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West Syndrome

Infantile Spasms


Presentation

  • Spectral components did not show significant differences compared to age matched controls at the moment of presentation.[ncbi.nlm.nih.gov]
  • 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]
  • Four of them have been followed up to the present time and have had MRI scans performed. Their present clinico-neurological features and MRI findings are described.[ncbi.nlm.nih.gov]
  • An 11-month-old girl had an onset of oculogyric crisis at 2 months and she presented with epileptic spasms and generalized tonic seizures with series formation at 3 months.[ncbi.nlm.nih.gov]
  • In this review, previous literature on optimal treatments of West syndrome and its refractory nature were briefly presented, followed by an introduction of recent publication of expert opinions from the US and Europe.[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]
  • At the last follow-up, normal group children also showed a favorable seizure prognosis (seizure control 100%).[ncbi.nlm.nih.gov]
  • Neonatal seizures occurred in 72 subjects (44.4%). The incidence of epilepsy and West syndrome was significantly higher in infants who experienced neonatal seizures than in those without seizure history.[ncbi.nlm.nih.gov]
  • , (7) etiology, (8) drug chosen as the initial treatment, (9) response to initial treatment regardless of the kind, (10) development of other seizure types after spasms were evaluated in terms of seizure and developmental outcome.[ncbi.nlm.nih.gov]
  • The generalized tonic seizures began with bi-frontal polyspikes on EEG. Our diagnosis was lissencephaly presenting with West syndrome and generalized tonic seizures. A total callosotomy was performed at 11 months.[ncbi.nlm.nih.gov]

Workup

  • 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
  • ., brainstem) and hypsarrhythmia is a secondarily generalized phenomenon produced by the brainstem.[ncbi.nlm.nih.gov]
  • For the WS patients the same dataset was obtained and compared again at the end of the study period, when hypsarrhythmia was no longer present.[ncbi.nlm.nih.gov]
  • This syndrome is associated with the pathognomonic EEG feature of hypsarrhythmia.[ncbi.nlm.nih.gov]
  • […] clinical spasms may manifest in absence of hypsarrhythmia [infantile spasms without hypsarrhythmia (ISW)].[ncbi.nlm.nih.gov]
  • PURPOSE: Hypsarrhythmia, the pathognomonic EEG pattern of West syndrome, is typically characterized by a high amplitude, arrhythmic, and asynchronous pattern.[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]
  • It consists of chaotic, high- to extremely high– voltage, polymorphic delta and theta rhythms with superimposed multifocal spikes and wave discharges. 13. Treatment:• Compared with other forms of epilepsy, West syndrome is difficult to treat.[de.slideshare.net]
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]
  • 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]
Cortical Tubers
  • The complicated clinical developments from WS with TS multiple cortical tubers related to epileptogenesis in addition to impairment of brain development.[ncbi.nlm.nih.gov]
White Matter Lesions
  • MRI showed white matter lesions with cerebral atrophy, particularly in the frontotemporal regions, and reversible abnormalities in the mesencephalon, thalami and globus pallidium resolving after fructose restriction in the diet.[ncbi.nlm.nih.gov]

Treatment

  • 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]
  • Despite potential side effects, hormonal therapy remains the main treatment for West syndrome. Here, we report on 10 patients receiving steroid treatment who presented with unusual, mostly hyperkinetic, movements.[ncbi.nlm.nih.gov]
  • Adrenocorticotropic hormone (ACTH), vigabatrin (VGB) and corticosteroids are the first-line treatments for WS.[ncbi.nlm.nih.gov]
  • It was shown that medically proven optimal treatment of West syndrome is not always the practical treatment of choice in Asian countries.[ncbi.nlm.nih.gov]
  • Therefore, the prevention of avoidable causes is at least as important as the treatment.[ncbi.nlm.nih.gov]

Prognosis

  • 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]
  • It is characterized by intractable seizures occurring almost daily, severe psychomotor retardation, poor prognosis and EEG abnormalities, known as hypsarrhythmia.[ncbi.nlm.nih.gov]
  • Although from a neuropediatric point of view, there are several benign epilepsy syndromes with a favorable prognosis, there are also several epilepsy syndromes with a poor prognosis in this age group.[onlinelibrary.wiley.com]
  • The incidence of WS is 1:3200 live births with an aetiology-dependent prognosis. Up to 80% of children with symptomatic WS suffer from mental retardation, and approximately 50% develop Lennox-Gastaut syndrome.[ncbi.nlm.nih.gov]

Etiology

  • Demographic features, treatment modalities, etiology, seizure and developmental outcome of 216 cases with West syndrome were recorded retrospectively.[ncbi.nlm.nih.gov]
  • RESULTS: The sample consisted of 15, six, 14, two, four, and 12 patients classified, on the basis of apparent time of acquisition of etiology, into the prenatal, term, preterm, postnatal, other, and no identified etiology groups, respectively.[ncbi.nlm.nih.gov]
  • We used survival analysis to investigate the influence of etiology and presence of epileptic discharges after the ACTH therapy on seizure outcome. RESULTS: Immediately after the ACTH therapy, etiology was associated with seizure outcome (p 0.003).[ncbi.nlm.nih.gov]
  • The etiology could not be ascertained in 16.6% of children. Favorable outcome was observed in 45 (30.4%) children with spasm cessation rate of 25.4% with prednisolone.[ncbi.nlm.nih.gov]
  • Etiology and investigations performed within 3 months of diagnosis were documented. RESULTS: From June 2012 to June 2014, a total of 251 infants were enrolled (53% male).[ncbi.nlm.nih.gov]

Epidemiology

  • 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]
  • 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]
  • Epidemiology The peak incidence is between 4 and 7 months of age, with 90% starting under 1 year of age. It is confined to infants and very small children.[patient.info]
Sex distribution
Age distribution

Pathophysiology

  • 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]
  • CONCLUSIONS: This unusual evolution, never previously reported, suggests that both electroclinical features mentioned above may share some pathophysiological processes genetically determined which produce a susceptibility to seizure and emphasizes that[ncbi.nlm.nih.gov]
  • In conclusion, subtraction ictal SPECT may be considered as a useful tool for presurgical evaluation of patients with West syndrome and investigation of the pathophysiology of spasms.[ncbi.nlm.nih.gov]
  • The definitive pathophysiology of the syndrome still remains unclear.[news-medical.net]

Prevention

  • 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]
  • Preventive measurements such as controlled diet and proper oral hygiene along with professional dental management are recommended in patients with west syndrome to avoid dental problems.[ncbi.nlm.nih.gov]
  • The authors also raise the possibility of prevention of mental deterioration if anticipatory treatment is started early.[ncbi.nlm.nih.gov]
  • Glucose transporter type 1 (GLUT-1) deficiency is a rare cause of preventable intellectual disability. Intellectual disability is due to refractory seizures in infancy and reduced supply of glucose to the brain.[ncbi.nlm.nih.gov]
  • Early treatment seems to be crucial for the prevention of irreversible brain damage.[ncbi.nlm.nih.gov]

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