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Epileptic Encephalopathy Type Lennox-Gastaut
Macrocephaly and Epileptic Encephalopathy

Presentation

Although not always present at the onset of seizures, most people with Lennox-Gastaut syndrome experience some degree of impaired intellectual functioning or information processing, along with developmental delays and behavioral disturbances. [ninds.nih.gov]

In some cases of Lennox-Gastaut syndrome no associated condition is present or presumed and the cause of the disorder is unknown. [rarediseases.org]

Severe behavioural disorders are nearly always present. There is also a tendency for psychosis to develop with time. [cochranelibrary.com]

Neurologic

  • Seizure

    Outcomes reported are percentage reductions in seizure frequency for total seizure frequency, atonic‐tonic seizures, absence seizures, myoclonic seizures and partial‐onset seizures. [cochranelibrary.com]

    These include myoclonic seizures, which are characterized by abnormal, jerky movements and may occur alone or in conjunction with atypical absence seizures; tonic-clonic seizures (once known as grand mal seizures), which last a couple of minutes and are [rarediseases.org]

    There may be periods of frequent seizures mixed with relatively seizure-free periods. [ninds.nih.gov]

    The most common seizure type is tonic seizures, which cause the muscles to stiffen (contract) uncontrollably. These seizures typically occur during sleep; they may also occur during wakefulness and cause sudden falls. [medlineplus.gov]

    Onset of seizures: 3 years. Cryptogenic LGS: 67%; symptomatic: 33%. Frequency of the seizures at onset: daily (83.3%) or weekly (16.7%). [ibecs.isciii.es]

  • Generalized Seizure

    This procedure is generally reserved for individuals who suffer from intractable seizures that lead to injuries (e.g., drop seizures or frequent generalized tonic-clonic seizures) or are potentially life-threatening. [rarediseases.org]

Treatment

Read More Read Less Treatment Treatment Lennox-Gastaut syndrome can be very difficult to treat. A combination of seizure medications and other treatments may be used to improve seizure control and other associated conditions. [ninds.nih.gov]

Treatments require evaluation early in its course. [cochranelibrary.com]

Treatment may require the coordinated efforts of a team of specialists. [rarediseases.org]

Treatment of Lennox-Gastaut syndrome. Cochrane Database Syst Rev. 2013 Feb 28;(2):CD003277. doi: 10.1002/14651858.CD003277.pub3. Review. Update in: Cochrane Database Syst Rev. 2021 Apr 7;4:CD003277. [medlineplus.gov]

Prognosis

Read More Read Less Prognosis Prognosis The prognosis for individuals with Lennox-Gastaut syndrome varies. There is no cure for the disorder. Complete recovery, including freedom from seizures and normal development, is very rare. [ninds.nih.gov]

Studies on the progress of LGS reveal a poor prognosis. Patients and methods. A retrospective study of 1629 patients with epilepsy was conducted. [ibecs.isciii.es]

The long‐term prognosis is poor; although the epilepsy often improves, complete seizure freedom is rare and conversely the mental and psychiatric disorders tend to worsen with time (Beaumanoir 2005). [cochranelibrary.com]

Pathophysiology

Lennox-Gastaut syndrome (LGS) is an epileptic encephalopathy that starts in childhood and has an unknown pathophysiological mechanism. Studies on the progress of LGS reveal a poor prognosis. Patients and methods. [ibecs.isciii.es]

Prevention

Other treatment options include dietary therapy with the ketogenic diet, vagus nerve stimulation, and epilepsy surgery (typically a corpus callostomy, which involves severing the band of nerve fibers that connect the two halves of the brain to prevent [ninds.nih.gov]

These impulses prevent seizures from occurring. The intensity and timing of the nerve impulses are determined based upon each individual’s needs. [rarediseases.org]

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