Mildly Impaired Foot Control in Long-Term Treated Patients with Wilson’s Disease

Abnormal gait is a common initial symptom of Wilson’s disease, which responds well to therapy, but has not been analyzed in detail so far. In a pilot study, a mild gait disturbance could be detected in long-term treated Wilson patients. The question still is what the underlying functional deficit of this gait disturbance is and how this functional deficit correlates with further clinical and laboratory findings. In 30 long-term treated Wilson patients, the vertical component of foot ground reaction forces (GRF-curves) was analyzed during free walking without aid at the preferred gait speed over a distance of 40 m. An Infotronic® gait analysis system, consisting of soft tissue shoes with solid, but flexible plates containing eight force transducers, was used to record the pressure of the feet on the floor. Parameters of the GRF-curves were correlated with clinical scores as well as laboratory findings. The results of Wilson patients were compared to those of an age- and sex-matched control group. In 24 out of 30 Wilson patients and all controls, two peaks could be distinguished: the first “heel-on” and the second “push-off” peak. The heights of these peaks above the midstance valley were significantly reduced in the patients (p < 0.05). The time differences between peaks 1 or 2 and midstance valley were significantly negatively correlated with the total impairment score (p < 0.05). Gait speed was significantly correlated with the height of the “push-off” peak above the midstance valley (p < 0.045). The GRF-curves of free walking, long-term treated patients with Wilson’s disease showed a reduced “push-off” peak as an underlying deficit to push the center of mass of the body to the contralateral side with the forefoot, explaining the reduction in gait speed during walking.

Finger abduction as a novel function of the extensor digitorum brevis manus muscle

A 25-year-old female presented with a chronic scapho-lunate ligament injury with development of carpal instability requiring reconstruction. During a standard dorsal longitudinal mid-line approach to the carpus, an extensor digitorum brevis manus (EDBM) muscle was found taking its origin from the dorsal wrist capsule overlying the lunate with innervation from the posterior interosseous nerve (PIN). Electrical stimulation of the muscle belly demonstrated abduction of the middle finger. The EDBM is a rare anatomical variant of the extensor compartment of the wrist and may be encountered during surgical approaches. Where possible these variant muscles should be carefully dissected off underlying structures, preserved and repaired at the conclusion of a procedure to ensure no perceived functional deficit to the patient. We present a case of a previously undescribed EDBM muscle function of pure finger abduction with no extension and a surgical technique of preserving its origin. We propose that the middle finger variant of the EDBM should be re-named the extensor digitorum brevis medius to reflect our findings.

Brachial Plexus Injuries – Review of the Anatomy and the Treatment Options

Brachial plexus injuries are still challenging for every surgeon taking part in treating patients with BPI. Injuries of the brachial plexus can be divided into injuries of the upper trunk, extended upper trunk, injuries of the lower trunk and swinging hand where all of the roots are involved in this type of the injury. Brachial plexus can be divided in five anatomical sections from its roots to its terminal branches: roots, trunks, division, cords and terminal branches. Brachial plexus ends up as five terminal branches, responsible for upper limb innervation, musculocutaneous, median nerve, axillary nerve, radial and ulnar nerve. According to the findings from the preoperative investigation combined with clinically found functional deficit, the type of BPI will be confirmed and that is going to determine which surgical procedure, from variety of them (neurolysis, nerve graft, neurotization, arthrodesis, tendon transfer, free muscle transfer, bionic reconstruction) is appropriate for treating the patient.

Compartment syndrome of the hand following iatrogenic intra-arterial administration of epinephrine during cardiopulmonary resuscitation

A 26-year-old woman developed acute compartment syndrome (ACS) of her right hand secondary to reperfusion syndrome. She suffered an out-of-hospital cardiac arrest following a pregabalin overdose. Attending paramedics mistakenly gave intra-arterial epinephrine into her right brachial artery. On resolution of her brachial artery spasm, she developed a reperfusion injury to her right hand and subsequently ACS. A four-incision fasciotomy with carpal tunnel decompression was performed and was successful in reversing focal ischaemia and an irreversible functional deficit. This case demonstrates an unusual case of hand ACS secondary to temporary limb ischaemia and reperfusion syndrome following iatrogenic intra-arterial epinephrine administration. We also summarise the current available literature on ACS of the hand including the aetiology, treatment and use of an intracompartmental monitor.

Nefroneural syndrome as a result of poisoning by diethylene glycol

Introduction: Diethylene glycol is a clear, hygroscopic, odorless and toxic liquid for humans. It is widely available in the industry, being widely used in the production of antifreeze, lubricants, cosmetics and plasticizers. Poisoning by ingesting this substance leads patients to an early neurological syndrome consisting of drunkenness, ataxia and, if severe, seizures and coma. Objectives: To review the central aspects of diethylene glycol intoxication, its toxic dynamics and the development of nephroneural syndrome. Methods: A literature review compiled from searches for articles in the PubMed and Medline databases was performed using the descriptors Dietilenoglicol; Ácido 2-hidroxietoxiacético; Toxicidade Renal; Álcoois tóxicos. Results: After ingestion, diethylene glycol is rapidly absorbed and distributed in the body. Metabolism occurs in the liver and the excretion of both the substance and its metabolite 2-hydroxyethoxyacetic acid (HEAA) is renal. HEAA is primarily responsible for kidney and neurological damage, which result in severe nephroneural syndrome, initially characterized by gastrointestinal changes, such as nausea, vomiting and abdominal pain, followed by metabolic acidosis and emerging kidney injury. After 72 hours of intoxication, damage to the optic nerve, functional deficit of cranial nerves, tetraparesis and peripheral neuropathy can occur, which can lead the patient to death or permanent disability. HEAA causes damage to renal and nerve cells to varying degrees depending on the amount of substance ingested or the susceptibility of the intoxicated patient. Conclusion: Early diagnosis and proper patient management, in addition to good industry practices, are essential for the eradication of this intoxication.

A novel nerve transection and repair method in mice: histomorphometric analysis of nerves, blood vessels, and muscles with functional recovery

Peripheral nerve transection is associated with permanent functional deficit even after advanced microsurgical repair. While it is difficult to investigate the reasons of poor functional outcomes of microsurgical repairs in humans, we developed a novel pre-clinical nerve transection method that allows reliable evaluation of nerve regeneration, neural angiogenesis, muscle atrophy, and functional recovery. Adult male C57BL/6 mice were randomly assigned to four different types of sciatic nerve transection: Simple Transection (ST), Simple Transection & Glue (TG), Stepwise Transection and Sutures (SU), and Stepwise Transection and Glue (STG). Mice were followed for 28 days for sciatic function index (SFI), and sciatic nerves and hind limb muscles were harvested for histomorphological and cellular analyses. Immunohistochemistry revealed more directional nerve fiber growth in SU and STG groups compared with ST and TG groups. Compared to ST and TG groups, optimal neural vessel density and branching index in SU and STG groups were associated with significantly decreased muscle atrophy, increased myofiber diameter, and improved SFI. In conclusion, our novel STG method represents an easily reproducible and reliable model with close resemblance to the pathophysiological characteristics of SU model, and this can be easily reproduced by any lab.