Alzheimer's disease and corticobasal degeneration presenting as corticobasal syndrome

The aim of this article is to compare patients with Alzheimer's disease (AD) pathology and corticobasal degeneration pathology (CBD) presenting as corticobasal syndrome (CBS). Clinicopathologic series was used. Five patients with AD and 11 patients with CBD were clinically diagnosed with CBS. Patients with AD pathology had an earlier age of onset than patients with CBD pathology (58 vs. 68 years, P = 0.004), but the two groups had similar disease duration and core features of CBS. Tremors were only present in CBD cases (73%, P = 0.026), but myoclonus was more common in AD than CBD (80 vs. 18%, P = 0.036). Neuropsychological testing showed similar degrees of memory impairment and attentional deficits. 99mTc‐HMPAO SPECT imaging demonstrated parietal hypoperfusion in AD patients and frontotemporal hypoperfusion in CBD patients. AD patients with clinical CBS have similar characteristics to CBD patients. Functional brain imaging may have greater utility than the clinical and neuropsychological features in differentiating AD presenting as CBS from CBD. © 2009 Movement Disorder Society

[1]  G. Wooten.  Principles and Practice of Movement Disorders , 2009 .

[2]  F. Baas,et al.  Screening for dystonia genes DYT1, 11 and 16 in patients with writer's cramp , 2009, Movement disorders : official journal of the Movement Disorder Society.

[3]  E. Foncke,et al.  Pediatric writer's cramp in myoclonus-dystonia: maternal imprinting hides positive family history. , 2009, European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society.

[4]  J. Matson,et al.  Rett syndrome in adults with severe intellectual disability: Exploration of behavioral characteristics , 2008, European Psychiatry.

[5]  G. Oliveira,et al.  Movement disorders in Rett syndrome: An analysis of 60 patients with detected MECP2 mutation and correlation with mutation type , 2008, Movement disorders : official journal of the Movement Disorder Society.

[6]  E. Altenmüller,et al.  A heterozygous frameshift mutation in PRKRA (DYT16) associated with generalised dystonia in a German patient , 2008, The Lancet Neurology.

[7]  R. Petersen,et al.  Neuropsychological findings in clinically atypical autopsy confirmed corticobasal degeneration and progressive supranuclear palsy. , 2008, Parkinsonism & related disorders.

[8]  Sonja W. Scholz,et al.  DYT16, a novel young-onset dystonia-parkinsonism disorder: identification of a segregating mutation in the stress-response protein PRKRA , 2008, The Lancet Neurology.

[9]  V. Fung,et al.  Myoclonus‐dystonia: significance of large SGCE deletions , 2008, Human mutation.

[10]  J. Schulz,et al.  Long-term EMG recordings differentiate between parkinsonian and essential tremor , 2008, Journal of Neurology.

[11]  P. Maciel,et al.  Abnormal movements in Rett syndrome are present before the regression period: A case study , 2007, Movement disorders : official journal of the Movement Disorder Society.

[12]  F. Cendes,et al.  The international cooperative ataxia rating scale in Machado‐Joseph disease. Comparison with the unified multiple system atrophy rating scale , 2007, Movement disorders : official journal of the Movement Disorder Society.

[13]  K Patterson,et al.  Focal cortical presentations of Alzheimer's disease. , 2007, Brain : a journal of neurology.

[14]  B. Miller,et al.  11C-PIB PET imaging in Alzheimer disease and frontotemporal lobar degeneration , 2007, Neurology.

[15]  J. Grafman,et al.  Alzheimer's disease presenting as corticobasal syndrome , 2006, Movement disorders : official journal of the Movement Disorder Society.

[16]  Lippincott Williams Wilkins,et al.  Scale for the assessment and rating of ataxia: Development of a new clinical scale , 2006, Neurology.

[17]  Paola Giunti,et al.  Reliability and validity of the International Cooperative Ataxia Rating Scale: A study in 156 spinocerebellar ataxia patients , 2006, Movement disorders : official journal of the Movement Disorder Society.

[18]  Joseph James Duffy,et al.  Clinicopathologic analysis of frontotemporal and corticobasal degenerations and PSP , 2006, Neurology.

[19]  E. Kerty,et al.  Long‐term botulinum toxin treatment increases employment rate in patients with cervical dystonia , 2005, Movement disorders : official journal of the Movement Disorder Society.

[20]  J. Jankovic,et al.  Comparison of botulinum toxin serotypes A and B for the treatment of cervical dystonia , 2005, Neurology.

[21]  William T. Hu,et al.  MRI correlates of alien leg‐like phenomenon in corticobasal degeneration , 2005, Movement Disorders.

[22]  C. Einspieler,et al.  Is the Early Development of Girls with Rett Disorder Really Normal? , 2005, Pediatric Research.

[23]  A. Berardelli,et al.  Validity of family history data on primary adult-onset dystonia. , 2004, Archives of neurology.

[24]  M. Doran,et al.  Pathological heterogeneity of clinically diagnosed corticobasal degeneration , 2003, Journal of the Neurological Sciences.

[25]  I. McKeith,et al.  SIC Task Force appraisal of clinical diagnostic criteria for parkinsonian disorders , 2003, Movement disorders : official journal of the Movement Disorder Society.

[26]  J. Mink,et al.  Movement disorders in children. , 2003, Pediatrics in review.

[27]  K. Poskitt,et al.  Rett Syndrome: Investigation of Nine Patients, including PET Scan , 2002, Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques.

[28]  F. Hanefeld,et al.  An update on clinically applicable diagnostic criteria in Rett syndrome. Comments to Rett Syndrome Clinical Criteria Consensus Panel Satellite to European Paediatric Neurology Society Meeting, Baden Baden, Germany, 11 September 2001. , 2002, European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society.

[29]  R. Giugliani,et al.  Neurologic findings in Machado-Joseph disease: relation with disease duration, subtypes, and (CAG)n. , 2001, Archives of neurology.

[30]  M H Lewis,et al.  Varieties of Repetitive Behavior in Autism: Comparisons to Mental Retardation , 2000, Journal of autism and developmental disorders.

[31]  R. Petersen,et al.  Highly active antiretroviral therapy reverses brain metabolite abnormalities in mild HIV dementia , 1999, Neurology.

[32]  S. Factor,et al.  Clinical comparison of tardive and idiopathic cervical dystonia , 1998, Movement disorders : official journal of the Movement Disorder Society.

[33]  M. Aramideh,et al.  Long-term effect of botulinum toxin on impairment and functional health in cervical dystonia , 1998, Neurology.

[34]  J. Trojanowski,et al.  Editorial on Consensus Recommendations for the Postmortem Diagnosis of Alzheimer Disease from the National Institute on Aging and the Reagan Institute Working Group on Diagnostic Criteria for the Neuropathological Assessment of Alzheimer Disease , 1997, Journal of neuropathology and experimental neurology.

[35]  M. Hallett,et al.  International Cooperative Ataxia Rating Scale for pharmacological assessment of the cerebellar syndrome , 1997, Journal of the Neurological Sciences.

[36]  S. Claes,et al.  Correlations between triplet repeat expansion and clinical features in Huntington's disease. , 1996, Archives of neurology.

[37]  M. H. Prins,et al.  Botulinum toxin versus trihexyphenidyl in cervical dystonia , 1996, Neurology.

[38]  C. Kitt,et al.  Preliminary Evidence for Neurodegenerative Changes in the Substantia Nigra of Rett Syndrome , 1995, Neuropediatrics.

[39]  G. Wenk.  Alterations in Dopaminergic Function in Rett Syndrome , 1995, Neuropediatrics.

[40]  B. Hagberg,et al.  Rett variants: a suggested model for inclusion criteria. , 1994, Pediatric neurology.

[41]  E. Tangalos,et al.  The short test of mental status. Correlations with standardized psychometric testing. , 1991, Archives of neurology.

[42]  G. Dellatolas,et al.  Spasmodic Torticollis — Review of 220 Patients , 1991, Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques.

[43]  J. Jankovic,et al.  Therapeutic uses of botulinum toxin. , 1991, The New England journal of medicine.

[44]  C. Marsden,et al.  Natural history of adult-onset idiopathic torticollis. , 1990, Archives of neurology.

[45]  梶龍兒,et al.  痙性斜頸患者におけるToronto Western Spasmodic Torticollis Rating Scale(TWSTRS)の評価者間信頼性の検討 , 2009 .

[46]  A. Antonietti,et al.  The role of stereotypies in overselectivity process in Rett syndrome. , 2009, Research in developmental disabilities.

[47]  C. Johannessen Landmark,et al.  Antiepileptic drugs in non-epilepsy disorders: relations between mechanisms of action and clinical efficacy. , 2008, CNS drugs.

[48]  M. Farrer,et al.  Rett Syndrome : An Overlooked Diagnosis in Women with Stereotypic Hand Movements , Psychomotor Retardation , Parkinsonism , and Dystonia ? , 2007 .

[49]  Irene Litvan,et al.  Corticobasal degeneration and its relationship to progressive supranuclear palsy and frontotemporal dementia , 2003, Annals of neurology.

[50]  A. Lang,et al.  Phenotypes and prognosis: clinicopathologic studies of corticobasal degeneration. , 2000, Advances in neurology.

[51]  E. Tangalos,et al.  Free and cued selective reminding test: MOANS norms. , 1997, Journal of clinical and experimental neuropsychology.

[52]  Shigenobu Nakamura,et al.  CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1 , 1994, Nature Genetics.

[53]  T. Yuasa,et al.  Stereotyped hand clasping: An unusual tardive movement disorder , 1993, Movement disorders : official journal of the Movement Disorder Society.

[54]  M. Brin,et al.  Idiopathic cervical dystonia: Clinical characteristics , 1991, Movement disorders : official journal of the Movement Disorder Society.

[55]  P. M. Fitzgerald,et al.  Rett syndrome and associated movement disorders , 1990, Movement disorders : official journal of the Movement Disorder Society.

[56]  S. Fahn,et al.  Analysis of open‐label trials in torsion dystonia using high dosages of anticholinergics and other drugs , 1988, Movement disorders : official journal of the Movement Disorder Society.