West syndrome

(redirected from Infantile epileptic encephalopathy)

West syn·drome

(west),
an encephalopathy in infancy characterized by infantile spasms, arrest of psychomotor development, and hypsarrhythmia.

West syn·drome

(west),
an encephalopathy in infancy characterized by infantile spasms, arrest of psychomotor development, and hypsarrhythmia.
An early onset condition characterised by a triad:
(1) Infantile spasms,
(2) Interictal EEG pattern termed hypsarrhythmia, and
(3) Mental retardation. Per the international classification, the diagnosis can be made in the presence of two of the three symptoms. The frequent onset of West syndrome in infancy suggests that CNS immaturity may play a key role in its pathogenesis. The clinical response to ACTH and glucocorticoids may be linked to suppression of corticotropin-releasing hormone production

West syndrome

Massive myoclonus Neurology An occasionally X-linked condition characterized by infantile spasms–seizures and 2º generalized epilepsy, hypsarrhythmia, encephalopathy with mental retardation and arrested psychomotor development, ± with immune dysfunction and death at an early age due to bronchopneumonia. See Salaam convulsions.

West syn·drome

(west sin'drōm)
An encephalopathy in infancy characterized by infantile spasms, arrest of psychomotor development, and hypsarrhythmia.

West,

Charles, English physician, 1816-1898.
West syndrome - an encephalopathy in infancy characterized by infantile spasms, arrest of psychomotor development, and hypsarhythmia.
References in periodicals archive ?
XEN901 is a potent, highly selective Nav1.6 sodium channel inhibitor being developed by Xenon for the treatment of epilepsy including treatment resistant adult and pediatric focal seizures, as well as rare, pediatric forms of epilepsy, such as EIEE13, an early infantile epileptic encephalopathy due to gain-of-function mutations in the SCN8A gene that encodes the Nav1.6 sodium channel.
Abulhijaa et al., "Early infantile epileptic encephalopathy associated with a high voltage gated calcium channelopathy," Journal of Medical Genetics, vol.
XEN1101 is a Kv7 potassium channel opener being developed by Xenon for the treatment of epilepsy including: treatment-resistant adult and pediatric focal seizures as well as rare, pediatric forms of epilepsy, such as EIEE7, an early infantile epileptic encephalopathy associated with mutations in the KCNQ2 gene encoding the Kv7.2 potassium channel; and potentially other neurological disorders.