Which of the following is true about inverse Retinitis pigmentosa?
Ans. (a) X linkedRef: A.K. Khurana 6th ed. /287INVERSE RETINITIS PIGMENTOSA* Inverse RP is a degeneration of the nerve cells in the macula, which is located in the center of the retinal. Inverse or central RP is characterized by a bilateral and often symmetrical loss of cone function in the presence of a reduced rod function. Individuals with macular degeneration secondary to a central vision loss experience difficulty reading, recognizing faces, shapes and contours of objects or things.* This is a general term for a disparate group of rod- cone dystrophies characterized by progressive night blindness, visual field constriction with a ring scotoma/ tubular vision, loss of acuity, waxy pallow optic disc, and an abnormal electroretinogram (ERG).* It occurs sporadically or in an autosomal recessive, dominant, or X-linked pattern.* MC inheritance pattern: Autosomal Recessive* Irregular black deposits of clumped pigment in the peripheral retina, called hone spicules because of their vague resemblance to the spicules of cancellous bone, give the disease its name.* No definite treatment. However, Vitamin A can slow down the progression.
X linked
Bony spicule in fovea
Bony spicule in para fovea
Progressive Choroid degeneration
Congenital and Developmental Disorders