NrurojLihror?2atoses: Pathological and Clinicul Ovcrvkv,

by S.M. Huson and R.A.C. Hughes (editors), 1993, Chapman & Hall, London, E79.00. ISBN 0 412 38920 7

After a long period in which medical interest in the ‘phakomatoses’

remained both rather dormant and restricted, the recent decades have

witnessed a revival of biomedical research into these disorders. Did

volume 14 of the Handbook of‘ Clinical Nrurology in 1976 provide a

synthesis of knowledge about all of them, in 1986 the New York Acad-

emy of Sciences organized meetings from which arose this volume on

‘Neurofibromatosis’, the genetic anchors of which have been defined.

The present volume edited by Huson and Hughes testifies to the

happy flight research has taken. In a magnificently produced book. 25

specialists have contributed chapters that make delightful reading no

clinician. and in particular no neurologist. must deny him- or herself.

Chapters on features clinically distinguishing neurofibromatosis types

I and II (and their overlap); on the molecular work identifying the

causative gene sites on chromosomes 17 and 22. respectively; on the

embryology and biology of crest cells and Schwann cclla: and 011 111c

neuropathology form a firm basis to engrave the pathogcnesl\ on the

reader’s mind. This is followed by chapters covering the r’ntirc range

of clinical manifestations, the epidemiology, and medical and aurglcai

treatment. Fascinating chapters exposing the diagnostic myth about thr*

‘Elephant Man’ as well as the likely diagnosis of rhc diaordcr hu hatI

(Proteus syndrome), and finally variants and related diseases ciosc thix

volume which merits compliments to the editors. [he authors. and rhc

publishing house.

The text is to the point and lucid. the many illustrations hate been

well chosen, the references have been selected on their pertlnencc. the

index is adequate, and, finally, the price is reasonable.

K.P.M. Bruyn, MD

Department of Neurology

Oudenrijn Hospital

Utrecht. The Netherlands