Table 1

Comparison of the type of study, rate of familial amyotrophic lateral sclerosis, source of case ascertainment and case inclusion criteria for each study

CountryYearsFALSTotal ALS% FALSAscertainmentDiagnosisComments
Group 1: prospective population based registry
 Puglia, Southern Italy1998–199921301.50A, B, C, D, G, KBii, H, J, L, N
 Piemonte, Northern Italy1995–20045312604.20A, D, HBii, N
 Scotland1989–19985912264.80A, B, D, G, H, JB, C
 South-East England2002–2006314726.60A, B, C, G, JBii, H
 Washington, USA1990–199491745.20B, GG, H, K, LPersonal communication with author
 San Francisco, USA1970–1986527087.30A, B, C, G, H, LA, D, H, J, NOnly paper to give criteria for familial ALS
 Uruguay2002–200361434.20A, B, H, JBi, I, K, MStates based on detailed family history
 Ireland1995–20095311704.50A, B, C, G, H, LBii, NUp to date registry data (November 2009)
Group 2: retrospective population based study
 New Zealand1985–2006102444.10B, C, D, LBi, G, I, K
 Rochester, USA1925–19983773.90EP
 Modena, Italy1990–199941432.80C, H, J, LBi
 Jefferson County, USA1998–20022365.50B, C, D, G, H, IBi, I, MPrevalence study looking for cluster
 Ontario, Canada1978–198261394.30C, G, HD, H, K, L
 Middle Finland1976–198154311.60D, HD, O
 Northern Sweden1969–198061284.70E, H, MA, D, OStates that 4.7% should be taken as a minimum value
 Cantabria, Spain1974–19853654.60ED, H, IPoor case ascertainment
 Sardinia, Italy1957–198041822.20CDPoor case ascertainment. FALS limited to first degree relative
 South-West Greece1990–200321331.50EBi, F, I, N
 Belgrade, Yugoslavia1985–19911581.70CD, NPoor ascertainment
 Hong Kong1989–19921841.20B, C, L, HBi, F, H, J, L, N
 Northern Denmark1974–198651862.70CD, O
 Hordaland, Norway1970–199071484.70E, F, HD, O
Group 3: prospective case series
Texas, USA1982–199411412009.50EE, NStates that data interpretation must be made in light of non-random enrolment
New York, USA1973–1977336684.90MD
Colorado, USA1989–199141672.40CD, E, N
Group 4: retrospective case series
 Brazil1977–200472512.80EBii, G, I, K, N
 London, UK1965–1984275804.70CDCase notes from 3 London hospitals. 37 FALS cases not verified—rate could be 10%
 Rochester, USAPre 19555589ED
 Lazio, Italy1987–2007235314.30EBii, N
 Novia Scotia, CanadaPre 19743525.80ED, F, H, J, L
 Leuven, Belgium1991–1995121408.60EBi, H, J, LEspecially high rate SOD1 mutation
 Israel1959–197533181C, I, MC, D, E, N
 Netherlands1970–1988203076.50ED, G, I, K
KeyAscertainmentKeyDiagnostic criteria
AProspective registerAPost mortem
BAll neurologistsBEl Escorial criteria
CMulticentre hospital recordsBiEEC definite, probable
DMulticentre discharge recordsBiiEEC definite, probable, suspect
ESingle centre hospital recordsCSimplified EFN criteria
FSingle centre discharge recordsDDiagnosed by neurologist pre-criteria
GALS association recordsEALS rated using institutions own scoring system
HDeath certificatesFSMA included
ICompulsory reportingGSMA excluded
JEMG archivesHPMA included
KControl drug registryIPMA excluded
LPrivate neurologistsJPLS included
MQuestionnaireKPLS excluded
LPSP included
MPSP excluded
NEMG >80% patients
OEMG <80% patients
  • ALS, amyotrophic lateral sclerosis; EEC, El Escorial criteria; FALS, familial amyotrophic lateral sclerosis; PMA, progressive muscular atrophy; PSP, progressive supranuclear palsy; SMA, spinal muscular atrophy; WFN, World Federation of Neurology.