Neurogenetic disorders and treatment of associated seizures.

Title

Neurogenetic disorders and treatment of associated seizures.

Creator

Faulkner MA; Singh Sanjay P

Identifier

Publisher

Pharmacotherapy

Date

2013

Subject

Humans; Treatment Outcome; Epilepsies; Angelman Syndrome/dt [Drug Therapy]; Anticonvulsants/tu [Therapeutic Use]; Epilepsies; Rett Syndrome/dt [Drug Therapy]; Seizures/dt [Drug Therapy]; Tuberous Sclerosis/dt [Drug Therapy]; Angelman Syndrome/co [Complications]; Anticonvulsants/ad [Administration & Dosage]; Anticonvulsants/ae [Adverse Effects]; KT Synthesis; Myoclonic/co [Complications]; Myoclonic/dt [Drug Therapy]; Rett Syndrome/co [Complications]; Seizures/co [Complications]; Tuberous Sclerosis/co [Complications]

Description

Seizures are a frequent complication associated with several neurogenetic disorders. Antiepileptic medications remain the mainstay of treatment in these patients. We summarized the available data associated with various antiepileptic therapies used to treat patients with neurogenetic disorders who experienced recurrent seizures. A MEDLINE search was conducted to identify articles and abstracts describing the use of antiepileptic therapy for the treatment of various neurogenetic syndromes. Of all the neurogenetic syndromes, only autism spectrum disorders, Angelman syndrome, Rett syndrome, Dravet syndrome, and tuberous sclerosis complex were identified as having sufficient published information to evaluate therapy. Some efficacy trends were identified, including frequent successes with valproic acid with clonazepam for epilepsy with Angelman syndrome; valproic acid, stiripentol, and clobazam (triple combination therapy) for epilepsy with Dravet syndrome; and vigabatrin for infantile spasms associated with tuberous sclerosis complex. Due to a paucity of information regarding the mechanisms by which seizures are generated in the various disorders, approach to seizure control is primarily based on clinical experience and a limited amount of study data exploring patient outcomes. Although exposure of the developing brain to antiepileptic medications is of some concern, the control of epileptic activity is an important undertaking in these individuals, as the severity of eventual developmental delay often appears to correlate with the severity of seizures. As such, early aggressive therapy is warranted. 2013 Pharmacotherapy Publications, Inc.
2013

Rights

Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).

Type

Journal Article

Citation List Month

Backlog

Pages

330-43

Issue

3

Volume

33

Citation

Faulkner MA; Singh Sanjay P, “Neurogenetic disorders and treatment of associated seizures.,” Pediatric Palliative Care Library, accessed October 28, 2021, https://pedpalascnetlibrary.omeka.net/items/show/14763.

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