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La Belle Indifference
Introduction
The term “la belle indifference” is a French term that translates to “beautiful ignorance.”[1] La belle indifference is defined as a paradoxical absence of psychological distress despite a serious medical illness or symptoms of a health condition.[2] This condition is most commonly associated with conversion disorder. According to the Diagnostic and Statistical Manual of Mental Disorders V (DSM 5), conversion disorder is also referred to as functional neurologic symptom disorder (FNSD). FNSD, or conversion disorder, is characterized by at least 1 neurological deficit with no medical or neurological etiology and is incompatible with any known medical or neurological disorders. Psychological distress or conflict may be manifested as a physical symptom in patients with conversion disorder. La belle indifference is not formally included in DSM 5 as a diagnosis. However, it is used in conjunction with conversion disorder.[3]
According to the DSM 5, the mere presence of la belle indifference does not confirm the diagnosis of conversion disorder. However, la belle indifference is most commonly seen in patients with conversion disorder, so this topic focuses primarily on its association with conversion disorder. For a full review of conversion disorder, please refer to the conversion disorder topic by StatPearls.[4]
In the DSM 5, conversion disorder is classified under somatic symptom and related disorders (SSD). Other diagnoses included under SSD include somatic symptom disorder, psychological factors affecting other medical conditions, illness anxiety disorder, and factitious disorder. The most common presentation of FNSD, or conversion disorder, includes weakness or paralysis of 1 side of the body or bilaterally, abnormal movements (including tremors, myoclonus, dystonic movements, etc.), seizures (psychogenic neuroleptic seizures), swallowing problems (globus), speech problems (dysphonia or aphonia), sensory loss (vision or olfactory issues) and syncopal episodes.
Etiology
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Etiology
The etiology of conversion disorder can be multifactorial. A vast majority of patients who present with FNSD have an identifiable stressor. However, the absence of a stressor does not exclude the presence of FNSD. It may be noted that a causal relationship between psychological distress and the occurrence of conversion is difficult to establish.
According to recent functional magnetic resonance imaging (fMRI) studies exploring a brain-based cognitive model for conversion disorder, there appears to be a disconnect between the neuronal networks of the anterior cingulate and prefrontal cortex, which points to the psychodynamic dissociation hypothesis.[2][5] These studies also suggest an association between depression, post-traumatic stress disorder (PTSD), and conversion disorder.[2] Personality disorders, mood, and anxiety disorders are the most common comorbidities seen in patients with conversion disorder.[6] People with maladaptive behaviors and poor coping strategies are at an increased risk of conversion disorder.
Psychosocial stressors, including neglect and physical or sexual abuse, are also implicated in the manifestation of conversion disorder. For example, dissociative phenomena and motor symptoms that are common in patients with PTSD are also seen in conversion disorder.[7][8][9]
Epidemiology
The incidence of conversion disorder in general hospital patients is around 5%.[10] The estimated prevalence of psychogenic non-epileptic seizures (PNES) is 33 per 100,000.[11] In another study that followed over 0.35 million individuals for 3 years, the incidence rate of PNES was 4.9 per 100,000.[12] In a study of the general population in New York, the incidence of conversion disorder was 11-22 per 100,000.[13]
Conversion disorder is more commonly seen in females than in males.[14][15] It is also commonly seen in individuals with low socioeconomic status, lower education, and rural population.[13][16] The onset of FNSD is generally in late adolescence and early adulthood. Forty-seven percent of individuals have comorbid anxiety or depression.[12] In patients with conversion disorder, over two-thirds of patients have a history of depression or trauma.[17][18]
History and Physical
A thorough physical and neurological exam should be conducted to exclude serious medical and neurological disorders. Neurological disorders that need to be ruled out include multiple sclerosis, stroke, Guillain-Barré syndrome, myopathies, polymyositis, and myasthenia gravis. Depending on the presentation, a focused neurological exam should be performed. Below are some tests for motor symptoms with high sensitivity and specificity for conversion disorder. A full range of neurological exams for motor, sensory, and gait are summarized by Oneal and Baslet.[19]
Hoovers sign (63% Sensitivity & 100% specificity): This test is commonly used to separate organic from nonorganic causes of weakness or paralysis. An examiner's hand is placed below the heel of the affected leg, and the patient is asked to flex the hip of the normal leg against resistance. In organic disorders, there should not be any pressure on the examiner's hand on the affected side, while pressure is felt in patients with FNSD.[20]
Variable strength (63% sensitivity and 97% specificity): The weakness is inconsistent with variable force at different locations.
Inconsistencies in the exam (13% sensitivity and 98% specificity): Individuals have an inconsistent presentation of signs and symptoms when performing voluntary activity and when they are being examined.
Co-contraction (17% sensitivity and 100% specificity): When asked to flex the elbow, the triceps and biceps are contracted.[21]
For tremors, distraction affecting the tremor (92% sensitivity and 94% specificity), tremor variability (22% sensitivity and 92% specificity), and tremor entertainment ( (91% sensitivity and 92% specificity) are commonly used.[22]
Evaluation
EEG and video-EEG should be ordered to rule out epileptic seizures. MRI is essential to rule out neurological disorders like multiple sclerosis or stroke. Labwork should be done to rule out Guillain-Barré syndrome, myopathies, polymyositis, and myasthenia gravis. Some patients with PNES may also have epileptogenic seizures.[23] The approximate incidence of epileptic seizures in patients with PNES is 10%.[24] In some cultures, seizure-like episodes are common during cultural rituals. Therefore, providers must carefully explore the religious and cultural association with symptoms in these individuals.
Treatment / Management
Treatment of FNSD poses a significant challenge due to the lack of empirical studies and RCTs.[16] A comprehensive treatment approach involving multiple specialties - primary care providers (PCPs), psychiatrists, neurologists, and psychologists is usually needed. The 3 P's approach -identifying predisposing factors, precipitating stressors, and perpetuating factors is suggested in the literature.[25](B3)
Cognitive-behavioral therapy (CBT) is identified as an effective treatment for SSD.[26][27] A pilot study of 16 PNES patients treated with CBT showed a significant reduction of symptoms at a 6-month follow-up.[28] In a study looking at the effectiveness of pharmacology, 7 out of 10 patients with conversion disorder showed improvement in motor symptoms with antidepressants.[29] Though several treatment strategies are suggested, there is no effective evidence-based treatment for FNSD.[27] Providers should focus on the effective treatment of comorbidities and the management of stressors and psychological trauma.(A1)
Differential Diagnosis
FNSD can be present in patients with physical illness. Patients with epileptogenic seizures may also have concurrent PNES.[23] Patients with significant and life-threatening physical illnesses may also present with dissociative symptoms and conversion. However, patients with la belle indifference do not appear distressed by their symptoms. FNSD is frequently seen in patients with multiple psychiatric comorbidities like depression, anxiety, PTSD, and personality disorders. Multiple sclerosis, stroke, Guillain-Barré syndrome, myopathies, polymyositis, and myasthenia gravis should be considered in the differential.
Prognosis
The prognosis of FNSD depends on the onset and duration of the symptoms. Acute onset and short duration with an identifiable stressor generally have a good prognosis. For a quarter of patients, the symptoms of conversion resolve within weeks.[16] In a longitudinal study of patients with psychogenic movement disorders, patients with psychiatric comorbidities have prolonged duration of symptoms.[30]
Complications
According to a systematic review by Stone et al., there is only a 4% chance of misdiagnosis of FNSD.[31] However, there remains a risk that a true neurological or medical etiology may be missed, and treatment is delayed in these cases. Hence an immediate referral by PCP for comprehensive screening and assessment by psychiatry and neurology is essential to arrive at an accurate diagnosis in a reasonable amount of time.
Consultations
Primary teams should consult neurology, psychology, and psychiatry for appropriate diagnosis, referral, and treatment of la belle indifference and FNSD.
Deterrence and Patient Education
A multidisciplinary treatment approach is needed to diagnose and treat FNSD. Engaging patients is critical for positive treatment outcomes. The first step towards patient engagement is the patient’s acceptance of their diagnosis. Educating the patient on their physical symptoms and how the diagnosis of FNSD was made should be explained so that the patient understands.[32]
Enhancing Healthcare Team Outcomes
A comprehensive screening and assessment by a multidisciplinary treatment team, including primary care providers, neurology, and psychiatry, are needed to diagnose and treat FNSD. Active collaboration and agreement regarding the diagnosis across specialties involved in patient care are required to prevent unnecessary consultations, testing, multiple hospital visits, and health care utilization. Patients should be educated on their presenting symptoms, and efforts should be made to engage the patient in treatment. Treatment outcomes are generally worse if the patient does not have buy-in in their diagnosis of FNSD or does not engage in treatment.[33]
References
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