INFANTILE MYOCLONIC ENCEPHALOPATHY

SYMPTOMS

Infantile myoclonic encephalopathy symptoms might include:

• Myoclonic seizures
• Tonic or focal motor seizures
• Abnormal electrical brain activity pattern (burst suppression pattern)
• Developmental delays
• Weak muscle tone
• Impaired awareness or vegetative state

DIAGNOSIS

Infantile myoclonic encephalopathy is diagnosed based on electroencephalogram (EEG) results and clinical aspects.

The most crucial test for diagnosing Infantile Myoclonic Encephalopathy is the EEG. Without treatment, a baby’s EEG displays a burst suppression pattern (high amplitude spikes followed by minimal brain activity or flattening of the brain waves), which is quite abnormal. Both while the baby is awake and when they are sleeping, these changes may be detected.

The term “hypsarrhythmia pattern” refers to an EEG with multifocal high amplitude spikes and a highly aberrant backdrop. This pattern may emerge as newborns grow and develop.

A useful method for searching for structural alterations in the brain that can result in Infantile myoclonic is through magnetic resonance imaging. Initial MRI results may be normal, but subsequent MRIs may reveal brain atrophy (shrinkage). The neurologist will decide whether the anomaly is the source of the Infantile myoclonic when the MRI is sometimes abnormal.

Additionally, blood work will be performed to check for genetic or metabolic issues.

TREATMENT

• Anti-seizure drugs are often utilized, although seizures caused by Infantile myoclonic encephalopathy are frequently challenging to manage. Clobazam (Onfi), clonazepam (Klonopin), topiramate (Topamax), zonisamide (Zonegran), phenobarbital, valproate (if the metabolic state is known), or felbamate (Felbatol) are among the medications that are often used. Drugs like vigabatrin (Sabril) may exacerbate seizures.
• Surgery for Epilepsy is a possibility for kids whose seizures begin in one region or involve one side of the brain. A hemispherectomy or focused resection, which removes a specific portion of the brain, may be an option in certain circumstances.
• Dietary treatment or devices: When medications are ineffective, other options may be explored, such as a vagus nerve stimulator, customized diets based on the underlying metabolic disorder, or the ketogenic diet.