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Methylmalonic acidemia (MMA) is a series of rare inherited organic acid metabolic disorders with variable and nonspecific clinical manifestations, in particular neurological symptoms such as vomiting, lethargy, etc. Even with timely treatment, patients may still have various degrees of neurological complications and can even die. The prognosis is mainly related to the type of genetic variants, level of metabolites, newborn screening, onset of disease and early initiation of treatment. This article has reviewed the prognosis of patients with various types of MMA and factors that may affect it.

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Objective To explore the efficacy of moderate dose bromocriptine by one-off oral administration in treatment of patients with non-acute prolactin (PRL) type pituitary adenoma.Methods Forty-three patients with definite diagnosis of non-acute prolactin type pituitary adenoma,admitted to our hospital from January 2016 to December 2017,were chosen in our study.All patients were administrated with 5 mg bromocriptine at 18 pm;the serum PRL levels were examined at 2,3 and 12 h after bromocriptine administration.The curative effects of these patients were judged by decline rate of PRL,and the curative effects of patients with different genders,different initial PRL levels and different ages were compared.Results In these 43 patients,bromocriptine showed excellent effect in 20 patients,obvious effect in 18 and invalid effect in 5.There were statistically significant differences in the efficacy of bromocriptine between different genders (P＜0.05).There were statistically significant differences in efficacy of patients with different genders after taking bromocriptine (P＜0.05).The female patients had obviously higher rate of obvious effect than the male patients.Patients with different initial PRL values had statistically significant differences in efficacy after taking bromocriptine (P＜0.05).Patients with PRL initial value of 40-200 ng/mL had significantly higher rate of obvious effect than patients with PRL initial value of ＞200 ng/mL.There was no significant difference in efficacy between patients ＜ 45 years old and patients＞45 years old (P＞0.05).Conclusion The treatment ofhyperprolactin caused by prolactin type pituitary adenoma with moderate dose bromocriptine by one-off oral administration is effective;female patients have obviously better effect than male patients;and for patients with initial prolactin＞200 ng/mL,the effect is better.

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Objective To explore prevention and treatment of complications following resection of pineal lesions via the modified Poppen approach.Methods From January 2008 to December 2017,62 patients received resection of pineal region lesions via the modified Poppen approach at Department of Neurosurgery,Anhui Provincial Hospital.Their clinical data and outcomes were reviewed.The incidences of postoperative complications were compared between the patients with differences in lesion diameter,tentorial angle and preoperative hydrocephalus.Results Of this cohort,total tumor resection was performed in 48,subtotal resection in 10 and partial resection in 4.Follow-ups from 3 to 12 months revealed 2 cases of hemianopia which were recovered 3 months later,7 cases of occipital lobe contusion,2 cases of monoparesis and 2 cases of intracranial infection.Of the 7 cases of occipital lobe contusion,3 had intracranial hematoma which had been removed by secondary surgery and 4 had ataxia.The incidences of postoperative hemianopia and monoparesis for the patients with a lesion ＞4.5 cm in diameter were significantly higher than for those with a lesion ≤4.5 cm in diameter (P＜0.05).The incidence of postoperative hemianopia for the patients with a tentorial angle ＞60° was significantly higher than for those with a tentorial angle ≤ 60° (P＜0.05).Conclusions Size and tentorial angle of the lesion may be closely associated with the complications following resection of pineal lesions via the modified Poppen approach.The key to reducing the postoperative complications lies in comprehensive preoperative assessments,familiarity with clinical anatomy of the Poppen approach,sophisticated surgical skills and an individualized surgical protocol.

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Objective To conclude the application experience of intraoperative ultrasound added with neuronavigation in resection of intracranial gliomas.Methods A retrospective analysis was performed on the clinical data of 60 patients with intracranial gliomas,performed resection in our hospital from January 2010 to June 2013 by intraoperative ultrasound added with neuronavigation; 32 of them had high-grade gliomas and 28 low-grade ones.Ultrasonoscopy of different grade gliomas was studied.Results The accuracy of localization was 100％ in 60 patients with intracranial gliomas.Ultrasonic image of high-grade gliomas enjoyed obvious hyperechogenicity and the boundaries were clear usually; ultrasonic image of low-grade ones were slightly hyperechogenic or isoechogenic and the boundaries were indistinct usually.Inhomogeneous hyperechogenic of the ultrasonoscopy of intracranial gliomas before resection and hypoechogenic of residual cavity after resection were noted,respectively.Total removal was achieved in 50 patients,subtotal resection in 6 and partial resection in 4.Brain swelling happened postoperatively in 1 and was remitted by decompressive craniectomy.Hemiplegia happened in 2,and no death occurred postoperatively.Conclusions Intraoperative ultrasound is important to the correct of brain shift,and has clear and important value to the choice of operation approach and incising cortex.Ultrasonic image of high-grade gliomas and calcified lesions enjoys the best view.Intraoperative ultrasound added with neuronavigation can raise resection rate of intracranial gliomas.

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Objective To discuss the reoperation method for recurrent pituitary apoplexy after the successful initial transnasal-sphenoidal surgery.Methods Twenty-one patients with pituitary apoplexy were found recurrence after previous tumor resection via transsphenoidal surgery in 9 months to 5 years.In the reoperation,transsphenoidal approach would be still used.The anterior wall of sphenoid sinus and the sellar floor were located accurately,and then the remnants of them were removed as large as possible to expand the range of bone window.Next,the intrasellar hematoma was cleared away gently,and the residual tumor was removed progressively and thoroughly to make sure the sellar diaphragm subsiding fully and finally removed the whole tumor.Results Most of recurrent tumors were found to be of soft texture with rich blood supply,and 17 cases were still apoplexy.In 18 cases of the 21 patients,total resection was achieved.However,for the other 3 cases with tumor aggressive growth,only massive resection was achieved,and underwent stereotactic radiosurgery postoperatively.Postoperative clinical symptoms were alleviated in 15 cases,whose hormones were also decreased.Temporary diabetes insipidus and hypopituitarism appeared in 5 and 3 cases respectively,but all of these postoperative complications were relieved after symptomatic treatment.Two cases of intraoperative cerebrospinal fluid leakage were resolved by packing the cavity with muscle and fat flaps.All cases were followed up for 3 months- 5 years,residual tumor enlarged in only 1 case of massive resection,which umderwent transsphenoidal surgery for the third time,no other cases recurred.Conclusion Operation via the transsphenoidal approach of recurrent pituitary apoplexy after successful initial transsphenoidal surgery could achieve high total resection rate.While the accurate locating,full expansion of the saddle bone window,and repeatedly scraping the apoplexy tumor to get a full subsidence for the sellar diaphragm,are the key points to remove tumors totally as well as to improve the symptoms.

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Objective To investigate the application of neurophysiological monitoring and microsurgi-eal technique in acoustic neurinoma resection, exploring the significance of neurophysiological monitoring in facial and auditory nerve reservation of acoustic neuronma microsurgery. Methods Accompanied with EMG and BAEP nerve monitoring, 113 patients harboring acoustic neuroma were treated surgically by the subocipi-tal retrosigmoid approach for reserving facial and auditory nerve. The facial nerve was stimulated to evaluate its function during late-operation. Postoperative facial and auditory nerve function were valuated in all the postop-erative following up. Results All of them were treated microsurgically via the suboccipitai retrosigmoid ap-proach. Total tumors resection was achieved in 102 cases (90.3%), subtotal resection in 6 cases(5.3%) and partial resection in 5 case (4.4%). The facial nerve was preserved anatomically in 98 cases (86.7%), the functional valuation of facial nerve according to the House-Brakman (H-B) postoperatively: 86 cases (76.1%) in class Ⅰ -Ⅱ , 12 cases(lO.6%) in class Ⅲ-Ⅳ and 15 cases (13.3%) in class Ⅴ-Ⅵ. The acoustic nerve was preserved anatomically in 40 cases (35.4%). It denoted the good function of facial nerve responsing sensi-tively to electrostimulation ≤ 4mA at the end of operation. Conclusion Assisted with the intraoperative electrophysiological monitoring techniques, it would be greatly facilitate the preservation of facial and auditory nerve in acoustic neuroma resection. Simultaneously, it would valuate the functional convalescence by late-op-erative electrostimulation in the postoperation.

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Objective To analysis the therapeutic effect of microsurgery through single-nostril transsphenoidal approach on pituitary adenomas and to study the surgical skill and postoperative complications about microsurgery for pituitary adenomas.Methods The clinical manifestations,pathological type, removal percentage,postoperative complications and follow-up data of 241 cases with pituitary ademoma by microsurgery through single-nostril transsphenoidal approach were analysed retrospectively.Results 171(7 1%)cases were achieved total tumor removal,28(11.6%)cases were achieved subtotal tumor removal,26(10.7%)cases were achieved most tumor removal and 16(6.6%)cases were achieved partial tumor removal.Most of patients achieved better results,however there were 38(15.8%)eases with diabetes insipidus,12(4.9%)cases with transient worse sight,4(1.6%)cases with cerebrospinal fluid leakage,2(0.8%)cases with oculomotor paralysis,and no death.The postoperative complications were cured and outcome was considered as good.The follow-up period was 1-36 months in 241 patients and the clinical symptoms were improved by different degrees.Conclusion Pituitary adenomas can be treated by microsurgery through single-nostril transsphenoidal approach due to the time of operation shorten,the postoperative complications.