surat tanprawate, md, frcp division of neurology
TRANSCRIPT
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MyoclonusMyoclonus
is a clinical sign defined as sudden,brief, shocklike, involuntarymovements caused by muscular contractions or inhibitions
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MyoclonusMy
oclonus
Sudden, brief, shock like involuntary jerks caused bycontraction or inhibition of one muscle or multiple muscle, irregular in rhythm and amplitudes
ClonusClonus
A series of rhythmic, uniphasic or monophasic (unidirectional) contraction andrelaxation of a group of muscles
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Hammond Hammond Convulsive tremor Convulsive tremor
FriedreichFriedreichParamyoklonus multipleParamyoklonus multiple
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PolymyoclonusPoly
myoclonus
Widespread,lightninglike, arrhythmic
contraction
MuscularMuscularcontractionscontractions
Positive myoclonus
MyoclonusMy
oclonussimplexsimp
lex
Single or are repeatedin a restricted groupof muscle
MM uscularuscular ii nhibitionsnhibitions
Negative myoclonus or
asterixis
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CC lassifilassifi cationcationEtiologies Physiological Essential Epileptic Symptomatic
Anatomicdistribution
Focal Segmental Multifocal Generalize
Provocative factor Spontaneous Reflex Action
Contraction pattern Rhythmic Arrhythmic Oscillatory
Clinicalneurophysiology testing
Cortical Cortical-subcortical Subcortical-supraspinal Spinal peripheral
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Where is the lesion?
Physiologic classificationWhat is the lesion? Etiologic classification
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Physiologic classificationPhysiologic classification
CorticalSubcortical : Thalamus, Brain stem
Spinal :Segmental,
PropriospinalPeripheral
Anatomicdistribution
Contractio
n pattern
Provicativ
e factor
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EtiologicEtiologicclassificat classificat
ionion
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ClassificationClassification
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Guideline for assessment of a patient withGuideline for assessment of a patient withmyoclonusmyoclonus
Physiologic classification: Hx, PE
Basic ancillary testing
Clinical neurophysiology testing to determinephysiological classification
Advanced neurophysiology for rareand specific diagnosis
Etiologies
Anatomicdistribution
Provocative factor
Contraction pattern
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Cortical myoclonusCortical myoclonus
Arising from the sensorimotorcortexConduct rapidly in the pyramidaltractsMost common type of myoclonusRostral to caudal distribution
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Cortical myoclonusCortical myoclonusTypeType CharacteristicCharacteristic PhysiologicPhysiologic
studystudySpontaneous corticalmyoclonus
Cortical reflex myoclonus
Epilepsia partialiscontinuae.g.PME
Lance Adam syndromeEncephalopathyStorage diseaseInfection: CJD, HIVDegeneration: CBD
Arrhythmic pattern
Focal or multi-focal jerk
Biggest hand , face
Spontaneous or induceby reflex or action
Giant SEP
EEG discharge
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Subcortical myoclonusSubcortical myoclonus
Spread either rostral or caudalfrom the brainstemAxial and bilateral movement
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Subcortical myoclonusSubcortical myoclonusOriginOrigin CharacteristicCharacteristic Physiologic studyPhysiologic study
Thalamus
Brain stem-Hyperexplexia-Reticular reflexmyoclonus-Palatal myoclonuse.g.HypoxiaMetabolic: renal, hepaticLymes diseaseNon-dopa responsiveparkinsonismPontine demyelinatinglesion
Asterixis in arm
Generalize and axial
Very stimulussensitive
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Spinal myoclonusSpinal myoclonus
Typically associated with a focallesion, with little spread of myoclonicactivity from spinal-generator sitesLesion may directly damage the cord changes in the afferent signaling from
peripheral and supraspinal structure
Rostral or caudal spread from thespinal cord level
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Spinal myoclonusSpinal myoclonusTypeType CharacteristicCharacteristic Physiologic studySegmental
Propriospinal
Rhythmic, notstimulus sensitive,limited bycontiguoussegment of cord
Rhythmic or notUsually thoracciclevelRecruit axial m. viaslowly conductionpolysynapticpropriospinalpathwayMore extensive
Typical: slow,bilateral of flex m.of trunk or lower
Long durationelectromyographic
burst that speed slowup and down
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Cortical myoclonusCortical myoclonus
Cause TumorInfectionIdiopathic
SyringomyeliaIschemic myelopathy
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Peripheral myoclonusPeripheral myoclonus
By alter sensory input and inducecentral reorganization withabnormal spinal sensory afferencecausing loss of inhibitory spinalinterneuron
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Peripheral myoclonusPeripheral myoclonusCharacteristicCharacteristic
Trauma
Nerve entrapment
Hemifacial spasm
Not stimulussensitive
Arrhythmic jerk
Persist during sleep
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Focal, SegmentalCortical, Spinal ,Peripheral
Rhythmic provocative
cortical +/- +
subcortical +/- ++
spinal + -
peripheral - -
Multifocal, GeneralizeCortical, subcortical
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Etiologic diagnosisEtiologic diagnosis
Physiologic
classification Cortical Subcortical
Spinal
Peripheral
Etiologicdiagnosis Physiologic Essential Epileptic
myoclonus Symptomatic
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Cortical myoclonusCortical myoclonus
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Part 1Part 1 :: Anatomic distribution patternAnatomic distribution pattern(History, physical examination)(History, physical examination)
Full historymode of myoclonusonsetpresence of otherneurological problemshistory of seizurescurrent and past drugor toxin exposurepast or currentmedical problemsfamily history
Physical examinationDistribution (may be focal,segmental, multifocal, hemi,generalised)
Temporal profile (includesrhythmic or irregular)ContinuousIntermittent (sporadic or trains)Activation profileRest (spontaneous myoclonus)Voluntary movement (actionmyoclonus)Reflex stimuli (any combinationof touch, light, sound, musclestretch)
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Physiologic classificationPhysiologic classification
Lancet Neurol 2004; 3: 598607
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Classification of myoclonusClassification of myoclonusI. Physiological
myoclonus (healthyindividuals)
Sleep jerks (eg, hypnic jerks) Anxiety induced Exercise induced Hiccough (singultus)
Benign infantilemyoclonus withfeeding
II. Essentialmyoclonus(primarysymptom, non-progressivehistory) Hereditary
(autosomaldominant) Sporadic
p ep c myoc onus se zuresp ep c myoc onus se zures
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Fragments of epilepsy Isolated epileptic myoclonic jerks Epilepsia partialis continua Idiopathic stimulus-sensitive myoclonus Photosensitive myoclonus Myoclonic absences in petit mal epilepsy
Childhood myoclonic epilepsy Infantile spasms Myoclonic astatic epilepsy (Lennox-Gastaut) Cryptogenic myoclonus epilepsy (Aicardi) Awakening myoclonus epilepsy of Janz (juvenile myoclonic
epilepsy)Benign familial myoclonic epilepsy (Rabot)Progressive myoclonus epilepsy: Balticmyoclonus (Unverricht- Lundborg)
. p ep c myoc onus se zures. p ep c myoc onus se zuresdominate, part of chronic seizuredominate, part of chronic seizure
disorder)disorder)
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IV. Symptomatic myoclonus (secondary,progressive, or static encephalopathy
dominates)Storage disease
Lafora body disease GM2 gangliosidosis (late infantile, juvenile) Tay-Sachs disease Gauchers disease (non-infantile
neuronopathic form) Krabbes leucodystrophy Ceroid-lipofuscinosis (Batten) Sialidosis (cherry-red spot) (types 1 and 2)
Spinocerebellar degenerations Ramsay-Hunt syndrome Friedreichs ataxia Ataxia-telangiectasia
Other spinocerebellardegenerations
Basal ganglia degenerations Wilsons disease Torsion dystonia Hallervorden-Spatz disease Progressive supranuclear palsy Huntingtons disease Parkinsons disease Multisystem atrophy Corticobasal degeneration Dentatorubropallidoluysian atrophy
Dementias Creutzfeldt-Jakob disease Alzheimers disease
Dementia with Lewy bodies Frontotemporal dementia Retts syndrome
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Infectious or post-infectious
Subacute sclerosing panencephalitis Encephalitis lethargica Arbovirus encephalitis Herpes simplex encephalitis Human T-lympnotropic virus IHIV Post-infectious encephalitis Miscellaneous bacteria
(streptococcus, clostridium, other)
Malaria
Syphilis Cryptococcus Lyme disease Progressive multifocal
leucoencephalopathy
Metabolic Hyperthyroidism Hepatic failure Renal failure Dialysis syndrome Hyponatraemia Hypoglycaemia
Non-ketotic hyperglycaemia Multiple carboxylasedeficiency
Biotin deficiency Mitochondrial dysfunction Hypoxia Metabolic alkalosis
Vitamin E deficiency
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Toxic and drug-inducedsyndromes
Physicalencephalopathies Post-hypoxia (Lance-
Adams) Post-traumatic Heat stroke Electric shock Decompression injury
Focal nervous systemdamage
CNSPost-strokePost-thalamotomy
Tumour TraumaInflammation (eg, multiplesclerosis)Moebius syndromeDevelopmentalIdiopathic
Peripheral nervous system TraumaHaematoma
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Malabsorption Coeliac disease Whipples disease
Eosinophilia-myalgiasyndrome
ParaneoplasticencephalopathiesOpsoclonus-myoclonussyndrome
Idiopathic Paraneoplastic
Infectious Other
Exaggerated startlesyndrome Hereditary Sporadic
Hashimotosencephalopathy
Multiple systemdegenerations
Allgrove syndrome DiGeorge syndrome Membraneous lipodystrophy
Unknown Familial Sporadic
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Part 2:Part 2:
Basic ancillary testingBasic ancillary testingElectrolytes (+/- including bismuth)GlucoseRenal function tests
Hepatic function testsParaneoplastic antibodiesDrug and toxin screen (if history suggests)Brain imaging
ElectroencephalographySpine imaging (if focal or segmental) Thyroid antibodies and function
P 3
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Part 3:Part 3: Clinical neurophysiology testing toClinical neurophysiology testing to
determine physiological classificationdetermine physiological classification
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Part 4:Part 4:Advanced testing for rare and specificAdvanced testing for rare and specific
diagnosesdiagnosesBody imaging for occultcancerCerebrospinal fluid exam(for infectious andinflammatory disorders, 14-3-3 protein for Creutzfeld- Jakob disease)
Tests for malabsorptiondisorders (eg, coeliac sprue,Whipples disease)Enzyme assays fordeficiency(eg,neuraminidase,biotinidase)Copper studies for Wilsonsdisease
Tissue biopsy of skin orleucocytes (eg, Lafora bodies,ceroid inclusions,etc.)Alpha-feto protein, cytogeneticanalysis, radiosensitivty of DNA
synthesis (ataxia-telangiectasia)Genetic testing for inheriteddisorders (eg, EPM1 gene,mitochondria genes, huntingtingene, etc)Mitochondria function studies(lactate, muscle biopsy, etc.)
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