Ataxia-Telangiectasia Complicated by Craniopharyngioma – A New Observation

Ataxia-telangiectasia is a rare autosomal recessive neurodegenerative disorder with high incidence of malignancy including leukemias, lymphomas, and solid tumors. Central nervous system tumors in ataxia telangiectasia include medulloblastomas and gliomas. We describe a 13-year-old girl with ataxia t...

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Bibliographic Details
Published in:Pediatric neurology 2006-10, Vol.35 (4), p.287-288
Main Authors: Masri, Amira T., Bakri, Faris G., Al-Hadidy, Azmy M., Musharbash, Awni F., Al-Hussaini, Maisa
Format: Article
Language:English
Subjects:
Adolescent
Ataxia Telangiectasia - complications
Ataxia Telangiectasia - diagnosis
Ataxia Telangiectasia - genetics
Biological and medical sciences
Brain - pathology
Consanguinity
Craniopharyngioma - complications
Craniopharyngioma - diagnosis
Craniopharyngioma - genetics
Craniopharyngioma - surgery
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
Experimental and animal immunopathology. Animal models
Fatal Outcome
Female
Humans
Hypophysectomy
Immunopathology
Lymphoma, Non-Hodgkin - complications
Lymphoma, Non-Hodgkin - diagnosis
Lymphoma, Non-Hodgkin - genetics
Magnetic Resonance Imaging
Medical sciences
Neoplasms, Multiple Primary - complications
Neoplasms, Multiple Primary - diagnosis
Neoplasms, Multiple Primary - genetics
Neurologic Examination
Neurology
Pituitary Neoplasms - complications
Pituitary Neoplasms - diagnosis
Pituitary Neoplasms - genetics
Pituitary Neoplasms - surgery
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Description
Summary:Ataxia-telangiectasia is a rare autosomal recessive neurodegenerative disorder with high incidence of malignancy including leukemias, lymphomas, and solid tumors. Central nervous system tumors in ataxia telangiectasia include medulloblastomas and gliomas. We describe a 13-year-old girl with ataxia telangiectasia who developed craniopharyngioma and non-Hodgkin’s lymphoma. To our knowledge, this is the first case of ataxia telangiectasia complicated by craniopharyngioma in the English literature.
ISSN:0887-8994
1873-5150
DOI:10.1016/j.pediatrneurol.2006.05.011