Myelopathy is one of the three major symptoms of superficial siderosis and is defined as an acute or subacute spinal cord dysfunction, slowly progressive, and caused by demyelination. Non superficial siderosis related conditions will also contribute negatively to the progression. Myelopathy is aggravated by arthritic changes (spondylosis) which cause narrowing of the spinal canal (spinal stenosis) resulting in compression of the spinal cord.

Myelopathy is typically diagnosed by a clinical exam. The signs and symptoms may include:

  • Neck, arm, leg or lower back pain.
  • Tingling, numbness or weakness.
  • Difficulty with fine motor skills, such as writing, using utensils or buttoning a shirt
  • Difficulty walking
  • upper motor neuron signs: weakness, clumsiness, altered tonus
  • hyperreflexia: increased or abnormal twitching or spastic reflexes 
  • lower motor neuron signs: muscle group weakness in the area of spinal cord compromise, muscle atrophy, hyporeflexia, muscle hypotonicity or flaccidity
  • sensory deficits
  • Loss of urinary or bowel control 
  • sexual dysfunction.

Myelopathy can also be evaluated by your neuropsychologist using Transcranial Magnetic Stimulation (TMS). This method measures the time required for the neural impulse to cross the pyramidal tracts. This time measurement is called Central Conduction Time (CCT).  Your physician will use this test to determine whether myelopathy exists, identify which section of the spinal cord is affected, and responsible for the myelopathy. It also allows your doctor to track the progression of the myelopathy

Please refer to the individual symptoms that are grouped under the myelopathy category for more specific information.



Sources: Superficial siderosis is a rare neurologic disease characterized by progressive sensorineural hearing loss, cerebellar ataxia, pyramidal signs, and neuroimaging findings revealing hemosiderin deposits in the spinal and cranial leptomeninges and subpial layer. The disease progresses slowly, and patients may present with mild cognitive impairment, nystagmus, dysmetria, spasticity, dysdiadochokinesia, dysarthria, hyperreflexia, and Babinski signs. Additional features reported include dementia, urinary incontinence, anosmia, ageusia, and anisocoria. Superficial siderosis MedGen UID: 831707 •Concept ID: CN226971 •Finding Orphanet: ORPHA247245
Chen R, Cros D, Curra A, Di Lazzaro V, Lefaucheur JP, Magistris MR, Mills K, Rösler KM, Triggs WJ, Ugawa Y, Ziemann U. The clinical diagnostic utility of transcranial magnetic stimulation: report of an IFCN committee. Clin Neurophysiol. 2008 Mar;119(3):504-32.
Dr. Atkinson, Patty (March 27, 2013). “Cervical Myelopathy”. Mayo Clinic News Network. Mayo Clinic
Living With SuperficialSiderosis Website PubMed Reference Library 

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