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PubMed2003

Spatial and temporal sequence learning in patients with Parkinson's disease or cerebellar lesions.

The functional role of different subcortical areas in sequence learning is not clear. In the current study, Parkinson's patients, patients with cerebellar damage, and age-matched control participants performed a serial reaction time task in which a spatial sequence and a temporal sequence were presented simultaneously. The responses were based on the spatial sequence, and the temporal sequence was incidental to the task. The two sequences were of the same length, and the phase relationship between them was held constant throughout training. Sequence learning was assessed comparing performance when both sequences were present versus when the dimension of interest was randomized. In addition, sequence integration was assessed by introducing phase-shift blocks. A functional dissociation was found between the two patient groups. Whereas the Parkinson's patients learned the spatial and temporal sequences individually, they did not learn the relationship between the two sequences, suggesting the basal ganglia play a functional role in sequence integration. In contrast, the cerebellar patients did not show any evidence of sequence learning at all, suggesting the cerebellum might play a general role in forming sequential associations.

Aged↗
PubMedDate not supplied

[Concurrence of Fahr's disease with cerebellar tumors].

Fara's disease or idiopathic calcification of the basal ganglia is a rare disease that is characterized by multiple petrificates in the area of the basal ganglia, caudate nucleus, and dentate nuclei of the cerebellum. As of now, only two cases of a concurrence of Fara's disease and brain tumors have been described. The authors present two more cases. Both cases are unique since the tumors occurred in the presence of Fara's disease symmetrically, as in the mirror, in the cerebellar hemispheres at the periphery of petrificates. This may be confirmed by the fact that astrocytic proliferation and hyperplasia around the calcified vessels are a cause of neoplasms.

Adult↗
PubMed1990

Ubiquitin immunoreactivity in kuru plaques in Creutzfeldt-Jakob disease.

Cerebellar kuru plaques in 2 cases of Creutzfeldt-Jakob disease were studied immunohistochemically. Similar to cerebellar senile plaques in Alzheimer's disease, many kuru plaques contained ubiquitin-positive, tau-negative small granular elements, presumably representing dystrophic neurites. Our results suggest that similar mechanisms are involved in neuritic changes in cerebellar plaques in Creutzfeldt-Jakob and Alzheimer's diseases despite differences of amyloid proteins in the plaques.

Aged↗
PubMedDate not supplied

Voice dysfunction in dysarthria: application of the Multi-Dimensional Voice Program.

Phonatory dysfunction is a frequent component of dysarthria and often is a primary feature noted in clinical assessment. But the vocal impairment can be difficult to assess because (a). the analysis of voice disorder of any kind can be challenging, and (b). the voice disorder in dysarthria often occurs along with other impairments affecting articulation, resonance, and respiration. A promising assessment tool is multi-parameter acoustic analysis, such as the Multi-Dimensional Voice Program (MDVP). Part 1 of this paper recommends procedures and standards for the acoustic analysis of voice, including (1). selection of the sample to be analyzed, (2). signal quality requirements, (3). availability of normative data for both genders and different ages of speakers, (4). reliability of analysis, and (5). correlation of acoustic results with results from other methods of analysis. In Part 2, acoustic data are reviewed for the dysarthria associated with Parkinson disease (PD), cerebellar disease, amyotrophic lateral sclerosis (ALS), traumatic brain injury (TBI), unilateral hemispheric stroke, and essential tremor. Tentative profiles of voice disorder are described for these conditions. These profiles may serve as hypotheses for future research. Although several issues remain to be resolved in the acoustic analysis of voice disorder in dysarthria, steps can be taken now to promote the reliability, validity, and clinical utility of such analyses. (1). As a result of this activity, the participant will be able to describe ways in which an optimal multi-dimensional analysis of voice can be performed with modern acoustic analysis systems. (2). As a result of this activity, the participant will be able to apply multi-dimensional acoustic analysis of voice to individuals who have a dysarthria-related voice disorder. (3). As a result of this activity, the participant will be able to identify major sources of normative data on the Multi-Dimensional Voice Program.

Amyotrophic Lateral Sclerosis↗
PubMed1999

Low insulin-like growth factor (IGF-1) in the cerebrospinal fluid of children with progressive encephalopathy, hypsarrhythmia, and optic atrophy (PEHO) syndrome and cerebellar degeneration.

PURPOSE: In patients with progressive encephalopathy, hypsarrhythmia, and optic atrophy (PEHO) syndrome, the pathophysiology underlying early progressive cerebellar and brainstem degeneration and severe epilepsy is unknown. Because insulin-like growth factor (IGF)-1 has been shown significantly to promote survival of cerebellar neurons, we wanted to see if the IGF system played a role in the pathogenesis of cerebellar atrophy. METHODS: We used a sensitive enzyme immunoassay kit for measuring cerebrospinal fluid (CSF) IGF-1 and insulin-like growth-binding protein (IGFBP)-3 in four groups of patients: PEHO syndrome patients (eight), PEHO-like patients (seven), age-matched controls (31), and patients with other types of cerebellar atrophy (11). RESULTS: Patients with PEHO syndrome and those with other progressive, degenerative cerebellar diseases had lower levels of CSF IGF-1 than the controls with other neurologic diseases. The CSF IGF-1 also allowed us to differentiate the "true" PEHO patients from the "PEHO-like" patients (those with similar clinical symptoms but without the typical neuroophthalmologic or neuroradiologic findings). The concentrations of IGFBP-3 did not significantly differ in any of the patient or control groups studied. CONCLUSIONS: CSF IGF-1 levels might be used as a marker of the degeneration of neurons in specific areas.

Atrophy↗
PubMed2006

Cachexia--induced cerebellar degeneration: involvement of serum TNF and MCP-1 in the course of experimental neoplastic disease.

Cerebellar degeneration may be recognized as a remote effect of a growing tumor. We have analysed serum concentrations of tumor necrosis factor-alpha (TNF-alpha), macrophage chemoattractant protein-1 (MCP-1), thyroxine and insulin to elucidate the pathomechanism which may be of importance for the development of central degeneration in cachectic Morris hepatoma bearing rats. Serum TNF-alpha and MCP-1 levels were evaluated by means of the ELISA system, while thyroxine and insulin were estimated by radioimmunoassay. Microscopic examination using hematoxylin-eosin, Nissl and Klüver-Barrera staining revealed an atrophy in the cerebellum, homogenization changes of Purkinje cells and decreased cell density of the granular layer. In the Morris hepatoma bearing animals serum MCP-1 content was elevated while TNF-alpha, thyroxine and insulin concentrations were decreased. This study has demonstrated that circulating TNF-alpha and MCP-1, together with decreased levels of insulin and thyroxine accompany and may produce a milieu of factors involved in mechanisms of the development of cerebellar degeneration in cachectic hepatoma bearing rats.

Animals↗
PubMed2004

Subclinical celiac disease with cerebellar ataxia.

We report an unusual case of celiac disease with cerebellar ataxia. Gastrointestinal signs and malabsorption were not found in this patient. We suggested that celiac disease should be taken into consideration in differential diagnosis of patients with cerebellar ataxia with unknown etiology.

Adult↗
PubMed1995

Oxidative phosphorylation diseases and cerebellar ataxia.

Oxidative phosphorylation (OXPHOS) diseases can be caused by mutations in nuclear genes or mitochondrial DNA (mtDNA) genes. mtDNA mutations include complex mtDNA rearrangements in which large segments of mtDNA are duplicated or deleted and point mutations in which single nucleotide substitutions occur within transfer RNA (tRNA) genes, ribosomal RNA (rRNA) genes, or mitochondrial genes encoding OXPHOS polypeptides. Although over 30 pathogenic mtDNA point mutations and over 60 different types of mtDNA deletions are known (Shoffner and Wallace, 1995; Wallace et al., 1994), only a subset of these mutations are associated with cerebellar ataxia. This review focuses on the clinical, biochemical, and genetic features of OXPHOS diseases caused by mtDNA mutations in which ataxia is a common manifestation.

Adult↗
PubMed2000

Essential tremor and cerebellar dysfunction clinical and kinematic analysis of intention tremor.

The cerebellum is assumed to play a major role in the pathophysiology of essential tremor (ET). As intention tremor is considered one of the classical features of cerebellar disease, we have assessed a large group of patients with ET for the semiology of the tremor and have performed objective quantitative analysis of a grasping movement in patients with ET, cerebellar disease and a normal control group. We found 25% of the patients to have a moderate or severe kinetic tremor with clear-cut features of a classical intention tremor. Another 33% of the patients had a mild intentional component of their kinetic tremor. Patients with intention tremor (ET(IT)) did not differ from those with predominant postural tremor (ET(PT)) with respect to alcohol sensitivity of the tremor and the frequency of a family history. ET(IT) patients were older and more often showed head and trunk involvement. The onset of this intention tremor has been assessed retrospectively. It was found to begin at a randomly distributed time interval after the onset of the postural tremor, but older patients had a shorter time to development of intention tremor. Quantitative accelerometry of postural tremor showed similar tremor frequencies in both patient groups, but ET(IT) patients had a slightly larger tremor amplitude. Quantitative analysis of a grasping movement using an infrared-camera system was performed in two subgroups of the patients with ET(PT) and ET(IT) and control groups with cerebellar disease or normal subjects. The intention tremor could be quantified objectively as an increased amplitude of curvature during the deceleration and target phase of the movement. The amplitude measurements of intention tremor were clearly abnormal and of comparable magnitude for ET(PT) and cerebellar disease. Additionally, the patients with ET(IT) had a significantly slowed grasping movement during the deceleration and target period. Hypermetria was significantly increased for the patients with ET(IT) and cerebellar disease. We conclude that intention tremor is a feature of ET. ET(IT) patients have abnormalities of their upper limb function compatible with cerebellar disease. This suggests that patients with more advanced ET show abnormalities of cerebellar functions.

Adolescent↗
PubMed1998

Lipopigment Changes in Purkinje Cells in Alzheimer's Disease.

Cerebellar tissue was examined from 22 patients with Alzheimer's disease (AD) and from an age-matched group of 20 non-diseased subjects. Intraneuronal lipopigment in the bodies of 1344 Purkinje cells (PCs) (32 per brain) was identified by fluorescence microscopy. The mean total area (per PC) of the outlines of discrete regions of lipopigment in a PC perikaryon for the AD-related group of PCs was significantly greater than the mean for the comparison group (p<0.001). Also, the two groups of PCs showed significant (</=0.05) differences in the mean number (per PC) of discrete regions of lipopigment in 11 size categories. The findings indicate a lysosomal abnormality in PCs in AD. The pattern of size distribution of lipopigment in PCs differed from that previously-reported for neurons of the frontal cortex. These differences may be associated with the absence of senile plaques and the presence of "diffuse" amyloid plaques in the cerebellum in AD

Journal Article↗
PubMed1974

Acquired pendular nystagmus with oscillopsia in multiple sclerosis: a sign of cerebellar nuclei disease.

In an unselected series of 644 cases of multiple sclerosis, 25 cases with acquired pendular nystagmus were found. Ten additional cases of pendular nystagmus in multiple sclerosis were investigated, and four cases from the literature are analysed. Acquired pendular nystagmus is purely sinusoidal in form, ceases with eye closure, is accompanied by oscillopsia, often monocular and vertical in direction, and never accompanied by optokinetic inversion. This is different from congenital nystagmus. Acquired pendular nystagmus in multiple sclerosis shows a high correlation with holding tremor of head and arm and with trunk ataxia, and must therefore be viewed as a result of lesions of cerebellar nuclei or their fibre connections with the brain-stem. Supporting evidence is discussed. The results fit into a theory of cerebellar function according to which the cerebellar nuclei are involved in the maintenance of positions.

Adult↗
PubMedDate not supplied

The neurobiological basis of movement initiation.

Simple reaction time (RT) is defined as the elapsed time between presentation of a single stimulus and onset of movement. In choice RT, there are at least two stimuli, requiring two distinct responses. The neurobiological basis of RT in humans has mostly been evaluated in patients with Parkinson's disease or cerebellar disease. Lesion studies in animals have assessed the different contributions of various subregions of the basal ganglia and the cerebellum. There is a prolongation of simple RT and in some cases of choice RT in Parkinson's disease. Both simple and choice RT are susceptible to modulation by brain dopamine levels. However, such is not invariably the case, attesting to the contribution of non-dopaminergic neurons in the sensori-motor slowing found in Parkinson's disease. An increase in simple RT and in choice RT are found in patients with cerebellar atrophy. The initiation of fast ballistic movements is associated with the dentate efferent system. This system is modulated by dopaminergic and glutamatergic pathways to the striatum.

Animals↗
PubMed1987

Juvenile Alzheimer's disease with cerebellar involvement.

We describe a sporadic case of Alzheimer's disease with cerebellar involvement in a man, who died at age 32 years after an illness lasting seven years, which was marked by progressive dementia and ataxia. The brain was quite atrophic and showed numerous senile plaques of all types and neurofibrillary tangles in the cerebral cortex with some involvement of the basal ganglia and diencephalon. There was cerebellocortical atrophy with numerous large, kurulike plaques. In addition, widespread severe congophilic angiopathy was noted.

Adult↗
PubMed1980

Changes in RNA synthesis and messenger RNA content in the cerebellum of rats with graft versus host disease.

Cerebellar RNA accumulation, synthesis, and functional capacity was studied in 14-day-old F1 hybrid rats subjected to neonatally induced graft versus host disease (GVHD). There was a decrease in RNA synthetic rate as measured by the uptake of labeled precursors into RNA. The decrease in total cerebellar RNA synthesis was reflected both in a reduced amount of Nissl substance, visible in cresyl violet-stained 10-micron-thick sections of cerebella, and in the total amount of cytoplasmic RNA isolated from individual cerebella from diseased animals compared with control littermates. Analysis of the RNA translational capacity in wheat germ protein synthesizing systems showed that RNA from experimental animals was also biologically less active. Qualitative differences between protein populations in control and diseased animals were analyzed by two-dimensional gel electrophoresis. There were few alterations in the steady state levels of cerebellar protein. However, two-dimensional gel electrophoresis of the peptides synthesized in vitro by RNA from control and diseased animals showed that there were several changes in the relative abundance of some mRNAs between the two RNA populations. These data show that the cerebellar RNA from rats with GVHD differs both qualitatively and quantitatively from that of controls.

Animals↗
PubMed1979

Alterations of ocular motility in cerebellar pathology. An electro-oculographic study.

Saccadic as well as smooth pursuit movements were studied by means of electro-oculograms in a group of 14 patients affected by cerebellar diseases. Ten patients had cerebellar atrophies, and four had undergone surgery for cerebellar tumors. Loss of gain of the pursuit system and metric alteration of saccades were the most striking abnormalities observed. Dysmetria was shown to be related to the amplitude of the movement and to the sector of the perimeter within which the movement occurs (movements occurring in the more eccentric sector were more disturbed). A tendency to produce saccades slower than normal was noted in patients affected by olivopontocerebellar atrophy. The clinical and pathophysiological significance of this finding is discussed with particular reference to Wadia-Swaami hereditary ataxia.

Adult↗
PubMedDate not supplied

Head nodding associated with intermittent esotropia.

Head nodding (to-and-fro turning about the vertical cervical axis) associated with abnormal eye movements may be seen in spasmus nutans and congenital nystagmus. In the absence of abnormal eye movements, it may be indicative of neurological disease (eg, cerebellar disease, basal ganglia dysfunction). We report a neurologically normal infant without nystagmus but with intermittent head nodding and intermittent esotropia, whose head movements manifested only when his eyes were straight. The head movements ceased with the occlusion of either eye or spontaneous onset of esotropia. When his head was forcibly stabilized, he immediately developed esotropia. The head movement presumably facilitated fusion, although the mechanism of action is unknown.

Esotropia↗