Opinions and clinical practices related to diagnosing and managing patients with psychogenic movement disorders: An international survey of movement disorder society members

Five hundred and nineteen members of the Movement Disorder Society completed a 22‐item questionnaire probing diagnostic and management issues in psychogenic movement disorders (PMD). When patients showed definite evidence of PMD with no other unexplained clinical features, approximately 20% said they informed patients of the diagnosis and requested no further neurological testing. The 51% who reported conducting standard neurological investigations to rule out organic causes before presenting the diagnosis to such patients had fewer years of fellowship training and fewer PMD patients seen per month. A non‐PMD diagnosis was correlated with patients' normal social or personal functioning, little or no employment disruption, lack of non‐physiologic findings, and lack of psychiatric history. Ongoing litigation was more predictive of the PMD diagnosis for US compared to non‐US respondents. Two thirds of respondents, more commonly younger and academic clinician researchers, refer PMD patients to a psychiatrist or mental health specialist while also providing personal follow up. Physician reimbursement, insurability of PMD patients, and ongoing litigation interfered with managing PMD patients to a greater extent in the US compared to non‐US countries. Acceptance of the diagnosis by the patient and identification and management of psychological stressors and concurrent psychiatric disorders were considered most important for predicting a favorable prognosis. These findings suggest that expert opinions and practices related to diagnosing and managing PMD patients differ among movement disorders neurologists. Some of the discrepancies may be accounted for by factors such as training, type of practice, volume of patients, and country of practice, but may also reflect absence of practice guidelines. © 2009 Movement Disorder Society     

Psychogenic palatal tremor

We describe a case of psychogenic palatal tremor. The diagnosis was supported by clinical criteria and neurophysiological testing, including frequency analysis and jerk‐locked back‐averaging. We discuss the differential diagnosis of palatal tremor as well as the role of neurophysiological testing in the diagnosis of psychogenic movement disorders. © 2005 Movement Disorder Society     

Unilateral lower limb rest tremor is not necessarily a presenting symptom of Parkinson's disease

Lower leg rest tremor is an uncommon symptom of neurological disease. Review of the files of 16 patients who presented with lower leg tremor (average age 58 ± 16 years; average disease duration 6.8 ± 8.5 years) yielded a diagnosis of Parkinson's disease (PD) in 5 and probable multiple system atrophy (MSA) in 3. In 4 patients with an indeterminate diagnosis, cardiac MIBG SPECT was positive in 3, indicating PD, and negative in one, suggesting MSA. Two patients each had psychogenic tremor and drug‐induced parkinsonism. Although lower leg tremor is considered an unusual presentation of PD, it should raise suspicions of MSA and other neurodegenerative disorders. © 2010 Movement Disorder Society     

Surgical therapy for multiple sclerosis tremor: a 12‐year follow‐up study

Background and purpose: Severe multiple sclerosis (MS) tremor causes disability poorly responsive to medication. Deep brain stimulation (DBS) or thalamotomy can suppress tremor, but long‐term outcomes are unclear. Methods: Nine patients with MS tremor underwent disability measures at baseline and 12 months post‐surgery (six thalamotomy, three DBS) in 1997–1998 (previously reported, Matsumoto et al., Neurology 2001;57:1876–82). We report the prospective 12‐year follow‐up of this cohort for tremor, disability, and death. Results: Surgery was initially successful in all. Tremor recurred in all patients within median 3 months, although two DBS patients were tremor‐free for 5 years. Median tremor‐free survival (tremor‐free time/survival time) was 4.3%. At 12‐year follow‐up, four survivors (two thalamotomy, two DBS) (Expanded Disability Status Scale scores 8–8.5) were severely disabled. Five patients were dead (four thalamotomy, one DBS) median 5.8 years post‐operative. Conclusions: Surgery benefit for severe tremor was overall short‐lived (median 3 months), with long‐term poor prognosis. Although two DBS patients had sustained 5‐year tremor‐suppression, the observed progressive disability and death in this cohort bear importance for long‐term success in future MS tremor surgery trials.     

Predictive value of nigrostriatal dysfunction in isolated tremor: A clinical and SPECT study

The overlap among tremor disorders is wide and complex because essential tremor patients may present resting tremor coexisting with postural tremor, while postural may coexist with resting tremor in Parkinson's disease. We investigated dopamine transporter binding in 61 subjects presenting with isolated atypical tremors defined as unilateral either postural, resting, or mixed (i.e. resting and postural) tremor, without rigidity or bradykinesia, by means of 123I‐FPCIT SPECT imaging at baseline. Patients were followed‐up clinically for 28.4 ± 7.2 months. Twenty‐five patients with baseline normal SPECT continued to present only tremor at follow‐up. Among 36 patients with abnormal SPECT, 23 (64%) developed PD, while the remaining 13 continued to present only tremor at follow‐up. The value of 123I‐FPCIT SPECT in predicting the evolution to PD was very high in a way independent from the first clinical presentation of tremor (Rest tremor, P = 0.015; Mixed tremor, P = 0.015; Postural tremor, P = 0.039; chi‐square test). Our data suggest that the clinical presentation of isolated tremors is insufficient to allow a precise early‐stage diagnosis, whereas the detection of presynaptic nigrostriatal dopaminergic dysfunction could lead to diagnosis of atypical tremor disorders at a very early stage. We suggest this disorder to be labeled as “isolated tremor with dopaminergic presynaptic dysfunction.” © 2008 Movement Disorder Society     

Incidence of Parkinson's disease and parkinsonism in northern Sweden: A population‐based study

The differential diagnosis of parkinsonian disorders is difficult, especially early in the course of the diseases. The clinical subtypes of Parkinson's disease (PD) have not so far been described in newly diagnosed patients. We present a prospective incidence cohort study of patients with idiopathic parkinsonian syndromes in the Umeå region in northern Sweden identified over a 4‐year period. The clinical diagnoses were re‐evaluated at follow‐up visits at 12 months. We found 138 patients with parkinsonism: 112 PD, 12 multiple system atrophy with predominant parkinsonism (MSA‐P), six progressive supranuclear palsy (PSP) and eight unclassifiable patients. The crude incidences for all age ranges per 100,000 were: PD 19.7 (95% confidence interval 16.1–23.3); MSA‐P 2.1 (1.1–3.7); PSP 1.1 (0.4–2.4); idiopathic parkinsonism 24.3 (20.2–28.4). Age‐standardized to the average Swedish population 2004–2007: PD 22.5 (18.3–26.7); MSA‐P 2.4 (1.2–4.2); PSP 1.2 (0.4–2.6); idiopathic parkinsonism 27.5 (22.9–32.1). The crude annual incidence rate for PD, with exclusion of patients with normal dopamine receptor uptake (FP‐CIT‐SPECT), was 18.8 per 100,000 (95% confidence interval 15.2–22.4), age‐adjusted to the average Swedish population 2004 to 2007: 21.5 (17.4–25.6). The incidence rates did not differ significantly between men and women. The cumulative incidence of PD up to 89 years of age was for men 3.4%, for women 2.6%, and for both sexes combined 2.9%. The annual incidence rates found for PD, idiopathic parkinsonism, MSA‐P and PSP are among the highest reported. © 2010 Movement Disorder Society     

Long‐term outcome of brief augmented psychodynamic interpersonal therapy for psychogenic nonepileptic seizures: Seizure control and health care utilization

Purpose: Most neurologists endorse psychotherapy as the treatment of choice for psychogenic nonepileptic seizures (PNES), but its effectiveness remains unproven, and there are no previous reports of long‐term outcome after psychotherapy. This study aimed to establish the outcome of brief augmented psychodynamic interpersonal therapy (PIT) for 47 patients with PNES in terms of seizures and health care utilization 31–65 months (median 50 months) after diagnosis. Methods: Participants completed questionnaires before starting therapy (Clinical Outcomes in Routine Evaluation Outcome Measure [CORE‐OM]; Patient Health Questionnaire [PHQ15]; Short‐Form Health Survey [SF‐36]). Forty‐seven of 66 consecutive patients (71%) also completed a follow‐up questionnaire about current seizure frequency, employment status, and health care utilization 42 months after the end of therapy (range 12–61 months). Factors associated with seizure outcome and predictors of seizure cessation were evaluated. Results: At follow‐up, 25.5% of patients had become seizure‐free; a further 40.4% achieved a seizure reduction of >50%. Logistic regression showed “economic activity” status to be the only significant baseline predictor of seizure cessation (p < 0.021). Health care utilization declined significantly from baseline to follow‐up (p < 0.039), suggesting minimum expected annual health care expenditure savings of £245 ($408). Discussion: These results indicate that this intervention is associated with a significant improvement in seizure frequency and health care utilization, suggesting that a randomized controlled study of the intervention is justified.     

Psychogenic tremors

We diagnosed 24 patients, 9 men and 15 women ranging in age from 15 to 78 years, with clinically established or documented psychogenic tremors. Clinical presentations were unique, with complex tremors (often resting, postural, and kinetic), unusual temporal profiles (abrupt onset with a variable course), absence of other neurologic signs, inconsistent and incongruous symptomatology, selective disability with ability to perform some functions despite severe tremors, distractibility that lessens or abolishes tremor, atypical tremorgraphic recordings with changing amplitude and frequency, unusual handwriting and drawing specimens, presence of multiple undiagnosed somatizations, unresponsiveness to all treatments, absence of documented disease by laboratory or radiographic tests, presence of psychiatric disease, spontaneous remissions, or recovery with psychotherapy. We present criteria for the diagnosis of psychogenic tremor.     

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     

Chronic deep brain stimulation in patients with tardive dystonia without a history of major psychosis

Tardive dystonia usually occurs with a delay after neuroleptic exposure in patients with major psychosis. A subgroup of patients, however, is given such medication for “mild depression” or “neurasthenia.” Tardive dystonia, in general, may respond favorably to pallidal deep brain stimulation (DBS). Nevertheless, it remains unclear thus far whether or not similar beneficial outcome is achieved with pallidal DBS in different subgroups of patients with tardive dystonia. Four women (mean age 59 years at surgery) underwent stereotactic pallidal DBS in the frame of an observational study. Tardive dystonia occurred secondary to medication with fluspirilene and haloperidol, and injection of long‐acting depot neuroleptics prescribed for mild depression or “nervousness.” Assessment included the Burke‐Fahn‐Marsden (BFM) scale preoperatively and at 12 months follow‐up. Extended follow‐up was available at a mean of 27.3 months postoperatively (range 16–36 months). There were no surgically related complications. All 4 patients experienced sustained statistically significant benefit from pallidal DBS. Mean improvement at 12 months was 77% for the BFM motor score (range, 45–91%; P = 0.043), and 84% at the last available follow‐up (range, 70–91%; P = 0.03). This was paralleled by improvement of the BFM disability score. Chronic pallidal DBS in patients with tardive dystonia without a history of major psychosis provides sustained improvement which is similar to that in other subgroups of patients with tardive dystonia. This effect is stable on extended follow‐up for up to 3 years. © 2010 Movement Disorder Society     

Clinicopathologic discrepancies in a population‐based incidence study of parkinsonism in olmsted county: 1991‐2010

Objective : The purpose of this study was to examine the discrepancies between the clinical diagnosis of parkinsonism and neuropathological findings in a population‐based cohort with parkinsonian disorders. Background : The specific clinical diagnosis of parkinsonism is challenging, and definite confirmation requires neuropathological evaluation. Currently, autopsies are seldom performed, and most brain autopsies represent atypical or diagnostically unresolved cases. Methods : We used a defined population‐based incidence cohort with clinical parkinsonism (n = 669) from the Rochester Epidemiology Project in Olmsted County, Minnesota, 1991‐2010. We reviewed reports of all patients who underwent neuropathologic examination at autopsy (n = 60; 9%) and applied consensus pathologic guidelines for neurodegenerative disease diagnosis. Results : Among the 60 patients examined pathologically, the median time from the last recorded clinical diagnosis to death was 7 years (range from 2 to 17 years). Clinical–pathological concordance was found in 52 cases (86.7%), whereas 8 (13.3%) had a clinical‐pathological discrepancy. Four patients with a clinical diagnosis of idiopathic Parkinson's disease had no pathological evidence of Lewy bodies or α‐synucleinopathy; of these, pathological diagnoses were Alzheimer's disease (2 cases), progressive supranuclear palsy (1 case), and vascular parkinsonism (1 case). Two patients with clinical diagnoses of "dementia with Lewy bodies" and one patient with an "unspecified parkinsonism" had a pathological diagnosis of Alzheimer's disease without concomitant α‐synuclein lesions. One patient with clinically diagnosed "progressive supranuclear palsy" had indeterminate pathological findings without α‐synuclein or Aβ‐ or tau‐immunoreactive lesions at autopsy. Conclusions : Overall, the clinical diagnoses of parkinsonian subtypes had good concordance with pathological confirmation (86.7%). However, clinical–pathological discrepancies were documented in 13.3%. © 2017 International Parkinson and Movement Disorder Society     

Historical and clinical features of psychogenic tremor: a review of 70 cases.

OBJECTIVES: To review the clinical characteristics and associated features found in patients with psychogenic tremor. METHODS: Ten-year retrospective review of charts of all patients and videotapes of fifty-one patients diagnosed by the senior author as having psychogenic tremor. RESULTS: Seventy patients fulfilled the diagnostic criteria for clinically definite psychogenic tremors. Psychogenic tremors usually started abruptly (73%), often with the maximal disability at onset (46%), and then took static (46%) or fluctuating (17%) courses. Psychogenic tremors usually started in one limb and spread rapidly to a generalized or mixed distribution. Spontaneous resolution and recurrence, easy distractibility together with entrainment and response to suggestion were characteristic features. Presence of functional symptoms and signs and refractoriness to conventional antitremor drugs were common. CONCLUSIONS: Psychogenic tremor is generally not a diagnosis of exclusion. The presence of characteristic features on history and especially clinical examination can permit an accurate diagnosis and avoid unnecessary investigations.     

Tremor dominant parkinsonism: Clinical description and LRRK2 mutation screening

Tremor dominant parkinsonism (TDP) is characterized by initial prominent resting and action tremor, mild parkinsonism, unpredictable response to medication, and a better prognosis than idiopathic Parkinson's disease (PD). We report on clinical features and longitudinal course of 26 patients suffering from TDP. Mean disease duration was 6.5 ± 3 years, 61.5% of patients had a positive family history of tremor, 73% did not need drug treatment, performance of 123I‐Ioflupane SPECT showed reduced striatal tracer uptake in 65.4% of patients, and odor identification testing was pathologic in all the patients tested (n = 22). Co‐occurrence of action and resting tremor were the most annoying and disabling symptoms in all the patients, whereas rigidity and/or bradykinesia were clinically irrelevant in most of them. We also sequenced the full coding region of the Leucine‐rich repeat kinase 2 gene (LRRK2) in all patients. We found a novel Val2390Met mutation that was not found in 864 chromosomes. Our results suggest a broader clinical heterogeneity related to LRRK2 mutations and points towards TDP as a subtype within the spectrum of PD, in which disabling tremor but otherwise mild parkinsonian signs and a better prognosis are the main characteristics. © 2007 Movement Disorder Society     

Idiopathic spinal myoclonus: A clinical and neurophysiological assessment of a movement disorder of uncertain origin

Spinal Myoclonus (SM) is characterized by brief and sudden movements caused by the activation of muscles belonging to adjacent spinal myotomes. Recent reports have indicated that “typical” clinical and electrophysiological features of SM can be mimicked voluntarily. A useful tool that can distinguish between organic and psychogenic jerks is the detection of a Bereitschaftspotential (BP). In this study, we looked for evidence of a BP in a cohort of patients with idiopathic SM. A clinical and neurophysiological assessment of 20 patients affected by idiopathic SM was performed. A video EEG‐EMG multichannel recording was performed in each patient to detect BP. An expert neurophysiologist (PB) reviewed the BP recordings and divided them into those showing a definite, possible, and no BP. A clinical assessment of the videoed movements was performed by two neurologists expert in movement disorders (KB and MJE) who indicated if the movements were compatible with organic or psychogenic myoclonus. A definite or possible BP was recorded in 15 out of 20 patients. Clinical raters agreed in their clinical opinion on 15 patients (75%). All patients where both raters agreed the movements appeared to be organic had definite or possible BP. BP are commonly seen in patients with idiopathic SM. There is discordance between clinicians in their clinical rating of SM as organic or psychogenic, but even in those patients where movements appear clinically to be organic, a BP is commonly detected, indicating that the aetiology is psychogenic. This suggests that BP recordings are a useful adjunct to clinical assessment in the accurate diagnosis of patients with idiopathic SM. © 2009 Movement Disorder Society     

Identification of psychogenic, dystonic, and other organic tremors by a coherence entrainment test.

The differentiation of psychogenic from organic tremors, particularly those of a dystonic nature, can be difficult on clinical grounds. Entrainment of tremulous movements of different body parts into a single rhythm has been used clinically as a means of distinguishing these tremor forms, based on the inability of a patient with hysterical tremor to generate voluntary tapping oscillations independent of their ongoing tremor oscillation. The coherence entrainment test is a quantified electrophysiological entrainment test performed on accelerometer or surface EMG tremor signals. This test was carried out on 25 patients referred with suspected psychogenic tremor or dystonic tremor and on 10 normal subjects attempting to tap two independent voluntary oscillations. Using established and new clinical diagnostic criteria, patients were assigned the following final clinical diagnoses: 6 cases of clinically definite dystonic tremor, 5 cases of probable dystonic tremor, 2 cases of classic essential tremor, 5 cases of clinically definite psychogenic tremor, 3 cases of probable psychogenic tremor and 4 uncertain cases. On comparing these clinical diagnoses with those reached by a coherence entrainment test subsequently carried out on each patient, there was 100% concordance in both clinically definite and clinically probable patients. In uncertain cases, when later clinical information came to light, this also corroborated with the coherence entrainment diagnosis. No normal subjects were able to "mimic" organic tremor. The coherence entrainment test appears to be a sensitive and specific means of distinguishing psychogenic tremor from dystonic and other organic tremors.     

Wine drinking and essential tremor: A possible protective role

The purpose of this study was to evaluate the possible association of cigarette smoking, coffee drinking, and wine consumption with essential tremor using a matched case–control design. Cases and controls were enrolled from 6 Movement Disorder centers in central‐southern Italy. Essential tremor was diagnosed according to Bain's criteria. Three unrelated healthy controls (not affected by neurological disorders) per each enrolled case, matched by sex and age (±5 years), were selected. A standardized questionnaire was administered to record demographic, epidemiological, and clinical data. All cases and controls underwent a standard neurological examination. Adjusted odds ratios and 95% confidence intervals were estimated using conditional logistic regression for the matched cases and controls. Eighty‐three patients with essential tremor (38 men and 45 women; mean age, 68.2 ± 8.6 years) and 245 matched control subjects (113 men and 132 women; mean age, 68.4 ± 9.7 years) were enrolled in the study. Multivariate analysis showed a significant negative association between essential tremor and wine consumption preceding the onset of disease (adjusted odds ratio, 0.23; 95% confidence interval, 0.08–0.64; P = .0005) with a significant dose effect (1–2 glass of wine per day: odds ratio, 0.32; 95% confidence interval, 0.10–0.95; P = .04; more than 3 glass of wine per day: odds ratio, 0.14; 95% confidence interval, 0.03–0.62; P = .01). In our sample no association between essential tremor and cigarette smoking or coffee drinking was found. Our data suggest a negative association between wine drinking and essential tremor, which could be explained by the long‐term neuroprotective effect of its antioxidant components. © 2011 Movement Disorder Society     

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     

Onset of intractability and its course over time: the Dutch study of epilepsy in childhood

Purpose: Intractability in epilepsy is difficult to define, and little is known about its onset, course, and duration. We investigated these aspects (as well as the occurrence of intractability) during long‐term follow‐up in patients with epilepsy, focusing on possible explanations for the variation in time of onset and duration of intractability. Methods: After diagnosis, 453 patients with childhood‐onset epilepsy had a 5‐year follow‐up with regular visits and data collection. Ten years later they received a questionnaire with items concerning epilepsy, which was completed by 413 patients resulting in a mean follow‐up of 15 years. Intractability during the first 5 years was compared with that in the last year of follow‐up. Intractability was defined as having no 3‐month remission during a 1‐year period despite adequate medical treatment. Key Findings: At least 12.1% of the cohort had a period of intractability during the 15‐year follow‐up, and 8.5% were intractable in the final year. Of the patients with idiopathic etiology 4.3% had a period of intractability versus 17.0% for those with cryptogenic, and 22.6% for those with remote symptomatic etiology (p < 0.001). Other risk factors at baseline were younger age at first seizure, generalized cryptogenic/symptomatic or localization‐related symptomatic epilepsy, mental retardation, and febrile convulsions before enrollment. The cumulative risk of a period of intractability was 6.1% (95% confidence interval [CI] 3.7–8.5) at 2 years follow‐up and 8.2% (95% CI 5.4–11.0) at 5 years. The mean time to onset of intractability during the first 5 years of follow‐up was 1.6 (95% CI 1.3–2.0; median 1.0) years and the mean duration of intractability during these 5 years was 3.3 (95% CI 2.8–3.8; median 3.6) years. Fifteen patients were intractable only during the first 5 years of follow‐up (group A), and 19 subjects were intractable both during the first 5 years and the last year of follow‐up (group B). Compared with group A, group B had shorter remission and a longer time to intractability during the first 5 years and more were intractable in the fifth year of follow‐up. Sixteen other patients had a late onset of intractability after 5 years of follow‐up, sometimes after long periods of remission (group C). No significant differences in baseline characteristics were found among groups A, B, and C, but slightly more children in groups B and C became mentally retarded during the follow‐up. In all groups, antiepileptic drugs were of little use in preventing and ending intractability. Significance: There is a large unpredictable variation in time of onset, course, and duration of intractability, with a higher chance of final intractability after a poor course during the first 5 years of follow‐up. The natural course of epilepsy probably best explains the variable course of intractability. The effect of medication seems to be minor.