Table 2

Relevant characteristics and neurological signs in cases with overlapping ALS and parkinsonism (n=13) with/without cognitive impairment in Papua, 2001–2012

Case numberALS criteria, Yahr stageSurvey yearVillageAgeSexOnset (age/year)Duration (year)Death (year)Tribe (origin)UMN signsLMN signsBulbar signsParkinsonian signCognitive impairmentFamily history
ALS-P 1Possible, IV2008Pies65F60/'0362009Auyu3/3++/−−/−+/+++/+++++8
ALS-P 2Possible, III2010/'11Ogorito76M70/'0562011Auyu3/3++/±−/−+/++/+++
ALS-P 3Possible, III2010/' 11/'12Homilikia69M65/'084AliveAuyu3/2++/−−/−+/++/+++
ALS-P 4Definite, III2008Bade34M18/'92172009Muyu3/3++/−−/−+++++/++++9
ALS-P 5Definite, III2012Sien60M58/'102AliveAuyu3/3++/+−/−+/++++
ALS-P 6Definite, III2012Benggo50M48/'102AliveAuyu2/2++/+−/−+++/−+++
ALS-P 7Possible, II2001Bade36MNCNCNCAuyu3/3+−/−−/−+++
ALS-P 8Possible, II2008/'10Bade23M11/'9812AliveAuyu3/4+−/−−/−+/++/±+
ALS-P 9Possible, III2010/'11Ogorito76M70/'0562011Auyu3/3+−/−−/−+/−+/+++
ALS-P 10Possible, III2008/' 10/'11Yibin52M44/'038AliveAuyu3/3+−/−−/−+/−+/+++
ALS-P 11Possible, III2011Getio55F53/'0922011Auyu3/3+±/−−/−+/++/+++
ALS-P 12Possible, IV2001Bade40MNCNCNCAuyu3/3+−/−−/−++++++
ALS-P 13Possible, III2012Bosma52M48/'084AliveAuyu3/4+−/−−/−++/++++
  • 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: 8, sister (P 6) and cousin (NC); 9, father (NC).

  • 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.