Congenital bilateral perisylvian syndrome(CBPS) is rare in literature,especially in China.In this article,we report the clinical and treatment of a patient with CBPS and discuss its mechanism,clinical features and therapy.This patient was a 28-year-old man.His main clinical features were pseudobulbar palsy,cognitive deficits and intractable epilepsy.MRI shows bilateral thickening of the cortex around the sylvian fissures which were deeper than normal and polymicrogyria.The electroencephalogram demonstrated slow spike in right temporal lobe and left frontal lobe.Rhythmal 4 Hz ? waves exist in left frontal and parietal lobe.As the epilepsy was poorly controlled by antiepileptic,section of the corpus callosum was carried out.After callosotomy,there was pronounced seizure reduction and intelligence development improvement.CBPS is characterized by pseudobulbar palsy,cognitive deficits,and bilateral perisylvian abnormalities in imaging studies.If intractable epilepsy is combined,callosotomy may be effective.

Objective To investigate the value of presurgical functional magnetic resonance imaging(fMRI) in identifying the eloquent brain areas and guiding the resection of cerebral tumors located in these areas.Methods A total of 10 patients(aged 51.3 years on average) with tumors near to the brain functional areas were enrolled in this study,among which 7 had weakness in the limbs,2 had convulsion,and 1 suffered from facial palsy.The preoperative Karnofsky scale of the patients was 82 on average.Before the fMRI,the patients have been confirmed as having tumors involving functional areas.They were treated by total or subtotal resection according to the anatomical relationship between activated areas and the tumor.Injuries to the activated areas should be avoided during the operation.After the treatment,the muscular strength,Karnofsky scales of the patients were tested.Postoperative MRI was carried out in all of the patients,and the results were compared with that before the operation.Results As shown by fMRI,the activated areas were compressed and dislocated by the tumor in 8 of the 10 patients.The minimal distance between the edge of the tumor and the activated spots was 0-26 mm(mean,13.6).Seven of the patients received total resection of the tumor,while the other 3 underwent subtotal resection.After the surgery,the muscular strength was improved in 7 cases;the convulsion disappeared in the 2 patients who had symptom before the treatment;and the one who had facial palsy was improved.One month after the surgery,the mean Karnofsky scale of the patients was 93.Re-examination by MRI performed one week after the operation showed that the tumor was disappeared in 7 patients,and reduced by 95% to 99% in the other three.Conclusions fMRI is valuable in the preoperative evaluation of the brain functional area near to a cerebral tumor.It can be used to guide the tumor resection while preserving the motor areas.

Meningomyelocele combined with squamous cell carcinoma is rare in literature. In this article,we report the clinical and treatment of a patient with meningomyelocele and squamous cell carcinoma and discuss its mechanism,clinical feature,therapy and prognosis.The patient was a 11-year-old Chinese boy.At the time of his birth he was noted to have a lumbosacral meningomyelocele,which was disrupted and the cerebral spinal fluid flew out when the child was six.The wound surface abrased and exudated repeatedly.Two months before admission,the meningomyelocele was disrupted again and the condition got worse.Inspection showed a meningomyelocele in the lower lumbar region 10 cm in diameter,consisting of a cauliflower-shaped swelling and a central crater containing black slough.The area smelled foul and was constantly draining serosanguineous fluid.Magnetic resonance imaging showed meningomyelocele associa-ted with spinal dysraphism and tethered cord syndrome.After thorough preparation,operation was undertaken.A perpendicular skin incision,which was carried down to the lumbar aponeurosis,allowed the main bulk of the tumour to be undercut and removed.The quick frozen pathological examination confirmed that it was squamous cell carcinoma.The skin and subcutaneous tissue were fruther resected and the vertebral canal explored until frozen section showed the excision edge was clear.Skin closure was achieved by a bi-pedicle advancement flap,some 10 cm wide and the secondary defect was closed with a thigh skin graft.Histological examination showed that the massive outgrowth was a well-differentiated squamous cell carcinoma.The postoperative recovery was uneventful and the wounds healed by primary intention.Although meningomyelocele combined with squamous cell carcinoma is rare in literature,the possibility of can-cerization should be considered when there is a long-term and non-healing ulcer (Marjolin ulcer) with foul smell in a meningomyelocele patient.

Objective:To explore the mechanism,clinical features,and prognosis of trigemino-cardiac reflex (TCR) during skull base operations.Methods:A retrospective analysis was performed on 291 patients with skull base disease from Jan.2009 to Oct.2015 in Peking University First Hospital.By reviewing the patients' operative data and anaesthetic electrical record,and we picked out all the patients who suffered from TCR during the operation and analyzed their surgical procedures,clinical features,influence factors,and prognosis.TCR was defined as a drop in mean arterial blood pressure (MABP) and the heart rate (HR) of more than 20％ to the baseline values before the stimulus and coinciding with the manipulation of the trigeminal nerve.Results:In all the 291 patients receiving skull base surgery,9 patients suffering TCR for 19 times during the operation were found.These 9 cases included three acoustice schwannomas,one trigeminal schwannoma,one petroclival meningioma,one epidermoid cyst in cerebellopontine angle,one cavernous sinus cavernous hemangioma,one pituitary adenoma,and one trigeminal neuralgia.The trigger of TCR was related to manipulation,retraction,and stimulation of the trunk or branches of trigeminal nerve.The baseline heart rate was 62-119/min [mean (79.4 ± 14.6) /min] and dropped about 29.0％-66.4％ (mean 44.3％) to 22-60/min [mean (44.2 ±9.6) /min] after TCR.The baseline mean arterial blood pressure was 75-103 mmHg [mean (87.5 ±7.8) mmHg] and dropped about 23.4％-47.2％ (mean 37.3 ％) to 45-67 mmHg [mean (54.9 ± 6.3) mmHg] after TCR.During the 19 times of TCR,heart rate and blood pressure could return to baseline in a short time while stopping manipulation (8 times),using atropine (8 times,dose 0.5-1.0 mg,mean 0.69 mg),using ephedrine hydrochloride (one time,15 mg),using epinephrine (one time,1 mg),and using dopamine (one time,2 mg).TCR also could be triggered again by a second stimulation.There was no relative cardiologic complication or neurological deficit in the postoperative 24 hours.Conclusion:TCR is a short neural reflex with a drop in blood pressure and heart rate coinciding with the manipulation of the trigeminal nerve in skull base surgery.Correct recognition,intensive observation,and essential management of TCR will lead to a good prognosis.

Cognitive impairment following subarachnoid hemorrhage (SAH) has aroused extensive attention because of its high incidence and bad influences in patients' life quality and social function. However, it's still not clear about its specific mechanism and there is still no effective treatment. Here, we make a review of the researches in incidence rate, pathogenesis, assessment methods and treatments of cognitive impairment following SAH in animal experiments and clinical trials, so as to deepen the understanding of its mechanism and find the potential therapeutic targets.