Myoclonus

Surat Tanprawate, MD, FRCP

Division of neurology
Chiang Mai University
 Myoclonus
 is a clinical sign defined as sudden, brief
, shocklike, involuntary movements cau
sed by muscular contractions or inhibiti
ons
 Clonus

A series of rhythmic, uniphasic or

monophasic (unidirectional) contraction and
relaxation of a group of muscles

Myoclonus

Sudden, brief, shock like involuntary jerks caused by

contraction or inhibition of one muscle or
multiple muscle, irregular in rhythm and amplitudes
 Hammond
Convulsive tremor

Friedreich
Paramyoklonus multiplex
Myoclonus simplex Muscular contracti
ons
Single or are repeated in a
restricted group of muscle Positive myoclonus

Polymyoclonus M uscular
inhibitions
Widespread,lightning like,
arrhythmic contraction Negative myoclonus or ast
erixis
 C lassification
 Etiologies  Provocative factor
– Spontaneous
– Physiological – Reflex
– Essential – Action

– Epileptic  Contraction pattern

– Rhythmic
– Symptomatic – Arrhythmic
 Anatomic – Oscillatory

distribution  Clinical neurophysiology

testing
– Focal – Cortical
– – Cortical-subcortical
Segmental – Subcortical-supraspinal
– Multifocal – Spinal
– Generalize – peripheral
 Where is the lesion?
– Physiologic classification
 What is the lesion?
– Etiologic classification
 Physiologic classification
Anatomic
distribution

Cortical
Subcortical:
Thalamus, Brain stem
Spinal:
Segmental, Propriospinal
Peripheral

Contraction Provicative
pattern factor
 Etiologic
classification
Classification
 Guideline for assessment of a patient with myoclonus

Physiologic classification: Hx, PE

Etiologies Provocative factor

Basic ancillary testing
Anatomic distribution Contraction pattern

Clinical neurophysiology testing to determine

physiological classification

Advanced neurophysiology for rare and

specific diagnosis
 Cortical myoclonus
 Arising from the sensorimotor cortex
 Conduct rapidly in the pyramidal tracts
 Most common type of myoclonus
 Rostral to caudal distribution
 Cortical myoclonus
Type Characteristic Physiologic study
Spontaneous cortical Arrhythmic pattern Giant SEP
myoclonus
Focal or multi-focal jerk EEG discharge
Cortical reflex
myoclonus
Biggest hand , face

Epilepsia partialis
Spontaneous or induce
continua by reflex or action
e.g.
PME
Lance Adam syndrome
Encephalopathy
Storage disease
Infection: CJD, HIV
Degeneration: CBD
 Subcortical myoclonus
 Spread either rostral or caudal from the
brainstem
 Axial and bilateral movement
 Subcortical myoclonus
Origin Characteristic Physiologic study
Thalamus Asterixis in arm

Brain stem Generalize and axial

-Hyperexplexia
-Reticular reflex Very stimulus sensitive
myoclonus
-Palatal myoclonus
e.g.
Hypoxia
Metabolic: renal, hepatic
Lyme’s disease
Non-dopa responsive
parkinsonism
Pontine demyelinating lesion
 Spinal myoclonus
 Typically associated with a focal lesion,
with little spread of myoclonic activity from
spinal-generator sites
 Lesion may
– directly damage the cord
– changes in the afferent signaling from
peripheral and supraspinal structure
 Rostral or caudal spread from the spinal
cord level
 Spinal myoclonus
Type Characteristic Physiologic study
Segmental Rhythmic, not stimulus
sensitive, limited by
contiguous segment of
cord

Long duration
Rhythmic or not
Propriospinal electromyographic
Usually thoraccic level burst that speed slow
Recruit axial m. via up and down
slowly conduction
polysynaptic
propriospinal pathway
More extensive
Typical: slow, bilateral
of flex m. of trunk or
lower limb
 Cortical myoclonus

 Cause
 Tumor
 Infection
 Idiopathic
 Syringomyelia
 Ischemic myelopathy
 Peripheral myoclonus
 By alter sensory input and induce central
reorganization with abnormal spinal sensory
afference causing loss of inhibitory spinal
interneuron
 Peripheral myoclonus
Characteristic

Trauma Not stimulus sensitive

Nerve entrapment Arrhythmic jerk

Hemifacial spasm Persist during sleep

Focal, Segmental Multifocal, Generalize
Cortical, Spinal ,Peripheral Cortical, subcortical

Rhythmic provocative

cortical +/- +

subcortical +/- ++

spinal + -

peripheral - -
 Etiologic diagnosis

Etiologic
Physiologic diagnosis
classification – Physiologic
– Cortical
– Essential
– Subcortical
– Epileptic
– Spinal myoclonus
– Peripheral – Symptomatic
Cortical myoclonus
 Part 1: Anatomic distribution pattern
(History, physical examination)
Full history Physical examination
 mode of myoclonus onset  Distribution (may be focal,
segmental, multifocal, hemi,
 presence of other generalised)
neurological problems  Temporal profile (includes
 history of seizures rhythmic or irregular)
 current and past drug or  Continuous
toxin exposure  Intermittent (sporadic or trains)
 past or current medical
 Activation profile
 Rest (spontaneous myoclonus)
problems  Voluntary movement (action
 family history myoclonus)
 Reflex stimuli (any combination of
touch, light, sound, muscle stretch)
Physiologic classification

Lancet Neurol 2004; 3: 598–607

 Classification of myoclonus
I. Physiological II. Essential myoclonus
myoclonus (healthy (primary symptom,
individuals) non-progressive
– Sleep jerks (eg, hypnic history)
jerks) – Hereditary (autosomal
– Anxiety induced dominant)
– Exercise induced – Sporadic
– Hiccough (singultus)
– Benign infantile
myoclonus with
feeding
III. Epileptic myoclonus (seizures dominate, part of
chronic seizure disorder)
 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: Baltic myoclonus
(Unverricht- Lundborg)
IV. Symptomatic myoclonus (secondary, progressive, or
static encephalopathy dominates)
 Storage disease  Other spinocerebellar degenerations
– Lafora body disease
– Basal ganglia degenerations
– GM2 gangliosidosis (late infantile, juvenile)
–
– Wilson’s disease
Tay-Sachs disease
– Gaucher’s disease (non-infantile – Torsion dystonia
neuronopathic form) – Hallervorden-Spatz disease
– Krabbe’s leucodystrophy – Progressive supranuclear palsy
– Ceroid-lipofuscinosis (Batten) – Huntington’s disease
– Sialidosis (cherry-red spot) (types 1 and 2) – Parkinson’s disease
 Spinocerebellar degenerations – Multisystem atrophy
– Ramsay-Hunt syndrome
– Corticobasal degeneration
– Friedreich’s ataxia
– Dentatorubropallidoluysian atrophy
– Ataxia-telangiectasia
 Dementias
– Creutzfeldt-Jakob disease
– Alzheimer’s disease
– Dementia with Lewy bodies
– Frontotemporal dementia
– Rett’s syndrome
 Infectious or post-infectious  Metabolic
– Subacute sclerosing
– Hyperthyroidism
panencephalitis
– Encephalitis lethargica – Hepatic failure
– Arbovirus encephalitis – Renal failure
– Herpes simplex encephalitis – Dialysis syndrome
– Human T-lympnotropic virus IHIV – Hyponatraemia
– Post-infectious encephalitis – Hypoglycaemia
– Miscellaneous bacteria – Non-ketotic hyperglycaemia
(streptococcus, clostridium, other) –
–
Multiple carboxylase
Malaria deficiency
– Syphilis – Biotin deficiency
– Cryptococcus
– Mitochondrial dysfunction
– Lyme disease
–
– Hypoxia
Progressive multifocal
leucoencephalopathy – Metabolic alkalosis
– Vitamin E deficiency
 Toxic and drug-induced  Focal nervous system
syndromes damage
– CNS
 Physical  Post-stroke
encephalopathies  Post-thalamotomy
– Post-hypoxia (Lance-  Tumour
Adams)  Trauma
– Post-traumatic  Inflammation (eg, multiple
– Heat stroke sclerosis)
 Moebius syndrome
– Electric shock
 Developmental
– Decompression injury  Idiopathic

– Peripheral nervous system

 Trauma

 Haematoma
 Malabsorption  Exaggerated startle
– Coeliac disease syndrome
– Whipple’s disease – Hereditary
 Eosinophilia-myalgia – Sporadic
syndrome  Hashimoto’s encephalopathy
 Paraneoplastic  Multiple system
encephalopathies degenerations
 Opsoclonus-myoclonus – Allgrove syndrome
syndrome – DiGeorge syndrome
– Idiopathic – Membraneous lipodystrophy
– Paraneoplastic  Unknown
– Infectious – Familial
– Other – Sporadic
 Part 2:
Basic ancillary testing
 Electrolytes (+/- including bismuth)
 Glucose
 Renal function tests
 Hepatic function tests
 Paraneoplastic antibodies
 Drug and toxin screen (if history suggests)
 Brain imaging
 Electroencephalography
 Spine imaging (if focal or segmental)
 Thyroid antibodies and function
 Part 3:
Clinical neurophysiology testing to determine
physiological classification
 Part 4:
Advanced testing for rare and specific diagnoses

 Body imaging for occult cancer  Tissue biopsy of skin or

 Cerebrospinal fluid exam (for
infectious and inflammatory
disorders, 14-3-3 protein for
Creutzfeld-Jakob disease)
 Tests for malabsorption
disorders (eg, coeliac sprue,
Whipple’s disease)
 Enzyme assays for deficiency
(eg,neuraminidase, biotinidase)
 Copper studies for Wilson’s
disease
leucocytes (eg, Lafora bodies,
ceroid inclusions,etc.)
 Alpha-feto protein, cytogenetic
analysis, radiosensitivty of DNA
synthesis (ataxia-telangiectasia)
 Genetic testing for inherited
disorders (eg, EPM1 gene,
mitochondria genes, huntingtin
gene, etc)
 Mitochondria function studies
(lactate, muscle biopsy, etc.)