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NEUROLOGY 2008;70:e27
© 2008 American Academy of Neurology
Resident and Fellow Section

Teaching NeuroImage: Cervical cord atrophy with dorsal root ganglionopathy in Sjögren syndrome

Chih-Chun Lin, MD and Ming-Jang Chiu, MD, PhD

From the Departments of Neurology, National Taiwan University Hospital, College of Medicine, National Taiwan University, Taipei, Taiwan.

Address correspondence and reprint requests to Dr. Ming-Jang Chiu, Department of Neurology, National Taiwan University Hospital, No. 7, Chung-Shan S. Rd., Taipei, 100, Taiwan mjchiu{at}ntu.edu.tw

A 59-year-old woman had had asymmetric numbness from her neck to both arms and left leg for 5 years. She had had dry eyes and mouth for many years. Examination showed sensory ataxia, generalized areflexia, positive Romberg test, and hand pseudoathetosis. Vibration sense was reduced in both lower limbs; the severity was more on the left side but was not length dependent. Joint position sense was impaired in all limbs while pain and temperature senses were comparatively preserved. No sensory level was demarcated. Electrophysiologic studies revealed absence of sensory action potentials in all sampled nerves and prolonged central conduction time from median nerves on somatosensory-evoked potentials.1,2 Her anti-SSA antibody, rheumatoid factor, and Schirmer test were positive. MRI (figure) showed cervical cord atrophy with increased T2 signal intensity at the dorsal column which implied a consequence of centripetal Wallerian degeneration from dorsal root ganglionopathy in a patient with Sjögren syndrome.


Figure 120
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  		Figure MRI showing increased signal intensity at the dorsal column (A) and atrophy (B) of the cervical cord on T2-weighted images

The axial image is at the level of C3/C4 and the cord atrophy extends from C2 to C6.

 

Disclosure: The authors report no conflicts of interest.


   REFERENCES
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 REFERENCES
 

  1. Malinow K, Yannakakis GD, Glusman SM, et al Subacute sensory neuronopathy secondary to dorsal root ganglionitis in primary Sjögren's syndrome. Ann Neurol 1986;20:535–537.[Medline]
  2. Lauria G, Pareyson D, Sghirlanzoni A. Neurophysiological diagnosis of acquired sensory ganglionopathies. Eur Neurol 2003;50:146–152.[Medline]



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