doi：10.3969/j.issn.1672⁃6731.2021.08.015

Background The petroclival area is located deep at the base of the skull, and the surrounding anatomical structure is complex, which brings great challenges to the safe removal of the lesions in this part of the operation. At present, the main surgical approaches for petroclival lesions are lateral craniotomy and endoscopic endonasal approach. This study explored the effectiveness and safety of endoscopic techniques in the resection of petroclival lesions via endonasal approach. Methods A total of 6 patients with petroclival lesions treated in The First Affiliated Hospital of Fujian Medical University from January 2018 to December 2020 were included. All patients underwent neuronavigation assisted endoscopic endosnasal approach resection of petroclival lesions. Results All the 6 patients successfully completed the operation. The average operation time was 4.53 h. After the operation, 3 cases were pathologically confirmed as chondrosarcoma (2 cases of WHO grade 2, one case of WHO grade 1), one case of schwannoma, one case of chordoma and one case of cholesterol granuloma. Except for one case of preoperative double vision that did not relieve postoperatively, the remaining 5 cases had preoperative symptoms alleviated to varying degrees. The average postoperative hospital stay was 5.67 d. The postoperative median follow⁃up was 14.07 months. There were no complications such as cerebrospinal fluid leakage, central nervous system infection, cranial nerve damage, no unplanned secondary operations, and no deaths within 3 months after the operation. Up to the last follow ⁃ up, no patients had recurrence. Conclusions Neuronavigation assisted endoscopic endonasal approach resection of petroclival lesions is relatively safe and effective. Different endoscopic techniques should be selected according to the tumor location and size.

Objective To summarize the key points of neuroendoscopic transcranial resection for cerebellopontine angle (CPA) tumors. Methods Twenty⁃eight patients with CPA tumors diagnosed and treated in Xiangya Hospital Central South University from January 2019 to December 2020 were included, and all of them underwent neuroendoscopic transcranial surgery to remove CPA tumors. Results All the 28 patients were successfully treated with neuroendoscopic transcranial resection of tumors in CPA region, and the tumors were totally removed. After operation, 9 cases of acoustic neuroma, 8 cases of meningioma, 9 cases of cholesteatoma, one case of hemangioblastoma, and one case of trigeminal neurinoma were confirmed by postoperative pathology. Postoperative facial nerve function was grade Ⅲ in 3 cases, grade Ⅱ in 6 cases, and grade Ⅰ in 19 cases. One patient had no obvious recovery of auditory nerve function injury, and 2 patients had dizziness, vertigo and nausea after operation. No complications such as cerebrospinal fluid leakage, meningitis and incision infection occurred, and no death occurred. At 3 months of follow ⁃ up, no tumor recurrence was seen on MRI, and no new neurological symptoms occurred. Conclusions Neuroendoscopic transcranial resection of CPA tumors has many advantages, but also has some shortcomings, and has a good development in the future.

Nerve stimulation treatment of epilepsy is to stimulate the central nervous system through electric current, thereby reducing the frequency and severity of epilepsy seizures. The current nerve stimulation methods that may be used to treat pediatric epilepsy include vagus nerve stimulation, responsive neurostimulator system, deep brain stimulation, chronic subthreshold cortical stimulation, and transcranial direct current stimulation. This article reviews the use of these neurostimulation techniques in epilepsy to analyze the effectiveness and safety of these treatments, and to provide references for the clinical treatment of intractable epilepsy in children.

Objective To analyze the clinical features and mechanism of reversible splenial lesion syndrome (RESLES) in adults. Methods and Results Twenty ⁃ three patients with reversible splenial lesion syndrome diagnosed and treated in the First Affiliated Hospital of Nanchang University from January 2017 to May 2019 were included. The clinical manifestations were headache, dizziness, fever, convulsions, disturbance of consciousness, all 23 patients in this group had decreased serum albumin levels. The common feature of head MRI was a reversible isolated lesion of the corpus callosum, which was round ⁃ shaped with clear borders, with high signal on T2WI, low signal on T1WI, high signal on DWI, and low signal on ADC, and the lesions gradually or completely disappeared on the 14th day of onset. Conclusions The etiology and clinical manifestations of reversible splenial lesion syndrome are complicated. MRI usually presents a reversible round ⁃ shaped lesion in the corpus callosum. Imaging lesions usually disappear earlier than clinical symptoms. The decrease of serum albumin level may be a risk factor for cytotoxic edema.

Objective To summarize the clinical, muscle pathology and molecular biological features of late⁃onset glycogen storage disease type Ⅱ (GSDⅡ). Methods and Results Five patients with late⁃onset GSD Ⅱ diagnosed and treated in The Fifth Affiliated Hospital of Zhengzhou University from January 2013 to January 2020 were selected. The main clinical manifestations of 5 patients were weakness of raising the head, weakness of the proximal extremities, decreased muscle tone, fatigue intolerance, and dyspnea. The activity of acid α⁃glucosidase (GAA) was significantly reduced. HE staining of muscular tissues in 5 patients showed vacuole⁃like changes of different sizes, different numbers, and irregular shapes in most muscle fibers. Modified Gomori trichrome (MGT) staining showed a large number of blue and purple particles deposited in the vacuole. Periodic acid⁃Schiff (PAS) staining showed the glycogen content in the vacuoles were increased in 4 cases, and the glycogen content in the vacuoles were lost in one case. GAA gene testing showed that 9 mutations were detected in 5 patients, and 4 cases were compound heterozygous mutations, which were derived from father and mother respectively. The c.1320_1322delGAT (p.Met440del) was a deletion mutation, c.2331G＞C (p.Thr777Thr) was a synonymous mutation, c.2237G＞A (p.Trp746*) was a nonsense mutation, c.877G＞A (p.Gly293Arg) was a missense mutation, c.2238G＞C (p.Trp746Cys) was a missense mutation, c.784G＞A (p.Glu262Lys) was a missense mutation, c.2014C＞T (p.Arg672Trp) was a missense mutation, and c. 2332⁃2A＞G was a splicing mutation. One case was homozygous mutation of c. 1432G＞A (p. Gly478Arg), which originated from the mother. Among them, c. 2331G＞C, c. 1432G＞A and c. 2332⁃2A＞G were reported for the first time at home and abroad. Conclusions The clinical manifestations of late⁃onset GSD Ⅱ are weakness of proximal limbs and dyspnea. The activity of GAA in peripheral serum is decreased significantly. Muscle tissue pathology is characteristic. GAA gene mutations are mainly compound heterozygous mutation, c.2331G＞C, c.1432G＞A and c.2332⁃2A＞G are new mutations, which extended the mutation spectrum of GAA gene.