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Childhood Disintegrative Disorder: A Rare Neurobiological Disorder

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Childhood disintegrative is a rare yet severe condition in which the child lacks or has a sudden drop in basic rudimentary skills.

Medically reviewed by

Dr. Rakesh Radheshyam Gupta

Published At September 7, 2023
Reviewed AtApril 1, 2024

Introduction:

Each receptive children are distinct, portraying unique characteristic traits. Some children learn stuff and stories quickly, while others possess a slow pace. So, those little ones seem lagging and need to be better combined with their peers. Subsequently, they might have difficulties following the daily routine. Cognitive health is to look at. Childhood disintegrative disorder might possess developmental delays. And the condition is rare, priming two out of 100,000 children globally.

What Is Childhood Disintegrative Disorder?

Childhood disintegrative disorder is a rare illness characterized by almost loss or retrogradation in the skills that were acquired previously. The skills like communication, social and behavioral are regressed as it has a late age of onset. The disorder is also known as Heller’s syndrome or disintegrative psychosis. Childhood disintegrative disorder has been merged into Autism spectrum disorder in accordance with the Diagnostic and Statistical Manual of Mental (DSM) Disorders, fifth edition. This is due to the symptoms of both conditions seem relevant.

How Does Childhood Disintegrative Disorder Differ From Autism?

Childhood disintegrative disorder and autism are distinct conditions, though they share certain core features and symptoms. However, childhood disintegrative disorder possesses severe impairments in comparison with autism. These impairments include severe epilepsy and lower intelligence quotient (IQ). Moreover, differences in childhood disintegrative disorders include more cognitive declines, faster regression, and higher developmental deficits.

What Causes Childhood Disintegrative Disorder?

The precise causes of childhood disintegrative disorder are still undisclosed. But, the researchers have identified certain risk factors stimulating the condition.

  • Family history of epilepsy, childhood disintegrative disorder, or autism.

  • Birth injury or trauma.

  • Premature birth.

  • Severe viral infections.

  • Exposure to toxic substances.

  • Chromosomal abnormalities or disruption.

In addition, childhood disintegrative disorder might co-occur with the succeeding conditions in people with late onset.

  • Epilepsy.

  • Lipid storage diseases (storage of excess toxic lipids or fat in the brain and nervous system).

  • Tuberous sclerosis (a cancerous genetic condition in which the tumor develops in the brain. Other tumor sites incorporate kidneys, lungs, heart, eyes, and skin).

  • Subacute sclerosis panencephalitis (in this condition, measles viruses cause infection, leading to brain inflammation). In addition, it damages the nervous system and nerve cells.

  • Leukodystrophy (the myelin sheath, an outer covering of the nerve, fails to develop in this illness, so the nerve cells are not reinforced enough).

What Are the Signs and Symptoms of Childhood Disintegrative Disorder?

The child remains physically and mentally fit, exhibiting no cognitive impairments. The child has healthy social, communication, and comprehension skills like other kids until the child meets two years. Hence, the period between two and ten years is the predominant one to experience the signs and symptoms. The child lacks a minimum of two skills of six themes, as demonstrated below.

  • Receptive language skills.

  • Expressive language skills.

  • Social and self-care skills.

  • Controlling bowel and bladder.

  • Motor skills.

  • Play skills.

Over and above that, the child lacks specific basic skills too. The impairment is also seen in at least two of the following skills.

  • Socialization.

  • Communication.

  • Restricted repetitive acts and behavior.

Features of Childhood Disintegrative Disorder:

Therefore, the child encountering disintegrative disorder has a deficit of rudimentary skills. And their conduct relies on these characteristics.

  • The onset of symptoms may be either abrupt or gradual.

  • The child will begin to have regrets about their conduct as the illness progresses.

  • They will lose the phrases or quest for words to communicate, so they can not even frame a sentence of three words.

  • Parents and caregivers could not notice social, verbal, language, self-care, emotional, and behavioral impairments before two years.

  • The child avoids hugs and cuddles while developing emotional and communal issues.

How Is Childhood Disintegrative Disorder Diagnosed?

Unfortunately, no such appropriate tool or equipment can diagnose the condition. But, medical care professionals review the child's history, family history, and symptoms. They will rule out the behavior changes through a complete clinical and mental examination. Therefore, specific tests and laboratory investigations will also be advised to look for underlying conditions or illnesses. An electroencephalogram, EEG, computerized tomography (CT), and magnetic resonance imaging (MRI) might be included as the childhood disintegrative disorder has a few subsequent differential diagnoses.

  • Brain tumor.

  • Mercury or lead poisoning.

  • Schizophrenia.

  • Seizure disorder.

  • Hypothyroidism.

  • Organophosphate exposure.

How Is Childhood Disintegrative Disorder Treated?

The child needs to gain the skills required for daily functioning, academics, and the entity. So, the childhood disintegrative condition can be controlled effectively with appropriate intervention and long-term palliative care. The succeeding intervention manages the symptoms as they are directed primarily at eventually relieving them by improving the rudimentary skills.

Pharmacological Drugs: Regarding the medications, no exact drug will work for the disintegrative disorder itself. However, certain drugs are prescribed to treat severe uncontrolled symptoms.

  • Stimulants, antipsychotics, and antidepressants like selective serotonin reuptake inhibitors for behavioral problems and aggression.

  • Antiepileptic drugs if in case of seizure episodes.

Behavioral Therapy: The intervention leans on how our thoughts reframe into actions. In a precise manner, how our moods and beliefs affect our behavior. So the therapist primarily works on the child’s negative beliefs as it would eventually reflect in one’s skills.

  • According to the American Academy of Pediatrics, Applied Behavior Analysis, ABA is regarded as an effective intervention for autism. So, it intends to bring about positive reinforcement, alleviate self-aggression and improve the overall quality of life.

  • Cognitive behavioral therapy, CBT, is also utilized to convert unhelpful thoughts into positive ones.

Environmental Therapy: Environmental treatment aims to treat physical and cognitive issues by detoxicating. Specifically, sensory enrichment therapy brings wonder over symptoms.

Prognosis: The outlook and prognosis seem nearly poor as the disorder requires long-term support that may continue lifelong. And in some cases, epileptic seizures are more presumably to develop. Subsequently, the adaptive skills may get worsen, leading to intellectual impairments.

Conclusion:

Childhood disintegrative disorder is a condition characterized by a lack of skills. The disorder is identified with the reversal of assimilated skills. It can be addressed and treated effectively with appropriate therapies. Parents, teachers, or caretakers ensure that to provide the right guidance with genuine care. And, yes, the skills can be retrieved to strive and thrive.

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Dr. Rakesh Radheshyam Gupta
Dr. Rakesh Radheshyam Gupta

Child Health

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