Does emergent implantation of a vagal nerve stimulator stop refractory status epilepticus in children?

PURPOSE: Status Epilepticus can be a serious life threatening event in epileptic patients. The definition of refractory or super-refractory Status Epilepticus was based on the therapeutic response to anti-epileptic and anesthetic drugs. Vagal Nerve Stimulation showed efficacy in treating drug-resistant epilepsy but there are only few reports on emergentplacement of Vagal Nerve Stimulator for refractory or super-refractory Status Epilepticus. METHODS: Among 49 children implanted at our Institution with Vagal Nerve Stimulation for drug-resistant epilepsy, the authors retrospectively identified those implanted for refractory or super-refractory Status Epilepticus, according with the current definitions. RESULTS: 4 patients were operated upon at ages ranging 7 to 17 months and reached the programmed output current of 1 mA over a time ranging from 24 to 36 h (fast ramping-up). In 3 out of 4 patient we observed the abrupt of Status Epilepticus; one patient was refractory both to drugs and Vagal Nerve Stimulation and later died, without recovering from SE. At follow up, ranging from 24 to 45 months, the remaining 3 patients showed a decrease of the seizures frequency >80% without relapse of Status Epilepticus; in all the patients, output current and/or Duty Cycle were increased later. CONCLUSION: VNS can be effective in treating refractory or super-refractory Status Epilepticus.

The Disease of the Italian Poet Giacomo Leopardi (1798-1837): A Case of Juvenile Ankylosing Spondylitis in the 19th Century?

Some authors sustained that the pessimistic thought of the Italian writer and philosopher Giacomo Leopardi (1798-1837) may be attributed to his unhappy life, characterized by several health problems. His philosophical theories appear as the result of depressive and melancholic state, related to his precarious health conditions, so limiting their intrinsic values. Several authors formulated various hypotheses on the diseases that Leopardi suffered from and postulated different theories on the cause of his early death. This article assumed that Leopardi may have been affected by juvenile ankylosing spondylitis, conditioning spinal deformities, relapsing-remitting uveitis, urinary tract and bowel tract problems, and acute arthritis. Chest deformity, as a complication of juvenile ankylosing spondylitis, may have caused progressive cardiorespiratory failure, worsened by recurrent bronchial and pulmonary complications, until his death caused by acute right ventricular heart failure. The acknowledgment of a physical cause of Leopardi's disease contributes to reevaluate his "cosmic pessimism" as an original expression of his thought, so leading a general revaluation of the figure of one of the most important European thinkers of the 19th century.

Effectiveness of Intraventricular Endoscopic Lamina Terminalis Fenestration in Comparison with Standard ETV: Systematic Review of Literature.

BACKGROUND: Endoscopic third ventriculostomy is a consolidated technique for the treatment of hydrocephalus. Despite its effectiveness and feasibility, several technical limitations about its use in certain situations have been described. Lamina terminalis-endoscopic third ventriculostomy (LT-ETV) has been proposed as an alternative technique. Authors systematically reviewed the literature in order to define the effectiveness and limits in comparison with standard ETV. METHODS: This systematic review followed the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) statement. It has also been registered with the PROSPERO International Prospective Register of Systematic Reviews (CRD42016041596). MEDLINE, Web of Knowledge, and EMBASE were independently searched. RESULTS: Seven studies were found to be eligible. A case of ours was added to the series, totaling 41 patients (mean patient age ± SD was 21.6 ± 20.7 years). Endoscopic findings leading surgeons to perform LT-ETV were abnormal ventricular anatomy (24, 57%), inadequate/insufficient interpeduncular subarachnoid space (11, 26%), a combination of both (5, 12%), and intraoperatory, unsatisfactory third ventricle floor fenestration (2, 5%). Most common pathologies were neurocysticercosis (12, 28.57%), aqueductal stenosis (8, 19%), tuberculous meningitis (4, 9.52%), and medulloblastoma (3, 7.14%). A flexible endoscope was the most used device (36 procedures, 86%), while not determining a statistical relevant diminution of complications in comparison with a rigid endoscope (P = 1.0). An overall success rate of 69% was registered, increasing to 89% if just the first year of follow-up was considered. CONCLUSIONS: LT-ETV can be considered a successful technical option when standard ETV cannot be performed, although more complex cerebrovascular anatomy is involved. Therefore we suggest that lateral terminalis fenestration is a valid technical option in experienced hands.

Vagal Nerve Stimulation in the Treatment of Drug-Resistant Epileptic Encephalopathies in Inborn Errors of Metabolism: Report of 2 Cases.

Patients affected by inborn errors of metabolism can develop catastrophic epilepsies ineligible for resective surgery. Few reports concerning vagal nerve stimulation in patients with epileptic encephalopathy in the context of metabolic diseases have been published in the literature. Drug-resistant epilepsies in metabolic disease could be a specific target for vagal nerve stimulation, although the efficacy of this technique in these patients still needs to be proved. The authors report our experience in treating refractory epilepsy with vagal nerve stimulation in 2 patients affected by inborn errors of metabolism. The first patient is a 23-year-old patient affected by glutaric aciduria type II, the other one is a 16-month-old child with nonketotic hyperglycinemia. Vagal nerve stimulation reduced seizures up to 50% in the first case and up to 90% in the second one.

Craniovertebral junction pathological features and their management in the mucopolysaccharidoses.

The mucopolysaccharidoses (MPS) are multisystemic inherited metabolic diseases caused by the deficiency of the enzymes involved in the degradation of glycosaminoglycans (GAGs), which variably involve the central nervous system, heart, lungs, and bones.Undegraded or only partly degraded GAGs accumulate in the extracellular matrix, joint fluid, and connective tissue leading to widespread tissue and organ dysfunction.The introduction of hematopoietic stem cell transplantation (HSCT) and enzyme replacement therapy (ERT) has positively affected the natural history of MPS patients and their life expectancy. However, the presence of spinal abnormalities and deposition of GAGs in soft tissues remains nearly unaltered.Abnormalities of the craniovertebral junction (CVJ) and GAG deposits can result in spinal cord compression with slowly progressive myelopathy or acute posttraumatic tetraplegia.The current paper discusses neuroimaging findings in a consecutive series of 42 MPS patients followed at our Center for Metabolic Diseases and their neurosurgical issues.Current recommendations for decompression and fusion will be discussed according to our experience and review of the literature.

Is preoperative functional magnetic resonance imaging reliable for language areas mapping in brain tumor surgery? Review of language functional magnetic resonance imaging and direct cortical stimulation correlation studies.

OBJECTIVE: Language functional magnetic resonance imaging (fMRI) has been used extensively in the past decade for both clinical and research purposes. Its integration in the preoperative imaging assessment of brain lesions involving eloquent areas is progressively more diffused in neurosurgical practice. Nevertheless, the reliability of language fMRI is unclear. To understand the reliability of preoperative language fMRI in patients operated on for brain tumors, the surgical studies that compared language fMRI with direct cortical stimulation (DCS) were reviewed. METHODS: Articles comparing language fMRI with DCS of language areas were reviewed with attention to the lesion pathology, the magnetic field, the language tasks used pre- and intraoperatively, and the validation modalities adopted to establish the reliability of language fMRI. We tried to explore the effectiveness of language fMRI in gliomas. RESULTS: Nine language brain mapping studies compared the findings of fMRI with those of DCS. The studies are not homogeneous for tumor types, magnetic fields, pre- and intraoperative language tasks, intraoperative matching criteria, and results. Sensitivity and specificity were calculated in 5 studies (respectively ranging from 59% to 100% and from 0% to 97%). CONCLUSION: The contradictory results of these studies do not allow consideration of language fMRI as an alternative tool to DCS in brain lesions located in language areas, especially in gliomas because of the pattern of growth of these tumors. However, language fMRI conducted with high magnet fields is a promising brain mapping tool that must be validated by DCS in methodological robust studies.

Oncogenic osteomalacia caused by a phosphaturic mesenchymal tumor of the thoracic spine.

Phosphaturic mesenchymal tumors that cause the paraneoplastic syndrome known as oncogenic osteomalacia are rare. The authors report on the case of a 57-year-old man with a history of osteomalacia and in whom was diagnosed a thoracic spine tumor at the T-4 level. Complete tumor resection was accomplished. The histological diagnosis was phosphaturic mesenchymal tumor (mixed connective tissue variant). After lesion removal, the paraneoplastic syndrome resolved. At the 24-month follow-up, no recurrence of the disease was observed. The clinical presentation, surgical technique, and follow-up in this case were reviewed in detail.