| The recognition of uncommon pediatric motor unit disorders or unusual clinical presentations of common illnesses, such as guillain-barre syndrome (GBS), have increased the need for electromyography (EMG) in childhood critical care units. There are two different clinical sets, one appropriate to newborns and infants and the other to older children. Some illnesses that present as an acute floppy infant are not found in the differential diagnosis of motor unit disorders in the older child or adult. These include spinal muscular atrophy, postvaccine poliomyelitis, intrauterine GBS, infantile botulism, and severe myopathies, such as myotonia dystrophy, and some glycogen storage diseases. An appreciation of the neurophysiological maturational norms is essential to an effective pediatric EMG consultation for children ages 0-3 years. Additionally, the neuromuscular complications of extended intubation and sepsis in children are gaining broader recognition. An increased dialogue between clinical neurophysiologists and pediatric neurologists and intensivists in both neonatal and pediatric intensive care units is essential. ( info) |
12/163. Importation of wild poliovirus into Qinghai Province--china, 1999. Indigenous wild poliovirus was last isolated in china in 1994. On October 13, 1999, a case of acute flaccid paralysis (AFP) in a 16-month-old boy was reported to public health authorities in Xunhua Autonomous County, Haidong Prefecture, Qinghai Province, china. Following onset of paralysis on October 12, the boy was no longer able to stand or walk. Two stool samples, taken within 14 days of onset of paralysis, were analyzed in the Qinghai provincial laboratory and yielded poliovirus. The isolates were later differentiated as wild poliovirus type 1 at the National poliovirus Laboratory in Beijing. Stool specimens from one of five children with whom the boy had contact yielded wild poliovirus type 1. This report describes this case of poliomyelitis and the public health response to the case in china. ( info) |
| The temporal bones of a 26-month-old white female with a paralytic syndrome clinically and pathologically identical to poliomyelitis were examined. The aetiological agent was unknown although a non-poliomyelitis enterovirus infection seemed likely. There was a complete absence of the cochlear neurons and substantially reduced peripheral and central axons with loss of some inner hair cells but preservation of outer hair cells. Scarpa's ganglion, and the geniculate ganglion were partially atrophied. The saccule and utricle were mildly dilated and Reissner's membrane of the apical turn was bulging. In two previous audiological studies a 10--20 dB bilateral sensorineural hearing loss was found in poliomyelitis patients and a neuronal lesion was postulated which is now supported by our findings. This is a rare example of an almost pure neural hearing loss. ( info) |
14/163. ulnar nerve palsy due to axillary crutch. A young lady with residual polio, using axillary crutch since early childhood, presented with tingling, numbness and weakness in ulnar nerve distribution of five months duration. Ulnar motor conduction study revealed proximal conduction block near the axilla, at the point of pressure by the crutch while walking. Distal ulnar sensory conduction studies were normal but proximal ulnar sensory conduction studies showed absence of Erb's point potential. These findings suggested the presence of conduction block in sensory fibers as well. Proper use and change of axillary crutch resulted in clinical recovery and resolution of motor and sensory conduction block. ( info) |
| BACKGROUND: Paralytic poliomyelitis due to the wild-type poliovirus has been eradicated in the united states because of effective immunization programs. In the postvaccination era, most cases are caused by other rna viruses, such as coxsackievirus or echovirus. The condition usually begins with a fever and upper respiratory tract or gastrointestinal tract symptoms that progress to a "paralytic" phase characterized by limb weakness, areflexia, and, occasionally, respiratory failure that superficially resemble guillain-barre syndrome. OBJECTIVE: To describe 2 patients with nonpoliovirus poliomyelitis and highlight the findings on magnetic resonance imaging of the spinal cord to distinguish these cases from variants of guillain-barre syndrome. DESIGN AND SETTING: Case series from an academic medical center. patients: Following a viral illness, the patients, aged 35 and 50 years, had painless, progressive, asymmetrical weakness in the arms followed by respiratory failure in one patient, and generalized limb weakness in the other patient, reaching a nadir in 1 week. Both patients had fevers but no signs of meningitis at onset. Tendon reflexes were absent or reduced in affected regions. The cerebrospinal fluid findings were as follows: mononuclear leukocyte counts of 100 000 cells/mm(3) and 700 000 cells/mm(3), respectively, and the protein level was above 10 g/dL in both patients. Compound muscle action potential amplitudes were reduced in some nerves with active denervation in clinically affected muscles, and F-responses were absent but there were no other demyelinating features. magnetic resonance imaging showed discrete T2-weighted signal changes of the ventral horns of the spinal cord, and one had elevated coxsackievirus titers in the serum. There was little recovery and significant atrophy in weak muscles after 3 years. CONCLUSIONS: The poliomyelitis syndrome still occurs in adults in developed countries. It has superficial similarities to a motor axonal variant of guillain-barre syndrome but can be distinguished by clinical, cerebrospinal fluid, and, perhaps specifically, magnetic resonance imaging characteristics. ( info) |
16/163. Paralytic poliomyelitis associated with the Sabin 3 revertant strain of poliovirus in bahrain. We report a case of vaccine-associated paralytic poliomyelitis (VAPP) in bahrain. The case occurred in an 8-week-old infant who had received a dose of oral polio vaccine (OPV) 7 days after birth. She was in contact with two vaccinees who had received OPV during the national immunisation campaign conducted 10 days before her birth. Specimens from the infant were sent to the WHO Collaborating Centre for Virus Reference and research Laboratory for serological testing and virus detection, including genomic sequencing. Clinical and virological features are presented of a case of VAPP caused by the Sabin 3 strain of poliovirus that had reverted towards neurovirulence. The case represents one in 51,879 first doses of OPV distributed between 1995 and 1998. In order to reduce further the risk of VAPP, the dose of OPV at birth has been discontinued and a sequential schedule of inactivated polio vaccine (IPV) followed by OPV will be recommended. ( info) |
17/163. Acute flaccid paralysis associated with circulating vaccine-derived poliovirus--philippines, 2001. Three cases of acute flaccid paralysis (AFP) associated with circulating vaccine-derived poliovirus (cVDPV) isolates were reported in the philippines during March 15-July 26, 2001. The first case-patient, a child aged 8 years from northern Mindanao island (500 miles south of Manila) who had received 3 doses of oral polio vaccine (OPV), had onset of paralysis on March 15. A second child, aged 3 years from Laguna province on Luzon island (60 miles south of Manila) who had received 3 OPV doses, presented with signs of meningitis but no paralysis on July 23. A third child, aged 14 months from Cavite province (25 miles from Manila and 45 miles north of Laguna province) who had received 2 OPV doses, had onset of paralysis on July 26. No patients had traveled outside of their province of residence since birth. Characterization of isolates from the three patients revealed type 1 polioviruses derived from Sabin vaccine strain type 1, with a 3% genetic sequence difference between Sabin 1 vaccine and vaccine-derived poliovirus (VDPV) isolates. The three polioviruses are not identical but are closely related (>99% sequence homology); they also appear to share an identical recombination site with a nonpolio enterovirus in the noncapsid region of the genome. ( info) |
18/163. A part of the ulnar nerve as an alternative donor nerve for functioning free muscle transfer: a case report. A patient with no active elbow flexion because of poliomyelitis-like syndrome underwent functioning free muscle transfer for elbow flexion reconstruction in which a part of the ulnar nerve was used as a donor motor nerve. Fourteen months after surgery the patient had achieved 120 degrees of active elbow flexion against gravity without functional deficit of the donor ulnar nerve. A part of the ulnar nerve can be used as an alternative donor motor nerve for reconstruction of chronic C5-C6 (or C5-7) brachial plexus injury. ( info) |
19/163. survival of wild polio by a patient with XLA. BACKGROUND: X-linked agammaglobulinemia (XLA) is a primary immunodeficiency characterized by failure of B-cell differentiation and hypogammaglobulinemia. In addition to being susceptible to bacterial infections, patients with XLA are also susceptible to enteroviruses. Systemic enterocytopathogenic human orphan virus (ECHO), coxsackie virus, and vaccine-related polio infections have caused severe morbidity and high mortality rates in XLA patients. OBJECTIVE: We report a 54-year-old male with molecularly defined XLA who survived wild poliomyelitis in childhood before the diagnosis of XLA. methods: At age 5, in 1951, the patient contracted wild polio, characterized by diarrhea and motor weakness. He subsequently developed recurrent sinusitis, bronchitis, and pneumonia, and at age 31 was found to be hypogammaglobulinemic and was started on immunoglobulin replacement. Laboratory evaluation at age 47 revealed an immunoglobulin g of 256 mg/dL, and B-cells (CD19) of 0.1%. mutation analysis of Bruton's tyrosine kinase revealed a 26-basepair deletion between nucleotides 146 and 173 within the plextrin homology domain, resulting in a frameshift and premature termination. CONCLUSIONS: Resolution of wild poliovirus infection is possible in patients with XLA. ( info) |
| In March 2001, a 13-month-old unvaccinated Roma (i.e., gypsy) girl from Bourgas, bulgaria, had onset of bilateral leg weakness. The National enterovirus Laboratory in the capital city of Sofia subsequently isolated a wild type 1 poliovirus in the patient's stool. In April, a second case, with wild type 1 poliovirus isolate was found in lambol located approximately 50 mileswest of Bourgas in an unvaccinated 26-month-old Roma girl who had onset of paralysis of both legs. Subsequent analyses indicated that these viruses were related closely to a strain isolated from Uttar Pradesh, india, in July 2000. A third confirmed case with clinical and serologic evidence of poliomyelitis was diagnosed in a 3-month-old Roma boy in Bourgas who had onset of paralysis on May 7. Following the identification of the poliovirus, the Bulgarian Ministry of Health implemented contact investigations, screening of children at high risk, retrospective record review, intensified acute flaccid paralysis (AFP) surveillance, and mass vaccinations. This report summarizes the outbreak investigation and supplemental vaccination activities in response to these polio cases. High routine vaccination coverage and certification standard AFP surveillance are necessary to detect rapidly and prevent the spread of poliovirus importations in areas and countries where polio is not endemic. ( info) |