Intelligence impairment, personality features and psychopathology disturbances in a family affected with CADASIL

  1. Domínguez Sánchez, Francisco Javier 1
  2. Lasa Aristu, Amaia 1
  3. Goñi Imízcoz, Miguel 2
  1. 1 Universidad Nacional de Educación a Distancia
    info

    Universidad Nacional de Educación a Distancia

    Madrid, España

    ROR https://ror.org/02msb5n36

  2. 2 Complejo Asistencial Universitario de Burgos
    info

    Complejo Asistencial Universitario de Burgos

    Burgos, España

    ROR https://ror.org/01j5v0d02

Aldizkaria:
The Spanish Journal of Psychology

ISSN: 1138-7416

Argitalpen urtea: 2011

Alea: 14

Zenbakia: 2

Orrialdeak: 936-943

Mota: Artikulua

DOI: 10.5209/REV_SJOP.2011.V14.N2.39 DIALNET GOOGLE SCHOLAR lock_openSarbide irekia editor

Beste argitalpen batzuk: The Spanish Journal of Psychology

Garapen Iraunkorreko Helburuak

Laburpena

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a small-vessel disease of the brain that is characterized by headache, recurring lacunar strokes, mood changes and progressive cognitive deterioration. The disease is transmitted with an autosomal dominant pattern and usually starts during midadulthood (at 30–50 years of age). Cognitive deficits in patients with CADASIL develop slowly. The dementia causes frontal-like symptoms and it typically develops after a history of recurrent stroke. We describe three patients from one Spanish family affected by this disease. All three cases underwent comprehensive clinical and neuropsychological examination, and were monitored for seven years. The results obtained in this study describe a) a significant loss of the intelligence quotient (IQ) and noticeable damage to abstract ability (g factor), b) mood and psychopathological disturbances (major depression and dysthymia), and c) a personality with neurotic features.

€ Ikusi finantzaketa

Finantzaketari buruzko informazioa

Support for this work was provided by the Research Promotion Plan of the Universidad Nacional de Educación a Distancia (UNED, 2011). We thank Carmen Callejas Hervás for her comments and advice on genetic issues.

Finantzatzaile

Erreferentzia bibliografikoak

  • Aben, I., Denollet, J., Lousberg, R., Verhey, F., Wojciechowski, F., & Honig, A. (2002). Personality and vulnerability to depression in stroke patients. A 1-year prospective follow-up study. Stroke, 33, 2391-2395. doi:10.1161/01.STR.0000029826.41672.2E
  • Amberla, K., Waljas, M., Tuominen, S., Almkvist, O., Poyhonen, M., Tuisku, S., Viitanen, M. (2004). Insidious cognitive decline in CADASIL. Stroke, 35, 1598-1602. doi:10.1161/01.STR.0000129787.92085.0a (Pubitemid 38823632)
  • American Psychiatric Association (2000). Diagnostic and Statistical Manual of Mental Disorders, (4th Ed.), Text Revision (DSM-IV-TR). Washington, DC: American Psychiatric Association.
  • Auer, D. P., Pütz, B., Gossl, Ch., Elbel, G. K., Passer, Th., & Dichgans, M. (2001). Differential lesion patterns in CADASIL and sporadic subcortical arteriosclerotic encephalopathy: MR imaging study with statistical parametric group comparison. Radiology, 218(2), 443-451. (Pubitemid 32121057)
  • Colom, R., Abad, F. J., García, L. F., & Juan-Espinosa, M. (2002). Education, Wechsler's Full Scale IQ, and g. Intelligence, 30, 449-462. doi:10.1016/SO160-2896(02)00122-8
  • Costa, R. M., Honjo, T., & Silva, A. J. (2003). Learning and memory deficits in Notch mutant mice. Current Biology, 13, 1348-1354. doi:10.1016/S0960-9822(03)00492-5 (Pubitemid 36953310)
  • Chabriat, H., Vahedi, K., Iba-Zizen, M. T., Joutel, A., Nibbio, A., Nagy, T. G, Lyon-Caen, O. (1995). Clinical spectrum of CADASIL: a study of 7 families. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy. Lancet, 346, 934-939. doi:10.1016/S0140-6736(95)91557-5
  • Davidson, R. J., Pizagalli, D., Nitschke, J. B., & Putnam, K. (2002). Depression: Perspectives from affective neuroscience. Annual Review of Psychology, 53, 545-574. doi:10.1146/annurev.psych. 53.100901.135148
  • Deary, I. J., Leaper, S. A., Murray, A. D., Staff, R. T., & Whaley, L. J. (2003). Cerebral white matter abnormalities and lifetime cognitive change: A 67-year follow-up of Scottish Mental Survey of 1932. Psychology and Aging, 18, 140-148. doi:10.1037/0882-7974.18.1.140 (Pubitemid 36293051)
  • Deary, I. J., & Matthews, G (1993). Personality traits are alive and well. The Psychologist, 6, 299-311.
  • Duncan, J. (1995). Attention, intelligence, and the frontal lobes. In M. S. Gazzaniga (Ed.), The cognitive neurosciences (pp. 721-733). Cambridge, MA: MIT Press/Bradford Books.
  • First, M. B., Spitzer, R. L., Gibbon, M., & Williams, J. B. W. (1999) Guia del usuario para la Entrevista Clinica Estructurada para los Trastornos del Eje I del DSM-1V. SC1D-I. Version Clinica [Structured Clinical Interview for DSM-IV Axis I Disorders, Clinician Version (SCID-CV)]. Barcelona, Spain: Masson.
  • First, M. B., Gibbon, M., Spitzer, R. L., Williams, J. B. W, & Benjamin, L. S. (1999). Guia del usuario para la Entrevista Clinica Estructurada para los Trastornos del Eje II del DSM-IV (SC1D-II) [Structured Clinical Interview for DSM-IV Personality Disorders, (SCID-II)]. Barcelona, Spain: Masson.
  • Fry, A. F., & Hale, S. (2000). Relationships among processing speed, working memory, and fluid intelligence in children. Biological Psychology, 54, 1-34. doi:10.1016/S0301-0511 (00)00051-X
  • Hathaway, S. R., & McKinley, J. C. (1988). Inventario Multifdsico de Personalidad de Minnesota [Minnesota Multiphasic Personality Inventory]. Madrid, Spain: TEA.
  • Jonas, B. S., & Mussolino, M. E. (2000). Symptoms of depression as a prospective risk factor for stroke. Psychosomatic Medicine, 62(4), 463-471. (Pubitemid 30604788)
  • Joutel, A., Corpechot, C, Ducros, A., Vahedi, K., Chabriat, H., Mouton, P., Tournier-Lasserve, E, (1996). Notch3 mutations in CADASIL, a hereditary adult-onset condition causing stroke and dementia. Nature, 38, 707-710. doi:10.1038/383707a0 (Pubitemid 26360644)
  • Leblanc, G G, Meschia, J. F., Stuss, D. T, & Hachinski, V. (2006). Genetics of vascular cognitive impairment. Stroke, 37, 248-255. doi:10.1161/01.STR.0000195177.61184.49 (Pubitemid 43732087)
  • Lesnik Oberstein, S. A. J., van der Boom, R., Middelkoop, H. A. M., Ferrari, M. D., Knaap, Y. M., van Houwelingen, H. C, Haan, J. (2003). Incipient CADASIL. Archives of Neurology, 60, 707-712. doi:10.1007/s00401-003-0701-6 (Pubitemid 36869243)
  • O'Sullivan, M., Barrick, T. R., Morris, R. G, Clark, C. A., & Markus, H. S. (2005). Damage within a network of white matter regions underlies executive dysfunction in CADASIL. Neurology, 65, 1584-1590. doi:10.1136/jnnp. 2004.045963 (Pubitemid 41081291)
  • Peters, N., Opherk, Ch., Danek, A., Ballard, C, Herzog, J., & Dichgans, M. (2005). The pattern of cognitive performance in CADASIL: a monogenic condition leading to subcortical ischemic vascular dementia. American Journal of Psychiatry, 162, 2078-2085. doi:10.1176/appi.ajp.162.11.2078 (Pubitemid 41598261)
  • Pinillos, J. L. (1982). Cuestionario de Personalidad CEP [CEP personality questionnaire]. Madrid, Spain: TEA.
  • Raven, J. C, Court, J. H., & Raven, J. (1996). Matrices Progresivas. Escala SPM General [Standard Progressive Matrices]. Madrid, Spain: TEA.
  • Roca, M., Parr, A., Thompson, R., Woolgar, A., Torralva, T., Antoun, N., Duncan, J. (2010). Executive function and fluid intelligence after frontal lobe lesions. Brain, 133, 234-247. doi:10.1093/brain/awp269
  • Ruchoux, M-M., & Maurage, C-A. (1997). CADASIL: cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy. Journal of Neuropathology and Experimental Neurology, 56, 947-964. (Pubitemid 27386125)
  • Steffens, D. C, Krishnan, R. R., Crump, C, & Burke, G L. (2002). Cerebrovascular disease and evolution of depressive symptoms in the Cardiovascular Health Study. Stroke, 33, 1636-1644. doi:10.1161/01.STR. 0000018405.59799.D5 (Pubitemid 34619971)
  • Trojano, L., Ragno, M., Manca, A., & Caruso, G (1998). A kindred affected by cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL): a 2-year neuropsychological follow-up. Journal of Neurology, 245, 217-222. doi:10.1007/s004150050208 (Pubitemid 28192696)
  • van den Boom, R., Lesnik Oberstein, S. A., Ferrari, M. D., Haan, J., & van Buchem, M. A. (2003). Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy: MR imaging findings at different ages - 3rd-6th decades. Radiology, 229(3), 683-690. doi:10.1148/radiol. 2293021354 (Pubitemid 37467569)
  • Verin, M., Rolland, Y., Landgraf, F., Chabriat, H., Bompais, B. Michael, A., Martinet, J. P. (1995). New phenotype of the cerebral autosomal dominant arteriopathy mapped to chromosome 19: migraine as the prominent clinical feature. Journal of Neurology, Neurosurgery and Psychiatry, 59, 579-585. doi:10.1136/jnnp.59.6.579
  • Wahlund, L. O., Barkhof, R, Fazekas, F., Bronge, L., Augustin, M., SjSgren, M Scheltens, P. (2001). A new rating scale for age-related white matter changes applicable to MRI and CT. Stroke, 32, 1318-1322. doi:10.1161/01.STR.32. 6.1318 (Pubitemid 32530416)
  • Wechsler, D. (1977). Escala de Inteligencia de Weschler para Adultos (WAIS) [Wechsler Adult Intelligence Scale]. Madrid, Spain: TEA.
  • Wechsler D. (1997). Escala de Inteligencia de Weschler para Adultos-III (WAIS-III) [Wechsler Adult Intelligence Scale - third edition (WAIS-III)]. Madrid, Spain: TEA.
Datuen iturria: Dialnet