Neurologic Involvement in Primary Immunodeficiency Disorders

Yildirim M., Ayvaz D. N., Konuskan B., GÖÇMEN R., Tezcan I., TOPÇU M., ...Daha Fazla

JOURNAL OF CHILD NEUROLOGY, cilt.33, sa.5, ss.320-328, 2018 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 33 Sayı: 5
  • Basım Tarihi: 2018
  • Doi Numarası: 10.1177/0883073817754176
  • Dergi Adı: JOURNAL OF CHILD NEUROLOGY
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Sayfa Sayıları: ss.320-328
  • Anahtar Kelimeler: primary immune deficiency, immunosuppression, agammaglobulinemia, neurologic, nervous system, NUCLEOSIDE PHOSPHORYLASE-DEFICIENCY, PATIENT, MUTATIONS, TRANSPLANTATION, ABNORMALITIES, AUTOIMMUNITY, DISEASES, LRBA
  • Hacettepe Üniversitesi Adresli: Evet

Özet

The nervous system may be affected in primary immune deficiency (PID) syndromes through infectious, autoimmune, neoplastic mechanisms, or as a primary feature of the syndrome. However certain neurologic problems do not conform to these etiopathogenetic groups. We retrospectively examined PID patients seen in neurology consultation between 2014 and 2017 in order to determine the spectrum of nervous system involvement. Among patients with confirmed neurologic problems (n = 35), common manifestations were encephalopathy and global developmental/cognitive delay. In 13 (37%) instances, the neurologic signs had no apparent relation with a treatment-related, infectious, or vascular complication and were considered as primary findings: acquired microcephaly, central nervous system malformation, or peripheral neuropathy. The diagnosis of PID was made after, and based on, the neurologic manifestation in 6 of 35 (17%) patients. Neurologic presentation may constitute the initial manifestation in some types of primary immune deficiency.