Ketogenic diet in patients with myoclonic status in non-progressive encephalopathy

Roberto Caraballo,Francesca Darra, Gabriela Reyes, Marisa Armeno, Araceli Cresta, Graciela Mestre, Bernardo Dalla Bernardina. Ketogenic Diet in Patients with Myoclonic Status in Non-Progressive Encephalopathy.  Seizure.  In print.

Highlights

•MSNPE is a well-defined epileptic syndrome refractory to antiepileptic drugs.

•Ketogenic diet is a good option treatment in patients with MSNPE.

•Ketogenic diet should be considered as treatment early in patients with MSNPE.

Abstract

Myoclonic status in non-progressive encephalopathy (MSNPE) is characterized by the recurrence of long-lasting atypical status epilepticus associated with attention impairment and continuous polymorphous jerks, mixed with other complex abnormal movements, in infants suffering from a non-progressive encephalopathy. The ketogenic diet (KD) has been used as an alternative to antiepileptic drugs (AEDs) for patients with refractory epileptic encephalopathies.

Purpose

In this study we assess the efficacy and tolerability of the KD in patients with MSNPE.

Methods

Between March 1, 1980 and August 31, 2013, 99 patients who met the diagnostic criteria of MSNPE were seen (58 patients in Verona and 41 patients in Buenos Aires). Six of these 99 patients were placed on the KD using the Hopkins protocol and followed for a minimum period of 24 months.

Results

Twelve months after initiating the diet, three patients had a 75% to 99% decrease in seizures, two had a 50% to 74% decrease in seizures, and the remaining child had a less than 50% seizure reduction. In five patients with a seizure reduction of more than 50%, the myoclonic status epilepticus disappeared within 6 months after starting the diet. All patients had very good tolerability and no adverse events were identified. In most of the patients AEDs were reduced.

Conclusion

The KD is a promising therapy for MSNPE, with most of our patients showing a more than 50% seizure reduction. In patients that responded well to the diet cognitive performance and quality of life also improved.

This study was performed in order to evaluate the efficacy and tolerability of the ketogenic diet (KD) in patients with myoclonic status in non–progressive encephalopathy (MSNPE). For MSNPE, the KD was a promising therapy, with most of the patients showing a more than 50% seizure reduction. Cognitive performance and quality of life were also improved in patients that responded well to the diet.

Methods

The clinicians observed 99 patients who met the diagnostic criteria of MSNPE (58 patients in Verona and 41 patients in Buenos Aires) between March 1, 1980, and August 31, 2013.

Using the Hopkins protocol, 6 of these 99 patients were placed on the KD and followed for a minimum period of 24 months.

Results

Three patients had a 75% to 99% decrease in seizures, 2 had a 50% to 74% decrease in seizures, and the remaining child had a less than 50% seizure reduction 12 months after initiating the diet.

The myoclonic status epilepticus disappeared within 6 months after starting the diet in 5 patients with a seizure reduction of more than 50%.

All patients had very good tolerability.

There were no adverse events.

Antiepileptic drugs (AEDs) were reduced in most of the patients.

https://www.mdlinx.com/neurology/medical-news-article/2017/07/14/encephalopathy-ketogenic-diet-myoclonic-status-non/7244098/?category=latest&page_id=1