Infantile Neuronal Ceroid Lipofuscinosis (Paperback)

Please note that the content of this book primarily consists of articles available from Wikipedia or other free sources online. INCL, also known as Infantile Neuronal Ceroid Lipofuscinoses or Santavuori Diseaseis a form of NCL and inherited as a recessive autosomal genetic trait. The disorder is progressive, degenerative and fatal, extremely rare worldwide - with approximately 60 official cases reported by 1982, perhaps 100 sufferers in total today - but relatively common in Finland due to the local founder effect. The development of children born with INCL is normal for the first 8-18 months, but will then flounder and start to regress both physically and mentally. Motor skills and speech are lost, and optic athropy causes blindness. A variety of neurological symptoms, such as epilepsy and myoclonic seizures, appear. The senses of hearing and touch remain unaffected, and sufferers have been known to enjoy music. The average lifespan of an INCL child is 9-11 years.