Startle myoclonus induced by Lyme neuroborreliosis: a case report.

INTRODUCTION: The normal startle response is a form of physiological myoclonus. Its anatomic origin is probably the brain stem. Pathologic startles are defined as reproducible exaggerated startle responses to trivial and not surprising stimuli. Symptomatic forms of an exaggerated startle response can be due to a variety of brain stem disorders. We have, however, found scant data about an exaggerated startle reflex induced by Lyme neuroborreliosis. We therefore report the case of a patient with this unusual presentation. CASE PRESENTATION: A 69-year old Caucasian man presented with a two-week history of a pronounced startle myoclonus, as well as a four-week history of double vision, gait disturbance and severe lancinating pain in his upper thoracic region. Neurological examination showed an excessive startle reaction of his upper trunk evoked by visual and tactile stimulation, a positive sign of Lhermitte, mild right-sided palsy of his sixth and seventh cranial nerve, moderate dysarthria, very brisk deep tendon reflexes, pallhypesthesia of his legs, and an atactic gait disturbance. A diagnosis of a Lyme neuroborreliosis was confirmed by cerebrospinal fluid examination. Under intravenous treatment with ceftriaxone, our patient improved considerably with complete remission in a follow-up at two months. CONCLUSIONS: This case illustrates the chameleon role that neuroborreliosis likes to play: although the wide spectrum of different symptoms that neuroborreliosis can present with has been described, to the best of our knowledge this is the first case report about a symptomatic form of a pathologic startle response as the predominating sign of Lyme neuroborreliosis.

Implementation and efficacy of selective sonographic screening for carotid disease before cardiac surgery.

BACKGROUND: Preoperative carotid sonography with consecutive preventive strategies might reduce stroke risk during cardiac surgery. Since routine sonography in all patients may be unfeasible, an approach to examine preselected patients was investigated. METHODS: A prognostic model predicting carotid disease was developed using the clinical data of 1,768 routinely examined patients. It recommended 1,018 of 4,814 patients of a following collective for selective sonography. Patients recommended for preoperative sonography were compared to those selected in clinical practice. RESULTS: Besides the evaluated predictor variables, a history of syncope/cardiogenic shock and of pulmonary disease was associated with patient selection for sonography in clinical practice, even though both variables were not associated with severe carotid disease. In patients who underwent sonography, although this was not recommended by the prognostic model, severe carotid disease was estimated lower than what was actually detected, suggesting a change in relative relevance of predicting variables along with the change in frequencies of patients' cardiovascular characteristics. CONCLUSION: Prognostic models for selective screening before cardiac surgery may require reevaluation over time, especially when baseline characteristics used for prediction have changed. Criteria used in clinical practice to select patients for screening may differ from those recommended by investigational studies.

Impaired cerebral autoregulation distal to carotid stenosis/occlusion is associated with increased risk of stroke at cardiac surgery with cardiopulmonary bypass.

OBJECTIVES: Severe carotid stenosis and occlusion are associated with an increased risk of stroke during and after cardiac surgery with cardiopulmonary bypass. Relevance of an impaired cerebral autoregulation caused by stenosis/occlusion is unknown. METHODS: We prospectively assessed the incidence of stroke in relation to severity of carotid disease and corresponding autoregulatory reserve in 2797 patients who had coronary artery bypass graft and/or valve surgery with cardiopulmonary bypass. Patients underwent preoperative carotid sonography and, in case of severe extracranial disease, transcranial Doppler sonography with carbon dioxide stimulation to assess cerebrovascular reserve capacity. RESULTS: Sixty-seven (2.4%) patients had an ischemic stroke, which was fatal in 5. Anterior hemispheric stroke occurred in 42 (1.9%) patients with no/low-grade stenosis, 6 (1.8%) with medium-grade stenosis, 1 (0.6%) with high-grade stenosis/occlusion and normal autoregulation, and 3 (27.3%) with high-grade stenosis/occlusion and exhausted autoregulatory reserve. Increased risk was observed in patients with high-grade stenosis/occlusion and exhausted autoregulatory reserve also after adjustment for potential confounders (adjusted odds ratio [OR] 28.3, 95% confidence interval [CI] 5.8-139.1). Stroke risk was not increased in patients with stenosis/occlusion and normal autoregulation (1.5%, adjusted OR 0.6, 95% CI 0.2-1.6). CONCLUSIONS: Cerebrovascular reserve capacity evaluated by preoperative transcranial Doppler carbon dioxide testing is a major determinant of stroke risk in patients with carotid artery stenosis/occlusion undergoing cardiac surgery with cardiopulmonary bypass. Its assessment facilitates identification of patients with an excess perioperative stroke risk.

Genetic analysis of de novo CD5+ diffuse large B-cell lymphomas suggests an origin from a somatically mutated CD5+ progenitor B cell.

CD5(+) diffuse large B-cell lymphomas (DLBLs) have recently been described as a particular subgroup of DLBLs. Classical banding and interphase cytogenetic analyses targeting ATM, TP53, and P16(INK4a) genes and the D13S25 locus from 13 CD5(+) DLBLs were compared with 55 CD5(-) DLBLs. Additionally, analysis of somatic mutations of the immunoglobulin heavy chain variable region (IgVH) genes were performed in CD5(+) DLBLs. CD5(+) DLBLs were somatically mutated (7 of 8 cases) and were negative for t(11;14)(q13;q32) and t(14;18)(q32;q21), whereas t(3;14)(q27;q32) was found in only one tumor. Trisomy 3 and gains on chromosomes 16/16p and 18/18q were significantly overrepresented in CD5(+) DLBLs. No ATM deletions were detected. The prevalence of deletions at the D13S25 locus was significantly higher in CD5(+) DLBLs (4 of 12 [33%]) compared with CD5(-) DLBLs (4 of 42 [10%]), as were p16(INK4a) deletions (33% versus 8%). On the basis of these findings, CD5(+) DLBLs are likely to arise from the same progenitor cell as the mutated variant of CD5(+) lymphocytic lymphoma/B-cell chronic lymphocytic leukemia (B-CLL).