Dennie-Marfan syndrome
Encyclopedia
Dennie–Marfan syndrome is a syndrome
in which there is association of spastic paraplegia of the lower limbs and mental retardation
in children with congenital syphilis
. Both sexes are affected, and the onset of the disease can be acute or insidious, with slow progression from weakness to quadriplegia
. Epilepsy
, cataract
, and nystagmus may be also be found.
The syndrome was described by Charles Clayton Dennie
in 1929, and Antoine Marfan
in 1936.
Syndrome
In medicine and psychology, a syndrome is the association of several clinically recognizable features, signs , symptoms , phenomena or characteristics that often occur together, so that the presence of one or more features alerts the physician to the possible presence of the others...
in which there is association of spastic paraplegia of the lower limbs and mental retardation
Mental retardation
Mental retardation is a generalized disorder appearing before adulthood, characterized by significantly impaired cognitive functioning and deficits in two or more adaptive behaviors...
in children with congenital syphilis
Congenital syphilis
Congenital syphilis is syphilis present in utero and at birth, and occurs when a child is born to a mother with secondary syphilis. Untreated syphilis results in a high risk of a bad outcome of pregnancy, including mulberry molars in the fetus. Syphilis can cause miscarriages, premature births,...
. Both sexes are affected, and the onset of the disease can be acute or insidious, with slow progression from weakness to quadriplegia
Quadriplegia
Tetraplegia, also known as quadriplegia, is paralysis caused by illness or injury to a human that results in the partial or total loss of use of all their limbs and torso; paraplegia is similar but does not affect the arms...
. Epilepsy
Epilepsy
Epilepsy is a common chronic neurological disorder characterized by seizures. These seizures are transient signs and/or symptoms of abnormal, excessive or hypersynchronous neuronal activity in the brain.About 50 million people worldwide have epilepsy, and nearly two out of every three new cases...
, cataract
Cataract
A cataract is a clouding that develops in the crystalline lens of the eye or in its envelope, varying in degree from slight to complete opacity and obstructing the passage of light...
, and nystagmus may be also be found.
The syndrome was described by Charles Clayton Dennie
Charles Clayton Dennie
Charles Clayton Dennie was an American dermatologist remembered for Dennie-Marfan syndrome and Dennie-Morgan fold.- External links :...
in 1929, and Antoine Marfan
Antoine Marfan
Antoine Bernard-Jean Marfan was a French pediatrician. He was born in Castelnaudary to Antoine Prosper Marfan and Adélaïde Thuries....
in 1936.