Neuroscience represents a dynamic area of biomedical research where neuroethical responsibilities for researchers are emerging. This paper is the companion piece to the English-language one also published in this issue of the Canadian Journal of Neurological Sciences. It serves as a review of recent advances in neuroethics through the lens of three cases: (1) incidental finding of anomalies in neuroimaging research; (2) creation of neurotechnologies that can lead to cognitive enhancement, and (3) responsible communication of research results. We propose and discuss a multidimensional framework of neuroethical responsibilities to help tackle these issues. The framework reiterates the fundamental role of scientific integrity, puts in the foreground social responsibilities pertaining to the eventual use of neuroscience knowledge, and highlights self-reflection in research and training of researchers.

Neuroscience represents a dynamic area of biomedical research where neuroethical responsibilities for researchers are emerging. This paper is the companion piece to the French-language one also published in this issue of the Canadian Journal of Neurological Sciences. It serves as a review of recent advances in neuroethics through the lens of three cases: (1) incidental finding of anomalies in neuroimaging research; (2) creation of neurotechnologies that can lead to cognitive enhancement, and (3) responsible communication of research results. We propose and discuss a multidimensional framework of neuroethical responsibilities to help tackle these issues. The framework reiterates the fundamental role of scientific integrity, puts in the foreground social responsibilities pertaining to the eventual use of neuroscience knowledge, and highlights self-reflection in research and training of researchers.

Since the early 1990s, DNA triplet repeat expansions have been found to be the cause in an ever increasing number of genetic neurologic diseases. A subset of this large family of genetic diseases has the expansion of a CAG DNA triplet in the open reading frame of a coding exon. The result of this DNA expansion is the expression of expanded glutamine amino acid repeat tracts in the affected proteins, leading to the term, Polyglutamine Diseases, which is applied to this sub-family of diseases. To date, nine distinct genes are known to be linked to polyglutamine diseases, including Huntington's disease, Machado-Joseph Disease and spinobulbar muscular atrophy or Kennedy's disease. Most of the polyglutamine diseases are characterized clinically as spinocerebellar ataxias. Here we discuss recent successes and advancements in polyglutamine disease research, comparing these different diseases with a common genetic flaw at the level of molecular biology and early drug design for a family of diseases where many new research tools for these genetic disorders have been developed. Polyglutamine disease research has successfully used interdisciplinary collaborative efforts, informative multiple mouse genetic models and advanced tools of pharmaceutical industry research to potentially serve as the prototype model of therapeutic research and development for rare neurodegenerative diseases.

A 29-year-old woman was found lying unconscious in the shower. There was a two-day history of headache and dizziness. In the emergency room, she was initially stuporous (Glasgow Coma Scale 10/15), afebrile, bradycardic and hypertensive. She exhibited roving, conjugate eye movements, left facial paresis (including frontalis), left ptosis, diffuse hypotonia, extensor plantar responses bilaterally and a 1.5 cm warm, fluctuant mass with surrounding erythema behind the left ear (Figure 1). Otoscopy revealed a bulge in the posterior wall of the left external auditory canal.

Hypoglossal nerve injury is a rare complication of anesthetic airway management. We report a case of unilateral hypoglossal nerve injury following the use of the laryngeal mask airway (LMA). A 48-year-old man with no past medical history sustained a proximal humerus fracture. His pre-operative examination was normal except for a humerus fracture. Anesthesia was induced and a size-3 LMA was inserted successfully on first attempt without difficulty. The cuff was inflated with the recommended 20 ml of air until there was no leak and maintained at a pressure of 10-15 cm H20. The surgery was uneventful and the duration of anesthesia was two hours. Peri-operatively the patient was hemodynamically stable without episodes of hypotension. The recovery staff noted approximately three hours after the surgery that his tongue was deviated to the left. The patient complained of difficulty swallowing and slurred speech. He had normal sensation of his tongue, taste was unchanged, and the gag reflex was intact. No other cranial nerves were involved and no other neurological deficiency was found. The following day, there was significantly less dysarthria and slight improvement in tongue movement. Conservative management included speech therapy and regular assessment. He continued to improve and complete recovery took place within two weeks.

Occipital condyle fracture (OCF) is a rare injury that was first described by Bell in 1817. In fact, there have been only 96 more reported cases of occipital condyle fractures from 1817 to 1994 of which only 58 survived. Occipital condyle fractures can sometimes go unnoticed or under-diagnosed as they are not always evident on plain radiographs of the cervical spine. Also, in rare cases OCFs can cause damage to the hypoglossal nerve which passes through the hypoglossal canal which is near the occipital condyle. The presence of specific symptoms and clinical signs should lead to the correct diagnosis. This paper describes a patient who was diagnosed with OCFs, but not hypoglossal nerve damage until 20 days following admission to hospital. We point out many factors that contributed to this delayed diagnosis, which ultimately caused severe discomfort to the patient.

Fibrous dysplasia (FD) of the bone is an idiopathic disorder in which bone-forming mesenchyme develops abnormally. Here, we describe an unusual case in an adult, with recurrent acute headache and third nerve palsy due to FD of the skull base and low-grade fibrosarcoma.

A previously healthy 32-year-old female office clerk, presented with an eight day history of acute-onset, severe, right occipital and retro-orbital continuous headache, associated with nausea and vomiting. She had required dimenhydrinate, acetaminophen with 15 mg codeine, and meperidine with only mild relief of the headache. She also described a 5-day history of right eye ptosis which had progressed to eye closure by the time of presentation. She had otherwise been well, with no systemic or constitutional symptoms and no previous history of headaches.

Neurofibromatosis Type I or von Recklinghausen’s neurofibromatosis is an autosomal dominant disorder with a high index of spontaneous mutations and extremely varied and unpredictable clinical manifestations. We present a case of sepsis secondary to an infected hematoma, enclosed within a massive neurofibroma.

A 42-year-old man presented to the emergency department with a one week history of fever and chills. He reported an increase in pain and size of a growth near his chest. The patient was noted to be febrile on arrival. On physical examination, a very large neurofibroma was seen extending from the right upper chest. Wound and blood cultures were obtained. Computed Tomography (CT) of the thorax revealed a hematoma contained within the large mass.

Pituitary carcinomas have been reported to metastasize systemically and, less commonly, along the craniospinal axis. Metastatic lesions have been reported in the cerebral cortex, cerebellum, spinal cord, leptomeninges, cervical lymph nodes, liver, ovaries, and bone. The authors are unaware of any other examples of subependymal metastases of a pituitary carcinoma. We report such a case.

Pituitary carcinoma is rare, accounting for roughly 0.2%1,3-5 of all pituitary tumors, with approximately 140 cases reported in the literature. These tumors are associated with a very high mortality, with 66% of patients dying within the first year after diagnosis. Pituitary carcinomas are differentiated from invasive pituitary adenomas by the presence of non-contiguous craniospinal tumor deposits and/or distant systemic metastases. The majority (88%) of these carcinomas prove to be hormone secreting, with prolactin secreting tumors being the most common. Invasive carcinomas evolve from hormone secreting pituitary adenomas after a latency period. Pituitary carcinomas have a predilection for systemic spread. The rate of systemic metastasis approaches 71% for prolactin producing tumors and 57% for ACTH producing tumors. Thirteen percent of tumors demonstrate both systemic and craniospinal patterns of metastatic spread.