Annals of Internal Medicine
CLINICAL CASES
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doi/10.7326/aimcc.2023.0634
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Publication date: 21 May 2024
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Corresponding Author
Alexander Chiang, BS; Alameda Highlands
Hospital, 1411 E 31st St, Oakland, CA
94602; e-mail, achiang4241@gmail.com.
How to Cite
Chiang A, Cho S, Seng P, et al. A case of
atypical intestinal botulism with rapid
recovery after delayed antitoxin
administration. AIM Clinical Cases.
2024;3:e230634.
doi:10.7326/aimcc.2023.0634
1/3
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CASE REPORT
A Case of Atypical Intestinal Botulism With Rapid Recovery After
Delayed Antitoxin Administration
Alexander Chiang, BS1 ; Shawn Cho, BS1 ; Panhaneath Seng, BA2 ; and Shireen Mirza, MD3
1 Medical student, California Northstate University, School of Medicine, Elk Grove, California
2 University of California Davis, School of Medicine, Sacramento, California
3 Kaiser Permanente Northern California, Harbor City, California
Keywords
Botulism, Antitoxins, Respiratory failure, Paralysis, Ptosis, Myasthenia gravis, Ingestion, Food botulism,
Wound botulism, Ice pack test, Botulinum antitoxin
Abstract
Botulism classically manifests with descending flaccid paralysis progressing to possible
respiratory failure. Atypical presentations often have symptomatic overlap with other
neuromuscular junction pathologies, making it difficult to diagnose and treat quickly. Here we
present the case of a 31-year-old woman hospitalized 6 days after elective abdominoplasty
for dysphagia, bilateral ptosis, and upper extremity weakness who, despite delayed initiation
of antitoxin therapy on day 14, showed rapid muscle tone recovery within 48 hours of
administration. This case discusses the variable manifestation of botulism which leads to
difficulty in diagnosis, and emphasizes the possibility of rapid recovery and even paralysis
reversal despite delayed antitoxin administration.
Background
Roughly 110 cases of botulism are reported annually in the United States, mostly in western
states, particularly California. Approximately 70% involve infant botulism, while the other 30%
are foodborne and wound botulism cases (1). Men and women are affected equally, except in
cases of wound botulism where the majority of cases occur in women. Patient presentation
typically involves symmetrical, descending flaccid paralysis of motor and autonomic nerves
(2, 3). This can initially present as diplopia, ptosis, slurred speech, and dysphagia. Foodborne
botulism may also be preceded by gastrointestinal symptoms such as nausea, vomiting,
abdominal pain, and diarrhea. If left untreated, this may lead to diffuse muscle weakness
and respiratory failure. Botulism is initially diagnosed based on clinical presentation, and
immediate treatment before confirmatory laboratory results is recommended to prevent
further symptom progression. Differential diagnosis includes polyradiculopathies (Guillain-Barré
and Miller Fisher syndromes), myasthenia gravis, Lambert-Eaton myasthenic syndrome, and
central nervous system diseases (3). Improvements in medical care (mechanical ventilation) and
further development of the botulinum antitoxin have significantly reduced mortality rates from
approximately 50% in the past to 5% (4).
Objective
We hope to contribute to the larger body of case reports shedding light on the sometimes
variable manifestation of botulism and how this can lead to difficulties in diagnosis. In
addition, we hope to highlight the possibility of rapid recovery in delayed botulinum antitoxin
administration as an area of future investigation.
Case Report
We discuss the case of a 31-year-old woman who presented to the emergency department
with acute throat discomfort, diplopia, and dizziness. Medical history was notable for gastric
bypass surgery several years ago, cosmetic forehead onabotulinumtoxinA injections every 4 to
6 months, and an elective abdominoplasty 6 days before admission. Otherwise, her medical
history was unremarkable for any prior health conditions. She also reported eating mushroom
derivatives to supplement her health. Upon physical examination, the patient was noted to
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Atypical Intestinal Botulism With Rapid Recovery After Delayed Antitoxin Administration ... Chiang et al.
have bilateral ptosis with nystagmus, slurred speech,
1+ strength in the upper extremities bilaterally, and weak
to absent reflexes in upper extremities bilaterally. Cerebral
spinal fluid analysis showed no elevation in leukocyte count
or albuminocytologic dissociation, and an ice pack test
for myasthenia gravis was deemed positive. Miller-Fisher
syndrome and myasthenia gravis were still suspected at the
time; therefore, steroid therapy with pyridostigmine and
intravenous immunoglobulin therapy were initiated while
serology results were pending. Despite initial treatment, the
patient’s course progressed to respiratory failure requiring
mechanical ventilation.
On day 13 of her admission, the patient began reporting
abdominal pain and was febrile for multiple days with no known
source. Abdominal computed tomography without contrast
revealed necrotic bowel, and the patient had an emergency
exploratory laparotomy. This revealed a necrotic right ascending
colon requiring right hemicolectomy and end ileostomy from
the cecum to hepatic flexure. Meanwhile, the patient’s anti-
acetylcholinesterase antibody test result was negative, at which
point botulism was considered. Antitoxin was administered
on day 14 with suspension of immunosuppression. Over the
next 48 hours, our patient exhibited significant improvement
in respiratory failure and overall muscle tone and strength. A
stool toxin assay on day 16 confirmed the diagnosis of botulism.
Eventually, the patient was discharged to a long-term acute care
facility and was decannulated postadmission day 50.
Discussion
Our patient has several possible botulism exposures. Typically,
foodborne botulism (or toxicoinfection) is due to home-canned
products or vegetable produce outbreaks listed by the Center
for Disease Control. One possible source of toxicoinfection
was the patient’s use of mushroom nutrition supplements (5).
However, toxicoinfection is less likely since the patient had no
prior gut health conditions (Crohn disease, ulcerative colitis, and
so forth) and there is a low incidence of foodborne botulism in
adults versus infants. Another possible exposure was her recent
abdominoplasty and cosmetic forehead onabotulinumtoxinA
injections, as some case reports have demonstrated rare surgical
tool contamination with botulinum spores despite proper
autoclave (6). If this were the case, our patient would be one
of the few cases of wound botulism documented in the United
States annually (7, 8). It is also important to note that the
patient’s surgery included the administration of prophylactic
antibiotics. This perturbation of her typical gut flora could have
enabled a level of dysbiosis capable of potentially enabling
rare spore germination (9). Given these considerations, we are
still uncertain which source is the ultimate cause. However,
it is still possible to reach the conclusion that our patient’s
rare combination of risk factors and recent surgery led to her
becoming particularly susceptible to spore germination from
either possible source of botulinum spores.
Our patient also had a delayed diagnosis due to her atypical
presentation and findings. Typically, foodborne botulism
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Annals of Internal Medicine: Clinical Cases e230634 (2024)
manifests 12 to 72 hours after ingestion with gastrointestinal
symptoms followed by neurologic dysfunction (9). In contrast,
wound botulism often manifests between 5 and 15 days after
ingestion with fever and inflammation before neurologic or
gastrointestinal dysfunction (10). Neither of these patterns,
however, directly match our patient’s presentation with
12 to 24 hours of bilateral cranial nerve palsies, respiratory
failure, and necrotic bowel. Additionally, physical examination
findings overlapped with disorders such as myasthenia gravis.
An “ice pack test,” consisting of placing an ice pack over a
patient’s eyes for 2 to 5 minutes with significant improvement
in ptosis, is typically considered positive and relatively specific
to myasthenia gravis (11). Several case reports, however, have
noted a positive ice pack test in botulism patients. This may be
due to the cooling effect, which increases acetylcholine and can
cause transient improvement in neuromuscular junction deficits
of several causes (12).
Despite delayed diagnosis, our patient had a remarkable
recovery almost immediately after receiving antitoxin. The
mainstay treatment of botulism, heptavalent botulinum
antitoxin, is composed of fragment antigen-binding
immunoglobulins F(ab) and F(ab’)2 that are active against
all botulinum toxin serotypes A–G (13). The amount of toxin
ingested and toxin serotypes both affect symptom duration and
recovery. Because the toxin inhibits soluble N-ethylmaleimide–
sensitive factor attachment protein receptor (SNARE) proteins
and impairs acetylcholine release, regeneration of SNARE is
required for reversal of symptoms and resumption of normal
acetylcholine release (10). Timely administration decreases
the risk of illness and death since antitoxins bind free-floating
molecules of toxin, allowing metabolization and excretion
of the remainder of the botulinum spores while SNARE
proteins regenerate. However, our current understanding is
that antitoxins only bind free toxin in blood to halt symptom
progression and cannot reverse paralysis (13).
Given this background, it is remarkable that our patient showed
such significant muscle strength improvement within 48 hours of
administration. Given the delay in administration, we would
typically expect a minimal response and a halt in symptom
progression rather than paralysis reversal (13). This response
could be related to ingestion of a smaller amount of spores,
or perhaps a serotype that had a much faster resolution of
symptoms (10). Unfortunately, our particular assay confirmed
the botulinum toxin but was unable to delineate serotype. While
the reason for our patient’s remarkable recovery is unclear, our
patient demonstrates that it may be beneficial to administer
antitoxin even incases of prolonged time to diagnosis.
Overall, our patient’s case demonstrates how botulism can be
initially misdiagnosed because of variable symptom onset and
rarity of incidence. Although the exact origin of toxemia in our
patient is unclear, her progression from respiratory failure to
necrotic bowel, and causes of delayed diagnosis are important
to note for recognition of future clinical cases. Furthermore, her
case may lead to further discussions in the future about certain
Atypical Intestinal Botulism With Rapid Recovery After Delayed Antitoxin Administration ... Chiang et al.
lifestyle exposures, diets, and surgical prophylactic antibiotic
usage.
In addition, our patient had an evident and rapid recovery
despite delayed antitoxin administration. This highlights the
efficacy of antitoxin even in atypical scenarios. Hopefully, this
case will help clinicians better understand the variability in
presentation and clinical management of botulism.
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