doi:10.

1093/brain/awp100

Brain 2009: 132; 26092616

| 2609

BRAIN
A JOURNAL OF NEUROLOGY

OCCASIONAL PAPER

The quicksand of forgetfulness: semantic dementia in One Hundred Years of Solitude

Katya Rascovsky, Matthew E. Growdon, Isela R. Pardo, Scott Grossman and Bruce L. Miller
Memory and Aging Center, UCSF Department of Neurology, San Francisco, CA, USA Correspondence to: Katya Rascovsky, UCSF Memory and Aging Center, 350 Parnassus Ave., Suite 905, San Francisco, CA 94143-1207, USA E-mail: krascovsky@memory.ucsf.edu

Downloaded from http://brain.oxfordjournals.org/ by guest on February 11, 2013

rquezs One Hundred Years of a Ma This multidisciplinary article compares the pattern of memory loss described in Gabriel Garc rquez depicts the plight of a Ma Solitude to that exhibited by patients with semantic dementia (SD). In his renowned novel, Garc Macondo, a town struck by the dreaded insomnia plague. The most devastating symptom of the plague is not the impossibility of sleep, but rather the loss of the name and notion of things. In an effort to combat this insidious loss of knowledge, the Arcadio Buend a, marked everything with its name: table, chair, clock, door, wall, bed, pan. Studying the protagonist, Jose innite possibilities of a loss of memory, he realized that the day might come when things would be recognized by their inscriptions but that no one would remember their use. The cognitive impairments experienced by Macondos inhabitants are remarkably similar to those observed in SD, a clinical syndrome characterized by a progressive breakdown of conceptual knowledge (semantic memory) in the context of relatively preserved day-to-day (episodic) memory. First recognized in 1975, it is now considered one of the main variants of frontotemporal lobar degeneration. Writing within the realm of magical realism rquez provides beautiful descriptions of the loss a Ma and investigating the power of language as a form of communication, Garc of the name and notion of things typical of the syndrome. He further speculates on ways to cope with this dissolution of Arcadios memory machine, strategies that resonate with meaning, ranging from the spell of an imaginary reality to Jose rquez created a striking literary a Ma attempts by semantic dementia patients to cope with their disease. Remarkably, Garc depiction of collective semantic dementia before the syndrome was recognized in neurology. The novel also provides an inspiring and human account of one towns ght against the quicksand of forgetfulness.

rquez; One Hundred Years of Solitude; magical realism; neurology and a Ma Keywords: semantic dementia; Gabriel Garc literature

, sino la que uno recuerda y co mo La vida no es la que uno vivio la recuerda para contarla. Life is not what one lived, but what one remembers and how one remembers it in order to recount it. rquez a Ma Gabriel Garc

Introduction
The insomnia plague
The mythical town of Macondo has been stricken with the dreaded insomnia plague. Despite precautions, the illness spread

Received January 27, 2009. Revised March 16, 2009. Accepted March 22, 2009. Advance Access publication May 15, 2009 The Author (2009). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

2610

| Brain 2009: 132; 26092616

K. Rascovsky et al. perceptual/visuospatial abilities or day-to-day memory. Drawing on Tulvings newly proposed distinction between memory for experienced events (episodic memory) and knowledge about the world (semantic memory) (Tulving, 1972), she argued that these patients exhibited a selective breakdown in semantic memory. Shortly afterward, Mesulam (1982) described a number of cases characterized by a slowly progressive aphasia without generalized dementia and coined the term Primary Progressive Aphasia (Mesulam, 1982). On autopsy, the majority of these patients showed non-Alzheimers pathology. It quickly became evident that there were at least two main subtypes of progressive aphasia: uent and non-uent. On the one hand, the non-uent variety was characterized by effortful, hesitant speech with agrammatism and phonemic paraphasias, but relatively preserved comprehension (Grossman, 2002). On the other hand, it became clear that the impairments of patients with uent aphasia went beyond the domain of language, and in fact mirrored those described by Warrington a few years earlier. This prompted Snowden (Snowden et al., 1989) to re-name the syndrome semantic dementia, as this name encapsulated the essence of a decit in representational knowledge. In 1992, Hodges and colleagues described ve similar cases and proposed criteria for the diagnosis of SD (Hodges et al., 1992). Semantic dementia is now recognized as the temporal variant within the frontotemporal lobar degeneration (FTLD) spectrum (Neary et al., 1998).

rsula Iguara ns lovingly prepared candy animals: through U Children and adults sucked with delight on the delicious green roosters of insomnia, the exquisite pink sh of insomnia, and the tender yellow ponies of insomnia, so that dawn on Monday, rquez, 1970). Initially, a Ma found the whole town awake (Garc Macondos inhabitants were not alarmed: If we dont sleep again, Arcadio Buend a said in good humour, so much the better, Jose That way we can get more out of life. Unfortunately, the most fearsome part of the sickness was not the impossibility of sleeping, but its inexorable evolution toward a more critical manifestation: a loss of memory.

Loss of the name and notion of things

The rst and most devastating aspect of this memory loss was the a was the loss of the name and notion of things. Aureliano Buend rst to notice this troubling symptom: One day he was looking for the small anvil that he used for laminating metals and he could not remember its name. His father told him: Stake. Aureliano wrote the name on a piece of paper that he pasted to the small anvil: stake. In that way he was sure of not forgetting it in the future. It did not occur to him that this was the rst manifestation of a loss of memory, because the object had a difcult name to remember. But a few days later he discovered that he had trouble remembering almost every object in the laboratory. Then he marked them with their respective names so that all he had to do was read the inscription in order to identify them. When his father told him about his alarm at having forgotten even the most impressive happenings of his childhood, Aureliano explained his Arcadio Buend a put it into practice method to him and Jose all through the house and later on imposed it on the whole village. With an inked brush he marked everything with its name: table, chair, clock, door, wall, bed, pan. He went to the corral and marked the animals and plants: cow, goat, pig, hen, cassava, caladium, banana. Little by little, studying the innite possibilities of a loss of memory, he realized that the day might come when things would be recognized by their inscriptions but that no one would remember their use.

Downloaded from http://brain.oxfordjournals.org/ by guest on February 11, 2013

Description
Patients with SD often present to medical attention with empty circumlocutory speech, prominent anomia and frequent semantic paraphasias. They tend to replace specic words with general terms (e.g. thing for chair) or prototype members of the same category (e.g. dog for rhinoceros). They also have difculty understanding the meaning of words and will often express their lack of familiarity with questions such as cow? What is a cow? Nevertheless, their speech output is uent, grammatically correct and free of phonological errors. The core semantic decit in SD can be formally demonstrated using a variety of tests. Semantic dementia patients exhibit severe decits in picture-naming, word-picture matching and tests of associative semantic knowledge (e.g. Pyramids and Palm Trees Test, Camel and Cactus Test) (Hodges et al., 1992; Howard and Patterson, 1992; Bozeat et al., 2000; Ikeda et al., 2006; Rogers et al., 2006; Hodges and Patterson, 2007). This failure to recognize or name objects is not due to perceptual decits, as can be seen in Fig. 1. Although SD patients are able to copy drawings accurately and with great attention to detail, they are unable to assign the correct name to each gure. Figure 2 best exemplies the dissolution of semantic knowledge in the disorder. When SD patients are spontaneously asked to draw various animals, they produce drawings that converge on a prototypical four-legged creature, and they fail to include the salient features that would distinguish these animals. The deterioration of representational knowledge typical of SD can be seen in all modalities. Thus, patients may have difculty with naming from verbal descriptions (Hodges et al., 1992a), identifying odours (Luzzi et al., 2007), non-verbal sounds (e.g. ringing

Semantic dementia
The cognitive impairments experienced by Macondos inhabitants are remarkably similar to those observed in semantic dementia (SD). Semantic dementia is a clinical syndrome characterized by a breakdown of conceptual knowledge (semantic memory) in the context of relatively preserved day-to-day (episodic) memory.

History
Although initially described by Pick (1892), SD is rst recognized in the neurological literature following an article by Warrington in 1975 (Warrington, 1975). She described 3 patients with progressive anomia accompanied by a loss of word and picture comprehension. These decits were apparent in the absence of phonological or syntactic impairments of language,

 rquez a Ma Semantic dementia in Garc

Brain 2009: 132; 26092616

| 2611

Downloaded from http://brain.oxfordjournals.org/ by guest on February 11, 2013

Figure 1 Animal copies by patient B.F. (shown on right) demonstrate retention of visuoperceptual ability in SD patients. Confrontation
naming tests highlight their semantic decits. When asked to name these animals, B.F. called the cow a dog and the elephant a cow. Reference drawings by Katharine Woodman-Maynard.

of a telephone) (Bozeat et al., 2000) and selecting appropriate colours for familiar black and white drawings (e.g. choosing yellow for a banana) (Breedin et al., 1994). Most patients exhibit decits naming and later recognizing familiar faces (Hodges and Graham, 1998; Snowden et al., 2004; Thompson et al., 2004), and some have difculties with actual object use (Hodges et al., 2000; Bozeat et al., 2002). In stark opposition to their profound semantic memory decits, SD patients reportedly have preserved memory for day-to-day events. Caregivers report that patients can remember prospective appointments, keep track of the preceding days and

weeks and, in general, continue to function adequately in their daily activities. Semantic dementia patients also show preserved performance on tests of visual recall and recognition (EdwardsLee et al., 1997; Graham et al., 2000; Hodges and Graham, 2001; Scahill et al., 2005). Other cognitive skills such as visuoperceptual and spatial ability, non-verbal problem solving, and working memory can be strikingly spared, even at relatively late stages of the disease (for a review, see Hodges et al., 1992; Hodges and Patterson, 1996; Hodges et al., 1998; Hodges et al., 1999; Snowden, 1999; Perry and Hodges, 2000; Hodges and Patterson, 2007).

2612

| Brain 2009: 132; 26092616

K. Rascovsky et al. Davies et al., 2005; Shi et al., 2005; Knibb et al., 2006; Alladi et al., 2007; Snowden et al., 2007; Davies et al., 2008). Most SD patients exhibit ubiquitin-positive tau-negative inclusions, while a minority of patients show either Picks Disease or Alzheimers Disease. Atrophy always predominates in the temporopolar and perirhinal cortices with relative sparing of adjacent temporal regions (Davies et al., 2008).

Semantic dementia in One Hundred Years of Solitude

Coined by German art critic Franz Roh in 1925, the phrase magical realism originally captured a post-expressionist style of realist painting that cast common objects in a new light so as to reveal their original mysticism masked by familiarity. Through later decades, the term evolved to describe a writing style rich in fantastical elements that blend into the backdrop of an otherwise ordinary reality. In his essay Magical Realism in Spanish American Literature, Luis Leal writes, In magical realism key events have no logical or psychological explanation. The magical realist does not try to copy the surrounding reality (as the Realists did) or to wound it (as the Surrealists did) but to seize the mystery that breathes behind things. . . . In magical realism the mystery does not descend to the represented world, but rather hides and palpitates behind it (Leal, 1995). Although increasingly recognized as an international movement, magical realism has undeniable roots in Latin American literature. rquezs One Hundred Years of Solitude is often cited as a Ma Garc one of the earliest and nest examples of modern magical realism. Throughout the novel, the lines between objective reality and dreams are consistently blurred, as seen in the character of Mauricio Babilonia, a mechanic who is forever surrounded by yellow butteries. Similarly, Remedios the Beauty, too beautiful and innocent for the lustful appetite of Macondos men, one day rises into the heavens while folding laundry. In the realm rquez a Ma of magical realism, the world as imagined by Garc is not dened by traditional rationalism, but rather by alternate realities that co-exist almost seamlessly in the lives of Macondos residents. rquezs masterful use of magical realism is on full a Ma Garc display in his depiction of the memory loss epidemic that ravages Macondo. While his conception of the plague is undoubtedly rquez, the magical realist, depicts the catasa Ma fantastical, Garc trophe as if it were an everyday reality. He probes the mystery that hides and palpitates behind mundane concepts, such as the names of everyday objects. This process leads him to a terrifying visionof a world in which people are unable to communicate the names of things, or to understand their usethat bears a striking resemblance to the clinical syndrome of SD.

Downloaded from http://brain.oxfordjournals.org/ by guest on February 11, 2013

Figure 2 Drawings by patient G.W. elucidate semantic decits

in spontaneous recall. The gures converge on a prototypical four-legged creature with a rounded abdomen. Distinguishing features are absent: the sh lacks ns, the rst bird lacks wings and the elephant lacks a trunk.

Anatomy
Semantic dementia is now recognized as the temporal variant within the FTLD spectrum (Neary et al., 1998). Quantitative MRI imaging studies of patients with SD have revealed focal atrophy of the temporopolar and perirhinal cortices, as well as the anterior fusiform gyri (Galton et al., 2001b; Chan et al., 2002; Rosen et al., 2002; Davies et al., 2004; Gorno-Tempini et al., 2004). The structural integrity of these areas has been shown to correlate with performance in semantic memory tasks (Galton et al., 2001b; Davies et al., 2004; Williams et al., 2005). As the disease progresses, atrophy may also be evident in anterior medial temporal lobe structures (Chan et al., 2001; Galton et al., 2001a) as well as ventromedial frontal regions (Mummery et al., 2000).

Pathology
There have been few SD cases with pathology reported in the literature (Rossor et al., 2000; Hodges et al., 2004;

Semantic loss
rquez provides a Ma Writing in the style of magical realism, Garc beautiful descriptions of the loss of the name and notion of things experienced by Macondos villagers. This is best

 rquez a Ma Semantic dementia in Garc Arcadio to combat the exemplied by the strategy devised by Jose effects of memory loss: The sign that he hung on the neck of the cow was an exemplary proof of the way in which the inhabitants of Macondo were prepared to ght against loss of memory: This is the cow. She must be milked every morning so that she will produce milk, and the milk must be boiled in order to be mixed with coffee to make coffee and milk. Arcadio realizes that memory loss extends beyond the mere Jose loss of names; rather than simply hanging a sign that reads this is a cow on its neck, he elaborates on the animals importance in the production of milk and, by extension, coffee. The sign encapsulates not just the name of the cow, but its purpose, or its semantic connection with other objects. In the same way, patients with SD struggle not only with naming objects, as shown in Fig. 1, but also with recalling how an object must be used, or how it might interact with other objects.

Brain 2009: 132; 26092616

| 2613

Many SD patients catalogue their retained vocabulary in an attempt to keep track of object names and concepts; this activity bears a striking resemblance to the strategy described in One Hundred Years of Solitude. In Fig. 3, one SD patient imposes order on a seemingly disconnected string of vocabulary words by linking specic words to the larger semantic categories that group them together and shed light on their purpose.

Autobiographical memory
Semantic dementia also affects remote autobiographical episodic memory in a rather unusual way. Using the Autobiographical Memory Interview, Graham and Hodges (1997) found that, although SD patients were successful in producing autobiographical memories for the most recent time-period, they were signicantly impaired at retrieving episodes from childhood and early adulthood, thereby reversing the pattern of retrograde amnesia exhibited by classic amnestic and Alzheimers disease patients.

Downloaded from http://brain.oxfordjournals.org/ by guest on February 11, 2013

Figure 3 Word list by patient L.H. reects her attempt to preserve vocabulary. She writes specic words followed by parenthetical descriptions of the category into which those words t, e.g. cucumber (food) and Cincinnati (city). She also groups words by category, e.g. blanket, pillow case, sheet, bed. These strategies help the patient not only to remember the names of objects but also to recall how these words can be grouped into larger semantic categories and therefore used in relation to each other.

2614

| Brain 2009: 132; 26092616

K. Rascovsky et al.

This sequence of progressive memory loss, starting with distant memories, resembles that of Macondos inhabitants: . . . when the sick person became used to his state of vigil, the recollection of his childhood began to be erased from his memory, then the name and notion of things, and nally the identity of people and even the awareness of his own being, until he sank into a kind of idiocy that had no past.

A town copes with loss of meaning

rquez speculates on strategies a small community might a Ma Garc use to cope with the dissolution of meaning. This description serves as a testament to the immense challenges posed by a progressive loss of semantic knowledge and, more importantly, to the unwavering and human need to maintain hope during difcult times. Initially, Macondos inhabitants cope with their memory loss Arcadios labelling method to ensure practically. They apply Jose that everythings name and function is known: At the beginning of the road in to the swamp they put up a sign that said MACONDO and another larger one on the main street that said GOD EXISTS. In all the houses keys to memorizing objects and feelings had been written. Eventually, despair led some villagers to abandon the method: The system demanded so much vigilance and moral strength that many succumbed to the spell of an imaginary reality, one invented by themselves, which was less practical for them but more comforting. Pilar Ternera was the one who contributed most to popularize that mystication when she conceived the trick of reading the past in cards as she read the future before. By means of that recourse the insomniacs began to live in a world built on the uncertain alternatives of the cards, where a father was remembered faintly as the dark man who had arrived at the beginning of April and a mother was remembered only as a dark woman who wore a gold ring on her left hand, and where a birth date was reduced to the last Tuesday on which a lark sang in the laurel tree. While some of Macondos inhabitants resign themselves to their Arcadio fate, some are not content to fade into oblivion. Jose combats the insomnia plague by building an encyclopedic memory machinea blueprint for villagers to make sense of a world that has lost its meaning: The artifact was based on the possibility of reviewing every morning, from beginning to end, the totality of knowledge acquired during ones life. He conceived of it as a spinning dictionary that a person placed on the axis could operate by means of a lever, so that in very few hours there would pass before his eyes the notions most necessary for life. The memory machine, which attempts to capture words, deni rquezs most a Ma tions and notions essential to life, is Garc fantastical solution to memory loss, but remarkably is the most

Downloaded from http://brain.oxfordjournals.org/ by guest on February 11, 2013

Figure 4 Patient R.B. lists in a yearly planner his residences across a 32-year period, reecting his attempt to retain basic autobiographical information and to carry a physical reminder of his identity on his person at all times. The mention of Bethlehem indicates the importance of religion in his life, showing again how the pocketbook planner is crucial to his self-identication.

similar to the coping mechanisms used by SD patients as they deal with progressive semantic loss. The excerpts in Figs. 4 and 5, taken from a pocket planner, paint the picture of a man ghting the gradual loss of valuable memories (his home and friends), who creates a portable memory machine to keep details of his life accessible. The patients journal, kept close at all times, acts as a physical manifestation of his remote past to partially alleviate the strain of losing his identity. The desperation of Jose Arcadio, who pens almost fourteen thousand entries for his machine, further resonates with the SD patient from Fig. 3, who methodically stores the totality of her vocabulary on sheets of paper. By committing words and knowledge to paper, diaries, or a even a spinning dictionary, patients with SD and Garc rquezs ctional characters share the desire to imbue their Ma fading semantic knowledge with permanence.

Discussion
rquez created a a Ma In One Hundred Years of Solitude, Garc literary depiction of collective semantic dementia before the syndrome was recognized in neurology. The memory plague section of the novel also provides an inspiring and human account of one towns ght against the quicksand of forgetfulness.

 rquez a Ma Semantic dementia in Garc

Brain 2009: 132; 26092616

| 2615

rquez a Ma process and a fantastical plague. As such, Garc achieves a masterful portrayal of SD not just in his description of the plagues ravaging effects on semantic knowledge, but even more vividly in his account of the affected peoples ability to maintain hope through various coping strategiesto preserve their fragile self-identity in the pages of word lists and pocketbook diaries.

Caveat
Any manuscript that attempts to uncover deeper meaning in One a Hundred Years of Solitude must end with a caveat. As Garc rquez himself points out: In general, [critics], with the investiMa ture of pontiffs, dont realize that a novel like One Hundred Years of Solitude completely lacks seriousness and is full of signals to close friends, signs that only they can decipher; so they assume the responsibility of decoding all the riddles in the book, running rquez and a Ma the risk of saying a lot of stupid things (Garc Apuleyo Mendoza, 1982). Whatever his intention, it is clear that rquez offers his readers insights into the nature of a Ma Garc knowledge and the scope of semantic memory. That his depiction of Macondos dreaded insomnia plague resonates so well rquezs a Ma with clinical accounts of SD is a testament to Garc perceptiveness and to the value of studying cognition in neurodegenerative disease.

Downloaded from http://brain.oxfordjournals.org/ by guest on February 11, 2013

Figure 5 Patient R.B. devotes another page from his pocketbook planner to familiar names. The page is not thematic, as entries alternate between using location, occupation and directions as descriptive factors. The inconsistency of details highlights the fragmented memory of once intact interpersonal relationships.

References
Alladi S, Xuereb J, Bak T, Nestor P, Knibb J, Patterson K, et al. Focal cortical presentations of Alzheimers disease. Brain 2007; 130: 263645. Bozeat S, Lambon Ralph MA, Patterson K, Garrard P, Hodges JR. Non-verbal semantic impairment in semantic dementia. Neuropsychologia 2000; 38: 120715. Bozeat S, Lambon Ralph MA, Patterson K, Hodges JR. When objects lose their meaning: what happens to their use? Cogn Affect Behav Neurosci 2002; 2: 23651. Breedin SD, Saffran EM, Coslett HB. Reversal of the concreteness effect in a patient with semantic dementia. Cognit Neuropsychol 1994; 11: 61760. Chan D, Fox N, Rossor M. Differing patterns of temporal atrophy in Alzheimers disease and semantic dementia. Neurology 2002; 58: 838. Chan D, Fox NC, Jenkins R, Scahill RI, Crum WR, Rossor MN. Rates of global and regional cerebral atrophy in AD and frontotemporal dementia. Neurology 2001; 57: 175663. Davies RR, Graham KS, Xuereb JH, Williams GB, Hodges JR. The human perirhinal cortex and semantic memory. Eur J Neurosci 2004; 20: 24416. Davies RR, Halliday GM, Xuereb JH, Kril JJ, Hodges JR. The neural basis of semantic memory: evidence from semantic dementia. Neurobiol Aging 2008. Advance Access published on March 25, 2008, doi:10.1016/j.neurobiolaging.2008.02.005. Davies RR, Hodges JR, Kril JJ, Patterson K, Halliday GM, Xuereb JH. The pathological basis of semantic dementia. Brain 2005; 128: 198495. Edwards-Lee T, Miller BL, Benson DF, Cummings JL, Russell GL, Boone K, et al. The temporal variant of frontotemporal dementia. Brain 1997; 120 (Pt 6): 102740. Galton CJ, Gomez-Anson B, Antoun N, Scheltens P, Patterson K, Graves M, et al. Temporal lobe rating scale: application to Alzheimers disease and frontotemporal dementia. J Neurol Neurosurg Psychiatry 2001a; 70: 16573.

rquez envision a world in which people a Ma Why does Garc lose their ability to communicate the names of everyday things? He gives some insight into his use of magical realism in the opening lines of the novel, in which he describes the village of Macondo in its earliest days: the world was so recent that many things lacked names, and in order to indicate them it was neces rquez implies that a Ma sary to point. With this description, Garc the ability to assign a name to an everyday object is a dening human achievement that has developed over time. In this light, the insomnia plague forces Macondo villagers into a primitive state by robbing them of their ability to remember the name and notion of things. rquezs probing investigation of the a Ma Remarkably, Garc power of words and everyday names leads him to a striking literary enactment of the clinical syndrome of SD and the problems faced by SD patients. His ctional characters, just like the SD patients discussed here, are acutely aware of the innite possibilities of a loss of memory. Both patients and characters attempt to preserve semantic meaning in more permanent forms that are free from the devastating effects of a neurodegenerative

2616

| Brain 2009: 132; 26092616

K. Rascovsky et al.
Luzzi S, Snowden JS, Neary D, Coccia M, Provinciali L, Lambon Ralph MA. Distinct patterns of olfactory impairment in Alzheimers disease, semantic dementia, frontotemporal dementia, and corticobasal degeneration. Neuropsychologia 2007; 45: 182331. Mesulam MM. Slowly progressive aphasia without generalized dementia. Ann Neurol 1982; 11: 5928. Mummery CJ, Patterson K, Price CJ, Ashburner J, Frackowiak RS, Hodges JR. A voxel-based morphometry study of semantic dementia: relationship between temporal lobe atrophy and semantic memory. Ann Neurol 2000; 47: 3645. Neary D, Snowden JS, Gustafson L, Passant U, Stuss D, Black S, et al. Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology 1998; 51: 154654. Perry RJ, Hodges JR. Differentiating frontal and temporal variant frontotemporal dementia from Alzheimers disease. Neurology 2000; 54: 227784. Pick A. Uber die Beziehungen der senilen Hirnatrophie zur Aphasie. (On the relationship between senile cerebral atrophy and aphasia.). Prager Medicinische Wochenschrift 1892; 17: 1657. Rogers TT, Ivanoiu A, Patterson K, Hodges JR. Semantic memory in Alzheimers disease and the frontotemporal dementias: a longitudinal study of 236 patients. Neuropsychology 2006; 20: 31935. Rosen HJ, Gorno-Tempini ML, Goldman WP, Perry RJ, Schuff N, Weiner M, et al. Patterns of brain atrophy in frontotemporal dementia and semantic dementia. Neurology 2002; 58: 198208. Rossor MN, Revesz T, Lantos PL, Warrington EK. Semantic dementia with ubiquitin-positive tau-negative inclusion bodies. Brain 2000; 123 (Pt 2): 26776. Scahill VL, Hodges JR, Graham KS. Can episodic memory tasks differentiate semantic dementia from Alzheimers disease? Neurocase 2005; 11: 44151. Shi J, Shaw CL, Du Plessis D, Richardson AM, Bailey KL, Julien C, et al. Histopathological changes underlying frontotemporal lobar degeneration with clinicopathological correlation. Acta Neuropathol 2005; 110: 50112. Snowden J, Neary D, Mann D. Frontotemporal lobar degeneration: clinical and pathological relationships. Acta Neuropathol 2007; 114: 318. Snowden JS. Semantic dysfunction in frontotemporal lobar degeneration. Dementia Geriatr Cognit Disord 1999; 336. Snowden JS, Goulding PJ, Neary D. Semantic dementia: a form of circumscribed cerebral atrophy. Behav Neurol 1989; 2: 16782. Snowden JS, Thompson JC, Neary D. Knowledge of famous faces and names in semantic dementia. Brain 2004; 127: 86072. Thompson SA, Graham KS, Williams G, Patterson K, Kapur N, Hodges JR. Dissociating person-specic from general semantic knowledge: roles of the left and right temporal lobes. Neuropsychologia 2004; 42: 35970. Tulving E. Episodic and semantic memory. In: Tulving E, Donaldson W, editors. Organisation of memory. New York: Academic Press; 1972. pp. 381403. Warrington EK. The selective impairment of semantic memory. Q J Exp Psychol 1975; 27: 63557. Williams GB, Nestor PJ, Hodges JR. Neural correlates of semantic and behavioural decits in frontotemporal dementia. Neuroimage 2005; 24: 104251.

Galton CJ, Patterson K, Graham K, Lambon-Ralph MA, Williams G, Antoun N, et al. Differing patterns of temporal atrophy in Alzheimers disease and semantic dementia. Neurology 2001b; 57: 21625. rquez G. One hundred years of solitude. G. Rabassa, a Ma Garc translator. 2006 edn. New York: HarperCollins; 1970. rquez G, Apuleyo Mendoza P. El olor de la guayaba. Bogota: a Ma Garc Grupo Editorial Norma, S.A.; 1982. Gorno-Tempini ML, Dronkers NF, Rankin KP, Ogar JM, Phengrasamy L, Rosen HJ, et al. Cognition and anatomy in three variants of primary progressive aphasia. Ann Neurol 2004; 55: 33546. Graham KS, Hodges JR. Differentiating the roles of the hippocampal complex and the neocortex in long-term memory storage: evidence from the study of semantic dementia and Alzheimers disease. Neuropsychology 1997; 11: 7789. Graham KS, Simons JS, Pratt KH, Patterson K, Hodges JR. Insights from semantic dementia on the relationship between episodic and semantic memory. Neuropsychologia 2000; 38: 31324. Grossman M. Frontotemporal dementia: a review. J Int Neuropsychol Soc 2002; 8: 56683. Hodges JR, Bozeat S, Lambon Ralph MA, Patterson K, Spatt J. The role of conceptual knowledge in object use evidence from semantic dementia. Brain 2000; 123 (Pt 9): 191325. Hodges JR, Davies RR, Xuereb JH, Casey B, Broe M, Bak TH, et al. Clinicopathological correlates in frontotemporal dementia. Ann Neurol 2004; 56: 399406. Hodges JR, Garrard P, Patterson K, editors Semantic dementia. New York: Wiley-Liss; 1998. Hodges JR, Graham KS. A reversal of the temporal gradient for famous person knowledge in semantic dementia: implications for the neural organisation of long-term memory. Neuropsychologia 1998; 36: 80325. Hodges JR, Graham KS. Episodic memory: insights from semantic dementia. Philos Trans R Soc Lond B Biol Sci 2001; 356: 142334. Hodges JR, Patterson K. Nonuent progressive aphasia and semantic dementia: a comparative neuropsychological study. J Int Neuropsychol Soc 1996; 2: 51124. Hodges JR, Patterson K. Semantic dementia: a unique clinicopathological syndrome. Lancet Neurol 2007; 6: 100414. Hodges JR, Patterson K, Oxbury S, Funnell E. Semantic dementia. Progressive uent aphasia with temporal lobe atrophy. Brain 1992; 115 (Pt 6): 1783806. Hodges JR, Patterson K, Ward R, Garrard P, Bak T, Perry R, et al. The differentiation of semantic dementia and frontal lobe dementia (temporal and frontal variants of frontotemporal dementia) from early Alzheimers disease: a comparative neuropsychological study. Neuropsychology 1999; 13: 3140. Howard D, Patterson K. Pyramids and palm trees: a test of semantic access from pictures to words. Bury St. Edmunds, Suffolk, England: Thames Valley Test Company; 1992. Ikeda M, Patterson K, Graham KS, Ralph MA, Hodges JR. A horse of a different colour: do patients with semantic dementia recognise different versions of the same object as the same? Neuropsychologia 2006; 44: 56675. Knibb JA, Xuereb JH, Patterson K, Hodges JR. Clinical and pathological characterization of progressive aphasia. Ann Neurol 2006; 59: 15665. Leal L. Magical realism in Spanish American literature (1967). In: Zamora LP, Faris WB, editors. Magical realism. Durham: Duke UP; 1995. p. 123.

Downloaded from http://brain.oxfordjournals.org/ by guest on February 11, 2013