Evaluation of QT Dispersion in Children with Breath Holding Spells

How to Cite This Article: Movahedian AH, Heidarzadeh Arani M, Motaharizad M, Mousavi GhA, Mosayebi Z. Evaluation of QT Dispersion in Children with Breath Holding Spells. Iran J Child Neurol. Winter 2016; 10(1):25-30.AbstractObjectiveBreath holding spells (BHS) are common involuntary reflexes in infancy and early childhood. Differential diagnosis should embrace Long QT Syndrome (LQTS) and paroxysmal abnormalities of rhythm. The aim of this study was to compare QT dispersion (QTd) in children with breath holding spells and normal controls.Materials & MethodsQT dispersion and Corrected QT(QTc) dispersion were measured in 12 lead surface electrocardiograms in 56 patients with BHS and compared with healthy children of the same age referred to the clinic for regular checkup visits.Results The most common type of BHS was cyanotic (83.9%). Seven patients (12.5%) had pallid and two patients (3.5%) had mixed spells. There was a history of breath holding spells in 33.9% of the children. QT dispersion was 61.6± 22.5 and 47.1±18.8 ms in patient and control groups, respectively. QTc dispersion (QTcd) was 104 ± 29.6 and 71.9 ±18.2 ms, respectively. There was a significant difference between patient and control groups in terms of QTd and QTcd (P<0.001).ConclusionQTd and QTcd were increased in children with BHS. Therefore, the evaluation of EKG for early diagnosis of rhythm abnormalities seems reasonable in these children

Atypical Ataxia Presentation in Variant Ataxia Telangiectasia: Iranian Case-Series and Review of the Literature

Ataxia-telangiectasia (AT) is a rare autosomal recessive neurodegenerative multisystem disorder. A minority of AT patients can present late-onset atypical presentations due to unknown mechanisms. The demographic, clinical, immunological and genetic data were collected by direct interview and examining the Iranian AT patients with late-onset manifestations. We also conducted a systematic literature review for reported atypical AT patients. We identified three Iranian AT patients (3/249, 1.2% of total registry) with later age at ataxia onset and slower neurologic progression despite elevated alpha-fetoprotein levels, history of respiratory infections, and immunological features of the syndrome. Of note, all patients developed autoimmunity in which a decrease of naïve T cells and regulatory T cells were observed. The literature searches also summarized data from 73 variant AT patients with atypical presentation indicating biallelic mild mutations mainly lead to an atypical phenotype with an increased risk of cancer. Variant AT patients present with milder phenotype or atypical form of classical symptoms causing under- or mis- diagnosis. Although missense mutations are more frequent, an atypical presentation can be associated with deleterious mutations due to unknown modifying factors