Executive cognitive deficits in primary dystonia.

Primary dystonia is a disorder of movement for which no consistent pathophysiology has been identified; in the absence of evidence to the contrary, it is assumed to be cognitively benign. We have studied a clinically heterogeneous group of 14 patients with primary dystonia on a battery of neuropsychological tests. Despite well-preserved speed of information processing, language, spatial, memory and general intellectual skills relative to normal controls, we have identified a constellation of attentional-executive cognitive deficits on the Cambridge Neuropsychological Test Automated Battery (CANTAB). Specifically, patients demonstrated significant difficulties negotiating the extra-dimensional set-shifting phase of the IED task. The implications of these findings for the pathophysiology of primary dystonia are discussed. This is, to the best of our knowledge, the first report of a significant cognitive deficit in patients with primary dystonia.     

Arthroscopic distal clavicle resection

The acromioclavicular joint is a potential source of pain in the shoulder. There are a variety of disorders that can affect this joint, including distal clavicle osteolysis, posttraumatic arthritis, osteoarthritis, and rheumatoid arthritis. Nonoperative treatment for this condition with nonsteroidal medication and activity modification can alleviate the pain. When conservative treatment is exhausted, surgical resection of the distal clavicle is often necessary. Arthroscopic resection of the distal clavicle preserves the acromioclavicular ligaments to prevent postoperative distal clavicle instability. The procedure is performed in either the beach chair or lateral position and requires the use of a shaver, electrocautery, and a burr for soft tissue and debridement and bone resection.     

Early experience with a novel plaque excision system for the treatment of complex coronary lesions.

The use of directional coronary atherectomy (DCA) in current practice has been limited. The SilverHawk System is a newly developed plaque excision device that aims to overcome the drawbacks of prior DCA platforms. The device was evaluated in a porcine coronary model and in a series of patients. Procedural variables along with outcomes were reviewed. Quantitative angiography (QCA) was performed and excised tissue fragments were weighed and examined histologically. In porcine cases, pretreatment MLD increased from 0.51 +/- 0.26 to 2.36 +/- 0.59 mm postdebulking and 19.9 +/- 7.6 mg of tissue was retrieved. In human cases, pretreatment MLD increased from 0.8 +/- 0.4 to 2.2 +/- 0.5 mm postdebulking and 15.2 +/- 7.8 mg of tissue was retrieved without complications. These data show that the SilverHawk System may offer significant utility in treating a wide variety of complex coronary lesions.     

Effect of the Aldehyde Dehydrogenase Inhibitor, Cyanamide, on the Ex Vivo Sensitivity of Murine Multipotent and Committed Hematopoietic Progenitor Cells to Mafosfamide (Asta Z 7557)

The ex vivo sensitivity of murine multipotent (CFU-GEMM) and committed (CFU-Mk, CFU-GM, BFU-E and CFU-E) hematopoietic progenitor cells to mafosfamide was quantified with and without concurrent exposure to cyanamide, an inhibitor of aldehyde dehydrogenase activity. In the absence of cyanamide, CFU-GEMM, CFU-Mk and CFU-GM were approximately equisensitive to mafosfamide while the erythroid progenitors were more sensitive to the drug. Cyanamide potentiated the cytotoxicity of mafosfamide toward CFU-GEMM and CFU-Mk, but not toward CFU-GM, BFU-E and CFU-E. Cellular aldehyde dehydrogenases are known to catalyze the oxidation of 4-hydroxycyclophos-phamide/aldophosphamide, the major intermediate in cyclophosphamide and mafosfamide activation, to the relatively nontoxic acid, carboxyphosphamide. Thus, our findings indicate that 1) murine CFU-GEMM contain the relevant aldehyde dehydrogenase activity, and 2) the relevant aldehyde dehydrogenase activity is retained upon differentiation to progenitors committed to the megakaryocytoid lineage, but lost upon differentiation to progenitors committed to the granulocytoid/monocytoid and erythroid lineages. The relative insensitivity of CFU-GM to mafosfamide is apparently due to a cellular determinant that influences their sensitivity to all cross-Unking agents since CFU-GM were found to be relatively insensitive to non-oxazaphosphorine cross-linking agents as well.     

Treatment of vasospasm with a 480-nm pulsed-dye laser

Laser energy at a wavelength of 480 nm was applied in 1-microseconds pulses of 3 to 10 mJ to two models of vasospasm. Rabbit common carotid arteries (CCA's) were constricted chronically by the application of human blood within a silicone sheath. Peak vasospasm developed 24 to 48 hours later, and persisted for up to 6 days. Endovascular laser treatment was delivered to 40 CCA's via a 200-microns diameter silica quartz fiber introduced through the femoral artery. The CCA caliber increased from 60% of the pre-vasospasm control diameter to a minimum post-laser diameter of 83% of control. No instances of laser-induced perforation or of arterial thrombosis were observed for up to 60 days after treatment. Prophylactic laser application to nine normal vessels was able to attenuate the development of vasospasm if blood was applied immediately thereafter (88% vs. 59% of control diameter, p less than 0.02), but not if blood was applied 7 days later. Studies in 16 normal CCA's established that there was a considerable margin between the laser energy required to induce dilatation and that which caused perforation, providing that the fiber remained relatively central within the artery. Morphological examination demonstrated focal loss of endothelial cells immediately after laser application, followed approximately 7 days later by the development of areas of intimal hyperplasia. Only minimal changes were observed in the medial or adventitial layers. In a second study, the basilar artery of seven dogs was constricted chronically by two intracisternal injections of autologous blood 3 days apart. Five dogs received endovascular laser treatment 7 or 10 days after the first injection, when basilar artery diameter was reduced to a mean of 61% and 77% of control, respectively. Immediately following treatment, basilar artery diameter increased to 104% and 102% of resting diameter, respectively. Both untreated and laser-treated arteries were smaller than the control diameter at 30 days (80% and 82%, respectively), but in each group the vasodilatory response to hypercapnia was preserved. These findings indicate that 1-microsecond laser pulses are well tolerated by systemic and cerebral arteries in two different animal models, and suggest that the 480-nm pulsed-dye laser may have an application for the treatment or prophylaxis of cerebral vasospasm.     

Ambulatory Surgery Patients May Be Discharged before Voiding after Short-acting Spinal and Epidural Anesthesia

Background: Voiding before discharge is usually required after outpatient epidural or spinal anesthesia because of concern about bladder overdistention and dysfunction. Shorter duration spinal and epidural anesthesia may allow return of bladder function before overdistention occurs in low-risk patients (those younger than age 70, not having hernia, rectal, or urologic surgery, and without a history of voiding difficulty), and predischarge voiding may not be necessary.

Methods: After institutional review board approval and informed consent, 201 low-risk ambulatory patients were prospectively studied in either a standard or accelerated pathway after undergoing spinal or epidural anesthesia with procaine, lidocaine, 2-chloroprocaine, or less than 7 mg bupivacaine; epinephrine was not used in any anesthetic. Standard pathway patients (n = 70) were required to void before discharge. Accelerated pathway (n = 131) patients were not required to void. (After randomization of an initial 163 patients to one of the two tracks, 38 additional patients were assigned to the accelerated pathway.) If accelerated pathway patients voided, they were discharged when all other discharge criteria were met. If they did not spontaneously void after block resolution, a bladder ultrasound (BUS) was performed. If the BUS indicated a urine volume of less than 400 ml, the patients were discharged and instructed to return to the emergency department if they were unable to void within 8 h of discharge. If the BUS indicated a urine volume of greater than 400 ml, the patients were reassessed in 1 h and were discharged if they could void spontaneously. If they could not void spontaneously, they were catheterized to facilitate discharge. All patients were contacted the next day to assess the return of normal bladder function.

Results: All standard pathway patients voided without difficulty, and were discharged in 153 +/- 49 (SD) min. 62 patients in the accelerated pathway voided spontaneously after resolution of their block and were discharged in 127 +/- 41 min. 46 patients were discharged with a BUS less than 400 ml in 120 +/- 42 min. 23 patients had a BUS greater than 400 ml: of these, 20 patients voided within an hour and were discharged in 162 +/- 45 min. Three were catheterized after 1 h, and were discharged in 186 +/- 61 min. Mean discharge time for all patients in the accelerated pathway was 22 min shorter than the standard pathway (P = 0.002). No patients had difficulty voiding or returned to the hospital for urinary problems. None reported new urologic symptoms.     

Infantile myofibromatosis

 Infantile myofibromatosis is a mesenchymal tumor most commonly seen in infancy. The tumors have a variable appearance on CT/MR and often simulate a more aggressive neoplasm. This report describes CT/MR findings in cases of infantile myofibromatosis with pathologic correlation. Discussion into the success of imaging in suggesting the correct diagnosis is also addressed. Infantile myofibromatosis is a mesenchymal disorder of infancy characterized by the presence of tumorous nodules in the skin, subcutaneous tissue, muscle, viscera, and bone. Cases of solitary and multiple lesions have been described. We present the clinical, histologic, and radiographic findings of one case of the solitary form of infantile myofibromatosis that was recently diagnosed at our hospital.     

Intracapsular implant rupture: MR findings of incomplete shell collapse

The objective of this study was to determine the frequency and significance of the MR findings of incomplete shell collapse for detecting implant rupture in a series of surgically removed breast prostheses. MR images of 86 breast implants in 44 patients were studied retrospectively and correlated with surgical findings at explanation. MR findings included (a) complete shell collapse (linguine sign), 21 implants; (b) incomplete shell collapse (subcapsular line sign, teardrop sign, and keyhole sign), 33 implants; (c) radial folds, 31 implants; and (d) normal, 1 implant. The subcapsular line sign was seen in 26 implants, the teardrop sign was seen in 27 implants, and the keyhole sign was seen in 23 implants. At surgery, 48 implants were found to be ruptured and 38 were intact. The MR findings of ruptured implants showed signs of incomplete collapse in 52% (n = 25), linguine sign in 44% (n = 21), and radial folds in 4% (n = 2). The linguine sign perfectly predicted implant rupture, but sensitivity was low. Findings of incomplete shell collapse improved sensitivity and negative predictive values, and the subcapsular line sign produced a significant incremental increase in predictive ability. MRI signs of incomplete shell collapse were more common than the linguine sign in ruptured implants and are significant contributors to the high sensitivity and negative predictive values of MRI for evaluating implant integrity.     

Post-operative progress of dystonia patients following globus pallidus internus deep brain stimulation

In the current era of functional surgery for movement disorders, deep brain stimulation (DBS) of the globus pallidus internus (GPi) is emerging as the favoured intervention for patients with dystonia. Here we report our results in 20 patients with medically intractable dystonia treated with GPi stimulation. The series comprised 14 patients with generalized dystonia and six with spasmodic torticollis. Although comparisons were limited by differences in their respective neurological rating scales, chronic DBS clearly benefited both patient groups. Data conveying the rate of change in neurological function following intervention are also presented, demonstrating the gradual but progressive and sustained nature of improvement following stimulation of the GPi in dystonic patients.