Myoclonus


Article Author:
Wael Ibrahim


Article Editor:
Sandeep Sharma


Editors In Chief:
Mohamed Hegazy
Arayamparambil Anilkumar
Najib Murr


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James Hughes
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Nazia Sadiq
Hajira Basit
Phillip Hynes
Tehmina Warsi


Updated:
5/5/2019 11:27:48 PM

Introduction

Myoclonus is defined as rapid, brief, jerky, or shock-like movements involving muscle or group of muscles. Among all hyperkinetic movement disorders, Myoclonus is considered to be the most rapid and brief. When caused by sudden muscle contraction, it is known as "positive myoclonus," while a brief loss of muscular tone results in "negative myoclonus" as in asterixis.[1]

Myoclonus is classified in different ways according to its physiology, anatomical site of origin, and etiology. As with most movement disorders, myoclonus can be focal, multifocal, segmental, or generalized.[2]

Myoclonus is one of the signs in a wide variety of nervous system disorders such as dystonia, multiple sclerosis, Parkinson disease, Creutzfeldt-Jakob disease (CJD), serotonin toxicity, Huntington disease, subacute sclerosing panencephalitis, Alzheimer disease, and Gaucher disease.

Etiology

Etiologically, myoclonus can be primary or secondary. Primary can be subdivided into physiological myoclonus (hypnic jerks which occurs in normal individuals), essential myoclonus (idiopathic or hereditary), epileptic myoclonus; while secondary myoclonus is called "symptomatic myoclonus" where the myoclonus is secondary to an underlying disorder.[1]

Moreover, myoclonus can be subdivided by the anatomical origin into the following:[3]

  1. Cortical (includes post-hypoxic action myoclonus, primary generalized myoclonic seizures, primary generalized epileptic myoclonus), 
  2. Subcortical (includes essential myoclonus, reticular reflex myoclonus, opsoclonus-myoclonus syndrome, hyperekplexia), 
  3. Spinal (includes propriospinal myoclonus and segmental spinal myoclonus), and 
  4. Peripheral (includes hemifacial spasm)

Since functional myoclonus is among the most common functional movement disorders, it is crucial to differentiate from organic myoclonus. This can be done through localization of the movements. Features in favor of an organic cause over a functional cause of myoclonus include consistent phenomenology, insidious onset, or response to benzodiazepines or antiepileptic medication.[4] The presence of spontaneous periods of remission, pronounced reduction of the myoclonus with distraction, acute onset, and sudden resolution favor the functional diagnosis of myoclonus.[3]

Epidemiology

In the United States, myoclonus is not common, with a lifetime prevalence of 8.6 per 100,000 population. After 50 years of age, the prevalence increases. In about a quarter of cases (27%), myoclonus is transient and usually drug-induced. About 8% of myoclonic cases are functional.[5] The average annual incidence of myoclonus (from 1976 to 1990) was 1.3 cases per 100 000 person-years.[3]

The most common type of myoclonus is symptomatic myoclonus representing 72% of all patients with myoclonus,[6] followed by epileptic myoclonus with around 17%, while essential myoclonus represents only 11% of all patients with myoclonus.[5]

Pathophysiology

Myoclonus can arise from several levels in the nervous system, ranging from the cerebral cortex to the peripheral nerves. Cortical myoclonus predominantly affects body regions with the largest cortical representations such as the hands and face, and as the motor areas of the cerebral cortex are most involved in voluntary action, the jerks are most manifest on action (i.e., action myoclonus).[3]

Brainstem motor systems are mainly involved in axial and bilateral movements and are near subcortical reflex centers. Thus, brainstem myoclonus is characterized by being generalized and stimulus sensitive (especially auditory stimuli). Its hallmark is auditory reflex jerks as in startle syndrome and brainstem reflex myoclonus.[3]

History and Physical

History taking in a patient with myoclonus should focus on the age of onset, course, and duration of disease, family history, precipitating factors such as drugs, triggers (auditory stimulus, movement), the body parts involved, and the rhythmicity of the myoclonus. The presence of associated neurological signs/symptoms or disorder help to identify the underlying etiology and/or pathology.[5]

The main clinical characteristics demarcating myoclonus from other movement disorders are its rapid onset, brief duration, and the single-event type of muscle activation.[7] Myoclonus can occur spontaneously (at rest) or during movement (i.e., action myoclonus) or can be provoked by external tactile or acoustic stimuli (i.e., reflex myoclonus).

Clinically, cortical myoclonus is more prominent in the hands and face and more commonly provoked by tactile stimuli. While, brainstem reflex myoclonus affects the arms bilaterally. spinal myoclonus tends to present as unilateral arrhythmic jerking in the arm and/or trunk; whereas repetitive jerking of only the trunk and abdomen, and bilaterally, may be due to propriospinal myoclonus.[8]

The presences of cognitive impairment, epilepsy, ataxia, other movement disorders or neurological signs usually point to symptomatic myoclonus. Meanwhile, acute or subacute onset should orientate toward a toxic, metabolic, or infectious aetiology. A progressive course should orientate toward a degenerative process, static encephalopathy, or progressive metabolic disease.[6]

Evaluation

The determination of the generator site in myoclonus is an important clue for diagnostic orientation. All neurophysiologic tools (electromyography [EMG], electroencephalography [EEG], evoked potentials) may be helpful.[9] EEG is useful for the identification of both ictal and interictal patterns in epileptic myoclonus (For example, generalized spikes and 3 to 6 Hz spike and wave pattern are typical for primary generalized myoclonic epilepsy).[3]

Imaging of the brain and spinal cord to determine structural or focal lesion.

Advanced testing for specific and rare diagnoses should be considered including, but not limited to, a cerebrospinal fluid exam, tissue biopsy of skin, and genetic testing for inherited disorders.[3]

Treatment / Management

Clinicians mainly manage myoclonus mainly by treating symptoms. The previously mentioned classification of myoclonus into cortical and subcortical helps in the guidance of pharmacotherapy since spinal myoclonus fails to respond to medications effective for cortical myoclonus and vice versa.[8]

In cortical myoclonus, first-line medications include valproate, clonazepam, and levetiracetam with topiramate and zonisamide used as second-class agents. Meanwhile, antiepileptic drugs as phenytoin, carbamazepine, and lamotrigine may cause worsening of cortical myoclonus symptoms. On the other hand, subcortical/spinal myoclonus responds to both clonazepam, and levetiracetam as first-line and second-line agents, respectively.[8]

Botulinum toxin can be offered as a treatment option in focal peripheral myoclonus.[10]

In regards to DBS in myoclonus, the most commonly reported indication is myoclonus–dystonia syndrome, and the target is usually both internal globus pallidus (GPi) and thalamic nuclei.[11]

Differential Diagnosis

Differential diagnosis of myoclonus includes similar movement disorders presenting as rapid and jerky movements. They vary in amplitude from mild, not causing movements across the joint as fasciculations and myokymia, to stronger movements as tremor, tics, and chorea; all of which need to be distinguished from myoclonus.[8]

The differential diagnosis can include:

  • Dystonia
  • Multiple sclerosis
  • Parkinson
  • CJD
  • Serotonin toxicity
  • Huntington disease
  • CJD
  • Subacute sclerosing
  • Panencephalitis 
  • Alzheimer disease
  • Some forms of Gaucher disease

Prognosis

The degree to which myoclonus improves with treatment depends on the underlying etiology. Certain types of myoclonus may be secondary to potentially treatable etiologies as an inflammatory disorder, or a metabolic disturbance. For example, in drug-induced myoclonus stopping the offending drug can be sufficient for complete resolution of myoclonic jerks.[12]

Complications

Myoclonus can be the cause of significant disability with impairment in activities of daily living and maybe depressive symptoms. This results from jerky movements occurring during rest, with muscle activation or by external stimuli as sound resulting in interference with performing or starting the desired correct movement for a specific task.[10]

Enhancing Healthcare Team Outcomes

A neurologist best manages patients with myoclonus. When a primary caregiver or nurse practitioner comes across a patient with a movement disorder, referral to a neurologist is recommended as the differential diagnosis is broad and the treatments do vary for each disorder.


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Myoclonus - Questions

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Which of the following statements best describes myoclonus?



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A patient develops rapid, brief, purposeless jerking movements of the right upper extremity. Loud sounds may precipitate the abnormal movements. What is the term for this?



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Which of the following drug classes is associated with drug-induced myoclonus?



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Which of the following myoclonic conditions responds to alcohol?



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A 40-year-old man presented to the emergency department with rapidly progressive encephalopathy in the past few days. His wife reported that two weeks ago patient experiences abnormal jerky movements more with loud sounds, double vision, stiffness in both upper and lower limbs and attacks of tachycardia, dizziness, and sweating. On examination, the patient is encephalopathic with marked rigidity. There are myoclonic jerks of all limbs. Ocular examination reveals ophthalmoplegia. Which antibody is expected in cerebrospinal fluid of this patient?



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Myoclonus - References

References

Badheka R,Barad NK,Sankhla CS, Pediatric movement disorders. Neurology India. 2018 Mar-Apr     [PubMed]
Eberhardt O,Topka H, Myoclonic Disorders. Brain sciences. 2017 Aug 14     [PubMed]
Dreissen YE,Cath DC,Tijssen MA, Functional jerks, tics, and paroxysmal movement disorders. Handbook of clinical neurology. 2016     [PubMed]
Mills K,Mari Z, An update and review of the treatment of myoclonus. Current neurology and neuroscience reports. 2015 Jan     [PubMed]
Espay AJ,Chen R, Myoclonus. Continuum (Minneapolis, Minn.). 2013 Oct     [PubMed]
Caviness JN, Treatment of myoclonus. Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics. 2014 Jan     [PubMed]
Lozsadi D, Myoclonus: a pragmatic approach. Practical neurology. 2012 Aug     [PubMed]
Vercueil L, Myoclonus and movement disorders. Neurophysiologie clinique = Clinical neurophysiology. 2006 Sep-Dec     [PubMed]
Borg M, Symptomatic myoclonus. Neurophysiologie clinique = Clinical neurophysiology. 2006 Sep-Dec     [PubMed]
Cassim F,Houdayer E, Neurophysiology of myoclonus. Neurophysiologie clinique = Clinical neurophysiology. 2006 Sep-Dec     [PubMed]
Caviness JN,Brown P, Myoclonus: current concepts and recent advances. The Lancet. Neurology. 2004 Oct     [PubMed]
Starr PA, Deep brain stimulation for other tremors, myoclonus, and chorea. Handbook of clinical neurology. 2013     [PubMed]

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