Outcome after corpus callosotomy in children with injurious drop attacks and severe mental retardation

2007 ◽  
Vol 29 (9) ◽  
pp. 577-585 ◽  
Author(s):  
Chaturbhuj Rathore ◽  
Mathew Abraham ◽  
Ravi Mohan Rao ◽  
Annamma George ◽  
P. Sankara Sarma ◽  
...  
Neurosurgery ◽  
2015 ◽  
Vol 78 (5) ◽  
pp. 743-751 ◽  
Author(s):  
Sarat P. Chandra ◽  
Nilesh S. Kurwale ◽  
Sarabjit Singh Chibber ◽  
Jyotirmoy Banerji ◽  
Rekha Dwivedi ◽  
...  

Abstract BACKGROUND: Corpus callosotomy is a palliative procedure especially for Lennox-Gastaut semiology without localization with drop attacks. OBJECTIVE: To describe endoscopic-assisted complete corpus callosotomy combined with anterior, hippocampal, and posterior commissurotomy. METHODS: Patients with drug refractory epilepsy having drop attacks as the predominant seizure type, bilateral abnormalities on imaging, and moderate to severe mental retardation were included. All underwent a complete workup (including magnetic resonance imaging). RESULTS: Patients (n = 16, mean age 11.4 ± 6.4 years, range 6-19 years) had a mean seizure frequency of 24.5 ± 19.8/days (range 1-60) and a mean intelligence quotient of 25.23 ± 10.71. All had syndromic diagnosis of Lennox-Gastaut syndrome, with the following etiologies: hypoxic insult (10), lissencephaly (2), bilateral band heterotropia (2), and microgyria and pachygyria (2). Surgery included complete callosotomy and the section of anterior and posterior commissure by microscopic approach through a mini craniotomy (11) and endoscopic-assisted approach (5). Complications included meningitis (1), hyperammonemic encephalopathy (2), and acute transient disconnection (5). There was no mortality or long-term morbidity. Mean follow-up was 18 ± 4.7 months (range 16-27 months). Drop attacks stopped in all. Seizure frequency/duration decreased >90% in 10 patients and >50% in 5 patients, and increased in 1 patient. All patients attained presurgical functional levels in 3 to 6 months. Child behavior checklist scores showed no deterioration. Parental questionnaires reported 90% satisfaction attributed to the control of drop attacks. The series was compared retrospectively with an age/sex-matched cohort (where a callosotomy only was performed), and showed better outcome for drop attacks (P < .003). CONCLUSION: This preliminary study demonstrated the efficacy and safety of complete callosotomy with anterior, hippocampal, and posterior commissurotomy in Lennox-Gastaut syndrome (drop attacks) with moderate to severe mental retardation.


2007 ◽  
Vol 82 (3) ◽  
pp. 239-241
Author(s):  
Anna Lauda-Świeciak ◽  
Olga Haus ◽  
Danuta Kurylak ◽  
Ewa Duszeńko ◽  
Krystyna Soszyńska

PEDIATRICS ◽  
1965 ◽  
Vol 36 (1) ◽  
pp. 62-66
Author(s):  
Edward J. O'Connell ◽  
Robert H. Feldt ◽  
Gunnar B. Stickler

The purpose of this study was to re-affirm our clinical impression that non-institutionalized children whose head circumference was below minus 2 standard deviations were mentally subnormal and frequently had growth failure. A group of 134 children with a head circumference below minus 2 standard deviations from the mean were studied, and all but one were mentally subnormal. The most severe mental retardation was noted in the group of children with a head circumference of minus 4 standard deviations or below. We found, as have others, that children with mental retardation have height and weights below the expected norm and that children with a head circumference below minus 2 standard deviations have even lower mean heights and weights. The head circumference of 31 children with growth failure and normal intelligence was normal for age and sex, therefore disproving the concept that the abnormally small child has a proportionally small head. In the child with growth failure, should the head be proportionally small (below minus 2 standard deviations), mental subnormality should be suspected. We feel that the head circumference measurement has taken on new clinical significance in that our data support its use in suspecting the association of mental subnormality in children with growth failure and a head circumference of below minus 2 standard deviations from the mean for age and sex.


1980 ◽  
Vol 2 (2) ◽  
pp. 41-50
Author(s):  
John M. Opitz

1. Approximately 3% of the population (6 to 7 million persons in the United States) is mentally retarded. Of these, severe mental retardation (IQ <50) occurs in about 10% (3 or 4 per 1,000 persons) and mild mental retardation (IQ 50 to 70) in 90%. 2. The high familial occurrence, the continuously variable phenotype shading into normality, and various genetic studies suggest that most of mild mental retardation represents the left end of the normal IQ distribution curve. Virtually no such cases can be found in the group of the severely retarded, either within or outside the institutions, suggesting that the majority of severe mental retardation represents discontinuous phenotypes due to chromosomal, environmental, mendelian, and multifactorial causes. 3. Some mild mental retardation represents syndromal occurrence (ie, mild PKU, rubella syndrome, Klinefelter syndrome); however, in most cases no anomalies are found, chromosomes are normal, height and head circumference fall within normal limits, and few have neurologic deficits, such as cerebral palsy and/or seizures. In the mildly retarded, personal, emotional and psychosocial problems predominate. The severely retarded are a biologically different group with a high incidence of gross neurologic disturbances, growth failure, abnormal head circumference, single or multiple malformations, and metabolic diseases. 4. The severely retarded are generally infertile, the mild retarded less fertile than average; however, a small minority among the latter contributes a disproportionately large number of retarded offspring to the next generation. 5. Most mental retardation can be evaluated on an outpatient basis for causal, pathogenetic, and prognostic factors. The evaluation can be economic, quick, reliable, painless, and efficient in most instances; however, CNS degenerative diseases may require a brief inpatient stay for biochemical evaluation. By all odds the most informative items in the work-up of the retarded are the (family and past) history and the (physical and neurologic) examination. Metabolic screening is usually not indicated in the malformed, neither are cytogenetic studies in the nonmalformed. 6. All patients with mental retardation deserve a diagnostic/causal evaluation and their families prognostic/genetic counseling. 7. Some 70% of mental retardation in the general population can be attributed to genetic causes. Genetic counseling in severe mental retardation is to prevent recurrence in siblings; in the mildly retarded much greater emphasis is placed on the prevention of retarded offspring.


1988 ◽  
Vol 2 ◽  
pp. 43-48
Author(s):  
Cam Wright

Down's Syndrome has long been associated with mental retardation. This has resulted in expectations of moderate or severe mental retardation in individuals with Down's Syndrome (Hopkins, 1983). Although there has been acceptance of the possibility of variability of attainments, a certain predictability of outcome has been assumed since Down's Syndrome is a condition resulting from known chromosomal abnormalities (Springer & Steele, 1980; Hopkins, 1983).


2017 ◽  
Vol 33 (11) ◽  
pp. 1883-1887 ◽  
Author(s):  
Keisuke Ueda ◽  
Sandeep Sood ◽  
Eishi Asano ◽  
Ajay Kumar ◽  
Aimee F. Luat

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