Dyke-Davidoff-Masson syndrome: case report of fetal unilateral ventriculomegaly and hypoplastic left middle cerebral artery

Prenatal ultrasonographic detection of unilateral cerebral ventriculomegaly arises suspicion of pathological condition related to cerebrospinal fluid flow obstruction or cerebral parenchimal pathology. Dyke-Davidoff-Masson syndrome is a rare condition characterized by cerebral hemiatrophy, calvarial thickening, skull and facial asymmetry, contralateral hemiparesis, cognitive impairment and seizures. Congenital and acquired types are recognized and have been described, mainly in late childhood, adolescence and adult ages. We describe a female infant with prenatal diagnosis of unilateral left ventriculomegaly in which early brain MRI and contrast enhanced-MRI angiography, showed cerebral left hemiatrophy associated with reduced caliber of the left middle cerebral artery revealing the characteristic findings of the Dyke-Davidoff-Masson syndrome. Prenatal imaging, cerebral vascular anomaly responsible for the cerebral hemiatrophy and the early clinical evolution have never been described before in such a young child and complete the acquired clinical descriptions in older children. Differential diagnosis, genetic investigations, neurophysiologic assessments, short term clinical and developmental follow up are described. Dyke-Davidoff-Masson syndrome must be ruled out in differential diagnosis of fetal unilateral ventriculomegaly. Early clinical assessment, differential diagnosis and cerebral imaging including cerebral MRI angiography allow the clinicians to diagnose also in early infancy this rare condition

Consensus recommendations for transcranial color-coded duplex sonography for the assessment of intracranial arteries in clinical trials on acute stroke

BACKGROUND AND PURPOSE: Transcranial color-coded duplex sonography has become a standard diagnostic technique to assess the intracranial arterial status in acute stroke. It is increasingly used for the evaluation of prognosis and the success of revascularization in multicenter trials. The aim of this international consensus procedure was to develop recommendations on the methodology and documentation to be used for assessment of intracranial occlusion and for monitoring of recanalization. METHODS: Thirty-five experts participated in the consensus process. The presented recommendations were approved during a meeting of the consensus group in October 2008 in Giessen, Germany. The project was an initiative of the German Competence Network Stroke and performed under the auspices of the Neurosonology Research Group of the World Federation of Neurology. RESULTS: Recommendations are given on how examinations should be performed in the time-limited situation of acute stroke, including criteria to assess the quality of the acoustic bone window, the use of echo contrast agents, and the evaluation of intracranial vessel status. The important issues of the examiners' training and experience, the documentation, and analysis of study results are addressed. One central aspect was the development of standardized criteria for diagnosis of arterial occlusion. A transcranial color-coded duplex sonography recanalization score based on objective hemodynamic criteria is introduced (consensus on grading intracranial flow obstruction [COGIF] score). CONCLUSIONS: This work presents consensus statements in an attempt to standardize the application of transcranial color-coded duplex sonography in the setting of acute stroke research, aiming to improve the reliability and reproducibility of the results of future stroke studies

Cerebellar induced differential polyglot aphasia: a neurolinguistic and fMRI study

Research has shown that linguistic functions in the bilingual brain are subserved by similar neural circuits as in monolinguals, but with extra-activity associated with cognitive and attentional control. Although a role for the right cerebellum in multilingual language processing has recently been acknowledged, a potential role of the left cerebellum remains largely unexplored. This paper reports the clinical and fMRI findings in a strongly right-handed (late) multilingual patient who developed differential polyglot aphasia, ataxic dysarthria and a selective decrease in executive function due to an ischemic stroke in the left cerebellum. fMRI revealed that lexical-semantic retrieval in the unaffected L1 was predominantly associated with activations in the left cortical areas (left prefrontal area and left postcentral gyrus), while naming in two affected non-native languages recruited a significantly larger bilateral functional network, including the cerebellum. It is hypothesized that the left cerebellar insult resulted in decreased right prefrontal hemisphere functioning due to a loss of cerebellar impulses through the cerebello-cerebral pathways

Benefit of Advanced 3D DSA and MRI/CT Fusion in Neurovascular Pathology.

Digital subtraction angiography provides excellent spatial and temporal resolution; however, it lacks the capability to depict the nonvascular anatomy of the brain and spinal cord.A review of the institutional database identified five patients in whom a new integrated fusion workflow of cross-sectional imaging and 3D rotational angiography (3DRA) provided important diagnostic information and assisted in treatment planning. These included two acutely ruptured brain arteriovenous malformations (AVM), a small superficial brainstem AVM after radiosurgery, a thalamic microaneurysm, and a spine AVM, and fusion was crucial for diagnosis and influenced further treatment.Fusion of 3DRA and cross-sectional imaging may help to gain a deeper understanding of neurovascular diseases. This is advantageous for planning and providing treatment and, most importantly, may harbor the potential to minimize complication rates. Integrating image fusion in the work-up of cerebrovascular diseases is likely to have a major impact on the neurovascular field in the future

The Dandy-Walker syndrome

The Dandy-Walker syndrome (DWS) has been defined as a congenital malformation of the structures of the posterior fossa characterised by cystic dilatation of the fourth ventricle, hypoplasia of the cerebellar vermis, enlargement of the posterior fossa, atresia of the foramina of Luschke and Magendie and associated hydrocephalus. Since its initial description definitions have been modified to include findings encountered in a particular case or series of cases. This lack of uniformity of the diagnostic criteria has made the objective assessment of management and outcome difficult. It has also resulted in terms such as DandyWalker variant, mega cisterna pouch and prominent cisterna posterior fossa cysts which magna, magna, do not persistent Blake's being applied to fit in with the particular criteria used for the series described

Hemorrhagic infarct of basal ganglia in cardiac arrest. CT and MRI findings. 2 cases

We report the CT and MRI findings in two cases of hemorrhagic infarct of the basal ganglia (BG), following out-of-hospital cardiac arrest (CA). In case 1, Brain-CT realized at day 2 showed bilateral and almost symmetric hemorrhagic infarct of the BG and infarct of the tectum of the mesencephalon. In case 2, MRI realized at day 6 showed hemorrhagic infarct of both lenticular nuclei on T2 GE images. In both cases there was no medical history and the cardiovascular and the coagulation profile were normal. In these cases, the lesions are observed earlier than reported in a few previous radiological cases. Similar lesions have been reported in pathological studies. These lesions seem occur early after CA. Reperfusion is probably responsible for the hemorrhagic transformation. The reason why some patients present either BG or brainstem infarct or both remains unclear. Bilateral and symmetric hemorrhagic infarct of the BG, especially of the Lenticular nuclei, and infarct of the dorsal pons and mesencephalic tegmentum seem to be a characteristic feature of profound and prolonged hypotension or of CA

Hypertrophic olivary degeneration after surgical removal of cavernous malformations of the brain stem: report of four cases and review of the literature

ManuscriptBackground: Hypertrophic olivary degeneration (HOD) is a pathological phenomenon that occurs after injury to the dentato-olivary pathway. Its hallmarks include hypertrophy of the olive with increased T2 signal intensity on magnetic resonance imaging, and often manifests with palatal tremor and oscillopsia clinically. Method: We report the cases of four patients who developed delayed HOD after surgical resection of pontine lesions. Findings: We discuss the anatomical and pathological details of this disease and review the few other reported cases of HOD after resection of lesions within the brainstem. Conclusions: HOD should be recognized as a possible complication of surgery within the brainstem and must be diagnosed promptly so that patients can be appropriately counseled and symptoms can be treated

Evalutation of fetal cerebral blood flow perfusion using three dimensional power doppler ultrasound angiography in fetuses affected by intrauterine growth retardation

The aim of the present study is to explore the possible use of 3D Power Doppler Angiography (3D-PDA) using VOCAL software (General Electric Healthcare, USA) in the assessment of different cerebral regions in normal and growth restricted fetuses (IUGR). This is a pilot study, that means a small experiment designed to test the method and gather information prior to a larger study.In Late Onset IUGR fetuses, presenting normal Bidimensional Doppler flow indices of umbilical and middle cerebral arteries, Vascularity Index (VI) and Vascular Flow Index (VFI) of the frontal zone of the fetal brain resulted increased demonstrating the \u201cfrontal brain sparing effect\u201d . On the other hand, these vascular parameters were decreased in the temporal zone suggesting a vascular redistribution during brain sparing effect according to a regional increase in bloody supply to the frontal region sprinkled mainly by the anterior cerebral artery. This shift may indicate that general cognitive functions, such as impulse control, language, memory, problem solving and socialization may be preferentially preserved suggesting a hierarchical order in the protection of the brain function