Ataxia-telangiectasia in a child with vaccine-associated paralytic poliomyelitis

Immunodeficiency and infections in ataxia-telangiectasia

“…These data are consistent with previously published reports. We are aware of only 1 patient with A-T who developed vaccine-acquired poliomyelitis, 34 1 patient with atypical A-T with severe failure to thrive who died from P jerovici pneumonia at age 15 months, 35 and 1 patient who died from disseminated herpes simplex virus infection. 36 Most patients with A-T have laboratory evidence of immune dysfunction, but systemic bacterial and opportunistic infections are uncommon.…”

Neurological sequelae of the dancing eye syndrome

“…In addition elevation of CSF interferon levels have been reported in non-malignancy associated cases [26]. Recently polymerase chain reaction studies have been used to attempt to look for chronic viral encephalitis by detecting small quantities of viral antigenic material in children with other neurological disorders [37]. This technique has not been applied to cases of DES.…”

Ataxia‐telangiectasia: recommendations for multidisciplinary treatment

“…81,82 Immunoglobulin replacement therapy Immunoglobulin replacement therapy, aiming at a serum immunoglobulin-G >6g/L, is indicated in all patients with a severe immunoglobulin-G deficiency (serum immunoglobulin-G <4g/L) 65,66 and in those with HIGM phenotype; it should also be considered for patients with milder humoral immune defects, recurrent infections, or low specific antibody responses despite booster immunizations. 76,77,[79][80][81][82][83] Inactivated vaccines are safe in ataxia-telangiectasia and are recommended as part of routine childhood vaccination programmes, except for patients who are on immunoglobulin replacement therapy. immunoglobulin-G 2 ) with normal specific polysaccharide antibody responses, immunoglobulin replacement therapy is not indicated.…”

“…76 Live oral polio vaccine should not be administered. 76,77,[79][80][81][82][83] Inactivated vaccines are safe in ataxia-telangiectasia and are recommended as part of routine childhood vaccination programmes, except for patients who are on immunoglobulin replacement therapy. Annual vaccination with inactivated influenza vaccine is recommended for all patients, including those receiving immunoglobulin replacement therapy.…”