Childhood Psychoses


Psychosis is a severe disruption of thought, perception, and behavior resulting in loss of reality testing. Psychosis can occur as part of a mood disorder, such as major depressive disorder or bipolar I disorder; between mood disorder episodes, as in schizoaffective disorder; or without mood disorder episodes, as in schizophrenia. Transient psychotic episodes can arise during times of psychological or physiologic stress in patients who are vulnerable because of personality, developmental, or genetic disorders. Delusions, hallucinations, disorganized thinking, and grossly disorganized behavior (positive symptoms) are key features that define psychoses across disorders, likely because of shared pathophysiologic mechanisms. Negative symptoms, on the other hand, are most typical of schizophrenia.

Delusions are fixed, unchangeable, false beliefs held despite conflicting evidence. They may include a variety of themes (persecutory, referential, somatic, religious, grandiose). Delusions are considered bizarre if they are clearly implausible. Hallucinations are vivid, clear, perceptual-like experiences that occur without external stimulus and have the full force and impact of normal perceptions. They may occur in any sensory modality; auditory hallucinations are the most common. Disorganized thinking is typically inferred from an individual's speech (loose associations, tangentiality, or incoherence). Grossly disorganized behavior may range from childlike silliness to catatonic behavior. Negative symptoms include diminished emotional expression, avolition, alogia (lack of speech), anhedonia (inability to experience pleasure), and asociality. Negative symptoms generally account for a substantial portion of the long-term morbidity associated with schizophrenia.

Given the centrality of hallucinations and delusions in making a diagnosis of a psychotic illness, their differentiation from developmentally normal fantasy is essential. When children are imagining, they control the fantasy and do not have the perceptual experience of seeing and hearing. When children are hallucinating, they do not control the hallucination. Almost two thirds of children will endorse at least 1 psychotic-like experience, most often a hallucination, and when not persistent or accompanied by distress, these experiences are not usually a cause for concern. The largest population-based study to date evaluating psychotic symptoms and neurocognition in youth 11-21 yr old found that those who endorsed more psychotic-like experiences than is typical for their age had reduced accuracy across neurocognitive domains, reduced global functioning, and increased risk of depression, anxiety, behavioral disorders, substance use, and suicidal ideation. Thus, psychotic-like symptoms that are frequent, distressing, and cause impairment signal a need for further evaluation and monitoring; however, only a small minority of these children will develop full-blown psychotic illnesses.

Schizophrenia Spectrum and Other Psychotic Disorders

Joseph Gonzalez-Heydrich
Heather J. Walter
David R. DeMaso

Schizophrenia spectrum and other psychotic disorders as described in the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5) include brief psychotic disorder, schizophreniform disorder, schizophrenia, schizoaffective disorder, substance/medication-induced psychotic disorder, psychotic disorder caused by another medical condition, catatonia associated with another mental disorder, catatonic disorder due to another medical condition, unspecified catatonia, delusional disorder, schizotypal personality disorder, and other specified/unspecified schizophrenia spectrum and other psychotic disorders.

Description

The schizophrenia spectrum and other psychotic disorders are primarily characterized by the active (or positive) symptoms of psychosis, specifically delusions, hallucinations, disorganized speech, or grossly disorganized or catatonic behavior. Brief psychotic disorder is characterized by the duration of 1 or more of these symptoms for at least 1 day but <1 mo followed by complete resolution. Emergence of symptoms may or may not be preceded by an identifiable stressor ( Table 47.1 ). Although brief, the level of impairment in this disorder may be severe enough that supervision is required to ensure that basic needs are met and the individual is protected from the consequences of poor judgment and cognitive impairment.

Table 47.1
From the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition, (Copyright 2013). American Psychiatric Association, p 94.
DSM-5 Diagnostic Criteria for Brief Psychotic Disorder

  • A

    Presence of 1 (or more) of the following symptoms. At least 1 of these must be (1), (2), or (3):

    • 1

      Delusions.

    • 2

      Hallucinations.

    • 3

      Disorganized speech (e.g., frequent derailment or incoherence).

    • 4

      Grossly disorganized or catatonic behavior.

    Note: Do not include a symptom if it is a culturally sanctioned response.

  • B

    Duration of an episode of the disturbance is at least 1 day but less than 1 mo, with eventual full return to premorbid level of functioning.

  • C

    The disturbance is not better explained by major depressive or bipolar disorder with psychotic features or another psychotic disorder such as schizophrenia or catatonia, and is not attributable to the physiologic effects of a substance (e.g., a drug of abuse, a medication) or another medical condition.

  • Specify if:

  • With marked stressor(s) (brief reactive psychosis): If symptoms occur in response to events that, singly or together, would be markedly stressful to almost anyone in similar circumstances in the individual's culture.

  • Without marked stressor(s) : If the symptoms do not occur in response to events that, singly or together, would be would be markedly stressful to almost anyone in similar circumstances in the individual's culture.

  • With postpartum onset : If onset is during pregnancy or within 4 wk postpartum.

If 2 or more psychotic symptoms persist from 1 mo up to 6 mo, the condition is called schizophreniform disorder ( Table 47.2 ). To meet DSM-5 criteria for schizophrenia , 2 or more psychotic symptoms must have been present for a significant time during 1 mo (unless suppressed by treatment), and the level of psychosocial functioning must be markedly below the level achieved before the onset (or there is failure in children to achieve the expected level of functioning). In addition, there must be continuous signs of the disturbance (prodromal, active, or residual symptoms) for at least 6 mo ( Table 47.3 ).

Table 47.2
From the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition, (Copyright 2013). American Psychiatric Association, pp 96–97.
DSM-5 Diagnostic Criteria for Schizophreniform Disorder

  • A

    Two (or more) of the following, each present for a significant portion of time during a 1 mo period (or less if successfully treated). At least 1 of these must be (1), (2), or (3):

    • 1

      Delusions.

    • 2

      Hallucinations.

    • 3

      Disorganized speech (e.g., frequent derailment or incoherence).

    • 4

      Grossly disorganized or catatonic behavior.

    • 5

      Negative symptoms (i.e., diminished emotional expression or avolition).

  • B

    An episode of the disorder lasts at least 1 mo but less than 6 mo. When the diagnosis must be made without waiting for recovery, it should qualified as “provisional.”

  • C

    Schizoaffective disorder and depressive or bipolar disorder with psychotic features have been ruled out because either (1) no major depressive or manic episodes have occurred concurrently with the active-phase symptoms; or (2) if mood episodes have occurred during active-phase symptoms, they have been present for a minority of the total duration of the active and residual periods of the illness.

  • D

    The disturbance is not attributable to the physiologic effects of a substance (e.g., a drug of abuse, a medication) or another medical condition.

  • Specify if:

  • With good prognostic features : This specifier requires the presence of at least 2 of the following features: onset of prominent psychotic symptoms within 4 wk of the first noticeable change in usual behavior or functioning; confusion or perplexity; good premorbid social and occupational functioning; and absence of blunted or flat affect.

  • Without good prognostic features : This specifier is applied if 2 or more of the above features have not been present.

Table 47.3
From the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition, (Copyright 2013). American Psychiatric Association, pp 99–100.
DSM-5 Diagnostic Criteria for Schizophrenia

  • A

    Two (or more) of the following, each present for a significant portion of time during a 1 mo period (or less if successfully treated). At least 1 of these must be (1), (2), or (3):

    • 1

      Delusions.

    • 2

      Hallucinations.

    • 3

      Disorganized speech (e.g., frequent derailment or incoherence).

    • 4

      Grossly disorganized or catatonic behavior.

    • 5

      Negative symptoms (i.e., diminished emotional expression or avolition).

  • B

    For a significant portion of the time since the onset of the disturbance, level of functioning in 1 or more major areas, such as work, interpersonal relations, or self-care, is markedly below the level achieved prior to the onset (or when the onset is in childhood or adolescence, there is failure to achieve expected level of interpersonal, academic, or occupational functioning).

  • C

    Continuous signs of the disturbance persist for at least 6 mo. This 6 mo period must include at least 1 mo of symptoms (or less if successfully treated) that meet Criterion A (i.e., active-phase symptoms) and may include periods of prodromal or residual symptoms. During these prodromal or residual periods, the signs of the disturbance may be manifested by only negative symptoms or by 2 or more symptoms listed in Criterion A present in an attenuated form (e.g., odd beliefs, unusual perceptual experiences).

  • D

    Schizoaffective disorder and depressive or bipolar disorder with psychotic features have been ruled out because either (1) no major depressive or manic episodes have occurred concurrently with the active-phase symptoms; or (2) if mood episodes have occurred during active-phase symptoms, they have been present for a minority of the total duration of the active and residual periods of the illness.

  • E

    The disturbance is not attributable to the physiologic effects of a substance (e.g., a drug of abuse, a medication) or another medical condition.

  • F

    If there is a history of autism spectrum disorder or a communication disorder of childhood onset, the additional diagnosis of schizophrenia is made only if prominent delusions or hallucinations, in addition to the other required symptoms of schizophrenia, are also present for at least a month (or less if successfully treated).

Individuals with schizophrenia can display inappropriate affect, dysphoric mood, disturbed sleep patterns, and lack of interest in eating, or food refusal. Depersonalization, derealization, somatic concerns, and anxiety and phobias are common. Cognitive deficits are observed, including decrements in declarative memory, working memory, language function, and other executive functions, as well as slower processing speed. These individuals may have no insight or awareness of their disorder, which is a predictor of nonadherence to treatment, higher relapse rates, and poorer illness course. Hostility and aggression can be associated with schizophrenia, although spontaneous or random assault is uncommon. Aggression is more frequent for younger males and for individuals with a past history of violence, non-adherence with treatment, substance abuse, and impulsivity.

The essential features of schizophrenia are the same in childhood as in adulthood, but it is more difficult to make the diagnosis. In children, delusions and hallucinations may be less elaborate, visual hallucinations may be more common, and disorganized speech may be better attributed to an autism spectrum or communication disorder. In a review of 35 studies of youth with schizophrenia, the most frequent psychotic symptoms were auditory hallucinations (82%), delusions (78%), thought disorder (66%), disorganized or bizarre behavior (53%), and negative symptoms (50%).

Epidemiology

Brief psychotic disorders have been reported to account for 9% of first-onset psychosis in the United States, with a 2 : 1 ratio in favor of females. The incidence of schizophreniform disorders in the United States appears as much as 5-fold less than that of schizophrenia. The lifetime prevalence of schizophrenia is approximately 0.3–0.7%, although variations are reported by race/ethnicity, across countries, and by geographic origin for immigrants. The male:female ratio is approximately 1.4 : 1. Males generally have worse premorbid adjustment, lower educational achievement, more prominent negative symptoms, and more cognitive impairment than females.

Clinical Course

Brief psychotic disorder most often appears in adolescence or early adulthood, with the average age of onset in the mid-30s, but can occur throughout the life span. A diagnosis of brief psychotic disorder requires full remission within 1 mo of onset and gradual return to premorbid level of function. The age of onset of schizophreniform disorder is similar to that of schizophrenia. Recovery from an episode of the disorder is within 6 mo; however, about two thirds of patients relapse and eventually receive a diagnosis of schizophrenia or schizoaffective disorder. Abrupt onset, confusion, absence of blunted affect, and good premorbid functioning predict a better outcome in schizophreniform disorder.

Schizophrenia typically develops between the late teens and the mid-30s; onset before adolescence is rare. The peak age at onset for the first psychotic episode is in the early to mid-20s for males and in the late 20s for females. The onset may be abrupt or insidious, but the majority of individuals manifest a slow and gradual development of symptoms, with about half of individuals complaining of depressive symptoms. The predictors of course and outcome are largely unexplained. The course appears to be favorable in approximately 20% of cases, and a small number of individuals are reported to recover completely. However, many remain chronically ill, with exacerbations and remissions of active symptoms, whereas others experience progressive deterioration. Most individuals diagnosed with schizophrenia require daily living supports. Positive symptoms tend to diminish over time, and negative symptoms are the most persistent, along with cognitive deficits.

Differential Diagnosis

The differential diagnosis for the psychotic disorders is broad and includes reactions to substances/medications (dextromethorphan, LSD, hallucinogenic mushrooms, psilocybin, peyote, cannabis, stimulants, inhalants; corticosteroids, anesthetics, anticholinergics, antihistamines, amphetamines); medical conditions causing psychotic-like symptoms ( Table 47.4 ); and other psychiatric disorders (depressive, bipolar, obsessive-compulsive, factitious, body dysmorphic, posttraumatic stress, autism spectrum, communication, personality). The differential diagnosis can be difficult because many conditions that can be mistaken for psychosis also increase the risk for it.

Table 47.4
Select Neurologic and Systemic Causes of Depression and/or Psychosis
Modified from Perez DL, Murray ED, Price BH: Depression and psychosis in neurological practice. In Daroff RB, Jankovic J, Mazziotta JC, et al, editors: Bradley's neurology in clinical practice, 7th ed, Philadelphia, 2015, Elsevier.
CATEGORY DISORDERS
Head trauma
  • Traumatic brain injury

  • Subdural hematoma

Infectious
  • Lyme disease

  • Prion diseases

  • Neurosyphilis

  • Viral infections/encephalitides (HIV infection/encephalopathy, herpes encephalitis, cytomegalovirus. Epstein-Barr virus)

  • Whipple disease

  • Cerebral malaria

  • Systemic infection

Inflammatory
  • Autoimmune encephalitis

  • Celiac disease

  • Systemic lupus erythematosus

  • Sjögren syndrome

  • Temporal arteritis

  • Hashimoto encephalopathy

  • Sydenham chorea

  • Sarcoidosis

Neoplastic
  • Primary or secondary cerebral neoplasm

  • Systemic neoplasm

  • Paraneoplastic encephalitis

Endocrine or acquired metabolic
  • Hepatic encephalopathy

  • Uremic encephalopathy

  • Dialysis dementia

  • Hypo/hyperparathyroidism

  • Hypo/hyperthyroidism

  • Addison disease, Cushing disease

  • Postpartum

  • Vitamin deficiency: vitamin B 12 , folate, niacin, vitamin C. thiamine

  • Gastric bypass–associated nutritional deficiencies

  • Hypoglycemia

  • Hyponatremia

Vascular
  • Stroke

  • Cerebral autosomal dominant aneriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)

Degenerative
  • Progressive supranuclear palsy

  • Huntington disease

  • Corticobasal ganglionic degeneration

  • Multisystem atrophy, striatonigral degeneration, olivopontocerebellar atrophy

  • Idiopathic basal ganglia calcifications, Fahr disease

  • Neuroacanthosis

  • Neurodegeneration with brain iron accumulation (NBIA)

  • Adrenoleukodystrophy

  • Metachromatic leukodystrophy

Demyelinating, dysmyelinating
  • Multiple sclerosis

  • Acute disseminated encephalomyelitis

  • Adrenoleukodystrophy

  • Metachromatic leukodystrophy

Inherited metabolic
  • Wilson disease

  • Posterior hom syndrome

  • Tay-Sachs disease

  • Neuronal ceroid lipofuscinosis

  • Niemann-Pick disease type C

  • Acute intermittent porphyria

  • Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS)

  • Cerebrotendinous xanthomatosis

  • Homocystinuria

  • Ornithine transcarbamylase deficiency

Syndromes
  • Williams

  • Prader-Willi

  • Fragile X

  • Deletion 22q11.2

  • ROHHAD

Epilepsy
  • Ictal

  • Interictal

  • Postictal

  • Forced normalization

  • Postepilepsy surgery

  • Lafora progressive myoclonic epilepsy

Medications
  • Analgesics

  • Androgens (anabolic steroids)

  • Antiarrhythmics

  • Anticonvulsants

  • Anticholinergics

  • Antibiotics

  • Antihypertensives

  • Antineoplastic agents

  • β-Blocking agents

  • Corticosteroids

  • Cyclosporin

  • Dopamine agonists

  • Oral contraceptives

  • Sedatives/hypnotics

  • Selective serotonin reuptake inhibitors (SSRIs) (serotonin syndrome)

Drugs of abuse
  • Alcohol

  • Amphetamines

  • Cocaine

  • Hallucinogens

  • Marijuana and synthetic cannabinoids

  • Methylenedioxymethamphetamine (MDMA, Ecstasy)

  • Phencyclidine

Drug withdrawal syndromes
  • Alcohol

  • Barbiturates

  • Benzodiazepines

  • Amphetamines

  • SSRIs

Toxins
  • Heavy metals

  • Inhalants

Other
  • Normal-pressure hydrocephalus

  • Ionizing radiation

  • Decompression sickness

ROHHAD, Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, autonomic dysregulation.

Autoimmune encephalitis caused by anti– N -methyl- d -aspartate (NMDA) receptor or other autoantibodies may manifest with psychosis, anxiety, depression, agitation, aggression, delusions, catatonia, visual or auditory hallucinations, disorientation, and paranoia in combination with sleep disturbances, autonomic dysfunction (hypoventilation), dyskinesias, movement disorders, seizures, memory loss, and a depressed level of consciousness ( Fig. 47.1 ). The electroencephalogram (EEG), cerebrospinal fluid (CSF), and MRI are usually, but not always, abnormal. The constellation of psychosis and encephalitic features should suggest the diagnosis, although at presentation, behavioral problems may be the dominant feature (see Chapter 616.4 ).

Fig. 47.1, Clinical characteristics of patients with anti–NMDA receptor encephalitis.

Determining when identifiable medical conditions are causing delirium with prominent psychotic symptoms may be difficult ( Table 47.5 and Table 47.6 ). In general, delirium due to medical causes is often associated with abnormalities in vital signs and the neurologic examination (including level of consciousness). A positive family or prior personal history of serious psychiatric illness is less likely. When psychotic symptoms are caused by identifiable medical conditions, there are often impairments in attention, orientation, recent memory, and intellectual function. Hallucinations may be caused by medical illness, but are often tactile, visual, or olfactory, whereas auditory hallucinations are more common in primary psychotic disorders. Patients whose hallucinations are caused by medical illness are more likely than patients with primary psychotic disorders to be aware that the hallucinations do not represent reality.

Table 47.5
Special Problems in the Differential Diagnosis of Delirium *
From Mendez MF, Padilla CR: Delirium. In Daroff RB, Jankovic J, Mazziotta JC, et al, editors: Bradley's neurology in clinical practice, 7th ed, Philadelphia, 2015, Elsevier.
CLINICAL FEATURE DELIRIUM DEMENTIAS SCHIZOPHRENIA DEPRESSION
Course Acute onset; hours, days, or more Insidious onset, months or years, progressive Insidious onset, ≥6 mo, acute psychotic phases Insidious onset, at least 2 wk, often months
Attention Markedly impaired attention and arousal Normal early; impairment later Normal to mild impairment Mild impairment
Fluctuation Prominent in attention arousal; disturbed day/night cycle Prominent fluctuations absent; lesser disturbances in day/night cycle Absent Absent
Perception Misperceptions; hallucinations, usually visual, fleeting; paramnesia Perceptual abnormalities much less prominent; paramnesia Hallucinations, auditory with personal reference May have mood-congruent hallucinations
Speech and language Abnormal clarity, speed, and coherence; disjointed and dysarthric; misnaming; characteristic dysgraphia Early anomia; empty speech; abnormal comprehension Disorganized, with a bizarre theme Decreased amount of speech
Other cognition Disorientation to time, place; recent memory and visuospatial abnormalities Disorientation to time, place; multiple other higher cognitive deficits Disorientation to person; concrete interpretations Mental slowing; indecisiveness; memory retrieval difficulty
Behavior Lethargy or delirium; nonsystematized delusions; emotional lability Disinterested; disengaged; disinhibited; delusions and other psychiatric symptoms Systematized delusions; paranoia; bizarre behavior Depressed mood; anhedonia; lack of energy; sleep and appetite disturbances
Electroencephalogram Diffuse slowing; low-voltage fast activity; specific patterns Normal early; mild slowing later Normal Normal

* The characteristics listed are the usual ones and not exclusive.

Table 47.6
From Perez DL Murray ED, Price BH: Depression and psychosis in neurological practice. In Daroff RB, Jankovic J, Mazziotta JC, editors: Bradley's neurology in clinical practice, 7th ed, Philadelphia, 2015, Elsevier.
Features Suggesting Neurologic Disease in Patients With Psychiatric Symptoms

Atypical Psychiatric Features

  • Late or very early age of onset

  • Acute or subacute onset

  • Lack of significant psychosocial stressors

  • Catatonia

  • Diminished comportment

  • Cognitive decline

  • Intractability despite adequate therapy

  • Progressive symptoms

History of Present Illness

  • New or worsening headache

  • Inattention

  • Somnolence

  • Incontinence

  • Focal neurologic complaints such as weakness, sensory changes, incoordination, or gait difficulty

  • Neuroendocrine changes

  • Anorexia/weight loss

Patient Medical History

  • Risk factors for cerebrovascular disease or central nervous system infections

  • Malignancy

  • Immunocompromised status

  • Significant head trauma

  • Seizures

  • Movement disorder

  • Hepatobiliary disorders

  • Abdominal crises of unknown cause

  • Biologic relatives with similar diseases or complaints

Unexplained Diagnostic Abnormalities

  • Screening laboratory tests

  • Neuroimaging studies or possibly imaging of other systems

  • Electroencephalogram

  • Cerebrospinal fluid

The diagnosis of a psychotic disorder should be made only after other explanations for the observed symptoms have been thoroughly considered. Mistakenly diagnosing psychosis when it is not present can lead to inappropriate use of antipsychotics with all their attendant risks, and mistakenly dismissing psychotic symptoms as nonpsychotic manifestations of, for example, autism or trauma can lead to long delays in treatment of the psychosis. The persistence, frequency, and form of possible psychotic symptoms, as well the degree of accompanying distress and functional regression, need to be considered in determining the likelihood of an underlying psychotic pathophysiology.

Comorbidity

In a review of 35 studies of youth with schizophrenia, rates of comorbidity approximated 34% for posttraumatic stress disorder, 34% for attention-deficit/hyperactivity and/or disruptive behavior disorders, and 32% for substance abuse/dependence.

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