Van Gogh Syndrome is seen in
## Core Concept
Van Gogh syndrome, also known as **Norrie disease**, is a rare genetic disorder characterized by severe visual impairment or blindness at birth, often associated with hearing loss and intellectual disability. It is caused by mutations in the **NDP** (Norrie disease protein) gene located on the X chromosome, making it an X-linked recessive disorder.
## Why the Correct Answer is Right
The correct answer, **Norrie disease**, is associated with Van Gogh syndrome because both terms refer to the same condition. Norrie disease is known for its early onset of severe visual impairment, and it often presents with additional symptoms such as hearing loss and psychiatric problems. The name "Van Gogh syndrome" might be used due to the reported psychiatric features and the historical reference to Vincent Van Gogh, who suffered from various mental health issues.
## Why Each Wrong Option is Incorrect
- **Option A:** This option is incorrect because, without specifying the condition, it's impossible to directly link it to Van Gogh syndrome or Norrie disease.
- **Option B:** Similarly, this option lacks specificity and does not directly relate to known conditions associated with Van Gogh syndrome.
- **Option D:** This option is also incorrect for the same reason as options A and B; it does not provide a recognizable condition related to Van Gogh syndrome.
## Clinical Pearl / High-Yield Fact
A key point to remember is that **Norrie disease** is an X-linked recessive disorder, meaning it predominantly affects males, who have only one X chromosome. Female carriers can exhibit milder symptoms or be asymptomatic but can pass the mutated gene to their offspring. Early diagnosis and genetic counseling are crucial for families affected by this condition.
## Correct Answer: C. Norrie disease.