Chiari malformation type I with depression and severe psychosis: Case report according to the role of the cerebellum in cognition and emotion

Abstract

Chiari malformation may have possible effect on cognitive and affective demonstrations. It seems necessary to pay attention to the underlying anatomical conditions in terms of diagnostic and therapeutic aspects when evaluating neuropsychiatric manifestations.

INTRODUCTION

Our patient is a 41-year-old woman with a history of treatment-resistant headaches. In the imaging survey, type 1 Chiari malformation was diagnosed. She suffered from exacerbation of headache accompanied by depressed episode and hallucinations. Chiari malformation may have possible effect on mood and psychotic manifestations.

Chiari type I is a neurological disease in which, as the volume of the posterior fossa decreases, the cerebellar tonsils pass through the foramen magnum to the subarachnoid space of cervical spine. ^1,2 In most cases, when a brain MRI is performed for various reasons, such as the presence of neurological manifestations, it is accidentally detected.³ In recent years, there have been reports of cognitive deficits such as impaired attention, memory, processing speed, and visuospatial performance related to it.⁴ Few reports have been made on the association of some psychiatric disorders with this malformation, such as anxiety disorders,⁵ mood disorders,⁶ attention deficit hyperactivity disorder (ADHD),⁷ and psychotic disorders.⁸

In this case report, we describe the case of a patient who underwent diagnostic imaging evaluations due to prolonged headaches and was diagnosed with type I Chiari. The patient experienced mood and psychotic manifestations, which, with regard to other neurological symptoms, makes it difficult to justify them only in the context of a psychiatric disorder.

CASE HISTORY

A 41-year-old woman with high school education was referred to our psychiatric center with suicidal idea and homicidal ideation toward her 7 and 10 years old children. The patient had depressed mood, anhedonia, hopelessness, restlessness, reduced concentration, and reduced energy. She also had decreased sleep and decreased appetite and 3 kg weight loss in the last month. Occasionally, she would see in the mirror a man telling her to kill herself and her children. Sometimes she heard voices giving her these orders. She also complained of severe headaches and dizziness, which began a few weeks before the recent episode. The headache was mainly in the back of the head and was exacerbated by bending forward and had worsening symptoms, followed by coughing. The pain was also compressive in nature and sometimes lasted for days. General neurological examination was within normal limits. She had no eye symptoms and also complained of neck pain. Eventually, she was diagnosed with major depressive disorder with psychotic feature. She was treated with sodium valproate 200 mg three times a day, citalopram 40 mg once a day, chlordiazepoxide 5 mg once at night, and trifluoprazine 5 mg three times a day. She was discharged with partial relief of symptoms, improvement in headache and stopping hallucinations. Two months after discharge, despite the continuation of medication, the symptoms had intensified further, leading to the current condition. About a month before the recent visit, she revealed headache in the back and neck pain again. She was experiencing the symptoms of the previous episode and was re-admitted to the psychiatric ward.

The first history of admission to psychiatric center was about 2 years ago. At that time, the patient was complaining of a headache that had developed several months earlier and had recently intensified. In addition, she had nausea, restlessness, hopelessness, depressed mood, and death wish with the idea of suicide. She had been admitted to a psychiatric hospital at the time and had been diagnosed with major depressive disorder. The patient also had no history of substance abuse or trauma in the past. Based on the severity of the headache, neurologic counseling was performed at that time, and in the imaging studies, she was diagnosed with type 1 Chiari malformation. (Figure 1) At that time, the patient underwent neurosurgery counseling, and due to significant headaches and lack of proper response to treatment, became a candidate for decompression surgery but the patient and her family were reluctant to have neurosurgery at the time.

[IMAGE OMITTED. SEE PDF]

In the recent episode, she underwent neurosurgery consultation again and underwent cognitive therapy due to the need for surgery in order to possible control of her symptoms. Cognitive-behavioral therapy was also performed to help improve the patient's mood symptoms until a decision was made for possible surgery. This was accompanied by a further improvement in the patient's psychiatric symptoms, including depressive ones.

DISCUSSION

In this report, we introduced a 41-year-old patient who has had episodes of major depression with headaches, nausea, and dizziness. Major depressive episode in the recent period has been characterized by psychosis in the feature of thoughts of pessimism accompanied by suicidal thoughts and a clear decline in individual performance. The patient also had auditory and visual hallucinations. Her response to psychiatric medications, including antidepressants and anti-psychotics, was relatively poor, despite the appropriate medication capacity. During the imaging examinations, she was diagnosed with Chiari malformation.

Ilankovic et al, reported the patient with acute episodes of repetitive psychosis over the course of 3 years with a number of neuropsychiatric signs and symptoms, such as insomnia, agitation, dysarthria, and dysphasia, that had been treated with antipsychotic medications with no obvious therapeutic response for many years. They emphasized the necessity of paying attention to all clinical aspects of patients who have referred with neuropsychiatric manifestations. They also emphasized the effect of this malformation on causing some neuropsychiatric symptoms through pressure on nerves and blood vessels.⁹

In this regard, Kandeger et al introduced the 36-year-old worker with symptoms of depression and suicidal thoughts and the diagnosis of major depressive disorder that were diagnosed with type I Chiari in brain MRI. He also suffered from hydrocephalus. They stated that one of the possible causes of depression in this disease was attributed to the effect of compression on the brain stem and serotonergic and noradrenergic imbalance.⁶ However, in this regard, a group of experts do not consider sufficient evidence of a link between affective disturbances and organic neuropathology. They cite the lack of proper response of behavioral manifestations to surgical treatments as another reason for this lack of association.¹⁰ Also, Garcia et al, reported depression and anxiety as common symptoms among patients with Chiari regardless of surgical status.¹¹

On the other hand, Noroozian evaluated the role of the cerebellum in cognition and emphasized its role not only in movement status but also in the field of intellect and emotion. She pointed out that the connection between the cerebellum and various areas of the brain through thalamus is a factor in its impact on cognition and behavior. She also noted the noradrenergic, serotonergic, and dopaminergic inputs from the brain stem to the cerebellum, and, by stating these, emphasized the role of the cerebellum in nonmotor function and suggested possible changes in behavior, mood, and higher levels of cognition in the context of structural or functional changes in the cerebellum.¹²

Steinberg et al¹³ also addressed several possible neuroanatomical explanations regarding the possible effect of anatomical malformations on cognitive and mood changes in patients with type 1 Chiari. In their review, these include various problems in the efferent and afferent pathways, and secondary cerebellar connection dysfunction with the dorsolateral prefrontal cortex and vestibular system, as well as vascular involvement due to anatomical changes, including the anterior and posterior inferior cerebellar artery (AICA&PICA).

Schmahmann also describes cerebellar posterior lobe lesions as an effective factor in the formation of cerebellar cognitive affective syndrome (CCAS). He attributed the identification of such a syndrome to a deeper understanding of the wide range of neurological and neuropsychiatric disorders associated with these lesions.¹⁴

In this regard, Guell et al examined motor and multiple nonmotor task fMRI activations in the cerebellum. These nonmotor activities included language, social, and emotion processing. They emphasized the role of the cerebellum in regulating the speed, capacity, and appropriateness of cognitive and mental processes, citing evidence from anatomical, clinical, and behavioral studies that the cerebellum is involved in cognitive and affective functions, in addition to motor control. They also reported the presence of triple representation of nonmotor task activation in the cerebellum based on their study.¹⁵

Chiari type 1 can be challenging both for physicians and for patients.¹⁶ Our patient had mood disorder with psychotic feature with Chiari type 1 malformation-the phenomenon that can be evaluated in different aspects and based on the materials presented in the literature and studies mentioned. The question is that which part of the patient's neuropsychiatric manifestations can be considered to be based on the patient's neuroanatomical status? And can surgery, in addition to being a way to improve neurological manifestations, be used in this patient and similar cases as a treatment to improve the patient's psychiatric condition? These are questions that need to be further evaluated in the future.

CONCLUSION

Our patient is an example of a patient with psychiatric manifestations with some neurological symptoms in which anatomical malformation of the central nervous system has been identified in subsequent evaluations. Chiari malformation may have possible effect on mood and psychotic manifestations, not only through the effect of compression on the brain stem, spinal cord or cervical spine, but also on the indirect side effects by making changes in neurotransmitters. Cerebellar nonmotor activities in relation to cognitive and affective functions have been considered in studies based on the analysis of multiple nonmotor task fMRI activations.

It seems that in evaluating patients with neuropsychiatric manifestations, it is necessary to consider the complex condition of the anatomical parts of the brain and to pay attention to the function and connections within different areas of the brain in order to achieve the best possible therapeutic outcome.

CONFLICT OF INTEREST

None declared.

AUTHOR CONTRIBUTIONS

RB: involved in literature check, wrote the initial draft of the manuscript, and reshaped it into this manuscript. HRN: involved in literature check, reviewed the manuscript, and edited the draft. MN: involved in literature check, reviewed the manuscript, and edited the draft AZ: involved in literature check, wrote the initial draft of the manuscript, and final submission. All the authors read and approved the final manuscript.

ETHICAL APPROVAL

We confirm that the manuscript has been read and approved by all named authors. The patient consented to the publication of her case.

CONSENT FOR PUBLICATION

We have obtained written consent from the patient and her husband to provide details about the case and scientific discussion while maintaining complete patient anonymity.

DATA AVAILABILITY STATEMENT

The data that support the points made in this study are available at the request from the corresponding author.

References

Yan H, Han X, Jin M, et al. Morphometric features of posterior cranial fossa are different between Chiari I malformation with and without syringomyelia. Eur Spine J. 2016;25(7):2202‐2209.

Botelho RV, Heringer LC, Botelho PB, Lopes RA, Waisberg J. Posterior fossa dimensions of chiari malformation patients compared with normal subjects: systematic review and meta‐analysis. World Neurosurg. 2020;138:521‐529.e2.

Frič R, Eide PK. Chiari type 1—a malformation or a syndrome? A critical review. Acta Neurochir. 2020;162(7):1513‐1525.

Houston JR, Hughes ML, Lien M‐C, et al. An electrophysiological study of cognitive and emotion processing in type I Chiari malformation. Cerebellum. 2018;17(4):404‐418.

Caykoylu A, Ekinci O, Albayrak Y, Kuloglu M, Deniz O. Arnold‐Chiari I malformation association with generalized anxiety disorder: a case report. Prog Neuropsychopharmacol Biol Psychiatry. 2008;32(6):1613‐1614.

Kandeger A, Guler HA, Egilmez U, Guler O. Major depressive disorder comorbid severe hydrocephalus caused by Arnold‐Chiari malformation. Indian J Psychiatry. 2017;59(4):520.

DuBow A, Mourot A, Tourjman SV. Chiari malformation and attention deficit hyperactivity disorder. Case Rep Med. 2020;2020:1‐3.

Di Genova C, Charitos S, Ba G, Viganò CA Case report: atypical psychotic onset of type I Arnold‐Chiari malformation. F1000Research. 2015;4:915.

Ilanković NN, Ilanković AN, Bojić V, Ilanković LM. Chiari I malformation in adults: epileptiform events and schizophrenia‐like psychosis. Psychiatr Danub. 2006;18(1–2):92‐96.

Novegno F. Clinical diagnosis—part II: what is attributed to Chiari I. Childs Nerv Syst. 2019;35(10):1681‐1693.

Rogers JM, Savage G, Stoodley MA. A systematic review of cognition in Chiari I malformation. Neuropsychol Rev. 2018;28(2):176‐187.

Noroozian M. The role of the cerebellum in cognition: beyond coordination in the central nervous system. Neurol Clin. 2014;32(4):1081‐1104.

Steinberg SN, Greenfield JP, Perrine K. Neuroanatomic correlates for the neuropsychological manifestations of Chiari malformation type I. World Neurosurg. 2020;136:462‐469.

Schmahmann JD. The cerebellum and cognition. Neurosci Lett. 2019;688:62‐75.

Guell X, Gabrieli JD, Schmahmann JD. Triple representation of language, working memory, social and emotion processing in the cerebellum: convergent evidence from task and seed‐based resting‐state fMRI analyses in a single large cohort. NeuroImage. 2018;172:437‐449.

Fischbein R, Saling JR, Marty P, et al. Patient‐reported Chiari malformation type I symptoms and diagnostic experiences: a report from the national conquer Chiari patient registry database. Neurol Sci. 2015;36(9):1617‐1624.