Treatment of Sinusoidal Obstruction Syndrome With Defibrotide in Pediatric Cancer Patients Following Nontransplant-associated Chemotherapy: A Case Report and Review of the Literature.

Sinusoidal obstruction syndrome (SOS), formerly veno-occlusive disease (VOD), in pediatric cancer patients often presents as a complication of hematopoietic stem cell transplantation, and less commonly secondary to nontransplant-associated chemotherapy. Therapy with defibrotide is well-described as standard care for transplant-associated SOS/VOD, but the treatment of nontransplant-associated SOS/VOD is less clear. We report a 3-year-old with relapsed Wilms tumor and recurrent SOS/VOD, with successful use of defibrotide during chemotherapy. A review of pediatric cancer patients with nontransplant-associated SOS/VOD treated with defibrotide revealed 83 patients, and 66 were in remission. This review supports early treatment with defibrotide in patients with nontransplant-associated SOS/VOD.

Telangiectasia Macularis Eruptiva Perstans of the Scalp Mimicking Angiosarcoma.

ABSTRACT: We present a case of a 74-year-old man with marked photodamage who was ultimately diagnosed with telangiectasia macularis eruptiva perstans (TMEP) of the scalp. The diagnosis was made more difficult because of the clinical and histological similarity of this case with an early angiosarcoma. TMEP is a benign and indolent rare subtype of cutaneous mastocytosis presenting clinically with red-brown telangiectatic macules, usually symmetrically distributed over the trunk and extremities. Although most cases are limited to the skin, systemic involvement can occur, and this can be a potentially life-threatening disease. Although also rare, in contrast to TMEP, cutaneous angiosarcoma is a highly malignant vascular tumor with a poor prognosis. This case highlights the importance of including TMEP on the differential diagnosis where vascular lesions of the scalp are observed.

Striatum-Mediated Deficits in Stimulus-Response Learning and Decision-Making in OCD.

Obsessive compulsive disorder (OCD) is a prevalent psychiatric disorder characterized by obsessions and compulsions. Studies investigating symptomatology and cognitive deficits in OCD frequently implicate the striatum. The aim of this study was to explore striatum-mediated cognitive deficits in patients with OCD as they complete a stimulus-response learning task previously shown to differentially rely on the dorsal (DS) and ventral striatum (VS). We hypothesized that patients with OCD will show both impaired decision-making and learning, coupled with reduced task-relevant activity in DS and VS, respectively, compared to healthy controls. We found that patients with OCD (n = 14) exhibited decision-making deficits and learned associations slower compared to healthy age-matched controls (n = 16). Along with these behavioral deficits, OCD patients had reduced task-relevant activity in DS and VS, compared to controls. This study reveals that responses in DS and VS are altered in OCD, and sheds light on the cognitive deficits and symptoms experienced by patients with OCD.

Wearable neural prostheses. Restoration of sensory-motor function by transcutaneous electrical stimulation.

In this article, we focus on the least invasive interface: transcutaneous ES (TES), i.e., the use of surface electrodes as an interface between the stimulator and sensory-motor systems. TES is delivered by a burst of short electrical charge pulses applied between pairs of electrodes positioned on the skin. Monophasic or charge-balanced biphasic (symmetric or asymmetric) stimulation pulses can be delivered. The latter ones have the advantage to provide contraction force while minimizing tissue damage.

The influence of electrode size on selectivity and comfort in transcutaneous electrical stimulation of the forearm.

Transcutaneous electrical stimulation (TES) is a technique to artificially activate motor nerves and muscles. It can be used for rehabilitation or the restoration of lost motor functions, e.g., in subjects with brain or spinal cord lesions. Apart from selectively activating motor nerves and muscles, TES activates sensory fibers and pain receptors, producing discomfort and pain. Clinicians try to minimize discomfort by optimizing stimulation parameters, electrode location, and electrode size. There are some studies that found optimal electrode sizes for certain stimulation sites (e.g., gastrocnemius), however the underlying effects why certain electrode sizes are preferred by patients is not well understood. We used a TES model consisting of a finite element (FE) model and a nerve model to assess the influence of different electrode sizes on the selectivity and the perceived comfort for various anatomies. Motor thresholds calculated using the TES model were compared with motor thresholds that were obtained from measurements performed on the forearm of ten human volunteers. Results of the TES model indicate that small electrodes (0.8 x 0.8 cm(2)) are more comfortable for thin fat layers (0.25 cm) and superficial nerves (0.1 cm) and larger electrodes (4.1 x 4.1 cm(2)) are more comfortable for thicker fat layers (2 cm) and deeper nerves (1.1 cm) at a constant recruitment.

A model for transcutaneous current stimulation: simulations and experiments.

Complex nerve models have been developed for describing the generation of action potentials in humans. Such nerve models have primarily been used to model implantable electrical stimulation systems, where the stimulation electrodes are close to the nerve (near-field). To address if these nerve models can also be used to model transcutaneous electrical stimulation (TES) (far-field), we have developed a TES model that comprises a volume conductor and different previously published non-linear nerve models. The volume conductor models the resistive and capacitive properties of electrodes, electrode-skin interface, skin, fat, muscle, and bone. The non-linear nerve models were used to conclude from the potential field within the volume conductor on nerve activation. A comparison of simulated and experimentally measured chronaxie values (a measure for the excitability of nerves) and muscle twitch forces on human volunteers allowed us to conclude that some of the published nerve models can be used in TES models. The presented TES model provides a first step to more extensive model implementations for TES in which e.g., multi-array electrode configurations can be tested.

Assessment of finger forces and wrist torques for functional grasp using new multichannel textile neuroprostheses.

New multichannel textile neuroprotheses were developed, which comprise multiple sets of transcutaneous electrode arrays and connecting wires embroidered into a fabric layer. The electrode arrays were placed on the forearm above the extrinsic finger flexors and extensors. Activation regions for selective finger flexion and wrist extension were configured by switching a subset of the array elements between cathode, anode, and off states. We present a new isometric measurement system for the assessment of finger forces and wrist torques generated using the new neuroprostheses. Finger forces (from the middle phalanxes) were recorded using five load cells mounted on a "grasp handle" that can be arbitrarily positioned in space. The hand and the grasp handle were rigidly mounted to a 6-degree of freedom load cell, and the forces and torques about the wrist were recorded. A vacuum cushion was used to comfortably fixate the forearm. The position and orientation of the forearm, wrist, fingers, and handle were recorded using a new three-dimensional position measurement system (accuracy <+/-1 mm). The measurement system was integrated into the real-time multichannel transcutaneous electrode environment, which is able to control the spatiotemporal position of multiple activation regions. Using the combined system and textile neuroprosthesis, we were able to optimize the activation regions to produce selective finger and wrist articulation, enabling improved functional grasp.

New multi-channel transcutaneous electrical stimulation technology for rehabilitation.

Transcutaneous (surface) electrical stimulation (TES) is a widely applied technique for muscle atrophy treatment, muscle force training, endurance training, pain treatment, functional movement therapy, and the restoration of motor functions. We present a new TES technology based on a multi-channel stimulation approach, which allows us to perform real-time spatial and temporal variations of the electrical current density on the skin surface and in deeper tissue layers. This new approach can generate a better muscle selectivity and improved muscle activation patterns compared to state of art TES systems, which operate with predetermined electrode positions. In simulations using a finite element model (FEM) of the distal arm we could show that the nerve activation in the muscle layer is not significantly influenced by the structure of the multi-channel electrode, if the gap between elements is less than 2 mm. Experiments in healthy volunteers allowed us to measure the selectivity of single finger activations. We could also show in stroke subjects that this novel multi-channel approach was able to generate selective finger and wrist extension movements that were strong enough to overcome flexion hyperactivity. For future applications in rehabilitation a full integration of the stimulation hardware into a garment sleeve would be helpful. Once fully integrated, this new technology has a high potential to increase the ease of use, stimulation and wear comfort. It is able to improve muscle selectivity compared to state of the art TES systems, and allows the implementation of a variety of new applications for the medical and consumer market.