MOVEMENT DISORDER Spartan a better view on the disorders
- 1. MOVEMENT DISORDERS BASAL GANGLIA/ NUCLEI Ganglia: cell bodies in the PNS Nuclei: cell bodies in the CNS Grey Matter - Consists of cell bodies - Color grey - d/t presence of Nissel Bodies (cc synthesizes proteins) White Matter - Consists of axons - Color white - d/t presence of Myelin Sheath (cc contains lipids) COMPOSITION OF BASAL GANGLIA: Corpus Striatum Codate Nucleus Subthalamic Nucleus Caudate Nucleus Neo Striatum CORPUS STRIATUM Putamen Lentiform Nucleus Globus Pallidus Putamen GLOBUS PALLIDUS PUTAMEN Smaller Larger Lighter Darker Medially Laterally MAIN FXN OF BASAL NUCLEI IS TIMING & SCALING OF MOVEMENT NEUROTRANSMITTERS INHIBITORY - Corpus Striatum: Dopamine - Cortex: GABA
- 2. EXCITATORY - Cortex to Corpus Striatum: Glutamate - Striatum: Acetylcholine ↓ Dopamine; ↑ Acetylcholine: Parkinsonism ↑Dopamine; ↓ Acetylcholine: Chorea (Sa Korea (Chorea) hindi lng maraming oppa maramin din DOPA) PATHWAYS DIRECT PATHWAY: FASCILITATORY INDIRECT PATHWAY: INHIBITATORY Over active IP Akinesia & rigidity ↓ Dopamine Under active DP Bradykinesia CORTEX STRIATUM (Afferent) INDIRECT PATHWAY GLOBUS PALLIDUS EXTERNUS THALAMUS GLOBUS PALLIDUS + SUBSTANTIA NIGRA PARS RETICULATA (Efferent) SUBTHALAMIC NUCLEUS DIRECT PATHWAY
- 3. PARKINSONISM 1◦/ IDIOPATHIC - Tremor (predominant sign) - Postural instability - Gait deviations - Disturbed group Late onset: > 40 y/o Early onset: < 40 y/o Young: > 21 y/o Juvenile: < 21 y/o PATHOGENESIS - Degeneration of Dopaminergic neurons in SNPC (Substantia Nigra Pars Compacta) - (-) Levy Bodies (Late) - 30% - 60% degeneration of neurons (clinical signs appear) CARDINAL SIGNS 1. Rigidity 2. Tremor 3. Postural instability 4. Bradykinesia RIGIDITY - Resistance to PROM, is non-velocity dependant (heaviness or stiffness) Types: 1. Lead-pipe: Resistance is constant all throughout the ROM. 2. Cogwheel: ratchet like. Only present is certain range. RIGIDITY (extra pyramidal) SPASTICITY Non – velocity dependant Velocity dependant Fast (+); Slow (+) Fast (+); Slow (-) Alpha (extrafusal mm fibers) Gamma (intrafusal mm spindles) R igid A lpha A lpha E xtrafusal mm fibers G amma G amma S pastic I ntrafusal mm S pindle
- 4. Basal ganglia: Resting TREMOR Cerebellar: Intention tremor - Involuntary oscillatory movements of body parts Frequency: 4 – 6 hz or 5 – 7 hz Relived by relaxation & unoccupancy feeling Aggravated by emotions, fatigue & stress M/c presenting sign M/c initial symptom Co-related c “Pill – rolling”: Index & thumb Tremor in LE is seen in supine BRADYKINESIA - Akinesia: Poverty of spontaneous movement - Hypokinesia: Reduced amplitude of movement CN affectation: CN3 CN7 CN9 CN7 - Facial mm - Hypomemia CN9 - Glossopharyngeal nerve - Hypokinetic speech/ Dysarthria - Siallorhea POSTURAL INSTABILTY - Impaired righting reflex (Balance) - Unstable BOS - Abnormal posture (Simian Posture) Forward head Cervical flexion & capital extension Protracted Sh ↑ Kyphosis ↓ Lordosis Striated Hand - Wrist flexion - Ulnar deviation - CCP flexion
- 5. - IP extension Striated Foot - DF - Inversion - Big toe extension Sitting Enbloc - Upon attempt to sit, pt collapse Freezing Phenomenon - Sudden stopping or sudden freezing upon turning or in contact of something or confrontation of an obstacle. ↑ Risk of falling - Anteriorly: Anteropulsion - Laterally: Lateropulsion - Posteriorly: Retropulsion POSTURAL INSTABILITY is the MOST DISABLING sign CLINICAL D/O MOTOR Gait - Festinating: ↑ speed c small step - Shuffling Gait: ↓ heel strike to foot flat mechanism Kinesia paradoxical - Pt has ability to perform rapid movement during range of emotional energy Micrographia - Small crammed hand writing Poverty of movement - Overall reduction in total amplitude of movement Myerson Sign - Glabellar tap reflex (Bridge of the nose) Stimulus: rep. tapping of glabellar Reflex: sustained eye blinking AUTONOMIC D/O - Orthostatic hypotension - GT immotility (m/c autonomic dysfxn)
- 6. - Constipation - Seborrhea (↑ production of sebum) - Urinary incontinence - Erectile dysfxn RESPERATORY D/O - Airway obstruction (RLD d/t weakness of posture) m/c respiratory d/o SPEECH & SWALLOWING D/O - Disarthria: Diff in articulation - Dysphagia - Mutism - Hypolinetic Speech (CN9) Monotone ↓ Volume Low tone Imprecise articulation Hoarseness - Siallorhea SENSORY - Postural Stress Syndrome (mm pain and jt pain d/t postural and mm imbalance) - Akathisia: feeling of inner restlessness and inability to stay still - Anosmia - Loss of proprioception COGNITIVE & PSYCHIATRIC D/O - Depression - Anxiety - Bradyphrenia: Slowness in thought process and pattern SLEEP D/O - Excessive daytime Somnolence - Insomnia MOST COMMON SYMPTOM REPORTED IN PD? _ TREMOR MOST CAUSE OF PAIN IN PD _ LIMB RIGIDITY MOST COMMON DEFORMITY IN PD _ KYPHOSIS
- 7. PROGNOSIS GOOD - Early tremor - Rigidity - FHx POOR Be A Good Citizen of PhiL - B radykinesia - A kinis - G ait dev - C og. Dysfxn - P ostural instability - L ate onset DIAGNOSIS - Hx, speech, interview, handwriting - 2/4 cardinal sign - Radiologic results - Apomorphine test (To exclude P+) Same sy/sx but diff parts of the brain (+) improvement: PD Take Levadopa (-) improvement: P+ HOEN & YAHR 1 (mild) – 5 (sever) I. Minimal, uinilateral involvement II. Bilateral involvement III. Impaired righting reflex, work affectation IV. All sx are present, standing & amb possible only c assist V. Bed, w/c confinement BEST STRETCHING TECHNIQUE _ CONTRACT, RELAX MOTOR LEARNING STRATEGIES Blocked practice - Same environment
- 8. Visual cues - Floor marks Rhythmic Auditory Stimulation - Metronome beat/ musical beat - Improve gait pattern PNF TECHNIQUES Ryhtmic Stretch: Trunk rotation Rhythmic Initiation: Improve initiation Rhythmic Rotation: Relaxation and stretching technique Pattern: D2 flexion (UE) D1 extension (LE) AMBULATION WEDGE - Toe: anteropulsive - Heel: retropulsive AD - Rolling walker: difficulty in initiation - Standard walker: difficulty in termination PHARMACOLOGY On : Off Phenomenon - Fluctuation in motor performance, worsening of pt’s condition towards the end of the expected timeframe of effectiveness - End of dose deterioration Honeymoon Period - Dramatic initial fxnal improvement SINEMET - Levadopa + carbidopa - MOA: activation of D1 & D2 receptors Carbidopa inhibits Dopamine decarboxylase found in the BBB - Intake at AM 1hr before exercise
- 9. ANTICHOLINERGIC - Restore balance between dopamine and Acetylcholine - Example: Trihexyphenidyl Benztopine DIPAMINE AGONIST - MOA: Stimulates D1 & D2 receptors - Example: Bromocriptine Pergolide AMANTADINE - Antiviral drug - MOA: Promotes dopasynthesis & re-uptake MOA – B INHIBITORS (Mono Amine Oxidase) - Example: Selegiline C – deprenyl - Considered as neuroprotective agent Early stage Delays progression Delays transition to L-dopa tx by 9 mons S elegiline A matadine L evadopa A nticholinergic D opa agonist SURGICAL Mx - Respond poorly to meds - Experience complications to meds 2◦ PARKINSONISM TeaM PD - T oxic - M etabolic - P ost infectious - D rug Induced
- 10. TOXIC - Pesticides - Industrial chem Manganese (m/c) Methanol Methylphenyltetrahydropiridine (NPTP) Carbon disulphide Cuanide METABOLIC - Wilson’s dse - Hypo/ Hyperparathyroidism (Ca accumulation) - Hyperthyroidism POST INFECTIOUS - Influenza - Encephalitis lethargica DRUG INDUCED - Neuroleptic - Anti-depressants - Anti-Htn PARKINSON’S PLUS (P+) SHY – DRAGERS DSE - Autonomic - (+) Tremor - Dsfxn of bladder & bowel - Abn BP, HR & RR WILSON’S DSE - Hypotenticular degen - Copper - EP sign - Tremor - Rigidity - Dystonic HUNTINGTON’S DSE - Autonomal dominant - (+) primitive reflex - (+) hypotonia striatum
- 11. A dolesence B ehavioral C horea D ementia TRIAD E nd (&) F amily Hx G rasp reflex H ypotonia PROGRESSIVE SUPRANUCLEAR PULSY (Surprise Nuc) - Contraction of frontalis - Eyes open - Difficulty in eyes closing - Nek dystonia - Vertical eye movement abnormality - Common in 7th decade MOVEMENT D/Os PD Putamen: Dystonia SC Striatum: Chorea GA Globus Palidus: Athetosis SH Subthalamus: Hemibalismus DYSTONIA - Sustained mm contraction that causes the limb to twist repetitively leading to abn posture 1. Focal - Single body part 2. Segmental - 1 or more contiguous body segment 3. Multifocal - 2 or more contiguous body segment
- 12. Cranial Dystonia - Jaw opening Tongue protrusion Platysma Cxn - Most common form of cranio-cervical dystonia - It also presents as: Tongue chewing Tongue curling Blapherospasm Cervical Dystonia - Most common form of focal dystonia - SCM (Torticolis) - Characteristics: rep spasmodic Limb Dystonia - Writers - Occupationals - Graphospasm - Characteristics: Abn pattern of hand and FA - Pattern: Hyper flexion & hyper extension of wrist and finger GUILLES TOURRRET SYNDROME Corrolalia: abscene words Copopraxia: obscene actions - Head & Sh thrashing - Frowning - Eye blinking - Hisses, barks & coughs CHOREA Characteristics: - Rapid, brief, jerky movements - Affected part (distal) - Lesion in striatum - Common in SLE, Syndenhams, ST vitus dance, RHD RHD → jt pain → Heart Dse Dr. Jones Ch → Chorea
- 13. ATHETOSIS - Slow, writhing, worm like movement - GP - Willson’s Dse & CP HEMIBALISMUS - Wild, flailing, violent - Proximal body - Subthalamus TICS - Intermittent MYOCLONUS - Thunder like, sudden movement, brief in presentation - Common in seizure & epileptic attack ASTEREXIS - AKA: Liver Clap - Asynchronized twist - Clonus of the UMNL