CURRICULUM VITAE

MICHAEL R. PRANZATELLI, M.D.

 

PERSONAL INFORMATION

     Office Address:     SIU School of Medicine
                                    Department of Neurology
                                    P.O. Box 19643
                                    Springfield, IL 62794-9643
                                    E-mail: mpranzatelli@siumed.edu                                        
                                    (217) 545-7635
                                    Fax: (217) 545-1903
    
     Citizenship:           U.S.A.

 

LICENSURE

     IL Medical License:                      No.: 036-101666
     IL Controlled Substances:            No.: 336-062052
     DEA Registration:                        No.: BP0270962

 

BOARD CERTIFICATION

     1977                       National Board of Medical Examiners; Certificate #: 169517
     1981                       American Board of Pediatrics; Certificate #: 27023
     1987                       American Board of Psychiatry and Neurology with
                                    Special Qualification in Child Neurology; Certificate #: 507

EDUCATION

Undergraduate and Medical:

     1964-68                  North Hills High School; Pittsburgh, PA
     1968-72                  Duquesne University; Pittsburgh, PA; Degree: B.S.;
                                    Majors: Biology/English
     1972-76                  Pennsylvania State University; Hershey, PA
                                    Degree: M.D.

Residency Training:

     1976-77                  Intern, Case Western Reserve University and Affiliate
                                    Hospitals, Cleveland, OH
     1977-79                  Resident, Pediatrics, Case Western Reserve University                                                                                                                                       and Affiliated Hospitals; Cleveland, OH
                                    Dr. Richard E. Behrman, Chairman
     1979-82                  Resident, Pediatric Neurology, University of Colorado,
                                    Denver, CO; Dr. David A. Stumpf, Chairman

Research Fellowships:

     1981-82                  Public Health Service, Post-Doctoral Trainee,
                                    National Research Service Award Institutional Grant (T32);
                                    Pediatric Stolinsky Laboratory; Dr. Donough O'Brien, Director
     1982-85                  Research Fellow in Neuropharmacology, Neurology
                                    Research Laboratory; Children's Hospital of Los Angeles,
                                    University of Southern California, Los Angeles, CA;
                                    Dr. S. Robert Snodgrass, Director

Additional Postgraduate Coursework:

     1983                       Neurochemistry; University of California at Los Angeles
                                    Los Angeles, CA
     1983                       Computerized Statistical Programs; University of Southern California
                                    Los Angeles, CA
     1994                       NIH Foundation for Advanced Education in the Sciences (FAES),
                                    BIO-TRAC 7
                                    "Basic Cell and Tissue Culture" (2 credits, lecture and laboratory)
     1994                       NIH  (FAES) BIO-TRAC 2
                                    "Recombinant DNA Technologies" (2 credits, lecture and laboratory)
     1995                       NIH  (FAES) BIO-TRAC 5
                                    "Separation Techniques" (2 credits, lecture and laboratory)

PROFESSIONAL EXPERIENCE

Faculty Appointments:

     1985-86                  Assistant Professor of Neurology
                                    College of Physicians and Surgeons, Columbia University
     1986-90                  Assistant Professor of Neurology and Pediatrics
                                    College of Physicians and Surgeons, Columbia University
     1990-95                  Associate Professor of Neurology, Pediatrics and
                                    Pharmacology, The George Washington University

 

     1995-97                  Associate Professor with Tenure,

22.    Pranzatelli MR. "Neuropharmacology of myoclonus." Tristate Child Neurology Society, New York, NY, 1988.

23.    Pranzatelli MR. "Serotonin in movement disorders." Columbia University, Movement
                  Disorder Group Lecture Series, New York, NY, 1985.

24.    Pranzatelli MR. "New approaches to myoclonus." Stanford University, Neurology Grand Rounds, Pale Alto, CA, 1988.

25.    Pranzatelli MR. "Pharmacologic advances in myoclonus." North Shore University Hospital/Cornell, Neurology Grand Rounds, New York, NY, 1990.

26.    Pranzatelli MR. "Serotonin receptors in childhood neural crest tumors: Implications for opsoclonus-myoclonus syndrome." Jefferson University, Neurology Grand Rounds, Philadelphia, PA, 1990.

27.   Pranzatelli MR. "Neurologic consequences of brainstem receptor plasticity." Yale University, Neurology Grand Rounds, New Haven, CT, 1990.

28.   Pranzatelli MR. "Pharmacologic basis and need for molecular genetic studies of serotonin receptors in progressive myoclonus epilepsies." The National Institutes of Health, NIAAA, Bethesda, MD, 1990.

29.    Pranzatelli MR. "Plasticity of brainstem serotonin receptors." The George Washington University, Pharmacology Department of Seminar, Washington, DC, 1990.

30.   Pranzatelli MR. "Pediatric movement disorders." Clinical Child Neurology Update, Neurology Department Course, Children's National Medical Center, 1990.

31.   Pranzatelli MR. "Advances in the treatment of myoclonus." The George Washington University, Neurology Grand Rounds, Washington, DC, 1990.

32.   Pranzatelli MR. "Neuropharmacology of myoclonus." The George Washington University, Nuclear Medicine Research Conference, Washington, DC, 1990.

33.   Pranzatelli MR. "Therapeutic implications of the serotonin hypothesis of myoclonus." The Johns Hopkins University, Neurology Grand Rounds, Baltimore, MD, 1991.

59.  Pranzatelli MR. “The serotonin revolution: New prospects for movement disorders.” SIU Neurology/Neurosurgery Grand Rounds, February 15, 2000.

60.  Pranzatelli, MR. “Pediatric movement disorders.” SIU Center for Family Medicine, Springfield, IL, February 16, 2000.

61.  Pranzatelli, MR. “Serotonin receptor plasticity in developing brain.” Sangamon Chapter of Society for Neuroscience, Springfield, IL, March 7, 2000.

62.  Pranzatelli MR. “PANDAS—fact or fiction.” 18th Annual Neurology Symposium. Neurologic Problems in Medicine. Springfield, IL, March 24, 2000.

63.  Pranzatelli, MR. “A case of the wobbles: Pediatric ataxia.” 1st Annual Pediatric Neurosciences Symposium. SIU School of Medicine, September 29, 2000.

64.  Pranzatelli, MR. “Strep in the headlines: PANDAS or Pandora syndrome?” 1st Annual Pediatric Neurosciences Symposium, SIU School of Medicine, September 29, 2000.

65.  Pranzatelli, MR. “Serotonin receptor plasticity in developing brain.” SIU Department of Physiology Seminar, Carbondale, IL, November 15, 2000.

66.  Pranzatelli, MR. “The formidable blood-brain barrier: Problems, strategies, and innovations.” Neurology Neuroscience Conference, Springfield, IL,  February 20, 2001.

67.  Pranzatelli, MR. “Those ‘spells’ that aren’t seizures.” 1st Annual Pediatric Neurosciences Symposium. SIU School of Medicine, September 29, 2000.

68.  Pranzatelli, MR. “Big heads, little heads.” Pediatrics Symposium, SIU School of Medicine, Springfield, IL, March 16, 2001.

69.  Pranzatelli, MR. “The pediatrician’s dilemma: PANDAS syndrome.” Pediatric Grand Rounds, St. Francis Medical Center, Peoria, IL, April 5, 2001.  

70.  Pranzatelli, MR. “CNS plasticity of serotonergic systems: Behavioral, neurochemical, and receptor correlates.” Neuroscience Seminar, University of Illinois College of Medicine, Peoria, IL, April 5, 2001.  

71.  Pranzatelli, MR. “The progressive myoclonus epilepsies: A frontier of neurobiology.” Clinical Conference, SIU School of Medicine, Springfield, IL, April 10, 2001.

72.  Pranzatelli, MR. “PANDAS syndrome: A breakthrough or streptomania?” 12th Annual Developmental-Behavioral Pediatrics Spring Conference, University of Illinois at Springfield, April 26, 2001. 

73.  Pranzatelli, MR. “Progressive myoclonus epilepsy: Genes, mice, drugs, and clinical tips.” Neurology Grand Rounds, University of Illinois College of Medicine, Peoria, IL, May 18, 2001.

74.  Pranzatelli, MR. “What’s special about movement disorders in kids?” Rehabilitation
Institute of Chicago, Chicago, IL, July 25, 2001.
 
75. Pranzatelli, MR. “Seeing the forest through the trees: Movement disorders in cerebral palsy.” Symposium on the Team Management of Cerebral Palsy, SIU School of Medicine, Springfield, IL, November 1, 2001.

76. Pranzatelli, MR. “CSF leukocytes immunophenotyping as a neurologist’s tool: Experience from a paraneoplastic disorder.” SIU School of Medicine, Neurology Grand Rounds, February 26, 2002.

77. Pranzatelli, MR. “Pediatric Neurology Workshop,” 20th Annual Neurology Symposium, SIU School of Medicine, March 15, 2002.

78. Pranzatelli, MR. “An unrecognized medical emergency–Paraneoplastic syndromes in childhood.” SIU School of Medicine, Pediatric Grand Rounds, March 28, 2002.

79.  Pranzatelli, MR.  “Immunological studies in children with neuroblastoma and a  paraneoplastic syndrome.”  St. John’s Hospital, School of Clinical Laboratory Sciences, April 4, 2002. 

80. Pranzatelli, MR. “Short cases in child neurology.”  Medicine for Today, Illinois Academy of Family Physicians, Springfield, IL, April 11, 2002. 

81. Pranzatelli, MR. “Immunophenotyping by flow cytometry.”  Research lecture, Department of Neurology, SIU School of Medicine, July 30, 2002.

82.  Pranzatelli, MR. Evaluation of the child in coma. 21st Annual Neurology Symposium, SIU School of Medicine, March 21, 2003.

83.  Pranzatelli, MR. “Novel immunologic markers in pediatric opsoclonus-myoclonus.” Pediatric Grand Rounds, Memorial Sloan-Kettering Cancer Center, New York City, June 5, 2003.

84.  Pranzatelli, MR. “Management of seizures.” Advances in Pediatric Neonatal Critical Care. Prairie Heart Institute, October 3, 2003.

85.  Pranzatelli, MR. “Expanding the boundaries of developmental delay.” Neurology/ Neurosurgery Grand Rounds. SIU School of Medicine, September 16, 2003.

86.  Pranzatelli, MR. “Movement disorders.” 3rd Annual Neuromotor Symposium - Cerebral Palsy: the Goal is Adulthood. Prairie Heart Institute, October 30, 2003.

87.  Pranzatelli, MR. “Catecholamines.” Neurology Resident Neuroscience Course, SIU School of Medicine, December 9, 2003.

88.  Pranzatelli, MR. “Serotonin.” Neurology Resident Neuroscience Course, SIU School of Medicine, January 15, 2004.

89.  Pranzatelli, MR. “Autistic spectrum disorders: A neurologist’s perspective.” 22nd Annual Neurology Symposium, SIU School of Medicine, March 19, 2004.

90.  Pranzatelli, MR. “Overview of childhood cerebellar ataxias.” SIU School of Medicine, Neurology Grand Rounds, May 4, 2004.

91.  Pranzatelli, MR. “Vincent van Gogh at the crossroads of neurology, psychiatry, and neuroscience. What is your diagnosis?” SIU School of Medicine, Neurology Grand Rounds, November 9, 2004.

92.  Pranzatelli, MR. “The neuroimmunology of opsoclonus-myoclonus, a paraneoplastic syndrome.” Wayne State University School of Medicine, Neurology Grand Rounds, Detroit, MI, November 19, 2004.

93. Pranzatelli, MR. “Advances in neuroimmunology: The B lymphocyte as a therapeutic target in autoimmune neurological disorders.” SIU School of Medicine, Neurology Grand Rounds, December 13, 2005.

94.  Pranzatelli, MR. Management of Tourette’s syndrome. 24th Annual Neurology Symposium, SIU School of Medicine, March 24, 2006.

95.  Pranzatelli, MR. “Less familiar faces of cancer: paraneoplastic syndromes.” Michael Hammond, M.D., Annual Memorial Lecture, Scottsdale Healthcare Shea, Scottsdale, AZ, April 28, 2006.

96.  Pranzatelli, MR. “The brain as a war zone: a medical emergency you don’t want to miss.” Michael Hammond, M.D., Annual Memorial Lecture, Scottsdale Healthcare Shea, Scottsdale, AZ, April 28, 2006.

97.  Pranzatelli, MR. “Chemokines: critical roles in the CNS.” SIU School of Medicine, Neurology Grand Rounds, October 17, 2006.

98. Pranzatelli, MR. “Therapeutic frontiers in paraneoplastic disorders: opsoclonus-myoclonus syndrome.” Pediatric Grand Rounds, University of Colorado, Denver, CO, November 17, 2006.

99. Pranzatelli, MR. “Sydenham chorea: the immunology of dance.” Neurology Grand Rounds, SIU School of Medicine, February 27, 2007.

100. Pranzatelli, MR. A Worse Problem than Cancer: Paraneoplastic Syndromes. The 20th Annual John H. Kendig Lecture Series, St. John’s Mercy Medical Center, St. Louis, MO, July 11, 2007.

101. Pranzatelli, MR. Friendly Fire and the Brain: Opsoclonus-Myoclonus Syndrome. The 20th Annual John H. Kendig Lecture Series, St. John’s Mercy Medical Center, St. Louis, MO, July 11, 2007.

 

MEDIA INTERVIEWS AND RELEASES

“Opsoclonus-Myoclonus Syndrome Awareness.” Fox 19, WXIX, Cincinnati, 2002.

“Changed Behavior.” The Herald News, Suburban Chicago News, May 9, 2003.

“Family Battles Insurance Over Treatment.” The Herald News, Suburban Chicago News, May 9, 2003.

“Caring for Mackenzie.” Las Vegas Review-Journal, November 9, 2003.

“SIU Pediatric Neurologist Treats Patients from the U.S. and Abroad for Rare Disorder.” Focal Point, January, 2004.

“Children with Rare Disorder Find Hope at Center in Springfield, IL.” St. Louis Post-Dispatch, Front Page, Monday, March 22, 2004.

“Sound Advice.” WUIS local public radio, May 9, 2004.

“Opsoclonus-Myoclonus Segment.” Channel 20 coverage of CMN Telethon, June 6, 2004.

“WAND-TV Check-Up on Health.” WAND-TV, “Sweeps Series,” November 15 and 16, 2004.

“Monster of the Mind.” The State Journal-Register, Springfield, IL, Health Section, Monday, November 29, 2004.

“Smiling Past the Pain.” Daily Herald, Chicago, IL, Front Page, Sunday, November 20, 2005.

 

SCHOLARSHIP AND RESEARCH

RESEARCH GRANTS: (approximately $3.5 million)

Competitive Peer-Review Awards:

1985-90           Myoclonus Research Foundation, Inc., "The Pharmacology of Myoclonus," $303,665 direct costs, role: P.I.

1987-90           National Institutes of Health grant 1 KO8 NS01158 (CIDA), NINDS,
                        "Serotonin Receptors and Myoclonus," $202,400 direct costs, $218,592
                        total costs, role: P.I.

1988-90           United Cerebral Palsy Education and Research Foundation and William
                        Randolph Hearst Foundation (R-381-88), "Perinatal Brain Injury:
                        Pharmacological Modification of Serotonin Receptors," $76,000 direct
                        and total costs, role: P.I.

1990-93           Food and Drug Administration, Orphan Products Development, FD-R-
                        000746-01/02, "The Efficacy and Safety of 5-Hydroxy-L-tryptophan in
                        Pediatric Myoclonic Disorders," $188,524 direct costs, $269,954 total
                        costs, role: P.I.

1993-94           Myoclonus Research Foundation, "Serotonin Receptor Plasticity and
                        Expression in Experimental Myoclonus," $10,000 direct and total costs, role: P.I.

1993-95           Food and Drug Administration, Orphan Products Development, FD-R-
                        000955-01, "Piracetam for Pediatric Myoclonus: Efficacy and Safety,"
                        $200,437 direct costs, $305,667 total costs, role: P.I.

1994-95           CRI Research Advisory Council, "Molecular Pharmacologic and
                        Immunologic Studies of Opsoclonus-Myoclonus," $13,500 direct costs,
                        $18,022 total costs, role: P.I.

1994-95           Myoclonus Research Foundation, Inc., "Molecular Pharmacologic and
                        Neuroimmunologic Investigation of the Pediatric Opsoclonus-Myoclonus
                        Syndrome," $42,145 direct and total costs, role: P.I.

1995-96           Supplement and Extension, Food and Drug Administration, Orphan
                        Products Development, FD-R-000955, "Piracetam for Pediatric
                        Myoclonus: Efficacy and Safety," $25,546 direct costs, $40,286 total
                        costs, role: .P.I.

1998-00           United Cerebral Palsy Research and Education Foundation, "Piracetam
                        in Myoclonic Cerebral Palsy," $82,519 direct costs, $94,897 total costs, role: P.I.

2000-03           American Medical Association Education and Research Foundation,
                        "Localization and Characterization of Autoantigens in OMS,” $29,973
                        total costs, role: P.I.

1. Illinois Department of Public Health, Excellence in Academic Medicine Award, “Opsoclonus-Myoclonus Syndrome in Children with Neuroblastoma: Search for Paraneoplastic Antibodies which Cause Cerebellar Dysfunction,” $50,000 direct and total costs, role: Co-P.I.

 

2004-07           Tourette Syndrome Association, “Immunophenotyping of Cerebrospinal Fluid Lymphocytes in Tourette Syndrome,” $51,000 direct costs, $56,100 total costs, role: P.I.

2006-07           The Chicago Institute of Neurosurgery and Neuroresearch (CINN) Foundation, “Childhood-Cancer-Associated Brain Disorders: Inflammatory Mediators as Therapeutic Targets,” $50,112 total costs, role P.I.

2007-08           The Chicago Institute of Neurosurgery and Neuroresearch (CINN) Foundation, “Leukocyte Chemokine Receptor Expression in Immune-Mediated Brain Injury.” $54,400, role P.I.
    
Corporate Grants:

1989-90           Bolar Pharmaceutical Co., Inc.,"The Efficacy and Safety of 5-Hydroxy-L-tryptophan in Pediatric Myoclonic Disorders," $24,000 direct and total
                        costs, role: PI

1992-94           UCB Pharmaceutical Sector, "A Clinical Trial of Piracetam in Myoclonic Disorders," $50,000 direct and total costs, role: PI

2002-04           William E. McElroy Charitable Foundation, “Opsoclonus-Myoclonus Syndrome in Neuroblastoma: Identification of Novel Cerebellar Autoantibodies,” $24,990 total costs, role: Co-I

2005-06          Pediatric Opsoclonus-Myoclonus Fund, “Immunologic Studies in Opsoclonus-Myoclonus Syndrome,” $50,000 total costs, role: PI

2005-07           Genentech, Inc./Biogen Idec, Co. “A Phase I Clinical Trial of Rituximab for Pediatric Opsoclonus-Myoclonus,” $214,315 total costs, role: PI

2005-08           Spastic Paralysis and Allied Diseases of the Central Nervous System Research Foundation, “Immunological Studies of Opsoclonus-Myoclonus Syndrome,”
$60,000 total costs, role: PI

2006-07          Pediatric Opsoclonus-Myoclonus Fund, “Comprehensive Analysis of Serum Cytokines as Biomarkers of Disease Activity in Opsoclonus-Myoclonus,” $50,000 total costs, role P.I.

2006-08          William E. McElroy Charitable Foundation, “A Study of Inflammatory Mediators in Pediatric Opsoclonus-Myoclonus,” $50,000 total costs, role P.I.

2006-07          Ronald McDonald House Charities, “B-cells and Autoantibodies in Childhood Opsoclonus-Myoclonus Syndrome,” $10,000 total costs, role P.I.

2007-08          Questcor Pharmaceuticals, “Analysis of Immunological Response to ACTH in Pediatric Opsoclonus-Myoclonus Syndrome.” $121,494 direct costs, $144,368 total costs, role P.I.

2007-08          Genentech/Biogen IDEC, “Effect of Rituximab on CSF and Serum Chemokine/ Cytokine Signaling in Pediatric Opsoclonus-Myoclonus Syndrome, $83,116 direct costs, $99,740 total costs, role P.I.

Institutional Grant Support:

1990-96           Children's Research Institute, Children's National Medical Center,
                        Washington, D.C., $800,000 total costs. Role: PI

1994-95           Board of Lady Visitors, Children's National Medical Center
SIU School of Medicine, $25,000

2000-06           Children’s Miracle Network, $240,000, total costs. Role: PI

Other Grant Support:

1987-89           Aitken Family Center for Coma Research

1987-90           Colleen Giblin Foundation for Pediatric Neurological Research

1988-90           NIH General Clinical Research Center Grant #RR00645

Grant Support for Student Fellowships in P.I. Lab:

1986, 88          Parkinson's Disease Foundation, Summer Student Fellowship

1992-96           W.T. Gill Summer Student Fellowship, The George Washington University

1992-96           Myoclonus Research Foundation Summer Student Fellowship

1992-96           NIH Minorities Apprentice Program, Children's Research Institute

2007                MPEE Program, SIU School of Medicine

CURRENT RESEARCH PROTOCOLS:

Identification of Immunologic Abnormalities in Children with Opsoclonus-Myoclonus Syndrome (IRB # 00-066)

Identification of Autoantibodies in Pediatric Paraneoplastic Opsoclonus-Myoclonus (IRB Exempt Review)

Immunophenotyping of Leukocyte Infiltrates in Neuroblastoma from Children with and without a Paraneoplastic Syndrome (IRB Exempt)

Evaluation of Piracetam in Pediatric Myoclonic Disorders (IRB #00-087)

Immunophenotyping of Cerebrospinal Fluid Lymphocytes in Tourette Syndrome (IRB #03-166)

Pediatric CSF Neurotransmitters and Metabolites (IRB Exempt Review)

Chemotherapy in Pediatric Opsoclonus-Myoclonus Syndrome (IRB Exempt Review)

A Study of Serum Cytokines, Chemokines, and Growth Factors in Healthy Children, Opsoclonus-Myoclonus Syndrome, and Neuroblastoma (IRB # 05-118)

A Phase I Clinical Trial of Rituximab for Pediatric Opsoclonus-Myoclonus (IND# 11,771) (IRB # 04-112) (ClinicalTrials.gov Identifier: NCT00244361)

 

RESEARCH SKILLS:

Basic Research:

1.   Neurochemistry - high performance liquid chromatography for assays of monoamines and their metabolites.
2.   Neurotransmitter receptor binding assays - for serotonin, dopamine, thyrotropin-releasing hormone, glycine, benzodiazepine receptors.
3.  Quantitative receptor autoradiography - regional mapping to study neuroplasticity of each serotonin receptor subtype in rat and human postmortem brain.
4.   Behavioral pharmacology - scoring and evaluation of behavioral components of the serotonin   syndrome, seizures, and myoclonus in rats; videotaping; use of automex quantitative       instruments, rotometers, photocell locomotor activity cages.
5.   Animal lesions - making stereotactic lesions, intracisternal and intraventricular injections of neurotoxins, drugs; rats and dogs.
6.   Fetal brain cell cultures - studies of cytotoxicity, dendritic growth and cell differentiation.
7.   Immunocytochemistry - staining for neuronal and glial markers and neurotransmitters in cultured cells and human brain sections.
8.   Immunofluorescence - immunophenotyping of cultured brain cells and human brain sections using double and triple labelling techniques.
9.   Radioimmunoassays - detection of ACTH and CRF in CSF.
10. Gel electrophoresis - PAGE and agarose.
11. Western blotting - CSF and serum proteins and human brain.
12. Immunophenotyping of CSF and blood by flow cytometry.
13. Cytokine and chemokine measurements by Luminex technology.

Clinical Research:

1. Internationally recognized authority - pediatric movement disorders, especially myoclonus
2. Clinical drug trials in children and adults - federally-funded placebo-controlled double-blinded
     drug studies in human myoclonic disorders.
3. Rating scales for movement disorders - devising and using rating scales for scoring and evaluation of pediatric movement disorders.
4. International center for pediatric opsoclonus-myoclonus syndrome – founding and directing center, developing and managing a clinical research team with research nurse practitioner, clinical  research assistants, and clinical trials coordinator.

5. Data analysis - expertise in computer and statistical requirements for data analysis.
6. Videodocumentation of movement disorders – use for blinded research scoring and documentation.
7.  Immunotherapies - experienced in the use of immunotherapies of various types, such as IA-PEX, IVIG, ACTH, steroids, chemotherapy, and mAbs, in children.
8.  Biomarkers and immunological staging of cerebrospinal fluid (CSF) in children – procedure for safe and atraumatic removal of CSF, even in toddlers.

RESEARCH FOCUS:

Paraneoplastic neurological disorders─ interface of brain and immune system function and dysfunction in a prototypic childhood autoimmune disorder, opsoclonus-myoclonus, associated with neuroblastoma

 

CLINICAL INTERESTS:

1.  Facilitating a compassionate state-of-the-art national clinical center for children with
myoclonic disorders, especially autoimmune in nature; multidisciplinary focus backed by basic research.

2. To maintain and further develop our international website on the paraneoplastic neurological
disorders of neuroblastoma: www.omsusa.org

3.  Developing a pediatric movement disorder service to meet regional healthcare needs; tics, Tourette syndrome, tremor, dystonia, chorea, paroxysmal choreoathetosis; restless legs syndrome, and many other disorders.

STATEMENT OF RESEARCH GOALS:

As a physician/scientist — pediatrician, neurologist, neuroscientist — I see my role as bridging the gap between basic science and clinical neurology. With an interest and background in developmental neurobiology, I have been working over the past 20 years as a P.I. in the study of pediatric movement disorders.

I began my career identifying the pharmacologic mechanisms which underlie the development of mammalian motor systems and the pathophysiology of those developmental neurological disorders which disrupt motor development and result in dyskinesias. These studies involved techniques ranging from neurochemical assays to behavioral pharmacology. I studied lesion- and drug-induced animal models of a prototypic dyskinesia, myoclonus, which is at once a hallmark developmental feature of motor development, and may also occur under other physiologic and pathologic conditions. These studies took me into the field of serotonin neuropharmacology, where I was involved in 5-HT receptor mapping studies through quantitative autoradiography and investigating the effect of neonatal brain lesions on 5-HT receptor ontogeny and myoclonus. In parallel clinical pharmacologic research, I evaluated the effect of serotonergic drugs on human myoclonus.

About 20 years ago, I focused on one pediatric myoclonic disorder caused by neuroblastoma, a paraneoplastic syndrome. I developed the National Pediatric Myoclonus Center (www.omsusa.org), a comprehensive and now international center with two programs, one clinical, the other basic. We have evaluated and created a database on 225+ children, which is the largest experience with this rare disorder in the world. The clinical research program is designed to provide compassionate care, state-of-the-art diagnostic testing, the most promising of new investigational drug treatments for myoclonus, and to obtain body fluid samples for laboratory research. It avails itself of the research techniques of many outside collaborators. We developed and advocate immunologic staging, an individualized approach, much as oncologists would do tumor staging, to identify high-risk children and to ensure that no one is under-treated or over-treated. Based on our data, we are also proponents of multi-modality immunotherapy for opsoclonus-myoclonus and have pioneered this approach.

The basic research program entails studies of the neuroimmunology of paraneoplastic disorders. The goal is to define the immunologic defect in the opsoclonus-myoclonus syndrome. In studies of CSF and sera from OMS patients, we immunophenotype by flow cytometry using monoclonal antibodies; measure cytokine/chemokine activity in CSF; culture B cells and T cells and measure lymphocyte responses in vitro, such as antibodies and lymphokines produced in response to various antigen-specific stimuli such as brain and tumor antigens. For neuroanatomic localization, we test for immunostaining of brainstem and cerebellum by CSF, sera, and conditioned media from lymphocyte cultures, using double immunofluorescence labelling and immunoenzymatic assays. To define the neuropharmacologic target of autoantibodies, we screen CSF and sera and B cell culture conditioned media using gel electrophoresis and Western blots of pediatric control brain for testing the existence of autoantibodies against several neurotransmitter system enzymes and receptors known to have a role in myoclonus, such as GABA, glutamate, and serotonin. The relationship of immunologic and molecular pharmacologic abnormalities to clinical variables is evaluated statistically, focusing on treatment status and clinical severity as main variables. We have also developed an in vitro model system for studies of cytotoxicity in opsoclonus-myoclonus, which is being used to study brain-immune interactions. The effect parameters include cell differentiation, dendritic proliferation, and cell death by apoptosis.

My research has lead to the discovery of immunological biomarkers of disease activity in pediatric opsoclonus-myoclonus syndrome. Finding expansion of B- and T-cells in CSF that correlates highly with disease severity, we developed a treatment innovation, namely the use of a selective anti-B-cell monoclonal antibody (rituximab). We demonstrated that rituximab normalizes the CSF B-cell immunophenotype with resulting clinical improvement. We now have a phase I clinical trial that we anticipate will lead to a phase II trial in the near future. Our cytokine program has identified cytokine/chemokine biomarkers of relapse. We are now studying the effect of conventional and investigational immunotherapies on the biomarkers, with the goal of applying or developing novel cytokine/chemokine blockers. Short-term cultures of lymphocyte subsets will be used in functional assays and mechanistic studies.

In summary, I believe that the two aspects of my research, pharmacology and immunology, are now converging on the relation of neurological and immunological abnormalities in paraneoplastic disorders. My research has broader implications for centrally-mediated autoimmune disorders. Also, learning how to down-regulate anti-tumor immune defenses may have important implications for tumor immunotherapy in the treatment of cancer.

BIBLIOGRAPHY

ORIGINAL RESEARCH REPORTS (peer-reviewed journals):

1.  Pranzatelli MR, Stumpf DA. The metabolic consequences of experimental intracranial hemorrhage. Neurology 35:1299-1303, 1985.

2.  Pranzatelli MR, Rubin G, Snodgrass SR. Serotonin-lesion myoclonic syndromes. I. Neurochemical profile and S1 receptor binding. Brain Research 364:57-66, 1986.

3.  Pranzatelli MR, Snodgrass SR. Serotonin-lesion myoclonic syndromes. II. Analysis of individual syndrome elements, locomotor activity, and behavioral correlations. Brain Research 364:67-76, 1986.

4.  Pranzatelli MR, Snodgrass SR. Antimyoclonic properties of S-2 serotonin receptor antagonists in the rat. Neuropharmacology 25:5-12, 1986.

5.  Pranzatelli MR, Snodgrass SR. Enhanced selective 5-HT depletions in the DHT rat model:
Denervation supersensitivity and recovery of function. Psychopharmacology 89:449-455, 1986.

6.  Pranzatelli MR, Snodgrass SR. Motor habituation in the DHT model: Bin analysis of daytime and nocturnal locomotor activity. Pharmacology Biochemistry and Behavior 24:1679-1686, 1986.

7.  Pranzatelli MR, Gantner C, Snodgrass SR. 3-Acetylpyridine lesions and four serotonergic
behavioral syndromes in the rat. Brain Research Bulletin 18:159-163, 1987.

8.  Pranzatelli MR, Schultz L, Snodgrass SR. High-dose clonidine motor syndrome: Relationship to serotonin syndrome. Behavioral Brain Research 24:221-232, 1987.

9.  Pranzatelli MR, Snodgrass SR. Harmala alkaloids and related B-carbolines: A myoclonic model and antimyoclonic drugs. Experimental Neurology 96:703-719, 1987.

10. Pranzatelli MR, Jappay E, Snodgrass SR. Effects of 5-HT receptor subtype-selective drugs on locomotor activity and motor habituation in the DHT adult rat model. Pharmacology Biochemistry and Behavior 27:497-504,1987.

11. Pranzatelli MR, Snodgrass SR. The guinea pig myoclonic model: Behavioral supersensitivity to 5-hydroxytryptophan induced by intracisternal 5,7-dihydroxytryptamine. European Journal of Pharmacology 143:237-242, 1987.

12. Pranzatelli MR. The comparative pharmacology of the behavioral syndromes induced by TRH and by 5-HT in the rat. General Pharmacology 19:205-211, 1988.

13. Pranzatelli MR, Dailey A, Markush S. The regulation of TRH and serotonin receptors: Chronic TRH and analog administration in the rat. Journal of Receptor Research 8:667-681, 1988.

14. Pranzatelli MR. Effect of chronic treatment with 5-hydroxytryptophan on cortical serotonin
receptors in the rat. Clinical Neuropharmacology 11:257-262, 1988.

15. Pranzatelli MR. Effect of antiepileptic and antimyoclonic drugs on serotonin receptors in vitro. Epilepsia 29:412-419, 1988.

16. Pranzatelli MR. In vivo and in vitro effects of adrenocorticotrophic hormone on serotonin receptors in neonatal rat brain. Developmental Pharmacology and Therapeutics 12:49-56, 1989.

17. Pranzatelli MR, Eng B.  Chronic ACTH treatment: Influence on 5-HT2 receptors and behavioral supersensitivity induced by 5,7-dihydroxytryptamine lesions.  Peptides 10:5-8, 1989.

18. Pranzatelli MR. Benzodiazepine-induced shaking behavior in the rat: Structure-activity and relation to serotonin and benzodiazepine receptors. Experimental Neurology 104:241-250, 1989.

19. Pranzatelli MR, Dollison AM, Hlibczuk V. Intracisternal 5,7-dihydroxytryptamine lesions in neonatal and adult rats: Comparison of response to 5-hydroxytryptophan. Developmental Neuroscience 11:205-211, 1989.

20. Chiriboga CA, Pranzatelli MR, De Vivo DC. Chronic ACTH treatment increases striatal dopamine D-2 receptor binding in developing rat brain. Brain Dev 11:197-200, 1989.

21. Pranzatelli MR, Huang Y, Dollison AM, Stanley M. Brainstem serotonergic hyperinnervation modifies behavioral supersensitivity to 5-hydroxytryptophan in the rat. Developmental Brain Research 50:89-99, 1989.

22. Pranzatelli MR, Balletti J. Identification of 5-HT1A recognition sites in human ganglioneuroblastoma. European Journal of Pharmacology 170:127-128, 1989.

23. Butler PD, Pranzatelli MR, Barkai AL. Regional central serotonin-2 receptor binding and
phosphoinositide turnover in rats with 5,7-dihydroxytryptamine lesions. Brain Research Bulletin 24:125-129,1990.

24. Pranzatelli MR. Evidence for involvement of 5-HT2 and 5-HT1C receptors in the behavioral effects of the 5-HT agonist 1 -(2,5-dimethoxy-4-iodophenylaminopropane (DOI). Neuroscience Letters 115:74-80, 1990.

25. Pranzatelli MR, Dollison AM, Huang Y-y. The functional significance of neonatal 5,7-
dihydroxytryptamine lesions in the rats: Response to selective 5-HT1A and 5-HT 2,1C agonists. Brain Research Bulletin 24:747-753, 1990.

26. Pranzatelli MR. Antimyoclonic effect of MK-801: A possible role for NMDA receptors in
developmental myoclonus of the neonatal rat. Clinical Neuropharmacology 13:329-338, 1990.

27. Pranzatelli MR. Neonatal 5,7-DHT lesions upregulate [3H]mesulergine-labelled spinal 5-HT1C binding sites in the rat. Brain Research Bulletin 25:151-153, 1990.

28. Pranzatelli MR. The regulation of 5-HT2 receptors in rat frontal cortex: Studies with a putative selective agonist and an antagonist. Biochemical Pharmacology 42:1099-1105, 1991.

29. Pranzatelli MR, Pluchino RS. The relation of central 5-HT1A and 5-HT2 receptors: Low dose agonist-induced selective tolerance in the rat. Pharmacology Biochemistry and Behavior 39:407-413, 1991.

30. Pranzatelli MR, Dailey A, Levy M, Dollison A. Absence of tolerance to the excitatory effects of benzodiazepines: Clonazepam-evoked shaking behavior in the rat. Pharmacology Biochemistry and Behavior 39:1021-1024,1991.

31. Pranzatelli MR. Regional central serotonin receptor binding in rats treated chronically with high-dose 5-hydroxy-L-tryptophan. Experimental Brain Research 87:340-344, 1991.

32. Pranzatelli MR, Balletti J. Characterization of 5-hydroxytryptamine1A -like binding sites in human ganglioneuroblastoma. Neuroscience Letters 132:117-120, 1991.

33. Pranzatelli MR, Albin RL, Cohen BH. Acute dyskinesias in young asthmatics on theophylline. Pediatric Neurology 7(3):216-219, 1991.

34. Pranzatelli MR, Pavlakis SG, Gould RJ, De Vivo DC. Hypothalamic-midbrain dysregulation
syndrome: Hypertension, hyperthermia, hyperventilation, and decerebration. Journal of Child Neurology 6(2):115-122,1991.

35. Pranzatelli MR, Tkach K. Regional glycine receptor binding in the p,p'-DDT myoclonic rat model. Archives of Toxicology 66:73-76, 1992.

36. Pranzatelli MR, Balletti J. Serotonin receptors in human neuroblastoma: A possible biologic tumor marker. Experimental Neurology 115:423-427, 1992.

37. Pranzatelli MR, Murthy JN Pluchino RS. Identification of spinal 5-HT1C binding sites in the rat: characterization of [3H]mesulergine binding. Journal of Pharmacology and Experimental Therapeutics 261:161-165, 1992.

38. Murthy JN, Pranzatelli MR. Brainstem 5-hydroxytryptamine1A binding sites are not down-regulated by agonists which induce tolerance in the rat. Journal of Receptor Research 12:287-297, 1992.

39. Heyes MP, Saito K, Crowley JS, Davis LE, Demitrack MA, Der M, Dilling LA, Elia J, Kruesi MJP, Lackner A, Larsen SA, Lee K, Leonard HL, Markey SP, Martin A, Milstein S, Mouradian MM, Pranzatelli MR, Quearry BJ, Salazar A, Smith M, Straus SE, Sunderland T, Swede SE, Tourtellotte WW. Quinolinic acid and kynurenine pathway metabolism in inflammatory and non-inflammatory neurologic disease. Brain 115:1249-1273, 1992.

40. Trifiletti R, Pranzatelli MR. ACTH binds to [3H]MK-801-labelled rat hippocampal NMDA receptors. European Journal of Pharmacology 226:377-379, 1992.

41. Pranzatelli MR, Martens JN. Plasticity and ontogeny of the central 5-HT transporter: effect of neonatal 5,7-dihydroxytryptamine lesions in the rat. Developmental Brain Research 70:191-195, 1992.

42. Pranzatelli MR. Serotonin receptor ontogeny: effects of agonists in one day old rats. Pharmacology Biochemistry and Behavior 43:1273-1277, 1992.

43. Pranzatelli MR, Murthy JN, Tailor PT. Novel regulation of 5-hydroxytryptamine1C receptors: down-regulation induced both by 5-HT1C agonists and antagonists. European Journal of Pharmacology 244:1-5, 1993.

44. Pranzatelli MR. Regional differences in the ontogeny of 5-hydroxytryptamine1C binding sites in rat brain and spinal cord. Neuroscience Letters 149:9-11, 1993.

45. Pranzatelli MR, Tailor PT, Razi P. Brainstem serotonin receptors in the guinea pig: implications for myoclonus. Neuropharmacology 32:209-215, 1993.

46. Pranzatelli MR, Gregory CM. High and low affinity 5-HT2 and 5-HT1C binding sites: responses to neonatal 5,7-DHT lesions in rat brain. Cytobios 75:197-209, 1993.

47. Toro C, Pascual-Leone A, Deuschl G, Tate E, Pranzatelli MR, Hallett M. Cortical tremor, a
common manifestation of cortical myoclonus. Neurology 43:2346-2352, 1993.

48. Pranzatelli MR, Kao PC, Tate ED, Chaves E, Chez M, Dobyns WE, Kang H, Rothner DA.
Autoantibodies to ACTH in opsoclonus-myoclonus. Neuropediatrics 24:131-133, 1993.

49. Pranzatelli MR, Tailor PT, Pluchino R, Gonzales W, Simmens S. DDT myoclonic/epileptic model: serotonin receptor binding and behavioral studies in the rat. Neurotoxicology 15(2):261-272, 1994.

50. Pranzatelli MR. Dissociation of the plasticity of 5-HT1A sites and 5-HT transporter sites.
Neurochemistry Research 19:311-315, 1994.

51. Pranzatelli MR, Durkin MM, Barkai AI. Quantitative autoradiography of 5-hydroxy- tryptamine binding sites in rats with neonatal 5,7-dihydroxytryptamine lesions. Developmental Brain Research 80:1-6, 1994.

52. Pranzatelli MR, Razi P. Drug-induced regulation of [125I]iodocyanopindolol-labelled
5-hydroxytryptamine1B receptors in the central nervous system. Neuropsychopharmacology 10:259-264,1994.

53. Pranzatelli MR, Galvan I. Ontogeny of [125I]iodocyanopindolol-labelled 5-hydroxytryptamine1B binding sites in the rat CNS. Neuroscience Letters 149:9-11, 1994.

54. Pranzatelli MR, Tailor PT. Modulation of brainstem 5-HT1C receptors by serotonergic drugs in the rat. General Pharmacology 25:1279-1284, 1994.

55. Pranzatelli MR, Mott S, Pavlakis S, Conry J, Tate E. The clinical spectrum of secondary
parkinsonism in childhood: A reversible disorder. Pediatric Neurology 10:131-140, 1994.

56. Pranzatelli MR, Tate E, Baldwin M. Clinical responses to 5-hydroxy-L-tryptophan in chronic pediatric opsoclonus-myoclonus suggest biochemical heterogeneity: a double-blinded placebo crossover pilot study. Clinical Neuropharmacology 17:103-116, 1994.

57. Pranzatelli MR, Huang Y-y, Tate E, Stanley M, Noetzel MJ, Gospe SM, Banasiak K. Cerebrospinal fluid 5-hydroxyindoleacetic acid and homovanillic acid in the pediatric opsoclonus-myoclonus syndrome. Annals of Neurology 37:189-197, 1995.

58. Papero PH, Pranzatelli MR, Margolis CJ, Tate E, Glass P. Neurobehavioral and psychosocial functioning of children with opsoclonus-myoclonus syndrome. Developmental Medicine and Child Neurology 37:915-932, 1995.

59. Pranzatelli MR, Tate E, Huang Y, Haas R, Bodensteiner J, Ashwal S, Franz D. The
neuropharmacology of progressive myoclonus epilepsy: response to 5-hydroxy-L-tryptophan.
Epilepsia 36:783-791, 1995.

60. Mott SH, Packer RJ, Vezina LG, Kapur S, Dinndorf PA, Conry JA, Pranzatelli MR, Quinones RR. Encephalopathy with parkinsonian features in children following bone marrow transplantations treated with high dose amphotericin B. Annals of Neurology 37:810-814, 1995.

61. Tate E, Ho H, Kim A, Pranzatelli MR. A sensitive and quantitative pediatric myoclonus evaluation scale. Journal of Neuroscience Nursing 27:287-292, 1995.

62. Pranzatelli MR, Galvan I, Tailor PT. Human brainstem serotonin receptors: characterization and implications for subcortical myoclonus. Clinical Neuropharmacology 19(6):507-514, 1996.

63. Pranzatelli MR, Durkin MM, Farmer M. Plastic responses of neonatal 5-hydroxytryptamine1B receptors to 5,7-dihydroxytryptamine lesions mapped by quantitative autoradiography. International Journal of Developmental Neuroscience 14:621-629, 1996.

64. Pranzatelli MR, Tate E, Wheeler A. A controlled trial of 5-hydroxy-L-tryptophan for ataxia in progressive myoclonus epilepsy. Clinical Neurology and Neurosurgery 98(2):161-164, 1996.

65.  Connolly AM, Pestronk A, Shobhna M, Pranzatelli MR, Noetzel MJ. Serum autoantibodies in childhood opsoclonus-myoclonus syndrome: an analysis of antigenic targets in neural tissues. Journal of Pediatrics 130:878-884, 1997.
 
66.  Lafreniere RG, Rochefort DL, Chretien N, Rommens JM, Cochius JL, Kalviainen R, Nousiainen U, Patry G, Farrell K, Soderfeldt B, Federico A, Hale BR, Cossio OH, Sorensen T, Pouliot MA, Kmiec T, Uldall P, Janszky J, Pranzatelli MR, Andermann F, Andermann E, Rouleau GA.  Unstable insertion in the 5’ flanking region of the cystatin B gene is the most common mutation in progressive myoclonus epilepsy type 1, EPM1. Nature Genetics 15:298-302, 1997.

67.  Bespalova IN, Adkins S, Pranzatelli M, Burmeister M. Novel cystatin B mutation and diagnostic PCR assay in Unverricht-Lündborg (Baltic) progressive myoclonus epilepsy patient.  American Journal of Medical Genetics (Neuropsychiatric Genetics) 74(5):467-471, 1997.

68.  Bespalova IN, Pranzatelli M, Burmeister M. G to C transversion at a splice acceptor site causes exon skipping in the cystatin B gene.  Mutation Research Genomics 382:67-74, 1997.

69.  Pranzatelli MR, Hanin I, Tate E, Kindel G, Bergin A, Brown CM, Habersang P, Habersang R, Mack KJ, Rosenbaum B, Selby KA. Cerebrospinal fluid free choline in movement disorders of pediatric onset.  European Journal of Pediatric Neurology 1:33-39, 1998.

70.  Pranzatelli MR, Huang YY, Tate E, Goldstein DS, Holmes CS, Goldstein EM, Ketner K, Kinast M, Lange BM, Sanz A, Shevell MI, Stanford RE, Taff IP. Monoaminergic effects of high dose corticotropin in corticotropin-responsive pediatric opsoclonus-myoclonus. Movement Disorders 13:522-528, 1998.

71. Pranzatelli MR, Tate ED. Chloral hydrate for progressive myoclonus epilepsy: A new look at an old drug. Pediatric Neurology 25:385-389, 2001.

72. Pranzatelli MR, Tate ED, Galvan I, Wheeler A. Controlled pilot study of piracetam for pediatric opsoclonus-myoclonus. Clinical Neuropharmacology 24:352-357, 2001.

73. Pranzatelli MR, Tate ED, Kinsbourne M, Caviness VS, Mishrah B. Forty-one year follow-up of childhood-onset opsoclonus-myoclonus: cerebellar atrophy, multiphasic relapses, response to IVIG. Movement Disorders 17(6):1387-1390, 2002.

74. Pranzatelli MR, Tate ED, Wheeler A, Bass N, Gold AP, Griebel ML, Gumbinas M, Heydemann PT, Holt PJ, Jacob P,  Kotagal S, Minarcik CJ, Schub HS. Screening for autoantibodies in children with opsoclonus-myoclonus-ataxia.  Pediatric Neurology 27(5):384-387, 2002.

75.  Pranzatelli MR, Travelstead AL, Tate ED, Allison TJ, Moticka EJ, Franz DN, Nigro MA, Parke JT, Stumpf DA, Verhulst SJ. B- and T-cell markers in opsoclonus-myoclonus syndrome: immunophenotyping of CSF lymphocytes. Neurology 62(9):1526-1532, 2004.

76.  Pranzatelli MR, Travelstead AL, Tate ED, Allison TJ, Verhulst SJ. CSF B-cell expansion in opsoclonus-myoclonus syndrome: a biomarker of disease activity. Movement Disorders 19(7):770-777, 2004.

77. Pranzatelli MR, Travelstead AL, Tate ED, Allison TJ, Lee ND, Fisher J, Jasty R. Immunophenotype of blood lymphocytes in neuroblastoma-associated opsoclonus-myoclonus. Journal of Pediatric Hematology/Oncology 26(11):718-723, 2004.

78.  Pranzatelli MR, Hyland K, Tate ED, Arnold LA, Allison TJ, Soori GS. Evidence of cellular immune activation in children with opsoclonus-myoclonus: cerebrospinal fluid neopterin. Journal of Child Neurology 19:919-924, 2004.

79. Pranzatelli MR, Tate ED, Travelstead AL, Longee D. Immunologic and clinical response to rituximab in a child with opsoclonus-myoclonus syndrome. Pediatrics 115(1):e115-e119, 2005.

80.  Tate ED, Allison TJ, Pranzatelli MR, Verhulst SJ. Neuroepidemiologic trends in 105 U.S. cases of pediatric opsoclonus-myoclonus. Journal of Pediatric Oncology Nursing 22(1):8-19, 2005.

81.  Pranzatelli MR, Chun KY, Moxness M, Tate ED, Allison TJ. Cerebrospinal fluid ACTH and cortisol in opsoclonus-myoclonus: effect of therapy. Pediatric Neurology 33:121-126, 2005.

82.  Pranzatelli MR, Tate ED, Dukart WS, Flint MJ, Hoffman MT, Oksa AE. Sleep disturbance and rage attacks in opsoclonus-myoclonus syndrome: response to trazodone. Journal of Pediatrics 147(3):372-378, 2005.

83. Pranzatelli MR, Tate ED, Travelstead AL, Barbosa J, Bergamini RA, Civitello L, Franz DN, Greffe BS, Hanson RD, Hurwitz C, Kalinyak KA, Kelfer H, Khakoo Y, Mantovani JF, Nicholson HS, Sanders JM, Wegner S. Rituximab (anti-CD20) adjunctive therapy for opsoclonus-myoclonus syndrome. Journal of Pediatric Hematology Oncology 28(9):585-593, 2006.

 

REVIEW ARTICLES (peer-reviewed journals):

84. Pranzatelli MR, Snodgrass SR. The pharmacology of myoclonus. Clinical Neuropharmacology 8: 99-130, 1985.

85. Pranzatelli MR, De Vivo DC. The pharmacology of Reye syndrome. Clinical Neuropharmacology 10: 96-125, 1987.

86. Pranzatelli MR. The neurobiology of opsoclonus-myoclonus. Clinical Neuropharmacology 15(3): 186-228, 1992.

87. Pranzatelli MR. Serotonin and human myoclonus: rationale for the use of serotonin receptor agonists and antagonists. Archives of Neurology 51: 605-617, 1994.

88. Pranzatelli MR. Putative neurotransmitter abnormalities in infantile spasms: CSF neurochemistry and response to drugs. Journal of Child Neurology 9: 119-129, 1994.

89. Pranzatelli MR. On the molecular mechanism of adrenocorticotrophic hormone: neurotransmitters and receptors. Experimental Neurology 125: 142-161, 1994.

90. Pranzatelli MR, Nadi NS. Mechanism of action of antiepileptic and antimyoclonic drugs. Advances in Neurology 67: 329-360, 1995

91. Pranzatelli MR. Update on pediatric movement disorders. Advances in Pediatrics 42: 415-463, 1995.

92. Pranzatelli MR. The immunopharmacology of the opsoclonus-myoclonus syndrome. Clinical Neuropharmacology 19:1-47, 1996.

93. Pranzatelli MR. Advances in the treatment of pediatric movement disorders. International Pediatrics 10: 250-259,1995.

94. Pranzatelli MR. Antidyskinetic drug therapy for pediatric movement disorders. Journal of Child Neurology 11:355-369, 1996.

95. Pranzatelli MR. The pharmacology of antimyoclonic drugs. Clinical Neuroscience 3:246-252, 1996.

96. Pranzatelli MR. Movement disorders in childhood. Pediatrics in Review 17:388-394, 1996.

97. Pranzatelli MR. Oral pharmacotherapy for the movement disorders of cerebral palsy. Journal of Child Neurology 11 (suppl 1) S13-S22, 1996.

98.  Pranzatelli MR. Paraneoplastic syndromes: An unsolved murder. Neuroimmunology issue, Seminars in Pediatric Neurology 7(2):118-130, 2000.

99.  Pranzatelli MR. Infantile spasms vs. myoclonus: Is there a connection? In: Epilepsy, infantile spasms, and developmental encephalopathy. International Review of Neurobiology 49: 285-314, 2002.
 
100.  Pranzatelli MR. A forward look at the therapy of pediatric movement disorders.  Seminars in Pediatric Neurology 10:103-110, 2003.

101.  Pranzatelli MR. Myoclonus in childhood.  Seminars in Pediatric Neurology 10:41-51, 2003.

 

BOOKS, BOOK CHAPTERS, AND OTHER REVIEWS:

102. Pranzatelli MR. The proposed role of neurotransmitter receptors in the pathophysiology of human myoclonic disorders. Medical Hypotheses 30: 55-60, 1989.

103. Pranzatelli MR, Pedley TA. Differential diagnoses in childhood. In: Dam M, Gram L (eds)
Comprehensive Epileptology - An International Perspective, Raven Press, 423-447, 1990.

104. Pranzatelli MR, De Vivo DC. Role of the central nervous system in blood pressure regulation. In: Loggie J (ed) Pediatric Hypertension, Chapter 4, Blackwell Scientific Publication, 52-63, 1991.

105. Pranzatelli MR. An approach to movement disorders of childhood. Pediatric Annals 22: 13-17, 1993.

106. Pranzatelli MR. Myoclonic disorders. Pediatric Annals 22: 33-37, 1993.

107. Pranzatelli MR. Miscellaneous movement disorders of childhood. Pediatric Annals 22: 65-68, 1993.

108. Pranzatelli MR. Autoimmunity: the missing link in neurobiology of CNS paraneoplastic syndromes.  Advances in Neuroimmunology 3:12-17, 1996. 

109. Pranzatelli MR. Use of 5,6- and 5,7-dihydroxytryptamine to lesion serotonin neurons. In: Kostrzewa RM (ed) Highly selective neurotoxins: Basic and clinical applications. Humana Press, Inc., Totowa, NY, 1997.

110.  Pranzatelli MR. Myoclonic disorders. I. Diagnosis and pharmacotherapy. Drugs of Today: Timely Topics in Medicine 33 (4):237-250, 1997.

111.  Pranzatelli MR. Myoclonic Disorders. II. Antimyoclonic drug sites and mechanisms of action.  Drugs of Today: Timely Topics in Medicine 33 (5):315-324,1997.

112.  Pranzatelli MR. Serotonergic drugs and movement disorders in humans. Drugs of Today: Timely Topics in Medicine 33 (6):379-392, 1997.

113.  Pranzatelli MR. Central neurological paraneoplastic syndromes.  Part 1. Clinical features, pathophysiology, and immunopharmacology. Drugs of Today 34 (5):415-430, 1998.

114.  Pranzatelli MR. Central neurological paraneoplastic syndromes.  Part 2.  Practical, theoretical, and mechanistic considerations of treatment with drugs and biologicals. Drugs of Today 34(6), 1998.

115.  Pranzatelli MR. Peripheral neurological paraneoplastic syndromes. Drugs of Today 34(7):625-639, 1998.

116.  Tate E, Pranzatelli MR. Unforgettable Faces: Through the Eyes of a Nurse Practitioner. Atheneum Press, 461 pp, 1999.

117.  Pranzatelli MR. A new age of CNS drug delivery.  Drug & Market Development 10 (4):129-136, 1999.

118.  Pranzatelli MR. Innovations in drug delivery to the central nervous system.  Drugs of Today 35(6):435-448, 1999.

119.  Pranzatelli MR. The new appeal of nicotinic acetylcholine receptors. Drug & Market Development 10(10):346-352,1999.

120.  Pranzatelli MR. Friendly Fire. Discover April 2000, p 35-36.

121.  Pranzatelli MR. Tremor disorders in children. International Tremor Foundation Spring 2000 Newsletter.  pp. 1-5.

122.  Pranzatelli MR. Opsoclonus-myoclonus syndrome. In The NORD Guide to Rare Diseases. Lippincott, Williams & Wilkins, Philadelphia, PA, 2003.

123.  Pranzatelli MR. Progressive myoclonus epilepsy. In The NORD Guide to Rare Diseases. Lippincott, Williams & Wilkins, Philadelphia, PA, 2003.

124. Pranzatelli, MR. An update on OMS and neuroblastoma. NB Journal, Spring Issue, 2004.

125.  Pranzatelli MR. Pathophysiology, neurophysiology, and pharmacology of human myoclonus. In LeDoux M (ed) Animal Models of Movement Disorders, Elsevier Inc: Amsterdam, pp 397-413, 2005.

126. Pranzatelli, MR. Opsoclonus-myoclonus syndrome: Diagnostic and therapeutic considerations. In Steinlin M, Kaufmann F, Fuhrer K, Strozzi S (eds) Aktuelle Neuropädiatrie 2004, Novartis Pharma Verlag, Nürnberg, pp 120-131, 2005.
 
127.  Pranzatelli MR. Myoclonus. In Singer HS, Kossoff EH, Hartman AL, Crawford TO (eds) Treatment of Pediatric Neurologic Disorders.Taylor & Francis, Boca Raton, pp 151-159, 2005.

128.  Kramer K, Pranzatelli MR. Management of neurologic complications. In Cheung N-K V, Cohn SL (eds) Neuroblastoma, Springer-Verlog, Berlin, pp 213-222, 2005.

129.  Pranzatelli MR. Opsoclonus-myoclonus-ataxia. In Fernandez-Alvarez E, Arzimanoglou A, Tolosa E (eds) Pediatric Movement Disorders, John Libbey Eurotext: Montrouge, France, pp 120-136, 2005.

130.  Pranzatelli, MR. Treatment of myoclonus. In Jankovic J, Tolosa E, Parkinson’s Disease & Movement Disorders, 5th Edition, Chapter 29, Lippincott Williams & Wilkins, pp 387-393, 2006.

131.  Pranzatelli MR.  In Movement Disorders in Children: A Clinical Update with Video Recordings. John Libbey Eurotext, pp 125-137, 2007.

132.  Blumkin L, Pranzatelli MR. Acquired ataxias, infectious and parainfectious. In Subramony SH and Durv A (eds) Ataxia Disorders I. Handbook of Clinical Neurology series, 3rd Edition, in press.

 

LETTERS TO THE EDITOR:

133. Pranzatelli MR, Snodgrass SR. Fenfluramine therapy for autism. Journal  of Autism and Developmental Disorders [letter] 15:439-441,1985.

134. Pranzatelli MR. Hypothalamic-midbrain dysregulation syndrome. Child Neurology [letter] 7: 116-117, 1992.

135. Pranzatelli MR, Franz D, Tate ED, Martens JM. Buspirone in progressive myoclonus epilepsy. Journal of Neurology Neurosurgery and Psychiatry [letter] 56(1): 114-115, 1993.

136. Pranzatelli MR. Dyskinesia secondary to aminophylline. Pediatric Neurology [letter] 1993.

137. Tate ED, Pranzatelli MR. Baltic myoclonus and crohn's disease. Movement Disorders [letter] 8(2) 252-253,1993.

138. Pranzatelli MR. Neuroblastoma masquerading as benign childhood ataxia: An unrecognized medical emergency. The Journal of Emergency Medicine, 26(2), 2004.

 

ABSTRACTS (platform presentations and posters):

1.  Pranzatelli MR, Rubin G, Snodgrass SR. Pharmacologic correlates of 5-HT lesion syndromes. Society for Neuroscience [abstr] 9: 1404, 1983.

2.  Pranzatelli MR, Stumpf DA. The metabolic consequences of intracranial hemorrhage: A dog model. Annals of Neurology [abstr] 14: 359, 1983.

3.  Pranzatelli MR, Jappay E, Snodgrass SR. Antimyoclonic properties of type-2 serotonin receptor (S2) antagonists. Neurology [abstr] 34 (Suppl 1): 267-268, 1984.

4.  Pranzatelli MR. In vitro activity of antimyoclonic and anticonvulsant drugs at central serotonin 5-HT1A, 5-HT1B, and 5-HT2 receptors in the rat. Society for Neurosci [abstr] 12: 363, 1986.

5.  Truong DD, Pranzatelli MR, Jackson-Lewis V, Fahn S. Intracerebroventricular glycine blocks myoclonus induced by p,p'-DDT in the rat. Annals of Neurology [abstr] 20: 149, 1986.

6.  Pranzatelli MR. Studied on the relation between glycine and serotonin in myoclonus. Neurology [abstr] 37 (Suppl 1): 283, 1987.

7.  Pranzatelli MR, Snodgrass SR, Gillis F. The role of the inferior olive in myoclonus in the DHT rat model. Neurology [abstr] 37 (Suppl 1): 283, 1987.

8.  Pranzatelli MR. The serotonin hypothesis of infantile spasms: Regional studies of ACTH effects in vitro and in vive on serotonin receptors in the neonatal and adult rat CNS. Annals of Neurology [abstr] 22: 437, 1987.

9.  Chiriboga CA, Pranzatelli MR, De Vivo DC. The effect of ACTH treatment on striatal dopamine D-2 receptors in developing rat brain. Annals of Neurology [abstr] 22: 433, 1987.

10. Dailey A, Pranzatelli MR. Motor abnormalities evoked by co-transmitters: Analysis of TRH and 5-HT syndromes. Society for Neuroscience [abstr] 13: 1037, 1987.

11. Butler PD, Pranzatelli MR, Barkai AL. Regional effects of 5,7-dihydroxytryptamine on 5-HT, receptors and phosphoinositide turnover in the rat. Society for Neuroscience [abstr] 13: 342, 1987.

12. Truong DD, Pranzatelli MR, Jackson-Lewis V, Fahn S. DDT-induced myoclonus in rats:
Effects of pharmacologic agents. Neurology [abstr] 37: 377, 1987.

13. Pranzatelli MR. Shaking behavior in the rat: Interaction between benzodiazepines and serotonin 5-HT1A agonists. Society for Neuroscience [abstr] 13: 799, 1987.

14. Pranzatelli MR, Gonzales W, Eng B. Serotonin and epilepsy: Altered responsiveness to the convulsant DDT in rats with 5,7-dihydroxytryptamine lesions. Neurology [abstr] 38 (Suppl 1): 229,1988.

15. Pranzatelli MR. Failure of recovery mechanisms to prevent the consequences of neonatal serotonin brain lesions. Neurology [abstr] 38 (Suppl 1): 303, 1988.

16. Pranzatelli MR, Levy M, Dailey A. Serotonin and benzodiazepine receptor binding in rats treated chronically with five classes of benzodiazepines. Neurology [abstr] 38 (Suppl 1): 302, 1988.

17. Pranzatelli MR. Spontaneous myoclonus in the neonatal rat: The role of NMDA receptors. Annals of Neurology [abstr] 24: 339, 1988.

18. Pranzatelli MR. Evaluation of experimental hyperglycinemia in the neonatal rat as a model of non-ketotic hyperglycinemia. Annals of Neurology [abstr] 24: 304, 1988.

19. Pranzatelli MR. Ontogeny of serotonergic behaviors in the rat: Putative selective agonists. Annals of Neurology [abstr] 24: 350, 1988.

20. Pluchino RS, Pranzatelli MR. Comparative acute and chronic effects of putative 5-HT2 and 5-HT1A agonists in the rat: Is there more than one "serotonin syndrome?" Society for Neuroscience [abstr] 14: 306,1988.

21. Pranzatelli MR. 5-HT2 receptor regulation in rat frontal cortex: Putative selective agonist and antagonist studies. Society for Neuroscience [abstr] 14: 609, 1988.

22. Pranzatelli MR, Carls A, Tkach K. The neuropharmacology of 5-hydroxytryptophan: Chronic effects on central serotonin receptors in the rat. Neurology [abstr] 39: 136, 1989.

23. Tkach K, Pranzatelli MR. The p,p'-DDT epileptic/myoclonic rat model: The role of brainstem and spinal glycine receptors. Neurology [abstr] 39: 116, 1989.

24. Pranzatelli MR, Dailey A, Markush S. TRH and two analogs: Influence on TRH and serotonin receptors in vivo and in vitro in the rat central nervous system. New York Academy of Sciences [abstr] 553: 503-504,1989.

25. Balletti J, Pranzatelli MR. Identification of 5-HT1A receptors in human ganglioneuroblastoma 0but not related tumors. Society for Neuroscience [abstr] 15: 486, 1989.

26. Hlibczuk V, Pranzatelli MR. Effects of neonatal 5,7-dihydroxytryptamine lesions on spinal 5-HT1C receptors. Society for Neuroscience [abstr] 15: 419, 1989.

27. Trifiletti RR, Pranzatelli MR. Central benzodiazepine receptor changes in the p,p'-DDT
myoclonic/epileptic rat hippocampus. Society for Neuroscience [abstr] 15: 1073, 1989.

28. Pranzatelli MR, Hlibczuk V, Dollison AM. Neonatal 5,7-dihydroxytryptamine lesion in the rat: response to selective 5-HT1A and 5-HT2 agonists. Society for Neuroscience [abstr] 15: 487, 1989.

29. Pranzatelli MR, Huang Y, Dollison AM, Stanley M. Brainstem serotonergic hyperinnervation modifies behavioral supersensitivity to 5-hydroxytryptophan in the rat. New York Academy of Science [abstr] 63:1989.

30. Pranzatelli MR, Balletti J, Levy M. Neurotransmitter receptors on human neural crest tumors: A new hypothesis for the opsoclonus-myoclonus syndrome. Annals of Neurology [abstr] 26: 474, 1989.

31. Dailey AT, Pranzatelli MR. The association of opsoclonus, myoclonus, and ataxia: A review of the literature. Child Neurology Society [abstr] 225: 1989.

32. Rosen B, Pranzatelli MR, Dailey AT, Cohen B, Gold AP. Acute infantile dyskinesias: Possible role of theophylline in an asthmatic. Child Neurology Society [abstr] 288: 1989.

33. Nordli DR, Pranzatelli MR, Gold AP. Failure of early therapy of congenital hypothyroidism toprevent neurological abnormality. Child Neurology Society [abstr] 288: 1989.

34. Trifiletti RR, Pranzatelli MR. The peripheral-type benzodiazepine receptor in a series of
neuroectodermal tumors of childhood. Neurology [abstr] 1990.

35. Trifiletti RR, Pranzatelli MR. Influence of ACTH and ACTH fragments on [3H]MK-801 binding to rat hippocampal NMDA receptors. Society for Neuroscience [abstr] 16:785, 1990.

36. Pranzatelli MR. Neonatal 5,7-DHT lesions alter [3H]mesulergine-labeIled 5-HT1C receptors in rat brain. Society for Neuroscience [abstr] 20: 16, 1990.

37. Durkin MM, Pranzatelli MR, Barkai AL, McEwen S, Pedley TA. Autoradiography of 5-HT1A sites in rats with neonatal 5,7-DHT lesions. Society for Neuroscience [abstr] 20: 16, 1990.

38. Pluchino R, Pranzatelli MR. Identification of spinal 5-HT1C binding sites. Society for Neuroscience [abstr] 16: 694, 1990.

39. Pranzatelli MR, Huang Y-y, Stanley M, Tate ED, Noetzel MJ, Lange BM. Cerebrospinal fluid monoamines in opsoclonus-myoclonus. Annals of Neurology [abstr] 30:503, 1991.

40. Pranzatelli MR, Tate ED, Haas R, Bodensteiner J. Hyperprolactinemia as an index of central serotonergic stimulation in patients with myoclonus treated with 5-hydroxy-L-tryptophan. Annals of Neurology [abstr] 30:503, 1991.

41. Ashwal S, Nadi NS, Huang Y-y, Chugani HT, Pranzatelli MR.