Table 1

Relevant  characteristics and neurological signs in cases with pure ALS (n=17) with/without cognitive impairment in Papua, 2001–2012

Case numberALS criteriaSurvey yearVillageAgeSexOnset (age/year)Duration (years)Death (year)Tribe (origin)UMN signsLMN signsBulbar signsParkinsonian signCognitive
impairment
Family
history
DTRBSMAFascTADysTRBKPI
U/LU/LU/LU/LU/L
ALS 1Definite2001Bade35MNCNCNCAuyu3/3+++/+++/+++
ALS 2Definite2001Ogoto43FNCNC2002Auyu3/3+++/+++/+++++
ALS 3Definite2002Senggo50M48/'002NCCitak3/3+++/++±/±±+1
ALS 4Definite2007Homilikia53M51/'0522007Auyu3/2+++/+++/+
ALS 5Definite2007/'08Bade51M49/'0632009Auyu3/4++/−−/−+
ALS 6Definite2008Bade21M11/'98112009Auyu4/4+++/++−/−2
ALS 7Definite2007/'08/'10Bade70F57/'97132010Maluku3/3+++/+++/−+3
ALS 8Probable2011Geturki53M48/'065AliveAuyu3/3+/+−/+
ALS 9Definite2011Muya41F36/'0662012Auyu3/3++/+−/−
ALS 10Probable2007/'10/'11/'12Osso65F59/'066AliveAuyu3/4++/−−/−+4
ALS 11Probable2007/'08/'10/'12Gimikya32M12/'9220AliveAuyu4/4+++/+++/−±+/−5
ALS 12Definite2010/'11/'12Bosma64M53/'0111AliveAuyu3/4+++/+++/−++6
ALS 13Definite2012Asset70F68/'102AliveAuyu3/3+/+−/−
ALS 14Probable2012Pies65F60/'075AliveAuyu3/4+/+−/−
ALS 15Definite2007Bade64F62/'0522007Maluku3/3+++/++−/−++7
ALS 16Definite2008/'10/'11Yibin25M13/'99132012Auyu3/4+++/+++/+++
ALS 17Definite2012Ogorito70M66/'084AliveAuyu3/3++/++/++
  • Age: at the time of final survey; Alive: alive in 2012; Deep tendon reflexes: −, absent; 1, hypoactive; 2, normal; 3, hyperactive; 4, markedly hyperactive with ankle clonus; Duration: between subjective onset of illness and last survey or death; Onset: subjective onset of illness.

  • Other signs are classified as: −, absent; ±, questionably present; +, present; ++, markedly present.

  • Family history: 1, cousin (possible ALS); 2, grandfather (NC); 3, sister (ALS 15); 4, cousin's daughter (P 16); 5, cousin (NC); 6, father (NC); 7, sister (ALS 7).

  • ALS, amyotrophic lateral sclerosis; BK, bradykinesia; BS, Babinski sign; DTR, deep tendon reflex; Dys, dysphagia/dysarthria; F, female; Fasc, fasciculation; LMN, lower motor neuron signs; M, male; MA, muscle atrophy with weakness; NC, not confirmed by examination; P, parkinsonism; PI, postural impairment; R, rigidity; T, tremor; TA, tongue atrophy; U/L: upper limbs/lower limbs; UMN, upper motor neuron signs; Yahr stage, the scale of Hoehn and Yahr.