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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Ayesha Anwar, M.B.B.S[2]

Historical Perspective

Personality defects were started to be recognized in the 18th century. Previously, all the diseases were a result of abnormalities with four bodily fluids; blood, phlegm, yellow bile, and black bile. The changes in them were also considered responsible for variations in mood. However, by the 18th century, Phillippe Pinel described a group of people having impulsive, irrational ways and behaviors while maintaining understanding, perception, judgment, and memory of the actions. This was the birth of recognition of personality disorders. In the 18th century, the term 'phrenology' was used to describe personality characteristics. It was believed that the origin of personality traits is from various facets in the cranium. Despite the discontinuation of the term, it remains significant as it laid the basis for the origin of PDs from the cerebral cortex. In the 19th century and early 20th century, different European psychologists started identifying and describing different personality traits and disorders. The term personality is derived from Greek word, 'persona,' the mask worn in theatres in ancient times to denote a character or social role. It is now used to define that aspect of the person which is discerned by other individuals. In the 1920s and 1930s, Sigmund Freud, known as the father of psychology and his colleagues, worked on the psychoanalytic classification and etiology of personality. They related personality traits with childhood characters. He presented the structural theory that unconscious mental conflicts influence the development of character and behavior [1]. This comprises three components of the brain; the id (primitive urges at birth), ego (mediator that maintains a balance between id and reality), and superego (conscience and moral values). They develop at different stages in life, and the interplay among them is responsible for shaping the personality of a person. Any fixation at any stage is responsible for the improper balance of id and ego and leads to interference in the appropriate and timely development of the superego. A person is born with the id and develops ego and superego at last. This laid down the foundation for further theories and explanations of PDs; however, it lacks the interaction and influences of social, cultural, environmental, and genetic factors in posing the personality in an individual. In the late 1900s, statistics was utilized to group together different definitions of personality structures. It was pioneered by Bernard Cattell. This employs a different number of dimensions to delineate personality systems. These dimensional models lead to DSM characterization of personality disorders according to DSM classifications. DSM II was established in 1986 and listed 10 PDs. It differs from DSM I due to the recognition stage in life being adolescence, while the former states that these disorders exist lifelong. DSM II was based on concepts of psychoanalysis and neuroses, while DSM III described PDs scientifically and clinically. DSM IV was established in 1994 with an updated version, DSM IV-TR, and uses a multiaxial approach to describe psychiatric illnesses with axis II reserved for personality disorder. This multiaxial system was abolished in DSM 5 and categorized the various disorders with related disorders [2]. It classifies PDs into three clusters, with each containing 3-4 disorders.

Classification

There are two approaches used to classify personality disorders; categorical and dimensional. Categorical classification is based on distinct operational criteria depending on behavioral characteristics. DSM-5 and ICD-10 both uses this approach. As compared to this, dimensional classification is based on the personality traits and using a quantitative distinction. It places normality at one end and disorder at other.

Pathophysiology

Personality disorders are related to multifactorial causes. Throughout time, a multitude of theories has been developed to explain the origin of these disorders. However still, the pathophysiology of PDs remains enigmatic. The five-factor model of personality was developed in the 1980s and 1990s, which demonstrated that it comprises five distinct traits. These include extraversion, Neuroticism, openness to experience/intellect, Agreeableness, and conscientiousness. A meta-analysis conducted by Saulsman and Page in 2004 reveals the association of personality disorders with the five-trait model. It concludes that extraversion is positively associated with disorders characterizing assertiveness or gregariousness like Histrionic and Narcissist. Neuroticism is positively associated with disorders causing emotional distress like Paranoid, Schizotypal, Borderline, Dependent, and Avoidant. Agreeableness is negatively associated with disorders characterized by interpersonal difficulties like Paranoid, Schizotypal, Antisocial, Borderline, and Narcissist. Those disorders which are distinguished by orderliness are positively associated with conscientiousness, like Obsessive-compulsive disorder. Schizoid is negatively associated with extraversion. Hence, PDs are primarily the result of positive correlation with Neuroticism and negative association with Agreeableness. Extraversion is associated in both ways [3]. It is a well-known fact that personality develops during childhood and interpersonal experiences and social interactions play a significant role in the development of PDs. Parental maltreatment, stress, and traumatic life events influence the personality adversely. In addition, genetic and prenatal factors also constitute a major role. Cluster-A PDs can have polymorphisms associated with the gene coding for dopamine 2-receptor (DRD2), catechol-0-methyltransferase (COMT), Dysbindin (DTNBP1), and D-aminoacid oxidase (DAAO). These genes are also associated with the development of schizophrenia, implying that both schizophrenia and schizotypal PD are related to dopaminergic dysfunction. Cluster B PDs have been found linked to polymorphisms in genes encoding serotonin transporter (5-HTTLPR), catabolic enzyme monoamine oxidase (MAOA), and tryptophan hydroxylase enzyme related genes TPH1 and TPH2. This demonstrates the relation of the development of borderline personality and antisocial disorder with dysfunction in the serotonin system. Cluster-C PDs are linked with polymorphisms of the dopamine 3-receptor (DRD3) gene and COMT, particularly obsessive-compulsive disorder[4]. Perinatal injuries like trauma, infections like encephalitis, and hemorrhage may also be contributing factors. Genetic factors interact with environmental stresses to result in PDs. Various parental behavior like excessive attachment, parental insensitivity or emotional neglect, physical and sexual abuse, and substance use disorders causes an essential impact on PDs development. Social bullying, racial discrimination, frequent dislocations during childhood, and lack of peer support are other risk factors.

Causes

Causative factors associated with PDs include:

  1. Genetic factors with mutations in genes involving dopamine and serotonin pathways such as DRD2, COMT, DTNBP1, DAAO, 5-HTTLPR, MAOA, DRD3,TPH1 and TPH2.
  2. Environmental factors like stresses, parental treatment, sexual abuse and substance use.

Differentiating Personality disorder from other Diseases

Personality disorders present with symptoms which corresponds to other psychiatric illnesses as well. It makes imperative to employ the [DSM-5] criterion to make the diagnosis of PD. Additionally, many patients with PDs also suffer from co-morbid conditions like mood disorders, substance abuse and organic brain lesions which have overlapping symptoms and signs with PDs. This requires a complete long history including duration of symptoms and developmental history and essential investigations. Boderline disorder needs to be differentiated from mood disorders like Bipolar disorder, anxiety and delusional disorder. Cluster-A disorders have to distinguished from delusional disorder (persecutory type), schizophreniform, bipolar disorder with psychotic symptoms and schizophrenia. Post-traumatic stress disorder (PTSD) can also have interchangeable presenting complaints to the cluster-C PDs.. Thus, Axis 1 disorders and Axis 2 disorders have similar presentation and needs to be evaluated and ruled out before making the diagnosis of Axis-2 disorders.

Epidemiology and Demographics

Worldwide pooled prevalence of personality disorder as found by meta-analysis of studies conducted from 21 countries is 7.8%. Global rates of cluster-A PD is 3.8%, cluster-B is 2.8% and cluster-C PD is 5% [5]. In United States (US), it is around 10%, with major disease burden contributed by obsessive-compulsive PD followed by narcissist and borderline PD. In the rest of countries, it varies [6]. OCD is twice common in females than males and 75% of individuals diagnosed with BPD are females. No sex predilection is found with rest. Narcissist PD is found in 20% of military personals, 17% first-year medical students and 6% forensic population.

Risk Factors

Risk in development of PDs is increased with following factors:

  1. Genetic factors
  2. Perinatal injuries like trauma, infections like encephalitis and hemorrhage
  3. Parental attachment, parental misconduct, abuse, insensitivity and emotional neglect
  4. Physical and sexual abuse
  5. Use of illegal drugs or substance abuse
  6. Social bullying and racial discrimination
  7. Frequent displacements in life or a major dislocation

Natural History, Complications and Prognosis

Personality disorders usually begin to develop in early adolescence and are diagnosed in early adulthood. The symptoms are usually apparent for a long time, indicating the long-term course of the disorder in life. Moreover, it also affects functioning in several aspects that can cause personal impairment and social distress. Stigmatization exists after the diagnosis is established and prevents individuals from seeking treatment at an earlier stage. However, with appropriate psychotherapy and keeping symptoms under control with medications, disease stability is achieved and even complete remission. A follow-along study performed by Skodol et al. demonstrated that remission was seen in the case of avoidant and schizotypal PD with a greater number of positive experiences and building interpersonal skills at a young age. Another ten years follow-up study to observe remission in BPD was done by Zanarini et al., which revealed that 80% of individuals achieved remission and their 16 years follow-up showed sustained symptomatic remission [7] [8]. The complications can occur at any stage and can add to a worsening prognosis. Among them, suicidality is of foremost significance. Others include injuries from fights and accidents, sexually acquired infections from presumptuous sex, and substance use disorder. It also adds to the morbidity by causing personal functional impairment and affecting family life. The mortality in PD is more than in the general population. A famous study spanning 24 years was performed on patients with PDs, and it was found that 5.9% of patients with BPD died by suicide vs. 1.4% of the comparison group and 14% vs. 5.5% with other non-suicide causes. In addition, those patients who did not achieve recovery were at higher risk of early death [9]. Thus, PDs follow a waxing and waning course throughout life with periods marked by flares and remission. The life expectancy in such individuals is influenced by psychotherapy initiation, treatment compliance, co-morbid conditions, and social support. In most cases, it is lesser than average in the normal individual.

Personality Change Due to Another Medical Condition

Personality changes are also associated with other medical conditions, such as frontal lobe lesions. The lesions (tumors, abscess, granuloma, or cystic lesion) present with changes in personality. Substance use disorders like marijuana, alcohol, amphetamine or cannibis) also manifest personality changes. Old patients in hospitals or home may develop delirium and exhibit personality changes. Other conditions associated with personality changes include:

  1. Endocrine disorders like hypothyroidism.
  2. Long-term steroid use OR hypercortisolism.
  3. Familial disorder like Huntington disease.
  4. Automimmune disorders involving central nervous system like Systemic lupus erythematosus (SLE).
  5. CNS infections like Meningoencephalitis.
  6. Autoimmune immunodeficiency syndrome (AIDS) or HIV.
  7. Traumatic brain injuries like chronic sub-dural hematoma.

Diagnosis

Diagnostic Criteria

The diagnosis of personality disorder is intricate as most patients present with symptoms related to depression and anxiety, and many times, two or more personality disorders co-exist. Also, an overlap in certain personality characteristics among different personality disorders. Therefore, the diagnosis of a personality disorder requires a specific criterion after a complete evaluation of cognitive, behavioral, interpersonal, and social features in an individual.

  • In DSM-5, the following criteria must be met:
    • A persistent and enduring pattern of behavior and traits that do not comply with one's culture involving  2 of the following: cognition (ways of perceiving and interpreting self, others, or events), affectivity (degree or range of a person's emotional response), interpersonal functioning and impulse control.
    • This maladaptive pattern causes significant distress and impaired responses in social, occupational, and other areas.
    • The onset is in early adolescence or early adulthood, and hence, the duration of symptoms is long.
    • The symptoms should be present for greater than one year if age is less than 18 years, and for antisocial PD, age should be greater than 18 years.
    • The symptomatology cannot be explained with any other psychological or neurological abnormality, and substance abuse disorders have been ruled out.
  • In ICD-10, World Health Organization (WHO) lists the following criteria:
    • The deeply engrained and enduring pattern of behavioral tendencies in various aspects of personality, including affectivity, impulse control, arousal, perception and thinking, and style of relating to others, producing personal and social disruption.
    • The PDs may co-exist with other mental disorders; however, the behavior is continuous and not limited to episodes of mental illness.
    • The inflexible attitude started in adolescence or early adulthood and diagnosed later in life.
    • It is of long duration and follows a stable course.
    • The symptoms are responsible for considerable personal distress and significant social impairment. [10]

At least 3 of these criteria must be met, and ICD-10 adds that 'for different cultures, it may be necessary to develop specific sets of criteria with regards to social norms, rules, and obligations.'[11]

History and Symptoms

History constitutes the first step in assessing for the personality disorder in any individual. An age of 18 years for a patient is essential in the diagnosis of personality disorders. Duration of symptoms is also critical. Details of education, employment, and personal and social relationships give an insight into interpersonal functioning. Conducting a clinical interview also helps in providing a comprehensive understanding of self-identity issues if present. Family history and history of substance abuse can provide a valuable contribution in assessment for diagnosis. Such patients usually present with symptoms like frequent mood swings, anger outbursts, unstable self-image, waning social relationships, suspiciousness towards others, over-emotionality, in-sensitivity and irresponsibility towards self and others, and inconsistency in goals. They are usually ignorant towards their own behavior and have ego-syntonic symptoms. All these symptoms need to present in more than one setting.

Physical Examination

There are no specific physical signs associated with personality disorders. The physical exam is essential to rule out organic disorders and substance use disorders. Depression and anxiety need to be ruled out by conducting their assessment tools. Patients with borderline personality disorders have an increased risk of suicide, and they may have self-inflicted wounds on the body or signs of attempted suicide attempts. A complete mental status examination needs to be conducted. The first is to examine appearance and behavior. Borderline personality disorder patients may exhibit defensive behavior. Those with a paranoid personality disorder will fail to maintain eye contact. The second is mood and affect; borderline personality disorder may reveal fleeting mood and emotional states with different questions or scenarios. This is also vital to assess suicide risk in the patient. Antisocial personality disorders may be homicidal and display a hostile attitude. Cognitive functions like attention, memory, orientation, language, and intelligence are normal. Mini-mental state examination (MMSE) can be conducted for this. Histrionic PD may manifest a ‘la belle indifference,’ meaning showing an apparent lack of concern regarding their own symptoms. Perception is normal though. Moreover, the thought process is usually unremarkable. It is imperative in paranoid personality disorder to ascertain that no thoughts of harm to others are present. However, insight and judgment may be affected depending on different scenarios in patients with variable personality disorders.

Laboratory Findings

These investigations are carried out to rule out other diagnosis. No definite abnormalities are associated with PDs. The laboratory tests conducted comprises:

  1. Vitamin B12, Vitamin D and ferritin levels.
  2. Thyroid function tests, fasting glucose and cortisol levels.
  3. Toxicology screen is done to differentiate substance abuse disorder from PD and also, as substance abuse is common with PSs.
  4. Sexually Transmitted disease screening is required. HIV patients may also present with personality changes. Moreover, PD patients have impulse control disorder and may get introduced with such infections.

Imaging

Computed Tomography scan (CT scan) and Magnetic resonance imaging (MRI) are essential to rule out organic neurological causes of the presenting symptomatology. The changes observed in borderline PD are found in hypothalamus and limbic system, if the childhood trauma was the triggering event for the disorder. Impulsivity in PD is associated with changes in frontal structures and aggression with changes in hippocampal and frontal structures [12]. Electroencephalographic (EEG) changes are observed in BPD, however, they are not diagnostic. A recent study conducted by Shankar et al, demonstrated the presence of sharp and spike waves in severe BPD and nonspecific slowing waves in mild and moderate form of BPD [13].

Assessment tools

Following assessment tools are considered:

Treatment

PD affects all aspects of individual life and causes interference with psychological and behavioral growth. It causes emotional distress and social impairment. It affects the quality of life grimly and has dire consequences on life years. Early recognition is crucial to start appropriate management and prevent complications from this debilitating condition. Management of PDs lacks evidence-based guidelines, and health authorities across the world have formulated their independent guidelines. American Society of Psychiatry guidelines exists only for BPD, while European guidelines are present for BPD, ASPD, and PD general. It includes acute treatment by hospitalization if there is a risk of self or other people harm and chronic management of the disorder. Indications for inpatient management include; suicidal intent and plan, impulse control loss, imminent danger to self and others, and severe symptoms impairing functioning and unresponsive to outpatient treatment. An initial assessment should be performed. The second step is designing a treatment plan and discussing it with the patient. Family support and patient education play a vital role in effective management. Prior to starting the therapy, it is essential to rule out PTSD, depression, and anxiety and manage them if these conditions co-exist. Substance use disorder needs to be recognized and treated as well

Psychotherapy

Psychotherapy is the mainstay and core management for PDs. It is a collaborative treatment that aims to improve the perception of the disease and rectify the response to social and personal problems with ameliorated behavior. Psychodynamic psychotherapy (PDT) focuses on self-reflection and the identification of perceptual distortions. It then enables an individual to develop adaptive responses to varying stimuli. Emotional conflicts, defence mechanisms and unconscious thoughts are recognised and analysed. This is then used to counter and resolve the unconscious conflicts and relational difficulties. It is performed twice to four weeklies for many months. Cognitive-behavioral therapy (CBT) is based on recognizing distortion in thought processes and rectify the cognition pattern, thus establishing emotional stability and behavioral regulation. It is done once weekly for many months to years. It is used in ASPD, BPD, and substance use disorder. Dialectical-behavioral therapy is s subtype of CBT that reinforces and integrates positive emotions, thoughts, and behaviors by changing the negative thinking patterns. The word 'dialect' means 'synthesis or integration of opposites.' It equips patients with new enhanced coping skills to manage their painful conflicting emotions and control their impulses and self-destructing behavior. It is a significant therapy in cluster-B PDs. Interpersonal therapy comprises individual sessions that focus on improving interpersonal and social relationships. It involves finding triggers such as adjustment difficulty, role transition or dispute, and interpersonal deficit; and working together with the individual to challenge them and establish new positive roles. It is used for mood disorders and can be used in BPD. It is conducted weekly for 6-12 months. Dynamic Group psychotherapy harnesses the dynamic existing among individuals and utilizes it to bring out constructive and optimistic behaviors. Feedback from patients is beneficial to produce a therapeutic response. It is also carried out weekly for months. A multi-wave study done by Clarkin et al. in 2007 studied the PDT, Dialectal behavioral therapy, and dynamic supportive therapy in the management of BPD. It demonstrated that PDT and DST were associated with improvement in anger and impulsivity, PDT and dialectical behavioral therapy lead to improvement in suicidality, and only PDT was found to be a predictor of verbal and direct assault [14]. European guidelines have the strongest recommendation for psychotherapy for BPD. Cognitive-behavioral therapy for ASPD is recommended by British and German guidelines. American society of Psychiatry recommends dialectical behavioral therapy and psychodynamic therapy for BPD.

Medical Therapy

No medical therapy is approved by Food and Drug administration, FDA for treatment of personality disorders. Pharmacotherapy is utilised to manage symptoms during acute decompensation and trait vulnerabilities. Mood dysregulatory symptoms like emotional lability, anger outbursts, depressive crashes, and other affective dysregulation symptoms are managed with (selective serotonin reuptake inhibitors) SSRIs or selective norepinephrine reuptake inhibitors (SNRIs) like venlafaxine. Mood stabilizers like lithium, valproate, carbamazepine, lamotrigine or topiramate are used as second line. Impulse control dyscontrol symptoms are self-mutilation, aggression, eroticism, reckless sex, extravagant spending and uncontrolled substance use. They are managed with SSRIs as first line and monoamine oxidase inhibitors (MAOIs) as second line [15]. British guidelines recommend against the use of medications for these symptoms [16]. Cognitive perceptual symptoms incorporate paranoia, delusions, hallucination, derealisation, depersonalization and suspiciousness. Low dose neuroleptics or antipsychotic medications are used. They help with psychotic symptoms as well as mood issues.

Case Studies

Case #1

Related Chapters


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