Neurology: Case of the Month

Test Yourself — Patient 71

Neuronal Ceroid Lipofuscinosis

1. Which of the following is NOT a hallmark clinical sign/symptom of neuronal ceroid lipofuscinosis (NCL)?
   • [ A ] Visual loss
   • [ B ] Seizures
   • [ C ] Psychomotor retardation
   • [ D ] Hearing loss
2. All of the neuronal ceroid lipofuscinoses (NCLs) are inherited in an autosomal recessive manner.
   • [ A ] True
   • [ B ] False
3. Which of the following associations is LEAST likely to occur?
   • [ A ] Adult-onset NCL associated with visual loss
   • [ B ] Tay-Sachs disease associated with visual loss
   • [ C ] Late-infantile NCL associated with a photoconvulsive response
   • [ D ] Infantile NCL associated with seizures
4. Which of the following features was not originally used to classify the neuronal ceroid lipofuscinosis (NCL) subtypes?
   • [ A ] Age of onset
   • [ B ] Clinical course
   • [ C ] Electron microscopic appearance of deposits
   • [ D ] Seizure type

 

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