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Department of Neurology

Houston, Texas

BCM neurologists see patients through the Baylor Clinic and some of the world's leading specialty clinics.
Department of Neurology
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Test Yourself — Patient #71

Neuronal Ceroid Lipofuscinosis

  1. Which of the following is NOT a hallmark clinical sign/symptom of neuronal ceroid lipofuscinosis (NCL)?

    Visual loss
    Seizures
    Psychomotor retardation
    Hearing loss

  2. All of the neuronal ceroid lipofuscinoses (NCLs) are inherited in an autosomal recessive manner.

    True
    False

  3. Which of the following associations is LEAST likely to occur?

    Adult-onset NCL associated with visual loss
    Tay-Sachs disease associated with visual loss
    Late-infantile NCL associated with a photoconvulsive response
    Infantile NCL associated with seizures

  4. Which of the following features was not originally used to classify the neuronal ceroid lipofuscinosis (NCL) subtypes?

    Age of onset
    Clinical course
    Electron microscopic appearance of deposits
    Seizure type

 

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