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Imaging of corticobasal degeneration syndrome

  • Diagnostic Neuroradiology
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Abstract

Introduction

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Diagnosing corticobasal degeneration is often difficult on the basis of clinical symptoms and radiological images. We aimed to clarify the imaging findings of corticobasal degeneration syndrome (CBDS).

Methods

Included in the study were 16 patients (8 men, 8 women, 46–75 years old) with clinically diagnosed CBDS. We evaluated the patients’ symptoms and signs, and MR and single-photon emission CT (SPECT) imaging findings.

Results

All the patients had cerebral atrophy. Asymmetric cerebral atrophy was observed in 13 patients (81%) predominantly contralateral to the side clinically more affected. Atrophy in the cerebral peduncle was observed in seven patients. FLAIR images showed hyperintensity in the subcortical white matter in the frontoparietal lobes in the clinically more affected side in 14 patients, and in the rolandic region in 13 patients. Asymmetric hypoperfusion in the frontoparietal lobes on SPECT images was observed in all of the patients, and in the basal ganglia in 11 patients.

Conclusion

CBDS might be unique in showing hyperintensity in the subcortical white matter in the rolandic region on FLAIR images with asymmetric atrophy predominantly contralateral to the side clinically more severely affected. Asymmetric atrophy in the cerebral peduncle without signal abnormalities was also characteristic of CBDS. Atrophy in the midbrain tegmentum was also seen in patients with CBDS.

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References

  1. Boeve BF, Lang AE, Litvan I (2003) Corticobasal degeneration and its relationship to progressive supranuclear palsy and frontotemporal dementia. Ann Neurol 54(Suppl 5):S15–S19

    Article  PubMed  Google Scholar 

  2. Lowe JS, Leigh N (2002) Corticobasal degeneration. In: Graham DI, Lantos PL (eds) Greenfield’s neuropathology, 7th edn, vol. 2. Arnold, London, pp 346–350

    Google Scholar 

  3. Shannon KM (2004) Corticobasal degeneration. In: Bradley WG, Daroff RB, Fenichel GM, Jankovic J (eds) Neurology in clinical practice, 4th edn, vol. 2. Butterworth Heinemann, Philadelphia, p 2142

    Google Scholar 

  4. Doran M, du Plessis DG, Enevoldson TP, Fletcher NA, Ghadiali E, Larner AJ (2003) Pathological heterogeneity of clinically diagnosed corticobasal degeneration. J Neurol Sci 216:127–134

    Article  PubMed  CAS  Google Scholar 

  5. Matsuda H, Yagishita A, Tsuji S, Hisada K (1995) A quantitative approach to technetium-99m ethyl cysteinate dimer; a comparison with technetium-99m hexamethylpropylene amine oxime. Eur J Nucl Med 22:633–637

    Article  PubMed  CAS  Google Scholar 

  6. Galton CJ, Patterson K, Xuereb JH, Hodges JR (2000) Atypical and typical presentations of Alzheimer’s disease: a clinical, neuropsychological, neuroimaging and pathological study of 13 cases. Brain 123:484–498

    Article  PubMed  Google Scholar 

  7. Kaida K, Takeda K, Nagata N, Kamakura K (1998) Alzheimer’s disease with asymmetric parietal lobe atrophy: a case report. J Neurol Sci 160:96–99

    Article  PubMed  CAS  Google Scholar 

  8. Giannakopoulos P, Hof PR, Bouras C (1994) Alzheimer’s disease with asymmetric atrophy of the cerebral hemispheres: morphometric analysis of four cases. Acta Neuropathol (Berl) 88:440–447

    CAS  Google Scholar 

  9. Grossman RI, Yousem DM (2003) Neuroradiology: the requisites, 2nd edn. Mosby, Philadelphia, p 387

    Google Scholar 

  10. Parent A (1996) Carpenter’s human neuroanatomy, 9th edn. Williams & Wilkins, Baltimore, pp 452–456

    Google Scholar 

  11. Tsuchiya K, Murayama S, Mitani K, Oda T, Arima K, Mimura M, Nagura H, Haga C, Akiyama H, Yamanouchi H, Mizusawa H (2005) Constant and severe involvement of Betz cells in corticobasal degeneration is not consistent with pyramidal signs: a clinicopathological study of ten autopsy cases. Acta Neuropathol 109:353–366

    Article  PubMed  Google Scholar 

  12. Boelmans K, Kaufmann J, Bodammer N, Heinze HJ, Niehaus L (2006) Corticospinal tract atrophy in corticobasal degeneration. Arch Neurol 63:462–463

    Article  PubMed  Google Scholar 

  13. Yagishita A, Oda M (1996) Progressive supranuclear palsy: MRI and pathological findings. Neuroradiology 38(Suppl 1):S60–S66

    Article  PubMed  Google Scholar 

  14. Oba H, Yagishita A, Terada H, Barkovich AJ, Kutomi K, Yamauchi T, Furui T, Shimizu T, Uchigata M, Matsumura K, Sonoo M, Sakai M, Takada K, Harasawa A, Takeshita K, Kohtake H, Tanaka H, Suzuki S (2005) New and reliable MRI diagnosis for progressive supranuclear palsy. Neurology 64:2050–2055

    Article  PubMed  CAS  Google Scholar 

  15. Doi T, Iwasa K, Makifuchi T, Takamiri M (1999) White matter hyperintensities on MRI in a patient with corticobasal degeneration. Acta Neurol Scand 99:199–201

    Article  PubMed  CAS  Google Scholar 

  16. Winkelmann J, Auer DP, Lechner C, Elbel G, Trenkwalder C (1999) Magnetic resonance imaging findings in corticobasal degeneration. Mov Disord 14:669–673

    Article  PubMed  CAS  Google Scholar 

  17. Mirra SS, Hyman BT (2002) Corticobasal degeneration. In: Graham DI, Lantos PL (eds) Greenfield’s neuropathology, 7th edn, vol. 2. Arnold, London, pp 231–232

    Google Scholar 

  18. Hecht MJ, Fellner F, Fellner C, Hilz MN, Heuss D, Neundorfer B (2001) MRI-FLAIR images of the head show corticospinal tract alterations in ALS patients more frequently than T2-, T1- and proton-density-weighted images. J Neurol Sci 186:37–44

    Article  PubMed  CAS  Google Scholar 

  19. Oba H, Araki T, Ohtomo K, Monzawa S, Uchiyama G, Koizumi K, Nogata Y, Kachi K, Shiozawa Z, Kobayashi M (1993) Amyotrophic lateral sclerosis: T2 shortening in motor cortex at MR imaging. Radiology 189:843–846

    PubMed  CAS  Google Scholar 

  20. Matsusue E, Kinoshita T, Ohama E, Ogawa T (2005) Cerebral cortical and white matter lesions in chronic hepatic encephalopathy: MR-pathologic correlations. AJNR Am J Neuroradiol 26:347–351

    PubMed  Google Scholar 

  21. Kitagaki H, Mori E, Hirono N, Hirono N, Ikejiri Y, Ishii K, lmamura T, Ikeda M, Yamaji S, Yamashita H, Shimomura T, Nakagawa Y (1997) Alteration of white matter MR signal intensity in frontotemporal dementia. AJNR Am J Neuroradiol 18:367–378

    PubMed  CAS  Google Scholar 

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Acknowledgement

We thank Shouki Takahashi, MD, for his advice.

Conflict of interest statement

We declare that we have no conflict of interest.

Author information

Author notes

  1. Masamichi Koyama

    Present address: Department of Nuclear Medicine, Cancer Institute Hospital, 3-10-6, Ariake, Koto-ku, Tokyo, 135-8550, Japan

Authors and Affiliations

  1. Department of Neuroradiology, Tokyo Metropolitan Neurological Hospital, 2-6-1 Musashidai, Fuchu, Tokyo, 183-0042, Japan

    Masamichi Koyama, Akira Yagishita & Yasuhiro Nakata

  2. Department of Neurology, Tokyo Metropolitan Neurological Hospital, 2-6-1 Musashidai, Fuchu, Tokyo, 183-0042, Japan

    Mitsuaki Bandoh

  3. Department of Neuropathology, Tokyo Metropolitan Neurological Hospital, 2-6-1 Musashidai, Fuchu, Tokyo, 183-0042, Japan

    Toshio Mizutani

  4. Department of Clinical Neuropathology, Tokyo Metropolitan Neurological Institute, 2-6 Musashidai, Fuchu, Tokyo, 183-8526, Japan

    Masaharu Hayashi

Authors
  1. Masamichi Koyama
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  2. Akira Yagishita
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  3. Yasuhiro Nakata
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  4. Masaharu Hayashi
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Corresponding author

Correspondence to Masamichi Koyama.

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Conflict of interest statement

We declare that we have no conflict of interest.

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Koyama, M., Yagishita, A., Nakata, Y. et al. Imaging of corticobasal degeneration syndrome. Neuroradiology 49, 905–912 (2007). https://doi.org/10.1007/s00234-007-0265-6

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  • DOI: https://doi.org/10.1007/s00234-007-0265-6

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