Repeated Suicide Attempts in a Patient With Stiff Person Syndrome

Stiff person syndrome (SPS) is a rare neurological disorder with large functional impacts (1). The physical manifestations of SPS include rigidity, stiffness, unpredictable spasms, frequent falls, and pain (1). Psychiatric comorbidities often include anxiety, depression, and phobias (1). A study conducted in the United Kingdom showed that SPS affected 1–2 people per million over a 5-year span. Among patients with SPS, 60%–80% have the presence of antibodies against glutamic acid decarboxylase (1). The exact role of autoantibodies in SPS pathogenesis is still under investigation, although all these antibodies target inhibitory synaptic transmission through gamma-aminobutyric acid, an inhibitory neurotransmitter, in the brain and spinal cord (1).

It is important for clinicians to recognize the signs and symptoms of SPS because there have been past occurrences of misdiagnosis. Case reports from the literature and a systematic review of articles published before May 2020 show how initial signs of SPS have often been misdiagnosed as primary psychiatric disorders, leading to potential delay in care (2, 3). In one case, although the patient had progressive stiffness and spasms for 11 years, he was treated for phobic symptoms before neurological evaluation (3). Early detection of psychiatric comorbidity in the context of SPS could provide great benefit for patients.

A systematic review revealed that the rate of psychiatric comorbidities among patients with SPS was greater than that of the general population, with relative risk estimates of 6.09–11.25, although the study reported small sample sizes (2). The prognosis of SPS is variable, and many patients have disability despite immunological and symptomatic treatment (4).

Here, we present a case of severe depression leading to multiple suicide attempts in a patient who was diagnosed with SPS along with other medical comorbid conditions.

Case Presentation

A 46-year-old female patient with a history of depression and no previously documented suicide attempts or psychiatric hospitalizations presented to the emergency department with multiple self-inflicted stab wounds. She reported that she had attempted suicide and expressed regret that her suicidal behaviors were nonfatal. Her wounds, inflicted by a knife, were 2-cm lacerations of the right neck, right chest, right abdomen, and right groin; 1-cm lacerations of the left chest and upper-left thigh; and a 6-cm laceration of the left abdomen. No signs of damage to large blood vessels, airways, or other vital organs were seen on computed tomography. Her serum ethyl alcohol level was negative.

The patient had a history of SPS, first diagnosed at the age of 38. At age 44, she suddenly developed blindness; there was concern that this may have been the result of a suicide attempt by ingestion of methanol, but the etiology was never elucidated. Her medical history included adrenal insufficiency, hypothyroidism, seizure disorders, meningioma, and decreased gastric motility. Her psychiatric history included insomnia, anxiety, and depression. In the year before presentation to the emergency department, she had multiple hospitalizations for seizures, worsening symptoms of SPS, and adrenal insufficiency.

Prior to admission, the patient was taking clonazepam (2 mg) and diazepam (5 mg) three times a day, escitalopram (15 mg) and levothyroxine (112 µg) daily, levetiracetam (1,250 mg) twice a day, hydrocortisone (20 mg in the morning and 10 mg in the afternoon), intravenous immunoglobulin therapy (1,280 mL monthly), botulinum toxin injections every 4 months, and rituximab (100 mg/10 mL) injections every 6 months. The patient did not have a relevant family psychiatric history. She lived at home with her husband and two children. Of note, her previous psychiatric management was escitalopram, which she was taking at the time of admission, and outpatient therapy, although the type of therapy was not specified.

Because the patient required further care for self-inflicted lacerations, for which she required 12 sutures (including repair of a deep wound in the left abdomen), she was admitted to the trauma surgery service. She refused treatment for her lacerations early in admission but later consented.

While in the emergency department, psychiatry was consulted. The patient reported intent to end her life by stabbing herself. She shared her struggles living with multiple medical conditions and expressed frustration about her reliance on her family. Her overall health declined steadily after the diagnosis of SPS. She reported having suicidal ideation two weeks prior to this hospitalization and had attempted to end her life by swallowing a handful of benzodiazepines. She aborted the attempt by vomiting because she thought about her children. The disposition recommendation from psychiatry was admission to an inpatient psychiatric unit once the patient was medically cleared, with criteria met for involuntary hospitalization.

The psychiatry consultation-liaison service followed the patient during her admission to trauma surgery service and recommended an increase in escitalopram to 20 mg daily. During initial follow-up interviews, the patient reported improved mood and less burdensomeness but was regretful that her suicidal behaviors were nonfatal. Four days after her initial encounter, she began to deny suicidal ideations. She was eventually medically cleared but was boarding on the surgery unit awaiting bed placement in the psychiatry unit where there was a shortage of inpatient beds. As her symptoms continued to improve, with consistent denials of suicidal ideation, she no longer met criteria for involuntary inpatient psychiatric hospitalization because she was not at imminent risk of harm to herself. The psychiatry consultation-liaison service recommended intensive outpatient care because she might have had difficulty acclimating to the acute psychiatry unit as a result of comorbid blindness, SPS spasticity, and overall frailty, but voluntary admission was presented as an option. Her husband was supportive of her choice and could provide supervision if needed. She expressed interest in inpatient psychiatric services. Two weeks after the initial presentation, with the availability of a bed, she was transferred to an inpatient psychiatry unit.

On the first day of psychiatric admission, the patient endorsed a depressed mood and decreased energy but denied further symptoms of depression. Brief inpatient cognitive-behavioral therapy (CBT) was utilized to target feelings of hopelessness and burdensomeness. The patient declined medication adjustments. The following day, she expressed a desire for discharge because of overall symptom improvement, along with perceived inaccessibility of therapeutic activities due to blindness and cold sensitivity. CBT was continued, and she was receptive to this treatment, focusing on thinking patterns and behaviors in her life. Through this therapy, she gained insight into the role SPS played in worsening her depressive symptoms.

On day 3, the treatment team recommended additional inpatient care for continued intervention with CBT, but the patient requested discharge. By telephone, the husband indicated that he felt comfortable with the patient being discharged and was supportive of outpatient treatment, agreeing to assist in maintaining a safe environment. After careful consideration of her current symptoms and requests, she was discharged home. Referral was made for intensive outpatient care on discharge.

Discussion

Although SPS is a rare condition, treatment options for depression among patients with comorbid SPS are important to investigate. In this case, a short trial of CBT showed positive initial results. Research has demonstrated the positive impact of CBT for anxiety and stiffness in SPS (5, 6). However, there is limited information on the impact of CBT on comorbid depression in the context of this disorder.

Aspects of life with SPS had a large impact on the patient’s mental health. A study exploring quality of life among 24 individuals with SPS showed lower quality-of-life scores in all domains, with markedly lower scores in physical and social functioning (7). The patient in our report engaged in therapy while on the unit and expressed a sense of isolation because of her diagnosis; few others understood her experience. She shared concerns of unforeseen functional decline, the rate at which she could deteriorate, and the burden it might place on her family. During CBT sessions, the technique of reframing was used to pose alternative perspectives, such as her family’s desire to care for her rather than the thought that she was a burden. Through reframing, she began to grieve the loss of mobility she faced in her life, having once been an athlete. Mindfulness was also used to nonjudgmentally appraise cognitive distortions, such as catastrophizing, with the emphasis to choose not to listen to these thoughts. With these positive initial advancements, CBT showed promise in addressing her depressive symptoms.

Although the CBT intervention was brief as a result of the patient’s desire for discharge, there were initial signs of improvement indicating the potential of this intervention. There is a need to balance specialization and generalization of CBT to allow for incorporation of this intervention in medical settings such as consultation-liaison service. Identifying commonalities in CBT modalities, such as psychoeducation and behavioral activation, rather than focusing on unique methods of delivery in specialized settings can reduce barriers to utilizing CBT (8). With this generalization, use of CBT in the consultation-liaison setting may have proved to be beneficial for this patient early in admission. One noteworthy limitation to this study is the uncertainty of the patient’s survival following hospitalization. Early screening of psychiatric comorbidity in SPS and direction regarding treatment may have prevented progression of depression in this case. Furthering awareness of the association of these comorbid conditions may allow clinicians, patients, and families to detect early indications that psychiatric treatment should begin.

Key Points/Clinical Pearls