• Although the exact cause is unknown, following respiratory tract infection (Mycoplasma pneumoniae), gastrointestinal infections (especially Campylobacterjejuni and rarely H.pylori), EBV, CMV, H.influenzae infections, Lyme disease or meningococcal serotype C, rabies, influenza and oral polio vaccination emerging
It is a neuropathy that mainly involves motor neurons, as well as sensory and autonomic nerves, and is characterized by weakness starting from the lower extremities and loss of symmetric reflexes.
Clinical Findings
• Weakness usually begins in the lower extremities and progresses progressively to involve the trunk, upper extremities and finally the bulbar muscles. This form of progression is called "Landry ascending paralysis".
• There is loss of symmetrical deep tendon reflex (DTR), loss of sensation, and weakness.
As a result of autonomic nerve dysfunction, hypotension, hypertension, tachycardia, arrhythmia, urinary retention, sweating occur.
• Proximal and distal muscles are involved relatively symmetrically, but asymmetry is found in 9% of patients.
• Affected children are irritable. The weakness may progress to an unwillingness or inability to walk, and then to flaccid tetraplegia. In some cases, paresthesias occur.
• Bulbar involvement occurs in approximately half of the cases. Although papilledema is seen in some cases, visual impairment is not clinically evident. Respiratory failure may develop. Dysphagia and facial weakness may be early signs of respiratory failure. Facial nerves may be involved.
• Miller-Fisher syndrome consists of acute external ophthalmoplegia, ataxia and areflexia.
• Urinary incontinence or urinary retention is a complication in about 20% of cases, but this is usually temporary.
Diagnosis
• Protein increase without cell increase in CSF (albuminocytological dissociation)
• Decrease in motor conduction velocity and decrease in amplitudes in EMG
• Inflammation and segmental demyelination are observed in peripheral nerves.
Treatment
• Due to the risk of respiratory failure in the acute period, they should be monitored in intensive care units, and if respiratory failure develops, they should be intubated.
• Hypertension, supportive treatment if hypotension has developed.
• Correction of liquid electrolyte disturbance.
• Treatment of constipation or incontinence.
• IVIG is primarily used in the treatment, if ineffective, immunosuppressive drugs and plasmapheresis can be used. Steroids are generally ineffective.
Prognosis
• 75% of cases return to normal in 1-12 months.
• Weakness in the lower extremities is seen in 20% of the cases.
• 5% of cases may die from respiratory failure, cardiovascular collapse or pulmonary embolism in the acute period.
• Conditions associated with Poor Prognosis:
- Cranial nerve involvement
- Intubation
- Maximum disability at the time of application
Chronic inflammatory demyelinating polyradiculoneuropathy is a progressive, slowly progressive Guillian Barre syndrome that may last for months to years, with recurrent attacks or never heal. Acute recurrence occurs in approximately 7% of children with GBS.
Patients usually have severe involvement, tetraplegia, bulbar and respiratory involvement are also seen. Hypo or areflexia may be seen. Motor involvement is in the foreground. There is no pleocytosis in the CSF and the protein is normal or slightly elevated. Nerve conduction velocity studies and sural nerve biopsy are abnormal. Steroids and IVIG are used in the treatment.
