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Myoclonus is usually classified physiologically to optimize treatment. Myoclonus is a precursor effect to myoclonus dystonia and most commonly begins in childhood or adolescence. [4] [5] Myoclonus is classified as cortical, subcortical, peripheral or spinal. Cortical myoclonus is the most common of these four and affects the upper limbs and face.
Lance–Adams syndrome (LAS) is a sequela of hypoxic encephalopathy due to respiratory arrest, airway obstruction, cardiac arrest, etc., several days after the onset of hypoxic encephalopathy. A condition that presents with functional myoclonus associated with increased cortical excitability in a few weeks.
Action myoclonus is the most disabling form of myoclonus and can affect the arms, legs, face, and even the voice. It is often associated with tonic-clonic seizures and diffuse neuronal disease such as post-hypoxic encephalopathy , uremia , and the various forms of PME, although, in the case of focal cerebral damage, the disease may be ...
Symptoms often include action or stimuli induced myoclonus, seizures, neuropathy, cognitive decline, and spike and wave or no cerebral discharges. [4] The prognosis of those diagnosed with PME is poor. The person often becomes reliant on a wheelchair, enters a vegetative state due to myoclonus, and has a shortened life expectancy. [4] [3]
Progressive myoclonus epilepsy is a disease associated with myoclonus, epileptic seizures, and other problems with walking or speaking. These symptoms often worsen over time and can be fatal. [6] MERRF syndrome is also known as myoclonic epilepsy with ragged-red fibers.
The primary diagnosis for JME is a good knowledge of patient history and the neurologist's familiarity with the myoclonic jerks, which are the hallmark of the syndrome. [38] Additionally, an EEG will indicate a characteristic pattern of waves and spikes associated with the syndrome such as generalized 4–6 Hz polyspike and slow wave discharges.
Palatal myoclonus The myoclonic triangle (also known by its eponym Triangle of Guillain-Mollaret or dentato-rubro-olivary pathway ) is an important feedback circuit of the brainstem and deep cerebellar nuclei which is responsible for modulating spinal cord motor activity.
Myoclonic astatic seizures: seizures that involve a myoclonic seizure followed immediately by an atonic seizure. This type of seizure is exclusive to MAE and is one of the defining characteristics of this syndrome. Tonic seizures: muscle stiffening or rigidity. This seizure is rare in this syndrome.