Gait abnormalities in psychogenic movement disorders.

An abnormal gait is not uncommon in patients with medically unexplained neurological symptoms, including those with other psychogenic movement disorders (PMDs). Previous studies have not evaluated the gait characteristics of patients with a variety of PMDs and there are no reports comparing PMDs with and without gait disturbances. We were interested in determining how those with and without additional involvement of gait differed and how PMD patients differed from those with a pure psychogenic gait disorder (PGD) in the absence of another PMD. We investigated gait features in a large series of patients with PMD (n = 279), dividing them into two groups (Group I with a normal gait and Group II with an abnormal gait). Group I included those with PMD with a normal gait and no change in the PMD while walking (I-1), and those with a change in PMD while walking, but not affecting gait (I-2). Group II was divided into those with PMD with additional abnormal gait (II-1) and those with pure psychogenic gait disorder without other abnormal movements (II-2). Excessive slowing of movement was more common in PMD patients with an abnormal gait (Group II) compared to those without (Group I). Slowness of gait was the most common feature in patients with PMD combined with a PGD (II-1) and buckling of the knee pattern was the most common type of pure PGD (II-2), followed by astasia-abasia.     

Psychogenic movement disorders

All varieties of movement disorders may be mimicked by a psychogenic disorder, most commonly tremor, dystonia, and myoclonus. Approximately 3% of patients seen in specialty clinics have a psychogenic movement disorder (PMD). The diagnosis of a PMD depends on not just ruling out an organic movement disorder, but moreover, recognizing features from the history and examination that are inconsistent or incongruous with an organic movement disorder. Most PMDs represent a conversion disorder, sometimes as part of a somatoform disorder; less common diagnoses include a factitious disorder or malingering. Co-morbid psychiatric illness is prevalent in patients with PMD including depression, anxiety, and personality disorders. Many PMDs remain chronic, but a multidisciplinary approach centering on psychiatric intervention can be successful. A shorter duration of symptoms and a co-existent treatable psychiatric disorder portend a better prognosis, whereas compensation and pending litigation are associated with a poorer prognosis.     

Clinical characteristics of 49 patients with psychogenic movement disorders in a tertiary clinic in Turkey

Patients admitted to movement disorders outpatient unit at a university hospital between January 2002 and June 2007 were screened for psychogenic movement disorders (PMDs). Out of 1,743 patients, 49 patients (2.8%), including four children, were diagnosed to have PMDs. Women to men ratio was 34/15. The mean age and the age‐at‐onset were 41 ± 17 years and 36 ± 15 years in the adult group, and 10 ± 2 and 9 ± 2 years in children. Among the whole group, 44% had tremor, 24% dystonia, 12% pure gait disorders, 8% parkinsonism, 6% chorea‐ballism, and 4% tic disorder. PMD developed acutely in 85% of patients, and distractibility was observed in 83%. Of the patients, 81% met the criteria for clinically established PMD, whereas 16% for documented and 2% for probable PMD. Although our data was obtained from a different culture, our results showed that hospital‐based frequency and phenomenological features between our PMD group and previously reported ones are similar. © 2009 Movement Disorder Society     

Transcultural comparison of psychogenic movement disorders.

Prompted by the lack of cross-cultural comparative data, and because a better understanding in the different clinical presentations of psychogenic movement disorders (PMDs) is relevant to neurological assessment and interventions, we compared the phenomenology, anatomical distribution, and functional impairment of PMDs in the United States and Spain. Consecutive patients diagnosed with PMD by a movement disorder specialist from one US site and from eight Spanish university centers were included in the study. The two groups were similar in their movement types, anatomical distribution, and functional impairment. PMDs were more prevalent in women than in men and were most common in upper and lower extremities. Gait and speech dysfunctions were distributed similarly in both countries. We found action tremor to be the most frequent PMD in both countries.     

Rating scale for psychogenic movement disorders: scale development and clinimetric testing.

We developed and tested the clinimetric properties of a scale for psychogenic movement disorders (PMDs). PMDs are disabling but lack any generally accepted treatment strategies. To develop treatments, means of assessing disease severity must be provided. No scale to assess PMDs existed. The PMD scale developed here rates 10 phenomena (rest tremor, action tremor, dystonia, chorea, bradykinesia, myoclonus, tics, athetosis, ballism, cerebellar incoordination), 2 functions (gait, speech), and 14 body regions. To study interrater agreement, three movement disorder neurologists independently rated 88 videotapes of PMD patients. Data analysis was performed using a kappa coefficient of agreement, Kendall's coefficient of concordance, Spearman correlations, and intraclass correlation coefficients. Validity and scale responsiveness were tested as well. All phenomena and speech and gait dysfunction occurred in the patient sample. A wide range of affected body regions, severity, and incapacitation was captured. Ratings showed excellent interrater reliability for presence or absence of each phenomenon (kappa range, 0.63 to 0.86). Kendall's concordance coefficients for phenomenology, function, and total PMD scores were 0.92, 0.93, and 0.91. Spearman correlations between raters ranged from 0.86 to 0.90. The scale was responsive to changes that occurred as a result of a neuropsychiatric intervention. The PMD scale adequately captures the complex movements of PMDs and can be used to assess PMDs and test the efficacy of intervention strategies.     

Psychogenic facial movement disorders: Clinical features and associated conditions

The facial phenotype of psychogenic movement disorders has not been fully characterized. Seven tertiary‐referral movement disorders centers using a standardized data collection on a computerized database performed a retrospective chart review of psychogenic movement disorders involving the face. Patients with organic forms of facial dystonia or any medical or neurological disorder known to affect facial muscles were excluded. Sixty‐one patients fulfilled the inclusion criteria for psychogenic facial movement disorders (91.8% females; age: 37.0 ± 11.3 years). Phasic or tonic muscular spasms resembling dystonia were documented in all patients most commonly involving the lips (60.7%), followed by eyelids (50.8%), perinasal region (16.4%), and forehead (9.8%). The most common pattern consisted of tonic, sustained, lateral, and/or downward protrusion of one side of the lower lip with ipsilateral jaw deviation (84.3%). Ipsi‐ or contralateral blepharospasm and excessive platysma contraction occurred in isolation or combined with fixed lip dystonia (60.7%). Spasms were reported as painful in 24.6% of cases. Symptom onset was abrupt in most cases (80.3%), with at least 1 precipitating psychological stress or trauma identified in 57.4%. Associated body regions involved included upper limbs (29.5%), neck (16.4%), lower limbs (16.4%), and trunk (4.9%). There were fluctuations in severity and spontaneous exacerbations and remissions (60%). Prevalent comorbidities included depression (38.0%) and tension headache (26.4%). Fixed jaw and/or lip deviation is a characteristic pattern of psychogenic facial movement disorders, occurring in isolation or in combination with other psychogenic movement disorders or other psychogenic features. © 2012 Movement Disorder Society     

Physiology of psychogenic movement disorders

Psychogenic movement disorders (PMDs) are common, but their physiology is largely unknown. In most situations, the movement is involuntary, but in a minority, when the disorder is malingering or factitious, the patient is lying and the movement is voluntary. Physiologically, we cannot tell the difference between voluntary and involuntary. The Bereitschaftspotential (BP) is indicative of certain brain mechanisms for generating movement, and is seen with ordinarily voluntary movements, but by itself does not indicate that a movement is voluntary. There are good clinical neurophysiological methods available to determine whether myoclonus or tremor is a PMD. For example, psychogenic myoclonus generally has a BP, and psychogenic stimulus-sensitive myoclonus has a variable latency with times similar to normal reaction times. Psychogenic tremor will have variable frequency over time, be synchronous in the two arms, and might well be entrained with voluntary rhythmic movements. These facts suggest that PMDs share voluntary mechanisms for movement production. There are no definitive tests to differentiate psychogenic dystonia from organic dystonia, although one has been recently reported. Similar physiological abnormalities are seen in both groups. The question arises as to how a movement can be produced with voluntary mechanisms, but not be considered voluntary.     

Psychogenic movement disorders in children

Psychogenic movement disorders (PMDs) are well characterized in adults, but childhood‐onset PMDs have not been extensively studied. We reviewed the medical records of children who were diagnosed in our clinic with PMDs since 1988 and identified 54 patients with PMDs, representing 3.1% of our pediatric movement disorder population and 5.7% of all PMD cases. The mean age at symptom onset was 14.2 years (±2.11, range 7.6–17.7). Similar to published data in adults, two‐thirds of children exhibited multiple PMD phenotypes, the most common being tremor followed by dystonia and myoclonus. Most PMDs were abrupt in onset, paroxysmal and triggered by identifiable physical or psychological trauma. As in adults, childhood PMDs were more likely to affect females, but there was no female predominance in children less than 13 years old. Although prior studies suggest that medically unexplained symptoms beginning in childhood often follow a benign course, this cohort of children experienced marked disability and morbidity related to PMDs, including prolonged school absences and unnecessary surgical procedures in more than one‐fifth of patients. © 2008 Movement Disorder Society     

Immediate vs. delayed treatment of psychogenic movement disorders with short term psychodynamic psychotherapy: Randomized clinical trial

ObjectiveIn subjects with psychogenic movement disorders (PMDs), we conducted a 6 month randomized, cross-over design study to assess the effect of 3 months of psychodynamic psychotherapy followed by observation by the neurologist vs. observation by the neurologist, then 3 months of psychiatric intervention.BackgroundPMDs are often disabling but no uniformly successful treatment strategies have been identified. Short term, open label psychodynamic psychotherapy has been successful in improving PMDs but whether PMDs improve equally well with neurological observation and support has not been tested.DesignFifteen patients with PMDs were randomized to immediate vs. delayed (after 3 months) weekly psychodynamic psychotherapy for 12 weeks. During the phase without psychiatric intervention, they were monitored by the treating neurologist. Patients were assessed at baseline, 3 and 6 months. Change in their movement disorder was assessed using a clinical global impression scale change (CGI-c), depression and anxiety using the Hamilton Depression Scale (HAM-D) and Beck Anxiety Inventory (Beck-A).ResultsFourteen women and one man, age 42.3 ± 11, disease duration 63.2 ± 73 months, were randomized to immediate (7 patients) or delayed (8 patients) treatment. Over the six month study, PMDs, depression and anxiety were significantly improved but time was the determinant factor without an independent effect of treatment assignment.ConclusionIn this group of PMD patients, where patients were kept within the medical system and involved in a research program, PMDs as well as depression and anxiety improved, but without specific benefit time-linked to psychotherapy as opposed to neurological observation and support.     

Transcutaneous electrical stimulation (TENS) for psychogenic movement disorders

Psychogenic movement disorders (PMDs) often result in disability and diminished quality of life, yet medical therapies are presently limited and largely ineffective. On the basis of previous reports that transcutaneous electrical nerve stimulation (TENS) is helpful for certain patients with organic movement disorders, the authors studied the effects of TENS in 19 patients with PMDs, utilizing the Psychogenic Movement Disorder Rating Scale (PMDRS) as well as patient-rated assessments of PMD magnitude, persistence, and disability. The PMDRS Severity score significantly improved after a mean follow-up of 6.9 months, and short duration of PMD was found to be the only identifiable predictor of a favorable outcome. Although the tingling sensation produced by TENS makes it poorly suited for a controlled clinical trial, the device has a favorable side-effect profile and is an acceptable palliative treatment for a subset of PMD patients.     

Hemifacial spasm: Clinical findings and treatment

Hemifacial spasm (HFS) is a peripherally induced movement disorder characterized by involuntary, unilateral, intermittent, irregular, tonic or clonic contractions of muscles innervated by the ipsilateral facial nerve. We reviewed the clinical features and response to different treatments in 158 patients (61% women) with HFS evaluated at our Movement Disorders Clinic. The mean age at onset was 48.5 ± 14.1 years (range: 15–87) and the mean duration of symptoms was 11.4 ± 8.5 (range: 0.5–53) years. The left side was affected in 56% instances; 5 patients had bilateral HFS. The lower lid was the most common site of the initial involvement followed by cheek and perioral region. Involuntary eye closure which interfered with vision and social embarrassment were the most common complaints. HFS was associated with trigeminal neuralgia in 5.1% of the cases and 5.7% had prior history of Bell's palsy. Although vascular abnormalities, facial nerve injury, and intracranial tumor were responsible for symptoms in some patients, most patients had no apparent etiology. Botulinum toxin type A(BTX-A)injections, used in 110 patients, provided marked to moderate improvement in 95% of patients. Seven of the 25 (28%) patients who had microvascular decompression reported permanent complications and the HFS recurred in 5 (20%). Although occasionally troublesome, HFS is generally a benign disorder that can be treated effectively with either BTX-A or microvascular decompression. © 1998 John Wiley & Sons, Inc. Muscle Nerve 21: 1740–1747, 1998     

Psychogenic movement disorders

Psychogenic movement disorders (PMD) are challenging to diagnose and to treat. Since the nineteenth century, PMDs were recognized and described in painstaking detail. In the modern neurology clinic, PMDs may comprise 2-25% of the patient population. Recognition of the various types of PMDs, differentiation from organic illness and an approach to PMDs are described in this article.     

Specificity of "peering at the tip of the nose" for a diagnosis of thalamic hemorrhage

BACKGROUND: Tonic inward and downward deviation of the eyes ("peering at the tip of the nose") is regarded as a unique feature of thalamic hemorrhage, but the mechanisms of this ocular finding remain obscure. OBJECTIVES: To describe 4 patients who showed tonic inward and downward deviation of the eyes from brainstem or thalamic lesions and to discuss the possible mechanisms involved. DESIGN: Case report. SETTING: Secondary and tertiary referral hospitals. RESULTS: One patient developed alternating esotropia with downward ocular deviation from thalamic hemorrhage compressing the midbrain. Two patients showed multiple infarctions in the territory of the posterior circulation with or without the involvement of the thalamus. Another patient had lateral pontine hemorrhage extending up to the midbrain tegmentum. Ocular bobbing preceded or accompanied tonic ocular deviation in 3 patients. CONCLUSIONS: Tonic inward and downward deviation of the eyes may develop in thalamic or brainstem lesions. Irritation or destruction of the neural structures involved in the vergence and vertical gaze may cause this ocular sign in mesodiencephalic lesions. Skew deviation and esotropia from abduction deficit may be involved in some patients. Ocular bobbing and tonic downward deviation may share a common pathogenesis.     

Hemifacial spasm non-motor and motor-related symptoms and their response to botulinum toxin therapy

Hemifacial spasm (HFS) is a chronic movement disorder which presents as clonic and/or tonic facial muscle contractions frequently accompanied by many other sensory (visual or auditory disturbances, pain), motor (facial weakness, trismus, bruxism, dysarthria) and/or autonomic (lacrimation, salivation) symptoms. The aim of the study was to assess the occurrence of HFS non-motor and motor-related symptoms and their responsiveness to botulinum toxin type A (BTX-A) therapy. 56 HFS patients were included in the open-label design study. Patients were examined three times: before BTX-A injection, and 2 and 12 weeks later. The occurrence of non-motor and motor-related symptoms was assessed by a special questionnaire, and the severity of HFS was rated by the Clinical Global Impression-Severity scale (CGI-S) and depression symptoms by the Beck Depression Inventory (BDI). Over 81% of the patients before BTX-A therapy reported HFS non-motor and motor-related symptoms. Almost 50% of the patients reported more than three symptoms. The most frequent symptoms were: tearing (44.5%), eye irritation (39.3%), facial paraesthesia (26.8%) and hearing of a “clicking” sound (25.0%). 2 weeks after BTX-A injection 75% of the patients did not report any symptoms and 20% reported only one or two. 3 months later the number of symptoms had increased again, with 57% of patients reporting at least one. The number of HFS non-motor and other symptoms did not correlate with the patients’ age, disease duration and the presence of neuro-vascular conflict, but were positively correlated with the CGI-S and BDI scores. This study showed that muscle contractions in HFS patients are commonly accompanied by non-motor and other motor-related symptoms and most of them are reduced following BTX-A treatment.     

A comparative study of primary and secondary hemifacial spasm

BACKGROUND: Hemifacial spasm (HFS) is a common movement disorder. OBJECTIVE: To evaluate possible differences in the demographic and clinical features between primary and secondary HFS. DESIGN: In-person interview using a standardized questionnaire to collect demographic and clinical data. SETTING: A multicenter study that included patients with HFS attending 3 Italian academic centers.Patients Two hundred fourteen patients with HFS. MAIN OUTCOME MEASURE: A complete neurological examination assessed the current muscle distribution of spasm and the presence of synkinetic movements between upper and lower facial muscles. RESULTS: The study sample comprised 214 patients with HFS, 81 men and 133 women, having a mean +/- SD age of 65.9 +/- 12.3 years; 164 patients were classified as having primary HFS and 50 patients (48 postparalytic and 2 symptomatic cases) were classified as having secondary HFS. Patients with primary and those with secondary HFS had similar mean +/- SD ages at onset (54.9 +/- 13.5 vs 57.0 +/- 12.8 years), male-female ratios (63:101 vs 18:32), right-sided-left-sided HFS (77:86 [1 bilateral] vs 21:28 [1 bilateral]), and frequencies of familial cases (2.9% vs 2.0%), respectively. Most patients (65.0%) with primary HFS had initial symptoms of periocular muscle contractions alone and had subsequent involvement of the lower facial muscles. Most patients (72.0%) with secondary HFS reported initial involvement of the upper and lower facial muscles simultaneously. Signs of synkinesis were present in primary (43.3%) and secondary (58.0%) HFS. CONCLUSIONS: Patients with primary and those with secondary HFS share common demographic and clinical features, including sex distribution, age at onset, affected side of HFS, synkinesis, and rarity of familial cases. Signs of synkinesis were present in significant proportions of patients with primary or secondary HFS. The 2 forms differed in clinical presentation.     

Babinski's clinical differentiation of organic paralysis from hysterical paralysis: effect on US neurology

BACKGROUND: The formalized neurological examination developed near the end of the 19th century, and clinicians searched for signs to differentiate weakness due to structural lesions of the central nervous system (organic paralysis) from weakness caused by hysteria. Joseph F. F. Babinski worked in the shadow of his mentor, Jean M. Charcot, until 1893, but then developed independent studies to examine patients with both types of weakness. OBJECTIVES: To elucidate the role of Babinski in differentiating organic paralysis from hysterical paralysis and to describe his influence on 2 US neurologists, Charles Gilbert Chaddock and Charles Franklin Hoover. DESIGN: Primary and secondary sources were studied to outline the discoveries of Babinski and to determine his influence on US neurology. RESULTS: Babinski described toe extension in cases of organic paralysis and specifically stated that this sign did not occur in cases of hysterical paralysis. Chaddock and Hoover were influenced by the work of Babinski and disseminated his discoveries to US neurologists, each developing additional techniques to differentiate the 2 forms of paralysis. Each considered his technique superior to the Babinski toe sign. CONCLUSIONS: Although Babinski was only modestly appreciated by his contemporary peers of French neurology, his influence on US neurology was substantial. The Babinski, Chaddock, and Hoover signs that demonstrate whether structurally related upper motor neuron weakness exists continue to be useful maneuvers in separating these forms of paralysis from psychogenic weakness.     

The neuropsychological profile of psychotic major depression and its relation to cortisol.

BACKGROUND: Our study described the neuropsychological profile of psychotic major depression (PMD) compared to nonpsychotic major depression (NPMD) patients and psychiatrically healthy controls (HC). We predicted that higher cortisol levels would be associated with greater cognitive deficits. METHODS: Twenty-nine PMDs, 24 NPMDs, and 26 HCs were recruited at Stanford University Medical Center. Psychiatric ratings, cortisol levels from 1800-0900 hours, and neuropsychological test data were obtained. RESULTS: PMDs had more severe cognitive impairments compared with NPMDs and HCs with the exception of simple verbal attention. PMDs had elevated mean cortisol levels from 1800 to 0100 hours which were significantly correlated with poorer verbal memory and psychomotor speed performance. Cortisol slopes from 1800 to 0100 hours were also significantly correlated with verbal memory and working memory. CONCLUSIONS: While PMDs' ability to attend passively to information appears intact, they have more difficulty processing, manipulating, and encoding new information. Elevated cortisol levels, as seen in PMD patients, are associated with poorer cognitive performance especially related to verbal memory for lists of words and working memory.     

Psychogenic movement disorders in children: A report of 15 cases and a review of the literature

Data on psychogenic movement disorders (PMD) in children are scarce, with most existing literature relating to adults only. We report 15 cases with the aim of highlighting the clinical characteristics, risk factors, comorbidity, treatment, outcome, and prognosis of PMD in children. Only 13% of cases had onset before age 10, with the mean age at onset being 12.3 years. Females were predominantly affected (F:M = 4:1). The most common types of movement disorders seen were dystonia (47%), tremor (40%), and gait disorders (13%). Multiple hyperkinetic phenomenologies were observed in many cases. Abrupt onset and precipitation by minor injuries, and stressful life events were commonly reported. Clinical clues on examination suggesting a psychogenic origin were similar to those identified in adults. A distinct feature of PMD in children was the predominant involvement of the dominant limb. The underlying psychiatric diagnosis was conversion disorder in the majority of cases. Time from symptom onset until diagnosis of a PMD varied broadly (between 2 weeks and 5 years). Treatment with cognitive and behavioral therapy and rehabilitation by a multidisciplinary team led to improvement in most cases. However, treatment was much more effective in children with a short time from symptom onset to diagnosis and treatment. © 2008 Movement Disorder Society     

Hemifacial spasm associated with epidermoid tumors of the cerebellopontine angle

Hemifacial spasm (HFS) is rarely due to serious compressive lesions, such as tumors, aneurysms, or vascular malformations, located in the cerebellopontine angle. Because of the interesting association of HFS with epidermoid tumors, we reviewed the records of all patients with HFS and all patients with intracranial epidermoid tumors seen from January 1975 to December 1986. Of the 18 patients who had epidermoid tumors of the cerebellopontine angle, 3 (17%) had a facial movement disorder that resembled HFS at sometime during their illness. There were 429 patients who had HFS with no obvious serious compressive lesion of the facial nerve. Therefore, HFS was associated with epidermoid tumor in 0.7% of cases. All 3 patients developed other findings due to involvement of adjacent neural structures. Patients with HFS have a low probability of having a serious compressive lesion, but those with atypical features should be evaluated for cerebellopontine angle masses such as epidermoid tumors.     

Functional end-plate recovery in long-term botulinum toxin therapy of hemifacial spasm: a nerve conduction study

Botulinum toxin type-A is currently thought to be effective and safe for hemifacial spasm (HFS). The pre-synaptic block of acetylcholine release at the neuromuscular junction induces depression of orbicularis oculi muscle compound motor action potential (CMAP). The aim of our study was to evaluate at what extent end-plate functional recovery is possible even in botulinum toxin treatments lasting up to 15 years. We examined 81 outpatients with primary HFS (mean treatment duration = 7.2 ± 4.2 years) who underwent neurophysiologic study, once clinical effect of the previous treatment had vanished. The mean CMAP amplitude, mean rectified amplitude of response 1 (R1) of the blink reflex and area of response 2 (R2) of treated orbicularis oculi muscle were measured in comparison to the controlateral side. Mean amplitude of the above mentioned parameters was slightly lower (about 20%; p < 0.001) in the treated side at the end of the follow-up period (4.7 ± 1.7 months). The CMAP amplitude reduction weakly correlated with the interval from last treatment, while other neurophysiologic parameters did not change due to treatment duration or total toxin amount. Our study demonstrates that botulinum toxin affects compound motor action potential and blink-reflex responses for at least 4–5 months in HFS patients. The residual block is slight and does not increase with repeated injections after several years of treatment. Our study, beside confirming the long-term efficacy of botulinum toxin treatment for HFS, provides neurophysiologic evidence that therapeutic effect may be obtained without hindering the regenerative potential of the nerve-muscle complex.