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Fig 3. Pathogenesis of GBS
小兒Guillain-Barre症候群復健成效:病例報告Rehabilitation for A Child with Guillain-Barré Syndrome – A case report 編號:76 陳春如 陳繼民 林高民 黃秀珍 賴旻信* 戴德森醫療財團法人嘉義基督教醫院 Introduction Discussion 40-60% patients have antecedent infections within 1 month Upper repiratory infections are the most common Gastrointestinal illness is the 2nd most common Achieve maximal severity within 4 weeks Minimal loss of sensation despite paresthesia Absent or diminshed tendon reflex Lumbar puncture: Albuminocytologic dissociation elevated CSF protein with a normal WBC count Mechanism of Plasmaphoresis Remove circulating antibodies, complement, and soluble biological response modifiers with total change of plasma volume (50ml/kg) for 5 times within 1~2 weeks Mechanism: IVIG Blockade of Fc receptors, provision of anti-idiotypic antibodies, interfere with complement activation and T cell IVIG is recommended for non-ambulant adult patients with GBS within 2 or 4 weeks Acute phase rehabilitation (I) Bowel and bladder management: LMN type Bladder catheterization is often needed in acute stage ~ 30% patients developed urinary tract infection Orthotics prescription for proper positioning and optimizing residual motor function Gentle strengthening: individualized programs Watch out for muscle shortening and joint contractures Fatigue persists in 80% of patient and is unrelated to age, duration or severity of the initial illness The recovery period may be as little as a few weeks or as long as a few years. About 30 percent of those with Guillain-Barré still have a residual weakness after 3 years. About 3 percent may suffer a relapse of muscle weakness and tingling sensations many years after the initial attack. Follow-up investigations showed a persistent muscular weakness at 60 days, 6 months, and 1 year after onset in 61%, 14%, and 10% of children Fig 2. Treatment Ladder of GBS Fig 3. Pathogenesis of GBS The syndrome is named after the French physicians Georges Guillain and Jean Alexandre Barré, who described it in 1916. Guillain-Barre Syndrome (GBS) is an acute acquired demylenating polyneuropathy presumed to be immune mediated. An incidence of 0.5-5/ children/year has been reported worldwide and High incidence (21%) among patient < 10y/o in Taiwan. 60% of cases do not have a known cause. Case Report An 10-year-old boy presented with thigh pain, distal-dominant upper limbs weakness and bilateral legs weakness, and inability to walk 3 weeks after an upper respiratory infection. He had no sensory and autonomic signs and no cranial nerve involvement during the course of the disease. Tendon reflexes were preserved except for an absent Achilles' tendon reflex. Cerebrospinal fluid analysis revealed albuminocytologic dissociation. Therefore, a diagnosis of pure motor Guillain-Barré syndrome (GBS) was made. Cerebrospinal fluid analysis revealed an albuminocytologic dissociation. Therefore, a diagnosis of atypical GBS was made. Based on the clinical findings, we considered his diagnosis as GBS following the upper upper respiratory infection. Aggressive rehabilitation programs and Intravenous immunoglobulin (IVIG) treatment, with 0.4 g/kg/day over 5 days was initiated but his clinical condition responded to the treatment weakly. Therefore, we started plasmapharesis for his poor response to the IVIG. The patient was discharged one month later. Tow months after discharge, at a follow-up visit, the patient showed gradual improvement of his symptoms, including the weakness in the upper and lower limbs and the DTRs became normal. A We started plasmaphoresis treatment for him. B B Fig 1. Clinical improvement of our patient’s condition with PEDI(Pediatric Evaluation of Disability Inventory). Note the red arrow when he was received plasmaphoresis.
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