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Neuropsychological and neuroimaging correlates in corticobasal degeneration

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Abstract

The aim of this study was to correlate neuropsychological and neuroimaging findings in corticobasal degeneration (CBD). Three patients with clinical criteria for CBD were examined by means of neuropsychological tests, brain magnetic resonance imaging (MRI), and flow and metabolism neuroimaging techniques. Neuropsychological assessment revealed impairment in executive functions, manual dexterity and motor programming with significant asymmetry between upper limbs. Ideomotor and oral apraxia were also detected, and memory deficits were observed in one patient. MRI revealed cortical dilation of the frontal and perirolandic regions, symmetrical in one case and asymmetrical in the other two cases. An increased T2 signal intensity in the posterolateral putamen and substantia nigra ipsilateral to the cortical atrophy was observed in one patient. Asymmetries of both frontal and parietal cortices and basal ganglia were detected in all three patients by 18-fluorodeoxyglucose positron emission tomography; temporal region hypometabolism was associated in one patient. These cortical and subcortical asymmetries were observed in two patients by single photon emission tomography with the tracer technetium Tc 99m hexamethyl propylenamine oxime; cortical asymmetry was observed in only one patient. The results showed that functional neuroimaging findings correlated well with neuropsychological aspects in CBD. Neuroimaging and neuropsychological correlations may contribute toward understanding anatomical and functional abnormalities associated with this neurodegenerative disorder.

Sommario

Nel presente studio vengono riportate le correlazioni neuropsicologiche e neuroradiologiche in tre pazienti affetti da una rara malattia neurodegenerativa, la degenerazione cortico-basale (DCB). Lo studio neuropsicologico ha mostrato un decadimento nelle funzioni esecutive, nella destrezza manuale e programmazione rnotoria con una asimmetria tra i due arti superiori e un'aprassia ideo-motoria e bucco facciale. La risonanza magnetica nucleare (RMN) dell'encefalo ha evidenziato una dilatazione corticale in sede frontale e peri-rolandica, con asimmetria controlaterale al lato, più affetto in due casi, e simmetrica in un caso; inoltre un aumentato segnale T2 in sede di putamen e della sostanza nera ipsilaterale all'atrofia corticale è stato osservato in un paziente. La PET con tracciante 18-fluorodeossiglucosio ha evidenziato un ipometabolismo asimmetrico sia della corteccia frontale e parietale the dei gangli della base. La SPECT con tracciante 99-tecnezio ha evidenziato un'asimmetria corticale e sottocorticale in due casi; in un caso è stata osservata un'asimmetria solo in sede corticale. Le nostre osservazioni sui pazienti con DCB hanno mostrato una precisa correlazione tra aspetti neuropsicologici e neuroradiologici. Tali correlazioni possono dare un contributo allo studio delle alterazioni anatomiche e funzionali associate con questo disturbo neurodegenerativo.

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References

  1. Gibb WRG, Luthert PJ, Marsden CD (1989) Corticobasal degeneration. Brain 112:1171–1192

    Google Scholar 

  2. Riley DE, Lang AE, Lewis A et al (1990) Cortical-basal ganglionic degeneration. Neurology 40:1203–1212

    Google Scholar 

  3. Rinne JO, Lee MS, Thompson PD, Marsden CD (1994) Corticobasal degeneration. A clinical study of 36 cases. Brain 117:1183–1196

    Google Scholar 

  4. Rebeiz JJ, Kolodny HE, Richardson EP Jr (1968) Corticodentatonigral degeneration with neuronal achromasia. Arch Neurol 18:20–33

    Google Scholar 

  5. Watts RL, Williams RS, Growdon JD, Young RR, Haley EC Jr, Beal MF (1985) Corticobasal ganglionic degeneration. Neurology 35[Suppl 1]:178

    Google Scholar 

  6. Lippa CF, Smith TW, Fontneau N (1990) Corticonigral degeneration with neuronal achromasia: A clinicopathological study of two cases. J Neurol Sci 98:301–310

    Google Scholar 

  7. Lippa CF, Cohen R, Smith TW, Drachman DA (1991) Primary progressive aphasia with focal neuronal achromasia. Neurology 41:882–886

    Google Scholar 

  8. Brown J, Lantos PL, Roques P, Fidani L, Rossor MN (1996) Familial dementia with swollen achromatic neurons and corticobasal inclusion bodies: A clinical and pathological study. J Neurol Sci 135:21–30

    Google Scholar 

  9. Litvan J, Agid Y, Goetz C et al (1997) Accuracy of the clnical diagnosis of corticobasal degeneration: A clinicopathologic study. Neurology 48:119–125

    Google Scholar 

  10. Leiguarda R, Lees AJ, Merello M, Starkstein S, Marsden CD (1994) The nature of apraxia in cortico-basal degeneration. J Neurol Neurosurg Psychiatry 57:455–459

    Google Scholar 

  11. Pillon B, Blin J, Vidailhet M et al (1995) The neuropsychological pattern of corticobasal degeneration: Comparison with progressive supranuclear palsy and Alzheimer's disease. Neurology 45:1477–1483

    Google Scholar 

  12. Massman PJ, Kreiter KT, Jankovic J, Doody RS (1996) Neuropsychological functioning in cortical-basal ganglionic degeneration: Differentiation from Alzheimer's disease. Neurology 46:720–726

    Google Scholar 

  13. Sawle GV, Brooks DJ, Marsden CD, Frackoviack RJ (1991) Corticobasal degeneration. A unique pattern of regional cortical oxygen hypometabolism and striatal fluorodopa uptake demonstrated by positron emission tomography. Brain 114:542–546

    Google Scholar 

  14. Eidelberg D, Dhawane V, Moeller JR et al (1991) The metabolic landscape of cortico-basal ganglionic degeneration: Regional asymmetries studied with positron emission tomography. J Neurol Neurosurg Psychiatry 54:856–862

    Google Scholar 

  15. Blin J, Vidailhet M, Pillon B, Dubois B, Feve JR, Agid Y (1992) Corticobasal degeneration: Decreased and asymmetrical glucose consumption as studied with PET. Mov Disord 7:348–354

    Google Scholar 

  16. Okuda B, Takeda M, Kawabata K, Sugita M, Fukuchi M (1995) Focal cortical hypoperfusion in corticobasal degeneration demonstrated by three dimensional surface dysplay with 1231MP: A possible cause of apraxia. Neuroradiology 37:642–644

    Google Scholar 

  17. Markus HS, Lees AJ, Lennox G, Marsden CD, Costa DC (1995) Patterns of regional cerebral blood flow in corticobasal degeneration studied using HMPAO SPECT: Comparison with Parkinson's disease and normal controls. Mov Disord 10:179–187

    Google Scholar 

  18. Nagasawa H, Tanji H, Momura H et al (1996) PET study of cerebral glucose metabolism and fluorodopa uptake in patients with corticobasal degeneration. J Neurol Sci 139:210–217

    Google Scholar 

  19. Brazzelli M, Carpitani E, Della Sala S, Splinner H, Zuffi M (1994) Milan overall dementia assessment. Organizzazioni Speciali, Florence

    Google Scholar 

  20. Folstein MF, Folstein SE, Hugh PR (1975) “Mini-Mental State”: A practical method for grading cognitive state of patients for the clinicians. J Psychiatr Res 12:189–198

    Google Scholar 

  21. Spinnler H, Tognoni G (1987) Standardizzazione e taratura italiana di tests neuropsicologici. Ital J Neurol Sci [Suppl 8]

  22. Luzzati C, Willmes K, De Bleser R (1996) L'Aachner aphasie test (AAT). Organizzazioni Speciali, Florence

    Google Scholar 

  23. Nelson HE (1976) A modified card sorting test sensitive to frontal lobe defect. Cortex 12:313–324

    Google Scholar 

  24. Lezak MD (1995) Neuropsychological assessment. Oxford University, Oxford

    Google Scholar 

  25. Golden CJ, Hammeke TA, Purisch AP (1985) The Luria-Nebraska neurophysiological battery. Western Psychological Services, Los Angeles

    Google Scholar 

  26. De Renzi E, Piezcuro A, Vignolo A (1968) Ideational apraxia: A quantitative study. Neuropsychologia 6:41–52

    Google Scholar 

  27. De Renzi E, Lucchelli F (1988) Ideational apraxia. Brain 111:1173–1185

    Google Scholar 

  28. De Renzi E, Motti F, Nichelli P (1980) Imitating gestures: A quantitative approach to ideomotor apraxia. Arch Neurol 37:6–10

    Google Scholar 

  29. Leiguarda RC, Pramstaller PP, Merello M, Starkstein S, Lees AJ, Marsden CD (1997) Apraxia in Parkinson's disease, progressive sopranuclear palsy, multiple system atrophy and neuroleptic-induced parkinsonism. Brain 120:75–90

    Google Scholar 

  30. Pramstaller PP, Marsden CD (1996) The basal ganglia and apraxia. Brain 119:319–340

    Google Scholar 

  31. Okuda B, Tachibana H, Kawabata K, Takeda M, Sugita M (1992) Slowly progressive limb-kinetic apraxia with a decrease in unilateral cerebral blood flow. Acta Neurol Scand 86:76–8135

    Google Scholar 

  32. Watson R, Fleet S, Gonzàlez Rothi LJ, Heilman KM (1986) Apraxia and the supplementary motor area. Arch Neurol 43:787–792

    Google Scholar 

  33. Tokumaru AM, O'uchi T, Kuru Y, Maki T, Murayama S, Horichi Y (1997) Corticobasal degeneration: MR and histopathologic comparison. AJNR Am J Neuroradiol 17:1849–1852

    Google Scholar 

  34. Alexander GE, Delong MR, Strick PP (1986) Parallel organization of functionally segregated circuits linking basal ganglia and cortex. Annu Rev Neurosci 9:357–381

    Google Scholar 

  35. Alexander GE, Crutcher MD, DeLong MR (1990) Basal ganglia-thalamocortical circuits: Parallel substrates for motor, oculomotor, ‘prefrontal’ and ‘limbic’ functions. Prog Brain Res 85:119–146

    Google Scholar 

  36. Bergeron MD, Pollanen MS, Black SE, Lang AE (1996) Unusual clinical presentations of cortico-basal ganglionic degeneration. Ann Neurol 40:893–900

    Google Scholar 

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Authors and Affiliations

  1. Department of Neurological and Visual Sciences, Policlinico Borgo Roma, I-37134, Verona, Italy

    E. Frasson, G. Moretto, M. Pampanin, C. Stegagno, R. Tanel & N. Rizzuto

  2. Institute of Radiology, Policlinico Borgo Roma, Verona, Italy

    A. Beltramello

  3. Rehabilitation Unit, Policlinico Borgo Roma, Verona, Italy

    N. Smania

Authors
  1. E. Frasson
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  2. G. Moretto
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  3. A. Beltramello
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  4. N. Smania
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  5. M. Pampanin
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  6. C. Stegagno
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  7. R. Tanel
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  8. N. Rizzuto
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Frasson, E., Moretto, G., Beltramello, A. et al. Neuropsychological and neuroimaging correlates in corticobasal degeneration. Ital J Neuro Sci 19, 321–328 (1998). https://doi.org/10.1007/BF00713860

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  • DOI: https://doi.org/10.1007/BF00713860

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