1
Movement Disorders
Source: DAMS, The Neurophile on YouTube
                                   Case                                    Table of Contents
 57, M
 (+) history of hypertension and hypothyroidism
 (+) right arm shaking for 2-years
          Onset         first noted in right thumb, then slowly
                         progressed to involve the hand
         Associate      occurs predominantly at rest
         d Factors
         Relieving      improves its movement
         Factors
 (-) changes in walking or balance
 (+) decreased sense of smell
 (+) constipation
 (-) changes in sleep pattern
 Medications: Lisinopril & Levothyroxine
 Overall this is a fairly mild presentation, so why is he in your office
 now?
   The arm shaking is now becoming bothersome.
1
Movement Disorders
                            HYPOKINETIC                                                          HYPOKINETIC
 Parkinsonism
 BRADYKINESIA
   ⁃ slowness in movement, slowness in initiation of movement,
  Arms: starts distally
   ⁃ Decreased manual dexterity of the fingers
   ⁃ Difficulty with tasks that require precision
          • Buttoning buttons
          • Typing shoelaces
          • Grasping pills
  Legs: foot dexterity decreased
     o Festinating Gait
          • Drag their legs when walking
          • Shorter steps
     o Difficulty in standing up
     o Getting out of the car
  When walking: less arm swinging
  Face:
     o Masked face (hypomimia)
     o Delayed nasal speech
     o Monotonous speech (soft low pitched voice, whispering)
                                  Parkinsonism
                                                                 1. BRADYKINESIA
                                                                    ⁃ slowness in movement, slowness in
                                                                      initiation of movement,
                                                                  Arms: starts distally
                                                                    ⁃ Decreased manual dexterity of the fingers
                                                                    ⁃ Difficulty with tasks that require precision
                                                                           • Buttoning buttons
                                                                           • Typing shoelaces
                                                                           • Grasping pills
                                                                  Legs: foot dexterity decreased
                                                                      o Festinating Gait
                                                                           • Drag their legs when walking
                                                                           • Shorter steps
                                                                      o Difficulty in standing up
                                                                      o Getting out of the car
                                                                  When walking: less arm swinging
                                                                  Face:
                                                                      o Masked face (hypomimia)
                                                                      o Delayed nasal speech
                                                                 Monotonous speech (soft low pitched voice,
                                                                 whispering)
                                                                 2.
                                                                                                                     Globus pallidus
                                                                 3.                                                  G-A-P
2
Movement Disorders
    1.   Athetosis                         Slow
         a.k.a. Hammond’s Disease          Sinous                               distal limb
                                           Writhing
                                        (crawling, snake-like movement)
    2.   Chorea (dance-like movement)   Non-rhythmic                                                                                                            Caudate
         a.k.a. St. Vitus Dance         Semi purposeful                                                                                                         Nucleus
                                        ETIOLOGY
                                          S    Sydenham chorea
                                          V    Vascular (Stroke)
                                          I    Increased RBC (Polycythemia
                                          T    Toxins (CO, Mn, Hg)
                                          U    Uremia
                                          S    SLE - MCC
                                               senile
                                          D    Drugs: AED (phenytoin, CBZ), levodopa, Lithium
                                          A    APLA Syndrome
                                          N    Neuroacanthocytosis
                                                   - AR
                                                   - peripheral smear: acanthocytes
                                                   - self-mutilating
                                                   - rubber man gait
                                                   - dystonia: sustained contraction of the leg
                                          C    Conception
                                                   - pregnancy: chorea gravidarum
                                                   - OCPs
                                          E    Endocrine
                                                   - Hyperthyroidism
                                                   - Hypo/hyperglycemia (transient chorea)
    3.   Hemiballismus                  Large amplitude flinging movement of the proximal limb                                                                  Subthala
                                        Usually unilateral                                                                                                      mic
                                                                                                                                                                nucleus
                                                                                                                                                                (contralate
                                                                                                                                                                ral lesion)
    4.   0                              neurodegeneration/ atrophy of the substantia nigra (hypopigmented)
                                        ETIOLOGY:
                                              1.  Drugs (mcc secondary parkinsonism)
                                                    DA antagonists
                                                         Typical Antipsychotics
                                                         Metoclopramide
                                                    DA depleters
                                                         Methyldopa
                                                         Tetrabenzine
                                                         CCB: Flunarazine, Cinnazarine
                                                         Amiodarone
                                                         Lithium
                                              2.  Genetic/Familial (5-15%)
                                                                      AD                          AR
                                                                      Classical PD
                                                       Genes          LRRK-2 (chr 12)             Parkin (chr 6)
                                                       mutated        ⍺ synuclein (chr 4)         PINK-1
                                                                                                  DJ-1
                                                          Age of         >40 years          <40 years
                                                          onset
                                               3.    Toxins – CO, Manganese, Heroi
                                               4.    Trauma
                                               5.    Idiopathic (85-90%) = PARKINSON’S DISEASE aka Paralysis agitans, Kampa Vatain Sanskrit (shaking tremors)
 Parkinson’s Disease                                                                                                                                            Substanti
                                          T      Tremors                                                 MC                                                     a nigra
                                                                                                         sympto
                                                                                                         m
                                                                                                         (85%)
                                                                                                         4-6 Hz
                                                                                                         resting
                                                                                                         (upper
                                                                                                         limb >
                                                                                                         LL)
                                          R      Rigidity
                                          A      Akinesia
3
Movement Disorders
                                                                                         P         Postural instability – common to falls                 Loss of
                                                                                                                                                          postural
                                                                                                                                                          reflexes
                                                                                                                                                          Stoope
                                                                                                                                                          d
                                                                                                                                                          posture
                                                                                                                                                          cannot
                                                                                                                                                          maintai
                                                                                                                                                          n the
                                                                                                                                                          spine
                                                                                                                                                          and
                                                                                         F         Festinating Gait
                                                                                         g         Kinesia paradoxa (increased acceleration when
                                                                                         K         running)
                                                                                         d
                                                                                         M         Micrographia
                                                                                         S         Monotonous speech (soft, low pitched voice
                                                                                                   ~whispering )
                                                                                         M         Mask-like face (aka hypomimia)
                                                                                         M         Meyerson sign (glabellar tap sign)
                                                                                                   sustained blinking after 5 taps on forehead
                                                                                         D         Depression
                                                                                         D         Dementia (usually after 1 year of onset)
                                                                                         A         Anosmia
                                                                                       1st (earliest) = anosmia
                                                                                       Last to happen = postural instability
                                                                                       Onset = asymmetrical but later it can be bilateral
                                                                                               
      If onset is bilateral = Atypical PD (PK+)
            1.        Progressive Supranuclear Palsy (PSP) aka Steel
                      Richardson Syndrome
                         PSPP form – resembles PK in early stages, responds to
                         levodopa
                         PSPR form (classical) – extended posture, defective
                         downward gaze, falls down easily, dementia, (-) tremors,
                         Gunslinger’s gait (flexed elbow, thus less arm swinging
                         while walking), omega sign (forehead muscle dystonia) or
                         procerus sign (vertical wrinkling due to sustained procerus
                         contraction)
 2.        3.     Dementia of Lewy Body                                                MC                                                                                                                                   NOT Only
             PK + Visual Hallucinations + Dementia (<1 year of onset)                                                                                                                                                       substranti
                                                                                                                                                                                                                            a nigra
                                                                                                                                                                                                                            but also
                                                                                                                                                                                                                            autonomi
                                                                                                                                                                                                                            c nervous
                                                                                                                                                                                                                            system or
                                                                                                                                                                                                                            cerebellu
                                                                                                                                                                                                                            m
 4.        5.       Multiple System Atrophy (MSA)                                      (+) Pisa sign – l;ateral bending of the spine
       MSAp (MORE COMMON)
        Autonomic instability
       (-) trmonrs
       Sym,metrical onset
       MSAC (cerebellar)
       Cerebellar symptoms + PK
 6.          7.    Coricobasilar Degeneration                                          Least common
      Similar to PK
      Asymmetrical onset
      Dystonia
      Alien limb phenomena unintended motor acitons like graspinfg, drifting of
      hand
      Myoclonus
 Treatment
 Problems :
 Decrease in Dopamine
 Increase in Acetylcholine
                                                                                       Decrease in Dopamine
                                                                                               1)        Levodopa
                                                                                                         MOA strictly speaking, Targets Rigidity & Bradykinesia
                                                                                                         but because of the enzyme decarbooxtylase, there will be Peripheral conversion ∫thus persistence of rigidity and
                                                                                                         bradykinesia)
4
Movement Disorders
                            Wearing of. effect or ON-OFF phenomenon
                            Thus
                             + Carbidopa/Benseraside
                     2)      Amantandine
                             Increases levels
                     3)       Dopamine Agonists
                     ( Pramipraxole
                     Ropinorole
                     Rotigotin
                     4)      Apomorphine (non-opioid) injectibkle
5
Movement Disorders
                                                            TREMORS                                                                                               RIGIDITY
                                                                                                                               - resistance to passive movement
                                                                                                                               - resistance between the flexors & extensors are the same (bidirectional)
                                                                                                                                                            BEST JOINT: wrist joint
 Resting Tremors                -   MC symptom (85%) in PD                                      Parkinson’s Disease          Lead pipe rigidity                         Extrapyramidal syndrome
                                -   4-6 Hz
                                -   Affects upper limb > LL
 Intention tremor                                                                               Cerebellar lesions           Cog wheel rigidity                           Parkinson’s Disease
                                                                                                                                                                                 UL = cogwheel
                                                                                                                                                                                 LL = leadpipe, because
                                                                                                                                                                                  itremors are more prominent
                                                                                                                                                                                  in UL
 Flapping tremors               negative = Drop of tone myoclonus = jerky                       Hepatic encephalopathy      Clasp knife rigidity                         UMN lesion
   a.k.a. asterixis, negative                                                                   Uremic encepalothtty        (spasticity)
 myoclonus                                                                                      CO2 narcosis (respiratory     - resistance of the flexors > extensors
                                                                                                   encephalopathy)               (unidirectional)
                                                                                                                               - velocity dependent
 Fine tremors                                                                                   Thyrotoxicosis
 Essential tremors              -   MC movement disorder affecting elderly and young age
                                -   Age onset at 70
                                -   6-10 Hz
                                -   Bilateral
                                -   Symmetrical
                                -   Kept on shaking
                                -   UL > head > voice/tongue>LL
                                -   50% multiple sites
                                -   Autosomal dominant inheritance
                                -   Increased in:
                                               stress and anxiety
                                               alcohol
                                -   Tx: beta blocker
                                               Propranolol [DOC]
                                               Primidone
                                -   If no response to medical tx -- > deep brain stimulation
                                    (DBS) for ventro intermediate nucleus of thalamus (VIN)