Age: 44.0
Sex: F
Vaccine: IPV
Symptoms: CSF glucose normal, CSF lymphocyte count normal, CSF myelin basic protein increased, CSF protein normal, CSF white blood cell count
Clinical narrative: Initial case was retrieved from scientific literature on 04 July 2011. A 44 year-old female with a history of common variable immunodeficiency and was receiving intravenous immune globulin. The patient experienced sudden paralysis of all four limbs and respiratory muscles, resulting in death. The patient was found, to have Type 2 vaccine-derived poliovirus in her stool. According to the report, the patient was probably infected 11.9 years prior when her child had received the oral poliovirus vaccine. Verbatim from the report: "In March 2009, the Department of Health investigated a case of acute flaccid paralysis associated with a positive stool culture for enterovirus. Case report: In December 2008, cough, purulent rhinorrhea, mild dyspnea, malaise, and a "low-grade fever" developed in a 44-year-old woman with long-standing common variable immunodeficiency; the symptoms, which she assumed were an exacerbation of her chronic sinusitis, resolved after 4 days. Two days later, cramping developed in her left calf and progressed over a period of 5 days to leg weakness, which was associated with a fall and ankle sprain, and the patient was hospitalized. On examination, she was afebrile and her cranial nerves were normal, without meningismus. Upper-extremity muscle strength was normal, and reflexes were "quite brisk". Measurements of lower-extremity muscle strength on the right and left sides, respectively, were as follows: iliopsoas, 3/5 and 1/5; quadriceps, 3/5 and 1/5; dorsiflexors, 2/5 and 1/5; and plantar flexors, 4/5 and 3/5. A trace right-ankle jerk was noted, but all other lower-extremity stretch reflexes were absent. The plantar (Babinski) reflex was downward bilaterally. Sensory responses to a light touch, temperature changes, and pinprick were intact, with severe pain noted in both legs. Common variable immunodeficiency was diagnosed in 1991, with subsequent diagnoses of chronic lymphoid interstitial pneumonia (on lung biopsy), liver cirrhosis with esophageal varices of grade 1 to 2, and spruelike enteropathy (on intestinal biopsy) with chronic diarrhea (which had worsened during the 2 months before the patient's hospitalization in 2008). She had undergone splenectomy in 2006 owing to portal hypertension. B cells were absent on flow cytometry, expression of Bruton's tyrosine kinase was normal, and she was heterozygous for an R202H mutation in the transmembrane activator and calcium-modulator and cyclophilin-ligand-interactor (TACI) protein. Frequent episodes of otitis media and sinusitis were treated with antibiotics. She was being treated with prednisone, 20 mg daily, and two different brands of intravenous immune globulin every 3 weeks, with multiple changes in the dose, before the onset of her neurologic symptoms. The only travel of possible relevance was with her family 2 months earlier. By hospital day 2, the weakness had progressed to involve both upper extremities. The patient became constipated and remained afebrile. The cerebrospinal fluid contained 6 white cells per cubic millimeter (51% lymphocytes) and glucose and protein levels were normal. Initial magnetic resonance imaging showed increased T2-weighted signal in the spinal-column parenchyma from T7 to the conus medullaris, without contrast enhancement. When imaging was repeated on hospital day 6, new areas of hyperintensity were noted in C3 through C7, findings that were consistent with anterior horn-cell disease. The patient's upper-extremity muscle weakness progressed (biceps strength, 3/5 on the right side and 2/5 on the left), and she continued to have severe muscular limb pain. Electromyography and nerve-conduction testing on hospital day 8 were consistent with acute motor axonal neuropathy or motor neuronopathy (anterior horn-cell disease). On hospital days 8 through 38, negative inspiratory force was reduced, and pulmonary infiltrates required noninvasive, bilevel positive airway pressure and intermittent endotracheal intubation. On hospital days 61 through 73, liver dysfunction worsened and pneumonia and respiratory failure ensued. These problems continued, and given the multiple coexisting illnesses and persistent neurologic deficits, the family chose to withdraw support on hospital day 92, and the patient died. No autopsy was performed. Although the initial viral cultures were negative, a stool sample obtained on hospital day 74 was found to contain enterovirus and was sent to the Department of Health for assessment of speciation. Investigators of reportable diseases, including polio, under state statute are classified as public health response, nonresearch by the Department of Health Institutional Review Board." The patient reportedly had received all recommended childhood vaccinations. The patient's child had received the primary series of oral poliovirus vaccine at 13.0, 12.8 and 12.6 years before the patient's isolate was collected. The patient had the first nonimported case of paralytic poliomyelitis and the second case of VDPV infection reported since the "discontinuation in 2000 of oral poliovirus vaccinations". The patient's history of receiving intravenous administration of immune globulin did not "prevent the acquisition of infection, stop the long-term poliovirus infection or prevent the development of paralytic poliomyelitis." Diagnostic and Laboratory findings included: MRI of the Spinal cord on hospital day 2 revealed the signal was increased on T(2)-weighted images from the lumbar spine to the seventh thoracic vertebra; on day six focal focal hyperintensity was seen on T(2)-weighted images in the anterior horns bilaterally, extending from the third through seventh cervical vertebra with mild enlargement of the spinal cord. Cerebrospinal fluid white-cell count was 6 x10(-3)/mm(3) and myelin basic protein on hospital day 1-30 was 17 ng/ml and on days 61-91 was 5.24. Blood white-cell count on hospital days 1-30 was 17.8 x10(-3)/mm(3); on hospital days 31-60 was 22.3 x10(-3)/mm(3); and on hospital days 61-91 was 25.5 x10(-3)/mm(3). Neutrophils on hospital days 1-30 were 17.1 x10(-3)/mm(3); on hospital days 31-60 was 18.7 x10(-3)/mm(3); and on hospital days 61-91 was 23.7 x10(-3)/mm(3). Documents held by sender: journal article.