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Piloerection as the sole symptom of epilepsy: A case report and review of literature*1Ji-Qing Qiu PhD, *2Yu Cui MD, 3Li-Chao Sun MD, 1Bin Qi PhD, 1Xiao-Bo Zhu PhD, 1Zhan-Peng Zhu PhD*JQ Qiu and Y Cui contributed equally to this work and are co-first authors
Departments of 1Neurosurgery, 2Otolaryngology and 3Emergency Medicine, The First Hospital of Jilin University, Changchun, Jilin, China Abstract
Piloerection is an involuntary erection of body hairs that usually has physiological correlates such as cold or a strong emotional experience. Piloerection may also be a rare manifestation of seizure. Here, we report a case of 54-year-old man who experienced pilomotor seizures from temporal lobe epilepsy. The patient presented with sudden piloerection and no loss of consciousness many times a day. Magnetic resonance imaging of the brain showed three lesions in the right hemisphere, with the largest lesion in the right temporal lobe. A video-EEG showed an ictal discharge in the delta range with right temporal onset. Digital subtraction angiography excluded arteriovenous malformation. The lesion in the right temporal lobe was resected. Immunohistochemistry confirmed a cerebral cavernous malformation. There was no further seizure. A review of the published literature revealed that ictal piloerection as a lone manifestation is rare. Most cases of pilomotor seizure originate in the temporal lobe. Close to four fifth of the cases has a structural lesion. EEG was able to confirm the diagnosis of ictal piloerection in the majority of cases.
Keywords: Piloerection, seizure, EEG
Neurology Asia 2018; 23(2) : 163 – 175
Address correspondence to: Dr Zhan-Peng Zhu, Department of Neurosurgery, The First Hospital of Jilin University, 71 Xinmin Street, Changchun, Jilin 130021, P.R. China. E-mail: [email protected]
INTRODUCTION
Piloerection is a neurovegetative phenomenon associated with fever, cold, and strong emotions, such as fear. 1 Accordingly, piloerection is typically accompanied by autonomic reactions including tachycardia, tachypnea, vasoconstriction, shivering, and heightened alertness.2 Piloerection can also a rare manifestation of seizure.3-9 The prevalence of pilomotor seizures in temporal lobe epilepsy is estimated at 1.2%.10 Piloerection as the lone symptom of seizure is said to be rare.3,5,11 We report here the case of a man that presented with piloerection as the only symptom of seizure from temporal lobe cavernoma. We also reviewed published literature on pilomotor seizures.
CASE REPORT
A 54-year-old right-handed man was admitted to our unit with a 2-year history of unexplained recurrent bouts of visible piloerection involving the whole body. The episodes were isolated without other accompanied symptom. Initially, the patient had 2-3 episodes per day of the said
piloerection; each episode lasting 10-20 seconds. Subsequently, the condition worsened, with the increased frequency of the attack up to 10 episodes per day, lasting up to 30-40 seconds each time. During these episodes, the patient did not experience other motor or sensory symptoms; there was no confusion or loss of awareness. The episodes usually occurred in the daytime, most often when under stress. The patient’s past medical and family history was unremarkable. His gestational development and birth history was also normal. He had no past illness that may give rise to development of epilepsy, such as head injury, febrile seizures, encephalitis, meningitis, or cerebrovascular disease. The patient’s physical, mental, and neurologic examinations, routine blood tests, and electrocardiogram were normal. MRI of the brain showed three lesions in the right hemisphere; the largest lesion, with a volume of 3.4 cm × 4.6 cm × 3.7 cm, was in the right temporal lobe. The other two lesions were in the right frontal lobe and the right insular cortex (Figure 1a-d). The
Neurology Asia June 2018
164
MRI brain was supportive of cavernous angioma. Digital subtraction angiography was normal and did not show any arteriovenous malformation (Figure 1e,f). As there has been previous reports of lesion in the temporal lobe causing ictal piloerection12, a diagnosis of epilepsy was suspected. A video-EEG was performed showing an ictal discharge in the delta range with right temporal onset. This event lasted 27s (Figure 2). Corresponding to the rhythmic wave burst, the patient had piloerection over his whole body. Thus, the EEG confirmed that the events were focal seizures. The patient was thus diagnosed to have temporal lobe epilepsy from cavernous malformation. He was treated with oral oxcarbazepine up to 600 mg/daily. This initially decreased the frequency
and severity of the seizures. After 3 months, frequency and severity of the seizures increased again and could not be controlled despite the use of other antiepileptic drugs (AEDs) (carbamazepine, benzodiazepines, phenytoin, gabapentin, phenobarbital, levetiracetam, and valproic acid alone and in combination). With only mild reduction in the frequency of the seizures, the patient was recommended to have surgery. The lesion in the right temporal lobe was resected (Figure 3, 4a). There was no neurological deficits postsurgery. The patient was maintained on oxcarbazepine 450mg/day, and has remained seizure free for 4 months during the last follow up. Histopathological examination of the resected tissue confirmed the diagnosis of cerebral cavernous malformation (Figure 4b).
Figure 1. Preoperative neuroimaging: Axial (a), sagittal (c), and coronal (d) magnetic resonance T2-weighted images revealed a 3.4 cm × 4.6 cm × 3.7 cm lesion in the right medial temporal lobe. Axial T2- fluid attenuated inversion recovery imaging revealed lesions in the right frontal lobe and right insular cortex (b). The lesions showed hypererintense center surrounded by hypointense ring suggestive of cavernoma. Digital subtraction angiography (e, f) excluded arteriovenous malformation.
Figure 2. Ictal EEG at the onset of piloerection showing irregular slow wave delta activity on the right temporal region (a-c).
165
DISCUSSION
Piloerection is usually characterized by involuntary erection of body hairs in response to psychophysiological triggers, including a strong emotional experience or cold.13 As mentioned above, piloerection can also rarely be a symptom of seizure11, particularly from temporal lobe epilepsy.14
A comprehensive literature search of the PubMed and Web of Science databases from inception to August 2017 using the key words ‘piloerection’, ‘goosebump’, ‘pilomotor’ and ‘seizure’ by two independent reviewers was also performed. The searches identified 36 cases in which piloerection was reported as a manifestation of seizure (Table 1). Together with our own case, 26 patients were men, and 10 patients were women (the gender of one patient was not mentioned), with Male : Female ration of 2.6 : 1, suggesting a male predominance. All the patients were adults, age ranged from 23 to 75 years. In three patients (cases 25, 34, 36)3,5,11,
piloerection was the lone seizure manifestation. This suggests that piloerection as a lone ictal manifestation is uncommon. In the majority of patients (25 patients), consciousness was preserved, implying that they were experiencing focal-aware seizures.15 The precise localization of pilomotor seizure is unknown. Animal studies and case reports
on humans implicate the hypothalamus, limbic system, orbital cortex, and the premotor area of the frontal lobe. In cats and/or monkeys, electrical or pharmacological stimulation of the hypothalamus, amygdaloid nuclei, and cingulated gyrus elicited piloerection, bilateral hypothalectomy abolished piloerection, and removal of the premotor area exaggerated piloerection. In humans, piloerection was also associated with changes in brain potentials in the premotor area.7,16 Piloerection occurs as a sympathetic reflex in response to cold, shock, stress, or fear. In pilomotor seizures, piloerection may thus be the initial symptom of a seizure or secondarily induced during the seizure in response to psychic symptoms such as fear. Ictal piloerection is often associated with autonomic symptoms involving the cardiovascular, cutaneous, gastrointestinal, genital, pupillary, respiratory, and urinary systems, implying the involvement of the autonomic and limbic system. As for the clinical pattern of spread of the piloerection, 20/36 (56%) had a focal or somatotopical pattern, whereas in 16/36 (44%) cases, the piloerection was bilateral in distribution. As physiological piloerection is usually bilateral in distribution, a focal or somatotopical pattern may thus help in the clinical diagnosis of ictal piloerection. Including the current case, based on CT/MRI,
Figure 3. Postoperative neuroimaging: CT scan following resection of the right temporal lobe (a, b).
Figure 4. Postoperative imaging showing the lesion (a) and cavernous malformation (Hemotoxylin and Eosin staining; 10×)(b).
Neurology Asia June 2018
166
Tabl
e 1:
Rev
iew
of p
revi
ousl
y re
port
ed c
ases
of p
iloer
ectio
n se
izur
es.
Cas
eA
utho
r/ye
arA
geSe
xD
istr
ibut
ion
of
pilo
erec
tion
Aur
aSy
mpt
oms
Con
scio
usIm
agin
g E
EG
Etio
logy
or
asso
ciat
ed
neur
olog
ical
di
seas
e
Surg
ery
Out
com
e
1La
ndau
et a
l. 19
5332
29M
Rt s
ide
of th
e
face
and
nec
k→
Rt a
rm a
nd
fore
arm
→ R
t le
g→ tr
unk
Feel
ing
of
stra
ngen
ess,
sadn
ess,
fear
, an
d un
real
ity
—Y
—Sl
ow w
ave
burs
t Fo
cal c
ereb
ral
infe
ctio
n of
st
rept
ococ
cic
sept
icem
ia
——
2M
ulde
r et a
l. 19
5433
25F
—A
sens
atio
n lik
e a
“qui
verin
g”
in th
e he
art;
swal
low
ing
Chi
llyN
—Sh
arp
wav
es
aris
ing
from
th
e lt
sylv
ian
fissu
re (a
fter
met
razo
l)
——
—
3M
ulde
r et a
l. 19
5433
43M
Thig
hsTi
nglin
g pa
rest
hesi
a in
the
abdu
ctor
regi
ons
of th
e th
igh
( som
etim
es
invo
lved
the
scro
tal a
rea)
—N
—Sl
ow w
aves
or
igin
atin
g fr
om a
focu
s in
the
lt te
mpo
ral l
obe
——
—
4B
rody
et a
l. 19
6024
53F
The
limbs
and
ch
est
Abd
omin
al
disc
omfo
rt,
naus
ea
Fairl
y ra
pid
and
deep
br
eath
ing
YA
focu
s of
irreg
ular
rh
ythm
s in
the
rt te
mpo
ral
area
Glio
blas
tom
a m
ultif
orm
eTu
mor
exci
sion
Ora
l dila
ntin
, co
mpl
ete
cess
atio
n of
se
izur
es
5A
nder
man
n et
al.
1984
2561
MR
t arm
An
unus
ual
chem
ical
odo
r. Th
e fe
elin
g lik
e a
vibr
atio
n in
the
abdo
men
risi
ng
to th
e he
ad
War
m, c
old,
a
chill
y fe
elin
g,
vibr
atio
n, o
r sh
iver
ing
in
the
rt le
g
YC
T: a
m
alig
nant
tu
mor
in
the
ante
rior
porti
on o
f th
e te
mpo
ral
lobe
, ins
ula,
an
d ba
sal
gang
lia
—G
liobl
asto
ma
An
exte
nsiv
e an
terio
r te
mpo
ral
lobe
ctom
y
Afte
r sur
gery
, se
izur
es
cont
inue
d fo
r se
vera
l wee
ks
Oxy
ence
phal
ogr
am: p
artia
lfil
ling
of th
epo
ster
orpo
rtion
of t
hert
late
ral
vent
ricle
of
the
tem
pora
l an
d oc
cipi
tal
horn
s
167
Tabl
e 1:
Rev
iew
of p
revi
ousl
y re
port
ed c
ases
of p
iloer
ectio
n se
izur
es.
Cas
eA
utho
r/ye
arA
geSe
xD
istr
ibut
ion
of
pilo
erec
tion
Aur
aSy
mpt
oms
Con
scio
usIm
agin
g E
EG
Etio
logy
or
asso
ciat
ed
neur
olog
ical
di
seas
e
Surg
ery
Out
com
e
1La
ndau
et a
l. 19
5332
29M
Rt s
ide
of th
e
face
and
nec
k→
Rt a
rm a
nd
fore
arm
→ R
t le
g→ tr
unk
Feel
ing
of
stra
ngen
ess,
sadn
ess,
fear
, an
d un
real
ity
—Y
—Sl
ow w
ave
burs
t Fo
cal c
ereb
ral
infe
ctio
n of
st
rept
ococ
cic
sept
icem
ia
——
2M
ulde
r et a
l. 19
5433
25F
—A
sens
atio
n lik
e a
“qui
verin
g”
in th
e he
art;
swal
low
ing
Chi
llyN
—Sh
arp
wav
es
aris
ing
from
th
e lt
sylv
ian
fissu
re (a
fter
met
razo
l)
——
—
3M
ulde
r et a
l. 19
5433
43M
Thig
hsTi
nglin
g pa
rest
hesi
a in
the
abdu
ctor
regi
ons
of th
e th
igh
( som
etim
es
invo
lved
the
scro
tal a
rea)
—N
—Sl
ow w
aves
or
igin
atin
g fr
om a
focu
s in
the
lt te
mpo
ral l
obe
——
—
4B
rody
et a
l. 19
6024
53F
The
limbs
and
ch
est
Abd
omin
al
disc
omfo
rt,
naus
ea
Fairl
y ra
pid
and
deep
br
eath
ing
YA
focu
s of
irreg
ular
rh
ythm
s in
the
rt te
mpo
ral
area
Glio
blas
tom
a m
ultif
orm
eTu
mor
exci
sion
Ora
l dila
ntin
, co
mpl
ete
cess
atio
n of
se
izur
es
5A
nder
man
n et
al.
1984
2561
MR
t arm
An
unus
ual
chem
ical
odo
r. Th
e fe
elin
g lik
e a
vibr
atio
n in
the
abdo
men
risi
ng
to th
e he
ad
War
m, c
old,
a
chill
y fe
elin
g,
vibr
atio
n, o
r sh
iver
ing
in
the
rt le
g
YC
T: a
m
alig
nant
tu
mor
in
the
ante
rior
porti
on o
f th
e te
mpo
ral
lobe
, ins
ula,
an
d ba
sal
gang
lia
—G
liobl
asto
ma
An
exte
nsiv
e an
terio
r te
mpo
ral
lobe
ctom
y
Afte
r sur
gery
, se
izur
es
cont
inue
d fo
r se
vera
l wee
ks
Cas
eA
utho
r/ye
arA
geSe
xD
istr
ibut
ion
of
pilo
erec
tion
Aur
aSy
mpt
oms
Con
scio
usIm
agin
g E
EG
Etio
logy
or
asso
ciat
ed
neur
olog
ical
di
seas
e
Surg
ery
Out
com
e
6G
reen
et a
l. 19
848
44M
Rt s
ide
of th
e
face
→ R
t arm
an
d le
g
Epig
astri
c se
nsat
ion,
an
unpl
easa
nt o
dor
A se
nsat
ion
of c
hills
YC
T: R
t te
mpo
ral
lobe
lesi
on
Shar
p an
d sl
ow w
aves
in
the
rt fr
onta
l an
d te
mpo
ral
regi
ons
Glio
blas
tom
a m
ultif
orm
eB
iops
ySe
izur
es
decr
ease
d,
then
in
crea
sed
7B
rogn
a et
al.
1986
2658
MA
rms→
nec
k→
head
A g
ener
aliz
ed
feel
ing
of
wei
ghtle
ssne
ss,
then
a w
arm
, ris
ing
visc
eral
se
nsat
ion
Epig
astri
c tin
glin
g; a
so
ur ta
ste
YM
RI:
a re
gion
of
incr
ease
d T1
- and
T2-
wei
ghte
d si
gnal
s in
the
lt m
esia
l te
mpo
ral a
rea
exte
ndin
g in
to th
e pa
rieta
l lob
e
Lt m
esio
basa
l te
mpo
ral
rhyt
hmic
sh
arp
wav
e di
scha
rges
Gra
de II
fib
rilla
ry
astro
cyto
ma
Bio
psy
—
8Ty
ndel
et a
l. 19
8627
56M
Legs
→ a
rms→
tru
nk→
hea
dan
unu
sual
smel
lN
ause
aY
CT:
a la
rge
ltte
mpo
ropa
riet
al, w
hite
mat
ter,
ring
enha
ncin
g le
sion
with
m
arke
d m
ass
effe
ct
Nor
mal
Glio
blas
tom
a m
ultif
orm
e
Die
d
9A
hern
et a
l. 19
88.17
35M
Bila
tera
l sid
eSu
dden
ly fe
el
depr
esse
d,
anxi
ous,
or
wea
k an
d he
ad
“tig
htne
ss”
Swea
t and
a
fear
ful f
acia
l ex
pres
sion
YC
T: rt
te
mpo
ral
hem
atom
a w
ith
surr
ound
ing
edem
a
norm
alTr
aum
atic
co
ntus
ion
in
the
rt an
terio
r te
mpo
ral l
obe
NO
ral c
, se
izur
e fr
ee
10Yu
et a
l. 19
9815
29M
Verte
x→lt
uppe
r and
lt
low
er li
mbs
Stra
nge
or fe
arfu
l se
nsat
ions
Mot
ionl
ess
star
ing
with
au
tom
atis
ms
of li
p-sm
acki
ng
and
fum
blin
g
YM
RI:
lt m
esia
l te
mpo
ral
abno
rmal
ities
Inte
ricta
l sp
ikin
g ov
er
the
lt te
mpo
ral
regi
on
h sc
lero
sis
Lt a
nter
ior
tem
pora
l lo
bect
omy
Seiz
ure
free
Lt p
ari
cran
ioto
my
Neurology Asia June 2018
168
Cas
eA
utho
r/ye
arA
geSe
xD
istr
ibut
ion
of
pilo
erec
tion
Aur
aSy
mpt
oms
Con
scio
usIm
agin
g E
EG
Etio
logy
or
asso
ciat
ed
neur
olog
ical
di
seas
eSu
rger
yO
utco
me
11Yu
et a
l. 19
9815
37M
Lt th
igh,
in
guin
al a
nd
scro
tal a
reas
→
lt ab
dom
en, l
t ch
est w
all,
lt ar
m, l
t fac
e →
ve
rtex
regi
on.
—C
hills
, pa
lpita
tions
an
d po
orly
de
scrib
ed
fear
ful
feel
ings
YC
T: a
mas
s le
sion
in th
e lt
sphe
noid
re
gion
Rhy
thm
ic
disc
harg
es
over
the
lt te
mpo
ral
regi
on
C o
ral,
seiz
ure
free
12R
oze
et a
l. 20
001
66M
Rt h
emib
ody
①
Epig
astri
c se
nsat
ion
with
a
feel
ing
of
thor
acic
op
pres
sio
n② fl
ushi
ng
of fa
ce a
nd
arm
s ③ a
se
nsat
ion
of
hot l
iqui
d in
the
rt ha
nd→
the
rt fo
ot→
th
e w
hole
bo
dy
Agi
tate
d an
d di
sorie
ntat
ed
as to
tim
e an
d pl
ace;
fa
lse
reco
gniti
ons,
conf
abul
a-tio
ns, a
nd
delu
sion
of
pers
ecut
ion
YC
T/M
RI:
norm
alSh
arp
wav
e di
scha
rges
at
thet
a fr
eque
ncie
s in
the
left
front
otem
pora
l re
gion
→ b
oth
hem
isph
eres
NC
ora
l, se
izur
e fr
ee
13C
utts
et a
l. 20
0228
49M
Entir
e bo
dyA
met
allic
tast
eA
har
d ep
igas
tric
sens
atio
n,
fatig
ue, a
se
nse
of ti
me
loss
—M
RI:
A
larg
e rt
fron
topa
rieta
l m
ass w
ith
exte
nsio
n in
to th
e rt
tem
pora
l lob
e
—A
stro
cyto
ma
Bio
psy
and
subt
otal
ex
cisi
on
of th
e tu
mor
14Sa
’ada
h et
al
. 200
21826
MLt
leg
and
foot
→ lt
thig
h,
ingu
inos
crot
al
area
, lt
abdo
men
and
ch
est w
all,
lt ar
m a
nd fa
ce →
ve
rtex
—U
nexp
lain
ed
bout
s of
shiv
erin
g se
nsat
ion,
pa
lpita
tions
, sw
eatin
g,
epig
astri
c di
scom
fort,
an
d po
orly
de
scrib
ed
fear
ful a
nd
stra
nge
feel
ings
YC
T: a
ne
opla
stic
le
sion
in th
e lt
sphe
noid
ar
ea
Inde
pend
ent
freq
uent
sh
arp-
wav
e co
mpl
exes
and
slow
-wav
e di
scha
rges
w
ith lt
te
mpo
ral
dom
inan
ce
Lt sp
heno
id
men
ingi
oma
or lt
tem
pora
l tip
con
tusi
on
Tum
or
rese
ctio
nC
ora
l, se
izur
e fr
ee
Hyp
eros
mol
a,no
n-ke
totic
hype
rgly
caem
iaMen
ingo
thel
iom
atou
sm
enin
giom
aor
trau
mat
icsu
bara
chno
idhe
mor
rhag
e
Cra
niot
omy
with
tum
orre
sect
ion
Dex
amet
has
one
and
phen
ytoi
nor
al, s
eizu
refr
ee
169
Cas
eA
utho
r/ye
arA
geSe
xD
istr
ibut
ion
of
pilo
erec
tion
Aur
aSy
mpt
oms
Con
scio
usIm
agin
g E
EG
Etio
logy
or
asso
ciat
ed
neur
olog
ical
di
seas
eSu
rger
yO
utco
me
15Sa
’ada
h et
al
. 200
21823
FLt
low
er
limb→
the
trunk
,lt u
pper
lim
b, lt
side
of
the
face
and
ve
rtex
Stra
nge
feel
ing
Mot
ionl
ess
star
ing
with
au
tom
atis
ms
of li
p sm
acki
ng,
swea
ting,
an
d fu
mbl
ing
YC
T/M
RI:
norm
alSl
ow-w
ave
activ
ity
from
bot
h si
des,
high
am
plitu
de
shar
p-sp
ike-
wav
e di
scha
rges
w
ith rt
fro
ntot
empo
ral
dom
inan
ce
Idio
path
icN
C o
ral,
seiz
ure
free
16Se
o et
al.
2003
3127
MR
t leg
→ rt
ar
m→
lt lim
bsB
ehav
iour
al
arre
st, c
ompl
ex
mot
or a
ctiv
ity
incl
udin
g pe
lvic
th
rust
ing,
w
hole
bod
y m
ovem
ents
, and
ra
rely
laug
hter
—N
Subt
ract
ed
icta
l SPE
CT
core
gist
ered
w
ith M
RI
show
ed
mul
tifoc
al
hype
r-pe
rfus
ed
area
s in
the
ante
rior
med
ial
fron
tal a
rea
Subd
ural
el
ectro
de
arra
ys: t
he
seiz
ures
wer
e or
igin
atin
g in
th
e an
terio
r m
edia
l fro
ntal
re
gion
.
Mild
cor
tical
dy
slam
inat
ion
Rt a
nter
ior
fron
tal
lobe
ctom
y
O o
ral,
seiz
ure
free
17D
ove
et a
l. 20
0419
26F
Who
le b
ody
Fear
and
pan
ic,
feel
ings
of
war
mth
and
na
usea
Col
d sh
iver
sY
MR
I: rt
mes
ial
tem
pora
l sc
lero
sis
Dis
char
ge
begi
nnin
g in
th
e rt
ante
rior
tem
pora
l lo
be a
nd
tach
ycar
dia
Rt m
edia
l te
mpo
ral
scle
rosi
s
NLo
raze
pam
or
al, s
eizu
re
free
18U
sui e
t al.
2005
2041
MLt
arm
A fu
nny
feel
ing
in th
e he
adEp
igas
tric
sens
atio
nN
MR
I: no
rmal
; PE
T-C
T:hy
pom
eta-
bolis
m in
the
lt fr
onto
tem
-po
ral r
egio
ns
Icta
l EEG
: R
hyth
mic
sp
ikin
g at
the
lt sp
heno
idal
el
ectro
de.
Intra
cran
ial
EEG
: ict
al
disc
harg
es in
th
e lt
mes
ial
tem
pora
l are
a
Mes
ial
tem
pora
l sc
lero
sis
Seiz
ure
free
Lt ante
rom
esi
al te
mpo
ral
lobe
ctom
y
Neurology Asia June 2018
170
Cas
eA
utho
r/ye
arA
geSe
xD
istr
ibut
ion
of
pilo
erec
tion
Aur
aSy
mpt
oms
Con
scio
usIm
agin
g E
EG
Etio
logy
or
asso
ciat
ed
neur
olog
ical
di
seas
eSu
rger
yO
utco
me
19W
iese
r et a
l. 20
0530
42M
Lt a
rm a
nd th
e fa
ce—
Fear
, ol
fact
ory,
hallu
cina
-tio
ns, a
ndar
rest
re
actio
ns
YIn
teric
al E
EG:
slow
ing
of th
e ba
ckgr
ound
ac
tivity
with
m
ixed
alp
ha
and
thet
a ac
tivity
. Ic
tal E
EG:
thet
a pa
ttern
al
tern
atin
g be
twee
n th
e si
des,
mos
tly
on th
e lt,
but
al
so o
n th
e rt
and
freq
uent
ly
with
co
ntra
late
ral
prop
agat
ion
Non
-pa
rane
opla
stic
lim
bic
ence
phal
itis
N
20M
asno
u et
al
. 200
614
35F
Bot
h ar
ms a
nd
legs
—B
ilate
ral
sens
atio
ns o
f ch
ill, c
olor
ed
phos
phen
es
in th
e rt
hem
i-fiel
d,
naus
ea,
thor
acic
co
mpr
essi
on
YM
RI:
decr
ease
of
sign
al
abno
rmal
ity
in th
e lt
h’,
atro
phy
of
the
lt h’
Inte
ricta
l EE
G: n
orm
al.
Icta
l EEG
: di
ffuse
fla
tteni
ng
of e
lect
rical
ac
tivity
fo
llow
ed b
y a
rhyt
hmic
slow
ac
tivity
with
a
max
imum
am
plitu
de o
n th
e lt
cent
ral
and
tem
pora
l ar
ea
—N
C o
ral,
seiz
ure
free
21La
m e
t al.
2010
972
MR
t hem
ibod
y Ta
chyc
ardi
a,
hype
rtens
ion,
w
arm
th, f
acia
l flu
shin
g
Palp
itatio
n,
anxi
ety,
oc
casi
onal
he
adac
he
YM
RI:
rt m
esia
l te
mpo
ral
T2 si
gnal
ch
ange
, en
hanc
emen
t, an
d su
bseq
uent
at
roph
y
Rt t
empo
ral
shar
p w
aves
→
alph
a fr
eque
ncy
disc
harg
es→
th
e en
tire
rthe
mis
pher
e →
the
cont
rala
tera
l te
mpo
ral l
obe
Lim
bic
ence
phal
itis
NLe
vetir
acet
am
and
valp
roic
ac
id o
ral,
seiz
ure
free
Imm
unos
up-
pres
sive
ther
apy,
seiz
ure
free
MR
I in
the
acut
e ph
ase:
non
-pa
rane
opla
stic
lim
bic
ence
phal
itis.
Afte
r 18
mon
ths,
MR
I: h
sele
rosi
s.
171
Cas
eA
utho
r/ye
arA
geSe
xD
istr
ibut
ion
of
pilo
erec
tion
Aur
aSy
mpt
oms
Con
scio
usIm
agin
g E
EG
Etio
logy
or
asso
ciat
ed
neur
olog
ical
di
seas
eSu
rger
yO
utco
me
22M
ittal
et a
l. 20
1021
57M
Bot
h up
per
extre
miti
esA
stra
nge
smel
l an
d sw
eaty
pa
lms
NY
MR
I: a
none
nhan
cing
m
ass
invo
lvin
g th
e rt
med
ial
tem
pora
l lob
e
Rt t
empo
ral
regi
on d
elta
sl
owin
g in
term
ixed
w
ith sh
arp,
sp
ike
wav
es
Ast
rocy
tom
a w
ith a
n ol
igod
endr
o-gl
ial
com
pone
nt
Rem
oval
of
les
ion
and
surr
oun-
ding
ep
ilep-
toge
nic
zone
Seiz
ure
free
23Pu
lighe
ddu
et a
l. 20
102
54M
Bot
h fo
rear
ms
—Ta
chyc
ardi
a,
anxi
ety
Y—
Abr
uptly
by
1 to
2 se
cond
s, ge
nera
l vo
ltage
de
crea
se,
incl
udin
g at
tenu
atio
n of
the
β ac
tivity
in th
e ce
ntra
l lea
ds,
with
som
e co
ntin
uatio
n in
the
post
erio
r hea
d re
gion
—N
Valp
roic
ac
id a
nd
leve
tirac
etam
or
al, s
eizu
res
redu
ced
24St
rzel
czyk
et
al. 2
01022
65F
Ipsi
late
ral
—Pa
raph
asia
—In
term
itten
t sl
owin
g an
d sh
arp
wav
es
over
the
lt te
mpo
ral l
obe
——
25Ya
o et
al.
2010
1130
FLt
leg
——
—M
RI:
norm
alIc
tal E
EG:
foca
l slo
w
wav
es o
n th
e rt
parie
tal a
nd
tem
pora
l lob
es
Vira
l en
ceph
aliti
sN
C o
ral,
seiz
ure
free
26Fi
sch
et a
l. 20
1229
68M
Diff
use
—N
onflu
ent
apha
sia,
em
otio
nal
dist
ress
, foc
al
myo
clon
ic
jerk
—M
RI:
a lt
tem
pora
l m
ass e
xten
- di
ng fr
om
the
tem
pora
l po
le to
the
pulv
inar
and
in
volv
ing
amyg
dala
an
d h’
Epile
ptic
di
scha
rges
ov
er lt
te
mpo
ral
deriv
atio
ns
Ana
plas
tic
astro
cyto
ma
Parti
al
lobe
ctom
y—
Lesi
onec
-to
my
with
post
erio
rre
sect
ion
ofth
e h’
CT/M
RI: A
nin
trave
ntric
ular
calc
ified
men
ingi
oma
Neurology Asia June 2018
172
Cas
eA
utho
r/ye
arA
geSe
xD
istr
ibut
ion
of
pilo
erec
tion
Aur
aSy
mpt
oms
Con
scio
usIm
agin
g E
EG
Etio
logy
or
asso
ciat
ed
neur
olog
ical
di
seas
eSu
rger
yO
utco
me
27H
ayka
l et
al. 23
75M
Bila
tera
l low
er
extre
miti
esA
hot
flas
h st
artin
g in
the
head
and
runn
ing
dow
n th
e bo
dy
Wor
d fin
ding
di
fficu
lties
, st
utte
ring
or
dysa
rthria
, an
d oc
casi
onal
ly
a ja
w tr
emor
YA
n ic
tal
disc
harg
e of
thet
a ra
nge
with
co
nsis
tent
lt
infe
rom
esia
l-an
terio
r te
mpo
ral o
nset
Her
pes z
oste
r en
ceph
aliti
sN
Lam
otrig
ine
oral
, sei
zure
fr
ee
28K
urita
et a
l. 20
137
38M
Bila
tera
l arm
s an
d ba
ck—
Chi
llsY
MR
I: hi
gh
inte
nsity
in
the
rt h’
, rt
uncu
s, rt
amyg
dala
, an
d sw
olle
n rt
h’. T
hen
rt h
atro
phy
Rhy
thm
ic
wav
es in
the
rt te
mpo
ral a
rea
and
grad
ually
be
cam
e sl
ow
and
irreg
ular
—N
C o
ral,
seiz
ure
free
29A
sha
et a
l. 20
146
66M
Rt a
rm→
lt ar
mN
ause
ous,
‘fun
ny’ f
eelin
g in
the
stom
ach,
tin
glin
g se
nsat
ion
on th
e rt
arm
Vis
ual
dist
urba
nces
an
d a
feel
ing
of
‘det
achm
ent
from
real
ity’;
post
-icta
l dy
spho
ria;
olfa
ctor
y an
d gu
stat
ory
hallu
cina
tions
of
‘bur
ning
ru
bber
’
—M
RI:
the
ill d
efine
d en
hanc
ing
lesi
on in
th
e su
perio
r m
edia
l as
pect
of
the
post
erio
r ho
rn o
f the
rig
ht la
tera
l ve
ntric
le
exte
ndin
g to
th
e sp
leni
um.
The
lesi
on
parti
ally
ex
tend
ing
into
the
tem
pora
l ho
rn o
f rig
ht la
tera
l ve
ntric
le.
NPr
imar
y gl
iobl
asto
ma
mul
tifor
me
Deb
ulki
ng
of th
e le
sion
Ant
i-epi
lept
ic
med
icat
ion,
se
izur
e fr
ee
30R
ocam
ora
et
al. 2
0145
40M
——
Swea
ting
on
hand
sY
MR
I: rt
h sc
lero
sis
Rt a
nter
ior
tem
pora
l lob
e se
izur
e on
set
CSF
ana
lysi
s:
Ma2
an
tibod
ies
.
NSe
izur
es
cont
inue
MRI
: mild
T2
hype
rinte
nsity
in th
e lt
mes
ial
tem
pora
l lob
e
173
Cas
eA
utho
r/ye
arA
geSe
xD
istr
ibut
ion
of
pilo
erec
tion
Aur
aSy
mpt
oms
Con
scio
usIm
agin
g E
EG
Etio
logy
or
asso
ciat
ed
neur
olog
ical
di
seas
eSu
rger
yO
utco
me
31R
ocam
ora
et
al. 2
0145
39M
Lt si
de—
Bod
y pa
rest
hesi
asY
MR
I: rt
h sc
lero
sis
Rt p
oste
rior
tem
pora
l lob
e se
izur
e on
set
Seru
m a
nd
CSF
ana
lysi
s:
LGI1
an
tibod
ies.
NIn
trave
nous
c’
, sei
zure
fr
ee
32R
ocam
ora
et
al. 2
0145
35F
Wid
e sp
read
Spee
ch a
rres
tO
ral
auto
mat
ism
sY
MR
I: lt
amyg
dala
and
h’
swel
ling
Lt a
nter
ior
tem
pora
l lob
e se
izur
e on
set
Seru
m
anal
ysis
: LG
I1
antib
odie
s.
NIn
trave
nous
c’
, sei
zure
fr
ee
33R
ocam
ora
et
al. 2
0145
32F
Rt a
rm—
Epig
astri
c se
nsat
ion
NM
RI:
lt h
scle
rosi
s Lt
ant
erio
r te
mpo
ral l
obe
seiz
ure
onse
t
CSF
ana
lysi
s:
anti-
Hu
antib
odie
s
NSe
izur
e co
ntin
ue
34R
ocam
ora
et
al. 2
0145
52M
Gen
eral
ized
——
—M
RI: b
oth
tem
pora
l lo
bes
hype
rinte
rsity
Rt t
empo
ral
lobe
with
th
eta-
delta
rh
ythm
ic
activ
ity
Seru
m
anal
ysis
: LG
I1
antib
odie
s.
NSe
izur
e co
ntin
ue
35Sy
mvo
ulak
is
et a
l. 20
164
64M
Bila
tera
l—
Feel
ing
of
anxi
ety,
naus
ea, a
se
nsat
ion
of
impe
ndin
g lo
ss o
fco
nsci
ousn
ess
YM
RI:
incr
ease
d si
gnal
in
tens
ity in
th
e in
ferio
r te
mpo
ral
gyru
s and
the
h’ b
ilate
rally
, w
ith
addi
tiona
l sm
all n
odul
ar
gado
liniu
m
enha
ncem
ent
in b
oth
tem
pora
l lo
bes
No
paro
xysm
al
activ
ity
Lim
bic
ence
phal
itis
N—
36W
hatle
y et
al
. 201
73—
—Lt
hem
ibod
y—
—Y
MR
I: ca
vern
ous
angi
omas
in
the
lt te
mpo
ral p
ole
and
lt gy
rus
rect
us
—C
aver
nous
an
giom
as—
—
C=c
arba
maz
epin
e; C
’=co
rtico
ster
oids
; C
T=co
mpu
ted
tom
ogra
phy;
EEG
=ele
ctro
ence
phal
ogra
m;
F =
fem
ale;
Fro
n=fr
onta
l; h=
hipp
ocam
pal;
h’=h
ippo
cam
pus;
Lt=
lef
t; M
= m
ale;
M
RI=
mag
netic
reso
nanc
e im
agin
g; N
=no;
O=o
xcar
baze
pine
; PET
=pos
itron
em
issi
on to
mog
raph
y; P
ari=
parie
tal;
Rt=
righ
t; Y
=yes
; — =
not
ava
ilabl
e
Neurology Asia June 2018
174
EEG and response to surgery, we estimated that the pilomotor seizures originated from the temporal lobe in 21/37 (57%, cases 6, 9, 10, 11, 14, 17, 18, 20, 21, 22, 24, 27, 28, 30, 31, 32, 33, 34, 35, 36, current case) cases3,5,7-9,14,15,17-23 and another 7/37 (20%, 4, 5, 7, 8, 13, 26, 29) had temporal lobe plus origin of the seizure.6,24-29 The discharges from the temporal lobe may spread to affect the central autonomic network resulting in in the pilomotor seizures and other autonomic manifestations. Based on CT/MRI including our case, there were 12 pilomotor seizures with pathology on the right side, and 11 cases with pathology on the left. There is thus no hemispheric predominance of pilomotor seizure. It has been said that when the epileptogenic zone is in the left hemisphere, ictal piloerection is often accompanied by ictal cold shiver.10
It is also noteworthy that structural lesion was found in the CT/MRI in 29/37 (78%) of the cases, including the present case. Multiple etiologies were identified, including glioma6,8,21,24-29, limbic encephalitis4,9,30, and as in our patient, cavernous malformation. Thus, in close to four fifth of the cases, the pilomotor seizure is associated with a structural lesion demonstrable by CT/MRI. Carbamazepine and oxcarbazepine are commonly used to treat pilomotor seizures.7,11,14-18,31 Resection of the lesion in the right temportal lobe was performed in the current case, and the patient was seizure free after surgery. Similar findings were reported in other published literature.6,15,18,20,21,24,28,31 This support that resection is an effective treatment for pilomotor seizures associated with cavernoma. In 29/33 (88%) of the cases, EEG abnormalities could be demonstrable in the ictal or interictal EEG recordings. EEG is thus a sensitive tool for the diagnosis of ictal piloerection. In conclusion, we report a man that presented with piloerection as the only symptom of seizure, confirmed by ictal EEG. The seizures from temporal lobe cavernoma was not responsive to AED, but was responsive to surgical resection. Clinicians should have increased awareness of pilomotor seizures.
ACKNOWLEDGEMENTS
This manuscript has been edited and proofread by Medjaden Bioscience Limited. The study was approved by the ethics committee of First Hospital of Jilin University. Informed parental consent was obtained in this case. We have received a signed release form from the patient parents authorizing the publication of her material.
DISCLOSURE
Financial support: None
Conflict of interest: None
REFERENCES
1. Siepmann T, Gibbons CH, Illigens BM, Lafo JA, Brown CM, Freeman R. Quantitative pilomotor axon reflex test: a novel test of pilomotor function. Arch Neurol 2012;69:1488-92.
2. Puligheddu M, Bortolato M, Barberini L, et al. Dissociation between neurovegetative signs and subjective symptoms in a case of idiopathic pilomotor seizures. Cogn Behav Neurol 2010;23:274-7.
3. Whatley BP, Jubenville C, Clarke DB, Sadler MR. Focal seizures with left hemibody piloerection related to left hemisphere cavernous angiomas. Epileptic Disord 2017;19:232.
4. Symvoulakis EK, Anyfantakis D, Zaganas I. Pilomotor seizures: an unusual presentation of limbic encephalitis. Acta Neurol Belg 2016;116:365-7.
5. Rocamora R, Becerra JL, Fossas P, et al. Pilomotor seizures: an autonomic semiology of limbic encephalitis? Seizure 2014;23:670-3.
6. Asha MJ, Tansey RJ, Gan YC. ‘Goose bumps’ as presenting feature of intraventricular glioblastoma multiforme. Br J Neurosurg 2014;28:276-7.
7. Kurita T, Sakurai K, Takeda Y, Kusumi I. Pilomotor seizures in temporal lobe epilepsy: A case report with sequential changes in magnetic resonance imaging. Epilepsy Behav Case Rep 2013;1:142-5.
8. Green JB. Pilomotor seizures. Neurology 1984;34:837-9.
9. Lam EM, Worrell GA, Laughlin RS. Semiology of the rare seizure subtype piloerection. Arch Neurol 2010;67:1524-7.
10. Stefan H, Pauli E, Kerling F, Schwarz A, Koebnick C. Autonomic auras: left hemispheric predominance of epileptic generators of cold shivers and goose bumps? Epilepsia 2002;43:41-5.
11. Yao Z, Luo C, Yao D, Zhou D. Localization of pilomotor seizure demonstrated by electroencephalography /functional magnetic resonance imaging. Neurol India 2010;58:758-60.
12. Loddenkemper T, Kellinghaus C, Gandjour J, et al. Localising and lateralising value of ictal piloerection. J Neurol Neurosurg Psychiatry 2004;75:879-83.
13. Benedek M, Kaernbach C. Physiological correlates and emotional specificity of human piloerection. Biol Psychol 2011;86:320-9.
14. Masnou P, Gagnepain JP, Fouad A, Ducreux D, Adams D. Pilomotor seizures associated with sequential changes in magnetic resonance imaging. Epileptic Disord 2006;8:232-7.
15. Yu H, Yen D, Yiu C, Chung W, Lirng J, Su M. Pilomotor seizures. Eur Neurol 1998;40:19-21.
16. Roze E, Oubary P, Chedru F. Status-like recurrent pilomotor seizures: case report and review of the literature. J Neurol Neurosurg Psychiatry 2000;68:647-9.
17. Ahern GL, Howard GF, 3rd, Weiss KL. Posttraumatic
175
pilomotor seizures: a case report. Epilepsia 1988;29:640-3.
18. Sa’adah MA, Shawabkeh A, Sa’adah LM, Inshasi J. Pilomotor seizures: symptomatic vs. idiopathic report of two cases and literature review. Seizure 2002;11:455-9.
19. Dove GH, Buchhalter JR, Cascino GD. Acute repetitive pilomotor seizures (goose bumps) in a patient with right mesial temporal sclerosis. Clin Neurophysiol 2004;115:2511-3.
20. Usui N, Kajita Y, Maesawa S, Endo O, Takebayashi S, Yoshida J. Pilomotor seizures in mesial temporal lobe epilepsy: a case confirmed by intracranial EEG. Seizure 2005;14:288-91.
21. Mittal S, Oni-Orisan A, Stenz J, Shah AK. Temporal neocortical origin of pilomotor seizures in association with an infiltrating glioma: a case confirmed by intracranial electroencephalography monitoring. J Neurosurg 2010;113:388-93.
22. Strzelczyk A, Nowak M, Bauer S, et al. Localizing and lateralizing value of ictal flatulence. Epilepsy Behav 2010;17:278-82.
23. Haykal MA, Abou-Khalil B. Pilomotor seizures: a video case report. Epileptic Disord 2012;14:76-9.
24. Brody IA, Odom GL, Kunkle EC. Pilomotor seizures: Report of a case associated with a cerebral glioma. Neurology 1960;10:993-7.
25. Andermann F, Gloor P. Pilomotor seizures. Neurology 1984;34:1623.
26. Brogna CG, Lee SI, Dreifuss FE. Pilomotor seizures. Magnetic resonance imaging and electroencephalographic localization of originating focus. Arch Neurol 1986;43:1085-6.
27. Tyndel F, Bayer N, Preiksaitis H. An additional pilomotor seizure. Neurology 1986;36:305.
28. Cutts J, Lee G, Berarducci M, Thomas C, Dempsey PK, Kadish SP. Goosebumps. Lancet 2002;360:690.
29. Fisch L, Megevand P, Badoud S, Seeck M, Burkhard PR. Pilomotor seizure: when paroxysmal gooseflesh heralds brain tumor. Neurology 2012;78:1189.
30. Wieser S, Kelemen A, Barsi P, et al. Pilomotor seizures and status in non-paraneoplastic limbic encephalitis. Epileptic Disord 2005;7:205-11.
31. Seo DW, Lee HS, Hong SB, Hong SC, Lee EK. Pilomotor seizures in frontal lobe epilepsy: case report. Seizure 2003;12:241-4.
32. Landau WM. March of piloerection as a convulsive equivalent. AMA Arch Neurol Psychiatry 1953;69:766-8.
33. Mulder DW, Daly D, Bailey AA. Visceral epilepsy. AMA Arch Intern Med 1954;93:481-93.
