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 Myoclonus

What Is Myoclonus?

Myoclonus is the medical term for sudden, rapid, brief, involuntary jerking of a muscle
or group of muscles. These shock-like movements may be caused by sudden muscle
contractions (positive myoclonus) or sudden losses of muscle tone (negative
myoclonus). Many different neurological disorders can cause myoclonus; therefore,
neurologists consider it a symptom of disease rather than a specific diagnosis. Indeed,
everyone experiences myoclonus on occasion. For example, "sleep starts," also known
as “hypnic jerks,” are the shock-like twitches that some people experience while
drifting off to sleep. This is a form of myoclonus that occurs in normal, healthy
individuals. Pathological myoclonus, however, may be extremely disabling.

Myoclonus may affect a small region (focal or segmental myoclonus), such as one
hand, or may produce violent jerks over the entire body (generalized myoclonus).
Myoclonic jerks may occur alone or in sequence, either in a pattern or randomly.
When patterned, they sometimes resemble more common forms of tremor. They may
occur infrequently or many times each minute. All types of myoclonus are similar
insofar as the movement cannot be controlled by the person who is experiencing it
and, in contrast to a tic, there is no warning or premonitory sensation. In more severe
cases, myoclonus can distort movement and severely limit a person’s ability to walk,
talk and eat. These types of myoclonus may indicate that an underlying condition of
the brain or nerves is causing the myoclonus.

Classifying the many different kinds of myoclonus is difficult, as the causes, clinical
effects, and responses to treatment vary greatly. It can be classified in a number of
ways. By distribution (body parts affected), myoclonus can be classified as focal,
multifocal, or generalized and by provoking factors as spontaneous and reflex.
Myoclonus can also be classified on physiology, meaning, the presumed source of its
origin in the nervous system: cortical (brain), subcortical (between the brain and
spinal cord), spinal, or peripheral (nerves outside of the central nervous system). It is
useful for physicians to classify myoclonus by physiology because the presumed
source of myoclonus (cortical, subcortical, spinal, or peripheral) helps guide the
physician towards the most effective treatment.

Myoclonus can occur at rest, when maintaining posture, or during action. Stimulus
sensitive myoclonus is a type of myoclonus triggered by outside stimuli such as lights,
noise or movement. It can be brought on by surprise as well.
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 Types of Myoclonus

Myoclonus can also be classified by etiology (the underlying cause), which is described
in more detail below.

Physiologic Myoclonus

Essential Myoclonus

Epileptic Myoclonus

Progressive Myoclonic Epilepsy (PME)

Secondary Myoclonus

Neurologic Diseases That Can Be Accompanied by

Myoclonus

Examples of neurologic diseases that can be accompanied by myoclonus are listed

below:

Multiple sclerosis
Parkinson's disease
Dementia with Lewy bodies
Corticobasal degeneration
Multiple system atrophy
Frontotemporal dementia
Alzheimer's disease
Creutzfeldt-Jakob disease
Stiff-person syndrome
Autoimmune encephalitis
Celiac disease

Several types of secondary myoclonus are described in further detail below:

Post-Hypoxic Myoclonus (Lance-Adams Syndrome)

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 Post-Hypoxic Myoclonus (Lance-Adams Syndrome)

Opsoclonus-Myoclonus Syndrome

Palatal Myoclonus

Peripheral Myoclonus

Diagnosis

The diagnostic approach to a patient with myoclonus has two objectives: (1)
identifying what part of the nervous system is producing myoclonus and (2)
establishing the cause. Clinicians are able to categorize myoclonus on the basis of its
distribution over the body, its electrophysiological characteristics, and its etiology
(cause).

Physicians may order certain tests to learn what is causing the myoclonus. These may
include:

Electroencephalogram (EEG): a record of the brain’s electrical activity

Electromyography (EMG): measures muscle activity and the electoral signals associated
with myoclonus
Imaging tests including MRI, CT scan, or PET scan to look for abnormalities such as
lesions or tumors
Blood tests to see if there any medical conditions that could be causing the myoclonus

Cause

Although some cases of myoclonus are caused by an injury to the peripheral nerves
(defined as the nerves outside the brain and spinal cord), most forms of myoclonus
are caused by a disturbance of the central nervous system (the brain and spinal cord).
Studies suggest that several locations in the brain are involved in myoclonus. One
such location is in the area of the brainstem located close to structures that are
responsible for the startle response, an automatic reaction to an unexpected stimulus
involving rapid muscle contraction.

The specific mechanisms underlying myoclonus are not yet fully understood.
Scientists believe that some types of stimulus-sensitive myoclonus may involve over
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 Scientists believe that some types of stimulus-sensitive myoclonus may involve over
excitability of the parts of the brain that control movement. These parts are
interconnected in a series of feedback loops called motor pathways. These pathways
facilitate and modulate communication between the brain and muscles. Key elements
of this communication are chemicals known as neurotransmitters, which carry
messages from one nerve cell, or neuron, to another by binding to a receptor.
Laboratory studies suggest that an imbalance between these chemicals may underlie
myoclonus. Receptors that appear to be related to myoclonus include those for two
important inhibitory neurotransmitters: serotonin, which constricts blood vessels and
promotes sleep, and gamma-aminobutyric acid (GABA), which helps the brain
maintain muscle control.

Treatment

If possible, the underlying cause of myoclonus should be corrected, but this is not
always possible. If the myoclonus is due to a medication side effect, then the
myoclonus usually resolves with discontinuation of that medication. If myoclonus
persists despite eliminating treatable causes, then the treatment of myoclonus
otherwise focuses on medications help reduce symptoms.

The drugs used to treat myoclonus usually possess anti-seizure properties. Epileptic
myoclonus and cortical (arising from the brain’s cerebral hemispheres) myoclonus
respond best to clonazepam and sodium valproate, which may be used alone or in
combination. Clonazepam is a tranquilizer and is commonly used to treat myoclonus.
Dosages of clonazepam are usually increased gradually until the patient improves or
side effects become bothersome. Drowsiness and loss of coordination are common
side effects. The beneficial effects of clonazepam may diminish over time if the patient
develops a tolerance for the medication.

If disability from the myoclonus is not adequately improved on either or both of these
medications, then medications such as levetiracetam (Keppra) can be added, which is
also very effective in treating myoclonus. Levetiracetam has also been shown to be
effective in posthypoxic myoclonus. Primidone may also be of value was an additional
drug, as well as clobazam and acetazolamide in severely affected patients.

Often, a single drug is not effective by itself, and combinations of medications are
frequently required.

When medications fail, botulinum toxin can be used. In certain types of myoclonus,
such as palatal myoclonus and hemifacial spasm, botulinum toxin (BTX) injections can
be very effective. This medication is directly injected into the muscles causing the
involuntary jerks at relatively low doses, allowing for relaxation of the muscles, thus
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 involuntary jerks at relatively low doses, allowing for relaxation of the muscles, thus
preventing the spasms.

Rarely, surgical intervention known as deep brain stimulation (DBS) is considered as a

last resort for certain forms of myoclonus. Further studies are needed to evaluate the
effectiveness of such treatment. Tumors that cause myoclonus in children with
opsoclonus-myoclonus may need to be surgically removed and/or treated with
chemotherapy and radiation.

Selected References

©2021 Joseph Jankovic, M.D.

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