The n e w e ng l a n d j o u r na l of m e dic i n e

Review Article

Dan L. Longo, M.D., Editor

Sensory Ganglionopathy
Anthony A. Amato, M.D., and Allan H. Ropper, M.D.​​

A
mong disorders that cause sensory loss, the most distinctive From Brigham and Women’s Hospital and
are those that affect the sensory ganglia. These disorders are called sen- Harvard Medical School, Boston (A.A.A.).
Address reprint requests to Dr. Amato at
sory neuronopathies or sensory ganglionopathies.1-5 The sensory ganglia Brigham and Women’s Hospital, 60 Fen-
contain the cell bodies of the sensory nerves and lie alongside both sides of the wood Rd., Boston, MA 02115, or at ­aamato@​
spinal cord, appended to the dorsal root of each spinal nerve. Their proximal pro- ­bwh​.­harvard​.­edu.

jections are the sensory nerve roots that enter the spinal cord, and their periph- N Engl J Med 2020;383:1657-62.
eral projections are the sensory fibers of peripheral nerves. The importance of DOI: 10.1056/NEJMra2023935
Copyright © 2020 Massachusetts Medical Society.
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 disor-
ders, 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. Paraneoplastic sensory ganglionopathy is
thought to be caused by cytotoxic T cells stimulated by antigens in the tumor that
cross-react with epitopes on sensory ganglia neurons. Sensory ganglionopathy as-
sociated with Sjögren’s syndrome or checkpoint inhibitors and idiopathic sensory
ganglionopathy are also likely to be mediated by cytotoxic T cells. The focus of
this review is on the acquired sensory ganglionopathies. Rare hereditary ganglion-
opathies (hereditary sensory and autonomic neuropathies and Fabry’s disease) are
beyond the scope of this review.

Cl inic a l Fe at ur e s
The clinical features of sensory ganglionopathies differ in many ways from the
more common peripheral neuropathies. First, because they are confined to sen-
sory neurons, sensory ganglionopathies do not cause weakness. Second, most
common polyneuropathies (e.g., those due to diabetes) result from axonal damage,
which leads to degeneration in the distal parts of the longest nerves and symptoms
that begin in the toes, ascend the legs, and only later appear in the fingers and
proximal parts of the body. In contrast, sensory ganglionopathies are not depen-
dent on the length of axons, and sensory features can start in any or all territories
innervated by sensory neurons, particularly in the face, scalp, oral mucosa, trunk,
and proximal limbs. Third, most sensory ganglionopathies affect the neurons of
large nerve fibers and, correspondingly, are associated with loss of touch, vibra-
tory perception, and proprioception; pseudoathetoid posturing that represents a
search for position in space; Romberg’s sign; and loss of tendon reflexes. Less
commonly, ganglionopathies affect only the neurons of small sensory fibers,
resulting in burning, tingling, and stabbing pains, with physical examination re-
vealing impaired pinprick and temperature perception but preservation of large-
fiber sensory functions and reflexes. Disease processes that affect the sensory
ganglia often also damage the autonomic ganglia, so patients with sensory gan-
glionopathies may also have dysautonomia (e.g., orthostatic hypotension, cardiac
arrhythmia, gastric dysmotility, and sweating disturbances).

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 The n e w e ng l a n d j o u r na l of m e dic i n e

Paraneoplastic Ganglionopathy
Table 1. Types and Causes of Acquired Sensory Ganglionopathies.*
The most common malignant process associated
Type of Ganglionopathy Causes with sensory ganglionopathy is small-cell lung
Paraneoplastic Small-cell lung cancer, bronchial carcinoma, carcinoma, but the syndrome has also been as-
breast cancer, ovarian cancer, prostate can- sociated with breast, ovarian, prostate, colon,
cer, lymphoma, neuroendocrine tumors, or
sarcoma
and gastric cancers, lymphoma, neuroendocrine
tumors, and other cancers.6-9 Sensory symptoms
Systemic autoimmune Sjögren’s syndrome, systemic lupus erythema-
tosus, MCTD, or rheumatoid arthritis can be acute or insidious and precede the diag-
nosis of cancer by weeks or months. Additional
Infection-related HIV, HTLV-1, EBV, Zika virus, enterovirus, or
VZV infection or leprosy paraneoplastic syndromes may develop, such as
Drug- or toxicity-related Platinum-based chemotherapy (cisplatin, oxali-
myelitis, motor neuropathy, the Lambert–Eaton
platin, or carboplatin) myasthenic syndrome, encephalitis, or cerebellar
Vitamin B6 (pyridoxine) toxicity degeneration.
Checkpoint inhibitors
Antineuronal autoantibodies directed against
Idiopathic Large-fiber sensory ganglionopathy a 35-to-40-kD protein or complex of proteins,
Small-fiber sensory ganglionopathy
the Hu antigen, anti–collapsin response media-
* EBV denotes Epstein–Barr virus, HIV human immunodeficiency virus, HTLV-1 tor protein 5 (CRMP5) antibody, or amphiphysin
human T-cell lymphotropic virus type 1, MCTD mixed connective-tissue disease, are found in serum or cerebrospinal fluid (CSF)
and VZV varicella–zoster virus.
from most patients with paraneoplastic sensory
ganglionopathy.7-9 Autopsy studies have shown
Because the proximal projections of sensory inflammation and loss of ganglion cells in the
ganglion cells form the posterior spinal roots dorsal root ganglia (Fig. 1A) and wallerian de-
that contain spinocerebellar fibers, ganglionop- generation of the posterior columns in the spinal
athies may cause a special form of ataxia that cord (Fig. 1B).6,10
simulates cerebellar disease but without dysar-
thria and nystagmus. The combination of proxi- Sjögren’s Syndrome
mal sensory symptoms and sensory loss with Polyneuropathies occur in 5 to 15% of patients
ataxia, with preserved strength, is the arche- with Sjögren’s syndrome, of which approximate-
typal sensory ganglionopathy syndrome. Most ly 40% are large-fiber sensory ganglionopathies
cases evolve over a period of days or weeks — and 20% are small-fiber sensory neuropathies or
more rapidly than typical peripheral neuropa- ganglionopathies.1-3,11-14 Symptoms may involve the
thies — and tend to become severe, affecting arms more than the legs and may be asymmetric
tactile sensibility to a degree that, for example,at the start but usually become generalized. Fa-
precludes a normal swallowing reflex because of cial and perioral numbness from involvement of
loss of pharyngeal sensation. Owing to these the trigeminal ganglia is common. Less than
seemingly inexplicable symptoms and the un- 50% of patients with Sjögren’s syndrome–related
usual topographic pattern of sensory loss with sensory ganglionopathy have sicca symptoms at
normal electrophysiological studies early in the the onset of the disease. Antinuclear, Ro (SSA),
disorder, patients with sensory ganglionopathies and La (SSB) antibodies are present in 10 to 55%
are often thought to have a functional neuro- of patients,3,14 but Schirmer’s test is positive
logic disorder. Criteria have been developed to (showing deficient lacrimation in keratoconjunc-
help diagnose sensory ganglionopathies.4 tivitis sicca) in more than 90%, rose bengal test-
ing is positive (showing staining of conjunctiva
when tearing is reduced) in nearly 70%, and
C ause s
salivary gland biopsy is positive in more than
The importance of sensory ganglionopathies in 90%.3,13 Less commonly, sensory ganglionopathy
general medicine is their association with under- complicates other rheumatologic diseases such
lying systemic disease, usually inflammatory or as systemic lupus erythematosus.
neoplastic, which is often manifested after the
development of neurologic symptoms (Table 1). Celiac Disease
In approximately half of patients, the disorder There is an uncertain relationship between celiac
remains idiopathic despite extensive evaluation. disease and sensory ganglionopathy.1-3,15 A con-

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 Sensory Ganglionopathy

Platins
A
Cisplatin, carboplatin, and oxaliplatin in large
cumulative doses cause sensory ganglionopa-
thy,1-3,19 with ataxia, pseudoathetoid movements,
and Lhermitte’s sign. Symptoms may begin to
appear as many as 8 weeks after the drug has
been stopped and may progress for up to
6 months. The proposed mechanism is inter-
strand and intrastrand DNA cross-linking that
preferentially damages dorsal root ganglion
neurons.

B Pyridoxine Toxicity
Pyridoxine (vitamin B6) in high doses (usually at
doses >2 g per day but possibly with doses as
low as 116 mg per day; recommended daily al-
lowance is 2 to 4 mg) has been associated with
dysesthesias and sensory ataxia.1-3,20 Studies in
animals have shown loss of dorsal root ganglion
cells and degeneration of the posterior column
tracts. Many, but not all, patients have improve-
ment after discontinuation of the pyridoxine.

Figure 1. Paraneoplastic Sensory Ganglionopathy
in a Patient with Anti-Hu Antibodies and Small-Cell
Lung Cancer.
A specimen from the cervical spinal cord shows marked
parenchymal and perivascular inflammation of the dor-
sal root ganglia, with loss of ganglion cells and fibrosis
(Panel A, hematoxylin and eosin), and a section from the
cervical cord shows marked pallor of the dorsal columns
(Panel B, arrows; Luxol fast blue–hematoxylin and eosin).
Images are reprinted from Amato and Anderson.6
founding issue is nutritional deficiency as a
cause of sensory neuropathy. Autopsies in three
patients with celiac disease and sensory symp-
toms showed inflammation in the dorsal root
ganglia with degeneration of the posterior col-
umns of the spinal cord.15 Some patients may
have improvement with a gluten-free diet, but
most do not.
Infections
Ganglionitis is a rare complication of human
immunodeficiency virus infection and is an even
more rare complication of human T-lympho-
tropic virus type 1, enterovirus, Zika virus, and
Epstein–Barr virus infections and leprosy.1-3,16-18
Varicella–zoster virus infection causes a focal
sensory ganglionitis but usually does not involve The diagnostic workup includes testing for serum
more than a few contiguous segments. autoantibodies (antinuclear, anti-Ro, and anti-La
Checkpoint Inhibitors
Immune checkpoint inhibitors may induce an
autoimmune attack against sensory ganglia.21
Checkpoint inhibitors may also worsen or un-
mask underlying autoimmune conditions (e.g.,
Sjögren’s syndrome) and lead to sensory gangli-
onopathy.22
Idiopathic Sensory Ganglionopathy
Approximately half of all cases of sensory gan-
glionopathy are idiopathic.1-5 Most idiopathic
cases are of the large-fiber variety, with sensory
ataxia developing in middle age, but the process
may begin at any time, including in childhood.
Idiopathic sensory ganglionopathy is more com-
mon in females than in males in all age groups.
Symptoms can develop over a period of hours or
insidiously over a period of years, and the course
may be monophasic, chronically progressive, or
relapsing. Idiopathic cases of the small-fiber
variety are less common and cause severe pain
and widely distributed superficial pain, some-
times appearing acutely after an infection (e.g.,
a urinary tract infection).5,23-26
Di agnos t ic E va luat ion

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 The n e w e ng l a n d j o u r na l of m e dic i n e

Table 2. Investigations to Evaluate Sensory Ganglionopathies.*

Variable Evaluation
Assessment in all cases Sensory- and motor-nerve conduction studies to look for demyelin-
ating 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 keratocon-
junctivitis sicca
Minor salivary gland biopsy
Celiac disease Consider testing for antigliadin and antitransglutaminase autoanti-
bodies
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 Consider testing for anti–trisulfated heparin disaccharide and
sensory ganglionopathies anti–fibroblast growth factor receptor 3 antibodies‡
Assessment of idiopathic small-fiber sensory Consider skin biopsies of distal leg and thigh to measure intraepider-
ganglionopathies mal 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.

antibodies), positron-emission tomography–com-
puted tomography to detect occult cancer, and
biopsy of suspicious masses (Table 2). Autoanti-
bodies directed against trisulfated heparin disac-
charide and fibroblast growth factor receptor 3
may be found in patients with sensory-predomi-
nant neuropathies or neuronopathies, but the
specificity and pathogenic role are unclear.27-30
Lip or parotid gland biopsy can be performed for
tissue confirmation of Sjögren’s syndrome if
serologic testing is negative. CSF examination in
patients with sensory ganglionopathy may show
elevated protein levels, mild pleocytosis, or oli-
goclonal bands, but these findings are neither
sensitive nor specific, and the CSF examination
is often normal. Sensory-nerve conduction stud-
ies in the common large-fiber sensory ganglion-
opathies show reduced amplitudes of sensory-
nerve action potentials (SNAPs) with relative
sparing of sensory latencies and conduction ve-
locities, but these tests may be normal early in
the disorder. Results of motor-nerve conduction
studies and needle electromyographic studies are
usually normal. The reductions in SNAPs can be
asymmetric or generalized, usually correspond-
ing to the clinical pattern of sensory symptoms
and loss. When the SNAPs in the arms (e.g.,
radial, median, or ulnar) or the blink reflex (as-
sessing trigeminal sensory ganglion) are affect-
ed but those in the lower legs (sural and super-
ficial peroneal) are normal, the findings support
a pattern of involvement that is not nerve-length–
dependent and is characteristic of a sensory
ganglionopathy. Sensory-nerve conduction tests,
which assess the neurophysiology of large-diam-
eter fibers and SNAP amplitudes, are normal in
patients with small-fiber ganglionopathies. In
such patients, autonomic testing may show ab-
normalities in sudomotor function, as well as
abnormal heart-rate and blood-pressure responses

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 Sensory Ganglionopathy

during the Valsalva maneuver and heart-rate cess.1-3,11-14 Treating an underlying cancer usually
variability with deep breathing. fails to lead to remission of the ganglionopathy,
Magnetic resonance imaging may show swell- but exceptions occur. It is likely that improve-
ing, increased signal on T2-weighted images, or ment with treatment is limited by destruction of
enhancement in the dorsal root ganglia, as well the cell body of the sensory neuron. However,
as degeneration of the posterior columns in the immunotherapy occasionally halts disease pro-
spinal cord.26,31-33 Dorsal root ganglia biopsy is gression, and some form of immunotherapy may
rarely recommended, although it can show a be undertaken if the neurologic syndrome is pro-
CD8+ T-cell–mediated attack directed against gressive and has a duration of days or weeks. A
sensory ganglia neurons in autoimmune cases. case series showed improvement in patients who
Sensory-nerve biopsy may reveal a preferential were treated within the first 2 months after the
loss of large myelinated fibers and an absence of onset of symptoms, with stabilization of the dis-
clusters of small regenerating fibers — a feature order in those treated within the first 8 months.34
that may help distinguish neuronopathy from
axonal neuropathy. (Once the cell body is dead, Sum m a r y
axons cannot regenerate.) However, these find-
ings are nonspecific, and nerve biopsy is infre- The sensory ganglionopathies are disabling syn-
quently performed. Skin biopsy with immuno­ dromes that, unlike typical sensory neuropa-
staining may reveal reduced intraepidermal thies, are characterized by symptoms that de-
nerve-fiber density in a pattern that is not nerve-velop in the face, trunk, and arms before they
length–dependent (e.g., a greater reduction in the appear in the distal legs; by sensory ataxia in the
density of fibers in the thigh or arm than in common large-fiber ganglionopathies; and by a
the distal leg).5,25,26 severe burning, tingling, and itchy sensation in
the less common small-fiber ganglionopathies.
T r e atmen t of Im mune-Medi ated Investigation is undertaken for underlying can-
Sensor y G a ngl ionopathie s cer and for Sjögren’s syndrome, but half of the
cases are idiopathic. Immunotherapies are not
A variety of immunotherapies (e.g., glucocorti- usually effective but may be useful in cases of
coids, immunosuppressive agents, plasma ex- recent onset.
change, intravenous immune globulin, and ritux- Dr. Amato reports serving on advisory boards for Sarepta,
imab) have been tried in different forms of Alexion, CSL Behring, and Strongbridge Pharma. No other po-
tential conflict of interest relevant to this article was reported.
paraneoplastic and other immune-mediated sen- Disclosure forms provided by the authors are available with
sory ganglionopathies but usually with little suc- the full text of this article at NEJM.org.

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