- Email: [email protected]
A census of movement disorders at a Thai university hospital
Journal of the Neurological Sciences 301 (2011) 31–34
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Journal of the Neurological Sciences j o u r n a l h o m e p a g e : w w w. e l s ev i e r. c o m / l o c a t e / j n s
A census of movement disorders at a Thai university hospital Roongroj Bhidayasiri a,b,⁎, Karn Saksornchai a, Lalita Kaewwilai a, Kammant Phanthumchinda a a Chulalongkorn Comprehensive Movement Disorders Center, Department of Medicine, Faculty of Medicine, Chulalongkorn University and King Chulalongkorn Memorial Hospital, Thai Red Cross Society, Bangkok, 10330, Thailand b Department of Neurology, Geffen School of Medicine at UCLA, Los Angeles 90095, USA
a r t i c l e
i n f o
Article history: Received 22 March 2010 Received in revised form 5 November 2010 Accepted 10 November 2010 Available online 4 December 2010 Keywords: Movement disorders Parkinson's disease Thailand
a b s t r a c t There is little information available on the number of patients with movement disorders seen by physicians in Thailand. The authors reviewed the medical records of all movement disorders patients seen at the Chulalongkorn Comprehensive Movement Disorders Center (CUMDS) in Bangkok, Thailand over a 4.5-year period to determine the number of patients with movement disorders and disease characteristics. A total of 1993 patients were assessed at CUMDS. Most of these patients had a diagnosis of parkinsonism (72%), including Parkinson's disease (PD) (60.9%), followed by tremor (9.6%), and dystonia (8.4%). The diagnostic accuracy of PD according to United Kingdom Parkinson's Disease Society Brain Bank clinical diagnostic criteria was 90.3%. The average referral period waiting for the consultation was more than 2 years. In spite of the limited availability of medical resources in Thailand, patients with movement disorders tend to seek specialist care and most often it is indicated. This finding documents the need for awareness of PD and other movement disorders by health professionals in Thailand, including the need for specialized training in movement disorders for physicians, including neurologists. © 2010 Elsevier B.V. All rights reserved.
Although the subspecialty of movement disorders has been established in neurology for more than 20 years, it is relatively new in Thailand and there are only a few clinics dedicated to movement disorders in the country. The Chulalongkorn Comprehensive Movement Disorders center (CUMDS) was recently established by the Thai Red Cross Society as the main tertiary care center in Thailand specializing in the treatment and study of Parkinson's disease (PD) and various other movement disorders. Currently, the center is staffed with two full-time board-certified neurologists with fellowship training in movement disorders, two clinical fellows, three clinical and research nurses, and two support personnel. A multidisciplinary approach is taken in the care of patients with movement disorders and there is strong collaboration between the Center and Neurosurgery, which has an active deep brain stimulation program, Psychiatry, Rehabilitation and the laboratories. In a recent survey of 2326 PD patients who attended the Parkinson's Disease Awareness Day, organized by the CUMDS and the Thai Red Cross Society on 10 July 2010, more than 90% of the patients indicated that they would like to be seen by a specialist in movement disorders. However, fewer than 30% of PD patients who attended the event had ever consulted a neurologist. This is consistent
with other surveys which show that most PD patients in Thailand are treated by general internists without specialty training in neurology. 63% of patients who completed the survey live outside of Bangkok and have to commute to PD clinics for their follow-up visits, which usually require a trip to the city every three months. Since the establishment of the Center in 2005, there has been a large increase in the number of referred patients from all over the country. With a staff of seven (two fellowship trained in movement disorders, two fellows in movement disorders, two nurses, and one study coordinator) operating three movement disorders outpatient clinics at CUMDS, we provided 2855 movement disorders consultations in 2007, 2957 consultations in 2008, and 2753 consultations in 2009. Despite this increasing number of movement disorders consultations provided since the opening of the Center in 2005, there has been a concurrent increase in demand so that we have adopted a referral-back policy in which uncomplicated movement disorders patients are transferred back to their primary physicians for follow-up. With this approach, we have been successful in reducing the number of new patients on the waiting list by more than 20% per year. Nevertheless, this situation indicates that there are still a large number of patients suffering from movement disorders who may not be able to get the specialist care they need, even after such a long waiting period, because of the following realities:
⁎ Corresponding author. Chulalongkorn Comprehensive Movement Disorders Center Division of Neurology, Chulalongkorn University Hospital 1873 Rama 4 Road Bangkok 10330, Thailand. Tel.: +662 256 4627; fax: +662 256 4630. E-mail address: [email protected] (R. Bhidayasiri).
• Limited access — very few PD clinics are available and they are located only in major cities, • Availability — there are only 278 board-certified neurologists in Thailand serving a population of 65 million populations, and
1. Introduction
0022-510X/$ – see front matter © 2010 Elsevier B.V. All rights reserved. doi:10.1016/j.jns.2010.11.010
32
R. Bhidayasiri et al. / Journal of the Neurological Sciences 301 (2011) 31–34
• Affordability of services — The National Health Service provides only basic antiparkinsonian medications, including levodopa bromocriptine and anticholinergics. This gap between the need for specialized care for movement disorders and service utilization can be addressed by developing additional specialized tertiary care centers. This study was designed to evaluate the number and clinical profiles of patients seen at outpatient clinics for movement disorders at Chulalongkorn University Hospital in Bangkok, Thailand, over a 4.5year period.
secondary dystonia were diagnosed according to a definition of sustained involuntary muscle contractions causing twisting or abnormal posture [4]. Clinical subtypes of dystonia were defined by topical distribution with standard clinical criteria. In patients with chorea, specific diagnostic information about underlying cause was obtained (drug history, genetic testing for Hungtington's disease, family history, and structural brain disease) [8]. Tics were defined as repetitive and intermittent movements of individual muscle groups that can be temporarily suppressed at the expense of rising stress levels [9]. The diagnosis of psychogenic movement disorders were adapted from Fahn et al [10].
2. Patients and methods 3. Results We identified all patients seen from January 2006 to May 2010 at the movement disorders clinics of the Chulalongkorn Comprehensive Movement Disorders Center (CUMDS), which is affiliated with Chulalongkorn University Hospital and Thai Red Cross Society. A standard data collection form was used to document the frequency of different types of movement disorders, personal data (present age and sex), patient characteristics (age-at-onset of the disease, age at presentation, and duration of illness before presentation), disease characteristics (initial and distribution of symptoms), etiologies, diagnostic evaluations and treatment. A movement disorders specialist (RB) established the movement disorders database and services at CUMDS in 2005 as well as a botulinum toxin clinic. Patients for the study were identified from this database and the medical records of these patients were reviewed to obtain demographic and clinical characteristics of each patient. Descriptive statistical analyses were done where appropriate. Data were analyzed using SPSS version 13.0 (Chicago, Illinois). Continuous data are expressed as mean (standard deviation, SD), median and ranges. This study was approved by the Ethics Committee of the Chulalongkorn University Hospital. All patients with primary complaints of movement disorders were considered for this study, assuming that the principal diagnosis was the main reason for referral. However, only patients with a minimal follow-up of three months were included and all patients were confirmed to have a movement disorder by a movement disorders specialist (RB). First, the movement disorders were categorized according to the presenting phenomenon dominating the clinical presentation, including parkinsonism, tremor, dystonia, chorea, myoclonus and tics. Different types of abnormal movements were defined by their standard clinical criteria [1–4]. Second, a specific etiology was investigated for the individual phenomenon of the movement disorders. A movement disorders specialist (RB) and one clinician (KS) reviewed the patients' records at least 3 months after the initial visit. The final diagnosis occasionally differed from the tentative initial diagnosis, depending on the clinical course during follow-up, and additional investigations, including neuroimaging (MRI of the brain, Fluorodopa positron emission tomography), genetic tests (SCA1, SCA2, SCA3, HD, and DYT1), and ancillary blood tests. Classification of participants with clinical signs of parkinsonism was done according to the United Kingdom Parkinson's Disease Society Brain Bank (UKPDSBB) criteria for Parkinson's disease (PD) [3] and consensus criteria for dementia with Lewy bodies, multiple system atrophy, progressive supranuclear palsy, corticobasal degeneration, and vascular parkinsonism [5]. Young-onset PD was defined as age-at-onset ≤ 40 years [6]. A positive family history of PD was defined as a history of parkinsonian features or reported physician's diagnosis of PD in a first-degree relative. Drug-induced movement disorders were diagnosed if dopamine receptor blocking agents were started within 6 months of the onset of symptoms and taken for at least 6 months [7]. A diagnosis of tremor disorders was made according to the consensus statement on tremor [1]. Primary and
From January 2006 to May 2010, 1993 patients were seen in the Outpatient Movement Disorders Clinic during 10,365 total patient visits. 19 patients were excluded due to incomplete medical records. There were 997 (50.5%) men and 977 (49.5%) women with the mean age of 62.7 ± 16.3 years (range: 4–99 years). Table 1 lists the patients by types of movement disorder, with their evaluation and diagnosis. There were 1421 (72%) patients with parkinsonism, of whom 1202 (60.9%) were diagnosed with Parkinson's disease (PD). 710 (59.1%) PD patients had an age of onset between 50 and 70 years while 87 (7.2%) PD patients had symptom onset before the age of 40 years, representing a group of young-onset PD. Family history of PD in a first-degree relative was obtained in 26 patients (2.2%). Tremor at rest was the most common presenting symptom (50.7%), followed by asymmetric rigidity (31.2%), bradykinesia (22.6%) and postural instability (8.5%). In our cohort, 420 PD patients (34.6%) were seen by us within 2 years of symptom onset while the rest of patients were seen after 3 years of disease onset. Motor fluctuations were documented in 26.4% of the patients. The average dose of levodopawas 483.4 ± 251.7 mg/day. Most PD patients (65.8%) had consultations scheduled every three months in order to evaluate their Table 1 Types of movement disorders seen at Chulalongkorn Comprehensive Movement Disorders Center (CUMDS). Types of movement disorders 1) Parkinsonism 1.1) Parkinson's disease 1.2) Secondary parkinsonism –Vascular parkinsonism 1.3) Parkinsonism-plus syndrome –Multiple system atrophy –Dementia with Lewy bodies –Corticobasal syndrome –Progressive supranuclear palsy –Alzheimer's disease with parkinsonism –Frontotemporal dementia with parkinsonism 2) Tremor 2.1) Essential tremor 2.2) Drug-induced tremor 2.3) Enhanced physiologic tremor 2.4) Psychogenic tremor 2.5) Holmes' tremor (from Wilson's disease) 2.6) Palatal tremor 2) Dystonia 2.1) Focal dystonia –Cervical dystonia –Writer's cramp –Blepharospasm –Spasmodic dysphonia 2.2) Segmental dystonia –Meige's syndrome 2.3) Hemidystonia 2.4) Generalized dystonia
N (%) (1974 = 100%)
Prevalence (2009)
1202 127 98 92 28 26 16 10 10 2
(60.9%) (6.4%) (4.9%) (4.7%) (1.4%) (1.3%) (0.8%) (0.5%) (0.5%) (0.1%)
396.8/100,000
189 159 18 5 3 3 1 166 116 69 12 11 5 34 25 3 13
(9.5%) (8.1%) (0.9%) (0.2%) (0.1%) (0.1%) (0.05%) (8.4%) (5.9%) (3.5%) (0.6%) (0.5%) (0.2%) (1.7%) (1.3%) (0.2%) (0.6%)
10.9/100,000
6.24/100,000
R. Bhidayasiri et al. / Journal of the Neurological Sciences 301 (2011) 31–34
clinical condition and to replenish their supply of antiparkinsonian medications, which are supported by the National Health Service. In advanced PD patients (15.2%), we follow them monthly in order to make frequent drug adjustments and assessments by a multidisciplinary team for possible surgical interventions, including pallidotomy and globus pallidus interna or subthalamic nucleus deep brain stimulation. Clinicopathologic studies are difficult to perform among Thai patients with parkinsonism, partly due to cultural and religion beliefs. However, the accuracy of a clinical diagnosis of PD on referred cases was assessed based on the UKPDSBB clinical diagnostic criteria. There were 89 patients who did not fulfill the UKPDSBB clinical diagnostic criteria due to incomplete medical records and some exclusion criteria, including neuroleptic treatment at onset of symptoms, supranuclear palsy, severe dementia with disturbances of memory, and an inadequate trial of levodopa. 20 patients had inadequate supportive criteria. As a result, there were 1312 patients who were referred to us with prior clinical diagnosis of PD. Among those, 1193 satisfied the UKPDSBB clinical diagnostic criteria for idiopathic PD, giving the accuracy of clinical diagnosis of 90.3%. However, we must be bear in mind that this high figure of diagnostic accuracy was based on a standard clinical diagnostic criterion, not necropsy studies. In our cohort, vascular parkinsonism (20.7%) was responsible for the majority of false positive cases, followed by essential tremor and progressive supranuclear palsy (13.8% equally), and corticobasal degeneration (8.6%) respectively. Tremor (excluding tremor at rest in PD) was the second most common movement disorder being referred to the clinic, representing 189 cases (9.6%). Most of these patients (84.1%) were diagnosed with essential tremor while the remaining diagnoses include druginduced tremor, enhanced physiologic tremor, Holmes' tremor (from midbrain infarct and Wilson's disease), palatal tremor and psychogenic tremor. There were 166 cases (8.4%) with dystonia. The majority (85.2%) of patients with dystonia had primary dystonia. Focal dystonia accounted for 74.4% of primary dystonia. Segmental (15.4%), generalized dystonia (8.4%) and hemidystonia (1.8%) accounted for the rest. The most common focal dystonia was cervical dystonia (44.2%), followed by writer's cramp (7.7%) and blepharospasm (7.1%). There were 24 cases (14.8%) of secondary dystonia in which the most common etiologies were tardive dystonia (52%), focal cerebral ischemia (25.2%), and perinatal injury (18.9%). There were 12 patients with Wilson's disease, thirteen patients with genetically proven Huntington's disease and ten patients with genetically proven spinocerebellar ataxia (SCA) (8 patients with SCA3, 1 patient with SCA2, 1 patient with SCA1). We saw a small number (20) of pediatric cases with movement disorders since most of the patients are followed by two pediatric neurologists. Among those, 13 patients was diagnosed with childhood-onset tics or Tourette's syndrome and five patients with primary dystonia in which none of the childhood-onset patients with primary dystonia tested positive for DYT1. 4. Discussion This study focused on the analysis of clinical patterns and specific etiologies of movement disorders in patients being referred to CUMDS during a 4.5-year period. The data suggest that patients with movement disorders represent a substantial proportion of patients with neurological disorders, comparable to that in developed countries [11]. While the prevalence of stroke from a survey of a medium-size community in Bangkok was 6.9/1000 [12], the prevalence of patients with movement disorders at CUMDS in 2009 was found to be relatively similar at 4.2/1000. However, this comparison of the prevalence is often difficult since the studies were conducted in different settings and designs. Despite a large number of movement
33
disorders patients at CUMDS, the frequency of movement disorders reported here is likely to be an underestimate due to limited certified personnel, time, and distance. Due to a long waiting list of more than 2 years for new referrals at the center, this problem probably reflects the pressing needs of patients with movement disorders requiring specialist care, and a lack of medical support in local communities, resulting in a large influx of cases within a few years of the establishment of the Center. The data also support the need for more training in movement disorders for physicians, including neurologists, in Thailand. According to the specialist registry of the medical council of Thailand, there are currently 278 board-certified neurologists in Thailand, with more than 50% residing in Bangkok [13]. There are only eight neurologists in the nation who have completed fellowship training in movement disorders. Since data on the prevalence of parkinsonism and other movement disorders in Thailand are not available, we are unable to estimate the percentage of affected patients seeking specialist care. However, the prevalence of movement disorders at CUMDS was similar to the data from the combined databases of the movement disorders clinics at Columbia University Medical Center (New York) and Baylor College of Medicine (Houston) for 21,766 patients encountered through 1996 in which parkinsonism, dystonia, and tremor represent the most common types of abnormal movements seen in these tertiary center clinics [14]. In addition, data collected in 1996 from another university hospital in Bangkok found that a total of 1100 patients with various movement disorders were treated with botulinum toxin [15]. Despite limitations in treatment availability and affordability locally, many patients travel to regional neurological centers and university hospitals to obtain specialist care. CUMDS has been conducting a national campaign for the development of a PD registry in Thailand in collaboration with the Ministry of Public Heath, the National Health Security Office, and the Bangkok Metropolitan Administration since January 2009 and the final results are expected to be available within the next few years [16]. Up to May 2010, there are 28,868 PD patients registered to the system, giving a crude prevalence of 396.8/100,000 population (95% CI 285.1–425.9). This study gives an overview of the number of different types of movement disorders and disease characteristics in patients with movement disorders at CUMDS, which is a tertiary movement disorders referral center in Thailand. The most significant limitation of this study is that its clinic-based design reflects the selection bias of the patients, necessitating further community-based studies. As a result, the spectrum of cases encountered may thus be subject to referral bias, with a tendency to see more severe or more complex movement disorders cases. Furthermore, the lack of resources may limit the potential for accurate diagnosis in some patients; for example patients with ataxia who may require advanced genetic tests (not limited to SCA1, SCA2, and SCA3). In spite of this limitation, clinic-based surveys like this help initiate the process of increasing professional awareness of PD and other movement disorders, which is still considered as a rather “unfamiliar” subspecialty to many general internists in Thailand. In addition, this study provides data for comparison with an earlier report and global perspectives of movement disorders in developing countries in which the number of affected individuals is not that uncommon as previously thought [17].
Acknowledgments This study was supported by Ratchadapiseksompoj faculty grant of the Faculty of Medicine, Chulalongkorn University and Parkinson's disease center development grant of the Thai Red Cross society. We thank Dr. Neil Brenden for the editorial assistance.
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References [1] Deuschl G, Bain P, Brin M. Consensus statement of the Movement Disorder Society on Tremor. Ad Hoc Scientific Committee Mov Disord 1998;13(Suppl 3):2–23. [2] Fahn S, Marsden CD, Van Woert MH. Definition and classification of myoclonus. Adv Neurol 1986;43:1–5. [3] Gibb WR, Lees AJ. The relevance of the Lewy body to the pathogenesis of idiopathic Parkinson's disease. J Neurol Neurosurg Psychiatry 1988;51:745–52. [4] Fahn S, Bressman SB, Marsden CD. Classification of dystonia. Adv Neurol 1998;78: 1–10. [5] Litvan I. Atypical parkinsonian disorders: clinical and research aspects. Totowa, New Jersey: Humana Press; 2005. [6] Golbe LI. Young-onset Parkinson's disease: a clinical review. Neurology 1991;41: 168–73. [7] Schrag A, Ben-Shlomo Y, Quinn NP. Cross sectional prevalence survey of idiopathic Parkinson's disease and Parkinsonism in London. BMJ 2000;321:21–2. [8] Bhidayasiri R, Truong DD. Chorea and related disorders. Postgrad Med J 2004;80: 527–34. [9] Jankovic J. Differential diagnosis and etiology of tics. Adv Neurol 2001;85:15–29.
[10] Hallett M, Cloninger RC, Fahn S, Jankovic J, Lang AE. The psychogenic movement disorders: neurology and neuropsychiatry. Lippincott Williams & Wilkins; 2005. [11] Wenning GK, Kiechl S, Seppi K, Mller J, Hogl B, Saletu M. Prevalence of movement disorders in men and women aged 50–89 years (Bruneck Study cohort): a population-based study. Lancet Neurol 2005;4:815–20. [12] Viriyavejakul A. Stroke in Asia: an epidemiological consideration. Clin Neuropharmacol 1990;13(Suppl 3):S26–33. [13] Towanabut S. Thai neurological information center data. Prasat Neurological Institute, Ministry of Health, Bangkok, Thailand; 2009. [14] Fahn S, Jankovic J. Principles and practice of movement disorders. Philadelphia: Churchill Livingstone Elsevier; 2007. [15] Poungvarin N. Prevalence of dystonia and occupational cramps in Thailand. In: Kimura J, Shibasaki H, eds. Recent advances in clinical neurophysiology Elsevier Science B.V.; 1996. [16] Bhidayasiri R, Wannachai N, Limpahandhu S, Asawawichienchinda T, Kaewwilai L, Decahastin R. Parkinson's disease registry in Thailand: an 18-month update. Mov Disord 2010;25:S256–7. [17] Muangpaisan W, Hori H, Brayne C. Systematic review of the prevalence and incidence of Parkinson's disease in Asia. J Epidemiol 2009;19:281–93.
Contents lists available at ScienceDirect
Journal of the Neurological Sciences j o u r n a l h o m e p a g e : w w w. e l s ev i e r. c o m / l o c a t e / j n s
A census of movement disorders at a Thai university hospital Roongroj Bhidayasiri a,b,⁎, Karn Saksornchai a, Lalita Kaewwilai a, Kammant Phanthumchinda a a Chulalongkorn Comprehensive Movement Disorders Center, Department of Medicine, Faculty of Medicine, Chulalongkorn University and King Chulalongkorn Memorial Hospital, Thai Red Cross Society, Bangkok, 10330, Thailand b Department of Neurology, Geffen School of Medicine at UCLA, Los Angeles 90095, USA
a r t i c l e
i n f o
Article history: Received 22 March 2010 Received in revised form 5 November 2010 Accepted 10 November 2010 Available online 4 December 2010 Keywords: Movement disorders Parkinson's disease Thailand
a b s t r a c t There is little information available on the number of patients with movement disorders seen by physicians in Thailand. The authors reviewed the medical records of all movement disorders patients seen at the Chulalongkorn Comprehensive Movement Disorders Center (CUMDS) in Bangkok, Thailand over a 4.5-year period to determine the number of patients with movement disorders and disease characteristics. A total of 1993 patients were assessed at CUMDS. Most of these patients had a diagnosis of parkinsonism (72%), including Parkinson's disease (PD) (60.9%), followed by tremor (9.6%), and dystonia (8.4%). The diagnostic accuracy of PD according to United Kingdom Parkinson's Disease Society Brain Bank clinical diagnostic criteria was 90.3%. The average referral period waiting for the consultation was more than 2 years. In spite of the limited availability of medical resources in Thailand, patients with movement disorders tend to seek specialist care and most often it is indicated. This finding documents the need for awareness of PD and other movement disorders by health professionals in Thailand, including the need for specialized training in movement disorders for physicians, including neurologists. © 2010 Elsevier B.V. All rights reserved.
Although the subspecialty of movement disorders has been established in neurology for more than 20 years, it is relatively new in Thailand and there are only a few clinics dedicated to movement disorders in the country. The Chulalongkorn Comprehensive Movement Disorders center (CUMDS) was recently established by the Thai Red Cross Society as the main tertiary care center in Thailand specializing in the treatment and study of Parkinson's disease (PD) and various other movement disorders. Currently, the center is staffed with two full-time board-certified neurologists with fellowship training in movement disorders, two clinical fellows, three clinical and research nurses, and two support personnel. A multidisciplinary approach is taken in the care of patients with movement disorders and there is strong collaboration between the Center and Neurosurgery, which has an active deep brain stimulation program, Psychiatry, Rehabilitation and the laboratories. In a recent survey of 2326 PD patients who attended the Parkinson's Disease Awareness Day, organized by the CUMDS and the Thai Red Cross Society on 10 July 2010, more than 90% of the patients indicated that they would like to be seen by a specialist in movement disorders. However, fewer than 30% of PD patients who attended the event had ever consulted a neurologist. This is consistent
with other surveys which show that most PD patients in Thailand are treated by general internists without specialty training in neurology. 63% of patients who completed the survey live outside of Bangkok and have to commute to PD clinics for their follow-up visits, which usually require a trip to the city every three months. Since the establishment of the Center in 2005, there has been a large increase in the number of referred patients from all over the country. With a staff of seven (two fellowship trained in movement disorders, two fellows in movement disorders, two nurses, and one study coordinator) operating three movement disorders outpatient clinics at CUMDS, we provided 2855 movement disorders consultations in 2007, 2957 consultations in 2008, and 2753 consultations in 2009. Despite this increasing number of movement disorders consultations provided since the opening of the Center in 2005, there has been a concurrent increase in demand so that we have adopted a referral-back policy in which uncomplicated movement disorders patients are transferred back to their primary physicians for follow-up. With this approach, we have been successful in reducing the number of new patients on the waiting list by more than 20% per year. Nevertheless, this situation indicates that there are still a large number of patients suffering from movement disorders who may not be able to get the specialist care they need, even after such a long waiting period, because of the following realities:
⁎ Corresponding author. Chulalongkorn Comprehensive Movement Disorders Center Division of Neurology, Chulalongkorn University Hospital 1873 Rama 4 Road Bangkok 10330, Thailand. Tel.: +662 256 4627; fax: +662 256 4630. E-mail address: [email protected] (R. Bhidayasiri).
• Limited access — very few PD clinics are available and they are located only in major cities, • Availability — there are only 278 board-certified neurologists in Thailand serving a population of 65 million populations, and
1. Introduction
0022-510X/$ – see front matter © 2010 Elsevier B.V. All rights reserved. doi:10.1016/j.jns.2010.11.010
32
R. Bhidayasiri et al. / Journal of the Neurological Sciences 301 (2011) 31–34
• Affordability of services — The National Health Service provides only basic antiparkinsonian medications, including levodopa bromocriptine and anticholinergics. This gap between the need for specialized care for movement disorders and service utilization can be addressed by developing additional specialized tertiary care centers. This study was designed to evaluate the number and clinical profiles of patients seen at outpatient clinics for movement disorders at Chulalongkorn University Hospital in Bangkok, Thailand, over a 4.5year period.
secondary dystonia were diagnosed according to a definition of sustained involuntary muscle contractions causing twisting or abnormal posture [4]. Clinical subtypes of dystonia were defined by topical distribution with standard clinical criteria. In patients with chorea, specific diagnostic information about underlying cause was obtained (drug history, genetic testing for Hungtington's disease, family history, and structural brain disease) [8]. Tics were defined as repetitive and intermittent movements of individual muscle groups that can be temporarily suppressed at the expense of rising stress levels [9]. The diagnosis of psychogenic movement disorders were adapted from Fahn et al [10].
2. Patients and methods 3. Results We identified all patients seen from January 2006 to May 2010 at the movement disorders clinics of the Chulalongkorn Comprehensive Movement Disorders Center (CUMDS), which is affiliated with Chulalongkorn University Hospital and Thai Red Cross Society. A standard data collection form was used to document the frequency of different types of movement disorders, personal data (present age and sex), patient characteristics (age-at-onset of the disease, age at presentation, and duration of illness before presentation), disease characteristics (initial and distribution of symptoms), etiologies, diagnostic evaluations and treatment. A movement disorders specialist (RB) established the movement disorders database and services at CUMDS in 2005 as well as a botulinum toxin clinic. Patients for the study were identified from this database and the medical records of these patients were reviewed to obtain demographic and clinical characteristics of each patient. Descriptive statistical analyses were done where appropriate. Data were analyzed using SPSS version 13.0 (Chicago, Illinois). Continuous data are expressed as mean (standard deviation, SD), median and ranges. This study was approved by the Ethics Committee of the Chulalongkorn University Hospital. All patients with primary complaints of movement disorders were considered for this study, assuming that the principal diagnosis was the main reason for referral. However, only patients with a minimal follow-up of three months were included and all patients were confirmed to have a movement disorder by a movement disorders specialist (RB). First, the movement disorders were categorized according to the presenting phenomenon dominating the clinical presentation, including parkinsonism, tremor, dystonia, chorea, myoclonus and tics. Different types of abnormal movements were defined by their standard clinical criteria [1–4]. Second, a specific etiology was investigated for the individual phenomenon of the movement disorders. A movement disorders specialist (RB) and one clinician (KS) reviewed the patients' records at least 3 months after the initial visit. The final diagnosis occasionally differed from the tentative initial diagnosis, depending on the clinical course during follow-up, and additional investigations, including neuroimaging (MRI of the brain, Fluorodopa positron emission tomography), genetic tests (SCA1, SCA2, SCA3, HD, and DYT1), and ancillary blood tests. Classification of participants with clinical signs of parkinsonism was done according to the United Kingdom Parkinson's Disease Society Brain Bank (UKPDSBB) criteria for Parkinson's disease (PD) [3] and consensus criteria for dementia with Lewy bodies, multiple system atrophy, progressive supranuclear palsy, corticobasal degeneration, and vascular parkinsonism [5]. Young-onset PD was defined as age-at-onset ≤ 40 years [6]. A positive family history of PD was defined as a history of parkinsonian features or reported physician's diagnosis of PD in a first-degree relative. Drug-induced movement disorders were diagnosed if dopamine receptor blocking agents were started within 6 months of the onset of symptoms and taken for at least 6 months [7]. A diagnosis of tremor disorders was made according to the consensus statement on tremor [1]. Primary and
From January 2006 to May 2010, 1993 patients were seen in the Outpatient Movement Disorders Clinic during 10,365 total patient visits. 19 patients were excluded due to incomplete medical records. There were 997 (50.5%) men and 977 (49.5%) women with the mean age of 62.7 ± 16.3 years (range: 4–99 years). Table 1 lists the patients by types of movement disorder, with their evaluation and diagnosis. There were 1421 (72%) patients with parkinsonism, of whom 1202 (60.9%) were diagnosed with Parkinson's disease (PD). 710 (59.1%) PD patients had an age of onset between 50 and 70 years while 87 (7.2%) PD patients had symptom onset before the age of 40 years, representing a group of young-onset PD. Family history of PD in a first-degree relative was obtained in 26 patients (2.2%). Tremor at rest was the most common presenting symptom (50.7%), followed by asymmetric rigidity (31.2%), bradykinesia (22.6%) and postural instability (8.5%). In our cohort, 420 PD patients (34.6%) were seen by us within 2 years of symptom onset while the rest of patients were seen after 3 years of disease onset. Motor fluctuations were documented in 26.4% of the patients. The average dose of levodopawas 483.4 ± 251.7 mg/day. Most PD patients (65.8%) had consultations scheduled every three months in order to evaluate their Table 1 Types of movement disorders seen at Chulalongkorn Comprehensive Movement Disorders Center (CUMDS). Types of movement disorders 1) Parkinsonism 1.1) Parkinson's disease 1.2) Secondary parkinsonism –Vascular parkinsonism 1.3) Parkinsonism-plus syndrome –Multiple system atrophy –Dementia with Lewy bodies –Corticobasal syndrome –Progressive supranuclear palsy –Alzheimer's disease with parkinsonism –Frontotemporal dementia with parkinsonism 2) Tremor 2.1) Essential tremor 2.2) Drug-induced tremor 2.3) Enhanced physiologic tremor 2.4) Psychogenic tremor 2.5) Holmes' tremor (from Wilson's disease) 2.6) Palatal tremor 2) Dystonia 2.1) Focal dystonia –Cervical dystonia –Writer's cramp –Blepharospasm –Spasmodic dysphonia 2.2) Segmental dystonia –Meige's syndrome 2.3) Hemidystonia 2.4) Generalized dystonia
N (%) (1974 = 100%)
Prevalence (2009)
1202 127 98 92 28 26 16 10 10 2
(60.9%) (6.4%) (4.9%) (4.7%) (1.4%) (1.3%) (0.8%) (0.5%) (0.5%) (0.1%)
396.8/100,000
189 159 18 5 3 3 1 166 116 69 12 11 5 34 25 3 13
(9.5%) (8.1%) (0.9%) (0.2%) (0.1%) (0.1%) (0.05%) (8.4%) (5.9%) (3.5%) (0.6%) (0.5%) (0.2%) (1.7%) (1.3%) (0.2%) (0.6%)
10.9/100,000
6.24/100,000
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clinical condition and to replenish their supply of antiparkinsonian medications, which are supported by the National Health Service. In advanced PD patients (15.2%), we follow them monthly in order to make frequent drug adjustments and assessments by a multidisciplinary team for possible surgical interventions, including pallidotomy and globus pallidus interna or subthalamic nucleus deep brain stimulation. Clinicopathologic studies are difficult to perform among Thai patients with parkinsonism, partly due to cultural and religion beliefs. However, the accuracy of a clinical diagnosis of PD on referred cases was assessed based on the UKPDSBB clinical diagnostic criteria. There were 89 patients who did not fulfill the UKPDSBB clinical diagnostic criteria due to incomplete medical records and some exclusion criteria, including neuroleptic treatment at onset of symptoms, supranuclear palsy, severe dementia with disturbances of memory, and an inadequate trial of levodopa. 20 patients had inadequate supportive criteria. As a result, there were 1312 patients who were referred to us with prior clinical diagnosis of PD. Among those, 1193 satisfied the UKPDSBB clinical diagnostic criteria for idiopathic PD, giving the accuracy of clinical diagnosis of 90.3%. However, we must be bear in mind that this high figure of diagnostic accuracy was based on a standard clinical diagnostic criterion, not necropsy studies. In our cohort, vascular parkinsonism (20.7%) was responsible for the majority of false positive cases, followed by essential tremor and progressive supranuclear palsy (13.8% equally), and corticobasal degeneration (8.6%) respectively. Tremor (excluding tremor at rest in PD) was the second most common movement disorder being referred to the clinic, representing 189 cases (9.6%). Most of these patients (84.1%) were diagnosed with essential tremor while the remaining diagnoses include druginduced tremor, enhanced physiologic tremor, Holmes' tremor (from midbrain infarct and Wilson's disease), palatal tremor and psychogenic tremor. There were 166 cases (8.4%) with dystonia. The majority (85.2%) of patients with dystonia had primary dystonia. Focal dystonia accounted for 74.4% of primary dystonia. Segmental (15.4%), generalized dystonia (8.4%) and hemidystonia (1.8%) accounted for the rest. The most common focal dystonia was cervical dystonia (44.2%), followed by writer's cramp (7.7%) and blepharospasm (7.1%). There were 24 cases (14.8%) of secondary dystonia in which the most common etiologies were tardive dystonia (52%), focal cerebral ischemia (25.2%), and perinatal injury (18.9%). There were 12 patients with Wilson's disease, thirteen patients with genetically proven Huntington's disease and ten patients with genetically proven spinocerebellar ataxia (SCA) (8 patients with SCA3, 1 patient with SCA2, 1 patient with SCA1). We saw a small number (20) of pediatric cases with movement disorders since most of the patients are followed by two pediatric neurologists. Among those, 13 patients was diagnosed with childhood-onset tics or Tourette's syndrome and five patients with primary dystonia in which none of the childhood-onset patients with primary dystonia tested positive for DYT1. 4. Discussion This study focused on the analysis of clinical patterns and specific etiologies of movement disorders in patients being referred to CUMDS during a 4.5-year period. The data suggest that patients with movement disorders represent a substantial proportion of patients with neurological disorders, comparable to that in developed countries [11]. While the prevalence of stroke from a survey of a medium-size community in Bangkok was 6.9/1000 [12], the prevalence of patients with movement disorders at CUMDS in 2009 was found to be relatively similar at 4.2/1000. However, this comparison of the prevalence is often difficult since the studies were conducted in different settings and designs. Despite a large number of movement
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disorders patients at CUMDS, the frequency of movement disorders reported here is likely to be an underestimate due to limited certified personnel, time, and distance. Due to a long waiting list of more than 2 years for new referrals at the center, this problem probably reflects the pressing needs of patients with movement disorders requiring specialist care, and a lack of medical support in local communities, resulting in a large influx of cases within a few years of the establishment of the Center. The data also support the need for more training in movement disorders for physicians, including neurologists, in Thailand. According to the specialist registry of the medical council of Thailand, there are currently 278 board-certified neurologists in Thailand, with more than 50% residing in Bangkok [13]. There are only eight neurologists in the nation who have completed fellowship training in movement disorders. Since data on the prevalence of parkinsonism and other movement disorders in Thailand are not available, we are unable to estimate the percentage of affected patients seeking specialist care. However, the prevalence of movement disorders at CUMDS was similar to the data from the combined databases of the movement disorders clinics at Columbia University Medical Center (New York) and Baylor College of Medicine (Houston) for 21,766 patients encountered through 1996 in which parkinsonism, dystonia, and tremor represent the most common types of abnormal movements seen in these tertiary center clinics [14]. In addition, data collected in 1996 from another university hospital in Bangkok found that a total of 1100 patients with various movement disorders were treated with botulinum toxin [15]. Despite limitations in treatment availability and affordability locally, many patients travel to regional neurological centers and university hospitals to obtain specialist care. CUMDS has been conducting a national campaign for the development of a PD registry in Thailand in collaboration with the Ministry of Public Heath, the National Health Security Office, and the Bangkok Metropolitan Administration since January 2009 and the final results are expected to be available within the next few years [16]. Up to May 2010, there are 28,868 PD patients registered to the system, giving a crude prevalence of 396.8/100,000 population (95% CI 285.1–425.9). This study gives an overview of the number of different types of movement disorders and disease characteristics in patients with movement disorders at CUMDS, which is a tertiary movement disorders referral center in Thailand. The most significant limitation of this study is that its clinic-based design reflects the selection bias of the patients, necessitating further community-based studies. As a result, the spectrum of cases encountered may thus be subject to referral bias, with a tendency to see more severe or more complex movement disorders cases. Furthermore, the lack of resources may limit the potential for accurate diagnosis in some patients; for example patients with ataxia who may require advanced genetic tests (not limited to SCA1, SCA2, and SCA3). In spite of this limitation, clinic-based surveys like this help initiate the process of increasing professional awareness of PD and other movement disorders, which is still considered as a rather “unfamiliar” subspecialty to many general internists in Thailand. In addition, this study provides data for comparison with an earlier report and global perspectives of movement disorders in developing countries in which the number of affected individuals is not that uncommon as previously thought [17].
Acknowledgments This study was supported by Ratchadapiseksompoj faculty grant of the Faculty of Medicine, Chulalongkorn University and Parkinson's disease center development grant of the Thai Red Cross society. We thank Dr. Neil Brenden for the editorial assistance.
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