What are the typical symptoms of myelomalacia?

Typical Symptoms of Myelomalacia

Myelomalacia presents primarily with progressive neurological deficits including sensory and motor dysfunction, with symptoms varying based on the location and extent of spinal cord damage. 1, 2

Clinical Presentation

Sensory Symptoms

• Paresthesia (abnormal sensations) including numbness, tingling, and burning sensations in a symmetric distribution 3
• Progressive loss of sensation, particularly affecting proprioception and vibratory sensitivity 3
• Elevated thresholds for detecting touch, temperature, and pain 3

Motor Symptoms

• Progressive weakness that typically begins distally and may progress proximally 3
• Difficulty with fine motor tasks and hand clumsiness when cervical spine is involved 1
• Wide-based, unsteady gait (ataxia) 1
• Muscle weakness that can range from mild to severe, potentially leading to paralysis 3
• Reduced deep tendon reflexes proportional to sensory loss 3

Autonomic Symptoms

• Bowel and bladder dysfunction, potentially progressing to incontinence in severe cases 1, 4
• Orthostatic hypotension may occur in some cases 3

Disease Progression

• In early stages, symptoms may be mild and limited to sensory changes 2
• As the condition progresses, motor deficits become more prominent 2
• Advanced cases may show central cystic degeneration, syrinx formation, and spinal cord atrophy 2
• In some cases, particularly with diffuse myelomalacia, there may be an "ascending syndrome" with cranial migration of neurological deficits 5

Clinical Patterns Based on Location

• Cervical myelomalacia typically presents with upper and lower extremity symptoms, though atypical presentations with only lower extremity symptoms can occur 1
• Thoracic myelomalacia often presents with lower extremity weakness and sensory changes below the level of the lesion 4
• Lumbar intumescence involvement carries a higher risk of progressive myelomalacia 6

Diagnostic Considerations

• Magnetic resonance imaging (MRI) is the gold standard for diagnosis, showing characteristic signal changes 2
• Early stage myelomalacia appears as high-intensity signal changes on T2-weighted MRI images 2
• Intermediate stage shows varying degrees of cystic necrosis of the central gray matter 2
• Late stage demonstrates central cystic degeneration, syrinx formation, and atrophy 2

Prognosis

• Early stage myelomalacia may be reversible depending on the severity of initial spinal cord injury 2
• Intermediate and late stages typically show either no change or progression of disease 2
• Diffuse or progressive myelomalacia has a poor prognosis and can be fatal, particularly when there is cranial migration of neurological signs 5

Risk Factors for Progressive Myelomalacia

• Location of spinal cord injury (lumbar intumescence carries higher risk) 6
• Delayed surgical intervention (>12 hours after loss of ambulation) increases risk 6
• Complete sensorimotor loss following intervertebral disc extrusion 6

Early recognition of symptoms and prompt intervention are crucial for managing myelomalacia, as early stages may be reversible while advanced stages typically have poor outcomes.