Literature DB >> 26105056

Consensus Paper: Revisiting the Symptoms and Signs of Cerebellar Syndrome.

Florian Bodranghien1, Amy Bastian2, Carlo Casali3, Mark Hallett4, Elan D Louis5, Mario Manto6, Peter Mariën7,8, Dennis A Nowak9,10, Jeremy D Schmahmann11, Mariano Serrao3,12, Katharina Marie Steiner13,14, Michael Strupp15, Caroline Tilikete16,17,18, Dagmar Timmann13,14, Kim van Dun8.   

Abstract

The cerebellum is involved in sensorimotor operations, cognitive tasks and affective processes. Here, we revisit the concept of the cerebellar syndrome in the light of recent advances in our understanding of cerebellar operations. The key symptoms and signs of cerebellar dysfunction, often grouped under the generic term of ataxia, are discussed. Vertigo, dizziness, and imbalance are associated with lesions of the vestibulo-cerebellar, vestibulo-spinal, or cerebellar ocular motor systems. The cerebellum plays a major role in the online to long-term control of eye movements (control of calibration, reduction of eye instability, maintenance of ocular alignment). Ocular instability, nystagmus, saccadic intrusions, impaired smooth pursuit, impaired vestibulo-ocular reflex (VOR), and ocular misalignment are at the core of oculomotor cerebellar deficits. As a motor speech disorder, ataxic dysarthria is highly suggestive of cerebellar pathology. Regarding motor control of limbs, hypotonia, a- or dysdiadochokinesia, dysmetria, grasping deficits and various tremor phenomenologies are observed in cerebellar disorders to varying degrees. There is clear evidence that the cerebellum participates in force perception and proprioceptive sense during active movements. Gait is staggering with a wide base, and tandem gait is very often impaired in cerebellar disorders. In terms of cognitive and affective operations, impairments are found in executive functions, visual-spatial processing, linguistic function, and affective regulation (Schmahmann's syndrome). Nonmotor linguistic deficits including disruption of articulatory and graphomotor planning, language dynamics, verbal fluency, phonological, and semantic word retrieval, expressive and receptive syntax, and various aspects of reading and writing may be impaired after cerebellar damage. The cerebellum is organized into (a) a primary sensorimotor region in the anterior lobe and adjacent part of lobule VI, (b) a second sensorimotor region in lobule VIII, and (c) cognitive and limbic regions located in the posterior lobe (lobule VI, lobule VIIA which includes crus I and crus II, and lobule VIIB). The limbic cerebellum is mainly represented in the posterior vermis. The cortico-ponto-cerebellar and cerebello-thalamo-cortical loops establish close functional connections between the cerebellum and the supratentorial motor, paralimbic and association cortices, and cerebellar symptoms are associated with a disruption of these loops.

Entities:  

Keywords:  A- or Dysdiadochokinesia; Affect; Ataxia; Cerebellar syndrome; Cerebellum; Cognition; Dysarthria; Dysmetria; Eye movements; Functional topography; Hypotonia; Language; Loops; Speech; Tremor

Mesh:

Year:  2016        PMID: 26105056      PMCID: PMC5565264          DOI: 10.1007/s12311-015-0687-3

Source DB:  PubMed          Journal:  Cerebellum        ISSN: 1473-4222            Impact factor:   3.847


  160 in total

Review 1.  An emerging concept. The cerebellar contribution to higher function.

Authors:  J D Schmahmann
Journal:  Arch Neurol       Date:  1991-11

2.  Gait pattern in inherited cerebellar ataxias.

Authors:  Mariano Serrao; Francesco Pierelli; Alberto Ranavolo; Francesco Draicchio; Carmela Conte; Romildo Don; Roberto Di Fabio; Margherita LeRose; Luca Padua; Giorgio Sandrini; Carlo Casali
Journal:  Cerebellum       Date:  2012-03       Impact factor: 3.847

3.  Impact of cerebellar lesion on syntactic processing evidenced by event-related potentials.

Authors:  M Adamaszek; K Strecker; C Kessler
Journal:  Neurosci Lett       Date:  2012-02-04       Impact factor: 3.046

4.  Intention tremor of the head in patients with essential tremor.

Authors:  Julie Leegwater-Kim; Elan D Louis; Seth L Pullman; Alicia G Floyd; Sarah Borden; Carol B Moskowitz; Lawrence S Honig
Journal:  Mov Disord       Date:  2006-11       Impact factor: 10.338

Review 5.  Cerebellar-induced apraxic agraphia: a review and three new cases.

Authors:  Hyo Jung De Smet; Sebastiaan Engelborghs; Philippe F Paquier; Peter P De Deyn; Peter Mariën
Journal:  Brain Cogn       Date:  2011-04-19       Impact factor: 2.310

6.  Metalinguistic deficits in patients with cerebellar dysfunction: empirical support for the dysmetria of thought theory.

Authors:  Xavier Guell; Franziska Hoche; Jeremy D Schmahmann
Journal:  Cerebellum       Date:  2015-02       Impact factor: 3.847

7.  Evidence for distinct cognitive deficits after focal cerebellar lesions.

Authors:  B Gottwald; B Wilde; Z Mihajlovic; H M Mehdorn
Journal:  J Neurol Neurosurg Psychiatry       Date:  2004-11       Impact factor: 10.154

8.  Cerebellum and ocular motor control.

Authors:  Amir Kheradmand; David S Zee
Journal:  Front Neurol       Date:  2011-09-01       Impact factor: 4.003

9.  Schmahmann's syndrome - identification of the third cornerstone of clinical ataxiology.

Authors:  Mario Manto; Peter Mariën
Journal:  Cerebellum Ataxias       Date:  2015-02-27

Review 10.  Mechanisms of cerebellar gait ataxia.

Authors:  Susanne M Morton; Amy J Bastian
Journal:  Cerebellum       Date:  2007       Impact factor: 3.648

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  82 in total

1.  The cerebellum does more than sensory prediction error-based learning in sensorimotor adaptation tasks.

Authors:  Peter A Butcher; Richard B Ivry; Sheng-Han Kuo; David Rydz; John W Krakauer; Jordan A Taylor
Journal:  J Neurophysiol       Date:  2017-06-21       Impact factor: 2.714

2.  Tremor Distribution and the Variable Clinical Presentation of Essential Tremor.

Authors:  Matteo Bologna; Isabella Berardelli; Giulia Paparella; Gina Ferrazzano; Luca Angelini; Patrizia Giustini; Danilo Alunni-Fegatelli; Alfredo Berardelli
Journal:  Cerebellum       Date:  2019-10       Impact factor: 3.847

Review 3.  Clinical presentations of Wilson disease.

Authors:  Samuel Shribman; Thomas T Warner; James S Dooley
Journal:  Ann Transl Med       Date:  2019-04

Review 4.  Cerebellum-from J. E. Purkyně up to Contemporary Research.

Authors:  František Vožeh
Journal:  Cerebellum       Date:  2017-06       Impact factor: 3.847

Review 5.  Cerebellar Cortex as a Therapeutic Target for Neurostimulation.

Authors:  Kim van Dun; Hiroshi Mitoma; Mario Manto
Journal:  Cerebellum       Date:  2018-12       Impact factor: 3.847

6.  The Working Life of People with Degenerative Cerebellar Ataxia.

Authors:  A Ranavolo; M Serrao; T Varrecchia; C Casali; A Filla; A Roca; A Silvetti; C Marcotulli; B M Rondinone; S Iavicoli; F Draicchio
Journal:  Cerebellum       Date:  2019-10       Impact factor: 3.847

7.  Neurocognitive Characterization of an SCA28 Family Caused by a Novel AFG3L2 Gene Mutation.

Authors:  Laszlo Szpisjak; Viola L Nemeth; Noemi Szepfalusi; Denes Zadori; Zoltan Maroti; Tibor Kalmar; Laszlo Vecsei; Peter Klivenyi
Journal:  Cerebellum       Date:  2017-12       Impact factor: 3.847

8.  Progression of Gait Ataxia in Patients with Degenerative Cerebellar Disorders: a 4-Year Follow-Up Study.

Authors:  Mariano Serrao; Giorgia Chini; Carlo Casali; Carmela Conte; Martina Rinaldi; Alberto Ranavolo; Christian Marcotulli; Luca Leonardi; Gaia Fragiotta; Fabiano Bini; Gianluca Coppola; Francesco Pierelli
Journal:  Cerebellum       Date:  2017-06       Impact factor: 3.847

Review 9.  Recent Advances in Cerebellar Ischemic Stroke Syndromes Causing Vertigo and Hearing Loss.

Authors:  Hyun-Ah Kim; Hyon-Ah Yi; Hyung Lee
Journal:  Cerebellum       Date:  2016-12       Impact factor: 3.847

Review 10.  Neurological disorders of gait, balance and posture: a sign-based approach.

Authors:  Jorik Nonnekes; Rianne J M Goselink; Evžen Růžička; Alfonso Fasano; John G Nutt; Bastiaan R Bloem
Journal:  Nat Rev Neurol       Date:  2018-01-29       Impact factor: 42.937

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