Dynamic MRI testing of the cervical spine has prognostic significance in patients with progressive upper-limb distal weakness and atrophy

Marc Gotkine, Alon Abraham, Vivian E. Drory, Zohar Argov, John Moshe Gomori, Sergiu C. Blumen

Research output: Contribution to journalArticlepeer-review

Abstract

Background: The syndrome of isolated progressive upper-limb distal weakness and atrophy results from disease processes affecting lower motor neurons originating in the cervical anterior horn gray matter. Lower motor neuron dysfunction restricted to the C7-T1 myotomes in the absence of neuropathy, upper motor neuron signs, sphincter disturbances or abnormality on conventional MR imaging is suggestive of either Hirayama disease (HD), or the initial manifestation of a progressive motor neuron disease such as amyotrophic lateral sclerosis (ALS). In HD the supposed etiologic mechanism is a mechanical compression of the cervical spinal cord during neck flexion; therefore, dynamic MRI (dMRI) of the cervical cord might help differentiate between these possibilities. Methods: This was a multi-center observational cohort study. Over a 4-year period between 8/2009 and 8/2013, 22 patients were identified as having a disease consistent with HD. We identified a subgroup of patients suspected of suffering fromactive progressive disease and prospectively followed themafter performing dynamic MRI studies of the cervical spine. Results: Twenty-two patients were identified as having a disease consistent with HD, of whom8 were defined as having actively progressive disease. Seven of these 8 patients demonstrated clear dynamic compression of the cervical spine during neck flexion. The patient who did not demonstrate the typical MRI changes associated with HD went on to develop generalized ALS. Conclusions: dMRI has a practical role in patients presenting with progressive upper-limb distal weakness and atrophy, and the presence of characteristic changes typical of HD may suggest a more optimistic prognosis.

Original languageEnglish
Pages (from-to)168-171
Number of pages4
JournalJournal of the Neurological Sciences
Volume345
Issue number1
DOIs
StatePublished - 15 Oct 2014

Keywords

  • ALS
  • Hirayama disease
  • Monomelic amyotrophy
  • Motor-neuron disease
  • Spinal cord

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