Chapter 9 - Cerebellar motor syndrome from children to the elderly
Introduction
This chapter is devoted to the cerebellar motor syndrome (CMS). In the field of clinical ataxiology, CMS is a major source of disabilities in both children and adults (Holmes, 1917; Musselman et al., 2014; Manto and Mariën, 2015). CMS is typically associated with errors in the metrics of voluntary movements. The lack of coordination in voluntary movements resulting in jerky movement is one of the signatures of a cerebellar lesion (Holmes, 1917, Holmes, 1939). Symptoms and motor signs gather speech deficits, impairments of limb movements, and abnormalities of posture/gait. The vestibulocerebellar syndrome (CMS) and Schmahmann syndrome (cerebellar cognitive affective syndrome) represent the two other cornerstones of clinical ataxiology and will not be discussed here (Schmahmann and Sherman, 1998; Manto and Mariën, 2015).
Section snippets
Anatomy and physiology of the motor cerebellum
Amongst the 10 lobules of the cerebellum, lobules I–V (anterior lobe) and lobules VI and VIII (from the posterior lobe) are considered as mainly sensorimotor. Phylogenetic studies have led to the subdivision of the cerebellum into: (1) the archicerebellum, corresponding to the flocculonodular lobe (vestibulocerebellum); (2) the paleocerebellum (or spinocerebellum), which receives projections from the spinal cord via the spinocerebellar pathways; and (3) the neocerebellum, whose growth in
General rules underlying the CMS
In many cerebellar ataxias, multiple mechanisms leading to motor deficits are often combined at various degrees and explain the timecourse of symptoms (Gilman et al., 1981): reduction in blood flow (in case of stroke), edema (following a trauma), invasion of cerebellar parenchyma (especially by a tumor), inflammatory response (for instance, during cerebellitis), immune response (during an immune-mediated cerebellar ataxia).
Edema in the posterior fossa is a risk factor for obstructive
Motor ataxia: a historic perspective
The general term of ataxia was defined by Garcin (1969) as “a disturbance of coordination which, quite independently of any motor weakness, alters the direction and extent of voluntary movement and impairs the sustained voluntary or reflex muscle contractions necessary for maintaining posture and equilibrium.”
Historically, three scientists have strongly influenced our current appraisal of the deficits of limb movements in cerebellar patients (Manto, 2002):
- 1.
Luciani (1891) initially reported three
Control of speech
From the anatomic perspective, the hemispheric lobule VI is particularly important because it includes the lip/tongue area of a sensorimotor homunculus (Ziegler, 2016). In addition, a tongue area is also located in lobule VIII. Lobule VI receives somatosensory afferent informations from oral and facial muscles via the inferior cerebellar peduncles (Stoodley and Schmahmann, 2010). This input conveys sensory information on the sensory state of speech organs. The cerebellar hemispheres receive
Control of limb movements
Ataxia of limbs includes to varying degrees dysmetria (hypermetria: overshoot; hypometria: undershoot), dysdiadochokinesia, cerebellar tremor (action tremor, postural tremor, kinetic tremor), isometrataxia, disorders of muscle tone (both hypotonia and cerebellar fits), and impaired check and rebound.
Writing
Handwriting is irregular in cerebellar ataxias. Letters are unequal in size and are irregularly spaced (Holmes, 1917). Some patients exhibit megalographia, which is abnormally large handwriting (Frings et al., 2010). Initially, mean letter height may appear normal but repeated writing of a similar sentence is associated with an increase in the size of letters. Larger handwriting has been observed in adults with focal cerebellar disorders, in adolescents with attention deficit-hyperactivity
Myoclonus
Myoclonus consists of brief (20–200-ms) contractions (positive myoclonus) or brief cessation of muscle tone (negative myoclonus). Myoclonic jerks can be localized or generalized.
Opsoclonus-myoclonus syndrome (OMS; also called “dancing-eye syndrome” due to multidirectional conjugate eye movements) is characterized by ataxia, myoclonic jerks, and opsoclonus (Pang et al., 2009). OMS occurs typically between 12 months and 3 years of age and is associated with cerebellar atrophy, especially at the
Posture and gait
Overall, children show less efficiency for the control of posture as compared to adults. Physiologically, the upright postural sway decreases markedly from the age of 3 to 5 years, followed by a slower decline after the age of 6 years (Usui et al., 1995). In addition, under the age of 10 postural sway is higher in boys than in girls.
Both children and adults use visual, vestibular, and proprioceptive information to maintain body posture as stable as possible, but the respective contributions of
Learning of complex motor skills
Delayed learning of complex motor skills may be a prominent feature in children with cerebellar disorders. This is probably related to specific pathophysiologic mechanisms in children. In particular, motor learning is distinct in children and in adults (Patrick et al., 2014). Learning is error-driven. Prior experience and not the size of the error improves motor learning in young children. Unexpectedly, children show an immaturity in terms of motor adaptation. Adaptation of the center of
The motor phenotype in ataxic cerebral palsy
Cerebral palsy, one of the most prevalent childhood disorders which is commonly attributed to perinatal asphyxia, impacts on development and motor skills (Paneth, 2008). The motor phenotype is classically divided into spastic (increased muscle tone), dyskinetic (athetosis, dystonia), and ataxic. The ataxic phenotype accounts for only about 2% of cases (McHale et al., 2000). Motor repercussions are considerable in terms of balance and daily life activities (Grecco et al., 2017). Gait is ataxic,
CMS corresponds to pure cerebellar lesions
The CMS reported in this chapter refers to pure cerebellar lesions. However, ataxic patients may suffer from a cerebellar-plus syndrome, manifesting especially with combinations of CMS with pyramidal, extrapyramidal, sensory, or autonomic deficits. This will impact on the motor phenotype. In children, numerous genetic and developmental disorders affect simultaneously the cerebellum and extracerebellar structures, especially the brainstem. In adults, MSA and ataxic hemiparesis are two classic
Rating CMS in children and in adults
The importance of an accurate clinical quantification of CMS for the follow-up of patients is obvious. Reliable scales are available to quantify motor ataxia. Scale for the Assessment and Rating of Ataxia (SARA) is an example (Schmitz-Hübsch et al., 2006). The scale takes into account speech (0–6 points), limb movements (0–16 points), and sitting/stance/gait (0–18 points). Global score varies from 0 (no motor ataxia) to 40 (most severe ataxia). In developing children, both SARA scores and
The prevailing hypothesis to explain CMS: disruption of internal models
In numerous tasks of daily life, an integration of spatial and temporal information in the millisecond range is required (Broersen et al., 2016). The capacity of the brain to generate predictions based on spatiotemporal cues is now considered as a critical phenomenon to perform movements with the correct timing and to perform adequate perceptual judgments. Several structures in the brain cooperate to plan and execute accurate spatial and temporal motor responses, especially the cerebellum and
Acknowledgments
Mario Manto is supported by the FNRS-Belgium and the Fonds Erasme.
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Cited by (22)
Neurophysiology of cerebellar ataxias and gait disorders
2023, Clinical Neurophysiology PracticeThe underpinnings of cerebellar ataxias
2022, Clinical Neurophysiology PracticeCitation Excerpt :The tremor induced by cooling the nucleus interpositus is more dependent on proprioceptive feedback than visual information (Flament et al., 1984). When the animal attempts to reach food, a 3 to 5 Hz action tremor is observed, suggesting that a key-function of the interpositus muscle is to damp oscillations of the limb (Manto, 2018). Kinetic tremor is characterized by oscillations between 2 and 7 Hz.
REAC neurobiological treatments in acute post-traumatic knee medial collateral ligament lesion
2020, HeliyonCitation Excerpt :Beyond the signs described above, referable to the trauma, all the subjects presented a disorder of the neuromotor control of the lower limbs, called functional dysmetria. Dysmetria, sometimes confused with heterometry, literally different measure, is an altered execution of voluntary movements, which can occur as a result of dysfunctions or lesions of the cerebellum and/or spinal cord [37]. It is opportune to remember that the dysmetria, in addition to involving the movement also involves thought and emotion [38] (emotional, affective, cognitive and relational functions).
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