Developmental malformations of human tongue and associated syndromes (review)

The development of the tongue begins as known, in the floor of the primitive oral cavity, when the human embryo is four weeks old.More specifically, the tongue develops from the region of the first three or four branchial arches during the period that the external face develops. Malformations of the tongue, are structural defects, present at birth and happening during embryogenesis. The most common malformations are:1. Aglossia2. Microglossia, which is always combined with other defects and syndromes, like Moëbius syndrome3. Macroglossia, which is commonly associated with cretinism, Down’s syndrome, Hunter’s syndrome, Sanfilippo syndrome and other types of mental retardation4. Accesory tongue5. Long tongue6. Cleft or Bifid tongue, condition very usual in patients with the orodigitofacial syndrome7. Glossitis Rhombica Mediana, a developmental malformation?8. Lingual thyroid.Malformations are extensively analysed and discussed.Le développement de la langue commence au niveau du plancher de la cavité orale primitive lorsque l’embryon humain est âgé de 4 semaines.Plus précisément, la langue se développe dans la région des trois ou quatre premiers arcs branchiaux durant la période du développement de la face externe. Les malformations de la langue correspondent à des défauts de structure présents à la naissance et survenant au cours de l’embryogenèse. Les malformations les plus communes sont:1. Aglossie2. Microglossie, qui est souvent combinée à d’autres anomalies ou syndromes, tel le syndrome de Moebius3. Macroglossie, qui est communément associée au crétinisme, au syndrome de Down, au syndrome de Hunter, au syndrome de Sanfilippo et à d’autres types de retard mental4. Langue accessoire5. Langue longue6. Langue bifide, condition très usuelle chez des patients présentant le syndrome orodigitofacial7. Glossite rhomboïde médiane (?)8. Thyroïde linguale.Les malformations sont analysées et discutées

Chemotherapy and diffuse low-grade gliomas: a survey within the European Low-Grade Glioma Network.

Diffuse low-grade gliomas (DLGGs) are rare and incurable tumors. Whereas maximal safe, functional-based surgical resection is the first-line treatment, the timing and choice of further treatments (chemotherapy, radiation therapy, or combined treatments) remain controversial. An online survey on the management of DLGG patients was sent to 28 expert centers from the European Low-Grade Glioma Network (ELGGN) in May 2015. It contained 40 specific questions addressing the modalities of use of chemotherapy in these patients. The survey demonstrated a significant heterogeneity in practice regarding the initial management of DLGG patients and the use of chemotherapy. Interestingly, radiation therapy combined with the procarbazine, CCNU (lomustine), and vincristine regimen has not imposed itself as the gold-standard treatment after surgery, despite the results of the Radiation Therapy Oncology Group 9802 study. Temozolomide is largely used as first-line treatment after surgical resection for high-risk DLGG patients, or at progression. The heterogeneity in the management of patients with DLGG demonstrates that many questions regarding the postoperative strategy and the use of chemotherapy remain unanswered. Our survey reveals a high recruitment potential within the ELGGN for retrospective or prospective studies to generate new data regarding these issues

Cavernous Malformations of the Central Nervous System: An International Consensus Statement

Introduction: Cavernous malformations (CM) of the central nervous system constitute rare vascular lesions. They are usually asymptomatic, which has allowed their management to become quite debatable. Even when they become symptomatic their optimal mode and timing of treatment remains controversial. Research question: A consensus may navigate neurosurgeons through the decision-making process of selecting the optimal treatment for asymptomatic and symptomatic CMs. Material and methods: A 17-item questionnaire was developed to address controversial issues in relation to aspects of the treatment, surgical planning, optimal surgical strategy for specific age groups, the role of stereotactic radiosurgery, as well as a follow-up pattern. Consequently, a three-stage Delphi process was ran through 19 invited experts with the goal of reaching a consensus. The agreement rate for reaching a consensus was set at 70%. Results: A consensus for surgical intervention was reached on the importance of the patient’s age, symptomatology, and hemorrhagic recurrence; and the CM’s location and size. The employment of advanced MRI techniques is considered of value for surgical planning. Observation for asymptomatic eloquent or deep-seated CMs represents the commonest practice among our panel. Surgical resection is considered when a deep-seated CM becomes symptomatic or after a second bleeding episode. Asymptomatic, image-proven hemorrhages constituted no indication for surgical resection for our panelists. Consensus was also reached on not resecting any developmental venous anomalies, and on resecting the associated hemosiderin rim only in epilepsy cases. Discussion and conclusion: Our Delphi consensus provides an expert common practice for specific controversial issues of CM patient management

State of the art and future prospects of nanotechnologies in the field of brain-computer interfaces

Neuroprosthetic control by individuals suffering from tetraplegia has already been demonstrated using implanted microelectrode arrays over the patients’ motor cortex. Based on the state of the art of such micro & nano-scale technologies, we review current trends and future prospects for the implementation of nanotechnologies in the field of Brain-Computer Interfaces (BCIs), with brief mention of current clinical applications. Micro- and Nano-Electromechanical Systems (MEMS, NEMS) and micro-Electrocorticography now belong to the mainstay of neurophysiology, producing promising results in BCI applications, neurophysiological recordings and research. The miniaturization of recording and stimulation systems and the improvement of reliability and durability, decrease of neural tissue reactivity to implants, as well as increased fidelity of said systems are the current foci of this technology. Novel concepts have also begun to emerge such as nanoscale integrated circuits that communicate with the macroscopic environment, neuronal pattern nano-promotion, multiple biosensors that have been “wired” with piezoelectric nanomechanical resonators, or even “neural dust” consisting of 10-100μm scale independent floating low-powered sensors. Problems that such technologies have to bypass include a minimum size threshold and the increase in power to maintain a high signal-to-noise-ratio. Physiological matters such as immunological reactions, neurogloia or neuronal population loss should also be taken into consideration. Progress in scaling down of injectable interfaces to the muscles and peripheral nerves is expected to result in less invasive BCI-controlled actuators (neuroprosthetics in the micro and nano scale). The state-of-the-art of current microtechnologies demonstrate a maturing level of clinical relevance and promising results in terms of neural recording and stimulation. New MEMS and NEMS fabrication techniques and novel design and application concepts hold promise to address current problems with these technologies and lead to less invasive, longer lasting and more reliable BCI systems in the near future

Acute-onset diplopia from intracranial hypertension due to torcular herophili obstruction by an hemorrhagic intradiploic epidermoid cyst

Background: Epidermoid cysts are benign slow-growing congenital lesions, constituting approximately 1% of all cranial tumors. Most of these lesions are located intradurally, while about 10-25% of them are located within the diploic spaces. Intradiploic epidermoid cysts are usually discovered incidentally and may remain asymptomatic for many years, but in rare instances, they may grow intracranially and produce brain compression. Sometimes, intradiploic epidermoid cysts may occlude the main cranial venous sinuses causing intracranial hypertension. Case Description: We present the case of a 24-year-old male harboring a paramedian right occipital intradiploic cyst with erosion of both outer and inner bony tables, which occluded the torcular herophili producing a worsening symptomatology with acute-onset diplopia from right sixth cranial nerve palsy; the patient also presented bilateral papilledema, but only reported mild headache and dizziness. Neuroradiological studies evidentiated a lesion compatible with intradiploic epidermoid cyst with intralesional hemorrhagic component, overlying and almost completely occluding the torcular herophili. Considering the fast worsening of symptomatology and the evidence of intracranial hypertension, the patient was operated on immediately after completion of clinical and radiological assessment. The lesion was radically removed with almost immediate reversal of signs and symptoms. Histopathology confirmed the diagnosis of epidermoid cyst with intralesional hemorrhagic components. Conclusion: Intradiploic epidermoid cysts may cause intracranial hypertension by occlusion of main cranial venous sinuses; intralesional hemorrhage may act as precipitating factor in occlusion of the torcular herophili, producing rapidly worsening intracranial hypertension, which requires prompt surgical treatment to reverse symptomatology. Radical surgical resection is necessary to avoid recurrence