Cerebral white matter: Neuroanatomy, clinical neurology, and neurobehavioral correlates

Annals of the New York Academy of Sciences

Volumn 1142, Issue , 2008, Pages 266-309

  Schmahmann, Jeremy D ^a   Smith, Eric E ^a   Eichler, Florian S ^a   Filley, Christopher M ^b,c  

^a MASSACHUSETTS GENERAL HOSPITAL   (United States)

^b UNIVERSITY OF COLORADO SCHOOL OF MEDICINE   (United States)

^c VETERANS AFFAIRS MEDICAL CENTER   (United States)

Author keywords

Cognition; Demyelination; Fiber tracts; Neuropsychiatry; Vascular dementia

Indexed keywords

AGING; ANTERIOR COMMISSURE; ARCUATE FASCICULUS; AUTOIMMUNE DISEASE; BEHAVIOR; BRAIN DISEASE; BRAIN INFECTION; BRAIN INJURY; BRAIN STEM; BRAIN TOXICITY; BRAIN TUMOR; CAPSULA INTERNA; CEREBROVASCULAR DISEASE; CINGULATE GYRUS; CLINICAL FEATURE; COGNITIVE DEFECT; COMPUTER ASSISTED TOMOGRAPHY; CORPUS CALLOSUM; DEGENERATIVE DISEASE; DEMYELINATING DISEASE; ENCEPHALITIS; EXTREME CAPSULE; FRONTOOCCIPITAL FASCICULUS; GENETIC DISORDER; HIPPOCAMPUS; HUMAN; HYDROCEPHALUS; INFERIOR LONGITUDINAL BUNDLE; MENTAL DISEASE; METABOLIC ENCEPHALOPATHY; MIDDLE LONGITUDINAL FASCICULUS; MURATOFF BUNDLE; NERVE FIBER; NERVE PROJECTION; NERVE TRACT; NEUROANATOMY; NEUROIMAGING; NEUROPATHOLOGY; NONHUMAN; NUCLEAR MAGNETIC RESONANCE IMAGING; PATHOPHYSIOLOGY; REVIEW; RISK ASSESSMENT; SAGITTAL STRATUM; SUPERIOR LONGITUDINAL BUNDLE; THALAMUS; UNCINATE FASCICULUS; WHITE MATTER;

EID: 54949136087     PISSN: 00778923     EISSN: 17496632     Source Type: Book Series    
DOI: 10.1196/annals.1444.017     Document Type: Review

References (309)

1. Neuburger, M. 1897. Die historische Entwicklung der experimentellen Gehirn- und Rückenmarksphysiologie vor Flourens. Ferdinand Enke Verlag, Stuttgart. Translated and edited, with additional material, by Edwin Clarke (1981) The Historical Development of Experimental Brain and Spinal Cord Physiology before Flourens. Johns Hopkins University Press. Baltimore and London.
2. Schmahmann, J.D. & D.N. Pandya. 2006. Fiber Pathways of the Brain. Oxford University Press. New York.
3. Schmahmann, J.D. & D.N. Pandya. 2007. Cerebral white matter-historical evolution of facts and notions concerning the organization of the fiber pathways of the brain. J. Hist. Neurosci. 16 : 237 267.
4. Geschwind, N. 1965. Disconnexion syndromes in animals and man. I. Brain 88 : 237 294.
5. Geschwind, N. 1965. Disconnexion syndromes in animals and man. II. Brain 88 : 585 644.
6. Filley, C.M., G.M. Franklin, R.K. Heaton & N.L. Rosenberg. 1988. White matter dementia. Clinical disorders and implications. Neuropsychiatry Neuropsychol. Behav. Neurol. 1 : 239 254.
7. Nauta, W.J.H. 1964. Some efferent connections of the prefrontal cortex in the monkey. In The Frontal Granular Cortex and Behavior. J.M. Waren & K. Akert, Eds. : 397 409. McGraw-Hill. New York.
8. Luria, A.R. 1966. Higher cortical functions in man. Prefaces to the English. In Authorized Translation from the Russian, by Basil Haigh. H.-L. Teuber & K.H. Pribram, Eds. : Basic Books. New York.
9. Pandya, D.N. & H.G. Kuypers. 1969. Cortico-cortical connections in the rhesus monkey. Brain Res. 13 : 13 36.
10. Jones, E.G. & T.P. Powell. 1970. An anatomical study of converging sensory pathways within the cerebral cortex of the monkey. Brain 93 : 793 820.
11. Mesulam, M.-M. 1981. A cortical network for directed attention and unilateral neglect. Ann. Neurol. 10 : 309 325.
12. Mesulam, M.-M. 2000. Principles of Behavioral and Cognitive Neurology. 2nd ed. Oxford University Press. New York.
13. Ungerleider, L.G. & M. Mishkin. 1982. Two cortical visual systems. In Analysis of Visual Behavior. D.J. Ingle, M.A. Goodale & R.J.W. Mansfield, Eds. : 549 586. MIT Press. Cambridge, MA.
14. Goldman-Rakic, P.S. 1988. Topography of cognition: parallel distributed networks in primate association cortex. Annu. Rev. Neurosci. 11 : 137 156.
15. Basser, P.J., J. Mattiello & D. LeBihan. 1994. MR diffusion tensor spectroscopy and imaging. Biophys. J. 66 : 259 267.
16. Wedeen, V.J., T.L. David, R.M. Weiskoff, et al. 1995. White matter connectivity explored by MRI. Hum. Brain Mapp. (Suppl 1 36.
17. Wedeen, V.J., R.P. Wang, J.D. Schmahmann, et al. 2008. Diffusion spectrum magnetic resonance imaging (DSI) tractography of fiber crossings. NeuroImage 41 : 1267 1277.
18. Conturo, T.E, N.F. Lori, T.S. Cull, et al. 1999. Tracking neuronal fiber pathways in the living human brain. Proc. Natl. Acad. Sci. USA 96 : 10422 10427.
19. Catani, M., R.J. Howard, S. Pajevic & D.K. Jones. 2002. Virtual in vivo interactive dissection of white matter fasciculi in the human brain. NeuroImage 17 : 77 94.
20. Garrison, F.H. 1969. History of neurology. Revised and enlarged with a bibliography of classical, original, and standard works in neurology. By Lawrence C. McHenry Jr. with a foreword by Derek E. Denny-Brown. Thomas. Springfield, IL.
21. Steno (Stensen) N. 1671. Dissertatio de cerebri anatome, spectatissimis viris dd Societatis apud dominum Thevenot collectae, dictata, atque è gallico exemplari. Parisiis edito 1669. Latinitate donata, opera & studio Guidonis Fanosii.
22. Reil, J.C. 1809. Archiv für die Physiologie. Halle, Curtschen Buchhandlung 9 : 136 208.
23. Burdach, K.F. (CF) (1819-1826). Vom Baue und Leben des Gehirns. Zweyter Band, 1822 Leipzig. Dritter band, 1826. Leipzig. In der Dyk'schen Buchhandlung.
24. Gall, F.J. & G. Spurzheim. 1810. Anatomie et Physiologie du Systeme Nerveux en general, et du Cerveau en particulier. Atlas. Paris, Chez F. Schoell.
25. Meynert, T. (1871 1872). Vom Gerhirne der Säugethiere. In Handbuch der Lehre von den Geweben des Menschen und der Thiere. Vol. 2. S. Stricker, Ed. : 694 808. Leipzig. Englemann. Translated by Henry Power in three volumes (1870-1873): Meynert T. (1872). The brain of mammals. In: Stricker S. (ed.) Manual of human and comparative histology. New Sydenham Society, London. Volume 2, pages 367-537.
26. Dejerine, J.J. 1895. Anatomie des Centres Nerveux. Rueff et Cie. Paris.
27. Wernicke, C. 1874. Der aphasische Symptomencomplex. Eine psychologische Studie auf anatomischer Basis. Breslau. Cohn.
28. Dejerine, J.J. 1892. Contribution à l'étude anatomo-pathologique et clinique des différentes variétés de cécité verbale. Mém. Soc. Biol. 4 : 61 90.
29. Schmahmann, J.D., Pandya, D.N. 2008. Disconnection syndromes of basal ganglia, thalamus and cerebrocerebellar systems. Cortex 44 : 1037 1066.
30. Schmahmann, J.D., D.N. Pandya, R. Wang, et al. 2007. Association fiber pathways of the brain: pParallel observations from diffusion spectrum imaging and autoradiography. Brain, 130 : 630 653.
31. Makris, N., A.J. Worth, A.G. Sorensen, et al. 1997. Morphometry of in vivo human white matter association pathways with diffusion-weighted magnetic resonance imaging. Ann. Neurol. 42 : 951 962.
32. Makris, N., D.N. Kennedy, S. McInerney, et al. 2005. Segmentation of subcomponents within the superior longitudinal fascicle in humans: a quantitative, in vivo, DT-MRI study. Cereb. Cortex 15 : 854 869.
33. Lehéricy, S., M. Ducros, A. Krainik, et al. 2004. 3-D diffusion tensor axonal tracking shows distinct SMA and pre-SMA projections to the human striatum. Cereb. Cortex 14 : 1302 1309.
34. Johansen-Berg, H., T.E. Behrens, E. Sillery, et al. 2005. Functional-anatomical validation and individual variation of diffusion tractography-based segmentation of the human thalamus. Cereb. Cortex 15 : 31 39.
35. Raichle, M.E., A.M. MacLeod, A.Z. Snyder, et al. 2001. A default mode of brain function. Proc. Natl. Acad. Sci. USA 98 : 676 682.
36. Greicius, M.D., K. Supekar, V. Menon & R.F. Dougherty. 2008. Resting-State Functional Connectivity Reflects Structural Connectivity in the Default Mode Network. Cereb. Cortex April 9, 2008. [Epub ahead of print]
37. Ghashghaei, H.T. & H. Barbas. 2002. Pathways for emotion: interactions of prefrontal and anterior temporal pathways in the amygdala of the rhesus monkey. Neuroscience 115 : 1261 1279.
38. Foltz, E.L. & L.E. White. 1962. Pain "relief" by frontal cingulumotomy. J. Neurosurg. 19 : 89 100.
39. Ballantine, H.T., W.L. Cassidy, N.B. Flanagan & R. Marino Jr. 1967. Stereotaxic anterior cingulotomy for neuropsychiatric illness and intractable pain. J. Neurosurg. 26 : 488 495.
40. Ballantine, H.T., A.J. Bouckoms, E.K. Thomas & I.E. Gitiunas. 1987. Treatment of psychiatric illness by stereotactic cingulotomy. Biol. Psychiatry 22L : 807 819.
41. Jenike, M.A., L. Baer, H.T. Ballantine, et al. 1991. Cingulotomy for refractory obsessive-compulsive disorder. Arch. Gen. Psychiatry 48 : 548 555.
42. Spangler, W.J., G.R. Cosgrove, H.T. Ballantine, et al. 1996. Magnetic resonance image-guided stereotactic cingulotomy for intractable psychiatric disease. Neurosurgery 38 : 1071 1078.
43. Price, B.H., I. Baral, G.R. Cosgrove, et al. 2001. Improvement in severe self-mutilation following limbic leucotomy: A series of 5 consecutive cases. J. Clin. Psychiatry 62 : 925 932.
44. Cosgrove, G.R. & S.L. Rauch. 2003. Stereotactic cingulotomy. Neurosurg. Clin. N. Am. 14 : 225 235.
45. Schmahmann, J.D. & D.N. Pandya. 2007. The complex history of the fronto-occipital fasciculus. J. Hist. Neurosci. 16 : 362 377.
46. Gazzaniga, M.S. 1967. The human brain is actually two brains, each capable of advanced mental functions. When the cerebrum is divided surgically, it is as if the cranium contained two separate spheres of consciousness. Sci. Am. 217 : 24 29.
47. Gazzaniga, M.S. 2000. Cerebral specialization and interhemispheric communication: does the corpus callosum enable the human condition? Brain 123 : 1293 1326.
48. Sperry, R.W. 1964. The great cerebral commissure. Sci. Am. 210 : 42 52.
49. Sperry, R. 1984. Consciousness, personal identity and the divided brain. Neuropsychologia 22 : 661 673.
50. Bogen, J.E. & G.M. Bogen. 1988. Creativity and the corpus callosum. Psychiatr. Clin. North Am. 11 : 293 301.
51. Demeter, S., D.L. Rosene & G.W. Van Hoesen. 1985. Interhemispheric pathways of the hippocampal formation, presubiculum, and entorhinal and posterior parahippocampal cortices in the rhesus monkey: the structure and organization of the hippocampal commissures. J. Comp. Neurol. 233 : 30 47.
52. Schmahmann, J.D. 1984. Hemi-inattention from right hemisphere subcortical infarction. Boston Society of Neurology and Psychiatry.
53. Tatemichi, T.K., D.W. Desmond, I. Prohovnik, et al. 1992. Confusion and memory loss from capsular genu infarction: a thalamocortical disconnection syndrome? Neurology 42 : 196 1979.
54. Chukwudelunzu, F.E., J.F. Meschia, N.R. Graff-Radford & J.A. Lucas. 2001. Extensive metabolic and neuropsychological abnormalities associated with discrete infarction of the genu of the internal capsule. J. Neurol. Neurosurg. Psychiatry 71 : 658 662.
55. Anderson, D. & A. Ahmed. 2003. Treatment of patients with intractable obsessive-compulsive disorder with anterior capsular stimulation. Case report. J. Neurosurg. 98 : 1104 1108.
56. Kumar, K., C. Toth & R.K. Nath. 1997. Deep brain stimulation for intractable pain: a 15-year experience. Neurosurgery 40 : 736 746.
57. von Bechterew, W. 1885. Zur Anatomie der Schenkel des Kleinhirns, insbesondere der Brückenarme. Neurologisches Centralblatt 4 : 121 125.
58. Schmahmann, J.D. 1991. An emerging concept: The cerebellar contribution to higher function. Arch. Neurol. 48 : 1178 1187.
59. Schmahmann, J.D. 2004. Disorders of the cerebellum. Ataxia, dysmetria of thought, and the cerebellar cognitive affective syndrome. J. Neuropsychiatry Clin. Neurosci. 16 : 367 378.
60. Bezman, L., A.B. Moser, G.V. Raymond, et al. 2001. Adrenoleukodystrophy: incidence, new mutation rate, and results of extended family screening. Ann. Neurol. 49 : 512 517.
61. Moser, H., K. Smith, P. Watkins, et al. 2000. X-linked adrenoleukodystrophy. In The Metabolic and Molecular Bases of Inherited Disease. C. Scriver et al., Eds. : 3257 3301. McGraw-Hill. New York.
62. van Geel, B.M., L. Bezman, D.J. Loes, et al. 2001. Evolution of phenotypes in adult male patients with X-linked adrenoleukodystrophy. Ann. Neurol. 49 : 186 194.
63. Eichler, F.S., A. Mahmood, D. Loes, et al. 2007. Magnetic resonance imaging detection of lesion progression in adult patients with X-linked adrenoleukodystrophy. Arch. Neurol. 659 664.
64. Edwin, D., L.J. Speedie, W. Kohler, et al. 1996. Cognitive and brain magnetic resonance imaging findings in adrenomyeloneuropathy. Ann. Neurol. 40 : 675 678.
65. Aubourg, P., N. Sellier, J.L. Chaussain & G. Kalifa. 1989. MRI detects cerebral involvement in neurologically asymptomatic patients with adrenoleukodystrophy. Neurology 39 : 1619 1621.
66. Melhem, E.R., P.B. Barker, G.V. Raymond & H.W. Moser. 1999. X-linked adrenoleukodystrophy in children: review of genetic, clinical, and MR imaging characteristics. AJR Am. J. Roentgenol. 173 : 1575 1581.
67. Melhem, E.R., D.J. Loes, C.S. Georgiades, et al. 2000. X-linked adrenoleukodystrophy: the role of contrast-enhanced MR imaging in predicting disease progression. AJNR Am. J. Neuroradiol. 21 : 839 844.
68. Eichler, F.S., P.B. Barker, C. Cox, et al. 2002. Proton MR spectroscopic imaging predicts lesion progression on MRI in X-linked adrenoleukodystrophy. Neurology 58 : 901 907.
69. Eichler, F.S., R. Itoh, P.B. Barker, et al. 2002. Proton MR spectroscopic and diffusion tensor brain MR imaging in X-linked adrenoleukodystrophy: initial experience. Radiology 225 : 245 252.
70. Schaumburg, H.H., J.M. Powers, C.S. Raine, et al. 1975. Adrenoleukodystrophy. A clinical and pathological study of 17 cases. Arch. Neurol. 32 : 577 591.
71. Eichler, F.S., J.Q. Ren, M. Cossoy, et al. 2008. Is microglial apoptosis an early pathogenic change in cerebral X-ALD? Ann. Neurol. 63 : 729 742.
72. Austin, J.H. 1959. Metachromatic sulfatides in cerebral white matter and kidney. Proc. Soc. Exp. Biol. Med. 100 : 361 364.
73. Heinisch, U., J. Zlotogora, S. Kafert & V. Gieselmann. 1995. Multiple mutations are responsible for the high frequency of metachromatic leukodystrophy in a small geographic area. Am. J. Hum. Genet. 56 : 51 57.
74. Hagberg, B. 1971. Clinical aspects of globoid cell and metachromatic leukodystrophies. Birth Defects Orig. Artic. Ser. 7 : 103 112.
75. Von Hirsch, T. & J. Peiffer. 1955. [Histological methods in differential diagnosis of leukodystrophy from lipoidosis.]. Arch. Psychiatr. Nervenkr Z Gesamte Neurol. Psychiatr. 194 : 88 104.
76. Austin, J., D. Armstrong, S. Fouch, et al. 1968. Metachromatic leukodystrophy (MLD). VIII. MLD in adults: diagnosis and pathogenesis. Arch. Neurol. 18 : 225 240.
77. Filley, C.M. & K.F. Gross. 1992. Psychosis with cerebral white matter disease. Neuropsychiatry Neuropsychol. Behav. Neurol. 5 : 119 125.
78. Hyde, T.M., J.C. Ziegler & D.R. Weinberger. 1992. Psychiatric disturbances in metachromatic leukodystrophy. Insights into the neurobiology of psychosis. Arch. Neurol. 49 : 401 406.
79. Faerber, E.N., J. Melvin & E.M. Smergel. 1999. MRI appearances of metachromatic leukodystrophy. Pediatr. Radiol. 29 : 669 672.
80. Van Der Voorn, J.P., W. Kamphorst, M.S. van der Knaap & J.M. Powers. 2004. The leukoencephalopathy of infantile GM1gangliosidosis: oligodendrocytic loss and axonal dysfunction. Acta Neuropathol. (Berl) 107 : 539 545.
81. Kobayashi, T., N. Shinnoh, I. Goto, et al. 1985. Galactosylceramide- and lactosylceramide-loading studies in cultured fibroblasts from normal individuals and patients with globoid cell leukodystrophy (Krabbe's disease) and GM1 gangliosidosis. Biochim. Biophys. Acta 835 : 456 464.
82. Miyatake, T. & K. Suzuki. 1972. Globoid cell leukodystrophy: additional deficiency of psychosine galactosidase. Biochem. Biophys. Res. Commun. 48 : 539 543.
83. Wenger, D., K. Suzuki & Y.S. Suzuki. 2000. Galactosylceramide lipidosis: Globoid cell leukodystrophy (Krabbe Disease). In The Metabolic and Molecular Basis of Inherited Disease. C. Scriver, et al. Eds. : 3669 3694. McGraw-Hill. New York.
84. Lyon, G., B. Hagberg, P. Evrard, et al. 1991. Symptomatology of late onset Krabbe's leukodystrophy: the European experience. Dev. Neurosci. 13 : 240 244.
85. Barone, R., K. Bruhl, P. Stoeter, et al. 1996. Clinical and neuroradiological findings in classic infantile and late-onset globoid-cell leukodystrophy (Krabbe disease). Am. J. Med. Genet. 63 : 209 217.
86. Farina, L., A. Bizzi, G. Finocchiaro, et al. 2000. MR imaging and proton MR spectroscopy in adult Krabbe disease. AJNR Am. J. Neuroradiol. 21 : 1478 1482.
87. Baram, T.Z., A.M. Goldman & A.K. Percy. 1986. Krabbe disease: specific MRI and CT findings. Neurology 36 : 111 115.
88. Nagar, V.A., M.A. Ursekar, P. Krishnan & B.G. Jankharia. 2006. Krabbe disease: unusual MRI findings. Pediatr. Radiol. 36 : 61 64.
89. Powers, J.M. 1996. A neuropathologic overview of the neurodystrophies and neurolipidoses. In Neurodystrophies and Neurolipidosis. Vol. 22. H.W. Moser, Ed. : Elsevier Science B.V.
90. Sourander, P., H.A. Hansson, Y. Olsson & L. Svennerholm. 1966. Experimental studies on the pathogenesis of leucodystrophies. II. The effect of sphingolipids on various cell types in cultures from the nervous system. Acta Neuropathol. (Berl) 6 : 231 242.
91. Takashima, S., A. Matsui, Y. Fujii & H. Nakamura. 1981. Clinicopathological differences between juvenile and late infantile metachromatic leukodystrophy. Brain Dev. 3 : 365 374.
92. Van Der Knaap, M.S., P.A. Leegwater, A.A. Konst, et al. 2002. Mutations in each of the five subunits of translation initiation factor eIF2B can cause leukoencephalopathy with vanishing white matter. Ann. Neurol. 51 : 264 270.
93. Van Der Knaap, M.S., J.C. Pronk & G.C. Scheper. 2006. Vanishing white matter disease. Lancet Neurol. 5 : 413 423.
94. Hanefeld, F., U. Holzbach, B. Kruse, et al. 1993. Diffuse white matter disease in three children: an encephalopathy with unique features on magnetic resonance imaging and proton magnetic resonance spectroscopy. Neuropediatrics 24 : 244 248.
95. Schiffmann, R., J.R. Moller, B.D. Trapp, et al. 1994. Childhood ataxia with diffuse central nervous system hypomyelination. Ann. Neurol. 35 : 331 340.
96. Scheper, G.C., J. Mulder, M. Kleijn, et al. 1997. Inactivation of eIF2B and phosphorylation of PHAS-I in heat-shocked rat hepatoma cells. J. Biol. Chem. 272 : 26850 26856.
97. Van Haren, K., J.P. van der Voorn, D.R. Peterson, et al. 2004. The life and death of oligodendrocytes in vanishing white matter disease. J. Neuropathol. Exp. Neurol. 63 : 618 630.
98. Marotti, J.D., S. Tobias & J.D. Fratkin. 2004. Adult onset leukodystrophy with neuroaxonal spheroids and pigmented glia: Report of a family, historical perspective, and review of the literature. Acta Neuropathol. (Berl) 107 : 481 488.
99. Freeman, S.H., B.T. Hyman, K.B. Sims, et al. 2008. Adult onset leukodystrophy with neuroaxonal spheroids: clinical, neuroimaging and neuropathologic observations. Brain Pathol. Apr 15. [Epub ahead of print].
100. Pavlakis, S.G., P.C. Phillips, S. DiMauro, et al. 1984. Mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes: a distinctive clinical syndrome. Ann. Neurol. 16 : 481 488.
101. Hirano, M., E. Ricci, M.R. Koenigsberger, et al. 1992. Melas: an original case and clinical criteria for diagnosis. Neuromuscul. Disord. 2 : 125 135.
102. Castillo, M., L. Kwock & C. Green. 1995. MELAS syndrome: imaging and proton MR spectroscopic findings. AJNR Am. J. Neuroradiol. 16 : 233 239.
103. Tanahashi, C., A. Nakayama, M. Yoshida, et al. 2000. MELAS with the mitochondrial DNA 3243 point mutation: a neuropathological study. Acta Neuropathol. 99 : 31 38.
104. Thomeer, E.C., W.M. Verhoeven, C.J. van de Vlasakker & J.L. Klompenhouwer. 1998. Psychiatric symptoms in MELAS; a case report. J. Neurol. Neurosurg. Psychiatry 64 : 692 693.
105. Kim, H.G., J.D. Schmahmann, K. Sims, et al. 1999. A neuropsychiatric presentation of mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes. Med. Psychiatry 2 : 3 9.
106. Leehey, M.A., R.P. Munhoz, A.E. Lang, et al. 2003. The fragile X premutation presenting as essential tremor. Arch. Neurol. 60 : 117 121.
107. Jacquemont, S., R.J. Hagerman, M. Leehey, et al. 2003. Fragile X premutation tremor/ataxia syndrome: molecular, clinical, and neuroimaging correlates. Am. J. Hum. Genet. 72 : 869 878.
108. Greco, C.M., R.F. Berman, R.M. Martin, et al. 2006. Neuropathology of fragile X-associated tremor/ataxia syndrome (FXTAS). Brain 129 : 243 255.
109. Grigsby, J., A.G. Brega, K. Engle, et al. 2008. Cognitive profile of fragile X premutation carriers with and without fragile X-associated tremor/ataxia syndrome. Neuropsychology 22 : 48 60.
110. Schmahmann, J.D. & D.N. Pandya. 1997. The cerebrocerebellar system. In The Cerebellum and Cognition. J.D. Schmahmann, Ed. Academic Press. San Diego. Int. Rev. Neurobiol. 41: 31-60.
111. Medana, I.M. & M.M. Esiri. 2003. Axonal damage: a key predictor of outcome in human CNS diseases. Brain 126 : 515 530.
112. Charcot, J.M. 1877. Lectures on the diseases of the nervous system delivered at La Salpêtrière. New Sydenham Society. London.
113. Kurtzke, J.F. 1970. Neurologic impairment in multiple sclerosis and the Disability Status Scale. Acta Neurol. Scand. 46 : 493 512.
114. Rao, S.M., G.J. Leo, L. Bernardin & F. Unverzagt. 1991. Cognitive dysfunction in multiple sclerosis. I. Frequency, patterns, and prediction. Neurology 41 : 685 691.
115. Boerner, R.J. & H.P. Kapfhammer. 1999. Psychopathological changes and cognitive impairment in encephalomyelitis disseminata. Eur. Arch. Clin. Neurosci. 249 : 96 102.
116. Filley, C.M. 2001a. The Behavioral Neurology of White Matter. Oxford University Press. New York.
117. Wattjes, M.P., M. Harzheim, G.G. Lutterbey, et al., 2008. Prognostic value of high-field proton magnetic resonance spectroscopy in patients presenting with clinically isolated syndromes suggestive of multiple sclerosis. Neuroradiology 50 : 123 129.
118. Zivadinov, R., M. Stosic, J.L. Cox, et al. 2008. The place of conventional MRI and newly emerging MRI techniques in monitoring different aspects of treatment outcome. J. Neurol. 255 (Suppl 1 61 74.
119. Stadelmann, C., M. Albert, C. Wegner & W. Brück. 2008. Cortical pathology in multiple sclerosis. Curr. Opin. Neurol. 1 : 229 234.
120. Catalaa, I., J.C. Fulton, X. Zhang, et al. 1999. MR imaging quantitation of gray matter involvement in multiple sclerosis and its correlation with disability measures and neurocognitive testing. AJNR Am. J. Neuroradiol. 20 : 1613 1618.
121. Filley, C.M. 2001. Neurobehavioral Anatomy. 2nd ed. University Press of Colorado. Boulder, CO.
122. de Seze, J., M. Debouverie, H. Zephir, et al. 2007. Acute fulminant demyelinating disease: a descriptive study of 60 patients. Arch. Neurol. 64 : 1426 1432.
123. Young, N.P., B.G. Weinshenker & C.F. Lucchinetti. 2008. Acute disseminated encephalomyelitis: current understanding and controversies. Semin. Neurol. 28 : 84 94.
124. Sonneville, R., S. Demeret, I. Klein, et al. 2008. Acute disseminated encephalomyelitis in the intensive care unit: clinical features and outcome of 20 adults. Intensive Care Med. 34 : 528 532.
125. Tenembaum, S., T. Chitnis, J. Ness & J.S. Hahn. International Pediatric MS Study Group. 2007. Acute disseminated encephalomyelitis. Neurology 68 (16 Suppl 2 S23 S36.
126. Navia, B.A., E-S. Cho, C.K. Petito & R.W. Price. 1986. The AIDS dementia complex: II. Neuropathology. Ann. Neurol. 19 : 525 535.
127. Jones, H.R., D.D. Ho, P. Forgacs, et al. 1988. Acute fulminating fatal leukoencephalopathy as the only mainfestation of human immunodeficiency virus infection. Ann. Neurol. 23 : 519 522.
128. Navia, B.A., B.D. Jordan & RW. Price. 1986. The AIDS dementia complex: I. Clinical features. Ann. Neurol. 19 : 517 524.
129. Tozzi, V., P. Narciso, S. Galgani, et al. 1993. Effects of zidovudine in 30 patients with mild to end-stage AIDS dementia complex. AIDS 7 : 683 692.
130. Fillippi, C.G., G. Sze, S.J. Farber, et al. 1998. Regression of HIV encephalopathy and basal ganglia signal intensity abnormality at MR imaging in patient with AIDS after the initiation of protease inhibitor therapy. Radiology 206 : 491 498.
131. Thurnher, M.M., E.G. Schindler, S.A. Thurnher, et al. 2000. Highly active antiretroviral therapy for patients with AIDS dementia complex: effect on MR imaging findings and clinical course. AJNR Am. J. Neuroradiol. 21 : 670 678.
132. Chiang, M-C., R.A. Dutton, K.M. Hayashi, et al. 2007. 3D patterns of brain atrophy in HIV/AIDS visualized using tensor-based morphometry. NeuroImage 34 : 44 60.
133. Greenlee, J.E. 2006 Nov. Progressive multifocal leucoencephalopathy in the era of natalizumab: a review and discussion of the implications. Int. MS J. 13 : 100 107.
134. von Einsiedel, R.W., T.D. Fife, A.J. Aksamit, et al. 1993. Progressive multifocal leukoencephalopathy in AIDS: a clinicopathologic study and review of the literature. J. Neurol. 240 : 391 406.
135. Langer-Gould, A., S.W. Atlas, A.J. Green, et al. 2005. Progressive multifocal leukoencephalopathy in a patient treated with natalizumab. N. Engl. J. Med. 353 : 375 381.
136. Moll, N.M., A.M. Rietsch, A.J. Ransohoff, et al. 2008. Cortical demyelination in PML and MS: Similarities and differences. Neurology 70 : 336 343.
137. Garrels, K., W. Kucharczyk, G. Wortzman & M. Shandling. 1996. Progressive multifocal leukoencephalopathy: clinical and MR response to treatment. AJNR Am. J. Neuroradiol. 17 : 597 600.
138. Arai, Y., Y. Tsutsui, K. Nagashima, et al. 2002. Autopsy case of the cerebellar form of progressive multifocal leukoencephalopathy without immunodeficiency. Neuropathology 22 : 48 56.
139. Kastrup, O., M. Maschke, H.C. Diener & I. Wanke. 2002. Progressive multifocal leukoencephalopathy limited to the brain stem. Neuroradiology 44 : 227 229.
140. Giancola, M.L., E.B. Rizzi, P. Lorenzini, et al. 2008. Progressive multifocal leukoencephalopathy in HIV-infected patients in the era of HAART: radiological features at diagnosis and follow-up and correlation with clinical variables. AIDS Res. Hum. Retroviruses 24 : 155 162.
141. West, S.G. 1994. Neuropsychiatric lupus. Rheum. Dis. Clin. North Am. 20 : 129 158.
142. Kozora, E., L.L. Thompson, S.G. West & B.L. Kotzin. 1996. Analysis of cognitive and psychological deficits in systemic lupus erythematosus patients without overt central nervous system disease. Arthritis Rheum. 39 : 2035 2045.
143. Kirk, A., A. Kertesz & M.J. Polk. 1991. Dementia with leukoencephalopathy in systemic lupus erythematosus. Can. J. Neurol. Sci. 18 : 344 348.
144. Kozora, E., D.B. Arciniegas, C.M. Filley, et al. 2005. Cognition, MRS neurometabolites, and MRI volumetrics in non-neuropsychiatric systemic lupus erythematosus. Cogn. Behav. Neurol. 8 : 159 162.
145. Filley, C.M. & B.K. Kleinschmidt-DeMasters. 2001. Toxic leukoencephalopathy. N. Engl. J. Med. 345 : 425 432.
146. Reddick, W.E., J.O. Glass, K.J. Helton, et al. 2005. Prevalence of leukoencephalopathy in children treated for acute lymphoblastic leukemia with high-dose methotrexate. AJNR Am. J. Neuroradiol. 26 : 1263 1269.
147. Haykin, M.E., M. Gorman, J. van Hoff, et al. 2006. Diffusion-weighted MRI correlates of subacute methotrexate-related neurotoxicity. J. Neurooncol. 76 : 153 157.
148. Hormes, J.T., C.M. Filley & N.L. Rosenberg. 1986. Neurologic sequelae of chronic solvent vapor abuse. Neurology 36 : 698 702.
149. Rosenberg, N.L., M.C. Spitz, C.M. Filley, et al. 1988. Central nervous system effects of chronic toluene abuse - clinical, brainstem evoked response and magnetic resonance imaging studies. Neurotoxicol. Teratol. 10 : 489 495.
150. Filley, C.M., R.K. Heaton & N.L. Rosenberg. 1990. White matter dementia in chronic toluene abuse. Neurology 40 : 532 534.
151. Rosenberg, N.L., B.K. Kleinschmidt-DeMasters, K.A. Davis, et al. 1988. Toluene abuse causes diffuse central nervous system white matter changes. Ann. Neurol. 23 : 611 614.
152. Filley, C.M., W. Halliday & B.K. Kleinschmidt-DeMasters. 2004. The effects of toluene on the central nervous system. J. Neuropathol. Exp. Neurol. 63 : 1 12.
153. Filley, C.M. 1998. The behavioral neurology of cerebral white matter. Neurology 50 : 1535 1540.
154. Kriegstein, A.R., D.C. Shungu, W.S. Millar, et al. 1999. Leukoencephalopathy and raised brain lactate from heroin vapor inhalation ("chasing the dragon"). Neurology 10 : 1765 1773.
155. Bartlett, E. & D.J. Mikulis. 2005. Chasing "chasing the dragon" with MRI: leukoencephalopathy in drug abuse. Br. J. Radiol. 78 : 997 1004.
156. Offiah, C. & E. Hall. 2008. Heroin-induced leukoencephalopathy: characterization using MRI, diffusion-weighted imaging, and MR spectroscopy. Clin. Radiol. 63 : 146 152.
157. Wolters, E.C., G.K. van Wijngaarden, F.C. Stam, et al. 1982. Leucoencephalopathy after inhaling "heroin" pyrolysate. Lancet 2 : 1233 1237.
158. Powell, H.C., R.R. Meyers & P.W. Lampert. 1980. Edema in Neurotoxic Injury. Williams & Wilkins. Baltimore.
159. Hinchey, J., C. Chaves, B. Appignani, et al. 1996. A reversible posterior leukoencephalopathy syndrome. N. Engl. J. Med. 334 : 494 500.
160. Adams, R.D. & C.S. Kubik. 1944. Subacute degeneration of the brain in pernicious anemia. N. Engl. J. Med. 231 : 1 9.
161. Chatterjee, A., R. Yapundich, C.A. Palmer, et al. 1996. Leukoencephalopathy associated with cobalamin deficiency. Neurology 46 : 832 834.
162. Stojsavljević, N., Z. Lević, J. Drulović & G. Dragutinović. 1997. A 44-month clinical-brain MRI follow-up in a patient with B12 deficiency. Neurology 49 : 878 881.
163. Lee, H.B. & C.G. Lyketsos. 2001. Delayed post-hypoxic leukoencephalopathy. Psychosomatics 42 : 530 533.
164. Molloy, S., C. Soh & T.L. Williams. 2006. Reversible delayed posthypoxic leukoencephalopathy. AJNR Am. J. Neuroradiol. 27 : 1763 1765.
165. Chen-Plotkin, A.S., K.T. Pau & J.D. Schmahmann. 2008. Delayed leukoencephalopathy after hypoxic-ischemic injury. Arch. Neurol. 65 : 144 145.
166. Choi, I.S. 1983. Delayed neurologic sequelae in carbon monoxide intoxication. Arch. Neurol. 40 : 433 435.
167. Sandson, T.A., R.B. Lilly & M. Sodkol. 1988. Kluver-Bucy syndrome associated with delayed post-anoxic leukoencephalopathy following carbon monoxide poisoning. J. Neurol. Neurosurg. Psychiatry 51 : 156 157.
168. Langlois, J., W. Rutland-Brown & K. Thomas. 2004. Traumatic Brain Injury in the United States: Emergency Department Visits, Hospitalizations and Deaths. Centers for Disease Control and Prevention, National Center for Injury Prevention. Atlanta, GA.
169. Adams, J.H., D.I. Graham, L.S. Murray & G. Scott. 1982. Diffuse axonal injury due to nonmissile head injury: an analysis of 45 cases. Ann. Neurol. 12 : 557 563.
170. Smith, D.H., D.F. Meaney & W.H. Shull. 2003. Diffuse axonal injury in head trauma. J. Head Trauma Rehabil. 18 : 307 316.
171. Kraus, M.F., T. Susmaras, B.P. Caughlin, et al. 2007. White matter injury and cognition in chronic traumatic brain injury: a diffusion tensor imaging study. Brain 130 : 2508 2519.
172. Alexander, M.P. 1995. Mild traumatic brain injury: pathophysiology, natural history, and clinical management. Neurology 45 : 1252 1260.
173. Adams, J.H., D.I. Graham & B. Jennett. 2000. The neuropathology of the vegetative state after an acute brain insult. Brain 123 : 1327 1338.
174. Filley, C.M. & B.K. Kleinschmidt-DeMasters. 1995. Neurobehavioral presentations of brain neoplasms. West. J. Med. 163 : 19 25.
175. Giese, A. & M. Westphal. 1996. Glioma invasion in the central nervous system. Neurosurgery 39 : 235 250.
176. Geer, C.P. & S.A. Grossman. 1997. Interstitial flow along white matter tracts: a potentially important mechanism for the dissemination of primary brain tumors. J. Neurooncol. 32 : 193 201.
177. Filley, C.M., B.K. Kleinschmidt-DeMasters, K.O. Lillehei, et al. 2003. Gliomatosis cerebri: neurobehavioral and neuropathological observations. Cogn. Behav. Neurol. 16 : 149 159.
178. Rollins, K.E., B.K. Kleinschmidt-DeMasters, J.R. Corboy, et al. 2005. Lymphomatosis cerebri as a cause of white matter dementia. Hum. Pathol. 36 : 282 290.
179. Grois, N., A.J. Barkovich, W. Rosenau & A.R. Ablin. 1993. Central nervous system disease associated with Langerhans' cell histiocytosis. Am. J. Pediatr. Hematol. Oncol. 15 : 245 254.
180. Grois, N.G., B.E. Favara, G.H. Mostbeck & D. Prayer. 1998. Central nervous system disease in Langerhans cell histiocytosis. Hematol. Oncol. Clin. North Am. 12 : 287 305.
181. Grois, N., D. Prayer, H. Prosch & H. Lassmann, CNS LCH Co-operative Group. 2005. Neuropathology of CNS disease in Langerhans cell histiocytosis. Brain 128 : 829 838.
182. Schmahmann, J.D., J.B. Weilburg & J.C. Sherman. 2007. The neuropsychiatry of the cerebellum - insights from the clinic. Cerebellum 6 : 254 267.
183. Whitsett, S.F., K. Kneppers, M.J. Coppes & R.M. Egeler. 1999. Neuropsychologic deficits in children with Langerhans cell histiocytosis. Med. Pediatr. Oncol. 33 : 486 492.
184. Schmahmann, J.D. & J.C. Sherman. 1998. The cerebellar cognitive affective syndrome. Brain 121 : 561 579.
185. Levisohn, L., A. Cronin-Golomb & J.D. Schmahmann. 2000. Neuropsychological consequences of cerebellar tumour resection in children: cerebellar cognitive affective syndrome in a paediatric population. Brain 123 : 1041 1050.
186. Del Bigio, M.R. 1993. Neuropathological changes caused by hydrocephalus. Acta Neuropathol. 85 : 573 585.
187. Del Bigio, M.R., M.C. da Silva, J.M. Drake & U.I. Tuor. 1994. Acute and chronic cerebral white matter damage in neonatal hydrocephalus. Can. J. Neurol. Sci. 21 : 299 305.
188. Adams, R.D., C.M. Fisher, S. Hakim, et al. 1965. Symptomatic occult hydrocephalus with "normal" cerebrospinal-fluid pressure. N. Engl. J. Med. 273 : 117 126.
189. Schwarzschild, M., G. Rordorf, K. Bekken, et al. 1997. Normal-pressure hydrocephalus with misleading features of irreversible dementias: a case report. J. Geriatr. Psychiatry Neurol. 10 : 51 54.
190. Marmarou, A., H.F. Young, G.A. Aygok, et al. 2005. Diagnosis and management of idiopathic normal-pressure hydrocephalus: a prospective study in 151 patients. J. Neurosurg. 102 : 987 997.
191. Earnest, M.P., S. Fahn, J.H. Karp & L.P. Rowland. 1974. Normal pressure hydrocephalus and hypertensive cerebrovascular disease. Arch. Neurol. 31 : 262 266.
192. Bech, R.A., M. Juhler, G. Waldemar, et al. 1997. Frontal brain and leptomeningeal biopsy specimens correlated with cerebrospinal fluid outflow resistance and B-wave activity in patients suspected of normal-pressure hydrocephalus. Neurosurgery 40 : 497 502.
193. Peters, A., D. Leahu, M.B. Moss & K.J. McNally. 1994. The effects of aging on area 46 of the frontal cortex of the rhesus monkey. Cereb. Cortex 4 : 621 635.
194. Peters, A. 2002. The effects of normal aging on myelin and nerve fibers: a review. J. Neurocytol. 31 : 581 593.
195. Makris, N., G.M. Papadimitriou, A. Van Der Kouwe, et al. 2007. Frontal connections and cognitive changes in normal aging rhesus monkeys: a DTI study. Neurobiol. Aging 28 : 1556 1567.
196. Hachinski, V.C., P. Potter & H. Merskey. 1986. Leuko-araiosis: an ancient term for a new problem. Can. J. Neurol. Sci. 13 (4 Suppl 533 534.
197. Hachinski, V.C., P. Potter & H. Merskey. 1987. Leuko-araiosis. Arch. Neurol. 44 : 21 23.
198. DeCarli, C., E. Fletcher, V. Ramey, et al. 2005. Anatomical mapping of white matter hyperintensities (WMH): exploring the relationships between periventricular WMH, deep WMH, and total WMH burden. Stroke 36 : 50 55.
199. O'Sullivan, M., P.E. Summers, D.K. Jones, et al. 2001. Normal-appearing white matter in ischemic leukoaraiosis: a diffusion tensor MRI study. Neurology 57 : 2307 2310.
200. Fernando, M.S., J.T. O'Brien, R.H. Perry, et al. 2004. Comparison of the pathology of cerebral white matter with post-mortem magnetic resonance imaging (MRI) in the elderly brain. Neuropathol. Appl. Neurobiol. 30 : 385 395.
201. Au, R., J.M. Massaro, P.A. Wolf, et al. 2006. Association of white matter hyperintensity volume with decreased cognitive functioning: the Framingham Heart Study. Arch. Neurol. 63 : 246 250.
202. de Leeuw, F.E., J.C. de Groot, M.L. Bots, et al. 2000. Carotid atherosclerosis and cerebral white matter lesions in a population based magnetic resonance imaging study. J. Neurol. 247 : 291 296.
203. de Leeuw, F.E., J.C. de Groot, M. Oudkerk, et al. 2002. Hypertension and cerebral white matter lesions in a prospective cohort study. Brain 125 (Pt 4 765 772.
204. Longstreth, W.T. Jr., T.A. Manolio, A. Arnold, et al. 1996. Clinical correlates of white matter findings on cranial magnetic resonance imaging of 3301 elderly people. The Cardiovascular Health Study. Stroke 27 : 1274 1282.
205. de Leeuw, F.E., J.C. de Groot, E. Achten, et al. 2001. Prevalence of cerebral white matter lesions in elderly people: a population based magnetic resonance imaging study. The Rotterdam Scan Study. J. Neurol. Neurosurg. Psychiatry 70 : 9 14.
206. Longstreth, W. Jr., E.K. Larsen, R. Klein, et al. 2007. Associations between findings on cranial magnetic resonance imaging and retinal photography in the elderly: the Cardiovascular Health Study. Am. J. Epidemiol. 165 : 78 84.
207. Khatri, M., C.B. Wright, T.L. Nickolas, et al. 2007. Chronic kidney disease is associated with white matter hyperintensity volume: the Northern Manhattan Study (NOMAS). Stroke 38 : 3121 3126.
208. Hassan, A., B.J. Hunt, M. O'Sullivan, et al. 2004. Homocysteine is a risk factor for cerebral small vessel disease, acting via endothelial dysfunction. Brain 127 (Pt 1 212 219.
209. Breteler, M.M., J.C. van Swieten, M.L. Bots, et al. 1994. Cerebral white matter lesions, vascular risk factors, and cognitive function in a population-based study: the Rotterdam Study. Neurology 44 : 1246 1252.
210. Schmidt, R., F. Fazekas, M. Hayn, et al. 1997. Risk factors for microangiopathy-related cerebral damage in the Austrian stroke prevention study. J. Neurol. Sci. 152 : 15 21.
211. Gurol, M.E., M.C. Irizarry, E.E. Smith, et al. 2006. Plasma beta-amyloid and white matter lesions in AD, MCI, and cerebral amyloid angiopathy. Neurology 66 : 23 29.
212. van Dijk, E.J., N.D. Prins, S.E. Vermeer, et al. 2004. Plasma amyloid beta, apolipoprotein E, lacunar infarcts, and white matter lesions. Ann. Neurol. 55 : 570 575.
213. Stenset, V., L. Johnsen, D. Kocot, et al. 2006. Associations between white matter lesions, cerebrovascular risk factors, and low CSF Abeta42. Neurology 67 : 830 833.
214. Carmelli, D., C. DeCarli, G.E. Swan, et al. 1998. Evidence for genetic variance in white matter hyperintensity volume in normal elderly male twins. Stroke 29 : 1177 1181.
215. Vermeer, S.E., M. Hollander, E.J. van Dijk, et al. 2003. Silent brain infarcts and white matter lesions increase stroke risk in the general population: the Rotterdam Scan Study. Stroke 34 : 1126 1129.
216. Smith, E.E., J. Rosand, K.A. Knudsen, et al. 2002. Leukoaraiosis is associated with warfarin-related hemorrhage following ischemic stroke. Neurology 59 : 193 197.
217. Ay, H., E.M. Arsava, J. Rosand, et al. 2008. Severity of leukoaraiosis and susceptibility to infarct growth in acute stroke. Stroke 39 : 1409 1413.
218. Kuller, L.H., W.T. Longstreth Jr., A.M. Arnold, et al. 2004. White matter hyperintensity on cranial magnetic resonance imaging: a predictor of stroke. Stroke 35 : 1821 1825.
219. Gorter, J.W. 1999. Major bleeding during anticoagulation after cerebral ischemia: patterns and risk factors. Stroke Prevention In Reversible Ischemia Trial (SPIRIT). European Atrial Fibrillation Trial (EAFT) study groups. Neurology 53 : 1319 1327.
220. Neumann-Haefelin, T., S. Hoelig, J. Berkefeld, et al. 2006. Leukoaraiosis is a risk factor for symptomatic intracerebral hemorrhage after thrombolysis for acute stroke. Stroke 37 : 2463 2466.
221. Smith, E.E., M.E. Gurol, J.A. Eng, et al. 2004. White matter lesions, cognition, and recurrent hemorrhage in lobar intracerebral hemorrhage. Neurology 63 : 1606 1612.
222. Longstreth, W.T. Jr., C. Dulberg, T.A. Manolio, et al. 2002. Incidence, manifestations, and predictors of brain infarcts defined by serial cranial magnetic resonance imaging in the elderly: the Cardiovascular Health Study. Stroke 33 : 2376 2382.
223. Dufouil, C., J. Chalmers, O. Coskun, et al. 2005. Effects of blood pressure lowering on cerebral white matter hyperintensities in patients with stroke: the PROGRESS (Perindopril Protection Against Recurrent Stroke Study) Magnetic Resonance Imaging Substudy. Circulation 112 : 1644 1650.
224. ten Dam, V.H., D.M. van den Heuvel, M.A. van Buchem, et al. 2005. Effect of pravastatin on cerebral infarcts and white matter lesions. Neurology 64 : 1807 1809.
225. Fernando, M.S., J.E. Simpson, F. Matthews, et al. 2006. White matter lesions in an unselected cohort of the elderly: molecular pathology suggests origin from chronic hypoperfusion injury. Stroke 37 : 1391 1398.
226. Pantoni, L. & J.H. Garcia. 1997. Pathogenesis of leukoaraiosis: a review. Stroke 28 : 652 659.
227. Holland, C.M., E.E. Smith, I. Csapo, et al. 2008. Spatial distribution of white-matter hyperintensities in Alzheimer disease, cerebral amyloid angiopathy, and healthy aging. Stroke 39 : 1127 1133.
228. De Reuck, J., D. Decoo, M. Marchau, et al. 1998. Positron emission tomography in vascular dementia. J. Neurol. Sci. 154 : 55 61.
229. De Reuck, J., D. Decoo, K. Strijckmans & I. Lemahieu. 1992. Does the severity of leukoaraiosis contribute to senile dementia? A comparative computerized and positron emission tomographic study. Eur. Neurol. 32 : 199 205.
230. Hatazawa, J., E. Shimosegawa, T. Satoh, et al. 1997. Subcortical hypoperfusion associated with asymptomatic white matter lesions on magnetic resonance imaging. Stroke 28 : 1944 1947.
231. Tohgi, H., H. Yonezawa, S. Takahashi, et al. 1998. Cerebral blood flow and oxygen metabolism in senile dementia of Alzheimer's type and vascular dementia with deep white matter changes. Neuroradiology 40 : 131 137.
232. Yao, H., S. Sadoshima, S. Ibayashi, et al. 1992. Leukoaraiosis and dementia in hypertensive patients. Stroke 23 : 1673 1677.
233. ten Dam, V.H., D.M. van den Heuvel, A.J. de Craen, et al. 2007. Decline in total cerebral blood flow is linked with increase in periventricular but not deep white matter hyperintensities. Radiology 243 : 198 203.
234. Joutel, A., C. Corpechot, A. Ducros, et al. 1996. Notch3 mutations in CADASIL, a hereditary adult-onset condition causing stroke and dementia. Nature 383 : 707 710.
235. Mayer, M., A. Straube, R. Bruening, et al. 1999. Muscle and skin biopsies are a sensitive diagnostic tool in the diagnosis of CADASIL. J. Neurol. 246 : 526 532.
236. Singhal, S., P. Rich, & H.S. Markus. 2005. The spatial distribution of MR imaging abnormalities in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy and their relationship to age and clinical features. AJNR Am. J. Neuroradiol. 26 : 2481 2487.
237. Harris, J.G. & C.M. Filley. 2001. CADASIL: Neuropsychological findings in three generations of an affected family. J. Int. Neuropsychol. Soc. 7 : 768 774.
238. Viswanathan, A., A. Gschwendtner, J.P. Guichard, et al. 2007. Lacunar lesions are independently associated with disability and cognitive impairment in CADASIL. Neurology 69 : 172 179.
239. Chabriat, H., K. Vahedi, M.T. Iba-Zizen, et al. 1995. Clinical spectrum of CADASIL: a study of 7 families. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy. Lancet 346 : 934 939.
240. Zhang-Nunes, S.X., M.L. Maat-Schieman, S.G. van Duinen, et al. 2006. The cerebral beta-amyloid angiopathies: hereditary and sporadic. Brain Pathol. 16 : 30 39.
241. Knudsen, K.A., J. Rosand, D. Karluk & S.M. Greenberg. 2001. Clinical diagnosis of cerebral amyloid angiopathy: validation of the Boston criteria. Neurology 56 : 537 539.
242. Smith, E.E. & S.M. Greenberg. 2003. Clinical diagnosis of cerebral amyloid angiopathy: validation of the Boston criteria. Curr. Atheroscler. Rep. 5 : 260 266.
243. Greenberg, S.M., M.E. Gurol, J. Rosand & E.E. Smith. 2004. Amyloid angiopathy-related vascular cognitive impairment. Stroke 35 (11 Suppl 1 2616 2619.
244. Neuropathology Group of the Medical Research Council Cognitive Function and Ageing Study (MRC CFAS). 2001. Pathological correlates of late-onset dementia in a multicentre, community-based population in England and Wales. Lancet 357 : 169 175.
245. Vernooij, M.W., A. Van Der Lugt, M.A. Ikram, et al. 2008. Prevalence and risk factors of cerebral microbleeds: the Rotterdam Scan Study. Neurology 70 : 1208 1214.
246. Chen, Y.-W., M.E. Gurol, J. Rosand, et al. 2006. Progression of white matter lesions and hemorrhages in cerebral amyloid angiopathy. Neurology 67 : 83 87.
247. Salat, D.H., E.E. Smith, D.S. Tuch, et al. 2006. White matter alterations in cerebral amyloid angiopathy measured by diffusion tensor imaging. Stroke 37 : 1759 1764.
248. Viswanathan, A., P. Patel, R. Rahman, et al. 2008. Tissue microstructural changes are independently associated with pre-index cognitive impairment in survivors of lobar intracerebral hemorrhage. Stroke 39 : 1988 1992.
249. Johnson, K.A., M. Gregas, J.A. Becker, et al. 2007. Imaging of amyloid burden and distribution in cerebral amyloid angiopathy. Ann. Neurol. 62 : 229 234.
250. Arriagada, P.V., J.H. Growdon, E.T. Hedley-Whyte & B.T. Hyman. 1992. Neurofibrillary tangles but not senile plaques parallel duration and severity of Alzheimer's disease. Neurology 42 (3 Pt 1 631 639.
251. de Groot, J.C., F.E. de Leeuw, M. Oudkerk, et al. 2000. Cerebral white matter lesions and cognitive function: the Rotterdam Scan Study. Ann. Neurol. 47 : 145 151.
252. Mosley, T.H. Jr., D.S. Knopman, D.J. Catellier, et al. 2005. Cerebral MRI findings and cognitive functioning: the Atherosclerosis Risk in Communities study. Neurology 64 : 2056 2062.
253. Longstreth, W.T. Jr., A.M. Arnold, N.J. Beauchamp Jr., et al. 2005. Incidence, manifestations, and predictors of worsening white matter on serial cranial magnetic resonance imaging in the elderly: the Cardiovascular Health Study. Stroke 36 : 56 61.
254. Prins, N.D., E.J. van Dijk, T. den Heijer, et al. 2005. Cerebral small-vessel disease and decline in information processing speed, executive function and memory. Brain 128 (Pt 9 2034 2041.
255. Schmidt, R., S. Ropele, C. Enzinger, et al. 2005. White matter lesion progression, brain atrophy, and cognitive decline: the Austrian Stroke Prevention study. Ann. Neurol. 58 : 610 616.
256. de Groot, J.C., F.E. de Leeuw, M. Oudkerk, et al. 2001. Cerebral white matter lesions and subjective cognitive dysfunction: the Rotterdam Scan Study. Neurology 56 : 1539 1545.
257. Lopez, O.L., W.J. Jagust, C. Dulberg, et al. 2003. Risk factors for mild cognitive impairment in the Cardiovascular Health Study Cognition Study: part 2. Arch. Neurol. 60 : 1394 1399.
258. Smith, E.E., S. Egorova, D. Blacker, et al. 2008. Magnetic resonance imaging white matter hyperintensities and brain volume in the prediction of mild cognitive impairment and dementia. Arch. Neurol. 65 : 94 100.
259. DeCarli, C., D. Mungas, D. Harvey, et al. 2004. Memory impairment, but not cerebrovascular disease, predicts progression of MCI to dementia. Neurology 63 : 220 227.
260. Vermeer, S.E., N.D. Prins, T. den Heijer, et al. 2003. Silent brain infarcts and the risk of dementia and cognitive decline. N. Engl. J. Med. 348 : 1215 1222.
261. Schneider, J.A., Z. Arvanitakis, W. Bang & D.A. Bennett. 2007. Mixed brain pathologies account for most dementia cases in community-dwelling older persons. Neurology 69 : 2197 2204.
262. Hubbard, B.M. & J.M. Anderson. 1981. A quantitative study of cerebral atrophy in old age and senile dementia. J. Neurol. Sci. 50 : 135 145.
263. Brinkman, S.D., M. Sarwar, H.S. Levin & H.H. Morris 3rd. 1981. Quantitative indexes of computed tomography in dementia and normal aging. Radiology 138 : 89 92.
264. Brun, A. & E. Englund. 1986. A white matter disorder in dementia of the Alzheimer type: a pathoanatomical study. Ann. Neurol. 19 : 253 262.
265. Englund, E. & A. Brun. 1990. White matter changes in dementia of Alzheimer's type: the difference in vulnerability between cell compartments. Histopathology 16 : 433 439.
266. Jellinger, K.A. 2002. Alzheimer's disease and cerebrovascular pathology: an update. J. Neural Transm. 109 : 813 836.
267. Atri, A., J.J. Locascio, J.M. Lin, et al. 2005. Prevalence and effects of lobar microhemorrhages in early-stage dementia. Neurodegener. Dis. 2 : 305 312.
268. Pfeifer, L.A., L.R. White, G.W. Ross, et al. 2002. Cerebral amyloid angiopathy and cognitive function: the HAAS autopsy study. Neurology 58 : 1629 1634.
269. Román, G.C. 2000. Binswanger disease: the history of a silent epidemic. Ann. N. Y. Acad. Sci. 903 : 19 23.
270. Román, G.C. 1996. From UBOs to Binswanger's disease. Impact of magnetic resonance imaging on vascular dementia research. Stroke 27 : 1269 1273.
271. Selnes, O.A., & H.V. Vinters. 2006. Vascular cognitive impairment. Nat. Clin. Pract. Neurol. 2 : 538 547.
272. Leys, D., J.P. Pruvo, M. Parent, et al. 1991. Could Wallerian degeneration contribute to "leuko-araiosis" in subjects free of any vascular disorder? J. Neurol. Neurosurg. Psychiatry 54 : 46 50.
273. Bozzali, M., A. Falini, M. Francheschi, et al. 2002. White matter damage in Alzheimer's disease assessed in vivo using diffusion tensor magnetic resonance imaging. J. Neurol. Neurosurg. Psychiatry 72 : 742 746.
274. Burton, E.J., R.A. Kenny, J. O'Brien, et al. 2004. White matter hyperintensities are associated with impairment of memory, attention, and global cognitive performance in older stroke patients. Stroke 35 : 1270 1275.
275. Marshall, G.A., R. Hendrickson, D.I. Kaufer, et al. 2006. Cognitive correlates of brain MRI subcortical signal hyperintensities in non-demented elderly. Int. J. Geriatr. Psychiatry 21 : 32 35.
276. Nordahl, C.W., C. Ranganath, A.P. Yonelinas, et al. 2005. Different mechanisms of episodic memory failure in mild cognitive impairment. Neuropsychologia 43 : 1688 1697.
277. Reed, B.R., J.L. Eberling, D. Mungas, et al. 2004. Effects of white matter lesions and lacunes on cortical function. Arch. Neurol. 61 : 1545 1550.
278. Soderlund, H., L.G. Nilsson, K. Berger, et al. 2006. Cerebral changes on MRI and cognitive function: the CASCADE study. Neurobiol. Aging 27 : 16 23.
279. Van Den Heuvel, D.M., V.H. ten Dam, A.J. de Craen, et al. 2006. Increase in periventricular white matter hyperintensities parallels decline in mental processing speed in a non-demented elderly population. J. Neurol. Neurosurg. Psychiatry 77 : 149 153.
280. Ylikoski, R., A. Ylikoski, T. Erkinjuntti, et al. 1993. White matter changes in healthy elderly persons correlate with attention and speed of mental processing. Arch. Neurol. 50 : 818 824.
281. Nordahl, C.W., C. Ranganath, A.P. Yonelinas, et al. 2006. White matter changes compromise prefrontal cortex function in healthy elderly individuals. J. Cogn. Neurosci. 18 : 418 429.
282. de Groot, J.C., F.E. de Leeuw, M. Oudkerk, et al. 2000. Cerebral white matter lesions and depressive symptoms in elderly adults. Arch. Gen. Psychiatry 57 : 1071 1076.
283. Firbank, M.J., J.T. O'Brien, S. Pakrasi, et al. 2005. White matter hyperintensities and depression-preliminary results from the LADIS study. Int. J. Geriatr. Psychiatry 20 : 674 679.
284. Thomas, A.J., J.T. O'Brien, S. Davis, et al. 2002. Ischemic basis for deep white matter hyperintensities in major depression: a neuropathological study. Arch. Gen. Psychiatry 59 : 785 792.
285. Iosifescu, D.V., P.F. Renshaw, I.K. Lyoo, et al. 2006. Brain white-matter hyperintensities and treatment outcome in major depressive disorder. Br. J. Psychiatry 188 : 180 185.
286. Taylor, W.D., D.C. Steffens, J.R. MacFall, et al. 2003. White matter hyperintensity progression and late-life depression outcomes. Arch. Gen. Psychiatry 60 : 1090 1096.
287. Bocti, C., R.H. Swartz, F.Q. Gao, et al. 2005. A new visual rating scale to assess strategic white matter hyperintensities within cholinergic pathways in dementia. Stroke 36 : 2126 2131.
288. Sheline, Y.I., J.L. Price, S.N. Vaishnavi, et al. 2008. Regional white matter hyperintensity burden in automated segmentation distinguishes late-life depressed subjects from comparison subjects matched for vascular risk factors. Am. J. Psychiatry 165 : 524 532.
289. Taylor, W.D., J.R. MacFall, D.C. Steffens, et al. 2003. Localization of age-associated white matter hyperintensities in late-life depression. Prog. Neuropsychopharmacol. Biol. Psychiatry 27 : 539 544.
290. Tullberg, M., E. Fletcher, C. DeCarli, et al. 2004. White matter lesions impair frontal lobe function regardless of their location. Neurology 63 : 246 253.
291. Starkstein, S.E., L. Sabe, S. Vazquez, et al. 1997. Neuropsychological, psychiatric, and cerebral perfusion correlates of leukoaraiosis in Alzheimer's disease. J. Neurol. Neurosurg. Psychiatry 63 : 66 73.
292. Sultzer, D.L., M.E. Mahler, J.L. Cummings, et al. 1995. Cortical abnormalities associated with subcortical lesions in vascular dementia. Clinical and position emission tomographic findings. Arch. Neurol., 52 : 773 780.
293. Yao, H., S. Sadoshima, Y. Kuwabara, et al. 1990. Cerebral blood flow and oxygen metabolism in patients with vascular dementia of the Binswanger type. Stroke 21 : 1694 1699.
294. Schmahmann, J.D. & D. Leifer. 1992. Parietal pseudothalamic pain syndrome. Clinical features and anatomical correlates. Arch. Neurol. 49 : 1032 1037.
295. Leventhal, C.M., J.R. Baringer, B.G. Arnason & C.M. Fisher. 1965. A case of Marchiafava-Bignami disease with clinical recovery. Trans. Am. Neurol. Assoc. 90 : 87 91.
296. Heilman, K.M. & G.W. Sypert. 1977. Korsakoff's syndrome resulting from bilateral fornix lesions. Neurology 27 : 490 493.
297. D'Esposito, M., M. Verfaellie, M.P. Alexander & D.I. Katz. 1995. Amnesia following traumatic bilateral fornix transection. Neurology 45 : 1546 1550.
298. Polyak, S. 1957. The Vertebrate Visual System. H. Klüver, Ed University of Chicago Press. Chicago, IL.
299. Groothuis, D.R., G.W. Duncan & C.M. Fisher. 1977. The human thalamocortical sensory path in the internal capsule: evidence from a small capsular hemorrhage causing a pure sensory stroke. Ann. Neurol. 2 : 328 331.
300. Fisher, C.M. 1979. Capsular infarcts: the underlying vascular lesions. Arch. Neurol. 36 : 65 73.
301. Aralasmak, A., J.L. Ulmer, M. Kocak, et al. 2006. Association, commissural, and projection pathways and their functional deficit reported in literature. J. Comput. Assist. Tomogr. 30 : 695 715.
302. Naeser, M.A., C.L. Palumbo, N. Helm-Estabrooks, et al. 1989. Severe nonfluency in aphasia. Role of the medial subcallosal fasciculus and other white matter pathways in recovery of spontaneous speech. Brain 112 : 1 38.
303. Davis, K.L., D.G. Stewart, J.I. Friedman, et al. 2003. White matter changes in schizophrenia: evidence for myelin related dysfunction. Arch. Gen. Psychiatry 60 : 443 456.
304. Nakamura, M., R.W. McCarley, M. Kubicki, et al. 2005. Fronto-temporal disconnectivity in schizotypal personality disorder: a diffusion tensor imaging study. Biol. Psychiatry 58 : 468 478.
305. Lamantia, A.S. & P. Rakic. 1990. Cytological and quantitative characteristics of four cerebral commissures in the rhesus monkey. J. Comp. Neurol. 291 : 520 537.
306. Innocenti, G.M. 1995. Exuberant development of connections, and its possible permissive role in cortical evolution. Trends Neurosci. 18 : 397 402.
307. Herbert, M.R., D.A. Ziegler, C.K. Deutsch, et al. 2003. Dissociations of cerebral cortex, subcortical and cerebral white matter volumes in autistic boys. Brain 126 : 1182 1192.
308. Filley, C.M., R.K. Heaton, L.M Nelson, et al. 1989. A comparison of dementia in Alzheimer's disease and multiple sclerosis. Arch. Neurol. 46 : 157 161.
309. Lafosse, J.M., J.R. Corboy, M.A. Leehey, et al. 2007. MS vs. HD: Can white matter and subcortical gray matter pathology be distinguished neuropsychologically? J. Clin. Exp. Neuropsychol. 29 : 142 154.