Neurologists
30 years of experience
Video profile
Accepting new patients
Washington Heights
Columbia University
710 W 168th St
New York, NY 10032
212-305-5548
Locations and availability (2)

Education ?

Medical School Score Rankings
University of Connecticut (1980)
  • Currently 3 of 4 apples
Top 50%

Awards & Distinctions ?

Appointments
New York-presbyterian Hospital
Columbia University College Of Physicians And Surgeons, Ny, Ny (1988 - Present)
Associations
American Board of Psychiatry and Neurology

Affiliations ?

Dr. Greene is affiliated with 10 hospitals.

Hospital Affilations

Score

Rankings

  • NewYork-Presbyterian / Weill Cornell
    525 E 68th St, New York, NY 10065
    • Currently 4 of 4 crosses
    Top 25%
  • New York Presbyterian Hospital / Columbia
    630 W 168th St, New York, NY 10032
    • Currently 4 of 4 crosses
    Top 25%
  • Harlem Hospital Center
    506 Malcolm X Blvd, New York, NY 10037
    • Currently 2 of 4 crosses
  • New York Presbyterian Hospital - Presby Campus
  • New York Presbyterian HospitalColumbia Presbyterian Center
  • New York Harbor Healthcare System
    423 E 23rd St, New York, NY 10010
  • New York Presbyterian / Westchester
    21 Bloomingdale Rd, White Plains, NY 10605
  • NewYork-PresbyterianColumbia
  • Presbyterian Hospital Acu
  • NewYork-Presbyterian/Columbia
  • Publications & Research

    Dr. Greene has contributed to 50 publications.
    Title The Pam-1 Aminopeptidase Regulates Centrosome Positioning to Ensure Anterior-posterior Axis Specification in One-cell C. Elegans Embryos.
    Date August 2010
    Journal Developmental Biology
    Excerpt

    In the one-cell Caenorhabditis elegans embryo, the anterior-posterior (A-P) axis is established when the sperm donated centrosome contacts the posterior cortex. While this contact appears to be essential for axis polarization, little is known about the mechanisms governing centrosome positioning during this process. pam-1 encodes a puromycin sensitive aminopeptidase that regulates centrosome positioning in the early embryo. Previously we showed that pam-1 mutants fail to polarize the A-P axis. Here we show that PAM-1 can be found in mature sperm and in cytoplasm throughout early embryogenesis where it concentrates around mitotic centrosomes and chromosomes. We provide further evidence that PAM-1 acts early in the polarization process by showing that PAR-1 and PAR-6 do not localize appropriately in pam-1 mutants. Additionally, we tested the hypothesis that PAM-1's role in polarity establishment is to ensure centrosome contact with the posterior cortex. We inactivated the microtubule motor dynein, DHC-1, in pam-1 mutants, in an attempt to prevent centrosome movement from the cortex and restore anterior-posterior polarity. When this was done, the aberrant centrosome movements of pam-1 mutants were not observed and anterior-posterior polarity was properly established, with proper localization of cortical and cytoplasmic determinants. We conclude that PAM-1's role in axis polarization is to prevent premature movement of the centrosome from the posterior cortex, ensuring proper axis establishment in the embryo.

    Title Myoclonus and Tremor Response to Thalamic Deep Brain Stimulation Parameters in a Patient with Inherited Myoclonus-dystonia Syndrome.
    Date April 2009
    Journal Clinical Neurology and Neurosurgery
    Excerpt

    We present a 74-year-old woman with inherited myoclonus-dystonia, with predominant myoclonus and a novel mutation in the epsilon-sarcoglycan gene. The patient reports a life-long history of rapid, jerking movements, most severe in the upper extremities as well as a postural and action tremor. Bilateral deep brain stimulation (DBS) of the ventral intermediate nucleus of the thalamus was performed, and the patient demonstrated moderate clinical improvement in myoclonus. We studied the effects on myoclonus and tremor of varying DBS frequency and amplitude. The frequency tuning curve for myoclonus was similar to that of tremor, suggesting similar mechanisms by which DBS alleviates both disorders.

    Title Motor Stereotypies in Children with Autism and Other Developmental Disorders.
    Date February 2009
    Journal Developmental Medicine and Child Neurology
    Excerpt

    The purpose of the study was to count and characterize the range of stereotypies--repetitive rhythmical, apparently purposeless movements--in developmentally impaired children with and without autism, and to determine whether some types are more prevalent and diagnostically useful in children with autism. We described each motor stereotypy recorded during 15 minutes of archived videos of standardized play sessions in 277 children (209 males, 68 females; mean age 4y 6mo [SD 1y 5mo], range 2y 11mo-8y 1mo), 129 with autistic disorder (DSM-III-R), and 148 cognitively-matched non-autistic developmentally disordered (NADD) comparison children divided into developmental language disorder and non-autism, low IQ (NALIQ) sub-groups. The parts of the body involved and characteristics of all stereotypies were scored blind to diagnosis. More children with autism had stereotypies than the NADD comparison children. Autism and, to a lesser degree, nonverbal IQ (NVIQ) <80, especially in females contributed independently to the occurrence, number, and variety of stereotypies, with non-autistic children without cognitive impairment having the least number of stereotypies and children with autism and low NVIQ the most. Autism contributed independently to gait and hand/finger stereotypies and NVIQ <80 to head/trunk stereotypies. Atypical gazing at fingers and objects was rare but virtually limited to autism. Stereotypies are environmentally modulated movement disorders, some highly suggestive, but not pathognomonic, of autism. Their underlying brain basis and genetic correlates need investigation.

    Title Tetrabenazine Therapy of Pediatric Hyperkinetic Movement Disorders.
    Date February 2007
    Journal Movement Disorders : Official Journal of the Movement Disorder Society
    Excerpt

    Tetrabenazine (TBZ), a presynaptic dopamine depletor and postsynaptic dopamine receptor blocker, is widely used for the treatment of hyperkinetic movement disorders in adults. However, reports of its use in children are limited. We review the efficacy and tolerability of TBZ therapy in 31 children with hyperkinetic movement disorders refractory to other medications. TBZ was effective in reducing the severity of movement disorders resistant to treatment with other medicines. When compared to adult patients, pediatric patients required higher doses. Side effects were similar to the adult population; however, children had a lower incidence of drug-induced Parkinsonism.

    Title Stiff Child Syndrome with Mutation of Dyt1 Gene.
    Date June 2006
    Journal Neurology
    Title Status of Fetal Tissue Transplantation for the Treatment of Advanced Parkinson Disease.
    Date June 2006
    Journal Neurosurgical Focus
    Excerpt

    In the first double-blind, placebo-controlled randomized study of fetal tissue transplantation for the treatment of patients with advanced Parkinson disease (PD), investigators found that implanted dopaminergic tissue can produce measurable improvement in young PD in the absence of medication (that is, the "off" state). The results of the study, however, also highlighted several serious limitations of transplantation. In the group of older patients in the study (in the typical age range of individuals afflicted with PD) no improvement was derived from the implant despite positron emission tomography-documented scan evidence that the graft survived and produced dopamine. Patients in the study were selected because they experienced motor fluctuations, and the transplant did not improve dyskinesias or the time required to remain "on" medication for any subgroup of patients, including young patients. Five of 33 implant-treated patients developed involuntary movements (dyskinesias or dystonia) that could not be eliminated by reducing antiparkinsonian medications. These included four patients with the best responses to transplantation. Finally, some sham-operated patients experienced a dramatic placebo effect lasting at least 1 year, which justified the controversial sham surgery. The authors believe that these problems must be solved before fetal tissue transplantation can be considered a therapeutic option for PD.

    Title A Single-blind, Open-label Trial of Sodium Oxybate for Myoclonus and Essential Tremor.
    Date April 2006
    Journal Neurology
    Excerpt

    The authors performed an open-label, rater-blinded, add-on study of sodium oxybate in 20 patients with ethanol-responsive myoclonus or essential tremor. Blinded ratings of videotaped examinations showed improvements in myoclonus at rest, stimulus-sensitive myoclonus, action myoclonus, functional performance, and postural and kinetic tremor. Tolerability was acceptable, and more than half of the patients chose to continue treatment after the trial. Double-blind placebo-controlled studies in these disorders are warranted.

    Title Reaction Time and Movement Time After Embryonic Cell Implantation in Parkinson Disease.
    Date July 2004
    Journal Archives of Neurology
    Excerpt

    BACKGROUND: Embryonic nigral cell implants are a novel treatment for Parkinson disease (PD). Reaction time (RT) and movement time (MT) analysis, validated quantitative measures of premovement neural processing and motor execution, can be used as objective physiological markers of motor performance in PD. OBJECTIVES: To gauge the change in motor performance in patients with PD who received implants, and to determine whether the physiological findings correlate with clinical outcome measures after transplantation. DESIGN: Double-blind, placebo-controlled trial.Patients Forty patients with levodopa-responsive, Hoehn and Yahr stage III or greater PD. INTERVENTIONS: Random assignment to embryonic tissue implants or placebo (sham) operation. MAIN OUTCOME MEASURES: Combined RT + MT scores measured preoperatively and at 4 and 12 months postoperatively in the "off" state. RESULTS: The difference in mean RT + MT scores between the sham and implant groups was statistically significant (P =.005) and was greatest in those 60 years or older (P =.003). Changes correlated with Unified Parkinson's Disease Rating Scale off scores at 4 (r = 0.87, P =.001) and 12 (r = 0.75, P =.01) months in those younger than 60 years. There was a significant deterioration in the sham surgery group at 12 months (P =.03) that was thought to be due to worsening in subjects 60 years and older (P<.001). CONCLUSIONS: The physiological measures detected significant changes in patients undergoing embryonic nigral cell implants and correlated directly with clinical outcome measures. Comprehensive analyses of RT paradigms can document subtle changes in motor performance over time, making them useful outcome measures in therapeutic trials of PD. These findings support further research into nigral cell implantation for PD.

    Title An Algorithm (decision Tree) for the Management of Parkinson's Disease (2001): Treatment Guidelines.
    Date March 2004
    Journal Neurology
    Title Extensor Digitorum Brevis Test and Resistance to Botulinum Toxin Type A.
    Date January 2003
    Journal Muscle & Nerve
    Excerpt

    We studied 22 patients with dystonia to determine the normal range of values for the extensor digitorum brevis (EDB) test, and to determine its sensitivity and specificity in detecting resistance to botulinum toxin type A (BTX-A). Three compound muscle action potentials (CMAPs) elicited by peroneal nerve stimulation were averaged before and 2 weeks after injection of 20 units of BTX-A into the EDB. Amplitude and area ratios were calculated by dividing the averaged postinjection CMAP by the averaged preinjection CMAP values. The difference in means of this ratio between clinically sensitive and resistant subjects was statistically significant (P < 0.002). A normal range of <0.45 for each ratio was determined by adding two standard deviations to the ratio mean of 14 clinically sensitive subjects. Four of five resistant patients had values outside the normal range. The EDB test is a simple quantitative method of detecting resistance to BTX-A, with a sensitivity of 80% and specificity of 94%.

    Title The Natural History of Embouchure Dystonia.
    Date February 2002
    Journal Movement Disorders : Official Journal of the Movement Disorder Society
    Excerpt

    Focal task-specific dystonias are unusual disorders of motor control, often affecting individuals who perform complex repetitive movements. Musicians are especially prone to develop these disorders because of their training regimens and intense practice schedules. Task-specific dystonia occurring in keyboard or string instrumentalists usually affects the hand. In contrast, there have been few descriptions of musicians with task-specific dystonia affecting the muscles of the face and jaw. We report detailed clinical observations of 26 professional brass and woodwind players afflicted with focal task-specific dystonia of the embouchure (the pattern of lip, jaw, and tongue muscles used to control the flow of air into a mouthpiece). This is the largest and most comprehensively studied series of such patients. Patients developed embouchure dystonia in the fourth decade, and initial symptoms were usually limited to one range of notes or style of playing. Once present, dystonia progressed without remission and responded poorly to oral medications and botulinum toxin injection. Patients with embouchure dystonia could be separated by the pattern of their abnormal movements into several groups, including embouchure tremor, involuntary lip movements, and jaw closure. Dystonia not infrequently spread to other oral tasks, often producing significant disability. Effective treatments are needed for this challenging and unusual disorder.

    Title Transplantation of Embryonic Dopamine Neurons for Severe Parkinson's Disease.
    Date March 2001
    Journal The New England Journal of Medicine
    Excerpt

    BACKGROUND: Transplantation of human embryonic dopamine neurons into the brains of patients with Parkinson's disease has proved beneficial in open clinical trials. However, whether this intervention would be more effective than sham surgery in a controlled trial is not known. METHODS: We randomly assigned 40 patients who were 34 to 75 years of age and had severe Parkinson's disease (mean duration, 14 years) to receive a transplant of nerve cells or sham surgery; all were to be followed in a double-blind manner for one year. In the transplant recipients, cultured mesencephalic tissue from four embryos was implanted into the putamen bilaterally. In the patients who received sham surgery, holes were drilled in the skull but the dura was not penetrated. The primary outcome was a subjective global rating of the change in the severity of disease, scored on a scale of -3.0 to 3.0 at one year, with negative scores indicating a worsening of symptoms and positive scores an improvement. RESULTS: The mean (+/-SD) scores on the global rating scale for improvement or deterioration at one year were 0.0+/-2.1 in the transplantation group and -0.4+/-1.7 in the sham-surgery group. Among younger patients (60 years old or younger), standardized tests of Parkinson's disease revealed significant improvement in the transplantation group as compared with the sham-surgery group when patients were tested in the morning before receiving medication (P=0.01 for scores on the Unified Parkinson's Disease Rating Scale; P=0.006 for the Schwab and England score). There was no significant improvement in older patients in the transplantation group. Fiber outgrowth from the transplanted neurons was detected in 17 of the 20 patients in the transplantation group, as indicated by an increase in 18F-fluorodopa uptake on positron-emission tomography or postmortem examination. After improvement in the first year, dystonia and dyskinesias recurred in 15 percent of the patients who received transplants, even after reduction or discontinuation of the dose of levodopa. CONCLUSIONS: Human embryonic dopamine-neuron transplants survive in patients with severe Parkinson's disease and result in some clinical benefit in younger but not in older patients.

    Title Sleep Episodes in Parkinson's Disease: a Wake-up Call.
    Date January 2001
    Journal Movement Disorders : Official Journal of the Movement Disorder Society
    Title Parkinsonism, Dystonia, and Hemiatrophy.
    Date October 2000
    Journal Movement Disorders : Official Journal of the Movement Disorder Society
    Excerpt

    Hemiatrophy has been reported in association with a variety of neurologic conditions, including parkinsonism. Patients with the hemiparkinson-hemiatrophy syndrome (HP-HA) have asymmetric parkinsonism with limb atrophy on the more affected side. Several authors have suggested that asymmetric brain damage early in life results in both atrophy and parkinsonism. Dopa-responsive dystonia (DRD) is a disease in which a deficiency of tetrahydrobiopterin, or, less commonly, of tyrosine hydroxylase, results in levodopa-responsive dystonia with parkinson features in children. We have recently identified four patients with DRD who had asymmetric dystonia and limb atrophy on the more affected side. Based on these patients, we suggest that a deficiency of the nigrostriatal dopamine system may, by itself, be sufficient to cause body atrophy and may underlie the limb atrophy in both DRD and HP-HA.

    Title Localization of a Gene for Myoclonus-dystonia to Chromosome 7q21-q31.
    Date November 1999
    Journal Annals of Neurology
    Excerpt

    Essential myoclonus-dystonia is a neurological condition characterized by myoclonic and dystonic muscle contractions and the absence of other neurological signs or laboratory abnormalities; it is often responsive to alcohol. The disorder may be familial with apparent autosomal dominant inheritance. We report a large kindred with essential familial myoclonus-dystonia and map a locus for the disorder to a 28-cM region of chromosome 7q21-q31.

    Title Falling Asleep at the Wheel: Motor Vehicle Mishaps in Persons Taking Pramipexole and Ropinirole.
    Date July 1999
    Journal Neurology
    Excerpt

    The authors report a new side effect of the dopamine agonists pramipexole and ropinirole: sudden irresistible attacks of sleep. Eight PD patients taking pramipexole and one taking ropinirole fell asleep while driving, causing accidents. Five experienced no warning before falling asleep. The attacks ceased when the drugs were stopped. Neurologists who prescribe these drugs and patients who take them should be aware of this possible side effect.

    Title The Role of the Dyt1 Gene in Secondary Dystonia.
    Date November 1998
    Journal Advances in Neurology
    Title Intrathecal Baclofen in the Treatment of Dystonia.
    Date November 1998
    Journal Advances in Neurology
    Title Exteroceptive and Interoceptive Stimuli in Dystonia.
    Date August 1998
    Journal Movement Disorders : Official Journal of the Movement Disorder Society
    Excerpt

    Some patients with torsion dystonia experience a dramatic increase or decrease in symptoms when performing specific activities. The activities that influence dystonic symptoms vary from person to person. An activity or sensory stimulus that reduces symptoms has been called a "sensory trick" or, in cervical dystonia, a "geste antagoniste." When a single activity induces symptoms of dystonia, the dystonia is called "task specific." We have discovered that in some patients, thinking about a sensory trick or task affects the dystonia in the same way as actually performing the activity. We present three representative patients, and discuss the relevance of this observation to the understanding of dystonia.

    Title Clinical Presentation and Pharmacological Therapy in Corticobasal Degeneration.
    Date August 1998
    Journal Archives of Neurology
    Excerpt

    BACKGROUND: To date, to our knowledge, there is no systematic presentation of treatment outcome in large series of patients clinically diagnosed as having corticobasal degeneration. OBJECTIVE: To evaluate the clinical presentation and treatment outcome of patients clinically diagnosed as having corticobasal degeneration. SUBJECTS: We gathered case patients seen in 8 major movement disorder clinics during the last 5 years who were diagnosed as having corticobasal ganglionic degeneration. METHODS: Using a chart review method, we recorded the clinical presentation, medications used, response to medications, and adverse effects. RESULTS: A total of 147 case patients were reviewed, 7 were autopsy proven. Parkinsonian features were present in all, other movement disorders in 89%, and higher cortical dysfunction in 93%. The most common parkinsonian sign was rigidity (92%), followed by bradykinesia (80%), gait disorder (80%), and tremor (55%). Other movement disorders were dystonia in 71% and myoclonus in 55%. Higher cortical dysfunction included dyspraxia (82%), alien limb (42%), cortical sensory loss (33%), and dementia (25%). Ninety-two percent of the case patients received dopaminergic drugs, which resulted in a beneficial effect for 24%. Parkinsonian signs were the elements improving the most and levodopa was the most effective drug. Benzodiazepines, primarily clonazepam, were administered to 47 case patients, which resulted in improvement of myoclonus in 23% and dystonia in 9%. The most frequent disabling adverse effects of drug trials in these case patients were somnolence (n = 24), gastrointestinal complaints (n = 23), confusion (n = 16), dizziness (n =12), hallucinations (n = 5), and dry mouth (n = 5). CONCLUSIONS: Pharmacological intervention was largely ineffective in the management of corticobasal degeneration, and new treatments are needed for ameliorating the symptoms of this syndrome.

    Title Barriers to Research Utilization for Oncology Staff Nurses and Nurse Managers/clinical Nurse Specialists.
    Date July 1998
    Journal Oncology Nursing Forum
    Excerpt

    To describe and compare barriers to research utilization faced by oncology staff nurses and nurse managers/clinical nurse specialists (CNSs) and to compare these to barriers identified by other nurses.

    Title Idiopathic Torsion Dystonia Linked to Chromosome 8 in Two Mennonite Families.
    Date November 1997
    Journal Annals of Neurology
    Excerpt

    The DYT1 locus on chromosome 9q34 is responsible for most childhood limb-onset idiopathic torsion dystonia (ITD). Linkage to DYT1 has been excluded in families with adult-onset, and predominantly cranial-cervical, ITD. We mapped a locus (DYT6) associated with prominent cranial-cervical ITD in two large Mennonite families to chromosome 8. An identical haplotype spanning 40-cM segregates with ITD in these families, suggesting a shared mutation from the recent past.

    Title Secondary Dystonia and the Dyti Gene.
    Date July 1997
    Journal Neurology
    Excerpt

    Early-onset (< 28 years) primary dystonia in most Ashkenazi Jews is due to a single founder mutation in the DYT1 gene on chromosome 9q34, as determined by very strong linkage disequilibrium with a haplotype of 9q34 alleles at surrounding marker loci. The role of this mutation in individuals with secondary causes for dystonia has never been tested, although environmental insults, such as neuroleptic exposure or perinatal asphyxia, are proposed to precipitate dystonia in genetically predisposed individuals. We assessed 9q34 haplotypes in 40 Ashkenazi patients with secondary dystonia; 25 had early onset of symptoms, including 15 with exposure to neuroleptic medication or perinatal asphyxia. Of the 25 patients with early onset, 9 were considered phenocopies of DYT1 having normal examinations except for dystonia, normal radiographic and other laboratory studies, and onset in a limb or the neck. Only one individual whose dystonia developed in the setting of a measles infection carried the associated haplotype. Our findings indicate that clinical diagnostic criteria that include historical information to detect tardive dystonia and perinatal asphyxia discriminate primary dystonia due to the DYT1 founder mutation. We found no evidence that the DYT1 founder mutation contributes to secondary dystonia.

    Title Exclusion of the Dyt1 Locus in Familial Torticollis.
    Date November 1996
    Journal Annals of Neurology
    Excerpt

    Clinical-genetic studies of idiopathic torsion dystonia (ITD) indicate that the DYT1 gene on chromosome 9q34 is responsible for most childhood limb-onset disease. The genetic basis of adult-onset ITD is less well studied. In most multiplex adult-onset ITD families, dystonia is limited to the cervical, cranial, or brachial muscles; in a few rare families, dystonia also involves the legs and trunk. Previous linkage studies have excluded the DYT1 locus in these atypical families. We studied two large non-Jewish families with adult-onset ITD limited to the cervical and brachial muscles and excluded the DYT1-containing region. This study further restricts the role of DYT1 to childhood limb-onset ITD and suggests that other genes are responsible for focal adult-onset ITD.

    Title Response to Botulinum Toxin F in Seronegative Botulinum Toxin A--resistant Patients.
    Date August 1996
    Journal Movement Disorders : Official Journal of the Movement Disorder Society
    Excerpt

    Botulinum toxin type A (btx A) injections are the most effective treatment for most patients with focal dystonia. Some patients who improve after btx A injections and later lose response have serologic evidence of antibodies to btx A with the mouse neutralization assay (seropositive patients). Another group of patients who lose response to btx A do not have detectable antibodies (seronegative patients). Seropositive patients may improve after injections of botulinum toxin type F (btx F), an alternative serotype of botulinum toxin. We treated nine seronegative resistant patients with btx F. None of these patients had muscle atrophy after injection with btx A. Five of the nine had improvement after btx F injection that was sustained for at least three consecutive btx F injections. This observation is consistent with the hypothesis that btx resistance in seronegative patients is caused by undetected antibodies to btx A. If this be the case, then there may be techniques for preventing or reversing btx resistance, as in the case of resistance of factor VIII in the treatment of hemophilia A.

    Title Periodontal Management of a Prominent Labial Frenum in an Adolescent with Gingival Overgrowth.
    Date April 1995
    Journal Pediatric Dentistry
    Title Dystonia in Ashkenazi Jews: Clinical Characterization of a Founder Mutation.
    Date December 1994
    Journal Annals of Neurology
    Excerpt

    A gene (DYT1) for idiopathic torsion dystonia maps to chromosome 9q34 in Ashkenazi Jewish families with early onset of symptoms. Further, there is linkage disequilibrium between DYT1 and a particular haplotype of alleles at 9q34 loci in this population. This implies that a large proportion of early-onset idiopathic torsion dystonia in Ashkenazi Jews is due to a founder mutation in DYT1. To characterize the phenotypic range of this mutation, we studied 174 Ashkenazi Jewish individuals affected with idiopathic torsion dystonia. We used GT(n) markers on chromosome 9q34 (D9S62, D9S63, and ASS) and classified individuals as having ("carriers"), not having ("noncarriers"), or being ambiguous with respect to a DYT1-associated haplotype. We assessed clinical features and found marked clinical differences between haplotype carriers and noncarriers. There were 90 carriers, 70 noncarriers, and 14 ambiguous individuals. The mean age at onset of symptoms was significantly lower in carriers than in noncarriers (12.5 +/- 8.2 vs 36.5 +/- 16.4 years). In 94% of carriers, symptoms began in a limb (arm or leg equally); rarely the disorder started in the neck (3.3%) or larynx (2.2%). In contrast, the neck, larynx, and other cranial muscles were the sites of onset in 79% of noncarriers; onset in the arms occurred in 21% and onset in the legs never occurred. Limb onset, leg involvement in the course of disease, and age at onset distinguished haplotype carriers from noncarriers with 90% accuracy. In conclusion, there are clinical differences between Ashkenazi Jewish individuals with idiopathic torsion dystonia who do or do not have a unique DYT1 mutation, as determined by a DYT1-associated haplotype of 9q34 alleles.(ABSTRACT TRUNCATED AT 250 WORDS)

    Title A Comparison of Oral Health Status and Need for Dental Care Between Abused/neglected Children and Nonabused/non-neglected Children.
    Date July 1994
    Journal Pediatric Dentistry
    Excerpt

    This paper compares oral health status and presence of untreated, decayed permanent teeth in abused/neglected children with nonabused/non-neglected controls. The sample comprised 903 children between 5 and 13 years old; 30 were confirmed cases of child abuse and 873 served as controls. Their oral health status was assessed by two calibrated dentists using the DMFS index. Presence of untreated, decayed teeth was determined from the decayed and unfilled component of the DMFS score. The data were analyzed using logistic regression so that the influence of other explanatory variables (sociodemographic characteristics) on oral health status and presence of untreated, decayed teeth could be controlled while the influence of abuse status was evaluated. Results show that abuse status is an important explanatory variable for both oral health status and presence of untreated, decayed teeth. While the impact of abuse status on oral health status is obscured by interactions with other explanatory variables, its impact on the presence of untreated, decayed teeth is clear. Abused children are eight times more likely to have untreated, decayed permanent teeth than nonabused children. Accordingly, it is recommended that confirmed cases of child abuse/neglect should be referred routinely for dental screening as part of their overall rehabilitation.

    Title Use of Botulinum Toxin Type F Injections to Treat Torticollis in Patients with Immunity to Botulinum Toxin Type A.
    Date December 1993
    Journal Movement Disorders : Official Journal of the Movement Disorder Society
    Excerpt

    Fifteen patients with torticollis who had been treated with repeated injections of botulinum toxin type A (botox A) developed antibodies to the toxin. This resulted in loss of benefit in the 13 patients who had improved with botox A injections and failure to develop muscle atrophy after injection in all 15 patients. Patients were then injected with botulinum toxin type F (botox F) in the same muscles that had been injected with botox A. Ten of the 15 improved after botox F injections, including 9 of the 12 patients who had improved with type A toxin. Six of 9 patients with pain had improvement in pain after botox F injections. Patients reported similar improvement with type F and type A toxins, but duration of benefit was approximately 3 months with type A and approximately 1 month with type F. Botox F is an effective treatment for torticollis in patients who are immune to botox A. The usefulness of type F toxin, however, is limited by short duration of benefit.

    Title American Responds to Cancer Pain: a Survey of State Pain Initiatives.
    Date August 1993
    Journal Cancer Practice
    Excerpt

    Though pharmacologic and technologic advances make it possible to control most cancer pain, it often goes untreated or undertreated, largely because of lack of knowledge or misguided attitudes about appropriate use of such measures. In response to the need to improve education of health professionals and patients about cancer pain management, the state of Wisconsin created a state cancer pain initiative. This program, now fully developed, has made substantial progress toward its goals and has become a demonstration project for the World Health Organization (WHO). This report describes the Wisconsin Cancer Pain Initiative (WCPI), its impact on the development of initiatives in other states, and outlines the findings of a 1992 American Cancer Society (ACS) survey of 26 similar initiatives, many of which are modeled after the WCPI.

    Title Baclofen in the Treatment of Idiopathic Dystonia in Children.
    Date May 1992
    Journal Movement Disorders : Official Journal of the Movement Disorder Society
    Excerpt

    Baclofen benefits some patients with adult onset dystonia, but few reports document the response to baclofen of children with idiopathic dystonia. Sixteen of 80 patients less than age 21 years with idiopathic dystonia seen by the Movement Disorder Group at Columbia-Presbyterian Medical Center in New York were treated with baclofen. Five had substantial improvement in symptoms, two had moderate improvement, and nine failed to benefit. Three of the improved patients had transient improvement on high dose anticholinergics, but had sustained, dramatic improvement when baclofen was added. At last follow-up, five patients maintained improvement for a mean 3.8 years (19 months-8 years) on a mean 79 mg of baclofen (40-120 mg). Response to baclofen did not correlate with age at onset of dystonia or age at onset of therapy, but did correlate with duration of symptoms before therapy (3 years for those who improved vs 7.8 years for those who did not: p less than .002 by t-test). Baclofen can be an effective treatment for childhood dystonia.

    Title Distant Effects of Locally Injected Botulinum Toxin: a Double-blind Study of Single Fiber Emg Changes.
    Date November 1991
    Journal Muscle & Nerve
    Excerpt

    We used single fiber electromyography (SFEMG) to study 42 patients who had enrolled in a double-blind, placebo-controlled trial undertaken to assess the efficacy of botulinum toxin (BTX) injection of neck muscles to treat torticollis. SFEMG in a limb muscle was performed before treatment, 2, and 12 weeks after injection of placebo or BTX. Before treatment, the mean jitter was 26.8 microsec in patients who were to receive BTX, and 25.7 microsec in the placebo group. Two weeks after injection, mean jitter in the group receiving BTX was 43.6 microsec. In the placebo group, it was 26.5 microsec (P = less than .05). Twelve weeks after injection, mean jitter in the BTX group was 35.5; for the placebo group it was 24.5. Fiber density did not change in any patient during the study. There were no remote clinical effects of BTX. Injection of BTX into muscles affected with focal dystonia is a promising and safe treatment, but there are subclinical effects on uninjected muscles.

    Title Connecting: a Catalyst for Caring.
    Date July 1991
    Journal Nln Publications
    Title What is It? Case 1, 1990: Progressive Unilateral Rigidity, Bradykinesia, Tremulousness, and Apraxia, Leading to Fixed Postural Deformity of the Involved Limb.
    Date January 1991
    Journal Movement Disorders : Official Journal of the Movement Disorder Society
    Title Treatment of Hyperkinetic Movement Disorders.
    Date May 1990
    Journal Neurologic Clinics
    Excerpt

    Movement disorders are subdivided based on a variety of criteria. One useful and popular approach to movement disorders, based on clinical phenomenology, categorizes these disorders into two groups, those displaying a poverty of movement (akinesia) and those displaying excessive movement (hyperkinesia). This article discusses diagnosis and treatment of the latter. By necessity, certain hyperkinesias such as hyperexplexia, akathisia, and restless leg syndrome are omitted or only briefly discussed. The major hyperkinesias, dystonia, tremor, tics, chorea (including tardive dyskinesia and ballism), and myoclonus are reviewed and a guide to practical management emphasizing symptomatic treatment is presented.

    Title Segregation Analysis of Idiopathic Torsion Dystonia in Ashkenazi Jews Suggests Autosomal Dominant Inheritance.
    Date April 1990
    Journal American Journal of Human Genetics
    Excerpt

    The results of segregation analysis applied to a family study of idiopathic torsion dystonia in Ashkenazi Jews are reported. The study is based on 43 probands (with age at onset prior to 27 years) from 42 nuclear families; pedigrees were extended systematically through all available first- and second-degree relatives, who were directly examined and videotaped. Final diagnoses were based on exam information and blinded videotape review. Segregation analysis demonstrated that the data are consistent with autosomal dominant inheritance with 30% penetrance. Recessive and polygenic inheritance were strongly rejected. There was no evidence for sporadic cases or new mutations. The high incidence and dominant inheritance of early-onset idiopathic torsion dystonia in Ashkenazi Jews suggests genetic homogeneity within this population, making it especially useful for linkage studies of this disorder.

    Title Idiopathic Dystonia Among Ashkenazi Jews: Evidence for Autosomal Dominant Inheritance.
    Date December 1989
    Journal Annals of Neurology
    Excerpt

    We studied families to clarify the mode of inheritance of idiopathic torsion dystonia among the Ashkenazim. Probands had symptoms before 28 years of age, had at least one Ashkenazi grandparent, and were ascertained independently of family history and not referred by another relative. All available first- and second-degree relatives were examined, and videotapes were made. Examination notes and blinded review of videotapes led to rating of dystonia as definite, probable, possible, or absent. We determined rates of illness for first- and second-degree relatives and calculated age-adjusted lifetime risks. The methods of maximum likelihood and likelihood ratio goodness-of-fit tests were used to estimate parameters and to test dominant and recessive models of inheritance. We studied 43 probands, 146 (90.1%) of 162 living first-degree relatives, and 96 (40.2%) of 239 living second-degree relatives. Nineteen relatives had definite dystonia, and 2 had probable dystonia. Using definite cases only, the age-adjusted risk for all first-degree relatives was 15.5% and for all second-degree relatives 6.5%, with no significant sex differences; parent, offspring, and sibling risks did not differ significantly. The risks were consistent with autosomal dominant inheritance with a penetrance estimated at 29.4% using definite cases only or 32.2% using definite and probable cases. Assuming a disease frequency of 1/15,000, the gene frequency was estimated to be 1/9000.

    Title Botulinum Toxin Injection for the Treatment of Oromandibular Dystonia.
    Date March 1989
    Journal The Annals of Otology, Rhinology, and Laryngology
    Excerpt

    Dystonia is a neurologic disorder characterized by abnormal, involuntary movements causing twisting and turning postures; it is postulated to be a disorder of central motor processing. The dystonias, when classified by region of the body involved, have been characterized as focal, segmental, and generalized. Focal dystonia can affect jaw mechanics, leading to forceful contraction of the jaw muscles and resulting in inappropriate deviation of the jaw. Localized injections of botulinum toxin have been used successfully in the management of other focal or segmental dystonias. We have treated 20 oromandibular dystonia patients with botulinum toxin. Six patients had only jaw and tongue involvement; 11 had blepharospasm and jaw involvement; and three had jaw involvement as part of a more generalized dystonia. Five patients had been diagnosed originally and treated as having temporomandibular joint syndrome. All but one of the patients had improvement of their symptoms with the toxin injections. The patients averaged 47% improvement with the injections.

    Title Localized Injections of Botulinum Toxin for the Treatment of Focal Dystonia and Hemifacial Spasm.
    Date January 1989
    Journal Movement Disorders : Official Journal of the Movement Disorder Society
    Excerpt

    Medical treatment of dystonia usually results in an incomplete response and is frequently unsuccessful. Peripheral surgical therapy is available for some focal dystonias, but may only offer temporary relief and may have unacceptable complications. We have used local injections of botulinum toxin into the appropriate muscles for treatment of disabling focal or segmental dystonia in 93 patients with torticollis, blepharospasm, oromandibular dystonia (OMD), limb dystonia, lingual dystonia, and dystonia adductor dysphonia, in addition to four patients with hemifacial spasm. Significant relief of motor symptoms was seen in 69% of the patients with blepharospasm and 64% of patients with torticollis; 74% of the latter group with pain experience relief. Relief of symptoms was noted in most patients with OMD and limb dystonia, and all with lingual dystonia, dystonic adductor spastic dysphonia, and those with hemifacial spasm. Benefit averaged 2 1/2-3 months initially; however some patients experienced longer relief with subsequent injections. Adverse effects were transient, although 2 patients developed antibodies against the toxin, and we documented evidence for distant effects in others. This approach of chemically weakening contracting muscles in focal dystonia offers many advantages over pharmacotherapy and surgical therapy. Additional experience is needed to explore the proper doses, and potential for long term adverse effects.

    Title Inheritance of Idiopathic Torsion Dystonia Among Ashkenazi Jews.
    Date August 1988
    Journal Advances in Neurology
    Excerpt

    The mechanism(s) of inheritance of primary dystonia are unclear. An autosomal recessive form among Ashkenazi Jews and an autosomal dominant form among non-Jews have been proposed. However, the patterns of inheritance, particularly among Ashkenazim, are controversial. In this report we have reviewed the literature particularly as it pertains to the mode of inheritance among Ashkenazim. We also report the results of a pilot study of the families of 25 independently ascertained Ashkenazi probands with onset of primary dystonia before age 27 years. A total of 91/98 living first-degree relatives were examined; of these 91, 86 were greater than or equal to 8 years of age at time of examination and were included in our analysis. Overall, 14/86 (16.3%) of first-degree relatives were affected. We found 11.4% (4/35) of parents, 22.2% (8/36) of siblings, and 13.3% (2/15) of offspring were definitely affected. This finding of an approximately equal risk to parents, siblings, and offspring is consistent with autosomal dominant transmission with a minimum penetrance of 32.6%. Our findings do not support autosomal recessive or multifactorial inheritance.

    Title Localized Injections of Botulinum Toxin for the Treatment of Focal Dystonia and Hemifacial Spasm.
    Date August 1988
    Journal Advances in Neurology
    Title The Role of the American Cancer Society in Cancer Public Education.
    Date September 1986
    Journal Seminars in Oncology Nursing
    Title The Association of Pediatric Oncology Nurses: the First Ten Years.
    Date March 1985
    Journal Oncology Nursing Forum
    Title The Pivotal Role of the Nurse in Hospice Care.
    Date September 1984
    Journal Ca: a Cancer Journal for Clinicians
    Title The Association of Pediatric Oncology Nurses: the First Ten Years.
    Date December 1983
    Journal Oncology Nursing Forum
    Title Injury in the Dental Laboratory.
    Date October 1983
    Journal Quintessence of Dental Technology
    Title Use of a Complete Denture As a Radiation Carrier.
    Date September 1983
    Journal The Journal of Prosthetic Dentistry
    Excerpt

    When surgical resection of intraoral neoplasms is contraindicated, treatment by surface mold irradiation may be used. Success or failure of this mode of treatment may depend on the fabrication of a surface mold that can deliver the prescribed amount of therapeutic radiation in the desired amount of time. A technique for modification of an existing complete denture prosthesis for use as a surface mold has been described. Patient cooperation is essential for successful use of this modified prosthesis.

    Title The Swinglock Removable Partial Denture--a Viable Alternative.
    Date February 1983
    Journal Mississippi Dental Association Journal
    Title Nursing Care in Childhood Cancer: Late Effects of Therapy.
    Date May 1982
    Journal The American Journal of Nursing
    Title Dystonia.
    Date
    Journal Current Treatment Options in Neurology
    Excerpt

    Therapy for most people with dystonia is symptomatic, directed at lessening the intensity of the dystonic contractions. For a small minority of patients (eg, those with dopa-responsive dystonia, Wilson's disease, or psychogenic dystonia), specific therapy directed at one of the many causes of dystonia is available. Before initiating treatment, clinicians need to decide if a patient has a form of dystonia amenable to such therapy. The most sensitive and least costly method to diagnose DRD is a therapeutic trial of levodopa. It is, therefore, recommended to treat all those with dystonia beginning in childhood or adolescence with low-dose levodopa. For patients with generalized or segmental signs who do not respond to levodopa, other oral medications, including anticholinergics, baclofen, and benzodiazepines, may provide mild to moderate relief; these medications are often given in combinations. For those with focal dystonia, most having adult-onset disease, botulinum toxin A injections often effectively control contractions. The injections produce transient weakness and need to be repeated, generally every 3 to 5 months. There is growing renewed interest in surgical treatment. Peripheral denervating procedures may be helpful for patients with torticollis who do not obtain adequate benefit with botulinum toxin A. The central procedures of pallidotomy and pallidal stimulation are under study; their place in the treatment of the many dystonia subtypes (eg, limb vs axial, generalized vs focal, primary vs secondary) still needs to be established. There are very few studies evaluating physical and psychological therapies or the impact of diet or lifestyle in dystonia. Most clinicians consider physical therapy, including massage, a potential adjunct to medical therapy, and psychological support and stress reduction may help individuals cope with this chronic and frequently disabling condition.


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