Discussion
Ataxia-telangiectasia (A-T) is a genetic neurodegenerative disorder described by several problems such as ataxia, telangiectasis, failure in motor ability, unusual eye movement, immune deficiency, pulmonary disorder, and susceptibility to hematologic malignancies due to the mutation in the ATM gene on the chromosome 11q22.23 (1, 2, 12). Infectious complications play indispensible role in the morbidity and mortality of the disease (6). The etiology of some hematologic disorders in A-T patients may be related to immune disorders such as thrombocytopenia. Furthermore, inflammation participates in the pathogenesis of the disease. It is demonstrated that inflammatory cytokines had the increased levels in A-T patients (13). These increased levels can be involved in inflammatory diseases such as suppurative rhinitis, gastroenteritis, acute otitis media, and parotiditis which were observed in our case. One of the characterizations of A-T is cerebellar neurodegeneration (14). In our patient, nervous system disorders were included ataxia and ocular nystagmus. In this respect, it is stated that the neurogenesis kinetics in A-T are changed. TheATM gene encodes a protein kinase which participates in the activation of cellular responses to DNA double-strand breaks and neural stability (15, 16). Hence, it is likely that these functions are disrupted by ATM mutation. The majority of A-T patients have immune problems such as defects in the productions and/or functions of IgA, IgG, and reduction in the number of CD4+ T cells. Moreover, it is reported that few A-T patients have HIgM syndrome or different viral and opportunistic infections (2). However, our case has chickenpox, zona, and HIgM syndrome with a significant frequency of CD4+ cells which the reason of this occurrence is not clearly known. Others have indicated that pulmonary disorders in A-T may be contributed to neurological disorders, regressive infectious, and lung disorder (2).