Progressive multifocal leukoencephalopathy in a lung transplant recipient.

Progressive multifocal leukoencephalopathy (PML) is a sub-acute, demyelinating disease of the brain caused by a human polyomavirus. We describe a patient with the onset of PML 7 months after lung transplantation. The patient was treated with immunosuppressive modulation and cidofovir, a new anti-viral therapy for PML, with stabilization of the symptoms. We also review the 4 additional reports in the literature of PML after heart and lung transplantation. Progressive multifocal leukoencephalopathy may become more prevalent as the population of heart and lung transplantation recipients increases.     

Cryopreserved cadaveric allografts for treatment of unexcised partial thickness flame burns: clinical experience with 12 patients

Partial thickness burns (PTB) usually heal within 3 weeks. Prevention of infection and desiccation of the wounds are crucial for optimal healing. Early tangential excision of the burn eschar and allografting prevent deepening of the burns, and are therefore advocated for treatment with the best functional and aesthetic results. For superficial partial thickness burns (SPTB) conservative use of topical antimicrobial agents with frequent dressing changes are implemented. We compared the conservarive treatment for PTBs and SPTBs to grafting cryopreserved cadaveric allografts with no prior excision.

Twelve patients with flame PTB areas were allografted after mechanical debridement without excision of the burn wounds. The allografts were cadaveric skin cryopreserved by programmed freezing and stored at −180°C for 30–48 months. Matching burns for depth and area were treated with silver sulfadiazine (SSD) one to two times daily until healing or debridement and grafting were required.

It was found that 80 per cent of the cryopreserved allografts adhered well and 76 per cent of the treated areas healed within 21 days, whereas only 40 per cent of the SSD-treated burns healed within 21 days.

Partial thickness burns can be treated successfully with viable human allografts (cryopreserved cadaveric skin) with no prior surgical excision. The burn wounds heal well within 3 weeks. For deep partial thickness burns (DPTB) treatment with allografts has no advantage if they have not been previously excised.     

Focal dystonia as the presenting sign in Creutzfeldt-Jakob disease.

A variety of movement disorders may occur during the course of prion disease. We describe a unique patient who had focal upper limb dystonia as the presenting symptom of familial codon 200 mutation-positive Creutzfeldt-Jakob disease.     

Bent knees and tiptoeing: late manifestations of end-stage Parkinson's disease.

We describe a unique gait phenomenon of bent knees in 9 patients with idiopathic Parkinson disease (mean age, 73.1 +/- 11.1 years), 3 of whom also manifested tiptoeing. The bent-knee posture appeared only during ambulation; in the recumbent position, full or nearly full extension was possible in all patients. The abnormality emerged after long-standing disease (6-23 years from onset) and failed to respond to dopaminergic treatment. Most of the patients also had bent spine (camptocormia). The pathogenesis of these phenomena are unknown, but they might represent a rare type of dystonia.     

Analysis of gene expression in MOG-induced experimental autoimmune encephalomyelitis after treatment with a novel brain-penetrating antioxidant

Accumulating data from experimental studies indicate that oxidative stress has a major role in the pathogenesis of multiple sclerosis (MS). It has been suggested that local production of reactive oxygen species, probably by macrophages, mediates axonal damage in both MS patients and the mouse model experimental autoimmune encephalomyelitis (EAE). We have shown previously that our novel brain-penetrating antioxidant, N-acetylcysteine amide (AD4), reduces the clinical and pathological symptoms, including inflammation and axonal damage in myelin oligodendrocyte glycoprotein (MOG)-induced chronic EAE in mice. The aim of this study was to examine the molecular mechanism by which AD4 exerts protection in MOG-induced EAE mice. Therefore, we analyzed gene-expression profile in the spinal cords of MOG-induced chronic EAE mice and compared them with MOG-induced mice treated with AD4, using a cDNA microarray. We found that MOG treatment up-regulated genes encoding growth factors, cytokines, death receptors, proteases, and myelin structure proteins, whereas MOG- and AD4-treated mice demonstrated gene expression profiles similar to that seen in na?ve healthy mice. In conclusion, our study shows that chronic AD4 administration suppresses the induction of various pathological pathways that play a role in EAE and probably in MS.     

HTL V-I from Iranian Mashhadi Jews in Israel is phylogenetically related to that of Japan,India, and South America rather than to that of Africa and Melanesia

A new endemic focus of human T-lymphotropic virus type I (HTL V-I) was recently reported among Mashhadi Jews, a group of immigrants from northeastern Iran to Israel. We extracted DNAs from fresh peripheral blood mononuclear cells (PBMCs) and/or gargle mouthwash from 10 HTL V-I carriers, who consisted of members of one family, and HTL V-I-associated myelopathy (HAM) and adult T-cell leukemia (ATL) patients. Long terminal repeat (LTR) regions of proviral DNAs were sequenced and analyzed phylogenetically. In a phylogenetic tree, all the Mashhadi HTL V-I isolates belonged to subtype A, one of the three subtypes of the cosmopolitan type of HTL V-I, and made a tight cluster distinct from the other isolates of subtype A from Japan, India, the Caribbean Basin, and South America. Although a few nucleotide substitutions were observed among the clones sequenced, no characteristic sequence variation was found in different disease manifestations, even in one family or different sources of DNA preparation.     

Using bone's adaptation ability to lower the incidence of stress fractures

In three prospective epidemiologic studies of the effect of pre-military-induction sport activities on the incidence of lower extremity stress fractures during infantry basic training, recruits who played ball sports (principally basketball) regularly for at least 2 years before basic training had a significantly lower incidence of stress fractures (13.2%, 16.7%, and 3.6% in the three studies, respectively) than recruits who did not play ball sports (28.9%, 27%, and 18.8%, respectively). Preinduction running was not related to the incidence of stress fracture. To assess the tibial strain environment during these sport activities, we made in vivo strain measurements on three male volunteers from the research team. Peak tibial compression and tension strain and strain rates during basketball reached levels 2 to 5.5 times higher than during walking and about 10% to 50% higher than during running. The high bone strain and strain rates that occurred in recruits while playing basketball in the years before military induction may have increased their bone stiffness, according to Wolff's Law. The stiffer bone could tolerate higher stresses better, resulting in lower strains for a given activity and a lower incidence of stress fractures during basic training.     

Safety and efficacy of a 6 French perclose arterial suturing device following percutaneous coronary interventions: a pilot evaluation

BACKGROUND: Arterial access site management after percutaneous coronary intervention (PCI) is a matter of increasing importance in this era of potent antiplatelet pharmacotherapy. We evaluated the safety and efficacy of a 6 French (Fr) Perclose suturing device in achieving rapid hemostasis of the access site after PCI and thus improving patient comfort. METHODS: The 6 Fr Perclose (Prostar) device consists of a suture-based closure device delivered via introducer sheath designed for suturing of the arteriotomy puncture site. Over a 3-month period, the device was used in 48 consecutive PCI treated patients (age, 62 13 years; 70% male; 44% post myocardial infarction) and in-hospital groin complication rate was compared to 48 consecutive patients (age, 64 12 years; 64% male; 33% post myocardial infarction) who had manual compression hemostasis. RESULTS: Antiplatelet glycoprotein IIb/IIIa antagonists were used more frequently during and following the procedure in 58% of Perclose-treated patients versus 42% of the manual compression group (p = 0.019). Leg immobilization duration was 3 4 hours in all patients sutured by the device; in patients with manual compression, the sheath was removed at an average of 4.8 2.5 hours after termination of the PCI and an additional 6 hours of leg immobilization were subsequently required. No difference in overall major complication rate was found between groups (6.2% in suture-mediated patients versus 9.3% in manual compression group; p = 0.60). In 3 patients (6.2%) treated using the device, adjunctive manual compression was required in addition to groin suturing due to technical failure or residual oozing from the arteriotomy site. CONCLUSION: The 6 Fr Perclose device can be safely used to achieve rapid hemostasis and the device may hasten bed mobilization of PCI-treated patients despite frequent use of potent antiplatelet pharmacotherapy during coronary interventions.