The medical therapy for cerebellar mutism syndrome: a case report and literature review.

PURPOSE: Cerebellar mutism syndrome (CMS) represents a major complication affecting many children that undergo surgery for posterior fossa lesions. Etiology and pathophysiology are still not fully understood. CMS deeply influences quality of life and recovery of these patients. An effective treatment has not been defined yet. This case-based review aims at analyzing the available evidence and knowledge to better delineate this phenomenon and to determine whether CMS can be successfully treated with pharmacological therapy. METHODS: Systematic research and retrieval of databases were conducted analyzing all papers where medical treatment of CMS was reported. A summary of the latest understanding and reports regarding definition, clinical manifestations, pathophysiology, management, and outcome of CMS has been conducted. RESULTS: Consensus on definition of this syndrome is lacking. CMS is the term accepted by the Posterior Fossa Society in 2016. Pathophysiology is still poorly understood but the most likely mechanism is injury along proximal components of the efferent cerebellar pathway. Nine papers describing positive effects of pharmacological therapy for CMS have been identified. Fluoxetine, zolpidem, bromocriptine, and midazolam are the drugs that seem to alleviate symptoms of CMS and improve recovery. To date, cognitive rehabilitation and physiotherapy are the only treatment options available. CONCLUSION: CMS has deep impact on affected children and their families. Despite attempts to identify preventive measures and treatment, cases still occur on a regular basis. Pharmacological treatments have been proposed to help reduce the symptoms of CMS with some promising results, but reports are limited; therefore, further studies are needed.

Complete section of the left vagus nerve does not preclude the efficacy of vagus nerve stimulation: illustrative case.

BACKGROUND: Vagus nerve stimulation (VNS) represents a valid therapeutic option for patients with medically intractable seizures who are not candidates for epilepsy surgery. Even when complete section of the nerve occurs, stimulation applied cranially to the involved nerve segment does not preclude the efficacy of VNS. Complete vagus nerve section with neuroma causing definitive left vocal cord palsy has never been previously reported in the literature. OBSERVATIONS: Eight years after VNS implant, the patient experienced worsening of seizures; the interrogation of the generator revealed high impedance requiring surgical revision. On surgical exploration, complete left vagus nerve section and a neuroma were found. Vocal cord atrophy was found at immediate postoperative laryngeal inspection as a confirmation of a longstanding lesion. Both of these events might have been caused by direct nerve injury during VNS surgery, and they presented in a delayed fashion. LESSONS: VNS surgery may be complicated by direct damage to the left vagus nerve, resulting in permanent neurological deficits. A complete section of the nerve also enables an efficacious stimulation if applied cranially to the involved segment. Laryngeal examination should be routinely performed before each VNS surgery to rule out preexisting vocal cord dysfunction.

White matter and cerebellar involvement in alternating hemiplegia of childhood.

OBJECTIVE: To determine whether brain volumetric and white matter microstructural changes are present and correlate with neurological impairment in subjects with alternating hemiplegia of childhood (AHC). METHODS: In this prospective single-center study, 12 AHC subjects (mean age 22.9 years) and 24 controls were studied with 3DT1-weighted MR imaging and high angular resolution diffusion imaging at 3T. Data obtained with voxel-based morphometry and tract-based spatial statistics were correlated with motor impairment using the International Cooperative Ataxia Rating Scale (ICARS) and Movement and Disability sub-scales of Burke-Fahn-Marsden Dystonia Rating Scale (BFMMS and BFMDS). RESULTS: Compared to healthy controls, AHC subjects showed lower total brain volume (P < 0.001) and white matter volume (P = 0.002), with reduced clusters of white matter in frontal and parietal regions (P < 0.001). No significant regional differences were found in cortical or subcortical grey matter volumes. Lower cerebellar subvolumes correlated with worse ataxic symptoms and global motor impairment in AHC group (P < 0.001). Increased mean and radial diffusivity values were found in the corpus callosum, corticospinal tracts, superior and inferior longitudinal fasciculi, subcortical frontotemporal white matter, internal and external capsules, and optic radiations (P < 0.001). These diffusion scalar changes correlated with higher ICARS and BFMDS scores (P < 0.001). INTERPRETATION: AHC subjects showed prevalent white matter involvement, with reduced volume in several cerebral and cerebellar regions associated with widespread microstructural changes reflecting secondary myelin injury rather than axonal loss. Conversely, no specific pattern of grey matter atrophy emerged. Lower cerebellar volumes, correlating with severity of neurological manifestations, seems related to disrupted developmental rather than neurodegenerative processes.

The use of opioids in children receiving intrathecal baclofen therapy.

PURPOSE: We hypothesized that children on chronic intrathecal baclofen therapy (ITB) may require less analgesics for postoperative pain control and are at higher risk of developing opioid-induced respiratory depression postoperatively. The aims of this study are to review children on chronic intrathecal baclofen therapy receiving opioids after major surgery and to determine the incidence complications in this population. METHOD: We conducted a retrospective cohort study comparing 13 children on ITB, who underwent posterior spinal fusion surgery, to 17 children with spina bifida that received the same surgery. RESULTS: On postoperative day 0 (POD 0), four children (40%) had respiratory depression in the baclofen group compared to none in the control group. Desaturation was significantly more frequent in children in the ITB group compared to those of the control group on POD 0; oversedation was recorded in 8 (80%) children in the baclofen group vs. 3 (17.6%) in the control group. Desaturation, respiratory depression, and oversedation were significantly more frequent on POD 0 in children in the baclofen group compared with children in the control group. CONCLUSIONS: The findings of the current study suggest that children on chronic intrathecal baclofen therapy require lesser amounts of opioids for postoperative pain control and are at a greater risk of developing postoperative respiratory depression and excessive sedation compared to patients without baclofen therapy.

Vagus nerve stimulation: Surgical technique of implantation and revision and related morbidity.

Indications for vagus nerve stimulation (VNS) therapy include focal, multifocal epilepsy, drop attacks (tonic/atonic seizures), Lennox-Gastaut syndrome, tuberous sclerosis complex (TSC)-related multifocal epilepsy, and unsuccessful resective surgery. Surgical outcome is about 50-60% for seizures control, and may also improve mood, cognition, and memory. On this basis, VNS has also been proposed for the treatment of major depression and Alzheimer's' disease. The vagus nerve stimulator must be implanted with blunt technique on the left side to avoid cardiac side effects through the classic approach for anterior cervical discectomy. The actual device is composed of a wire with three helical contacts (two active contacts, one anchoring) and a one-pin battery. VNS is usually started 2 weeks after implantation with recommended settings of stimulation (1.0-2.0 mA; 500 µs pulse width; 20-30 Hz; 30 s ON, 5 min OFF). The complications of VNS therapy are early (related to surgery) and late (related to the device and to stimulation of the vagus nerve). Early complications include the following: intraoperative bradycardia and asystole during lead impedance testing, peritracheal hematoma, infections (3-8%), and vagus nerve injury followed by hoarseness, dyspnea, and dysphagia because of left vocal cord paralysis. Delayed morbidity due to the device includes late infections or problems in wound healing; other more rare events are due to late injury of the nerve. Late complications due to nerve stimulation include delayed arrhythmias, laryngopharyngeal dysfunction (hoarseness, dyspnea, and coughing), obstructive sleep apnea, stimulation of phrenic nerve, tonsillar pain mimicking glossopharyngeal neuralgia, and vocal cord damage during prolonged endotracheal intubation. The laryngopharyngeal dysfunction occurs in about 66% of patients and is usually transitory and due to the stimulation of the inferior (recurrent) laryngeal nerve. A true late paralysis of the left vocal cord is often partial and rare (1-2.7%), and usually transitory, and may be caused by previous surgical trauma (i.e., damage of nerve fibers and/or of their blood supply) or to a supposed chronic denervation during stimulation. Surgery for complete removal or revision and replacement of the device is to be considered in cases of device malfunction (4-16.8%), failure of VNS therapy, intolerable side effects, or because of patient's specific request. As described in the literature, the surgical techniques of lead revision and replacement are two: sharp and blunt dissection of helical electrodes and replacement; and blunt dissection combined with ultrasharp low-voltage cautery dissection. The incidence of left vocal cord palsy after vagus nerve stimulator replacement/revision is slightly higher than that of first implantation (4.9% vs. 3.8%). A de novo implantation in a naive segment of the left or right vagus nerve may be considered in specific cases; the use of the right vagus nerve is a rare exception that may be chosen with an acceptable result.

Deep Brain Stimulation of the Anterior Limb of the Internal Capsule May Be Efficacious for Explosive Aggressive Behaviour.

BACKGROUND: Intermittent explosive disease (IED) is a psychiatric disorder characterized by intermittent attacks of rage and violence frequently resistant to pharmacological therapy. Deep brain stimulation (DBS) of the posteromedial hypothalamus has been applied with fair results and clinical improvement with some surgical morbidity due to neurovegetative side effects. The anterior limb of the internal capsule/ventral capsule/ventral striatum (VC/VS) has never been used alone as a target for this disease. OBJECTIVES: The aim of this study is to evaluate the efficacy of bilateral DBS of the VC/VS for the treatment of IED. METHODS: We performed bilateral DBS of the VC/VS in a 21-year-old patient with IED. This young man had a traumatic birth complicated by hypoxia, and he showed a mild mental impairment. Different pharmacological treatments were carried out with no results before DBS was proposed to the patient's relatives after multidisciplinary approval. RESULTS: After 22 months of high-frequency monopolar bilateral DBS of the VC/VS, the patient showed a significant improvement. Postoperative 18F-FDG PET-CT studies ruled out a reduction of the hypermetabolic areas located in the limbic system previously detected in pre-operative investigations. CONCLUSIONS: Bilateral DBS of the VC/VS may be considered for the treatment of IED without the risk of neurovegetative side effects.

[Mission woman: a survey on the perception of the "Service of Welcome and Listening" offered in the Emergency Department at the San Camillo Hospital in Rome by women victims of violence]. / Mission woman: indagine sulla percezione del "Servizio di Accoglienza e Ascolto" nel Pronto Soccorso dell'Az. Osp. S.Camillo-Forlanini di Roma da parte delle donne vittime di violenza.

INTRODUCTION: The project Mission Woman was set up to describe how the service offered by the Emergency Department of the Hospital San Camillo-Forlanini in Rome was perceived by women victims ( or supposed to be) of violence. The objective of this investigation is to frame clients' perception about the service offered by staff in terms of sensitivity, recognition and management of the issue. The access to the Emergency Room is the first contact of the client with a helping relationship which goes beyond the simple provision of medical care. METHODS: A questionnaire devised for the purpose was filled in by women who have asked for help to the Service "Door Woman" . The Service "Door Woman", set up in 2009, aimed to welcome and listen women victims of violence with the collaboration of different operators in the emergency department: nurses, doctors, psychologists and social workers. The project developed within the Hospital S. Camillo-Forlanini is managed by both professionals of the Emergency Department together with the Charity "Be Free". The service is open 24/7, all year, the clients could be women or children and could receive assistance or medical, nursing, psychosocial and legal counselling. The triage nurses are those who through their expertise could first identify the victims of violence and to introduce them in the path. CONCLUSION: Despite the level of injuries, who access to the Emergency Room for single or repeated events of violence, may have decided to claim in court and not hide seeking.. The research aims to understand what are the theoretical and practical deficiencies of staff in the management of a issue which is not only medical but also social and legal, as well as structural and organizational weaknesses of the service.

Imaging of neuropathies about the hip.

Neuropathies about the hip may be cause of chronic pain and disability. In most cases, these conditions derive from mechanical or dynamic compression of a segment of a nerve within a narrow osteofibrous tunnel, an opening in a fibrous structure, or a passageway close to a ligament or a muscle. Although the evaluation of nerve disorders primarily relies on neurological examination and electrophysiology, diagnostic imaging is currently used as a complement to help define the site and aetiology of nerve compression and exclude other disease possibly underlying the patient' symptoms. Diagnosis of entrapment neuropathies about the hip with US and MR imaging requires an in-depth knowledge of the normal imaging anatomy and awareness of the anatomic and pathologic factors that may predispose or cause a nerve injury. Accordingly, the aim of this article is to provide a comprehensive review of hip neuropathies with an emphasis on the relevant anatomy, aetiology, clinical presentation, and their imaging appearance. The lateral femoral cutaneous neuropathy (meiralgia paresthetica), femoral neuropathy, sciatic neuropathy, obturator neuropathy, superior and inferior gluteal neuropathies and pudendal neuropathy will be discussed.

The frequency of regulatory CD3+CD8+CD28- CD25+ T lymphocytes in human peripheral blood increases with age.

Aging is commonly associated with immune deficiency and dysregulation. The aging of the immune system involves a progressive reduction in naïve T cell output associated with thymic involution and peripheral expansion of oligoclonal memory T cells. We have investigated frequency, phenotype, and function of CD3+CD8+CD28(-)CD25+ T cells in healthy volunteers over a wide age range. We demonstrate that the frequency of CD3+CD8+CD28(-)CD25+ T cells in healthy volunteers increases with age. Peripheral CD3+CD8+CD28(-)CD25+ T cells share phenotypic and functional features with CD3+CD4+CD25+ regulatory T cells (Tregs): In particular, they strongly express CTLA-4 and forkhead box P3. We observed that in vitro, functional titration assays of CD3+CD8+CD28(-)CD25+ T cells show equivalent regulatory function in young and elderly donors, with suppression of proliferation and cytokine production in response to polyclonal T cell stimulation. Finally, CD3+CD8+CD28(-)CD25+ T cells seem to specifically express the CD122 receptor. Altogether, these observations demonstrate an increase in peripheral blood CD8+ Tregs associated with aging.