Paroxysmal Psychogenic Movement Disorders

Marie Vidailhet

Frédéric Bourdain

Philippe Nuss

Jean-Marc Trocello

ABSTRACT

Paroxysmal psychogenic movement disorders are rare, but may be underdiagnosed because of two major limitations: the definition of “paroxysmal” and the positive diagnosis of “psychogenic” disorder. Several subtypes are identified (in order of frequency), such as paroxysmal dystonia and dyskinesia, stereotypies, myoclonus, and tics, and are illustrated by clinical “vignettes.” The features that are the most useful clues to distinguish psychogenic from “organic” paroxysmal manifestations are discussed. Paroxysmal psychogenic movement disorders are rarely isolated (either intricate with other movement disorders or associated with other somatic symptoms over time). Nevertheless, paroxysmal psychogenic movement disorders share with the other psychogenic movement disorders common mechanisms, therapeutic strategies, and prognosis.

Psychogenic movement disorders may represent up to 20% of patients in a movement disorders clinic. Although the proportion of the different types of movement disorders varies from one tertiary referral center to another, the most frequent psychogenic movement disorders are, in descending order, tremor, dystonia, myoclonus, parkinsonism, gait disorders, and tics (1, 2, 3, 4, 5, 6). Paroxysmal movement disorders are not mentioned in most of the series. The most likely reason is that even though many psychogenic movement disorders occur paroxysmally, they are not selected out and labeled separately as paroxysmal psychogenic movement disorders. Furthermore, paroxysmal movement disorders are often categorized as pseudoseizures and are, therefore, excluded from the series of psychogenic movement disorders.

The diagnosis of paroxysmal psychogenic movement disorder (as against an organic disorder) is very difficult and suffers two major limitations: the definition of “paroxysmal,” and the positive diagnosis of “psychogenic” disorder applied to this particular category, as the field of “organic” paroxysmal disorders is still expanding with some difficulties in the classifications of the various subtypes.

DEFINITION: PAROXYSMAL

It is not easy to set the limits of the label “paroxysmal” because it refers, in some definitions, both to duration and to triggering factors (7,8). It can also be defined as abrupt onset, recurrence, or intensification of symptoms. The symptoms occur suddenly out of a background of normal motor behavior. Important characteristics such as the frequency, severity, type of movements, and duration are not included in some definitions, and features may vary from one attack to another, as well. The triggering factors can be similar to those incriminated in organic paroxysmal disorders (9, 10, 11) (e.g., kinesigenic, continuous exertion, startle, coffee, tea, alcohol, fasting, lack of sleep, fatigue, anxiety, stress, excitement, emotion), but can also include suggestion, distraction maneuvers, or emotional reactions (to the examiner, the family, or the environment).

It would be artificial to restrain the diagnosis of psychogenic paroxysmal disorders to the movements that “look like” paroxysmal dyskinesias (9, 10, 11). Paroxysmal psychogenic movement disorders do not only overlap with the clinical features of organic paroxysmal dyskinesias, but may be phenomenologically similar to tremor, myoclonus,
tics, stereotypies, and other movement disorders. In a subgroup of sporadic paroxysmal dyskinesias, mainly representing nonfamilial, nonkinesigenic dyskinesias, the prevalence rate of psychogenic origin was 61% (5).

We use the term “paroxysmal” to describe a hyperkinetic movement disorder that occurs abruptly and unpredictably out of a background of normal motor activity and that generally lasts less than 24 hours [i.e., arbitrary definition by analogy with transient ischemic attacks (TIA) and paroxysmal movement disorders related to TIA, and by analogy with secondary paroxysmal dyskinesias]. In most cases, psychogenic paroxysmal movement disorders usually last only for a few seconds or minutes, often less than 1 hour.

POSITIVE DIAGNOSIS OF PSYCHOGENIC PAROXYSMAL MOVEMENT DISORDERS

The usual diagnostic criteria for psychogenic movement disorders (2,9) are partially inoperative in the situation of paroxysmal phenomena. One cannot rely on the clues such as “abrupt onset,” “paroxysmal” or “paroxysmal worsening,” “spontaneous remission,” or “over-time variability,” as they also can be observed in organic disorders. Moreover, organic movement disorders can be temporarily and voluntarily suppressed (tics) or restrained (chorea, akathisia) or may appear with voluntary movements (dystonia).

As a consequence, the important clues are restricted to a few items: incongruity with known organic movement disorders, selective disability (excessive slowness or clumsiness in voluntary movements), multiple somatizations, associated “odd” neurologic signs (e.g., weakness, false sensory loss), litigation, and secondary gain (2,3,6). Response to placebo or suggestion is to be considered with caution (up to 20% of organic disorders improve with placebo) (4).

Despite all these caveats, the diagnosis of paroxysmal psychogenic disorder should be a positive diagnosis, based on a set of clinical “clues,” possibly supplemented by further diagnostic testing. Furthermore, a diagnostic workup for possible organic disorders should be performed. It also implies multiple observations under various conditions (patient alone, with family, with the staff, at home) at different times by experienced movement disorder specialists with a background in neuropsychiatric strategies (2,3,6).

CLINICAL PRESENTATION

Paroxysmal Dystonia and Paroxysmal Dyskinesias

Illustrative Cases

Case 1

A 17-year-old girl (right-handed) without prior medical history is the youngest of eight children. When her family moved to another town, she had to adapt to a new school that she did not like, and she was rather lonely and distressed. She abruptly presented paroxysmal “dystonic” movements of the left hand, without any triggering factor. She clenched her fist, and flexed her elbow for 10 to 20 seconds, and meanwhile she could answer questions, smile, and behave normally. At the end of the episode, she could normally open her hand, without any sensory or motor abnormality. Then she said, “it is all over,” sighed, and smiled. She was a childish, dependent girl, and she tried not to answer questions. Several episodes were triggered by suggestion. None occurred when secretly observed by the examiner and the family, but the episodes were markedly increased when the mother was present and especially when the girl had to go to school. Clinical examination and MRI were normal. The episodes completely disappeared when the girl was taken from school during the holidays and never reappeared thereafter (with a follow-up of 1 year). She was diagnosed as having psychogenic paroxysmal dystonia.

This observation illustrates several clues in favor of the psychogenic origin: an abrupt onset and end, the influence of suggestion, the fact that the episodes only occurred when somebody was around and concerned, the identification of a triggering factor and of its influence on the movement disorder (disappearance of dystonia when the triggering factor was removed), and predisposing factors (passive-dependent, immature personality). Favorable prognostic factors included young age, abrupt onset and short duration of evolution, identified triggering factor, and no underlying severe psychiatric disorder.

Case 2

A 38-year-old woman complained of the abrupt onset of twitching movements of the left upper lip, immediately after dental anesthesia 8 years earlier. She complained of transient paresthesias in the area of the movement disorder.

These movements mimicked those of a rabbit’s mouth for less than 1 minute, increased with suggestion and during physical examination, and appeared mainly in stressful situations. She sometimes complained of pain in the precise location where anesthesia had been performed a few years earlier. Local injection of lidocaine led to resolution of the movement disorder for a few hours. She was then told that a local injection would “cure” the problem. This placebo test consisted of the injection of 0.1 mL of saline solution 1 centimeter over the lip. The paroxysmal movement immediately disappeared and did not recur for 3 weeks. She came back and asked for another “therapeutic” injection.

Neurologic examination was normal. Cranial and brain MRI were normal and extensive blood tests were normal.

She had a histrionic personality, and was deeply depressed. She described difficulties in her marriage (with conjugal violence and ongoing divorce procedure), and was the mother of an 8-year-old autistic child with little help at home. She described herself as “trapped between
fate and duty.” She recognized that she would recover from her symptoms when her family (at least marital) problems would be solved. Her brother testified that the movement disorder could disappear for several weeks during vacations or when she was less anxious.

She was diagnosed with paroxysmal facial dystonia.

This observation illustrates the role of rationalization (as she makes a connection between the dental anesthesia and the onset of the movement disorder), the utility of the placebo test, the recurrence of the movement disorder in a difficult personal and familial context, and a partial insight into the phenomenon (as the patient knows that the disappearance of the symptoms depends on the improvement of her personal life).

Comments

By definition, paroxysmal dystonia and paroxysmal dyskinesias consist of intermittent dystonia, chorea, athetosis, ballism, or any combination of these hyperkinetic disorders. Initially, paroxysmal dyskinesias were classified according to the duration of the attack and the precipitant factor [paroxysmal kinesigenic choreoathetosis or PKC (12), paroxysmal dystonic choreoathetosis or PDC (13,14), paroxysmal exercise-induced dystonia or PED] (15). A revised classification (8) is mainly based on precipitating factors, phenomenology, duration of attacks, and etiology: paroxysmal kinesigenic dyskinesias (PKD) induced by sudden movement; paroxysmal, nonkinesigenic dyskinesia (PNKD); and PED induced after prolonged exercise (8).

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