Guillain-Barr é Syndrome . Mohamed Sulaiman Al-Houqani. Case 1.
Mohamed Sulaiman Al-Houqani
A 17-year-old male developed flu-like symptoms, severe diarrhoea and abdominal pain 4 days after attending a dinner party at which he had eaten a chicken. Three other people who had attended the same party developed gastrointestinal symptoms. These symptoms settled within a few days. Stool cultures taken from all four individuals grew Campylobacter jejuni. About 10 days after the onset of diarrhoea, he developed diffuse aching around his shoulders and buttocks and pins and needles in his hands and feet. Over the next week the sensory changes worsened and spread to involve his arms and legs. His limbs became progressively weaker and 8 days after the onset of neurological symptoms he could not hold a cup or stand unaided. He was admitted to hospital and found to have severe symmetrical distal limb weakness and ‘glove and stocking’ sensory loss to the elbows and knees. Nerve conduction studies showed evidence of a mixed motor and sensory neuropathy and examination of his cerebrospinal fluid (CSF) showed a very high total protein level at 4g/l but without any increase in the number of cells in the CSF. High titres of IgM and IgG antibodies to Campylobacter jejuni were found in his peripheral blood.
A diagnosis was made of the Guillain–Barré syndrome (acute inflammatory polyneuropathy) probably triggered by Campylobacter jejuni infection. He was treated with high-dose intravenous immunoglobulin but his condition deteriorated with respiratory muscle weakness and he required mechanical ventilation. His condition slowly improved and he was able to breathe spontaneously after 2 weeks. His strength and sensory symptoms slowly improved with vigorous physiotherapy but 1 year after the initial illness he still had significant weakness in his hands and feet.
A previously healthy 15-year-old black adolescent, gravida 1, para 0, at week 10 of gestation had a 10-day history of progressive weakness and paresthesia of the lower extremities. There was no history of a preceding infection or flu-like illness. The patient reported no symptoms in the upper extremities or shortness of breath. Neurologic examination revealed bilateral foot drop, with 4/5 strength of proximal muscles of the lower extremities and normal strength in the upper extremities and bulbar muscles. Sensory examination was remarkable for symmetrically decreased sense of vibration, light touch, and proprioception in the lower extremities (below the knees). Deep tendon reflexes were absent in the lower extremities and only trace reflexes were present in the upper extremities. Plantar flexor response was present bilaterally.
Baseline vital capacity was 2.47 L (87% of predicted value for patient age). Thyroid function tests and screening for heavy metals, vitamin B12, and folate yielded normal results; erythrocyte sedimentation rate was within normal limits. Serum protein electrophoresis revealed a normal migration pattern. The patient did not consent to lumbar puncture. Motor nerve conduction studies revealed markedly increased distal latency of the peripheral nerves of upper and lower extremities, as well as unelicitable F wave of the peroneal nerves, consistent with acute demyelinating peripheral polyneuropathy. An antiñperipheral nerve antibody panel revealed mild elevation of GM1 antibodies (12; normal <10), with normal values for asialo-GM1, GD1a, and GD1b.
Pelvic ultrasonography revealed a normal intrauterine pregnancy, good correlation between size and gestational age, and a live and active fetus. After obstetric consultation, the patient had four sessions of plasmapheresis, with a calculated plasma-to-crystalloid exchange rate of 200 to 250 mL. The patient and fetus tolerated the procedure well. Progression of motor symptoms was arrested and a mild improvement was noticed immediately after the third plasma exchange session. Follow-up spirometries were normal and the patient did not require assisted ventilation. Follow-up ultrasonography did not show any signs of fetal distress. Aggressive physical therapy was begun and the patient was ambulatory on discharge from the hospital, 9 days after admission.
The patient received follow-up care at the High Risk Obstetrical Clinic and had an uneventful course until gestational week 20, when a decrease in frequency of fetal movements prompted immediate ultrasonography. Intrauterine fetal death was detected, and the patient was readmitted for an induced vaginal delivery. Failure to deliver the placenta required cervical dilation with evacuation of uterine contents and curettage.
Pathologic examination of placental tissue revealed CMV placentitis.
is an acute inflammatory demyelinating polyneuropathy characterized by progressive muscle weakness and areflexia