Infantile Spasms, Hypsarhythmia, and Mental Retardation

JAMA ◽  
1962 ◽  
Vol 182 (5) ◽  
pp. 523 ◽  
Author(s):  
J. Gordon Millichap
Keyword(s):  
Mental Retardation ◽  
Infantile Spasms

Infantile Spasms Due to Unilateral Cerebral Infarcts

PEDIATRICS ◽  
1987 ◽  
Vol 79 (6) ◽  
pp. 1024-1026
Author(s):  
LUIS A. ALVAREZ ◽  
SHLOMO SHINNAR ◽  
SOLOMON L. MOSHÉ
Keyword(s):  
Mental Retardation ◽  
Genetic Disorders ◽  
Infantile Spasms ◽  
Seizure Disorder ◽  
Neurologic Examination ◽  
Cerebral Infarcts ◽  
Symptomatic Group ◽  
High Incidence ◽  
Underlying Disorder ◽  
Group 1

Infantile spasms are an age-specific seizure disorder that occur in infants with no known underlying disorder or prior neurologic insult (cryptogenic group) as well as in infants with a variety of genetic disorders or known prior neurologic insult (symptomatic group).1-8 The presence of infantile spasms is associated with a high incidence of developmental retardation (87%)3 even in previously normal infants.3,5-7 Although there are many contradictory studies, it is generally believed that the infants in the symptomatic group, especially those with abnormal findings on neurologic examination prior to the onset of the seizures, have a significantly higher incidence of mental retardation and epilepsy than the infants in the cryptogenic group.1-9

scholarly journals Infantile Spasms: Little Seizures, BIG Consequences

2006 ◽  
Vol 6 (3) ◽  
pp. 63-69 ◽  
Author(s):  
W. Donald Shields
Keyword(s):  
Mental Retardation ◽  
Seizure Control ◽  
Early Recognition ◽  
Infantile Spasms ◽  
Diagnostic Evaluation ◽  
Proper Treatment ◽  
Important Impact

Infantile spasms is one of the “catastrophic childhood epilepsies” because of the difficulty in controlling seizures and the association with mental retardation. However, early recognition, a careful diagnostic evaluation, and proper treatment may allow some children to attain seizure control and to achieve a normal, or at least much improved, level of development. Thus, there is the opportunity to have an important impact in the lives of these unfortunate children and their families.

scholarly journals Functional Consequences of Mutations inCDKL5, an X-linked Gene Involved in Infantile Spasms and Mental Retardation

2006 ◽  
Vol 281 (42) ◽  
pp. 32048-32056 ◽  
Author(s):  
Ilaria Bertani ◽  
Laura Rusconi ◽  
Fabrizio Bolognese ◽  
Greta Forlani ◽  
Barbara Conca ◽  
...  
Keyword(s):  
Mental Retardation ◽  
Infantile Spasms ◽  
Linked Gene ◽  
Functional Consequences

scholarly journals West Syndrome: How Early is Early for its Presentation?

2013 ◽  
Vol 33 (3) ◽  
pp. 239-242
Author(s):  
Bikash Shrestha ◽  
Kamal Kiran ◽  
Ranjit Ghulliani
Keyword(s):  
Mental Retardation ◽  
Neonatal Period ◽  
Infantile Spasms ◽  
West Syndrome ◽  
Rare Presentation

West syndrome is the triad of mental retardation, infantile spasms and characteristic hypsarrhythmia on electroencephalograph. West syndrome usually presents in mid-infancy period. We present this as a rare presentation of West syndrome in the neonatal period. DOI: http://dx.doi.org/10.3126/jnps.v33i3.8639   J. Nepal Paediatr. Soc. 2013;33(3):239-242

scholarly journals Ohtahara syndrome progressing to West syndrome

2021 ◽  
Vol 8 (5) ◽  
pp. 941
Author(s):  
Tapan Patel ◽  
Shivani Patel
Keyword(s):  
Mental Retardation ◽  
Epileptic Encephalopathy ◽  
Infantile Spasms ◽  
West Syndrome ◽  
Epilepsy Syndrome ◽  
Ohtahara Syndrome ◽  
Severe Epilepsy

West syndrome is a severe epilepsy syndrome composed of the triad of infantile spasms, hypsarrhythmia on electroencephalography (EEG) and mental retardation. It is sometimes due to the progression of a rare and fatal condition called early infantile epileptic encephalopathy (Ohtahara syndrome). Here we describe the case of a 3 year old male, who is a known case of West syndrome, presenting with recurrent breakthrough convulsions.

Cognitive-Behavioral Consequences of Epilepsy

1994 ◽  
Vol 15 (9) ◽  
pp. 367-372
Keyword(s):  
Mental Retardation ◽  
Side Effects ◽  
Normal Development ◽  
Psychosocial Problems ◽  
Brain Dysfunction ◽  
Juvenile Myoclonic Epilepsy ◽  
Infantile Spasms ◽  
Behavioral Consequences ◽  
The Family ◽  
Child Syndrome

Children who have epilepsy (two or more unprovoked seizures) are at risk of experiencing cognitive and behavioral consequences related to: 1) the underlying brain dysfunction that resulted in the seizures, 2) the side effects of anticonvulsant drugs, and 3) psychosocial problems in the family, including the vulnerable child syndrome. Although certain epilepsy syndromes, such as infantile spasms, are associated with mental retardation or progressive intellectual decline, others typically are associated with normal development. Children who have "absence" attacks, juvenile myoclonic epilepsy, and benign Rolandic seizures, for example, generally are not impaired cognitively. When children are intellectually normal prior to the onset of epilepsy, intellectual decline is rare regardless of the number of seizures.

scholarly journals Mutations of CDKL5 Cause a Severe Neurodevelopmental Disorder with Infantile Spasms and Mental Retardation

2004 ◽  
Vol 75 (6) ◽  
pp. 1079-1093 ◽  
Author(s):  
Linda S. Weaving ◽  
John Christodoulou ◽  
Sarah L. Williamson ◽  
Kathie L. Friend ◽  
Olivia L.D. McKenzie ◽  
...  
Keyword(s):  
Mental Retardation ◽  
Neurodevelopmental Disorder ◽  
Infantile Spasms

scholarly journals Array-CGH detection of a de novo 0.7-Mb deletion in 19p13.13 includingCACNA1Aassociated with mental retardation and epilepsy with infantile spasms

Epilepsia ◽  
2009 ◽  
Vol 50 (11) ◽  
pp. 2501-2503 ◽  
Author(s):  
Stéphane Auvin ◽  
Muriel Holder-Espinasse ◽  
Marie-Dominique Lamblin ◽  
Joris Andrieux
Keyword(s):  
Mental Retardation ◽  
Array Cgh ◽  
De Novo ◽  
Infantile Spasms
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