toxin related paralysis including botulism and GBS

This patient’s illness is consistent with a foodborne process that is followed by oculobulbar findings and a flaccid descending paralysis. An important consideration with such a clinical scenario is botulism, which is mediated by a neurotoxin that can be produced when food contaminated with Clostridium botulinum has germination of the botulinum spores. Such an illness can occur in a setting in which food has not been refrigerated for a prolonged period, as occurred with this patient. After the classic GI symptom of vomiting, patients with botulism may develop dysfunction of the ocular cranial nerves, with cranial nerve 3 involvement (most often in the form of accommodative paresis, ptosis, ophthalmoparesis, and dilated pupils) and cranial nerve 6 palsy being most common. Bulbar palsy, which denotes the impaired function of cranial nerves 9, 10, 11, and 12, may manifest as dysarthria, dysphagia, and dysphonia, all of which this patient had. The neuro-ophthalmic signs in botulism are usually bilateral. The descending paralysis that this patient developed had the classic tempo of rapidly progressing after the development of the oculobulbar findings. When botulism is suspected, guidance from the Centers for Disease Control and Prevention is to contact the local or state health department’s emergency on-call staff immediately to arrange emergency expert clinical consultation because laboratory confirmation can be performed only by certain municipal and state public health laboratories and by the Centers for Disease Control and Prevention’s National Botulism Laboratory. Botulinum antitoxin is the only specific therapy for botulism and should be administered as quickly as possible once the diagnosis is suspected because laboratory confirmation may take days.

Even though this patient is at risk for carbon monoxide poisoning because of the generator he was using in his home, he does not have the headache or encephalopathy that are usually present. There has been no history to suggest depression of consciousness. With carbon monoxide poisoning, oculobulbar and proximal muscle weakness would be very unlikely early findings.

In contrast to botulism, Guillain-Barré syndrome is an ascending form of paralysis that occurs on the basis of demyelination. Areflexia is highly characteristic of demyelinating processes, but this patient has present, although diminished, reflexes. Pupillary abnormalities are extremely uncommon in patients with Guillain-Barré syndrome. Often occurring as the immunologic sequela of a diarrheal illness caused by Campylobacter jejuni, Guillain-Barré syndrome tends to follow the GI illness by weeks, not by hours or days as occurred in this patient. Because of demyelination, Guillain-Barré syndrome tends to be painful, but this patient had no pain.

Lambert-Eaton myasthenic syndrome is a neuromuscular junction disorder that may occur on the basis of either a paraneoplastic phenomenon or as a primary autoimmune disorder. Reviews have stated that more than half of the cases are associated with small cell lung cancer, which this patient had and for which he was receiving treatment. The primary clinical manifestation is proximal muscle weakness. Deep tendon reflexes are usually absent, and there is often autonomic dysfunction. The tempo of the muscle weakness in Lambert-Eaton is usually not as progressive and nonfluctuating as in this patient’s findings.12345

A 56-year-old man presents with blurred and double vision. Two days before presentation, the patient developed vomiting and diarrhea, followed by difficulty swallowing. One day before presentation, he developed weakness when trying to lift things and, over the last 1 to 2 h, when trying to ambulate. He reports no pain or other sensory symptoms.

For the past 9 days, he has been living in his home that was flooded and lost power after a Category 5 hurricane. Three days before the onset of any of his symptoms, he started using a home generator to supply light and to power a fan in one of the rooms of his home, but his food has not been refrigerated. His medical history is significant for small cell lung cancer diagnosed 3 months earlier and presently being treated. On physical examination, the patient is afebrile with normal BP and pulse. His mental status is normal. There is bilateral ptosis and inability to abduct either eye past the midline. His pupils are symmetric, mildly dilated, and sluggishly reactive to light and accommodation. His voice has a nasal tone when he speaks, and there is some difficulty in articulating words. His gag reflex is diminished. There is 2 of 5 strength in the patient’s ability to lift his arms and legs upward against resistance. Deep tendon reflexes are symmetric but slightly diminished. Sensory examination results are normal.

On the basis of this presentation, what is the most likely diagnosis?

Footnotes

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  2. Bilusic M, Pattathil J, Brescia M, et al. Recurrent bulbar paralysis caused by botulinum toxin type B. Clin Infect Dis. 2008;46(8):e72-e74. PubMed ↩

  3. Chatham-Stephens K, Fleck-Derderian S, Johnson SD, et al. Clinical features of foodborne and wound botulism: a systematic review of the literature, 1932-2015. Clin Infect Dis. 2017;66(suppl_1):S11-S16. PubMed ↩

  4. Dembek ZF, Smith LA, Rusnak JM. Botulism: cause, effects, diagnosis, clinical and laboratory identification, and treatment modalities. Disaster Med Public Health Prep. 2007;1(2):122-134. PubMed ↩

  5. Rao AK, Sobel J, Chatham-Stephens K, et al. Clinical guidelines for diagnosis and treatment of botulism, 2021. MMWR Recomm Rep. 2021;70(2):1-30. PubMed ↩