Rating Parkinson's Disease: A Contemporary Eye to Archival Patients

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Authors: Anne P. Jeanjean MD, PhD and Genevieve Aubert MD, PhD

Article first published online: 31 JUL 2014 | DOI: 10.1002/mdc3.12078


 

Today, a careful medical history and clinical examination remain the basis for the diagnosis of Parkinson's disease (PD).[1, 2] This neurodegenerative condition is characterized by motor symptoms and signs, as well as by cognitive, autonomic, and psychiatric features, sleep disturbance, and sensory symptoms. The major motor features of PD consist of the four hallmarks—tremor, rigidity, bradykinesia, and postural instability—also referred to as parkinsonism.

Rating PD is important in order to follow-up patients and assess new treatments. Detailed clinical scoring tools have been developed. The most utilized is the UPDRS,[3] which was revised in 2008. This new version, which was sponsored and commissioned by the International Parkinson and Movement Disorder Society (MDS), is termed the MDS-sponsored UPDRS revision (MDS-UPDRS).[4] The detailed assessment still prioritizes the motor aspects of the disease, with the motor examination accounting for half of the total score. The MDS-UPDRS is intented to be used worldwide. Non-English versions in several languages have been recently proposed.

Arthur Van Gehuchten (1861–1914), the first Belgian professor of neurology, recognized the potential of cinematography for didactic purposes and documentary support.[5, 6] He filmed extensively and conceived the plan of a systematic cinematographic atlas of neurological diseases between 1905 and 1914. Twelve different patients with possible or probable PD are illustrated in this film archive.[7] We present here these original film clips and, in view of the universal interest of MDS-UPDRS, we analyze these one-century-old films with a contemporary eye inspired by the MDS-UPDRS.

Patients and Methods

Van Gehuchten's films are kept at the Cinematek (Royal Belgian Film Archive [CRB], Brussels), courtesy of the descendants of Arthur Van Gehuchten, under the scientific authority of one of the authors (G.A.). The original 35-mm nitrate films have been restored by the CRB where they are kept, together with later copies in 16-mm film. This collection amounts to approximately 3 hours of short sequences, ranging from a few seconds to a few minutes. An acetate copy of all the films was obtained and digitalized. The two authors independently searched the complete film collection for sequences showing patients with possible PD. Twelve different patients with possible or probable PD were identified. Videos 1 to 12 are available on our website (www.arthurvangehuchten.be). For each patient, the best copy, technically speaking, was chosen (Table 1).

Table 1. Data on patients and films
Patient No. 1 2 3 4 5 6 7 8 9 10 11 12
  1. a

    Carer: an accompanying supporting person is seen on the film.

  2. b

    Figure in VG's book: Figure number in Van Gehuchten's textbook Les Maladies Nerveuses.[8]

  3. na, not available.

Gender M M M M M M M na M M M F
Duration of film(s) 50 24 42 72 53 36 32 38 21 14 49 35
Nude                 +      
Carera     +                 +
Figure in VG's textbookb 389–390 396                 393–394  
Standing sequence + + + + + +   +     + +
Sitting sequence +     +                
Arising from chair sequence     + +     +          
Walking sequence +   + + + + +   + + + +
Turning sequence +   + + + +     + + + +
Close-up face   +   + + +           +
Close-up hands +   + + + +   +     + +
 

The complete published work of Van Gehuchten, papers and books, was searched for mention of PD. Van Gehuchten's neurological textbook was first published posthumously, after the end of World War I, in 1920. The archives of the Université Catholique de Louvain retain the second part of the original handwritten manuscript of this work, which contains the chapter dedicated to PD. The selected films were matched to the figures in the first published edition of Van Gehuchten's textbook ([8]; the complete text, as well as the manuscript, are available on the website: www.arthurvangehuchten.be). Unfortunately, we do not have detailed files with history, signs and symptoms, and complete clinical examination of each patient. Neither do we have any information on duration and progression of illness, nor on treatment. This explains that, according to UK Parkinson's Disease Society Brain Bank clinical diagnostic criteria, a definite diagnosis cannot be assessed because we lack supportive prospective positive criteria. Thus, we will consider the patients shown in these films as possible or probable PD. It must be reminded that, in 1914, at the beginning of WWI, the German invaders burned down many buildings in the center of Louvain, including Van Gehuchten's house. All his personal things, books, documents, and files, disappeared in the fire.

Given the age of the films, it was impossible to provide patients' consent and to suppose that the patients on the sequences could be identified.

The films were scrutinized with a specific look at the illustrated semiology. The MDS-UPDRS part III was the inspiration in the assessment and rating of the motor signs (Table 2). Part I and II, as well as part IV, were not evaluable because they are based on the patients' medical history. Given that the MDS-UPDRS has many items with a specific protocol for their assessment, full scoring was impossible. However, it was possible to score many items in most patients, because the patients were moving spontaneously, sometimes in their home environment. The two authors rated the films independently. In addition, Christopher Goetz, an internationnally known expert in movement disorders and MDS-UPDRS scoring, independently gave his experienced opinion. When the score was not congruent, the authors had a second look together at the films and reached a final score by consensus.

Table 2. Motor signs shown in the films
Patient No. 1 2 3 4 5 6 7 8 9 10 11 12
  1. Motor signs rated according to MDS-UPDRS. When difficult to assess we chose the minimal score.

  2. ur, unable to rate

Facial expression 2 4 3 4 2 2 ur ur ur ur ur 1
Arising from chair ur ur 1 0 ur ur 1 ur ur ur ur ur
Gait 2 ur 2 2 2 1 2 ur 2 2 2 1
Freezing of gait 0 ur 2 1 0 0 0 ur 0 0 1 0
Posture 3 ur 3 2 2 1 4 ur 2 2 2 1
Body bradykinesia 3 ur 3 2 2 2 2 ur 2 2 2 2
Postural tremor of right hand ur ur ur ur ur 2 ur 3 ur ur ur ur
Postural tremor of left hand ur ur ur ur ur 4 ur 3 ur ur ur ur
Rest tremor of right hand 4 ur 3 4 3 3 0 2 1 2 2 3
Rest tremor of left hand 3 ur 2 4 3 3 3 2 1 2 3 2
Rest tremor of feet/legs 4 ur ur 2 ur ur ur ur ur ur ur ur
Rest tremor of lip/jaw 2 2 ur 4 2 ur ur ur ur ur ur 0
 

Results

Altogether, we found film clips of 12 different patients with possible or probable PD (Fig. 1). Table 1 itemizes the characteristics of patients and films. Ten patients are clearly male. Only 1 patient is a female, and she is a nun. One patient in a black garment may be either a woman or a priest in cassock. Age ranges approximately from 40 to 70. Three patients are filmed inside the hospital, always in the same room, well lit by large windows, whereas the others are filmed outdoors, in different settings, some probably at home. One patient is filmed completely nude. Ten patients are filmed alone. Two are accompanied by a nun. One patient (patient 1) was apparently filmed twice on two different occasions because he wears different clothes in the two sequences. Because the overall clinical function was similar in the two films, we combined the two sequences for analysis.

Figure 1. A frame from each patient's film. Patients are labeled by a number from 1 to 12, from top left to bottom right. Full videos are available on the website: www.arthurvangehuchten.be.






 

Table 2 summarizes the motor signs displayed by the patients and their rating severity.

A masked facies with decreased blinking is observed in 7 patients, ranging from minimal to severe with a typical stiff frozen poker-faced expression in 2. Two patients (1 and 12) are shown smiling, looking at ease in front of the camera.

Arising from a chair is filmed in 3 patients.[1, 3, 4] This maneuver is normal in 1 and abnormal in 2, particularly in patient 3, who gets up slowly with some hesitation and a tendency to fall backward, but is still able to get up without help.

Gait is illustrated in 10 patients, most often with the patient walking toward and away from the camera. All patients are able to walk without assistance. The semiflexed posture of arms and legs, the lack of arm swing, and the slowness of stride and shuffling is particularly well exemplified in patient 3. Patient 7 is filmed in a profile view, with the patient walking from left to right and returning. In this condition, the typical flexed posture of trunk is particularly obvious. Some elements of freezing of gait either on starting or on turning, or on both, are illustrated in 3 patients. Start hesitation is particularly noticeable in patient 3. Turning en bloc is visible in several patients. Also noticeable are stuttering movements when turning, with halt in patient 3, and with backward step in patient 9.

Postural abnormalities with axial rigidity are illustrated in 10 patients either while standing or walking. In patient 4, the characteristic flexed posture is corrected by the patient; whether spontaneously or upon command cannot be determined here. In patient 1, lateral flexed posture and leaning to the right is particularly highlighted by filming the patient spinning around. A peculiar fixed posturing of the right hand is observed in patient 5.

Body bradykinesia is conspicuous in 10 patients, with all degrees of severity of slowness, hesitancy, and poverty of movement. In some patients, for instance, patient 7, an asymmetry of this feature is well illustrated, with the left side of the body much more affected. There was, of course, no task, as described in the MDS-UPDRS to specifically illustrate akinesia that was, however, evaluated from spontaneous movement, often performed in a normal environment.

Tremor is well documented in all patients and shown in close-up in 8 patients. Rest tremor of the arms, legs, chin, lips, and tongue is illustrated. Hand tremor is usually rather coarse, and classical pill rolling is observed only in a close-up of the hands of patient 11. Worsening of hand tremor while walking can be observed in patient 3. Patient 1, filmed sitting quietly, presents severe tremor of the four extremities. Conspicuous tremor of the chin, lips, and tongue is particularly noticeable in a close-up of patient 2. Unilateral as well as bilateral tremor of the hands, symmetric or asymmetric (for instance, patient 7), is shown. In patient 6, ample rest tremor of hands is temporarily reduced, particularly on the right side, when the patient stretches both his arms forward.

Only patient 12 is shown speaking, with apparently a normal fluency, but given that these are silent films, hypophonia or slurring cannot be assessed.

No dyskinesias, chorea or dystonia, are observed in any film.

Van Gehuchten discussed PD extensively in his textbook, in the section devoted to functional diseases, in a 13-page, 10-illustration chapter. Eight figures of the book show four different patients. Three of them could be matched with film clips (Table 1). The illustrations depict posture, hand position, and facies. A fourth patient, a woman, is shown in three figures, but the matching films were not found. The last two illustrations show samples of tremor-affected writing and micrographia. Not one of Van Gehuchten's many articles was devoted to PD. No specific clinical files or details of any of these 12 patients could be found.

Discussion

We present here a collection of films from over a century ago, illustrating the clinical features of PD. These iconographic documents brilliantly demonstrate the didactic qualities of Arthur Van Gehuchten. Indeed, they can still be used today to teach young clinicians to recognize the various motor signs of the disease and appreciate their severity.

We tried to analyze the films with a contemporary look, using the latest clinical rating scale for PD (MDS-UPDRS). This scale is divided into four sections. Three of them are based on the medical history and could not be used here. Part III, Motor Examination, was the inspiration for our analysis. We acknowledge that the attempt to rate these archival films with the new UPDRS is fraught with issues and potential inaccuracies, and that the exact guidelines could not be followed where specific instructions to the patient should have been given. Even if clearly present, rigidity could not be scored, as is still the case today, when attempting to score MDS-UPDRS on videos. All in all, approximately half of the motor examination could be rated with confidence. This helped us to precisely describe the clinical picture of each patient and draw attention to the clinical signs of PD that were considered as the most important in Van Gehuchten's time: tremor, rigidity, slowness of movement, and abnormal walking. The MDS-UPDRS has gained a very large geographical extension, given that it is used worldwide. With our attempt at using it on archival films, we tried to give the new scale a temporal extension, spanning over one century.

In his textbook, Van Gehuchten considered two main symptoms: tremor and hypertonia of striated muscles. He described the characteristic slow rest tremor, its distal predominance, usually starting in one hand, and its progressive extension. Tremor is well documented in the films not only in arms and legs, but also in the chin, lips, and tongue, sometimes in close-up. Van Gehuchten considered hypertonia as the most important symptom. Van Gehuchten did not specifically consider akinesia and considered slowness and difficulty in executing voluntary movements as a consequence of rigidity. The typical gait characteristics were well described by Van Gehuchten and are fully illustrated in most of the films. Patients are shown walking, sometimes almost running, with short steps, loss of arm swing, and typical flexed posture. Freezing of gait is different from that observed today, being more an extreme bradykinesia during walk than a sudden arrest while walking. Postural instability is conspicuous, with patients taking a few steps backward while turning.

It must be emphasized that the films are a pure observation of everyday life situations, such as walking or arising from a chair, without any intervention from a medical attendant or examiner. No specific maneuver is utilized to show rigidity or akinesia. As such, there are no instances of a physician examining passive joint movement or instructing the subjects to perform repetitive hand or foot movements. Cogwheel phenomenon had been described by the Italian neurologist, Camillo Negro, in idiopathic PD (iPD) in 1901, but this description waited 20 years to be widely recognized by the scientific and clinical community.[9] Although Van Gehuchten described resistance to passive movements, he did not specifically mention the cogwheel phenomenon.

In Van Gehuchten's time, few nonmotor signs had been described. Intelligence was thought to be intact. Besides the classic motor signs, Van Gehuchten pointed to other associated symptoms: drooling, sweating, and muscular pain. Micrographia and tremor-affected writing are illustrated in Van Gehuchten's textbook.

In most of the patients presented here, the diagnosis of iPD remains the most probable in 2014. The parkinsonian “variants” diseases were described long after Van Gehuchten's death. Indeed, Shy-Drager syndrome was described in 1960, PSP in 1963, corticobasal degeneration in 1968, and MSA in 1969. Van Gehuchten mentioned that reflexes were always normal in PD, but that sometimes walking might have cerebellar properties at the beginning.[8] In several patients (patients 1, 2, 4, 6, 7, 8, 11, and 12), a typical parkinsonian tremor is obvious, making any other diagnostic hypothesis very doubtful.

The marked predominance of male patients shown in this series of films is intriguing. Recent epidemiological studies have disclosed that men are approximately 1.5 times more frequently affected than women. Furthermore, the 6 cases described by James Parkinson were male.[10] It has been proposed that field neurology could explain this feature by favoring the encounter of male patients in the streets of early 19th century London.[11] In his textbook, Van Gehuchten did not mention a male predominance. In his large cinematographic production, both genders are almost equally represented, so we cannot propose a lower access of women to health service or shyness before the camera as possible explanations for this male predominance. We know that Van Gehuchten was behind the camera and patients do not give the impression of feeling ill at ease.

In Van Gehuchten's time, neither the nature nor the topography of the initial lesion of PD was known. The anatomist Van Gehuchten seemed annoyed to have to consider this distinctive neurological syndrome as a functional disease, together with chorea, epilepsy, and hysteria, as opposed to organic diseases with a fixed, identifiable lesion. But, referring to lenticular degeneration, recently described by Kinnier Wilson,[12] he concluded his discussion of the pathology of PD with a visionary statement that, most probably, there must be an unknown lesion in one of the basal nuclei.

Van Gehuchten did not consider secondary forms of parkinsonism. Indeed, the epidemics 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 synthesized. In Van Gehuchten's time, treatment of PD was limited to sedatives (potassium bromide and morphine) and various natural or synthetic anticholinergic alkaloids (duboisine, hyosciamine, hyoscine, and solanine). In addition, Van Gehuchten, in his textbook,[8] recommended several treatments based on the observation that emotions increase tremor and that sleep reduces it. Therefore, he suggested rest, sleep, and avoidance of any excitatory treatment. He underlined that all those medications have a partial effect only, mostly by decreasing tremor. Today, the cornerstone of treatment is levodopa, which is the most effective therapy. However, motor fluctuations as well as adventitious involuntary movements can be observed during treatment and can profoundly modify the motor semiology of PD.

Movement and gait disorders were frequently recorded by the pioneers of medical cinematography. This is well known through published articles with film descriptions, which have been traced.[6] However, surviving films of neurological patients before 1914 are extremely scarce. Despite an extensive search, we were unable to locate any film of patients with PD in France, Germany, the UK, or the United States, from this time period. The oldest surviving moving pictures of patients with PD were taken by Gheorghe Marinescu's team in Bucharest, Romania.[13] One sequence of 12 seconds, showing a group of 6 patients walking with typical gait and posture, has been recently edited and published. Another important neurological film archive is kept at the National Cinema Museum in Turin. In 1908, Negro collaborated with his friend Roberto Omegna, the owner of the then largest movie studios in Italy, and filmed several patients with various neurological disorders.[14] Entitled “La Nevropatologia” (neuropathology), it includes at least two clips of patients with PD in the 31-minute copy we could view. The patients are filmed sitting, arising from a chair, and walking, but no medical examination is shown, particularly passive movement of major joints.

In conclusion, we present an exceptional collection of archival films that illustrate the variety of clinical motor signs of PD, unaffected by l-dopa treatment. We tried to use here, as much as seemed possible, the new MDS-UPDRS, a wonderful method to score the severity of symptoms and signs of PD. Altogether, this represents a unique lesson in the semiology of PD, as shown in patients filmed over 50 years before the advent of l-dopa therapy.

 

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