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Dissociated motor development (DMD) is considered when the baby starts independent walking late, with normality of the other fields of development. There is evidence that babies with DMD present an atypical crawling pattern and hypotonia. To investigate the frequency and characteristics of DMD, neurological examination was performed monthly in 177 healthy full-term babies from 6 months age, in urban and rural zone samples in Brazil. Among 20 children with atypical crawling, none presented hypotonia neither did they start independent walking late. The means of the ages at the begining of atypical crawling and independent walking acquisitions, 7.40mo (SD 1.4) and 12.76mo (SD 2.5) respectively, did not differ from the group with crossed crawling pattern. Thus, in this sample of Brazilian healthy children we did not find cases with DMD.

Abstract Background Cerebral palsy (CP) patients have motor limitations that can affect functionality and abilities for activities of daily living (ADL). Health related quality of life and health status instruments validated to be applied to these patients do not directly approach the concepts of functionality or ADL. The Child Health Assessment Questionnaire (CHAQ) seems to be a good instrument to approach this dimension, but it was never used for CP patients. The purpose of the study was to verify the psychometric properties of CHAQ applied to children and adolescents with CP. Methods Parents or guardians of children and adolescents with CP, aged 5 to 18 years, answered the CHAQ. A healthy group of 314 children and adolescents was recruited during the validation of the CHAQ Brazilian-version. Data quality, reliability and validity were studied. The motor function was evaluated by the Gross Motor Function Measure (GMFM). Results Ninety-six parents/guardians answered the questionnaire. The age of the patients ranged from 5 to 17.9 years (average: 9.3). The rate of missing data was low (disability index with the visual analogue scales was not significant; for the convergent validity CHAQ disability index had a strong correlation with the GMFM (0.77); for the divergent validity there was no correlation between GMFM and the pain and overall evaluation scales; for the criterion validity GMFM as well as CHAQ detected differences in the scores among the clinical type of CP (p < 0.01); for the construct validity, the patients' disability index score (mean:2.16; SD:0.72) was higher than the healthy group (mean:0.12; SD:0.23)(p < 0.01). Conclusion CHAQ reliability and validity were adequate to this population. However, further studies are necessary to verify the influence of the ceiling effect on the responsiveness of the instrument.

Introduction Behavioral tests of auditory processing have been applied in schools and highlight the association between phonological awareness abilities and auditory processing, confirming that low performance on phonological awareness tests may be due to low performance on auditory processing tests. Objective To characterize the auditory middle latency response and the phonological awareness tests and to investigate correlations between responses in a group of children with learning disorders. Methods The study included 25 students with learning disabilities. Phonological awareness and auditory middle latency response were tested with electrodes placed on the left and right hemispheres. The correlation between the measurements was performed using the Spearman rank correlation coefficient. Results There is some correlation between the tests, especially between the Pa component and syllabic awareness, where moderate negative correlation is observed. Conclusion In this study, when phonological awareness subtests were performed, specifically phonemic awareness, the students showed a low score for the age group, although for the objective examination, prolonged Pa latency in the contralateral via was observed. Negative weak to moderate correlation for Pa wave latency was observed, as was positive weak correlation for Na-Pa amplitude.

Introduction: Behavioral and electrophysiological auditory evaluations contribute to the understanding of the auditory system and of the process of intervention., Objective: To study P300 in subjects with severe or profound sensorineural hearing loss., Methods: This was a descriptive cross-sectional prospective study. It included 29 individuals of both genders with severe or profound sensorineural hearing loss without other type of disorders, aged 11 to 42 years; all were assessed by behavioral audiological evaluation and auditory evoked potentials., Results: A recording of the P3 wave was obtained in 17 individuals, with a mean latency of 326.97ms and mean amplitude of 3.76V. There were significant differences in latency in relation to age and in amplitude according to degree of hearing loss. There was a statistically significant association of the P300 results with the degrees of hearing loss (p=0.04), with the predominant auditory communication channels (p<0.0001), and with time of hearing loss., Conclusions: P300 can be recorded in individuals with severe and profound congenital sensorineural hearing loss; it may contribute to the understanding of cortical development and is a good predictor of the early intervention outcome., (Copyright © 2014 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.)

Background: The Manual Ability Classification System (MACS) has been widely used to describe the manual ability of children with cerebral palsy (CP); however its reliability has not been verified in Brazil., Objective: To establish the inter- and intra-rater reliability of the Portuguese-Brazil version of the MACS by comparing the classifications given by therapists and parents of children with CP., Method: Data were obtained from 90 children with CP between the ages of 4 and 18 years, who were treated at the neurology and rehabilitation clinics of a Brazilian hospital. Therapists (an occupational therapist and a student) classified manual ability (MACS) through direct observation and information provided by parents. Therapists and parents used the Portuguese-Brazil version of the MACS. Intra- and inter-rater reliability was obtained using unweighted Kappa coefficient (k) and intra-class correlation coefficient (ICC). The Chi-square test was used to identify the predominance of disagreements in the classification of parents and therapists., Results: An almost perfect agreement resulted among therapists [K=0.90 (95% CI 0.83-0.97); ICC=0.97 (95%CI 0.96-0.98)], as well as with intra-rater (therapists), with Kappa ranging between 0.83 and 0.95 and ICC between 0.96 and 0.99 for the evaluator with more and less experience in rehabilitation, respectively. The agreement between therapists and parents was fair [K=0.36 (95% CI 0.22-0.50); ICC=0.79 (95% CI 0.70-0.86)]., Conclusions: The Portuguese version of the MACS is a reliable instrument to be used jointly by parents and therapists.

Background: Several studies have demonstrated the importance of using the Gross Motor Function Classification System (GMFCS) to classify gross motor function in children with cerebral palsy, but the reliability of the expanded and revised version has not been examined in Brazil (GMFCS E & R)., Objective: To determine the intra- and inter-rater reliability of the Portuguese-Brazil version of the GMFCS E & R applied by therapists and compare to classification provided by parents of children with cerebral palsy., Method: Data were obtained from 90 children with cerebral palsy, aged 4 to 18 years old, attending the neurology or rehabilitation service of a Brazilian hospital. Therapists classified the children's motor function using the GMFCS E & R and parents used the Brazilian Portuguese version of the GMFCS Family Report Questionnaire. Intra- and inter-rater reliability was obtained through percentage agreement and Cohen's unweighted Kappa statistics (k). The Chi-square test was used to identify significant differences in the classification of parents and therapists., Results: Almost perfect agreement was reached between the therapists [K=0.90 (95% confidence interval 0.83-0.97)] and intra-raters (therapists) with K=1.00 [95% confidence interval (1.00-1.00)], p<0.001. Agreement between therapists and parents was substantial (k=0.716, confidence interval 0.596-0.836), though parents classify gross motor impairment more severely than therapists (p=0.04)., Conclusions: The Portuguese version of the GMFCS E & R is reliable for use by parents and therapists. Parents tend to classify their children's limitations more severely, because they know their performance in different environments.

The goal of this study was to examine the prevalence, assessment and management of pediatric pain in a public teaching hospital. The study sample consisted of 121 inpatients (70 infants, 36 children, and 15 adolescents), their families, 40 physicians, and 43 nurses. All participants were interviewed except infants and children who could not communicate due to their clinical status. The interview included open-ended questions concerning the inpatients' pain symptoms during the 24 h preceding data collection, as well as pain assessment and pharmacological/non-pharmacological management of pain. The data were obtained from 100% of the eligible inpatients. Thirty-four children/adolescents (28%) answered the questionnaire and for the other 72% (unable to communicate), the family/health professional caregivers reported pain. Among these 34 persons, 20 children/adolescents reported pain, 68% of whom reported that they received pharmacological intervention for pain relief. Eighty-two family caregivers were available on the day of data collection. Of these, 40 family caregivers (49%) had observed their child's pain response. In addition, 74% reported that the inpatients received pharmacological management. Physicians reported that only 38% of the inpatients exhibited pain signs, which were predominantly acute pain detected during clinical procedures. They reported that 66% of patients received pharmacological intervention. The nurses reported pain signs in 50% of the inpatients, which were detected during clinical procedures. The nurses reported that pain was managed in 78% of inpatients by using pharmacological and/or non-pharmacological interventions. The findings provide evidence of the high prevalence of pain in pediatric inpatients and the under-recognition of pain by health professionals.

Introduction:  This is an objective laboratory assessment of the central auditory systems of children with learning disabilities., Aim:  To examine and determine the properties of the components of the Auditory Middle Latency Response in a sample of children with learning disabilities., Methods:  This was a prospective, cross-sectional cohort study with quantitative, descriptive, and exploratory outcomes. We included 50 children aged 8-13 years of both genders with and without learning disorders. Those with disorders of known organic, environmental, or genetic causes were excluded., Results and Conclusions:  The Na, Pa, and Nb waves were identified in all subjects. The ranges of the latency component values were as follows: Na = 9.8-32.3 ms, Pa = 19.0-51.4 ms, Nb = 30.0-64.3 ms (learning disorders group) and Na = 13.2-29.6 ms, Pa = 21.8-42.8 ms, Nb = 28.4-65.8 ms (healthy group). The values of the Na-Pa amplitude ranged from 0.3 to 6.8 ìV (learning disorders group) or 0.2-3.6 ìV (learning disorders group). Upon analysis, the functional characteristics of the groups were distinct: the left hemisphere Nb latency was longer in the study group than in the control group. Peculiarities of the electrophysiological measures were observed in the children with learning disorders. This study has provided information on the Auditory Middle Latency Response and can serve as a reference for other clinical and experimental studies in children with these disorders.

Neurological findings in a three-year-old child with meroacrania provide new insights into how the nervous system develops and functions in the absence of superior levels of control from the time of origin. The girl is the first child of a non-consanguineous white Brazilian couple, born at term, weighing 2650 g and measuring 44 cm in length. Upon examination at 43 months, she had quadriplegia, global hypotonia with occasional body hypertonia in a decorticate posture, hyperreflexia, ankle clonus, and extensor plantar response. This case allowed us to verify that, in the absence of upper structures and subcortical nuclei, there are clear signs that suggest corticospinal primacy in motor functions without a substitute pathway. Sound orientation responses suggest the independence of the vestibular-acoustic-ocular system, and manifestations of responsiveness to the environment raise questions about consciousness., (Copyright © 2010 Elsevier B.V. All rights reserved.)

Background: Spinal muscular atrophy is a common autosomal recessive neuromuscular disorder caused by mutations in the SMN1 gene. Identification of spinal muscular atrophy carriers has important implications for individuals with a family history of the disorder and for genetic counseling. The aim of this study was to determine the frequency of carriers in a sample of the nonconsanguineous Brazilian population by denaturing high-performance liquid chromatography (DHPLC)., Methods: To validate the method, we initially determined the relative quantification of DHPLC in 28 affected patients (DHPLC values: 0.00) and 65 parents (DHPLC values: 0.49-0.69). Following quantification, we studied 150 unrelated nonconsanguineous healthy individuals from the general population., Results: Four of the 150 healthy individuals tested (with no family history of a neuromuscular disorder) presented a DHPLC value in the range of heterozygous carriers (0.6-0.68)., Conclusions: Based on these results, we estimated there is a carrier frequency of 2.7% in the nonconsanguineous Brazilian population, which is very similar to other areas of the world where consanguineous marriage is not common. This should be considered in the process of genetic counseling and risk calculations., (Copyright © 2011 S. Karger AG, Basel.)

We report an 18-month-old Charcot-Marie-Tooth type 1A (CMT1A) patient who developed a rapid-onset neuropathy, with proximal and distal weakness, and non-uniform nerve conduction studies. The neuropathy responded well to immunomodulation, confirming the coexistence of an inherited and an inflammatory neuropathy. Unexpected clinical and/or electrophysiological manifestations in CMT1A patients should alert clinicians to concomitant inflammatory neuropathy. In addition, this association raises reflections about disease mechanism in CMT1A.

With increasing evidence of adverse health effects of lower lead levels (below 10microg/dL in whole blood), studies on novel internal dose biomarkers are needed. This study aimed at: (1) assessing the lead exposure by measuring this element in whole blood (Pb-blood), serum (Pb-serum), and 3 different types of saliva: whole (Pb-whole-saliva), submandibular/sublingual (Pb-sub-saliva) and parotid saliva (Pb-parotid-saliva); (2) correlating Pb-blood with Pb-serum; and (3) relating Pb-blood and Pb-serum with the lead found in the different salivas. The study population included 444 children aged 6 to 8 years attending 4 government schools in the district of Campos Eliseos, in Ribeirao Preto, Sao Paulo State, Brazil. Whole blood, serum, parotid, submandibular/sublingual ("sub-saliva"), and whole saliva were collected in trace element-free tubes. Lead concentrations were determined by inductively coupled plasma-mass spectrometry (ICP-MS). Median Pb-blood and Pb-serum were 2.1microg/dL and 0.4microg/L, respectively. Ten percent of the children had Pb-blood between 4.0 and 9.4microg/dL. Boys showed higher Pb-blood than girls (2.3 vs. 2.0microg/dL, p<0.0003). Lead concentrations in whole, sub, and parotid saliva were 1.7, 1.4, and 1.3microg/L, respectively. No significant correlations were found between Pb-blood and Pb-serum, between Pb-blood and lead in the 3 different salivas, or between Pb-serum and Pb-whole-saliva or Pb-sub-saliva. However, there was a weak statistically significant correlation between Pb-serum and Pb-parotid-saliva. In conclusion, the mean Pb-blood concentration was 2.4microg/dL, with 10% of the children exhibiting Pb-blood between 4.0 and 9.4microg/dL. Boys presented higher Pb-blood. Results suggest that Pb-blood has no correlation with Pb-serum or lead in the 3 different salivas collected here. A weak but statistically significant correlation between Pb-serum and Pb-parotid-saliva was found. Our results also point to the need for carrying out more studies on sources of exposure and lead levels in children in Brazil.

The course of myoclonic astatic epileptic syndrome (MAES) is variable and little information is available about cortical functions in the presence of the disease. The objective of the present study was to assess the phonological working memory (PWM) and the verbal language of six patients between 8 and 18 years old, on treatment for at least 5 years, and good control of seizures, diagnosed in the Service of Epilepsy of Hospital of Clinics of Ribeirão Preto, São Paulo University in Brazil. The Test of Repetition of Meaningless Words was used to assess PWM. Video-recorded of language samples were collected during spontaneous and directed activities for the study of verbal language and pragmatics. A qualitative analysis showed that all patients presented deficits in the execution of the PWM test and only one patient showed poor mastery of all aspects studied. These finds contribute to strategies of treatment for language problems of patients with MAES, focusing on PWA.

The goal of this study was to assess the relation between gender, age, motor type, topography and gross motor function, based on the Gross Motor Function System of children with cerebral palsy. Trunk control, postural changes and gait of one hundred children between 5 months and 12 years old, were evaluated. There were no significant differences between gender and age groups (p=0.887) or between gender and motor type (p=0.731). In relation to body topography most children (88%) were spastic quadriplegic. Most hemiplegics children were rated in motor level I, children with diplegia were rated in motor level III, and quadriplegic children were rated in motor level V. Functional classification is necessary to understand the differences in cerebral palsy and to have the best therapeutic planning since it is a complex disease which depends on several factors.

Unlabelled: Adjustments during postural control have been recognized in the process of the integration of movement and cognition. The objective of the present work was to describe postural changes and to verify if there is a correlation between postural adjustment and attention span in 7-month-old infants during 1 min of viewing an animated puppet., Method: Twenty-nine healthy infants (14 males) born from 31 to 39 weeks (median 36) were placed in a prone position and filmed watching a puppet during 1 min. The analysis of the images allowed us to catalogue the changes in position, the frequency of these changes, and the attention span. The following items were quantified: total number of infant positions, positions with weight transfer, changes in support, axis, decubitus, trunk and cervical movements, timing and pauses in visual attention., Results: Twenty-one infants stayed in the prone position during most of the recording, and eight chose sitting position before starting the session. Two groups were studied according to the main position throughout the filming, one in prone and the other in sitting positions, although they could rolling or crawling. For prone group the attention span was positively correlated with the number of positions with weight transfer (r=0.53, p=0.04), negatively correlated with trunk movements (r=-0.63, p=0.01), and not correlated with birth or current weight. This work suggests that changes in the trunk movements and weight transfer are different traits related to the attention in 7-month-old infants.

Background: Cerebral palsy (CP) patients have motor limitations that can affect functionality and abilities for activities of daily living (ADL). Health related quality of life and health status instruments validated to be applied to these patients do not directly approach the concepts of functionality or ADL. The Child Health Assessment Questionnaire (CHAQ) seems to be a good instrument to approach this dimension, but it was never used for CP patients. The purpose of the study was to verify the psychometric properties of CHAQ applied to children and adolescents with CP., Methods: Parents or guardians of children and adolescents with CP, aged 5 to 18 years, answered the CHAQ. A healthy group of 314 children and adolescents was recruited during the validation of the CHAQ Brazilian-version. Data quality, reliability and validity were studied. The motor function was evaluated by the Gross Motor Function Measure (GMFM)., Results: Ninety-six parents/guardians answered the questionnaire. The age of the patients ranged from 5 to 17.9 years (average: 9.3). The rate of missing data was low (<9.3%). The floor effect was observed in two domains, being higher only in the visual analogue scales (< or = 35.5%). The ceiling effect was significant in all domains and particularly high in patients with quadriplegia (81.8 to 90.9%) and extrapyramidal (45.4 to 91.0%). The Cronbach alpha coefficient ranged from 0.85 to 0.95. The validity was appropriate: for the discriminant validity the correlation of the disability index with the visual analogue scales was not significant; for the convergent validity CHAQ disability index had a strong correlation with the GMFM (0.77); for the divergent validity there was no correlation between GMFM and the pain and overall evaluation scales; for the criterion validity GMFM as well as CHAQ detected differences in the scores among the clinical type of CP (p < 0.01); for the construct validity, the patients' disability index score (mean:2.16; SD:0.72) was higher than the healthy group (mean:0.12; SD:0.23)(p < 0.01)., Conclusion: CHAQ reliability and validity were adequate to this population. However, further studies are necessary to verify the influence of the ceiling effect on the responsiveness of the instrument.

It is difficult to precisely measure articular arc movement in newborns using a goniometer. This article proposes an objective method based on trigonometry for the evaluation of lower limb abduction. With the newborn aligned in the dorsal decubitus position, 2 points are marked at the level of the medial malleolus, one on the sagittal line and the other at the end of the abduction. Using the right-sided line between these 2 points and a line from the medial malleolus to the reference point at the anterior superior iliac spine or umbilical scar, an isosceles triangle is drawn, and half of the inferential abduction angle is obtained by calculating the sine. Twenty healthy full-term newborns comprise the study cohort. Intersubject and intrasubject variability among the abduction angle values (mean [SD], 37 degrees [4] degrees) is low. This method is advantageous because the measurement is precise and because the sine can be used without approximation.

This study examined the effects of motor stimulation via treadmill on the behavior of male gerbils after external carotid ischemic brain lesion. The animals were assigned to five groups; ischemic with no stimulation (SIG), ischemic with stimulation (SIG 12/24/48/72h after surgery), non-ischemic with no stimulation (CC), non-ischemic with stimulation (CE) and sham, surgery without occlusion with no stimulation (SH). All the animals were tested in the open-field (OF) and rotarod (RR), 4 days after surgery in order to evaluate exploratory behaviors and motor performance. Data were submitted to one-way variance (ANOVA) and Dunnett's post hoc comparisons. SIG and SIG 12 groups showed a significant decrease in motor response (crossing) when compared to the control group (CC) (F=20.65, P<0.05) in the OF. SIG 12 group showed an increase in grooming behavior (F=23.136, P<0.05) and all ischemia groups (SIG, SIG12/24/48/72) spent less time on the RR (F=10.40, P<0.05), when compared to the control group (CC). Histological analyses show extensive lesions in the hippocampus and neostriatum for all groups with ischemia (SIG, SIG12/24/48/72), which are structures involved in the organization of motor behavior. Interestingly, the most pronounced damage was found in animals submitted to motor stimulation 12h after ischemia which can be correlated to the increased number of grooming behavior showed by them in the OF. These findings suggest that motor stimulation through treadmill training improve motor behavior after ischemia, except when it starts 12h after surgery.

Aim: To examine the components of auditory middle latency responses (AMLRs) in a sample of healthy children to establish their properties., Methods: Thirty-two children of both genders aged between 10 to 13 years, with no neurological disorders, were included in the study. Data were analyzed statistically by descriptive statistics (mean + SD) and by analysis of variance using the F test. AMLRs were investigated with toneburst stimuli at 50, 60 and 70 dB HL., Results and Conclusions: The mean latencies of the components were Na = 20.79 ms, Pa = 35.34 ms, Nb = 43.27 ms, and Pb = 53.36 ms, in 70 dB HL. The mean values for the NaPa amplitude ranged from 0.2 to 1.9 mV (M = 1.0 mV). The amplitude increased and latency decreased with increasing sound intensity. Inclination of the NaPa wave complex was present in some cases, which deserves attention in similar studies or in children with speech, language and auditory processing difficulties., Conclusion: This study provides additional information about AMLRs and may be a reference for others clinical and experimental studies in children.

To verify the reach of development delay investigation, we brought the experience in the pediatrics, infantile neurology and clinical genetics diagnoses, with resources of a tertiary health care, in 73 children, from 1 to 47 months age, between 1999 and 2001, attending a Stimulation Program of the Association of Parents and Friends of Exceptional Children of Batatais-SP. With a transversal and prospective method, six groups were identified: motor disturbances, dysmorphisms, malnutrition, macrocephaly, microcephaly and motor delay. In the analysis of the contribution of the antecedents, physical or laboratory exams to the diagnosis, it stands out the brain image in the groups "motor disturbances" and "macrocephaly"; and for the remaining groups, the physical examination and maternal data. The causes were detected in 48 (66%), being 38.4% of environmental and 24.6% genetics origin. It is emphasized the specialist evaluation, and the need of appropriate flow of information in the net of health.

The aim of this study was to verify developmental aspects of fetal movements through 2D ultrasonic recordings of normal fetuses, product of low risk pregnancies. A qualitative analysis of the several types of movements was performed in a sample of six fetuses, each four weeks, from 12th to 40th gestational week. In the close period to 20th gestational week was registered the largest prevalence of movements. It was observed that certain movements disappear, and others stay or appear through period. Complex movements (breathing, deglutition and suction) were more frequent after the 20th week than before. In spite of the fetuses have presented a general motor pattern, individual variations were observed. These findings are in consonance with literature, toward the existence of certain motor patterns, with emergence and disappearance of the several types of movements according to the gestational age, and individual variations that suggest specificity.

Valproic acid has been widely used for the treatment of epilepsy. Although it is usually well tolerated, it has been associated with some side effects. A poor studied side effect is the hyperammonemia, which independs from the drug hepatotoxic action. The hyperammonemia may occurs just after the beginning or during the treatment and is characterized by vomiting, progressive impairment of consciousness, focal neurologic signs and increased seizure frequency. We report boy a 6 year-old boy who presented with hyperammonemia during the use of valproic acid within the therapeutic range. Complementary investigation was negative for aminoacidopathy, organic acidemia and urea cycle disorders. The hypothesis of secondary effect to the valproic acid was reinforced by the normalization of ammonia levels after drug withdrawal. The pathogenesis of valproate-induced hyperammonemia have been discussed. We conclude that routine monitoring of ammonia blood concentration are strongly recommended in patients under valproic acid treatment.

The correction of the age of pre-term infants for the motor evaluation has been the accepted practice but it has not been clear in other areas. This study compared indicators of the acquisition and development of language, considering corrected and chronological ages. Twenty healthy infants born between the 28th and 36th week of gestation (median 32 weeks), weighing 800g to 2380g (median 1590g), 9 AGA and 11 SGA, were followed up to 15 months age. As a reference for normality, evaluation of Costa et al. (1992) was used, which groups predictable behavior in 5 levels. For receptive language, considering the chronological age, normal performance occurred at all levels except for Level I (0-3 months). For expressive language, considering the chronological age, 6 (12%) of the 50 evaluations showed normal performance. With their age corrected, in 16 evaluations (40%) the infants achieved the expected level, mainly at 6 and 12 months age. On the whole, for the chronological age, there was a larger number of AGA with normal performance (p<0.05). We conclude that with the use of the Costa method, it was unnecessary to correct the age for receptive language evaluation, and that, for the expressive, the high frequency of normal results at the corrected ages for 6 and 12 months, suggests that these ages constitute periods of intensification of vigilance.

Cerebral infarcts in children present peculiar characteristics either due to their diversity of causes or due to the unknown nature of the causes. The etiologies of cerebral infarct were reviewed in children from zero to 15 years old, attended at a tertiary hospital, in Ribeirão Preto (Brazil), from 1990 to 1997, adopting the modified Trial of ORG 10172 in Acute Stroke Treatment (TOAST) criteria of classification; 1-Atherosclerosis in large arteries; 2-Cardioembolic; 3-Occlusion of small vessels; 4-Other etiologies; 5-Undetermined cause. Thirty-nine children were included, 18 males and 21 females, aged 2 months to 15 years, mean age 5.67. The largest group, N=22 (56.4%), included children with "other etiologies", 7 of them aged under two years. The most common etiology was dehydration and septic shock leading to brain hypoperfusion and watershed infarcts. Nine (23%) children had "Undetermined etiology", 7 (17.9%) cardioembolic subtype and none had atherosclerosis. Laboratory improvement is needed for the large number of patients without a defined cause, and the high proportion of children with dehydration in the group with a determined cause emphasizes the need for preventive health actions among infants and children.

Purpose: To describe two patients with encephalocraniocutaneous lipomatosis (ECCL) and to review the literature on this disorder., Methods: Brain and orbit CT scans were performed on two patients with ECCL. Both patients were examined by the same ophthalmologist and neurologist during at least a 2-year follow-up period., Results: Unilateral skull hamartomas, intracranial abnormalities, epibulbar choristomas, and ocular adnexal changes including a specific form of cicatricial upper eyelid retraction were present in both patients., Conclusions: ECCL is a special form of oculocerebrocutaneous disease that has significant adnexal findings that are essential for the diagnosis of this rare disorder.

Ninety four neonates with hypoxic ischemic encephalopathy HIE attended at the University of Ribeirão Preto since 1982 were studied in terms of the neurological alterations during the acute phase and outcome over a mean period of 47 months. From 43 newborns with HIE I, 40 recovered within 96 hours and 3 died. Among 40 infants with HIE II, 37.5% recovered within the first week, and the others continued abnormal beyond the 7th day. All 11 infants with HIE III died before the second month of life. The HIE I group had no motor sequelae. Among the HIE II group, 34.5% showed cerebral palsy and 17.7% neuromotor retardation. 80.0% of those with sequelae persisted abnormal beyond 7th day of life, during the acute phase of the HIE. Epilepsy occurred in 17.5% of cases with HIE grade II, only among those with neuromotor sequelae. The IQ test did not show statistically significant difference between the HIE I, II without motor sequelae and the control groups. The authors reaffirm the value of the findings in the acute phase of HIE on the outcome of these patients.

A rare case of recurrent meningitis due to congenital anterior sacral meningocele and agenesis of the sacral and coccygeal vertebrae is described. An autosomal dominant inheritance is demonstrated for lower cord malformation, and environmental factors (chromic acid or fumes) are discussed.

Non-ketotic hyperglycinemia is one of inborn metabolic errors that manifest by epileptic seizures of difficult control from the first days of life in hypotonic newborn children. The lack of enzyme that catalyzes the conversion of glycine to hydroxymethyltetrahydrofolic acid, carbon dioxide and ammonia, in liver and brain, results in increased concentration of glycine in blood. It is reported in this study a case of non-ketotic hyperglycinemia diagnosed in neonatal period and characterized by hypotonia and non-controlled multiple seizures. The clinical and electroencephalographic findings, treatment as well as anatomopathologic study are discussed.

A case of neonatal apnea is reported. Clinical, laboratory, radiological and pathological findings are discussed. The failure of response to therapeutic procedures is suggestive of different cause than anoxia.

Report of a case of Maple syrup urine disease in a female neonate, with diagnosis at 26th day of life. The neurological picture consisted of alternating periods of hyper with hypotonicity, seizures, lethargy, poor feeding and respiratory arrest. Demonstration of elevation of plasma branched-chain amino-acids, was the most widely available confirmatory test, and the therapy with MSUD resulted in improvement of the patient.