Longitudinal effects of noninvasive positive-pressure ventilation in patients with amyotrophic lateral sclerosis

OBJECTIVE:To evaluate the duration of benefit on symptoms, quality of life, and survival derived from the use of noninvasive positive-pressure ventilation by patients with amyotrophic lateral sclerosis. DESIGN: In this prospective, cohort study, 30 of 36 consecutively referred symptomatic patients tolerated nightly noninvasive positive-pressure ventilation and undertook pulmonary function testing and 12 symptom and quality-of-life instruments concerning sleep quality, daytime sleepiness, physical fatigue, mental fatigue, and depression that were administered during a 10-mo period. RESULTS: With treatment, there was a significant improvement in the majority of patients in sleep quality, daytime sleepiness, physical fatigue, and depression; however, significant improvements lasted for up to 10 mo only in sleep quality. Partial pressure of arterial oxygen, partial pressure of arterial carbon dioxide, and oxyhemoglobin saturation remained stable or even improved for up to 7 mo during use of part-time noninvasive positive-pressure ventilation. A total of 14 patients had survival prolonged by continuous dependence on noninvasive positive-pressure ventilation. CONCLUSIONS: Noninvasive positive-pressure ventilation provides a long-lasting benefit on symptoms and quality of life indicators for amyotrophic lateral sclerosis patients and should be offered to all patients with symptoms of sleep disordered breathing or inspiratory muscle dysfunction. It can also prolong tracheostomy-free survival.     

Management of haemorrhagic type moyamoya disease with intraventricular haemorrhage during pregnancy

Summary Moyamoya (“hazy puff-of-smoke”) disease represents a rare condition with progressive narrowing and occlusion of basal cranial vessels with secondary specific neoangiogenesis; we report on a 25-year-old primigravida with known moyamoya disease who suffered from acute bilateral intraventricular haemorrhage at 24 weeks gestation. She underwent bilateral external ventricular drainage and intraventricular recombinant tissue plasminogen activator (r-TPA) lysis was performed. At 34 weeks’ gestation, a healthy girl was delivered via Caesarean section. Encephalomyosynangiosis (EMS) and extra-intracranial (EC/IC) bypass surgery were performed six and eight months after delivery, respectively. The patient recovered almost completely and showed only mild residual deficits. Prompt diagnosis and immediate interdisciplinary treatment might have been the key for optimal maternal and neonatal outcome in our patient.     

Emergency endovascular stent-grafting for life-threatening acute type B aortic dissections

Background

There is still a considerable controversy regarding optimal treatment for patients with acute type B aortic dissection. Patients with complicated disease are particularly challenging for cardiovascular treatment. Early surgery for acute dissections of the descending aorta with life-threatening complications is known to carry a high mortality. Endovascular stent grafting is developing as an alternative treatment mainly for chronic stages of type B aortic dissection. It is not clear whether endovascular stent grafting is safe and effective in emergency treatment of acute type B aortic dissection.

Methods

In 10 patients (7 men, 3 women; mean age, 59.2 years; range, 46 to 65 years), endovascular stent grafting was performed within 11.0 ± 5.9 hours (range, 4 to 24 hours) of diagnosis of complications. Indications for acute intervention included contained rupture, hematothorax, life-threatening malperfusion, and refractory pain. Using a retrograde endovascular route after surgical exposure of the femoral artery, self-expanding stent prostheses consisting of polyester-covered Nitinol (Talent, World Medical; mean diameter, 40 ± 4 mm; length, 10 cm) were placed into the descending aorta distal to the subclavian artery. Before discharge and on follow-up visits, imaging of the aorta was performed using computed tomography.

Results

In 9 of 10 patients (90%), the primary entry could be completely occluded with the endovascular stent. Early mortality was 20% (2 of 10): 1 patient died after disruption of the intimal layer distal to the stent, and 1 patient died in hemorrhagic shock after surgical fenestration of the abdominal aorta for persistent malperfusion. Three patients (30%) required consecutive surgical treatment: indications included acute development of retrograde type A aortic dissection, acute stent dislocation by fractured wires and secondary leakage, and late formation of an aneurysm of the descending aorta 6 months after endovascular stent grafting. There were no surgical or late deaths.

Conclusions

Our experience provides some evidence that early mortality of life-threatening acute type B aortic dissection may be reduced by emergency endovascular stent grafting and that this form of treatment is a promising therapeutic option. Refinements, especially in stent design and application, may further improve the prognosis of patients in the life-threatening situation of complicated acute type B aortic dissection.     

Comparative clinical studies on parenteral and oral premedication in childhood with special regard to the volume and acidity of the gastric juice

90 children of age between 1 and 10 years who were scheduled for adenotomy were randomized into 3 premedication-groups. Group 1 received no premedication, group 2 chlorprothixene 1 mg/kg i.m. and group 3 chlorprothixene 2 mg/kg orally. Prior to induction of anaesthesia 63.3% of the unpremedicated children were anxious or crying, in contrast to 23.3% of the children given oral or i.m. premedication. With respect to the reaction on venepuncture with a Butterfly G 23, there was no significant difference between the groups. The volume of gastric acid (median values) was 0.056 ml/kg in group 1, 0.063 ml/kg in group 2, and 0.068 ml/kg in group 3, with a pH of 2.0, 1.5 and 2.0 respectively. One hour after operation 44.8% of the children in group 1, 92.6% in group 2 and 84.6% in group 3 were calm or sleeping. Premedication definitively improves the pre- and postoperative management of children. We now recommend oral chlorprothixene for the premedication of children because the effect of this oral premedication is equivalent to i.m. application, and the oral route has no influence on the quantity or acidity of gastric contents.     

Cerebrospinal fluid and serum from patients with inflammatory polyradiculoneuropathy have opposite effects on sodium channels

The effects of cerebrospinal fluid (CSF) and serum from patients having Guillain–Barré syndrome (GBS) or chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) on voltage-dependent Na⁺ channels were compared. Bathing human myoballs in CSF substantially reduced their Na⁺ currents (by >40% with 8 of 10 patients) elicited at 1 Hz under whole-cell recording conditions. This was because, at the resting potential, more Na⁺ channels were inactivated (left-shift of the h_∞ curve). CSF from patients with other neurological diseases (OND) produces a similar, but smaller, effect. In contrast, serum samples from the same GBS and OND patients caused an increase of the Na⁺ currents by reducing the number of Na⁺ channels inactivated at the resting potential. This right-shift of the h_∞ curve is in part explained by the effect of serum albumin. We confirm that the CSF of most GBS and CIDP patients contains factors inhibiting voltage-dependent Na⁺ currents. There is no indication that such factors are effective in the serum of these patients. © 1995 John Wiley & Sons, Inc.     

Interstitial photodynamic therapy of nonresectable malignant glioma recurrences using 5-aminolevulinic acid induced protoporphyrin IX

BACKGROUND AND OBJECTIVE: Limited knowledge of the light and temperature distribution within the target volume in combination with non-selective accumulation of the applied photosensitizers (PS) has hampered the clinical relevance of interstitial photodynamic therapy (iPDT) for treatment of malignant glioma patients. The current pilot study focused on the development and the clinical implementation of an accurate and reproducible irradiation scheme for iPDT using 5-aminolevulinic acid (5-ALA) induced protoporphyrin IX (PPIX) as a selectively working PS. STUDY DESIGN/MATERIALS AND METHODS: Monte Carlo simulations of fluence rate and heat transport simulations were performed using the optical properties of normal brain tissue infiltrated by tumor cells (absorption coefficient micro(a) = 0.2 cm(-1), reduced scattering coefficient: micro'(s) = 20 cm(-1)). A modified 3-D treatment-planning software was used to calculate both, the treatment-volume and the exact position of the light diffusers within the lesion. The feasibility and the risk of iPDT were tested in 10 patients with small and circumscribed recurrent malignant gliomas. RESULTS: The optimum distance between the implanted light diffusers was determined to be 9 mm with regard to both fluence rate and temperature distribution. For this distance a temperature increase above 42 degrees C was not expected to occur. Up to six cylindrical light diffusers were stereotactically implanted to achieve a complete irradiation of the tumor volume, which was possible in every single patient (mean tumor volume: 5.9 cm3). The total applied light fluence was between 4,320 J and 11,520 J. Side effects of iPDT were not observed. Median survival was 15 months. CONCLUSION: 5-ALA iPDT in combination with a 3-D treatment-planning (which was based on optical and thermal simulations) is a safe and feasible treatment modality. The clinical impact of these findings deserves further prospective evaluation.