Sensory axonopathy in hereditary distal spinal muscular atrophy

A girl of 14 year is presented with a distal spinal muscular atrophy (SMA) with autosomal recessive inheritance. The technical findings are in agreement with the diagnosis. Light microscopical examination of sural nerve biopsy, including teased fiber studies and morphometry, showed no abnormalities. Electron microscopical investigation however demonstrated axonal pathology. The question arises if distal SMA is a distal axonopathy mainly of motor nerves, but to some extent also of sensory nerves.     

Effect of naloxone on detrusor-sphincter dyssynergia in the chronic spinal cat

Naloxone, an opioid peptide antagonist, has been reported to facilitate voiding in neurologic bladder disorders, but its effects on the neural micturition reflex arc are poorly understood. We studied the effect of naloxone in 34 male adult cats, spinalized at C5-C6 level 7 to 119 days previously. Each cat served as its own control. The following tests were performed: Urethral pressure profiles, cystosphincterograms with the urethro-vesical junction opened and closed and mechanograms of the detrusor, and the circular and longitudinal urethral muscles. The study included (1) the effects of anesthesia of the bladder and pelvic nerve, as well as that of the urethral and pudendal nerves; (2) the action of naloxone; and (3) the action of oxymorphone. Our results demonstrated that naloxone (1) increased somatic (osteotendinous and nociceptive) reflexes and aggravated spasticity; (2) increased vegetative micturitional and sexual reflexes, in particular the urethra-urethral contraction reflex, aggravating the spasmodic contractions of the external sphincter; and (3) increased the frequency and intensity of the mass reflex. In consequence, we suggest that naloxone is contraindicated in cases of spinal cord lesions with detrusor-sphincter dyssynergia syndrome.     

Unusual course of an epidural rhabdomyosarcoma of the upper thoracic spine

Summary This report deals with a case of rhabdomyosarcoma in the upper thoracic spine. It is of particular interest, not only for the rarity of type and location of this tumour, but for its clinical course, which presented fluctuations of neurological status, included an acute demonstration of complete paraplegia followed by full recovery after conservative treatment, and gradual relapsing of neurological deficit, one year later.     

Primary spinal paragangliomas: a clinicopathological and immunohistochemical study of 30 cases

Aims: Extra-adrenal paragliomas are neoplasms which have been the subject of much debate regarding parameters to establish their biological behaviour. This study describes the clinicopathological and immunohistochemical features of 30 cases of spinal paragliomas. Methods and results: There were 15 male and 15 female patients. The median age at diagnosis was 46 years (range 20–74 years). Fourteen patients presented with back pain, two with numbness of the lower extremities, one with difficulty in walking and one with spinal cord compression. Nineteen tumours were located in the lumbar region, six in the cauda equina, two in the filum terminale, two in the thoracic region and one in the cervical region. All patients underwent gross total excision. The size of the tumours ranged from 10 to 50 mm. Histologically, 18 neoplasms showed alveolar (Zellballen) pattern, seven a spindle component, two eosinophilic granular cells suggestive of oncocytic metaplasia, two melanin pigment and one ganglion cells. Positive immunohistochemical results include: neuron-specific enolase 23/23 (100%), synaptophysin 21/23 (91%), S100 protein 22/23 (95%, sustentacular cells), leu-enkephalin 11/23 (47%), somatostatin 8/23 (34%), focal glial fibrillary acidic protein 7/23 (30%), focal keratin 5/23 (21%), neurofilament proteins 3/23 (13%) and adrenocorticotrophic hormine (ACTH) 1/23 (4%). Follow-up information obtained in 20 patients show 17 patients alive over a period of 6–216 months. One patient had bone metastases. Two patients died of unrelated causes, including one of congestive heart failure and one of myocardial infarction. Conclusions: In our experience, spinal paragangliomas behave as slow-growing tumours susceptible to potential cure by total excision. We agree with the current World Health Organization (WHO) classification as grade I tumours. Less than 1% may be locally aggressive. Spinal paragangliomas immunoreact not only for conventional neuroendocrine markers but also for peptides including somatostatin and ACTH and focally for the epithelial marker keratin.     

肛门病手术应用低浓度小剂量利多卡因局麻的效果

目的观察低浓度小剂量利多卡因在肛门病手术中的应用效果。方法选择接受肛门病手术者100例,手术时用0．25％盐酸利多卡因5-20ml,根据病情和手术需要作局部浸润麻醉。结果通过100例的肛门手术观察,结果显示麻醉满意顺利83例,麻醉良好17例,无1例麻醉无效失败。总有效率100％。此法麻醉效果确切,未发现严重不良反应。结论此法具有易于掌握,效果好而安全的优越性,适于临床应用和推广。     

Alfentanil or fentanyl during isoflurane–based anaesthesia for day–care knee arthroscopy?

Forty patients agreed to participate in a study to compare whether fentanyl or alfentanil used as analgesic is associated with quicker recovery following anaesthesia for outpatient arthroscopy procedure. Psychomotor tests including choice reaction time (CRT), perceptive accuracy test (PAT) and finger tapping test (FTT) were done prior to induction of anaesthesia with propofol (2–3 mg–kg^-1). Patients were then divided into two groups: Group F (fentanyl) received 0.1 mg fentanyl prior to start of surgery and thereafter 0.05 mg every 30 min during the procedure. Group A (alfentanil) received 0.5 mg alfentanil prior to the onset of surgery and 0.25 mg every 15 min thereafter. Anaesthesia was then maintained using isoflurane (0.5–2%) in oxygen and air (Fio₂ 0.33) during spontaneous respiration with a face mask in a Bain's system. Psychomotor tests were repeated every 45 min postoperatively. Clinical recovery, visual analogue pain intensity score (VAS) and time to discharge home were also assessed by a nurse blind to the method used. Patients in Group A returned to baseline values on the FTT after 90 min while those in Group F did not return to baseline values until 135 min after the end of the operation. Clinical recovery and time to discharge home ("home ready") were also significantly longer in Group F. There was no difference in recovery as seen in the PAT and CRT between the groups. Also, there was no difference in the incidence of side effects and the pain intensity (VAS) scores were similar in the two groups at all time periods. We conclude that recovery following alfentanil is quicker compared to fentanyl when anaesthesia is based on isoflurane.     

Epidural pethidine and bupivacaine in labour

A double-blind randomised study was performed to assess the value of the addition of pethidine 50 mg to the initial dose of bupivacaine given for epidural analgesia in labour. Forty-nine patients received either 1 ml of saline (n = 24), or 50 mg of pethidine (n = 25), added to 9 ml of 0.25% bupivacaine as an initial injection for intrapartum epidural analgesia. There was a significant increase in the mean duration of analgesia in the pethidine group. However, pethidine did not increase the speed of onset of analgesia, or improve the quality of analgesia.     

Preoperative metoprolol improves cardiovascular stability and reduces oxygen consumption after thoracotomy

Background : Increased sympathetic activity perioperatively and associated cardiovascular effects play a central role in cardiovascular complications. High thoracic epidural blockade attenuates the sympathetic response, but even with complete pain relief, haemodynamic and endocrine responses are still present. Beta–adrenoceptor blockade is effective in situations with increased sympathetic activity. This study was designed to evaluate the perioperative haemodynamic effect of preoperative βblockade and its influence on the haemodynamic aspects of the surgical stress response.
Methods : Thirty–six otherwise healthy patients undergoing elective thoracotomy for lung resection were randomised doubleblinded to receive either 100 mg metoprolol or placebo preoperatively. Anaesthesia was combined high thoracic epidural block and general anaesthesia. The epidural analgesia was continued during recovery. Patients were monitored with ECG, pulse oximetry, invasive haemodynamic monitoring, arterial blood gases and electrolytes.
Results : After induction of anaesthesia the mean arterial pressure (MAP) decreased in both groups, and decreased further in the placebo group after initiation of the epidural block. The heart rate (HR) was slightly less throughout the observation period after metoprolol. Peroperatively, the only difference in measured haemodynamics was a marginally higher MAP after metoprolol. Postoperative cardiac index (CI) was lower with a lower variability and cardiac filling pressures were slightly higher in the metoprolol group. The oxygen consumption index was higher after placebo throughout the observation period, with no difference in the oxygen delivery.
Conclusion. We found that preoperative β–blockade during combined general anaesthesia and high thoracic epidural blockade stabilised perioperative HR and CI and decreased total oxygen consumption.