术中CT导航在先天性颅颈畸形腹侧减压中的应用

目的 探讨术中CT与导航系统在先天性颅颈交界区畸形腹侧减压手术中的应用价值.方法 先天性颅颈交界区畸形患者19例,其中男11例,女8例,年龄13 ～58岁,平均33.9岁,患者术中行CT扫描,根据重建结果制定个性化的腹侧减压方案,术中在导航引导下实施手术,术中验证减压效果,根据Nurick分级评价随访结果.结果 术中CT及导航系统应用顺利,验证准确度平均为1.6 mm(1.2 ～2.0 mm).其中4例患者术中第一次CT验证显示减压不充分,进一步磨除残留骨质后,再次术中CT验证均获得满意的减压效果,术中无神经、血管损伤等手术相关并发症发生.术后3个月根据Nurick分级,其中15例较术前至少改善1级,4例患者症状较术前无明显改善.结论 术中CT扫描结合导航系统不仅提高了手术成功率,也减少了术中并发症的发生.     

Myasthenia gravis: diagnostic and management dilemmas

This review focuses on the diagnostic tests that may help to confirm myasthenia in patients without acetylcholine receptor antibodies, including the newly discovered anti-muscle-specific receptor tyrosine kinase antibody and other investigations. We discuss management dilemmas, particularly those that revolve around treatments whose efficacy is questionable or unproved, such as thymectomy. We review the important issue of how treatments are assessed, and examine future treatment trial designs.     

Neonatal seizures: dilemmas in workup and management

There is a pressing need for consistent, evidence-based guidelines in the management of neonatal seizures by pediatric neurologists and neonatologists. Israeli pediatric neurologists and neonatologists completed a 20-item, self-administered questionnaire on choices of antiepileptic drugs, treatment of intractable neonatal seizures (unremitting seizures after 3 medications), treatment duration, and recommended workup. The responding 36/55 (65%) neurologists and 66/112 (59%) neonatologists made similar antiepileptic drug choices (phenobarbital as first line, phenytoin as second line, and benzodiazepines as third line). Antiepileptic treatment duration was similar for both groups, but varied considerably within them (range, 1-52 weeks). Neurologists tended to recommend longer treatment for seizures secondary to asphyxia or hemorrhage. Neurologists and neonatologists recommended different antiepileptic drugs for intractable neonatal seizures: valproic acid and topiramate by neurologists, vs lidocaine and benzodiazepines by neonatologists (P = 0.0023). Fewer neurologists recommended continuous electroencephalography monitoring after asphyxia than neonatologists (40% vs 70.5%, P = 0.013). These responses reflect both similarities and inconsistencies of the two groups in diagnosing and treating neonatal seizures. Our findings call for controlled clinical trials to establish protocols for (1) diagnosing neonatal seizures, (2) studying the efficacy and safety of new-generation antiepileptic drugs, and (3) determining optimal duration of drug administration.     

Court-mandated treatment: dilemmas for hospital psychiatry.

The authors present clinical material to illustrate the special treatment and management problems posed by different types of mentally ill offenders; they suggest that court-mandated hospital treatment is often destructive and unrelated to the needs of the patient, the community, and the mental institution. The failure to create new kinds of institutions, combining modalities derived from the hospital and correctional systems, is traced to poor communication among the disciplines involved. The "mentally ill offender" is caught in the interplay of these systems and is consequently both their victim and victimizer.     

Long-term follow up of transoral anterior decompression and posterior fusion for irreducible bony compression of the craniovertebral junction

The aim of this study was to examine the long-term outcomes of treating irreducible craniovertebral junction bony compression via the transoral approach, with a minimum of 4 years of follow-up. Between 1996 and 2005, the transoral transpharyngeal approach was used for 10 patients who underwent anterior decompression and posterior fusion and two who underwent only anterior decompression. All patients were monitored intraoperatively using combined somatosensory/motor evoked potentials. All patients reported substantial or complete resolution of pain. Six out of the eight patients with preoperative myelopathy experienced an improvement of one Ranawat classification level. Solid fusion was achieved in all patients, and no patient had iatrogenic neurological deficits or cerebrospinal fluid leaks. Two patients experienced wound dehiscence requiring resuturing, one experienced velopalatine incompetence, and one experienced wound infection. There was no re-growth of the bony lesion during follow up. Transoral repair provided generally acceptable long-term results for irreducible craniovertebral junction bony compression. Intraoperative spinal cord monitoring may be useful for preventing neurological complications.     

Somatosensory and brainstem auditory evoked potential in congenital craniovertebral anomaly; effect of surgical management.

Clinical features and evoked potential recordings were analysed in 32 patients with congenital atlantoaxial dislocation before and after surgery. Seven patients (group 1) had atlantoaxial dislocation, while 22 patients had associated basilar invagination (group 2). In both groups, pyramidal tract signs, posterior column signs, wasting of the upper limbs, and abnormality of somatosensory evoked potentials (SSEP) were similar. Conversely, lower cranial nerve involvement and abnormal brainstem auditory evoked potentials (BAEP) were significantly more in patients with basilar invagination (p less than 0.05). All seven patients in group 1 and 17 patients in group 2 were operated upon. Clinical and electrophysiological deterioration were significant in patients with basilar invagination (group 2), following posterior fixation compared with group 1. Among the patients in group 2, who clinically deteriorated following posterior fixation, seven had transoral excision of odontoid and six of them improved both clinically and electrophysiologically. Two patients in group 2 had odontoid excision before posterior fixation, and in both the evoked potentials improved postoperatively. In group 1 the patient's BAEP remained unaffected following posterior fixation, however, in group 2, eight patients over 53% showed improvement in brainstem function following posterior fixation. This study shows the value of evoked potentials in congenital atlantoaxial dislocation, and rationalizes the surgical procedure in these patients. In patients with basilar invagination, odontoid excision is the preferred first stage procedure.     

Dynamic CT scan of the craniovertebral junction: a role in the management of os odontoideum

Os odontoideum is an uncommon abnormality of the cranio-vertebral junction (CVJ) that exists as a separate ossicle apart from a hypoplastic dens. Its genesis and natural history have been debated, and its proper treatment remains uncertain. A 48-year-old woman complained of persistent upper neck pain and paraesthesia of her left side. Magnetic resonance imaging of the CVJ demonstrated an os odontoideum. Dynamic computed tomography scan of the CVJ showed a reduction of the space available for the spinal cord to 50% from extended to flexed position. The patient underwent posterior spinal fusion of C1-C2 using a sublaminar titanium hook and rods fixed in moderate extension. We discuss the usefulness of the dynamic computed tomography (CT) scan in the evaluation of atlantoaxial motion and the management of this pathology.     

Microvascular decompression for trigeminal neuralgia: recurrences and complications.

BACKGROUND: The purpose of this study was to review the complications and pain recurrences after microvascular decompression (MVD) for trigeminal neuralgia. METHODS: This is a retrospective review of 156 patients undergoing MVD in the last 25 years at Princess Alexandra Hospital, Brisbane, Australia. Patients were contacted by telephone and a questionnaire was used for the interview. RESULTS: The probability of becoming pain free after MVD in our study was 0.93. There were no deaths and the incidence of serious complication was 2%. Our complication profile is similar to other authors. Pain recurrences occurred in 18% of patients over a 25 year period. This was most likely within two years of surgery and thereafter occurred at a rate of 2-3.5% a year. Seventy-four percent of patients were still pain free at 10 years. In half of the patients the recurrence was not as severe as the initial occurrence of pain. Thirty percent of recurrences occurred on the opposite side. Eighteen percent of patients were not pain free despite any intervention. CONCLUSIONS: Microvascular decompression in experienced hands has an excellent pain outcome in the majority of patients. Major complications are uncommon.     

Myasthenia gravis: management of myasthenic crisis and perioperative care

Myasthenic crisis may be defined as respiratory failure or delayed postoperative extubation for more than 24 hours resulting from myasthenic weakness. Myasthenic crisis results from weakness of upper airway muscles leading to obstruction and aspiration, weakness of respiratory muscles leading to reduced tidal volumes, or from weakness of both muscle groups. About one-fifth of patients with myasthenia gravis experience crisis, usually within the first year of illness. Over the last four decades, prognosis from myasthenic crisis has dramatically improved from a mortality rate of 75% to the current rate of less than 5%. Common precipitating factors for myasthenic crisis include respiratory infections, aspiration, sepsis, surgical procedures, rapid tapering of immune modulation, beginning treatment with corticosteroids, exposure to drugs that may increase myasthenic weakness, and pregnancy. Myasthenic crisis should not be fatal, as long as patients receive timely respiratory support and appropriate immunotherapy to reduce myasthenic weakness of the upper airway and respiratory muscles. Myasthenic patients with oropharyngeal or respiratory muscle weakness should receive preoperative plasma exchange or intravenous immunoglobulin therapy to a minimal level of weakness to prevent postoperative complications.     

Transdermal rotigotine for the perioperative management of Parkinson’s disease

Continuous delivery of antiparkinsonian medication during a perioperative period is desirable to avoid ‘off’-symptom complications in surgical patients with concomitant Parkinson’s disease (PD). Fourteen PD patients undergoing surgery under general anesthesia were switched from oral dopaminergic medication to transdermally delivered 24-h rotigotine (median dose 12 mg/24 h) for the perioperative period. Rotigotine treatment was considered feasible by patients, their anesthesiologists and neurologists with good control of PD symptoms and easy switching and re-switching of PD medication.     

三叉神经痛显微血管减压术中岩静脉处理策略的研究

目的 在三叉神经痛显微血管减压手术中,观察岩静脉形态特点及其对手术显露的影响,分析岩静脉的处理方式对预后的影响.方法 纳入2009年1月至2010年1月首都医科大学宣武医院功能神经外科经小脑水平裂入路行显微血管减压术的三叉神经痛患者94例,回顾性分析其岩静脉的主干及属支情况、进入岩上窦的位置、术中电凝切断及术后并发症的情况.结果 94例患者中,岩静脉主干有1～3支者分别占22.3％(21例)、62.8％(59例)、14.9％(14例),静脉属支有1～5支者分别占4.3％(4例)、21.3％(20例)、64.9％(61例)、7.4％(7例)、2.1％(2例).责任血管明确者83例(88.3％),可疑11例(11.7％),其中岩静脉为责任血管者6例(6.4％).术中岩静脉完全保留者58例(61.7％),主干切断者4例(4.3％),属支切断者32例(34％).术后出现小脑梗死1例(岩静脉属支切断者),经治疗后未遗留明显神经功能缺损;小脑出血1例(切断岩静脉主干者),行后颅窝减压术后残留头晕、走路不稳等症状.结论 经小脑水平裂入路是三叉神经痛显微血管减压术较理想的手术入路.多数情况下术中不必切断岩静脉,必要时可以切断主干或部分属支,但切断后极少数患者会发生小脑梗死或出血.     

血液回抽吸技术在巨大动脉瘤手术中的应用

目的 探讨床突旁大型或巨大动脉瘤手术中血液回抽吸技术的方法和效果.方法 12例颈内动脉床突旁段大型或巨大动脉瘤均采用翼点入路,颈内动脉临时阻断孤立动脉瘤后用18号套管针在颈部颈内动脉阻断的远端穿刺,将套管针尾部用负压吸引器连接到血液回收装置,在不切开动脉瘤的情况下使动脉瘤塌陷.术中应用脑电图和体感诱发电位监测.结果 8例血液逆向抽吸后动脉瘤明显塌陷,将动脉瘤壁与周围结构剥离后夹闭.4例逆向抽吸后动脉瘤塌陷不明显,其中3例切开动脉瘤去除瘤内血栓后夹闭动脉瘤,1例改做动脉瘤孤立并行大隐静脉移植颈外动脉-大脑中动脉搭桥.8例术后行DSA检查,动脉瘤均夹闭满意.按改良COS评分术后良好为10例,差为1例,死亡l例.结论 血液回抽吸方法能使大部分床突旁大型或巨大动脉瘤塌陷,从而获得安全和满意的夹闭.术中应用电生理监测有助于发现早期的脑缺血和判断动脉瘤夹闭后有无载瘤动脉狭窄或误夹.     

Hypertrophic cranial pachymeningitis in countries endemic for tuberculosis: diagnostic and therapeutic dilemmas.

Hypertrophic cranial pachymeningitis (HCP) is an uncommon disorder with few studies correlating clinical, imaging and histopathological features. The aim of this study was to describe clinical and laboratory observations and therapeutic options of patients with HCP. Eleven patients with HCP (M:F 6:5; age range, 23-52 years) were evaluated over 10 years. Etiology was ascertained by MRI and laboratory tests and confirmed by biopsy of meninges and/or brain (7), nasal mucosa (1), mediastinal lymph node (1), muscle (2) or conjunctiva (2). Salient clinical features were headache (7), multiple cranial neuropathies (8), visual disturbances (6), seizures (2) and hemiparesis (2). Abnormal tests included: rapid erythrocyte sedimentation rate (3), positive serum venereal disease testing (1), chest CT abnormalities (4/6) and positive Mantoux test (2/5). Cerebrospinal fluid changes (10/11) revealed the following: cell count 0-47/mm(3); protein 14-95 mg/mL; and glucose of 44-79 mg/mL. Contrast MRI revealed a variable extent of thickened dura mater in all patients. Histopathology (n=11) confirmed chronic inflammation (100%) and provided specific etiology in six (vasculitis [2], sarcoidosis [2], tuberculosis [1], Wegener's granulomatosis [1]). Treatment included steroids only (4), anti-tubercular therapy with steroids (5), penicillin (1) and cyclophosphamide and plasmapheresis (1). During follow-up (27.0+/-26.3 months) there was significant recovery (9/9). On serial imaging (4), the lesion remained the same in three and resolved partially in one patient. HCP, despite frequently posing diagnostic and therapeutic challenges, has favorable outcome when treated appropriately.