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Injuries to the immature optic radiation (OR) are associated with thinning of the retinal nerve fiber layer and corresponding visual field (VF) defects. The aim of the current study was to seek evidence for causal retrograde trans-synaptic degeneration by exploring the correspondence between the localization and extension of the injury to the OR and the structure of the macular ganglion cell complex, and the relation to VF function. Seven adults (age range 18–35) with visual dysfunction secondary to white-matter damage of immaturity and six healthy adults (age range 22–33) underwent magnetic resonance imaging (MRI). Fiber tractography was used to generate the geniculate projections to the dorsal and ventral striate cortex, delineated by retinotopic functional MRI mapping. The structure of the macular ganglion cell complex was measured with optical coherence tomography. The tractography showed overlaps between the dorsal and ventral geniculo-striate projections. However, in four patients with inferior VF defects, the dorsal projections were to a large extent traversing the space normally solely occupied by ventral projections. This is consistent with structural changes to the OR and suggests of re-organization upon injury. Diffusion parameters were significantly different between patients and controls, and most pronounced in the dorsal geniculo-striate projections, with a pattern indicating primary injury. The macular ganglion cell complex was significantly thinner in the patients and most pronounced in the superior sectors; a pattern particularly evident in the four patients with inferior VF defects. The ratio of the mean thickness of the macular ganglion cell complex in the superior and inferior sectors significantly correlated with the axial and mean diffusivities in the contra- and ipsilateral dorsal striate projections. The results suggest a causal link between injuries to the superior portion of the immature OR and secondary thinning in the macular ganglion cell complex, resulting in inferior VF defects.

BACKGROUND AND PURPOSE: Diffusion-weighted MR imaging and fiber tractography can be used to investigate alterations in white matter tracts in patients with early acquired brain lesions and cerebral palsy. Most existing studies have used diffusion tensor tractography, which is limited in areas of complex fiber structures or pathologic processes. We explored a combined normalization and probabilistic fiber-tracking method for more realistic fiber tractography in this patient group. MATERIALS AND METHODS: This cross-sectional study included 17 children with unilateral cerebral palsy and 24 typically developing controls. DWI data were collected at 1.5T (45 directions, b=1000 s/mm2). Regions of interest were defined on a study-specific fractional anisotropy template and mapped onto subjects for fiber tracking. Probabilistic fiber tracking of the corticospinal tract and thalamic projections to the somatosensory cortex was performed by using constrained spherical deconvolution. Tracts were qualitatively assessed, and DTI parameters were extracted close to and distant from lesions and compared between groups. RESULTS: The corticospinal tract and thalamic projections to the somatosensory cortex were realistically reconstructed in both groups. Structural changes to tracts were seen in the cerebral palsy group and included splits, dislocations, compaction of the tracts, or failure to delineate the tract and were associated with underlying pathology seen on conventional MR imaging. Comparisons of DTI parameters indicated primary and secondary neurodegeneration along the corticospinal tract. Corticospinal tract and thalamic projections to the somatosensory cortex showed dissimilarities in both structural changes and DTI parameters. CONCLUSIONS: Our proposed method offers a sensitive means to explore alterations in WM tracts to further understand pathophysiologic changes following early acquired brain injury.

The development of interventional neuroradiology (INR) has increased rapidly during the past few years. A majority of cerebral aneurysms today are treated using more and more sophisticated endovascular techniques dedicated to the different types of aneurysms (fusiform/saccular, large and giant/small, wide neck/small neck, ruptured/unruptured). Also, endovascular techniques for the treatment of acute ischemic stroke are increasingly being used with highly effective results, particularly in the management of large-vessel occlusions, whereas angioplasty and stenting have a significant place in the prevention of stroke by treating cervical and intracranial stenoses. INR is the principal technique for the treatment of intracranial and spinal dural arteriovenous fistulas and also plays a significant role in the management of brain and spinal arteriovenous malformations, as well as in the presurgical embolization of cranial and spinal hypervascular tumors and in the devascularization of patients with, for example, epistaxis. Although the creation and development of INR were initiated by neuroradiologists, other medical specialists have become progressively more involved with the techniques, particularly neurosurgeons and, to a lesser extent, neurologists. Because it was a new medical practice, no specific medical training was initially available but, over time, several scientific societies began to propose guidelines for the training and practice of INR.1,2 Until now, training in INR has been a matter between trainee and teacher and, thus, the result has often been less-than-optimal training with a long learning curve, with patients paying a high price. The Union Europeenne des Medecins Specialistes (UEMS)/European Union of Medical Specialists) is the officially recognized and permanent consulting nongovernmental organization for European medical institutions; today it represents 35 national organizations and ≈1.6 million medical specialists. The UEMS is involved in professional issues important to European specialists and has a significant influence on how these issues are formulated in the directives and laws …

Diffusion MRI improves detection of abnormalities in white matter tracts in cerebral palsy (CP). Relationships between diffusion measurements and hand function are largely unexplored. We aimed first to assess microstructure of corticofugal fibers, and second to explore associations between tract injury as assessed by quantitative analysis of diffusion MRI and hand function in children with unilateral CP. Methods: In this cross-sectional study, 15 children with unilateral CP (6 boys, median age 12.4 years, min 7.2, max 17) and 24 controls were included (9 boys, median age 12.7 years, min 8.8, max 17.3). Hand function was assessed with the Box and Blocks (B&B) test. Magnetic resonance diffusion data ( b value = 1,000 s/mm 2 , 45 directions) were collected on a 1.5-T scanner. Fractional anisotropy (FA), mean diffusivity (MD), and tensor eigenvalues were measured bilaterally in the cerebral peduncle (ROI1), the posterior limb of the internal capsule (PLIC, ROI2), and corticofugal fibers connecting these regions. Results: In children with CP, FA in both ROIs and the partial tract corresponding to the affected hand was significantly lower compared to controls. This was caused by an increase in diffusivity perpendicular to the tract. After controlling for age, mean FA contralateral to the affected hand correlated with B&B scores, which was independent of lesion type or number of voxels in the partial tract, cerebral peduncle, or PLIC. Conclusions: FA in corticofugal fibers is a sensitive marker of damage to the motor system and correlates with hand function in CP. Using FA may improve early prediction of outcome. Neurology ® 2011;77:775–783

(CP). METHOD Visual field function was assessed using the confrontation technique and Goldmann perimetry in 29 children (15 males, 14 females; age range 7‐17y, median age 11y) with unilateral CP classified at Gross Motor Function Classification System (GMFCS) level I and Manual Ability Classification System levels I to III. The type and extent of brain lesions were determined using cerebral imaging. RESULTS Eighteen children had subnormal visual field function. The visual fields were severely restricted in six. The underlying brain lesions were malformation (n=7), white matter damage of immaturity (WMDI; n=13), and cortical‐subcortical lesions (n=9). Visual field function could be correlated with the pattern of brain damage in children with cortical‐subcortical lesions or extensive lesions caused by malformation or WMDI. Total homonymous hemianopia was common in the cortical‐subcortical group but rare in children with malformation or WMDI. Five children had normal visual field function despite having malformation or WMDI involving parts of the brain usually encompassing the visual system. INTERPRETATION Visual field function may be preserved by plasticity of the immature brain in children with malformation and WMDI. Severely restricted visual fields were more often associated with lesions occurring later in the developing brain. All children with severely restricted visual fields were identified by the confrontation technique. Goldmann perimetry was a suitable method to identify relative visual field defects. The cerebral lesions in children with cerebral palsy (CP) often involve parts of the brain that contain the posterior visual system (geniculate body, optic radiation, occipital cortex). Thus, visual field function may be affected in children with CP. Visual field function can be mapped using various techniques and the results expressed as the outer limits of the visual field, i.e. the furthest points at which an individual can see a strong and large stimulus, or, as the sensitivity with which an individual is able to see a small and weak object in the central field. Procedures in which a white ball is moved from the periphery towards the fixation line, with or without an arc perimeter, are referred to here as confrontation techniques, and can be used to identify the outer limits of the visual field. Goldmann perimetry is a manual perimetry technique in which changes in the size and intensity of a stimulus are used to detect relative visual field

SUMMARY The pathophysiology of pseudotumor cerebri is unclear, but may relate to an abnormality in water transport in the brain. The authors performed MR imaging in seven children with pseudotumor cerebri; the signal intensity in the white matter was normal in all patients. These data suggest that periventricular brain water content is not increased markedly in children with pseudotumor cerebri. The authors speculate that this may relate to the establishment of an equilibrium between increased resistance to cerebrospinal fluid outflow and increased brain stiffness, occurring as a consequence of increased cerebral blood-volume and/or interstitial pressure. RESUME Imagerie par resonance magnetique dans la pseudotumeur cerebrale La physiopathologie de la pseudotumeur cerebrale n'est pas claire mais pourrait etre reliee a une anomalie due au transport hydrique dans le cerveau. Les auteurs ont pratique des IRM chez sept enfants avec pseudotumeur cerebrale; l'intensite du signal dans la substance blanche etait normale chez tous les patients. Les donnees suggerent que ce contenu hydrique cerebral peri-ventriculaire n'est pas accru de facon marquee chez les enfants porteurs de pseudotumeur cerebrale. Les auteurs se demandent si cela ne pourrait s'expliquer par un equilibre entre une resistance accrue a la circulation du liquide cephalo-rachidien et un accroissement de la raideur cerebrale, apparaissant comme une consequence de l'accroissement du volume sanguin cerebral et/ou de la pression interstitielle. ZUSAMMENFASSUNG Magnet-Resonanz-Tomographie beim Peudolumor cerebri Die Pathophysiologic des Pseudotumor cerebri ist unklar, hangt aber vielleicht mit einer Unregelmasigkeit des Wassertransports im Gehirn zusammen. Die Autoren haben bei sieben Kindern mit Pseudotumor cerebri Kernspintomogramme gemacht; bei alien Patienten war die Intensitat in der weisen Substanz normal. Diese Daten lassen vermuten, das der periventrikulare Wassergehalt bei Kindern mit Pseudotumor cerebri nicht stark erhoht ist. Die Autoren sind der Meinung, das dies mit der Herstellung eines Gleichgewichtes zwischen erhohtem Widerstand gegen Liquorausflus und erhohter Hirnverhartung zusammenhangt, was als Folge eines erhohten cerebralen Blutvolumens und/oder Interstitiellen Druckes auftritt. RESUMEN Imagen por Resonancia Magnetica en el pseudotumor cerebral La fisiopatologia del pseudotumor cerebral no esta clara, pero puede relacionarse con una anomalia en el transporte del agua en el cerebro. Los autores practicaron RM en siete ninos con pseudotumor cerebral; en todos los pacientes la densidad de la substantia blanca era normal. Estos hallazgos sugieren que el contenido de agua periventricular no esta muy aumentado en ninos con pseudotumor cerebral. Los autores especulan que puede estar en relacion con el establecimiento de un equilibrio entre un aumento de la resistencia del curso del LCR y un aumento de la rigidez cerebral, como consecuencia de un aumento del volumen de sangre cerebral y/o de la presion intersticial.

SUMMARY Computed tomography (CT) of the brains of 70 children with permanent cortical visual impairment (CVI) and of 25 children who recovered from their cortical visual loss were analysed and the findings were correlated with clinical signs and symptoms. The 70 children with CVI were divided into groups depending on the known or suspected pathophysiology of CVI. Asphyxia caused permanent CVI in 34 children, of whom 16 were preterm, 17 were term and one lost vision later in life. Congenital brain malformations were the second largest group, followed by trauma, infections and shunt failure. 25 children recovered their visual acuities, but six of them were left with homonymous hemianopia. The results identified various clinical characteristics of the groups. CT scanning was helpful in understanding the pathophysiology of CVI, and provided useful information for the prognosis of visual recovery. ReSUMe Scanner cerebral d'enfants avec troubles visuels corticaux Les tomodensitometries (CT) des cerveaux de 70 enfants presentant des troubles visuels corticaux permanents (CVI) et de 25 enfants ayant recupere leur perte visuelle corticale ont ete etudiees et les donnees correlees avec les signes cliniques et les symptomes. Les 70 enfants avec CVI ont ete diffuses en deux groupes en fonction d'une physiopathologie de CVI connue ou suspectee. L'asphyxie provoquait des CVI permanents chez 34 enfants dont 16 prematures, 17 nes a terme et un ayant perdu la vision plus tardivement au cours de la vie. Les manifestations congenitales cerebrales constituaient le second groupe le plus etendu suivi par le traumatisme, l'infection et l'echec de valve. 25 enfants recuperent leur acuite visuelle mais une hemianopsie homonyme persista chez six d'entre eux. Les resultats permettent d'isoler des caracteristiques cliniques des groupes varies. Le scanner CT fut utile dans la comprehension de la physiopathologie des CVI et fournit des renseignements utiles pour le pronostic de la recuperation visuelle. ZUSAMMENFASSUNG Komputertomographie des Gehirns bei Kindern mit kortikaler Sehstorung Die Komputertomogramme (CT) des Gehirns von 70 Kindern mit permanenter kortikaler Sehstorung (CVI) und 25 Kindern, die einen kortikalen Sehverlust hatten, wurden analysiert und die Ergebnisse wurden mit klinischen Befunden und Symptomen korreliert. Die 70 Kinder mit CVI wurden entsprechend der bekannten oder vermuteten Pathophysiologie der CVI in Gruppen eingeteilt. Bei 34 Kindern wurde die CVI durch Asphyxie verursacht, davon waren 16 Fruhgeborene, 17 am Termin geboren und eins verlor das Sehvermogen erst spater. Die zweitgroβte Gruppe bildeten die kongenitalen Hirnmiβbildungen, gefolgt von Traumen, Infektionen und Ventilkomplikationen. 25 Kinder erlangten ihre Sehkraft wieder, aber sechs behielten eine homonyme Hemianopsie. Die Untersuchungsergebnisse deckten verschiedene klinische Charakteristika bei den Gruppen auf. Die CT-Untersuchungen waren fur das Verstandnis der Pathophysiologie von Bedeutung und erbrachten verwertbare Informationen fur die Prognose der Wiedererlangung des Sehvermogens. RESUMEN TAC cerebral en nihos con alteraciones del cortex cerebral La TAC cerebral de 70 ninos con alteracion visual cortical permanente (ACV) y de 25 ninos que se recuperaron de su perdida cortical visual, fueron analizadas y los hallazgos se correlacionaron con signos y sintomas clinicos. Los nifios con ACV fueron divididos en grupos segun la fisiopatologia conocida o sospechada de su ACV. La asfixia era la causa de ACV permanente en 34 ninos, de los cuales 16 eran pretermino, 17 eran a termino y uno con perdida de la vision mas tardia. El segundo grupo mas grande fueron las malformaciones cerebrales, seguidas de trauma infecciones y fallo en derivaciones. 25 ninos recuperaron sus agudezas visuales pero a diez les quedo una hemianopsia homonima. Los resultados identificaron varias caracteristicas clinicas de los grupos. La TAC fue util para entender la fisiopatologia de la ACV y proporciono una informacion util para el pronostico de la recuperacion visual.

The suggested link between autism and cerebellar dysfunction formed the background for a Swedish clinical study in 2001. Thirty-two children (17 females, 15 males; mean age 12y, SD 3y 10mo; range 6 to 21y) with a clinical suspicion of non-progressive congenital ataxia were examined, and parents were interviewed about the presence of neuropsychiatric problems in the child. Twelve children had simple ataxia, eight had ataxic diplegia, and 12 had 'borderline' ataxia. All but one of the 32 children had a mild to moderate gross motor disability according to Gross Motor Function Classification System (15 were categorized as level I, 16 as level II, and one child as level IV). Neuroimaging and neuropsychological testing were achieved in most cases. There was a strong association between learning disability* and autism spectrum disorder (often combined with hyperactivity disorder) on the one hand, and both simple and borderline 'ataxia' on the other, but a weaker link between ataxic diplegia and neuropsychiatric disorders. A correlation between cerebellar macropathology on neuroimaging and neuropsychiatric disorders was not supported. Congenital ataxia might not be a clear-cut syndrome of cerebellar disease, but one of many signs of prenatal events or syndromes, leading to a complex neurodevelopmental disorder including autism and learning disability.

The importance of UEMS is well known to many medical specialists in Europe. UEMS (Union Europeenne des Medecins Specialistes/European Union of Medical Specialists) today represents 35 national organisations, some 1.6M medical specialists, and is involved in professional issues important to European specialists. UEMS, as an officially recognised and permanent consulting organisation to the European Institutions, has a significant influence on how these issues are formulated in Directives and Laws introduced in the European Parliament. UEMS is the one and only representation of medical specialists for the European Commission and Parliament. Most decisions regarding medical specialists are prepared in cooperation with UEMS. The full members of UEMS are the National Medical Associations; each specialty is represented by a Section or a Division. To have a UEMS Section or Division means that your specialty is recognised in Europe. The Division of Neuroradiology is the face and voice of European Neuroradiology in Brussels and thus in Europe. The Division truly represents all European neuroradiologists as a "Union" and is representing neuroradiologists in professional and legal issues. Scientific issues, however, are managed by the Scientific societies, while educational and training are shared interests.

International audience; This paper reports work in progress on X-ray angiography acquired under stereotactic conditions. The objective is to be able to match multimodality images (typically MRI and X-ray) without a stereotactic frame but with stereotactic precision. We have identified potential problems and have studied them in detail. We conclude that, although the overall application is feasible, much work remains to be done on the estimation of the X-ray system conic projection and on automatic matching based on vascular structures.

Response: We thank Lee et al1 for their interest in the European Union of Medical Specialists (UEMS) Training Charter for Interventional Neuroradiology (INR). Setting standards for training where none exist can be perceived as controversial because it may imply that previous training has been insufficient or needs to be improved in one way or another. Assembling training charters, including detailed curricula defining the content of a training program, is an important task within any specialty; it is in fact so important that UEMS is using the training curriculum as the definition of a specialty. Whether there is a need to expand the number of specialists within a specialty or not is not the issue, when training guidelines are nonexistent. The important issue is to define the body of knowledge and formulate a curriculum that is accepted by those practicing the specialty. In doing so, the first priority must be patient safety, whereas professional issues, such as prestige and more or less open turf battles, must be of a lower priority. This training charter in INR is also the first such document which has been prepared and approved conjointly by representatives from all …

A case of congenital myotonic dystrophy is reported which was complicated by the development of a hydrocephalus that needed ventricular-peritoneal shunting at the age of 4 months. Although dilatation of cerebral ventricles is a common feature in these patients, an occlusive hydrocephalus has not so far been reported.

UEMS—European Union of Medical Specialists—is alobby and consulting organisation representing physicianstrained in a medical speciality in all those countries that aremembers of the European Union—EU.UEMS is organised in sections, each medical specialityrepresented by a section. To have a section in UEMS isequal to being a recognised medical speciality in Europe.There are sections of radiology, neurosurgery and neurol-ogy, just to mention a few pertinent specialities. TheSection chairmen are members of a General Assembly ofUEMS and the Council of UEMS is the executivecommittee of the organisation.TheCouncilofUEMStook,forEuropeanNeuroradiology,a very important decision in Bratislava on October 12th 2007when it approved the proposal of organising a “Division ofNeuroradiology”. This was unanimously supported by theUEMS Section of Radiology when it met in Vienna duringECRinMarchof2008.InaninauguralmeetinginBrusselsonSeptember 13th 2008, the “Division of Neuroradiology” wasformed. The officers of the Division were elected and as thefirst President of this division Professor Olof Flodmarkfrom Stockholm was elected. Professor Massimo Galluccifrom Italy was elected as General Secretary and ProfessorPeter Barsi of Hungary as Treasurer. Neuroradiology is, tothis date, the only division formed within the“family” ofRadiology.It has been a long sought goal for Neuroradiology tobecome represented in UEMS and to be recognised as adefined body of knowledge, speciality or at least as a superspeciality within the family of Radiology. The process thatended with the inauguration of the Division of Neuroradiol-ogy into UEMS in September 2008 started in the ExecutiveCommittee of European Society of Neuroradiology—ESNR—in the year of 1998. At this time neurology showeda significant interest in including Neuroradiology as asubspeciality of clinical Neurology [1]. The same year aproposal from ESNR to EAR, European Association ofRadiology, to allow Neuroradiology to be represented inUEMS within the family of Radiology, was turned down.Professor Pierre Lasjaunias wrote in 1998 and 1999, duringhis presidency of ESNR, the first documents outlining aproject with the outline of training in Neuroradiology [2]andlater the expressed goal of making Neuroradiology repre-sented in UEMS. Professor Lasjaunias wrote: “It is nowcrucial for our specialty to be actively represented togetherwith neurology and neurosurgery at the UEMS.” [3]Thework continued in several seminars during the presidency ofOlof Flodmark and in January 2002, the European Board ofNeuroradiology—EBNR—was formed.EBNR was deliberately formed on the pattern of asection of UEMS in order to create an organisation thatwould be possible to transform, with as little effort aspossible, into a section or division of UEMS. Thus EBNRestablished committees for accreditation, audit, certifica

Enhanced and non-enhanced computed tomography (CT) brain scans were performed within 72 h of surgery on 21 children in whom brain tumors had been resected totally or subtotally, and scans were repeated at varying intervals thereafter. Biopsies of the resection margins were performed in 12 patients at the end of the surgical procedure. The immediate CT scan showed enhancement in the resection margin in 13 of the 21 patients and in 9 of the 13, the enhancement disappeared on follow-up scans. There was discordance between the results of immediate CT scan examination and the biopsies of the resection margins in 7 of the 12 cases. The advantages and disadvantages of an immediate postoperative scan versus a more delayed CT scan are discussed.

CT brain scans were used to evaluate the most likely etiology of hemiplegic cerebral palsy in a group of children born at term. The population based study group comprised 83 children. The CT findings were classified to reflect the phase of brain maturation during which the injury causing hemiplegia most likely had occurred. The most common finding (37%) was periventricular atrophy considered to represent periventricular leukomalacia (PVL). PVL is a form of hypoxic-ischemic damage typical of the immature brain and most commonly seen as a complication of preterm birth. As this lesion was found in children born at term it was considered to reflect a cerebral injury that had occurred in utero. Cerebral maldevelopment, due to a very early intrauterine lesion, was detected in 17%, while cortical-/subcortical lesions, previously considered to be of major importance in hemiplegia, were seen in just 16% of the children. Altogether the CT findings indicated a prenatal origin of hemiplegia in 57%. Assessing the time of injury based on clinical records concurred with prenatal origin in 32% of the children thought to have prenatal origin of hemiplegia by CT. The objective information available from careful review of imaging information such as CT brain scans, is an important adjunct to clinical data. We propose that assessment of brain morphology, by means of imaging, should be integrated with clinical assessment when evaluating the etiology of brain lesions in children with congenital motor impairment.

4 patients with acquired arteriovenous shunts located in the wall of an ectatic vein of Galen (VG) are reported. They represent so-called VG dural arterio-venous malformations (DAVM). These shunts involve the vasa vasorum normally present in the VG wall. Physiopathology, clinical signs and age groups are usually the same as those encountered in other DAVMs; conversely they are totally different from the other mural shunts of the VG, detected in the pediatric population (true VG arteriovenous malformations), and leading to different clinical symptoms. Venous approach in these VG DAVMs is almost always contraindicated unless secondary occlusion of VG afferent has spontaneously excluded the pouch from the cerebral venous circulation.

As part of a multidisciplinary study, the rate of autism spectrum disorder (ASD), learning disability (LD), and brain abnormalities was examined in 20 participants (12 males, 8 females; age range 8mo-17y, mean age 8y 1mo) diagnosed as falling within the oculoauriculovertebral spectrum (OAV). A neuropsychiatric examination was performed, including standardized autism diagnostic interviews. Two individuals met diagnostic criteria for autism, one for autistic-like condition, and five for autistic traits. Four patients had mild LD, three severe LD, two profound LD, and two borderline intellectual functioning. Neuroimaging indicated cerebral abnormalities in more than half of the patients. Abnormalities of white/grey matter were found in more than half of examined individuals; enlargement of ventricles in more than a third. Results indicate that at least a subgroup of ASD may be associated with errors in early embryonic brain development. Awareness of the coexistence of OAV/ASD is important in habilitation care of individuals with OAV.

The use of magnetic resonance imaging (MRI) has resulted in the detection of an increasing number of children with an apparently leukodystrophic white matter. Laboratory tests and the clinical presentation, however, often do not correspond to any known entity and the course is sometimes not progressively deteriorating. Such children with white-matter changes and no known diagnosis were the subject of this Swedish multicentre study, in which MRI findings and clinical data from 100 children considered to have white-matter abnormalities were assessed during the period 1992-1995. At re-evaluation of MR images by an established "white-matter group" of neuroradiologists, paediatric neurologists, neurologists and neurochemists, the MRI signal of the white matter was considered normal in eleven children and eleven had mainly a grey matter affection. Of the remaining 78 children with white matter abnormalities, a diagnosis was found in 32, but in 46 children no diagnosis could be established. A progressive downhill course characterised 17, probably representing hitherto undefined types of leukodystrophies. Five children had a relapsing-remitting course, and in 11 it was difficult to establish whether the course was progressive or stationary. The disease was non-progressive in 13. This group of non-leukodystrophic white-matter changes obviously represents maldevelopments of myelin formation, thus dys- or hypomyelination rather than demyelination.

UMMARY This population-based study refers to 78 Swedish children with non-progressive ataxia from a total population of 3.1 million inhabitants. Inclusion criteria were ataxic gait without any signs of spasticity, dyssynergia, dysmetria and intention tremor. CT and/or MRI studies were available from 70 patients (90%). Infratentorial pathology was revealed in 27%. and findings were considered normal in 61%. If CT was normal, of recent date and of good quality, MRI did not add any new information. In half of the cases with pathological CT, however, MRI provided new information. The origin was considered prenatal in 45% (familial in 17%), perinatal in 4% and unclassifiable in 51%. 60% were mentally retarded; in the rest, cognitive development was near normal (18%) or normal (22%). Speech development was delayed in 88%, and 58% had visual dysfunction. RESUME Ataxie non evolutive. Olivine, pathologie cerebrate et handicaps chez 78 enfants suedois Cette etude de population porte sur 78 enfants suedois avec ataxie non evolutive, provenant ď unc population de 3.1 millions de habitants. Lcs criteres ď appartenance comprcnaient une demarche ataxiquc avec ou sans signes de spasticite, une dyssynergie, une dysmetrie et un tremblement intcntionnel. Des scanners et/ou une imagerie IRM etaient presents pour 70 enfants (90%). Une pathologie sous-tentorielle fut trouvee dans 27% des cas, et les donnees etaient considerees comme normales dans 61% des cas. Si le scanner etait normal, recent et de bonne qualitye, ITRM ne fournissait pas ď informations supplementaires. Cependant dans la moitie des cas de scanners anormaux, ľ IRM apportait de nouvclles donnees. ľ origine a ete consideree comme pre-natalc dans 45% des cas (17% des cas familiaux), 4% peri-natale et non classablc dans 51%. 60%etaient retardes mentaux; pour les autres, le developpement cognitif etait proche de la normale (18%) ou normal (22%). Le developpement du langage etait retarde dans 88% des cas. et il y avail une dysfonction visuelle dans 58% des cas. ZUSAMMENFASSUNG Nicht progressive Ataxie. Ursacheit. Hirnpathologie mul Storungen bei 78 schwedischen Kindern Im Rahmen dieser Populationsstudie wurden 78 schwedische Kinder mit einer nicht progressiven Ataxie aus einer Gesamtbevolkerung von 3.1 Millioncn untcrsucht. Die Kritericn fur die Aufnahme in die Studie waren ataktischer Gang ohne Zeichen einer Spastik, Dyssynergie, Dysmetrie und Intcnsionstremor. CT - und/oder MRT Untcrsuchungen lagen bei 70 Patienten vor (90%). Bei 27% fand sich eine infratentorielle Patholgie und bei 61% waren die Befunde normal. War das CT normal, vor kurzem durchgefiihrt und qualitativ in Ordnung, ergaben sich aus dem MRT keine neuen Informationen. Bei der Halfte der Falle mit pathologischem CT jedoch crgab das MRT neue Informationen. Die Ursache wurde in 45% (17% familiar) als pranatal und in 4% als perinatal angesehen und war in 5% nicht einzuordncn. 60% waren gcistig retardicrt. die restlichen hatten einc nahezu normale (18%) bzw. normale (22%) kognitive Entwicklung. Die Sprachentwicklung war bei 80% vcrzogert und 58% hatten visuelle Storungen. RESUME Ataxia no progresiva. Origen, patologia cerebral yalteraciones en 78 ninos suecos El estudio de poblacion base se refiere a 78 ninos suecos con ataxia no progresiva en una poblacion total de 3.1 millones de habitantcs. Los criterios de inclusion fueron una marcha ataxica sin signos de espasticidad. dis-sinergia, dismctria y temblor intencional. En 70 pacientes (90%) se practiceo TAC o IRM. Se hallo una patologia infratentorial en el 27% y los hallazgosfueron eonsiderados normales en el 61%. Si la TAC era normal, realizada recientemente y de buena calidad. la IRM no proporciono ninguna nueva informacion. Sin embargo, en la mitad de los casos con TAC patologica, la IRM proporciono nueva informacion. Se considero que el origen era prenatal en el 45% (familiar en el 17%). perinatal en el 4% y sin clasificaren el 51%. El 60% eran retrasados mentales; en el resto el desarrollo cognitivo era casi normal (18%) o normal (22%). El desarrollo del lenguaje estaba retrasado en el 88% y el 58% tenia una disfuncion visual.

This study has analysed the results of CT scans of the brains in children with congenital hemiplegia. The material consists of 111 out of a total of 151 children with this cerebral palsy syndrome in a population-based series. We have classified the morphological findings in five groups. The groups are designed to reflect the phase of maturation of the brain when the insult happened. The groups are: 1. Maldevelopment, 2. Periventricular atrophy, 3. Cortical-/subcortical atrophy, 4. Miscellaneous, 5. Normal. In contrast to previous reports we found a high proportion (17%) with maldevelopment. However the dominating morphological pattern was periventricular atrophy, consistent with a hypoxic-ischemic insult to the immature brain, seen in 42%. Cortical and/or subcortical atrophy was found in 12%. Three children (3%) presented with morphological patterns not possible to classify. The group with no pathology according to CT was 26%.

Magnetic resonance imaging is presently the best modality for evaluating gray/white matter anatomy. However, this modality is expensive and not yet readily available in all countries. Most neuronal migration abnormalities can be accurately diagnosed by computed tomography (CT) provided excellent scanning technique is used. Abnormalities of neuronal migration were identified with CT brain scans in 68 children and were categorized as focal heterotopias (11 patients), multifocal heterotopias (19 patients) of diffuse migration disorders (38 patients). The principal indications for imaging were correlated to the radiological features. Neuropathological confirmation of the migration abnormality was obtained in 8 patients. An increased awareness by both clinicians and radiologists together with optimal CT technique are essential for the accurate diagnosis of migration disorders in children.

Three cases of animal bites causing central nervous system injury in children are reported. Two infants suffered compound depressed skull fractures as a result of dog bites to the head. An older child suffered direct injury to the spinal cord from a tiger bite. In 2 cases, Pasteurella meningitis occurred. Pitfalls in the management of this type of problem are discussed.

We report the clinical, radiological, and neuropathological features of selective brainstem injury in an asphyxiated term infant. Disproportionate injury to thalamus, basal ganglia, and brainstem with relative sparing of cortex and subcortical white matter is observed occasionally after acute total asphyxia. Although this entity has been well documented neuropathologically, the specific computed tomographic findings have not been reported previously to our knowledge, and the clinical correlation is recognized rarely.

Aicardi's syndrome occurs in females and is associated with profound mental retardation, agenesis of the corpus callosum, chorioretinal lacunae, and infantile spasms. We report a distinctive combination of abnormalities which was observed on real-time cranial ultrasound scans of two infants with Aicardi's syndrome. These abnormalities include bilateral prominent cysts in the choroid plexus in conjunction with agenesis of the corpus callosum. Recognition of these radiologic findings may suggest the diagnosis of Aicardi's syndrome in early infancy even prior to the onset of seizures or visualization of chorioretinal lacunae. (J Child Neurol 1989;4:307-310).

A case is reported of methotrexate leukoencephalopathy presenting as an intracerebral rim-enhancing mass lesion of CT scan. The implications of this rare presentation of methotrexate leukoencephalopathy for therapy are discussed.

Two visually impaired children with occipital infarctions are presented. One patient has profound impairment of his primary visual pathway but has good vision for traveling, while the other child presented with the symptoms in reverse. We believe that these two patients provide further evidence that the primary visual pathway is used for conscious visual analysis and that the collicular visual system serves as the subconscious visual guidance for locomotion.

Fifty patients with permanent cortical visual impairment were evaluated. They had a characteristic behaviour profile, usually with residual sight but poor visual attention. 30 of the 50 also had damage to the anterior visual pathway. Visual evoked potential mapping was shown to have a clear advantage over visual evoked responses, and using that in conjunction with CT and clinical data enabled several subgroups of cortical visual impairment to be identified. The diagnosis probably is more common than previously recognised, and should be suspected when there is greater delay in visual development in other areas and the degree of visual loss is unexplained by ocular findings. Using traditional criteria for cortical blindness may mean that many children are not diagnosed, which has serious implications for their rehabilitation.

Although aseptic meningitis, lethargy and irritability occur frequently in Kawasaki disease and infantile polyarteritis nodosa, other neurological manifestations are rare. The authors report one case of Kawasaki disease and one of infantile polyarteritis nodosa, both associated with acute hemiplegia. Both patients had received courses of oral corticosteroids for their underlying disease prior to the onset of the hemiplegia. Pathological studies, as well as the four previously reported cases, are reviewed.