Parkinsonism: Onset, progression, and mortality
TL;DR: Controversy over the effectiveness of therapeutic measures for parkinsonism is due partially to this wide variability and to the paucity of clinical information about the natural history of the syndrome.
Abstract: PARKINSONISM, described in its entirety over one hundred and fifty years ago,’ rarely presents itself as a diagnostic problem. In consequence, little scrutiny has been directed to the marked variability of this frequently encountered neurological syndrome and to the progression of the disease in large groups of patients. As with most chronic neurological disorders, marked diversity can be expected to exist in age and mode of onset, relative prominence of the cardinal signs and symptoms, rate of progression, and resultant degree of functional impairment. Controversy over the effectiveness of therapeutic measures for parkinsonism is due partially to this wide variability and to the paucity of clinical information about the natural history of the syndrome. It is also re-
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TL;DR: The pathological findings in 100 patients diagnosed prospectively by a group of consultant neurologists as having idiopathic Parkinson's disease are reported, and these observations call into question current concepts of Parkinson's Disease as a single distinct morbid entity.
Abstract: Few detailed clinico-pathological correlations of Parkinson's disease have been published. The pathological findings in 100 patients diagnosed prospectively by a group of consultant neurologists as having idiopathic Parkinson's disease are reported. Seventy six had nigral Lewy bodies, and in all of these Lewy bodies were also found in the cerebral cortex. In 24 cases without Lewy bodies, diagnoses included progressive supranuclear palsy, multiple system atrophy, Alzheimer's disease, Alzheimer-type pathology, and basal ganglia vascular disease. The retrospective application of recommended diagnostic criteria improved the diagnostic accuracy to 82%. These observations call into question current concepts of Parkinson's disease as a single distinct morbid entity.
9,411 citations
TL;DR: This study traces the course of the pathology in incidental and symptomatic Parkinson cases proposing a staging procedure based upon the readily recognizable topographical extent of the lesions.
8,452 citations
Cites background from "Parkinsonism: Onset, progression, a..."
...A: age, G: gender, nfp: neurofibrillary pathology, stages I–VI, a : stages of cortical –amyloid deposition A–C (0 = no AD-related lesions), PD: PD stages 1–6, clin: clinical diagnoses (−, no mention of PD symptoms in the clinical protocols, PD I–V: clinically diagnosed cases are indicated by PD and—if available—the Hoehn and Yahr stages I–V [47])....
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TL;DR: It is suggested that age-related attrition of pigmented nigral cells is not an important factor in the pathogenesis of Parkinson's disease and the regional selectivity of PD is relatively specific.
Abstract: The micro-architecture of the substantia nigra was studied in control cases of varying age and patients with parkinsonism. A single 7 mu section stained with haematoxylin and eosin was examined at a specific level within the caudal nigra using strict criteria. The pars compacta was divided into a ventral and a dorsal tier, and each tier was further subdivided into 3 regions. In 36 control cases there was a linear fallout of pigmented neurons with advancing age in the pars compacta of the caudal substantia nigra at a rate of 4.7% per decade. Regionally, the lateral ventral tier was relatively spared (2.1% loss per decade) compared with the medial ventral tier (5.4%) and the dorsal tier (6.9%). In 20 Parkinson's disease (PD) cases of varying disease duration there was an exponential loss of pigmented neurons with a 45% loss in the first decade. Regionally, the pattern was opposite to ageing. Loss was greatest in the lateral ventral tier (average loss 91%) followed by the medial ventral tier (71%) and the dorsal tier (56%). The presymptomatic phase of PD from the onset of neuronal loss was estimated to be about 5 yrs. This phase is represented by incidental Lewy body cases: individuals who die without clinical signs of PD or dementia, but who are found to have Lewy bodies at post-mortem. In 7 cases cell loss was confined to the lateral ventral tier (average loss 52%) congruent with the lateral ventral selectivity of symptomatic PD. It was calculated that at the onset of symptoms there was a 68% cell loss in the lateral ventral tier and a 48% loss in the caudal nigra as a whole. The regional selectivity of PD is relatively specific. In 15 cases of striatonigral degeneration the distribution of cell loss was similar, but the loss in the dorsal tier was greater than PD by 21%. In 14 cases of Steele-Richardson-Olszewski syndrome (SRO) there was no predilection for the lateral ventral tier, but a tendency to involve the medial nigra and spare the lateral. These findings suggest that age-related attrition of pigmented nigral cells is not an important factor in the pathogenesis of PD.
3,181 citations
TL;DR: A clinical diagnostic classification based on a comprehensive review of the literature regarding the sensitivity and specificity of the characteristic clinical features of PD is proposed: Definite, Probable, and Possible.
Abstract: The clinical diagnosis of Parkinson disease (PD) is based on the identification of some combination of the cardinal motor signs of bradykinesia, rigidity, tremor, and postural instability, but few attempts have been made to develop explicit diagnostic criteria. We propose a clinical diagnostic classification based on a comprehensive review of the literature regarding the sensitivity and specificity of the characteristic clinical features of PD. Three levels of diagnostic confidence are differentiated: Definite, Probable, and Possible. The diagnoses of Possible and Probable PD are based on clinical criteria alone. Neuropathologic confirmation is required for the diagnosis of Definite PD in patients with the clinical diagnosis of Possible or Probable PD. Criteria for histopathologic confirmation of PD are also presented.
2,747 citations
TL;DR: In this six-month study of patients under 75 years of age with severe motor complications of Parkinson's disease, neurostimulation of the subthalamic nucleus was more effective than medical management alone.
Abstract: BACKGROUND: Neurostimulation of the subthalamic nucleus reduces levodopa-related motor complications in advanced Parkinson's disease. We compared this treatment plus medication with medical management. METHODS: In this randomized-pairs trial, we enrolled 156 patients with advanced Parkinson's disease and severe motor symptoms. The primary end points were the changes from baseline to six months in the quality of life, as assessed by the Parkinson's Disease Questionnaire (PDQ-39), and the severity of symptoms without medication, according to the Unified Parkinson's Disease Rating Scale, part III (UPDRS-III). RESULTS: Pairwise comparisons showed that neurostimulation, as compared with medication alone, caused greater improvements from baseline to six months in the PDQ-39 (50 of 78 pairs, P=0.02) and the UPDRS-III (55 of 78, P<0.001), with mean improvements of 9.5 and 19.6 points, respectively. Neurostimulation resulted in improvements of 24 to 38 percent in the PDQ-39 subscales for mobility, activities of daily living, emotional well-being, stigma, and bodily discomfort. Serious adverse events were more common with neurostimulation than with medication alone (13 percent vs. 4 percent, P<0.04) and included a fatal intracerebral hemorrhage. The overall frequency of adverse events was higher in the medication group (64 percent vs. 50 percent, P=0.08). CONCLUSIONS: In this six-month study of patients under 75 years of age with severe motor complications of Parkinson's disease, neurostimulation of the subthalamic nucleus was more effective than medical management alone. (ClinicalTrials.gov number, NCT00196911 [ClinicalTrials.gov].).
2,497 citations
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TL;DR: In this paper, the authors present a conciliatory explanation for the present publication, in which, it is acknowledged, that mere conjecture takes the place of experiment; and, that analogy is the substitute for anatomical examination, the only sure foundation for pathological knowledge.
Abstract: PREFACE The advantages which have been derived from the caution with which hypothetical statements are admitted, are in no instance more obvious than in those sciences which more particularly belong to the healing art. It therefore is necessary, that some conciliatory explanation should be offered for the present publication: in which, it is acknowledged, that mere conjecture takes the place of experiment; and, that analogy is the substitute for anatomical examination, the only sure foundation for pathological knowledge. When, however, the nature of the subject, and the circumstances under which it has been here taken up, are considered, it is hoped that the offering of the following pages to the attention of the medical public, will not be severely censured. The disease, respecting which the present inquiry is made, is of a nature highly afflictive. Notwithstanding which, it has not yet obtained a place in the classification of nosologists; some have regarded its characteristic symptoms as distinct and different diseases, and others have given its name to diseases differing essentially from it; whilst the unhappy sufferer has considered it as an evil, from the domination of which he had no prospect of escape. The disease is of long duration: to connect, therefore, the symptoms which occur in its later stages with those which mark its commencement, requires a continuance of observation of the same case, or at least a correct history of its symptoms, even for several years. Of both these advantages the writer has had the opportunities of availing himself, and has hence been led particularly to observe several other cases in which the disease existed in different stages of its progress. By these repeated observations, he hoped that he had been led to a probable conjecture as to the nature of the malady, and that analogy had suggested such means as might be productive of relief, and perhaps even of cure, if employed before the disease had been too long established. He therefore considered it to be a duty to submit his opinions to the examination of others, even in their present state of immaturity and imperfection. To delay their publication did not, indeed, appear to be warrantable. The disease had escaped particular notice; and the task of ascertaining its nature and cause by anatomical investigation, did not seem likely to be taken up by those who, from their abilities and opportunities, were most likely to accomplish it. That these friends to humanity and medical science, who have already unveiled to us many of the morbid processes by which health and life is abridged, might be excited to extend their researches to this malady, was much desired; and it was hoped, that this might be procured by the publication of these remarks. Should the necessary information be thus obtained, the writer will repine at no censurewhich the precipitate publication of mere conjectural suggestions may incur: but shall think himself fully rewarded by having excited the attention of those, who may point out the most appropriate means of relieving a tedious and most distressing malady.
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265 citations
TL;DR: The opportunity has been taken to make available more detailed extensions of the system of disease classification and also to encompass related statistical areas, and this revision coincides with the appearance of two volumes of an international classification of procedures in medicine.
Abstract: In most countries of the world vital and health statistics have, from the beginning of this year, been classified in accordance with a new revision of the International Classification of Diseases (ICD). This revision, the ninth (ICD-9), is available in several languages in two volumes: the tabular list and the index. Although the number of Other CABI sites categories has been increased by 90, its main structure is very similar to previous revisions but it contains many innovations coincident upon the need to exploit the potential of flexibility arising from the proliferation of data-retrieval systems in recent years. Many of the changes are particularly appropriate to the use of the ICD for the coding of morbidity statistics, and include the basis of dual classification by aetiology manifestation (\"daggers and asterisks \") and for the first time a set of rules for the selection of a single cause for such applications as the collection of data related to admissions to hospital. One further aspect worthy of note is a recommendation for enhanced information on maternal and perinatal mortality, advocating a form of \" perinatal death certificate \"-a recommendation that for civil registration reasons it may not presently be possible to implement in many countries. What particularly characterizes this revision is that the opportunity has been taken to make available more detailed extensions of the system of disease classification and also to encompass related statistical areas. In 1974 WHO issued a glossary of mental disorders designed to improve the international comparability of mental health statistics and this, updated (7), is now incorporated within the mental disorders chapter (V) of ICD-9. It is also published separately, with a detailed index and an introduction of general as well as specific interest. The needs of oncology for a classification based on the topography, morphology and behaviour of tumours are met by a further volume (ICD-O) (2) which provides a coding system based on all these axes and which is compatible with ICD-9. WHO have also issued a supplementary classification for use in dentistry and stomatology (3). There has also been a change in the suggested method of presentation of statistics, with a new combined list of causes forming a basic tabulation list. The composition of this list is included in ICD-9 volume 1 and also as a separate volume with an index (4). This latter volume is primarily intended for the production of vital statistics in those countries which have limited coding resources, but should also be of value for use in special where the full detail of the ICD is not required or as an index to tabulated output. One possible danger of this particular volume is that occasional users may fail to realize that the numbers referred to in its index are those of the basic tabulation list and not ICD numbers. As well as enhancing the system of coding diseases, this revision coincides with the appearance of two volumes of an international classification of procedures in medicine. The first volume (5) includes sets of codes for diagnostic, laboratory and preventive procedures, as well as for operations and other therapeutic procedures. The second volume (6) provides a classification of radiology, radiotherapy, drugs, medicines and biological agents. All these classifications are based on the best information available internationally and provide a wealth of detail suitable for statistical analysis; equally, the use of abbreviated codes should also be helpful in surveys or if objectives are more ISBN : 0116905522 Record Number : 19792701461 Publisher : Geneva, Switzerland. Language of text : not specified Language of summary : not specified Indexing terms for this abstract: Organism descriptor(s) : man Descriptor(s) : aetiology, causes of death, classification, drugs, fetal death, glossaries human diseases, mental disorders, morbidity, mortality, neoplasms, perinatal mortality radiotherapy, statistical analysis, statistics, surveys, techniques, trauma, WHO Identifier(s) : cancers, causal agents, death rate, etiology, foetal death, medicines, mental illness, pharmaceuticals, psychiatric disorders, statistical methods, terminologies, traumas, World Health Organization Broader term(s) : Homo, Hominidae, primates, mammals, vertebrates, Chordata, eukaryotes limited. If all that is now made available by WHO is used to the full, both internationally and nationally, then 1979 should be an important milestone along the road of disease classification and vital statistics. (1) WORLD HEALTH ORGANIZATION. Mental disorders: glossary and guide to their classification in accordance with the Ninth Revision of the International Classification of Diseases. 96 pp. 1978. [ISBN 92 4 154137 7] [Sw.fr. 12.-]. (2) WORLD HEALTH ORGANIZATION. ICD-O: International Classification of Diseases for Oncology, pp. xxiii + 131. 1976 [ISBN 92 4 154056 7] [Sw. fr. 20.-]. (3) WORLD HEALTH ORGANIZATION. Application of the International Classification of Diseases to Dentistry and Stomatology. ICD-DA. 2nd ed. 150 pp. 1978. [ISBN 92 4 154132 6] [Sw. fr. 20.-]. (4) WORLD HEALTH ORGANIZATION. International Classification of Diseases Ninth Revision: Basic Tabulation List with alphabetical index. 331 pp. 1978. [ISBN 92 4 154133 4] [Sw. fr. 15.]. (5) WORLD HEALTH ORGANIZATION. International Classification ofProcedures in Medicine. Vol. 1. Various paginations. 1978. [ISBN 92 4 154124 5] [Sw. fr. 15.-]. (6) WORLD HEALTH ORGANIZATION. International Classification of Procedures in Medicine. Vol. 2. Various paginations. 1978. [ISBN 92 4 154125 3] [Sw. fr. 15.-]. J.S.A. Ashley
182 citations
TL;DR: Postencephalitic parkinsonism most often appeared in the second and third decade of life, and contemporary clinicians of the time were struck by the number of children and young adults affected.
Abstract: VON ECONOMO'S disease (encephalitis lethargica or type A encephalitis) occurred chiefly in the period 1919-1926, and its incidence diminished rapidly thereafter. It continues to be of interest today because of the unusual and remarkable sequelae—the parkinsonian syndrome—which sooner or later appeared in many of its survivors. This syndrome was as likely to appear in a mild as in a severe case of encephalitis lethargica, could develop after a latent period of apparent recovery extending for several years or more, and might even appear in persons who had never had an overt attack of encephalitis. In contrast to paralysis agitans, postencephalitic parkinsonism most often appeared in the second and third decade of life, and contemporary clinicians of the time were struck by the number of children and young adults affected. Wilson noted that "in preepidemic days juvenile paralysis agitans was an absolute rarity but now is commonplace."1Moreover, there
170 citations