A Case of Localized, Unilateral (Cephalic) Wound Botulism
A Case of Localized, Unilateral (Cephalic) Wound Botulism
Kara Jacobs Slifka 1 2
Jo Ann Harris 0
Von Nguyen 1 2
Carolina Luquez 1
Tejpratap Tiwari 3
Agam K. Rao 1
0 Stormont-Vail Regional Health , Topeka, Kansas , USA
1 Division of Foodborne , Waterborne, and Environmental Diseases , Centers for Disease Control and Prevention , Atlanta , Georgia
2 Epidemic Intelligence Service
3 Division of Bacterial Diseases, National Center for Immunization and Respiratory Diseases, Centers for Disease Control and Prevention , Atlanta , Georgia
We describe a rare presentation of botulism originally presenting with exclusively unilateral cranial nerve deficits following a puncture wound to the face. Cephalic tetanus was initially suspected but laboratory testing confirmed botulism. Botulism caused by local diffusion of toxin from a contaminated head wound can be confused with cephalic tetanus.
Wound botulism and cephalic tetanus are both potentially fatal,
toxin-mediated neurological illnesses. They require early
clinical diagnosis and rapid treatment. Wound botulism occurs
when Clostridium botulinum germinates in the anaerobic
milieu of a wound and produces botulinum toxin, which enters the
blood circulation and exerts its effect systemically [
is classically described as an acute-onset illness beginning with
bilateral symmetric cranial nerve (CN) palsies, which may
progress to descending flaccid paralysis [
]. Dysphagia, facial
paralysis, ptosis, dysarthria, diplopia, and impaired gag reflex
are among the manifestations of CN palsies that can be seen in
patients with botulism. Rarely, asymmetric and even some
unilateral CN palsies have been reported [
]. Symptoms result
from blockade of voluntary motor and autonomic cholinergic
junctions by botulinum toxin [
Tetanus, a vaccine-preventable disease, is caused by wound
colonization with Clostridium tetani and production of
tetanus toxins that closely resemble botulinum toxin in amino acid
sequence. Tetanus toxin acts at the myoneuronal junction of
skeletal muscle and, eventually, the central nervous system, where
it blocks inhibitory impulses, resulting in characteristic spastic
paralysis of generalized and localized tetanus [
tetanus is a rare form of tetanus resulting from a head wound,
and may cause, in contrast to generalized tetanus, flaccid palsies
of CNs VII, III, IV, VI, and XII, usually presenting in a unilateral
fashion. The exact pathophysiology of the flaccid paralysis is not
fully understood . Trismus (lockjaw) usually precedes facial
paralysis and dysphagia; dysarthria and nystagmus may also be
present. Progression to generalized tetanus occurs in about
twothirds of cases and can result in respiratory compromise [
Cases of botulism are confirmed by specialized laboratory
testing in reference laboratories. Wound botulism is confirmed
by detection of botulinum toxin in serum, or isolation of
C. botulinum from a wound. Confirmatory testing for tetanus
is uncommon; C. tetani yield on culture [
] and real-time
polymerase chain reaction (PCR) assays for detection of C. tetani
neurotoxin gene fragments are not widely available [
A 14-year-old boy playing stickball was wounded by a
broomstick splinter that penetrated his right maxillary area under the
eye. The boy experienced no fever, trauma to the right eye, vision
changes, or drainage. Three days later, he visited his
pediatrician, was diagnosed with cellulitis at the puncture site, and was
prescribed oral cefadroxil. After 2 days, the boy complained of
new-onset double vision, right-sided facial droop, and decreased
sensation. Within 2 days, he had difficulty swallowing solids and
his parents observed worsening facial swelling and difficulty
speaking. A computed tomographic scan of the head showed a
3-cm foreign body deeply embedded in the right
infratemporal fossa. Ten days after the injury, an otolaryngologist noted
trismus, dysphagia, dysarthria, diplopia, and right-sided facial
weakness; he was admitted for surgical removal of the splinter.
On admission, the patient was afebrile. Examination showed
diffuse cellulitis surrounding the right eye and a pinpoint
puncture wound above the right malar eminence. Neurologic
examination demonstrated unilateral, right-sided deficits including
ptosis, decreased sensation in the area of swelling, weakness
in the distribution of the facial nerve (CN VII), and
impairment of lateral, superior, and inferior gaze consistent with
CN III and VI palsies (Figure 1). Moderate trismus was again
noted. Immunizations were up-to-date, including the 5-dose
childhood tetanus toxoid series and a booster dose of a tetanus
toxoid-containing vaccine 1 year before illness [
]. No sick
contacts, injection drug use, or home-canned food
consumption were reported.
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White blood cell count and lumbar puncture results were
within normal limits. Ertapenem and vancomycin were initiated
and the foreign body was removed in the operating room on
hospital day (HD) 1; purulent material and the extracted splinter were
cultured. Cephalic tetanus was the leading diagnosis based on
unilateral CN impairments and trismus. Human tetanus immune
globulin (500 units) was administered on HD2, HD4, and HD6.
Electrodiagnostics performed on HD5 revealed unilateral
findings; there was involvement of the right facial motor nerve with
relative sparing of the left facial nerve and markedly decreased
motor unit potentials on the right. Over the next few days, the
patient developed worsening dysphagia. On HD7, the patient
experienced severe respiratory distress and was transferred to
the pediatric intensive care unit. A tracheostomy was performed
to facilitate removal of respiratory secretions. By this day, the
patient’s truncal tone was decreased and he was unable to extend
his neck. For the first time on HD7, 17 days after the injury,
physicians suspected bilateral CN XII impairment demonstrated by
poor tongue mobility and CN XI impairment demonstrated by
weakness of the right sternocleidomastoid muscle. They observed
subtle left-sided ptosis on HD8 and loss of the left nasolabial fold
on HD9. The patient’s extremities were not hyperreflexic.
On HD7, cultures of 2 specimens obtained during surgery
(a specimen obtained from a pocket of pus surrounding the
wound and the broom splinter) grew a Clostridium species
preliminarily identified as Clostridium species non-perfringens.
Treating physicians, who maintained suspicion that C.
botulinum might have been the causative agent, sent serum and the
2 Clostridium cultures to the Centers for Disease Control and
Prevention (CDC). CDC tested the serum specimen by mouse
bioassay and the 2 cultures by botulinum neurotoxin gene
realtime PCR and enzyme-linked immunosorbent assay. The serum
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Figure 2. Unilateral right-sided paralysis with inability to close the right eye due
to tetanus toxin from wound on right forehead. Facial paralysis in the distribution of
cranial nerve VII resulted in the inability to raise the right oral commissure and
inability to close the right eye (Dan Higgins, illustrator). This artist rendition is modeled
after a photograph depicting a patient with cephalic tetanus.
specimen was negative for botulinum toxin, and the 2 cultures
were positive for C. botulinum type A. These results were
available 27 days after illness onset but by HD12, right-sided
frontalis muscle function and extraocular muscle movements were
improved, the patient was able to lift his head from the bed, and
he was transferred from the hospital to a rehabilitation
facility. Botulinum antitoxin was not indicated because of clinical
The boy’s illness began differently from the classically described
appearance of botulism; all signs were unilateral and the
trismus and CN deficits after a head wound were features
consistent with cephalic tetanus (Figure 1). Risus sardonicus, which
occurs with generalized tetanus, was absent; however, not all
cases of cephalic tetanus progress to generalized tetanus, so this
absence was not considered unusual. Continuous consideration
of alternative diagnoses by the clinical team led to renewed
suspicion for botulism, and laboratory testing confirmed it.
All naturally occurring botulism syndromes are attributed to
the systemic action of botulinum toxin that enters circulation to
produce the classically described bilateral and symmetric CN
palsies and flaccid paralysis of extremities [
]. Seven (7%) patients
with botulism due to toxin type A in the United States during
2010–2013 had completely unilateral CN deficits on HD1, but
all became bilateral within 2 days (CDC, unpublished data). The
presence of a mix of unilateral and bilateral CN deficits has been
reported rarely but, to our knowledge, we here describe the first
confirmed case of botulism that became bilateral after several days
of completely unilateral CN palsies. We speculate that the
patient experienced localized paralysis resulting from slow diffusion
of botulinum toxin from the injury site into facial, orbital,
pharyngeal, and subsequently, cervical tissues—similar to the
localized diffusion of botulinum toxin that occurs with cosmetic or
therapeutic injection. The late-course postural manifestations of
sternocleidomastoid and possibly trapezius weakness, along with
mild contralateral CN deficits and respiratory symptoms, might
have been due to slow diffusion of toxin from the postoperative
wound site along fascial planes, or to toxin diffusion into
circulation. While sensory deficits have sometimes been observed with
], in this case, these may be due to pressure on
local sensory nerves from edema around the embedded splinter.
The similarity in presentation of cephalic tetanus and botulism
in a patient with a head or neck wound is illustrated in a
previous report of a patient with atypical tetanus that was initially
treated as wound botulism [
]. In that case, illness presented
with flaccid paralysis and ptosis but progressed to generalized
tetanus and, similar to our case, occurred in an adolescent
immunized against tetanus. Typically, CN VII palsies are reported with
cephalic tetanus; involvement of multiple CNs has been reported
]. Initial involvement of CNs III, IV, and VI are more
commonly reported in botulism, which can involve other CNs . As
seen in Figure 1, the patient in our case had right facial paralysis
with ptosis and loss of the nasolabial fold. In comparison, the
patient in Figure 2, a 5-year-old boy with cephalic tetanus, had right
facial paralysis and inability to close the right eye. The similarity
of their appearances might be explained by a similar mechanism
underlying cephalic tetanus and botulism. The botulism patient
has multiple CN palsies including III and VI, which can occur
with cephalic tetanus but is more common in botulism [
During 2005–2014, 94% (207/220 cases) of wound botulism
cases in the United States were due to limb wounds in
intravenous drug abusers; 6% were due to wounds of other types [
The trauma-induced wound botulism described in this report
is uncommon and resembles in some ways iatrogenic botulism
from botulinum toxin injections. Licensed commercial
preparations of botulinum toxins are increasingly injected for cosmetic
or therapeutic purposes (eg, ablation of facial wrinkles and
cervical dystonia relief). They produce limited unilateral paralysis
of a target muscle through localized diffusion of the injected
toxin and might present with clinical features similar to those
in this case if diffusion of toxin occurs beyond the intended
muscle. Rarely, systemic effects of injected toxin including
bilateral CN deficits and paralysis have been described, presumably
due to entry of toxin from high-dose injections into circulation.
Whether the initial presentation was unilateral CN deficits is
To our knowledge, this is the first reported case of botulism
initially presenting with persistent, completely unilateral CN
palsies. However, 17 days after the injury the CN findings
became bilateral. We attribute this presentation to local,
ipsilateral diffusion of botulinum toxin from a contaminated facial
wound. Given the expanded use of botulinum toxin injections
for therapeutic purposes, it is possible that treatment-related
cases similar to this case may occur. Botulinum toxin should
be considered in addition to cephalic tetanus in a patient with
unilateral CN palsies and a head wound or localized botulinum
Acknowledgments. We thank Dr Clifton Jones, Dr Daniel Katz, Dr
Babak Marefat, and Dr Casey Cordts of Pediatric Hospitalists, Pediatric
Intensive Care Team of Stormont Vail Health; Dr Anna Acosta of Division
of Bacterial Diseases, National Center for Immunizations and Respiratory
Diseases, Centers for Disease Control and Prevention (CDC); Dr James
Sejvar of Division of High-Consequence Pathogens and Pathology, National
Center for Emerging and Zoonotic Infectious Diseases, CDC; Dr Jeremy
Sobel of Division of Foodborne, Waterborne, and Environmental Diseases,
National Center for Emerging and Zoonotic Infectious Diseases, CDC; and
Dan Higgins, CDC illustrator.
Disclaimer. The findings and conclusions in this report are those of the
authors and do not necessarily represent the official position of the CDC.
Financial support. The findings and conclusions in this presentation
are those of the author(s) and do not necessarily represent the official
position of the CDC.
Supplement sponsorship. This article appears as part of the supplement
“Botulism,” sponsored by the Centers for Disease Control and Prevention.
Potential conflicts of interest. All authors: No reported conflicts of
interest. All authors have submitted the ICMJE Form for Disclosure of
Potential Conflicts of Interest. Conflicts that the editors consider relevant to
the content of the manuscript have been disclosed.
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