When the soloist lowers her
Strad and takes a bow,
she reveals the violin’s mark
on her throat, which makes me think
of Mozart, who rehearsed so hard
and gripped his quill so tight
his hand became a claw
by the time he turned twenty-nine.

Compared to them, I can’t complain-
my injuries are nothing more than
a calloused finger from writing
progress notes, a backache from
sitting with a thousand weightless
secrets, and a neck stiffened by nodding
“yes, yes,” in time to my patients’
sad love songs, each beat
a measure of the hours I practice,
the score I still have to learn.

Articles

When the soloist lowers her Strad and takes a bow, she reveals the violin’s mark on her throat, which makes me think of Mozart...

Musician Injuries

Every practicing psychiatrist has seen a patient whose presentation resembles Chad’s. Chad consulted me almost 2 decades ago, but I remember him well. It was a few months before his 60th birthday. He was “facing mortality” and thought he might be getting depressed. Chad complained of trouble concentrating and said that simple tasks like balancing his checkbook had become more difficult. His legal work no longer engaged him, and he was spending more time on the Internet.

Chad still enjoyed working in his garden and was busy preparing the flowerbeds for a late-summer celebration of his wedding anniversary. He dearly loved his wife, but he was living with a long-standing conflict. He had “always known” that he was gay, and he had kept this a secret for his entire marriage. Now that he was turning 60, he had regrets. It was in this context that he had discovered gay pornography on the Internet. He told me that he was “becoming addicted to the porn,” and that sometimes he even watched while in his law office.

The changes in Chad seemed psychologically understandable and, yet, I had a nagging feeling that this was not an ordinary depression or “late-in-life crisis.” Chad seemed to be lacking in motivation more than suffering from a depressive mood. I also worried that perhaps his judgment was becoming impaired. In other words, Chad was an almost 60-year-old man who was reflecting on his life, rethinking choices he had made, and feeling mildly depressed; but it was possible that, in addition, Chad had a covert medical condition such as a sleep disorder or perhaps an early dementia.

Psychiatrists are the most intensively trained in the diagnosis of discrete, well-described medical/neurological conditions that might be masquerading as primary psychiatric disorders.

A medical work-up, including a neurology consultation, found no evidence of dementia. The patient’s symptoms were attributed to depression, and reassurance was recommended.

Chad stopped seeing me after 8 months. He felt somewhat better after talking about his life in psychotherapy and taking an SSRI. But his sexual preoccupations continued, and he planned to seek help from a psychologist who specialized in “sexual addiction.”

Chad’s story does not end well. He was suffering from a disease that would end his life, but it took 2 years of declining functioning before MRI evidence of his disorder became clear. Chad’s wife wrote to tell me that Chad had received a diagnosis of frontotemporal dementia (FTD). I always think of Chad when I learn of new findings about FTD. Much has been discovered about this disorder in the years since I saw him.

FTD is the second most common cause of dementia (after Alzheimer disease) in the population 

 The age of onset is usually between ages 45 and 65 but can range from 30 years to over 80. 

 is estimated to be about 15 to 22 per 100,000.

Chad had the most common subtype of FTD, now called the behavioral variant (bvFTD). Patients with bvFTD frequently present with psychiatric symptoms, often years before other features of the disease emerge.

 These individuals may look like they have late-onset major depression, psychosis, bipolar disorder, or OCD. Mental health professionals are very likely to be consulted, either by the patient or by family members. This, in and of itself, makes it important for psychiatrists to be familiar with FTD.

FTD is a brain disorder. But, in masquerading as what psychiatrists call primary psychiatric disorders, FTD challenges us to re-examine how we think about the relationship between “mental illness” and “brain disease.” Indeed, FTD inspires us to expand our knowledge-base and our clinical acumen into realms beyond the usual boundaries of our field.

In this article I will present a summary of what psychiatrists need to know about FTD. I have included summaries of case examples from the literature to convey the wide variety of possible psychiatric presentations of bvFTD.

In 1892, the Czech psychiatrist Arnold Pick described the first case of what would today be classified as a FTD. The term FTD actually refers to a large class of neurodegenerative disorders that vary in both clinical presentation and underlying pathology, but all of these disorders manifest with cortical degeneration in the frontal and temporal lobes.

FTD has been the subject of intensive research, including efforts to categorize various clinical and neuropathological subtypes. Researchers have found an association between FTD and mutations in a small number of genes (most commonly, 

). There also are accumulations of protein aggregates (for example, tau or TDP-43) in neural tissue, and it is hypothesized that these may spread from cell to cell in a way that is similar to what occurs in prion disease.

 that functional connectivity patterns may differ in bvFTD and Alzheimer disease.

With many of the clinical subtypes of FTD, patients and their families are likely to consult with neurologists because the first symptoms are disturbances of language or movement, making it clear that the problem is probably a brain disorder. These types of FTD include primary progressive aphasias, progressive supranuclear palsy, corticobasal syndrome, and FTD with motor neuron disease. (Amyotrophic lateral sclerosis [ALS] is the most common motor neuron disease in adults.)

 Patients who have the most common type of FTD, the behavioral variant (bvFTD), are much more likely to come to the attention of a psychiatrist because they first present with changes in behavior and personality, impaired executive functioning, and/or symptoms that mimic late-onset primary psychiatric disorders. BvFTD often erodes qualities that are considered to be the very essence of the patient’s temperament, character, identity, and sense of values.

The most common early symptoms of bvFTD include various combinations of the following:

 Disinhibition manifests as behaviors that are socially inappropriate, impulsive, or careless, or that exhibit poor judgment and lack of consideration of consequences. Poor manners, lack of social decorum, and an absence of any sense of embarrassment are characteristic. Patients may also be irritable, superficially jocular and euphoric, or depressive.

 Apathy may express itself as inertia, disinterest, social withdrawal, and/or a lack of engagement, drive, or motivation.

 Lack of empathy may manifest as self-involvement, diminished interpersonal warmth, loss of sympathy for others, and/or lack of regard for the effect of the individual’s behaviors on the feelings of others.

 Repetitive, stereotyped, or perseverative behaviors may include simple movements or speech patterns or more complex rituals and compulsions.

 Substantial changes in food preferences and eating habits may involve a shift to over-consumption of sweets and carbohydrates, binge eating, gluttony, and substantial weight gain. Hyperorality eventually may include mouthing of non-food objects.

 Impairment of executive functions is common, while memory and spatial orientation are relatively preserved.

The diagnostic challenge of bvFTD for psychiatrists. 

The gradual emergence of symptoms in bvFTD can make it difficult, early on, to recognize this disorder as being something other than situational. In addition, when patients and their families believe the problem is psychiatric, this “frames” the problem and may bias the initial diagnostic impression for the clinicians with whom they consult.

Patients with early bvFTD are also a diagnostic challenge because of the substantial overlap between the symptoms of FTD and the symptoms of certain primary psychiatric conditions: disinhibition and hypomania or ADHD; stereotyped/repetitive behaviors and OCD; and also apathy and depression. MDD is the most common psychiatric diagnosis given to patients who eventually receive a diagnosis of bvFTD.

 included this testimony from the wife of “A 31-Year-Old Man with Personality Changes and Progressive Neurologic Decline.”

 This patient was remarkably young and had an unusually rapid downhill course. The wife succinctly describes characteristic behavioral changes of bvFTD in her husband and, sadly, the number of medical professionals who missed his diagnosis.

During my pregnancy, my gregarious husband became socially withdrawn. His behavior became erratic: he bought things we could not afford, began having trouble meeting deadlines and following the work dress code, and withdrew from his friends. He became obsessed with listening repeatedly to Harry Potter audio books, to the extent that when I was in labor, I had to ask him to put his iPod away.

By the time our child was 6 months old, my husband was functioning so poorly that I did not feel comfortable leaving them alone together. Accompanying him to a routine appointment, I said to his primary care physician, “My husband is acting so weird, does he have a brain tumor?” She was the first of eight medical and mental health professionals who failed to make the diagnosis.

This patient died just after his child turned 4. The cause of his bvFTD was thought to have been a spontaneous mutation in the MAPT gene, one of a small set of genes that have been associated with FTD.

Most cases of FTD are due to mutations that are sporadic (spontaneous).

 However, in 40% of FTD cases there is a family history of dementia, psychiatric disease, and/or motor neuron disease, and in 10% to 20% of these cases the mutations are autosomal dominant.

Behavioral variant FTD associated with mutations in 

In 2011, 2 groups of researchers independently identified the most common genetic cause for bvFTD as an expansion mutation (extra hexanucleotide repeats: GGGGCC) on chromosome 9 (C9) of the 

 This mutation was also found to be probably the most common genetic cause of ALS and the FTD-ALS complex.

Realizing that sound information about the subset of FTD patients who exhibit the 

 mutation would be important for practicing psychiatrists, the American Neuropsychiatric Association (ANPA) Committee on Research reviewed the literature on psychiatric aspects of this mutation and published their report in February 2017.

The committee found that patients with expansion mutations in the 

 gene may experience psychiatric symptoms for 4 or 5 years before the emergence of the more typical manifestations of bvFTD. In addition, neuroimaging results initially may be negative, and one cannot count on the presence of a family history of dementia; many patients have only a family history of what sound like psychiatric disorders.

The ANPA Committee literature review also elucidated what is currently known about the most common psychiatric presentations of FTD patients with C9orf72 mutations.

The most common psychiatric presentations in FTD patients with 

. Psychotic symptoms appear to be the most common and may take the form of “various combinations of delusions or overinvested ideas and multimodal hallucinations.”

 The delusions may be persecutory, jealous, religious/mystical, or grandiose, and somatic preoccupations are common. Generally, in the published literature, the patients’ psychotic symptoms were not responsive to antipsychotic medications.

Less often, onset of bipolar disorder with mania after the age of 40 was the manifestation of 

 mutations. The small number of patients with late-onset bipolar disorder with mania who were reported in the literature tended to respond to treatment with lithium. In addition, patients with 

 expansion mutations may present with other mood disturbances, including “depressive episodes with or without catatonia, dysphoria with suicidal thoughts or attempts, and depression-related cognitive disturbances (‘pseudodementia’).”

Late-onset OCD has been described as part of the prodrome of 

 mutations, but OCD-like symptoms are commonly seen as part of the presenting picture in bvFTD even in patients who do not have this specific mutation.

As part of their longitudinal studies on aging and neurodegenerative disease, the University of California, San Francisco Memory and Aging Center found that some of their patients had 

. This gave them an opportunity to prospectively follow individuals who carried this mutation and to “characterize psychiatric symptomatology during the earliest phases of the illness.”

 In 2015, they published a paper that included 3 of these patients, summarized below.

FTD developed in each of these 3 patients; however, given that these are single case examples, one cannot assume a simple causal relationship between the 

 gene mutation and the patient’s psychiatric symptoms. These case examples demonstrate how the diagnosis of neuropsychiatric disorders is a dynamic process that unfolds over time and that it is important to include FTD in the differential when encountering these kinds of presentations.

 Mr. M first began to have delusions and auditory hallucinations at age 58. He heard “voices from other continents that spoke to him through his hearing aid and eyeglasses.” He would cover mirrors to keep from being observed and hide in the closet to elude the Mafia. He also began to think that he was a “sex god,” and he went into his neighbors’ homes with the idea that they were interested in having sex with him. These delusions and hallucinations lessened over 2 years, giving way to apathy and a voracious appetite; often he would eat whatever was in front of him, food as well as non-food objects.

 Ms. G had a first manic episode at age 53. Her symptoms included rapid thought, grandiose notions such as intentions to start a new religion, poor sleep, and then over-familiarity with strangers. She was hospitalized and responded to treatment with lithium. Diagnosed with bipolar disorder, she was stable on valproic acid until age 63. At this point her husband felt that her cognition was below her baseline, and she was seen at the Memory and Aging Center. There, she was found to have very mild executive dysfunction and verbal memory impairment; she received a diagnosis of mild cognitive impairment. Two years later, her affect had become strange and she became disinhibited. By age 68 she had ALS and was wheelchair-bound.

. At age 60, Ms. A began to display rigid, obsessive-compulsive behaviors, including checking email compulsively and obsessively organizing household items. A community physician diagnosed OCD. Then, over the next 2 years, she became increasingly apathetic and withdrawn. Her judgment deteriorated, her empathy diminished, and she became disinhibited. Some of her behaviors included personal postings on an Internet sex site, losing money in an on-line scam, and making inappropriate remarks in public.

Pointers for psychiatrists in evaluating patients who might have bvFTD

With current information about bvFTD, how might one approach the psychiatric evaluation of a patient like Chad? The 

 provides some important points to help the psychiatrist when evaluating patients with suspected bvFTD.

Early identification of bvFTD allows patients to have time to put their affairs in order and to participate in planning for their own future care. In addition, an accurate diagnosis is extremely helpful to family members who often are befuddled by the changes in their loved ones’ behaviors and who also may be facing extremely difficult social, financial, and/or legal predicaments.

Among mental health professionals, psychiatrists are the most intensively trained in the diagnosis of discrete, well-described medical/neurological conditions that might be masquerading as primary psychiatric disorders. When it comes to bvFTD, psychiatrists have an especially important role in the early identification of patients who may have this disorder.

Dr. Schildkrout is Assistant Professor of Psychiatry, Part-time, Harvard Medical School, Beth Israel Deaconess Medical Center, Boston, MA. She is the author of 2 books, 

Unmasking Psychological Symptoms: How Therapists Can Learn to Recognize the Psychological Presentation of Medical Disorders

Masquerading Symptoms: Uncovering Physical Illnesses That Present as Psychological Problems

 Ducharme S, Bajestan S, Dickerson BC, Voon V. 

Psychiatric presentations of C9orf72 mutation: what are the diagnostic implications for clinicians?

Network dysfunction in Alzheimer’s disease and frontotemporal dementia: implications for psychiatry

 Miller BL, Dickerson BC, Lucente DE, et al. 

Case 9-2015: a 31-year-old man with personality changes and progressive neurologic decline

 Block NR, Sharon JS, Karydas AM, et al. Frontotemporal dementia and psychiatric illness: emerging clinical and biological links in gene carriers. 

Here: what psychiatrists need to know about frontotemporal dementia. Case examples convey the variety of possible psychiatric presentations of behavioral variant FTD. 

Frontotemporal Dementia: A Brain Disease That Challenges Definitions of Mental Illness

I am often asked by psychiatrists who are not trained in psychodynamic psychiatry about what I do and how I think about patients and their treatments. In response, I often say that a key difference lies in how psychodynamic psychiatrists view clinical material, evaluate patients, and make-or know when to withhold, depending on timing-interpretations. We also often turn to supervisors, consultants, and peers. With the goal of engaging more colleagues and encouraging them to pursue this particular mode of treatment, I present the following fictionalized case based on a real patient but with fabricated identifying information.

Sophia is single, in her early 30s, and lives with a roommate in an apartment in the city in which I practice. She commutes into the city daily, where she works full time as an executive administrative assistant. Many years ago, she received a diagnosis of depression and was treated with an SSRI and psychotherapy. She has no medical history and has never been suicidal.

Sophia initially presented to me for evaluation with the chief complaint of “I don’t feel that connected to my life.” She was referred by her longtime psychiatrist (Dr. A), who initially treated her with psychotherapy and later with medication. Dr. A recognized early on that Sophia might benefit from psychoanalysis and discussed this as an option with her. She was interested but did not feel ready to commit until many years later.

During my evaluation, Sophia stated that she was now able to commit the time and effort for psychodynamic psychotherapy, since she was planning to leave her job and return to school full time. She was interested in improving her relationships with people, especially men, and wanted to figure out why she has had such difficulty in choosing a satisfying career path. Sophia had begun to feel that time was running out and wanted resolve these matters before life passed her by.

At the time of my evaluation, Sophia was planning to pursue a career in medicine. She had enrolled in a graduate program but continued to work full time. For the first year of treatment with me (consisting of twice-weekly psychotherapy for 3 years and then converting to 4-times-a-week, on-the-couch psychoanalysis), she continued to struggle with whether she wanted to commit to a career in medicine, become a therapist, or perhaps choose a different career path altogether, such as one as a physicist (she was doing very well in her physics classes).

Her vague way of relating, complaints of poor memory, and not knowing what she wanted to do with her life or whom she wanted to be with made me quite skeptical about the benefits of psychoanalytic treatment for this patient. Her identity diffusion as well as a significant Axis I MDD that might well deteriorate in the face of such an intensive treatment made me initially question Dr. A’s recommendation for psychoanalysis. Furthermore, she had fled treatment with Dr. A on several occasions over the years.

Sophia presented herself and others in her life in a vague, 2-dimensional manner. I think she sensed my wariness and countered in like fashion, often reminding me that she was not committed to staying in town (she was seriously considering transferring to a school in a distant city), to her current job or plans to attend medical school, and therefore by inference to our treatment. In addition to Dr. A, a consultant at the time of the evaluation also considered Sophia to be sufficiently structured to tolerate psychoanalysis. I tentatively agreed but reserved judgment in the early years of treatment, as I kept treating her in a psychodynamic psychotherapy.

I learned that Sophia was born to married parents in the suburbs, with 2 siblings (she is the middle child). She had graduated at the top of her class in high school and attended a prestigious college, where she had a double major in the biological sciences and humanities. She had intended to go to medical school after receiving her BA. Yet, she continually questioned her capabilities, constantly comparing herself with her peers, which negatively affected her self-confidence and left her questioning whether she had what it took to become a physician.

Sophia described her father as an energetic, personable man “who values intelligence and education.” He has his own consulting firm and also teaches at a local college. Her mother, “a depressive woman,” gave up her career as an accounting assistant to join her husband’s consulting firm, which she now regrets. Sophia’s mother believes the firm is struggling financially because of the rigidly obsessional nature of Sophia’s father and resulting inability to complete projects and bill clients on time.

Sophia’s mother often asked her for money. Before her treatment, Sophia would always readily give it to her, yet worried about ever being able to own a house or even a car. When I questioned Sophia about this, she expressed guilt over being so fortunate in life and felt a duty to help others in need. Sophia regularly paid for lunches and dinners out with friends and family and showered them with extravagant gifts, even when they could well afford to pay for themselves. They rarely, if ever, reciprocated.

Sophia’s older brother is single (previously engaged) and a successful investment banker. Sophia described their relationship as contentious and competitive. For example, they did not speak for a year after she won a game of Monopoly. Her younger sister is single and lives with roommates in another city and works as a paralegal. She and Sophia are extremely close and speak on the phone or text daily. Sophia describes her sister as outgoing, athletic, and adventurous and wonders why “such a catch” remains single.

Sophia has had 2 heterosexual relationships in her lifetime. The first relationship was in high school, and the second was with a man that she met in college and dated on and off over the years. The first relationship was primarily sexual, with a boy who would not acknowledge her as his girlfriend. She described him as athletic, outgoing, and ambitious. Sophia was heartbroken to discover that he was seeing other girls. She became very depressed with increased tearfulness, anhedonia, and loss of appetite; she dropped out of intramural sports and became increasingly socially isolated.

Her second relationship began in her freshman year of college. He was not a student but lived in town and was a distant friend of the family. He held a series of manual labor jobs and had dropped out of school in the eighth grade. They dated on and off while she was in college and maintained a long-distance relationship after she graduated. They became engaged around that time, but Sophia developed cold feet and broke off the engagement. Soon after, he met someone else and fathered a child. Learning this news was the main trigger for Sophia’s second depressive episode.

Over the years, their relationship continued in an on-again, off-again manner; his marriage eventually dissolved and they continued to maintain contact, even living together for a brief time. Each time they spoke of marriage and children, Sophia got cold feet and ended things, although she desperately wanted a partner and children.

In treatment, Sophia initially presented herself as a model patient, showing up early for appointments, rarely canceling, and paying on time, if not early. This continued for the first 2 years, after which things began to change. Around that time, she began canceling and missing sessions, showing up and paying late, and questioning the treatment in general.

When this shift occurred, I initially found myself colluding with Sophia’s passive-aggressive behaviors, allowing myself to become distracted by her apologies and promises that she was going to improve her behavior. This assuaged my anger until the next time. I soon began to realize that we were avoiding speaking about what might be going on in the treatment that made her cancel or be late for her appointments.

This eventually came to a head after I came back from vacation one summer. Sophia returned from the break stating that she wanted to have fewer sessions each week and also requested early morning appointments. Rather than exploring these requests and what they meant to her, I discouraged her from doing so and told her that I did not have any early morning appointments available. I was commuting into the city daily and felt very reluctant to give her early appointments in order to avoid the heavy rush hour traffic.

Sophia responded by canceling more sessions in the following months, showing up late on a number of occasions, and questioning the treatment and whether we were suited to be working together. After I repeatedly asked her about and tried to explore the missed and late sessions, one day she exclaimed:

 It would be easier to get here on time than talk about why I was 45 minutes late. It would just be easier to be on time. I don’t really enjoy having to dissect why I was late. I get that it says something, I guess I just don’t want to explore what it is saying. I’d just rather be on time.

 But you haven’t been. What’s that about?

 Being late all the time says something about how much or little I prioritize being here. It’s not something I would come out and say, necessarily. Well, I guess I just did. . . . Yeah, but I guess being late says maybe I don’t respect your time as much as I should.

At the time, I was struck by how devalued and not prioritized I felt as a result of Sophia’s declaration. Suddenly, I realized that perhaps she was making me feel the way she feels most of the time out in the world. Knowing this intellectually and really feeling it firsthand emotionally were 2 completely different things, and I REALLY felt it in this moment. I also realized that I had not been prioritizing Sophia either. So I responded:

 You saying that, makes me wonder how much of a priority you feel you are with me and with others.

 Um, priority, I don’t think I’m top priority for anyone. I factor into peoples’ priorities, like my parents. But now that I’m older I don’t feel top priority with them either. With you, I don’t know what priority I am. As MUCH as I’ve been coming here and we’ve been working together, that says something. But like, I guess I don’t know what kind of priority I am to other people.

 Maybe sometimes it’s just better not to look, than to look and realize you are not a priority.

 Well, yeah, definitely. If you’re not looking at it, then you don’t need to be concerned. I’d rather just not be aware. It creeps into my awareness at times, but it’s pretty simple to think of other things and distract myself.

At the time, and even now in looking back, I experienced this session as a turning point in my work with Sophia. I was beginning to prioritize and think more about her. Perhaps she found both my emotional absence and then my renewed interest equally distressing. I wish I had said that to her in this session, that sometimes it’s just as difficult to realize that you are being noticed and paid attention to by another as it is to realize that you may not be.

I wish I could say this session marked a magical turning point with Sophia, and that after this breakthrough she suddenly started coming more regularly to sessions and stopped being late. It did help somewhat, but not drastically, and certainly not right away. More importantly, however, it helped provide me with more insight into Sophia’s experience of being in the world, which she had carefully kept hidden, and her conflicted feelings about me realizing it. Furthermore, she surprised me with her willingness to show up and reflect on events such as these during the sessions. As a result, I have begun to realize that this has been the goal all along, and that the behavioral changes were secondary to being able to consistently show up and reflect on what is going on in the treatment.

Over time, I have observed positive changes in Sophia’s mental status. She has become better groomed; her hair is longer and attractively styled. She went clothes shopping for the first time in years, began to develop a style, and became interested in accessories, spending a few sessions discussing purses and shoes with me. She also started to wear contact lenses, which has further helped in gauging her increased eye contact. She consistently attempts to be more social (attending parties, going out with men, considering online dating, and reaching out to family and friends for increased contact). In the beginning, Sophia’s affect was very constricted. Her countenance has since relaxed considerably, and she now shows a spontaneous range of emotions during sessions, including laughing, smiling, and expressing sadness.

I often say that a key difference between general psychiatry and psychodynamic psychiatry lies in how psychodynamic psychiatrists view clinical material, evaluate patients, and make-or know when to withhold, depending on timing-interpretations.

Her cloudy, vague disposition has gradually given way to a more clearly expressed sense of self and an improved memory (she continued to do remarkably well in her graduate classes, often earning top scores on exams). Both she and I have come to understand the vagueness and confusion as defenses that prevent others from seeing and knowing her. She has become more aware of and in touch with her anger, envy, and competitive strivings, and better able to tolerate emotional intimacy without fleeing.

Since the early days, I have carried with me a mental image of Sophia, beautiful and strong, laughing in a carefree manner with her peers. A consultant remarked on this being reminiscent of Hans Loewald, who believed it was important in analytic work to have such a mental snapshot in mind: “An image of the patient, not as he is at the moment, but as he may yet become. The analyst, in other words, holds this image safe in keeping for the patient until such a time as he may lay claim to it as his own.” I have often thought, in looking back, that it was perhaps this very image of Sophia that kept me going when things felt most difficult. I like to think that she sensed it too-my faith in her-and that this is what helped keep her in treatment despite her worst doubts and wishes to defeat me by proving me wrong.

Dr. A and my consultant were right: psychoanalysis was ultimately the treatment that helped Sophia change. Her predominant defenses are in fact higher order and include intellectualization, reaction formation, and repression. She possesses several strengths, including her formidable intelligence, subtle humor, considerable compassion, and strong work ethic. She does not and has never abused substances or alcohol, remains adherent with her medications, and has been able to maintain many long-term relationships with friends-dating back to childhood.

One day, early in treatment, I asked whether she thought she had a sense of herself but kept it hidden from others and, to some extent, herself. Or did she really not know? Without missing a beat, she firmly said the former. Something in me believed her, or wanted to believe her. Sure enough, this hidden self has slowly but surely begun to unfurl. And (perhaps as a result) we have certainly had our share of rough patches, yet we both still continue to show up and talk about it, which ultimately is what matters.

I am grateful to David Lopez, MD, and Jennifer Downey, MD, of the American Academy of Psychoanalysis and Dynamic Psychiatry (AAPDP) for their assistance in publishing this article. AAPDP is the affiliate society of the American Psychiatric Association, dedicated to the field of psychodynamic psychiatry. Its mission is to promote the continuing education of psychodynamic principles in the evaluation and treatment of patients in clinical, medical education, and residency training settings.

Jacobs TJ. Hans Loewald: an appreciation. In: Jacobs TJ. 

The Possible Profession: The Analytic Process of Change

Loewald H. On the therapeutic action of psychoanalysis. 

Dr. Hyun is Assistant Clinical Professor of Psychiatry at Columbia University in New York and is former Trustee of the American Academy of Psychoanalysis and Dynamic Psychiatry. She has a private practice in New York and New Jersey. Dr. Hyun reports no conflicts of interest concerning the subject matter of this article.

The author presents a fictionalized case based on a real patient to encourage colleagues to pursue psychodynamic psychiatry.

Psychodynamic Psychiatry: A Case Report

All other clinicians either will receive a CME Attendance Certificate or may choose any of the types of CE credit being offered.

To identify the psychiatric and neurological implications of anti-NMDA receptor encephalitis.

At the end of this CE activity, participants should be able to:

• Recognize when a patient may be presenting with anti-NMDA receptor encephalitis

• Describe the progression of anti-NMDA receptor encephalitis symptoms

• Identify psychiatric and neurological signs and symptoms

• Describe the differential diagnosis for anti-NMDA receptor encephalitis symptoms

This continuing medical education activity is intended for psychiatrists, psychologists, primary care physicians, physician assistants, nurse practitioners, and other health care professionals who seek to improve their care for patients with mental health disorders.

CME Credit (Physicians): This activity has been planned and implemented in accordance with the Essential Areas and policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint providership of CME Outfitters, LLC, and Psychiatric Times. CME Outfitters, LLC, is accredited by the ACCME to provide continuing medical education for physicians.

CME Outfitters designates this enduring material for a maximum of 1.5 

™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Note to Nurse Practitioners and Physician Assistants: AANPCP and AAPA accept certificates of participation for educational activities certified for 

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-methyl-D-aspartate receptor (NMDAR) encephalitis was first reported in 2005 but not identified as a diagnostic entity until 2007.

 At that time, 12 female patients were identified; they ranged in age from 14 to 44 years. All presented with various psychiatric and neurological symptoms, including personality and behavioral changes, paranoia, agitation, confusion, catatonia, autonomic instability, dyskinetic movements, seizures, hypoventilation requiring mechanical ventilation, hyperthermia (less often hypothermia), and coma.

All 12 patients had serum/cerebrospinal fluid (CSF) antibodies to the NR1 subunit of the NMDAR. Moreover, 11 had ovarian teratomas, of which 5 were examined and found to have nervous tissue expressing NR2 subunits that reacted with patient antibodies. The teratoma was removed in 8 patients, followed by immunotherapy that resulted in improvement of symptoms or full recovery. Two of three patients who did not have the tumor resected died.

Anti-NMDAR encephalitis is recognized as an autoimmune disorder in which IgG autoantibodies are directed against the NR1 subunit of the NMDAR. Binding of the antibodies to NMDARs induces internalization of the receptors in synapses, which is a mechanism that agrees with the glutamate hypothesis of schizophrenia in which NMDAR antagonists cause psychotic symptoms. The condition can present as a paraneoplastic syndrome that is often but not always associated with ovarian teratoma. However, in many patients no tumor can be found. A general definition of “paraneoplastic syndrome” is one that is caused by cancer but not by direct invasion so that most are the result of hormones, cytokines, and immune responses from or as a result of the tumor.

Hundreds of cases of anti-NMDAR encephalitis have been reported, and many patients -possibly up to 40% -are initially hospitalized on psychiatric units.

 Some patients may present with psychiatric symptoms without neurological involvement, and there may not be any differences in demographics, psychiatric symptoms, or outcomes compared with patients who have neurological symptoms. In Dalmau’s original case series, 77% of cases were initially seen by psychiatrists and 23% by neurologists.

 Because of the significant morbidity and mortality associated with NMDAR encephalitis, it is essential that psychiatrists in all settings but especially in inpatient, emergency, and consultation-liaison roles be acquainted with the presentation and diagnosis of the condition.

Most cases of anti-NMDAR encephalitis occur in young women, although ages range from 2 months to 85 years. Prodromal symptoms are common (more than 75% of patients) and usually appear as a viral syndrome with headache, fever, and respiratory and gastrointestinal symptoms. The initial psychiatric symptoms may include prominent visual and auditory hallucinations, paranoia, delusions, agitation, memory loss, depression, and eating disorders (

The condition usually progresses to include serious neurological symptoms such as seizures, abnormal movements, autonomic instability, and hypoventilation. Seizures may include any type but especially generalized tonic-clonic or partial complex. Dyskinesias may be of any type but include orofacial, complex extremity, abdominal and pelvic movements, abnormal postures, and rigidity. A frequent pattern seen in case reports is admission to a psychiatric unit followed by transfer to an ICU after the onset of serious neurological symptoms. At this point, treatment usually requires a multidisciplinary approach.

Similar symptoms may be observed in children, although the youngest may have less apparent psychiatric symptoms and more prominent abnormalities of sleep, speech, and/or gait that progress to motor abnormalities, dyskinesias, and seizures. Tumors are found less often in pediatric cases. In older patients (older than 45 years), behavioral changes, seizures, and cognitive problems are frequent; males represent a greater proportion of cases than in younger groups; and fewer have an identifiable tumor compared with younger patients. Tumors, especially in older patients, can include not only ovarian tumors but also carcinomas and tumors of the breast, lung, and thymus as well as melanocytic nevi. In male cases, fewer tumors are found but have included schwannoma and mediastinal and testicular teratomas.

Psychiatric misdiagnosis can occur before the onset of neurological symptoms. Early diagnoses have included schizophrenia, schizoaffective disorder, bipolar disorder, depression, conversion disorder, and anorexia nervosa (

). However, most cases of schizophrenia, new-onset psychosis, and depression do not involve anti-NMDAR antibodies. Although most patients with anti-NMDAR encephalitis have no prior psychiatric histories, some do -including schizophrenia and autism, or positive family histories of mental illness. In cases of anti-NMDAR encephalitis in adults with preexisting autism or intellectual disability, initial presentations may be mistaken for new-onset primary psychosis or medication effects.

One case report describes a young woman with a 7-year history of schizophrenia who received a diagnosis of anti-NMDAR encephalitis, although the relationship of her prior schizophrenia to the newly diagnosed encephalitis could not be discerned.

 In another case report, a young woman with a 14-year history of neuropsychiatric symptoms presented as an outpatient with symptoms of word-finding difficulties, later diagnosed as anti-NMDAR encephalitis.

Anti-NMDAR encephalitis can occur during pregnancy and can resemble postpartum depression and neuroleptic malignant syndrome (NMS). Many symptoms of NMS (rigidity, hyperthermia, elevated serum creatine kinase, rhabdomyolysis) may also occur in anti-NMDAR encephalitis. Testing for anti-NMDAR antibodies should therefore be considered in any patient with acute-onset psychiatric symptoms who has neurological or autonomic abnormalities, including rigidity and altered consciousness. (See Sansing and colleagues

 for an in-depth discussion of differential diagnoses for anti-NMDAR encephalitis, NMS, and serotonin syndromes.)

Psychiatrically and neurologically, the differential diagnosis is quite broad (

). A key factor for recognizing this disorder is that it often follows a pattern of syndrome development. In about 70% of cases, prodromal symptoms develop, including headache, fever, and gastrointestinal and upper respiratory tract symptoms. Usually within 2 weeks, psychiatric symptoms emerge, which often leads to psychiatric assessment. These symptoms may include anxiety, insomnia, delusions, mania and paranoia, possibly social withdrawal, and stereotypical behaviors. Short-term memory loss occurs as does disrupted language.

The condition progresses to decreased responsiveness with agitation, catatonia, abnormal movements, and autonomic instability and “storms,” as well as hypoventilation that may require ventilation. Seizures including status epilepticus can occur at any stage, can overlap with abnormal movements, and can decrease over the course of the illness.

The diagnosis is often made on the presence of neurological impairment, including abnormal movements, speech problems, rigidity, and altered consciousness as indications of something other than primary psychiatric disorders, although these are not universal. Drug screening and medication history may help rule out intoxication. Encephalitis from other causes including infections and autoimmune responses can be assessed with basic laboratory screenings, metabolic testing, immunological testing, MRI findings, and response to treatments such as thiamine and corticosteroids. Once anti- NMDAR encephalitis is diagnosed, care of the patient usually involves psychiatric consultation on ICU, surgery, medical, and/or neurological units.

Very small and even microscopic tumors can cause anti-NMDAR encephalitis, and the condition can occur as a complication of herpes simplex encephalitis. Initial laboratory studies can be normal, electroencephalograms (EEGs) can show non-specific slowing, and CSF analysis may show only lymphocytic pleocytosis. Confirmation of the diagnosis is based on positivity of the serum or, more importantly, the CSF for antibodies to the GluN1 NMDA receptor subunit. Serum testing alone may result in false positive or negative results.

Following tumor resection, if a tumor is found, treatment often begins with first-line immunotherapy (corticosteroids, plasma exchange, intravenous immunoglobulin). Second-line immunotherapy (rituximab, cyclophosphamide) may become necessary and increases the likelihood of better outcome.

Psychiatric management includes psychotropic medications, although the response can be poor especially before a precise diagnosis is made. Irritability toward caregivers and family is often reported, and suicidal ideation with severe agitation and aggression can occur. ECT has been used successfully in some cases of catatonia but is not well studied and not always successful. Some treatment recommendations include atypical antipsychotics for behavioral symptoms, valproic acid for mood dysregulation, and benzodiazepines for catatonia. Medically induced comas have sometimes been required in the most psychiatrically severe cases.

Recovery depends on many factors, but Dalmau’s original case series remains a general guide: 75% of patients recovered, although some still had mild residual deficits, and 25% had poor outcomes with severe residual deficits or died. Because anti-NMDAR encephalitis is potentially curable, early diagnosis and treatment including tumor removal and immunotherapy are key to best outcomes. Antipsychotic medications can help control psychotic symptoms; however, be aware that, especially before diagnosis, rigidity and autonomic instability from anti-NMDAR encephalitis can be mistaken for NMS, and antipsychotic medications can cause NMS in these patients.

Improvement of psychiatric and neurological symptoms during treatment can be observed within days -in some cases hours -after tumor resection, but full recovery may be a slow process, perhaps taking several months, with possible relapses. Most neurological symptoms may improve, but executive functions and severe psychiatric symptoms, including suicidality and aggression, can persist for many months, possibly requiring inpatient psychiatric treatment. Lengthy outpatient psychiatric treatment with antipsychotic medication may be necessary. Recurrences caused by recurring tumors have also been reported.

Much remains to be learned about the various presentations of anti- NMDAR encephalitis. A case report, for example, described an adolescent who presented to an outpatient service and received a diagnosis of atypical anorexia nervosa without secondary amenorrhea.

 She was treated as an outpatient until 3 weeks later when she was admitted with a seizure. On day 23 of hospitalization, hallucinations developed that did not respond to antipsychotic medications. They were initially diagnosed as dissociative because the neurological workup was mostly non-specific. The diagnosis of anti- NMDAR encephalitis was confirmed 36 days after admission.

No tumor was found, but near-baseline functioning was achieved with immunotherapy after 10 months. The case is atypical because most patients with anti-NMDAR encephalitis do not have an identifiable eating disorder.

Future research on anti-NMDAR encephalitis will contribute to our knowledge of psychosis, autoimmunity, and glutamatergic function; although the extent to which the condition applies to specific conditions such as schizophrenia remains unknown. However, the recognition of anti-NMDAR encephalitis has already led to further research that contributed to the discovery of other autoimmune synaptic encephalitides. And, the elucidation of anti-NMDAR encephalitis has led to new approaches to the diagnosis of catatonia, movement abnormalities, memory problems, seizures, and limbic encephalitis.

Jenny, a 23-year-old white graduate student with no psychiatric history, has had a flu-like condition with headache and nausea for about 10 days, and she has been having problems in school because of unusual memory loss. At home, she seems lethargic during the day but is unable to sleep at night. Her family reports that Jenny has become irritable, which is out of character for her. They brought her to the ED after she was found wandering confused in the yard.

An uncle has bipolar disorder, and the family is concerned she may have the same condition. She has a temperature of 100ºF, but baseline blood work and physical and neurological examination results are normal. A brain CT scan is unremarkable. Psychiatric assessment finds symptoms consistent with mild depression, but she is alert and oriented. There are no findings that support admission. Antibiotics are prescribed, and the patient is discharged.

A few days later she returns to the ED after becoming agitated and paranoid and experiencing auditory hallucinations. She is admitted to the psychiatric unit, where she exhibits bizarre behavior including giggling, echolalia, and posturing. She has frequent cycling leg movements and believes that staff members are plotting against her and that her food is poisoned. She is hyperkinetic, screaming, hallucinating, and sexually disinhibited. Antipsychotic medications do not seem effective, and prominent orofacial dyskinetic movements develop.

She experiences altered mental status followed by a generalized tonic-clonic seizure, although she has no history of seizures. An EEG shows nonspecific slowing, and leukocytosis is noted in the CSF. She does not return to her previous level of consciousness, autonomic instability develops, and she is transferred to the ICU.

In the ICU she requires sedation and mechanical ventilation. Subsequent EEGs reveal only nonspecific slowing, and a brain MRI scan shows non-specific small hyperintensities. During an extensive work-up, anti-NMDA receptor antibodies are found in her CSF. CT scanning of the chest, abdomen, and pelvis reveals a small right ovarian mass. The tumor is surgically resected and diagnosed as a teratoma.

Three days after the operation, she starts to improve significantly and is extubated. She receives first-line immunotherapy before discharge to a rehabilitation unit. Several months later, her neurological and psychiatric examinations are normal and she returns to academic study.

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Assistant Clinical (Adjunct) Professor of Psychiatry, VCU [Virginia Commonwealth University] School of Medicine, Department of Psychiatry, Richmond, VA

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-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. 

. Oxford, UK: Oxford University Press; 2011.

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 Dalmau J, Gleichman AJ, Hughes EG, et al. Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. 

 Chen SL, Lee SY, Chang YH, et al. Inflammation in patients with schizophrenia: the therapeutic benefits of risperidone plus add-on dextromethorphan. 

 Heekin RD, Catalano MC, Frontera AT, Catalano G. Anti-NMDA receptor encephalitis in a patient with previous psychosis and neurological abnormalities: a diagnostic challenge. 

 Sansing LH, Tuzun E, Ko MW, et al. A patient with encephalitis associated with NMDA receptor antibodies. 

 Mechelhoff D, van Noort BM, Weschke B, et al. Anti-NMDA receptor encephalitis presenting as atypical anorexia nervosa: an adolescent case report. 

 Florance NR, Davis RL, Lam C, et al. Anti-

-methyl-D-aspartate receptor (NMDAR) encephalitis in children and adolescents. 

 Barry H, Byrne S, Barrett E, et al. Anti-

-methyl-D-aspartate receptor encephalitis: review of clinical presentation, diagnosis, and treatment. 

 Suhs KW, Wegner F, Skripuletz T, et al. Heterogeneity of clinical features and corresponding antibodies in seven patients with anti-NMDA receptor encephalitis. 

 Maccaferri GE, Rossetti AO, Dalmau J, Berney A. Anti-

-methyl-D-aspartate receptor encephalitis: a new challenging entity for consultation-liaison psychiatrists. 

This articles focuses on the psychiatric and neurological implications of anti-NMDA receptor encephalitis. 

Anti-NMDA Receptor Encephalitis: Diagnostic Issues for Psychiatrists

I don’t know about you, but I’m exhausted. I’m sure it has something to do with my arthroscopic surgery to repair a torn meniscus and the resulting tendonitis in my other ankle due to too much pressure on an old injury (skateboarding 40 years ago, don’t ask). It’s possible my inability to do my usual relaxation therapy by digging in my garden is playing a role. And it’s possible it’s the several hours a day of rehab and having to walk around with a boot on the leg with the tendonitis, though it’s more likely the onslaught of the crazy news (I was tempted to title this “Bizarro World Redux,” for those of you who read my “Bizarro World”editorial in the April issue).

My wife and I are both political junkies, so we almost always watch the network and cable news at the end of the workday. Lately, though, I’ve been feeling like Alex in

 trying to listen to music from his favorite composer, Beethoven, after he was treated with the Ludovico technique. For those who don’t remember the book or the movie, the Ludovico technique is described in the book as a form of aversive conditioning treatment.

In Alex’s case, it was used to treat his severely violent behavior by playing Beethoven and giving a nausea-inducing drug while he was watching extremely violent movie scenes. In my case, watching the news even without any nausea-inducing drugs or Beethoven is nonetheless inducing extreme fatigue and fear of nausea.

I’m trying (unsuccessfully) to go cold turkey. Whether it’s the latest Russia-related campaign news, the sadistically misnamed American Healthcare Act, or the latest attempts to dismantle anything the government did between 2008 and 2016, I don’t even have to watch TV anymore. I have endless loops of all this going through my brain paired with the face of my state’s senior senator, Mitch McConnell. Even my wife and I trying to think up potential 

Here's how I've managed to get my own mind off the news. What about you? 

Vol 34 No 8

Musician Injuries

Frontotemporal Dementia: A Brain Disease That Challenges Definitions of Mental Illness

Psychodynamic Psychiatry: A Case Report

Anti-NMDA Receptor Encephalitis: Diagnostic Issues for Psychiatrists

What a Long Strange Trip It’s Been

The Healing Power of Photographs

Improving Mental Health: Four Secrets in Plain Sight

Treatment for Cannabis Use Disorders: A Case Report

E-Cigarettes, Vaping, and Other Electronic Nicotine Products: Harm Reduction Pathways or New Avenues for Addiction?

Understanding and Addressing Sleep Disruptions in Alcohol Use Disorders

ADHD and Substance Use: Current Evidence and Treatment Considerations