Skip to main content
SpringerLink
Log in

Idiopathic Late Onset Cerebellar Ataxia (ILOCA), and Cerebellar plus Syndrome

  • Reference work entry
Handbook of the Cerebellum and Cerebellar Disorders

Abstract

Spinocerebellar ataxias (SCAs), also called spinocerebellar degenerations, comprise a large group of slowly progressive neurodegenerative diseases characterized by truncal and limb ataxias as the cardinal clinical features. Sporadic degenerative ataxias include idiopathic late-onset cerebellar ataxia (ILOCA), ILOCA with cerebellar-plus syndrome, and multiple system atrophy (MSA). The clinical presentations of ILOCA (CCA) are characterized by late ages at onset with slow progression and pure cerebellar ataxia with markedly ataxic gait with relative preservation of coordination in the upper limbs. ILOCA with cerebellar-plus syndrome is characterized by cerebellar ataxia together with additional neurological features including pyramidal signs, mild dementia, supranuclear ophthalmoplegia, optic atrophy, dysphagia, parkinsonism, sphincter disturbances, hypopallaesthesia, or chorea. MSA is a sporadic neurodegenerative disorder characterized by various combinations of autonomic dysfunction, cerebellar symptoms, parkinsonism, and pyramidal signs. MSA has recently been classified into two subtypes: MSA-C (characterized by cerebellar ataxia) and MSA-P (characterized predominantly by parkinsonism). Among these sporadic neurodegenerative ataxias, ILOCA (CCA) is characterized by slow progression rate, while patients with MSA-c show faster progression compared to other neurodegenerative ataxias. Although there are no curative treatments available to prevent disease progression, continuous physiotherapeutic interventions in ataxia patients are encouraged.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution

Institutional subscriptions

References

  • Abele M, Weller M, Mescheriakov S, Burk K, Dichgans J, Klockgether T (1999) Cerebellar ataxia with glutamic acid decarboxylase autoantibodies. Neurology 52(4):857–859

    Article  PubMed  CAS  Google Scholar 

  • Adams RD, Vanbogaert L, Vandereecken H (1964) Striato-Nigral degeneration. J Neuropathol Exp Neurol 23:584–608

    PubMed  CAS  Google Scholar 

  • Baloh RW, Yee RD, Honrubia V (1986) Late cortical cerebellar atrophy. Clinical and oculographic features. Brain 109(Pt 1):159–180

    Article  PubMed  Google Scholar 

  • Berciano J, Boesch S, Perez-Ramos JM, Wenning GK (2006) Olivopontocerebellar atrophy: toward a better nosological definition. Mov Disord 21(10):1607–1613

    Article  PubMed  Google Scholar 

  • Critchley M, Greenfield JG (1948) Olivoponto-cerebellar atrophy. Brain 71(4):344–364

    Article  PubMed  CAS  Google Scholar 

  • Darnell RB, Posner JB (2003) Paraneoplastic syndromes involving the nervous system. N Engl J Med 349(16):1543–1554

    Article  PubMed  CAS  Google Scholar 

  • Déjerine J, André-Thomas A (1900) L’atrophie olivo-pontocerebelleuse. Nouv Icongr Salpet 13:330–370

    Google Scholar 

  • Eggermont E (2006) Recent advances in vitamin E metabolism and deficiency. Eur J Pediatr 165(7):429–434

    Article  PubMed  CAS  Google Scholar 

  • Fox SH, Nieves A, Bergeron C, Lang AE (2003) Pure cerebello-olivary degeneration of Marie, Foix, and Alajouanine presenting with progressive cerebellar ataxia, cognitive decline, and chorea. Mov Disord 18(12):1550–1554

    Article  PubMed  Google Scholar 

  • Furneaux HF, Reich L, Posner JB (1990) Autoantibody synthesis in the central nervous system of patients with paraneoplastic syndromes. Neurology 40(7):1085–1091

    Article  PubMed  CAS  Google Scholar 

  • Gilman S, Low PA, Quinn N, Albanese A, Ben-Shlomo Y, Fowler CJ et al (1999) Consensus statement on the diagnosis of multiple system atrophy. J Neurol Sci 163(1):94–98

    Article  PubMed  CAS  Google Scholar 

  • Gilman S, Wenning GK, Low PA, Brooks DJ, Mathias CJ, Trojanowski JQ et al (2008) Second consensus statement on the diagnosis of multiple system atrophy. Neurology 71(9):670–676

    Article  PubMed  CAS  Google Scholar 

  • Graham JG, Oppenheimer DR (1969) Orthostatic hypotension and nicotine sensitivity in a case of multiple system atrophy. J Neurol Neurosurg Psychiatry 32(1):28–34

    Article  PubMed  CAS  Google Scholar 

  • Greenfield JG (1954) The spinocerebellar degenerations. Blackwell, Oxford

    Google Scholar 

  • Hadjivassiliou M, Gibson A, Davies-Jones GA, Lobo AJ, Stephenson TJ, Milford-Ward A (1996) Does cryptic gluten sensitivity play a part in neurological illness? Lancet 347(8998):369–371

    Article  PubMed  CAS  Google Scholar 

  • Hadjivassiliou M, Grunewald RA, Chattopadhyay AK, Davies-Jones GA, Gibson A, Jarratt JA et al (1998) Clinical, radiological, neurophysiological, and neuropathological characteristics of gluten ataxia. Lancet 352(9140):1582–1585

    Article  PubMed  CAS  Google Scholar 

  • Harding AE (1981) “Idiopathic” late onset cerebellar ataxia. A clinical and genetic study of 36 cases. J Neurol Sci 51(2):259–271

    Article  PubMed  CAS  Google Scholar 

  • Ilg W, Synofzik M, Brotz D, Burkard S, Giese MA, Schols L (2009) Intensive coordinative training improves motor performance in degenerative cerebellar disease. Neurology 73(22):1823–1830

    Article  PubMed  CAS  Google Scholar 

  • Ilg W, Brotz D, Burkard S, Giese MA, Schols L, Synofzik M (2010) Long-term effects of coordinative training in degenerative cerebellar disease. Mov Disord 25(13):2239–2246

    Article  PubMed  Google Scholar 

  • Kanazawa I, Kwak S, Sasaki H, Mizusawa H, Muramoto O, Yoshizawa K et al (1985) Studies on neurotransmitter markers and neuronal cell density in the cerebellar system in olivopontocerebellar atrophy and cortical cerebellar atrophy. J Neurol Sci 71(2–3):193–208

    Article  PubMed  CAS  Google Scholar 

  • Manto M, Marmolino D (2009) Cerebellar ataxias. Curr Opin Neurol 22(4):419–429

    Article  PubMed  Google Scholar 

  • Marie P, Foix C, Alajouanine T (1922) De l’atrophie cerebelleuse tardive a predominance corticale. Rev Neurol (Paris) ii:849–885, 1082-111

    Google Scholar 

  • Massano J, Costa F, Nadais G (2008) Teaching neuroImage: MRI in multiple system atrophy: “hot cross bun” sign and hyperintense rim bordering the putamina. Neurology 71(15):e38

    Article  PubMed  Google Scholar 

  • Ormerod IE, Harding AE, Miller DH, Johnson G, MacManus D, du Boulay EP et al (1994) Magnetic resonance imaging in degenerative ataxic disorders. J Neurol Neurosurg Psychiatry 57(1):51–57

    Article  PubMed  CAS  Google Scholar 

  • Polo JM, Calleja J, Combarros O, Berciano J (1991) Hereditary ataxias and paraplegias in Cantabria, Spain. An epidemiological and clinical study. Brain 114(2):855–866

    Article  PubMed  Google Scholar 

  • Saiz A, Arpa J, Sagasta A, Casamitjana R, Zarranz JJ, Tolosa E et al (1997) Autoantibodies to glutamic acid decarboxylase in three patients with cerebellar ataxia, late-onset insulin-dependent diabetes mellitus, and polyendocrine autoimmunity. Neurology 49(4):1026–1030

    Article  PubMed  CAS  Google Scholar 

  • Schols L, Bauer P, Schmidt T, Schulte T, Riess O (2004) Autosomal dominant cerebellar ataxias: clinical features, genetics, and pathogenesis. Lancet Neurol 3(5):291–304

    Article  PubMed  Google Scholar 

  • Selim M, Drachman DA (2001) Ataxia associated with Hashimoto’s disease: progressive non-familial adult onset cerebellar degeneration with autoimmune thyroiditis. J Neurol Neurosurg Psychiatry 71(1):81–87

    Article  PubMed  CAS  Google Scholar 

  • Shy GM, Drager GA (1960) A neurological syndrome associated with orthostatic hypotension: a clinical-pathologic study. Arch Neurol 2:511–527

    Article  PubMed  CAS  Google Scholar 

  • Sillevis Smitt P, Kinoshita A, De Leeuw B, Moll W, Coesmans M, Jaarsma D et al (2000) Paraneoplastic cerebellar ataxia due to autoantibodies against a glutamate receptor. N Engl J Med 342(1):21–27

    Article  PubMed  CAS  Google Scholar 

  • Sitrin MD, Lieberman F, Jensen WE, Noronha A, Milburn C, Addington W (1987) Vitamin E deficiency and neurologic disease in adults with cystic fibrosis. Ann Intern Med 107(1):51–54

    PubMed  CAS  Google Scholar 

  • Spillantini MG, Crowther RA, Jakes R, Cairns NJ, Lantos PL, Goedert M (1998) Filamentous alpha-synuclein inclusions link multiple system atrophy with Parkinson’s disease and dementia with Lewy bodies. Neurosci Lett 251(3):205–208

    Article  PubMed  CAS  Google Scholar 

  • Tsuji S, Onodera O, Goto J, Nishizawa M (2008) Sporadic ataxias in Japan–a population-based epidemiological study. Cerebellum 7(2):189–197

    Article  PubMed  CAS  Google Scholar 

  • Wakabayashi K, Yoshimoto M, Tsuji S, Takahashi H (1998) Alpha-synuclein immunoreactivity in glial cytoplasmic inclusions in multiple system atrophy. Neurosci Lett 249(2–3):180–182

    Article  PubMed  CAS  Google Scholar 

  • Wenning GK, Tison F, Seppi K, Sampaio C, Diem A, Yekhlef F et al (2004) Development and validation of the Unified Multiple System Atrophy Rating Scale (UMSARS). Mov Disord 19(12):1391–1402

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Neurology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan

    Dr. Shoji Tsuji

Authors
  1. Dr. Shoji Tsuji
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Shoji Tsuji .

Editor information

Editors and Affiliations

  1. Unité d'Etude du Mouvement (UEM), FNRS, Neurologie ULB Erasme, Bruxelles, Belgium

    Mario Manto

  2. Ataxia Unit, Cognitive and Behavioral Neurology Unit, Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA

    Jeremy D. Schmahmann

  3. Neuroscience Institute of the Cavalieri-Ottolenghi Foundation (NICO), University of Turin, Orbassano, Turin, Italy

    Ferdinando Rossi

  4. Molecular and Integrative Neuroscience Department (MIND), The Scripps Research Institute, California, CA, USA

    Donna L. Gruol

  5. Department of Integrative Physiolgy, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan

    Noriyuki Koibuchi

Rights and permissions

Reprints and permissions

Copyright information

© 2013 Springer Science+Business Media Dordrecht

About this entry

Cite this entry

Tsuji, S. (2013). Idiopathic Late Onset Cerebellar Ataxia (ILOCA), and Cerebellar plus Syndrome. In: Manto, M., Schmahmann, J.D., Rossi, F., Gruol, D.L., Koibuchi, N. (eds) Handbook of the Cerebellum and Cerebellar Disorders. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-1333-8_98

Download citation

Publish with us

Policies and ethics

Search

Navigation