Overview
Idiopathic acute transverse myelitis (ATM) is a rare neurological disorder characterized by acute inflammation of the spinal cord that occurs without an identifiable underlying cause. The term 'idiopathic' distinguishes it from transverse myelitis that occurs secondary to known conditions such as multiple sclerosis, neuromyelitis optica spectrum disorder, systemic autoimmune diseases, or infections. The inflammation damages the myelin sheath (the protective covering of nerve fibers) and can also injure the nerve cells themselves, disrupting communication between the spinal cord and the rest of the body. The condition primarily affects the nervous system and can present with a rapid onset of motor weakness or paralysis in the legs (and sometimes arms, depending on the level of spinal cord involvement), sensory disturbances including pain, numbness, tingling, and abnormal sensitivity below the level of the lesion, as well as bowel and bladder dysfunction. Symptoms typically develop over hours to days and can range from mild to severe. A characteristic feature is a clearly defined sensory level, meaning there is a distinct boundary on the body below which sensation is altered. Back pain at the level of the inflammation is also commonly reported. There is no cure for idiopathic acute transverse myelitis, and treatment focuses on reducing spinal cord inflammation and managing symptoms. First-line therapy typically involves high-dose intravenous corticosteroids (such as methylprednisolone). For patients who do not respond adequately to steroids, plasma exchange (plasmapheresis) or intravenous immunoglobulin (IVIG) may be considered. Long-term management includes physical rehabilitation, pain management, and treatment of bladder and bowel complications. Recovery is variable: approximately one-third of patients recover well, one-third have moderate residual deficits, and one-third experience severe permanent disability. Early and aggressive treatment is associated with better outcomes.
Sporadic
Usually appears on its own, not inherited from a parent
Variable
Can begin at different ages, from infancy through adulthood
Treatments
No FDA-approved treatments are currently listed for Idiopathic acute transverse myelitis.
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Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Idiopathic acute transverse myelitis.
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Common questions about Idiopathic acute transverse myelitis
What is Idiopathic acute transverse myelitis?
Idiopathic acute transverse myelitis (ATM) is a rare neurological disorder characterized by acute inflammation of the spinal cord that occurs without an identifiable underlying cause. The term 'idiopathic' distinguishes it from transverse myelitis that occurs secondary to known conditions such as multiple sclerosis, neuromyelitis optica spectrum disorder, systemic autoimmune diseases, or infections. The inflammation damages the myelin sheath (the protective covering of nerve fibers) and can also injure the nerve cells themselves, disrupting communication between the spinal cord and the rest of
How is Idiopathic acute transverse myelitis inherited?
Idiopathic acute transverse myelitis follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
Which specialists treat Idiopathic acute transverse myelitis?
14 specialists and care centers treating Idiopathic acute transverse myelitis are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.