clinical and electrodiagnostic follow up of a case of food borne

clinical and electrodiagnostic follow up of a case of food borne

CASE REPORT
CLINICAL AND ELECTRODIAGNOSTIC FOLLOW UP OF A CASE OF
FOOD BORNE BOTULISM
Nilgün Cengiz1, Hande Türker,1 Meral Kiziltan2
1
2
Department of Neurology, Ondokuzmayıs University School of Medicine, Samsun,Turkey
Department of Neurology, Istanbul University Cerrahpasa School of Medicine Istanbul, Turkey
ABSTRACT
In the following case report, we describe the follow up of electrodiagnostic studies together with the clinical pattern in a 40
year old woman diagnosed as botulinum intoxication caused by home-made canned green beans. She had dyplopia, blurred vision,
weakness and paresthesias in all four extremities, difficulty of swallowing and breathing and bifascial paralysis. Repetetive nerve
stimulation and blink reflex studies were performed during the follow up of the case. Serologic tests couldn’t be performed.
Antitoxin therapy was initialised immediately, recovery of symptoms began in about a month’s time and she was symptom free in
three months. Her electrodiagnostic tests also improved and were found to be normal. We believe that diagnosis of botulinum
intoxication on clinical grounds is far more important than it is supposed because electrodiagnosis may not be always very typical
and serologic tests may not be available. Therefore we believe that initialising the antitoxin therapy is life saving and should be
performed immediately, even if the diagnosis is mostly on clinical grounds.
Keywords: Botulinum intoxication, electrodiagnosis and botulinum intoxication
GIDA ALIMIYLA İLİŞKİLİ BOTULİNUM İNTOKSİKASYONLU BİR
OLGUNUN KİLİNİK VE ELEKTOFİZYOLOJİK İZLEMİ
ÖZET
Bu olgu sunumunda ev yapımı konserve tüketimi sonrasında clostridium botulinum intoksikasyonu gelişen 40 yaşındaki bir
bayan hastaya ait klinik ve elektrofizyolojik izlem rapor edilmektedir. Olguda diplopi, bulanık görme, güçsüzlük ve her dört
ekstremitede parestezilerin yanısıra yutma ve solunum güçlüğü ile bifasyal paralizi gelişmiştir. Olgunun izlemi sırasında ardışık sinir
uyarımı ve göz kırpma refleksi çalışmaları yapılmıştır. Serolojik testlerin yapılamadığı olguda, klinik tablo ve elektrofizyolojik
incelemelerle tanı konmuş ve derhal antitoksin tedavisi başlatılmıştır. Semptomlarda bir ay içinde düzelme başlamış, üç ayda
hastadaki semptomlar ortadan kalkmıştır. Elektrodiyagnostik izlemde de bulguların normale döndüğü izlenmiştir. Bu olguda
elektrodiyagnostik testler tipik sonuçlar göstermemiştir. Olgunun izlemiyle varılan kanı, botulinum intoksikasyonu tanısının daha çok
kliniğe dayandığı durumlarda bile, antitoksin tedavisine derhal başlamanın hayat kurtarıcı olduğu yönündedir.
Anahtar Kelime: Botulinum intoksikasyonu, elektrodiyagnoz ve botulinum intoksikasyonu
5)inadvertent botulism. A clinical pattern of
descending weakness is characteristic of all five
forms. Almost all human cases of botulism are
caused by one of three serotypes (A, B, or E).
Classic and wound botulism were the only two
forms known until the last quarter of this century.
Wound botulism was rare until the past decade. It
is caused by local production of toxin by
Clostridium botulinum after wound infection 3.
Although it is a rare variant of botulism, it is
increasingly being reported in drug users who
inject subcutaneously 4. Infant botulism, first
described in 1976, is now the most frequently
reported form especially in the USA. Food borne
INTRODUCTION
Botulism is a rare but serious and potentially
fatal ilness.The Botulinum intoxication is caused
by the exotoxin of Clostridium Botulinum which
has a generalised effect on the neuromuscular
junction involving both striated and smooth
muscles. Botulinum toxin causes its major effect
by blocking neuromuscular transmission in
autonomic and motor nerve terminals. The types
A,B and E account for human cases 1,2. Since the
discovery of the toxin about 100 years ago, five
clinical forms of botulism have been described: 1)
classic or foodborne botulism; 2) wound botulism;
3) infant botulism; 4) hidden botulism;
Corresponding author: Dr. Hande Türker, Ondokuzmayıs University,
School of Medicine, Department of Neurology, Samsun,Turkey
E-mail: [email protected]
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Marmara Medical Journal 2004;17(1);89-92
Marmara Medical Journal 2004;17(1);89-92
Hande Türker
Clinical and Electrodiagnostic Follow up of A Case of Food Borne Botulism
abolished and she had generalised hypotonia.
Nerve conduction studies of sensory nerves were
in normal limits whereas M amplitudes were quite
low in some of the motor nerves.
Electromyographic studies showed that there was
no voluntary motor activity in fascial muscles
while there was a decline in the recruitment
pattern of extremity muscles. In the repetetive
stimulation of the trapezius muscle which was
performed in the first week, all findings were in
normal limits (Stimulations were performed with
2Hz, 3Hz and 20 Hz frequencies). No blink reflex
was obtained in the first week. Regarding the
story, the neurological signs and electrodiagnostic
tests, a diagnosis of botulinum intoxication was
made and as the clinical signs were progressing
rapidly and in the descending fashion, antitoxin
therapy was initialised and the patient was given
A,B and E type antitoxin each given in 600 000
U/day, I.V and in slow infusion form.
botulism is the most severe and debilitating form,
caused by ingestion of the toxin 2. In our country
many of the reported cases are food borne and
mostly from home canned vegetables because
preparing such food is seen quite often in rural
areas of Turkey 2,5,6. Food-borne butulism may
manifest as an outbreak but there are also single
cases. Onset is within 12-36 hours after ingestion,
signs of gastroenteritis with diarrhea, nausea and
vomiting usually precede muscle weakness which
often is generalised.Ocular and bulbar signs such
as diplopia, ptosis, dysarthria and dysphagia are
common.
Weakness
decends
usually
symmetrically to involve muscles of the trunk,
respiratory system and limbs.Proximal muscles
and upper extremities are usually more prone to
involvement. Autonomic manifestations include
dry mouth, dilated, fixed or poorly reactive pupils,
blurred vision, constipation, ileus and urinary
retention 1,2,3. Identification of the toxin in the
patient’s serum confirms the diagnosis.
Electrodiagnosis is mostly helpful. Nerve
conduction studies show normal amplitude and
latency of sensory action potentials. A small
compound muscle action potential elicited by a
single shock further declines with repetetive
stimulation at a small rate. Repetetive stimulation
at 20-50 Hz is the most spesific test, showing an
incremental response in most patients 7. Single
fiber EMG has shown increased jitter and
blocking and some reduction in fiber density
8,9,10,11,12
. Spesific therapy is the antitoxin therapy
which should be administered early because it is
unlikely to be effective after 3 days of exposure.
In this condition only supportive therapy can be
applied 7.
Follow-up
A week after the antitoxin therapy, there
were no changes in the neurological examination
of the patient.The clinical improvement began
gradually after this first week and in her first
month’s check, her muscle strength was found to
be +4/5, her deep tendon reflexes came back and
she hadn’t ophthalmoplegia any more while light
reflex was intact in both eyes. Her clinical
improvement became more clear in the second
month’s control and her neurological exam was
found to be normal in the third month.
Electrodiagnostic tests were repeated in the
second week and in the third month. In the second
week, the repetetive nerve stimulation test showed
a mild decrement (10 Hz stimulation) while it was
normal in the third month (3 Hz stimulation)
(Fig.1-2 respectively). Blink reflex couldn’t be
obtained in the first two weeks whereas in the
third month its latency was in normal limits with a
slight reduction in the amplitude (Fig.3-4
respectively).
CASE PRESENTATION
A forty year old female patient who had
nausea and vomiting besides blurred vision and
generalised weakness of all four extremities was
admitted to the emergency room. Her complaints
had begun 2 days after she ate some of the home
canned green beans she had prepared before. The
day after the meal she had a fierce nausea and
vomiting and two days later she complained of
having blurred vision, diplopia, weakness and
paresthesias in all extremities, difficulty of
swallowing and breathing. She realised that she
couldn’t shut her eyes properly and that her fascial
muscles were weak. In neurological exam, she
had bilateral ptozis, bilateral total external
ophtalmoplegia, bilateral mydriasis and fascial
diplegia. She also had dysphonia and
dysphagia.She had quadriparesis involving all
extremities by 3/5. All deep tendon reflexes were
Figure I: Repetitive stimulation in the second week
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Marmara Medical Journal 2004;17(1);89-92
Hande Türker
Clinical and Electrodiagnostic Follow up of A Case of Food Borne Botulism
timing and severity of illness 8. Cox N. and Hinkle
R. reported that a high index of suspicion is
important for the diagnosis and prompt treatment
of infant botulism, because this disease can
quickly progress to respiratory failure 14. Unless
SFEMG is used electrodiagnostic studies may not
be helpful and diagnosis on clinical grounds is
more essential than it is believed to be, especially
in sporadic cases. Our patient happened to be one
of these. Signs of internal and external
ophthalmoplegia,
dry
mouth,
descending
paralysis, obstipation with weakness, absence of
fever and lucid sensorium as cardinal symptoms
should always bring botulism to mind 15. We
thought in the same way in our diagnostic
approach and made a clinical diagnosis firstly, the
electrodiagnostic tests supported our clinical
diagnosis partially (Findings of low amplitude
CMAPs in nerve conduction studies and finding
decremental response in the begining are helping
but foundation of no incremental response in
higher frequencies of stimulation is of no help).
The frequency of diagnostic findings in cases of
botulism intoxication seem to be quite different
from each other in case reports published so far.
Some of them claim that serology is more
important while the others stress the importance
of electrodiagnostic studies. In a presentation of a
case of two boys with symptoms of food borne
botulism , the diagnosis was made by serology
(mouse neutralization test), whereas the EMG
showed negative results 15. Graf WD. et
al.suggested that electrodiagnosis was not a
reliable tool as far as their 11-year review of
toxin-confirmed cases had reflected 16. In a report
of seven patients with foodborne botulism, the
clinical picture was characterized by mild
symptoms with a long latency of onset and by
involvement of cranial and upper limb muscles;
only one patient, a child, developed respiratory
failure. Spores of Clostridium botulinum were
found in stools in some but not all cases.
Conventional neurophysiological tests had low
sensitivity; abnormal findings were present only
in the patient with severe clinical involvement, in
whom compound muscle action potentials
(CMAPs) appeared to be reduced .Repetitive
nerve stimulation at a high rate showed
pseudofacilitation and not true posttetanic
facilitation, but single-fiber electromyography
(SFEMG) showed abnormalities of neuromuscular
transmission in every case 9. Clay SA. et al imply
that the EMG pattern of brief, small motor unit
potentials, in the context of the clinical syndrome
may be diagnostic for acute infantile cases 17,
almost alike our case. Graf WD. et al reported a
Figure II: Repetitive stimulation in the third month
Figure III-IV: Blink two weeks and third month respectively
DISCUSSION
Rapid diagnostic approach is very important
in life threatening diseases and botulinum
intoxication is one of these.The diagnosis is a
clinical one, confirmed by electrodiagnostic tests
and by testing stool for the organism, C.
botulinum, or its toxin in serum and feces 13.
Although serologic tests and electrodiagnosis are
important tools in making the diagnosis, serology
may be negative and electrodiagnostic results may
not always be typical. The principal
electrodiagnostic feature, an incremental response
on high rates of repetitive nerve stimulation, has
variable sensitivity and may not always be useful
as a diagnostic test given the vagaries of test
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Marmara Medical Journal 2004;17(1);89-92
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Clinical and Electrodiagnostic Follow up of A Case of Food Borne Botulism
3)
case where results of electrodiagnosis were
negative but enema effluent contained adequate
concentrations of organism and toxin to confirm
the diagnosis 16. L. Mulleague et al. wrote that the
diagnosis of botulism was based on clinical
findings, but EMG remained the most useful
discriminatory investigation. They also implied
that the diagnosis should be confirmed by toxin
bioassay, although occasional false-negatives can
occur 18. Early diagnosis and associated therapy
overcome the necessity of intubation and
prolonged intensive care 19. Although the
diagnostic approches may show slight variaties it
is certain that the important point is rapid
diagnosis and therapy in botulinum intoxication.
Our case confirms this very clearly. In this case
report we also underline the importance of clinical
and electrophysiological follow up in such
patients thus the clinical diagnosis is confirmed
and it is important supplying information of
prognosis and patterns of healing. We performed a
follow up of our patient up to 3 month’s time both
clinically and electrodiagnostically. Here we
performed a blink reflex study as well as the
repetetive test. Although we hadn’t had an
increment but a decrement pattern at the
beginning, our follow up study showed a normal
repetetive test finally and although the blink reflex
couldn’t be obtained at the beginning, the follow
up test showed a normal response. We also
followed up a pattern of healing which quite
overlapped the electrophysiological improvement.
We emphasize the importance of clinical
diagnosis and that making it rapidly is life saving.
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