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PubMed1997

[Neurodegeneration: aging and dementia. Etiopathogenic role of electron transport disorders. Therapeutic possibilities].

The neurodegenerative disorders (Parkinson's disease, Alzheimer's dementia, Huntington's disease, cerebellar degeneration) are common medical and social problems. The late onset diseases and slow neurodegeneration is connected with excitotoxins and alteration of mitochondrial electron transport chain. In elderly, congenital and acquired defects of mitochondrial complexes cause formation of free radicals. The overstimulation of excitatory amino acid receptors interfere with the cellular energy metabolism and also forming reactive oxygen species. The impaired energy metabolism make neuronal cells vulnerable to the excitotoxic damage. In these ways, excitotoxicity may be the final common pathway of neuronal death in a variety of neurodegenerative diseases. Potential therapeutic strategies would be use receptor antagonist or drugs to bypass energetic defects.

Aged↗
PubMed2000

Paraneoplastic cerebellar degeneration in pediatric Hodgkin disease.

Paraneoplastic cerebellar degeneration (PCD) is a rare neurological complication in adults with extracerebral neoplasms. It is characterized by a diffuse cerebellar dysfunction, usually leading to severe neurological sequelae. In childhood, this complication is extremely rare. We report on PCD as primary manifestation of Hodgkin disease (HD) in a thirteen-year old boy. On magnetic resonance imaging, irreversible atrophy of the cerebellum developed within three months. Antibodies against Purkinje cells were detectable at diagnosis and normalised after successful treatment of the lymphoma. Cerebellar symptoms, however, only partially resolved. The necessity of a search for a malignant tumour is emphasised in the presence of an otherwise unexplained, subacutely developing, diffuse cerebellar dysfunction.

Adolescent↗
PubMed1989

[Visual suppression test].

The visual suppression test is one of the methods measuring the function of visual fixation. It is carried out by recording the caloric nystagmus by electronystagmography and comparing the maximum slow phase velocity of caloric nystagmus in the darkness and the slow phase velocity in the light with eyes opened. In 50 normal adults aged 16 to 31 years, the score of the visual suppression of the slow phase velocity of caloric nystagmus was 69.9 +/- 18.5%. Visual suppression was also studied in 125 cases with various kinds of vestibular diseases. The results showed that: 1. In cases with peripheral vestibular diseases, the visual suppression was normal (visual suppression over 50%). 2. In cases with cerebellar diseases, the visual suppression might be reduced (visual suppression 40%-10%) or abolished (visual suppression under 10%). It means that the flocculus or nodulus had been directly or indirectly impaired. 3. In brain stem lesions, the visual suppression might be reduced, abolished or the caloric nystagmus might even show augmentation in light (visual suppression negative). It means that the paramedian pontine reticular formation had been impaired. 4. In case of CP angle tumor, owing to compression to the vestibulo-cerebellum or brain stem, the visual suppression might be abnormal. 5. In case of congenital nystagmus, visual suppression varied from normal to abolished. Thus, the visual suppression test is useful in differential diagnosis of the central and peripheral vestibular diseases.

Adolescent↗
PubMed2001

Perturbed step initiation in cerebellar subjects: 2. Modification of anticipatory postural adjustments.

Although ataxias of stance and gait are frequent manifestations of cerebellar disease, the number of human studies examining stance or gait in cerebellar subjects is limited. In the present study, we examined whether anticipatory postural adjustments were impaired in cerebellar subjects during perturbed and unperturbed step initiation. The first aim was to show possible abnormalities in timing, force and kinematic parameters of anticipatory postural adjustments in unperturbed stepping in cerebellar subjects. Second, we examined the ability of cerebellar subjects to modify anticipatory postural adjustments associated with step initiation in response to a backward translation. Finally, we asked whether cerebellar subjects (and controls) make use of predictive knowledge of perturbation amplitude in perturbed stepping. Only few abnormalities of anticipatory postural adjustments were found in cerebellar subjects compared to controls. Both in the unperturbed and perturbed step conditions, force production as well as step length and step velocity were reduced in cerebellar subjects compared to controls, suggesting compensatory slowing. Cerebellar subjects also appeared to be less able to use predictive information of perturbation amplitude to scale anticipatory postural adjustments than control subjects. Nevertheless, in unperturbed steps, temporal parameters of anticipatory postural adjustments were preserved in cerebellar subjects. When subjects voluntarily initiated a step in response to the surface translation, both control and cerebellar subjects adapted by executing the anticipatory postural adjustments for step more rapidly. Furthermore, both control and cerebellar subjects were able to use online information regarding perturbation amplitude to scale parameters of step initiation in perturbed stepping. Overall, our findings suggest that the cerebellum is neither critical for the basic motor program underlying unperturbed step initiation nor for many adaptive changes occurring during perturbed step initiation. Like its role in predictive scaling of automatic postural responses to external perturbations, the cerebellum appears to be important for predictive adaptation of anticipatory postural adjustments during step initiation.

Adolescent↗
PubMed1977

The shaking patient. Diagnosis and management of tremor.

Tremor can be categorized into three general types: resting, action, and intention, Each requires different therapy. Resting tremor is present when the hands are at rest; it disappears with movement. It is characteristic of Parkinson's disease and responds to treatment with L-dopa either alone or in combination with a decarboxylase inhibitor. Action tremor is maximal when the hands are outstretched to the front; it may persist during movement. It is not rare and is often misread as a sign of Parkinson's disease. Propranolol is beneficial. Intention tremor occurs with movement and is characteristic of cerebellar disease. Pharmacologic agents are not helpful. The only known effective treatment is stereotaxic surgery.

Amantadine↗
PubMed1999

Amyloid precursor proteins in the cerebellar cortex of Alzheimer's disease patients devoid of cerebellar beta-amyloid deposits: immunocytochemical study of five cases.

Five human brains affected by Alzheimer's disease (AD), but without cerebellar amyloid (A beta) deposits, were investigated for the presence and location of amyloid precursor proteins (APP). This was parallel to 6 AD brains with A beta deposits, 6 young controls and 6 age-matched controls. Antibodies against A beta and two epitopes of APP (amino and carboxy terminals, APP(60-100) and APP(643-695), respectively) were employed. Accumulations of APP in neurons (mainly Purkinje cells) and glial cells in the upper part of the molecular layer were far greater than those in age-matched control brains and similar to those in AD brains with A beta deposits. This suggests that changes in APP production and/or metabolism occur before A beta deposition, or that these changes can occur without amyloidogenic processing. More than 60% of positive Purkinje neurons were of normal appearance; most of them showed both APP(60-100) and APP(643-695) immunoreactivity, but a small number (<21%) reacted with only a single antiserum. A small number of Golgi, Lugaro and granule cells were APP immunopositive. In all cases, stellate and basket cells were negative, as were most glial cells other than those of the molecular layer. Folia showed two different appearances, which were particularly well displayed in three cases: "strongly" immunopositive folia with high reactivity in Purkinje cells and other neurons, and "weakly" immunopositive folia with low neuronal reactivity, but with a large number of positive glial cells in the molecular layer. The results are discussed in relation to the possible existence of types or stages of the AD process and local factors, including specific and non-specific cell factors, in the induction of APP accumulation. All these 5 cases were female, but the Apo-E 4 genotype was displayed only in two cases.

Adult↗
PubMed1999

Classification of tremor and update on treatment.

Tremor is a symptom of many disorders, including Parkinson's disease, essential tremor, orthostatic tremor, cerebellar disease, peripheral neuropathy and alcohol withdrawal. Tremors may be classified as postural, rest or action tremors. Symptomatic treatment is tailored to the tremor type. Combination therapy with carbidopa and levodopa remains the first-line approach for parkinsonian tremor. Essential tremor may be amenable to propranolol or primidone. Propranolol may be useful in treating alcohol withdrawal tremor, and isoniazid may control the cerebellar tremor associated with multiple sclerosis. Clonazepam may relieve orthostatic tremor. Other agents are also available for the treatment of tremor. When medical therapy fails to control the tremor, surgical options such as thalamotomy, pallidotomy and thalamic stimulation should be considered in severe cases. Thalamic stimulation, the most recent of these surgical approaches, offers the advantage over ablative procedures of alleviating tremor without the creation of a permanent lesion.

Diagnosis, Differential↗
PubMed2005

Degree of cerebellar ataxia correlates with three-dimensional mri-based cerebellar volume in pure cerebellar degeneration.

The aim of the present study was to compare the severity of cerebellar ataxia as measured by the International Cooperative Ataxia Rating Scale (ICARS) by Trouillas et al. [ J Neurol Sci 1997;145:205-211] with the cerebellar volume in chronic cerebellar disease. Fifteen patients with pure cerebellar degeneration were investigated. Seven patients suffered from spinocerebellar ataxia type 6, 5 from idiopathic late-onset cerebellar ataxia, 2 from autosomal dominant cerebellar ataxia type III and 1 from episodic ataxia type 2. Volumetric analysis was based on individual three-dimensional MR images. Total ICARS score significantly inversely correlated with the cerebellar volume (r = -0.805, p < 0.0001), correlations between ICARS subscores and cerebellar volume were significant for upper and lower limb ataxia, ataxia of posture and gait, and dysarthria, but not for the oculomotor subscore. The results suggest that the degree of cerebellar atrophy in pure cerebellar degenerative disorders is accompanied by comparable functional impairment (i.e. degree of cerebellar ataxia).

Adult↗
PubMed2006

Depth perception in cerebellar and basal ganglia disease.

There is increasing evidence that the cerebellum and the basal ganglia serve not only a role in motor control but also in visual perception. Patients with Parkinson's disease (PD) as well as patients with cerebellar lesions exhibit impairments of vision that are not fully explained by ocular motor deficits. It is less clear to which extent these visual deficits contribute to an impaired control of visually guided movements. This study examined whether a dysfunction of the cerebellum or the basal ganglia induces impairments in depth perception, which affect action. We employed an illusionary display, the Ames trapezoidal window, to determine the ability of PD patients (n=10) and patients with spinocerebellar ataxia (SCA) (n=6) to process depth cues when estimating object slant. Participants either pointed to the edges of the window (motor judgement) or verbally indicated the perceived orientation of the display (verbal judgement). To control for ocular and limb motor deficits, participants judged the slant of a non-illusionary display in a second task. Slant estimation of the non-illusionary window was not impaired in either patient group when compared to control subjects (all P>0.2). In contrast, SCA as well as PD patients exhibited significantly greater slant estimation errors than controls when pointing to the illusionary window (P=0.005). In addition, both patient groups made larger errors than controls in their verbal judgements during binocular viewing of the illusion (P=0.005), but not during monocular viewing (P>0.2). In sum, the present findings point towards a role for both the basal ganglia and cerebellum for the processing of visual information about depth. Since the deficits were seen both in the context of action and perception and were only partially reconciled by the availability of binocular depth cues, we conclude that basal ganglia as well as cerebellar disease may affect the visual perception of depth.

Adult↗
PubMed1997

[Cerebellar atrophy in Minamata disease: comparison with spino-cerebellar degeneration on MR images].

We evaluated atrophic patterns of the cerebellar vermis in seven patients with Minamata disease (MD) and nine patients with spino-cerebellar degeneration (SCD) on MR images. Twenty-five control subjects were also examined. The cerebellar vermis was divided into superior, middle, and inferior parts by the primary fissure and the prepyramidal fissure on the median sagittal T1-weighted MR image. The length and area of each part were measured. In the patients with SCD, there were no significant differences in the degree of atrophy among the three parts. However, MR images of the patients with MD showed more severe atrophy in the middle and inferior parts than in the superior part. Atrophy of the superior part was less frequently observed in MD patients.

Adult↗
PubMed1998

Neck and other muscle pains in autonomic failure: their association with orthostatic hypotension.

Neck pain in the suboccipital and paracervical region ('coathanger' configuration) is often reported by patients with autonomic failure and orthostatic hypotension. The frequency of this pain, along with pains in the buttock and calf regions, was determined by questionnaire in two major groups with primary chronic autonomic failure--pure autonomic failure (PAF) and multiple system atrophy (MSA). Comparisons were made with Parkinson's disease, cerebellar degeneration and other disorders in which neurological symptoms overlap but in which there was neither autonomic failure nor orthostatic hypotension. Neck pain was present in 93% of patients with PAF, 51% of patients with MSA and 38-47% of the non-autonomic groups. Buttock pain was present in smaller but similar proportions (8-19%) of each group, like calf pain (23-37%). Neck pain in PAF and MSA differed from that in the other groups in being relieved by sitting or lying flat and in being associated with factors that lower blood pressure in these patients. Buttock pain was posturally related in PAF and MSA; for calf pain there was no difference between groups. Neck pain was related to the degree of orthostatic hypotension; in PAF patients, whose postural blood-pressure fall was greater than that in MSA, there was a greater frequency of neck pain.

Adult↗
PubMed1998

Quantitative study of Stewart-Holmes test.

Stewart-Homes test (SHT) is an ordinary neurological test performed for the diagnosis for cerebellar disease. We developed a quantitative method based on SHT carried out on the upper limb. Nineteen cerebellar patients and fifteen volunteer healthy subjects were tested. During the test, acceleration of forearm and two associated surface EMGs over biceps and triceps brachialis were recorded and analyzed. (1) Acceleration curve showed an oscillating pattern with flexion and extension over the elbow in both groups, but in cerebellar patient group, the acceleration oscillation wave was more pronounced and latencies of peak acceleration were significantly longer. (2) Correspondingly, the EMGs timing parameters were also different between the two groups: in patient group, ceasing isometric biceps contraction was delayed; rebound EMGs bursts over both biceps and triceps were prolonged. (3) Modelization of the oscillation of acceleration curve with dampened oscillation model showed that in patient group the oscillating amplitude attenuated much more slowly than in control group. A standard curve was established for detecting the acceleration profile abnormalities of SHT in cerebellar patients.

Adult↗
PubMed1997

Cerebellar presentation of multiple system atrophy.

Early diagnosis of multiple-system atrophy (MSA) is important in patients presenting with late-onset cerebellar ataxia because it has a less favourable prognosis than other degenerative ataxic disorders. We report cerebellar presentation of MSA in a series of 16 patients, 3 of whom later developed parkinsonism. Two-thirds of them had early evidence of impaired postural reflexes with a history of recurrent falls. Some of these had a narrow-based, unsteady gait, unlike the more classic broad-based gait ataxia of cerebellar disease. On review of the patients' histories, genitourinary dysfunction (particularly impotence) was present at the onset of, or preceding, cerebellar ataxia in 60% of patients, but this had often been attributed to age, or to urological or gynaecological causes. External striated anal or urethral sphincter electromyography (EMG) demonstrated features of chronic denervation and reinnervation in 14 (93%) of 15 patients, consistent with degeneration in Onuf's nucleus as occurs in MSA. Autonomic function tests were abnormal in 9 (64%) of 14 patients. Our data suggest that close enquiry into genitourinary function and analysis of the gait disorder can be useful pointers to a diagnosis of MSA in patients with an unexplained adult-onset progressive cerebellar syndrome, and that sphincter EMG is the most useful investigation in this context.

Adult↗