Exophytic bulbar pilocytic astrocytoma and post-operative cerebral salt wasting syndrome.

Cerebral salt wasting syndrome (CSWS) is a well-described consequence of several neurological disorders. Although the exact etiology of CSWS is still not completely elucidated, it is believed that the hypothalamus plays a pivotal role in the genesis of this disorder. We report for the first time 3 cases of CSWS occurring during the post-operative course following surgical resection of exophytic bulbar pilocytic astrocytomas in children. Since these 3 cases shared in common a medial implication of the medulla, we suggest that specific interconnectivity between the dorso-medial portion of the medulla oblongata and the hypothalamus might thus represent an anatomical pathway of interest in the pathogenesis of CSWS. Our findings suggest that the resection of medially located exophytic bulbar tumors might constitutes a risk factor in the development of CSWS. Particular care should thus be carried towards the prompt detection and treatment of CSWS in the post-operative courses of exophytic bulbar tumors.

Pituitary choriocarcinoma in an adolescent male: tumor-derived CG and GH delay diagnosis.

BACKGROUND: Primary intracranial germ cell tumors usually present in the first two decades of life, often with precocious puberty. The most common location is in the pineal gland; suprasellar germ cell tumors are rare. We present an additional case of a suprasellar choriocarcinoma producing GH, and review the literature. CASE: This French Canadian, 17 year-old male presented to the ER with a history of mild weight loss and an episode of syncope while hiking in Mexico, but with no other neurological symptoms. Puberty began at age 13 years (growth spurt: 15-16 years), and he attained an adult height within genetic target by age 16 years. Past medical history was negative except for myopia diagnosed during childhood. System review revealed increased thirst and nocturia. The mother was treated for an oligo-astrocytoma in 2007. Clinical examination showed a euthyroid, well-looking young man with 20 ml testicles. Endocrine evaluation revealed elevated testosterone, mildly elevated PRL, borderline low FT4, and decreased IGF-I, morning cortisol and urine osmolality; tumor markers were positive in serum and CSF (hCG>50 IU/L, AFP>10 ng/mL). A transphenoidal biopsy of a 4.5 cm, homogeneous, non-calcified, suprasellar mass was compatible with the diagnosis of choriocarcinoma and stained intensely for hCG and hGH, presumably the placental variant (GH-V) as previously found in vitro in choriocarcinoma cell lines. Combined chemotherapy and irradiation led to tumor regression and undetectable serum hCG to 36 months of follow-up. He is doing well with no evidence of tumor progression and is on complete hormone replacement therapy. CONCLUSIONS: Choriocarcinomas can have a hormonal profile that delays the development of symptoms, due to hCG stimulation of both the gonadal and thyroid axes. This report corroborates previous in vitro evidence that choriocarcinoma cells are able to make GH-V. To what extent the patient's tumor-derived GH contributed to his normal growth is not known. Prognosis for this intracranial neoplasm is very reserved, although combined radiotherapy and chemotherapy has been successful in our patient now 36 months post-diagnosis.

[The organization of the transition from childhood to adulthood in pediatric neurosurgery in Canada]. / L'organisation de la transition enfant à adulte en neurochirurgie pédiatrique au Canada.

The problem of the transition from childhood to adulthood in Canadian neurosurgery results from the individuation of pediatric neurosurgery. Except for transition programs for specific diseases, such as spina bifida or neurofibromatosis or cerebrovascular diseases, which are followed by adult surgical-endovascular teams, the transition from pediatric to adult care is generally left to the initiative of patients and their family. This reflects both the organization of healthcare and the geographic specificities of Canada. A harmonious transition from childhood to adulthood should result from coordination between neurosurgeons and between the different hospital networks. Transition teams, such as those already in operation for spina bifida, should be expanded to other diseases. Patient associations have a key role to play in this change.

Reliability and clinical relevance of perceptual evaluation of pathological voices.

The perceptual GIRBAS scale for deviant voice quality was tested in 5 different institutes on 943 voice patients. Each voice was evaluated separately by 2 professionals. The interrater and intrarater correlation is satisfactory for G, R and B. Experience with the scale significantly improves the interrater agreement. Further, GIRBAS/acoustical correlations were investigated by using the multidimensional voice program (MDVP) of Kay. Principal component analysis allows reduction of 21 MDVP parameters to 5 clinically relevant clusters, with a typical acoustical measurement for each of them. The GIRBAS scale seems to be a valuable instrument for clinical practice.

Quantitative rating of video-laryngostroboscopy: a reliability study.

The purpose of this study is to examine the most reliable and valid way for rating and comparing videolaryngostroboscopic recordings with a differentiated protocol. 30 high quality videostroboscopic recordings (2-5 minutes) of all kinds of vocal fold pathology were rated independently by 7 experienced laryngologists/phoniatricians, using a standardized and exhaustive protocol form. Interobserver agreement is high for amplitude, mucosal wave, and type of closure/symmetry, is satisfactory for regularity, consistency and vibration of the lesion (if any), and is rather moderate to low for grade of glottic closure and symmetry. As a general rule, raters trained with the protocol form show higher agreement than raters without such experience. Intraobserver consistency is good for all parameters.