Guillain-Barré Syndrome Mohamed Sulaiman Al-Houqani
Case 1 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.
Case 2 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.
Guillain-Barré syndrome: is an acute inflammatory demyelinating polyneuropathy characterized by progressive muscle weakness and areflexia • It has an annual incidence of 0.6 to 2.4 cases per 100,000 population and occurs at all ages and in both sexes • With the marked decline in the incidence of polio, Guillain-Barré syndrome is now the most common cause of acute flaccid paralysis in healthy people
PATHOGENESIS • Peripheral nerve demyelination in Guillain-Barré syndrome is believed to be immunologically mediated • Humoral factors and cell-mediated immune phenomena have been implicated in the damage of myelin and/or the myelin-producing Schwann cells
Guillain-Barré syndrome has been reported to follow • vaccinations • epidural anesthesia • thrombolytic agents • It has been associated with some systemic processes, such as • Hodgkin's disease • SLE • Sarcoidosis, and • infection with Campylobacter, Lyme disease, EBV, CMV, HSV, mycoplasma, and recently acquired HIV infection
Campylobacter infection • Campylobacter infection is the most commonly identified precipitant of Guillain-Barré syndrome • A case-control study involving 103 patients with the disease found that 26% of affected individuals had evidence of recent C. jejuni infection compared with 2% of household and 1% of age-matched controls • Seventy percent of those infected with C. jejuni reported a diarrheal illness within 12 weeks before the onset of the neurologic illness
The main lesions are acute inflammatory demyelinating neuropathy and, particularly in patients with Campylobacter-associated disease, acute axonal degeneration • These changes may be caused by cross-reacting antibodies to GM1 ganglioside (present in high concentrations in peripheral nerve myelin) formed in response to similar epitopes expressed by the infecting Campylobacter strain • However, mechanisms other than molecular mimicry may be associated with the production of antibodies to GM1 ganglioside
The Guillain-Barré syndrome variant known as Miller Fisher syndrome, in which the cranial nerves are affected, is also associated with Campylobacter infection • In these patients cross-reacting antibodies to GQ1b ganglioside, which is present in cranial nerve myelin, have been found
CLINICAL FEATURES • Two-thirds of patients develop the neurologic symptoms 2-4 weeks after what appears to be a benign respiratory or gastrointestinal infection • The initial symptoms are fine paresthesias in the toes and fingertips, followed by lower extremity weakness that may ascend over hours to days to involve the arms, cranial nerves, and in severe cases the muscles of respiration
CLINICALFEATURES • Early in the course, patients frequently complain of aching or sciatica-like lower back or leg pain • At some point during their illness, up to 25 percent of patients require mechanical ventilation • More than 90% of patients reach the nadir of their function within two to four weeks, with return of function occurring slowly over weeks to months
PhysicalExamination • Symmetric limb weakness with diminished or absent reflexes • Minimal loss of sensation despite paresthesias • Signs of autonomic dysfunction are present in 50 percent of patients, including • Cardiac dysrhythmias (asystole, bradycardia, sinus tachycardia, and atrial/ventricular tachyarrhythmias) • Orthostatic hypotension • Transient or persistent hypertension • Paralytic ileus • Bladder dysfunction • Abnormal sweating
DIAGNOSTIC STUDIES • Electrophysiologic studies are the most specific and sensitive tests for diagnosis of the disease • They demonstrate a variety of abnormalities indicating evolving multifocal demyelination • Slowed nerve conduction velocities • Partial motor conduction block • Abnormal temporal dispersion • Prolonged distal latencies • A normal study after several days of symptoms, makes the diagnosis of Guillain-Barré syndrome unlikely
DIAGNOSTIC STUDIES • After the first week of symptoms, analysis of the cerebrospinal fluid (CSF) typically reveals • normal pressures • few cells (typically mononuclear) • an elevated protein conc. (greater than 50 mg/dL) • Early in the course (less than one week), protein levels may not yet be elevated, but only rarely do they remain persistently normal • If CSF pleocytosis is noted, other diseases associated with Guillain-Barré syndrome eg, HIV infection, Lyme disease, malignancy, and sarcoidosis should be considered
TREATMENT • The main modalities of therapy for Guillain-Barré syndrome include • Plasmapheresis and • Administration of intravenous immune globulin
Plasmapheresis • Plasma exchange is recommended for patients who • Are unable to walk unaided • Demonstrate worsening vital capacities • Require mechanical ventilation • Have significant bulbar weakness • As a result of the cost, risk, and discomfort to the patient, plasma exchange is generally not used for ambulatory patients with mild disease or for patients whose symptoms are no longer progressing after three weeks