Dynamic analysis of muscles and the internal structure of the peripheral nerve as biomarkers of amyotrophic lateral sclerosis: A pilot study with ultrasound imaging.

INTRODUCTION: The aim of this study was to determine the behaviour of ultrasound biomarkers of fascicle density and muscle strength in patients with amyotrophic lateral sclerosis (ALS). METHODS: We conducted an observational, cross-sectional pilot study of 14 patients with ALS (28.6% women) and 14 controls. Bilateral cross-sectional ultrasound scans were performed in the abductor pollicis brevis (APB) and tibialis anterior (TA) muscles, with recording of muscle thickness (MT) at rest and in contraction, and the difference in thickness. In the median, sciatic, and common peroneal nerves, we analysed the cross-sectional area (CSA), number of fascicles (NF) and fascicle density (FD). Analyses were nested by laterality. RESULTS: Intra- and interrater agreement regarding NF was very good, with a minimum detectable error of < 0.7%. In patients with ALS, MT was lower in the APB both at rest (P = .003; g-Hedges = 1.03) and in contraction (P = .017; g-Hedges = 0.78) and in TA at rest (P = .002; g-Hedges = 0.15) and in contraction (P = .001; g-Hedges = 0.46), with lower thickening capacity. In the nerves, patients displayed lower CSA, with lower NF and higher FD. Significant correlations were found between MT of the ABP and Medical Research Council (MRC) scores for muscle strength (r = 0.34; r2 = 12%; P = .011) and with revised ALS Functional Rating Scale scores (r = 0.44; r2 = 19%; P < .001). The difference in TA thickening correlated with MRC scores (r = 0.30; r2 = 15%; P = .003) and with revised ALS Functional Rating Scale scores (r = 0.26; r2 = 7%; P = .049). NF in the sciatic nerve showed a significant correlation with MRC scores (r = 0.35; r2 = 12%; P = .008). CONCLUSION: MT measurements derived from dynamic testing together with NF and FD may be useful biomarkers for monitoring patients with ALS and establishing a prognosis.

[Quality control of Doppler diagnoses in vascular neurology]. / Control de calidad del diagnóstico mediante Doppler en neurología vascular.

INTRODUCTION: The use of ultrasonography in the study of extracranial pathologies is becoming widespread among the different neurology services in our community. Its main limitation is that it is a technique that depends on the explorer. AIMS: The purpose of this study is to determine the reliability of our Neurosonology laboratory by validating its results. PATIENTS AND METHODS: We conducted a retrospective study by selecting the first 100 consecutive patients (with ischemic stroke) who had been examined in the year 2001 in the Neurosonology laboratory in our hospital. These subjects had to fulfil the requirement of having been submitted to both a Doppler study (carotid and transcranial) and a vascular neuroimaging study using magnetic resonance angiography (MRA) or panarteriography of the supra-aortic trunks (SAT) and of the brain by means of femoral puncture. RESULTS: In the study of the SAT the Doppler detected stenosis or occlusion with a sensitivity of 91.4% and a specificity of 92.3% and, therefore, a positive predictive value (PPV) of 86.4%. 75% of the occlusions were diagnosed with three false positives (which were in fact critical stenoses above 95% instead of occlusion). As regards the evaluation of the transcranial Doppler (TCD), the sensitivity of the Doppler scan to detect stenoses above 50% is situated at 79%, with a PPV of 93%. For diffuse atheromatosis, however, sensitivity is 92.8%, and specificity is 93%. DISCUSSION: Findings agree with those offered by other studies conducted in our area using this technique, and show the reliability of this technique carried out in our Neurosonology laboratory. We therefore believe that Doppler echography of the SAT and TCD can be used for achieve an initial evaluation of cerebrovascular pathologies.

[Direct carotid-cavernous fistulas: endovascular treatment using a detachable balloon]. / Fístulas carotidocavernosas directas: tratamiento endovascular mediante balón desprendible.

INTRODUCTION: Carotid cavernous fistulas are abnormal communications between the cavernous sinus and the internal and/or external carotid artery. They may be spontaneous or acquired. Two basic types are distinguished as a function of the aetiology, clinical features and treatment. The direct type is usually due to trauma while the indirect is of varied aetiology. CLINICAL CASE: We present a series of seven cases of direct carotid-cavernous fistulas, four post-traumatic and three spontaneous, with initial symptoms of ocular involvement and ophthalmoparesia predominantly. All were treated, in our hospital, by embolization with a detachable balloon in the venous variant of the fistula in six cases and by carotid occlusion in one case. Only one severe complication due to distal embolization occurred. CONCLUSIONS: It is necessary to consider the diagnosis of carotid-cavernous fistulas in patients complaining of acute alterations of ocular motility, headache and/or other ocular symptoms, especially when there is a clinical history of head injury. There should be minimal delay between diagnosis and treatment since there is a high risk of hemorrhage and irreversible sequelae caused by alteration of the venous drainage when they are long-standing. The treatment of choice for direct fistulas is by detachable balloon, which has been shown to be superior to other techniques.

Tumor necrosis factor alpha as a uremic toxin: correlation with neuropathy, left ventricular hypertrophy, anemia, and hypertriglyceridemia in peritoneal dialysis patients.

Tumor necrosis factor alpha (TNF alpha) is usually excreted by the kidney. In dialysis patients, it accumulates. TNF alpha has been implicated in the pathogenesis of malnutrition, diabetic neuropathy, and erythropoietin resistance. We studied TNF alpha plasma levels in 49 stable peritoneal dialysis (PD) patients, with the aim of correlating those levels with the presence and severity of peripheral neuropathy, hypertrophic cardiomyopathy, and anemia. Kt/Vurea' residual renal creatinine clearance (CrC), nutritional markers, and general biochemistry were also determined. The average plasma level of TNF alpha was 67 +/- 32 pg/mL (range: 18.1-156.3 pg/mL; normal value 3-20 pg/mL). No correlation was observed between TNF alpha and KT/Vurea' but a negative correlation with CrC was seen (r: -0.37, p < 0.05). TNF alpha levels were higher in patients with neuropathy as compared to patients with normal results (72.5 +/- 32 pg/mL vs 44 +/- 22 pg/mL, p < 0.05). Patients with neuropathy also showed a lower CrC value (1.5 +/- 1.7 mL/min vs 3.9 +/- 2.6 mL/min, p < 0.01). TNF alpha levels were higher in patients with left ventricular hypertrophy (LVH) with respect to normal individuals (70.4 +/- 32 pg/mL vs 38.5 +/- 20.8 pg/mL, p < 0.05). Patients with severe LVH showed the lowest CrC value. A direct, significant relationship was found between TNF alpha levels and weekly erythropoietin dose (r: 0.41, p < 0.05). Patients with hypertriglyceridemia or taking lipid-lowering agents showed a positive linear correlation between TNF alpha and triglycerides (r = 0.7, n = 14, p < 0.05). These data suggest that accumulation of TNF alpha may contribute to the development or maintenance of some neurologic, hematologic, and cardiac complications of uremic syndrome. Loss of residual renal function conditions an increment in TNF alpha levels. These data continue to add support to the idea that TNF alpha may be considered a uremic toxin.