Clinical syndromes andepidemiology of the FTLD-spectrum disorders in Latin          America
Outline• Clinical syndromes of FTLD.• Prevalence and incidence of FTLD.• Estimating number of cases of the FTLD in LA.• Me...
Outline• Clinical syndromes of FTLD.• Prevalence and incidence of FTLD.• Estimating number of cases of the FTLD in LA.• Me...
“On the relationship between senile  cerebral atrophy and aphasia”            .… The patient had marked aphasia: his under...
Arnold Pick and “focal dementia”“….the clinical findings of senile dementia can be interpreted as a mosaic ofcircumscribed...
Alzheimer and histopathological descriptionALZHEIMER A. Über eigenartige Krankheitsfälledes späteren Alters. Zeitschrift f...
“Pick’s disease” or “Pick complex”?                                 Pick’s disease• Europe: Clinical diagnosis, with or wi...
Fronto-temporal lobar degeneration• Clinical, genetic, and pathological heterogeneous group of disorders.• Is a macro-anat...
International consensus criteria for behavioural                              variant FTDA.   Early behavioural disinhibit...
Primary Progressive Aphasia      Progressive aphasia nonfluent                        Semantic dementia                   ...
Outline• Clinical syndromes of FTLD.• Prevalence and incidence of FTLD.• Estimating number of cases of the FTLD in LA.• Me...
Estimated growth of dementiaThe number of people with dementia will roughly double every 20 years,         with the bigges...
Prevalence of different types of dementia by                             age group   Prevalence of dementia in Canada, by ...
Studies of prevalence of FTLD                                                              Point estimate perLocation     ...
Studies of incidence of FTLDLocation                    Cases (n)    Case definition   Rate per 100,000 per year    95 % C...
Frequency of FTLD in Brescia county according    to age at onset of symptoms (n=226)        22/100,000        78/100,000  ...
The very high estimates of prevalence in persons over           age 65 years don’t reflect neuropathological FTLD         ...
Outline• Clinical syndromes of FTLD.• Prevalence and incidence of FTLD.• Estimating number of cases of the FTLD in LA.• Me...
Prevalence of dementia in eight Latin American       studies, according to age groups               Nitrini R, et al. Inte...
Prevalence of dementia according to gender between          pooled data of Latin American and European studies            ...
Causes of dementia in the 103 cases: Lima-Peru    Diagnosis                            n                          %    Pro...
Causes of dementia in individuals over 65 years             of age: Population-based three studies in LA                  ...
Prevalence of presenile dementia           in a tertiary outpatient clinic: Sao Paulo-BrazilEtiology                      ...
Estimating number of cases of the FTLD in Peru                                                     Point      Estimating  ...
Outline• Clinical syndromes of FTLD.• Prevalence and incidence of FTLD.• Estimating number of cases of the FTLD in LA.• Me...
Studies of survival in FTLD                                               Basis of        No of    Mean aged      Delay in...
FTD follows a more malignant diseasecourse than AD once dementia is clinically recognized                            Rasco...
Behavioral variant DFT (bvDFT) progresses more          rapidly than other subtypes                        Roberson ED, et...
Survival in semantic dementia overlaps           Alzheimer’s disease                    Roberson ED, et al. Neurology 2005...
Patients with definite bvDFT have a poor prognosiswhich is worse if language deficits are also present                    ...
Outline• Clinical syndromes of FTLD.• Prevalence and incidence of FTLD.• Estimating number of cases of the FTLD in LA.• Me...
Evaluation of costs of Alzheimer-type dementiain Bs As according to patient’s place of residence              Allegri RF, ...
Evaluation of costs of Alzheimer-type dementia        in Bs As by severity of dementia             Allegri RF, et al. Inte...
Direct costs of Alzheimer’s, frontotemporal and  vascular dementia in Argentina: 2002-2008               Rojas G, et al. I...
Drug-medicines cost analysis of Alzheimer’s, fronto-   temporal and vascular dementia in Argentina                Rojas G,...
Conclusions•   Historically, researchers have used a varied nomenclature to describe FTLD.•   Clinical profile and the und...
Nilton Custodioniltoncustodio@neuroconsultas.com       www.neuroconsultas.com        neuroconsultas        twitter.com/neu...
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Clinical Syndromes and Epidemiology of the FTLD-Spectrum Disorders in Latin America

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Belohorizonte 06 de Junio 2012

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Clinical Syndromes and Epidemiology of the FTLD-Spectrum Disorders in Latin America

  1. 1. Clinical syndromes andepidemiology of the FTLD-spectrum disorders in Latin America
  2. 2. Outline• Clinical syndromes of FTLD.• Prevalence and incidence of FTLD.• Estimating number of cases of the FTLD in LA.• Mean survival time of FTLD.• Economic impact of FTLD in LA.
  3. 3. Outline• Clinical syndromes of FTLD.• Prevalence and incidence of FTLD.• Estimating number of cases of the FTLD in LA.• Mean survival time of FTLD.• Economic impact of FTLD in LA.
  4. 4. “On the relationship between senile cerebral atrophy and aphasia” .… The patient had marked aphasia: his understanding of speech was substantially, though not completely, disturbed. He could understand simple questions about generalities and about things familiar to him; other things he did not understand at all. The patient was over-talkative. If his sentences were about simple things they were occasionally correct, otherwise they were unintelligible. This was partly because correct words were used in the wrong order and partly because some of his words were nonsensical. This occasionally resulted from the re-arrangement of consonants; so, for example, for the target word ‘locomotive’, he produced ‘colmolotive’; for the word ‘Kleiderkasten’, he said ‘Reideklasten’; and so on…In reading aloud, for Ostende he responded ‘Oste, ost, u te te, Ostus, tentinde……. PICK A. Über die Beziehungen der senilen Atrophie zur Aphasie. Prager Medizinische Wochenschrift,17: 165-167, 1892.
  5. 5. Arnold Pick and “focal dementia”“….the clinical findings of senile dementia can be interpreted as a mosaic ofcircumscribed deficits of higher mental abilities (‘Herderscheinungen’); this factmay fail to be revealed when the process of atrophy occurs simultaneously atmany places, thereby masking the appearance of the single symptoms” PICK A. Senile Hirnatrophie als Grundlage für Herderscheinungen. Wiener Klinische Wochenschrift, 14:403-404, 1901.
  6. 6. Alzheimer and histopathological descriptionALZHEIMER A. Über eigenartige Krankheitsfälledes späteren Alters. Zeitschrift für die gesamteNeurologie und Psychiatrie, 4: 356-385, 1911.
  7. 7. “Pick’s disease” or “Pick complex”? Pick’s disease• Europe: Clinical diagnosis, with or without pathologically proven Pick bodies.• America: Pathological diagnosis, irrespective of the clinical presentation. Pick complex CBD PSP PPA bvFTD FTLD-ALS
  8. 8. Fronto-temporal lobar degeneration• Clinical, genetic, and pathological heterogeneous group of disorders.• Is a macro-anatomical descriptive term reflecting the relatively selective involvement of frontal and temporal lobes.• The clinical spectrum: – Predominant behavioral symptoms: behavioral variant FTLD (bvFTD). – Deterioration of language function: Primary Progressive Aphasia (PPA) • Progressive Nonfluent Aphasia (PNFA). • Semantic Dementia (SD). • Logopenic Progressive Aphasia (logopenic PPA).
  9. 9. International consensus criteria for behavioural variant FTDA. Early behavioural disinhibition.B. Early apathy or inertia.C. Early loss of sympathy or empathy.D. Early perseverative, stereotyped or compulsive/ritualistic behaviour.E. Hyperorality and dietary changes.F. Neuropsychological profile: executive/generation deficits with relative sparing of memory and visuospatial functions. Rascovsky K, et al. Brain 2011;134:2456-2477.
  10. 10. Primary Progressive Aphasia Progressive aphasia nonfluent Semantic dementia Logopenic aphasiaAt least one of the following: Both of the following: Both of the following: Agrammatism.  Impaired confrontation naming  Impaired single-word retrieval in Effortful, halting speech with inconsistent  Impaired single-word comprehension spontaneous speech and naming. speechsound errors and distortions At least 3 of the following:  Impaired repetition of sentences and phrases.At least 2 of 3 of the following:  Impaired object knowledge, particularly for low frequency or At least 3 of the following: Impaired comprehension of syntactically complex sentences. low-familiarity items  Speech (phonologic) errors in spontaneous speech and naming. Spared single-word comprehension.  Surface dyslexia or dysgraphia  Spared single-word comprehension Spared object knowledge  Spared repetition. and object Knowledge.  Spared speech production (grammar  Spared motor speech. and motor speech)  Absence of frank agrammatism.
  11. 11. Outline• Clinical syndromes of FTLD.• Prevalence and incidence of FTLD.• Estimating number of cases of the FTLD in LA.• Mean survival time of FTLD.• Economic impact of FTLD in LA.
  12. 12. Estimated growth of dementiaThe number of people with dementia will roughly double every 20 years, with the biggest increases in developing countries Alisson Abbott. Nature 2011;475:S2-S4
  13. 13. Prevalence of different types of dementia by age group Prevalence of dementia in Canada, by type of Prevalence of different types of dementia dementia, sex and age group- rates per 1,000 in the 45- to 64-year age group per 100,000 population in Cambridgeshire Women Men Both Prevalence rate/1,000 rate/1,000 rate/1,000 Diagnosis (95% CI)All dementia 86 69 80 65-74 28 19 24 Alzheimer’s disease 15.1 75-84 116 104 111 Frontotemporal dementia 15.1 85+ 371 287 345Alzheimer’s disease 58 38 51 Vascular dementia 6 65-74 14 5 10 75-84 78 55 69 Huntington’s disease 14 85+ 288 196 260Vascular dementia 12 19 15 65-74 4 8 6 Parkinsonian syndromes 5 75-84 19 31 24 85+ 46 52 48CSHA Working group. Neurology 2000;55:66-73 Ratnavalli E, et al. Neurology 2002;85:1615
  14. 14. Studies of prevalence of FTLD Point estimate perLocation Cases (n) Case definition 95 % CI 100,000 in 45–64 y/oZuid-Holland, Netherlands 55 bvFT only 4.0 2.8 – 5.7(Rosso et al. 2003)Cambridgeshire, UK 11 bvFTD + PPA 15 8.4 – 27.0(Ratnavalli et al. 2002)London, UK 18 bvFTD 15.4 9.1 – 24.3(Harvey et al. 2003)Brescia, Italy 213 bvFTD + PPA 22 17 – 27(Borroni et al. 2010)Ibaraki, Japon 17 bvFTD only 2.0 1.3 – 3.2(Ikejima et al. 2009) Knopman D, Roberts R. J Mol Neurosc 2011;45:330-335
  15. 15. Studies of incidence of FTLDLocation Cases (n) Case definition Rate per 100,000 per year 95 % CIRochester, Minnesota 4 bvFTD + PPA 4.1 (rango edad: 40-69) 2.8 – 5.7(Knopman et al. 2004)Cambridgeshire, UK 16 bvFTD + PPA 3.5 (rango edad: 45-64) 8.4 – 27.0(Mercy et al. 2008)Girona, Spain 14 bvFTD + SD 2.7 (rango edad: 45-64) 1.3 – 3.2(Garre-Olmo et al. 2010) Knopman D, Roberts R. J Mol Neurosc 2011;45:330-335
  16. 16. Frequency of FTLD in Brescia county according to age at onset of symptoms (n=226) 22/100,000 78/100,000 54/100,000 n=108 n=97 n=21 Borroni B, et al. J Alzheimers Dis 2010;19:111-116
  17. 17. The very high estimates of prevalence in persons over age 65 years don’t reflect neuropathological FTLD Total Functional disability 1998 criteria Common sample and neuroimaging sample sample (n=176) sample (n=154) (n=152) (n=137)Gender (F/M) 72/104 65/89 64/88 60/77Age at onset 58.1 (10.9) 58.4 (11.1) 57.8 (10.9) 58.1 (11.1)Age at initial evaluation 61.5 (10.9) 61.7 (11.0) 61.3 (10.9) 61.5 (11.0)Age at death 66.1 (11.6) 66.4 (11.7) 65.8 (11.6) 65.8 (11.6)Education 14.2 (3.5) 14.3 (3.4) 14.2 (3.5) 14.2 (3.5)MMSE 22.2 (7.0) 22.5 (6.9) 22.2 (7.1) 22.3 (7.1)Duration: onset-initial evaluation 3.2 (2.7) 3.2 (2.6) 3.2 (2.6) 3.3 (2.6)Duration: onset-death 7.8 (3.9) 7.6 (3.9) 7.7 (3.9) 7.6 (3.9)Duration: initial evaluation-death 4.6 (3.9) 4.4 (3.1) 4.5 (3.3) 4.3 (3.1) Rascovsky K, et al. Brain 2011;134:2456-2477.
  18. 18. Outline• Clinical syndromes of FTLD.• Prevalence and incidence of FTLD.• Estimating number of cases of the FTLD in LA.• Mean survival time of FTLD.• Economic impact of FTLD in LA.
  19. 19. Prevalence of dementia in eight Latin American studies, according to age groups Nitrini R, et al. International Psychogeriatrics 2009;21:622-630.
  20. 20. Prevalence of dementia according to gender between pooled data of Latin American and European studies Latino american studies European studies Women Men Women MenAge Dem Partic. Prevalence Dem Partic. Prevalence Preval. Preval. (%) n n (%) (95% CI) n n (%) (95% CI) (%)65-69 149 5620 2.65 (2.25-3.10) 79 3 479 2.27 (1.80-2.81) 1.0 1.670-74 196 4781 4.10 (3.55-4.69) 65 2 317 2.81 (2.17-3.57) 3.1 2.975-79 293 3802 7.71 (6.89-8.59) 112 1 888 5.93 (4.90-7.09) 6.0 5.680-84* 291 2326 12.51 (11.17-13.94) 162 1 489 10.88 (9.34-2.55) 12.6 11.085-89 281 1244 22.59 (20.30-24.97) 182 960 18.96 (16.49-21.55) 20.2 12.890+ 189 500 37.80 (33.56-42.28) 105 390 26.92 (22.54-31.67) 30.8 22.1
  21. 21. Causes of dementia in the 103 cases: Lima-Peru Diagnosis n % Probable AD 51 49.5 Possible AD 7 6.8 Vascular dementia 9 8.7 AD with CVD 16 15.5 Parkinson’s dementia 3 2.9 Lewy-body dementia 2 1.9 Frontotemporal dementia 2 1.9 Undetermined cause 13 12.7 Custodio N, et al . An Fac Med 2008;69(4):233-238
  22. 22. Causes of dementia in individuals over 65 years of age: Population-based three studies in LA Lima Catanduva Sao PauloDiagnosis (Total sample: 1532) (Total sample: 1656) (Total sample: 1563) n % n % n %AD 58 56.3 65 55.1 64 59.8VD 9 8.7 11 9.3 17 15.9AD+VD 16 15.5 17 14.4 9 8.4PD 3 2.9 4 3.4 1 0.9FTD 2 1.9 3 2.6 - -LBD 2 1.9 2 1.7 1 0.9Vitamin B12 deficiency - - 1 0.8 - -Alcoholic dementia - - - - 5 4.7Undetermined cause 13 12.7 15 12.7 10 9.3Total 103 100.0 118 100.0 107 100.0
  23. 23. Prevalence of presenile dementia in a tertiary outpatient clinic: Sao Paulo-BrazilEtiology n %Vascular dementia 52 36.9Probable AD 18 12.8Possible AD 12 8.5Traumatic brain injury 13 9.2Frontotemporal dementia 7 5.0Alcoholic dementia 7 5.0Normal pressure hydrocephalus 6 4.2Depression 6 4.2Anoxic encephalopaty 4 2.8Miscellanea 16 11.3 Fujihara S, et al. Arq Neuropsquiatr 2004;62:592-595.
  24. 24. Estimating number of cases of the FTLD in Peru Point Estimating Censo No of estimate number of 2007 cases per 100,000 cases Over 65 y/o 1’764,687 2 6 105 15 622 45-64 y/o 4’147,131 - 22 912 Number of cases of FTLD in Peru: 727 - 1017
  25. 25. Outline• Clinical syndromes of FTLD.• Prevalence and incidence of FTLD.• Estimating number of cases of the FTLD in LA.• Mean survival time of FTLD.• Economic impact of FTLD in LA.
  26. 26. Studies of survival in FTLD Basis of No of Mean aged Delay in Survival fromLocation diagnosis subjects at diagnosis diagnosis onset or diagnosisSurvival from diagnosis ClinicalSan Francisco (Robertson et al. 2005) 177 58.5 ± 9.4 4.5 ± 2.9 3.6 ± 0.4 diagnoses PathologicallySan Diego (Rascovsky et al. 2005) 70 65 ± 9.4 4.0 ± 2.8 4.2 confirmed ClinicalSidney (Garcin et al. 2009) 91 57.2 ± 8.2 3.6 ± 2.5 4.2 ± 0.8 diagnoses PathologicallyCambridge and Sidney (Hodges et al. 2003) 61 61.5 ± 7.6 3 3.0 ± 0.4 confirmedSurvival from onset PathologicallyRochester MN (Josephs et al 2005) 45 57.3 ± 11.1 - 6.6 confirmed PathologicallyPhiladelphia (Xie et al. 2008) 71 61.0 ± 9.5 1 ±1 6.6 ± 0.5 confirmed ClinicalNetherlands (Chiu et al. 2010) 354 57.5 ± 8.9 - 9.9 ± 0.7 diagnoses Knopman D, Roberts R. J Mol Neurosc 2011;45:330-335
  27. 27. FTD follows a more malignant diseasecourse than AD once dementia is clinically recognized Rascovsky K, et al. Neurology 2005;65:397-403
  28. 28. Behavioral variant DFT (bvDFT) progresses more rapidly than other subtypes Roberson ED, et al. Neurology 2005;65:719-725
  29. 29. Survival in semantic dementia overlaps Alzheimer’s disease Roberson ED, et al. Neurology 2005;65:719-725
  30. 30. Patients with definite bvDFT have a poor prognosiswhich is worse if language deficits are also present Garcin B, et al. Neurology 2009;73:1656-1661
  31. 31. Outline• Clinical syndromes of FTLD.• Prevalence and incidence of FTLD.• Estimating number of cases of the FTLD in LA.• Mean survival time of FTLD.• Economic impact of FTLD in LA.
  32. 32. Evaluation of costs of Alzheimer-type dementiain Bs As according to patient’s place of residence Allegri RF, et al. International Psycogeriatrics 2007;19:705-718
  33. 33. Evaluation of costs of Alzheimer-type dementia in Bs As by severity of dementia Allegri RF, et al. International Psycogeriatrics 2007;19:705-718
  34. 34. Direct costs of Alzheimer’s, frontotemporal and vascular dementia in Argentina: 2002-2008 Rojas G, et al. International Psycogeriatrics 2011;23:554-561
  35. 35. Drug-medicines cost analysis of Alzheimer’s, fronto- temporal and vascular dementia in Argentina Rojas G, et al. International Psycogeriatrics 2011;23:554-561
  36. 36. Conclusions• Historically, researchers have used a varied nomenclature to describe FTLD.• Clinical profile and the underlying pathological changes are heterogeneous in FLTD.• Two broad presentations are recognized: progressive deterioration in social function and personality and insidious decline in language skills.• Several research groups have used passive surveillance methods to estimate prevalence or incidence of the cognitive syndromes of the FTLDs.• Epidemiological studies suggest that FTLD is the second most common cause of young onset dementia after AD.• bvDFT progresses more rapidly than other subtypes, which is worse if language deficits are also present.• In Latin America, the costs to treat DFT are higher than for AD, but less than for DV, and has high costs in the use of psychotropic drugs.
  37. 37. Nilton Custodioniltoncustodio@neuroconsultas.com www.neuroconsultas.com neuroconsultas twitter.com/neuroconsultas neuroconsultas.blogspot.com slideshare.net/neuroconsultas
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