**7. References**

200 Neuroimaging for Clinicians – Combining Research and Practice

Fig. 24. Once the microwire was in the aneurysm a Snare was deployed to grab the microwire and the Marksman microcatheter was advanced using the snare over the microwire into the Pipeline device by pulling the microwire back out through the PCoA (Figure 24 A, B). Once distal access was reestablished additional Pipeline Devices were used to complete the endovascular reconstruction of the aneurysm. The aneurysm appeared almost completely obliterated at the end of the procedure (Figure 24 C) and remained

An in-depth knowledge of intracranial and extracranial collateral anastomoses, overt or hidden, is crucial for a neurointerventionist to devise optimal endovascular strategies to manage a host of pathological conditions; to ascertain potential pitfalls; and ultimately, to

obliterated at the 6-month follow-up (Figure 24 D).

**6. Conclusion** 


**11** 

*Spain* 

**Haemolytic-Uraemic Syndrome:** 

**and Neurocognitive Outcome** 

**Neurologic Symptoms, Neuroimaging** 

*Neurology (1) and Nephrology (2) Departments of Sant Joan de Déu Children's Hospital of Barcelona, Barcelona University (U.B.)* 

Ana Roche Martínez1, Pilar Póo Argüelles1, Marta Maristany Cucurella1, Antonio Jiménez Llort2, Juan A Camacho2 and Jaume Campistol Plana1

Haemolytic-Uraemic Syndrome (HUS) was first described in 1955 by Gasser (Pérez del Campo et al., 2000) and defined as a multi-systemic syndrome, due to the association of microangyopathic haemolytic anemia, thrombopenia and multiorganic aggression. HUS affects mainly kidneys and leads to acute renal failure with high levels of urea and creatinin; it often involves digestive and central nervous systems. Central nervous system (CNS) lesions, typically at the basal ganglia, may also affect cortico-subcortical areas and in so doing determines motor and neurocognitive outcome, and modify the patients' quality of

Incidence of HUS varies among continents, highly influenced by migration movements, and it is estimated to be around 18/100,000 in children younger than 5 years old. Some countries, like Argentina and South Africa, are considered "endemic", with a steady and relatively high incidence of HUS during all the seasons of the year; other areas, such as Canada, most of the European countries, and the west coast of the USA, are said to be "epidemic", with sporadic cases and a lower incidence of HUS compared to Latin America and Africa during most of the year, but with self-limited relapses during summertime

HUS etiology is diverse and physiopathologic mechanisms are not yet well known, but infective microorganisms are frequently involved, especially *Escherichia coli,* serotype O15:H7; this bacteria is able to produce a toxic protein (vero-toxin –VT- or Shiga-toxin –Stx), which "recognizes" the endothelial cells and provokes an endothelial lesion (Scheiring, 2010). Other bacteria seem to be involved in different cases of HUS, like *Salmonella enteritidis*  and *Streptococcus pneumonia*e (De Loos et al., 2002; Prestidge & Wong, 2009). Mutations in genes coding for different components of the complement system seem to be a risk factor for HUS (Skerka et al., 2009). However, the etiologic agent remains unidentified in most

Clinical presentation in the acute phase includes acute renal failure (100% of patients), often high blood pressure (HBP) due to a volume surcharge (35-40% of patients), and neurological

**1. Introduction** 

life.

(Exeni, 2001).

patients.

