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Descending paralysis resulting from occult wound botulism.

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Descending Paralysis
Resulting from Occult
Wound Botulism
Samuel Rapoport, MD, PhD,* and Paul B. Watkins, MDIS
~
~-
A 30-year-old male drug abuser developed ophthalmoplegia, bulbar paralysis, and limb weakness responsive to edrophonium. However, potentiation of a lowamplitude evoked muscle action potential was produced
with repetitive nerve stimulation at 10 Hz,and the clinical and electrophysiological data suggested the diagnosis
of botulism. T h e source of botulism type B toxin was
a clinically obscure cyst produced by subcutaneous
infiltration of cocaine two weeks prior to the onset of
symptoms. T h e patient improved with chronic administration of pyridostigmine bromide and plasmapheresis.
Wound botulism may be underdiagnosed because of
confusion with inflammatory neuropathy or myasthenia
gravis. Neuromuscular transmission studies in patients
with acute craniosomatic paralysis can prevent such
oversights.
Rapoport S, Watkins PB: Descending paralysis
resulting from occult wound botulism.
Ann Neurol 16:359-361, 1984
Ophthalmoplegia with bulbar paresis and limb muscle
weakness progressing over several days are frequently
occurring manifestations of acute cranial polyneuropathy, myasthenia gravis, and botulism { S ] . In
patients evidencing such clinical symptoms, differentiation among these three disorders is usually straightforward. Myasthenia gravis is typically diagnosed by
a dramatic improvement of the paralysis in response
to administration of acetylcholinesterase inhibitors
[ 4 ];acute polyneuropathy (AP) produces loss of deep
tendon reflexes and, at the peak of the illness, an
elevation in the cerebrospinal fluid (CSF) protein level
with an albuminocytological dissociation [S, 201. Botulism, when food borne, usually occurs in epidemics and
can be diagnosed from a history of associated gastrointestinal symptoms following consumption of the implicated food. When the source of botulism has been
From the Departments of *Neurology and ?Medicine, The New
York Hospital-Cornell University Medical Center, New York, N Y
10021.
$Present address: Department of Gastroenterology,Medical College
of Virginia, Richmond, VA 23005.
Received July 12, 1983, and in revised form Jan 27, 1984. Accepted
for publication Feb 12, 1984.
Address reprint requests to Dr Rapoport, Department of Neurology, The New York Hospital, 525 E 68th St, New York, NY
10021.
a wound, clinical reports suggest that the wound is
readily evident; most have occurred in agricultural
settings {lo, 1I}.
We recently treated a patient who had acute onset of
ophthalmoplegia, bulbar paralysis, and upper extremity
weakness. His symptoms responded dramatically to
acetylcholinesterase inhibitors, and his CSF manifested
a rise in protein without pleocytosis. Nevertheless, the
cause of neurological dysfunction was demonstrated to
be a clinically occult cyst colonized with Clostridia
bottllinurn producing B toxin.
A 30-year-old male drug abuser was well until three days
prior to admission, when he developed diplopia and photophobia. Over the next day he developed dysphonia, dysarthria, dysphagia, and a dry mouth. During the day prior to
admission generalized weakness appeared, most prominently
in the upper extremities. In the emergency room he complained of dyspnea and was ataxic, requiring assistance to
walk. H e had no constitutional symptoms. All food that he
had consumed in the previous week had been shared by
others who were in good health.
H e exhibited a pronounced bilateral ptosis and lowamplitude dysarthric speech but was alert and frightened. His
blood pressure was 130/85 mm Hg, pulse 72 beats per minute, respiratory rate 18 per minute, vital capacity 3 L, and
body temperature 36.2"C. There was a 1.5 x 1.5 cm, subcutaneous cystlike structure, without evidence of inflammation, on the left arm. This abnormality was not prominent and
the patient had no complainrs referable to it, stating that he
had infiltrated cocaine at this site during attempted intravenous injection two weeks earlier.
His mucous membranes were red and dry but without
exudates. Bowel sounds were reduced, and the bladder was
distended. His pupils were dilated and reacted sluggishly to
light and accommodation; he exhibited diminished vertical
eye movements, bilateral palatal weakness, neck flexor weakness, inability to protrude the tongue, and bilateral arm weakness, most marked proximally. Sensation was normal to all
modalities, and deep tendon reflexes were preserved.
Administration of 10 mg of edrophonium intravenously
substantially improved his extraocular movements, grip
strength, and vital capacity. A lumbar puncture revealed 5
mononuclear cells per microliter, a protein level of 17 mg/dl,
and a glucose level of 51 mg/dl. Median nerve conduction
velocity was 55 d s (normal, 50 to 67 d s ) . The F response
latency was 30.3 ms (normal for height, up to 36.0 ms). The
maximal amplitude of the evoked muscle action potential was
only 0.5 mV (normal, 5 to 25 mV). Repetitive stimulation at
10 Hz produced a 30% potentiation of the tenth action potential and a 70% potentiation of the thirtieth action potential.
Based on clinical and electrophysiologicaldata, a presumptive diagnosis of botulism was made. The source remained
obscure. Cultures of the stool and gastric aspirate were assayed for C. botulintlm and were negative. Treatment with
pyridostigmine bromide was instituted. Edrophonium tests
were repeated daily during peak and trough pyridostigmine
effectiveness and confirmed that this therapy was improving
the patient's functional capacity. Despite therapy with pyr-
359
idostigmine the patient's muscle strength and vital capacity
deteriorated progressively. Respiratory support was instituted on the fourth hospital day, and on the fifth hospital
day deep tendon reflexes could not be elicited in the upper
extremities and the left arm was noted to be weaker than the
right.
Plasmapheresis, which had been reported successful in
treating both myasthenia gravis and cranial polyneuritis [ 14,
171, was instituted on the fifth hospital day. Following exchange of 3.5 L of plasma, vital capacity, extraocular movements, and grip strength improved. A total of five plasma
exchanges were performed during the patient's hospital
course.
O n the sixth day 0.2 cc of serosanguineous fluid was aspirated from the still asymptomatic cyst on the left arm.
Anaerobic cultures grew gram positive spore-forming rods
which were subsequently demonstrated to produce C.
botulinum type B. The cyst was surgically excised on the
eighth hospital day and was found to be confined strictly to
the subcutaneous tissues. Intraoperative cultures also grew C.
botulzizum type B. Nine million units per day of penicillin was
administered for one week beginning on hospital day 8. A
repeat lumbar puncture revealed 3 white blood cells per microliter, a protein level of 3 7 mg'dl, and a glucose level of 5 5
mgidl.
The patient's muscular function improved, and he was successfully removed from the respirator on the fifteenth hospital day. Inability to swallow and hypophonia persisted for 8
more days, although the vocal cords appeared normal by
endoscopic examination.
The patient was discharged after 28 days of hospitalization.
During the month following discharge he reported symptoms
of dysphagia and dysphonia that developed and progressed
during the course of the day. These have since abated, and he
is presently in good health and has returned to his usual level
of activity.
Discussion
Although medical dictum has long advocated that botulism be considered in the differential diagnosis of
acute idiopathic polyneuritis [S], most clinicians give
the possibility only fleeting thought. O n a probabilistic
basis their attitude is justified. Most cases of noninfant
botulism are food borne and are associated with gastrointestinal symptoms that commence 2 to 18 hours after
consumption of the contaminated material [121. Of the
known cases of wound botulism, none has been described with paralysis as the initial complaint. Rather,
all patients have suffered a serious wound, and
neurological symptoms developed 4 to 14 days later
1111. The present case illustrates that the source of
botulinus toxin need not be readily apparent to either
physician or patient. Our patient offered no complaints
relative to the cyst, and the cyst appeared so benign
that it might have been overlooked as the source of the
patient's intoxication.
The present case illustrates that the distinction between botulism and cranial AP can be difficult, if not
impossible, to make on the basis of the clinical exami-
nation alone. Both diseases are characterized by descending paralysis with a predominant extraocular muscle weakness. Other cardinal features of AP also occur
in botulism. In an audit of botulism cases reported to
the Center for Disease Control { 121, ataxia occurred in
17%, depressed deep tendon reflexes in 40%, and
paresthesias in 14%. Miosis, with or without pupillomotor paralysis, occurs with equal incidence in both
diseases 17, 191. Autonomic dysfunction, although
more frequent in botulism 1121, is also common in AP.
Sphincter disturbance is present in 2 5 p of patients
with AP [ 131, and tachycardia, orthostatic hypotension, and vasomotor symptoms are s o frequencly seen
in AP [ 1, 31 that they are used in support of the diagnosis [2].
CSF findings cannot be relied on for differentiation.
A normal CSF protein level occurs at presentation in
17%' of cases of AP and rises to peak at the time that
the patient exhibits maximal neurological dysfunction
{ 171. Thus, when the clinical picture overwhelmingly
points to AP, a single normal CSF protein determination is often ignored in assigning a diagnosis. The present case provides an example of a clinical syndrome
compatible with AP in which the CSF protein level was
normal at presentation and rose to more than twice the
initial value during the course of the illness. Had C.
botulznum not been isolated from the cyst, the clostridial colonization could have resolved spontaneously
and the patient would have improved gradually, a
course compatible with AP. The increased incidence of
AP among drug addicts 118 } would have been added to
the potential diagnostic error.
Microbiological techniques are not always useful fur
diagnosing wound botulism. In 32g, of cases wound
botulism toxin has not been detected in the serum and
C. botub'num has not been isolated from the wound. In
these cases a presumptive diagnosis has been made on
the basis of the clinical examination and the presence of
potentiation of the evoked muscle action potential on
neuromuscular transmission studies [ 1 I]. Neuromuscular transmission studies appear to be a more sensitive
test than the mouse toxin neutralization assay in the
diagnosis of wound botulism. Only 20%. of patients
with wound botulism have detectable toxin in their
sera, whereas the neuromuscular transmission studies
were diagnostic in 100% of patients in whom they
were performed [ I 13. Electrodiagnostic tests also reliably distinguish between botulism and AP. Once the
diagnosis of wound botulism has been made, wide surgical excision with debridement of the wound and use
of systemic antibiotics have been recommended [ 153.
The efficacy of botulism antitoxin in wound botulism
has not been established El21. Of 23 patients reported
in the literature with wound botulism who exhibited
neurological symptoms [c), 111, 61% received antitoxin; the course of illness did not appear to differ in
360 Annals of Neurology Vol 16 No 3 September 1984
these patients and those who did not receive antitoxin.
Most cases of food-borne botulism occurring in
epidemics are treated with antitoxin. Occasionally
patients with food-borne botulism do not receive antitoxin and appear not to suffer from their lack of therapy [12f. Antitoxin has been shown to influence favorably only type E botulism [GI, and type E has never
been reported in a case of wound botulism. The administration of antitoxin carries a 21% incidence of serum
sickness [ 161.
The use of plasmapheresis, to our knowledge, has
not been reported previously in botulism. The patient
appeared to benefit: he demonstrated an immediate
increase in vital capacity, mostly after the first exchange, as well as marked improvement in his extraocular movements as recorded with a video recorder
before and after the exchange. It is difficult to assess
the efficacy of plasmapheresis in a single case of a disease whose natural history is to improve. Nevertheless,
the procedure appears to deserve a treatment trial.
References
1. Appenzeller 0, Marshall J: Vasomotor disturbance in LandryGuillain-Barre syndrome. Arch Neurol 9:368-372, 1963
2. Asbury A K Diagnostic considerations in Guillain-Barre syndrome. Ann Neurol 9 (suppl):1-5, 1981
3. Birchfield RI, Shaw CM: Postural hypotension in the GuillainBarre syndrome. Arch Neurol 10:149-157, 1964
4. Brooke MH: A Clinician's View of Neuromuscular Diseases.
Baltimore, Williams 81 Wilkins, 1977, pp 77-78
5. Cherington M: Acute muscle weakness. In Vinken PJ, Bruyn
GW (eds): Handbook of Clinical Neurology, Vol 41. Part 2,
Diseases of Muscle. New York, Elsevier, 1979, pp 287-293
6. Dolman CE: Human botulism in Canada (1919-1973). Can
Med J 110:191-198, 1974
7. Eliazan TS, Spire JP, Andiman RM, et al: Syndrome of acute
idiopathic ophthalmoplegia with ataxia and areflexia. Neurology
(Minneap) 21:281-292, I971
8. Fisher M: An unusual variant of acute idiopathic polyneuritis
(syndrome of ophthalmoplegia, ataxia and areflexia). N Engl J
Med 255:57-65, 1956
9. Fullerton P, Gogna NK, Stoddart R: Wound botulism. Med J
Aust 1:662-663, 1980
10. Gutmann L, Pratt L: Pathophysiologic aspects of wound botulism. Arch Neurol 33:175-179, 1976
11. Hikes DC, Manoli A: Wound botulism. J Trauma 21:68-71,
1982
12 Hughes JM, Blumenthal JR, Merson MH, et al: Clinical features
of type A and B food-borne botulism. Ann Intern Med 95:442445, 1981
13 Kennedy RH, Danielson MA, Mulder DW, Kurland LT: Guillain-Barre syndrome: a 42-year epidemiologic and clinical study.
Mayo Clin Proc 5393-99, 1978
14 Littlewood R, Baiada S: Successful plasmapheresis in the MillerFisher syndrome. Br Med J 282:778, 1981
15 Merson MH, Dowell V R Epidemiologic, clinical and laboratory
aspects of wound botulism. N Engl J Med 289:1005-1010,
1973
16 Merson MH, Hughes JM, Dowell VR, et al Current trends in
botulism in the United Stares JAMA 229 1305-1308, 1974
17 Pinching AJ, Peters DK, Newsome-Davis J Remission of myas-
thenia gravis following plasma exchange. Lancet 2:1373-1376,
1976
18. Smith WR, Wilson AF: Guillain-Barre syndrome in heroin addiction. JAMA 231:1367-1368, 1975
19. Terranova W, Palumbo JW, Breman JG: Ocular findings in botulism type-B. JAMA 241:475-477, 1979
20. Wiederholt WC, Mulder DW, Lambert EH: The LandryGuillain-Barre-Stohl syndrome or polyradiculoneuropathy: historical review, report on 97 patients and present concepts. Mayo
Clin Proc 39:427-449, 1964
Value of Ultrasound for
Identification of Acute
Hemorrhagic Necrosis
of Thalamus and Basal
Ganglia in an Asphyxiated
Term Infant
Katherine L. Kreusser, MD,*
Robert E. Schmidt, MD, PhD,t
Gary D . Shackelford, MD,S
and Joseph J. Volpe, MD"§
~
~
An infant with profound intrapartum and postpartum
asphyxia demonstrated striking bilateral echodensities
in the region of the thalamus and basal ganglia on
ultrasound scan. Neuropathological examination confirmed the presence of symmetrical areas of hemorrhagic
necrosis involving the thalamus, caudate, putamen, and
globus pallidus. The findings demonstrate the value of
ultrasonography in the identification of this variety of
perinatal hypoxic-ischemic injury.
Kreusser KL, Schmidt RE, Shackelford G D ,
Volpe JJ. Value of ultrasound for identification
of acute hemorrhagic necrosis of thalamus and
basal ganglia in an asphyxiated term infant.
Ann Neurol 16.361-363, 1984
A variety of neuropathological disorders have been described in association with perinatal hypoxic-ischemic
encephalopathy [ 2 , 4, 6, 7). The locus of involvement
relates principally to the gestational age of the infant
and to the precise nature, timing, and severity of the
From the Departments of "Pediatrics, *Neurology, t Pathology
(Neuropathology), $Radiology, and §Biological Chemistry, Washington University School of Medicine, St Louis, M O 63110
Received Sept 7, 1983, and in revised form Feb 3, 1984 Accepted
for publication Feb 4, 1984
Address reprint requests to Dr Volpe, St Louis Chddren's Hospital,
PO Box 14871, St Louis, M O 63178
36 1
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