Symptoma

West Syndrome

Infantile Spasms

Information was recorded pertaining to the age at onset and presentation, etiology, and associated co-morbidities; results of electroencephalography (EEG) and neuroimaging; treatment given; and final outcome. [ncbi.nlm.nih.gov]

  • Hypopigmented Macule

    Hypomelanosis of Ito (HI) is a rare neurocutaneous disorder characterized by hypopigmented macules along the Blaschko line and multiple abnormalities, including seizures, developmental retardation, ophthalmologic defects, and chromosome mosaicism. [ncbi.nlm.nih.gov]

  • Short Extremities

    He was observed to have short extremities, and radiographs were consistent with achondroplasia. The serum alkaline phosphatase level was 2 IU/dL. [ncbi.nlm.nih.gov]

  • Seizure

    On the tenth day of treatment, he developed frequent partial seizures, characterized by being motionless during the seizure with eye deviation to the right. [ncbi.nlm.nih.gov]

We propose that a cost-effective workup for those without obvious cause after initial clinical evaluation and MRI includes an array comparative genomic hybridization (aCGH) followed by an epilepsy gene panel if the microarray is not definitive, serum [ncbi.nlm.nih.gov]

Diagnostic testing at Children’s Memorial Hermann Hospital includes a detailed metabolic workup, spinal tap and high-resolution imaging to look for structural defects. [med.uth.edu]

Diagnostic testing at Children’s Memorial Hermann Hospital includes a detailed metabolic workup, spinal tap, and high-resolution imaging to look for structural defects. [utphysicians.com]

The NISC proposed a cost-effective workup for those children in whom initial clinical assessment and brain MRI failed to identify a cause: array comparative genomic hybridization followed by an epilepsy gene panel, and, if genetic testing is not “definitive [pediatricneurologybriefs.com]

  • Hypsarrhythmia

    […] clinical spasms may manifest in absence of hypsarrhythmia [infantile spasms without hypsarrhythmia (ISW)]. [ncbi.nlm.nih.gov]

  • Multifocal Spikes

    Interictal The background EEG is often highly disorganized with high voltage irregular slow waves intermixed with multifocal spikes and polyspikes, this is termed 'hypsarrhythmia'. [epilepsydiagnosis.org]

    The interictal EEG pattern is described as hypsarrhythmic as it is characterised by asynchronous and high-amplitude slow waves and multifocal spikes. Both fast and slow variants, depending on the aetiology, have been reported. [orpha.net]

    Hypsarrhythmia is a characteristic interictal pattern of chaotic, high to extremely high-voltage polymorphic delta and theta rhythms with superimposed multifocal spikes and wave discharges. [patient.info]

  • Spike-and-Slow-Waves

    […] and slow-wave activity (Gibbs and Gibbs 1952). [medlink.com]

    Interictal EEG recording during n-REM sleep and wakefulness displayed diffuse and asynchronous high voltage spikes and slow waves suggesting the hypsarrhythmic pattern (Figure 1 A). Figure 1 A. [bmcneurol.biomedcentral.com]

    (B) Representative EEG with spike and slow waves. (C) Representative EEG with a normal background. [nrronline.org]

    It associates IS with a typical EEG pattern called hypsarrhythmia consisting of random, high-voltage, nonsynchronous spikes and slow waves and developmental delay. [nature.com]

  • Multifocal Independent Spikes

    Other patterns, such as multifocal independent spike discharges (MISD), are present in the remainder. Although infantile spasms may be associated with other EEG abnormalities, hypsarrhythmia virtually never occurs in other epilepsy syndromes. [ncbi.nlm.nih.gov]

IVIG was administered for 3 consecutive days (initial IVIG treatment) at dosages ranging from 100 to 500mg/kg/day. If spasms disappeared within 2weeks of the initial treatment, maintenance IVIG treatment was commenced. [ncbi.nlm.nih.gov]

Prognosis Many studies have examined the long-term prognosis of patients with infantile spasms. [web.archive.org]

At the last follow-up, normal group children also showed a favorable seizure prognosis (seizure control 100%). [ncbi.nlm.nih.gov]

What is the prognosis and life expectancy for West syndrome? Due to the various causes of West syndrome, each individual will have a somewhat different prognosis. [medicinenet.com]

Demographic features, treatment modalities, etiology, seizure and developmental outcome of 216 cases with West syndrome were recorded retrospectively. [ncbi.nlm.nih.gov]

Epilepsia 49:1027–1037 CrossRef PubMed Google Scholar Brna PM, Gordon KE, Dooley JM, Wood EP (2001) The epidemiology of infantile spasms. [link.springer.com]

Murphy, C.C. and Yeargin-Allsopp, M. (1999) The descriptive epidemiology of infantile spasms among Atlanta children. [dx.doi.org]

The first chapter, titled "Background," gives historical perspectives, definitions of various epileptologic terms used throughout the book, a detailed review of epidemiology of West syndrome and IS, and basic pathophysiology of seizures as a function [jamanetwork.com]

Summary Epidemiology The incidence is estimated at between 1 and 1.6/100,000 live births. Boys are more often affected than girls. Clinical description Onset occurs between 3 and 7 months of age in 50-70% of cases. [orpha.net]

Further study is needed to elucidate whether or how the immune system alteration is involved in the pathophysiology of WS. Copyright 2009 Elsevier B.V. All rights reserved. [ncbi.nlm.nih.gov]

The first chapter, titled "Background," gives historical perspectives, definitions of various epileptologic terms used throughout the book, a detailed review of epidemiology of West syndrome and IS, and basic pathophysiology of seizures as a function [jamanetwork.com]

Preventable causes still constitute a substantial portion of the etiological causes of West syndrome. Therefore, the prevention of avoidable causes is at least as important as the treatment. [ncbi.nlm.nih.gov]

Topical application of fluoride varnish can usually be used safely to prevent and treat enamel whit spot lesion. [jisppd.com]