New-Onset Psychosis Associated With Dandy-Walker Variant in an Adolescent Female Patient

A 14-year-old Asian adopted girl with no previous psychiatric history was brought for outpatient psychiatric evaluation to assess “paranoid ideas and her misperception of reality.” She had been attending a summer camp when she developed acute feelings of severe anxiety. She reported having a headache, “feeling faint” with tingling and numbness in her arms,” and feeling “cold and clammy.” These symptoms lasted for a few minutes and she reported feelings of impending doom. She could not relate a precipitating event to these symptoms and reported that the ominous feelings persisted after the physical symptoms subsided. She became preoccupied that “bad things were going to happen.”

She was assessed by her pediatrician, who noted elevated heart rate but normal electrocardiogram and laboratory work-up. Several days after this initial complaint, the child reported to her parents that the faces of her friends were “colored red,” and she reported feelings of paranoia regarding people staring at her. Her pediatrician referred her to a child psychiatrist, who initiated a trial of risperidone, titrated up to an oral dose of 2.25 mg daily.

During the titration period, she continued to express paranoid ideation that people were hiding behind trees to spy on her and that her parents were going to kidnap her. She expressed to her parents that she heard a voice talking about a dead body and referenced that she understood this to mean that she was going to die or should die. She expressed fears that the police had come into her home and “moved words around in magazines” and had plans to arrest her for viewing internet pornography. She admitted that she had been viewing pornographic material, which she originally disguised as “health issue websites” for over a year and felt an enormous amount of guilt over this. Her mother described her thought-process as “loose and disconnected.” She admitted to feelings of racing thoughts, difficulty concentrating, excessive worry, and difficulty falling asleep secondary to this. She also expressed ideas of reference that patients in the waiting room of her appointments were all there because of her. She also had thoughts of self-doubt and low self-esteem. She denied use of drugs, alcohol, or tobacco; denied suicidal or homicidal ideations, and was able to articulate her paranoid ideations. She was referred for a more comprehensive psychiatric evaluation and psychological testing.

The child was adopted at the age of 2 from an Asian orphanage; she weighed only 17 pounds at the time of adoption and was delayed with regard to developmental milestones; however, with proper care, nutrition, and support, she was able to recover fully and continued to reach subsequent milestones at the appropriate ages. Her menses started at age 13. No family history is available for her; her adopted sister, also from Asia, is of no biological relation.

On initial interview with the current psychiatrist, she was quiet, shy, appeared younger than stated age, was well-groomed, and wearing appropriate clothing. She was cooperative, with normal speech and fair eye contact. She had no trouble separating from her parents, but did appear slightly anxious at times. Her affect was blunted, and she repeated that her mood and thoughts were “much better” with the medication. She admitted to previous feelings of agitation and anxiety. She endorsed feelings of sadness, decreased concentration, increased “thinking;” she denied hopelessness or helplessness, admitted to auditory hallucinations as previously noted; denied thought-broadcasting or thought-insertion. She endorsed difficulty with sleep, specifically, trouble falling asleep and early awakening. Her insight was fair, and her judgment was impaired. She exhibited low self-esteem and overwhelming guilt regarding her internet activities and believed that she needed to be punished. Review of her personal journal entries exhibited mood-congruent beliefs of self-reproach, guilt, poor self-esteem, and paranoid thinking. She was referred to a psychologist for psychological testing, with the following results:

The concept of psychosis, as applied to children, is very challenging in many aspects. Children’s concepts of reality change over the course of normal development, and mature adult conceptualizations of reality are achieved only as an adolescent. Many children have normatively appropriate beliefs in fantasy figures, which would not, in themselves, suggest psychosis. It is important to note that comorbid associations with conditions such as mental retardation or other developmental disorders might pose additional problems in assessing psychotic process in children.¹⁰

Many children who develop psychosis have disturbances of behavior and cognition before onset of symptoms and marked delays in motor and verbal milestones. Such children also have impaired social skills and school achievements. Symptoms frequently seen in children with psychosis are the following: speech disturbances, inability to distinguish dreams from reality, visual and auditory hallucinations, vivid and bizarre thoughts and ideas, diminished interest, confused thinking, extreme moodiness, odd behavior, stereotypy, disinheriting, ideas that others are “out to get them” (paranoia), confusion of television with reality, and severe problems making and keeping friends.¹¹

As referenced earlier, there are few cases of DWV associated with psychiatric symptoms in the literature. Turner et al.¹² reported the first case of schizophrenic-like psychosis in association with DWV. An 18-year-old woman presented with paranoia and hallucinations; subsequent computed tomography (CT) scan showed Dandy-Walker variant with hypoplasia of cerebellar hemispheres and vermis without evidence of hydrocephalus or supratentorial abnormalities. Her symptoms resolved without treatment. The authors were careful to note that the patient’s symptoms of psychosis and the presence of Dandy-Walker variant may have been coincidental, as they were also unable to rule out the possible concomitant use of illicit drugs.¹²

A second reported case of psychosis and DWV was described by Papazisis et al.¹³ in a 20-year-old man who was diagnosed with early schizophrenia and comorbid obsessive-compulsive disorder. CT scan showed DWV with hypoplasia of the cerebellar vermis, dilation of the fourth ventricle and mild enlargement of the rest of the ventricular system without enlargement of the posterior fossa. Papazisis et al.¹³ suggest evidence for the direct relationship between cerebellar abnormalities and psychosis, specifically involving higher-level cognitive deficits, such as impaired judgment and abstraction, mental arithmetic, and immediate and recent memory loss.¹³ Before this proposal, Andreasen et al.^14,15 had suggested an elaborate model involving the frontal, cerebellar, and thalamic regions of the brain, connected through cortical-subcortical-cerebellar circuitry.^14,15

Langarica and Peralta¹⁶ also report a case of a 52-year-old woman with no past psychiatric history, who presented with paranoia, thought-withdrawal, and psychosis. On the basis of her brain CT scan, a diagnosis of Mega Cisterna Magna was confirmed. She was treated effectively with olanzapine (7.5 mg daily) and had no reported acute exacerbation on 1-year follow-up.¹⁶

In a case unrelated to psychosis, Prakash et al.¹⁷ report on a 14-year-old young man diagnosed with hyperkinetic conduct disorder and mental retardation who was found to have imaging reports consistent with Dandy-Walker malformation.¹⁷ This is an example of a psychiatric disorder associated with brain-imaging abnormalities, but confounded by the diagnosis of mental retardation and presumed developmental delays.

The relationship between schizophrenia and cerebellar malformations has been documented in previous research studies.¹⁸ In a controlled study, the authors examined brain-imaging on over 50 patients and concluded that patients diagnosed with schizophrenia presented with significantly lower volumes of the entire vermis. Specifically, the posterior vermian area was smaller for participants with schizophrenia. This is consistent with findings in patients who presented with cerebellar lesions affecting affect; thus, the conclusion that these specific cerebellar lesions are noted in patients diagnosed with negative and positive symptoms of schizophrenia.¹⁸ Abnormalities of the cerebellum, especially in the vermis, have been linked with the pathophysiology of schizophrenia and are detected not only in chronic patients but at the time of onset of this condition.^19,20

Even though this patient’s MRI does not demonstrate abnormalities in the cerebellar vermis, we cannot exclude the possibility that the enlarged lateral ventricles and the enlarged inferior areas of the cerebellum are contributing to the psychotic symptomatology, including negative symptoms, such as blunted affect and anhedonia. Treatment with antipsychotics has ameliorated her paranoid and psychotic symptoms, but every time the antipsychotic medication dose is reduced, her symptoms return. Continued treatment with regard to her Dandy-Walker variant condition may include shunt placement, but it is mainly focused on treating the developmental problems, with physical or occupational therapy as well as ongoing supportive psychotherapy to improve self-esteem and general coping skills.

Despite the listed findings, further research is necessary to fully understand the possible correlation between the development of the brain, specifically the cerebellum and posterior fossa, and psychosis. The importance of recognizing new-onset psychosis and its potential relation to malformations in the brain needs to be addressed. With the malformation present since birth, early detection is vital to understanding the manifestation of psychotic symptoms and tailoring treatment options accordingly, as well as educating providers and parents about the possibility of developing future psychiatric disorders. This review highlights the necessity for a complete evaluation, including imaging studies, when presented with a child or adolescent with psychosis. At the root of the issue is a need for more research on the relationship between Dandy-Walker syndrome and psychiatric illness.