What matters in white matter dementia?

Caixeta, Leonardo

doi:10.1590/S1980-57642008DN10200004

Abstract

Dementia studies has primarily focused on disorders of the cerebral cortex and subcortical gray matter, what originated the concepts of cortical and subcortical dementias respectively. Dementia related mainly with cerebral white matter have received less attention. We present five different cases, each one illustrative of a dementia subtype that could be assigned under the category of 'white matter dementia': CADASIL, progressive subcortical gliosis, progressive multifocal leucoencephalopathy, normopressure hydrocephalus and brain injury. Besides that, recent clinical and scientific literature on white matter dementia was reviewed. The composition of exuberant psychiatric symptoms and personality changes (mainly apathy, but also desinhibition) with neurological signs (pyramidal alone or associated with extrapyramidal signs, ataxia and urinary incontinence) and with specific cognitive impairment (mentioned above), should rise strongly the possibility of a white-matter dementia, instead of a cortical or subcortical form of dementia.

Key words:
dementia; white matter; neuropsychology; psychiatric symptoms.

Resumo

Os trabalhos na área de demência têm enfocado primordialmente os distúrbios do córtex cerebral e da substância cinzenta subcortical, o que originou os conceitos de demência cortical e subcortical, respectivamente. A demência relacionada sobretudo com a substância branca tem recebido menor atenção. Apresentamos cinco casos ilustrativos de subtipos que pudessem ser considerados na categoria de 'demência da substância branca': CADASIL, gliose subcortical progressiva, leucoencefalopatia multifocal progressiva, hidrocefalia de pressão normal e traumatismo craniano. Além disso, conduzimos uma revisão da literatura científica atual sobre demência da substância branca. A composição de sintomas psiquiátricos exuberantes e mudanças de personalidade (sobretudo apatia, mas também desinibição) com sinais neurológicos (sinais piramidais isolados ou em associação com sinais extrapiramidais, ataxia e incontinência urinária) e com prejuízo cognitivo específico (mencionados acima), deve levantar fortemente a possibilidade de uma demência da substância branca, ao invés de uma forma de demência do tipo cortical ou subcortical.

Palavras-chave:
demência; substância branca; neuropsicologia; sintomas psiquiátricos.

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References

¹
Filley CM. Neurobehavioral aspects of cerebral white matter disorders. Psychiatr Clin North Am 2005;28:685-700.
²
Filley CM. White matter and behavioral neurology. Ann N Y Acad Sci 2005;1064:162-183.
³
Filley CM, Franklin GM, Heaton RK. White matter dementia: clinical disorders and implications. Neuropsychiatr Neuropsychol. Behav Neurol 1988;1:239-254.
⁴
Filley CM.The behavioral neurology of cerebral white matter. Neurology 1998;50:1535-1540
⁵
Filley CM. The behavioral neurology of white matter. New York: Oxford University Press; 2001:201-218.
⁶
Rao SM. White matter disease and dementia. Brain Cogn 1996;31:250-268.
⁷
Hurley RA, Tomimoto H, Akiguchi I, Fisher RE, Taber KH. Binswanger's Disease: an Ongoing Controversy. J Neuropsychiatr Clin Neurol 2000;12:301-304.
⁸
Filley CM, Thompson LL, Sze CI, Simon JA, Paskavitz JF, Kleinschmidt-DeMasters BK. White matter dementia in CADASIL. J Neurol Sci 1999;163:163-167.
⁹
de Castro P, Aranguren A, Arteche E, Otano M. Cognitive deterioration in multiple sclerosis. An Sist Sanit Navar 2002;25:167-178.
¹⁰
Dale RC. Acute disseminated encephalomyelitis. Semin Pediatr Infect Dis 2003;14:90-95.
¹¹
Major EO, Ault GS. Progressive Multifocal Leukoencephalopathy: clinical and laboratorial observations in a viral induced demyelinating disease in the immunodeficient patient. Curr Opin Neurol 1995;8:1084-189.
¹²
Thurnher MM. Progressive multifocal leukoencephalopathy in AIDS: initial and follow-up CT and MRI. Neuroradiology 1997;39:611-618.
¹³
Steere AC. Lyme Disease. N Engl J Med 2001:345:115-125.
¹⁴
Andreula C. Cranial viral infections in the adult. Eur Radiol 2004;14(Suppl 3):E132-44.
¹⁵
Tektonidou MG, Varsou N, Kotoulas G, Antoniou A, Moutsopoulos HM. Cognitive deficits in patients with antiphospholipid syndrome: association with clinical, laboratory, and brain magnetic resonance imaging findings. Arch Intern Med 2006;166:2278-2284.
¹⁶
Kirk A, Kertesz A, Polk MJ. Dementia with leukoencephalopathy in systemic lupus erythematosus. Can J Neurol Sci 1991;18:344-348.
¹⁷
Tullberg M, Hultin L, Ekholm S, Mansson JE, Fredman P, Wikkelso C. White matter changes in normal pressure hydrocephalus and Binswanger disease: specificity, predictive value and correlations to axonal degeneration and demyelination. Acta Neurol Scand 2002;105:417-426.
¹⁸
Horster F, Surtees R, Hoffmann GF. Disorders of intermediary metabolism: toxic leukoencephalopathies. J Inherit Metab Dis 2005;28:345-356.
¹⁹
Singhal BS. Leukodystrophies: Indian Scenario. Indian J Pediatr 2005;72:315-318.
²⁰
Reynolds E. Vitamin B12, folic acid, and the nervous system. Lancet Neurol 2006;5:949-960.
²¹
Nakayama N, Okumura A, Shinoda J et al. Evidence for white matter disruption in traumatic brain injury without macroscopic lesions. J Neurol Neurosurg Psychiatry 2006;77:850-855.
²²
Martinez-Mata AM, Martinez-Pardavila R, de Arriba-Villamor C, Espinosa-Mogro H. Cerebral gliomatosis with development of multifocal glioblastoma. Rev Neurol 1999;28:781-3.
²³
Geschwind N. Disconnexion syndromes in animals and man. Brain 1965; 88:237-294.
²⁴
Mesulan MM. Large-scale neurocognitive networks and distributed processing for attention, language, and memory. Ann Neurol 1990;28:597-613.
²⁵
Mesulan MM. Behavioral neuroanatomy: large-scale neural networks, association cortex, frontal syndromes, the limbic system, and hemispheric specializations. In: Mesulan MM, editor. Principles of Behavioral And Cognitive Neurology. 2 ed., New York: Oxford University Press; 2000:1-120.
²⁶
Merriam AE, Hegarty AM. Brain white-matter lesions and psychosis. Br J Psychiatr 1989;155:868-9.
²⁷
Miller BL, Lesser IM, Boone K, et al. Brain white-matter lesions and psychosis. Br J Psychiatr 1989;155:73-78.
²⁸
Eslinger PJ, Grattan LM, Damasio H, Damasio AR. Developmental consequences of childhood frontal lobe damage. Arch Neurol 1992;49:764-769.
²⁹
McAllister TW. Neuropsychiatric sequelae of head injuries. Psychiatr Clin North Am 1992;15:395-413.
³⁰
Gur RC, Parker IK, Hungerbuhler JP, et al. Differences in the distribution of gray and white matter in the human cerebral hemispheres. Science 1980;207:1226-1228.
³¹
Lafosse JM, Corboy JR, Leehey MA, et al. Neuropsychological support for the concept of white matter dementia. Neurology 2002;58(Suppl 3):A355-A356.

Publication Dates

Publication in this collection
Apr-Jun 2007

This is an open-access article distributed under the terms of the Creative Commons Attribution License

[1] ¹
Filley CM. Neurobehavioral aspects of cerebral white matter disorders. Psychiatr Clin North Am 2005;28:685-700.

[2] ²
Filley CM. White matter and behavioral neurology. Ann N Y Acad Sci 2005;1064:162-183.

[3] ³
Filley CM, Franklin GM, Heaton RK. White matter dementia: clinical disorders and implications. Neuropsychiatr Neuropsychol. Behav Neurol 1988;1:239-254.

[4] ⁴
Filley CM.The behavioral neurology of cerebral white matter. Neurology 1998;50:1535-1540

[5] ⁵
Filley CM. The behavioral neurology of white matter. New York: Oxford University Press; 2001:201-218.

[6] ⁶
Rao SM. White matter disease and dementia. Brain Cogn 1996;31:250-268.

[7] ⁷
Hurley RA, Tomimoto H, Akiguchi I, Fisher RE, Taber KH. Binswanger's Disease: an Ongoing Controversy. J Neuropsychiatr Clin Neurol 2000;12:301-304.

[8] ⁸
Filley CM, Thompson LL, Sze CI, Simon JA, Paskavitz JF, Kleinschmidt-DeMasters BK. White matter dementia in CADASIL. J Neurol Sci 1999;163:163-167.

[9] ⁹
de Castro P, Aranguren A, Arteche E, Otano M. Cognitive deterioration in multiple sclerosis. An Sist Sanit Navar 2002;25:167-178.

[10] ¹⁰
Dale RC. Acute disseminated encephalomyelitis. Semin Pediatr Infect Dis 2003;14:90-95.

[11] ¹¹
Major EO, Ault GS. Progressive Multifocal Leukoencephalopathy: clinical and laboratorial observations in a viral induced demyelinating disease in the immunodeficient patient. Curr Opin Neurol 1995;8:1084-189.

[12] ¹²
Thurnher MM. Progressive multifocal leukoencephalopathy in AIDS: initial and follow-up CT and MRI. Neuroradiology 1997;39:611-618.

[13] ¹³
Steere AC. Lyme Disease. N Engl J Med 2001:345:115-125.

[14] ¹⁴
Andreula C. Cranial viral infections in the adult. Eur Radiol 2004;14(Suppl 3):E132-44.

[15] ¹⁵
Tektonidou MG, Varsou N, Kotoulas G, Antoniou A, Moutsopoulos HM. Cognitive deficits in patients with antiphospholipid syndrome: association with clinical, laboratory, and brain magnetic resonance imaging findings. Arch Intern Med 2006;166:2278-2284.

[16] ¹⁶
Kirk A, Kertesz A, Polk MJ. Dementia with leukoencephalopathy in systemic lupus erythematosus. Can J Neurol Sci 1991;18:344-348.

[17] ¹⁷
Tullberg M, Hultin L, Ekholm S, Mansson JE, Fredman P, Wikkelso C. White matter changes in normal pressure hydrocephalus and Binswanger disease: specificity, predictive value and correlations to axonal degeneration and demyelination. Acta Neurol Scand 2002;105:417-426.

[18] ¹⁸
Horster F, Surtees R, Hoffmann GF. Disorders of intermediary metabolism: toxic leukoencephalopathies. J Inherit Metab Dis 2005;28:345-356.

[19] ¹⁹
Singhal BS. Leukodystrophies: Indian Scenario. Indian J Pediatr 2005;72:315-318.

[20] ²⁰
Reynolds E. Vitamin B12, folic acid, and the nervous system. Lancet Neurol 2006;5:949-960.

[21] ²¹
Nakayama N, Okumura A, Shinoda J et al. Evidence for white matter disruption in traumatic brain injury without macroscopic lesions. J Neurol Neurosurg Psychiatry 2006;77:850-855.

[22] ²²
Martinez-Mata AM, Martinez-Pardavila R, de Arriba-Villamor C, Espinosa-Mogro H. Cerebral gliomatosis with development of multifocal glioblastoma. Rev Neurol 1999;28:781-3.

[23] ²³
Geschwind N. Disconnexion syndromes in animals and man. Brain 1965; 88:237-294.

[24] ²⁴
Mesulan MM. Large-scale neurocognitive networks and distributed processing for attention, language, and memory. Ann Neurol 1990;28:597-613.

[25] ²⁵
Mesulan MM. Behavioral neuroanatomy: large-scale neural networks, association cortex, frontal syndromes, the limbic system, and hemispheric specializations. In: Mesulan MM, editor. Principles of Behavioral And Cognitive Neurology. 2 ed., New York: Oxford University Press; 2000:1-120.

[26] ²⁶
Merriam AE, Hegarty AM. Brain white-matter lesions and psychosis. Br J Psychiatr 1989;155:868-9.

[27] ²⁷
Miller BL, Lesser IM, Boone K, et al. Brain white-matter lesions and psychosis. Br J Psychiatr 1989;155:73-78.

[28] ²⁸
Eslinger PJ, Grattan LM, Damasio H, Damasio AR. Developmental consequences of childhood frontal lobe damage. Arch Neurol 1992;49:764-769.

[29] ²⁹
McAllister TW. Neuropsychiatric sequelae of head injuries. Psychiatr Clin North Am 1992;15:395-413.

[30] ³⁰
Gur RC, Parker IK, Hungerbuhler JP, et al. Differences in the distribution of gray and white matter in the human cerebral hemispheres. Science 1980;207:1226-1228.

[31] ³¹
Lafosse JM, Corboy JR, Leehey MA, et al. Neuropsychological support for the concept of white matter dementia. Neurology 2002;58(Suppl 3):A355-A356.

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