REVIEWS

P E R I P H E R A L N E U R O PAT H I E S

CIDP and other inflammatory

1
Aston Brain Centre, School of
Life & Health Sciences, Aston
University, Aston Triangle,
Birmingham B4 7ET, UK.
2
Regional Neuromuscular
Service, University Hospitals
Birmingham, Birmingham
B15 2WB, UK.
3
Department of Neurology,
University Hospital Essen,
Hufelandstrasse 55,
D-45147 Essen, Germany.
4
Department of Neurology,
Medical Faculty, Research
Group for Clinical and
Experimental
Neuroimmunology,
Heinrich-Heine University,
Moorenstrasse 5, 40225
Düsseldorf, Germany.
5
Weill Cornell Medicine-Qatar,
Education City, PO Box
24144, Doha, Qatar.
6
Division of Cardiovascular
Medicine, University of
Manchester, 46 Grafton
Street, Manchester M13 9NT,
UK.
Correspondence to Y.A.R.
y.rajabally@aston.ac.uk
doi:10.1038/nrneurol.2017.123
Published online 15 Sep 2017
neuropathies in diabetes —
diagnosis and management
Yusuf A. Rajabally1,2, Mark Stettner 3,4, Bernd C. Kieseier4, Hans-Peter Hartung4
and Rayaz A. Malik5,6
Abstract | Distal symmetric polyneuropathy (DSPN) is the most common neuropathy to occur in
diabetes mellitus. However, patients with diabetes can also develop inflammatory neuropathies,
the most common and most treatable of which is chronic inflammatory demyelinating
polyradiculoneuropathy (CIDP). Whether diabetes is a risk factor for CIDP remains under debate.
Early studies suggested that patients with diabetes were at increased risk of CIDP, but
epidemiological studies failed to confirm the association, and subsequent data have re‑opened
the debate. Inadequate interpretation of investigations and differentials between CIDP and other
neuropathies that can occur in diabetes, such as DSPN, diabetic radiculoplexus neuropathies and
vasculitic multiple mononeuropathy, might mean that CIDP is under-recognized. Despite a
response rate of >80% to first-line therapies for CIDP in patients with or without diabetes, those
with diabetes often present with greater disability owing to late referral and axonal pathology
attributed to DSPN. The increasing worldwide prevalence of diabetes creates an urgent need to
improve identification of potentially treatable neuropathies, such as CIDP. In this Review, we
consider the features of CIDP in patients with diabetes, and discuss how these features can be
used to differentiate the condition from other neuropathies. We also review the management
options for CIDP and other inflammatory neuropathies in patients with diabetes.
Diabetic neuropathy is the most prevalent chronic with neurological deficits that are not typical of DSPN,
complication of diabetes mellitus (referred to through­ inflammatory ­neuropathies must be considered.
out this Review as diabetes). The term diabetic In this Review, we consider the features of CIDP in
­neuropathy is normally used to refer to the forms patients with diabetes, and discuss how these features
of neuro­pathy that are most prevalent among patients can be used to differentiate the condition from other
with diabetes, which are distal symmetric polyneuro­ neuropathies. We also review the management options
pathy (DSPN) and diabetic autonomic neuropathies1–4; for CIDP and other inflammatory neuropathies in
DSPN presents in 10–15% of patients with newly diag­ patients with diabetes.
nosed type 2 dia­betes, and occurs in 36.8% of patients
who have had ­diabetes for >10 years5,6. However, not all Clinical features of CIDP
patients with diabetes and neuropathy symptoms have CIDP is a heterogeneous entity caused by an
these typical diabetic neuro­pathies1–4,7, and patients immune-mediated inflammatory process that involves
with diabetes can develop inflammatory neuropathies. nerve roots, plexuses and peripheral nerve trunks. In its
These inflammatory neuropathies include diabetic typical form, CIDP produces symmetrical proximal and
radiculoplexus neuro­pathies and vasculitic multiple distal muscle weakness in all four limbs, large-fibre sen­
mononeuropathies, but the most common and most sory loss, and reduced or absent reflexes9. CIDP is usually
treatable is chronic inflammatory demyelinating poly­ progressive over at least 8 weeks, although it can occur in
neuropathy (CIDP). Therefore, neuropathies that are not a relapsing–remitting pattern with long periods of remis­
typically associated with diabetes and might be treatable sion10–12. Atypical CIDP might be as common as typical
should be actively sought and managed in patients with forms13, and is diverse, including focal and multifocal
diabetes8, and when a patient with diabetes presents asymmetric, distal, pure motor and pure sensory forms9.

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Key points Societies–Peripheral Nerve Society (EFNS–PNS) criteria)

• The main neuropathy that occurs in diabetes is distal symmetric polyneuropathy
(DSPN), but inflammatory neuropathies can also occur
• Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is the most
common and most treatable inflammatory neuropathy in patients with diabetes,
although the extent of its occurrence in association with diabetes is debated
• Other inflammatory neuropathies that occur in diabetes are diabetic radiculoplexus
neuropathies and vasculitic multiple mononeuropathy
• Diagnosis of CIDP in the presence of diabetes can be made mainly on the basis of
clinical characteristics and specific electrophysiological criteria; cerebrospinal fluid
analysis, imaging and neuropathology are occasionally helpful
• The amenability of CIDP to treatment makes its identification important
• First-line treatment options for CIDP in diabetes include intravenous immunoglobulin
and corticosteroids; plasma exchange can be used when these treatments are
ineffective, and immunosuppression can occasionally be considered in refractory
disease
Is diabetes a risk factor for CIDP?
The frequency of CIDP among patients with diabetes is
uncertain. An association between CIDP and diabetes
has been acknowledged for several decades, but the con­
dition remains poorly recognized owing to the concur­
rent existence of a typical diabetic neuropathy in many
patients. A report published in 1986 described a patient
with diabetes and a relapsing inflammatory demyelinat­
ing polyneuropathy, and is probably one of the earliest
published descriptions of CIDP in a patient with dia­
betes14. Subsequent studies published in the late 1990s also
described the association, and emphasized the importance
of its recognition, given the potential for treatment15,16.
Epidemiological data on the association between CIDP
and diabetes entered the literature from the early 2000s.
A retrospective study from the USA showed that 30 of 87
patients with CIDP had diabetes17. In a French series of
100 consecutive patients with diabetes (74 of whom had
type 2 diabetes) and neuropathy who were referred to a
secondary or tertiary centre, nine had CIDP18. Another
North American non-population-based study demon­
strated that the risk of CIDP in patients with diabetes
was 11‑fold higher than in those without diabetes19.
Furthermore, the frequency of CIDP was the same in
type 1 and type 2 diabetes. The same study showed that
the odds of an individual with CIDP having diabetes were
>20‑fold higher than in those with myasthenia gravis or
amyotrophic lateral sclerosis (ALS). The authors acknow­
ledged referral bias as a potential confounding factor,
because patients with typical diabetic neuropathy would
not undergo electrophysiological studies under normal
circumstances. However, they emphasized the very high
frequency of CIDP in patients with diabetes, and felt
that the difference in frequency when compared with
other neuromuscular conditions could not be explained
by selection bias, as the frequencies of diabetes in their
patients with myasthenia gravis or ALS were similar to
those observed in previous population-based ­case–control
epidemiological studies.
In an epidemiological study conducted in the UK,
seven of 46 (15.2%) patients with CIDP (diagnosed
according to the European Federation of Neurological
had diabetes20. In another study from South East England,
ten of 101 (10%) people with CIDP had dia­betes21. Both
of these studies were uncontrolled, but identi­fied a higher
rate of diabetes among people with CIDP than had been
reported among the general population (5–6%)22. In a
study published in 2016, the prevalence of CIDP and dia­
betes was investigated by using a health insurance admin­
istrative claims database (Phar-Metrics Plus™ Database,
Watertown, Massachusetts, USA)23 of over 100 million
patients across the USA. People aged >65 years were
under-represented, and diagnostic criteria for CIDP and
diabetes were lacking, but the frequency of CIDP was six
per 100,000 patients without diabetes and 54 per 100,000
patients with diabetes, a ninefold increase.
Other epidemiological data suggest no associ­ation
of CIDP with diabetes. A large population-based Italian
study of 4,334,225 people showed that among 155
patients with CIDP, only 9% had diabetes, equating to
a standardized morbidity ratio of only 1.07 (95% CI,
0.58–1.80) for diabetes in CIDP24. The methods used to
diagnose diabetes might have been suboptimal, however,
as they relied exclusively on fasting blood glucose ­levels
or the fact that an individual used oral anti­diabetic drugs.
Epidemiological data from the USA have also challenged
the association between CIDP and dia­betes25. Among
260,000 inhabitants of Olmsted County, Minnesota,
the prevalence of CIDP was 8.9 per 100,000 people,
and only one of 23 patients with CIDP (4.3%) had dia­
betes, compared with 14 of 115 (12%) age-matched and
sex-matched controls. Diabetes was diagnosed on the
basis of documented treatment with antidiabetic medi­
cation, two or more fasting blood glucose level values
>126 mg/dl, two or more non-fasting blood glucose level
values >200 mg/dl, or a coded diagnosis in the medical
records. The odds ratio for prevalent diabetes among
patients with CIDP was 0.3 (95% CI 0.04–2.50), and did
not change after adjustment for age, sex and previously
diagnosed diabetes.
The variability in the reported prevalence of coexist­
ing CIDP and diabetes might be attributed to difficul­
ties in accurate identification of individuals with the two
conditions, partly owing to different diagnostic criteria
for CIDP and diabetes, and variation among the popu­
lations studied. Concurrent neuropathic symptoms of
DSPN, the rigour with which electrodiagnostic studies
are undertaken, and the interpretation of the electro­
diagnostic data could contribute to overdiagnosis or
underdiagnosis of CIDP.
Diagnosis of CIDP in diabetes
The same criteria are used to diagnose CIDP in patients
with and without diabetes, and diagnostic information
is provided by a variety of techniques.
Electrophysiology. Diagnosis of CIDP relies heav­
ily on the detection of demyelination by means of
electrophysio­logical tests. Several electrodiagnostic cri­
teria for CIDP have been proposed in the past 30 years26.
A multicentre European study in which different criteria
were evaluated found that the EFNS–PNS criteria offer

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the best combination of sensitivity and specificity27.
These criteria have since become the most widely used
in clinical research worldwide28, and their use is advis­
able in routine clinical practice, as the high sensitivity
minimizes the likelihood that the diagnosis of a treatable
condition will be missed.
Cerebrospinal fluid analysis. Cerebrospinal fluid (CSF)
protein levels are increased in most patients with CIDP.
However, as protein levels can be normal in >50% of
atypical forms29, the precise global sensitivity of this
marker is uncertain, although a figure as high as 95%
has been reported for typical forms30. However, a sub­
stantial proportion of patients with diabetes — in par­
ticular, those with a longer disease duration — can have
high levels of CSF protein31. Consequently, making a
diagnosis of CIDP as opposed to another neuropathy
in a patient with diabetes is challenging on the basis of
CSF protein levels alone, although levels >1 g/l seem
to be exceptional in patients with diabetes unless they
have CIDP18. Use of this value as a cut-off — which, for
similar reasons, has been proposed for distinguishing
between patients with Charcot–Marie–Tooth disease
and those with Charcot–Marie–Tooth disease associated
with CIDP32 — might be helpful.
Imaging. Various imaging techniques have been added
to the diagnostic armamentarium for CIDP in the past
15 years. Magnetic resonance neurography (MRN) might
aid the diagnosis of CIDP by enabling identification of
nerve hypertrophy and/or hyperintensity33,34. Gadolinium
enhancement on MRI, particularly in hypertrophied
nerve roots, might also contribute by indicating the pres­
ence of an inflammatory process, as occurs in CIDP35.
Whether MRN might be useful for differentiating CIDP
from other neuropathies in patients with diabetes is cur­
rently unknown, particularly because several studies have
shown that MRN detects proximal lesions in patients with
DSPN36–38 — this observation raises doubts about the
specificity of changes detected by MRN in patients with
CIDP and diabetes, as opposed to diabetes alone.
A study published in 2017 has shown that diffusion
tensor imaging (DTI) MRN can identify nerve lesions
in patients with type 1 diabetes, reflecting proximal and
distal nerve fibre pathology39. However, nerve abnormal­
ities on DTI have also been described in CIDP40, and
further research is needed to determine whether the
technique can distinguish between the two conditions.
Finally, ultrasound imaging has indicated that cross-­
sectional nerve area enlargement is present in DSPN41,42
and CIDP43,44, and an association between nerve size and
CIDP disease status has been reported45. Future stud­
ies might determine whether CIDP and DSPN can be
differentiated according to sites and patterns of nerve
size abnormalities.
Neuropathology. Early reports of CIDP emphasized
the value of nerve biopsy findings46, but the relevance
of histology is currently a matter of debate among spe­
cialists. As nerve biopsy is an invasive procedure, sam­
ples from healthy controls are rare, so establishing the
normal parameters, which would enable a reliable dis­
ease biomarker to be established, is difficult47. The over­
whelming consensus is that detection of demyelination
in semi-thin or ultra-thin sections is neither specific nor
sensitive for CIDP48, particularly when attempting to
distinguish CIDP from demyelinating hereditary poly­
neuropathies49,50. Total T-cell and macrophage counts,
which are markers of immune-mediated nerve damage,
also have low sensitivity for CIDP diagnosis51,52, although
the presence of perivascular macrophage clusters has
been proposed as a valid criterion to differentiate inflam­
matory neuropathy from other forms of neuropathy with
high sensitivity and specificity53. Some evidence suggests
that other inflammatory markers, such as endoneurial
oedema or the activity of matrix metallo­proteinase‑9
(MMP-9), in patients with diabetes and CIDP have
higher diagnostic value54,55, but these studies were explor­
atory, and the methods used are not widely available47.
For peripheral neuropathology, the quality of the reports
is highly dependent — more so than for other diagnostic
work-ups — on the availability of specialized laborato­
ries and expertise. The consensus among neuromuscular
physicians is that a nerve biopsy is not needed for the
initial diagnosis of CIDP, as it is unlikely to provide a
conclusive answer. Nevertheless, the method might prove
helpful in cases of atypical CIDP48, for the exclusion of
differential diagnoses, and for further investigation when
patients do not respond to treatment.
An alternative technique that has the potential to
aid diagnosis of CIDP is corneal confocal microscopy
(CCM), a noninvasive and rapid ophthalmological ima­
ging technique that can quantify axonal loss in a variety
of peripheral neuropathies56. CCM may be a viable surro­
gate end point for early diagnosis of DSPN, stratification
of patients with diabetes according to the severity of their
neuropathy, and assessment of response to treatment57,58.
In a cross-sectional study, patients with CIDP, multifocal
motor neuropathy, or neuropathy with MGUS (mono­
clonal gammopathy of undetermined significance) were
compared with healthy controls (in total, 182 patients
and controls were included). Reduced corneal nerve fibre
density, branch density and length were associated with
Langerhans cell infiltration around corneal nerves (FIG. 1)
and correlated with the degree of neurological deficits59.
Further longitudinal studies are required to determine
the utility of this technique for assessing progression of
disease and response to treatment.
Distinguishing CIDP
The challenge of determining whether a patient has CIDP
is greatest in patients with diabetes, because these indi­
viduals can have neurological deficits that resemble CIDP
phenotypes but are caused by other neuropathies. These
neuropathies can include the typical diabetic neuropathy,
or the atypical inflammatory neuropathies. Here, we dis­
cuss the features of these conditions, and how they can
be distinguished from CIDP in patients with diabetes.
DSPN. DSPN is characterized by early involvement
of small fibres, which leads to pain and dysaesthe­
sias1,4,60,61, and later involvement of large fibres, which

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a b strength, proprioceptive function and reflexes — is

­paramount to avoid delays in diagnosis of CIDP.
c d
Figure 1 | Corneal confocal microscopy images in
health, diabetes and CIDP. Images Nature
areReviews | Neurology
of the sub-basal
layer of the cornea. a | Sub-basal central corneal nerves
(arrow) in a healthy individual, without cell infiltrates.
b | Density of central corneal nerve fibres is decreased in
a patient with diabetes, and the number of cell infiltrates
is low. c | Sub-basal layer in a patient with chronic
inflammatory demyelinating polyneuropathy (CIDP). The
number of corneal nerve fibres is lower than in a healthy
individual, and there is a high density of mainly dendritic
cell infiltrates (inset magnification). d | Sub-basal layer in a
patient with CIDP who has a low number of corneal nerve
fibres at the inferior whorl and mainly non-dendritic cell
infiltrates (inset magnification). Scale bars 200 μm. Part d
modified with permission from Wiley and Sons ©
Stettner, M. C. et al. Neurology 3, 88–100 (2016).
can cause numbness and loss of protective sensation62.
Neurological assessment reveals distal sensory loss to
touch and pinprick in both feet63. Typically, sensory
deficits are greater than motor deficits, symptoms are
symmetrical, and progression is slow, so if other pres­
entations indicate an alternative aetiology2,64–66, early
referral to a neurologist is recommended8.
The presentation of CIDP contrasts with the pre­
dominant small-fibre involvement in DSPN, and
the overt motor impairment that is characteristic of
advanced DSPN67, although evidence from the past
2 years suggests that patients with type 2 diabetes or
impaired glucose tolerance without clinically evident
neuropathy can develop an early subclinical reduc­
tion in proximal and distal strength, and an altered
gait68,69. The typically rapid progressive course of CIDP
also contrasts with the evolution of DSPN, which pro­
gresses slowly over years; consequently, rapid progres­
sion of neurological disability in a patient with diabetes
is deemed to be a red flag for a neuropathy other than
DSPN, and CIDP should be considered. Some forms
of CIDP can develop insidiously, however, with pain,
fatigue or mild neurological deficits, making the dif­
ferential diagnosis more difficult70–73, particularly in
patients with diabetes. In this context, regular follow‑up
with neurological e­ valuation — especially of proximal
A degree of electrophysiological motor conduc­
tion slowing may be observed in DSPN. The slowing
seen in DSPN, however, is generally not to the degree
that is observed in CIDP. In initial descriptions of the
association between CIDP and diabetes, most patients
with either disorder met the highly stringent and spe­
cific American Academy of Neurology (AAN) diag­
nostic criteria for CIDP15,16. A study published in 2013
revealed that although 54% of patients with DSPN had
a demyelin­ating form — either pure or combined with
axonal loss — the degree of slowing was variable, and
mild slowing that did not meet the EFNS–PNS diagnos­
tic criteria for CIDP was present in many individuals74.
In patients with type 1 diabetes, this slowing was associ­
ated with poor glycaemic control. The same degree of
conduction slowing had been suggested in a previous
study75. In a subsequent study, patients with demyelin­
ating DSPN were compared with patients who had
CIDP and dia­betes76. Neuropathy scores were worse and
demyelin­ation was clearly more marked among patients
with CIDP and diabetes than among patients with
demyelin­ating DSPN. Interestingly, patients with CIDP
were older, in keeping with the known higher prevalence
of the disorder with age20. Furthermore, these patients
had a shorter duration of diabetes and better glycaemic
control than patients with DSPN, which seems to con­
firm the involvement of different pathophysiological
­mechanisms in concurrent CIDP.
In summary, patients with diabetes and CIDP can be
reliably differentiated, in most cases, from those with
diabetes and DSPN with slow motor conductions, by
using adequate electrophysiological parameters and
cut-offs. Motor conduction velocities <70% of the lower
limit of normal, distal motor latencies >150% of the
upper limit of normal, and conduction block >50% are,
when detected in multiple nerves, highly specific for
CIDP. Distal compound muscle action potential disper­
sion is also helpful in identifying the demyelination that
occurs in CIDP, as this feature is infrequently observed
in axonal neuropathies77,78. F‑wave prolongation might
be less helpful76–78.
Sural nerve biopsy studies in CIDP can reveal pathol­
ogy that is similar to that seen in DSPN: demyelin­
ation and remyelination (the onion bulb formation) is
a charac­teristic feature (FIG. 2)51. Endoneurial oedema
has also been observed in CIDP, particularly in acute-­
onset CIDP54. In a study of sural nerve biopsy samples,
epi­neurial perivascular T-cell infiltrates and immuno­
reactivity for MMP‑9 was found in patients with CIDP
and diabetes, but these features were absent in patients
with DSPN, suggesting that they could represent a
­biomarker for CIDP55.
A diagnostic tool that combines clinical, electro­
physio­logical, CSF protein and serological markers of
autoimmunity to identify CIDP in patients with dia­
betes has been proposed, and was validated in a small
numbers of patients — 12 with CIDP and diabetes, 18
with CIDP only, and 27 with DSPN only79. The param­
eters that were used comprised elements that support

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a b c

Figure 2 | Histopathological features in CIDP. a | Staining against CD3 with diaminobenzidine (DAB) reveals perivascular
T-cell infiltrates (brown) in the sural nerve of a patient with chronic inflammatory demyelinatingNature Reviews | Neurology
polyradiculoneuropathy
(CIDP); cell nuclei are counterstained with haematoxylin and appear blue (magnification 200x). b | In the endoneurium,
large numbers of CD68‑positive foamy macrophages can be seen with DAB staining (brown; magnification 200x).
c | Semi-thin nerve sections in CIDP reveal a large degree of myelin loss as well as thinning of the myelin (toluidine blue
staining, magnification 400x).

a diagnosis of CIDP, derived from characteristic fea­
tures of CIDP, and elements that contradict a diagnosis
of CIDP, derived from the characteristics of the axonal
neuropathy of DSPN (TABLE 1).
Diabetic radiculoplexus neuropathies. Diabetic
radiculoplexus neuropathy (also known as dia­
betic  amyotrophy, Bruns–Garland syndrome or
­diabetic poly­radiculoneuropathy) typically involves the
lumbosacral plexus80–82 or, less frequently, the cervical
plexus, and usually occurs in middle-aged to elderly
men with type 2 diabetes83. The life-long incidence
among patients with type 2 diabetes is ~1%84. Diabetic
lumbosacral radiculoplexus neuropathy (DLRPN) pre­
sents with extreme unilateral thigh pain with weak­
ness, starting proximally and monolaterally, and often
extending distally and contralaterally. Pain and sensory
symptoms occur early, and weakness and muscle wasting
are long-term features of the condition. Weight loss is
common, and can be substantial. Upper-limb involve­
ment can occur. DLRPN can also occur in a painless
form, which is considered by some to be a ‘diabetic
CIDP’, given that its onset is symmetrical and slower
than that of DLRPN, and that the symptoms are more
widespread and frequently involve the upper limbs85,86.
In a study of 23 patients with slowly progressive, pain­
less, symmetrical, motor-predominant neuropathy,
nerve biopsy demonstrated a reduction in myelinated
fibre density with epineurial vessel wall inflammation
and microvasculitis87, similar to that seen in the typical
painful form88,89. In both the painless and painful forms,
CSF protein levels are raised and electrophysiology can
reveal mild conduction slowing. The disease course is
monophasic, and patients recover without treatment,
although the condition can last for up to 18 months80,82
and recovery can take up to 3 years90. Multiple s­ tudies
have suggested that DLRPN has an autoimmune
basis, as indicated by the microvasculitis that has been
demonstrated pathologically88,89.
Diabetic cervical radiculoplexus neuropathy
(DCRPN) has similar characteristics to DLRPN 82.
Onset is acute in most patients, pain is the presenting
symptom in >60% of patients, weakness is an almost
universal feature, and sensory symptoms and weight
loss are common. Panplexopathy is also common, as
is contra­lateral spreading. Concurrent lumbosacral
or thoracic involvement occurs in 25% of cases. CSF
protein levels are mildly raised, and electrophysiology
indicates axonal rather than demyelinating involvement.
Abnormal MRI findings, including increased T2 signals,
nerve hypertrophy and, in rare cases, contrast enhance­
ment, are common. As in the case of DLRPN, the disease
course is monophasic.
Diabetic radiculoplexus neuropathies can be difficult
to distinguish from CIDP, particularly given the proxi­
mal motor weakness and possible bilateral involvement.
However, the acute presentation and the prominence of
pain in diabetic radiculoplexus neuropathy is not typical
of CIDP, and is sufficient to enable a correct diagnosis. In
addition, the conduction slowing that can be observed
in diabetic radiculoplexus neuropathy is not sufficient to
meet the stringent criteria for CIDP87.
Vasculitic multiple mononeuropathies. Vasculitic
multiple mononeuropathy (also called mononeuritis
multi­plex), which does not resemble the radiculoplexus
neuropathies, can occur in diabetes91. This condition
presents with painful mononeuropathies that manifest
in succession over days to weeks. The distinction of vas­
culitic multiple mononeuropathies from CIDP is usually
straightforward owing to the mononeuropathic pattern
and the acute and painful presentation, which are not
features of CIDP.
CIDP presenting as acute inflammatory neuropathy.
The progressive course of CIDP distinguishes the condi­
tion from the acute inflammatory polyneuropathies, or
Guillain–Barré syndrome, in which the nadir is reached
by week 4. However, ~15% of patients with CIDP can
experience an acute onset that is indistinguishable from
that of Guillain–Barré syndrome92, although the subse­
quent relapsing or progressive course confirms the diag­
nosis of CIDP93. The absence of a preceding infection,
facial weakness, dysautonomia or a need for mechan­
ical ventilation indicate CIDP, as does the presence of
­sensory signs92,93.

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Management of CIDP in diabetes In addition to IVIg and corticosteroids102,103, a third

CIDP is the most common and treatable inflammatory
neuropathy that occurs in patients with diabetes. The
main first-line therapies are intravenous immunoglobu­
lin (IVIg) or corticosteroids, and their use is based on
the knowledge that CIDP results from aberrant immune
responses to peripheral nerve antigens (FIG. 3). The inflam­
mation that results from this response is charac­terized by
nerve oedema, endoneurial infiltration by macrophages,
perivascular infiltration by T cells53,94, and increased
expression of cytokines and other inflammatory molecules
in the CSF and blood95–97. Breakdown of the blood–nerve
barrier is the critical initial stage that leads to the passage
of activated T cells and entry of inflammatory mediators,
which further contribute to permeability of the blood–
nerve barrier and upregulation of the neural inflammatory
response. CD4+ and CD8+ T cells and macrophages cluster
around endoneurial vessels and mediate demyelination98.
Various hypoth­eses have been proposed about the under­
lying mechan­isms that trigger the immune response99–101,
but a full u
­ nderstanding remains elusive.
evidence-based option for first-line treatment of CIDP is
plasma exchange. However, this approach is used less fre­
quently than the other two owing to its impracticality and
the fact that the response is short-term and n ­ ecessitates
repeated treatment104.
The decision whether to use IVIg or steroids depends
on the individual patient’s comorbidities, the type of
CIDP, and the disease severity. In patients with diabetes,
corticosteroids are generally avoided, as they can result
in deterioration of glycaemic control. However, caution
is needed with IVIg in the presence of multiple vascular
risk factors, including diabetes105,106. Similarly, history of
a recent thromboembolic event should lead to avoid­
ance of IVIg. Motor CIDP should be treated with IVIg,
as corticosteroids can cause worsening of symptoms107.
The latest comparative study of IVIg and corticosteroids
showed that the former is also less likely to be stopped as
a result of ineffectiveness or adverse effects108. This find­
ing suggests that IVIg may be preferred for severe disabil­
ity that requires rapid improvement, although long-term

Table 1 | Selected features of DSPN, CIDP and CIDP in patients with diabetes
Feature DSPN
Clinical • Early small-fibre involvement, pain,
presentation dysaesthesia, numbness
• If autonomic neuropathy: tachycardia,
orthostatic hypotension, gastroparesis,
constipation, diarrhoea, erectile
dysfunction, neurogenic bladder and
sudomotor dysfunction1–4,60,61,157,158
• Progression over years
Diagnostics • Electrophysiology: sensorimotor
polyneuropathy, mild slowing of NCV
with normal latencies74,75
• CSF protein levels might be slightly
elevated31
Histology • Myelinated fibre loss
• Reduction in unmyelinated axon
density and diameter
• Increase in unassociated Schwann cell
profile density
• Endoneurial microangiopathy (luminal
narrowing, endothelial cell hyperplasia
and hypertrophy, pericyte cell loss and
basement membrane thickening160–171)
Treatment • Symptomatic
• Improvement of glycaemic control
might limit progression in type 1
diabetes, but has no effect in type 2
diabetes
• No evidence to support use of
immunotherapy
CIDP, chronic inflammatory demyelinating neuropathy; CSF, cerebrospinal fluid; DSPN, distal symmetric peripheral neuropathy; EFNS–PNS, European Federation
of Neurological Societies–Peripheral Nerve Society; LLN, lower limit of normal; MRN, magnetic resonance neurography; NCV, nerve conduction velocity;
ULN, upper limit of normal.
CIDP
• Symmetrical proximal and distal muscle
weakness, sensory loss to large-fibre
modalities (vibration and proprioception)
and reduced or absent reflexes9
• Often greater motor than sensory deficits
• Rapid progression over months (at least
8 weeks)
• Subgroups: focal and multifocal
asymmetric presentation, distal forms,
pure motor forms and pure sensory forms
• Electrophysiology: high-sensitivity and
high-specificity EFNS–PNS criteria met
• Increased CSF protein levels are common
• MRN and ultrasound might be
helpful in some cases for detecting
heterogeneously thickened and
hyperintense nerves
• Sural nerve biopsy might be helpful
in some cases for demonstrating
inflammatory features47
• Demyelination and remyelination (onion
bulb formation)
• Endoneurial oedema and epineurial
perivascular T‑cell infiltrates51,54
• Intravenous immunglobulin or
corticosteroids as first-line therapy
• Plasma exchange118
• Cyclophosphamide131,132 or rituximab133
in refractory disease
CIDP in patients with diabetes
• Features of CIDP without diabetes
• Older patients
• Short duration of diabetes
• Good glycaemic control76
• Electrophysiology: NCV to <70% of the
LLN, distal motor latency >150% of the
ULN, and conduction block >50%, all in
multiple nerves, highly specific; distal
compound muscle action potential
dispersion83,87–89
• CSF: not specific to distinguish from
DSPN, but levels >1 g/l are unusual in
diabetes32,159
• Sural nerve biopsy might be helpful
in some cases for demonstrating the
inflammatory features of CIDP47
• Epineurial perivascular T‑cell infiltrates
• Immunoreactivity for matrix
metalloproteinase‑9 (REF. 55)
• Corticosteroids to be avoided
• Iintravenous immunoglobulin as first-line
therapy
• Plasma exchange118
• Cyclophosphamide131,132 or rituximab133
in refractory disease

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Systemic immune compartment BNB Peripheral nerve Myelin

APC Apoptosis

T cell Compact myelin

Schwann
T cell Macrophage cell

C5b–9
IFN-γ
TNF P0 P2 MBP
T cell TH1
IL-4
IL-6 Non-compact myelin
IL-10 Extracellular
TGF-β
Antibodies
B cell TH2
Intracellular
MAG Cx32 GLP

Figure 3 | Current model of immunopathogenesis in CIDP. The immune response in chronic inflammatory
Nature Reviewscells
demyelinating polyneuropathy (CIDP) involves cellular and humoral immune responses. Antigen-presenting | Neurology
(APCs),
such as dendritic cells or macrophages, activate autoreactive T cells. These T cells can cross the blood–nerve barrier
(BNB) to enter the peripheral nerve, a process mediated in part by chemokines, cellular adhesion molecules and
metalloproteinases (orange circles). In addition, T cells can activate B lymphocytes via secretion of cytokines, such as IL‑4
and IL‑6. Within the PNS, T cells are re‑exposed to the target antigen by local APCs (a macrophage is depicted), thereby
reactivating these cells and driving clonal expansion within the peripheral nerve. CD4+ T cells can differentiated into
T-helper 1 (TH1) cells, which, once activated by APCs, release pro-inflammatory cytokines and drive the inflammatory
process, or TH2 cells, which control and mitigate inflammation by secreting anti-inflammatory mediators. Macrophages
are predominantly activated by TH1 cells, and exhibit increased phagocytic activity, produce cytokines and release toxic
mediators, such as nitric oxide, that can directly damage Schwann cells and contribute to axonal damage. In addition,
pro-inflammatory cytokines, such as tumour necrosis factor (TNF) or IFNγ, are locally produced by B cells, and contribute
to demyelination and axonal damage. Antibodies can mediate demyelination by antibody-dependent cellular
cytotoxicity, can block epitopes that are functionally relevant to nerve conduction, and can activate the complement
system via the classical pathway, yielding pro-inflammatory mediators and the lytic C5b–9 terminal complex.
Termination of the inflammatory response is mediated, in part, by macrophages through induction of T-cell apoptosis
and release of anti-inflammatory cytokines, such as IL‑10 and transforming growth factor (TGF)-β. Myelin proteins, such
as P0, P2, myelin basic protein (MBP), myelin-associated glycoprotein (MAG), connexin 32 (Cx32) and glucagon-like
peptide (GLP), are putative target antigens in CIDP.

follow‑up of patients in this trial and in a previous retro­
spective study suggest that corticosteroids induce a
higher remission rate and longer remission times than
IVIg109,110. Consequently, cautious use of corticosteroids
might be considered, even for patients with diabetes and
mild disease.
Options for corticosteroid therapy are oral
dexametha­sone pulse treatment, intravenous methyl­
prednisolone, or a daily oral regime of prednisolone.
Current evidence indicates that pulse therapy provides
faster onset of benefit and has a better safety profile than
the other options111: pulse therapy has been associated
with significantly lower rates of sleeplessness and moon
facies. In a comparison of pulse therapy with daily oral
regimens, a significantly lower risk of weight gain >3 kg
and a near-significant reduced risk of raised blood sugar
were also observed with pulse therapy111. Long-term
treatment with any of these corticosteroids requires
regular monitoring. For IVIg treatment, dosage require­
ments are variable, and should be titrated to the minimal
effective dose in each case. Treatment withdrawal trials
should be considered at regular intervals112; trials can be
attempted yearly, although the frequency depends on
individual patients’ circumstances.
Response rates to treatment for CIDP are compa­
rable between patients with and without diabetes15,113.
However, the benefit in patients with diabetes might
be limited by underlying DSPN85. Indeed, more-severe
baseline axonal loss predicts a poor treatment response114.
Efforts to identify electrophysiological predictors of
treatment response in CIDP initially yielded negative
results115, although a report published in 2015 suggested

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that fulfilment of the AAN or EFNS–PNS diagnostic Prognosis

criteria for CIDP is associated with a higher chance of
a therapeutic response116. Another study published in
2015 showed that greater demyelination in patients with
CIDP and diabetes was associated with better treatment
responses117. This finding implies that in order to be diag­
nosed with CIDP, patients with diabetes should fulfil at
least two EFNS–PNS CIDP criteria for demyelination,
and that more-stringent cut-off points are needed to
define demyelination. In fact, cut-offs of 30% beyond
the normal values for distal latency, motor conduction
velocity and minimum F‑wave latency enabled better
prediction of a CIDP treatment response in patients with
diabetes than did lower cut-offs, and cut-offs of only 10%
produced a comparable level of prediction in patients
without diabetes.
For CIDP in patients with diabetes that is refractory
to initial monotherapy, subsequent treatment should be
identical to that for refractory CIDP without diabetes.
Plasma exchange and combination therapies (in particu­
lar, IVIg or plasma exchange with corticosteroids) should
be considered10,118.
No evidence currently supports the use of immuno­
suppressants in CIDP119,120 in any context. Randomized
controlled trials in patients with CIDP have demon­
strated no efficacy of azathioprine, methotrexate or
IFNβ1a121–124. Furthermore, evidence for efficacy of cyclo­
sporin125,126, mycophenolate mofetil127, natalizumab128 and
alemtuzumab129,130 is limited. A phase III clinical trial of
fingolimod (NCT01625182) was terminated early owing
to futility (reported by Hartung et al., American Academy
of Neurology, Boston, 2017). Immunosuppressants are
probably less justifiable for patients with diabetes and
pre-existing axonal loss as a result of DSPN, as the
potential for neurological recovery in these patients is
lower. Nevertheless, if a patient with diabetes is severely
affected by recent-onset CIDP, immunosuppression
should be considered. In a series of patients with treat­
ment-refractory CIDP, the responder rate to cyclophos­
phamide was >70%, and improvements in quality-of‑life
measures were seen; the treatment regimens that were
used included high-dose cyclophosphamide without
stem-cell rescue131,132. This treatment requires excep­
tional consideration after obtaining carefully informed
consent; all necessary precautionary measures — the use
of antibacterial, antifungal and uroprotective agents —
must be taken and the patient must be closely monitored
for adverse effects, particularly infectious, haematological
and urological effects. Rituximab might be an alternative
therapeutic option, as it has been used successfully in case
series of patients with refractory CIDP, including patients
with diabetes133–135.
A few case reports have shown that autologous
haemato­poietic stem cell transplantation can be effec­
tive for refractory CIDP136–140, and in a multicentre case
series of 11 Swedish patients with CIDP, this procedure
induced remission in eight patients for the 2‑year obser­
vation period141. Despite these encouraging data, a careful
risk–benefit analysis should always be performed before
embarking on this aggressive approach, which can cause
considerable morbidity and even death.
Few studies have reported on the outcome of treatment in
patients with CIDP and diabetes. Some useful informa­
tion can be derived from those that have been published,
but most were small, had a retrospective design, and used
assessment tools that are now considered outdated.
In two separate case series from the USA and Italy,
all 14 patients with diabetes and CIDP responded to
immunomodulatory and/or immunosuppressive treat­
ments15,16. In the US study, modified Rankin scale (mRS)
scores improved by 2 points in six patients, and by
1 point in one other15. Similarly, in the Italian study, mRS
score improved by 2 points in six patients and by 1 point
in another, and Neurological Disability Scores also sub­
stantially improved in all patients, both overall and in
the motor component16. In a comparison of 14 patients
who had CIDP and diabetes with 60 patients who had
‘idiopathic’ CIDP, the proportion of patients who
responded to IVIg, corticosteroids, plasma exchange or
cyclophosphamide — measured with the mRS — was
similar in the two groups85. However, improvements in
MRC (Medical Research Council) and mRS scores were
significantly greater among the patients with idiopathic
CIDP than among patients with CIDP and diabetes85.
Baseline characteristics of the groups were comparable,
except that the patients with diabetes were older, and had
a higher frequency of gait imbalance and a greater degree
of axonal loss. In a series of nine patients with CIDP
from a cohort of 100 patients with diabetes, only two of
four who were treated with corticosteroids responded,
and none of three who received IVIg responded 18.
In another comparison of CIDP patients with and with­
out diabetes (n = 6 and n = 8, respectively), all patients
responded to corticosteroids alone or in combination
with plasma exchange, IVIg or azathioprine. The patient
groups were comparable in all respects except age and
the degree of axonal loss, both of which were greater in
patients with CIDP and diabetes86.
Few prospective studies of the treatment of CIDP
in patients with diabetes have been published. In one
open-label, 4‑week study, 26 patients who met the
highly specific AAN diagnostic criteria for CIDP142
were treated with IVIg at a standard dose of 400 mg/kg
daily for 5 days143. For 21 of the 26 patients (80.8%), the
Neuropathy Impairment Score improved by >5 points
by week 4 after treatment. For one in six patients, scores
improved by the end of the 5‑day infusion, and lower-­
limb function had improved significantly 4 weeks after
treatment. Only the presence of conduction block pre­
dicted a treatment response and absence of relapse; age,
sex, duration of diabetes, CSF protein levels and glycated
haemoglobin did not. Of the five patients who did not
respond to IVIg, two improved slightly with plasma
exchange and corticosteroids in combination. The global
response rate to all treatment, therefore, was 88.5%.
Six patients had a relapsing course, but five of these
individuals responded to IVIg or plasma exchange. In
another prospective study, 16 patients with diabetes and
a demyelin­ating neuropathy that fulfilled AAN diagnos­
tic criteria for CIDP144 were followed up for 40 months.
Nine had type 1 diabetes, and seven had type 2 diabetes.

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All participants had experienced proxi­mal and dis­
tal limb weakness for 4–12 months before their initial
examination, and all were treated with IVIg. Neuropathy
Impairment Scores improved for 14 patients (87.5%),
and mean summated conduction velocities improved in
the upper and lower limbs, although motor and sensory
axonal loss progressed.
The largest study to date is a retrospective compara­
tive analysis of 134 patients with CIDP: 67 with diabetes
and 67 without diabetes113. In this analysis, the diagnosis
of CIDP was based on expert opinion and use of diagnos­
tic criteria that did not include electrophysiological meas­
ures145, and were previously found to be highly specific
but less sensitive than the EFNS–PNS criteria for CIDP27.
Assessment of response was based on a combination of
physician and patient evaluations, including the Toronto
Clinical Neuropathy Score (TCNS). Patients with CIDP
and diabetes had significantly higher TCNSs and a
greater extent of proximal weakness than those without
diabetes, but significantly fewer of these patients received
treatment (57% versus 93%, P <0.0001). No overall dif­
ference was seen in the responses to treatment with
IVIg, corticosteroids, plasma exchange, azathioprine or
mycophenolate mofetil, although the patients with dia­
betes were less likely to respond to IVIg (52%, compared
with 87% for patients without diabetes, P <0.0001). In
the whole cohort, only a shorter duration of neuropathy
had a favourable influence on response; age, the pres­
ence or duration of diabetes, the severity of neuropathy,
and the degree of axonal loss had no impact. Besides its
retrospective design, this study was limited by the fact
that validated functional scales for evaluation of CIDP,
such as the Overall Neuropathy Limitations Scale and
the Inflammatory Rasch-Built Overall Disability Score,
were not used, the possibility that cases of DLRPN were
misclassified as CIDP because electrophysiology was
not mandatory for diagnosis, and the possibility of
selection bias owing to the relatively low treatment rate
among patients with CIDP and diabetes. Also of note, the
response rate among the 67 patients with CIDP without
diabetes — selected from a cohort of 1,950 people with
CIDP to be age-matched and sex-matched to the patients
with diabetes — was higher than that reported in a pre­
vious therapeutic trial146, but was comparable to the rate
reported for treatment-naive patients with CIDP147.
Managing other inflammatory neuropathies
Radiculoplexus neuropathies. Treatments for painful
DLRPN and DCRPN are mainly symptomatic. No evi­
dence currently indicates a benefit of immunotherapy
for these conditions in diabetes83, and trials might be
difficult to conduct owing to the heterogeneity of the
presentation. For patients who have extreme pain that
is refractory to analgesics, intravenous steroids could be
considered in selected cases, but do not seem to ­hasten
recovery of strength83. Small case series, including fewer
than 20 patients in total, have suggested that IVIg is
beneficial, but no evidence has been generated in ran­
domized controlled trials83. Similarly, case series indicate
that plasma exchange is beneficial, but these studies also
included fewer than 20 patients in total. Comparative
analysis has suggested that untreated patients even­
tually improve to a similar degree to those receiving
IVIg or plasma exchange148. Furthermore, the clinical
descriptions of some patients who responded to treat­
ment imply that they had CIDP rather than DLRPN149.
Descriptions of the same treatment being ineffective also
contradict the positive findings150. Owing to these uncer­
tainties, no evidence base supports any treatment for
diabetic radiculoplexus neuropathies, particularly con­
sidering that corticosteroids impair glycaemic c­ ontrol in
this population151.
Distinguishing CIDP from microvasculitis can be dif­
ficult87, but in practice, patients for whom either diagno­
sis is reasonable should be considered to have CIDP and
treated accordingly. A proportion of patients with clini­
cally typical CIDP without diabetes do not fulfil electro­
physiological criteria for demyelination27,145, so denying
these patients treatment is inappropriate. Furthermore,
the technical expertise required for an adequate diag­
nostic neuropathology report greatly limits the use of
nerve biopsies in this urgent setting, so the clinical and
electrophysiological findings are often relied on to make
a therapeutic decision. Nevertheless, the possibility of
monophasic disease must be kept in mind to avoid
unnecessary overtreatment and therapeutic escalation152.
Vasculitic multiple mononeuropathy. If vasculitic mul­
tiple mononeuropathy is suspected, an exhaustive diag­
nostic work‑up for vasculitic neuropathy is indicated,
and if the work‑up is negative, nerve biopsy — preferably
to sample a clinically affected sensory nerve — should
be performed for pathological confirmation of vasculitic
multiple mononeuropathy. Patients with and without
diabetes should receive identical treatment for proven
or probable vasculitic multiple mononeuropathy, which
is the same as the treatment for nonsystemic vasculitic
neuropathy without diabetes153.
Conclusions and practice implications
The most common neuropathy in patient with diabetes
is DSPN, but other neuropathies — including radiculo­
plexus neuropathies, vasculitic multiple mononeuro­
pathies and CIDP — can occur and must be diagnosed
because they require different treatment approaches.
DLRPN and DCRPN are generally easy to recognize,
although they can be painless and extensive, making
their identification more difficult. The course of these
conditions is monophasic, and the prognosis is generally
favourable.
CIDP is the most treatable neuropathy that can occur
in diabetes. The frequency of CIDP among patients with
diabetes remains controversial, but the rapidly progres­
sive disability caused by CIDP warrants awareness among
health-care professionals of the association and the
potential for immunomodulatory therapy. The diagnosis
of CIDP in diabetes is primarily made clinically, and elec­
trophysiological measurements in CIDP with dia­betes
are less straightforward than in CIDP without diabetes.
Nevertheless, currently recommended cut-offs for demy­
elination remain highly specific for CIDP in patients with
diabetes9. No diagnostic criteria offer perfect sensitivity,

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however, and even when criteria are not fulfilled, a high
clinical suspicion of CIDP warrants a treatment trial,
especially if the neuropathy is disabling. In the absence
of widely available, adequately interpreted nerve his­
tology, this approach seems to be a pragmatic way to
prevent patients missing out on effective treatment. To
avoid unnecessarily prolonged immunotherapy, regular
monitoring, followed by reduction and withdrawal of
treatment if necessary, is highly advisable, particularly in
patients with diabetes, because these individuals have an
elevated risk of thromboembolism. The frequency of the
treatment review can be decided on a case‑by-case basis,
but should be at least yearly.
Response rates to treatment for CIDP seem to be
similar in patients with and without diabetes, although
underlying DSPN will limit the response, and full nor­
malization of function and strength might not occur.
Nevertheless, neurologists should not be deterred from
attempting and escalating treatment as they would for
patients with CIDP without diabetes. Treatment rates
for CIDP in patients with diabetes are low113, indicating
that these individuals are frequently denied potentially
effective treatment. Early treatment is advisable because
delaying immunotherapy can reduce its effectiveness.
Whether the presence of diabetes reduces the response
rate to IVIg in patients with CIDP is uncertain, but this
treatment remains the most appropriate first-line ther­
apy, particularly because it avoids the risk of worsening
glycaemic control that is associated with corticosteroids.
In future, a biomarker for CIDP might provide the
much-needed diagnostic certainty required for earlier
intervention, especially in patients with diabetes, for
whom diagnostic confirmation could be more helpful
and earlier intervention more beneficial. Novel anti­
bodies against nodal proteins have been identified in
CIDP in the past few years154–156, and these discoveries
provide encouragement with regard to biomarker identi­
fication, although the antibodies identified apply only to
a small proportion of patients with CIDP. Histology per­
formed on skin and nerve biopsy samples is one imaging
biomarker approach, but is impractical in routine clinical
practice55. Corneal confocal microscopy might be use­
ful for identifying corneal axonal loss and infiltration of
Langerhans cells in CIDP59, but longitudinal studies are
required to assess the effects of CIDP therapy on these
markers. In the meantime, increased awareness, detailed
and repeated clinical evaluation, adequate interpretation
of electrophysiology, and appropriate and timely con­
sideration of therapeutic options remain essential in the
routine care of patients with CIDP and diabetes.

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Author contributions
Y.A.R., B.C.K and R.M. researched data for and wrote the
article. All authors made substantial contributions to discus-
sion of the content and reviewed and/or edited the
­manuscript before submission.
Competing interests statement
Y.A.R. has received speaker and/or consultancy honoraria
from CSL Behring, LfB, Grifols, BPL, Octapharma and
Kedrion, has received educational sponsorships from LfB,
CSL Behring and Baxter, and has obtained research grants
from CSL Behring and LfB. M.S. has received honoraria for
consulting or lecturing and travel expenses for attending
meetings from Biogen Idec, Genzyme, Novartis, Sanofi
Aventis, UCB, Grifols and TEVA, and has received financial
support for research from Bayer Health Care and UCB. B.C.K.
has received honoraria for lecturing, travel expenses for
attending meetings, and financial support for research from
Bayer Health Care, Biogen, Genzyme/Sanofi Aventis, Grifols,
Merck Serono, Mitsubishi Europe, Novartis, Roche, Talecris
and TEVA. He is currently also an employee of Biogen.
H.-P.H. has received fees for consulting, speaking and serving
on steering committees from Baxelta, CSL Behring, Kedrion,
Novartis, Octapharma and LfB. R.A.M. has received honor­
aria for speaking or consultancy from Pfizer, Lilly and
Novo Nordisk.
Publisher’s note
Springer Nature remains neutral with regard to jurisdictional
claims in published maps and institutional affiliations.
Review criteria
A search for original English-language, full-text articles was
performed in PubMed using the search terms “chronic inflam-
matory demyelinating polyneuropathy”, “CIDP”, “demyelinat-
ing neuropathy”, “diabetes”, “diabetic neuropathy”, “diabetic
proximal amyotrophy”, “diabetic cervical radiculoplexus
neuro­pathy” and “diabetic lumbar radiculoplexus neuro­
pathy”. The reference lists of identified papers were searched
for further relevant articles.

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