Saba - Imaging in Neurodegenerative Disorders

Considerate la vostra semenza:
fatti non foste a viver come bruti,
ma per seguir virtute e canoscenza.

Consider your origin;
you were not born to live like brutes,
but to follow virtue and knowledge.

Dante Alighieri, Italian poet (12651321)
Divine Comedy
Canto XXVI, lines 118120.

Stilicidi casus lapidem cavat, uncus aratri
Continual dropping wears away a stone

Lucretius, Roman poet and philosopher (c.99 BCc.55 BC)
De Rerum Natura
Book I, line 313

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ISBN 9780199671618

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This book is dedicated to Tiziana

I am honoured to have been asked by Dr Saba to write one chapter for his fabulous new book in addition to writing this foreword. A foreword is generally an introduction that describes a book and specifically its subject, contents, scope, and aims. So here I go.

Subject: As its title clearly states, this book deals with degenerative disorders of the brain and spinal cord. However, it goes much further than what one would expect from its title. Keep reading.

Contents: Authors from 13 different countries have contributed 35 chapters dealing with all imaginable aspects of neurodegeneration. The contents of these chapters go beyond imaging to provide a clear, concise, and complete framework with which to understand these important and often neglected disorders.

Scope: The book begins with five chapters on the clinical aspects of degenerative disorders of the central nervous system, including their genetics and economic impact. Various techniques, current and future, that are and will be used to evaluate these disorders are described in the next six chapters. The following 17 chapters are each dedicated to one disorder or a group of disorders and are complemented by another describing their differential diagnoses. The often overlooked topic of post-traumatic neurodegeneration is well explained in three chapters arranged by anatomical regions. The last four chapters deal with a variety of topics but concentrate on different treatments and their imaging.

Aims: What Dr Saba set out to accomplish is to give neuroradiologists, neurologists, and other interested specialists a book that, while concentrating on imaging, is wide enough in its approach and contents to satisfy and enlighten all of those interested in neurodegeneration. I believe that the book you hold in your hands has clearly accomplished this goal.

Yes, there are other tomes on the same topic. Some, however, cover clinical aspects, molecular genetics, and/or biomarkers while relegating neuroimaging to a lesser status (or ignoring it completely). Others deal with specific topics such as proteins, inflammation, inhibitor molecules, and protein aggregates and although they also contain wonderful information they are far away from what we clinicians do and what we require in order to take care of our patients. That is the strength of Dr Sabas book: it contains enough basic science to make it illuminating while emphasizing the imaging and clinical aspects of these diseases. Read it, enjoy it, and learn from it.

Mauricio Castillo
Professor of Radiology and Chief of Neuroradiology,
University of North Carolina at Chapel Hill
Editor in Chief, American Journal of Neuroradiology
May 2014

Neurodegenerative diseases are characterized clinically by their insidious onset and chronic progression. Neurodegenerative disease clinical syndromes are often categorized by whether they initially affect cognition, movement, strength, coordination, sensation, or autonomic control. Frequently, however, patients will present with symptoms and signs referable to more than one system. Either involvement of several systems can occur concomitantly, or else by the time the patient has functionally declined enough to seek medical attention multiple systems have become involved. While the term neurodegenerative implies it is the loss of neurons that cause disease, it is possible that neuronal demise is merely the final stage of a preceding period of neuronal dysfunction. It is difficult to know whether clinical decline is associated with actual neuron loss, or with a period of neuronal dysfunction that precedes neuron loss. Also, particular neurodegenerative diseases are aetiologically heterogeneous. In addition to syndromically defining neurodegenerative diseases by what neuroanatomical system is involved, these disorders are broken down along other clinical lines. Early (childhood, young adulthood, or middle age) versus late (old age) onset is an important distinction. Some clinically similar neurodegenerative diseases are subcategorized by their age of onset, despite the fact at the molecular level different forms of a particular disease may have very little in common. Sporadic onset versus Mendelian inheritance constitutes another important distinction, and many named neurodegenerative diseases have both sporadic (Mendelian inheritance is not recognizable) and Mendelian subtypes.

Neurodegenerative disorders constitute a group of frequently diagnosed conditions in neurological clinics, but diagnosing these diseases can prove particularly intimidating to clinicians, because often the diagnosis cannot be critically confirmed by a simple test. New imaging modalities have advanced to the point of high resolution, morphological, metabolic, and functional analysis. CT, MRI, nuclear medicine, and molecular imaging have recently emerged as outstanding non-invasive techniques for the study of the neurodegenerative disorders.