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- Shafer, Audrey
This posthumously published collection of essays by Dr. Klawans, an eminent neurologist and writer, explores the interactions between patient, family and neurologist and the implications of specific neurologic diseases. Klawans's special interest in neurology is movement disorders, such as Huntington's chorea and Parkinson's disease, but his outside interests range from evolutionary biology to classical music. His essays, therefore, focus on single patients or families, but the author weaves thoughts about his other interests into each "case."
The book is divided into two sections, "The Ascent of Cognitive Function" and "The Brain's Soft Spots: Programmed Cell Death, Prions, and Pain." In a brief preface, Klawans declares that this book is "more than just a set of clinical tales about interesting and at times downright peculiar patients" from his 35 years of practice, but rather it "humbly grapples with the 'whys' of our brain, not the 'hows.'" (pp. 9-10) In the preface, as well as in one essay, Klawans acknowledges the work and impact of fellow neurologist-writer Oliver Sacks ("Oliver is truly the father of us all." p 12).
The title essay concerns a six-year-old girl who was found, locked and completely speech-deprived, in a closet. Because she is still within the window of opportunity for language acquisition, "Lacey" quickly learns to speak, unlike Victor, the Wild Boy of Aveyron, whose story was immortalized in the François Truffaut film, L'enfant Sauvage. Klawans uses these stories as a launch pad to discuss the evolution of language, including a proposal that the cavewoman, not the man, was responsible for development of the human species as she taught her offspring language.
Other chapters focus on patients with epilepsy, Parkinson's disease, localized and hemispheric stroke, "painful-foot-and-toe syndrome, " and Creutzfeldt-Jakob disease. Two particularly memorable chapters concern Huntington's chorea and Refsum's disease. The chapter, "Anticipation," explores the profound ethical concerns of genetic testing for Huntington's chorea as applied to three generations of one particular family. In the chapter, "The Hermit of Thief River Falls," Klawans recollects his first year as a neurology resident, and his care of a reclusive patient with a rare eponymous illness, Refsum's disease--just in time for a visit by Refsum himself, a famous Norwegian neurologist.
The book concludes with a speculative "afterthought" about genetics, evolution, and the importance of extended "juvenilization" --the protracted post-natal development of Homo sapiens. This essay intertwines some of the threads regarding speech development and evolutionary biology, particularly brain development, that were introduced earlier in the text.