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Moving pictures of Parkinson's disease

2011; Elsevier BV; Volume: 378; Issue: 9805 Linguagem: Inglês

10.1016/s0140-6736(11)61765-0

1474-547X

Anne Jeanjean, Geneviève Aubert,

Neurology and Historical Studies

Today, video is the ideal way to record the clinical features of Parkinson's disease; it is widely used as a teaching aid and to discuss difficult cases. From the earliest clinical reports, visual images have characterised medical accounts of Parkinson's disease. What are the iconographic landmarks of this visually so striking disease? The modern history of Parkinson's disease begins with an essay, published by James Parkinson in London in 1817. Although several of his other works are embellished with frontispiece, prints, woodcuts, and engravings, his 1817 essay does not include images. In one paragraph, Parkinson describes the clinical picture of shaking palsy (paralysis agitans): “Involuntary tremulous motion, with lessened muscular power, in parts not in action and even when supported; with a propensity to bend the trunk forward, and to pass from a walking to a running pace: the senses and intellects being uninjured”. Parkinson illustrates this description with six case sketches, and as an early example of field neurology his seminal descriptions are anchored almost exclusively in visual inspection. The first clinical images of patients with this disease come from Jean-Martin Charcot and his associates at the Salpêtrière hospital in Paris. Besides leaving us graphic illustrations of this illness, Charcot had a major role in defining it as a distinct neurological disorder. In acknowledgment of Parkinson's first description, he proposed to call this syndrome Parkinson's disease. This renaming was informed by several considerations. First, Charcot underlined that the term paralysis was inaccurate because muscular strength is generally not decreased, at least for a long time in the evolution of the disease. Second, the French word paralysie had an ominous connotation to the layman, evoking paralysie générale, or general paresis, with its dreaded accompaniment of dementia. Finally, although Charcot recognised that shaking or tremor was a major manifestation of the disease, he emphasised that it was not present in all patients and thus not required for the diagnosis. During his famous Tuesday lessons, before an assembly of physicians, interns, and students, Charcot taught his audience to observe patients standing, walking, and performing various manoeuvres. On several occasions, he presented patients with Parkinson's disease. He then elaborated on the other cardinal features of the disease: the slowness in executing movements and the muscular stiffness responsible for the masked facial expression, frozen posture and attitude. An avid sketcher, Charcot occasionally drew his Parkinsonian patients. He was fortunate to have as a medical assistant Paul Richer who was also a talented artist. Thanks to his dual background, Richer could artistically translate pathological clinical features. His legacy in the iconography of Parkinson's disease includes two-dimensional depictions of patients with Parkinson's disease—vivid pencil or pen and ink drawings and engravings—alongside three-dimensional renditions of stunningly realistic figurines. In 1878, Charcot opened a photographic laboratory at the Salpêtrière. Albert Londe, a skilful photographer, became the key figure in this department. In close and friendly collaboration with Richer, Londe captured the abnormal postures of Parkinsonian patients. With his interest in the anatomy and physiology of the moving human body, Richer was not, however, satisfied with these static representations. At that time, the photographer Eadweard James Muybridge and the French physiologist Etienne-Jules Marey were pioneering the analysis of movement with different techniques of time lapse photography and chronophotography. Assisted by an American physician, Francis Xavier Dercum, Muybridge recorded patients with various gait disorders or abnormal movements in his masterwork, Animal Locomotion, published in 1887. Oddly enough, no patients with shaking palsy are included in this volume. Prompted by Richer's interest in this approach, Londe built several types of multiple-lens camera. With these devices, they obtained series of consecutive photographs allowing them to analyse the typical gait of patients with Parkinson's disease. Around 1895, the world discovered cinematography with the first public screening of films in Paris. Although the French medical establishment viewed this new medium with a degree of suspicion, Londe experimented with the new technique and, together with Richer, filmed at least one patient with Parkinson's disease, but only isolated blurred frames have survived. These early films were made of cellulose nitrate, a highly unstable organic material, which is why most films from the beginning of cinematography have been irretrievably lost. The oldest surviving film of patients with Parkinson's disease was taken in Romania. With the recently developed techniques of chronophotography and cinematography, Gheorghe Marinescu studied normal and abnormal gait. He published several papers on organic or hysteric hemiplegia, paraplegia, and ataxia, which were illustrated with drawings after the stills. Among the retrieved films, a short sequence of 12 seconds shows a group of lined up patients walking abreast with the typical gait and posture of Parkinson's disease. An extensive collection of archival neurological films does exist: these were made by the Belgian anatomist and neurologist Arthur Van Gehuchten (1861–1914). At the beginning of the 20th century, Van Gehuchten recognised the potential of cinematography for capturing signs of neurological diseases as a didactic and documentary support of the medical record. He decided to make a comprehensive cinematographic collection about patients with nervous diseases, and, from 1905 on, he filmed intensively. Van Gehuchten personally dealt with all the steps of the process from shooting to screening. He did not hesitate to go to the patients' home to film them. He developed the exposed footage himself, selected the sequences, and finalised the montage. A pioneer of multimedia teaching, he enlivened his lectures for medical students or at scientific meetings with photographic and cinematographic projections. An important part of this collection has survived and is kept at the Cinematek (Royal Belgian Film Archive) in Brussels. It amounts to about 3 hours of short sequences, ranging from a few seconds to a few minutes. Van Gehuchten's scientific, clinical, educational, and cinematographic labour was cut brutally short with his untimely death in 1914, at the age of 53 years. He had just completed his textbook Les Maladies Nerveuses, which was published posthumously in 1920. It is richly illustrated with photographs and stills from film strips. Van Gehuchten discussed Parkinson's disease extensively in his textbook, devoting 13 pages of text and ten illustrations to the topic. Five figures can be matched with surviving film footage. Two other illustrations show samples of tremor-affected writing and micrographia. Van Gehuchten did not consider secondary forms of Parkinsonism. Indeed, the epidemic of encephalitis lethargica had not yet swept the world and drugs, such as neuroleptics and other dopamine antagonists known to induce Parkinsonism, had not yet been synthesised. Van Gehuchten's collection includes film clips of 12 patients with Parkinson's disease. Far better than a drawing or a photograph, these films perfectly catch and render the archetypal clinical features of the disease (see webvideos 1–6). Bradykinesia is conspicuous in all patients, with all degrees of severity of slowness, hesitancy, and poverty of movement. The tremulous agitation, mostly of the limbs, but sometimes of the whole body is abundantly documented. Conspicuous tremor of chin, lips, and tongue is exemplified in a close-up of one patient. No dyskinesias, chorea, or dystonia are observed in any film. Gait is illustrated with the patient usually walking towards and away from the camera. The axial rigidity and flexed posture of trunk, arms, and legs, lack of arm swing, together with slowness of stride and shuffling are all well exemplified. Freezing of gait on starting or on turning is recorded. Also noticeable are festination or stuttering movements when turning, with a tendency to propel backwards or forwards. In one patient, a lateral tilt of the trunk is particularly highlighted by filming the patient spinning around. A masked facies with decreased blinking is observed in all patients, ranging from minimal to severe with a typical stiff frozen poker-faced expression. This does not preclude two patients from smiling at the camera and their physician. On watching these films we felt ties of compassion and confidence between the physician modestly hidden behind the camera and his silent patients, often filmed in their familiar surroundings like a garden or a farmyard. These extraordinarily well-preserved films combine a precise iconography of Parkinson's disease with outstanding historical interest, all the more so as the clinical features documented here are those of patients filmed before the advent of dopa therapy. Dopa, the first rational and most effective symptomatic treatment of Parkinson's disease, led to a new era of treatment. In 1967, Georges Cotzias reported the improvement of several manifestations of Parkinsonism by dopa. However, it soon became clear that long-term therapeutic benefits were hampered by motor fluctuations and the appearance of various involuntary movements. These early reports were documented by cinematographic records. In the 21st century, video recordings remain an essential part of the patient file and in the evaluation of new therapeutic approaches such as enteral L-dopa or deep brain stimulation. As long as we do not have a cure for this disabling movement disorder, we will be haunted by these moving images. Download .mov (15.92 MB) Help with mov files Webvideo 1The first sequence in a garden, shows the patient walking. The axial rigidity and flexed posture are somewhat asymmetric with a lateral tilting of the trunk towards the right. The semi-flexed posture of arms, held tight to the chest, is typical. The hands are close together in front of the trunk, the forefingers almost touching each other, and there is a complete loss of arm swing. Bilateral hand tremor is conspicuous. The next sequence shows the patient in a courtyard, standing and spinning around. This particularly highlights the general abnormal posture, the flexion of the whole upper part of the body and the lateral tilt of the trunk. The next sequence presents the same patient, probably somewhat later; the abnormal posture and slowness of movements are more pronounced. Now we see him sitting quietly in a chair facing the camera; bilateral rest tremor of all four limbs is impressive. Finally, when spinning around, tremor of the arms is more marked on the left. In the different sequences, the patient seems completely at ease and even smiles to the camera. Two figures in Van Gehuchten's textbook, Les Maladies Nerveuses show this patient. Download .mov (8.06 MB) Help with mov files Webvideo 2Conspicuous tremor of chin, lips, and tongue is particularly exemplified in a close-up of this patient. Throughout the whole sequence, a masked facies with fixed gaze and decreased blinking is observed. One figure in Van Gehuchten's textbook, Les Maladies Nerveuses shows this patient. Download .mov (11.13 MB) Help with mov files Webvideo 3This sequence is filmed in Van Gehuchten's usual setting for indoor shots, in a hospital room well-lit by large windows. The patient sits on a chair facing the camera. He gets up with difficulty but without the help of the accompanying nun. He then walks with very short steps towards the camera. Start hesitation is noticeable. The patient then turns around and walks away from the camera. Turning en bloc, freezing of gait on turning and on starting, and stuttering movements when turning, are illustrated. Postural instability is conspicuous, as well as a general slowness and poverty of movements (body bradykinesia), more marked on the right side. A close-up on the hands highlights the semi-flexed position of the forearms and the tremor, more marked on the right side. Download .mov (16.39 MB) Help with mov files Webvideo 4This young patient is filmed in a courtyard, in front of a large door. The first sequence, a close-up of the face shows a loss of facial expression with decreased blinking, and chin tremor. In a second sequence, the patient walks backwards and forwards in front of the camera, with short stiff steps and turns en bloc in several steps. Axial rigidity is conspicuous as well as complete loss of arm swing. The attitude of the upper arms is peculiar: the arm are in slight abduction, the forearms semi-flexed with the hands in flexion. A last sequence highlights bilateral hand tremor in close-up. Download .mov (10.32 MB) Help with mov files Webvideo 5We are in a garden. It is a profile view of a man sitting on a chair. He stands up, walks three steps toward another chair, sits down and goes back again in a stereotyped toing and froing. The stooped attitude, trunk bent forward, is characteristic; we can speak here of camptocormia. The leaning movement of trunk is increased when the patient stands up and begins to walk. According to the time-honoured expression, the patient seems to run after his gravity centre; it is known as the anteropulsion movement. The upper limbs are asymmetric : the left arm is frozen in semi-flexion, held tight to the chest, and there is a tremor of the left hand. The right arm is definitely more supple. It is this right arm that the patient uses to aid himself in sitting or standing. Download .mov (10.12 MB) Help with mov files Webvideo 6A young nun is accompanied by an elderly colleague. The first sequence shows her walking towards the camera, turning back and walking away. Complete loss of arm swing is clear. The second sequence shows the patient in three-quarter position, quietly standing, with rest tremor of right wrist. During this sequence the patient has an animated conversation and smiles.