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]

Published on May 6, 2014 West syndrome case presentation 1. Welcome to Clinical Meeting Presented by DR. Amlendra K. Yadav DR. Chandra Shekhar Bhagat Resident (phase-A) 2. [slideshare.net]

They also present with no abnormal findings during any form of examination and there is no evidence of a presumptive trigger for the spasms. [news-medical.net]

• Developmental Delay

  Proximal duplication of chromosome 14q, including the FOXG1 gene located on 14q12, is a rare condition characterised by developmental delay, dysmorphic craniofacial features, epilepsy, and severe speech delay. [ncbi.nlm.nih.gov]

  Habilitation Therapy As the child gets developmental delay both in physical and mental ability, the chil should be given Physiotherapy, Occupational therapy and Special education regularly. [icddelhi.org]

  At the end of our study, global developmental delay persisted in 89.72%. Conclusion: The main aetiologies of West syndrome in our context are the sequelae of neonatal asphyxia and viral embryofoetopathies. [dx.doi.org]

• Shivering

  Even as I type this, that thought makes me shiver and tear up to think that we could have that kind of response from Nicholas. Unfortunately, it is not yet available nationwide due to differing state regulations on medical marijuana use. [articles.complexchild.com]

  But even today, after so many years of intensive campaign, any person who goes for a blood test would be shivering with fear till he knows that the blood report is negative for HIV. That is the fear psychosis of the AIDS. [loksabhahindi.nic.in]

• Vomiting

  […] months - - my husband and myself were commenting that we never read any of our current experiences in our parent handbook - that the hospital gave us on the night we took our little boy home as we were feeding our son via g-tube - and trying to catch his vomit [epilepsy.com]

  There was no history of fever, vomitting, respiratory problem, loss of consciousness or trauma. 5. [slideshare.net]

  It more often occurs among whites, and it most commonly affects people who are: Between ages 20 and 30 Older than age 60 Symptoms of Goodpasture Syndrome The first signs of Goodpasture syndrome may include: Fatigue Nausea and vomiting Difficulty breathing [webmd.com]

  • h/o cough-10days• No H/O LOC ,vomiting, fever, trauma.• No autonomic involvement• Perinatal history-uneventful.• Immunization up to date.• No similar history in the family.• No history of drug intake. 8. [de.slideshare.net]

• Muscle Spasm

  Skip Navigation Topic Overview Infantile spasms (West syndrome) are muscle spasms that affect a child's head, torso, and limbs. Infantile spasms usually begin before the age of 6 months. [healthy.kaiserpermanente.org]

  Topic Overview Infantile spasms (West syndrome) are muscle spasms that affect a child's head, torso, and limbs. Infantile spasms usually begin before the age of 6 months. Most children with infantile spasms have below-average intelligence. [uofmhealth.org]

  A 4-year-old boy with West syndrome, characterised by epileptic seizures and severe muscle spasm, was referred to the paediatric dentistry clinic at School of Clinical Dentistry, Sheffield. [ncbi.nlm.nih.gov]

  Topic Overview Infantile spasms (West syndrome) are muscle spasms that affect a child's head, torso, and limbs. Infantile spasms usually begin before the age of 6 months. Most children with infantile spasms have below-normal intelligence. [healthlinkbc.ca]

• Suggestibility

  Over the past two decades a few patients with favourable cognitive outcome and with total recovery from seizures were identified among the cryptogenic group suggesting an idiopathic aetiology. [ncbi.nlm.nih.gov]

  The frequent onset of West syndrome in infancy suggests that CNS immaturity may play a key role in its pathogenesis. [medical-dictionary.thefreedictionary.com]

• Seizure

  Synonyms include “lightning” or “jackknife convulsions” and “salaam seizures” ( Appleton 1993 ). [medlink.com]

  (intractable seizures), Utah, Vermont, Washington and West Virginia (intractable seizures). [medicalmarijuanainc.com]

  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]

  Two patients had pyridoxine (B6)-dependent seizures, confirmed by oral B6 trial. [journals.sbmu.ac.ir]

• Convulsions

  Epileptic spasms infantile spasms juvenile spasms Generalized flexion epilepsy Infantile epileptic encephalopathy Infantile myoclonic encephalopathy jackknife convulsions Massive myoclonia Salaam spasms electroclinical epileptic syndrome MESH:D013036 [wikidata.org]

  West: On a peculiar form of infantile convulsions. Letter. Lancet, 1840-1841, I: 724-725. T. E. Cone Jr: On a peculiar form of infantile convulsions (hypsarrhythmia) as described in his own infant son by Dr. W.J. West in 1841. [whonamedit.com]

  On a peculiar form of infantile convulsions. Lancet. 1841;1:724–725. [Original article] [West Syndrome] Newnham W. [litfl.com]

  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. [medical-dictionary.thefreedictionary.com]

  Patterns of circadian and ultradian rhythms in the heart rate (HR) are described in a full-term baby with birth asphyxia and convulsions. [ncbi.nlm.nih.gov]

• Stroke

  Metabolic syndrome is a group of five risk factors that increase the likelihood of developing heart disease, diabetes, and stroke. [healthline.com]

  Neurological Disorders and Stroke » [brainfacts.org]

  Some infants may have had a brain injury, such as a stroke or meningitis. Others may have been born with a genetic mutation or have problems with brain development. [chp.edu]

  The 54-hole tournament is stroke play. For information, check www.fsga.org. [theledger.com]

  Tuberous Sclerosis Alliance, including more videos about infantile spasms Infantile spasms information from the National Institute of Neuroloical Disorders and Stroke (NINDS) at the National Institutes of Health Facebook Live on "Infantile Spasms and [epilepsy.com]

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]

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]

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

  West syn·drome ( west ), an encephalopathy in infancy characterized by infantile spasms, arrest of psychomotor development, and hypsarrhythmia. [medical-dictionary.thefreedictionary.com]

  Overview West syndrome is a severe epileptic encephalopathy occurring in infancy that comprises specific seizure types consisting of spasms and interictal EEG pattern known as hypsarrhythmia, together with psychomotor regression. [medlink.com]

  Similarly, some children with infantile spasms never exhibit hypsarrhythmia. The absence of hypsarrhythmia is thought to be a good sign, but this has not been clearly established. [infantilespasmsproject.org]

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

• Hypsarrhythmia

  West syn·drome ( west ), an encephalopathy in infancy characterized by infantile spasms, arrest of psychomotor development, and hypsarrhythmia. [medical-dictionary.thefreedictionary.com]

  Overview West syndrome is a severe epileptic encephalopathy occurring in infancy that comprises specific seizure types consisting of spasms and interictal EEG pattern known as hypsarrhythmia, together with psychomotor regression. [medlink.com]

  Similarly, some children with infantile spasms never exhibit hypsarrhythmia. The absence of hypsarrhythmia is thought to be a good sign, but this has not been clearly established. [infantilespasmsproject.org]

  […] 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]

• Spike-and-Slow-Waves

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

  Interictal EEG displays diffuse and asynchronous high voltage spikes and slow waves suggesting the hypsarrhythmic pattern. B. Ictal EEG shows a myoclonic jerk of the upper limbs, synchronous with the generalized discharges of 4 Hz spike-wave. [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]

Treatment is unconventional, with adrenocorticotropic hormone (ACTH), vigabatrin, and high doses of oral steroids being the most effective drugs. • Effective treatment of infantile spasms should produce both cessation of spasms and resolution of hypsarrhythmia [medlink.com]

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]

Treatment optionsCommonly used first-line treatments for infants withWest syndrome include the following: ACTH Vigabatrin Prednisone Pyridoxine (vitamin B-6)Second-line treatments include the following: Benzodiazepines Valproic acid Lamotrigine Topiramate [de.slideshare.net]

It is felt that a shorter time between diagnosis and treatment will have a less deleterious effect on development compared to a longer lead-time to treatment. [rarediseases.org]

Prognosis With advancement of Science, the prognosis of children with Infantile Spasm has improved a lot during the last decades. Prognosis depends on the type of the Infantile Spasm. In general the cognitive prognosis is not so good. [icddelhi.org]

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]

Masson. [ 2 ] Trevathan, E., 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]