What is it?
What is Schilder’s disease?
- Schilder’s disease is a very rare, progressive, degenerative, demyelinating disorder of the central nervous system (CNS) that usually begins in childhood. It resembles multiple sclerosis (MS) both in some of its symptoms and its pathology (widespread demyelination of the brain). Because there are no specific criteria for diagnosis, there is debate about the most appropriate way to definitively diagnose the disease. Some suggest that there have been only 9-28 cases of definitively diagnosed Schilder’s disease since it was first described in 1912 by Paul Schilder.
- Schilder’s disease is thought to be a variant of MS.
- As the disease progresses, larger and larger patches of demyelination occur, interfering with motor movement, speech, personality, hearing and vision, ultimately affecting the vital functions of respiration, heart rate, blood pressure.
- Note: Schilder’s disease is not the same as Addison-Schilder disease (adrenoleukodystophy).
What other terms are sometimes used to refer to Schilder’s disease?
- Diffuse cerebral sclerosis
- Diffuse cerebral sclerosis of Schilder
- Myelinoclastic diffuse sclerosis
Is there another disease that contains the name Schilder that is sometimes confused with Schilder’s disease?
- Yes, Addison-Schilder disease (adrenoleukodystrophy), ), a rare inherited disease characterized by a biochemical abnormality in the myelin.
What causes Schilder’s disease?
- The underlying cause of Schilder’s disease is unknown. Schilder’s disease often occurs shortly after an infectious illness and may begin with headache, a general feeling of discomfort or illness, and fever. Symptoms are caused by widespread patches of demyelination throughout the brain and spinal cord, resulting in slowed transmission of nerve signals.
Who gets Schilder’s disease and when does it appear?
- Schilder’s disease is extremely rare and affects children and young adults, mostly boys. Most patients diagnosed with Schilder’s disease are between the ages of 7 and 12 years of age.
How is Schilder’s disease similar to multiple sclerosis (MS)?
- Schilder’s disease resembles MS in both its pathology (widespread demyelination of the brain) and some of its symptoms (difficulties with movement, speech, memory, among others).