Co-Planar Stereotaxic Atlas of the Human Brain—3-Dimensional Proportional System: An Approach to Cerebral Imaging, J. Talairach, P. Tournoux. Georg Thieme Verlag, New York (1988), 122 pp., 130 figs. DM 268

Neurofibromatosis type 2 (NF2) is a monogenic dominantly inherited disease predisposing carriers to develop nervous system tumours. To identify the genetic defect, the region between two flanking polymorphic markers on chromosome 22 was cloned and several genes identified. One is the site of germ-line mutations in NF2 patients and of somatic mutations in NF2-related tumours. Its deduced product has homology with proteins at the plasma membrane and cytoskeleton interface, a previously unknown site of action of tumour suppressor genes in humans.

Alteration in a new gene encoding a putative membrane-organizing protein causes neuro-fibromatosis type 2

https://www.cell.com/cell/pdf/0092-8674(93)90406-G.pdf

A novel moesin-, ezrin-, radixin-like gene is a candidate for the neurofibromatosis 2 tumor suppressor

Objective. —Neurofibromatosis 1 and neurofibromatosis 2 are autosomal dominant genetic disorders in which affected individuals develop both benign and malignant tumors at an increased frequency. Since the original National Institutes of Health Consensus Development Conference in 1987, there has been significant progress toward a more complete understanding of the molecular bases for neurofibromatosis 1 and neurofibromatosis 2. Our objective was to determine the diagnostic criteria for neurofibromatosis 1 and neurofibromatosis 2, recommendations for the care of patients and their families at diagnosis and during routine follow-up, and the role of DNA diagnostic testing in the evaluation of these disorders. Data Sources. —Published reports from 1966 through 1996 obtained by MEDLINE search and studies presented at national and international meetings. Study Selection. —All studies were reviewed and analyzed by consensus from multiple authors. Data Extraction. —Peer-reviewed published data were critically evaluated by independent extraction by multiple authors. Data Synthesis. —The main results of the review were qualitative and were reviewed by neurofibromatosis clinical directors worldwide through an Internet Web site. Conclusions. —On the basis of the information presented in this review, we propose a comprehensive approach to the diagnosis and treatment of individuals with neurofibromatosis 1 and neurofibromatosis 2.

https://jamanetwork.com/journals/jama/articlepdf/417288/jama_278_1_042.pdf

The diagnostic evaluation and multidisciplinary management of neurofibromatosis 1 and neurofibromatosis 2.

Introduction Histological Classification of CNS Tumours Definitions and Explanatory Notes Tumours of Neuroepithelial Tissue Astrocytic Tumours Ependymal Tumours Mixed Gliomas Choroid Plexus Tumours Neuroepithelial Tumours of Uncertain Origin Neuronal and Mixed Neuronal-Glial Tumours Pineal Parenchymal Tumours Embryonal Tumours Tumours of Cranial and Spinal Nerves Schwannoma Neurofibroma Malignant Peripheral Nerve Sheath Tumor Tumours of the Meninges Tumours of Menigothelial Cells Mesenchymal, Non-menigothelial Tumours Primary Melanocytic Lesions Lymphomas and Haemopoietic Neoplasms Germ Cell Tumours Cysts and Tumour-like Lesions Tumours of the Sellar Region Local Extensions from Regional Tumours Metastatic Tumours Unclassified Tumours Illustrations Subject Index Metat Cy Tu

Histological Typing of tumours of the Central Nervous System

SIR,-The editorial by Dr Tony Smith' reviews the European Commission document III/8267/89 but not the commission's proposals (111/3603/90EN) of February 1990. These sweeping proposals should alert doctors and their patients to the powerful centralised bureaucracy which, it is proposed, should be established for the approval of new medicines in the European Community. Document III/3603/90-EN proposes a centralised procedure for approving products from biotechnology (and other important new drugs if the producers wish) and a decentralised machinery for other drugs. Thus the centralised procedure will handle the new drugs for which the risk of unexpected adverse effects is greatest. In both procedures ifmember states \"are unable to reach a bilateral agreement, the matter will be referred for binding arbitration at Community level.\" Thus the proposal is for absolute power overriding national sovereignty in a sensitive aspect of public health. With respect to monitoring adverse drug reactions quite impractical timetables are proposed with a requirement that all information should be passed to companies. This would break the confidentiality of the yellow card scheme and seriously damage our only fully national method of postmarketing surveillance. It is important that these proposals should not be agreed and that doctors alert themselves to this issue.

https://www.bmj.com/content/bmj/301/6743/122.4.full.pdf

Shy-Drager syndrome presenting as isolated paralysis of vocal cord abductors.

Stenotic malformations of the internal auditory meatus (IAM) are rare. They are known to symptomatically mimic vestibular schwannomas leading to potential diagnostic error. We present a case (along with literature review) where a stenotic IAM was clinically and radiologically misdiagnosed as a vestibular schwannoma.

Stenosis of the internal auditory meatus masquerading as bilateral vestibular schwannomas: a cautionary tale.

Clivus meningioma is an uncommon tumour arising from the region of the synchrondrosis between the basal portions of the sphenoid and occipital bones. We report a case of a patient with a large meningioma of the clivus whose presenting symptom was auditory change. Alteration of hearing is a rare mode of presentation for such a tumour although it may present at a later stage in about 50 per cent of cases. The discrepancy between the size of the tumour and the paucity of physical findings, the value of a multiple test auditory screening strategy, and the surgical approach in this case are discussed.

An unusual posterior cranial fossa tumour causing hearing loss.

/pdf/patient-satisfaction-in-ear-surgery-xr4o4iwb2l.pdf

Richard T. Ramsden

Papers

Shy-Drager syndrome presenting as isolated paralysis of vocal cord abductors.

Stenosis of the internal auditory meatus masquerading as bilateral vestibular schwannomas: a cautionary tale.

An unusual posterior cranial fossa tumour causing hearing loss.

Patient satisfaction in ear surgery.

Middle earfunction inrheumatoid arthritis