Severe sensory ganglionopathy as a manifestation of mixed connective tissue disease

Abstract

Sensory ganglionopathies (SG) are a rare but distinct clinical subgroup of peripheral neuropathies characterized by damage to dorsal root ganglia. Typical manifestations include early gait and limb ataxia, widespread diminished or absent deep tendon reflexes accompanied by Romberg sign and pseudoathetoid movements.The diagnosis of SG is valuable since it may prompt towards early recognition of an underlying malignancy or autoimmune disorder. We report the case of a female diagnosed with mixed connective tissue disease (MCTD) along with severe SG. To our knowledge, such disease association has not been reported yet. The pathophysiology in cases linked to MCTD is unclear and asks for further studies. Moreover, the important degree of disability associated with this condition highlights the need for effective therapies’ development.

Keywords

• mixed connective tissue disease
• dorsal root ganglia
• sensory ganglionopathy
• sensory neuronopathy