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PubMed2001

High curvature and jerk analyses of arm ataxia.

We investigated high curvature analysis (HCA) and integrated absolute jerk (IAJ) for differrentiating healthy and cerebellopathy (CB) patients performing pointing tasks. Seventeen CB patients and seventeen healthy controls were required to move a pointer at their preferred pace between two 50.8 cm laterally spaced targets while standing with theirarm extended in front of their body. HCA was used to quantify the frequency of sharp turns in the horizontal-plane (anterior-posterior and medio-lateral) velocity trajectory of the hand-held pointer. IAJ was assesssed by integration of absolute jerk (second time derivative of velocity) time histories in the anterior-posterior and medio-lateral directions. HCA scores and IAJ scores were then compared between CB patients and healthy controls; for both analyses, higher scores indicateless smooth movements. We hypothesized that CB patients would have less smooth movement trajectories than healthy controls due to upper extremity ataxia asssociated with cerebellar disease and degeneration. We found that CB patients had higher HCA scores than healthy controls (P = 0.014). Although CB patients had higher IAJ scores in both anterior-posterior (P = 0.060)and medio-lateral (P = 0.231) directions compared to the healthy controls, the differences were not significant. The difference in sensitivity between the HCA andthe IAJ analysis might be explained by primitive neural activation commands, ubiquitous though only evident with some cerebellar dysfunctions, which produce submovements which are themselves minimal jerk curves. We conclude that HCA may be a useful tool for quantifying upper extremity ataxia in CB patients performing a repeated pointing task.

Adult↗
PubMed1990

The finger-tapping test. A quantitative analysis.

A quantitative analysis of the so-called finger-tapping test was performed on 111 normal subjects. Quantitative analysis was also performed on 17 patients with cerebellar diseases, 14 with parkinsonism, and 14 with hemiparesis. All analyses were performed in a simple fashion using an 8-bit microcomputer fed through an electrocardiographic apparatus. The results in normal subjects were as follows: (1) tapping frequency lowered with advancing age; (2) men tapped faster than women; and (3) tapping with the dominant finger was faster than tapping with the nondominant finger in normal subjects. Tapping frequency can distinguish patients with motor dysfunctions of cerebellar, basal ganglia, and cerebral origins from normal subjects. Only the time-sequential histograms of tapping intervals could distinguish the motor dysfunctions studied.

Adult↗
PubMed2002

Impaired vertical phoria adaptation in patients with cerebellar dysfunction.

PURPOSE: To determine whether phoria adaptation to a vertical prism disparity is altered in patients with cerebellar dysfunction. METHODS: With a computer-aided haploscope, adaptive responses of fusion-free eye position to a 10- or 30-minute period was measured in subjects wearing a 3-prism diopter vertical prism over one eye. Thirteen patients with well-documented cerebellar diseases who did not have manifest ocular misalignment or limited versional eye movement and age-matched healthy subjects participated. RESULTS: The mean +/- SD percentage of vertical phoria adaptation was 13% +/- 22% and 20% +/- 16% for the 10- and 30-minute adaptations, respectively. These levels were significantly smaller than the respective ones in the age-matched control group (P < 0.001, repeated measures MANOVA). Seven (54%) of 13 patients, including two with genetically confirmed pure cerebellar lesions (spinocerebellar ataxia type 6), showed markedly reduced responses to both the 10- and 30-minute adaptations. In all three patients with acute cerebellar ataxia, the adaptive response was improved at the same time as remission of cerebellum-associated neurologic deficits. CONCLUSIONS: Phoria adaptation to vertical binocular disparity is frequently impaired in patients with cerebellar dysfunction. These results bolster the hypothesis that phoria adaptation is a cerebellar-dependent response.

Adaptation, Ocular↗
PubMed2004

A model of cerebellum stabilized and scheduled hybrid long-loop control of upright balance.

A recurrent integrator proportional integral derivative (PID) model that has been used to account for cerebrocerebellar stabilization and scaling of transcortical proprioceptive feedback in the control of horizontal planar arm movements has been augmented with long-loop force feedback and gainscheduling to describe the control of human upright balance. The cerebellar component of the controller is represented by two sets of gains that each provide linear scaling of same-joint and interjoint long-loop stretch responses between ankle, knee, and hip. The cerebral component of the model includes a single set of same-joint linear force feedback gains. Responses to platform translations of a three-segment body model operating under this hybrid proprioception and force-based long-loop control were simulated. With low-velocity platform disturbances, "ankle-strategy"-type postural recovery kinematics and electromyogram (EMG) patterns were generated using the first set of cerebeller control gains. With faster disturbances, balance was maintained by including the second set of gains cerebellar control gains that yielded "mixed ankle-hip strategy"-type kinematics and EMG patterns. The addition of small amounts of simulated muscular coactivation improved the fit to certain human datasets. It is proposed that the cerebellum switches control gainsets as a function of sensed body kinematic state. Reduction of cerebellar gains with a compensatory increase in muscular stiffness yielded posture recovery with abnormal motions consistent with those found in cerebellar disease. The model demonstrates that stabilized hybrid long-loop feedback with scheduling of linear gains may afford realistic balance control in the absence of explicit internal dynamics models and suggests that the cerebellum and cerebral cortex may contribute to balance control by such a mechanism.

Biomechanical Phenomena↗
PubMed1997

Therapeutic and research implications.

Investigations into the relationship between the cerebellum and nonmotor processing have produced a substantial body of evidence which seems to require a revision of accepted notions about the functional role of the cerebellum. This chapter presents a perspective on the contemporary and possible future therapeutic and research implications of these findings. These include the need for patients and their families to know of the behavioral consequences of cerebellar disease processes; potential approaches for improvement through rehabilitation therapies; and future treatment strategies, such as electrical stimulation of the cerebellum and psychosurgical approaches applied to the cerebellum. In addition, some areas of basic science investigation that could prove informative in understanding this relationship are addressed. It will be important to obtain a more complete characterization of the anatomy, physiology, and functional topography of the cerebellum in humans and in animal models, and a greater understanding of the clinical consequences of cerebellar lesions.

Animals↗
PubMed1997

Classical conditioning.

Evidence has amassed from research in humans indicating that the cerebellar circuitry serving as the substrate for eyeblink classical conditioning is similar to that in nonhuman primates. In patients with bilateral cerebellar lesions or neurodegenerative cerebellar disease, few conditioned eyeblink responses are produced with either the ipsilesional or the contralesional eye. Cerebellar patients with lateralized lesions, like rabbits with experimentally produced unilateral cerebellar lesions, produce relatively normal conditioned responses (CRs) with the contralesional eye and few or no CRs with the ipsilesional eye. Age-related deficits in eyeblink classical conditioning appear in humans and rabbits in middle age. In normal aging in many species, including humans, there is Purkinje cell loss in cerebellar cortex. In rabbits, the Purkinje cell number correlates highly with the rate of learning, regardless of age. Positron emission tomography imaging of normal young adults during eyeblink conditioning reveals changes in activity in the cerebellum. Timed interval tapping, a task that assesses cerebellar function, also predicts performance on eyeblink conditioning. In dual-task conditions involving simultaneous performance of eyeblink conditioning and timed interval tapping, eyeblink conditioning is impaired. Investigations of patients with lesions or neurodegenerative disease not involving the cerebellum demonstrate that acquisition of CRs is possible, although prolonged in the case of hippocampal cholinergic disruption. Evidence to date suggests that the human analogue of the rabbit interpositus nucleus, the globose nucleus, is essential for the production of the conditioned eyeblink response and that cerebellar cortical Purkinje cells play a role in normal acquisition.

Aging↗
PubMedDate not supplied

What can acquired nystagmus tell us about congenital forms of nystagmus?

For several forms of acquired nystagmus, animal models exist, mathematical hypotheses have been proposed, and treatments are available. What insights could acquired nystagmus provide for congenital forms of nystagmus? Acquired periodic alternating nystagmus (PAN) is caused by instability of the velocity storage mechanism for vestibular eye movements; an adaptive mechanism produces the oscillations that have a period of about 4 minutes. Surprisingly, the ability of individuals with congenital forms of nystagmus to adapt their eye movements to new visual demands has received little study. Acquired pendular nystagmus (APN) may arise from instability in the neural integrator for eye movements; identification of the neurotransmitters contributing to normal gaze holding made it possible to identify candidate drugs for treatment of APN. Similar knowledge of the biology underlying of congenital forms of nystagmus might similarly suggest effective drugs. Downbeat nystagmus (DBN) is caused by cerebellar disease, which includes structural lesions affecting the flocculus and paraflocculus, and calcium channelopathies, such as episodic ataxia type 2 (EA2), for which a mouse model and effective treatment is available. Since some congenital forms of nystagmus are genetic in origin, then the possibility arises that they may be caused by a channelopathy, a hypothesis that suggests novel drugs for evaluation in randomized controlled trials.

Animals↗
PubMed2000

Classically conditioned withdrawal reflex in cerebellar patients. 1. Impaired conditioned responses.

The role of the cerebellum in the classically conditioned, human lower-limb-withdrawal reflex was studied in ten patients with pure cerebellar diseases (CBL), ten patients showing additional extracerebellar symptoms (CBL+), and in 11 sex- and age-matched normal controls (CTRL). Where conditioning was successful, the electrically evoked, unconditioned response was preceded by a tone-conditioned response (CR). CR incidence was variable, with best results in the CTRL, significantly less in CBL, and lowest in CBL+. Although CRs could be established in subjects in all groups, a continuous increase in the CR incidence in the course of the recording session was observed primarily in CTRL. In CBL and CBL+, such a characteristic reflex acquisition was rather the exception. CR onsets in CBL were within the range of those in CTRL, but CR amplitude was significantly lower in CBL. Cerebellar patients with circumscribed lesions behaved differently in our motor-learning paradigm, depending on the lesion site. Patients suffering from pathology of the posterior inferior cerebellum showed a mean CR incidence within the lower range of CTRL. In contrast, if the anterior and superior cerebellum was affected, few or even no CRs were observed. Our findings thus provide evidence that the human cerebellum is required for the acquisition and the retention of this specific conditioned limb-withdrawal reflex. In particular, anterior and superior parts of the cerebellum appear to be involved. Thus, an expansion of the current concept of clinically based, functional compartmentalization is suggested, such that anterior and superior cerebellar regions must be intact to establish plastic changes required for the acquisition of the conditioned withdrawal response.

Adult↗
PubMed1996

Dietary beta-alanine results in taurine depletion and cerebellar damage in adult cats.

We have used the taurine analogue, beta-alanine, to perturb the taurine concentrations in taurine-supplemented and taurine-deprived adult cats. By using 5% beta-alanine in the drinking water for 20 weeks, both groups of cats had greatly reduced brain taurine concentrations. Taurine-supplemented cat brain accumulated relatively small amounts of beta-alanine whereas taurine-deprived cats accumulated large amounts of beta-alanine. The cerebellum of cats treated with beta-alanine had a number of pathological changes compared with similar cats drinking water alone. The changes were more severe in the taurine-deprived cats, and included reduced numbers of granule and Purkinje cells, with many of those remaining appearing pyknotic and dying. Long swollen fibers were seen in the white matter, resembling Rosenthal fibers described in some human cerebellar diseases. There was also prominent gliosis. Using antibodies to beta-alanine and taurine, beta-alanine was localized in Purkinje cell soma and dendrites, in Golgi II cells, and in some granule cells, especially in taurine-deprived cats treated with beta-alanine. Taurine appears to have been virtually eliminated from Purkinje and granule cells, and concentrated in Golgi II cells and glia. We conclude that beta-alanine is responsible for these neurotoxic pathological changes.

Administration, Oral↗
PubMed2001

The gait disorder of advanced essential tremor.

Gait disturbances of patients with essential tremor (ET) have been described anecdotally, but have never been investigated quantitatively. Recent studies provided evidence for a cerebellar-like hand tremor in some patients with ET. Therefore, we designed a study to assess cerebellar-like abnormalities of leg function. Twenty-five patients with ET, eight patients with cerebellar diseases (CD) and 21 age-matched healthy subjects were studied for their normal and tandem gait using a three-dimensional gait analysis system. During normal walking, CD and ET patients showed only slight abnormalities. However, ET patients exhibited abnormalities in tandem gait with an increased number of mis-steps and a broad-based, ataxic and dysmetric gait which was indistinguishable from the findings in CD. When ET patients were separated into groups of those with or without intention tremor of the hands, the gait disorder was found to be much more pronounced in the intention tremor group. Patients with this gait disorder were more severely disturbed in their activities of daily living, and suffer from an advanced stage of ET. The present results quantitatively describe a gait disturbance in advanced ET which affects tandem gait, but leaves normal gait almost unaffected. This is strong evidence for a cerebellar-like disturbance in ET.

Adult↗
PubMed2002

Medulloblastoma or cerebellar dysplastic gangliocytoma (Lhermitte-Duclos disease)?

Dysplastic cerebellar gangliocytoma is a rare benign tumor associated with specific neuroimaging findings of abnormal laminated or folial pattern in the posterior fossa. Some authors thus proposed that it could be diagnosed by neuroimaging studies alone. We encountered a patient with medulloblastoma in which the neuroimaging findings mimicked those of dysplastic gangliocytoma. In patients with a posterior fossa tumor suggestive of a dysplastic gangliocytoma on neuroimaging studies, a pathologic confirmation is necessary.

Cerebellum↗
PubMed2004

On the role of the cerebellum in exploiting temporal contingencies:; evidence from response times and preparatory EEG potentials in patients with cerebellar atrophy.

Patients with degenerative cerebellar disease were compared to healthy controls in their ability to adapt behaviour to temporal contingencies, both according to instructions and according to acquired experience. Participants had to press the cued key whenever the inside of a clock face changed its colour, which could occur when the pointer, rotating once every 4s, was at "10h" or at "12h" or at "2h". Probabilities varied between blocks at which of these three time points the colour change occurred, with participants being instructed accordingly. Response times correlated intraindividually with these instructed "a priori" probabilities in control participants only. Subjectively, at any moment, probabilities of occurrence depend on whether the imperative colour change had occurred before, thus may be better described by conditional ("a posteriori") probabilities. Indeed, when response times were correlated to a posteriori rather than a priori probabilities, correlations increased in both groups equally from their different a priori levels. The amplitudes of preparatory EEG negativity before responding tended to obey to the same relationships, suggesting that the difference between groups was not due to pure motor impairment. Thus, these data suggest that patients with cerebellar atrophy are more impaired in implementing and using task-relevant information in a top-down manner than in learning to modify task-relevant contingencies.

Adult↗
PubMed1991

Direction and amplitude precuing has no effect on automatic posture responses.

Automatic postural responses of leg muscles to the sudden displacement of standing support were investigated under four different conditions of information given to subjects in advance. Results from three groups of subjects were compared: 6 normal subjects, 10 patients with cerebellar disease, and 9 patients with Parkinson's disease. Specifically, each subject was provided with visual information about the direction and/or the amplitude of an upcoming platform tilt. For the control situation no advance information on the characteristics of platform tilt was provided. Neither the latencies nor the integrals of postural EMG-responses showed alterations with advance information. In contrast, in a control experiment in which 3 normal subjects had to perform large or small forward or backward voluntary movements of the body around the ankle joint, shorter onset-latencies of leg muscle EMG responses were observed with increasing complexity of the advance information. These results suggest that, unlike voluntary movements, postural responses to rapid surface tilts do not benefit from advance visual information on direction or amplitude of a postural disturbance.

Adult↗
PubMed1987

Dysplastic disease of the cerebellum of an adult horse.

A 4-year-old horse was evaluated at the Colorado State University Veterinary Teaching Hospital for rapidly progressing cerebellar disease. Euthanasia was elected and at postmortem examination a proliferative mass encompassing the right side of the cerebellum was discovered. The lesion was characterized by large, convoluted, vascular folia and absence of the core of central white matter. Histologically, there was a diminution or loss of the internal granule cell layer, cavitation of the central white matter, and absence of Purkinje cells. The molecular layer was thickened with myelinated axons originating from large neurons aberrantly located in this zone. The pathology of this cerebellar mass closely resembles that seen in the cerebellum of humans with Lhermitte-Duclos disease. Mature adults are most commonly affected and clinical signs associated with this disease are often vague. The disease is thought to result from hypertrophy of granule cells possibly due to an early maturation or migration defect. Because of the late age of onset and the rarity of the disorder, its pathogenesis has been difficult to discover. Awareness that other mammalian species may be affected with a similar disorder may lead to an understanding of the causative developmental defect.

Animals↗