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Review Malignant Tourette syndrome. 2007
Cheung MY, Shahed J, Jankovic J. · Parkinson's Disease Center and Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine, Houston, Texas 77030, USA. · Mov Disord. · Pubmed #17566119 No free full text.
Abstract: The aim of this work was to draw attention to potentially life-threatening symptoms associated with Tourette syndrome (TS) and to explore their relationship to TS comorbidities. Medical records of all patients with TS evaluated at our Movement Disorders Clinic between July 2003 and July 2006 were reviewed. Data on patients with malignant TS, defined as >or=2 emergency room (ER) visits or >or=1 hospitalizations for TS symptoms or its associated behavioral comorbidities, were entered into a dataset and analyzed. Five illustrative cases are described. Of 333 TS patients evaluated during the 3-year period, 17 (5.1%) met the criteria for malignant TS. Hospital admission or ER visits were for tic-related injuries, self-injurious behavior (SIB), uncontrollable violence and temper, and suicidal ideation/attempts. Compared with patients with nonmalignant TS, those with malignant TS were significantly more likely to have a personal history of obsessive compulsive behavior/disorder (OCB/OCD), complex phonic tics, coprolalia, copropraxia, SIB, mood disorder, suicidal ideation, and poor response to medications. Although TS is rarely a disabling disorder, about 5% of patients referred to a specialty clinic have life-threatening symptoms. Malignant TS is associated with greater severity of motor symptoms and the presence of >or=2 behavioral comorbidities. OCD/OCB in particular may play a central role in malignant TS; obsessive compulsive qualities were associated with life-threatening tics, SIB, and suicidal ideation. Malignant TS is more refractory to medical treatment than nonmalignant TS.
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Review Contemporary assessment and pharmacotherapy of Tourette syndrome. 2006
Scahill L, Erenberg G, Berlin CM, Budman C, Coffey BJ, Jankovic J, Kiessling L, King RA, Kurlan R, Lang A, Mink J, Murphy T, Zinner S, Walkup J, Anonymous00057. · Yale Child Study Center, 230 South Frontage Road, P.O. Box 207900, New Haven, CT 06520, USA. · NeuroRx. · Pubmed #16554257 No free full text.
Abstract: To develop a guide to clinical assessment and pharmacotherapy for children and adults with Tourette syndrome (TS), we reviewed published literature over the past 25 years to identify original articles and reviews on the assessment and pharmacological treatment of Tourette syndrome, attention-deficit/hyperactivity disorder (ADHD) and obsessive-compulsive disorder (OCD). The literature search also included a survey of reviews published in book chapters. The assessment section was compiled from several reviews. Pharmacological treatments were classified into those with strong empirical support (as evidenced by two positive placebo-controlled studies for tics, OCD, or ADHD in TS samples); modest empirical support (one positive placebo-controlled study), or minimal support (open-label data only). We conclude that accurate diagnosis, including identification of comorbid conditions, is an essential step toward appropriate treatment for patients with TS. In many patients with TS, symptom management requires pharmacotherapy for tics or coexisting conditions. The evidence supporting efficacy and safety for medications used in patients with TS varies. But this evidence offers the best guide to clinical practice.
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Review Tourette's syndrome and the law. free! 2006
Jankovic J, Kwak C, Frankoff R. · Parkinson's Disease Center and Movement Diorders Clinic, Department of Neurology, Baylor College of Medicine, Houston, TX 77030, USA. · J Neuropsychiatry Clin Neurosci. · Pubmed #16525075 links to free full text
Abstract: Diminished legal responsibility and mental capacity have been used in defense of individuals with neurological disorders charged with legal misdemeanors, including criminal behavior. The purpose of this report is to 1) critically examine the mechanisms that may predispose patients with Tourette's syndrome (TS) to potentially, legally liable behaviors; 2) report the results of a nation-wide review of state, federal, and appellate cases involving TS; and 3) instigate awareness within the professional legal community regarding unrecognized organically-based behaviors that may predispose TS patients to unwanted legal disciplinary action. TS is a common neurological movement disorder of childhood onset associated with behavioral comorbidities, including impulse control problems, exhibition of obscene language or gestures, rage attacks, inappropriate obsessions, and other behaviors. To our knowledge, there are no studies (to date) addressing the potential impact of TS on the legal system. A comprehensive review of the neurobehavioral mechanisms underlying comorbid issues in TS is outlined. A comprehensive review of all cases tried in state and federal courts between 1985 and 2003, in which TS was somehow implicated, was conducted using the Westlaw database. As of October, 2003, TS was implicated in more than 150 cases found in the federal and state databases, 21 of which were criminal. Other cases are categorized as civil rights, criminal, education, family, labor, and social security cases. The authors conclude that TS rarely leads to criminal behavior, but patients with TS who have behavioral comorbidities are at risk of being involved with the legal system. The medical-legal community must learn to recognize the vulnerability of this patient population to potential mistreatment by the courts of justice.
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Review The effect of deep brain stimulation on quality of life in movement disorders. free! 2005
Diamond A, Jankovic J. · Department of Neurology, Parkinson's Disease Center and Movement Disorders Clinic, Baylor College of Medicine, 6550 Fannin Street, Suite 1801, Houston, TX 77030, USA. · J Neurol Neurosurg Psychiatry. · Pubmed #16107348 links to free full text
Abstract: Deep brain stimulation (DBS) is a viable treatment alternative for patients with Parkinson's disease (PD), essential tremor (ET), dystonia, and cerebellar outflow tremors. When poorly controlled, these disorders have detrimental effects on the patient's health related quality of life (HRQoL). Instruments that measure HRQoL are useful tools to assess burden of disease and the impact of therapeutic interventions on activities of daily living, employment, and other functions. We systematically and critically reviewed the literature on the effects of DBS on HRQoL in PD, ET, dystonia, and cerebellar outflow tremor related to multiple sclerosis.
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Review Psychogenic movement disorders: diagnosis and management. 2004
Thomas M, Jankovic J. · Department of Neurology, Parkinson's Disease Center and Movement Disorders Clinic, Baylor College of Medicine, Houston, Texas, USA. · CNS Drugs. · Pubmed #15139799 No free full text.
Abstract: Psychogenic movement disorders (PMDs) are best defined as hyper- or hypo-kinetic movement disorders, often associated with gait disorders, that cannot be directly attributed to a lesion or dysfunction of the nervous system and which are derived in most cases from psychological or psychiatric causes. There are a variety of PMDs including tremor, dystonia, parkinsonism, gait disorders and, even, unusual forms including paroxysmal dyskinesias. As has been recognised in the recent literature, PMDs cannot be strictly classified into clearly defined psychiatric disorders such as somatoform, dissociative or conversion disorders. In this review, we discuss the diagnosis of various PMDs (including hyper- and hypo-kinetic disorders; and current evidence for underlying comorbid disorders) and the current therapeutic approach to them. The therapy of PMDs is not well established, is very challenging to the clinician, and a better outcome can be achieved in the setting of a team approach involving movement disorders specialists, psychiatrists and therapists who specialise in cognitive-behavioural techniques. Current pharmacological and non-pharmacological approaches to treatment focus on therapy of underlying comorbid psychiatric and psychological issues, although compliance is a major concern.
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Review Tourette's syndrome. 2001
Jankovic J. · Parkinson's Disease Center and Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine, Houston, TX 77030, USA. · N Engl J Med. · Pubmed #11642235 No free full text.
This publication has no abstract.
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Article Cognitive declines following bilateral subthalamic nucleus deep brain stimulation for the treatment of Parkinson's disease. 2008
York MK, Dulay M, Macias A, Levin HS, Grossman R, Simpson R, Jankovic J. · Baylor College of Medicine, Department of Neurology, 6501 Fannin, NB302, Houston, Texas 77030, USA. · J Neurol Neurosurg Psychiatry. · Pubmed #17965146 No free full text.
Abstract: BACKGROUND: We investigated the cognitive and psychiatric outcome 6 months after bilateral subthalamic nucleus deep brain stimulation (DBS) for the treatment of Parkinson's disease (PD) using a disease control group. METHODS: 23 patients who underwent DBS were compared with 28 medically treated patients with PD at baseline and at 6 months for neuropsychological measures. In addition to the group outcomes, we report reliable change indices (RCI) and a dementia caseness analysis. RESULTS: Patients who underwent DBS demonstrated a significant decline in verbal memory compared with the control group (p<0.003), and trends for decline on oral information processing, including verbal fluency, timed transcription and word naming. Patients who underwent DBS demonstrated declines in attention, set shifting and semantic fluency but these changes were similar to the rate of decline in the PD group. RCI indicated that patients who underwent DBS demonstrated clinically significant declines in verbal fluency (p<0.01) and inhibition of a dominant response (p<0.003), with trends for declines in set shifting (p<0.02) and verbal long term recall (p<0.08), indicative of frontostriatal dysfunction. Patients who underwent DBS did not demonstrate significant changes in depression, anxiety or psychological distress scores. The caseness analysis revealed that one of the patients who underwent DBS (4%) converted to dementia over 6 months compared with none of the PD controls. CONCLUSIONS: Our findings demonstrated that patients who underwent DBS experienced declines in verbal recall and trends for declines in oral information processing 6 months following surgery, even when good motor outcome was achieved. Potential candidates should be counselled about the risk of mild frontostriatal cognitive declines following DBS to weigh the risks and benefits of surgery.
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Article Essential tremor among children. free! 2004
Jankovic J, Madisetty J, Vuong KD. · Department of Neurology, Parkinson's Disease Center and Movement Disorders Clinic, Baylor College of Medicine, 6550 Fannin St, Suite 1801, Houston, TX 77030, USA. · Pediatrics. · Pubmed #15520096 links to free full text
Abstract: OBJECTIVE: To characterize the clinical and therapeutic aspects of essential tremor (ET) among children. BACKGROUND: ET, an autosomal dominant disorder, has been studied extensively among adults, but little is known regarding its occurrence, clinical characteristics, treatment, and prognosis in pediatric populations. Often stigmatized as a disorder of the elderly, ET may be misdiagnosed among children. Previous studies of childhood-onset ET were limited by small sample sizes. METHODS: Clinical data, including gender, age at onset, family history, associated disorders, and response to treatment, were collected for consecutive patients diagnosed with childhood-onset ET at the Movement Disorders Clinic at Baylor College of Medicine. RESULTS: Of the 39 patients with ET, 29 (74.4%) were male. The mean age at onset was 8.8 +/- 5.0 years, and the mean age at evaluation was 20.3 +/- 14.4 years. A family history of tremor was noted for 79.5% of the patients. Eighteen (46.2%) had some neurologic comorbidity, such as dystonia, which was noted for 11 patients (28.2%). Only 24 of the patients (61.5%) were treated with a specific antitremor medication; 5 of the 12 patients treated with propranolol experienced improvement. CONCLUSIONS: Concomitant movement disorders, such as dystonia, are common among patients with childhood-onset ET, which supports the concept that ET is a heterogeneous disorder. Treatment strategies used for adult patients with ET seem to be effective also for children with ET, although controlled therapeutic trials in this population of patients with ET are lacking.
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Article Examination of the SGCE gene in Tourette syndrome patients with obsessive-compulsive disorder. 2004
de Carvalho Aguiar P, Fazzari M, Jankovic J, Ozelius LJ. · Department of Molecular Genetics, Albert Einstein College of Medicine, Bronx, New York 10461, USA. · Mov Disord. · Pubmed #15368614 No free full text.
Abstract: Mutations in the epsilon-sarcoglycan gene (SGCE) have been reported in families with myoclonus-dystonia (M-D). In addition to abnormal movements, obsessive-compulsive disorder (OCD) has also been described in families with M-D. OCD is a common feature in another movement disorder, namely Tourette syndrome (TS). The comorbidity of these disorders suggests that common genetic factors might be involved in their susceptibility. To evaluate this, we performed two sets of experiments. An association study using a polymorphism within an intron of the SGCE gene was assessed in patients with TS and OCD versus controls, and the SGCE gene itself was screened for mutations in all TS/OCD patients, followed by direct sequencing of the gene in a limited number of these patients. No correlation was found by either method.
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Article Psychogenic hemifacial spasm. free! 2001
Tan EK, Jankovic J. · Parkinson's Disease Center and Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine, Houston, TX 77030, USA. · J Neuropsychiatry Clin Neurosci. · Pubmed #11514645 links to free full text
Abstract: Facial spasms that distort facial expression are typically due to facial dystonia, tics, and hemifacial spasm (HFS). Psychogenic facial spasms, however, have not been well characterized. The authors sought to 1) determine prevalence of psychogenic facial spasm in patients referred for evaluation of HFS and 2) draw attention to clinical characteristics and potential diagnostic pitfalls. Among 210 consecutive patients referred for evaluation of HFS, 5 (2.4%) received diagnoses of psychogenic facial spasm. All patients were female; mean age was 34.6 years (range 26-45) and mean symptom duration 1.1 years (range 2 wk-2 yr). Onset was left-sided in 3 patients, and the lid was the initial site affected in 2 patients. This series of patients shows that facial spasms, although usually of neurovascular etiology, may be the initial or only manifestation of a psychogenic movement disorder, often associated with an underlying depression.
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Article Bilineal transmission in Tourette syndrome. 1999
Hanna PA, Janjua FN, Contant CF, Jankovic J. · Parkinson's Disease Center and Movement Disorders Clinic, Baylor College of Medicine, Houston, TX 77030, USA. · Neurology. · Pubmed #10489047 No free full text.
Abstract: OBJECTIVE: To investigate the frequency and pattern of bilineal transmission in families of patients with Tourette syndrome (TS) compared with normal control subjects. METHODS: The study population consisted of two groups: 1) consecutive patients with TS with both parents (51 family sets; 153 individuals), and 2) normal control subjects randomly selected from public schools (20 family sets; 60 individuals). All patients with TS, normal control subjects, and their parents were evaluated for evidence of TS and associated features. Structured interviews and detailed questionnaires designed to assess tics, obsessive-compulsive behavior (OCB), and attention deficit disorder (ADD) were administered to all people in both groups. RESULTS: In addition to tics, 43 (84.3%) patients with TS had ADD, 33 (64.7%) had OCB, and 31 (60.8%) had both ADD and OCB. In 42 (82.4%) of the 51 patients, at least one parent exhibited features of TS; unilineal transmission (only one parent with tics, OCB, or ADD) was present in 29 (56.9%) TS families and an additional 13 (25.5%) TS families manifested evidence of bilineal transmission (both parents affected). More fathers than mothers of patients with TS had tics (31.4% versus 15.7%), whereas more mothers had OCB than did fathers (33.3% versus 15.7%). Features of ADD were equally distributed among fathers (35.3%) and mothers (33.3%) of patients with TS. Eight of 1,142 (0.7%) children in the general school population had some evidence of TS. One of 40 parents of the normal control subjects (2.5%) had symptoms of ADD, but none of the parents of normal control subjects manifested any features of TS or OCB. CONCLUSIONS: This study found evidence for bilineal transmission in one fourth of TS families. Features of the TS spectrum were rare in normal control families. Evidence of tics, OCB, and ADD should be investigated in both parents of patients with TS.
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Article Attitudes toward neurosurgical procedures for Parkinson's disease and obsessive-compulsive disorder. free! 1999
Mathew SJ, Yudofsky SC, McCullough LB, Teasdale TA, Jankovic J. · Department of Psychiatry and Behavioral Sciences, Parkinson's Disease Center, Houston, TX, USA. · J Neuropsychiatry Clin Neurosci. · Pubmed #10333998 links to free full text
Abstract: Similar neurosurgical procedures exist for Parkinson's disease (PD) and obsessive-compulsive disorder (OCD). Because PD is seen as a brain disease and OCD as a disease of the mind, neurologists and psychiatrists may be more aware of and more optimistic toward neurosurgery for PD than for OCD. A questionnaire was sent to randomized American Psychiatric Association and American Academy of Neurology members, and 569 of 1,188 eligible members (47.9%) responded. Some 82.8% of the psychiatrists and 27.4% of the neurologists were aware of neurosurgical procedures for OCD, whereas 84.7% of psychiatrists and 99.4% of neurologists were aware of neurosurgery for PD (P < 0.001). Of psychiatrists, 74.1% would refer appropriate patients for OCD neurosurgery, 67.4% for PD neurosurgery (P = 0.15); of neurologists, 25.6% would refer for OCD, 94.3% for PD (P < 0.001). Specialty affected willingness to refer for OCD neurosurgery. Specialty and degree of contact with neurosurgeons affected willingness to refer for PD neurosurgery. There is poor physician awareness of neurosurgical options for OCD compared with PD, as well as a risk-benefit bias against OCD surgery by the neurologists surveyed.
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