Childhood Disintegrative Disorder: Clinical Features and Diagnostic Criteria

Waleed Ahmad

Published online by MRCPsych UK: Tuesday, 03 May 2022

Introduction

There is a loss of skills in several areas of development and deficits in social, communicative, and behavioural functioning that follow normal development in this condition. Often the condition follows a prodromic period during which children develop obscure symptoms; they become restive, irritable, anxious, and overactive. Impoverishment follows this and then loss of speech and language, accompanied by behavioural disintegration. Sometimes the loss of skills is persistently progressive (especially if there is an underlying progressive neurological condition), but more often, the decline over some months and then a slight improvement. The prognosis is usually abysmal, and it leaves most individuals with severe intellectual disability. There is uncertainty about the extent to which this condition differs from autism. Sometimes, the disorder can be because of some associated encephalopathy, but clinicians should make the diagnosis on the behavioural features. If the condition occurs because of an underlying neurological condition, clinicians should record that separately.

Diagnostic Features

1. Diagnosis requires a healthy development during the first 2 years of life, followed by a loss of skills; qualitatively, abnormal social functioning accompanies this.
2. A profound regression of language, level of play, social skills, adaptive behaviours, and bowel, or bladder control are common.
3. Patients also lose interest in the environment and develop stereotypes, mannerisms, and social, and communication deficits.
4. Unlike dementia, there is no evidence of organic disease, and the lost skills may recover. Thus, the ICD-10 has classified the syndrome as a pervasive developmental disorder, instead of dementia.1

Inclusions and Exclusions

The ICD-10 includes the following under childhood disintegrative disorder:

1. Symbiotic psychosis
2. Heller disease
3. Disintegrative psychosis
4. Dementia infantilis

However, the following are excluded:

1. Selective mutism
2. Schizophrenia
3. Rett disorder
4. Acquired epileptic aphasia

With autistic disorder, Asperger syndrome, and atypical autism, the DSM-5 and ICD-11 classifications have subsumed it under autism spectrum disorders.2-3

About the Author

Waleed Ahmad, consultant psychiatrist at the Department of Psychiatry, Mercy Teaching Hospital, Peshawar, Faculty member at the Department of Psychiatry and Behavioural Sciences, Peshawar Medical College, Peshawar, 25 000, KP, Pakistan. Email: dr.waleed@outlook.com

Copyright

Copyright © The Author(s), 2022.

Bibliography

1. The ICD-10 Classification of Mental and Behavioural Disorders: Clinical Descriptions and Diagnostic Guidelines. 10th ed. World Health Organization; 1992.
2. Association AP. Diagnostic and Statistical Manual of Mental Disorders. 5th ed. American Psychiatric Association; 2013.
3. Organization WH. International Classification of Diseases, Eleventh Revision (ICD-11). World Health Organization. Updated February 11, 2022. Accessed April 25, 2022. https://icd.who.int/browse11/l-m/en

Conversion Disorder

A 16-year-old girl presents with multiple unconsciousness. These started about six months ago and have become progressively worse. During the episodes, there is the jerky movement of the body but no urinary or faecal incontinence. These symptoms last for a few minutes and are followed by drowsiness. Neurological examination is normal.

What differential diagnoses would you consider in this case?

What other information would you enquire from the family about the episodes?

Which specific investigations would you like to order?

Differential Diagnosis

Conversion disorder (F44.5 Dissociative convulsions) suggested by the episodes of unconsciousness and lack of findings on neurological examination and absence of incontinence.

Epilepsy suggested by the recurrent brief episodes of unconsciousness, accompanied by jerky movements and followed by drowsiness

Vasovagal syncope suggested by the brief episodes of unconsciousness, jerky movements (may occur) and normal physical examination

Paroxysmal arrhythmia, which also can cause episodic unconsciousness

History of the Fits

A detailed account of (what happens during) the episodes

Examining a videotape if they have recorded it.

The duration of the fits

Whether there is a clonic phase

Whether they experience an aura. 

The occurrence of headache after the episode

Whether the patient remembers the events during the episode

Whether the fits are stereotypic in presentation

Whether the patient shouts or moans during the episodes ,

Whether the eyes remain open or closed during the episode

Whether they observe cyanosis

Tongue bite and location of the injury

The pattern of occurrence

The Frequency of the episodes

The weather there is a special time of occurrence

Fits/jerky movements during sleep

Precipitating factors




A 17-year-old girl is admitted in a psychiatry ward. She was brought unconscious with a reported history of 5-6 episodes of unconsciousness daily, each episode lasting more than an hour but not associated with body movements, tongue bite or urinary incontinence. These episodes started after her engagement to a middle-aged entrepreneur who is already married. Family members and neighbours believe she is under the influence of black magic. Radiological and lab investigations are unremarkable.

What is your provisional diagnosis?

What psychosocial explanations could you use to help the patient and her family?

How will you manage this patient?

Diagnosis

Conversion disorder (F44.5 Dissociative convulsions)

Functional neurological symptom disorder (DSM-5)

Useful Psychosocial Explanations

Experiencing a queasy stomach when talking in front of a vast audience.

Stress can worsen or cause hypertension.

Stress may worsen or cause even stress ulcers

Management

Inform the patient about the results of the assessment and the lack of evidence of an underlying physical condition.

Emphasise that the symptoms are real, well recognised and familiar to the clinician and strictly avoid giving the impression that there is 'nothing wrong' with them.

Explain the role of psychosocial factors in all medical conditions. Provide socially acceptable examples of diseases that often are deemed stress-related (e.g., peptic ulcer disease, hypertension).

Provide common examples of emotions producing symptoms (e.g., queasy stomach when talking in front of an audience) and examples of how the subconscious influences behaviour (e.g., nail-biting, pacing, foot tapping). Then offer and discuss a psychosocial explanation of the patient's symptoms.

Reassure the patient that the condition is temporary and, with motor disorders, because of a problem in converting willed intention into action.

Provide positive reinforcement/suggestion that the symptoms can improve and encourage the patient to overcome them.

Provide a graceful way for the patient to improve and allow time for a gradual recovery.

Avoid reinforcing symptoms or disability, including the provision of wheelchairs and stretchers. If the condition is long term, help remove the identified reinforcements.

Treat any associated psychiatric disorder (commonly anxiety or depression) and provide possible help with any related social issues.

Allow adequate time for the patient and their partner/family to ask questions.



Conversion disorder




Sorry for your loss, sorry to hear about your loss in vision/ sensation etc

(If vision – ask 1 or both eyes, red? Painful? Fluctuating vision loss?)

What do you think has caused this?

Happened before?

Do you think it might link with recent stresses in your life?

Do you know anyone with this, anyone in the family with this, or have you read about it?

Reassured by the Drs?

Do you think you have a particular illness?

Apart from this, do you have any other physical symptoms? How often do you see the GP?

What now? Treatment or believe you need investigations?

How have other people reacted?



Any court case pending?



Drugs and Alcohol? Impact – i.e. social situation- off sick? – stresses Risk – to self, to others, from others, neglect



MSE

Depression?

Anxiety – PTSD, GAD, OCD

Psychosis

Insight



PPHx

Meds



PMHx- thyroid, HI, epilepsy

FHx

Forensic – any court case pending?



For Dissociative seizures: include – any tongue biting, physical injuries sustained during seizures? Any incontinence? When to they occur, ABC?



Non-epileptic seizures are common in people who have epilepsy.


Tests for conversion disorder


Biological?


Evoked potentials

Findings:

disturbed somatosensory perception;

diminished or absent on side of defect.


Psychometric tool?

Halstead–Reitan battery.


Findings

Mild cognitive impairment

attentional deficits

visuoperceptual changes


Personality assessment

Objective: Minnesota Multiphasic Personality Inventory-2 (MMPI-2)

Rorschach test


Findings?

increased instinctual drives,

sexual repression

inhibited aggression


Hysterical aphonia TX?


Drug-assisted interview

E.g. intravenous amobarbital (Amytal) (100 to 500 mg) in slow infusion


Test for dissociative stupor?

Lorazepam challenge test


Patient has an a taxi gait. Does not fit any pattern. Patient does not fall to ground. Organic causes ruled out. What is this?

Atasia abasia


Patient has transient blindness for few hours. Tracking movements and pupil ary response is absent. Patient recovers two hours later. What is it

Amurosis fugax

Pupillary response and tracking movements are not absent in conversion disorder.


Patient says she is deaf. You think she is not. Conversion suspected. How will you prove your point?


Bring two metal plates, Bang heavily near her bed while she is asleep. If it awakens her, congratulate her that her hearing is normal. Okay, that was a joke, but the loud noise test during sleep is true.


Patient is having gtc fits. You are not sure whether they are conversion or epileptic. She is seizing right in front of you. Test?


Eye-opening

Check pupils

Babinski reflex


Babinski is absent in seizures and in postictal state


Psychotherapy of choice?

Insight-oriented therapy


Pathological jealousy. What ethical principles are related?


Tarasoff warning

mental health professionals have a duty to protect individuals who are being threatened with bodily harm by a patient.




































Motor Symptoms of Conversion Disorder. The International Classification of Diseases 10th edition has enlisted the following motor symptoms of conversion disorder. 
Involuntary movements
Tics
Torticollis
Blepharospasm
Seizures
Falling
Abnormal gait, Atasia-abasia
Weakness/paralysis
Aphasia
>> Dissociative Motor Disorders

ICD 10 Multiaxial System

In multi-axial diagnosis, a patient’s problems are viewed within a broader context, which includes clinical diagnosis, assessment of disability, and psychosocial factors. In ICD-10, multi-axial diagnoses are made along three axes, as follows:

Axis I: clinical diagnoses

This includes all disorders, both psychiatric and physical, including learning disability and personality disorders. 

Axis II: disabilities

Conceptualized in line with WHO definitions of impairments, disabilities, and handicaps, this covers a number of specific areas of functioning that are rated on a scale of 0–5 (‘no disability’ to ‘gross disability’):

Personal care: personal hygiene, dressing, feeding, etc.

Occupation: expected functioning in paid activities, studying, homemaking, etc.

Family and household: participation in family life.

Functioning in a broader social context: participation in the wider community, including contact with friends, leisure, and other social activities. 

Axis III: contextual factors

The factors considered to contribute to the occurrence, presentation, course, outcome, or treatment of the present Axis I disorder(s). They include problems related to:

Negative events in childhood.

Education and literacy.

Primary support group, including family circumstances.

Social environment.

Housing or economic circumstances.

(Un)employment.

Physical environment.

Certain psychosocial circumstances.

Legal circumstances.

Family history of disease or disabilities.

Lifestyle or life-management difficulties.

Classification of Depression According to the International Classification Diseases, Tenth Revision (ICD-10)

Waleed Ahmad

The ICD-10 has comprehensively sub-classified into various categories based on the clinical profile of symptoms and the course of symptoms. 

Based on the course, it may be a depressive episode, recurrent (major) depressive disorder, persistent depressive disorder or dysthymia, recurrent brief depression, etc. Depression may also be either unipolar or bipolar or it may occur in  

1. A first depressive episode, duration of at least15 days, is classified as a depressive episode (F32). If the first depressive episode severe and rapid onset, duration less than 15 days still depressive episode (F32).  
2. A depressive episode can be
1. mild (2 core symptoms, 2 other symptoms from the list) (32.0)
2. moderate (2 core symptoms, 3 or preferably 4 other symptoms) (32.1)
3. Severe (3 core symptoms, 4 other symptoms) without psychotic symptoms (32.2) (no delusion, hallucination or stupor)
4. Severe with psychotic symptoms (above plus either delusions, hallucinations or stupor) (F32.3)
5. Delusions can be mood-congruent or incongruent (neutral delusions e.g. delusions of reference are considered mood incongruent. None of them counts towards schizoaffective disorder unless one of the first-rank) 
6. A mild and moderate depressive episode can be 
1. with somatic syndrome (four or more somatic symptoms, or three very severe somatic symptoms)
2. without somatic syndrome (three or less somatic symptoms, not severe) 
7. A severe depressive episode always has a somatic syndrome 
8. Psychotic symptoms occur only in severe depression 
9. An episode of melancholic depression and agitated depression is coded under the severe depressive episode 
10. A single episode with atypical features→Other depressive episodes (F32.8)
11. An episode of masked depression NOS also coded under Other depressive episodes (F32.8) 
12. If depressive symptoms fluctuate/alternate with non-depressive symptoms e.g. worry, tension, distress also coded under F32.8
13. The second episode of depression changes the diagnostic category to recurrent depressive disorder (F33) 
14. A long history of typical depressive episodes, current episode hypomanic, the category remains recurrent depressive disorder (F33) 
15. Few brief hypomanic episodes but most of the episodes were depressive, the category remains the same
16. A long history of depressive episodes, current episode manic, category changes to bipolar 
17. A patient develops an episode that persists for long, fulfils criteria of depression, →persistent depression (F33.8 other recurrent mood disorders) 
18. An episode of subthreshold depressive symptoms persists for two years→dysthymia 
19. An episode of mild or moderate depression, followed by a two-year history of subthreshold depressive symptoms →dysthymia 
20. An episode of subthreshold depressive symptoms, current episode mild/moderate depression, → NOT dysthymia. Call it double depression? recurrent depressive disorder (? not clarified) current episode mild/moderate depression
21. Seasonal affective disorder coded under F33 (current mild or moderate episode only i.e. Cannot be severe) 
22. A patient has monthly episodes of depression that last less than two weeks, usually 2-3 days. Symptomatic criteria for d episode only depressive episodes can be fulfilled →recurrent be monthly episodes if depressive disorder F38.10
23. A patient has symptoms of both mania and depression which are equally prominent and fulfil criteria for depression and mania or hypomania by the number and severity of symptoms, last for two weeks→mixed affective episode 
24. A patient has symptoms of both mania and depression which alternate within hours, and fulfil criteria for depression as well as mania or hypomania by the number and severity of symptoms, the episode lasts for two weeks→mixed affective episode