Peripheral nerves are pathologically small in cerebellar ataxia neuropathy vestibular areflexia syndrome: a controlled ultrasound study.

BACKGROUND AND PURPOSE: Sensory neuronopathy is a cardinal feature of cerebellar ataxia neuropathy vestibular areflexia syndrome (CANVAS). Having observed that two patients with CANVAS had small median and ulnar nerves on ultrasound, we set out to examine this finding systematically in a cohort of patients with CANVAS, and compare them with both healthy controls and a cohort of patients with axonal neuropathy. We have previously reported preliminary findings in seven of these patients with CANVAS and seven healthy controls. METHODS: We compared the ultrasound cross-sectional area of median, ulnar, sural and tibial nerves of 14 patients with CANVAS with 14 healthy controls and 14 age- and gender-matched patients with acquired primarily axonal neuropathy. We also compared the individual nerve cross-sectional areas of patients with CANVAS and neuropathy with the reference values of our laboratory control population. RESULTS: The nerve cross-sectional area of patients with CANVAS was smaller than that of both the healthy controls and the neuropathy controls, with highly significant differences at most sites (P < 0.001). Conversely, the nerve cross-sectional areas in the upper limb were larger in neuropathy controls than healthy controls (P < 0.05). On individual analysis, the ultrasound abnormality was sufficiently characteristic to be detected in all but one patient with CANVAS. DISCUSSION: Small nerves in CANVAS probably reflect nerve thinning from loss of axons due to ganglion cell loss. This is distinct from the ultrasound findings in axonal neuropathy, in which nerve size was either normal or enlarged. Our findings indicate a diagnostic role for ultrasound in CANVAS sensory neuronopathy and in differentiating neuronopathy from neuropathy.

Deep brain stimulation in ireland for Parkinson's disease and essential tremor.

Deep brain stimulation (DBS) is highly effective neurosurgery for idiopathic Parkinson's disease (IPD), essential tremor (ET) and primary dystonia. DBS involves stereotactic surgical implantation of a battery-operated stimulator into deep brain nuclei. Irish patients are referred abroad for DBS and have to travel repeatedly for pre and post-operative care resulting in stress, anxiety and hardship. Safe pre and post-operative care of these complex, ageing patients is compromised by the absence of a DBS service in Ireland. Moreover, both DBS surgery and the subsequent post-operative care abroad incurs substantial cost to the state. The Dublin Neurological Institute at the Mater Misericordiae University Hospital (DNI) is a non-profit institute for the care of patients with neurological diseases. The DNI developed, in collaboration with the Mater Private Hospital (MPH) and the Walton Centre, Liverpool, a DBS programme in 2008/2009. We performed DBS at the Mater Campus on three carefully selected patients from a cohort of movement disorder patients attending the DNI and continue to provide pre-operative assessment and post operative care for patients following DBS in Ireland and abroad.

Alexia without agraphia.

Alexia without agraphia (also called pure alexia or word blindness) was the first of the disconnection syndromes (syndromes caused by disconnection of the right from the left cerebral hemisphere through interruption of the communication pathways between them) to be described. Déjerine in 1892 reported a patient who developed this syndrome after an infarct of the left occipital lobe and splenium of the corpus callosum. We describe a patient who developed alexia without agraphia due to an embolic left occipital lobe infarct extending to the posterior commissure and splenium of the corpus callosum.

Danazol: an effective option in acquired amegakaryocytic thrombocytopaenic purpura.

Acquired amegakaryocytic thrombocytopaenic purpura (AATP) is a rare haematological condition characterised by isolated thrombocytopaenia with normal other cell lines. It is often initially misdiagnosed as immune thrombocytopaenic purpura but has characteristic bone marrow findings of reduced megakaryocyte numbers. The optimal treatment of AATP is not clearly defined but revolves around immunosuppressive therapies. We report a case of successful treatment of AATP with danazol, an antioestrogenic medication. We also review the aetiologies and pathogenesis of the disorder and suggest that danazol should be considered as an effective alternative to potent immunosuppression in AATP.

Building a neighborhood network: interorganizational collaboration to prevent child abuse and neglect.

Policymakers have identified the community-based human services network as a promising model for restructuring services in neighborhoods affected by poverty, but researchers have found this approach complex and difficult to implement. An increase in networks and partnerships among service providers suggests that organizations are learning to work together, yet little is known about the processes of their collaboration. This article reports findings from an organizational analysis of one interorganizational collaboration in which executive directors and frontline practitioners from seven agencies worked for five years to create, implement, and institutionalize a community-based service network of informal and formal family support programs to help prevent child abuse and neglect. This article presents a theoretical framework for understanding community-based networks and interorganizational collaboration and describes and analyzes how collaboration was used as a method to build a new service network, the characteristics of the network, and factors that facilitated the collaborative processes.

Nonprofit organizations and innovation: a model of neighborhood-based collaboration to prevent child maltreatment.

Public policymakers increasingly are contracting with nonprofit organizations (NPOs) for innovations in the creation of new service systems in low-income communities. Interorganizational collaboration and cooperation are essential to such innovation. Neighborhood-based institutional arrangements require social work practitioners to work across multiple systems simultaneously--skills that most are not trained to possess. This article develops a theoretical and conceptual framework for neighborhood-based collaboration by NPOs; analyzes the main concepts of innovation in the design and implementation of a collaboration to prevent child maltreatment in an undervalued neighborhood; and draws implications for social policy, social work practice, and social work research.