Schilder's Disease
Definition
Schilder's disease is a form of multiple sclerosis that strikes in childhood.
Description
Schilder's disease is a very rare progressive degenerative disease that affects children. It resembles multiple sclerosis both in its symptoms (difficulties with movement and speech) and its pathology (widespread demyelination of the brain). Demyelination refers to the destruction of the myelin that normally encases nerve fibers. Myelin is the fatty white substance that wraps around nerve fibers, providing insulation and allowing nerve signals to move quickly. Without myelin, nervous transmission is significantly slowed. As the disease progresses, larger and larger patches of demyelination occur, interfering with motor movement, speech, personality, hearing and vision. Ultimately, the vital functions (respiration, heart rate, blood pressure) are affected, leading to the individual's death.
Demographics
Schilder's disease is exceedingly rare. Because there are no specific criteria for the diagnosis, there continues to be debate among researchers and clinicians regarding the most appropriate way to definitively diagnose the disease and collect data on its frequency and incidence. Some sources suggest that there have only been nine cases of definitively diagnosed Schilder's disease since it was originally described in the German medical literature in 1912.
Most patients with Schilder's disease are diagnosed between the ages of seven and twelve years of age.
Causes and symptoms
The underlying cause of Schilder's disease is unknown. Symptoms of the disease are caused by widespread patches of demyelination throughout the brain and spinal cord, resulting in slowed, faulty nervous transmission.
Symptoms of Schilder's disease include weakness of one side of the body (hemiparesis), slowness of movement (psychomotor retardation), paralysis of all four extremities (quadraparesis), seizures, difficulty with speech (dysarthria), visual and hearing impairment, irritability, memory problems, personality changes, and gradual loss of awareness and responsiveness. Over time, patients become unable to maintain their nutritional status and become increasingly thin and malnourished. Bowel and bladder function are often lost as the disease progresses.
Some children have a relentlessly progressive course of the disease, culminating in death. Other children have remissions and exacerbations, with each subsequent exacerbation more severe and each remission less complete, until death supervenes.
