Radiologic and histologic findings in Sjögren's sensory neuronopathy

Achados radiológicos e histológicos da neuronopatia sensitiva da síndrome de Sjögren

A 27-year-old woman presented with a two-year history of asymmetric paresthesia in her hands and feet. Neurological examination revealed global areflexia, marked loss of position sense and normal motor strength in all limbs. Nerve conduction studies showed absence of sensory potentials with no abnormalities in electromyography. These findings were compatible with a diagnosis of sensory neuronopathy. Spine MRI demonstrated hyperintensity in the posterior columns (Figures A and B). Salivary gland biopsy (Figure C) was diagnostic for Sjögren's syndrome. Sensory neuronopathy is a rare type of peripheral neuropathy, which can occur among Sjögren's syndrome patients and usually antedates the diagnosis of Sjögren's syndrome1^,2.

Figure
Axial T2 gradient-echo images (A and B) show hyperintensity involving the posterior columns of the spinal cord at cervical (A) and thoracic (B) levels (arrows). Histopathology images (C) shows lymphocytic infiltrate with complete destruction of the excretory duct. Acinic serous metaplasia and thrombi in small blood vessels are also seen (H&E, original magnification X200).

References

• Gwathmey KG. Sensory neuronopathies. Muscle Nerve 2016 Jan;53(1):8-19. https://doi.org/10.1002/mus.24943
  » https://doi.org/10.1002/mus.24943
• Martinez ARM, Nunes MB, Nucci A, França Junior MC. Sensory neuronopathy and autoimmune diseases. Autoimmune Dis. 2012;2012: 873587. https://doi.org/10.1155/2012/873587
  » https://doi.org/10.1155/2012/873587

Publication Dates

• Publication in this collection
  10 Jan 2020
• Date of issue
  Dec 2019

History

• Received
  01 June 2019
• Accepted
  16 July 2019