Among disorders that cause sensory loss, the most distinctive are those that affect the sensory ganglia. These disorders are called sensory neuronopathies or sensory ganglionopathies. The importance of sensory ganglia, in addition to the unique topography of sensory loss that occurs when they are diseased, lies in their close association with general medical disorders, and paraneoplastic disorders in particular. Furthermore, ganglia may be susceptible to autoimmune attack because of the fenestrated endothelial cells that form a permeable blood–nerve barrier.
Investigations to Evaluate Sensory Ganglionopathies
|Assessment in all cases||Sensory- and motor-nerve conduction studies to look for demyelinating or axonal physiology (results should be axonal and purely sensory in large-fiber sensory ganglionopathy but may be normal in small-fiber sensory ganglionopathy)
Consider cerebrospinal fluid testing
|Assessment according to specific cause|
|Paraneoplastic syndrome||Testing for anti-Hu, anti-CRMP5, and anti-amphiphysin antibodies SPEP or IFE assays for monoclonal gammopathy, which can be seen with lymphomas and plasmacytomas
Whole-body PET-CT to look for cancer, followed by diagnostic biopsy
|Sjögren’s syndrome||Testing for ANA, anti-Ro, and anti-La antibodies
Testing with Schirmer’s test or rose–bengal staining for keratoconjunctivitis sicca
Minor salivary gland biopsy
|Celiac disease||Consider testing for antigliadin and antitransglutaminase autoantibodies
Endoscopy and tissue biopsy†
|Infection||HIV serologic testing (also consider testing for HTLV-1, EBV, VZV, Zika virus, leprosy)|
|Vitamin toxicity||Measurement of vitamin B6 (pyridoxine) level|
|Assessment of idiopathic large- and small-fiber sensory ganglionopathies||Consider testing for anti–trisulfated heparin disaccharide and anti–fibroblast growth factor receptor 3 antibodies‡|
|Assessment of idiopathic small-fiber sensory ganglionopathies||Consider skin biopsies of distal leg and thigh to measure intraepidermal nerve-fiber density|
*ANA denotes antinuclear antibodies, CRMP5 collapsin response mediator protein 5, IFE immunofixation electrophoresis, PET-CT positron-emission tomography–computed tomography, and SPEP serum protein electrophoresis.
† Antigliadin and antitransglutaminase autoantibodies are not specific for celiac disease or the associated subtype of
peripheral neuropathy. They may be seen in axonal sensory and sensorimotor ganglionopathies.
‡ Anti–trisulfated heparin disaccharide and anti–fibroblast growth factor 3 receptor antibodies are not specific for the subtype of peripheral neuropathy. They may be seen in axonal sensory and sensorimotor ganglionopathies, and their presence does not indicate an immunologic cause of the neuropathy.
- Amato AA, Ropper AH. Sensory Ganglionopathy. N Engl J Med. 2020 Oct 22;383(17):1657-1662. [Medline]
- Gwathmey KG. Sensory neuronopathies. Muscle Nerve. 2016 Jan;53(1):8-19. [Medline]
Created Jun 17, 2021.