Epilepsy with myoclono-astatic crisis

Epilepsy with Myoclonic-Astatic Crisis is a form of epilepsy characterized by a combination of myoclonic seizures and astatic seizures. This condition is also known as Doose Syndrome, named after the German pediatrician Hermann Doose who first described it in the 1960s. Epilepsy with Myoclonic-Astatic Crisis is considered a rare and severe form of epilepsy that typically begins in early childhood, usually between the ages of 1 and 5 years.

Symptoms and Diagnosis

The hallmark of Epilepsy with Myoclonic-Astatic Crisis is the presence of myoclonic seizures, which are sudden, brief involuntary muscle jerks, and astatic seizures, which lead to falls due to a sudden loss of muscle tone. These seizures can be very frequent and are often resistant to treatment. The diagnosis is primarily clinical, based on the characteristic seizure types and age of onset. Electroencephalogram (EEG) findings in these patients often show specific patterns that help in confirming the diagnosis.

Treatment

Treatment of Epilepsy with Myoclonic-Astatic Crisis is challenging and often requires a combination of antiepileptic drugs (AEDs). Medications such as valproate, lamotrigine, and clobazam are commonly used. The ketogenic diet, a high-fat, low-carbohydrate diet that has been found to be effective in controlling seizures in some cases of epilepsy, may also be beneficial for patients with this condition. In refractory cases, where seizures are not controlled with medication, surgical options may be considered.

Prognosis

The prognosis for individuals with Epilepsy with Myoclonic-Astatic Crisis varies. Some children may achieve good seizure control with treatment and can have a normal or near-normal developmental outcome. However, in others, the condition may be resistant to treatment, leading to developmental delays and ongoing seizure activity. Early and aggressive treatment is crucial in improving the long-term outlook for these patients.

Epidemiology

Epilepsy with Myoclonic-Astatic Crisis is a rare condition, accounting for about 1-2% of all childhood epilepsies. It affects males slightly more often than females. The exact prevalence and incidence of the condition are not well-documented due to its rarity.

See Also

• Epilepsy
• Myoclonic seizure
• Astatic seizure
• Ketogenic diet
• Antiepileptic drug

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