About 50% of Italian patients with ALS had some degree of cognitive impairment, in keeping with a previous Irish study, despite the largely different genetic background of the two populations. The lower educational attainment in patients with ALS-FTD indicated a possible role of cognitive reserve in ALS-related cognitive impairment. ALS-ECI and ALS-NECI may represent discrete cognitive syndromes in the continuum of ALS and FTD.

Sparganosis is a severe parasitic infection caused by the larvae of Spirometra mansoni, also called "sparganum." In human hosts, the Spirometra mansoni larva commonly targets the subcutaneous tissue or muscle. Sometimes it can also migrate into the brain, resulting in cerebral sparganosis, mainly characterized by focal neurological symptoms such as seizures and radiological "wandering lesions" on magnetic resonance images (MRIs). Clinical cases of cerebral sparganosis have been reported worldwide, mainly in Asian countries, but also in North America, South America and Australia. Only two cases have been previously reported in Europe. A 29-year-old male from Bolivia, who lived in Spain, presented to our service for seizures and a multicystic brain lesion, initially suspected to be a dysembryoplastic neuroepithelial tumor (DNET). He underwent gross total resection of the mixed solid/cystic lesion. Pathology revealed gliosis, multiple interconnected cystic cavities with fibrous walls, inflammatory cell infiltration and no necrotizing granulomatous reaction. Inside the cavities, a parasitic form was identified as the larva of the cestode Spirometra mansoni. At 1-year follow-up, the patient had no deficits and was seizure free. Clinicians should be alerted to the possible existence of this rare entity in Europe, especially in patients from endemic areas with a possible infection history as well as "wandering lesions" on the MRI.

Background:Herpes simplex virus encephalitis (HSVE) is the most morbid clinical syndrome associated with the human herpes virus. Despite treatment with appropriate dosages of acyclovir, neurologic relapse of HSV infection have been reported after cranial surgery. Rarely, neurological deterioration due to postinfectious inflammatory response without demonstrable HSV reactivation may recur following cranial surgery.Case Description:We report a case of a 17-year-old girl who presented with a HSVE relapse on the 6th postoperative day following resective surgery for medically refractory epilepsy and review the literature. Postinfectious inflammatory reaction may be the underlying mechanism in cases with no HSV identified on cerebrospinal fluid (CSF) or brain polymerase chain reaction (PCR), such as in the current case.Conclusion:HSVE must be suspected in patients with previous history of HSVE and postoperative fever associated with an altered state of consciousness and/or seizures. Considering the high mortality and morbidity rates associated with HSVE, an adequate prophylactic administration of acyclovir should be considered for patients with previous history of HSVE undergoing neurosurgical procedures, especially when surgery involves the site of a previous herpetic lesion.

BACKGROUND Recommendations on the management of brain arteriovenous malformations (bAVM) with respect to pregnancy are based upon conflicting literature. OBJECTIVE To systematically review the reported risk and annualized rate of first intracranial hemorrhage (ICH) from bAVM during pregnancy and puerperium. METHODS MEDLINE, EMBASE, and Scopus databases were searched for relevant articles in English published before April 2018. Studies providing a quantitative risk of ICH in bAVM during pregnancy were eligible. RESULTS From 7 initially eligible studies, 3 studies met the criteria for providing quantitative risk of first ICH bAVM during pregnancy. Data from 47 bAVM ICH during pregnancy across 4 cohorts were extracted for analysis. Due to differences in methodology and definitions of exposure period, it was not appropriate to combine the cases. The annualized risk of first ICH during pregnancy for these 4 cohorts was 3.0% (95% confidence interval [CI]: 1.7-5.2%); 3.5% (95% CI: 2.4-4.5%); 8.6% (95% CI: 1.8-25%); and 30% (95% CI: 18-49%). Only the last result from the last cohort could be considered significantly increased in comparison with the nonpregnant period (relative rate 6.8, 95% CI: 3.6-13). The limited number of eligible studies and variability in results highlighted the need for enhanced rigor of future research. CONCLUSION There is no conclusive evidence of an increased risk of first hemorrhage during pregnancy from bAVM. Because advice to women with bAVM may influence the management of pregnancy or bAVM with significant consequences, we believe that a retrospective multicenter, case crossover study is urgently required.

BackgroundLarge fat embolus is a rare but potential reversible cause of ischaemic stroke.Methods and resultsWe describe the neurosurgical management of a complete right internal carotid artery occlusion due to a large fat embolus, caused by a mitral valve replacement.ConclusionKnowledge of acute cerebral ischaemia due to large fat embolism and its hallmark ‘hypodense artery’ is mandatory. Extracranial to intracranial bypass is a feasible rescue treatment after failure of endovascular embolectomy.