The NF-κB1/p50 Subunit Influences the Notch/IL-6-Driven Expansion of Myeloid-Derived Suppressor Cells in Murine T-Cell Acute Lymphoblastic Leukemia.

T-cell acute lymphoblastic leukemia is an aggressive neoplasia due to hyper-proliferation of lymphoid progenitors and lacking a definitive cure to date. Notch-activating mutations are the most common in driving disease onset and progression, often in combination with sustained activity of NF-κB. Myeloid-derived suppressor cells represent a mixed population of immature progenitors exerting suppression of anti-cancer immune responses in the tumor microenvironment of many malignancies.

Botulinum Toxin Type A for the Treatment of Neuropathic Pain in Neuro-Rehabilitation.

Pain is a natural protective mechanism and has a warning function signaling imminent or actual tissue damage. Neuropathic pain (NP) results from a dysfunction and derangement in the transmission and signal processing along the nervous system and it is a recognized disease in itself. The prevalence of NP is estimated to be between 6.

Efficacy of botulinum toxin type B for the treatment of primary palmar hyperhidrosis: a prospective, open, single-blind, multi-centre study.

Primary palmar hyperhidrosis is a distressing and disabling condition that can produce social, psychological and occupational problems. Although the use of botulinum toxin type A (BoNT-A) has been reported as an efficacious and safe intervention to improve palmar hyperhidrosis, only one study concerned botulinum toxin type B (BoNT-B) in this disorder. The aim of study was to evaluate the efficacy and safety of BoNT-B in treating primary palmar hyperhidrosis.

High doses of a new botulinum toxin type A (NT-201) in adult patients with severe spasticity following brain injury and cerebral palsy.

Background: Spasticity can be a severe disabling disorder requiring high-dose injections of botulinum toxin type A (BoNT-A). Efficacy and safety of high BoNT-A doses in reducing multi-level spasticity of subjects with brain injury and cerebral palsy were investigated. Pain and functional outcome were also assessed.

Efficacy and safety of higher doses of botulinum toxin type A NT 201 free from complexing proteins in the upper and lower limb spasticity after stroke.

Botulinum toxin type A (BTX-A) represents the gold standard therapy for focal spasticity after stroke, with low prevalence of complications, reversibility, and efficacy in reducing spastic hypertonia. Current guidelines suggest the employment of a dosage up to 600 units (U) of BTX-A to treat spasticity after stroke, to avoid important adverse effects and the development of antibodies against the neurotoxin. In recent years, NT 201, a new BTX-A free of complexing proteins, has been used for treating several movement disorders, showing safety and efficacy in upper limb spasticity.

Botulinum toxin use in neuro-rehabilitation to treat obstetrical plexus palsy and sialorrhea following neurological diseases: a review.

In neuro-rehabilitation, botulinum toxin (BTX) as adjunct to other interventions can result in a useful therapeutic tool treating disabled people. Other than spasticity, numerous motor and non motor disorders can complicate clinical course and hamper rehabilitative process of neurological impaired patients. A review of BTX use in treating muscular imbalance of children with obstetrical brachial plexus palsy and in reducing sialorrhea following neurological diseases including amyotrophic lateral sclerosis (ASL), Parkinson disease and cerebral palsy (CP) is provided.

Botulinum toxin type B for sialorrhoea in children with cerebral palsy: a randomized trial comparing three doses.

Aim: The aim of the present study was to evaluate the efficacy and safety of three doses of botulinum toxin type B (BoNT-B) in reducing persistent sialorrhoea in children with cerebral palsy (CP).

Method: Children with CP and refractory sialorrhoea were randomized to one of four groups: a control group and three experimental groups receiving a low (1500 mouse units [MU]), medium (3000 MU), or high (5000 MU) dose of BoNT-B respectively, into bilateral salivary glands. Drooling was measured using the Thomas-Stonell rating scale, and the weight and the number of bibs used per day were counted in all children at baseline, 4, and 12 weeks after BoNT-B injection.

Long-term functional outcome and health status of patients with critical illness polyneuromyopathy.

Objective: The aim of this study was to investigate the long-term functional outcome and health status of patients with critical illness polyneuromyopathy (CIPNM).

Method And Subjects: One hundred and twenty-four consecutive survival intensive care unit patients admitted to a neuro-rehabilitation Unit from January 2003 to December 2007 were identified. Patients with proven CIPNM by the electromyography were prospectively followed.

Botulinum toxin type A in the healing of a chronic buttock ulcer in a patient with spastic paraplegia after spinal cord injury.

Background: Pressure sores are a significant secondary complication in spinal cord injured patients. A new indication for botulinum toxin type A is described here, in treatment of recurrent muscular spasms that had hampered the healing of a chronic buttock ulcer in a subject with severe spastic paraplegia.

Case Report: A 27-year-old man with spastic paraplegia following traumatic spinal cord lesion at the thoracic level had developed recurrent severe muscular spasms, particularly involving the buttock region, with an Ashworth scale score of 3-4 and a Spasm Frequency Scale of grade 4.

Functional outcome and health status of injured patients with peripheral nerve lesions.

Background: Peripheral nerve lesions (PNLs) can complicate the clinical course and outcome of multiply injured patients. Since this often impedes recovery, it can be a significant burden for both patients and clinicians. The objective of the present study was to investigate the long-term outcome and health status of patients with PNL.

Botulinum toxin type A in the healing of ulcer following oro-mandibular dyskinesia in a patient in a vegetative state.

Objective: Use of botulinum toxin is expanding as clinical studies demonstrate new potential therapeutic applications. In rehabilitation, botulinum toxin is predominantly used as adjunct therapy for the treatment of spasticity, but it may prove useful for other atypical clinical situations.

Case History: A 73-year-old man had a severe sub-arachnoid haemorrhage following the rupture of a giant aneurism of the middle left cerebral artery.

Botulinum toxin type-A and plaster cast treatment in children with upper brachial plexus palsy.

Background And Purpose: Electrical stimulation, physical therapy and occupational therapy remain the main treatment for children with upper brachial plexus palsy (UBPP), when surgery has been excluded. A pilot study was undertaken to investigate whether botulinum toxin type A (BoNT-A) and plaster casting, as adjunct to the physical therapy, decreased muscle contracture and improved the position and function of the impaired arm.

Method: Twenty-two children (mean age 5.

Risk factors for spread of primary adult onset blepharospasm: a multicentre investigation of the Italian movement disorders study group.

Objectives: Little is known about factors influencing the spread of blepharospasm to other body parts. An investigation was carried out to deterrmine whether demographic features (sex, age at blepharospasm onset), putative risk, or protective factors for blepharospasm (family history of dystonia or tremor, previous head or face trauma with loss of consciousness, ocular diseases, and cigarette smoking), age related diseases (diabetes, hypertension), edentulousness, and neck or trunk trauma preceding the onset of blepharospasm could distinguish patients with blepharospasm who had spread of dystonia from those who did not.

Methods: 159 outpatients presenting initially with blepharospasm were selected in 16 Italian Institutions.

Intrasphincteric injection of botulinum toxin is effective in long-term treatment of esophageal achalasia.

We investigated the long-term efficacy and safety of intrasphincteric injections of botulinum toxin (100 U) in 57 patients with esophageal achalasia. One month after treatment, 50 patients had improved (88%); both symptom score and LES pressure were significantly reduced (P < 0.001).

[The use of type A botulin toxin in the treatment of detrusor-sphincter dyssynergia].

The detrusor-sphincter dyssynergia (DSD) is a common and significant problem for patients with spinal cord lesions. If not treated, DSD can lead to severe and potentially lethal complications (urinary tract infections and renal damage). BTX inhibits acetylcholine release at the neuromuscular junction, producing muscle chemical denervation in the site of injection.

Controlled trial of botulinum toxin injection versus placebo and pneumatic dilation in achalasia.

Background & Aims: Intrasphincteric injection of botulinum toxin has been suggested as an alternative treatment modality in esophageal achalasia. A controlled trial comparing botulinum toxin, placebo, and pneumatic dilation is reported.

Methods: Sixteen patients received random intrasphincteric injections of either botulinum toxin or saline.

Attenuation of the early anterior negativity of median nerve somatosensory evoked potential in the MPTP-treated monkey.

Median nerve somatosensory evoked potentials (SEP) were recorded in 7 Cynomolgus monkeys, before and after the administration of N-Methyl 1,4 Phenyl 1,2,3,6 tetrahydropiridine (MPTP), a neurotoxin which induces a parkinsonian syndrome in primates. Following MPTP administration, the amplitude of the negative component recorded at 15 ms over the frontal derivations (N15) decreased by 70% or more. This amplitude reduction was not modified by administration of dopamine precursors.

Maps of somatosensory evoked potentials (SEPs) to mechanical (tapping) stimuli: comparison with P14, N20, P22, N30 of electrically elicited SEPs.

Bit mapped color imaging of SEPs was recorded from 19 derivations in 11 healthy volunteers after electrical stimulation of the median nerve at the wrist, index finger digital nerve stimulation, and mechanical stimulation of the index fingertips by an electromechanically driven vibrating thin metallic plate. The latencies of SEP components increased for the various stimulation modalities, being shortest after median nerve stimulation at the wrist and longest after mechanical stimulation of the index fingertips. The scalp distribution of SEPs to mechanical stimuli was, however, the same as other SEPs, independently of the stimulation employed, and components corresponding to N20 and P22 were recorded only contralaterally to the stimulated side.

Methyl bromide myoclonus: an electrophysiological study.

We report a case of myoclonus from overnight exposure to methyl bromide. Myoclonus was either spontaneous or induced by somatosensory stimulation or voluntary movements, multifocal and sometimes generalized. Median SEP showed normal size P14-N20, but giant parietal P25, N33 and frontal P22-N30 waves.

Orthostatic tremor: report of two cases and an electrophysiological study.

Two patients with legs tremor present on standing, but none on walking or sitting, are reported. Tremor was not exclusive or orthostatism and was also evoked by strong tonic contraction of leg muscles. Synchronous EMG bursts were recorded in antagonistic muscle groups at 8-10 Hz in the first patient and at 16 Hz in the second.

Associated central and peripheral demyelination: an electrophysiological study.

A case is reported in which retrobulbar neuritis preceded Guillain-Barré syndrome by 4 weeks. The visual evoked potential latencies were prolonged. After peripheral nervous system signs had cleared, median and peroneal somatosensory evoked potentials showed prolonged cervical N13, scalp N20 and L3-scalp conduction times.

Serial recording of VEPs to different stimuli in multiple sclerosis patients.

VEPs to different spatial frequencies (1,2,4 cycles per degree) and different stimulus configurations (vertical, horizontal gratings and checkerboard) were recorded in 47 Multiple Sclerosis patients and a corresponding group of control subjects. Serial recordings were performed in periods of 1-6 years. We show that the highest diagnostic yield in MS is for VEPs of higher spatial frequency than 1 cpd, and that vertical gratings give reliable results when high spatial frequencies are used, whereas checkerboard stimuli elicit consistent VEPs only when the check size is 30'-15' of arc of visual angle.

Strümpell's familial spastic paraplegia: an electrophysiological demonstration of selective central distal axonopathy.

Three patients with autosomal dominant Strümpell's familial spastic paraplegia (SFSP) were evaluated by means of somatosensory evoked potentials (SEPs) from upper and lower limb and determination of sural nerve conduction velocity. Findings of normal sural nerve conduction but reduced amplitude and poor definition of SEPs with normal latencies on peroneal nerve stimulation support a pattern of central nervous system degeneration characterized by a selective involvement of centrally directed axons within the gracile fasciculi.