Pure progressive amnesia: An atypical amnestic syndrome?

We report on M.S., an 83-year-old patient with isolated pure progressive amnesia. This rare, recently identified, form of amnesia has been described in elderly patients. Neuropathological studies suggest that this syndrome is an atypical clinical presentation of Alzheimer's disease. The aim of our study was to characterize the neuropsychological pattern of pure progressive amnesia in comparison with other amnestic syndromes and memory dissociations reported in the literature. Our results indicate that pure progressive amnesia is characterized by a highly unusual dissociation in the realm of memory, with severe deficits on tests based on recognition and recall of verbal and visual single items, contrasting with relatively preserved anterograde autobiographical and spatial memory and normal recall of complex material such as stories. These findings suggest that memory for single items could depend on an independent system. One hypothesis is that M.S.'s unusual memory profile results from relative dysfunction of the ventral medial temporal lobe pathway. An alternative explanation implicates cognitive reserve. Further studies are required in order to progress on this matter. In any case, pure progressive amnesia is a clinical syndrome that may provide further insight into the organization of declarative memory.

[1]  Mortimer Mishkin,et al.  Preserved Recognition in a Case of Developmental Amnesia: Implications for the Acaquisition of Semantic Memory? , 2001, Journal of Cognitive Neuroscience.

[2]  P. Maruff,et al.  The neuropsychology of preclinical Alzheimer's disease and mild cognitive impairment , 2000, Neuroscience & Biobehavioral Reviews.

[3]  D G Gadian,et al.  Hierarchical organization of cognitive memory. , 1997, Philosophical transactions of the Royal Society of London. Series B, Biological sciences.

[4]  M. Torrens.  Co-Planar Stereotaxic Atlas of the Human Brain—3-Dimensional Proportional System: An Approach to Cerebral Imaging, J. Talairach, P. Tournoux. Georg Thieme Verlag, New York (1988), 122 pp., 130 figs. DM 268 , 1990 .

[5]  G. Waldemar,et al.  Years of severe and isolated amnesia can precede the development of dementia in early-onset Alzheimer’s disease , 2005, Neurocase.

[6]  P. Goldman-Rakic,et al.  Dual pathways connecting the dorsolateral prefrontal cortex with the hippocampal formation and parahippocampal cortex in the rhesus monkey , 1984, Neuroscience.

[7]  Carlo Caltagirone,et al.  Recognition memory for single items and for associations in amnesic patients , 2004, Neuropsychologia.

[8]  M. Mishkin,et al.  Differential effects of early hippocampal pathology on episodic and semantic memory. , 1997, Science.

[9]  Michael S. Gazzaniga,et al.  Memory Lost and Regained Following Bilateral Hippocampal Damage , 1999, Journal of Cognitive Neuroscience.

[10]  L. Squire,et al.  Selective memory loss , 1996, Neurology.

[11]  Pascale Piolino,et al.  Autobiographical memory and autonoetic consciousness: triple dissociation in neurodegenerative diseases. , 2003, Brain : a journal of neurology.

[12]  D. Perani,et al.  Isolated amnesia with slow onset and stable course, without ensuing dementia: MRI and PET data and a six-year neuropsychological follow-up. , 1996, Dementia.

[13]  Henri M. Duvernoy.  The human hippocampus , 1988 .

[14]  Robert T. Knight,et al.  Effects of extensive temporal lobe damage or mild hypoxia on recollection and familiarity , 2002, Nature Neuroscience.

[15]  Walter Kukull,et al.  Progression to dementia in patients with isolated memory loss , 1997, The Lancet.

[16]  H. Buschke,et al.  Screening for dementia by memory testing , 1988, Neurology.

[17]  B. Sahakian,et al.  Is knowledge of famous people disproportionately impaired in patients with early and questionable Alzheimer's disease? , 2002, Neuropsychology.

[18]  Sven Joubert,et al.  Preserved visual recognition memory in an amnesic patient with hippocampal lesions , 2005, Hippocampus.

[19]  M. W. Brown,et al.  Episodic memory, amnesia, and the hippocampal–anterior thalamic axis , 1999, Behavioral and Brain Sciences.

[20]  H. Soininen,et al.  MR volumetric analysis of the human entorhinal, perirhinal, and temporopolar cortices. , 1998, AJNR. American journal of neuroradiology.

[21]  Guy B. Williams,et al.  The human perirhinal cortex and semantic memory , 2004, The European journal of neuroscience.

[22]  J. Lucas,et al.  Disorders of memory. , 2005, The Psychiatric clinics of North America.

[23]  R. Stern,et al.  The influence of cognitive reserve on memory following electroconvulsive therapy. , 2003, The Journal of neuropsychiatry and clinical neurosciences.

[24]  B. Seltzer,et al.  A direct inferior parietal lobule projection to the presubiculum in the rhesus monkey , 1979, Brain Research.

[25]  P. Maruff,et al.  An optimized method for estimating intracranial volume from magnetic resonance images , 2000, Magnetic resonance in medicine.

[26]  N. Hunkin,et al.  Memory for single items, word pairs, and temporal order of different kinds in a patient with selective hippocampal lesions , 2001, Cognitive neuropsychology.

[27]  H Eichenbaum,et al.  Functional organization of the hippocampal memory system. , 1996, Proceedings of the National Academy of Sciences of the United States of America.

[28]  Gérard Deloche,et al.  Test de dénomination orale d'images , 1997 .

[29]  B. Milner,et al.  Disorders of learning and memory after temporal lobe lesions in man. , 1972, Clinical neurosurgery.

[30]  B. Deweer,et al.  [Investigation of Memory Performance With a Cued-recall Test in Alzheimers-disease] , 1994 .

[31]  J. Greene.  Contributions to Neuropsychological Assessment , 1995 .

[32]  Russell A. Epstein,et al.  The Parahippocampal Place Area Recognition, Navigation, or Encoding? , 1999, Neuron.

[33]  Permanent global amnesia with unknown etiology , 1993, Neurology.

[34]  Fabrice Wendling,et al.  Recollection of vivid memories after perirhinal region stimulations: synchronization in the theta range of spatially distributed brain areas , 2005, Neuropsychologia.

[35]  N. Hunkin,et al.  Relative sparing of item recognition memory in a patient with adult‐onset damage limited to the hippocampus , 2002, Hippocampus.

[36]  HighWire Press.  Philosophical Transactions of the Royal Society of London , 1781, The London Medical Journal.

[37]  E G Tangalos,et al.  Memory function in very early Alzheimer's disease , 1994, Neurology.

[38]  J. Becker,et al.  Focal temporal lobe dysfunction in probable Alzheimer's disease predicts a slow rate of cognitive decline , 1996, Neurology.

[39]  Y. Agid,et al.  Are explicit memory disorders of progressive supranuclear palsy related to damage to striatofrontal circuits? Comparison with Alzheimer's, Parkinson's, and Huntington's diseases , 1994, Neurology.

[40]  M. Hasselmo,et al.  The hippocampus as an associator of discontiguous events , 1998, Trends in Neurosciences.

[41]  E. Barbeau,et al.  Evaluation of visual recognition memory in MCI patients , 2004, Neurology.

[42]  R. Caselli,et al.  From slowly progressive amnesic syndrome to rapidly progressive Alzheimer disease. , 1998, Alzheimer disease and associated disorders.

[43]  D G Gadian,et al.  Amnesia and the organization of the hippocampal system , 1998, Hippocampus.

[44]  Andrew P. Yonelinas,et al.  Sparing of the familiarity component of recognition memory in a patient with hippocampal pathology , 2005, Neuropsychologia.

[45]  H. Eichenbaum.  A cortical–hippocampal system for declarative memory , 2000, Nature Reviews Neuroscience.

[46]  H Eichenbaum,et al.  Memory for items and memory for relations in the procedural/declarative memory framework. , 1997, Memory.

[47]  H. Eichenbaum,et al.  Two functional components of the hippocampal memory system , 1994, Behavioral and Brain Sciences.

[48]  M. Mishkin,et al.  A selective mnemonic role for the hippocampus in monkeys: memory for the location of objects , 1988, The Journal of neuroscience : the official journal of the Society for Neuroscience.

[49]  A. Sirigu,et al.  Pure Topographical Disorientation: A Definition and Anatomical Basis , 1987, Cortex.

[50]  D. Perani,et al.  Primary amnesia of insidious onset with subsequent stabilisation. , 1994, Journal of neurology, neurosurgery, and psychiatry.

[51]  N. Foster,et al.  Mild cognitive impairments predict dementia in nondemented elderly patients with memory loss. , 2001, Archives of neurology.

[52]  P. Caffarra,et al.  Isolated degenerative amnesia without dementia: An 8-year longitudinal study , 1996 .

[53]  G. Deloche.  Test de dénomination orale d'image : DO 80 , 1997 .

[54]  H. Braak,et al.  Neuropathological stageing of Alzheimer-related changes , 2004, Acta Neuropathologica.

[55]  John P. Aggleton,et al.  When is the perirhinal cortex necessary for the performance of spatial memory tasks? , 2004, Neuroscience & Biobehavioral Reviews.

[56]  M. Didic,et al.  A permanent pure amnestic syndrome of insidious onset related to Alzheimer's disease , 1998, Annals of neurology.