Review Article
How common are the “common”
neurologic disorders?
D. Hirtz, MD; D.J. Thurman, MD, MPH; K. Gwinn-Hardy, MD; M. Mohamed, MPH;
A.R. Chaudhuri, PhD; and R. Zalutsky, PhD
Abstract—Objective: To estimate the current incidence and prevalence in the United States of 12 neurologic disorders.
Methods: We summarize the strongest evidence available, using data from the United States or from other developed
countries when US data were insufficient. Results: For some disorders, prevalence is a better descriptor of impact; for
others, incidence is preferable. Per 1,000 children, estimated prevalence was 5.8 for autism spectrum disorder and 2.4 for
cerebral palsy; for Tourette syndrome, the data were insufficient. In the general population, per 1,000, the 1-year
prevalence for migraine was 121, 7.1 for epilepsy, and 0.9 for multiple sclerosis. Among the elderly, the prevalence of
Alzheimer disease was 67 and that of Parkinson disease was 9.5. For diseases best described by annual incidence per
100,000, the rate for stroke was 183, 101 for major traumatic brain injury, 4.5 for spinal cord injury, and 1.6 for ALS.
Conclusions: Using the best available data, our survey of a limited number of disorders shows that the burden of
neurologic illness affects many millions of people in the United States.
NEUROLOGY 2007;68:326–337
Current accurate estimates of the numbers of people
affected by neurologic disorders are needed to understand the burden of these conditions, to plan research on their causes and treatment, and to assess
preventive interventions. Estimating the incidence
and prevalence of neurologic disorders can be challenging. For some diseases, there are few published
data and for others, available estimates vary greatly.1 We surveyed the literature to develop the best
possible estimates of the incidence and prevalence in
the United States of 12 neurologic disorders across
the life span that are commonly seen by neurologists
and have substantial morbidity or mortality. Our approach was similar to the American Academy of Neurology’s process for evidence-based guidelines for
clinical practice.2 We defined four classes of evidence
(table 1) and searched the literature for relevant
Additional material related to this article can be found on the Neurology
Web site. Go to www.neurology.org and scroll down the Table of Contents for the January 30 issue to find the title link for this article.
studies. We based our estimates for incidence and
prevalence on the strongest evidence available.
Methods. For each disorder, we searched PubMed using the
terms epidemiology, incidence, and prevalence; we restricted the
search to English-language articles published between January 1,
1990, and January 31, 2005. We screened titles and abstracts and
selected relevant articles, excluding case reports and small case
series. We identified additional studies from reference lists and
from recent reviews. Approximately 500 articles were reviewed.
Criteria for evaluating the strength of evidence considered
time frame, case-finding strategy, case definition, and source of
diagnosis (table 1):
• Time frame. Because rates of disease occurrence as well as
methods of diagnosis or data collection often change over
time, we gave our top rating (A) to studies in which some or
all of the data were obtained from 1990 or later.
• Case finding and sample size. An A rating required either
the great majority of cases in a population to be included or
a representative population-based sample. Studies of exceptionally small samples with wide CIs (e.g., from less than
one-half to more than twice the point estimate) were rated
as C.
• Case definition. An A rating was reserved for studies with
clearly defined and consistently applied diagnostic criteria
reflecting currently accepted disease definitions.
• Source of diagnosis. An A rating required either an expert
diagnostician who had applied the case criteria to each case
or a validated source with known high predictive values.
Editorial, see page 322
See also pages 338 and 384
From the National Institutes of Neurological Disorders and Stroke/National Institutes of Health (D.H., K.G.-H., M.M., A.R.C., R.Z.), Bethesda, MD; and
National Center for Chronic Disease Prevention and Health & Promotion/Centers for Disease Control and Prevention (D.J.T.), Atlanta, GA.
Disclosure: The authors report no conflicts of interest.
Received June 20, 2006. Accepted in final form November 6, 2006.
Address correspondence and reprint requests to Dr. Deborah Hirtz, NIH/NINDS, NSC, Room 2212, 6001 Executive Blvd., Bethesda, MD 20892; e-mail:
[email protected]
326
Copyright © 2007 by AAN Enterprises, Inc.
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Table 1 Classes of evidence for studies on neurologic disorders
Time frame
A. Data refer to time period that includes years from 1990 or
later
B. Data refer to time period that includes years from 1970 to
1989 but not later
C. Data refer to 1969 or earlier
Many authors did not adjust their data to the age distribution
of a standard population. To improve comparisons across studies
that reported incidence or prevalence data stratified by age, we
recalculated summary rates standardized through direct adjustment to the age distribution of the US population in 2000.3 We did
not standardize rates from studies limited to children and we
could not standardize rates if age-specific data were lacking.
Where studies reported separate data from multiple time periods,
we considered only the most recent data (see appendix E-1 on the
Neurology Web site at www.neurology.org).
D. Data refer to an undisclosed time period
Case-finding and sample size
A. Evaluate all eligible population members (or adequate-sized
random sample) or search of all relevant referral sources, with
likelihood of identifying substantial majority of targeted cases
B. Some potentially relevant case-finding sources omitted
C. Case-finding strategy that may lead to an unrepresentative
sample or significant over- or underascertainment
D. No information disclosed or sample size inadequate to provide
confident estimate of incidence or prevalence
Case definition
A. Clearly defined and consistent with generally accepted
clinical/laboratory criteria
B. Less precisely defined with minor deviations from generally
accepted criteria
C. Modification or partial use of generally accepted criteria
D. Other or no criteria applied
Source of diagnosis
A. Specialist or fully validated source (including self-report) with
known positive predictive values
B. Self-report with specified criteria or partially validated source
C. Nonvalidated but well-defined diagnostic source
D. Self-report without specified criteria or poorly defined source
Using these criteria, we categorized articles as Classes I
through IV (table 2). At least two of the authors (D.H., D.J.T.,
K.G.H., R.Z.) independently reviewed each article and resolved
any differences in classification with the rest of the authors. When
four or more Class I articles were not available from the United
States or Canada, we included data from other developed countries. Class III and IV evidence was reviewed but not included in
determining estimates of incidence and prevalence, unless there
was little or no Class I or II evidence.
We defined incidence as the number of new cases occurring in
a specified population during a given interval and expressed it as
new cases per 100,000 population per year. Prevalence was defined as the number of cases existing in a population at a specific
time point. Prevalence includes all cases, whether of recent or
remote onset, until such time as the condition fully resolves or
death occurs, expressed as a proportion: existing cases per 1,000
population.
Table 2 Determination of class of evidence
Class
Distribution of criteria*
I
All A
II
One or more B; no C or D
III
One or more C; no D
IV
One or more D
* From table 1: time frame, case-finding strategy, case definition,
and source of diagnosis.
Neurologic disorders with onset early in life.
1. Autism. The autism spectrum disorders (ASD) are
classified according to the Diagnostic and Statistical
Manual of Mental Disorders, Fourth Edition, Text Revision (DSM-IV-TR) as pervasive developmental disorders that include autistic disorder, Rett syndrome,
childhood disintegrative disorder, and Asperger disorder as well as pervasive developmental disorder not
otherwise specified.4 Prevalence is reported in preference to incidence because the age at ascertainment is
variable. We found no studies of prevalence in adults,
although the disorder is lifelong.
There were seven Class I5-11 (one from the United
States) and seven Class II12-18 (two from the United
States) prevalence studies (see table E-1 on the Neurology Web site at www.neurology.org). From the
Class I studies, the median prevalence of autistic
disorder was 2.4 per 1,000 (range 0.7 to 4.1), and of
ASD was 5.8 per 1,000 (range 1.3 to 6.7). The ratio of
boys to girls was approximately 4:1.
These numbers are larger than have been previously reported, and there is widespread concern
about an apparent increase in autism.19,20 In light of
current controversy, two articles (one US, one Canadian) published after completion of our literature
search support the prevalence figure of approximately 6 per 1,000 for ASD.21,22 There are major limitations in our ability to compare recent and older
data; for example, diagnostic criteria have changed,
public services are more available, societal attitudes
have evolved, and case ascertainment methods have
shifted to population screening.
2. Cerebral palsy (CP). CP is a nonprogressive
impairment of movement or posture originating either perinatally or very early in life, usually in the
first year.23 Studies of prevalence have been performed in populations of children, although the disability is lifelong. Many studies include separate
data for infants born preterm because their risk of
CP is higher.
Eight European24-31 and one US32 Class I studies
yielded a median prevalence of 2.4 per 1,000, and
there was remarkable consistency (range 2.0 to 2.5
(table E-2a). Four European and two US Class II
studies also yielded a median prevalence of 2.4 per
1,000 but with a broader range (1.2 to 3.2).33-38
For preterm infants, from nine Class I24-27,29,31,32,39,40
and two Class II41,42 studies the median prevalence of
CP among survivors with birth weights of 1,500 to
2,499 grams was 11.2 per 1,000 (range 6.2 to 13.9);
the corresponding median among those with birth
weights less than 1,500 grams was 63.5 per 1,000
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NEUROLOGY 68
327
(range 28 to 185) (table E-2b). For infants weighing
less than 600 grams at birth, in a single Class III
study, of the 25% of infants who survived, 75% had
CP.43 In all studies, the lower the birth weight was
and the younger the gestational age was, the higher
the prevalence of CP. Infants born in multiples have
a higher risk of developing CP than singletons, but
this risk is largely related to the fact that they are
more commonly preterm.44 Estimates do not vary appreciably between studies from the 1980s and from
1990 or later. However, in the current decade, improved survival rates at earlier gestational ages as
well as increased rates of prematurity and multiple
births may be causing an increase in children with
CP.25,32,41,43-49
3. Tourette syndrome. The diagnosis of Tourette
syndrome (Gilles de la Tourette syndrome) is based
on the presence of multiple vocal and motor tics
present for at least 1 year with onset by age 18
years.4 DSM-IV50 criteria (published in 1994) required that there be some impairment or distress as
a result of the symptoms, and in contrast, DSM-IIIR51 (published in 1987) and DSM-IV-TR4 (2000) do
not include that requirement. Study populations or
samples are usually relatively small, resulting in
wide estimated CIs. Symptoms vary in frequency
and intensity and may go unrecognized by those who
suffer them, their families, or their physicians.52
No Class I studies were found. Four Class II
studies53-56 (including one from the United States)
yielded a median prevalence of 3.5 per 1,000 (range
0.4 to 11.0) (table E-3). The median prevalence in
four Class III studies (two from the United States)
was 7.2 per 1,000 (range 1.5 to 18.5).54,57-59 Prevalence peaks between ages 7 and 16 years with a male
predominance of about 5:1 (range 1.6 to 10:1).52 The
prevalence in special education students is much
higher than in the general population.59
Difficulties in case identification make accurate
estimates of prevalence challenging, and methods of
case ascertainment differ considerably among studies. Correspondingly, estimates from Class II studies
in several countries vary greatly (nearly 30-fold), and
the validity of the median estimate of 3.5 per 1,000 is
suspect.
Neurologic disorders with onset at any age.
4. Migraine. Operational diagnostic criteria published by the International Headache Society (IHS)
have helped standardize recent epidemiologic studies.60 Many migraine sufferers do not seek medical
care; thus, a study that relies on medical records is
likely to substantially underestimate this problem.
Population surveys are crucial in epidemiologic studies of migraine. Studies variously reported 1-year
prevalence, annual incidence, and lifetime prevalence. The episodic nature of the disorder makes
these susceptible to recall bias (e.g., people may forget an episode from decades earlier). Because the
diagnosis of migraine headache is based on history
rather than on physical examination, we considered
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NEUROLOGY 68
studies that employed structured questionnaires using IHS criteria to be equivalent to having the diagnosis made by an expert without direct examination
(table 1).
Four Class I61-64 and eight Class II65-70,72,73 studies
(five from the United States) estimating the 1-year
prevalence of migraine in adult populations yielded a
median prevalence of 121 per 1,000 (range 50 to 158)
(table E-4a). Studies including adults that compared
rates by sex consistently reported lower 1-year rates
for men, with a median man-to-woman rate ratio of
0.36. In adults, prevalence varies little by age (figure
E-1). Two Class II studies found a prevalence in children of 70 and adolescents of 106 per 1,000 (median
88).74,75
There was a single Class II study that found an
age-adjusted annual incidence of 370 per 100,000,
with a man-to-woman rate ratio of 0.28.73 From four
Class I64,71,76,77 (one US) and three Class II65,68,73 (one
US) studies, the median lifetime prevalence for men
and women together was 159 per 1,000 (range 109 to
234) (table E-4b).
5. Epilepsy. The International League Against
Epilepsy has defined epilepsy as recurrent nonprovoked seizures.78 Excluded from this definition are a)
acute symptomatic seizures provoked by known precipitants such as head trauma, b) febrile seizures in
young children with no history of nonfebrile seizures,
and c) neonatal seizures. Using these criteria, we
excluded several studies with broader definitions.
In four Class I79-82 (all European) and six Class
II83-88 studies of incidence (three from the United
States), the median estimate was 46 per 100,000 per
year (range 32 to 71; table E-5a). The subgroup of
four studies including all age groups yielded a similar median estimate of 48 per 100,000.81,82,86,87 The
incidence of epilepsy is related to age; in studies
restricted to children or adolescents, the median estimate was 57 per 100,000 per year (range 41 to 65).
Higher incidence rates occurred among infants
younger than 1 year and among people older than 60
years (figure E-2).79-86,88,89 Most studies did not show
significant differences by sex.
Six Class I90-95 (all European) and seven Class
81,85,87,89,96-98
II
(three from the United States) studies
addressed prevalence (table E-5b). For the three
Class I studies in children and adolescents up to age
19, the median value was 3.9 per 1,000 (range 3.6 to
5.1).90,92,94 Among the Class II studies that included
all age groups, the median estimate was 7.1 per
1,000 (range 4.0 to 8.9) (table E-5b).81,87,96-98
6. Multiple sclerosis (MS). Most of the studies
that we reviewed relied on diagnostic criteria proposed by Poser et al. in 198399; most included both
definite and probable cases, with definite cases accounting for most. Epidemiologic studies of MS have
been hampered by variability in clinical findings,
lack of a single, accurate diagnostic test, and delays
in diagnosis. In addition, changes in diagnostic tools
and criteria may have altered observations of incidence and prevalence.
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Earlier epidemiologic studies drew attention to
variation in prevalence by latitude,100 suggesting
that climatic, environmental, or infectious exposures— or varying genetic predispositions—are involved in the pathogenesis of MS.101 A systematic
meta-analysis has found that the apparent relationship between MS and latitude diminishes when estimates are age and sex adjusted to a common
standard population.102
Among all 22 Class I studies of incidence (one
from the United States),103-124 the median estimate of
the annual incidence of MS was 4.2 per 100,000
(range 0.8 to 12.0) (table E-6a). The median estimate among Mediterranean countries (Greece,103
Italy,104-111 Malta,112 and Spain113-115) was 3.7 (range
0.8 to 6.7) per 100,000, whereas the median among
more northern European countries116-123 was 4.6
(range 3.8 to 12.0). A study from North America
(Minnesota)124 using data from 1985 through 2000
reported 7.4 cases per 100,000 population per year.
Among the 39 Class I studies of MS prevalence
(table E-6b),103-106,108-115,118-141 the median was 0.9 per
1,000 (range 0.2 to 2.3). Studies in Mediterranean
countries103-115,131-133,135-138 yielded a median prevalence
per thousand of 0.6 (range 0.2 to 1.6), and other
European countries101,116-123,125,126,129,130,139,140 of 1.2
(range 0.7 to 1.8). The four studies from North America covering populations in Alberta127,128 and Minnesota124,141 provided a median estimate of 2.0 per 1,000
(range 1.7 to 2.3). The peak age of MS onset is approximately 30 years (figure E-3), and few cases are
diagnosed before age 15 or after 50. Both incidence
and prevalence are approximately twice as high
among women as in men.
7. Stroke. Stroke includes both ischemic and
nontraumatic hemorrhagic insults to the brain. We
did not include studies of asymptomatic cerebral infarctions diagnosed only from brain imaging procedures, studies of perinatal stroke, or data on TIAs.
Most studies of incidence addressed only first
strokes.
We identified 11 Class I142-152 (two from the United
States) and 14 Class II (seven from the United
States) studies153-165a that reported combined incidence of ischemic and hemorrhagic stroke (table
E-7a). Overall rates varied greatly by age range. The
median annual incidence of first stroke in studies
encompassing all ages was 183 per 100,000. The median value in studies excluding older ages (upper
bounds from 15 to 49 years) was 10 per 100,000 per
year. Data from Class I142-148,150,151 and Class II153,156-163
studies suggest that the risk of stroke roughly doubles with each decade of age during adulthood (figure
E-4). The lowest risk is seen among children after 1
year of age153-155; the highest is among persons aged
85 years or older, with a median annual rate exceeding 2,000 per 100,000.
The summary rate ratios by sex (table E-7a) do
not show a consistent difference; however, most
studies show somewhat higher age-specific rates
among men. Nine studies of US communities
focused on disparities in incidence by race or
ethnicity.152,154,155,164,165a,165b,166-168 These studies suggest higher risk among Hispanics and African Americans; in the latter group, rates appear to be roughly
twice those of whites (table E-7b).
Most studies attributed 80% or more of all strokes
to ischemia (median 81%). Lower figures were seen
in studies focused on children or populations younger
than 50 years of age (median 51%),142,153-155 and populations in Japan (median 62%).148,158,162 In one Class
I and two Class II studies addressing prevalence
(ever having a stroke), results varied by age range
(table E-7c).161,169,170 A study in Rochester, MN, suggests a prevalence of 1% in the white population of
all ages161; a national survey suggests a prevalence of
nearly 2% for US adults aged 25 to 74 years.170 As
predicted by results for incidence, older populations
have a much higher prevalence, as demonstrated by
an Italian study.171
8. Traumatic brain injury. The Centers for Disease Control and Prevention (CDC) defines traumatic brain injury (TBI) as craniocerebral trauma
associated with decreased level of consciousness, amnesia, other neurologic or neuropsychological abnormalities, skull fracture, intracranial lesions, or
death.172 The majority of studies in the United States
are focused on injuries that lead to hospital admission or result in death.
Of six Class I studies in the United States,173-178
four173,175-177 addressed the combined incidence of fatal and hospitalized TBI among all age groups (table
E-8) with a median annual incidence of 101 per
100,000 (range 91 to 105 per 100,000). Rates among
men were approximately twice those of women; rates
were higher among adolescents and young adults as
well as among seniors (figure E-5). The proportion of
TBIs resulting in death either immediately or during
acute hospital care varied from 18 to 23%. The Class
I study of hospitalized patients with nonfatal TBIs
found an incidence rate of 70 per 100,000.,178 and the
Class I study that addressed TBI hospitalizations
and deaths only among children younger than 20
years found a rate of 74 per 100,000.174
The estimated annual rates of presumed mild
TBIs treated only in outpatient facilities or hospital
emergency departments (EDs) from two Class II
studies in the United States were 392 TBI-related
ED visits per 100,000 population179 and 540 combined ED or outpatient visits per 100,000.180
We found no Class I, II, or III studies that directly
measured the prevalence of TBI-related disability in
general populations. One Class II study181 reported a
prevalence of disability of 37% in a population-based
sample of patients followed 1 or more years after
hospitalization for TBIs. Based on this figure and
taking account of temporal trends in TBI incidence
rates, the CDC has estimated that nearly 2% of the
entire US population has TBI-related disabilities.177
Because it is a proportion derived using assumptions
of long-term survival and mathematical models, we
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NEUROLOGY 68
329
cannot classify the level of this evidence, although it
clearly indicates a high prevalence.
9. Spinal cord injury. Most studies of the incidence of spinal cord injury (SCI) include traumatic
lesions that result in complete or incomplete functional interruption of spinal pathways and varying
degrees of paraparesis or tetraparesis, consistent
with American Spinal Injury Association criteria.182
Both transient and permanent manifestations are
included. Some studies of incidence are limited to
acute injuries that result in hospital admission; a
few also include immediate fatalities not admitted to
hospitals. Prevalence is defined as the proportion of
a population with persisting neurologic sequelae as a
result of SCI.
Four Class I183-186 and one Class II187 studies from
North America yielded a median annual incidence
rate of 4.5 per 100,000 (range 3.9 to 7.7) (table E-9).
Rates are approximately four times greater among
men and relatively higher among adolescents and
young adults (figure E-6). Analysis by level of lesion
showed that a slight majority of injuries resulted in
quadriplegia or quadreparesis.185,186,188
Prevalence data are scant; one Class III study
that surveyed a sample of the US population estimated that 0.72 people per 1,000 had a history of
traumatic SCI.189
Disorders with onset later in life. 10. Alzheimer
disease (AD). We limited our review to studies that
specifically addressed the incidence and prevalence
of AD rather than dementia generally. Most studies
used the clinical diagnostic criteria of the National
Institute of Neurological and Communicative Diseases and Stroke/Alzheimer’s Disease and Related
Disorders Association,190 the Cambridge Examination for Mental Disorders of the Elderly,191 or the
DSM-III-R criteria.51 Although some of these criteria
distinguish between probable and possible cases of
AD, most of the studies did not. Nursing home placement is typical in end-stage AD, and so we did not
use studies excluding long-term care residents.
Age ranges varied substantially across studies.
Because AD rates increase with age, it was difficult
to compare studies including younger ages with
those limited to older ages. Therefore, among studies
including younger age groups that had age-specific
data available, we recalculated the overall rates to
include only older populations (usually aged 65 or
older).
Eight Class I studies (three from the United
States) yielded a median annual incidence of AD of
1,178 per 100,000 population aged 65 years or older
(range 1,005 to 3,554)192-199 (table E-10a). Combining
these studies with six Class II studies200-205 (five from
the United States) increased the median to 1,275.
Between the seventh and ninth decades of life, the
incidence of AD increases steeply with age (figure
E-7). Most studies reporting rates by sex indicated a
higher risk for women with a median men-to-women
rate ratio of 0.54.182,194-197,202,203
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Five Class I studies of AD prevalence (one from
the United States) in both sexes yielded a median
estimate of 48 per 1,000 population (range 31 to 122)
for seniors (variously defined as 65 years or older, 70
years of older, or 75 years or older)198,206-209 (table
E-10b). With the addition of data from three Class II
studies210-212 (one from the United States), the median prevalence became 67 (range 31 to 130).
11. Parkinson disease (PD). We limited our review to studies using widely accepted diagnostic criteria.213,214 The studies evaluated PD occurrence
using differing age breakdowns, making cross-study
comparisons difficult. To improve the comparability
of studies that provided age-specific rates, we recalculated the rates provided to include only persons 65
years or older or 70 years or older, depending on the
age strata reported (tables E-11a and E-11b). Although in all cases the final diagnosis was confirmed
by physical examination, variations in screening
methods among Class I articles may have contributed to variations in findings.
We identified three Class I215-217 (one from the
United States) and five Class II218-222 (two from the
United States) studies of incidence. For the two studies that reported all age groups, the median incidence was 14 per 100,000 (range 12 to 15). The
median estimate from studies restricted to populations aged 65 or older or 70 years or older was much
higher: 160 (range 62 to 332).216-219,221,222 Controversy
exists about whether incidence plateaus around age
80 or keeps increasing, but data are too meager to
draw firm conclusions (figure E-8). PD appears to be
more common in men than women, with a median
rate ratio of 1.8 (range 1.4 to 2.0).215-219,221
There were six Class I215,223-227 (all European) and
six Class II220,222,228-231 (one from the United States)
studies of prevalence. Eleven reported a median
prevalence of 9.5 per 1,000 population for persons
aged at least 65 or 70 years (range 7.0 to 43.8).
12. ALS. Most studies of ALS published since
the mid-1990s used the El Escorial criteria for case
definitions.232 We included other studies if they used
similar criteria, although a few Class II studies addressed the broader category of motor neuron disease. We excluded studies of spinal muscular
atrophy of infancy and childhood.
Nine Class I233-241 (one from the United States) and
10 Class II242-251 (one from the United States) studies
of ALS incidence (table E-12a) were found. Most of
these studies identified cases from a combination of
hospital and clinic administrative data, neurologist
practices, or vital records; in all cases, a neurologist
made or reviewed the diagnosis. The median annual
rate of incidence among studies that included all
ages was 1.6 per 100,000 (range 0.7 to 2.5). From
Class I studies, only the median annual rate (all
ages) was 2.1 per 100,000 (range 0.7 to 2.5). Most
studies showed somewhat lower rates among women,
and the median rate ratio of men to women was 1.3.
The distribution of reported age-specific rates is
shown in figure E-9. In general, the data show an
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acceleration in the upward trend for rates after age
40. Estimated rates in the seventh decade of life are
broadly distributed around five per 100,000. Beyond
this age, wide disparities in reported rates do not
allow confident generalizations.
We identified one Class I236 (Italy) and seven
Class II242,244,245,247,248,251,252 (one United States) studies
addressing ALS prevalence (table E-12b). Results did
not vary widely, with a median reported prevalence
of 0.04 per 1,000 for all ages. Consistent with the
limited survival with this condition, prevalence is
quite low. ALS is weakly associated with male sex
and strongly associated with older age.
Discussion. The disorders included here are not
the only causes of neurologic morbidity and mortality nor are they necessarily the most common. We
selected a limited number of disorders of the CNS
that neurologists are likely to diagnose and treat and
that are generally presumed to have a high burden
of illness. We did not address other frequent and
burdensome conditions, such as chronic pain, mental
retardation, peripheral neuropathies, or sleep disorders, some of which lack well-established diagnostic
criteria or are treated primarily by non-neurologists.
We considered disorders that are primarily psychiatric to be beyond our scope. A few disorders originally
considered, such as muscular dystrophy, were omitted because of insufficient studies. Some disorders,
such as CNS neoplasms, are systematically tracked
and their incidence is summarized in other sources.253 Other important disorders, such as essential
tremor, restless legs syndrome, and vascular dementia, were not included but may merit future reviews.
Comparisons with an earlier review1 of the epidemiology of a range of similar disorders may be informative. The previous review did not evaluate the
quality of available evidence and often used broader
inclusion criteria. Adding to the difficulty of inferring real changes, case definitions, and methods of
case ascertainment have since evolved. We are unable to make age-adjusted comparisons between this
and the earlier report, and the recent shift in the
distribution of the US population toward older ages
could result in somewhat higher crude rates for conditions that mainly arise late in life. Given these
limitations, the current and previous results for epilepsy and migraine prevalence are very similar. For
CP, the data also appear consistent, taking age into
account. For Tourette syndrome and autism, there
were no earlier estimates given, which may reflect
recently increased recognition of these conditions.
Our estimate of MS prevalence rate is approximately
50% higher than the earlier estimate. Whether this
reflects improvements in diagnosis or whether the
incidence is actually increasing deserves further
study. The reported incidence of hospitalized TBI
has decreased about 50%. It is likely that this reflects more restrictive hospital admission criteria, although improvements in motor vehicle safety may
also contribute.254 Data are too sparse to support con-
clusions regarding an apparent increase in SCI incidence rate. The incidence rates of ALS appear
similar in both reviews, as do the incidence rates of
PD, considering that the earlier review encompassed
broader categories of parkinsonism and that there
have been changes in the age distribution of the
population.
Although stroke mortality has decreased,149 the
comparison of our data with the earlier review suggests a possible increase in stroke incidence, despite studies showing an earlier decline over
several decades.255,256 Possible explanations might
include more sensitive diagnostic methods using
neuroimaging and a significant increase in the proportion of older adults in the population. Finally,
although the earlier review included all types of
dementia, in comparison, the current rates of AD
alone are substantially higher. Factors cited earlier such as an increase in the proportion of older
people in the population and better case ascertainment may be important, but the possibility of a
real increase in the incidence of dementia merits
further research.
For most disorders, insufficient Class I evidence
from the United States is available. Additional highquality data are often available from non-US developed countries, perhaps in part because more
centralized health care systems facilitate case ascertainment and study. With caution, findings obtained
from one population may be generalized to others to
the extent that the demographic, cultural, and environmental characteristics of the populations are similar. We recognize, however, that even among
developed countries, differences in culture, age distribution, organization of health care and record
keeping, genetic susceptibility, environmental exposures, and the socioeconomic composition of populations might influence the occurrence and detection of
the conditions that we reviewed.
Although essential to any consideration of disease
burden, neither incidence nor prevalence captures
the full impact of disorders in human and economic
dimensions. These broader consequences are encompassed by the term burden of illness. Estimates of
human burden must consider not only the frequency
of a disease but also the years of life lost and the
duration, character, and degree of disability and suffering as well as the impact on family and society.
Various summary measures that incorporate these
considerations have been devised, including disability–adjusted life-years257 and quality-adjusted lifeyears.258 Such measures require data on age at onset,
age at death, and some quantitative metric of disability and suffering. On the economic side, direct
costs of treatment must be included, as well as lost
wages and productivity both of patients and their
caregivers.
Conclusions. Table 3 summarizes the results for
the 12 disorders that we reviewed. For each disorder,
consideration of incidence and prevalence immediately raises second-order questions that are critical
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331
Table 3 Summary of evidence: Incidence and prevalence of 12 neurologic disorders
Median estimates
Annual incidence
Prevalence
Class of
evidence
Range
of ages
included (y)
Rate/
100,000
No.*
Rate/
1,000
Autism spectrum disorders
I, II
2–15
—
—
5.8
Cerebral palsy
I, II
3–13
—
—
2.4
Disorder
Tourette syndrome
Rate ratio,
M/F†
Age(s), y, of
peak incidence
500,000‡
4.2
—
207,000‡
1.3
—
No.*
II
7–17
—
—
3.5§
301,000
4.8
—
Migraine
I, II
12–65
—
—
121
35,461,000
0.4
—
Epilepsy
I, II
All
48
142,000
7.1
2,098,000
I
All
4.2
12,000
0.9
266,000
Multiple sclerosis
1
⬍1, ⱖ80
0.5
30
I
All
101
298,000
—
—
2.1
20, ⱖ80
Spinal cord injury
I,II
All
4.5
13,000
—
—
4.2
20
ALS
I, II
All
1.6
5,000
0.04
12,000
1.3
ⱖ60
Stroke
I, II
All
183
541,000
2,956,000
1.1
ⱖ80
ⱖ65
1,093
401,000
—
—
—
Alzheimer disease
I,II
ⱖ65
1,275
468,000
67
2,459,000
0.5
ⱖ80
Parkinson disease
I,II
ⱖ65
160
59,000
9.5
349,000
1.8
ⱖ70
Traumatic brain injury
10
* Estimated number of cases in United States in 2005, rounded to nearest 1,000.
† Ratio of rates among males to rates among females.
‡ Estimated number of cases among children younger than 21 years of age only.
§ Data inadequate for firm estimate.
to understanding the disease and its impact on
public health. For many neurologic disorders, we
need better studies of incidence and prevalence to
improve the accuracy of estimates, to enable more
confident generalizations to broader populations,
and to assess trends. Beyond incidence and prevalence, additional research is needed to allow a more
complete estimate of burden of illness that includes
potential years of life lost, productive years lost to
disability, impact on caregivers, and economic costs.
Acknowledgment
The authors appreciate the assistance of Dallas W. Anderson,
PhD, Lynda Anderson, PhD, Colleen Boyle, PhD, Gary Franklin,
MD, MPH, Gary Friday, MD, MPH, Kurt Greenlund, PhD, Gary
Gronseth, MD, MPH, and Paul Scherr, PhD, who provided critical
advice in the preparation of this manuscript.
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How common are the "common" neurologic disorders?
D. Hirtz, D. J. Thurman, K. Gwinn-Hardy, M. Mohamed, A. R. Chaudhuri and R.
Zalutsky
Neurology 2007;68;326-337
DOI: 10.1212/01.wnl.0000252807.38124.a3
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Downloaded from www.neurology.org at Washington University on August 19, 2010