Prepare for the American Board of Professional Psychology (ABPP) Exam with flashcards and multiple-choice questions. Each question includes insights and explanations to help you excel. Get ready for your certification journey!

Multiple Choice

Which disorder features telangiectasias on the bridge of the nose and cheeks, onset by age 3–5, cerebellar vermis degeneration causing progressive gait ataxia, and immunodeficiency?

This pattern reflects a neurodegenerative-immunodeficiency syndrome where facial telangiectasias appear early, accompanied by progressive ataxia due to cerebellar vermis degeneration and immune dysfunction. The nasal bridge and cheek telangiectasias in a child around ages 3–5, together with a worsening gait that points to cerebellar involvement, and a tendency toward infections from immune deficiency, are the hallmarks of this condition. The underlying cause is an ATM gene mutation that impairs DNA repair, leading to neurodegeneration, immunoglobulin deficits (often IgA), and elevated alpha-fetoprotein levels. This combination is characteristic of Ataxia-telangiectasia, and does not align with Rett, Williams, or Prader-Willi syndromes, which have different primary features such as regression with hand-wringing, unique cardiovascular/cognitive profiles, or hypotonia and hyperphagia, respectively.

This pattern reflects a neurodegenerative-immunodeficiency syndrome where facial telangiectasias appear early, accompanied by progressive ataxia due to cerebellar vermis degeneration and immune dysfunction. The nasal bridge and cheek telangiectasias in a child around ages 3–5, together with a worsening gait that points to cerebellar involvement, and a tendency toward infections from immune deficiency, are the hallmarks of this condition. The underlying cause is an ATM gene mutation that impairs DNA repair, leading to neurodegeneration, immunoglobulin deficits (often IgA), and elevated alpha-fetoprotein levels. This combination is characteristic of Ataxia-telangiectasia, and does not align with Rett, Williams, or Prader-Willi syndromes, which have different primary features such as regression with hand-wringing, unique cardiovascular/cognitive profiles, or hypotonia and hyperphagia, respectively.