Parkinsons Disease ICD-9CM Code: 332.0: Idiopathic Parkinsons Disease,Primary 332.

1: Parkinsons Disease, Secondary

1. Definition

A. Is A Degenerative Disorder Involving The Dopamine Secreting Neurons Of The Substantia Nigra & Locus Ceruleus 2. Pathophysiology: A. The Disease Involves A Lack of Dopamine In These Regions Which Send Their Impulses To The Basal Ganglia. 3. Epidemiology: A. Is A Disease Of Adulthood With Most Cases Manifested By The Sixth Decade: Usually Occurs Between 45-65 Years Of Age B. Men/Women 1/1 C. There Are Familial & Sporadic Causes For The Disease D. Mutations In A Gene Coding For A Protein Involved In The Neuronal Synapse Termed Alpha-Synuclein Account For Some Of The Familial Cases 4. Causes: A. Sporadic B. Genetic: 1. Autosomal Dominant: A. Alpha-Synuclein Gene Mutation B. Duplications C. Triplications D. LRRK2 Mutations 2. Autosomal Recessive A. Parkin B. DJ-1 C. PINK-1

C. Secondary Parkinsonism: I. Neurodegenerative Diseases (Sporadic or Genetic) A. Progressive Supranuclear Palsy B. Multiple-System Atrophy C. Corticobasal Degeneration D. Dementia With Lewy Bodies E. Alzhemiers Disease F. ALS-Parkinsonism-Dementia Complex Of Guam G. Huntingtons Disease H. Neuroacanthocytosis I. Spinocerebellar Ataxias (eg. SCA-3, SCA-2) J. Wilsons Disease K. Pantothenate Kinase-Associated Neurodegeneration ( Hallervorden Spatz Disease) L. Neuroferritinopathy M. Fahrs Disease ( Calcification of the Basal Ganglia) N. Dopa-Responsive Dystonia II. Drugs: A. Neuroleptics B. Metoclopramide C. Prochlorperazine D. Tetrabenazine E. Reserpine F. Cinnarizine G. Flunarazine H. Alpha-Methyldopa I. Lithium

Toxic: A. MPTP B. Manganese C. Carbon Monoxide D. Mercury III. Infectious: A. Encephalitis Lethargica B. Other Encephalitis: (Including HIV) C. Subacute Sclerosing Panencephalitis D. Creutzfeldt-Jakob Disease IV. Vascular: A. Atherosclerosis B. Amyloid Angiopathy V. Neoplastic: A. BrainTumor B. Other Mass Lesion VI. Others: A. Head Trauma B. Multiple Sclerosis 5. Pathology: A. Loss Of Melanin Containing Neurons In The Locus Ceruleus, Substantia Nigra & Dorsal Motor Nucleus Of The Vagal Nerve.

6. Clinical Features: 1. Rigidity 2. Masked (Expressionless) Facies 3. Stooped Posture 4. Festinating Shuffling Gait 5. Bradykinesia: Slowing Of Voluntary Movements 6. Pill Rolling Tremor 7. Autonomic Dysfunction: A. Othostatic Hypotension B. Dysphagia: Difficulty Swallowing C. Sialorrhea: Excess Salivation D. Constipation E. Urinary Bladder Dysfunction F. Sexual Dysfunction G. Sweating Dysfunction:Hyperhidrosis 8. Nighttime Sleep Dysfunction & Daytime Sleepiness 9. Neuropsychological Dysfunction: A. Attention and Executive Deficits B. Language Problems: Hypophonia & Dysarthria C. Visuoperceptual Impairments D. Possible Dementia E. Anxiety, Depression F. Psychosis 7. Clinical Course: 1. Steady Progression Over A Period Of Ten Years 2. Dementia May Occur In A Minority Of Cases 3. Death Is The Result Of Intercurrent Infection Or Trauma From Frequent Falls Caused By Postural Instability

8. Treatment: A. Medical Therapy: 1. Levodopa Therapy: A. Levodopa + Carbidopa: Sinemet 2. MAOI Inhibitors: Selegiline (Eldepryl) & Rasagiline 3. Dopamine Receptor Agonists: A. Ropinirole,Pramipexole, Pergolide or Bromocryptine Apomorphine, L-Dopa 4. Amantadine: Augments Release & Prevents Reuptake of Dopamine 5. Anticholinergic Agents: A. Benztropine (Cogentin) , B. Trihexyphenidyl (Artane) C. Biperiden, D. Ethopropazone Secondary Anti-Cholinergic Agents: A. Benadryl B. Amitriptyline C. Clozapine 6. Catechol 0 Methyl Transferase Inhibitors: A. Entacapone B. Nitecapone C. Tolcapone 7. Anxiety and Depression: A. Anxiolytics: 1. Benzodiazepines 2. Buspirone 3. Mirtazapine 4. Nefazadone 5. SSRIs 6. Trazadone 7. Tricyclic Antidepressants 8. Venlafaxine

B. Antispychotics: 1. Atypical Antipsychotics: A. Olanzapine B. Risperidone C. Quetiapine D. Ziprasidone E. Aripiprazole F. Clozapine 8. Investigational Pharmacological Agents: A. L-Dopa Preparations: 1. Valdova 2. Duodopa 3. L-Dopa Methyl Ester 4. Etilevodopa B. Dopamine Agonists: 1. Ropinirole 24 Hour Release . 2. Rotigotine Patch 3. Lisuride Patch 4. Piribedil 5. SLV308 6. Sumanirole C. MAO Inhibitors: 1. Safinamide: Phase 3 Trials 2. Brasofensone: D. Agents With Novel Mechanisms 1. Istradefylline 2. B11B014/V2006 3. E2007 4. CEP-1347 5. Glial Derived Neurotropic Factor 6. Minocycline 7. Creatine 8. Coenzyme Q10 9. SIB-15084

E. Investigational Agents For Dyskinesia: A. Sarizotan B. Fipamezole C. ACP-103 D. Levetiracetam 3. Deep Brain Stimulation: A. Deep Brain Thalamic Stimulation B. Deep Brain Stimulation Of The Globus Pallidus Interna C. Deep Brain Stimulation Of The Subthalamic Nucleus D. Pallidotomy: Unilateral Or Bilateral E. Thalamotomy: Unilateral Or Bilateral 4. Investigational: Intracerebral Drug Infusion: A. Glial Cell Line Derived Neurotropic Factor: 1. Promotes Survival Of Dopaminergic & Motor Neurons 2. Neurturin: GDNF Analog 5. Investigational Therapies: A. Stem Cell Therapy B. Fetal Transplantation C. Gene Therapy 6. Behavioral Speech, Voice and Swallowing Treatment