Case 2: Acute Ascending Paralysis in a 4-Year-Old Boy

Benjamin M. Greenberg, MD

Disclosures
In This Article

Differential Diagnosis

Acute inflammatory demyelinating polyradiculoneuropathy (Guillain-Barré syndrome)
Porphyrias
Critical illness neuropathy
Diphtheria
Toxins (arsenic, thallium, organophosphates, lead, ciguatoxin, tetrodotoxin, tick paralysis, buckthorn)
Vasculitis
Inflammatory meningoradiculopathies (Lyme disease, HIV, CMV)

Botulism
Myasthenia gravis

Hypokalemia, hypophosphatemia, hypomagnesemia
Rhabdomyolysis
Polymyositis
Intensive care myopathy

Poliomyelitis
Rabies
Transverse myelitis
Basilar artery thrombosis

  • Acute inflammatory demyelinating polyradiculoneuropathy (Guillain-Barré syndrome)

  • Toxins (arsenic, thallium, organophosphates, lead, ciguatoxin, tetrodotoxin, tick paralysis, buckthorn)

  • Inflammatory meningoradiculopathies (Lyme disease, HIV, CMV)

  • Poliomyelitis

  • Botulism

  • Rabies

We narrowed it down to 2 likely possibilities: Guillain-Barré syndrome or botulism.

Acute inflammatory demyelinating polyradiculoneuropathy (AIDP), or Guillain-Barré syndrome, is clinically defined by weakness (usually ascending), parasthesias, and areflexia. It is commonly associated with a prodromal respiratory or gastrointestinal illness that occurs 1-3 weeks before the cardinal symptoms appear. The CSF demonstrates a normal cell count with an elevated protein (so-called albumino-cytologic dissociation). Electrodiagnostically, patients have conduction block, slowing of conduction velocity, decreased CMAP amplitudes, and other signs of segmental demyelination. However, early on, the CSF and nerve conduction studies may be normal.

Botulism is caused by 1 of 8 toxins secreted by Clostridium botulinum. The toxin inhibits release of acetylcholine via an intracellular enzymatic process (zinc-dependent endoprotease that cleaves the polypeptides responsible for exocytosis). The disease typically occurs after ingesting contaminated canned foods, usually home-processed, primarily canned vegetables, fish, fruits, and condiments (honey).

Botulism typically produces a descending paralysis. Symptoms include blurred vision, dysphagia, dysarthria, ophthalmoplegia, weakness, and areflexia, and can progress to complete paralysis and respiratory arrest. Electrodiagnostic tests reveal reduced CMAP amplitudes and facilitation of CMAP amplitude with repetitive stimulation (but nerve conduction studies may be normal early).

  • Guillain-Barré syndrome

  • Botulism

  • Something else

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