Long-term motor deficit in brain tumour surgery with preserved intra-operative motor-evoked potentials.

Muscle motor-evoked potentials are commonly monitored during brain tumour surgery in motor areas, as these are assumed to reflect the integrity of descending motor pathways, including the corticospinal tract. However, while the loss of muscle motor-evoked potentials at the end of surgery is associated with long-term motor deficits (muscle motor-evoked potential-related deficits), there is increasing evidence that motor deficit can occur despite no change in muscle motor-evoked potentials (muscle motor-evoked potential-unrelated deficits), particularly after surgery of non-primary regions involved in motor control. In this study, we aimed to investigate the incidence of muscle motor-evoked potential-unrelated deficits and to identify the associated brain regions. We retrospectively reviewed 125 consecutive patients who underwent surgery for peri-Rolandic lesions using intra-operative neurophysiological monitoring. Intraoperative changes in muscle motor-evoked potentials were correlated with motor outcome, assessed by the Medical Research Council scale. We performed voxel-lesion-symptom mapping to identify which resected regions were associated with short- and long-term muscle motor-evoked potential-associated motor deficits. Muscle motor-evoked potentials reductions significantly predicted long-term motor deficits. However, in more than half of the patients who experienced long-term deficits (12/22 patients), no muscle motor-evoked potential reduction was reported during surgery. Lesion analysis showed that muscle motor-evoked potential-related long-term motor deficits were associated with direct or ischaemic damage to the corticospinal tract, whereas muscle motor-evoked potential-unrelated deficits occurred when supplementary motor areas were resected in conjunction with dorsal premotor regions and the anterior cingulate. Our results indicate that long-term motor deficits unrelated to the corticospinal tract can occur more often than currently reported. As these deficits cannot be predicted by muscle motor-evoked potentials, a combination of awake and/or novel asleep techniques other than muscle motor-evoked potentials monitoring should be implemented.

Transethmoidal encephalocele endoscopically treated in a newborn: a case report.

Congenital encephalocele is a very rare entity, with herniation of normal brain or gliotic tissue through a defect in the skull. The objective is to present a newborn child diagnosed with transethmoidal encephaloceles at birth. She developed respiratory problems, feeding difficulties, and failure to thrive since the first days of life and so required early surgery at her 33th day of life, through an endoscopic nasal approach. Technical difficulties encountered, complications, and management are discussed. To the best of our knowledge, this is the first report of endoscopic treatment of transethmoidal encephalocele in a newborn. Further studies are needed to understand the best way to repair the dural defect in this rare condition.

Life-threatening secondary hemophagocytic lymphohistiocytosis following vagal nerve stimulator infection in a child with CHD2 myoclonic encephalopathy: a case report.

Vagus nerve stimulation (VNS) is a surgical treatment available for patients affected by generalized refractory epilepsy. The authors report the case of a 15-year-old girl affected by CHD2-related myoclonic encephalopathy and BLM haploinsufficiency due to a deletion of 15q25.3q26.2 region, who suffered from secondary hemophagocytic lymphohistiocytosis (SHLH) after a VNS wound infection. SHLH has sporadically been described in epileptic patients. Based on indirect evidence that shows immune dysregulation in patients with CHD2 mutations and BLM mutations, we hypothesize that the genetic background of this patient may have played a critical role in the development of the syndrome.

Rehabilitation pathways in adult brain tumor patients in the first 12 months of disease. A retrospective analysis of services utilization in 719 patients.

PURPOSE: Although previous studies have demonstrated the efficacy of rehabilitation programs for brain tumor (BT) patients and the positive impact on quality of life of functional gain, there are few studies focusing on specific rehabilitation management of brain tumor patients. METHODS: With the aim to evaluate the pattern of rehabilitation care and health services utilization in patients with brain tumor, we retrospectively analyzed administrative data on a large cohort of brain tumor patients diagnosed during the period 2008-2009 in the Lazio Region. Pattern of rehabilitation care was analyzed during a follow-up of 12 months after diagnosis. Data for this study were gathered and linked from two sources: (1) hospital discharge records stored into the Lazio Regional Health System database and (2) rehabilitation pathways database including inpatient and outpatient interventions files. RESULTS: We identified 789 patients with an ICD-9-CM code of 191.** in the Regional Health System databases in the study period. Among 719 patients included in this study, 92 (12.8 %) were treated with inpatient rehabilitation program, 22 (3.1 %) received an outpatient personalized program with intensive rehabilitation plan, and 85 (11.8 %) received outpatients rehabilitation interventions. CONCLUSIONS: Our retrospective analysis on the rehabilitation service utilization in a large cohort of BT patients shows that a limited number of patients received rehabilitation interventions during the first year after diagnosis. Nevertheless, the rehabilitation needs in BT patients are largely unmet and the lack of defined physical and cognitive rehabilitation strategies may negatively affect the functional independence and the short- and long-term quality of life.