CLINICAL AND LABORATORY OBSERVATIONS
Ataxia-telangiectasia in a child with vaccine-associated paralytic poliomyelitis K. R. E. Pohl, BSc, MBBS, DCH, MRCP(UK), J. D. Farley, MBBS, FRCP(C), J. E. Jan, FRCP(C), a n d A. K. Junker, MD, FRCP(C) From the Departments of Neurology and Pediatrics, University of British Columbia and the British Columbia Centre for Disease Control, Vancouver, British Columbia, Canada
Vaccine-acquired poliomyelitis d e v e l o p e d in a nonimmunized 10-month-old boy. At a g e 4 years, alaxia-telangiectasia was recognized. We conclude that the occurrence of vaccine-related poliomyelitis warrants a detailed assessment of immunity, and that, in patients with ataxia-telangiectasia, the use of live vaccines may be hazardous, even in those with apparently normal immunity. (J PEDIATR1992;121:405-7) C a n a d a currently has an average of about one or two cases per year of paralytic poliomyelitis. It is estimated t h a t one third of the cases are vaccine associated, occurring either in recipients of oral poliomyelitis vaccine or in n o n i m m u n i z e d persons who had contact with vaccine recipients.l W e report on a previously nonimmunized male infant in whom paralytic poliomyelitis developed at the age of 10 months and whose condition was proved to be caused by a vaccine-originated strain of virus, acquired through a c o m m u n i t y contact. H e subsequently was seen by physicians at the age of 4 years with clinical and laboratory features of ataxia-telangiectasia. CASE REPORT At the age of 10 months, this previously healthy boy with an unremarkable birth history and early developmental milestones had a febrile illness with marked coryza, fever, and fretfulness. The diagnosis of a viral illness was made. Six days later it was noted that he was reluctant to use his right leg and had become constipated. There was reduced movement and tone, with absent tendon reflexes in the right lower extremity. Sensation was normal. Radiologic examination of the right leg showed no abnormalities. Progressive painless paralysis of the right leg evolved. A clinical diagnosis of paralytic poliomyelitis was made. Investigations revealed a low peripheral leukocyte count with lymphoeytes 0.575 x 10 9 cells/L (normal, 2.3 to 8.0 X 10 9 cells/L). The cerebrospinal fluid was normal except for a protein level of 1.09
Submitted for publication Feb. 3, 1992; accepted March 25, 1992. Reprint requests: K. R. E. Pohl, MRCP(UK), Neurology Department, British Columbia's Children's Hospital, 4480 Oak St., Vancouver, British Columbia V6H 3V4, Canada. 9/22/38129
gm/L; CSF cultures were sterile. An enterovirus was isolated from the stool, and was identified as poliovirus type III by virus neutralization studies at the provincial laboratory of the British Columbia Centre for Disease Control. Confirmation was undertaken by the Canadian National Viral Division of the Bureau of Biological Health and Welfare. A vaccinelike strain of type III polio virus was indicated by intratypic serodifferentiation with the McBride test and oligonucleotide genetic characterization.2 During the convalescent phase of the poliomyelitis, quantitative IgG, IgM, IgA, and IgE levels were studied and found to be normal. Serologic study indicated IgG antibody to type III (but not I or II) of poliovirus. AT CSF OPV PCR VAPP
Ataxia-telangiectasia Cerebrospinal fluid Oral poliovirus vaccine Polymerase chain reaction Vaccine-associated paralytic poliomyelits
This child had never received poliovirus immunizations. His family belonged to a religious sect of about 600 people. Refusal of all immunizations or delay until school entry was common within this group. The only recognized contact of our patient with a recently immunized child was with a 2-year-old cousin, a regular playmate, who had received a second dose of trivalent OPV 7 weeks before the patient's febrile illness began. At 4 years 6 months of age the patient was referred for orthopedic assessmentof a dislocated right hip and shortening of his right leg as a result of chronic postpoliomyelitissyndrome. Reassessment at that stage revealed a cooperative child with mild motor delay (first noticed at 18 months of age) but no obvious regression. Computed tomography brain imaging had shown no abnormalities at age 2 years. On examination the boy had the striking neurocutaneous stigmata of AT with florid telangiectasia on the face and conjunctivae.
405
406
Clinical and laboratory observations
He had some difficulty speaking and a tendency to drool. He had truncal ataxia and athetoid-like movements of his upper limbs, together with impaired fine motor coordination. He had minimal limb ataxia and no associated nystagmus. A clinical diagnosis of AT was made; it was supported by findings of an elevated serum a-fetoprotein level of 100 #g/L (normal
Ataxia-telangiectasia in a child with vaccine-associated paralytic poliomyelitis K. R. E. Pohl, BSc, MBBS, DCH, MRCP(UK), J. D. Farley, MBBS, FRCP(C), J. E. Jan, FRCP(C), a n d A. K. Junker, MD, FRCP(C) From the Departments of Neurology and Pediatrics, University of British Columbia and the British Columbia Centre for Disease Control, Vancouver, British Columbia, Canada
Vaccine-acquired poliomyelitis d e v e l o p e d in a nonimmunized 10-month-old boy. At a g e 4 years, alaxia-telangiectasia was recognized. We conclude that the occurrence of vaccine-related poliomyelitis warrants a detailed assessment of immunity, and that, in patients with ataxia-telangiectasia, the use of live vaccines may be hazardous, even in those with apparently normal immunity. (J PEDIATR1992;121:405-7) C a n a d a currently has an average of about one or two cases per year of paralytic poliomyelitis. It is estimated t h a t one third of the cases are vaccine associated, occurring either in recipients of oral poliomyelitis vaccine or in n o n i m m u n i z e d persons who had contact with vaccine recipients.l W e report on a previously nonimmunized male infant in whom paralytic poliomyelitis developed at the age of 10 months and whose condition was proved to be caused by a vaccine-originated strain of virus, acquired through a c o m m u n i t y contact. H e subsequently was seen by physicians at the age of 4 years with clinical and laboratory features of ataxia-telangiectasia. CASE REPORT At the age of 10 months, this previously healthy boy with an unremarkable birth history and early developmental milestones had a febrile illness with marked coryza, fever, and fretfulness. The diagnosis of a viral illness was made. Six days later it was noted that he was reluctant to use his right leg and had become constipated. There was reduced movement and tone, with absent tendon reflexes in the right lower extremity. Sensation was normal. Radiologic examination of the right leg showed no abnormalities. Progressive painless paralysis of the right leg evolved. A clinical diagnosis of paralytic poliomyelitis was made. Investigations revealed a low peripheral leukocyte count with lymphoeytes 0.575 x 10 9 cells/L (normal, 2.3 to 8.0 X 10 9 cells/L). The cerebrospinal fluid was normal except for a protein level of 1.09
Submitted for publication Feb. 3, 1992; accepted March 25, 1992. Reprint requests: K. R. E. Pohl, MRCP(UK), Neurology Department, British Columbia's Children's Hospital, 4480 Oak St., Vancouver, British Columbia V6H 3V4, Canada. 9/22/38129
gm/L; CSF cultures were sterile. An enterovirus was isolated from the stool, and was identified as poliovirus type III by virus neutralization studies at the provincial laboratory of the British Columbia Centre for Disease Control. Confirmation was undertaken by the Canadian National Viral Division of the Bureau of Biological Health and Welfare. A vaccinelike strain of type III polio virus was indicated by intratypic serodifferentiation with the McBride test and oligonucleotide genetic characterization.2 During the convalescent phase of the poliomyelitis, quantitative IgG, IgM, IgA, and IgE levels were studied and found to be normal. Serologic study indicated IgG antibody to type III (but not I or II) of poliovirus. AT CSF OPV PCR VAPP
Ataxia-telangiectasia Cerebrospinal fluid Oral poliovirus vaccine Polymerase chain reaction Vaccine-associated paralytic poliomyelits
This child had never received poliovirus immunizations. His family belonged to a religious sect of about 600 people. Refusal of all immunizations or delay until school entry was common within this group. The only recognized contact of our patient with a recently immunized child was with a 2-year-old cousin, a regular playmate, who had received a second dose of trivalent OPV 7 weeks before the patient's febrile illness began. At 4 years 6 months of age the patient was referred for orthopedic assessmentof a dislocated right hip and shortening of his right leg as a result of chronic postpoliomyelitissyndrome. Reassessment at that stage revealed a cooperative child with mild motor delay (first noticed at 18 months of age) but no obvious regression. Computed tomography brain imaging had shown no abnormalities at age 2 years. On examination the boy had the striking neurocutaneous stigmata of AT with florid telangiectasia on the face and conjunctivae.
405
406
Clinical and laboratory observations
He had some difficulty speaking and a tendency to drool. He had truncal ataxia and athetoid-like movements of his upper limbs, together with impaired fine motor coordination. He had minimal limb ataxia and no associated nystagmus. A clinical diagnosis of AT was made; it was supported by findings of an elevated serum a-fetoprotein level of 100 #g/L (normal
