Heller’s Syndrome (also known as (CDD) Childhood Disintegrative Disorder and disintegrative psychosis) is an extremely rare syndrome. Incidence is only one per 50,000 children, about sixty times less frequent than autism. However, research often questions the prevalence due to the similarity between Heller’s Syndrome and autism causing under-diagnosis / misdiagnosis.
The characteristics and clinical presentation is very similar to autism…so similar that Heller’s syndrome has been seen by some as a form of late-onset autism with its own distinct etiology. Other researchers have considered the condition to be a childhood dementia because they believe that the brain deposition of amyloid is the cause of the condition. However, the pathophysiology of that theory has yet to be proven.
The syndrome was first identified by Theodore Heller, an Austrian educator, in 1908, 35 years before Leo Kanner first described autism.
Unlike autism, those with Heller’s Syndrome have normal development for the first 3-4 years of life. There is a sudden, unforeseen and marked deterioration of physical, cognitive, emotional, and behavioral ability. This loss may be gradual or grossly immediate. The effective behavior is specifically similar to the social interaction and “autistic -like” restrictive and repetitive communication and language impairments of children with autism. Autistic children never develop that normal skill set to lose it.
The aftermath of Heller’s syndrome can take several paths. Most children have a slight recovery period, where some skills such as: continence, language, and social interaction skills make some recovery. Other cases involve the child’s loss of skill ending, but there is no improvement or re-learning of lost skills. A few cases involve the continual progression of loss of skill, where the child has a high probability of death.
There is not a single captive factor known. Recent research focuses on a combination of genetic susceptibility and prenatal / environmental stress causing greater brain deposition of amyloid and disrupting synaptic transmission.