In Context
Focal Point Apathy: who cares? Stoicism, an ancient Hellenistic school of thought, advocated the development of self-control to overcome the irrational destructive emotions that interfere with logical reasoning. Apatheia (from a-pathos, without passion) referred to the ideal of stoic calm. Apathy entered medical nomenclature when Édouard Brissaud first described apathie in Parkinson’s disease.1 He underlined the importance of an accurate interview to reveal apathy, and emphasised that “the majority of parkinsonian patients—not to say all—seem indifferent to everything around them … Closed in themselves, they appear indifferent to this world, and their apathy seems absolute”.1 The notion of apathy as a clinical syndrome was built on case reports and lesional studies that described patients with preserved cognition but a striking poverty of spontaneous active behaviours. François Naville introduced the term “bradyphrenia” to describe the absence of interest, initiative, and concentration noted in postencephalitic parkinsonism after the historical pandemic encephalitis lethargica. Patients were reported “to sit and do nothing unless exhorted to rouse themselves”.2 Oliver Sacks described the “awakening” from such a chronic lethargic state when levodopa was introduced for Parkinson’s disease.3 Loss of auto-activation was noted in patients with psychic akinesia who had no spontaneous mental activity, but “could nevertheless be engaged in complex behaviours with almost normalised cognitive performance in reaction to external stimulation”.4 Robert Marin pointed out the absence of interest of the medical community in apathy, despite its high prevalence across many brain disorders. His operative definition of apathy and the creation of the first rating scale5 stimulated research on the neurobiological correlates and clinical relevance of apathy in neurodegenerative diseases.6
Paul Krack, Javier Pagonabarraga, Antonio P Strafella, Jaime Kulisevsky 1
2 3 4
5 6
Brissaud E. Vingt-troisième leçon. Nature et pathogénie de la maladie de Parkinson. In: Meige H, ed. Leçons sur les maladies nerveuses: la Salpêtrière, 1893–1894. Paris: Masson, 1895: 488–501. Naville F. Etudes sur les complications et les sequelles mentales de l’encephalite epidemique. Encephale 1922; 17: 369–75. Sacks OW. Awakenings. London: Picador, 1973. Laplane D, Baulac M, Widlöcher D, Dubois B. Pure psychic akinesia with bilateral lesions of basal ganglia. J Neurol Neurosurg Psychiatry 1984; 47: 377–85. Marin RS. Differential diagnosis and classification of apathy. Am J Psychiatry 1990; 147: 22–30. Pagonabarraga J, Kulisevsky J, Strafella AP, Krack P. Apathy in Parkinson’s disease: clinical features, neural substrates, diagnosis, and treatment. Lancet Neurol 2015; 14: 518–31.
www.thelancet.com/neurology Vol 14 May 2015
Lifeline Ronit Pressler qualified in medicine at the Free University Berlin (Berlin, Germany) in 1992, moved to London, UK, in 1995 to study for her PhD at King’s College London and, in 2008, became a consultant in paediatric clinical neurophysiology at Great Ormond Street Hospital, London, where she is clinical lead of the epilepsy monitoring unit. Her research focuses on the diagnosis and treatment of neonatal seizures. What has been the greatest achievement of your career? Coordinating the EU-funded NEMO project together with Geraldine Boylan, consisting of a fantastic team of neonatologists, neurologists, neurophysiologists, pharmacologists, clinical scientists, and statisticians.
See Articles page 469
And the greatest embarrassment? My spelling (I am dyslexic): “capri-dopa” (final medicine exams), “William the conker” (helping my son with his homework), and “data enteritis” (NEMO protocol). What do you think is the most neglected field of science or medicine at the moment? Drug development for babies, particularly in the area of neurology. What inspired you? My parents, and watching the athletes at the 2012 Paralympics in London overcome adversity. What is your greatest regret? When I was 16, I nearly ran away with a traveling circus. I sometimes wish I had… How do you relax? Travelling the world with my husband and sons—Noah and Joshua; failing this, going for a run along the Grand Union canal. What items do you always carry with you? A book, my phone, and enough change for a cup of coffee. If you could have dinner tonight with a famous person of your choice (dead or alive), who would it be? Janusz Korczak, paediatrician and children’s author, my childhood hero. If you were Bill Gates, how would you spend your fortune? Make school education for children compulsory and possible everywhere in the world. What keeps you awake at night? Nothing—since my boys stopped waking me on a regular basis. What would be your advice to a newly qualified doctor? Never give up: not on your patients or their parents, if you do paediatrics, and most of all not on yourself. What is your worst habit? Candy Crush. 465
Focal Point Apathy: who cares? Stoicism, an ancient Hellenistic school of thought, advocated the development of self-control to overcome the irrational destructive emotions that interfere with logical reasoning. Apatheia (from a-pathos, without passion) referred to the ideal of stoic calm. Apathy entered medical nomenclature when Édouard Brissaud first described apathie in Parkinson’s disease.1 He underlined the importance of an accurate interview to reveal apathy, and emphasised that “the majority of parkinsonian patients—not to say all—seem indifferent to everything around them … Closed in themselves, they appear indifferent to this world, and their apathy seems absolute”.1 The notion of apathy as a clinical syndrome was built on case reports and lesional studies that described patients with preserved cognition but a striking poverty of spontaneous active behaviours. François Naville introduced the term “bradyphrenia” to describe the absence of interest, initiative, and concentration noted in postencephalitic parkinsonism after the historical pandemic encephalitis lethargica. Patients were reported “to sit and do nothing unless exhorted to rouse themselves”.2 Oliver Sacks described the “awakening” from such a chronic lethargic state when levodopa was introduced for Parkinson’s disease.3 Loss of auto-activation was noted in patients with psychic akinesia who had no spontaneous mental activity, but “could nevertheless be engaged in complex behaviours with almost normalised cognitive performance in reaction to external stimulation”.4 Robert Marin pointed out the absence of interest of the medical community in apathy, despite its high prevalence across many brain disorders. His operative definition of apathy and the creation of the first rating scale5 stimulated research on the neurobiological correlates and clinical relevance of apathy in neurodegenerative diseases.6
Paul Krack, Javier Pagonabarraga, Antonio P Strafella, Jaime Kulisevsky 1
2 3 4
5 6
Brissaud E. Vingt-troisième leçon. Nature et pathogénie de la maladie de Parkinson. In: Meige H, ed. Leçons sur les maladies nerveuses: la Salpêtrière, 1893–1894. Paris: Masson, 1895: 488–501. Naville F. Etudes sur les complications et les sequelles mentales de l’encephalite epidemique. Encephale 1922; 17: 369–75. Sacks OW. Awakenings. London: Picador, 1973. Laplane D, Baulac M, Widlöcher D, Dubois B. Pure psychic akinesia with bilateral lesions of basal ganglia. J Neurol Neurosurg Psychiatry 1984; 47: 377–85. Marin RS. Differential diagnosis and classification of apathy. Am J Psychiatry 1990; 147: 22–30. Pagonabarraga J, Kulisevsky J, Strafella AP, Krack P. Apathy in Parkinson’s disease: clinical features, neural substrates, diagnosis, and treatment. Lancet Neurol 2015; 14: 518–31.
www.thelancet.com/neurology Vol 14 May 2015
Lifeline Ronit Pressler qualified in medicine at the Free University Berlin (Berlin, Germany) in 1992, moved to London, UK, in 1995 to study for her PhD at King’s College London and, in 2008, became a consultant in paediatric clinical neurophysiology at Great Ormond Street Hospital, London, where she is clinical lead of the epilepsy monitoring unit. Her research focuses on the diagnosis and treatment of neonatal seizures. What has been the greatest achievement of your career? Coordinating the EU-funded NEMO project together with Geraldine Boylan, consisting of a fantastic team of neonatologists, neurologists, neurophysiologists, pharmacologists, clinical scientists, and statisticians.
See Articles page 469
And the greatest embarrassment? My spelling (I am dyslexic): “capri-dopa” (final medicine exams), “William the conker” (helping my son with his homework), and “data enteritis” (NEMO protocol). What do you think is the most neglected field of science or medicine at the moment? Drug development for babies, particularly in the area of neurology. What inspired you? My parents, and watching the athletes at the 2012 Paralympics in London overcome adversity. What is your greatest regret? When I was 16, I nearly ran away with a traveling circus. I sometimes wish I had… How do you relax? Travelling the world with my husband and sons—Noah and Joshua; failing this, going for a run along the Grand Union canal. What items do you always carry with you? A book, my phone, and enough change for a cup of coffee. If you could have dinner tonight with a famous person of your choice (dead or alive), who would it be? Janusz Korczak, paediatrician and children’s author, my childhood hero. If you were Bill Gates, how would you spend your fortune? Make school education for children compulsory and possible everywhere in the world. What keeps you awake at night? Nothing—since my boys stopped waking me on a regular basis. What would be your advice to a newly qualified doctor? Never give up: not on your patients or their parents, if you do paediatrics, and most of all not on yourself. What is your worst habit? Candy Crush. 465
