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Spielmeyer-Vogt Disease



Spielmeyer-Vogt Disease Causes


Spielmeyer-Vogt disease can be inherited in an autosomal recessive pattern. Six genes have now been defined to cause Spielmeyer-Vogt disease both in children and adults.


Spielmeyer-Vogt Disease Definition


Spielmeyer-Vogt disease is a progressive genetic disorder where defective lipid metabolism that causes blindness, neurological deterioration, dementia leading to total incapacitation within years and death within 10-15 years. It is the most common type of a group of disorders called neuronal ceroid lipofuscinosis (NCLs). The disease is also known as Batten disease.


Spielmeyer-Vogt Disease Diagnosis


Diagnosis is based on the amount of deposits present in the skin samples in combination with other symptoms and criteria.


Spielmeyer-Vogt Disease Symptoms and Signs


Early symptoms of Spielmeyer-Vogt disease normally begins to show between the ages of four to ten including the following: Vision problems Seizures Learning delays Echolalia or repeating the words spoken Stumbling or clumsiness Poor circulation in hands and feet Shrinkage of body fat and muscle mass Breath holding periods Spine curvature Constipation Teeth grinding As time pass, children with Spielmeyer-Vogt disease will experience worsening symptoms including progressive loss of sight, mental impairment, and seizures get worse. Additionally, speech and motor skills may be loss gradually.


Spielmeyer-Vogt Disease Treatment


Clinical trials were made to study a gene therapy method and neural stem cell injections in the brain of a suffering individual. Though the two have gained quite favorable results, there is still no established evidence that it can accurately cure Spielmeyer-Vogt disease.


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