How Knowledgeable Are Spine Surgeons Regarding EMG-NCS for Cervical Spine Conditions? An International Aospine Survey.

Study Design: Cross-sectional, international survey.

Objectives: To evaluate the knowledge of spine surgeons regarding the use of electromyography (EMG) and nerve conduction studies (NCS) for degenerative cervical spine conditions (DCC).

Methods: All members of AO Spine International were emailed an anonymous survey to evaluate their clinical knowledge about the use of EMG and nerve conduction studies for DCC.

Randomized Trial of Botulinum Toxin Type A in Hereditary Spastic Paraplegia - The SPASTOX Trial.

Background: Hereditary spastic paraplegia presents spasticity as the main clinical manifestation, reducing gait quality and producing incapacity. Management with botulinum toxin type A (BoNT-A) is not well elucidated. The objective of the current study was to evaluate the efficacy and safety of BoNT-A in patients with hereditary spastic paraplegias.

Misdiagnosis and diagnostic delay in non-paraneoplastic sensory neuronopathies.

Methods: Sensory neuronopathies (SN) are a group of peripheral nerve disorders characterized by multifocal non-length-dependent sensory deficits and sensory ataxia. Its recognition is essential not only for proper management but also to guide the etiological investigation. The uncommon SN clinical picture and its rarity set the conditions for the misdiagnosis and the diagnostic delay, especially in non-paraneoplastic SN.

Deafness and Vestibulopathy in Cerebellar Diseases: a Practical Approach.

Cerebellar ataxias are a clinically heterogeneous group of neurological disorders. Besides the cerebellum, several forms of hereditary ataxias or non-genetic ataxias also affect other areas of the brain. Some forms of cerebellar ataxias may have cochlear and vestibular involvement and may present with deafness and symptoms or signs of vestibulopathy (dizziness, nystagmus and diplopia).

SPG11-related parkinsonism: Clinical profile, molecular imaging and l-dopa response.

Background: Molecular imaging has proven to be a powerful tool to elucidate degenerated paths in a wide variety of neurological diseases and has not been systematically studied in hereditary spastic paraplegias.

Objectives: To investigate dopaminergic degeneration in a cohort of 22 patients with hereditary spastic paraplegia attributed to SPG11 mutations and evaluate treatment response to l-dopa.

Methods: Patients and controls underwent single-photon emission computed tomography imaging utilizing Tc-TRODAT-1 tracer.

mutations cause widespread white matter and basal ganglia abnormalities, but restricted cortical damage.

mutations are the major cause of autosomal recessive Hereditary Spastic Paraplegia. The disease has a wide phenotypic variability indicating many regions of the nervous system besides the corticospinal tract are affected. Despite this, anatomical and phenotypic characterization is restricted.

Derek Denny-Brown: the man behind the ganglia.

The authors present an historical review about the main contributions of Professor Derek Denny-Brown to neurology. Some of his achievements include the first description of sensory neuronopathies, and some of the essential textbooks on the function and anatomy of the basal ganglia. In 2016, on the 35th anniversary of his death, modern neurologists are still strongly influenced by his legacy.

[Prevalence of oral mucosal alterations in Brazilian adolescents held in two juvenile re-education centers].

The purpose of the present study was to verify the prevalence of oral mucosal alterations in Brazilian adolescents institutionalized. A total of 231 adolescents, all male were examined. The criteria used for clinical diagnostic of the lesions were the former proposed by SB 2000 (Brazil).

Clinical, histological and immunohistochemical features of ectomesenchymal chondromyxoid tumor.

Objective: Ectomesenchymal chondromyxoid tumor is a rare oral soft tissue neoplasm that should be differentiated from other neural and chondromyxoid entities. The aim of this study was to report the clinical, histological, and immunohistochemical features of 3 additional cases of this condition.

Methods: Clinical data were obtained from the clinical records and all cases were evaluated through light microscopy and immunohistochemistry to cytokeratins, vimentin, S100 protein, desmin, smooth muscle actin, and glial fibrilary acidic protein.

Advanced osteosarcoma of the maxilla: a case report.

Osteosarcomas are primary malignant bone tumours in which mesenchymal cells produce osteoid. It is generally the most common malignant bone neoplasm, although lesions of the jaw are uncommon. Osteosarcoma of the jaw (JOS) presents a lower incidence of metastasis and a better prognosis than osteosarcoma of the long bones.

Congenital granular cell tumor (congenital epulis): a lesion of multidisciplinary interest.

Congenital granular cell tumor (CGCT), or congenital epulis, is a very uncommon benign soft tissue lesion that usually arises from the alveolar mucosa of neonates and may cause respiratory and feeding problems. We report a case of a 3-day-old female newborn, who presented an intraoral tumor mass which was protruding from her mouth, and compromising feeding. Under general anesthesia, the lesion was completely removed and the patient had an uneventful postoperative course.