Batten's Disease

Topics: Batten disease, Neuronal ceroid lipofuscinosis, Stem cell Pages: 7 (2367 words) Published: December 13, 2012
Batten's Disease

People cease to exist each and every day. With every passing minute, over one hundred people breathe their last and depart from this world. Death comes by many means: deprivation of food, war, epidemic, disease, and old age. Diseases are accountable for death in many people. A prominent family of ailments is neurological disorders. Batten disease is one subset of neurological disorders. Battens assaults its victims in the childhood years and eventually takes their lives as well.

Batten disease is relatively anomalous; thus, abounding measures of people do not know the explanation or history of the disorder. Batten disease is classified as a fatal autosomal recessive condition. It is the most prevalent form of the category of disorders recognized as Neuronal Ceroid Lipofuscins, which are routinely referred to as NCLs. The overall ramifications of the disease are neurodegenerative issues with the patients (Batten Disease, 1 of 6). In 1903, the first reported case was unearthed when Dr. Fredrick Batten researched and eventually discovered the disorder. The disease was entitled Batten disease in honor of the scientist (Batten Disease, 2 of 6).

There are multiple types of Batten disease; moreover, infantile Battens is one specimen. The most habitual time for this form to commence is amid the ages of six months and two years. Expeditious progression is a major attribute of this infantile Battens. Growth problems like Microcephaly, or an underdeveloped brain and a small head, are also customary. Sharp, concise muscle contractions -Myoclonic jerks- are conventional in infantile Battens. Although some sufferers of infantile Batten disease endure the ailment until approximately six years of age, a considerable amount of patients only live to the age of five (Batten Disease, 2 of 6). Alternative types of Batten disease, such as late infantile Batten disease, manifest later in the victim’s life.

Late infantile Batten disease generally originates two to four years subsequent to birth. Ataxia affects the majority of late infantile Batten disease sufferers. This means that the patients undergo a deprivation of muscle coordination. Seizures with no response to anti-convulsion drugs are also perceptible. Late infantile Batten disease has an accelerated development, and the victims most commonly have a lifespan of eight to twelve years (Batten Disease, 2 of 6). While late infantile Battens acts upon youthful children, juvenile Batten disease assaults children at an older age.

When the patient matures to be between the ages of five and ten, juvenile Batten disease initiates its onset. Certain symptoms are associated with this form of Battens. Vision loss is very rife; moreover, blindness is the ultimate conclusion later in the development. Seizures, although less common, are apparent as well. An additional symptom of juvenile Batten disease that plagues children is motor disruptions transpiring late in the disease. This type of Battens usually shortens the lifespan of the sufferer into the advanced teenage years and early twenties. This form is the most frequent type of Batten disease (What Is Batten Disease?, 1 of 3). Along with juvenile Batten disease, adult Batten disease is another type of this disorder.

Adult Battens develops in patients that are under the age of 40 while the others all occur in the early stages of life. It is also the rarest type of the disease. Gradual progression is a dominant characteristic. Adult Battens has more moderate symptoms than the other forms. A major example of this is that blindness does not affect patients with adult Battens. A shortened lifespan is apparent as well; however, the stolen years differ from case to case. This author was astonished that all forms of Batten disease shorten the lifespan of all sufferer to some degree (Batten Disease, 2 of 6).

Understanding Batten disease requires one to examine the causes of the ailment...
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