Parkinsonism: Onset, Progression, and Mortality
Total Page:16
File Type:pdf, Size:1020Kb
Parkinsonism: onset, progression, and mortality Margaret M. Hoehn, M.D., and Melvin D. Yahr, M.D. PARKINSONISM,described in its entirety over lated to differences in terminology regarding one hundred and fifty years ago,’ rarely pre- the type of parkinsonism and the degree of its sents itself as a diagnostic problem. In conse- severity at the time of treatment. Consequent- quence, little scrutiny has been directed to the ly, it is difficult to determine whether treat- marked variability of this frequently encoun- ment really influences the course of the disease tered neurological syndrome and to the progres- and to what extent symptomatic relief is sig- sion of the disease in large groups of patients. nificant. It seems clear that, at a time when As with most chronic neurological disorders, many new approaches to the treatment of marked diversity can be expected to exist in parkinsonism are being suggested, information age and mode of onset, relative prominence of this sort is of paramount importance. of the cardinal signs and symptoms, rate of With this in mind, a study was made of 856 progression, and resultant degree of functional patients bearing the diagnosis of paralysis impairment. Controversy over the effectiveness agitans, Parkinson’s disease, or parkinsonism of therapeutic measures for parkinsonism is who were seen at the Vanderbilt Clinic of the due partially to this wide variability and to Columbia-Presbyterian Medical Center from the paucity of clinical information about the 1949 to 1964, inclusively. natural history of the syndrome. It is also re- MATERIAL AND METHODS From the Department of Neurology, College of Phy- .From this group of 856 patients, 54 were sicians and Surgeons, Columbia University, and the excluded for the following reasons: 39 were Neurological Service, New York Neurological Institute of the Columbia-Plesbyterian Medical Center, New York City found to have essential or familial tremor with Read in part at the seventeenth annual meeting of the none of the classical signs of parkinsonism, American Academy of Neurology in Cleveland, Ohio, on April 30, 1965 and 15 were omitted either because of incor- Supported by a United States Public Health Service grant rect diagnosis or because patients were lost to (NB 05 184) and contract (PH 43-64-54) follow-up and insufficient information was Dr. Hoehn’s address -is Neurological Institute, 710 West 168th Street, New York, New York 10032. avaiIable to establish a d4nite diagnosis of Neurology / Volume 17 / May 1967 427 428 NEUROLOGY parkinsonism. The remaining 802 patients ex- standard error of differences between means hibited some or all of the accepted cardinal and proportions using the Student’s “t” tables. signs: “rest” tremor, plastic rigidity, paucitv or delayed initiation of movement, slowness. CLASSIFICATION and impaired postural and righting reflexes. For this review, the classification of the The following data were recorded on all pa- syndrome of parkinsonism was based on a tients: age, sex. date of onset of parkinsonism, combination of clinical data and presumed initial symptoms, age and date of death, dura- etiology. An attempt was made to name and tion of illness, and family history of parkinson- define each type of parkinsonism in such a ism or other neurological disease. During the manner as to be clinically applicable while two-year period 1963 and 1964, 263 of the recognizing that it might not necessarily be patients were examined for additional or more pathologically distinct from other types. detailed information, and McBee punch cards Primary parkinsonism or Parkinson’s disease: were used for recording 77 factors concerning The term “Parkinson’s disease” is equated to past health, associated diseases, neurological “paralysis agitans” and has been reserved for signs, progression of disease, and degree of what, at present, is considered the primary or disability. “idiopathic” form of the disease. Six hundred In any study of a chronic disease statistical and seventy-two patients were so classified problems arise because of sampling distortion. after thorough review of their histories failed Patients may be omitted from the group being to reveal a disease process that couId be con- studied either because they died of other sidered etiologic in the production of their causes before seeking medical attention for parkinsonism. The presence of associated neu- their parkinsonism or because during the years rologic abnormalities, unless clearly attribut- of a lengthy illness they stopped attending the able to an independent disease process (such clinic and are lost to follow-up. These errors as cervical spondylosis 0s peripheral nerve in- were partially corrected by including in pro- jury) excluded the case from this diagnostic gression and mortality analyses only those 271 category. patients who were first seen within two years The mean age at onset of the disease in of the onset of parkinsonism and by extensive these patients was 55.3 years (Table 1) and tracing of Iost patients. SuppIementary infor- is in accord with previous rep0rts.~-l3Although mation has been gained from death certificates, the range is wide, in two-thirds of the patients private physicians and relatives, and hospital the disease began between the ages of 50 and nursing home records. Autopsy findings, and 69 (Table 2). In Manschot’s review in when available, were correlated with entries 19045 the onset in 65.9%of his patients also oc- on death certificates. Statistical significance curred between the ages of 51 and 70. In the was determined by subjecting all data to the present study there were 268 women, with a chi-square test or by calculations based on the mean age at onset of 54.8 years, and 404 men, TABLE 1 PARKINSONISM: AGE AT ONSET Number 7Age at onset (years)? of cases* Type of parkinsonism Mean Range 672 A. Primary parkinsonism: Parkinson’s disease 55.3 & 11.3 17 to 89 B. Secondary parkinsonism associated with: 96 ( 1 ) Encephalitis lethargica 28.2 2 8.8 12 to 53 (82) Definite 28.3 f 9.1 12 to 53 (14) Probable 27.9 % 7.2 14 to 39 22 (2) Other 49.3 f 14.8 14 to 69 C. Indeterminate parkinsonism associated with: 12 Encephalitis lethargica 50.3 2 7.7 40 to 66 *A total of 802 cases of parkinsonism, at the Neurology Clinic, Columbia-Presbyterian Medical Center, 1949- 1964, inclusively PARKINSONISM: ONSET, PROGRESSION, AND IMORTALITY 429 with a mean age at onset of 55.6 years. This ---I" difference is not significant and is in contrast to most other studies reporting the age at on- 65-69 set to be slightly lower in men than in wom- en.6?8,10 In addition, the distribution of ages or\- h \i at onset is the same for both sexes. During the fifteen years from 1949. to 1964 there has been a gradual increase in the pro- portion of older patients attending the clinic, a finding consistent with other rep0rts.~112The mean age at onset has risen from 50.9 years in those patients first seen in 1949 to 58.3 years in those first seen in the 1960-1964 period. Similarly, the age at first examination in the Neurology Clinic has risen from 54.3 years in 1949 to 61.9 years in 1960-64. The gradually shifting proportions of the age dis- tribution are illustrated in Figure 1 according to the year the patients were first examined. During this same period the time interval between the onset of parkinsonism and the first medical examination at the clinic has remained '"'O 1 1 approximately constant for those patients -1 I I -1 whose age at onset was over 55 years (Fig. 2). 1949 1950-1954 1955-1959 1960-1964 However, patients who were younger when (46) (981 (93) (262) the disease began have been waiting longer YEAR OF FIRST EXAMINATION in recent years before attending the clinic: Fig. 1. Distribution of ages at onset of pri- from a mean of 3.5 years in 1949 to six years inary parkinsonism in 499 patients first seen in 1960-1964. This contributes to the shift to at the Neurology Clinic, Columbia-Presby- older ages in recent years, as might the in- terian Medical Center creasing age of the population as a whole. The increasing interval between onset and first cluded in this group if their parkinsonism is clinic attendance does not seem to be related associated with a presumptive etiologic agent to decreasing severity of the disease in young and/or signs which suggest that their parkin- patients, and it may well be that other socio- sonism is a fragment of a more diffuse disease economic factors play a role. of systems not ordinarily involved in the Secondary parkinsonism: Patients are in- classical syndrome. TABLE 2 PRIMARY PARKINSONISM* : DISTRIBUTION OF AGE AT 0,NSET BY SEX r-------Men+-----, r-------Men+-----, --------Wo~n----, ,Total men and wo- Age at ornet No. % No. 70 NO. 70 Under 30 7 1.7 5 1.9 12 1.8 30-39 29 7.2 27 10.1 56 8.3 40-49 72 17.8 44 16.4 116 17.3 50-59 143 35.4 94 35.1 237 35.3 60-69 112 27.7 77 28.7 189 28.1 70-79 39 9.7 19 7.1 58 8.6 2 0.5 2 0.7 4 0.6 go+ - - - 404 268 672 ~ "672 cases of primary parkinsonism, Neurology Clinic, CPMC, 1949-1964,inclusively 430 NEUROLOGY 6 YEAR OF FIRST EXAMINATION 5 -1960-1964 .......... 1955-1959 ---a- 1950-1954 ----1949 4 m Fig. 2. Mean intervals in years be- e: of 43 tween onset disease and first w examination for various ages at on- >. set in 499 patients with primary parkinsonism 2 1 0 I I I 1 <45 45-54 55-64 6%- (79) (115) (206) (99) AGE AT ONSET 11 Postencephalitic parkinsonism.