What Is a Cone Dystrophy?

Pyramid dysfunction syndrome, also known as congenital panchromatic blindness, is a comprehensive symptom characterized by congenital color blindness, amblyopia, shame, and nystagmus. It is often neglected in infancy and is often detected by parents in early childhood. It is often easily misdiagnosed as optic nerve atrophy, macular degeneration, congenital nystagmus, amblyopia and snoring. There were also nystagmus and photophobia within months of life.

Etiology of Pediatric Pyramid Dysfunction Syndrome

The cause of pyramidal dysfunction syndrome is unknown.

Pediatric pyramidal dysfunction syndrome examination

Electroretinogram

Patients with this disease may have a myopic electroretinogram disappeared, but a scotopic electroretinogram is generally normal.

Visual evoked potential

The initial response to visual evoked potential disappears or is delayed in patients with this disease

3. Initial curve of dark adaptation curve (cone function)

There are many changes, and the final threshold (rod function) is mostly dark adaptation of normal patients, which is more agile than ordinary people.

Diagnosis of pyramidal dysfunction syndrome in children

Diagnosis can be made based on the above clinical manifestations.

Differential diagnosis of pyramidal dysfunction syndrome in children

From the development of the disease, the syndrome can be divided into two categories, identified as follows:

1. Resting pyramidal dysfunction

Lesions are congenital and do not develop.

2. Progressive pyramidal dysfunction

Most scholars believe that the second type may be a new type of hereditary retinal dysfunction due to acquired, selective, and progressive pyramidal dysfunction. It is also called progressive pyramidal degeneration or progressive pyramidal dystrophy. Its clinical manifestations are basically the same as those of the first category, but it has good fixation ability, mostly without nystagmus, previous vision and color vision functions are normal, and the lesions are progressive.

Children with pyramidal dysfunction syndrome complications

Difficulty moving due to poor vision and color vision disorders.

Pediatric pyramidal dysfunction syndrome treatment

Pyramid dysfunction syndrome is a congenital and hereditary disease, so there is no effective treatment method. Wearing colored glasses to reduce strong light stimulation, in addition to reducing photophobia symptoms, some patients can still slightly improve vision.