CJD
Ismael calandri
26/7/2021
Estadísticos descriptivos
| Characteristic | Afectiva, N = 41 | Clásica, N = 181 | Cognitiva, N = 171 | Heidenhain, N = 141 | Oppenheimer, N = 111 | p-value2 |
|---|---|---|---|---|---|---|
| Demográficos | ||||||
| Edad | 62 (60, 66) | 64 (63, 73) | 64 (55, 72) | 64 (60, 69) | 63 (57, 68) | 0.8 |
| Nacionalidad | >0.9 | |||||
| No | 0 (0%) | 1 (5.6%) | 0 (0%) | 0 (0%) | 0 (0%) | |
| Si | 4 (100%) | 17 (94%) | 17 (100%) | 14 (100%) | 11 (100%) | |
| Derivado desde otro profesional / centro | 0.6 | |||||
| No | 2 (50%) | 14 (78%) | 11 (65%) | 11 (79%) | 9 (82%) | |
| Si | 2 (50%) | 4 (22%) | 6 (35%) | 3 (21%) | 2 (18%) | |
| Antecedentes médicos | ||||||
| Historia familiar de demencia | 0.11 | |||||
| No | 2 (50%) | 14 (78%) | 15 (88%) | 14 (100%) | 9 (82%) | |
| Si | 2 (50%) | 4 (22%) | 2 (12%) | 0 (0%) | 2 (18%) | |
| Historia familiar de enfermedad psquiatrica | 0.3 | |||||
| No | 3 (75%) | 17 (94%) | 17 (100%) | 13 (93%) | 11 (100%) | |
| Si | 1 (25%) | 1 (5.6%) | 0 (0%) | 1 (7.1%) | 0 (0%) | |
| Hisitoria familiar de enfermedad por priones | 0.11 | |||||
| No | 3 (75%) | 18 (100%) | 16 (94%) | 14 (100%) | 11 (100%) | |
| Si | 1 (25%) | 0 (0%) | 1 (5.9%) | 0 (0%) | 0 (0%) | |
| Trabaja en área de salud | ||||||
| No | 4 (100%) | 18 (100%) | 17 (100%) | 14 (100%) | 11 (100%) | |
| Antecedente de cirugías previas | 0.5 | |||||
| No | 1 (25%) | 8 (44%) | 9 (53%) | 9 (64%) | 4 (36%) | |
| Si | 3 (75%) | 10 (56%) | 8 (47%) | 5 (36%) | 7 (64%) | |
| Antecedente de traumatismo encéfalo-craneano | 0.11 | |||||
| No | 4 (100%) | 18 (100%) | 16 (94%) | 13 (93%) | 8 (73%) | |
| Si | 0 (0%) | 0 (0%) | 1 (5.9%) | 1 (7.1%) | 3 (27%) | |
| Antecedentes psiquiatricos | 0.2 | |||||
| No | 3 (75%) | 14 (78%) | 16 (94%) | 14 (100%) | 10 (91%) | |
| Si | 1 (25%) | 4 (22%) | 1 (5.9%) | 0 (0%) | 1 (9.1%) | |
| Clínica | ||||||
| Sintomas corticales focales (total) | 0.7 | |||||
| No | 0 (0%) | 1 (5.6%) | 1 (5.9%) | 2 (14%) | 2 (18%) | |
| Sí | 4 (100%) | 17 (94%) | 16 (94%) | 12 (86%) | 9 (82%) | |
| Síntomas corticales difusos (total) | 0.8 | |||||
| No | 0 (0%) | 2 (11%) | 3 (18%) | 2 (14%) | 3 (27%) | |
| Si | 4 (100%) | 16 (89%) | 14 (82%) | 12 (86%) | 8 (73%) | |
| Síntomas visuales (total) | <0.001 | |||||
| No | 3 (75%) | 17 (94%) | 14 (82%) | 2 (14%) | 8 (73%) | |
| Si | 1 (25%) | 1 (5.6%) | 3 (18%) | 12 (86%) | 3 (27%) | |
| Síntomas conductuales (total) | 0.2 | |||||
| No | 1 (25%) | 7 (39%) | 9 (53%) | 7 (50%) | 9 (82%) | |
| Si | 3 (75%) | 11 (61%) | 8 (47%) | 7 (50%) | 2 (18%) | |
| Síntomas psiquiátricos (total) | 0.13 | |||||
| No | 1 (25%) | 7 (39%) | 10 (59%) | 6 (43%) | 9 (82%) | |
| Si | 3 (75%) | 11 (61%) | 7 (41%) | 8 (57%) | 2 (18%) | |
| Alteración en ciclo sueño-vigilia | >0.9 | |||||
| No | 2 (50%) | 7 (39%) | 9 (53%) | 7 (50%) | 5 (45%) | |
| Si | 2 (50%) | 11 (61%) | 8 (47%) | 7 (50%) | 6 (55%) | |
| Insomnio | 0.3 | |||||
| No | 1 (25%) | 12 (67%) | 12 (71%) | 10 (71%) | 5 (45%) | |
| Si | 3 (75%) | 6 (33%) | 5 (29%) | 4 (29%) | 6 (55%) | |
| Alteración en tono muscular | >0.9 | |||||
| No | 2 (50%) | 8 (44%) | 10 (59%) | 8 (57%) | 5 (45%) | |
| Si | 2 (50%) | 10 (56%) | 7 (41%) | 6 (43%) | 6 (55%) | |
| Ataxia (total) | 0.024 | |||||
| No | 2 (50%) | 8 (44%) | 11 (65%) | 2 (14%) | 2 (18%) | |
| Si | 2 (50%) | 10 (56%) | 6 (35%) | 12 (86%) | 9 (82%) | |
| Mioclonías | 0.6 | |||||
| No | 1 (25%) | 3 (17%) | 7 (41%) | 4 (29%) | 3 (27%) | |
| Si | 3 (75%) | 15 (83%) | 10 (59%) | 10 (71%) | 8 (73%) | |
| Startle | >0.9 | |||||
| No | 3 (75%) | 14 (78%) | 14 (82%) | 11 (79%) | 9 (82%) | |
| Si | 1 (25%) | 4 (22%) | 3 (18%) | 3 (21%) | 2 (18%) | |
|
1
Median (IQR); n (%)
2
Kruskal-Wallis rank sum test; Fisher's exact test
|
||||||
Retraso diagnóstico
## Call: survfit(formula = Surv(data$timedx) ~ 1, data = data)
##
## 4 observations deleted due to missingness
## n events median 0.95LCL 0.95UCL
## 64.0 64.0 13.1 11.7 19.1
## Call: survfit(formula = Surv(data$timedx) ~ data$Variantes, data = data)
##
## 4 observations deleted due to missingness
## n events median 0.95LCL 0.95UCL
## data$Variantes=Afectiva 4 4 20.2 7.00 NA
## data$Variantes=Clásica 18 18 11.8 10.43 23.1
## data$Variantes=Cognitiva 17 17 24.3 12.14 52.4
## data$Variantes=Heidenhain 14 14 10.7 7.14 28.3
## data$Variantes=Oppenheimer 11 11 13.4 12.57 NA
Demora a la RMN
## Call: survfit(formula = Surv(data$timermn) ~ data$Variantes, data = data)
##
## 4 observations deleted due to missingness
## n events median 0.95LCL 0.95UCL
## data$Variantes=Afectiva 4 4 22.00 6.29 NA
## data$Variantes=Clásica 18 18 11.21 8.29 19.0
## data$Variantes=Cognitiva 17 17 23.71 11.14 51.1
## data$Variantes=Heidenhain 14 14 9.43 5.86 27.9
## data$Variantes=Oppenheimer 11 11 13.71 13.14 NA
¿Cuales son las causas de la demora Dx?
Regresion de cox
## beta HR (95% CI for HR) wald.test p.value
## d_derivado 0.17 1.2 (0.67-2.1) 0.33 0.56
## v_Cognitiva -0.85 0.43 (0.23-0.8) 7.2 0.0074
## v_Clasica 0.46 1.6 (0.9-2.8) 2.5 0.11
## timermn -0.29 0.75 (0.7-0.81) 61 5.2e-15
## Dx_diferencial_IM 0.019 1 (0.62-1.7) 0.01 0.94
## Dx_diferencial_DM 0.53 1.7 (0.23-13) 0.27 0.6Que pasen por una institución previa no parece enlentecer el diagnóstico pero sí pertenecer a la variante cognitiva y el tiempo en que se demoran en hacerle la resonancia
Tratamiento
| Characteristic | Afectiva, N = 41 | Clásica, N = 181 | Cognitiva, N = 171 | Heidenhain, N = 141 | Oppenheimer, N = 111 | p-value2 |
|---|---|---|---|---|---|---|
| solumedrol | 0.4 | |||||
| No | 1 (25%) | 12 (67%) | 10 (59%) | 9 (64%) | 9 (82%) | |
| SI | 3 (75%) | 6 (33%) | 7 (41%) | 5 (36%) | 2 (18%) | |
| IVIG | 0.7 | |||||
| No | 4 (100%) | 16 (89%) | 17 (100%) | 13 (93%) | 10 (91%) | |
| Si | 0 (0%) | 2 (11%) | 0 (0%) | 1 (7.1%) | 1 (9.1%) | |
| Recibió tratamiento con hipnóticos | 0.4 | |||||
| No | 2 (50%) | 10 (56%) | 12 (71%) | 5 (36%) | 7 (64%) | |
| Si | 2 (50%) | 8 (44%) | 5 (29%) | 9 (64%) | 4 (36%) | |
| Recibió tratamiento para sus mioclonías | 0.3 | |||||
| No | 3 (75%) | 13 (72%) | 15 (88%) | 9 (64%) | 6 (55%) | |
| Si | 1 (25%) | 5 (28%) | 2 (12%) | 5 (36%) | 5 (45%) | |
| Recibió tratamiento antipsicótico | 0.8 | |||||
| No | 2 (50%) | 10 (56%) | 11 (65%) | 6 (43%) | 7 (64%) | |
| Si | 2 (50%) | 8 (44%) | 6 (35%) | 8 (57%) | 4 (36%) | |
|
1
n (%)
2
Fisher's exact test
|
||||||
#Tiempos de sobrevida
Tiempos hasta la pérdida funcional
## Call: survfit(formula = Surv(TimeDADV, DAVD) ~ Variantes, data = data)
##
## 4 observations deleted due to missingness
## n events median 0.95LCL 0.95UCL
## Variantes=Afectiva 4 4 19.14 1.57 NA
## Variantes=Clásica 18 13 10.57 8.00 NA
## Variantes=Cognitiva 17 15 23.57 10.29 56.3
## Variantes=Heidenhain 14 10 8.71 4.29 NA
## Variantes=Oppenheimer 11 6 39.00 14.57 NA
## Tiempo hasta la muerte
## Call: survfit(formula = Surv(TimeDead, Muerte) ~ 1, data = data)
##
## 4 observations deleted due to missingness
## n events median 0.95LCL 0.95UCL
## 64.0 25.0 66.9 44.0 150.3
## Call: survfit(formula = Surv(TimeDead, Muerte) ~ Variantes, data = data)
##
## 4 observations deleted due to missingness
## n events median 0.95LCL 0.95UCL
## Variantes=Afectiva 4 3 40.2 9.0 NA
## Variantes=Clásica 18 6 30.6 26.4 NA
## Variantes=Cognitiva 17 7 84.6 66.9 NA
## Variantes=Heidenhain 14 3 NA 17.1 NA
## Variantes=Oppenheimer 11 6 101.3 44.0 NA
## Call: survfit(formula = Surv(TimeDead, Muerte) ~ p_resultado14.3.3,
## data = data)
##
## 4 observations deleted due to missingness
## n events median 0.95LCL 0.95UCL
## p_resultado14.3.3=Desconocida 18 4 101.3 60.0 NA
## p_resultado14.3.3=Negativa 14 6 44.0 20.1 NA
## p_resultado14.3.3=No realizada 14 4 169.1 27.6 NA
## p_resultado14.3.3=Positiva 18 11 66.9 30.6 NA
¿Afecta el tratamiento la sobrevida?
## beta HR (95% CI for HR) wald.test p.value
## solumedrol -0.28 0.76 (0.32-1.8) 0.4 0.53
## IVIG 0.31 1.4 (0.31-5.9) 0.17 0.68
## t_ttoinsomnio 0.61 1.8 (0.73-4.6) 1.7 0.19
## t_ttomioclonias 0.76 2.1 (0.86-5.4) 2.7 0.1
## t_ttoAntipsicoticos 1.1 3.1 (1.3-7.3) 6.4 0.011