*
i&E
Elsevie;Scicnce
Publishers
B.V.
Ail
rights
reserved
0304-3959/9;?/$OS.Oil
~~l~-rn~t~~ati~~ hollowing brachial plexus injury sustained at birth Eugene
Rossitch,
Sr., W. Jerry Oakes, Janice Uvelmen-Levitt
and Blaine S. Nashold, Jr.
Self-mutilation after deafferentation injuries has been reported only rarely in adult humans. This Summary behavior has been found to be similar to that observed in animals that have been subjected to cxperimcntal deafferentation. We present a child with a brachial plexus injury sustained at birth who began to bite her analgesic digits. Self-mutilation behavior in humans is reviewed and its relevance to current deaff~rentat~on pain animai models is examined. This behavior in humans further validates the current animal model of dcaffcrcntati~~il pain. Key words: Self-mutilation;
Deafferentation;
Brachial plexus avulsion; Infancy; Kiumpke’s palsy
Introduction Brachial plexus injuries can occur in children by various mechanisms. The commonest cause, however, arises as a corn~~~cat~on of childbirth. Upper plexus injuries (Et-h type) are more common than lower ptexus injuries (Klumpke type). The Erb-type injury usuaily occurs due to excessive traction to the shoulder during breech deliveries or to the head irl cephalic presentations when the angle between the neck and shoulder is widened. The Klumpke type can occur after a maneuver which produces traction on the arm when it is in a hyperabducted position. Self-mutilation after deafferentation injuries is rarely reported in adult humans (Aibe-Fessard et al. 1990: Procacci and Maresca 19901. It is, however, seen more frequently in other conditions (Sweet X9Sf; Levitt 1985). The self-mutilation foifowing deafferentation is similar to what is seen in animals which have undergone deafferentation procedures in the laboratory (Basbaum 1974; Albc-Fessard et al. 1979; Wall et al. 1979; Rossitch et al. 1987; Bennett and Xie 1988). This observation in humans provides further validation of this nnimal model of chronic pain.
In this paper we present a l-year-old child with a Klumpke-type brachial plexus injury sustained at birth, who began subsequently to bitt her analgesic digits. Self-mutiI~ti~~n behavior in humans is reviewed and its relevance to current deaffercntati~~n animal mod& is examined.
Case report N.H. the
I?; :I I-year-old
left
hrachial
extracting upper
ww
phy was performed left hrachi;il that
could
was
not
the
begin
exhibited On
There
he
was
not
i
week
Eleutromyogra;\ lesion of the A my&gram.
with
clinically prior
to
dilficulty
P;cralysis 01 the left
birth.
rrxvts most affected.
at
of deltoid
root
avulsions,
indicated
in
this
to her clinic
visit.
the
voluntarily.
there
WLIS no evidence There
and fiaccid.
and the 4th digit
I
year
or
According
emotiomtlly
WB\ no assoctatcd of
age.
there
to the mother, upset
when
ircmtching wils
or wrist on the left, although
function
the vertical
or evening.
irritable
There
in the fingers
not discolored. areflexic
it
morning
to
this behavior.
beyond
elicited
inlet.
after
injury
was
to bite the 4th and Sth digits of her left h;md. The biting
examination
evidence
there
rnen~i~~occ~c~ :zssociated
Approximately
tended
movement
the pelvic
immediately
the lower
since
in the early
child
well
plexus with
child.
II traumatic
Apparently
f I monthsof ageand confirmed
at
performed
occurred
through
noted
have shown
t-year-old
who suhtaincd
at birth.
her shoulders
extremity
child
female
plexus
no
evidence
Although
was some
elbow
lift her arm could
to passive
syndrome
nnd the left
of Homer‘s
not he
extension. Iris 5~:s
in the ieft arm. The rtrm W;~S
The tip of the ieft 5th digit
had zlso been bitten.
Hexion
resistance
was no sensation
of
there W;LScle:u
on that side. The child could
plane.
hhe
behavior.
had been bitten
hut to zi Iesser extent
(Fig.
off.
1f.
Fig. 1. Photograph of deafferentated hand showing self-mutilation of the 4th and 5th digit.
The patient was fitted with a hand splint which stopped the biting behavior.
Disttussion Human self-mutilation has been rarely reported in association with deafferentation states. However, it may be more prevalent in clinical practice than is evident from published reports. For example, this chiid was seen by several physicians a few days prior to visiting our clinic. In spite of the mother showing the mutilated digits to her physicians, the significance of the biting was not appreciated. Albe-Fessard et al. (1990) have discussed 4 adult cases in which seIf-mutilation occurred after deafferentation injury of the brachial plexus. One patient grasped his paralyzed hand and bit it because of a paroxysm of pain. The same patient, when cured of the pain by a dorsal horn lesion, never self-mutilated again. Two other patients wore gloves at night to avoid selfmutiIation. Procacci and Maresca (1990) reported 2 similar adult cases of self-mutilation after brachial plexus avulsions. These patients reported ‘paraesthesic’ sensations in their entire limb, especially the hand, along with a continuous burning pain. At night they bit the nails and fingers of the affected limbs. gearing gloves at night stopped this behavior in both patients. Our patient tended to bite in the evenings or early morning. Similarly, a splint stopped the self-mutilation. Interestingly, human self-mutilation following deafferentation has several features in common with animal autotomy. First, most human adults as well as the child presented here have tended to bite at night or in the evening. Rats have also been observed to bite most often in the early evening as they awaken from sleep (Duckrow and Taub 1977). Second, the biting behavior
was delayed in this child, appearing at 12 months of age. Similarly, monkeys that have undergone deaf’ferentation prior to delivery do not begin to bite for at least 3 months (Berman and Berman 1973). Finally. gloves or splints have been successful in stopping the abnormal behavior in both humans and monkeys 03erman and Berman 1973). Sweet (1981) has stated that self-mutilation is not uncommon in certain young children who are congenitally analgesic. These infants have been observed to typically bit their fingers, lips and tongue. In summarizing the Iiterature, Sweet concluded that these patients do not typically complain of pain. From these case reports, he deduced that the biting was a consequence of analgesia and was not motivated by subjective pain. However, Sternbach (1968) and others (Levine and Gordon 1982; Owens 1984) have discussed the probIems associated with the evatuation of pain in very young children and have given good reasons to cast doubt on Sweet’s deduction. It was unclear how the absence of peripherally induced pain could be a motivating factor for self-mutilation when other sensory and motor functions were preserved. Levitt has stated that, although no strong assertions can be made about the subjective sensations of the preverbaf congenitally analgesic infant, there are reports of non-painful dysesthesias in some of these patients at maturity (Levitt 1985). Moreover, the presence of peripheral sensory neuropathies in some of these cases suggests that congenital anaigesia in human infants resembles the deafferentation syndrome in subhumans (Dyck et al. 1983). The child we have reported showed evidence of irritability during self-mutilation. The irritability could have been secondary to abnormal disturbing sensations in her affected limb. The pattern of expression of the deafferentation syndrome in animals closely follows various aspects of the subjective sensations as reported by patients with deafferentation injuries. There is variability in occurrence, there is a time lag in divelopment, and the syndrome is worsened by stress. Evidence of human self-mutilation following brachial plexus deafferentation injuries further validates this animal model.
References Albe-Fessard, D., Nashold, B.S., Jr., Lombard, M.D., Yamaguchi, Y. and Boureau, F., Rat after dorsal rhizotomy: a possible animal model for chronic pain, Adv. Pain Res. Ther., 3 (1979) 761-766. Albe-Fessard, D., Giamberardino, M.A. and Rampin, O., Comparison of different animal models of chronic pain. Adv. Pain Res. Ther., 13 (1990) 11-27. Basbaum, A.I., Effects of central lesions on disorders by multiple dorsal rhizotomy in rats, Exp. Neural., 42 (1974) 490-501. Bennett, G.J. and Xie, Y., A peripheral mononeuropathy in rat that produces disorders of pain sensation like those seen in man, Pain. 33 (1988) 87-107.
21 I Berman,
A.J. and Berman,
of movement Neural., Duckrow,
38 (1973) R.B.
Exp. Neural..
Dyck, P.J.. Mellinger. i-itchy.
P.C.,
Brimijoin,
thy, Brain, Levine.
J.D.
Levitt,
M.,
J.R.,
multiple
on
dorsal
33-41. Feaiey,
RD.,
E.H..
Not
S.J.. McDonald.
Dysesthesias
mans: a review
N.C..
Go,
V.L.,
Kao,
*indifference
to
sensory and aut(~n~~rni~ neuropa-
Rev.. IO (lSXS, 247-290.
Pain in infancy: conceptual
Pain in prelingual
vocalization,
children
Pain, 14 (1982) in humans
and experimental
and its 8%Y3.
and subhu-
studies, Brain
and methodological
issueh.
213-230. M.. Autotomy
Rossitch, E., Jr.. Oveimen-Levitt. The deafferentation neurotensin
(letter),
Pain. 43 (1990) 394.
J.. Lyeriy, M.A.,
syndrome
and
Sternbach,
R.A.,
Pain,
Press, New York. Sweet,
W.H.,
Animal
tion from human
and self-mutilation
of clinical
M.E..
Pain 20 (IY84)
Nashoid,
B.S.. Jr..
in the rat: effects of intraventricu-
choiecystokinin.
Exp.
Neural..
YX (19x7)
Analysis.
Academic
26>4-275.
106 (19X.7) 373-390. by pain-induced
Owem.
iar
T.J., Horowitz.
and Lambert,
of heredita~
of diphenyihydantoin
by unilateral
54 (1977)
Da&e,
W.S.
and Gordon,
evaluation
effect
J.F., Reagan,
W.J..
pain’ hut varieties
Exp.
Procacci. P. and Maresca.
A., The
in rats produced
J.W..
the ontogenesis
sensory feedback.
170-176
and Taub.
self-mutilation rhizotomy,
D., Fetal deafferentation:
in the absence of peripheral
Res.
genital
an&&a
A Psychophysiological
196X. 1X5 pp. models of chronic experience (Part
1 of
pain: their possible vaiida-
with posterior
rhiz(~t~~rny and con-
the second John J. &mica
Lecture).
Pain. IO (19X1) 275-295. Wall.
P.D.,
Scadding.
and prevention 175-1X2.
J.W. and Tomkiewicz.
of experimental
M.M.,
The production
anesthesia doloresa. Pain, h ( 1979)
i&E
Elsevie;Scicnce
Publishers
B.V.
Ail
rights
reserved
0304-3959/9;?/$OS.Oil
~~l~-rn~t~~ati~~ hollowing brachial plexus injury sustained at birth Eugene
Rossitch,
Sr., W. Jerry Oakes, Janice Uvelmen-Levitt
and Blaine S. Nashold, Jr.
Self-mutilation after deafferentation injuries has been reported only rarely in adult humans. This Summary behavior has been found to be similar to that observed in animals that have been subjected to cxperimcntal deafferentation. We present a child with a brachial plexus injury sustained at birth who began to bite her analgesic digits. Self-mutilation behavior in humans is reviewed and its relevance to current deaff~rentat~on pain animai models is examined. This behavior in humans further validates the current animal model of dcaffcrcntati~~il pain. Key words: Self-mutilation;
Deafferentation;
Brachial plexus avulsion; Infancy; Kiumpke’s palsy
Introduction Brachial plexus injuries can occur in children by various mechanisms. The commonest cause, however, arises as a corn~~~cat~on of childbirth. Upper plexus injuries (Et-h type) are more common than lower ptexus injuries (Klumpke type). The Erb-type injury usuaily occurs due to excessive traction to the shoulder during breech deliveries or to the head irl cephalic presentations when the angle between the neck and shoulder is widened. The Klumpke type can occur after a maneuver which produces traction on the arm when it is in a hyperabducted position. Self-mutilation after deafferentation injuries is rarely reported in adult humans (Aibe-Fessard et al. 1990: Procacci and Maresca 19901. It is, however, seen more frequently in other conditions (Sweet X9Sf; Levitt 1985). The self-mutilation foifowing deafferentation is similar to what is seen in animals which have undergone deafferentation procedures in the laboratory (Basbaum 1974; Albc-Fessard et al. 1979; Wall et al. 1979; Rossitch et al. 1987; Bennett and Xie 1988). This observation in humans provides further validation of this nnimal model of chronic pain.
In this paper we present a l-year-old child with a Klumpke-type brachial plexus injury sustained at birth, who began subsequently to bitt her analgesic digits. Self-mutiI~ti~~n behavior in humans is reviewed and its relevance to current deaffercntati~~n animal mod& is examined.
Case report N.H. the
I?; :I I-year-old
left
hrachial
extracting upper
ww
phy was performed left hrachi;il that
could
was
not
the
begin
exhibited On
There
he
was
not
i
week
Eleutromyogra;\ lesion of the A my&gram.
with
clinically prior
to
dilficulty
P;cralysis 01 the left
birth.
rrxvts most affected.
at
of deltoid
root
avulsions,
indicated
in
this
to her clinic
visit.
the
voluntarily.
there
WLIS no evidence There
and fiaccid.
and the 4th digit
I
year
or
According
emotiomtlly
WB\ no assoctatcd of
age.
there
to the mother, upset
when
ircmtching wils
or wrist on the left, although
function
the vertical
or evening.
irritable
There
in the fingers
not discolored. areflexic
it
morning
to
this behavior.
beyond
elicited
inlet.
after
injury
was
to bite the 4th and Sth digits of her left h;md. The biting
examination
evidence
there
rnen~i~~occ~c~ :zssociated
Approximately
tended
movement
the pelvic
immediately
the lower
since
in the early
child
well
plexus with
child.
II traumatic
Apparently
f I monthsof ageand confirmed
at
performed
occurred
through
noted
have shown
t-year-old
who suhtaincd
at birth.
her shoulders
extremity
child
female
plexus
no
evidence
Although
was some
elbow
lift her arm could
to passive
syndrome
nnd the left
of Homer‘s
not he
extension. Iris 5~:s
in the ieft arm. The rtrm W;~S
The tip of the ieft 5th digit
had zlso been bitten.
Hexion
resistance
was no sensation
of
there W;LScle:u
on that side. The child could
plane.
hhe
behavior.
had been bitten
hut to zi Iesser extent
(Fig.
off.
1f.
Fig. 1. Photograph of deafferentated hand showing self-mutilation of the 4th and 5th digit.
The patient was fitted with a hand splint which stopped the biting behavior.
Disttussion Human self-mutilation has been rarely reported in association with deafferentation states. However, it may be more prevalent in clinical practice than is evident from published reports. For example, this chiid was seen by several physicians a few days prior to visiting our clinic. In spite of the mother showing the mutilated digits to her physicians, the significance of the biting was not appreciated. Albe-Fessard et al. (1990) have discussed 4 adult cases in which seIf-mutilation occurred after deafferentation injury of the brachial plexus. One patient grasped his paralyzed hand and bit it because of a paroxysm of pain. The same patient, when cured of the pain by a dorsal horn lesion, never self-mutilated again. Two other patients wore gloves at night to avoid selfmutiIation. Procacci and Maresca (1990) reported 2 similar adult cases of self-mutilation after brachial plexus avulsions. These patients reported ‘paraesthesic’ sensations in their entire limb, especially the hand, along with a continuous burning pain. At night they bit the nails and fingers of the affected limbs. gearing gloves at night stopped this behavior in both patients. Our patient tended to bite in the evenings or early morning. Similarly, a splint stopped the self-mutilation. Interestingly, human self-mutilation following deafferentation has several features in common with animal autotomy. First, most human adults as well as the child presented here have tended to bite at night or in the evening. Rats have also been observed to bite most often in the early evening as they awaken from sleep (Duckrow and Taub 1977). Second, the biting behavior
was delayed in this child, appearing at 12 months of age. Similarly, monkeys that have undergone deaf’ferentation prior to delivery do not begin to bite for at least 3 months (Berman and Berman 1973). Finally. gloves or splints have been successful in stopping the abnormal behavior in both humans and monkeys 03erman and Berman 1973). Sweet (1981) has stated that self-mutilation is not uncommon in certain young children who are congenitally analgesic. These infants have been observed to typically bit their fingers, lips and tongue. In summarizing the Iiterature, Sweet concluded that these patients do not typically complain of pain. From these case reports, he deduced that the biting was a consequence of analgesia and was not motivated by subjective pain. However, Sternbach (1968) and others (Levine and Gordon 1982; Owens 1984) have discussed the probIems associated with the evatuation of pain in very young children and have given good reasons to cast doubt on Sweet’s deduction. It was unclear how the absence of peripherally induced pain could be a motivating factor for self-mutilation when other sensory and motor functions were preserved. Levitt has stated that, although no strong assertions can be made about the subjective sensations of the preverbaf congenitally analgesic infant, there are reports of non-painful dysesthesias in some of these patients at maturity (Levitt 1985). Moreover, the presence of peripheral sensory neuropathies in some of these cases suggests that congenital anaigesia in human infants resembles the deafferentation syndrome in subhumans (Dyck et al. 1983). The child we have reported showed evidence of irritability during self-mutilation. The irritability could have been secondary to abnormal disturbing sensations in her affected limb. The pattern of expression of the deafferentation syndrome in animals closely follows various aspects of the subjective sensations as reported by patients with deafferentation injuries. There is variability in occurrence, there is a time lag in divelopment, and the syndrome is worsened by stress. Evidence of human self-mutilation following brachial plexus deafferentation injuries further validates this animal model.
References Albe-Fessard, D., Nashold, B.S., Jr., Lombard, M.D., Yamaguchi, Y. and Boureau, F., Rat after dorsal rhizotomy: a possible animal model for chronic pain, Adv. Pain Res. Ther., 3 (1979) 761-766. Albe-Fessard, D., Giamberardino, M.A. and Rampin, O., Comparison of different animal models of chronic pain. Adv. Pain Res. Ther., 13 (1990) 11-27. Basbaum, A.I., Effects of central lesions on disorders by multiple dorsal rhizotomy in rats, Exp. Neural., 42 (1974) 490-501. Bennett, G.J. and Xie, Y., A peripheral mononeuropathy in rat that produces disorders of pain sensation like those seen in man, Pain. 33 (1988) 87-107.
21 I Berman,
A.J. and Berman,
of movement Neural., Duckrow,
38 (1973) R.B.
Exp. Neural..
Dyck, P.J.. Mellinger. i-itchy.
P.C.,
Brimijoin,
thy, Brain, Levine.
J.D.
Levitt,
M.,
J.R.,
multiple
on
dorsal
33-41. Feaiey,
RD.,
E.H..
Not
S.J.. McDonald.
Dysesthesias
mans: a review
N.C..
Go,
V.L.,
Kao,
*indifference
to
sensory and aut(~n~~rni~ neuropa-
Rev.. IO (lSXS, 247-290.
Pain in infancy: conceptual
Pain in prelingual
vocalization,
children
Pain, 14 (1982) in humans
and experimental
and its 8%Y3.
and subhu-
studies, Brain
and methodological
issueh.
213-230. M.. Autotomy
Rossitch, E., Jr.. Oveimen-Levitt. The deafferentation neurotensin
(letter),
Pain. 43 (1990) 394.
J.. Lyeriy, M.A.,
syndrome
and
Sternbach,
R.A.,
Pain,
Press, New York. Sweet,
W.H.,
Animal
tion from human
and self-mutilation
of clinical
M.E..
Pain 20 (IY84)
Nashoid,
B.S.. Jr..
in the rat: effects of intraventricu-
choiecystokinin.
Exp.
Neural..
YX (19x7)
Analysis.
Academic
26>4-275.
106 (19X.7) 373-390. by pain-induced
Owem.
iar
T.J., Horowitz.
and Lambert,
of heredita~
of diphenyihydantoin
by unilateral
54 (1977)
Da&e,
W.S.
and Gordon,
evaluation
effect
J.F., Reagan,
W.J..
pain’ hut varieties
Exp.
Procacci. P. and Maresca.
A., The
in rats produced
J.W..
the ontogenesis
sensory feedback.
170-176
and Taub.
self-mutilation rhizotomy,
D., Fetal deafferentation:
in the absence of peripheral
Res.
genital
an&&a
A Psychophysiological
196X. 1X5 pp. models of chronic experience (Part
1 of
pain: their possible vaiida-
with posterior
rhiz(~t~~rny and con-
the second John J. &mica
Lecture).
Pain. IO (19X1) 275-295. Wall.
P.D.,
Scadding.
and prevention 175-1X2.
J.W. and Tomkiewicz.
of experimental
M.M.,
The production
anesthesia doloresa. Pain, h ( 1979)
