Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

acute neurologic deficits

1st line – corticosteroid

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ACUTE
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acute neurologic deficits
1st line – 

corticosteroid

There are no controlled trials of corticosteroids for TM, but the approach is extrapolated from treatment of acute multiple sclerosis (MS) attacks.

Treatment with 3 to 5 consecutive daily infusions of corticosteroids is associated with more rapid recovery of neurologic deficits in MS, and clinical experience suggests similar results in TM.[79]

Some clinicians follow the intravenous therapy with an oral prednisone taper for 5 to 14 days.

Side effects of corticosteroids include hyperglycemia, hypertension, paresthesias, tremor, anxiety, insomnia, and increased risk of infection, but resolve quickly after therapy is discontinued.

Primary options

methylprednisolone: 1000 mg intravenously once daily for 3-5 days

Consider – supportive care + acute rehabilitation

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ACUTE
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acute neurologic deficits
Consider – 

supportive care + acute rehabilitation

Treatment recommended for SOME patients in selected patient group

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 is managed by stretching exercises, antispasticity drugs (e.g., baclofen, tizanidine), and therapeutic botulinum toxin injections.[79]

Acute urinary retention may be managed by bladder catheterization.

Deep vein thrombosis prophylaxis (heparin or enoxaparin with compression) should always be considered in immobilized patients.

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.

2nd line – plasma exchange (plasmapheresis)

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ACUTE
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acute neurologic deficits
2nd line – 

plasma exchange (plasmapheresis)

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.[23][77][82] Intravenous immunoglobulin may be considered but there are no randomized controlled trials that have assessed its efficacy.[23][77][8]

A randomized controlled crossover trial for patients with severe, corticosteroid-refractory central nervous system demyelinating events showed that clinically meaningful improvement occurred more frequently during plasma exchange (42%) compared with sham exchange (6%).[77] This study included some patients with TM. The protocol utilized a total of 7 exchanges administered every other day.

Risks of plasma exchange include line infection, sepsis, thrombosis, and bleeding.

Consider – supportive care + acute rehabilitation

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ACUTE
|
acute neurologic deficits
Consider – 

supportive care + acute rehabilitation

Treatment recommended for SOME patients in selected patient group

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 is managed by stretching exercises, antispasticity drugs (e.g., baclofen, tizanidine), therapeutic botulinum toxin injections.[79]

Acute urinary retention may be managed by bladder catheterization.

Deep vein thrombosis prophylaxis should always be considered in immobilized patients.

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.

ONGOING

idiopathic TM

1st line – observation

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ONGOING
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idiopathic TM
1st line – 

observation

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

Therapy aimed at preventing future attacks is not required for single-event TM, where diagnostic evaluation does not reveal risk for recurrent events or a specific underlying diagnosis.

at risk for multiple sclerosis (typical demyelinating lesions on MRI)

1st line – MS disease-modifying therapies

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ONGOING
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at risk for multiple sclerosis (typical demyelinating lesions on MRI)
1st line – 

MS disease-modifying therapies

Acute partial TM is associated with high risk for multiple sclerosis (MS) when the brain MRI shows typical demyelinating lesions.

Disease-modifying therapies (DMT) aim to reduce the risk of MS relapses and disability progression. Selection of initial therapy is guided by characteristics of the disease course, imaging findings, and patient characteristics and preferences. The 2018 American Academy of Neurology practice guidelines on DMT for people with MS address questions such as how to select the initial therapy and subsequent therapies, how best to monitor treatment response, and when to switch or discontinue treatment.[81] For detailed information on the management of MS, please see our Multiple sclerosis topic.

aquaporin-4 autoantibody seropositive

1st line – immunosuppressive treatment

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ONGOING
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aquaporin-4 autoantibody seropositive
1st line – 

immunosuppressive treatment

Longitudinally extensive TM should prompt testing for aquaporin-4 (AQP4)-IgG and myelin oligodendrocyte glycoprotein-IgG antibodies.[9][26] AQP4-IgG seropositive patients have a very high risk for recurrent TM or optic neuritis (more than 50% risk of recurrence at 1 year).[5] Long-term immunosuppressive therapy is recommended. The most commonly used therapies are azathioprine, mycophenolate, and rituximab.[83][84][85][86][87]

Primary options

azathioprine: consult specialist for guidance on dose

OR

mycophenolate mofetil: consult specialist for guidance on dose

OR

rituximab: consult specialist for guidance on dose

myelin oligodendrocyte glycoprotein-IgG autoantibody seropositive

1st line – immunosuppressive treatment

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ONGOING
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myelin oligodendrocyte glycoprotein-IgG autoantibody seropositive
1st line – 

immunosuppressive treatment

Longitudinally extensive TM should prompt testing for aquaporin-4-IgG and myelin oligodendrocyte glycoprotein (MOG)-IgG antibodies.[9][26] 

There is a high risk of relapse if patients remain seropositive for MOG-IgG. The treatment approach includes an extended taper of oral prednisone over 4 to 6 months, with serologic 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] 

Primary options

prednisone: consult specialist for guidance on dose

OR

prednisone: consult specialist for guidance on dose

-- AND --

azathioprine: consult specialist for guidance on dose

or

mycophenolate mofetil: consult specialist for guidance on dose

or

rituximab: consult specialist for guidance on dose

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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