Usefulness of multiparametric computerized tomography findings in the differential diagnosis of stroke mimics of epileptic origin: A preliminary study. / Utilidad de la tomografía computarizada cerebral multiparamétrica en el diagnóstico diferencial de patología comicial en el código ictus. Estudio preliminar.

OBJECTIVE: Thirty percent of the patients for whom code stroke is activated have stroke mimics, the most common being epilepsy. Our purpose was to evaluate the usefulness of multiparametric CT for differentiating between seizure-related symptoms and vascular events. MATERIAL AND METHODS: We conducted a retrospective observational study; data were gathered prospectively during one year. We studied multiparametric CT images of patients admitted following code stroke activation and finally diagnosed with epilepsy. RESULTS: The study included a total of 11 patients; 36% were men and mean age was 74.5 years. Three patients had right hemisphere syndrome, 4 displayed left hemisphere syndrome, and the remaining 4 had isolated aphasia. Maximum time from symptom onset to multiparametric CT study was 8.16hours. Perfusion CT results were normal in 2 patients. Nine patients showed longer or shorter times to peak (Tmax); cerebral blood volume (CBV) and cerebral blood flow (CBF) maps varied. EEG was performed a maximum of 47.6hours after symptom onset. Four patients showed findings compatible with status epilepticus, 2 displayed focal epileptiform activity, and 5 showed post-ictal slowing ipsilateral to perfusion CT abnormalities. CONCLUSION: The most sensitive parameter for differentiating between stroke and epilepsy in our series was increased time to peak in multilobar cortical locations in the absence of large-vessel occlusion and basal ganglia involvement. Multiparametric CT is a fast, readily available, and useful tool for the differential diagnosis of acute-onset neurological signs of epileptic origin in patients initially attended after code stroke activation.

Longitudinal studies of ischemic penumbra by using 18F-FDG PET and MRI techniques in permanent and transient focal cerebral ischemia in rats.

At present, the goal of stroke research is the identification of a potential recoverable tissue surrounding the ischemic core, suggested as ischemic penumbra, with the aim of applying a treatment that attenuates the growth of this area. Our purpose was to determine whether a combination of imaging techniques, including (18)F-FDG PET and MRI could identify the penumbra area. Longitudinal studies of (18)F-FDG PET and MRI were performed in rats 3 h, 24 h and 48 h after the onset of ischemia. A transient and a permanent model of focal cerebral ischemia were performed. Regions of interest were located, covering the ischemic core, the border that progresses to infarction (recruited tissue), and the border that recovers (recoverable tissue) with early reperfusion. Analyses show that permanent ischemia produces severe damage, whereas the transient ischemia model does not produce clear damage in ADC maps at the earliest time studied. The only significant differences between values for recoverable tissue, (18)F-FDG (84±2%), ADC (108±5%) and PWI (70±8%), and recruited tissue, (18)F-FDG (77±3%), ADC (109±4%) and PWI (77±4%), are shown in (18)F-FDG ratios. We also show that recoverable tissue values are different from those in non-infarcted tissue. The combination of (18)F-FDG PET, ADC and PWI MRI is useful for identification of ischemic penumbra, with (18)F-FDG PET being the most sensitive approach to its study at early times after stroke, when a clear DWI deficit is not observed.

Somatotroph tumor progression during pegvisomant therapy: a clinical and molecular study.

CONTEXT: There is concern that pegvisomant could be associated with a higher risk of tumor growth. The rate and possible determinants of this tumor growth are unknown. OBJECTIVE: The objective of the study was to investigate the clinical, immunohistological, and molecular factors conditioning tumor growth in patients taking pegvisomant. DESIGN AND SETTING: This was a cross-sectional study performed from 2004 to 2010 in four university hospitals in Spain. PATIENTS: Seventy-five acromegalic patients with active disease resistant to somatostatin analogs treated with pegvisomant were followed up for a mean of 29 ± 20 months. MAIN OUTCOME MEASURES: Magnetic resonance images before initiation of pegvisomant, at 6 months, and then yearly were examined in all patients. Immunohistological and molecular studies were performed in tumors that grew. RESULTS: A significant increase in tumor size was observed in five patients (6.7%). Absence of previous irradiation (P = 0.014) and shorter duration of prepegvisomant somatostatin analog therapy (P < 0.001) were associated with an increased risk of tumor growth. A stepwise multivariate linear regression analysis (R(2) = 0.334, P < 0.001) identified the duration of somatostatin analog therapy prior to pegvisomant (beta = -4.509, P = 0.014) as the only significant predictor of tumor growth. In those tumors that grew, GH expression and insulin receptor expression were higher (P = 0.033 in both cases) than in the control group. CONCLUSIONS: No previous radiotherapy, shorter duration of prepegvisomant somatostatin analog therapy, and higher tumor expression of GH and insulin receptor could be risk factors for tumor growth during pegvisomant therapy.

Progressive multifocal leukoencephalopathy after stem cell transplantation, unsuccessfully treated with cidofovir.

We report a patient with progressive multifocal leukoencephalopathy (PML) after autologous stem cell transplantation (SCT) for non-Hodgkin's lymphoma (NHL). This is an unusual association, and to date only seven cases have been reported. This is the first case of PML after SCT treated with cidofovir, and the fifth case treated with this drug in a patient without human immunodeficiency virus (HIV) infection. In the previous four patients treated with cidofovir the outcome was discouraging, as was the case in this patient.

Intraoral mandibular distraction osteogenesis: special attention to treatment planning.

PURPOSE: To demonstrate our experience using internal devices for unidirectional distraction osteogenesis in treating different mandibular hypoplasias (with or without maxillary deformities). An algorithmic table for diagnosis, and treatment planning is presented. PATIENTS AND METHODS: Twenty internal distraction devices were used in 16 patients with mandibular hypoplasia. Deficiency in length of the mandible was calculated on three-dimensional computed tomography scans. The device was activated by a transcutaneous pin on the fifth postoperative day. Distraction was achieved at rates of 0.5 mm/12 h. After a variable period of consolidation the devices were removed. Mean follow-up was 18 months. RESULTS: Successful distraction osteogenesis was achieved in all patients. No premature consolidation or pseudoarthrosis was observed. Improvement of facial aesthetics was produced in all cases. Final occlusion was excellent in those cases where no simultaneous maxillary deformity was present. Orthodontic treatment was applied in all cases. Results remained stable one year postoperatively. CONCLUSIONS: The occlusal results obtained in this series show that we can plan distraction as a definitive treatment in cases with isolated mandibular hypoplasia. When an additional maxillary deformity is present, mandibular distraction must be performed first if indicated, but a maxillary procedure will be necessary later.

[Bullous emphysema and neurofibromatosis]. / Enfisema bulloso y neurofibromatosis.

Clinical and image findings in a 43 year-old smoker man with advanced lung bullous emphysema and von Recklinhausen's neurofibromatosis are presented. Bullous emphysema and subpleural neurofibromas were detected in a chest computed tomographic study. The possible association or independence of these two conditions is discussed.

[Daily defined doses of parkinsonian drugs in Alcoi]. / Dosis diarias definidas de fármacos antiparkinsonianos en Alcoi.

BACKGROUND: Some authors have suggested that the use of DDD (defined daily doses) of L-dopa may be useful as an indicator of the frequency of Parkinson's disease. AIM: To determine the measurement for L-dopa and selegiline in Alcoi. MATERIAL AND METHODS: We have obtained the intakes for L-dopa and selegiline between January and December, 1990 and we have calculated the DDD/1,000 inhab/day. RESULTS: During the study period, we have found that the mean intakes were 18,000.969 mg and 53,800 mg for L-dopa and selegiline, respectively. And so, the L-dopa intake in Alcoi was 0.60 DDD/ 1,000 inh, and the selegiline intake was 0.21 DDD/1,000 inh. DISCUSSION: Comparing the other data of the literature, specially in scandinavian countries, the obtained intake for L-dopa was low, and that could indicate that the frequency of the disease in Alcoi was lower.

Schinzel-Giedion syndrome: report of two sibs.

We report on two further cases, a sister and a brother, with Schinzel-Giedion syndrome. Both presented the following manifestations: "coarse face" with midface retraction, agenesis of corpus callosum, bilateral hydronephrosis, and typical skeletal anomalies. Patient 1 had a malignant sacrococcygeal teratoma. This is the third case of malignancy in this syndrome. Patient 2 died shortly after birth.

Cardiovascular reflexes in Parkinson disease.

We have investigated the autonomic function of 95 patients with Parkinson disease (PD) by testing their cardiovascular reflexes and compared the results with those of 53 healthy volunteers. 51.1% of the patients were on antiparkinsonian therapy. The patients showed a smaller heart rate response to deep breathing with a mean exhalation-inspiration difference of 87.73 +/- 7 (p: 0.0005), a smaller heart rate response to standing with a mean 30: 15 index of 1.18 +/- 0.25 (p: 0.01), and a smaller blood pressure rise to handgrip with a mean rise of 9.53 +/- 8.76 mmHg (p: 0.005). We found a higher percentage of patients with established sympathetic lesion (41% vs 34%) or atypical patterns of global autonomic function involvement (33% vs 26%), but the percentage of subjects with parasympathetic lesion was similar in both, patients and controls (16% vs 15%). We found no significant difference on cardiovascular performance between treated and untreated patients. The patients with autonomic impairment were older than the patients with normal autonomic function. Our findings suggest that patients with PD have a true autonomic dysfunction.

Radiohumeral synostosis, femoral bowing, other skeletal anomalies and anal atresia, a variant example of Antley-Bixler syndrome?

We report a newborn with radiohumeral synostosis, femoral bowing, anal atresia, a prominent nose (pear shaped nose), slender ribs, long tapering fingers with distal camptodactyly, genital hypoplasia and a neonatal humeral fracture. Among the possible differential diagnoses a variant example of Antley-Bixler syndrome is considered to be the most likely final diagnosis.

[Fulminant meningoencephalitis due to Listeria monocytogenes]. / Meningoencefalitis fulminante por Listeria monocytogenes.

We present a case of meningoencephalitis due to Listeria monocytogenes type IV in a patient in which the diagnosis of small-cells malignant diffuse non-Hodgkin's lymphoma had been established. He was hospitalized with vague clinical manifestations and without showing any neurological focus. Hours later, he presented clinical brain stem semiology. Within a 24-hour period, Listeria monocytogenes was isolated from blood and cephalorhachidian fluid (being the first time that this microorganism had been isolated for the past 20 years in our laboratory). The patient evolved to a state of coma, with Cheyne-Stokes's respiration, abolition of pupillary reflexes, decerebration position and exitus laetalis. The affection of the central nervous system has been fully documented. However, its fulminant presentation, as in the case that we present here, is less frequent, although it has been described in the literature by other authors, like Finegold et al., with cases in which the interval between the onset of the neurological focality and death ranged from 8 to 16 hours. The antibiogram showed a wide sensibility to several groups of drugs, despite the fast evolution of the disease that prevented the modification of the initial antibiotherapy (Vancomicine and imipenem).

Cardiovascular reflexes in patients with malignant disease.

Although some authors have reported clinical features of autonomic dysfunction in patients with malignant disease only a few systematic studies have been carried out. We have investigated the autonomic function of 48 patients with carcinoma or lymphoma by testing their cardiovascular reflexes, and compared their results with those of 62 healthy volunteers. Our patients showed a smaller increase in heart rate and a greater fall in blood pressure on standing up, as well as a smaller rise in blood pressure in response to contralateral handgrip. The study group showed a higher percentage of established sympathetic lesion (54.2% vs 27.4%, p: 0.008) and of atypical pattern of global autonomic function impairment (50% vs 24.2%, p: 0.03) than the control group. Our data suggest that patients with malignant disease suffer from a definite autonomic dysfunction.

Allogeneic bone marrow transplantation versus chemotherapy in the treatment of childhood acute lymphoblastic leukemia in second complete remission.

Seventy-six patients between the ages of 2 and 17 years with acute lymphoblastic leukemia (ALL) achieved a second complete remission induced by polychemotherapy. Twenty-one had an HLA-identical donor and underwent allogeneic bone marrow transplantation (BMT) after conditioning with total body irradiation and cyclophosphamide. The remaining 55 patients lacked a suitable donor and received intensive chemotherapy as treatment. Fifteen patients were excluded from the analysis because they relapsed within 3 months after achieving a second complete remission. Three of the 21 BMT patients died of transplant-related complications and seven relapsed between 90 and 480 days after transplantation. Eleven patients are alive and disease free at 5.5-71 months with an actuarial survival of 47.1%; eight patients are on a plateau extending from 22 to 71 months. Thirty-three patients treated with chemotherapy died from relapse and seven are alive and disease free 7.5-99 months from the second remission, with an actuarial survival of 9%. The probability of survival was significantly higher in the BMT group (p less than 0.025). The probability of remaining in complete remission in the BMT group was 58.5% versus 10.9% in the chemotherapy group (p less than 0.005). Our results show that BMT is the best alternative therapy for children affected by ALL who have had a relapse in the marrow.

Effects of sulindac on myeloid precursors growth in vitro.

We have studied the effects of sulindac and its main active metabolites on human bone marrow progenitors. Granulocytic-macrophagic colony forming units (CFU-GM) techniques were used for assessment. As in vitro inhibitory action of this drug or its metabolites on CFU-GM growth could not be demonstrated. These results refute the clinically presumed toxicity of sulindac on human bone marrow progenitors at pharmacological doses.

Lack of in vitro colony formation in a patient with severe aplastic anemia after spontaneous autologous hematologic reconstitution.

A case of idiopathic severe aplastic anemia with spontaneous complete remission is described. Hematologic parameters normalized spontaneously 94 days after onset. However, the ability of the patient's bone marrow cells to form granulocytic-macrophagic colony-forming units (CFU-GM) or erythroid burst-forming units (BFU-E) was depressed until the 288th day, in spite of the normalization of blood counts. Incubation of the patient's bone marrow cells with antilymphocytic globulin prior to the culture experiment normalized the number of CFU-GM and BFU-E in correlated studies. Coculture of a target marrow with several concentrations of the patient's lymphocytes or serum resulted in a complete inhibition of CFU-GM. BFU-E growth was inhibited by the patient's lymphocytes, but not by serum, which rather showed burst-promoting activity. The inhibitory effect on target bone marrow persisted for 288 days, when it disappeared concomitantly with the restoration of spontaneous CFU-GM and BFU-E growth.