{"id":3163,"date":"2018-02-21T22:28:55","date_gmt":"2018-02-21T16:58:55","guid":{"rendered":"https:\/\/healthool.com\/?p=3163"},"modified":"2022-01-12T13:35:55","modified_gmt":"2022-01-12T08:05:55","slug":"batten-disease","status":"publish","type":"post","link":"https:\/\/healthool.com\/batten-disease\/","title":{"rendered":"Batten Disease"},"content":{"rendered":"

What is Batten disease<\/h2>\n

A batten disease is a genetic disorder of the nervous system. It belongs to the group of disorder called Neuronal Ceroid Lipofuscinoses (NCL).<\/p>\n

Medical practitioners used the term Batten disease to describe all forms of Neuronal Ceroid Lipofuscinoses.<\/p>\n

There are four types of NCL and they are the following:<\/em><\/p>\n

    \n
  1. Congenital NCL \u2013<\/strong> It is the severe form of NCL characterized by severely small head and seizures. The chance of survival is extremely low. Babies born with congenital NCL die soon after birth. (1, 2<\/strong>)<\/sup><\/li>\n
  2. Infantile NCL \u2013<\/strong> The clinical manifestations start to appear between six months and 2 years of age. The progression of symptoms is rapid that the patient usually dies before the age of 5. Patients with infantile NCL have an extremely small head (microcephaly), growth is altered, and have myoclonic jerks. (1, 2, 3<\/strong>)<\/sup><\/li>\n
  3. Late infantile NCL \u2013<\/strong> It affects children between 2 to 4 years old. The clinical manifestations include ataxia (loss of muscle coordination) and seizures. The patient usually dies between the ages 8 and 12 years old. (3, 4<\/strong>)<\/sup><\/li>\n
  4. Adult NCL \u2013<\/strong> The progression of the condition is slow and the symptoms are mild. The clinical manifestations begin before the patient reaches 40 years old. (4<\/strong>)<\/sup><\/li>\n<\/ol>\n

    Batten Disease Pictures<\/h2>\n

    \"Children<\/p>\n

    Image 1: Children living with a Batten disease.
    \nPicture Source: www.primehealthchannel.com<\/p>\n

    \"A<\/p>\n

    Photo 2: A baby with an infantile batten disease condition.
    \nPicture Source: homepages.ihug.co.nz<\/p>\n

    \"Children<\/p>\n

    Image 3: Children with late infantile batten disease.
    \nPicture Source: bigtennetworks.files.wordpress.com<\/p>\n

    What are the symptoms of Batten’s Disease?<\/h2>\n

    The symptoms primarily depend on the type of Batten disease. Each type has distinct clinical manifestation, which sets it apart from the rest. Doctors carefully and thoroughly observe the signs and symptoms as they used them to differentiate one type to another.<\/p>\n

    However, there are classic signs and symptoms to watch out for. They are the following:<\/p>\n