450 www.thelancet.com/neurology Vol 13 May 2014

In Context

LifelineBart van der Worp is a neurologist at the University Medical Centre in Utrecht, Netherlands. As a clinical investigator of the Dutch Heart Foundation, one focus of his research is temperature management to improve outcome after stroke. Another interest is identifi cation of potential sources of bias in animal stroke studies to improve translation from bench to bedside.

If you had not entered your current profession, what would you have liked to do?Write novels. Like possibly 10% of the population, I think I have the talent but not the time.

Who was your most infl uential teacher, and why?Jan van Gijn, an eminent clinician, researcher, and teacher, who introduced evidence-based medicine to neurology in the Netherlands in the early 1980s.

What would be your advice to a newly qualifi ed doctor?Read Samuel Shem’s The House of God. Almost four decades after its publication, the book still contains valuable lessons, and many of its so-called laws are still valid.

What is your favourite fi lm, and why?Pulp fi ction by Quentin Tarantino, because of its unconventional structure and the superb dialogue.

What keeps you awake at night?Underfunding and over-regulation of academic clinical trials. And slow recruitment.

What is your worst habit?I annoy PhD students with my uncontrollable urge to rewrite considerable parts of the texts they have written. Regrettably, the same happens to my texts when they are reviewed by a British coauthor.

What was your fi rst experiment as a child?Impressed by the numbers of leaves falling to the ground each autumn, and unaware of the carbon cycle as an 8-year old, I aimed to test whether the earth gradually increased in diameter. I grew beans in a large glass jar, let the plants rot, and measured the soil’s thickness at weekly intervals. The experiment ended when my mother decided that there was no room for a compost heap in our house.

What is the most memorable comment from your school reports?In the third grade of secondary education, my English teacher noted that my marks could be as good as those of my younger sister if I would at least make an eff ort. Remarkably, this comment had the desired eff ect.

What was the most memorable comment you ever received from a referee?In 2003, an angry exposition that fl owed over two pages of A4, explaining that the value of animal experiments for stroke was self-evident and asking how I had dared to question the validity of some experiments.

Focal pointTackling ataxiaBefore her untimely death, just as she was poised to take over as head of the Department of Clinical Neurology at the Institute of Neurology (London, UK), Anita Harding (1952–95) was a woman of many fi rsts: she was the fi rst female professor of clinical neurology in Britain; and out of her neurogenetics laboratory at the Institute of Neurology came the fi rst description of pathogenic mitochondrial DNA mutations in Kearns-Sayre syndrome1 and the fi rst report of prenatal diagnosis of NARP (neurogenic weakness, ataxia, and retinitis pigmentosa) based on mitochondrial DNA analysis.2

Harding, who was brought up in Birmingham, UK, studied medicine at the Royal Free Hospital Medical School (London, UK), qualifying in 1975. She followed her studying with periods as house physician to Professor Peter K Thomas, who would later become her husband, and at the Medical Research Council Clinical Genetics Unit at the Institute of Child Health (London, UK). As an early advocate for the potential of recombinant DNA technology as a way to uncover the pathophysiology of the disorders she saw in the clinic, she acquired the expertise in molecular genetics to enable her to set up her neurogenetics laboratory. Later, along with Mary Davis Goddard, she would establish one of the largest service laboratories for neurogenetic diseases in the UK, at the National Hospital for Neurology and Neurosurgery, London.

In collaboration with John Morgan-Hughes, John Clark, and the then graduate student Ian Holt,1,2 she blazed a trail in the fi eld of neuromuscular disorders and the understanding of the peripheral neuropathies, dystonias, mitochondrial disease, and the classifi cation of the hereditary ataxias.3,4

The obituaries of Harding make much of her joie de vivre, and her great humour is perhaps best exemplifi ed by her reaction on learning of her terminal bowel cancer; she is widely reported to have quipped “at least I won’t have to buy Windows 95”. 5

Steven Goodrick1 Holt IJ, Harding AE, Morgan-Hughes AJ. Deletions of mitochondrial DNA

in patients with mitochondrial myopathies. Nature 1988; 331: 717–19.2 Harding AE, Holt IJ, Sweeney MG, Brockington M, Davis MB. Prenatal

diagnosis of mitchondrial DNA8993 T→G disease. Am J Hum Genet 1992; 50: 629–33.

3 Veláquez-Pérez L, Rodríguez-Labrada R, Canales-Ochoa N, et al. Progression of early features of spinocerebellar ataxia type 2 in individuals at risk: a longitudinal study. Lancet Neurol 2014; 13: 482–89.

4 Harding AE. Clinical features and classification of the inherited ataxias. Adv Neurol 1993; 61: 1–14.

5 Poulton J and Huson SM. Anita Harding (1952–95): In memoriam. Am J Human Genet 1996; 58: 235.

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