Psychogenic neurologic disorders

Victor W Mark MD (Dr. Mark of the University of Alabama at Birmingham has no relevant financial relationships to disclose.)
Originally released April 18, 2001; last updated December 18, 2015; expires December 18, 2018

This article includes discussion of psychogenic neurologic disorders, functional neurologic disorder, functional movement disorder, conversion disorder, and hysteria. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Psychogenic neurologic disorders are the simulations (with varying degrees of insight) of well-known disorders of neurologic functions (ie, experiences or movements) which lack the confirmatory laboratory or imaging findings of the disorders that they model. The author reviews the more common presentations, underlying mechanisms, and treatment of psychogenic neurologic disorders. The most common forms of psychogenic disorders are attentionally-moderated and lack a sense of agency by the patient for the disorder. Chronic psychogenic conditions are associated with focal cerebral structural changes, either increases or atrophy. Therefore, effects of the disorder extend beyond behavioral. The disturbances may favorably respond to intensive psychotherapy, physical therapy, or repetitive transcranial magnetic stimulation. This article also reviews variants, including Munchausen syndrome, Munchausen syndrome by proxy, and Ganser syndrome.

Key points

 

• Psychogenic neurologic disorders are commonly encountered in general neurologic practices and, hence, knowing their manifestations and treatment is essential for clinical care.

 

• Such disturbances represent, on some level of awareness, the simulation of usually familiar neurologic disorders that have involuntary etiologies, yet their personally controllable bases are not be recognized by the patients.

 

• Despite being self-controllable, such disturbances are generally disabling unless expert professional care is provided.

 

• There is no consistent association between psychogenic disturbances and posttraumatic stress or sexual abuse.

 

• Psychogenic neurologic disturbances respond best to empathetic concern by the clinician; demonstration that the disorder lacks a structural or permanent etiology; can be improved with distraction; and praise for gradual improvement by means of the patient's guided attempts to reduce sources of distress. Cognitive behavioral therapy (CBT) is emerging as a successful intervention.

 

• Although most forms of psychogenic neurologic disorder are relatively benign, the finding of Munchausen syndrome by proxy (the false reporting of illness by caregivers of dependent individuals) demands rapid intervention.

Historical note and terminology

The term "hysteria" was originally applied to diverse female behavioral disorders that were attributed from classical times to a "wandering uterus" (Zimmer 2004). Offray de La Mettrie, for example, published in 1738 an account of episodic catalepsy (waxy immobility of the limbs) in a woman that he attributed to hysteria arising from amenorrhea (Walusinski 2012). Eighteenth century treatments for hysteria were radical and untested, including bloodletting, beatings, diet, fresh air, and writing (Meek 2013). In the 19th century the term “hysteria” came to specify psychogenic disorders following the impression that primarily women simulate medical disorders for secondary gain. In contrast, men are thought to be more susceptible to hypochondriasis, the preoccupation with diverse bodily complaints (Crimlisk and Ron 1999). In 1859, Briquet published a landmark study that used "hysteria" to describe symptoms affecting diverse bodily systems. Psychogenic disorders were subsequently emphasized by the works of Charcot, Freud, and Janet (Crommelinck 2014).

Reynolds published in 1869 a prescient overview of disorders of motor control and sensation that appeared to stem from an ideological fixation and were amenable to compassionate behavioral retraining (Reynolds 1869). Reynolds rejected terming such patients “hysteric” as the term was used at the time. In 1888 Blocq comprehensively described a case series of the acute inability to stand and walk despite full motor control of the legs while supine (which he termed “astasia-abasia,” a term that continues today) (Okun and Koehler 2007). Although he doubted that the disturbance had a purely psychologic etiology, his pathophysiologic hypothesis—that marked emotional distress can aggravate cerebral inhibition over spinal walking mechanisms—is surprisingly similar to some current pathophysiologic hypotheses for psychogenic disturbances (see below). Elsewhere, a good overview of the history of the recognition of psychogenic seizures has been provided (LaFrance and Devinsky 2004). The neurologic interest into the causation, physiological basis, and treatment of psychogenic disorders that arose from investigations in the latter part of the 19th century became overshadowed, for the most part, by the advent of psychoanalysis (Crommelinck 2014). A return to interest in the neurophysiological basis of psychogenic disorders began in the 1960s and continues to the present.

The term "psychogenic neurologic disorder" is not universally accepted. In PubMed, the earliest appearance of “psychogenic” in respect to the disorders that are reviewed here dates to a 1916 report describing 2 cases of astasia-abasia (Mack 1916). The term psychogenic is used here in a broad sense to refer to clinical impairments that resemble "conventional" neurologic disorders but are modeled by the patient (or caregiver) rather than originating from the uncontrived pathophysiologic mechanisms that underlie conventional neurologic disorders. Alternate terms for the attentionally-mediated psychogenic disturbances include "functional disorder," "nonorganic disorder," "dissociative disorder," “psychogenic-neuropsychiatric disorder” (Williams et al 2005), and for behavioral patterns that resemble epileptic seizures, "nonepileptic seizures," "psychogenic nonepileptic seizures” (abbreviated PNES), "pseudoseizures," or "psychogenic pseudoseizures." Although these alternate terms are widely used, they have met with objections (Bye and Nunan 1992; Kuyk et al 1997), and none has been universally accepted. Edwards and Bhatia emphatically recommend the term “functional neurologic disorder” (Edwards and Bhatia 2012) on the grounds that patients find this less objectionable than rival terms (Stone et al 2002b). However, the term "psychogenic neurologic disorder" will be used here to emphasize the critical role of an individual's attitudes, expectations, and self-awareness in the evolution of the clinical manifestations for the wide variety of disturbances that will be discussed, even though for many patients “psychogenic” connotes psychopathology. But when discussing the attentionally-mediated disorder with patients, clinical experts recommend the term “functional” preceding the deficit instead (eg, functional weakness), to emphasize the reversibility of the disorder (Reuber et al 2005; Stone et al 2005).

These foregoing terms generically refer to the modeled elaboration of a neurologic disorder. The following subtypes must be distinguished. "Conversion disorder" or "hysteria" refers to an attentionally-mediated psychogenic disorder without sense of self-agency. "Somatization disorder" or "Briquet syndrome" is a variant of psychogenic disorder in which nonspecific bodily complaints appear (eg, fatigue, insomnia, irritable bowel) without resembling specific neurologic disorders and without objective physiologic disturbance (Khouzam and Field 1999; Stone et al 2005). "Malingering" is the fully aware simulation of a medical disorder (frequently neurologic) for personal gain, particularly for money, material goods, or improved access to specific privileges (eg, transfer from jail). "Factitious disorder" is the willful simulation of a medical disorder without clear financial or opportunistic gain (Bauer and Boegner 1996). Instead, sufferers have a need for an enhanced feeling of control or attention. "Munchausen syndrome" is a variant of factitious disorder (often with diverse complaints), in which the patient undergoes frequent clinic or hospital evaluation, sometimes resulting in invasive, even injurious, testing or treatment. The term was coined by Asher (Asher 1951), who thought that the wide meanderings of afflicted patients from clinic to clinic and their elaborate health histories resembled the fantastic travels regaled by a fictitious character depicted by Raspe in 1785, Baron Munchausen (Pankratz 1986). (This individual was likely inspired by the real Baron Münchhausen; consequently, German spellings of this disorder also appear.) "Munchausen syndrome by proxy" refers to a caregiver's bearing false evidence of medical illness in another individual who is incompetent to represent himself (a child, in most cases described thus far) (Meadow 1977). Finally, Ganser syndrome is a controversial disorder that involves, among other features, the suggestion of simulated confabulation (Ganser 1898).

Other disorders may be "psychogenic" in that they are behaviorally induced, but their fundamental mechanism is a recognized physiological disturbance without voluntary control once it commences. Thus, certain truly epileptic seizures may be "psychogenic" in that they may be consistently induced by specific thoughts (Blanke et al 1999; Cohen et al 1999). However, in some instances, patients may intentionally induce such seizures for personal gain (Koutroumanidis et al 1998), so such individuals may share motives that underlie other psychogenic disorders referred to above. Such seizures are a form of reflex epilepsy that is triggered by internal rather than external stimuli. The Valsalva maneuver, when it is purposefully used to cause syncope, is another example of a psychogenic disturbance in this second sense. In this regard, one may also include instances of transient global amnesia that are precipitated by acute emotional distress (eg, immediately after a motor vehicle crash despite no loss of consciousness), for which short-term hippocampal dysfunction is suspected (Noel et al 2008). Some case reports have associated severe acute psychological stress with either profound diffuse cerebral hypometabolism and protracted memory loss (Markowitsch et al 1998) or complex regional pain syndrome (Grande et al 2004).

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