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The Cerebellar Cognitive Affective Syndrome and the Neuropsychiatry of the Cerebellum

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Handbook of the Cerebellum and Cerebellar Disorders

Abstract

The traditional teaching that the cerebellum is purely a motor control device no longer appears to be valid, if indeed it ever was. Current evidence indicates that there is functional topographic organization in the cerebellum such that the cerebellar anterior lobe, adjacent part of lobule VI, and lobule VIII and their associated nuclei are principally engaged with motor control. In contrast, the great expanse of the cerebellar posterior lobe (lobules VI and VII) along with lobule IX and associated nuclei are engaged in the modulation of supramodal, higher order functions. This connectional and functional arrangement manifests clinically in two overarching clinical syndromes. Lesions of the motor cerebellum result in the cerebellar motor syndrome of ataxia, dysmetria, dysarthria, and oculomotor abnormalities. Lesions of the cognitive and limbic cerebellum lead to the cerebellar cognitive affective syndrome (CCAS), both in adults and in children, characterized by impairments in executive function, visual spatial processing, linguistic deficits, and affective dysregulation. The affective component of the CCAS, conceptualized as the neuropsychiatry of the cerebellum, has been grouped according to five major domains: attentional control, emotional control, autism spectrum disorders, psychosis spectrum disorders, and social skill set. Within each of these domains, behaviors may reflect cognitive overshoot or undershoot, akin to the disorder of motor control seen in the cerebellar motor syndrome. This chapter focuses on the behavioral neurology and neuropsychiatry of the cerebellum in a number of different disease states, and draws attention to the fact that this rapidly evolving field of neuroscience has clinically relevant ramifications for adults, children, and infants with cerebellar dysfunction. The chapter aims to enhance the recognition of the CCAS and its theoretical underpinnings, and to highlight the promise these new insights hold for novel interventions in patients with neurobehavioral and psychiatric disease linked to the cerebellum.

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References

  • Aarsen FK, Van Dongen HR, Paquier PF et al (2004) Long-term sequelae in children after cerebellar astrocytoma surgery. Neurology 62(8):1311–1316

    Article  CAS  PubMed  Google Scholar 

  • Ackermann H, Vogel M, Petersen D et al (1992) Speech deficits in ischaemic cerebellar lesions. J Neurol 239(4):223–227

    Article  CAS  PubMed  Google Scholar 

  • Adamaszek M, D’Agata F, Ferrucci R et al (2017) Consensus paper: cerebellum and emotion. Cerebellum 16(2):552–576

    Article  CAS  PubMed  Google Scholar 

  • Adrian E (1943) Afferent Ares in the cerebellum connected with the limbs. Brain 66:289–315

    Article  Google Scholar 

  • Ahmadian N, van Baarsen K, van Zandvoort M, Robe PA (2019) The cerebellar cognitive affective syndrome-a meta-analysis. Cerebellum 18(5):941–950

    Article  PubMed  PubMed Central  Google Scholar 

  • Albazron FM, Bruss J, Jones RM et al (2019) Pediatric postoperative cerebellar cognitive affective syndrome follows outflow pathway lesions. Neurology 93(16):e1561–e1571

    Article  PubMed  PubMed Central  Google Scholar 

  • Alexander MP, Gillingham S, Schweizer T et al (2012) Cognitive impairments due to focal cerebellar injuries in adults. Cortex 48(8):980–990

    Google Scholar 

  • Allin M, Matsumoto H, Santhouse AM et al (2001) Cognitive and motor function and the size of the cerebellum in adolescents born very pre-term. Brain 124(Pt 1):60–66

    Article  CAS  PubMed  Google Scholar 

  • Amarenco P, Hauw JJ (1990) Cerebellar infarction in the territory of the superior cerebellar artery: a clinicopathologic study of 33 cases. Neurology 40(9):1383–1390

    Article  CAS  PubMed  Google Scholar 

  • Amarenco P, Hauw JJ, Henin D et al (1989) Cerebellar infarction in the area of the posterior cerebellar artery. Clinicopathology of 28 cases. Rev Neurol (Paris) 145(4):277–286

    CAS  Google Scholar 

  • Amarenco P, Roullet E, Goujon C et al (1991) Infarction in the anterior rostral cerebellum (the territory of the lateral branch of the superior cerebellar artery). Neurology 41(2 (Pt 1)):253–258

    Article  CAS  PubMed  Google Scholar 

  • Anderson CM, Teicher MH, Polcari A et al (2002) Abnormal T2 relaxation time in the cerebellar vermis of adults sexually abused in childhood: potential role of the vermis in stress-enhanced risk for drug abuse. Psychoneuroendocrinology 27(1–2):231–244

    Article  PubMed  Google Scholar 

  • Anderson CM, Maas LC, Frederick B et al (2006) Cerebellar vermis involvement in cocaine-related behaviors. Neuropsychopharmacology 31(6):1318–1326

    Article  CAS  PubMed  Google Scholar 

  • Annoni JM, Ptak R, Caldara-Schnetzer AS et al (2003) Decoupling of autonomic and cognitive emotional reactions after cerebellar stroke. Ann Neurol 53(5):654–658

    Article  PubMed  Google Scholar 

  • Argyropoulos GPD, van Dun K, Adamaszek M et al (2020) The cerebellar cognitive affective/Schmahmann syndrome: a task force paper. Cerebellum 19(1):102–125

    Article  CAS  PubMed  Google Scholar 

  • Assal F, Valenza N, Landis T et al (2000) Clinicoanatomical correlates of a Fou rire prodromique in a pontine infarction. J Neurol Neurosurg Psychiatry 69(5):697–698

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Babinski J (1899) De l’asynergie cérébelleuse. Rev Neurol 7:806–816

    Google Scholar 

  • Baillieux H, De Smet HJ, Lesage G et al (2006) Neurobehavioral alterations in an adolescent following posterior fossa tumor resection. Cerebellum 5(4):289–295

    Article  PubMed  Google Scholar 

  • Barth A, Bogousslavsky J, Regli F (1993) The clinical and topographic spectrum of cerebellar infarcts: a clinical-magnetic resonance imaging correlation study. Ann Neurol 33(5):451–456

    Article  CAS  PubMed  Google Scholar 

  • Bauman ML, Kemper TL (1985) Histoanatomic observations of the brain in early infantile autism. Neurology 35(6):866–874

    Article  CAS  PubMed  Google Scholar 

  • Bauman ML, Kemper TL (2005) Neuroanatomic observations of the brain in autism: a review and future directions. Int J Dev Neurosci 23(2–3):183–187

    Article  PubMed  Google Scholar 

  • Berger A, Sadeh M, Tzur G et al (2005) Task switching after cerebellar damage. Neuropsychology 19(3):362–370

    Article  PubMed  Google Scholar 

  • Berman AF, Berman D, Prescott JW (1978) The effect of cerebellar lesions on emotional behavior in the rhesus monkey. In: Cooper IS, Riklan M, Snider RS (eds) The cerebellum, epilepsy and behavior. Plenum Press, New York, pp 277–284

    Google Scholar 

  • Berquin PC, Giedd JN, Jacobsen LK et al (1998) Cerebellum in attention-deficit hyperactivity disorder: a morphometric MRI study. Neurology 50(4):1087–1093

    Article  CAS  PubMed  Google Scholar 

  • Blackwood N, Ffytche D, Simmons A et al (2004) The cerebellum and decision making under uncertainty. Brain Res Cogn Brain Res 20(1):46–53

    Article  PubMed  Google Scholar 

  • Bolk L (1906) In: Bohn F (ed) Das Cerebellum der Saugetiere. Fischer, Jena

    Google Scholar 

  • Botez-Marquard T, Botez MI (1993) Cognitive behavior in heredodegenerative ataxias. Eur Neurol 33(5):351–357

    Article  CAS  PubMed  Google Scholar 

  • Botez-Marquard T, Leveille J, Botez MI (1994) Neuropsychological functioning in unilateral cerebellar damage. Can J Neurol Sci 21(4):353–357

    Article  CAS  PubMed  Google Scholar 

  • Bower JM (1997) Control of sensory data acquisition. In: Schmahmann JD (ed) Cerebellum and cognition. Academic, San Diego, pp 489–513

    Google Scholar 

  • Brady RO Jr, Gonsalvez I, Lee I et al (2019) Cerebellar-prefrontal network connectivity and negative symptoms in schizophrenia. Am J Psychiatry 176(7):512–520

    Article  PubMed  PubMed Central  Google Scholar 

  • Braga-Neto P, Pedroso JL, Alessi H et al (2012) Cerebellar cognitive affective syndrome in Machado Joseph disease: core clinical features. Cerebellum 11(2):549–556

    Google Scholar 

  • Brodal P (1978) The corticopontine projection in the rhesus monkey. Origin and principles of organization. Brain 101(2):251–283

    Article  CAS  PubMed  Google Scholar 

  • Brodal A (1981) Neurological anatomy in relation to clinical medicine. Oxford University Press, New York

    Google Scholar 

  • Bruce V, Young A (1986) Understanding face recognition. Br J Psychol 77(Pt 3):305–327

    Article  PubMed  Google Scholar 

  • Brunet E, Sarfati Y, Hardy-Bayle MC et al (2000) A PET investigation of the attribution of intentions with a nonverbal task. NeuroImage 11(2):157–166

    Article  CAS  PubMed  Google Scholar 

  • Burk K, Bosch S, Globas C et al (2001) Executive dysfunction in spinocerebellar ataxia type 1. Eur Neurol 46(1):43–48

    Article  CAS  PubMed  Google Scholar 

  • Calarge C, Andreasen NC, O’Leary DS (2003) Visualizing how one brain understands another: a PET study of theory of mind. Am J Psychiatry 160(11):1954–1964

    Article  PubMed  Google Scholar 

  • Castellanos FX, Giedd JN, Berquin PC et al (2001) Quantitative brain magnetic resonance imaging in girls with attention-deficit/hyperactivity disorder. Arch Gen Psychiatry 58(3):289–295

    Article  CAS  PubMed  Google Scholar 

  • Catsman-Berrevoets CE, Aarsen FK (2010) The spectrum of neurobehavioural deficits in the Posterior Fossa Syndrome in children after cerebellar tumour surgery. Cortex 46(7):933–946

    Article  PubMed  Google Scholar 

  • Catsman-Berrevoets CE, van Dongen HR, Zwetsloot CP (1992) Transient loss of speech followed by dysarthria after removal of posterior fossa tumour. Dev Med Child Neurol 34(12):1102–1109

    Article  CAS  PubMed  Google Scholar 

  • Chaves CJ, Caplan LR, Chung CS et al (1994) Cerebellar infarcts in the New England Medical Center posterior circulation stroke registry. Neurology 44(8):1385–1390

    Article  CAS  PubMed  Google Scholar 

  • Cher LM, Hochberg FH, Teruya J et al (1995) Therapy for paraneoplastic neurologic syndromes in six patients with protein A column immunoadsorption. Cancer 75(7):1678–1683

    Article  CAS  PubMed  Google Scholar 

  • Chheda M, Sherman J, Schmahmann JD (2002) Neurologic, psychiatric and cognitive manifestations in cerebellar agenesis. Neurology 58(Suppl 3):356

    Google Scholar 

  • Chirino-Pérez A, Marrufo-Meléndez OR, Muñoz-López JI et al (2021) Mapping the cerebellar cognitive affective syndrome in patients with chronic cerebellar strokes. Cerebellum. https://doi.org/10.1007/s12311-021-01290-3

  • Cooper IS, Riklan M, Amin I et al (1978) A long-term follow-up study of cerebellar stimulation for the control of epilepsy. In: Cooper IS (ed) Cerebellar stimulation in man. Raven Press, New York, pp 19–38

    Google Scholar 

  • Cooper FE, Grube M, Elsegood KJ et al (2010) The contribution of the cerebellum to cognition in Spinocerebellar Ataxia Type 6. Behav Neurol 23(1–2):3–15

    Article  PubMed  PubMed Central  Google Scholar 

  • Courchesne E, Yeung-Courchesne R, Press GA et al (1988) Hypoplasia of cerebellar vermal lobules VI and VII in autism. N Engl J Med 318(21):1349–1354

    Article  CAS  PubMed  Google Scholar 

  • D’Agata F, Caroppo P, Baudino B et al (2011) The recognition of facial emotions in spinocerebellar ataxia patients. Cerebellum 10(3):600–610

    Article  PubMed  Google Scholar 

  • D’Mello AM, Crocetti D, Mostofsky SH, Stoodley CJ (2015) Cerebellar gray matter and lobular volumes correlate with core autism symptoms. Neuroimage Clin 7:631–639

    Article  PubMed  PubMed Central  Google Scholar 

  • Demirtas-Tatlidede A, Freitas C, Cromer JR et al (2010) Safety and proof of principle study of cerebellar vermal theta burst stimulation in refractory schizophrenia. Schizophr Res 124(1–3):91–100

    Article  PubMed  PubMed Central  Google Scholar 

  • Devos D, Schraen-Maschke S, Vuillaume I et al (2001) Clinical features and genetic analysis of a new form of spinocerebellar ataxia. Neurology 56(2):234–238

    Article  CAS  PubMed  Google Scholar 

  • Dietze DD Jr, Mickle JP (1990) Cerebellar mutism after posterior fossa surgery. Pediatr Neurosurg 16(1):25–31. discussion 31

    Article  PubMed  Google Scholar 

  • Dimova PS, Bojinova VS, Milanov IG (2009) Transient mutism and pathologic laughter in the course of cerebellitis. Pediatr Neurol 41(1):49–52

    Article  PubMed  Google Scholar 

  • Dow RS (1974) Some novel concepts of cerebellar physiology. Mt Sinai J Med 41(1):103–119

    CAS  PubMed  Google Scholar 

  • Dow RS, Moruzzi G (1958) The physiology and pathology of the cerebellum. University of Minnesota Press, Minneapolis

    Google Scholar 

  • Doyon J, Song AW, Karni A et al (2002) Experience-dependent changes in cerebellar contributions to motor sequence learning. Proc Natl Acad Sci U S A 99(2):1017–1022

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Dreher JC, Grafman J (2002) The roles of the cerebellum and basal ganglia in timing and error prediction. Eur J Neurosci 16(8):1609–1619

    Article  PubMed  Google Scholar 

  • Dum RP, Strick PL (2003) An unfolded map of the cerebellar dentate nucleus and its projections to the cerebral cortex. J Neurophysiol 89(1):634–639

    Article  PubMed  Google Scholar 

  • Duncan GW, Parker SW, Fisher CM (1975) Acute cerebellar infarction in the PICA territory. Arch Neurol 32(6):364–368

    Article  CAS  PubMed  Google Scholar 

  • Exner C, Weniger G, Irle E (2004) Cerebellar lesions in the PICA but not SCA territory impair cognition. Neurology 63(11):2132–2135

    Article  PubMed  Google Scholar 

  • Fiez JA, Petersen SE, Cheney MK et al (1992) Impaired non-motor learning and error detection associated with cerebellar damage. A single case study. Brain 115(Pt 1):155–178

    Article  PubMed  Google Scholar 

  • Frick RB (1982) The ego and the vestibulocerebellar system: some theoretical perspectives. Psychoanal Q 51(1):93–122

    Article  CAS  PubMed  Google Scholar 

  • Frings M, Dimitrova A, Schorn CF et al (2006) Cerebellar involvement in verb generation: an fMRI study. Neurosci Lett 409(1):19–23

    Article  CAS  PubMed  Google Scholar 

  • Gambardella A, Annesi G, Bono F et al (1998) CAG repeat length and clinical features in three Italian families with spinocerebellar ataxia type 2 (SCA2): early impairment of Wisconsin Card Sorting Test and saccade velocity. J Neurol 245(10):647–652

    Article  CAS  PubMed  Google Scholar 

  • Gardner RJ, Coleman LT, Mitchell LA et al (2001) Near-total absence of the cerebellum. Neuropediatrics 32(2):62–68

    Article  CAS  PubMed  Google Scholar 

  • Garg S, Sinha VK, Tikka SK, Mishra P, Goyal N (2016) The efficacy of cerebellar vermal deep high frequency (theta range) repetitive transcranial magnetic stimulation (rTMS) in schizophrenia: a randomized rater blind- sham controlled study. Psychiatry Res 243:413–420

    Article  PubMed  Google Scholar 

  • Garrard P, Martin NH, Giunti P et al (2008) Cognitive and social cognitive functioning in spinocerebellar ataxia : a preliminary characterization. J Neurol 255(3):398–405

    Article  CAS  PubMed  Google Scholar 

  • Geschwind DH (1999) Focusing attention on cognitive impairment in spinocerebellar ataxia. Arch Neurol 56(1):20–22

    Article  CAS  PubMed  Google Scholar 

  • Gierga K, Schelhaas HJ, Brunt ER et al (2009) Spinocerebellar ataxia type 6 (SCA6): neurodegeneration goes beyond the known brain predilection sites. Neuropathol Appl Neurobiol 35(5):515–527

    Article  CAS  PubMed  Google Scholar 

  • Gondim FA, Parks BJ, Cruz-Flores S (2001) “Fou rire prodromique” as the presentation of pontine ischaemia secondary to vertebrobasilar stenosis. J Neurol Neurosurg Psychiatry 71(6):802–804

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Gorman MP (2010) Update on diagnosis, treatment, and prognosis in opsoclonus-myoclonus-ataxia syndrome. Curr Opin Pediatr 22(6):745–750

    Article  PubMed  Google Scholar 

  • Gottwald B, Wilde B, Mihajlovic Z et al (2004) Evidence for distinct cognitive deficits after focal cerebellar lesions. J Neurol Neurosurg Psychiatry 75(11):1524–1531

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Greve KW, Stanford MS, Sutton C et al (1999) Cognitive and emotional sequelae of cerebellar infarct: a case report. Arch Clin Neuropsychol 14(5):455–469

    CAS  PubMed  Google Scholar 

  • Grill J, Viguier D, Kieffer V et al (2004) Critical risk factors for intellectual impairment in children with posterior fossa tumors: the role of cerebellar damage. J Neurosurg 101(2 Suppl):152–158

    PubMed  Google Scholar 

  • Grodd W, Hulsmann E, Ackermann H (2005) Functional MRI localizing in the cerebellum. Neurosurg Clin N Am 16(1):77–99

    Article  PubMed  Google Scholar 

  • Gross-Tsur V, Ben-Bashat D, Shalev RS et al (2006) Evidence of a developmental cerebello-cerebral disorder. Neuropsychologia 44(12):2569–2572

    Article  PubMed  Google Scholar 

  • Gudrunardottir T, Morgan AT, Lux AL et al (2016) Consensus paper on post-operative pediatric cerebellar mutism syndrome: the Iceland Delphi results. Childs Nerv Syst 32(7):1195–1203

    Article  PubMed  Google Scholar 

  • Guell X, Hoche F, Schmahmann JD (2015) Metalinguistic deficits in patients with cerebellar dysfunction: empirical support for the dysmetria of thought theory. Cerebellum 14:50–58

    Article  PubMed  Google Scholar 

  • Guiang RL Jr, Ellington OB (1977) Acute pure vertiginous dysequilibrium in cerebellar infarction. Eur Neurol 16(1–6):11–15

    Article  PubMed  Google Scholar 

  • Habas C, Kamdar N, Nguyen D et al (2009) Distinct cerebellar contributions to intrinsic connectivity networks. J Neurosci 29(26):8586–8594

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Hartmann-von Monakow K, Akert K, Kunzle H (1981) Projection of precentral, premotor and prefrontal cortex to the basilar pontine grey and to nucleus reticularis tegmenti pontis in the monkey (Macaca fascicularis). Schweiz Arch Neurol Neurochir Psychiatr 129(2):189–208

    CAS  PubMed  Google Scholar 

  • Heath RG (1977) Modulation of emotion with a brain pacemaker. Treatment for intractable psychiatric illness. J Nerv Ment Dis 165(5):300–317

    Article  CAS  PubMed  Google Scholar 

  • Heath RG, Harper JW (1974) Ascending projections of the cerebellar fastigial nucleus to the hippocampus, amygdala, and other temporal lobe sites: evoked potential and histological studies in monkeys and cats. Exp Neurol 45(2):268–287

    Article  CAS  PubMed  Google Scholar 

  • Heath RG, Franklin DE, Shraberg D (1979) Gross pathology of the cerebellum in patients diagnosed and treated as functional psychiatric disorders. J Nerv Ment Dis 167(10):585–592

    Article  CAS  PubMed  Google Scholar 

  • Herman-Bert A, Stevanin G, Netter JC et al (2000) Mapping of spinocerebellar ataxia 13 to chromosome 19q13.3-q13.4 in a family with autosomal dominant cerebellar ataxia and mental retardation. Am J Hum Genet 67(1):229–235

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Hickey CL, Sherman JC, Goldenberg P et al (2018) Cerebellar cognitive affective syndrome: insights from Joubert syndrome. Cerebellum Ataxias 5:5

    Article  PubMed  PubMed Central  Google Scholar 

  • Hoche F, Guell X, Sherman JC, Vangel MG, Schmahmann JD (2016) Cerebellar contribution to social cognition. Cerebellum 15(6):732–743

    Article  PubMed  PubMed Central  Google Scholar 

  • Hoche F, Guell X, Vangel MG, Sherman JC, Schmahmann JD (2018) The cerebellar cognitive affective/Schmahmann syndrome scale. Brain 141(1):248–270

    Article  PubMed  Google Scholar 

  • Hoffmann M, Cases LB (2008) Etiology of frontal network syndromes in isolated subtentorial stroke. Behav Neurol 20(3):101–105

    Article  PubMed  Google Scholar 

  • Hoffmann M, Schmitt F (2004) Cognitive impairment in isolated subtentorial stroke. Acta Neurol Scand 109(1):14–24

    Article  PubMed  Google Scholar 

  • Hokkanen LS, Kauranen V, Roine RO et al (2006) Subtle cognitive deficits after cerebellar infarcts. Eur J Neurol 13(2):161–170

    Article  CAS  PubMed  Google Scholar 

  • Holmes G (1917) The symptoms of acute cerebellar injuries due to gunshot wounds. Brain 40:461–535

    Article  Google Scholar 

  • Holmes G (1939) The cerebellum of man (Hughlings Jackson memorial lecture). Brain 62:1–30

    Article  Google Scholar 

  • Hoppenbrouwers SS, Schutter DJ, Fitzgerald PB et al (2008) The role of the cerebellum in the pathophysiology and treatment of neuropsychiatric disorders: a review. Brain Res Rev 59(1):185–200

    Article  CAS  PubMed  Google Scholar 

  • Horton LC, Frosch MP, Schmahmann JD (2011) Spinocerebellar Ataxia type 7 (SCA7): a 13-year longitudinal study of clinical course, neuropathological features, and phenotype-genotype correlations. 63rd annual meeting of the American Academy of Neurology. Honolulu, HI

    Google Scholar 

  • Ishikawa K, Watanabe M, Yoshizawa K et al (1999) Clinical, neuropathological, and molecular study in two families with spinocerebellar ataxia type 6 (SCA6). J Neurol Neurosurg Psychiatry 67(1):86–89

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Ito M (1984) The cerebellum and neural control. Raven Press, New York

    Google Scholar 

  • Ito M (1997) Cerebellar microcomplexes. In: Schmahmann JD (ed) Cerebellum and cognition. Academic, San Diego. Int Rev Neurobiol 41:475–489

    Google Scholar 

  • Ivry R (1997) Cerebellar timing systems. In: Schmahmann JD (ed) Cerebellum and cognition. Academic, San Diego. Int Rev Neurobiol 41:555–573

    Google Scholar 

  • Jawaid A, Rauf MA, Usman U et al (2008) Post-infarct cerebellar cognitive affective syndrome: a case report. J Pak Med Assoc 58(7):415–417

    PubMed  Google Scholar 

  • Kalashnikova LA, Zueva YV, Pugacheva OV et al (2005) Cognitive impairments in cerebellar infarcts. Neurosci Behav Physiol 35(8):773–779

    Article  CAS  PubMed  Google Scholar 

  • Karatekin C, Lazareff JA, Asarnow RF (2000) Relevance of the cerebellar hemispheres for executive functions. Pediatr Neurol 22(2):106–112

    Article  CAS  PubMed  Google Scholar 

  • Kase CS, White JL, Joslyn JN et al (1985) Cerebellar infarction in the superior cerebellar artery distribution. Neurology 35(5):705–711

    Article  CAS  PubMed  Google Scholar 

  • Kelly RM, Strick PL (2003) Cerebellar loops with motor cortex and prefrontal cortex of a nonhuman primate. J Neurosci 23(23):8432–8444

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Kemper TL, Bauman M (1998) Neuropathology of infantile autism. J Neuropathol Exp Neurol 57(7):645–652

    Article  CAS  PubMed  Google Scholar 

  • Kingma A, Mooij JJ, Metzemaekers JD et al (1994) Transient mutism and speech disorders after posterior fossa surgery in children with brain tumours. Acta Neurochir 131(1–2):74–79

    Article  CAS  PubMed  Google Scholar 

  • Kish SJ, El-Awar M, Schut L et al (1988) Cognitive deficits in olivopontocerebellar atrophy: implications for the cholinergic hypothesis of Alzheimer’s dementia. Ann Neurol 24(2):200–206

    Article  CAS  PubMed  Google Scholar 

  • Koeppen AH (2011) Friedreich’s ataxia: pathology, pathogenesis, and molecular genetics. J Neurol Sci 303(1–2):1–12

    Google Scholar 

  • Koide R, Kobayashi S, Shimohata T et al (1999) A neurological disease caused by an expanded CAG trinucleotide repeat in the TATA-binding protein gene: a new polyglutamine disease? Hum Mol Genet 8(11):2047–2053

    Article  CAS  PubMed  Google Scholar 

  • Konarski JZ, McIntyre RS, Grupp LA et al (2005) Is the cerebellum relevant in the circuitry of neuropsychiatric disorders? J Psychiatry Neurosci 30(3):178–186

    PubMed  PubMed Central  Google Scholar 

  • Kossorotoff M, Gonin-Flambois C, Gitiaux C et al (2010) A cognitive and affective pattern in posterior fossa strokes in children: a case series. Dev Med Child Neurol 52(7):626–631

    Article  PubMed  Google Scholar 

  • Krienen FM, Buckner RL (2009) Segregated fronto-cerebellar circuits revealed by intrinsic functional connectivity. Cereb Cortex 19(10):2485–2497

    Article  PubMed  PubMed Central  Google Scholar 

  • Kumral E, Kisabay A, Atac C et al (2005) Spectrum of the posterior inferior cerebellar artery territory infarcts. Clinical-diffusion-weighted imaging correlates. Cerebrovasc Dis 20(5):370–380

    Article  PubMed  Google Scholar 

  • Lagarde J, Hantkie O, Hajjioui A et al (2009) Neuropsychological disorders induced by cerebellar damage. Ann Phys Rehabil Med 52(4):360–370

    Article  CAS  PubMed  Google Scholar 

  • Lee H, Sohn SI, Cho YW et al (2006) Cerebellar infarction presenting isolated vertigo: frequency and vascular topographical patterns. Neurology 67(7):1178–1183

    Article  CAS  PubMed  Google Scholar 

  • Leggio MG, Silveri MC, Petrosini L et al (2000) Phonological grouping is specifically affected in cerebellar patients: a verbal fluency study. J Neurol Neurosurg Psychiatry 69(1):102–106

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Leiner HC, Leiner AL, Dow RS (1986) Does the cerebellum contribute to mental skills? Behav Neurosci 100(4):443–454

    Article  CAS  PubMed  Google Scholar 

  • Levisohn L, Cronin-Golomb A, Schmahmann JD (2000) Neuropsychological consequences of cerebellar tumour resection in children: cerebellar cognitive affective syndrome in a paediatric population. Brain 123(Pt 5):1041–1050

    Article  PubMed  Google Scholar 

  • Lilja A, Hamalainen P, Kaitaranta E et al (2005) Cognitive impairment in spinocerebellar ataxia type 8. J Neurol Sci 237(1–2):31–38

    Article  PubMed  Google Scholar 

  • Limperopoulos C, Soul JS, Gauvreau K et al (2005a) Late gestation cerebellar growth is rapid and impeded by premature birth. Pediatrics 115(3):688–695

    Article  PubMed  Google Scholar 

  • Limperopoulos C, Soul JS, Haidar H et al (2005b) Impaired trophic interactions between the cerebellum and the cerebrum among preterm infants. Pediatrics 116(4):844–850

    Article  PubMed  Google Scholar 

  • Limperopoulos C, Bassan H, Gauvreau K et al (2007) Does cerebellar injury in premature infants contribute to the high prevalence of long-term cognitive, learning, and behavioral disability in survivors? Pediatrics 120(3):584–593

    Article  PubMed  Google Scholar 

  • Long J, Liang Y (1994) Cerebellar infarction. Analysis of 22 cases. Chin Med J 107(3):186–188

    CAS  PubMed  Google Scholar 

  • Maas RPPWM, Killaars S, van de Warrenburg BPC, Schutter DJLG (2021) The cerebellar cognitive affective syndrome scale reveals early neuropsychological deficits in SCA3 patients. J Neurol. https://doi.org/10.1007/s00415-021-10516-7

  • Maeshima S, Osawa A (2007) Stroke rehabilitation in a patient with cerebellar cognitive affective syndrome. Brain Inj 21(8):877–883

    Article  PubMed  Google Scholar 

  • Malm J, Kristensen B, Karlsson T et al (1998) Cognitive impairment in young adults with infratentorial infarcts. Neurology 51(2):433–440

    Article  CAS  PubMed  Google Scholar 

  • Manes F, Villamil AR, Ameriso S et al (2009) “Real life” executive deficits in patients with focal vascular lesions affecting the cerebellum. J Neurol Sci 283(1–2):95–98

    Article  PubMed  Google Scholar 

  • Mantovan MC, Martinuzzi A, Squarzanti F et al (2006) Exploring mental status in Friedreich’s ataxia: a combined neuropsychological, behavioral and neuroimaging study. Eur J Neurol 13(8):827–835

    Article  CAS  PubMed  Google Scholar 

  • Mariotti C, Alpini D, Fancellu R et al (2007) Spinocerebellar ataxia type 17 (SCA17): oculomotor phenotype and clinical characterization of 15 Italian patients. J Neurol 254(11):1538–1546

    Article  PubMed  Google Scholar 

  • Maryniak A, Roszkowski M (2005) Cognitive and affective disturbances in children after surgical treatment of cerebellar tumors. Neurol Neurochir Pol 39(3):202–206

    PubMed  Google Scholar 

  • Miall RC, Weir DJ, Wolpert DM et al (1993) Is the cerebellum a smith predictor? J Mot Behav 25(3):203–216

    Article  CAS  PubMed  Google Scholar 

  • Middleton FA, Strick PL (1994) Anatomical evidence for cerebellar and basal ganglia involvement in higher cognitive function. Science 266(5184):458–461

    Article  CAS  PubMed  Google Scholar 

  • Mier D, Lis S, Neuthe K et al (2010) The involvement of emotion recognition in affective theory of mind. Psychophysiology 47(6):1028–1039

    PubMed  Google Scholar 

  • Milandre L, Broca P, Sambuc R et al (1992) Les crises épileptiques au décours des accidents cérébrovasculaires: analyse clinique de 78 cas. Rev Neurol 148:767–772

    CAS  PubMed  Google Scholar 

  • Moberget T, Doan NT, Alnæs D et al (2018) Cerebellar volume and cerebellocerebral structural covariance in schizophrenia: a multisite mega-analysis of 983 patients and 1349 healthy controls. Mol Psychiatry 23(6):1512–1520

    Article  CAS  PubMed  Google Scholar 

  • Molinari M, Leggio MG, Silveri MC (1997) Verbal fluency and agrammatism. Int Rev Neurobiol 41:325–339

    Article  CAS  PubMed  Google Scholar 

  • Molinari M, Filippini V, Leggio MG (2002) Neuronal plasticity of interrelated cerebellar and cortical networks. Neuroscience 111(4):863–870

    Article  CAS  PubMed  Google Scholar 

  • Molinari M, Chiricozzi FR, Clausi S et al (2008) Cerebellum and detection of sequences, from perception to cognition. Cerebellum 7(4):611–615

    Article  PubMed  Google Scholar 

  • Mostofsky SH, Reiss AL, Lockhart P et al (1998) Evaluation of cerebellar size in attention-deficit hyperactivity disorder. J Child Neurol 13(9):434–439

    Article  CAS  PubMed  Google Scholar 

  • Moulton E, Elman I, Pendse G et al (2011) Aversion-related circuity in the cerebellum: responses to noxious heat and unpleasant images. J Neurosci 31(10):3795–3804

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Murakami N, Yoshida M, Hashizume Y et al (1989) A nosological study of a patient showing ataxia & lower motor neuron involvement. Rinsho Shinkeigaku 29(9):1116–1121

    CAS  PubMed  Google Scholar 

  • Naeije G, Rai M, Allaerts N, Sjogard M, De Tiège X, Pandolfo M (2020) Cerebellar cognitive disorder parallels cerebellar motor symptoms in Friedreich ataxia. Ann Clin Transl Neurol 7(6):1050–1054

    Article  PubMed  PubMed Central  Google Scholar 

  • Nakamura K, Jeong SY, Uchihara T et al (2001) SCA17, a novel autosomal dominant cerebellar ataxia caused by an expanded polyglutamine in TATA-binding protein. Hum Mol Genet 10(14):1441–1448

    Article  CAS  PubMed  Google Scholar 

  • Nashold BS Jr, Slaughter DG (1969) Effects of stimulating or destroying the deep cerebellar regions in man. J Neurosurg 31(2):172–186

    Article  PubMed  Google Scholar 

  • Neau JP, Arroyo-Anllo E, Bonnaud V et al (2000) Neuropsychological disturbances in cerebellar infarcts. Acta Neurol Scand 102(6):363–370

    Article  CAS  PubMed  Google Scholar 

  • Nixon PD, Passingham RE (2001) Predicting sensory events. The role of the cerebellum in motor learning. Exp Brain Res 138(2):251–257

    Article  CAS  PubMed  Google Scholar 

  • O’Reilly JX, Beckmann CF, Tomassini V et al (2010) Distinct and overlapping functional zones in the cerebellum defined by resting state functional connectivity. Cereb Cortex 20(4):953–965

    Article  PubMed  Google Scholar 

  • Ohara S, Iijima N, Hayashida K et al (2007) Autopsy case of opsoclonus-myoclonus-ataxia and cerebellar cognitive affective syndrome associated with small cell carcinoma of the lung. Mov Disord 22(9):1320–1324

    Article  PubMed  Google Scholar 

  • Olivito G, Lupo M, Iacobacci C et al (2018) Structural cerebellar correlates of cognitive functions in spinocerebellar ataxia type 2. J Neurol 265(3):597–606

    Article  CAS  PubMed  Google Scholar 

  • Oscarsson O (1965) Functional Organization of the Spino- and Cuneocerebellar Tracts. Physiol Rev 45:495–522

    Article  CAS  PubMed  Google Scholar 

  • Palmer SL, Hassall T, Evankovich K et al (2010) Neurocognitive outcome 12 months following cerebellar mutism syndrome in pediatric patients with medulloblastoma. Neuro-Oncology 12(12):1311–1317

    PubMed  PubMed Central  Google Scholar 

  • Parker KL, Kim YC, Kelley RM et al (2017) Delta-frequency stimulation of cerebellar projections can compensate for schizophrenia-related medial frontal dysfunction. Mol Psychiatry 22(5):647–655

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Parvizi J, Anderson SW, Martin CO et al (2001) Pathological laughter and crying: a link to the cerebellum. Brain 124(Pt 9):1708–1719

    Article  CAS  PubMed  Google Scholar 

  • Parvizi J, Joseph J, Press DZ et al (2007) Pathological laughter and crying in patients with multiple system atrophy-cerebellar type. Mov Disord 22(6):798–803

    Article  PubMed  Google Scholar 

  • Paulus KS, Magnano I, Conti M et al (2004) Pure post-stroke cerebellar cognitive affective syndrome: a case report. Neurol Sci 25(4):220–224

    Article  CAS  PubMed  Google Scholar 

  • Penn HE (2006) Neurobiological correlates of autism: a review of recent research. Child Neuropsychol 12(1):57–79

    Article  PubMed  Google Scholar 

  • Picard H, Amado I, Mouchet-Mages S et al (2008) The role of the cerebellum in schizophrenia: an update of clinical, cognitive, and functional evidences. Schizophr Bull 34(1):155–172

    Article  PubMed  Google Scholar 

  • Pollack IF, Polinko P, Albright AL et al (1995) Mutism and pseudobulbar symptoms after resection of posterior fossa tumors in children: incidence and pathophysiology. Neurosurgery 37(5):885–893

    Article  CAS  PubMed  Google Scholar 

  • Premack D, Woodruff G (1978) Does the chimpanzee have a theory of mind? Behav Brain Sci 1:515–526

    Article  Google Scholar 

  • Ramnani N, Behrens TE, Johansen-Berg H et al (2006) The evolution of prefrontal inputs to the cortico-pontine system: diffusion imaging evidence from Macaque monkeys and humans. Cereb Cortex 16(6):811–818

    Article  PubMed  Google Scholar 

  • Rapin I (1999) Autism in search of a home in the brain. Neurology 52(5):902–904

    Article  CAS  PubMed  Google Scholar 

  • Ravizza SM, McCormick CA, Schlerf JE et al (2006) Cerebellar damage produces selective deficits in verbal working memory. Brain 129(Pt 2):306–320

    Article  PubMed  Google Scholar 

  • Reis DJ, Doba N, Nathan MA (1973) Predatory attack, grooming, and consummatory behaviors evoked by electrical stimulation of cat cerebellar nuclei. Science 182(114):845–847

    Article  CAS  PubMed  Google Scholar 

  • Rekate HL, Grubb RL, Aram DM et al (1985) Muteness of cerebellar origin. Arch Neurol 42(7):697–698

    Article  CAS  PubMed  Google Scholar 

  • Richter S, Dimitrova A, Maschke M et al (2005) Degree of cerebellar ataxia correlates with three-dimensional MRI-based cerebellar volume in pure cerebellar degeneration. Eur Neurol 54(1):23–27

    Article  PubMed  Google Scholar 

  • Richter S, Aslan B, Gerwig M et al (2007) Patients with chronic focal cerebellar lesions show no cognitive abnormalities in a bedside test. Neurocase 13(1):25–36

    Article  PubMed  Google Scholar 

  • Riklan M, Marisak I, Cooper IS (1974) Psychological studies of chronic cerebellar stimulation in man. In: Cooper IS, Riklan M, Snider RS (eds) The cerebellum, epilepsy and behavior. Plenum Press, New York, pp 285–342

    Chapter  Google Scholar 

  • Ritvo ER, Freeman BJ, Scheibel AB et al (1986) Lower Purkinje cell counts in the cerebella of four autistic subjects: initial findings of the UCLA-NSAC Autopsy Research Report. Am J Psychiatry 143(7):862–866

    Article  CAS  PubMed  Google Scholar 

  • Riva D, Giorgi C (2000) The cerebellum contributes to higher functions during development: evidence from a series of children surgically treated for posterior fossa tumours. Brain 123(Pt 5):1051–1061

    Article  PubMed  Google Scholar 

  • Ronning C, Sundet K, Due-Tonnessen B et al (2005) Persistent cognitive dysfunction secondary to cerebellar injury in patients treated for posterior fossa tumors in childhood. Pediatr Neurosurg 41(1):15–21

    Article  PubMed  Google Scholar 

  • Sadeh M, Cohen I (2001) Transient loss of speech after removal of posterior fossa tumors – one aspect of a larger neuropsychological entity: the cerebellar cognitive affective syndrome. Pediatr Hematol Oncol 18(7):423–426

    Article  CAS  PubMed  Google Scholar 

  • Sasaki H, Kojima H, Yabe I et al (1998) Neuropathological and molecular studies of spinocerebellar ataxia type 6 (SCA6). Acta Neuropathol 95(2):199–204

    Article  CAS  PubMed  Google Scholar 

  • Schmahmann JD (1991) An emerging concept. The cerebellar contribution to higher function. Arch Neurol 48(11):1178–1187

    Article  CAS  PubMed  Google Scholar 

  • Schmahmann JD (1996) From movement to thought: anatomic substrates of the cerebellar contribution to cognitive processing. Hum Brain Mapp 4:174–198

    Article  CAS  PubMed  Google Scholar 

  • Schmahmann JD (1998) Dysmetria of thought. Clinical consequences of cerebellar dysfunction on cognition and affect. Trends Cogn Sci 2:362–370

    Article  CAS  PubMed  Google Scholar 

  • Schmahmann JD (2000a) Cerebellum and brainstem. In: Toga A, Mazziotta J (eds) Brain mapping: the systems. Academic, San Diego, pp 207–259

    Chapter  Google Scholar 

  • Schmahmann JD (2000b) The role of the cerebellum in affect and psychosis. J Neurolinguistics 13:189–214

    Article  Google Scholar 

  • Schmahmann JD (2001) The cerebellar cognitive affective syndrome: clinical correlations of the dysmetria of thought hypothesis. Int Rev Psychiatry 13:313–322

    Article  Google Scholar 

  • Schmahmann JD (2004) Disorders of the cerebellum: ataxia, dysmetria of thought, and the cerebellar cognitive affective syndrome. J Neuropsychiatr Clin Neurosci 16(3):367–378

    Article  Google Scholar 

  • Schmahmann JD (2007) Cerebellum and spinal cord – principles of development, anatomical organization, and functional relevance. In: Brice A, Pulst S (eds) Spinocerebellar degenerations: the ataxias and spastic paraplegias. Elsevier, New York, pp 1–60

    Google Scholar 

  • Schmahmann JD (2019) The cerebellum and cognition. Neurosci Lett 688:62–75

    Article  CAS  PubMed  Google Scholar 

  • Schmahmann JD (2020) Pediatric post-operative cerebellar mutism syndrome, cerebellar cognitive affective syndrome, and posterior fossa syndrome: historical review and proposed resolution to guide future study. Childs Nerv Syst 36(6):1205–1214

    Article  PubMed  Google Scholar 

  • Schmahmann JD, Pandya DN (1989) Anatomical investigation of projections to the basis pontis from posterior parietal association cortices in rhesus monkey. J Comp Neurol 289(1):53–73

    Article  CAS  PubMed  Google Scholar 

  • Schmahmann JD, Pandya DN (1991) Projections to the basis pontis from the superior temporal sulcus and superior temporal region in the rhesus monkey. J Comp Neurol 308(2):224–248

    Article  CAS  PubMed  Google Scholar 

  • Schmahmann JD, Pandya DN (1992) Course of the fiber pathways to pons from parasensory association areas in the rhesus monkey. J Comp Neurol 326(2):159–179

    Article  CAS  PubMed  Google Scholar 

  • Schmahmann JD, Pandya DN (1993) Prelunate, occipitotemporal, and parahippocampal projections to the basis pontis in rhesus monkey. J Comp Neurol 337(1):94–112

    Article  CAS  PubMed  Google Scholar 

  • Schmahmann JD, Pandya DN (1997a) Anatomic organization of the basilar pontine projections from prefrontal cortices in rhesus monkey. J Neurosci 17(1):438–458

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Schmahmann JD, Pandya DN (1997b) The cerebrocerebellar system. In: Schmahmann JD (ed) Cerebellum and cognition. Academic, San Diego. Int Rev Neurobiol 41:31–60

    Google Scholar 

  • Schmahmann JD, Pandya DN (2006) Fiber pathways of the brain. Oxford University Press, New York

    Book  Google Scholar 

  • Schmahmann JD, Pandya DN (2008) Disconnection syndromes of basal ganglia, thalamus, and cerebrocerebellar systems. Cortex 44(8):1037–1066

    Article  PubMed  PubMed Central  Google Scholar 

  • Schmahmann JD, Sherman JC (1997) Cerebellar cognitive affective syndrome. In: Schmahmann JD (ed) Cerebellum and cognition. Academic, San Diego. Int Rev Neurobiol 41:433–440

    Google Scholar 

  • Schmahmann JD, Sherman JC (1998) The cerebellar cognitive affective syndrome. Brain 121(Pt 4):561–579

    Article  PubMed  Google Scholar 

  • Schmahmann JD, Doyon J, Toga A et al (2000) MRI atlas of the human cerebellum. Academic, San Diego

    Google Scholar 

  • Schmahmann JD, Rosene DL, Pandya DN (2004a) Motor projections to the basis pontis in rhesus monkey. J Comp Neurol 478(3):248–268

    Article  PubMed  Google Scholar 

  • Schmahmann JD, Ko R, MacMore J (2004b) The human basis pontis: motor syndromes and topographic organization. Brain 127(Pt 6):1269–1291

    Article  PubMed  Google Scholar 

  • Schmahmann JD, Weilburg JB, Sherman JC (2007) The neuropsychiatry of the cerebellum – insights from the clinic. Cerebellum 6(3):254–267

    Article  PubMed  Google Scholar 

  • Schmahmann JD, Gardner R, MacMore J et al (2009a) Development of a brief ataxia rating scale (BARS) based on a modified form of the ICARS. Mov Disord 24(12):1820–1828

    Article  PubMed  PubMed Central  Google Scholar 

  • Schmahmann JD, MacMore J, Vangel M (2009b) Cerebellar stroke without motor deficit: clinical evidence for motor and non-motor domains within the human cerebellum. Neuroscience 162(3):852–861

    Article  CAS  PubMed  Google Scholar 

  • Schmahmann JD, Pierce S, MacMore J, L’Italien GJ (2021) Development and validation of a patient-reported outcome measure of Ataxia. Mov Disord. https://doi.org/10.1002/mds.28670

  • Schoch B, Dimitrova A, Gizewski ER et al (2006) Functional localization in the human cerebellum based on voxelwise statistical analysis: a study of 90 patients. NeuroImage 30(1):36–51

    Article  CAS  PubMed  Google Scholar 

  • Schweizer TA, Levine B, Rewilak D et al (2008) Rehabilitation of executive functioning after focal damage to the cerebellum. Neurorehabil Neural Repair 22(1):72–77

    Article  PubMed  Google Scholar 

  • Schweizer TA, Alexander MP, Susan Gillingham BA et al (2010) Lateralized cerebellar contributions to word generation: a phonemic and semantic fluency study. Behav Neurol 23(1–2):31–37

    Article  PubMed  PubMed Central  Google Scholar 

  • Scott RB, Stoodley CJ, Anslow P et al (2001) Lateralized cognitive deficits in children following cerebellar lesions. Dev Med Child Neurol 43(10):685–691

    Article  CAS  PubMed  Google Scholar 

  • Silveri MC, Leggio MG, Molinari M (1994) The cerebellum contributes to linguistic production: a case of agrammatic speech following a right cerebellar lesion. Neurology 44(11):2047–2050

    Article  CAS  PubMed  Google Scholar 

  • Snider RS (1950) Recent contributions to the anatomy and physiology of the cerebellum. Arch Neurol Psychiatr 64(2):196–219

    Article  CAS  Google Scholar 

  • Snider SR (1982) Cerebellar pathology in schizophrenia – cause or consequence? Neurosci Biobehav Rev 6(1):47–53

    Article  CAS  PubMed  Google Scholar 

  • Snider R, Eldred E (1951) Electro-anatomical studies on cerebro-cerebellar connections in the cat. J Comp Neurol 95(1):1–16

    Article  CAS  PubMed  Google Scholar 

  • Snider RS, Stowell A (1944) Receiving areas of the tactile, auditory, and visual systems in the cerebellum. J Neurophysiol 7:331–357

    Article  Google Scholar 

  • Sokolovsky N, Cook A, Hunt H et al (2010) A preliminary characterisation of cognition and social cognition in spinocerebellar ataxia types 2, 1, and 7. Behav Neurol 23(1–2):17–29

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Steinlin M, Zangger B, Boltshauser E (1998) Non-progressive congenital ataxia with or without cerebellar hypoplasia: a review of 34 subjects. Dev Med Child Neurol 40(3):148–154

    Article  CAS  PubMed  Google Scholar 

  • Steinlin M, Imfeld S, Zulauf P et al (2003) Neuropsychological long-term sequelae after posterior fossa tumour resection during childhood. Brain 126(Pt 9):1998–2008

    Article  PubMed  Google Scholar 

  • Stoodley CJ (2014) Distinct regions of the cerebellum show gray matter decreases in autism, ADHD, and developmental dyslexia. Front Syst Neurosci 8:92

    Article  PubMed  PubMed Central  Google Scholar 

  • Stoodley CJ, Schmahmann JD (2009a) The cerebellum and language: evidence from patients with cerebellar degeneration. Brain Lang 110(3):149–153

    Article  PubMed  Google Scholar 

  • Stoodley CJ, Schmahmann JD (2009b) Functional topography in the human cerebellum: a meta-analysis of neuroimaging studies. NeuroImage 44(2):489–501

    Article  PubMed  Google Scholar 

  • Stoodley CJ, Valera EM, Schmahmann JD (2010) An fMRI study of intra-individual functional topography in the human cerebellum. Behav Neurol 23(1–2):65–79

    Article  PubMed  PubMed Central  Google Scholar 

  • Stoodley CJ, MacMore JP, Makris N, Sherman JC, Schmahmann JD (2016) Location of lesion determines motor vs. cognitive consequences in patients with cerebellar stroke. NeuroImage Clin 12:765–775

    Article  PubMed  PubMed Central  Google Scholar 

  • Stoodley CJ, D’Mello AM, Ellegood J et al (2017) Altered cerebellar connectivity in autism and cerebellar-mediated rescue of autism-related behaviors in mice. Nat Neurosci 20:1744–1751

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Storey E, Forrest SM, Shaw JH et al (1999) Spinocerebellar ataxia type 2: clinical features of a pedigree displaying prominent frontal-executive dysfunction. Arch Neurol 56(1):43–50

    Article  CAS  PubMed  Google Scholar 

  • Strick PL, Dum RP, Fiez JA (2009) Cerebellum and nonmotor function. Annu Rev Neurosci 32:413–434

    Article  CAS  PubMed  Google Scholar 

  • Suenaga M, Kawai Y, Watanabe H et al (2008) Cognitive impairment in spinocerebellar ataxia type 6. J Neurol Neurosurg Psychiatry 79(5):496–499

    Article  CAS  PubMed  Google Scholar 

  • Sugihara I, Shinoda Y (2004) Molecular, topographic, and functional organization of the cerebellar cortex: a study with combined aldolase C and olivocerebellar labeling. J Neurosci 24(40):8771–8785

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Tavano A, Grasso R, Gagliardi C et al (2007) Disorders of cognitive and affective development in cerebellar malformations. Brain 130(Pt 10):2646–2660

    Article  PubMed  Google Scholar 

  • Tedesco AM, Chiricozzi FR, Clausi S et al (2011) The cerebellar cognitive profile. Brain 134(Pt 12):3672–3686

    Google Scholar 

  • Tei H, Sakamoto Y (1997) Pontine infarction due to basilar artery stenosis presenting as pathological laughter. Neuroradiology 39(3):190–191

    Article  CAS  PubMed  Google Scholar 

  • Terao S, Sobue G, Izumi M et al (1996) Infarction of superior cerebellar artery presenting as cerebellar symptoms. Stroke 27(9):1679–1681

    Article  CAS  PubMed  Google Scholar 

  • Thach WT (1987) Cerebellar inputs to motor cortex. CIBA Found Symp 132:201–220

    CAS  PubMed  Google Scholar 

  • Thach WT (1997) Context-response linkage. In: Schmahmann JD (ed) Cerebellum and cognition. Academic, San Diego. Int Rev Neurobiol 41:599–611

    Google Scholar 

  • Thieme A, Roeske S, Faber J et al (2020) Validation of a German version of the cerebellar cognitive affective/ Schmahmann Syndrome Scale: preliminary version and study protocol. Neurol Res Pract 2:39

    Article  PubMed  PubMed Central  Google Scholar 

  • Tiemeier H, Lenroot RK, Greenstein DK et al (2010) Cerebellum development during childhood and adolescence: a longitudinal morphometric MRI study. NeuroImage 49(1):63–70

    Article  PubMed  Google Scholar 

  • Trouillas P, Takayanagi T, Hallett M et al (1997) International Cooperative Ataxia Rating Scale for pharmacological assessment of the cerebellar syndrome. The Ataxia Neuropharmacology Committee of the World Federation of Neurology. J Neurol Sci 145(2):205–211

    Article  CAS  PubMed  Google Scholar 

  • Tsai PT, Hull C, Chu Y et al (2012) Autistic-like behaviour and cerebellar dysfunction in Purkinje cell Tsc1 mutant mice. Nature 488(7413):647–651

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Turkel SB, Shu Chen L, Nelson MD et al (2004) Case series: acute mood symptoms associated with posterior fossa lesions in children. J Neuropsychiatr Clin Neurosci 16(4):443–445

    Article  Google Scholar 

  • Turkel SB, Brumm VL, Mitchell WG et al (2006) Mood and behavioral dysfunction with opsoclonus-myoclonus ataxia. J Neuropsychiatr Clin Neurosci 18(2):239–241

    Article  Google Scholar 

  • Urban PP, Marx J, Hunsche S et al (2003) Cerebellar speech representation: lesion topography in dysarthria as derived from cerebellar ischemia and functional magnetic resonance imaging. Arch Neurol 60(7):965–972

    Article  PubMed  Google Scholar 

  • van Dongen HR, Catsman-Berrevoets CE, van Mourik M (1994) The syndrome of ‘cerebellar’ mutism and subsequent dysarthria. Neurology 44(11):2040–2046

    Article  PubMed  Google Scholar 

  • Van Overwalle F, Manto M, Cattaneo Z et al (2020) Consensus paper: cerebellum and social cognition. Cerebellum 19(6):833–868

    Article  PubMed  PubMed Central  Google Scholar 

  • Vaquero E, Gomez CM, Quintero EA et al (2008) Differential prefrontal-like deficit in children after cerebellar astrocytoma and medulloblastoma tumor. Behav Brain Funct 4:18

    Article  PubMed  PubMed Central  Google Scholar 

  • Vokaer M, Bier JC, Elincx S et al (2002) The cerebellum may be directly involved in cognitive functions. Neurology 58(6):967–970

    Article  CAS  PubMed  Google Scholar 

  • Voogd J (2004) Cerebellum and precerebellar nuclei. In: Paxinos G, Mai JK (eds) The human nervous system, 2nd edn. Elsevier Academic Press, Amsterdam, pp 321–392

    Chapter  Google Scholar 

  • Voogd J, Glickstein M (1998) The anatomy of the cerebellum. Trends Neurosci 21(9):370–375

    Article  CAS  PubMed  Google Scholar 

  • Wassmer E, Davies P, Whitehouse WP et al (2003) Clinical spectrum associated with cerebellar hypoplasia. Pediatr Neurol 28(5):347–351

    Article  PubMed  Google Scholar 

  • Watson PJ (1978) Nonmotor functions of the cerebellum. Psychol Bull 85(5):944–967

    Article  CAS  PubMed  Google Scholar 

  • Weilburg JB, Bear DM, Sachs G (1987) Three patients with concomitant panic attacks and seizure disorder: possible clues to the neurology of anxiety. Am J Psychiatry 144(8):1053–1056

    Article  CAS  PubMed  Google Scholar 

  • Wells EM, Walsh KS, Khademian ZP et al (2008) The cerebellar mutism syndrome and its relation to cerebellar cognitive function and the cerebellar cognitive affective disorder. Dev Disabil Res Rev 14(3):221–228

    Article  PubMed  Google Scholar 

  • White M, Lalonde R, Botez-Marquard T (2000) Neuropsychologic and neuropsychiatric characteristics of patients with Friedreich’s ataxia. Acta Neurol Scand 102(4):222–226

    Article  CAS  PubMed  Google Scholar 

  • Whiting BA, Barton RA (2003) The evolution of the cortico-cerebellar complex in primates: anatomical connections predict patterns of correlated evolution. J Hum Evol 44(1):3–10

    Article  CAS  PubMed  Google Scholar 

  • Williams RS, Hauser SL, Purpura DP et al (1980) Autism and mental retardation: neuropathologic studies performed in four retarded persons with autistic behavior. Arch Neurol 37(12):749–753

    Article  CAS  PubMed  Google Scholar 

  • Wisoff JH, Epstein FJ (1984) Pseudobulbar palsy after posterior fossa operation in children. Neurosurgery 15(5):707–709

    Article  CAS  PubMed  Google Scholar 

  • Wolf U, Rapoport MJ, Schweizer TA (2009) Evaluating the affective component of the cerebellar cognitive affective syndrome. J Neuropsychiatr Clin Neurosci 21(3):245–253

    Article  Google Scholar 

  • Wolff JE, Huttermann U, Askins MA (2007) Quantifying health status outcomes in pediatric medulloblastoma patients. Anticancer Res 27(1B):523–529

    PubMed  Google Scholar 

  • Wollmann T, Barroso J, Monton F et al (2002) Neuropsychological test performance of patients with Friedreich’s ataxia. J Clin Exp Neuropsychol 24(5):677–686

    Article  PubMed  Google Scholar 

  • Woolsey CN (1952) Summary of the papers on the cerebellum. Res Publ Assoc Res Nerv Ment Dis 30:334–336

    Google Scholar 

  • Zawacki TM, Grace J, Friedman JH et al (2002) Executive and emotional dysfunction in Machado-Joseph disease. Mov Disord 17(5):1004–1010

    Article  PubMed  Google Scholar 

  • Ziemus B, Baumann O, Luerding R et al (2007) Impaired working-memory after cerebellar infarcts paralleled by changes in BOLD signal of a cortico-cerebellar circuit. Neuropsychologia 45(9):2016–2024

    Article  CAS  PubMed  Google Scholar 

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Acknowledgments

Supported in part by the National Ataxia Foundation, the MINDlink foundation, and Mrs. Mary Jo Reston. The assistance of Jason MacMore is gratefully acknowledged.

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Authors and Affiliations

  1. Ataxia Center, Laboratory for Neuroanatomy and Cerebellar Neurobiology, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA

    Jeremy D. Schmahmann

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Editors and Affiliations

  1. CHU-Charleroi, University of Mons, Charleroi, Belgium

    Mario U. Manto

  2. Department of Neuroscience, The Scripps Research Institute, La Jolla, CA, USA

    Donna L. Gruol

  3. Ataxia Center, Laboratory for Neuroanatomy and Cerebellar Neurobiology, Department of Neurology Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA

    Jeremy D. Schmahmann

  4. Department of Integrative Physiology, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan

    Noriyuki Koibuchi

  5. Department of Pathology and Immunology, Baylor College of Medicine, Houston, TX, USA

    Roy V. Sillitoe

Section Editor information

  1. Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA

    Jeremy D. Schmahmann

  2. University of Mons, Mons, Belgium

    Mario Manto

  3. CHU-Charleroi, Charleroi, Belgium

    Mario Manto

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Schmahmann, J.D. (2022). The Cerebellar Cognitive Affective Syndrome and the Neuropsychiatry of the Cerebellum. In: Manto, M.U., Gruol, D.L., Schmahmann, J.D., Koibuchi, N., Sillitoe, R.V. (eds) Handbook of the Cerebellum and Cerebellar Disorders. Springer, Cham. https://doi.org/10.1007/978-3-030-23810-0_77

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