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© 2020 Association of Anaesthetists.BACKGROUND  The use of intraoperative monitoring (IOM) in glioma surgery is a widely adopted and clinically validated adjunct to define safe zones of resection for the neurosurgeon. However, the role of IOM in cases of a significant preexisting motor deficit is questionable. CASE DESCRIPTION  We describe a case of a 25-year-old with a recurrent presentation of a left paracentral glioblastoma, admitted with intratumoral hemorrhage and subsequent acute severe right-sided weakness. The patient underwent a redo left parietal craniotomy and 5-aminolevulinic acid-guided resection with IOM. The severity of the weakness was not reflected by the pre- and intraoperative cortical motor evoked potentials (MEPs) that were reassuring. The patient's hemiparesis recovered to full power postoperatively. CONCLUSIONS  Preoperative weakness is traditionally accepted as a relative contraindication to IOM and therefore its usefulness is questioned in this context. Our case challenges this assumption. We present the clinical course, review the cranial and spinal literature including the reliability of IOM in cases of preoperative motor deficit, and discuss the need for tailor-made IOM strategies. Georg Thieme Verlag KG Stuttgart · New York.BACKGROUND  The treatment of spinal intramedullary arteriovenous malformations (AVMs) presents the risk of spinal cord ischemia because of the vascular nidus and their feeding arteries involving and supplying the spinal cord parenchyma. The multimodal approach includes endovascular embolization and microsurgical excision, both benefiting from intraoperative neurophysiologic monitoring. We present a case study of a patient who underwent several staged embolizations and open surgery for microsurgical excision. PATIENT  A 32-year-old man who presented with a recurrent glomus-type intramedullary AVM in the cervical spinal cord, located at the C5-C6 segment, with progressive neurologic deterioration. METHODS  Somatosensory evoked potentials (SEPs) and transcranial motor evoked potentials (MEPs) were performed during three embolizations, a provocative test, and surgery, in addition to D-wave during microsurgical excision. RESULTS  Abolished hand MEP and drop in SEP during a provocative test guided the surgeon to embolize from a safer vessel with no acute neurologic deficit after three embolizations. Before surgery, an angiography showed the left posterior spinal artery supplying the AVM. After resecting the vascular nidus from the spinal parenchyma, left-hand MEP decreased in amplitude and later abolished, and SEP decreased. Interestingly, no D-wave or distal MEPs were affected. Weakness in the left hand immediately and 2 weeks postoperatively advocates for metameric spinal cord ischemia with preservation of long spinal cord pathways. CONCLUSIONS  Intraoperative neurophysiologic monitoring correlates with neurologic outcome after endovascular and surgical treatment of a cervical AVM. Intraoperative monitoring provides continuous functional information of long and metameric spinal cord pathways, which is critical when deciding on the vessel to be embolized and during microsurgical excision where the surgeon is in less control of the AVM hemodynamic flow. Georg Thieme Verlag KG Stuttgart · New York.Endometriosis is a disorder in women which is characterized by extrauterine manifestations. We describe a case of cerebellar endometriosis in a 39-year-old woman who underwent posterior fossa decompression multiple times without establishing a correct diagnosis. this website Her neurologic status progressively worsened due to chronic hydrocephalus and brainstem compression by cysts. Late in the clinical course, histology from the cyst wall was taken that revealed endometriosis with clear cells and positive immunohistology for progesterone and estrogen receptors. Treatment with gestagens was started but did not improve the patient's status. In patients with chronic recurring intracranial cysts and hydrocephalus, cerebral endometriosis should be considered. Georg Thieme Verlag KG Stuttgart · New York.Two patients with insular and striatal postnatal scar had epileptic spasms (ES) that were asymmetrical and the only seizure type, whereas none of the usual ictal symptoms of insular seizures occurred. Ictal electroencephalography (EEG) showed the high-amplitude slow-wave characteristic of ES. Vigabatrin remained efficient for over 4 years for one patient and right into the third decade for the other one. Such ES are distinct from infantile and late onset spasms. Furthermore, these observations suggest that in ES insular epilepsy triggers paroxysmal activation of the striatum, and that vigabatrin inhibits the striatal startle motor program, thus interrupting the corticostriatal loop. Georg Thieme Verlag KG Stuttgart · New York.The congenital myasthenia syndromes (CMS) are a group of autosomal recessive or autosomal dominant diseases that affect neuromuscular junctions. CMS caused by AGRN mutations is very uncommon typically characterized by ptosis, mild weakness, and proximal limb weakness. We report the case of an 8-year-old female who exhibited the onset of motor development retardation from infancy and slow progression to proximal muscle weakness. Repeated nerve stimulation at 3 Hz showed a clear decrement with 17%. Whole exon sequencing showed an AGRN gene compound heterozygous mutation (c.5009C >T and c.5078T > C). She was treated with salbutamol but without improvement. Then pseudoephedrine was adapted as a treatment choice and obtained remarkable curative effect. We have summarized and analyzed 12 patients who have been reported in the literature. An early age of onset and muscle weakness in the lower limbs are the main feature of an early AGRN gene mutation. Both types of AGRN-related CMS respond favorably to ephedrine. This is the first report showing that pseudoephedrine is effective as a choice for the treatment of AGRN-related CMS. Georg Thieme Verlag KG Stuttgart · New York.This systematic review and meta-analysis sought to evaluate the efficacy of platelet-rich fibrin (PRF) membranes versus subepithelial connective tissue grafts (SCTGs) in the coverage of Miller class I and II gingival recessions. After the inclusion and exclusion criteria were applied, the quality of seven articles (Cohen's Kappa = 0.9) was evaluated using the Jadad scale. The MEDLINE/PubMed, Cochrane, and Web of Science databases were consulted, and manual searches were performed in the most popular periodontics journals. The studies included considered a total of 122 patients, 203 surgical fields on which SCTGs were used, and 205 surgical fields on which PRF was used. The parameters analyzed were probing depth, clinical attachment level, gingival recession, and keratinized mucosa. The minimum follow-up period accepted was 6 months. A statistically significant difference between the SCTG and PRF groups was found only in the case of keratinized mucosa. However, gingival recession, clinical attachment level, and probing depth parameters in the PRF group were found to be statistically equal to those of the SCTG group (the gold standard) (p ≥0.