Hereditary Peripheral Neuropathies

Hereditary Peripheral Neuropathies deals with the Charcot-Marie-Tooth group of neuropathies and related primary hereditary neuropathies. The knowledge in this field has grown exponentially during the last ten years. The book is divided into two sections. The first section deals with the clinical presentation, electrophysiological features and differential diagnosis of these disorders as well as with the general biology of the peripheral nerve. The second section gives a detailed account of the known disease entities. The book will be interesting for both the clinician with a special interest in PNS diseases as well as for the researcher. TOC:Structure and function of the normal peripheral nerve.- Approach to the patient with suspected hereditary neuropathy.- Clinical evaluation. Electrodiagnostic evaluation. Differential diagnosis. Principles of pathology and nerve biopsy. Specific Neuropathies.- Overview of the Classification and Genetics (incl. full description of rare forms). CMT1 and HNPP. CMT2, dominant intermediate CMT and CMTX. Distal HMN. HSAN. Hereditary neuralgic amyotrophy. Genetic testing (tests and strategies). Genetic counselling. Medical treatment. Surgical treatment. Animal models. Appendix (adresses of self-help groups, laboratories, neurologic and surgical centers).