In Huntington's disease, myoclonus — sudden, brief, involuntary muscle contractions, axial or in the extremities — can resemble epileptic seizures while remaining distinct from them. When these episodes reach a severity that impairs a patient's functional capacity, a specific evidence-based pharmacological approach is indicated.
The patient has Huntington's disease and presents with myoclonus: sudden, brief, involuntary contractions — axial, in the extremities, or generalised — similar to the spasms and jerks seen in epileptic seizures but not related to epilepsy. The functional impact of these episodes is the key factor that drives the need for targeted management.
When myoclonus impairs functional capacity in this setting, evidence-based guidelines recommend pharmacological management involving specific agents that may be used alone or in combination; additional therapeutic alternatives apply for particular subtypes of myoclonus.
DOI: 10.3389/fneur.2019.00710
Myoclonus refers to sudden muscle contractions, brief and involuntary, axial, in extremities or generalized, similar to spams and jerks in epileptic seizures but not related epilepsy.
In case of myoclonus impacting the functional capacity of the patients, treatment with sodium valproate or clonazepam, used alone or in combination, and in escalating doses, is recommended (Grade C).
Levetiracetam is a therapeutic alternative for the same indication.
In case of myoclonus of cortical origin that is not associated with epileptic seizures, piracetam has a marketing authorization (Grade C).
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