[Epilepsy in children with congenital hemiparesis secondary to perinatal ictus]. / Epilepsia en niños con hemiparesia congénita secundaria a infartos cerebrales vasculares perinatales.

The objective was to describe the frequency, mode of presentation and characteristics of epilepsy in children with congenital hemiparesis (CH). It is a etrospective, descriptive and multicenter study, based on the collection of data from the clinical records of patients from 0 to 19 years with CH secondary to perinatal infarction in different centers of the community of Catalonia. A total of 310 children were included (55% males and 45% females), from a total of 13 centers in Catalonia. Average age of onset of the crises was 2 ± 1 year. Epilepsy was present in 29.5% (n = 76), among which the most frequent vascular subtype was arterial presumed perinatal ischemic stroke (51.3%), followed by neonatal arterial ischemic stroke (18.4%), periventricular venous infarction (15.8%), neonatal hemorrhagic stroke (10.5%) and neonatal cerebral sinovenous thrombosis (3.9%). Semiology of the most frequent seizures was motor focal in 82%, followed by focal motor with secondary bilateralization in 23%, focal discognitive in 13.5%, generalized by 2% and spasms in 6.5%. The 67.3% were controlled with monotherapy and the drugs used were valproate, levetiracetam or carbamazepine. The antecedent of electrical status during sleep was identified in 3 patients, all associated with extensive lesions that included the thalamus. Of the total number of children with epilepsy, 35% began with neonatal seizu res in the first 3 days of life. The 30% of children with perinatal stroke and CH present a risk of epilepsy during childhood. Children with ischemic strock have the highest risk, so they will require a follow-up aimed at detecting prematurely the epilepsy and start a treatment.

El objetivo fue describir la frecuencia, modo de presentación y características de la epilepsia en niños con hemiparesia congénita (HC). Estudio retrospectivo, descriptivo y multicéntrico, basado en la recolección de datos de las historias clínicas de pacientes de 0 a 19 años con HC secundaria a infarto perinatal en diferentes centros de la comunidad de Cataluña. Se incluyeron 310 niños (55% varones y 45% mujeres) de un total de 13 centros de Cataluña. Edad media del debut de las crisis fue de 2 ± 1 año. Presentaron epilepsia el 29.5% (n = 76), el subtipo vascular más frecuente fue el infarto presumiblemente perinatal (51.3%), seguido del accidente isquémico arterial neonatal (18.4%), infarto hemorrágico venoso periventricular (15.8%), infarto hemorrágico neonatal (10.5%) y trombosis venosa neonatal (3.9%). La semiología de las crisis más frecuente fue la focal motora en un 82%, seguida de las focales motoras con bilateralización secundaria en el 23%, focales discognitivas en 13.5%, generalizadas 2% y espasmos 6.5%. El 67.3% se controló con monoterapia y los fármacos empleados fueron el valproato, levetiracetam o carbamacepina. Se identificó el antecedente de estatus eléctrico durante el sueño en 3 pacientes, todos asociados a lesiones extensas que incluían al tálamo. Del total con epilepsia, el 35% debutaron con convulsiones neonatales en los primeros 3 días de vida. El 30% con accidente cerebrovascular perinatal y HC presentan riesgo de padecer epilepsia durante la infancia. Aquellos con infartos isquémicos tienen el riesgo más alto, por lo que requerirán un seguimiento dirigido a detectar precozmente la epilepsia e iniciar tratamiento.

Epilepsia en niños con hemiparesia congénita secundaria a infartos cerebrales vasculares perinatales / Epilepsy in children with congenital hemiparesis secondary to perinatal ictus

El objetivo fue describir la frecuencia, modo de presentación y características de la epilepsia en niños con hemiparesia congénita (HC). Estudio retrospectivo, descriptivo y multicéntrico, basado en la recolección de datos de las historias clínicas de pacientes de 0 a 19 años con HC secundaria a infarto perinatal en diferentes centros de la comunidad de Cataluña. Se incluyeron 310 niños (55% varones y 45% mujeres) de un total de 13 centros de Cataluña. Edad media del debut de las crisis fue de 2 ± 1 año. Presentaron epilepsia el 29.5% (n = 76), el subtipo vascular más frecuente fue el infarto presumiblemente perinatal (51.3%), seguido del accidente isquémico arterial neonatal (18.4%), infarto hemorrágico venoso periventricular (15.8%), infarto hemorrágico neonatal (10.5%) y trombosis venosa neonatal (3.9%). La semiología de las crisis más frecuente fue la focal motora en un 82%, seguida de las focales motoras con bilateralización secundaria en el 23%, focales discognitivas en 13.5%, generalizadas 2% y espasmos 6.5%. El 67.3% se controló con monoterapia y los fármacos empleados fueron el valproato, levetiracetam o carbamacepina. Se identificó el antecedente de estatus eléctrico durante el sueño en 3 pacientes, todos asociados a lesiones extensas que incluían al tálamo. Del total con epilepsia, el 35% debutaron con convulsiones neonatales en los primeros 3 días de vida. El 30% con accidente cerebrovascular perinatal y HC presentan riesgo de padecer epilepsia durante la infancia. Aquellos con infartos isquémicos tienen el riesgo más alto, por lo que requerirán un seguimiento dirigido a detectar precozmente la epilepsia e iniciar tratamiento.

The objective was to describe the frequency, mode of presentation and characteristics of epilepsy in children with congenital hemiparesis (CH). It is a etrospective, descriptive and multicenter study, based on the collection of data from the clinical records of patients from 0 to 19 years with CH secondary to perinatal infarction in different centers of the community of Catalonia. A total of 310 children were included (55% males and 45% females), from a total of 13 centers in Catalonia. Average age of onset of the crises was 2 ± 1 year. Epilepsy was present in 29.5% (n = 76), among which the most frequent vascular subtype was arterial presumed perinatal ischemic stroke (51.3%), followed by neonatal arterial ischemic stroke (18.4%), periventricular venous infarction (15.8%), neonatal hemorrhagic stroke (10.5%) and neonatal cerebral sinovenous thrombosis (3.9%). Semiology of the most frequent seizures was motor focal in 82%, followed by focal motor with secondary bilateralization in 23%, focal discognitive in 13.5%, generalized by 2% and spasms in 6.5%. The 67.3% were controlled with monotherapy and the drugs used were valproate, levetiracetam or carbamazepine. The antecedent of electrical status during sleep was identified in 3 patients, all associated with extensive lesions that included the thalamus. Of the total number of children with epilepsy, 35% began with neonatal seizu res in the first 3 days of life. The 30% of children with perinatal stroke and CH present a risk of epilepsy during childhood. Children with ischemic strock have the highest risk, so they will require a follow-up aimed at detecting prematurely the epilepsy and start a treatment.

Pattern of Corticospinal Projections Defined by Brain Mapping During Resective Epilepsy Surgery in a Patient with Congenital Hemiparesis and Intractable Epilepsy.

BACKGROUND: Congenital or early-onset brain structural lesions often cause contralateral hemiparesis, cognitive deficits, developmental delays, and seizures. Seizure is the most debilitating condition, as it greatly impairs quality of life in both the affected individuals and their caregivers and prevents them from active social participation. CASE DESCRIPTION: A 34-year-old man with hemiparesis and early-onset seizures since childhood owing to a congenital brain lesion developed intractable seizures in the last 2 years and was subsequently admitted for resective epileptic surgery. During the operation, we employed an innovative intraoperative neurophysiologic monitoring technique. In contrast to routine application for transcranial stimulation, we recorded compound muscle action potentials over the bilateral limb muscles simultaneously, instead of over the contralateral muscles only, to determine the patterns of the corticospinal projections. Transcranial stimulation over the bilateral hemispheres was applied before craniotomy, and direct cortical stimulation over the lesioned hemisphere was applied after craniotomy. By integrating both approaches, we could first identify the pattern of corticospinal projections before craniotomy and then accurately define the noneloquent area, which guided the resection to successfully accomplish the surgical goal. CONCLUSIONS: This technique is simple because no patient participation is required. We believe that it has the potential to replace conventional preoperative functional magnetic resonance imaging and transcranial magnetic stimulation in resective epilepsy surgery, particularly for young patients. Not only can it improve the safety of surgical procedures, but also it can help predict functional outcome.

Using theoretical models from adult stroke recovery to improve use of noninvasive brain stimulation for children with congenital hemiparesis.

Noninvasive brain stimulation has been widely used for adults with stroke to improve upper limb motor function. A recent study by Kirton and colleagues (Kirton A, Ciechanski P, Zewdie E, Andersen J, Nettel-Aguirre A, Carlson H, Carsolio L, Herrero M, Quigley J, Mineyko A, Hodge J, Hill M. Neurology 88: 259-267, 2017) applied noninvasive brain stimulation to children with congenital hemiparesis but found no significant effect of noninvasive brain stimulation on motor function. Here, we explore theories about cortical reorganization in both adult and children with hemiparesis and discuss how to improve the approaches of noninvasive brain stimulation to generate optimal motor improvement and development for children with congenital hemiparesis.

Preferencia manual precoz: signo de alarma de accidente cerebrovascular perinatal. Serie de casos / Early handedness: Warning sign of perinatal stroke. Case series

Accidente cerebrovascular presuntamente perinatal es la expresión utilizada para los casos en los que se diagnostica un accidente cerebrovascular antiguo por sus secuelas y no por la sintomatología aguda. Muchos accidentes cerebrovasculares presuntamente perinatales tienen como primera manifestación una hemiparesia congénita, la cual se hace notoria entre el cuarto y el octavo mes de vida como preferencia manual precoz. Es por esto por lo que la preferencia manual clara y persistente desarrollada antes del año de vida debe asumirse como un signo de alarma de una probable secuela motora. En este trabajo, revisamos la historia clínica de 15 casos de accidente cerebrovascular presuntamente perinatal para evaluar la edad en la que la consulta derivó en el diagnóstico, el motivo de consulta y la edad de desarrollo de la preferencia manual.

Presumed perinatal ischemic stroke is the term used for cases in which an old stroke is diagnosed by the consequences of it and not by the acute symptoms. Many presumed perinatal ischemic strokes have congenital hemiparesis as the first manifestation, which is usually noticed between the fourth and eighth month of life as early hand preference. That is why the clear and persistent handedness developed before one year of age must be assumed as a warning sign of probable motor sequelae. In this paper we review the medical records of 15 cases of presumed perinatal ischemic stroke to assess the age at which the consultation led to the diagnosis, reason for consultation and age at development of handedness.

[Preschool education impact on child development]. / El impacto de la educación inicial en el desarrollo infantil.

Presumed perinatal ischemic stroke is the term used for cases in which an old stroke is diagnosed by the consequences of it and not by the acute symptoms. Many presumed perinatal ischemic strokes have congenital hemiparesis as the first manifestation, which is usually noticed between the fourth and eighth month of life as early hand preference. That is why the clear and persistent handedness developed before one year of age must be assumed as a warning sign of probable motor sequelae. In this paper we review the medical records of 15 cases of presumed perinatal ischemic stroke to assess the age at which the consultation led to the diagnosis, reason for consultation and age at development of handedness.

Accidente cerebrovascular presuntamente perinatal es la expresión utilizada para los casos en los que se diagnostica un accidente cerebrovascular antiguo por sus secuelas y no por la sintomatología aguda. Muchos accidentes cerebrovasculares presuntamente perinatales tienen como primera manifestación una hemiparesia congénita, la cual se hace notoria entre el cuarto y el octavo mes de vida como preferencia manual precoz. Es por esto por lo que la preferencia manual clara y persistente desarrollada antes del año de vida debe asumirse como un signo de alarma de una probable secuela motora. En este trabajo, revisamos la historia clínica de 15 casos de accidente cerebrovascular presuntamente perinatal para evaluar la edad en la que la consulta derivó en el diagnóstico, el motivo de consulta y la edad de desarrollo de la preferencia manual.

Predicting hand function after hemidisconnection.

Hemidisconnections (i.e. hemispherectomies or hemispherotomies) invariably lead to contralateral hemiparesis. Many patients with a pre-existing hemiparesis, however, experience no deterioration in motor functions, and some can still grasp with their paretic hand after hemidisconnection. The scope of our study was to predict this phenomenon. Hypothesizing that preserved contralateral grasping ability after hemidisconnection can only occur in patients controlling their paretic hands via ipsilateral corticospinal projections already in the preoperative situation, we analysed the asymmetries of the brainstem (by manual magnetic resonance imaging volumetry) and of the structural connectivity of the corticospinal tracts within the brainstem (by magnetic resonance imaging diffusion tractography), assuming that marked hypoplasia or Wallerian degeneration on the lesioned side in patients who can grasp with their paretic hands indicate ipsilateral control. One hundred and two patients who underwent hemidisconnections between 0.8 and 36 years of age were included. Before the operation, contralateral hand function was normal in 3/102 patients, 47/102 patients showed hemiparetic grasping ability and 52/102 patients could not grasp with their paretic hands. After hemidisconnection, 20/102 patients showed a preserved grasping ability, and 5/102 patients began to grasp with their paretic hands only after the operation. All these 25 patients suffered from pre- or perinatal brain lesions. Thirty of 102 patients lost their grasping ability. This group included all seven patients with a post-neonatally acquired or progressive brain lesion who could grasp before the operation, and also all three patients with a preoperatively normal hand function. The remaining 52/102 patients were unable to grasp pre- and postoperatively. On magnetic resonance imaging, the patients with preserved grasping showed significantly more asymmetric brainstem volumes than the patients who lost their grasping ability. Similarly, these patients showed striking asymmetries in the structural connectivity of the corticospinal tracts. In summary, normal preoperative hand function and a post-neonatally acquired or progressive lesion predict a loss of grasping ability after hemidisconnection. A postoperatively preserved grasping ability is possible in hemiparetic patients with pre- or perinatal lesions, and this is highly likely when the brainstem is asymmetric and especially when the structural connectivity of the corticospinal tracts within the brainstem is asymmetric.

Mirror movements in unilateral spastic cerebral palsy: Specific negative impact on bimanual activities of daily living.

AIM: Mirror movements are involuntary movements of the other hand during voluntary unimanual movements. Some, but not all children with unilateral spastic cerebral palsy (USCP) show this phenomenon. In this observational study, we investigated whether these mirror movements have a specific negative impact on bimanual activities of daily living. METHODS: Eighteen children (six girls; age range, 6-16 years; mean age, 12 years 1 month; SD, 3 years 3 month) with USCP, nine with and nine without mirror movements, underwent the Jebsen Taylor Hand Function Test (unimanual capacity) and the Assisting Hand Assessment (bimanual performance). In addition, we measured the time the participants needed for the completion of five activities we had identified as particularly difficult for children with mirror movements. RESULTS: Multivariate analysis demonstrated that mirror movements indeed have a specific negative impact on bimanual performance (Assisting Hand Assessment) and on the time needed for the completion of these five particularly difficult activities. This effect was independent from unimanual capacity. CONCLUSION: Functional therapies in children with USCP and mirror movements should address this phenomenon.

Microstructure of transcallosal motor fibers reflects type of cortical (re-)organization in congenital hemiparesis.

BACKGROUND: Early unilateral brain lesions can lead to different types of corticospinal (re-)organization of motor networks. In one group of patients, the contralesional hemisphere exerts motor control not only over the contralateral non-paretic hand but also over the (ipsilateral) paretic hand, as the primary motor cortex is (re-)organized in the contralesional hemisphere. Another group of patients with early unilateral lesions shows "normal" contralateral motor projections starting in the lesioned hemisphere. AIM: We investigated how these different patterns of cortical (re-)organization affect interhemispheric transcallosal connectivity in patients with congenital hemiparesis. METHOD: Eight patients with ipsilateral motor projections (group IPSI) versus 7 patients with contralateral motor projections (group CONTRA) underwent magnetic resonance diffusion tensor imaging (DTI). The corpus callosum (CC) was subdivided in 5 areas (I-V) in the mid-sagittal slice and volumetric information. The following diffusion parameters were calculated: fractional anisotropy (FA), trace, radial diffusivity (RD), and axial diffusivity (AD). RESULTS: DTI revealed significantly lower FA, increased trace and RD for group IPSI compared to group CONTRA in area III of the corpus callosum, where transcallosal motor fibers cross the CC. In the directly neighboring area IV, where transcallosal somatosensory fibers cross the CC, no differences were found for these DTI parameters between IPSI and CONTRA. Volume of callosal subsections showed significant differences for area II (connecting premotor cortices) and III, where group IPSI had lower volume. INTERPRETATION: The results of this study demonstrate that the callosal microstructure in patients with congenital hemiparesis reflects the type of cortical (re-)organization. Early lesions disrupting corticospinal motor projections to the paretic hand consecutively affect the development or maintenance of transcallosal motor fibers.

Postural adaptation during arm raising in children with and without unilateral cerebral palsy.

Postural sway during arm movements were related to the size of the base of support (BOS) and the limits of stability (LOS) of children with unilateral cerebral palsy (USCP) and typically developing (TD) children. For half of the trials the mechanical disturbance due to the rapid arm movement was increased by attaching small weights at the wrists. The participants stood with both feet on a large force plate, which recorded the displacements of the center of pressure (CoP). The results showed that in the children with USCP the LOS forward and toward the non-dominant (more-affected) side were smaller than in the TD children whereas the LOS backward and toward the dominant (less-affected) side did not differ between the two groups. When rapidly moving the arms the children with USCP swayed over a larger portion of their base of support in the forward direction and toward their more-affected side. In addition, the maximal sway toward the more-affected side during arm movement exceeded the LOS while balance was maintained. These effects increased when the movements were performed with the weights at the wrists. These results show that an area of permissible sway, which was not spontaneously explored during the leaning task, was required to maintain balance during the supra-postural task. Training to enlarge the LOS that includes weight shifts toward the more-affected side might reduce the area of the BOS that is self-perceived as less secure.

Reduced integrity of sensorimotor projections traversing the posterior limb of the internal capsule in children with congenital hemiparesis.

There is reduced integrity of corticospinal projections that traverse the posterior limb of the internal capsule (PLIC) in children with unilateral cerebral palsy (CP). It remains unclear whether there are changes in integrity of other projections traversing the PLIC. Forty children with congenital hemiparesis and 15 typically developing children underwent structural and diffusion-weighted MRI. All children with congenital hemiparesis showed lesions to the periventricular white matter. Structural images were parcellated into 34 cortical regions per hemisphere and posterior limb of the internal capsule was identified. PLIC connections to each cortical region were extracted using probabilistic tractography. Differences between hemispheres for each cortical projection (asymmetry index (AI)) and tract microstructure (fractional anisotropy (FA), mean diffusivity (MD)) were assessed. The results showed that 17 children (42.5%) with congenital hemiparesis showed bilateral lesions on structural MRI. Projections to the primary motor cortex (precentral gyrus and paracentral lobule) showed greater asymmetry in unilateral CP group compared to typically developing children and indicate reduced projections on the hemisphere contralateral to the impaired limb (i.e., contralateral hemisphere). Reduced FA and increased MD were also observed for connections with the primary motor cortex, primary sensory cortex (postcentral gyrus) and precuneus on the contralateral hemisphere in children with congenital hemiparesis. Similar changes were observed between children with unilateral and bilateral lesions on structural MRI. Notably, microstructural changes were associated with deficits in both sensory and motor function. The findings further unravel the underlying neuroanatomical correlates of sensorimotor deficits in children with congenital hemiparesis.

Congenital hemiparesis, unilateral polymicrogyria and epilepsy with or without status epilepticus during sleep: a study of 66 patients with long-term follow-up.

AIM: We retrospectively analysed the electroclinical features, treatment, and outcome in patients with unilateral polymicrogyria (PMG), focussing on epileptic syndrome with or without encephalopathy, with status epilepticus during sleep (ESES) or continuous spikes and waves during slow sleep (CSWS) syndrome. METHODS: From June 1990 to December 2012, 39 males and 27 females, aged 5-26 years, were studied. We did not include patients with bilateral PMG or cases with unilateral PMG associated with other cerebral lesions. The mean follow-up period was 12 years (range: 3-22 years). RESULTS: Mean age at epilepsy onset was 6.5 years. Focal motor seizures occurred in all cases and 25 had secondary generalised seizures. Six patients also had complex focal seizures. Interictal EEG recordings showed focal spikes in all cases. For 43 of 53 patients with epilepsy, aged 2-9.5 years, the electroclinical features changed. An increase in frequency of focal motor seizures was reported in 20 patients, negative myoclonus occurred in 32 patients, atypical absences in 25 patients, and positive myoclonus in 19 patients. All patients had a continuous symmetric or asymmetric pattern of spike-wave activity during slow-wave sleep. CONCLUSION: For patients presenting with congenital hemiparesis, negative or positive myoclonus, and absences and focal motor seizures with ESES/CSWS, unilateral PMG should be considered. Brain MRI is mandatory to confirm this cortical malformation. The most commonly used treatments were clobazam, ethosuximide, and sulthiame, alone or in combination. For refractory cases, high-dose steroids were administered and surgery was performed in two patients. Outcome was relatively benign.

Immediate improvement of motor function after epilepsy surgery in congenital hemiparesis.

Hemispherectomy often leads to a loss of contralateral hand function. In some children with congenital hemiparesis, however, paretic hand function remains unchanged. An immediate improvement of hand function has never been reported. A 17-year-old boy with congenital hemiparesis and therapy-refractory seizures due to a large infarction in the territory of the middle cerebral artery underwent epilepsy surgery. Intraoperatively, electrical cortical stimulation of the affected hemisphere demonstrated preserved motor projections from the sensorimotor cortex to the (contralateral) paretic hand. A frontoparietal resection was performed, which included a complete disconnection of all motor projections originating in the sensorimotor cortex of the affected hemisphere. Surprisingly, the paretic hand showed a significant functional improvement immediately after the operation. This observation demonstrates that, in congenital hemiparesis, crossed motor projections from the affected hemisphere are not always beneficial, but can be dysfunctional, interfering with ipsilateral motor control over the paretic hand by the contralesional hemisphere.