Intracranial meningeal melanocytoma associated with ipsilateral nevus of Ota. Case report

In this report, the authors review the case of a man with a neurocutaneous syndrome. He presented with an intracerebral melanocytoma associated with a blue nevus of the scalp; its location and its appearance during childhood supported the diagnosis of a nevus of Ota. Meningeal melanocytomas are increasingly being diagnosed, but remain rare. Primary meningeal malignant melanoma is the first differential diagnosis to eliminate. Despite their common embryonic origin. the association of a melanocytoma with a nevus of Ota is rare. A nevus of Ota exhibits the same melanocytic proliferation and affects the trigeminal nerve territory. An ocular effect is not always observed. In contrast to an ocular lesion, a nevus of Ota rarely transforms into a malignant melanoma. It is found only among caucasians. During 4 years of follow-up review after surgery, the patient remained asymptomatic. Other than antiepileptic therapy, he received no complementary treatment and cerebral imaging revealed no evidence of recurrence.     

Ischemic preconditioning specifically restores complexes I and II activities of the mitochondrial respiratory chain in ischemic skeletal muscle

OBJECTIVE: Defective mitochondrial function has been reported in patients presenting with peripheral arterial disease, suggesting it might be an important underlying mechanism responsible for increased morbidity and mortality. We therefore determined the effects of prolonged ischemia on energetic skeletal muscle and investigated whether ischemic preconditioning might improve impaired electron transport chain and oxidative phosphorylation in ischemic skeletal muscle. METHODS: Thirty rats were divided in three groups: the control group (sham, n = 9) underwent 5 hours of general anesthesia without any ischemia, the ischemia-reperfusion (IR) group (n = 11) underwent 5 hours ischemia induced by a rubber band tourniquet applied on the left root of the hind limb, and in the third group, preconditioning (PC group, n = 10) was performed just before IR and consisted of three cycles of 10 minutes of ischemia, followed by 10 minutes reperfusion. Maximal oxidative capacities (V(max)) of the gastrocnemius muscle and complexes I, II, and IV of the mitochondrial respiratory chain were determined using glutamate-malate (V(max)), succinate (V(s)), and N, N, N,'N'-tetramethyl-p-phenylenediamine dihydrochloride ascorbate as substrates. RESULTS: Physiologic characteristics were similar in the three groups. Ischemia reduced V(max) by 43% (4.5 +/- 0.4 vs 7.9 +/- 0.5 micromol O(2)/(min x g dry weight), P < .01) and V(s) by 55% (2.9 +/- 0.3 vs 6.3 +/- 0.4 micromol O(2)/min/g dry weight; P < .01) in the IR and sham groups, respectively, and impairments of mitochondrial complexes I and II activities were evident. Of interest was that preconditioning prevented ischemia-induced mitochondrial dysfunction. Both V(max) and V(s) were significantly higher in the PC rats than in IR rats (+32% and +41%, respectively; P < .05), and were not different from sham values. CONCLUSIONS: Ischemic preconditioning counteracted ischemia-induced impairments of mitochondrial complexes I and II. These data support that ischemic preconditioning might be an interesting approach to reduce muscular injuries in the setting of ischemic vascular diseases.     

A difficult airway is not more prevalent in patients suffering from spasmodic torticollis: a case series

PURPOSE: We designed this retrospective study to assess the frequency of difficult airway and difficult intubation in patients with spasmodic torticollis and compare it to that of the general population. METHODS: After Institutional Review Board approval, data were collected from the charts of all the patients with spasmodic torticollis who underwent selective peripheral denervation at our institution between 1988 and 2001. The intubation grade was determined using the Cormack and Lehane laryngoscopic classification. The best laryngeal view was recorded. RESULTS: Data from 342 patients were available for analysis. Fourteen patients had a difficult airway. In two patients, intubation was difficult with three attempts at laryngoscopy in one patient and use of fibreoptic bronchoscopy in the other. Twelve (3.5%) patients presented with laryngoscopic grades of III or IV. The combined prevalence of laryngoscopic view grade III and IV and difficult intubation was 4.4%. CONCLUSIONS: This study assesses the frequency of difficult intubation in patients suffering from spasmodic torticollis. When compared to the general population, these patients do not appear to have a higher frequency of difficult airway or difficult intubation.     

Cardiovascular disease prevalence in patients with inflammatory arthritis, diabetes mellitus and osteoarthritis: a cross-sectional study in primary care

ABSTRACT: BACKGROUND: There is accumulating evidence for an increased cardiovascular burden in inflammatory arthritis, but the true magnitude of this cardiovascular burden is still debated. We sought to determine the prevalence rate of non-fatal cardiovascular disease (CVD) in inflammatory arthritis, diabetes mellitus and osteoarthritis (non-systemic inflammatory comparator) compared to controls, in primary care. METHODS: Data on CVD morbidity (ICPC codes K75 (myocardial infarction), K89 (transient ischemic attack), and/or K90 (stroke/cerebrovascular accident)) from patients with inflammatory arthritis (n = 1,518), diabetes mellitus (n = 11,959), osteoarthritis (n = 4,040) and controls (n = 158,439) were used from the Netherlands Information Network of General Practice (LINH), a large nationally representative primary care based cohort. Data were analyzed using multi-level logistic regression analyses and corrected for age, gender, hypercholesterolemia and hypertension. RESULTS: CVD prevalence rates were significantly higher in inflammatory arthritis, diabetes mellitus and osteoarthritis compared with controls. These results attenuated - especially in diabetes mellitus - but remained statistically significant after adjustment for age, gender, hypertension and hypercholesterolemia for inflammatory arthritis (OR = 1.5 (1.2-1.9)) and diabetes mellitus (OR = 1.3 (1.2-1.4)). The association between osteoarthritis and CVD reversed after adjustment (OR = 0.8 (0.7-1.0)). CONCLUSIONS: These results confirm an increased prevalence rate of CVD in inflammatory arthritis to levels resembling diabetes mellitus. By contrast, lack of excess CVD in osteoarthritis further suggests that the systemic inflammatory load is critical to the CVD burden in inflammatory arthritis.     

Multiple-level thoracolumbar burst fractures in teenaged patients

The purpose of this study was to examine the characteristics of multiple-level burst fractures in teenaged patients. Five teenaged patients were identified with this injury pattern. The mean age at injury was 17.6 years. All five patients underwent a posterior fusion and an attempted decompression through ligamentotaxis. One of the five went on to have an anterior decompression. Four of the five patients had spinal instrumentation. Neurologic deficit was present in four patients. The proximal fracture was most often responsible for the neurologic deficit when present. The average length of follow-up was 4.5 years. There was no significant neurologic recovery after hospital discharge. Three of the five patients had minimal or no back pain at latest follow-up. The authors conclude that multiple burst fractures should be treated individually based on their clinical and radiographic characteristics.     

Long-term results of less than total parathyroidectomy for hyperparathyroidism in multiple endocrine neoplasia type 1

BACKGROUND: Our aim was to assess long-term results after less than total parathyroidectomy for hyperparathyroidism in multiple endocrine neoplasia type 1. METHODS: Of 1888 patients undergoing operation at our institution for primary hyperparathyroidism between 1972 and 2001, 83 (4.4%) had multiple endocrine neoplasia type 1. Outcome data were available for 79; 66 underwent subtotal parathyroidectomy, 55 (83%) of these with bilateral thymectomy. In 13 patients, only grossly enlarged glands were resected (mean 1.1 per patient) as the syndrome of multiple endocrine neoplasia type 1 was not yet evident or the initial exploration was performed elsewhere. RESULTS: Follow-up has been 48 +/- 51 months (mean + SD). Intraoperative serum PTH assay decay in 20 patients was suggestive of cure in 18 patients, none of whom required reoperation. Nine patients (11%) required reoperation (3 required reoperation twice) after a mean interval of 77 +/- 53 months. Subtotal parathyroidectomy resulted in a lesser reoperation rate than resection of grossly enlarged glands (7% vs 30%, P =.02). At the time of review, 63 patients (80%) were normocalcemic, 10 (13%) hypocalcemic (2 after unsuccessful delayed autograft), and 7% hypercalcemic (none after reoperation). By Kaplan-Meier analysis, the rate of surgical cure (patients who are nonhypercalcemic) is 60% and 51% at 10 and 15 years, respectively. CONCLUSION: Subtotal parathyroidectomy reduces the need for reoperation. Selective reoperation leads to long-lasting biochemic cure.     

Quantitative in vivo islet potency assay in normoglycemic nude mice correlates with primary graft function after clinical transplantation

Reliable assays are critically needed to monitor graft potency in islet transplantation (IT). We tested a quantitative in vivo islet potency assay (QIVIPA) based on human C-peptide (hCP) measurements in normoglycemic nude mice after IT under the kidney capsule. QIVIPA was initially tested by transplanting incremental doses of human islets. hCP levels in mice were correlated with the number of transplanted islet equivalents (r(2) = 0.6, P<0.01). We subsequently evaluated QIVIPA in eight islet preparations transplanted in type 1 diabetic patients. Conversely to standard criteria including islet mass, viability, purity, adenosine triphosphate content, or glucose stimulated insulin secretion, hCP in mice receiving 1% of the final islet product was correlated to primary graft function (hCP increase) after IT (r(2)=0.85, P<0.01). QIVIPA appears as a reliable test to monitor islet graft potency, applicable to validate new methods to produce primary islets or other human insulin secreting cells.