Tabla I. Tests neuropsicológicos 2018: media (desviación estándar). |
|
Dígitos directos, span a |
5,16 (0,969) |
Dígitos inversos, span a |
3,74 (1,290) d |
Localización directos, span a |
5,17 (1,117) |
Localización inversos, span a |
4,23 (1,278) d |
Letras y números, span a |
4,00 (0,830) |
Test de retención visual de Benton-forma C a |
5,47 (2,583) |
Cubos-total a |
3,19 (1,662) d |
Cubos-tiempo a |
7,58 (4,766) |
Brief Test of Attention-percentiles a |
39,61 (29,681) |
Trail Making Test-B a |
158,26 (95,358) d |
Recuerdo libre total b |
–0,52 (1,151) |
Recuerdo libre a corto plazo b |
–0,26 (0,965) |
Recuerdo con claves a corto plazo b |
–0,16 (1,098) |
Recuerdo libre a largo plazo b |
–0,23 (1,146) |
Recuerdo con claves a largo plazo b |
–0,13 (1,204) |
Perseveraciones b |
0,06 (1,063) |
Intrusiones en el recuerdo libre b |
0,03 (1,278) |
Reconocimiento-aciertos b |
0 (1,125) |
DEX-Sp total de la familia a |
21,91 (17,167) d |
Memoria de trabajo c |
0,55 (0,506) |
Habilidades visuoconstructivas c |
0,29 (0,461) e |
Memoria verbal c |
0,77 (0,425) |
Atención alternante c |
0,29 (0,461) e |
Atención sostenida c |
0,68 (0,475) |
DEX-SP: versión española del cuestionario disejecutivo. a Puntuaciones directas; b Puntuaciones estandarizadas (media, 0; desviación estándar, 1); c Dominios neurocognitivos (1, preservado; 0, afectado); d Tests afectados basándose en los puntos de corte; e Dominios más afectados. |
Tabla II. Estadísticos de contraste 2015-2018. |
|||||
Tests |
Basal, 2015 |
Seguimiento, 2018 |
Z a |
Sig. asintótica (bilateral) |
|
Memoria de trabajo |
Dígitos directos, span |
5,42 (1,349) |
5,17 (0,963) |
–0,918 b |
0,359 |
Dígitos inversos, span |
4,29 (1,301) |
3,83 (1,090) |
–1,238 b |
0,216 |
|
Localización directos, span |
5,29 (1,781) |
5,04 (1,065) |
–0,852 b |
0,394 |
|
Localización inversos, span |
4,42 (0,881) |
4,35 (1,112) |
–0,262 b |
0,794 |
|
Letras y números |
4,54 (1,532) |
4,08 (0,881) |
–1,968 b |
0,049 d |
|
Habilidades visuoconstructivas y visuoespaciales |
Test de retención visual de Benton-forma C |
8,00 (3,022) |
5,61 (2,675) |
–3,179 b |
0,001 d |
Cubos total |
3,71 (1,681) |
3,38 (1,789) |
–0,639 b |
0,523 |
|
Atención alternante |
Trail Making Test-B |
167,21 (111,717) |
159,83 (99,627) |
–0,070 b |
0,945 |
Memoria verbal |
Recuerdo libre total |
–0,08 (1,100) |
–0,54 (1,103) |
–1,615 b |
0,106 |
Recuerdo libre a corto plazo |
–0,04 (0,859) |
–0,25 (0,944) |
–1,355 b |
0,175 |
|
Recuerdo de claves a corto plazo |
–0,08 (1,060) |
–0,13 (1,076) |
–0,421 b |
0,674 |
|
Recuerdo libre a largo plazo |
–0,08 (1,139) |
–0,17 (1,129) |
–0,925 b |
0,355 |
|
Recuerdo de claves a largo plazo |
–0,04 (1,233) |
–0,08 (1,176) |
–0,292 b |
0,770 |
|
Reconocimiento |
0,33 (1,167) |
0,13 (0,992) |
–1,140 b |
0,254 |
|
Discriminación |
0 (1,063) |
0,17 (0,816) |
–0,690 c |
0,490 |
|
Sesgos |
0,333 (0,963) |
0,083 (0,775) |
–1,364 b |
0,172 |
|
Comportamiento disejecutivo |
DEX-Sp |
24,74 (17,552) |
21,55 (17,478) |
–0,597 b |
0,551 |
DEX-SP: versión española del cuestionario disejecutivo. a Prueba de los rangos con signo de Wilcoxon; b Basado en los rangos positivos; c Basado en los rangos negativos; d p < 0,05. |
Tabla III. Diagnóstico neuropsicológico: frecuencias. |
||||
Basal, 2015 |
Porcentaje válido |
Seguimiento, 2018 |
Porcentaje válido |
|
Demencia en la enfermedad de Steinert |
3 |
12,5% |
6 |
19,3% |
Deterioro cognitivo leve unidominio |
3 |
12,5% |
9 |
29% |
Deterioro cognitivo leve multidominio |
12 |
50% |
13 |
41,9% |
Sin déficits significativos |
6 |
25% |
3 |
9,6% |
Neuropsychological profile in patients with myotonic dystrophy type 1: a four-year follow-up study Introduction. Myotonic dystrophy type 1 (MD1), or Steinert’s disease, is a multisystemic disorder of autosomal dominant inheritance, whose adult variant usually presents with multidomain cognitive impairment and affects patients’ functionality and quality of life. Aim. To study the four-year history of cognitive functioning in a sample of patients with the adult variant of MD1. Patients and methods. The neurocognitive functions of a sample of 31 patients with MD1 are evaluated, of whom 24 repeat the test administered four years ago in the Neurology Service of the Complejo Hospitalario of Navarra. Data are collected from the cognitive domains that are most related to the deficits that usually present in MD1. Results. The follow-up evaluation found that the visuospatial and visuoconstructive functions and alternating attention of the patients who underwent the study were affected, as was their daily functioning reported by the family. These results are in line with those obtained four years earlier, with no significant deterioration observed between the two measurements. A higher incidence of cognitive impairment was also displayed in 2018, with some cases of progression to dementia in Steinert’s disease. Conclusion. Neurocognitive progression in MD1 seems to respond to a progressive pattern of degeneration, linked to the functions that are most affected from the beginning of the sequelae phase and which usually correspond to the domains of working memory, alternating attention, and visuospatial and visuoconstructive abilities. Key words. Cognitive impairment. Dysexecutive syndrome. Myotonic dystrophy. Neurodegenerative pattern. Neuropsychological progress. |