resonance imaging.[47][48][79][80]

Immunosuppressive therapy of indefinite duration is recommended for patients who are seropositive for aquaporin-4 (AQP4) auto-antibody because of a persistent high relapse risk.[5]

There is also a high risk of relapse if patients remain seropositive for myelin oligodendrocyte glycoprotein (MOG)-IgG. The treatment approach includes an extended taper of oral prednisolone over 4 to 6 months with serological reassessment and initiation of immunosuppressive preventive therapy (such as azathioprine, mycophenolate, or rituximab) if MOG-IgG antibody persists or there is interim clinical relapse.[59][60]

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