3 Early Infantile Epileptic Encephalopathy

Also Known As:

Epileptic Encephalopathy, Early Infantile, 3; Early Myoclonic Encephalopathy; Myoclonic Epilepsy, Neonatal, With Suppression-Burst Pattern

Networked: 16 relevant articles (1 outcomes, 1 trials/studies)

Relationship Network

Disease Context: Research Results

Nervous System Diseases: 14178
- Central Nervous System Diseases: 4213
  - Brain Diseases: 15694
    - Epilepsy: 37791
      - Generalized Epilepsy: 896
        Infantile Spasms: 1454
        3 Early Infantile Epileptic Encephalopathy: 16
      - Epileptic Syndromes: 883
        Infantile Spasms: 1454
        3 Early Infantile Epileptic Encephalopathy: 16

Related Diseases

1.	Seizures (Absence Seizure)
2.	Infantile Epileptic-Dyskinetic Encephalopathy
3.	Nonketotic Hyperglycinemia
4.	Infantile Spasms (West Syndrome)
5.	Myoclonic Epilepsies (Myoclonic Encephalopathy)

Experts

1.	Ishikawa, Nobutsune: 1 article (10/2019)
2.	Itai, Toshiyuki: 1 article (10/2019)
3.	Kato, Mitsuhiro: 1 article (10/2019)
4.	Kobayashi, Masao: 1 article (10/2019)
5.	Kobayashi, Yoshiyuki: 1 article (10/2019)
6.	Matsumoto, Naomichi: 1 article (10/2019)
7.	Miyatake, Satoko: 1 article (10/2019)
8.	Tani, Hiroo: 1 article (10/2019)
9.	Tateishi, Yuichi: 1 article (10/2019)
10.	Kawatani, Masao: 1 article (09/2019)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to 3 Early Infantile Epileptic Encephalopathy:

1.	FenfluramineIBA 10/01/2022 - "Biallelic variants in SCN1B can result in early myoclonic encephalopathy, and adjunctive treatment with fenfluramine may be an effective treatment for SCN1B-related DEE. "
2.	Anticonvulsants (Antiepileptic Drugs)IBA 04/01/2003 - "Early myoclonic encephalopathy (EME) is a rare epileptic syndrome characterized by neonatal onset of severe recurrent seizures of multiple types often resistant to antiepileptic drugs (AEDs). " 03/01/2016 - "The most common epileptic encephalopathies are Ohtahara syndrome, early myoclonic encephalopathy, epilepsy of infancy with migrating focal seizures, West syndrome and Dravet syndrome, which are usually unresponsive to traditional antiepileptic medication. " 01/01/1998 - "Lamotrigine can be effective therapy for juvenile myoclonic epilepsy and eyelid myoclonia with absences when used alone and, in conjunction with other antiepileptic drugs (AED) (usually valproate) for early myoclonic encephalopathy, myoclonic-astatic epilepsy and particularly, epilepsy with myoclonic absences. "
3.	Glucose Transporter Type 1IBA 01/01/2002 - "The review also discusses the epileptic syndromes seen in neonates, including early myoclonic encephalopathy, Ohtahara syndrome, pyridoxine dependency, and glucose transporter type 1 syndrome."
4.	Neuregulin-1 (Neuregulin 1)IBA 03/01/2009 - "Early myoclonic encephalopathy caused by a disruption of the neuregulin-1 receptor ErbB4."
5.	Vigabatrin FDA Link 01/01/2006 - "Here we present the vigabatrin experience in two patients with early myoclonic encephalopathy owing to nonketotic hyperglycinemia (glycine encephalopathy). " 01/01/2006 - "Vigabatrin caused rapidly progressive deterioration in two cases with early myoclonic encephalopathy associated with nonketotic hyperglycinemia."
6.	Valproic Acid (Depakote)FDA LinkGeneric 01/01/1998 - "Lamotrigine can be effective therapy for juvenile myoclonic epilepsy and eyelid myoclonia with absences when used alone and, in conjunction with other antiepileptic drugs (AED) (usually valproate) for early myoclonic encephalopathy, myoclonic-astatic epilepsy and particularly, epilepsy with myoclonic absences. "
7.	SaltsIBA 04/02/2015 - "Here we report six unrelated individuals with mutations in salt-inducible kinase 1 (SIK1) in a series of 101 persons with early myoclonic encephalopathy, Ohtahara syndrome, and infantile spasms. "
8.	Retinaldehyde (Retinal)IBA 01/01/2000 - "A neurodegenerative disorder with early myoclonic encephalopathy, retinal pigmentary degeneration and nephronophthisis."
9.	Phosphotransferases (Kinase)IBA 04/02/2015 - "Here we report six unrelated individuals with mutations in salt-inducible kinase 1 (SIK1) in a series of 101 persons with early myoclonic encephalopathy, Ohtahara syndrome, and infantile spasms. "
10.	Phenobarbital (Luminal)FDA Link 09/01/2019 - "A case of early myoclonic encephalopathy with intractable seizures successfully treated with high-dose phenobarbital."

Therapies and Procedures

1.	Ketogenic Diet 10/01/2022 - "This study describes a child with early myoclonic encephalopathy and a compound heterozygous variant in the SCN1B gene (p.Arg85Cys and c.3G>C/p.Met1), along with the child’s clinical response to anti-seizure medications (ASMs) and the ketogenic diet. " 06/01/2019 - "Ketogenic diet (KD) has been trialled only in a small group of patients with neonatal NKH and early myoclonic encephalopathy, in whom significant improvements in seizure control were reported. " 09/01/2012 - "We report three cases affected by neonatal non ketotic hyperglycinemia and early myoclonic encephalopathy treated with ketogenic diet. " 09/01/2012 - "Ketogenic diet in early myoclonic encephalopathy due to non ketotic hyperglycinemia."
2.	Therapeutics 12/01/2012 - "There is no effective therapy for early myoclonic encephalopathy and the prognosis is poor." 12/01/2012 - "There is no effective therapy for early myoclonic encephalopathy. " 01/01/1998 - "Lamotrigine can be effective therapy for juvenile myoclonic epilepsy and eyelid myoclonia with absences when used alone and, in conjunction with other antiepileptic drugs (AED) (usually valproate) for early myoclonic encephalopathy, myoclonic-astatic epilepsy and particularly, epilepsy with myoclonic absences. "