Movement disordersMovement disorders

• Disturbance in speed or fluency of voluntary movement

or• the presence of unintended extra movement• Extrapyramidal diseases • Hyperkinetic excessive amt of spontanous

motor activity and abnormal involuntary move occur

• Hypokinetic Cx by akinesia or bradykinesia in which purposeful motor activity is absent or reduced

Basal gangliaBasal ganglia

• Basal ganglia regulate the initiation,scaling and control of the amplitude and direction of movement

• Movement disorders arise from biochemical or structural abnormalities in these structures.

..con..con

• Clearing house

• during an intended /projected movement one set of activities is facilitated all the other are surpassed

• BRAKE prevents the target structures from generating unwanted motor activity

• Switch selects which of the many available will be activated at any given time

BRAKE

SWITCH

Evaluation of the pt Evaluation of the pt

• Hx and P/E• Drug exposure • systemic illnesses• family hx• developmental milestones• other neurological abnormalities,neuroimaging• agg/ptting factors• degree of functional impairment• Onset/progression• psychosocial issues• Neuor psychiatric abnormalities

Parkinson’s diseaseParkinson’s disease

• Most common eg of neurodegenerative disease that results from decreased dopaminergic transmission with in the striatum.

• Characterized by bradykinesia tremor rigidity flexed posture/shuffling gait• Idiopathic in 75%• Familial 5%• Other causes 20%• Strongest risk factor is age >60yrs others male, family Hx ,rural living• Coarse 10-25yrs

PathogenesisPathogenesis

• Degeneration of dopaminergic neurones

• LB containing alpha synuclein as the main component

• I/V of the anterior olfactory nuclei

• Locus corleulus

• Basal forebrain amygdala

• substantia nigra pars compacta

ManifestationsManifestations

• Bradykinasia the most disabling• bulbar • limbs RegidityTremorGait /posture Non motor features sensory symptoms Neuropsychiatric Autonomic CognitiveDDx

DDxDDx

• Cognitive abnormalities • Hyperactive tendon reflexes• Babniski’s signs cerebellar signs• Ocular motility abnormalities• Prominent postural instability • Major autonomic manifestations• Poor response to levodopa• Symmetry of symptoms

……CONCON

• Lack of tremor

• Differentiating these syndromes may take 2-4 YRS

Ddx for pdDdx for pd

Drug therapyDrug therapy

• Levodopa• Dopamin agonists Bromocriptin/pergolide pramipexol/ropinirol• MAO inhibitors selegilin• COMT inhibitors entecapone telcapone• Anticholinergics/Amantadin

……concon

• Initiate when the disease starts to interfere with the functional status of the pt

• Which to initiate depends on the age Phenotype of the disease cognitive symptoms stage of the disease cost

TherapyTherapy

• Goal is to restore dopamin transmission in the striatum

Principles

1. Maintain the function and the QOL

2. Avoid drug induced complications

3. Prevention of secondary disabilities

4. Neuroprotecive therapies

……concon

• Levodopa is the well established as the most effective drug for the symptomatic Rx of PD

• Particularly effective for the akinetic Sx.tremor and regidity can also respond

• Initiate with small doses 100/25• ½ tab• Complete absence of response to doses of • 1-1.5gm strongly suggests that the original diagnosis of

PD was incorrect• Motor fluctuations• dyskinesia/dystonia• 50% of the pts treated for >5yrs

..con..con

• Initiate with dopamin agonists

• Titrate slowly

• Add levodopa when sym fail tobe controlled

• Lesser control of motor symptoms

• Higher neuropsychiatric symptoms

• Delay the onset of the motor s/e of early initiated levodopa

AugmentationAugmentation

• Selegilin less effective sx ?neuroprotective• Added to alleviate tremor or to prevent the wearing effect of

levodopa• Anticholinergic particularly to control the resting tremor and

dystonia.• Amantadin for drug induced dyskinesia• Non motor Sx antidepressants atypical antipsychotic • Neuroprotective• Surgery/DBS

DystoniaDystoniaSustained muscle contraction which Sustained muscle contraction which produces repetitive movements with produces repetitive movements with

twisting and abnormal posturingtwisting and abnormal posturingDirectional quality with specific patternDirectional quality with specific pattern

• Action dystonia voluntary mov. Which use the affected grp of muscle results in dystonia or worsens the condition

• Overflow those muscle grps that are not called for the particular action could get involved

• Sensory tricks specific procedures tend to abolish or decrease it for some time

• Intensified by stress • Tremor

PathophysiologyPathophysiology

Primary UKRole of dopamin levodopa Parkinson’s Antipsychotic drugsImpaired inhibition of multiple levels ‘disturbed surround inhibition’Enlarged sensory motor representation

ClassificationClassification

Anatomical distributionAgeEtiology Primary Secondary dystonia plus ParoxysmalGenetics DTY1-13

Topographic classTopographic class

Focal dystoniasFocal dystonias

Adult onset(4 and 5th decades)primary dystonia F:M 3:1Coarse extremely variablePeriodic → action → overflow rest → posture → Contracture30% progress to segemental some to multifocal Most cases stabilize with in 2-3yrs Remission is common in cervical dystonias

Cervical dystoniasCervical dystonias

• Commonest of all focal dystonias• Jerky head movements / unnatural neck

postions• Neighboring muscle i/v• Commonest dystonia that responds to neck

tricks• 2/3 neck pain• 1/3 head tremor• 10%brief spontanous remission

BlepherospasmBlepherospasm

• Intermittent/sustained closure of the eye

• Grimacing

• May begin in one eye

• Photophobia /excessive blinking

• Interferance in ADL →fun blindness

• Sensory tricks singing hamming talking laughing and yawning

Blepher…Blepher…

Oromandibular dystoniaOromandibular dystonia

• Jaw,mouth,lower face• tongue may be involved• Trismus/bruxism protrusion of the jaw• Jaw pain difficulty of eating/speaking• Sensory tricks chewing gum ,talking placing toothpick• Spasmodic dystonia either adduction or abduction of the vocal

cords • Adducted strangled hoarse restricted• Speech is interrupted by sudden short pauses followed by abrupt

burst and become less and less understandable• Abducted breathy whispering in quality • unable to speak loudly• Sensory tricks pressing the hand over the abdomen •

Oromandibular dystoniaOromandibular dystonia

Limb dystoniaLimb dystonia

• Upper limb tend to be task specific• The activites which are affected most of the time

are those which require highly specialized precise actions or extremely repetitive movements

• Writers cramp• Musicians • Tennis players• rarely in the lower limbs

Hand dystoniaHand dystonia

..con..con

• Lower limbs primarily the ankle and the foot • Inward turning of the foot and upward turning of

the sole • Onset is during childhood• Tend to generalize• Adult hood needs diagnostic evaluation• Hemidystonia type of multifocal dystonia • Secondary to underlying causes• Generalized

Foot dystoniaFoot dystonia

Treatment of dystoniasTreatment of dystonias

• Social support• Rehabilitation• Drug therapy Anticholinergics benzodiazepines Baclofene dopaminergic drugs antidopaminergics• Botulinum toxin Localized disease• Surgical therapy

CHOREACHOREA

• Irregular involuntary rapid and uncontrolled mov

• Random flow from one part to the other

• Hand face legs and trunk

• Mov are continually changing

• Hemiballismus differs from chorea in its amplitude of mov and rotator components of the motion

CausesCauses

• Static injury

cp,encephalitis,kernicturus

• Heriditary/degenerative

• Chemical /metabolic

Wilson’s ,vitE def,hypoparathyroidism

• Immune mediated

• Medications neuroleptics/CCBs/AEDs

ConCon

• Sydenham’s chorea • 18_36% of pts with ARF• 1-8mon a/f initiating infectn• Insidious in onset• Emotional changes• Vasculitis of the BG cortex cerebellum• Gradual imp within 12-15 wks

Huntington’s choreaHuntington’s chorea

• Heriditary disease that is Cx by chorea,dementia and emotional abnormalities• Earlier age of onset 35-45• Coarse 15-20yrs• Chorea peaks in the first 10 yrs • Then bradykinesia/regidity may predominate the coarse• Westphalt syndrome• Dementia• Emotional instabilities disorders of affect explosive emotional dis

Treatment Treatment

• Multidisciplinary Mx

• Avoid injuries,exhaustion,and dehydration

• Neuroleptics haloperidol

pimozide

• Benzodiazepines

• AEDs valproate

TicsTics

• Stereotyped mov that may be repeated or continues

• Similar to normal mov but involuntary• Sense of build up and temporary relief • Boys are more affected• Tend to be ass with OCDs and HADS• Waxing and waning coarse • Resolve spontaneously• Release phenomenon due to damage to the SN• ?? Infectious cause PANDAS

CONCON

• Quality of life

• Neuroleptics halloperidol pimozide

• Atypical anti psychotics clozapine

resperidone

• Clonidin,clonazepam,antidepressant

TremorTremor

• Involuntary rhythmic osscilatory• 10-20 fold of PD• Commonest mov’t disorder• Significant disability but often remains unrecognized Writing speech fine manipuln ADL feeding drinking dressing• classificn• by the body parts the tremor involvs LIMBS head TRUNKAL palatal….

ConCon

• Rest and action1. Rest when the muscles are not activated2. Action when there is voluntary muscle contraction kinetic with any form of voluntary con postural isometric intention Task specific Using frequency Physiological high frequency 8-12 Pathological LOW frequency <7•

Essential tremorEssential tremor

• Bilateral largely postural/kinetic

• i/v the hands and forearm

• persistent and visible

• In the absence of other neurological findings and physiological abnormalities

• M=F

• 40-60 yrs

• Precise mechanism is unknown• Pathology/structural lesion• Centeral source of tremerogenic oscillation ‘pacemaker’• Olivocerebellar rubral circuit

• Tremor syndromes1. enhanced physiological2. indeterminate tremor syndrom3. orthostatic tremor syndrome4. task specific syndromes5. Holme’s tremor6. Drug induced or toxic…..

RxRx

• Primidon/propranolol

• Benzodiazipines

• Gabapentin/topimerate

• Thalamotomy/DBS

ReferencesReferences

• Harrison’s 16th edt.

• Uptudate 14.3

• www.wemove.com

• Nejm august 2006

Keep moving