BLINK REFLEX ROLE IN ALGORITHMIC GENETIC TESTING OF
INHERITED POLYNEUROPATHIES
WEI WANG, MD,1,2 WILLIAM J. LITCHY, MD,1 JAY MANDREKAR, PhD,3 PETER J. DYCK, MD,1 and
CHRISTOPHER J. KLEIN, MD1,4,5
1
Department of Neurology, Mayo Clinic, 200 First Street, SW, Rochester, Minnesota, 55905 USA
2
Department of Neurology, China-Japan Friendship Hospital, Beijing, China
3
Biomedical Statistics and Informatics, Mayo Clinic, Rochester, Minnesota, USA
4
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA
5
Department of Medical Genetics, Mayo Clinic, Rochester, Minnesota, USA
Accepted 8 July 2016
ABSTRACT: Introduction: In severely affected inherited polyneur-
opathy patients, primary demyelination can be difficult to deter-
mine by routine extremity limb nerve conduction studies (NCS).
Blink reflexes may help classify severe polyneuropathies as either
axonal or demyelinating. However, blink reflex studies have not
been studied systematically in any genetically confirmed cohort.
Methods: Patients with a genetic diagnosis who had undergone
blink reflex testing and extremity NCS were identified retrospec-
tively. Blink reflex R1 latency, extremity NCS, and severity were
compared. Results: We identified 26 demyelinating and 23
axonal, genetically confirmed cases, including 20 with PMP22
duplications. In 12 (25%), the ulnar CMAP amplitude was !0.5
mV making electrophysiological classification difficult. However,
the R1-latency cutoff of >13 ms (demyelinating) robustly classi-
fied all patients regardless of severity. Conclusions: We show that
blink reflex studies are reliable for identification of inherited demy-
elinating polyneuropathy regardless of severity and can facilitate
algorithmic decisions in genetic testing.
Muscle Nerve 55: 316–322, 2017
Nerve conduction studies (NCS) are important
for distinguishing inherited demyelinating from
axonal peripheral neuropathies. This electrophysi-
ological distinction is important in algorithmic
ordering of genetic testing.1 Recent advances in
comprehensive genetic testing by next generation
sequencing (NGS) are changing genetic test order-
ing practice by allowing evaluation of a large num-
ber of genes at reduced cost.2 Nevertheless
ordering PMP22 duplication testing first in patients
with demyelinating nerve conduction and length-
dependent progressive neuropathy is recom-
mended because of the high pretest probability of
finding PMP22 duplications (50–70%).1,3–5 This
can reduce the genetic noise by reducing the
Additional Supporting Information may be found in the online version of
this article.
Abbreviations: c-HSP, complicated-hereditary spastic paraplegia;
CMAP, compound muscle action potential; CMT, Charcot-Marie-Tooth
disease; EMG, electromyography; FAP, familial amyloid polyneuropathy;
HSN, hereditary sensory neuropathies; NCS, nerve conduction studies;
MCV, motor conduction velocity; NGS, next generation sequencing; NIS,
neuropathy impairment score; PN, polyneuropathy; SNAP, sensory nerve
action potential.
Key words: blink reflex; Charcot-Marie-Tooth disease; demyelinating pol-
yneuropathy; genetic testing; inherited polyneuropathy; PMP22
Correspondence to: C. J. Klein; e-mail: klein.christopher@mayo.edu
C 2016 Wiley Periodicals, Inc.
V
Published online 16 July 2016 in Wiley Online Library (wileyonlinelibrary.com).
DOI 10.1002/mus.25250
316 Blink Reflex & Inherited PN
variants of unknown significance seen in large
genetic panel testing through NGS.
The ulnar motor forearm conduction velocity
(ulnar MCV) has been chosen by some authors to
distinguish axonal from demyelinating disorders
using !38 m/s as the value for identification of
demyelination in inherited polyneuropathy.3,6
However, conduction velocities are no longer reli-
able when ulnar motor compound muscle action
potential (CMAP) amplitudes are <0.5 mV,7,8 and
electrophysiological classification of neuropathy
becomes challenging.
Proximal extremity NCS are useful for patho-
physiologic classification of severe peripheral neu-
ropathies.1,9 However, they can be technically
difficult to perform to ensure maximal responses,
and accurate distance measurement for determining
nerve conduction velocities is a challenge.10
Although blink reflex studies are technically easy to
perform and are reproducible, we found only 1 case
report of the use of this approach in a genetically
confirmed inherited polyneuropathy, an EGR2 muta-
tion.11 An earlier study predating genetic mutation
discoveries also suggested that a prolonged R1 blink
response may be useful, because a markedly pro-
longed R1 is associated with severely abnormal nerve
conduction.12 Consistent with this observation was
that the facial motor study commonly had pro-
longed latency in inherited demyelinating cases.13
Normative data for blink reflex studies have been
generated by different groups, with 13 ms being the
upper limit of normal for the R1 response for indi-
viduals > age 2 years14 and R2 being less useful due
to much greater variability.12,15
Here, we evaluated the use of blink reflex R1
latency to distinguish demyelinating from axonal
inherited polyneuropathies through a study of blink
reflex responses in genetically confirmed patients.
We investigated whether there is a correlation
between blink reflex, nerve conduction abnormality,
and severity of neurological impairment.
MATERIALS AND METHODS
Participants. After institutional
review board
approval, we queried inherited polyneuropathy
MUSCLE & NERVE March 2017
patients diagnosed by genetic testing in the Mayo
Clinic database from January 1, 1999, to October
31, 2015. Over 300 patient records were reviewed.
All patients had signed consent agreeing to share
their health information for research study. Inclu-
sion criteria included: (1) Clinical diagnosis of
Charcot-Marie-Tooth disease (CMT); hereditary
motor and sensory neuropathies, hereditary senso-
ry neuropathies (HSN), complicated-hereditary
spastic paraplegia (c-HSP); or familial amyloid pol-
yneuropathy (FAP). (2) A known genetic mutation
linked with either axonal or demyelinating inher-
ited polyneuropathy; (3) Standard electrodiagnos-
tic studies performed in the Mayo Clinic
Neurophysiology laboratory with available nerve
conduction tracings, including ulnar motor con-
duction studies. Extremity temperatures were mea-
sured and confirmed to be >308C in the lower
extremities and >328C in the upper extremities. A
blink reflex study was done at the same time as
other studies. (4) Standardized neurological physi-
cal examination available for calculation of neuro-
logical impairments within 1 month of
electrodiagnostic study. Patients who had superim-
posed other neuropathy diagnosis were excluded,
such as neuropathy associated with Sj€ ogren syn-
drome, or chronic inflammatory demyelinating
polyneuropathy, where prolonged R1 latency has
been reported previously.15,16 A total of 49 patients
met all of the above criteria and were selected for
this study.
One major purpose of our study was to demon-
strate the utility of blink reflex R1 latency for iden-
tification of primary demyelinating patients who
would potentially benefit from PMP22 duplication
stand-alone testing. Therefore, these 49 patients
were further divided into 2 groups based on the
causal gene mutations reported in the Human
Gene Mutation Database and Online Mendelian
Inheritance in Man databases. Group 1 (n 5 26,
genetically confirmed primarily demyelinating)
included patients who had a genetic mutation
linked with primary demyelinating polyneuropathy.
Group 2 (n 5 23, genetically confirmed “axonal”)
patients had a genetic mutation which linked with
inherited polyneuropathy but without evidence of
primary demyelination.17 When multiple electro-
diagnostic studies were available, only the first
study was included. Summation of the distal stimu-
lated CMAP amplitude and summated antidromi-
cally stimulated distal sensory nerve action
potential (SNAP) amplitudes were calculated using
available uniformly studied NCS of fibular, tibial,
and ulnar motor, and sural and median sensory
NCS.
Neuropathy impairment scores (NIS) were cal-
culated based on a standardized neurological
Blink Reflex & Inherited PN
physical examination, including weakness of dis-
tributed muscles of head, trunk, and proximal and
distal segments of limbs with scoring from 1 to 4
points of the 24 muscle groups assessed from 0 to
192 points (NIS motor score); activity of the 5
commonly evaluated reflexes were categorized as
normal (0), decreased (1), or absent (2), with pos-
sible NIS reflex scores ranging from 0 to 20 points;
touch-pressure, vibration, joint motion, and pin-
prick were evaluated in the toes and fingers and
scored as normal (0), decreased (1), or absent (2),
with an NIS sensory score ranging from 0 to 32
points; the most severe patients had a maximal
NIS total score of 244 points.18
Data Analysis. The R1 latency was compared
between 2 patient groups (demyelinating vs axo-
nal) with knowledge of the prior normal reference
range (8–13 ms).12,15 Correlation and regression
analyses were done: (1) between R1 latency and
ulnar motor conduction velocity and ulnar motor
CMAP amplitude; and (2) between NIS score and
R1 latency, summated CMAP amplitude, and ulnar
CMAP amplitude.
RESULTS
Clinical Features. At the time of the electrodiag-
nostic study, the median age of 26 CMT1 patients
in group 1 (Supplementary Table S1, available
online) was 44 years (range: 5–70 years). Among
them, 21 were CMT1A patients with a PMP22 gene
mutation (20 of 21have PMP22 duplication), 2
CMT1C patients with an LITAF mutation, 2
CMT1D patients with an EGR2 mutation, and 1
CMT1B patient with an MPZ mutation. The symp-
tom onset age varied significantly: 4 patients had
neurological symptoms at age <10 years, 13
patients between 10 and 20 years, 6 between 20
and 40 years, and 3 patients >40 years. All 26
patients in group 1 had distal leg weakness (NIS
motor scores ranged from 2 to 112), and 24 had
sensory symptoms. The average of the total NIS
score summing motor, sensory, and reflex deficits
was 53.56 points (range: 10–158 points). Group 2
(S2) includes 23 axonal inherited neuropathy
patients with a median age of 43 years (range:
6–74 years) at the time of electrodiagnostic evalua-
tion. Among them, 12 were CMT2 patients (4
patients had an MFN2 gene mutation, 3 patients
had an MPZ mutation, and 1 patient had a BAG3
gene mutation), 2 were intermediate form CMT
patients with a GJB1 mutation, 5 were HSN
patients (2 patients had an SPTLC2 gene muta-
tion), 4 were FAP patients with a TTR mutation.
The average of the NIS total score was 57.47 points
(range: 17–120 points). None of the patients in
either group 1 or group 2 had a clinical history of
facial palsy. No significant difference was found
MUSCLE & NERVE March 2017 317
 Table 1. Blink reflex in genetically confirmed inherited polyneuropathy patients.

a. All genetically confirmed polyneuropathy patients (n 5 49)

Group 1 (n 5 26) (genetically demyelination) Group 2 (n 5 23) (genetically axonal) P-Value

Gender 7 female/19 male 9 female/14 male 0.54
Age of onset 20.3 6 3.6 years 26.9 6 3.8 years 0.21
Age at evaluation 44.3 6 3.8 years 42.9 6 4.0 years 0.79
NIS 53.56 6 6.39 points 57.47 6 6.79 points 0.68
R CMAP 4.8 6 1.2 mV 6.8 6 1.3 mV 0.23
Ulnar CMAP 3.9 6 0.8 mV 5.9 6 0.8 mV 0.07
Ulnar MCV 21.3 6 1.7 mV 49.8 6 1.7 mV <0.0001
R1 latency 17.1 6 0.5 mV 11.3 6 0.5 mV <0.0001

b. Inherited polyneuropathy patients with ulnar CMAP amplitude ! 0.5mV (n 5 12)

Group 1 (n 5 7) (genetically demyelination) Group 2 (n 5 5) (genetically axonal) P-Value

Gender 4 female/3 male 1 female/4 male 0.29
Age at evaluation 52.0 6 4.4 years 65.4 6 5.2 years 0.08
NIS 96.11 6 10.16 points 82.45 6 12.02 points 0.41
R CMAP 0.3 6 0.2 mV 0.3 6 0.2 mV 0.83
Ulnar CMAP 0.20 6 0.08 mV 0.22 6 0.09 mV 0.86
Ulnar MCV 20.8 6 3.9 mV 39.0 6 3.9 mV 0.02
R1 latency 18.5 6 1.2 mV 11.9 6 1.3 mV 0.004

between the 2 groups in age at evaluation or NIS
scores (Table 1).
Extremity NCS. In group 1 (n 5 26), 3 patients
had absent ulnar CMAPs, 4 more patients had
ulnar motor amplitudes ! 0.5 mV, 14 patients had
decreased ulnar CMAP amplitudes, and 5 patients
had normal ulnar CMAP amplitudes. Aside from 3
patients with absent ulnar CMAPs, the average
ulnar MCV of the 23 patients was 21.3 m/s (range:
12–38 m/s). Among 22 patients who had sural and
median sensory NCS, 18 of 22 (81.8%) had an
absent summated SNAP. In group 2 (n 5 23), 1
patient had an absent ulnar CMAP, 4 more
patients had ulnar CMAP amplitude !0.5 mV, and
6 patients had decreased ulnar CMAP amplitudes;
the remaining 12 patients had normal ulnar CMAP
amplitude. The average ulnar MCV of 22 patients
was 49.8 m/s (range: 30–67 m/s). Combining
groups 1 and 2, a total of 12 patients (Table 2)
were found to have either significantly reduced
ulnar CMAP amplitude (!0.5 mV) or completely
absent ulnar CMAPs, including 7 from the demye-
linating group and 5 from the axonal group. In
these 12 patients, no other proximal nerve conduc-
tion study was available to review. Six of 12 patients
could have been misclassified by ulnar MCV using
a cutoff value of 38 m/s. Three of 4 patients, who
had absent ulnar CMAPs (and no obtainable fibu-
lar or tibial CMAPs and would have been inter-
preted to have an axonal process had mutations
linked with CMT1. Among the 4 patients from
group 2 who had obtainable ulnar CMAPs with
318 Blink Reflex & Inherited PN
severely decreased amplitude (!0.5 mV), 3 had
ulnar MCV between 30 and 38 m/s.
Blink Reflex Study. All 26 patients in group 1 had
prolonged R1 response latency on a blink reflex
study (>13 m/s). A patient with absent ulnar
CMAP and prolonged R1 latency is shown as exam-
ple in Figure 1. One patient with a PMP22 duplica-
tion had an absent R1 response. The mean value
of R1 latency of the other 25 patients in group 1 is
17.1 ms (range: 13.3–24.6 ms), and markedly pro-
longed R1 latency ("16 ms) was present in 12 of
25 (48%) patients; 14 of 26 (53.8%) patients also
had prolonged ipsilateral R2 latency. The differ-
ence between ipsilateral and contralateral R2 laten-
cy was within 8 ms for all patients (data not
shown). All 23 patients in group 2 had normal R1
(<13 m/s) and R2 latencies (1 patient had the
blink reflex study at age 6 years, and only R1 laten-
cy was recorded), including the 5 patients with
absent or severely decreased ulnar CMAP ampli-
tudes (!0.5 mV). The mean value of R1 latency
was 11.3 ms in group 2 (range: 9.2–12.8 ms). One
patient with severely decreased ulnar CMAP ampli-
tude and normal R1 latency is shown as an exam-
ple in Figure 1.
Correlation and Regression Analysis. All patients in
group 1 had R1 prolongation (>13 ms), and all
patients in group 2 had normal R1 latency regard-
less of ulnar CMAP amplitude; nonetheless, a cor-
relation between R1 latency and ulnar CMAP
amplitude (Fig. 2A; r 5 0.43; P 5 0.03; n 5 25; 1
patient with absent R1 in group 1 was not includ-
ed) was found in group 1, but, no such correlation
MUSCLE & NERVE March 2017
 Table 2. Inherited PN patients with severe status (n 5 12).

a. Group 1 (genetically demyelinating, n57)

G-# Sex AOO (y) Gene mutation Diagnosis AOE (y) NIS score R1 of BR (ms) R-CMAP U M. Amp U MCV
G1-1 F 16 PMP22 duplication CMT1A 52 158 NR 0 NR NR
G1-2 M 47 PMP22 duplication CMT1A 65 94.75 21.4 NA 0.5 18
G1-4 F 12 PMP22 duplication CMT1A 51 86 18.3 0 NR NR
G1-14 M 55 PMP22 duplication CMT1A 64 74 15.6 0.4 0.4 23
G1-19 M 2 PMP22 c.281 del G CMT1A 29 103 23.6 0.3 0.3 14
G1-24 F 18 EGR2 p.R381C CMT1D 50 70 13.4 1.3 0.2 28
G1-25 F 21 EGR2 p.D355G CMT1D 53 87 18.7 0 NR NR

b. Group 2 (genetically axonal, n 5 5)

G-# Sex AOO (y) Gene mutation Diagnosis AOE (y) NIS score R1 of BR (ms) R-CMAP U M. Amp U MCV
G2-12 M 3 BSCL2 p.S154L c-HSP 71 63 12.6 0.4 0.4 37
G2-13 M 10 GJB1 p.I28N17 CMT,X1 46 75.75 12.4 0 NR NR
G2-20 F 59 TTR p.A140S FAP 65 94 12.8 0.6 0.3 52
G2-21 M 63 TTR p.V50M FAP 71 115.5 10.8 0.3 0.3 37
G2-23 M 68 TTR p.V50M FAP 74 64 11 0.1 0.1 30

AOO: age of onset of neurological symptom; AOE; age of electrophysiology evaluation; BR: blink reflex (ms); R-CMAP: including fibular motor, tibia motor,
ulnar motor (mV); U M. Amp: Ulnar motor distal amplitude (mV); U MCV: Ulnar MCV (m/s). NA: value not available; NR: no response.

was found in group 2 patients (r 5 0.02; P 5 0.92;
n 5 23). There was a strong negative correlation
between R1 response and ulnar MCV (Fig. 2B;
r 5 0.80; P < 0.001; n 5 45; 4 patients with absent
ulnar response were not included). The patients
with R1 " 16 ms had a mean NIS of 58 (10–103)
versus NIS mean of 42 (14–74) among patients
with R1 < 16 ms. In group 1 patients, the NIS cor-
relates with summated CMAP amplitude (Fig. 3A;
r 5 0.72; P < 0.001; n 5 21) and ulnar amplitude
(r 5 0.66; P 5 0.001; n 5 21), but not with R1
response latency (Fig. 3B; r 5 0.29; P 5 0.20;
n 5 21).
DISCUSSION
Since the original proposal of an electrophysio-
logical classification of hereditary neuropathies by
Dyck and Lambert in 1968,19 NCS have remained
important in the classification and diagnoses of
inherited polyneuropathies.9,20 Here, we studied

FIGURE 1. Example cases with blink reflexes compared with ulnar motor NCS. (A) Patient 4 in Group 1 (demyelinating) had R1 latency
prolongation (>13 ms) and an absent ulnar CMAP. (B) Patient 23 in Group 2 (axonal) had normal R1 latency (8–13 ms) and severely
decreased ulnar CMAP amplitude.

Blink Reflex & Inherited PN MUSCLE & NERVE March 2017 319
FIGURE 2. Correlation between R1 latency and ulnar CMAP amplitude and ulnar MCV in patients with genetically confirmed inherited
polyneuropathy. (A) All genetic demyelinating patients (n 5 25) had prolongation of R1 latency (>13 ms), and all genetic axonal
patients (n 5 23) had normal R1 latency (8–13 ms). (B) Regression analysis between R1 latency and ulnar motor conduction velocity
(n 5 45). The Pearson coefficient (r) and P value are shown.

the blink reflex as an alternative to proximal
extremity NCS in the classification of primary
demyelinating vs axonal inherited polyneuropathy
patients. This is especially helpful when distal
extremity responses have severely reduced ampli-
tudes which diminish the reliability of conduction
velocities. Specifically, our results indicate that all
genetically confirmed demyelinating patients
(group 1) had prolonged R1 latencies (>13 ms),
whereas all genetically confirmed axonal patients
(group 2) had normal R1 latencies (!13 ms).
Although historically an R1 latency >16 ms was
assumed to be the cutoff value for primary demye-
linating in our EMG lab, our results show that an
R1 latency >13 ms is more sensitive without loss of
specificity in distinction between demyelinating
and axonal forms of inherited neuropathy. The
patients reviewed in this study had variable severity
ranging from mild to severely affected, and 25%
had absent or very low ulnar motor CMAP ampli-
tudes. Age was also variable at evaluation (5–74
years). In severe cases, without other proximal

FIGURE 3. Correlations between neuropathy impairment score (NIS), R-CMAP amplitude, and R1 latency in patients with genetically
confirmed demyelinating polyneuropathy (n 5 21). (A) Significant correlation between NIS and RCMAP amplitude (including fibular, tibi-
al, and ulnar distal CMAP amplitudes). (B) No significant correlation between NIS and R1 latency was found. Pearson coefficients (r)
and P values are shown.

320 Blink Reflex & Inherited PN MUSCLE & NERVE March 2017

FIGURE 4. Blink reflex utility in algorithmic testing of inherited
polyneuropathy. PMP22 duplication testing should first be
ordered in inherited polyneuropathy patients with a prolonged
R1; if it is negative and the patient has a nonspecific inherited
polyneuropathy phenotype, targeted gene panel NGS testing
can be considered.
studies available, blink reflex R1 latency prolonga-
tion was the only EDX indicator to differentiate
demyelinating from axonal neuropathy. As an
example, a PMP22 duplication patient (NIS 5 158)
with flail extremities (including proximal muscles)
had an absent R1, whereas another with similar
severity (NIS 5 120) and a TRPV4 axonal mutation
had a normal R1 latency. This study emphasizes
that blink reflex R1 latency prolongation is a sensi-
tive and specific indicator for primary demyelinat-
ing inherited polyneuropathy regardless of severity.
Our findings have potentially broader implica-
tions. Specifically, because there is 100% stratifica-
tion of demyelinating vs axonal inherited varieties
by R1 latencies in multiple genetic varieties,
including 20 with PMP22 duplications, a simplified
algorithm of nerve conduction stratification for
genetic test ordering could be considered (Fig. 4).
Because there is a high probability of finding a
PMP22 duplication in inherited demyelinating
cases, this test could first be ordered in patients
with an ulnar MCV ! 38 m/s or a prolonged R1
(especially when the ulnar CMAP amplitude
is ! 0.5 mV or the CMAP is absent). Other condi-
tions where individual candidate gene testing
might be suggested from a combination of normal
blink R1 latency and slowed ulnar MCV would
include TTR (FAP) and GJB1 (CMTX1), where
clinical findings and inheritance pattern can sup-
port focused genetic testing. Otherwise, in patients
with a nonspecific polyneuropathy that is assumed
Blink Reflex & Inherited PN
to be inherited, targeted gene panel NGS testing
could be considered.2 In addition, blink reflex R1
latency can be helpful in filtering discovered DNA
variants. For example, in Dejerine-Sottas pheno-
types, point mutations in PMP22, EGR2, GJB1, and
MPZ genes account for the majority of patients
with very slow nerve conductions !15 m/s.1 In our
cohort, 1 patient with a PMP22 gene point muta-
tion (c.281 del G) was seen with an R1 of 23.6 ms
and an ulnar motor conduction velocity of 14 m/s.
Extremity NCS and needle EMG studies are
important to provide localization, severity, and
prognosis. Among CMT1 patients, there is a strong
correlation between distal nerve CMAP amplitudes
and clinical severity.21,22 One extended longitudi-
nal study showed that extreme conduction slowing
associates with more rapid distal motor amplitude
declines.23 In our study, we found no correlation
between NIS score and R1 latency, but there was a
good correlation between severity (NIS) and sum-
mated CMAP amplitudes. The observation of a
strong correlation between ulnar MCV reduction
and R1 latency prolongation in demyelinating
patients is consistent with the diffuse Schwann cell
disorder that is known in PMP22 and other prima-
ry demyelinating conditions.24,25
The approach of considering a focused nerve
conduction evaluation by R1 blink reflex for gene
testing stratification could be especially attractive
in children. This approach would allow for only
NCS at a single site. Also in consideration of clini-
cal trials the current pediatric CMT impairment
scoring has forgone nerve conduction testing in
favor of validated symptoms and functional out-
come measures.26 Such a scoring approach may
not detect the improvement of Schwann cell func-
tion that may occur early in PMP22 mutations. In
children, the R1 oligosynaptic reflex is fully mature
from 2 years on, and therefore, issues of age corre-
lations will be less problematic, whereas nerve con-
duction velocities in the extremities keep
increasing in both diseased and healthy subjects
into the mid-teen years.14,19,27,28 Our study suggests
that R1 latency might be considered to be a sensi-
tive approach for evaluating initial proximal
changes in CMT treatment trials for both children
and adults. This is particularly important in the
study of CMT1A, the most common type of CMT,
where early changes may be difficult to find in
even the best clinical designs.29,30
In summary, we have shown that blink reflex
studies are a reliable tool for identifying inherited
demyelinating polyneuropathy, which is important
in algorithmic decisions in genetic test ordering.
This study shows that a prolonged blink reflex R1
latency (>13 ms) is a specific characteristic of
demyelinating inherited polyneuropathy patients
MUSCLE & NERVE March 2017 321
that can be used as a sensitive tool in assisting
stratification of neuropathy classification regardless
of severity.
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