INFECTIOUS DISORDERS WITH DYSPHAGIA & DYSARTHRIA AS DOMINANT FEATURES
Saturday, August 16, 2008
Introduction
Involvement of cranial nerves nine through twelve is less frequent in meningeal infections, perhaps because their dysfunctions are less obvious, and they are less carefully examined. Involvement of their nuclei in the brainstem is, however, common in polio, leading to severe dysphagia, dysarthria, and respiratory difficulties. Thus, polio must be considered in unvaccinated individuals who have traveled abroad.
Botulism & Pseudobotulism
Although botulism is a disorder of the neuromuscular junction rather than peripheral nerves, patients with botulism commonly present with dry mouth, dysarthria, dysphagia, diplopia, and impaired ocular accommodation. Rapid recognition of this disease is important. Prompt administration of antitoxin along with ventilatory support is essential in the management of botulism. In addition, recognition of the disease should prompt an intensive search for contaminated foodstuffs, which are most often the cause of the condition in adults. Victims who have only early symptoms may be recognized only because they were identified as sharing certain foodstuffs with individuals who have clear-cut disease.
A cluster of signs and symptoms similar to those in botulism may occur after ingestion of any part of the plant, Jimson weed (Datura stramonium); the syndrome may mimic botulism in patients who present with dysphagia; dilated, fixed pupils; and dry mouth (pseudobotulism), which is often accompanied by visual or auditory hallucinations. The Datura plant contains the anticholinergics atropine and scopolamine, which are highly concentrated in the seeds and can cause serious illness or death. Teenagers seeking mind-altering experiences sometimes experiment with Datura, only to die, probably from the cardiotoxic principle in the plant. They usually present with the pseudobotulism syndrome (fixed, dilated pupils and dry mouth), which is distinguishable from true botulism primarily by the history of contact with the Datura plant or its seeds and by the hallucinations, which are not a feature of true botulism. If the cardiovascular system is stable, the patients usually improve over time without therapy, other than emptying the stomach, using activated charcoal, or both; however, if the patients manifest profound toxicity, including bradycardia or tachycardia, the use of the antidote physostigmine should be considered.
Datura poisoning might also suggest rabies to the initial observer, if the hallucinations are mistaken for encephalopathy and if the dysphagia is mistaken for hydrophobia.
Rabies
Rabies should also be considered in the differential diagnosis of patients presenting with dysphagia and dysarthria, with or without hydrophobia and laryngeal spasms. Rabies must be considered in any patient who has traveled to third-world countries and who has had any contact with potentially infected animals. Skunks, raccoons, and bats are the main animal reservoirs of rabies in the United States.
Treatment with human immune globulin and diploid cell-derived vaccine is very effective for individuals exposed to or bitten by rabid animals, but it is ineffective once neurological symptoms have developed. It is important for persons in close contact with the victims, whose body fluids may be infectious or for others who have had contact with the same or other infected animals to receive postexposure prophylaxis. A detailed review of the indications and procedures for postexposure prophylaxis has been provided by the Centers for Disease Control and Prevention (CDC/MMWR, 1999).
Involvement of cranial nerves nine through twelve is less frequent in meningeal infections, perhaps because their dysfunctions are less obvious, and they are less carefully examined. Involvement of their nuclei in the brainstem is, however, common in polio, leading to severe dysphagia, dysarthria, and respiratory difficulties. Thus, polio must be considered in unvaccinated individuals who have traveled abroad.
Botulism & Pseudobotulism
Although botulism is a disorder of the neuromuscular junction rather than peripheral nerves, patients with botulism commonly present with dry mouth, dysarthria, dysphagia, diplopia, and impaired ocular accommodation. Rapid recognition of this disease is important. Prompt administration of antitoxin along with ventilatory support is essential in the management of botulism. In addition, recognition of the disease should prompt an intensive search for contaminated foodstuffs, which are most often the cause of the condition in adults. Victims who have only early symptoms may be recognized only because they were identified as sharing certain foodstuffs with individuals who have clear-cut disease.
A cluster of signs and symptoms similar to those in botulism may occur after ingestion of any part of the plant, Jimson weed (Datura stramonium); the syndrome may mimic botulism in patients who present with dysphagia; dilated, fixed pupils; and dry mouth (pseudobotulism), which is often accompanied by visual or auditory hallucinations. The Datura plant contains the anticholinergics atropine and scopolamine, which are highly concentrated in the seeds and can cause serious illness or death. Teenagers seeking mind-altering experiences sometimes experiment with Datura, only to die, probably from the cardiotoxic principle in the plant. They usually present with the pseudobotulism syndrome (fixed, dilated pupils and dry mouth), which is distinguishable from true botulism primarily by the history of contact with the Datura plant or its seeds and by the hallucinations, which are not a feature of true botulism. If the cardiovascular system is stable, the patients usually improve over time without therapy, other than emptying the stomach, using activated charcoal, or both; however, if the patients manifest profound toxicity, including bradycardia or tachycardia, the use of the antidote physostigmine should be considered.
Datura poisoning might also suggest rabies to the initial observer, if the hallucinations are mistaken for encephalopathy and if the dysphagia is mistaken for hydrophobia.
Rabies
Rabies should also be considered in the differential diagnosis of patients presenting with dysphagia and dysarthria, with or without hydrophobia and laryngeal spasms. Rabies must be considered in any patient who has traveled to third-world countries and who has had any contact with potentially infected animals. Skunks, raccoons, and bats are the main animal reservoirs of rabies in the United States.
Treatment with human immune globulin and diploid cell-derived vaccine is very effective for individuals exposed to or bitten by rabid animals, but it is ineffective once neurological symptoms have developed. It is important for persons in close contact with the victims, whose body fluids may be infectious or for others who have had contact with the same or other infected animals to receive postexposure prophylaxis. A detailed review of the indications and procedures for postexposure prophylaxis has been provided by the Centers for Disease Control and Prevention (CDC/MMWR, 1999).
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