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DIFFERENTIATING MOVEMENT DISORDERS. B. WAYNE BLOUNT, MD, MPH PROFESSOR, EMORY S.O.M. QUESTION # 1. WHICH OF THE BELOW ARE FEATURES TO USE IN DIFFERENTIATING MOVEMENT DISORDERS? A. WHETHER OR NOT THE MOVEMENTS ARE HYPOKINETIC VS. HYPERKINETIC B. PRESENCE OF A TREMOR C. TYPE GAIT
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DIFFERENTIATING MOVEMENT DISORDERS B. WAYNE BLOUNT, MD, MPH PROFESSOR, EMORY S.O.M.
QUESTION # 1 • WHICH OF THE BELOW ARE FEATURES TO USE IN DIFFERENTIATING MOVEMENT DISORDERS? • A. WHETHER OR NOT THE MOVEMENTS ARE HYPOKINETIC VS. HYPERKINETIC • B. PRESENCE OF A TREMOR • C. TYPE GAIT • D. COGWHEEL VS PLASTIC RIGIDITY
ROADMAP • AN APPROACH TO DIFFERENTIATION • EVALUATION • THE TYPES: • HYPOKINETIC • HYPERKINETIC • PARKINSON’S
EVALUATION • HISTORY & PHYSICAL : • DETERMINE HYPO- OR HYPER- KINETIC • DETERMINE WHICH COMPONENTS OF MOTOR CONTROL ARE AFFECTED • CLASSIFY BODY PART, ACTIVATION CONDITION, FREQUENCY AND AMPLITUDE • CLUSTER SIGNS & SX TO DETERMINE DX
TYPES OF MOVEMENT DISORDERS • HYPOKINETIC • HYPERKINETIC
HYPOKINETIC CHARACTERISTICS • PARKINSONIAN APPEARANCE • DIMINISHED INITIATION • SLOWED EXECUTION • RIGIDITY • MAY HAVE RESTING TREMOR
QUESTION # 2 WHICH OF THE FOLLOWING ARE HYPOKINETIC DISORDERS? • A. PARKINSON’S • B. AIDS • C. HUNTINGTON’S • D. TARDIVE DYSKINESIA
HYPOKINETIC DISORDERS • PARKINSONISM • PROGRESSIVE SUPRANUCLEAR PALSY • LACUNAR STATE • TOXIC • HYPOTHYROID • HYPOPARATHYROID • SHY-DRAGER • TAURINE DEFICIENCY
HYPERKINETIC CHARACTERISTICS • INCREASED MOVEMENTS • DYSTONIA • ATHETOSIS • CHOREA • DYSKINESIA • TICS • TREMOR • MYOCLONUS
HYPERKINETIC DISORDERS (CHOREA) • HUNTINGTON’S • AIDS • TARDIVE DYSKINESIA • HYPERTHYROIDISM • DRUG-INDUCED • POST-HEMIPLEGIC CHOREOATHETOSIS
BALLISMUS • INVOL JERKING & FLINGING OF PROXIMAL MUSCLES • STROKE • TRAUMA • MULTIPLE SCLEROSIS • INFECTIONS
DYSTONIA • ABN TONE ANYWHERE; IMPAIRED MOVEMENT • MEIGE’S SYNDROME • BLEPHAROSPASM, OROMANDIBULAR DYSTONIA • TORTICOLLIS • TARDIVE DYSTONIA • HYPOCALCEMIA
MYOCLONUS • BRIEF, LIGHTNING-LIKE CTX OF A MUSCLE • JAKOB-CREUTZFELDT • ALZHEIMER’S AIDS • ANOXIA • TOXIC-METABOLIC ENCEPHALOPATHY
TICS • BRIEF, RAPID, INVOL MOVES; STEREOTYPICAL & REPETITIVE • IDIOPATHIC CHRONIC MOTOR TIC • CARBON MONOXIDE PSN-ING • TARDIVE DYSKINESIA WITH TICS
TREMOR • REPETITIVE, REGULAR OSCILLATORY MOVES; IRREG CTX OF OPPOSING MUSCLES, USU INVOL • EXAGGERATED PHYSIOLOGIC TREMOR • ESSENTIAL TREMOR • PARKINSONIAN TREMOR • CEREBELLAR TREMOR
CHOREA & ATHETOSIS • CHOREA: • BRIEF, PURPOSELESS, INVOLUNTARY MOVEMENTS OF EXTREMITIES AND FACE • ATHETOSIS: • WRITHING MOVEMENTS
QUESTION # 3 FOR WHICH OF THE FOLLOWING DO WE KNOW THE PATHOPHYSIOLOGY? • A. TARDIVE DYSKINESIA • B. HUNTINGTON’S CHOREA • C. SHY DRAGER • D. NONE OF THE ABOVE
PATHOPHYSIOLOGY • PARKINSON’S : DOPAMINE DEPLETION IN SUBSTANTIA NIGRA; LEWY BODIES • PROGRESSIVE SUPRANUCLEAR PALSY CELL LOSS, GLIOSIS & NEUROFIBRILLARY TANGLES IN MESENCEPHALIC-DIENCEPHALIC JXN • LACUNAR STATE : NECROSIS/OCCLUSION OF ARTERIES IN CAUDATE, PUTAMINE, GLOBUS, THALAMUS & INT. CAPSULE
PATHOPHYSIOLOGY • HUNTINGTON’S : NEURONAL LOSS OF CAUDATE & PUTAMEN; DEPLETED GAMMA- AMINOBUTYRIC ACID • TARDIVE DYSKINESIA : AFTER > 3 MO EXPOSURE TO NEUROLEPTIC AGENT • Now have 1 FDA approved drug for Huntington’s : tetrabenazine (8/08)
QUESTION # 4 WHICH OF THE FOLLOWING IS 1 OF THE CLASSIC TRIAD FOR PARKINSON’S • A. RESTING TREMOR • B. ACTION TREMOR • C. COGWHEEL REFLEXES • D. SHUFFLING GAIT
PARKINSON’S • MEAN AGE @ ONSET = 58-62 • HIGHEST PREVALENCE IN 70’S • INSIDIOUS ONSET • CLASSIC TRIAD : • BRADYKINESIA • RIGIDITY • RESTING TREMOR • (POSTURAL INSTABILITY)
Question # 5 • True or False ? Early PD has little or no motor complaints. • A. True • B. False
EARLY PD • MUSCLE WEAKNESS • DYSTONIA • ANXIETY • INSOMNIA • LITTLE C/0 MOVEMENT DISORDER • MOTOR PROBLEMS ON P.E.
PARKINSON’S BRADYKINESIA • FACIAL HYPOMIMIA (APATHY LOOK) • LONG LATENCY RESPONSES • SLOW, SHUFFLING GAIT • DIMINISHED ARM SWING • EN BLOC TURNS • DROOLING • MICROGRAPHIA • HYPOPHONIC SPEECH
Question # 6 • The rigidity in Parkinson’s is different between the upper extremities and the lower extremities. • A. True • B. False
PARKINSON’S RIGIDITY • COGWHEEL TYPE IN UPPER LIMBS • PLASTIC HYPERTONICITY IN LEGS
PARKINSON’S TREMOR • RESTING • DISAPPEARS WITH ACTION • ALTERNATING FLEXION & EXTENSION MOVEMENTS OF THE FINGERS AND WRISTS • “PILL-ROLLING”
OTHER COMMON PD SIGNS • FORWARD FLEXION OF THE NECK • FLEXION OF LIMBS • AUTONOMIC DYSFUNCTION : • ORTHOSTASIS, IMPOTENCE • DEMENTIA, DEPRESSION • SLEEP & SWALLOWING DISTURBANCE • FATIGUE
PD EXAM • MENTAL STATUS • CRANIAL NERVES • SENSATION • MOTOR : • TONE POSTURE • STRENGTH GAIT • REFLEXES KINESIS • COORDINATION
QUESTION # 7 TO MAKE THE DIAGNOSIS OF PARKINSON’S, YOU NEED: • A. 1 OF 3 CARDINAL SIGNS + 2 LESSER SIGNS • B. 2 OF THE 3 CARDINAL SIGNS • C. 3 OF THE 3 CARDINAL SIGNS • D. 2 OF THE 3 CARDINAL SIGNS + 2 LESSER SIGNS
PD DIAGNOSIS • > 2 OF 3 CARDINAL SIGNS • ABSENCE OF 2ND-ARY CAUSE
PD WORK-UP • CT OR MRI • IF CLASSIC FEATURES PRESENT, LITTLE ELSE NECESSARY
PD DIFFERENTIAL • OTHER PARKINSONIAN DISORDERS ; • PROGRESSIVE SUPRANUCLEAR PALSY • SHY DRAGER • LACUNAR STATE • TOXIC • DEPRESSION • METABOLIC : • HYPOTHYROID • HYPOPARATHYROID
PD TREATMENT • RELIEVE SYMPTOMS • PREVENT COMPLICATIONS • SLOW DISEASE PROGRESSION (THEORETICAL)
PD PHARMACOTHERAPY STIMULATE DOPAMINE RECEPTORS • INHIBIT DOPAMINE METABOLISM • ANTI- HISTAMINES/CHOLINERGICS • INCREASE DOPAMINE LEVELS
QUESTION # 8 WHICH OF THE FOLLOWING IS THE IST LINE TREATMENT FOR PARKINSON’S • STIMULATE DOPAMINE RECEPTORS B. INHIBIT DOPAMINE METABOLISM C. ANTI- HISTAMINES/CHOLINERGICS D. INCREASES DOPAMINE LEVELS
INCREASE DOPAMINE LEVELS • LEVODOPA-CARBIDOPA (SINEMET) • AMANTADINE (SYMMETREL)
INCREASE DOPAMINE LEVELS • LEVODOPA IS DOPAMINE PRECURSOR • CARBIDOPA BLOCKS PERIPHERAL CONVERSION • THE MOST EFFICACIOUS TREATMENT • FREQUENT DOSING • DON’T USE IN GLAUCOMA • LONG USE : “ON-OFF”, “WEAR OFF” • AMANTADINE IS SYNERGISTIC WITH LEVODOPA
STIMULATE DOPAMINE RECEPTORS • BROMOCRIPTINE (PARLODEL) • PERGOLIDE (PERMAX) • PRAMIPEXOLE (MIRAPEX) • ROPINIROLE (REQUIP)
STIMULATE RECEPTORS • ADJUNCT TO LEVODOPA • START WHEN LEVODOPA IS LOW-TO- MEDIUM DOSAGE ( < 600 MG/DAY) • AS MONOTHERAPY, THEY MAY NOT PROVIDE ADEQUATE IMPROVEMENT. • PTs NOT RESPONSIVE TO LEVODOPA UNLIKELY TO BE TO RECEPTOR AGONISTS • 1ST 2 ON SLIDE : RETROPERITONEAL & PULMONARY FIBROSIS
INHIBIT DOPAMINE METABOLISM • SELEGILINE (ELDEPRYL) MAO INHIBITOR • TOLCAPONE (TASMAR) COMT : CATECHOL O-METHYLTRANSFERASE INHIBITOR • ENTACAPONE COMT
INHIBIT METABOLISM • PREVENTS BREAKDOWN OF DOPAMINE & ALLOWS MORE TO REACH CNS • ADJUNCTS TO LEVODOPA, ESPECIALLY IN PTs WITH SX FLUCTUATIONS OR DO NOT RESPOND TO OTHER RX
OTHERS DIPHENHYDRAMINE (BENADRYL) • TRIHEXYPHENIDYL (ARTANE) • BENZTROPINE (COGENTIN) • ? BOTULINUM TOXIN ? • ADJUNCTIVE RX TO LEVODOPA, ESP FOR TREMOR • DIFFICULT IN OLDER PTs • BEST IN DRUG-INDUCED PARKINSONISM
Question # 9 • Which of the following is the preferred surgical technique for Parkinson’s? • A. Pallidotomy • B. Deep Brain Stimulation • C. Thalamotomy
SURGERY FOR PD THALAMOTOMY PALLIDOTOMY • DEEP BRAIN STIMULATION (DBS) • HAS REPLACED THE –OTOMIES • May increase suicide risk, esp in those already depressed
HOW TO SELECT MEDS • EACH PT NEEDS INDIVIDUAL RX • CONSIDER AGE, OCCUPATION & LIFESTYLE • CONSIDER FUNCTIONAL IMPAIRMENT & WHICH SX IS MOST BOTHERSOME • CONSIDER EFFICACY & SIDE EFFECTS
THERAPY : MOTOR SX • START EARLY • LEVODOPA/CARBIDOPA FIRST: CR FORMULATION • THEN ADD COMT • THEN DOPAMINE AGONIST • MAO-B INHIBITOR • DBS
NON-MOTOR SX • PSYCHOSIS : ATYPICAL ANTIPSYCHOTIC • ORTHOSTASIS : STOCKINGS; FLUDROCORTISONE • CONSTIPATION : BOWEL HYGIENE
OTHER TREATMENTS • NUTRITION • NORMAL PROTEIN • HIGH FIBER • VITAMINS OK, BUT NO SPEC. BENEFIT • EXERCISE can improve motor fxn • PHYSICAL & OCC THERAPY • No Treatment is neuroprotective
BIBLIOGRAPHY • CHARLES PD, ESPER GJ ET AL. CLASSIFICATION OF TREMOR & UPDATE ON TREATMENT. AFP MAR 15, 1999;59. • SMAGA S. TREMOR. AFP OCT 15, 2003; #8. • YOUNG R. UPDATE ON PARKINSON’S DISEASE. AFP APR 15, 1999; #8. • BAGHERI M, ET AL. RECOGNITION & MANAGEMENT OF TOURETTE’S SYNDROME & TIC DISORDERS. AFP APR 15, 1999; #8. • Dewey RB. Management of motor disorders in PD. Neurology 2004;62:S3-S7.