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How to diagnose and differentially diagnose patients with acute onset muscle weakness and respiratory dysfunction? The latest issue of the Clinical Reasoning Series of Neurology reported a case of a 13-year-old male patient with acute muscle weakness and shortness of breath.
Let’s learn the clinical reasoning process together.
Yimaitong compiles and compiles, please do not reprint without authorization.
A 13-year-old boy with fatigue, vomiting, shortness of breath, and difficulty walking for 4 days.
The patient was a 13-year-old boy who presented to the doctor for 4 days of fatigue, vomiting, shortness of breath and difficulty walking.
The patient was in good health and a recent history of viral upper respiratory tract infection.
Physical examination in the emergency room showed weakness in the lower limbs and right arm.
In the next few hours, the patient's condition deteriorated and was urgently intubated due to respiratory distress and was referred to the Children's Hospital.
Physical examination showed that he was awake, alert and oriented, and able to answer questions with finger movements.
Normal pupil size, sensitive to light; mild downward vision nystagmus, incomplete eye closure, tongue weakness, asymmetric ptosis; loss of vomiting reflex; normal muscle volume, decreased diffuse muscle tone; proximal limb muscle strength 2 /5, distal muscle strength 3-4/5; no obvious abnormality in reflexes; normal feeling; altogether and gait unmeasured.
Questions to ponder: 1.
What are the differential diagnoses? What differential diagnosis needs to be considered? The differential diagnosis of acute onset weakness with respiratory dysfunction includes myasthenia gravis (MG), juvenile myasthenia gravis (JMG), Lambert-Eton myasthenia syndrome (LEMS), hyperthyroidism, Guillain-Barré syndrome (GBS) and botulism.
MG is an autoimmune disease.
Its antibodies target the postsynaptic neuromuscular junction (NMJ), which causes muscle weakness in the medulla oblongata and extremities, which is fluctuating, fatigue, and painless, and improves after rest.
Patients with JMG often present with ptosis and other ocular symptoms, progress to general weakness, and rarely suffer from respiratory distress.
LEMS is an autoimmune or paraneoplastic disease characterized by ptosis, autonomic dysfunction, hyporeflexia, proximal and progressive muscle weakness.
However, considering the acute manifestations and the age of the patient, the disease is unlikely; and the disease is rare in children, and currently only 12 cases of LEMS have been reported in children.
Hyperthyroidism also needs to be considered.
The patient has local ophthalmoplegia, but no other signs and Graves ophthalmopathy, which is unlikely.
Thyroid crisis can cause acute respiratory failure, changes in mental status, and general weakness, but it is not common in children.
GBS usually appears 2-4 weeks after gastrointestinal or respiratory diseases, and is usually accompanied by ascending weakness, pain, and difficulty walking.
About 50% of children suffer from autonomic dysfunction, including unstable blood pressure, intestinal obstruction, and bladder dysfunction.
The variation of GBS, Miller-Fisher syndrome (MFS) can be manifested as a triad of ataxia, ophthalmoplegia, and decreased reflexes.
Although rare, acute respiratory failure can occur with GBS in children.
Botulinum toxin can block the release of neurotransmitters at the NMJ, resulting in a decrease in neurotransmitters and flaccid paralysis.
Normally, the incubation period is about 12-48 hours after ingestion.
The patient, his parents and a friend had eaten canned mung beans at home about 4 days before the consultation, but the others had no symptoms.
Questions to ponder: 1.
What diagnostic tests are needed? What further inspections are required? Laboratory tests including thyroid stimulating hormone, blood cell count, metabolic kit or systemic inflammation markers showed no abnormalities; toxicology screening showed no obvious abnormalities; anti-acetylcholine receptor, muscle-specific receptor tyrosine kinase antibody, anti-GQ1b Antibody and voltage-gated calcium channel antibodies are negative; anti-Titin antibody is mildly positive (commonly seen in patients with MG with thymoma, but this patient does not have thymoma on CT); pyrithione test does not support JMG; day 5 and day 10 The CSF test was normal on day 10; the spinal cord and head MRI were normal on day 10; the nerve conduction examination on the day of admission showed that the amplitude of the ulnar nerve motor conduction wave was reduced, the blink response disappeared, and the repetitive nerve stimulation (RNS) was normal. The patient was treated with glycerin suppositories for constipation, but glycerin invalidated the fecal botulinum toxin test, and no glycerin-free sample was obtained; serum botulinum toxin test was not performed.
Questions to ponder: 1.
What is the most likely diagnosis? What is the final diagnosis? Because the patient has a history of family canned food contact, food-borne botulism needs to be considered.
However, there was no pupil abnormality, weakness, no progress, and little support.
In the end, the patient considered the MFS variant of GBS, and received intravenous immunoglobulin (IVIG) treatment for this, and his hand muscle strength improved slightly within a week.
But for the next month, he was still weak and underwent a tracheotomy.
The patient subsequently developed sweating and intermittent hypertension, which was considered an autonomic dysfunction in GBS.
Two weeks after the first administration, gamma globulin was injected intravenously again, and his condition improved slightly.
The electrodiagnostic test was repeated 40 days after the onset of the initial symptoms, and it showed that axonal motor neuropathy was accompanied by mild to moderate denervation, which is acute motor axonal neuropathy (AMAN).
On the 24th day of hospitalization, a friend of the patient went to see a doctor.
He experienced fatigue, proximal weakness, difficulty walking, and dysphagia almost at the same time as the patient, but now the symptoms are stable; the only abnormality is a decrease in bilateral upper limb muscle volume , The proximal muscle strength was 4/5, and it was difficult to get up in the supine position; the patient stated that he had eaten the same mung bean as the patient in this case.
The full set of myositis, creatinine kinase level, and neuromuscular autoimmune disease screening were all normal.
The electro-diagnostic test performed on the 32nd day of the onset of symptoms showed that the RNS was normal, the motor unit amplitude was reduced, and the early recovery, suggesting acute myopathy.
Before further examination of the second patient, 47 days after the onset of symptoms in this patient, the mother of this patient was hospitalized with acute respiratory failure; her canned green bean botulinum toxin tested positive.
What caused AMAN? Botulinum poisoning is a rare and potentially fatal disease.
The subtypes include wounds, infants, intestinal toxemia, and foodborne. In order for botulinum toxin spores to germinate and produce heat-labile botulinum toxin, the environment must be anaerobic, non-acid, low in salt and low in sugar, which is common in household canned vegetables and fermented fish.
In the systemic circulation, neurotoxins irreversibly bind to the presynaptic motor neuron terminals and block the transmission of acetylcholine through NMJ, resulting in bulbar weakness, respiratory failure, and symmetric flaccid paralysis.
Other common symptoms include ophthalmoplegia, Other cranial nerve palsy, pupil abnormalities, and severe constipation; autonomic nerve disorders manifested as dry mouth, heart rate, and blood pressure fluctuations.
The diagnosis can be confirmed by testing stool, serum or suspicious food sources.
Although serum toxin tests are helpful in diagnosis, they usually only test positive within 16 days of admission.
Treatment includes early diagnosis, supportive treatment and timely antitoxin treatment.
For this patient, antitoxins are unlikely to work 54 days after symptoms appear, and circulating toxins are unlikely to remain.
The non-specific binding of IVIG to circulating toxins may delay the progression of the disease, but its slight improvement may be related to the natural course of the disease.
This case emphasizes the difficulty of quickly and accurately diagnosing botulinum poisoning.
Early symptoms of GBS in children may overlap with those of botulinum poisoning, including respiratory failure, weakness of the limbs and medulla oblongata, and weakened reflexes.
The salient features of GBS, such as elevated cerebrospinal fluid protein and electrophysiological abnormalities, may be delayed up to two weeks.
Botulinum poisoning may have reduced CMAP amplitude and increased response to rapid RNS, but it is rare.
Although the patient did not have a definitive test for botulinum toxin, and his electrodiagnostic study was inconclusive, his clinical course and exposure history finally proved to be consistent with botulinum neurotoxicity.
Reference: Clinical Reasoning: A Teenager with Shortness of Breath and Difficulty Walking.
Neurology.
2021 Jan 27;10.
1212/WNL.
0000000000011544.
Let’s learn the clinical reasoning process together.
Yimaitong compiles and compiles, please do not reprint without authorization.
A 13-year-old boy with fatigue, vomiting, shortness of breath, and difficulty walking for 4 days.
The patient was a 13-year-old boy who presented to the doctor for 4 days of fatigue, vomiting, shortness of breath and difficulty walking.
The patient was in good health and a recent history of viral upper respiratory tract infection.
Physical examination in the emergency room showed weakness in the lower limbs and right arm.
In the next few hours, the patient's condition deteriorated and was urgently intubated due to respiratory distress and was referred to the Children's Hospital.
Physical examination showed that he was awake, alert and oriented, and able to answer questions with finger movements.
Normal pupil size, sensitive to light; mild downward vision nystagmus, incomplete eye closure, tongue weakness, asymmetric ptosis; loss of vomiting reflex; normal muscle volume, decreased diffuse muscle tone; proximal limb muscle strength 2 /5, distal muscle strength 3-4/5; no obvious abnormality in reflexes; normal feeling; altogether and gait unmeasured.
Questions to ponder: 1.
What are the differential diagnoses? What differential diagnosis needs to be considered? The differential diagnosis of acute onset weakness with respiratory dysfunction includes myasthenia gravis (MG), juvenile myasthenia gravis (JMG), Lambert-Eton myasthenia syndrome (LEMS), hyperthyroidism, Guillain-Barré syndrome (GBS) and botulism.
MG is an autoimmune disease.
Its antibodies target the postsynaptic neuromuscular junction (NMJ), which causes muscle weakness in the medulla oblongata and extremities, which is fluctuating, fatigue, and painless, and improves after rest.
Patients with JMG often present with ptosis and other ocular symptoms, progress to general weakness, and rarely suffer from respiratory distress.
LEMS is an autoimmune or paraneoplastic disease characterized by ptosis, autonomic dysfunction, hyporeflexia, proximal and progressive muscle weakness.
However, considering the acute manifestations and the age of the patient, the disease is unlikely; and the disease is rare in children, and currently only 12 cases of LEMS have been reported in children.
Hyperthyroidism also needs to be considered.
The patient has local ophthalmoplegia, but no other signs and Graves ophthalmopathy, which is unlikely.
Thyroid crisis can cause acute respiratory failure, changes in mental status, and general weakness, but it is not common in children.
GBS usually appears 2-4 weeks after gastrointestinal or respiratory diseases, and is usually accompanied by ascending weakness, pain, and difficulty walking.
About 50% of children suffer from autonomic dysfunction, including unstable blood pressure, intestinal obstruction, and bladder dysfunction.
The variation of GBS, Miller-Fisher syndrome (MFS) can be manifested as a triad of ataxia, ophthalmoplegia, and decreased reflexes.
Although rare, acute respiratory failure can occur with GBS in children.
Botulinum toxin can block the release of neurotransmitters at the NMJ, resulting in a decrease in neurotransmitters and flaccid paralysis.
Normally, the incubation period is about 12-48 hours after ingestion.
The patient, his parents and a friend had eaten canned mung beans at home about 4 days before the consultation, but the others had no symptoms.
Questions to ponder: 1.
What diagnostic tests are needed? What further inspections are required? Laboratory tests including thyroid stimulating hormone, blood cell count, metabolic kit or systemic inflammation markers showed no abnormalities; toxicology screening showed no obvious abnormalities; anti-acetylcholine receptor, muscle-specific receptor tyrosine kinase antibody, anti-GQ1b Antibody and voltage-gated calcium channel antibodies are negative; anti-Titin antibody is mildly positive (commonly seen in patients with MG with thymoma, but this patient does not have thymoma on CT); pyrithione test does not support JMG; day 5 and day 10 The CSF test was normal on day 10; the spinal cord and head MRI were normal on day 10; the nerve conduction examination on the day of admission showed that the amplitude of the ulnar nerve motor conduction wave was reduced, the blink response disappeared, and the repetitive nerve stimulation (RNS) was normal. The patient was treated with glycerin suppositories for constipation, but glycerin invalidated the fecal botulinum toxin test, and no glycerin-free sample was obtained; serum botulinum toxin test was not performed.
Questions to ponder: 1.
What is the most likely diagnosis? What is the final diagnosis? Because the patient has a history of family canned food contact, food-borne botulism needs to be considered.
However, there was no pupil abnormality, weakness, no progress, and little support.
In the end, the patient considered the MFS variant of GBS, and received intravenous immunoglobulin (IVIG) treatment for this, and his hand muscle strength improved slightly within a week.
But for the next month, he was still weak and underwent a tracheotomy.
The patient subsequently developed sweating and intermittent hypertension, which was considered an autonomic dysfunction in GBS.
Two weeks after the first administration, gamma globulin was injected intravenously again, and his condition improved slightly.
The electrodiagnostic test was repeated 40 days after the onset of the initial symptoms, and it showed that axonal motor neuropathy was accompanied by mild to moderate denervation, which is acute motor axonal neuropathy (AMAN).
On the 24th day of hospitalization, a friend of the patient went to see a doctor.
He experienced fatigue, proximal weakness, difficulty walking, and dysphagia almost at the same time as the patient, but now the symptoms are stable; the only abnormality is a decrease in bilateral upper limb muscle volume , The proximal muscle strength was 4/5, and it was difficult to get up in the supine position; the patient stated that he had eaten the same mung bean as the patient in this case.
The full set of myositis, creatinine kinase level, and neuromuscular autoimmune disease screening were all normal.
The electro-diagnostic test performed on the 32nd day of the onset of symptoms showed that the RNS was normal, the motor unit amplitude was reduced, and the early recovery, suggesting acute myopathy.
Before further examination of the second patient, 47 days after the onset of symptoms in this patient, the mother of this patient was hospitalized with acute respiratory failure; her canned green bean botulinum toxin tested positive.
What caused AMAN? Botulinum poisoning is a rare and potentially fatal disease.
The subtypes include wounds, infants, intestinal toxemia, and foodborne. In order for botulinum toxin spores to germinate and produce heat-labile botulinum toxin, the environment must be anaerobic, non-acid, low in salt and low in sugar, which is common in household canned vegetables and fermented fish.
In the systemic circulation, neurotoxins irreversibly bind to the presynaptic motor neuron terminals and block the transmission of acetylcholine through NMJ, resulting in bulbar weakness, respiratory failure, and symmetric flaccid paralysis.
Other common symptoms include ophthalmoplegia, Other cranial nerve palsy, pupil abnormalities, and severe constipation; autonomic nerve disorders manifested as dry mouth, heart rate, and blood pressure fluctuations.
The diagnosis can be confirmed by testing stool, serum or suspicious food sources.
Although serum toxin tests are helpful in diagnosis, they usually only test positive within 16 days of admission.
Treatment includes early diagnosis, supportive treatment and timely antitoxin treatment.
For this patient, antitoxins are unlikely to work 54 days after symptoms appear, and circulating toxins are unlikely to remain.
The non-specific binding of IVIG to circulating toxins may delay the progression of the disease, but its slight improvement may be related to the natural course of the disease.
This case emphasizes the difficulty of quickly and accurately diagnosing botulinum poisoning.
Early symptoms of GBS in children may overlap with those of botulinum poisoning, including respiratory failure, weakness of the limbs and medulla oblongata, and weakened reflexes.
The salient features of GBS, such as elevated cerebrospinal fluid protein and electrophysiological abnormalities, may be delayed up to two weeks.
Botulinum poisoning may have reduced CMAP amplitude and increased response to rapid RNS, but it is rare.
Although the patient did not have a definitive test for botulinum toxin, and his electrodiagnostic study was inconclusive, his clinical course and exposure history finally proved to be consistent with botulinum neurotoxicity.
Reference: Clinical Reasoning: A Teenager with Shortness of Breath and Difficulty Walking.
Neurology.
2021 Jan 27;10.
1212/WNL.
0000000000011544.
