Papers by Silvia Intruvini
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[West's syndrome in patients with cerebral paralysis and periventricular leukomalacia: a good response to treatment]
PubMed, Sep 1, 1997
Objective: To evaluate the therapeutic response of West's syndrome (WS) associated with cerebral ... more Objective: To evaluate the therapeutic response of West's syndrome (WS) associated with cerebral paralysis (CP) secondary to periventricular leucomalacia (PVL). Material and methods: We made a retrospective analysis of the clinical histories of 10 patients with SW and CP secondary to periventricular leucomalacia. We studied 10 patients, 9 boys and 1 girl with a current age of between 3 and 11 years, 8 premature newborn babies and two full term new born babies. Results: The infantile spasms (IS) started between the ages of 4-10 months (average age 8.3 months). In 100% of cases they came in runs. In one patient alone they were associated with partial motor crises. EEG showed typical hypsarrhythmia in 8 cases, asymmetrical hypsarrhythmia in 1 case and modified hypsarrhythmia in another case. Seven patients were given ACTH, associated with valproic acid in 4 cases and benzodiazepines in 2 cases. The remaining 3 patients were treated with valproate as the only drug. The IS disappeared and the EEG became normal within 14 days of the start of treatment in 9 patients who remain symptom-free after between 2 and 11.9 years follow-up. In one patient there was partial control of the spasms, and the clinical picture cleared up 10 months after starting treatment with vigabatrine. All 10 patients had spastic quadriplegia. All had mental retardation of between slight and serious degree. The cerebral CT and MR showed signs compatible with PVL and in 3 cases there was associated diffuse cerebral atrophy. Conclusions: We have found a good electro-clinical response in 10 patients with WS, CP and PVL. The results are in agreement with those of other authors.
A comprehensive evaluation of adaptive and cognitive functioning is very useful in making a relia... more A comprehensive evaluation of adaptive and cognitive functioning is very useful in making a reliable diagnosis of autism spectrum disorders (ASD). Objectives: 1. To explore the relationship between adaptive behavior, developmental level and autistic symptom severity in a group of children referred for possible ASD evaluation; 2. To evaluate the existence of a specific adaptive behavior performance profile for children with ASD in comparison to other developmental disorders. Methods: Sixty children (age range: 24 to 62 months) were assessed for autistic symptoms, intellectual functioning, and adaptive behavior. Diagnosis of autism was based on DSM-IV criteria, developmental and medical history, and the results of direct assessment and parental interview. Results: No significant differences were found between the ASD and the non-autistic developmental disorder groups in sex or chronological age. Children on the ASD group showed a significantly lower Visual Reception Mullen Score. On the Vineland Adaptive Behavior Scales there were no differences in the Daily Living or Motor Skills standard scores; however children on the ASD group performed significantly lower on the Socialization and Communication scores. A differential adaptive behavior performance profile was seen on the ASD group in which Motor Skills > Daily Living Skills > Socialization > Communication different from the adaptive behavior profile from the non-autistic developmental disorder group: Socialization > Motor Skills > Daily Living Skills > Communication. Conclusions: Significant adaptive deficits, with a characteristic profile, were observed in ASD in comparison with peers with non-autistic developmental disorders (even when matched for cognitive level).
Status dystonicus (SD) is a medical emergency leading to a significant morbidity and mortality. I... more Status dystonicus (SD) is a medical emergency leading to a significant morbidity and mortality. It can affect either patients with primary or secondary dystonia. SD is characterized by episodes of generalized muscle contraction sustained and refractory to medical treatment. Usually is possible to identify triggers as fever, infections or abrupt discontinuation of treatment. From a therapeutic point of view there are no evidence-based management guidelines in SD. OBJECTIVE: we report on four patients with status dystonicus describing their clinical manifestations, treatments and evolution. MATERIALS AND METHODS: we reviewed medical records of patients admitted to our department with a diagnosis of status dystonicus from January 2009 to June 2012. RESULTS: we present four male patients with secondary dystonia with usual drug treatment. Range of age at diagnosis was 4-13 years. Fever was the most frequent trigger. Three patients required intravenous benzodiazepines without mechanical ventilation needed; two of which showed unfavorable response, and required implementation of baclofen intrathecal infusion pump, achieving the resolution of the SD and decline in basic medication. Only one patient was managed optimizing oral medication. CONCLUSION: Status dystonicus is a very difficult therapeutic management condition. Early use of surgical techniques, such as baclofen intrathecal infusion pump should be considered before treatment failure or relapse with intravenous benzodiazepines. We emphasize the importance of preventing and controlling triggers. There is an urgent need to develop evidence-based treatment guidelines.
A comprehensive evaluation of adaptive and cognitive functioning is very useful in making a relia... more A comprehensive evaluation of adaptive and cognitive functioning is very useful in making a reliable diagnosis of autism spectrum disorders (ASD). Objectives: 1. To explore the relationship between adaptive behavior, developmental level and autistic symptom severity in a group of children referred for possible ASD evaluation; 2. To evaluate the existence of a specific adaptive behavior performance profile for children with ASD in comparison to other developmental disorders. Methods: Sixty children (age range: 24 to 62 months) were assessed for autistic symptoms, intellectual functioning, and adaptive behavior. Diagnosis of autism was based on DSM-IV criteria, developmental and medical history, and the results of direct assessment and parental interview. Results: No significant differences were found between the ASD and the non-autistic developmental disorder groups in sex or chronological age. Children on the ASD group showed a significantly lower Visual Reception Mullen Score. On the Vineland Adaptive Behavior Scales there were no differences in the Daily Living or Motor Skills standard scores; however children on the ASD group performed significantly lower on the Socialization and Communication scores. A differential adaptive behavior performance profile was seen on the ASD group in which Motor Skills > Daily Living Skills > Socialization > Communication different from the adaptive behavior profile from the non-autistic developmental disorder group: Socialization > Motor Skills > Daily Living Skills > Communication. Conclusions: Significant adaptive deficits, with a characteristic profile, were observed in ASD in comparison with peers with non-autistic developmental disorders (even when matched for cognitive level).
Acknowledgement of translators, 2020
Developmental Medicine & Child Neurology, 2020
Portuguese Spanish Camila Ara ujo Santos Santana Carolina Ayllon Carolina Corsi Patricio Barria A... more Portuguese Spanish Camila Ara ujo Santos Santana Carolina Ayllon Carolina Corsi Patricio Barria Ana Carolina de Campos Maria Brinnand Egmar Longo Marcia Cabezas Mariana Martins dos Santos Marco Casartelli S ılvia Pav~ao Hugo Galvez Isabella Pess ota Sudati Silvia Intruvini Juan Pablo Manterola Maria Jose Martinez Caceres Gustavo Pelligra Jose Manuel P erez Cordova Mario Petersen Veronica Robles Marcel Rupcich Veronica Schiariti Roselyn Valerin Allen Winter
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Papers by Silvia Intruvini