Early myoclonic encephalopathy

Common Name(s)

Early myoclonic encephalopathy

Early infantile epileptic encephalopathy is characterized by onset during the first months of life of erratic refractory seizures, usually myoclonic. The prognosis is poor, and most children with the condition either die within 1 to 2 years after birth or survive in a persistent vegetative state. The EEG pattern often shows a suppression-burst pattern with high-voltage bursts of slow waves mixed with multifocal spikes alternating with isoelectric suppression phases ({2:Molinari et al., 2005}). For a general phenotypic description and a discussion of genetic heterogeneity of EIEE, see EIEE1 ({308350}).
 

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Condition Specific Organizations

Following organizations serve the condition "Early myoclonic encephalopathy" for support, advocacy or research.

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Early myoclonic encephalopathy" returned 6 free, full-text research articles on human participants. First 3 results:

Successful treatment of early myoclonic encephalopathy using lidocaine and carbamazepine.
 

Author(s): Kousuke Nakano, Katsuhiro Kobayashi, Satoshi Maniwa, Nobuyuki Kodani, Yoko Ohtsuka

Journal: Epileptic Disord. 2013 Sep;15(3):352-7.

 

We report two female infants with early myoclonic encephalopathy (EME) whose intractable focal seizures were suppressed with lidocaine and carbamazepine (CBZ). Although EME is a form of early-onset epileptic encephalopathy characterised by myoclonus and focal seizures that are highly ...

Last Updated: 20 Sep 2013

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Dextromethorphan in the treatment of early myoclonic encephalopathy evolving into migrating partial seizures in infancy.
 

Author(s): Yin-Hsuan Chien, Ming-I Lin, Wen-Chin Weng, Jung-Chieh Du, Wang-Tso Lee

Journal: J. Formos. Med. Assoc.. 2012 May;111(5):290-4.

 

Epileptic encephalopathy with suppression-burst in electroencephalography (EEG) can evolve into a few types of epileptic syndromes. We present here an unusual case of early myoclonic encephalopathy that evolved into migrating partial seizures in infancy. A female neonate initially ...

Last Updated: 4 Jun 2012

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Functional cortical deafferentation from the subcortical structures in a patient with early myoclonic encephalopathy: a functional neuroimaging study.
 

Author(s): Mieko Hirose, Kazuhiro Haginoya, Hiroyuki Yokoyama, Soichiro Tanaka, Mitsugu Uematsu, Tomohiro Kaneta, Masaru Takayanagi, Kimiya Kon, Yasushi Yoshihara, Kazuie Iinuma, Shigeru Tsuchiya

Journal: Epilepsia. 2010 Apr;51(4):699-702.

 

We, for the first time, used functional neuroimaging analyses for a girl with early myoclonic encephalopathy (EME). The interictal single photon emission computed tomography (SPECT) and [18F]-fluoro-D-deoxyglucose positron emission tomography (FDG-PET) at 1 month of age showed hypoperfusion ...

Last Updated: 16 Apr 2010

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Reviews from the PubMed Database

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The terms "Early myoclonic encephalopathy" returned 1 free, full-text review articles on human participants. First 3 results:

Early-onset epileptic encephalopathies: Ohtahara syndrome and early myoclonic encephalopathy.
 

Author(s): Jules C Beal, Koshi Cherian, Solomon L Moshe

Journal: Pediatr. Neurol.. 2012 Nov;47(5):317-23.

 

Ohtahara syndrome and early myoclonic encephalopathy are the earliest presenting of the epileptic encephalopathies. They are typically distinguished from each other according to specific clinical and etiologic criteria. Nonetheless, considerable overlap exists between the two syndromes ...

Last Updated: 9 Oct 2012

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Symptoms, Diagnosis, and Treatment

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Genetics of Severe Early Onset Epilepsies
 

Status: Recruiting

Condition Summary: Epilepsy; Epileptic Encephalopathy; Ohtahara Syndrome; Infantile Spasms; Dravet Syndrome; Malignant Migrating Partial Epilepsy of Infancy; Early Myoclonic Epileptic Encephalopathy

 

Last Updated: 1 Aug 2016

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