“When Will All of This End?”: A 65-Year-Old Man With Amyotrophic Lateral Sclerosis and Psychiatric Distress

See commentary by Ho and Berry

Amyotrophic lateral sclerosis (ALS) is a rare, progressive, and incurable neurodegenerative disease affecting both upper motor neurons in the brain and lower motor neurons in the bulbar and spinal segments. Prevalence is higher in Western countries, with men affected more than women. Risk increases with age and reaches a peak between 60 and 79 years (age-adjusted prevalence: 6.6:100,000).¹ Approximately 15% of ALS cases are familial and linked to about 40 known genes (most commonly C9orf72, TARDBP, SOD1, and FUS), while the rest are sporadic mutations.

Although ALS is characterized by phenotypic heterogeneity, the most common forms comprise a bulbar or spinal onset, each accounting for about 30% of the cases. All phenotypic variants are characterized by progressive weakness of voluntary skeletal muscles, beginning in the limbs and extending to the trunk and later involving swallowing, speaking, and breathing, in the case of the spinal variant, or vice versa in the case of the bulbar variant, eventually leading to significant functional decline and respiratory failure.² Sphincter and extraocular muscles are typically spared, which allows for some individuals to communicate with computer assisted eye tracking devices in advanced phases of the disease. The prognosis for a patient diagnosed with ALS is on average 3–5 years.

Although the more traditional view of ALS has been that of a predominantly motor neurodegenerative disorder, clinicians and researchers recognize that cognitive and behavioral disturbances affect between 30% and 50% of ALS patients. Specifically, difficulty with executive function, apathy, irritability, changes in eating habits, and disregard for hygiene are commonly seen even in the absence of a diagnosis of frontotemporal dementia, which shares a common genetic background with ALS and can affect 15% of ALS patients. Moreover, depression, anxiety, sleep disturbances, and pseudobulbar affect causing emotional lability are commonly seen at different stages of ALS and are now considered part of the condition.

Given the complex clinical manifestations of ALS, treatment of individuals with this disease should involve a multidisciplinary team. Interventions are typically focused on slowing the disease process with riluzole and edaravone and on supporting adequate nutrition and respiratory care. Psychotropic medications are often used to treat depression and anxiety. There are no guidelines on the treatment of emotional and cognitive symptoms of ALS, and the choice of psychotropic drugs is based on individual factors including psychiatric history, comorbid conditions, and concomitant medications, along with functional status. Additionally, no psychosocial interventions for ALS-specific adjustment have been validated with well-designed and controlled studies.

The following case illustrates the psychiatric challenges patients with ALS face. The patient, “Michael,” received both psychotherapy and psychopharmacology. We first introduce the patient and discuss his background, clinical presentation, and course of psychotherapy. Next, we discuss the treatments for Michael’s neuropsychiatric symptoms and medication management strategies.

Neurological and Psychopharmacological Perspective

Dr Alice Flaherty

When treating depressed patients with neurodegenerative illness, it’s natural to wonder whether the depression is reactive to the hardships of disease or an organic effect of neuronal death. In ALS, the answer is surprising: people with ALS are less likely to be depressed than those with other neurodegenerative diseases such as Parkinson disease and Alzheimer disease. Moreover, depression prevalence decreases with ALS progression.³ Why?

Although ALS is primarily a disease of motor neurons, about 10% of ALS patients have temporal lobe cell death that causes frontotemporal dementia (FTD). FTD can protect against depression, at least in late stages, because it causes prominent apathy and lack of empathy. However, most patients with FTD are not apathetic. Could they have other protective personality traits? A study titled “Are People With ALS Particularly Nice?” showed that ALS patients are more agreeable and less neurotic than controls.⁴

We should consider whether ALS patients are less depressed because other people are nicer to them. ALS’s status as a rare tragedy rather than a chronic disease protects patients from caregiver compassion fatigue, as does the fact that ALS patients are not prone to the psychosis and impulsivity of Alzheimer disease and late Parkinson disease. A cold numerical estimate of public sympathy might be the ratio of National Institutes of Health (NIH) research dollars to patient prevalence. In 2017, NIH research dollars per patient were $0.49 for dementia, $305 for Parkinson disease, and $2,053 for ALS.⁵

Whether it is ALS neurology or sociology that on average protects against depression, neither was enough to protect the subject of this case presentation from his fears and family stress. In any case, etiology’s prime importance is to guide treatment, and both “reactive” and “organic” depression can benefit from both psychotherapy and antidepressants. ALS physical symptoms guide treatment more than cause does. The risk of respiratory suppression made benzodiazepines dangerous for this patient. Neuroleptics do not suppress respiration, but long-acting ones caused too much daytime sedation. The shorter half-life of quetiapine or risperidone might have been useful. Homebound ALS patients rarely see full daylight, so phototherapy can help both circadian dysregulation and mood. Slow letter-by-letter communication shrank the content of psychotherapy visits; emails gave him a freer voice. Treatment guidelines developed for groups work best when they are not boundaries, but heuristics to guide what approach to try first with each individual.

Existential and Palliative Care Perspective

Dr Yvan Beaussant

In the context of serious illness, an existential and palliative orientation can significantly impact patient care. The existential dimension of an individual’s experience encompasses the concerns that arise when confronting the boundaries of life, leading to a renegotiation of the relationship with self, others, life, and death.⁶ Michael, the patient in the presented case, embodies numerous existential themes such as meaning, purpose, values, self-identity, hope, time, and connection.⁷ His experience with an incurable and progressive illness profoundly affects all aspects of his being, including his physicality, relationships, and existential reflections on the value and duration of his life.

Understanding and acknowledging the existential dimension of illness provides an alternative perspective to patients’ distress, going beyond potentially pathologizing constructs such as depression, suicidality, and anxiety. By recognizing existential experiences as a process, health care professionals can foster growth, healing, and self-awareness.^8,9 Interestingly, data on psychedelic-assisted therapy to improve psycho-existential distress are promising, suggesting ways Michael’s experience with psilocybin mushrooms might have been further integrated into his existential therapy.¹⁰ Lastly, integrating the existential dimension highlights the importance of interdisciplinary care in addressing questions of meaning, purpose, dignity, and spirituality.

Turning to palliative care, it is essential to consider the role it played in this patient’s case. The guidelines for ALS patient care recommend incorporating a palliative approach from the time of diagnosis.¹¹ This approach focuses on maximizing quality of life through relieving symptoms; providing emotional, psychological, and spiritual support; removing obstacles to a peaceful death; and supporting the family in bereavement. Although the case emphasizes optimal symptom control and psycho-existential support, it raises questions about end-of-life (EOL) discussions and the goals of care.

Understanding the extent of EOL conversations, advance care planning, and the management of impending life-threatening complications according to Michael’s preferences is crucial. As his functional capacity declines, decisions regarding life-sustaining measures such as non-invasive ventilation and G-tube feeding become increasingly complex. Anticipating the decline of his ability to communicate and entertain himself via assistive technology raises questions about the ethical limits of sustaining his life and the potential use of interventions like terminal sedation or medical assistance in dying.¹² The importance of preserving his sense of control for Michael suggests that engaging in these discussions and establishing a plan aligning with his definition of a reasonable life might have been empowering for him.

Psychodynamic Perspective

Dr Margaret Cramer

Physical loss. Freud states that, “The ego is first and foremost a bodily ego.”¹³ With his existential and phenomenological focus, Binswanger considered that we don’t just have a body; we are a body.¹⁴ Physicality is integral to identity, so understanding Michael’s previous relationship to his body could be useful.

Personality loss. Catastrophic illness can be a catalyst for either growth or regression. This patient’s self-absorption and need to control others may be an artifact of illness, psychological adaptation, or exacerbation of character style. A family meeting might elucidate his premorbid relational functioning.

Past loss in the present. Michael’s concerns are both contemporary worries and proxies for grievances of a painful past. His complaint that his children are not interested in him refers to his own childhood. His early life suggested enduring experiences of “unfairness” and emotional abandonment, proving that he was unworthy of attention on the basis of an affectional bond alone. He used guilt induction, financial control, and passive aggression to keep people close. Caregivers had to be paid or manipulated. His increasing disability and the prospect of dying likely reawakened yearnings for maternal care and love, emerging in the love/infatuation for a caregiver. Although the target of his feelings might not have been appropriate, the tender affects associated with it were entirely relevant to treatment.

Ambivalence. When Michael says, “It is too much to handle” and “When will all of this end?” he expresses his desire for/fear of death. Exploring this might have created an experience of being deeply understood by a caring other. Candid discussions about the desire for death can help patients feel less alone, make life-as-it-is feel more possible, and actually undermine the desire to die. For providers, becoming better acquainted with defenses against mortality becomes a treatment imperative.

Termination. When death is imminent, the concept of “short-term treatment” requires redefinition. Holding the certainty of the patient’s death within the treatment relationship could lead to greater frankness about provider availability. Michael’s initial goal for treatment was to find “a space to talk about what I’m going through.” Of all the things he meant by this, facing the end of his life was at the heart of the matter.

Dialectical Behavior Therapy Perspective

Dr Rebecca Harley

One of the “calling cards” of DBT is its versatile skills toolbox, teaching mindfulness, distress tolerance, emotion regulation, and interpersonal effectiveness (IE) skills in language that patients and treaters tend to like. We hear Dr Lester use DBT skills throughout this case—the IE work, for instance, when Dr Lester and Michael clarify the patient’s interpersonal goals and then craft more effective communications to his children, leading to a much-needed sense of rebuilding in those relationships.

Underpinning the skills toolbox, DBT is rooted in dialectical philosophy, a basic tenet of which is that two (or more) opposing things can be true simultaneously. This is a problem for us humans, however, because oftentimes we struggle to reconcile opposing thoughts, feelings, needs, or points of view. Rather than siding with one side and minimizing the opposing side, or vacillating between the two polarities, the challenge of dialectical awareness is to hold the truth of both sides. Applied to a therapy context, we as clinicians must hold contradiction and tolerate how that feels. We invite our patients to hold contradiction with us, along with the accompanying feelings we both have. It can feel deeply uncomfortable and, when it works, also deeply relieving.

Michael’s case is full of dialectics—many true, often painful things for which there is no one answer: his intense present-moment concerns about physical symptoms and caregiver and family relationships on the one hand, and looming awareness of his mortality on the other. The wish to live and the wish to hurry death along. Hope and hopelessness. I don’t see these polarities as problematic, although of course they pain us deeply. It makes sense to hope for that which is possible—in Michael’s case, things like connecting to the in-the-moment pleasure of watching Netflix or feeling supported by his care providers as they patiently wait for him to type his thoughts with his eyes. Equally valid is the co-occurring experience of hopelessness and its companion, sorrow, in response to that which is not possible: Michael will not survive ALS. While he lives, he will never use his voice to speak again, and he literally cannot lift a finger on his own behalf.

DBT points to validation as a core therapeutic tool for difficult, dialectical situations. When we offer our patients validation, we communicate that we are listening, that we see them, and that their reactions are understandable. We say, “Yes and Yes. It makes sense that you would feel this and also that you would feel that. I am interested in taking the journey with you as we work together to hold awareness of all of it.” I suspect that, to this patient, validation of his concerns, both large and small, felt precious. Isolated and alone inside an unmoving body, the experience of being seen and understood was almost certainly a lifeline.

Integrative Perspective

Dr Jonah N. Cohen

Drs Lester and Vitolo present the case of Michael, a man with ALS who struggled to adjust to the disease. This case reminds us of the varied considerations when working with someone at the end of their life, including the importance of developmental history, psychological and existential distress, medical complications, and grief. Moreover, this case emphasizes the essential role of a multidisciplinary approach over and above any particular theoretical modality.

Dr Flaherty allows us to zoom out of an intrapsychic lens and consider more societal, cultural, and psychopharmacological factors. ALS patients are treated differently and, on average, have lower levels of depression than their counterparts. Perhaps there is something about being locked-in our own bodies that forges stronger feelings of empathy in others, including therapists, psychiatrists, and caregivers. Moreover, despite the acknowledgment of social factors, Dr Flaherty also emphasizes the real suffering inside this patient’s symptoms and the need for optimized pharmacologic management.

Dr Beaussant discusses the case from an existential and palliative care perspective. Dr Beaussant reminds us how Michael’s case needs to be thought about beyond symptoms and to consider meaning, purpose, and identity. Of course, this is hard to do when our patients are exhibiting acute symptoms, but perhaps even more important in these moments so as to not over-pathologize. Finally, Dr Beaussant emphasizes the importance of a multidisciplinary approach in involving palliative care.

Dr Cramer discusses the role of dialectics by another name: conflict. In psychodynamic theory, unconscious conflict leads to compromises that can cause suffering. Dr Cramer discusses how this patient’s experience of unfairness and emotional abandonment led him to use guilt and passive aggression. Psychodynamic therapy might have helped Michael understand that these strategies were not actually likely to engender the love he wanted. We needed to help him think of other ways to find the love he desired, which might have meant working toward better tolerating his vulnerability. Furthermore, Dr Cramer discusses ways—largely inside the therapeutic relationship—that might have helped Michael feel less alone and more understood by another, things he lacked developmentally.

Finally, Dr Harley discusses the idea of dialectics—that two things that are opposite can be true simultaneously. In life, suffering often occurs in the denial of simultaneous and opposite truths—that we can love and hate the same person, that we can think we do not need help but yearn for it at the same time, and that we can both want to die and want to live. Dr Harley offers us a frame that not only acknowledges these contradictions but also reminds us that inside contradiction is something not just permissible but beautifully human. Michael faced some of these contradictions and struggled to try to reconcile them. He wanted to live but also wanted to know when his suffering would end; he was aggressive and sometimes controlling of others but longed for more connections with his family, friends, and caregivers.

This case illustrates the importance of working together in a multidisciplinary effort to provide the highest quality care (medical, psychological, social) to our patients most in need. Conversations such as these are essential to this work.

Article Information

Published Online: December 13, 2023. https://doi.org/10.4088/JCP.23ct15038
© 2023 Physicians Postgraduate Press, Inc.
Submitted: July 25, 2023; accepted October 5, 2023.
To Cite: Lester EG, Vitolo OV, Flaherty A, et al. “When will all this end?”: a 65-year-old man with amyotrophic lateral sclerosis and psychiatric distress. J Clin Psychiatry. 2024;85(1):23ct15038
Author Affiliations: Harvard Medical School, Boston, Massachusetts (Lester, Vitolo, Flaherty, Beaussant, Cramer, Harley, Cohen); Department of Psychiatry, Massachusetts General Hospital, Boston (Lester, Vitolo, Flaherty, Cramer); Department of Neurology, Massachusetts General Hospital, Boston (Flaherty); Department of Psychosocial Oncology and Palliative Care, Dana-Farber Cancer Institute, Boston, Massachusetts (Beaussant); Dialectical Behavior Therapy Program, Massachusetts General Hospital, Boston (Harley); Center for Anxiety and Traumatic Stress Disorders, Massachusetts General Hospital, Boston (Cohen).
Corresponding Author: Ethan G. Lester, PhD, One Bowdoin, Ste 100, Boston, MA 02114 ([email protected]).
Relevant Financial Relationships: Dr Vitolo reports employment by Cerevance Ltd. No other authors report conflicts of interest.
Funding/support: Cross Talk is financially supported by the Endowment for the Advancement of Psychotherapy at Massachusetts General Hospital.
Role of the sponsor: The funding agency had no role in the preparation, review, or approval of the manuscript or the decision to submit the manuscript for publication.
Statement of De-identification: Patient information has been deidentified and the patient’s name has been changed to protect confidentiality.

Clinical Points

• Patients with progressive, terminal neurodegenerative diseases like amyotrophic lateral sclerosis (ALS) are at increased risk for emotional distress secondary to their illness. Many interventions for these patients are primarily biomedical, and comparatively less attention has been paid to interventions addressing co-occurring psychological and psychiatric impacts of the illness.
• In addition to the physical toll ALS takes on patients and families, complex psychosocial factors (eg, family dynamics, occupational stress) can pose additional burdens for patients and produce more rapid and devastating health declines for patients with ALS.
• By taking an integrated, multidisciplinary approach to ALS care, and by prioritizing mental health, we can begin to address the additional challenges that patients with ALS face as part of their illness experience.