Ornithine transcarbamylase deficiency(OTCD)is a most common ornithine cycle (urea cycle) disorder. It is a X-link inherited disorder caused by
Humans ; Hyperammonemia/etiology* ; Liver Transplantation ; Nervous System Diseases/prevention & control* ; Ornithine Carbamoyltransferase Deficiency Disease/therapy*

Chemotherapy-induced peripheral neuropathy (CIPN) is a serious clinical problem and challenging for oncologists. CIPN is often a persistent adverse consequence of certain chemotherapeutic agents and more cancer survivors will experience CIPN leading to chronic pain and worsening quality of life. However, the available and effective strategies for clinical treatment of CIPN are very limited. Oncologists are frequently obliged to decrease or stop neurotoxic anticancer drugs, with a possible deleterious impact on the oncological prognostic. The challenges faced by CIPN include further study on the pathological mechanism, dose threshold, incidence, risk factors and clinical characteristics of CIPN; lack of diagnostic criteria and tools of CIPN; lack of effective and standardized CIPN prevention and treatment programs. The current update of research results on these challenging issues of CIPN will provide more decision-making evidence for oncologists to diagnose and treat CIPN. Therefore, Committee of Neoplastic Supportive-Care of China Anti-Cancer Association and Cancer Clinical Chemotherapy Committee of China Anti-Cancer Association convenes some experts to summarize the recent literatures and discuss to reach the consensus about recommendations for the definition, pathophysiological mechanism, assessment, prevention, and treatment of CIPN.
Antineoplastic Agents/adverse effects* ; Consensus ; Humans ; Neoplasms/drug therapy* ; Peripheral Nervous System Diseases/prevention & control* ; Quality of Life

Chemotherapy-induced peripheral neuropathy (CIPN) is one of the most common and severe adverse effects related to cancer treatment. Unfortunately, although several agents and protocols have been proposed, no prophylactic strategies have yet to be proven useful. Therefore, new alternative therapies have been considered for CIPN prevention. Herbal medicine in Japan, called Kampo medicine, is derived from traditional Chinese medicine. Goshajinkigan (GJG) is a Kampo medicine, that is comprised of ten herbs. The aim of this work is to analyse the results of pre-clinical and clinical studies on the potential applications of GJG in CIPN prevention.
Antineoplastic Agents ; adverse effects ; Drugs, Chinese Herbal ; therapeutic use ; Humans ; Medicine, Kampo ; Peripheral Nervous System Diseases ; chemically induced ; prevention & control ; Phytotherapy

OBJECTIVETo discuss the diagnosis and treatment of peripheral nerve injury in the earthquake.

METHODSFourteen patients with peripheral nerve injury injured in the earthquake were involved the retrospective study. All cases accepted the timely diagnosis and treatment including anastomosis and repair of the nerve and other conservative treatments. Then the therapeutic effects were observed.

RESULTSAll 14 patients got short-term follow-up and attained the improvement in their symptoms of nerve injury.

CONCLUSIONPeripheral nerve injury has a high incidence in the earthquake. Prevention is very important. The timely and effective treatment should be taken according to spot situations and traumatic conditions of casualties in earthquake.

China ; Earthquakes ; Female ; Follow-Up Studies ; Humans ; Male ; Peripheral Nerve Injuries ; Peripheral Nerves ; surgery ; Peripheral Nervous System Diseases ; diagnosis ; prevention & control ; surgery ; therapy ; Retrospective Studies

BACKGROUNDThe awareness, treatment and prevention of chronic diseases are generally poor among the elderly population of China, whereas the prevention and control of chronic diseases in elderly veteran communities have been ongoing for more than 30 years. Therefore, investigating the awareness status of chronic disabling neurological diseases (CDND) and common chronic diseases (CCD) among elderly veterans may provide references for related programs among the elderly in the general population.

METHODSA cross-sectional survey was conducted among veterans ≥60 years old in veteran communities in Beijing. The awareness of preventive strategies against dementia, Alzheimer's disease (AD), Parkinson's disease (PD), sleep disorders, cerebrovascular disease (CVD) and CCD such as hypertension, and the approaches used to access this information, including media, word of mouth (verbal communication among the elderly) and health care professionals, were investigated via face-to-face interviews.

RESULTSThe awareness rates for CCD and CVD were approximately 100%, but that for AD was the lowest at <10%. The awareness rates for sleep disorders, PD and dementia, were 51.0-89.4%. Media was the most commonly selected mode of communication by which veterans acquired knowledge about CCD and CVD. Media was used by approximately 80% of veterans. Both health care professionals and word of mouth were used by approximately 50% of veterans. With respect to the source of information about CDND excluding AD, the rates of the use of health care professionals, word of mouth and media were 10.6-28.2%, 56.5-76.5%, and approximately 50%, respectively.

CONCLUSIONSThe awareness of CDND among elderly veterans was significantly lower than that of CCD. More information about CDND should be disseminated by health care professionals. Appropriate guidance will promote the rapid and extensive dissemination of information about the prevention of CDND by media and word-of-mouth peer education.

Aged ; Aged, 80 and over ; Awareness ; physiology ; Chronic Disease ; prevention & control ; Cross-Sectional Studies ; Female ; Health Knowledge, Attitudes, Practice ; Humans ; Male ; Nervous System Diseases ; prevention & control ; Veterans ; statistics & numerical data

OBJECTIVETo discuss the prevention and management of the neurological complications during treatment for severe scoliosis.

METHODSSeventy-one patients with the coronal Cobb angle more than 80 degrees underwent operation. The average coronal Cobb angle was 96.6 degrees (80 degrees - 135 degrees ), and 31 patients combined with kyphotic deformity with average sagittal Cobb angle 83.0 degrees (52 degrees - 145 degrees ). Fourteen patients were treated with posterior pedicle screw fixation alone, 21 combined with posterior wedge resection, 34 with staged operation, and 2 with combined anterior and posterior approach. Intraoperative somatosensory evoked potentials (SEP) monitoring and wake up test were used in 61 cases, and wake up test was used alone in 10 cases.

RESULTSThe average coronal curve correction was 59.2% (average 39.6 degrees ) and sagittal curve correction was 61.6% (average 31.9 degrees ). Thirty-nine patients were followed up with average 51 months (5 - 81 months). Among them, 33 patients achieved solid spinal fusion and the rate of correction loss was 2.1%. Two patients underwent reoperation because of the breakages of the internal fixation. Among 8 patients with neurological dysfunction pre-operatively, 3 patients obtained complete recovery, 1 patient incomplete recovery, and the rest had not recovered because of post-poliomyelitis syndrome. Four of 5 patients developed neurological dysfunction post-operatively obtained complete recovery and 1 partial recovery.

CONCLUSIONSHalo-pelvic distraction and apical vertebra osteotomy are useful for increasing the correction rate and decreasing the neurological dysfunction. Intraoperative SEP monitoring combined with wake up test can call attention to early nerve injury. Early using of glucocorticoids and dehydration therapy promptly post-operatively is benefit to prevent neurological complications.

Adolescent ; Adult ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Humans ; Male ; Monitoring, Intraoperative ; Nervous System Diseases ; etiology ; prevention & control ; therapy ; Osteotomy ; methods ; Postoperative Complications ; prevention & control ; therapy ; Scoliosis ; pathology ; surgery ; Traction ; methods

OBJECTIVETo assess impact of different brain protection techniques upon postoperative temporary neurological dysfunction in aortic surgery with the aid of deep hypothermic circulatory arrest.

METHODSFrom January 2003 to December 2005, 78 patients who met the inclusion criteria entered the present cohort, 43 of whom were under the aid of deep hypothermic circulatory arrest plus retrograde cerebral perfusion (RCP group) and the other 35 under deep hypothermic circulatory arrest plus selective antegrade cerebral perfusion (SCP group). The present and grades of postoperative temporary neurological dysfunction were assessed by independent observers with the same criterion. The impact of duration of deep hypothermic circulatory arrest upon the postoperative temporary neurological dysfunction was also evaluated.

RESULTSThe incidence of postoperative temporary neurological dysfunction was significantly higher in the RCP group than in the SCP group (15, 34.9% vs. 4, 11.4%, P<0.05). And long duration of deep hypothermic circulatory arrest (more than 50 min) has a negative impact on the postoperative temporary neurological dysfunction rate.

CONCLUSIONSApplying selective antegrade cerebral perfusion as the brain protection technique and shortening the duration of deep hypothermic circulatory arrest can reduce the incidence of temporary neurological dysfunction and preserve cerebral function more effectively.

Adult ; Aged ; Aged, 80 and over ; Aorta ; surgery ; Brain ; blood supply ; physiopathology ; Circulatory Arrest, Deep Hypothermia Induced ; adverse effects ; Female ; Humans ; Male ; Middle Aged ; Nervous System Diseases ; etiology ; prevention & control ; Perfusion ; methods ; Postoperative Complications ; etiology ; prevention & control

Near-infrared spectroscopy (NIRS), a noninvasive optical method, utilizes the characteristic absorption spectra of hemoglobin in the near-infrared range to provide information on cerebral hemodynamic changes in various clinical situations. NIRS monitoring have been used mainly to detect reduced perfusion of the brain during orthostatic stress for three common forms of orthostatic intolerance (OI); orthostatic hypotension, neurally mediated syncope, and postural orthostatic tachycardia syndrome. Autonomic function testing is an important diagnostic test to assess their autonomic nervous systems for patients with symptom of OI. However, these techniques cannot measure dynamic changes in cerebral blood flow. There are many experimentations about study of NIRS to reveal the pathophysiology of patients with OI. Research using NIRS in other neurologic diseases (stroke, epilepsy and migraine) are ongoing. NIRS have been experimentally used in all stages of stroke and may complement the established diagnostic and monitoring tools. NIRS also provide pathophysiological approach during rehabilitation and secondary prevention of stroke. The hemodynamic response to seizure has long been a topic for discussion in association with the neuronal damage resulting from convulsion. One critical issue when unpredictable events are to be detected is how continuous NIRS data are analyzed. Besides, NIRS studies targeting pathophysiological aspects of migraine may contribute to a deeper understanding of mechanisms relating to aura of migraine. NIRS monitoring may play an important role to trend regional hemodynamic distribution of flow in real time and also highlights the pathophysiology and management of not only patients with OI symptoms but also those with various neurologic diseases.
Absorption ; Autonomic Nervous System ; Brain ; Cerebrovascular Circulation ; Complement System Proteins ; Diagnostic Tests, Routine ; Epilepsy ; Hemodynamics ; Humans ; Hypotension, Orthostatic ; Methods ; Migraine Disorders ; Nervous System Diseases* ; Neurons ; Orthostatic Intolerance* ; Perfusion ; Postural Orthostatic Tachycardia Syndrome ; Rehabilitation ; Secondary Prevention ; Seizures ; Spectroscopy, Near-Infrared* ; Spectrum Analysis ; Stroke ; Syncope

OBJECTIVETo study the mechanism of bortezomib inducing peripheral neuropathy and the reversing affection of reduced glutathione.

METHODSFemale Wistar rats were randomly divided into three groups. Group 1, treatment with bortezomib; Group 2, treatment with bortezomib and reduced glutathione; Group 3, saline control group. Drugs were administrated on the 1st, 4th, 7th and 11th day for the three groups. The amorphous of sciatic nerve and dorsal root ganglion (DRG) were observed by electron microscope on 14th and 42nd day. On 14th day, laser confocal microscopy was used to detect reactive oxygen species (ROS) of DRG neuron obtained from the rats by treated with DCFH-DA after primary culture.

RESULTSOn 14th day, morphology of sciatic nerve and DRG changed in both group 1 and 2. On 42nd day, the amorphous became normally in group 1. On 14th day, ROS releasing from DRG neuron was increased obviously in group 1 (P < 0.01), while decreased in both group 2 and 3, and the difference between the latter two groups had no statistical significance (P = 0.210).

CONCLUSIONReleasing ROS to injure mitochondrion and endoplasmic reticulum maybe involved in bortezomib induced peripheral neuropathy. Although reduced glutathione can inhibit ROS release, it has no obviously reversal effect for peripheral neuropathy.

Animals ; Boronic Acids ; adverse effects ; Bortezomib ; Female ; Glutathione ; therapeutic use ; Peripheral Nervous System Diseases ; chemically induced ; metabolism ; prevention & control ; Pyrazines ; adverse effects ; Rats ; Rats, Wistar ; Reactive Oxygen Species ; metabolism

The clinical effect of laparoscopic rectal cancer curative excision with pelvic autonomic nerve preservation (PANP) was investigated. This study evaluated the frequency of urinary and sexual dysfunction of 149 male patients with middle and low rectal cancer who underwent laparoscopic or open total mesorectal excision with pelvic autonomic nerve preservation (PANP) from March 2011 to March 2013. Eighty-four patients were subjected to laparoscopic surgery, and 65 to open surgery respectively. The patients were followed up for 12 months, interviewed, and administered a standardized questionnaire about postoperative functional outcomes and quality of life. In the laparoscopic group, 13 patients (18.37%) presented transitory postoperative urinary dysfunction, and were medically treated. So did 12 patients (21.82%) in open group. Sexual desire was maintained by 52.86%, un-ability to engage in intercourse by 47.15%, and un-ability to achieve orgasm and ejaculation by 34.29% of the patients in the laparoscopic group. Sexual desire was maintained by 56.36%, un-ability to engage in intercourse by 43.63%, and un-ability to achieve orgasm and ejaculation by 33.73% of the patients in the open group. No significant differences in urinary and sexual dysfunction between the laparoscopic and open rectal resection groups were observed (P>0.05). It was concluded that laparoscopic rectal cancer radical excision with PANP did not aggravate or improve sexual and urinary dysfunction.
Adult ; Autonomic Nervous System ; injuries ; Humans ; Laparoscopy ; adverse effects ; Male ; Middle Aged ; Peripheral Nerve Injuries ; etiology ; prevention & control ; Postoperative Complications ; Rectal Neoplasms ; surgery ; Sexual Dysfunction, Physiological ; etiology ; Urologic Diseases ; etiology