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At least 415 records · Page 23Linked to original sources

Force platform recordings in the diagnosis of primary orthostatic tremor.

Primary orthostatic tremor (OT) consists of rhythmical muscle contractions at a frequency of around 16 Hz, causing discomfort and/or unsteadiness while standing. Diagnosis has hitherto relied on recording Electromyography (EMG) from affected muscles. The main aim of this study was to see if the characteristic postural tremor in OT can be identified with force platforms. We also quantified postural sway in OT patients to assess their degree of objective unsteadiness. Finally, we investigated the time relations between bursts of activity in the various affected muscle groups. Subjects stood on a force platform with concurrent multichannel surface EMG recordings from the lower limbs. Seven patients with clinical and EMG diagnosis of OT were examined and the force platform data compared with those of 21 other neurological patients with postural tremor and eight normal controls. All OT patients had high frequency peaks in power spectra of posturography and EMG recordings (12--16 Hz). No such high frequency activity was evident in patients with Parkinson's disease, cerebellar degenerations, essential tremor or in healthy controls. Additionally, OT patients showed increased sway at low frequencies relative to normal controls, suggesting that the unsteadiness reported by OT patients is at least partly due to increased postural sway. Examination of EMG timing showed fixed patterns of muscle activation when maintaining a quiet stance within but not across OT patients. These data show a high correlation between EMG and posturography and confirm that OT may be diagnosed using short epochs of force platform recordings.

Blood Pressure↗

Unusual cerebello-pontine angle tumours.

Fifty-nine unusual cerebello-pontine angle tumours have been studied. These lesions represent 19.3 per cent or 1 in 5 of a series of 305 cerebello-pontine angle tumours of which the rest, 246 (80.7 per cent), were acoustic neuromas. An analysis of the relative incidence, histology and presenting clinical features has been carried out. The various radiographical features and imaging techniques used to diagnose these fascinating tumours have been described and also the otoneurosurgical procedures necessary to excise them.

Adolescent↗

Radio-necrosis of the temporal bone presenting as cerebellopontine angle lesion.

We report a case of osteoradionecrosis of the temporal bone which presented with symptoms and signs of cerebellopontine angle lesion. The clinical, radiological, histological and bacteriological findings are reported. The occurrence of osteoradionecrosis of the temporal bone and the factors which predispose to it are discussed with particular emphasis on the time lapse between radiotherapy and its development. This presentation has not previously been reported.

Bone Diseases↗

Leucocyte glutamate dehydrogenase in various hereditary ataxias.

Leucocyte Glutamate Dehydrogenase (GDH) activity was measured in 44 patients with various forms of ataxia and 44 age and sex-matched normal controls. The only significant change found was a moderate decrease in activity in Friedreich's ataxia and a few patients with OPCA. This decreased activity is not primary to the disease but probably reflects a regulatory defect affecting mitochondrial membranes in these patients.

Ataxia↗

Saccadic intrusions and oscillations.

There are a number of different types of involuntary saccadic eye movements which differ from normal saccades in that they take the fovea away from its target. This article presents a classification and description of these abnormal eye movements and indicates how they may be used to increase the precision of neurologic diagnosis.

Adult↗

Oculomotor and vestibular findings in autosomal recessive spastic ataxia of Charlevoix-Saguenay.

Electronystagmographic recordings were made of oculomotor and vestibular function in 11 patients with autosomal recessive spastic ataxia of Charlevoix-Saguenay. All had horizontal gaze nystagmus, marked impairment of smooth ocular pursuit and optokinetic nystagmus, and defective fixation suppression of caloric nystagmus. Many had saccadic dysmetria, but saccade velocity was probably unaffected. Abnormalities pointing to brainstem disturbance were sparse. The findings are thought to indicate mainly diffuse cerebellar disease, with particular involvement of vermis and vestibulo-cerebellum.

Adolescent↗

[Nondeclarative memory--neuropsychological findings and neuroanatomic principles].

The contents of long-term memory will influence behaviour, even if the acquired knowledge or the original learning episode are not remembered. These phenomena have been termed "non-declarative" or "implicit" memory, and they are contrasted with "declarative" or "explicit" memory which is characterised by conscious search and retrieval procedures. Non-declarative memory encompasses non-associative learning, simple conditioning, priming effects as well as motor, perceptual and cognitive skill acquisition. The dissociation of both forms of memory is documented by studies in health subjects which indicated that experimental manipulations or drugs may differentially affect declarative and non-declarative memory processes. Damage to the medial temporal or the medial thalamic regions is known to result in declarative memory deficits whereas non-declarative memory is largely unaffected by such lesions. Animal research and clinical findings indicate that several components of non-declarative memory such as motor and cognitive skill acquisition or certain types of classical conditioning are dependent upon the integrity of the basal ganglia or the cerebellum. These issues are therefore of increasing importance for the understanding of extrapyramidal and cerebellar diseases. This paper presents recent neuropsychological findings and neuroanatomical data relating to the issue of non-declarative memory.

Animals↗

[Degenerative diseases of the extrapyramidal and spinocerebellar system--differential diagnostic references].

Degenerative disorders of the extrapyramidal and spinocerebellar system often have a genetic cause; however, pathogenesis usually is still unknown. Disturbance of neurotransmitter metabolism in Parkinson disease or in some dystonic syndromes offers new therapeutic approaches; in choreatic or athetoid syndromes biochemical factors are only known in some parts. Clinical and neurophysiological differentiation of various dyskinetic disorders is essential to reach better understanding. In children early signs and symptoms are seen which may be caused by different factors and are difficult to diagnose. Similar problems are to be seen in hereditary ataxias which sometimes begin in childhood: Clinical findings are essential to plan for additional investigations.

Athetosis↗

Unusual cause of cerebellar abscess: occipital dermal sinus and dermoid cyst.

Cerebellar abscess induced by a contiguous dermal sinus is a rare event. In a large series of acute posterior fossa abscedation, otogenic suppuration is the cause in 93% of the cases, while hematogenous infection is infrequent, probably because of the relatively weak bloodflow in this part of the central nervous system. The authors present an occipital dermal sinus and dermoid cyst revealed by a cerebellar abscess. The literature is reviewed briefly, treatment and prophylactic measures are discussed.

Brain Abscess↗

The importance of routine magnetic resonance imaging in trigeminal neuralgia diagnosis.

The aim of the study was to determine the frequency of structural lesions diagnosed on magnetic resonance imaging (MRI) in a sample of patients with trigeminal neuralgia (TN) and to compare history and clinical features between the groups with and without structural lesions. Clinical records and MRI findings of 42 consecutive patients diagnosed with TN at the National Dental Centre, Singapore, and who underwent routine MRI examinations at diagnostic workup between April 1997 and March 1999 were retrospectively studied. Of these, 6 (14.3%; 95% confidence interval, 0 to 28.8%) were diagnosed with an associated structural lesion. Mean age of the group with structural lesions was 53.3 years (standard deviation, 10.9 years) at presentation, there was female predominance (M:F = 1:2), and all (100.0%) gave a typical pain history and had unilateral involvement of a single division of the trigeminal nerve. Two patients (33.3%) had other cranial nerve abnormalities. Three patients (50.0%) had complete, 2 patients (33.3%) had partial, and 1 patient (16.7%) had poor response to medical therapy. None of these variables was statistically different from the group without structural lesions (cranial nerve abnormalities were not compared). In conclusion, the frequency of structural lesions associated with TN in our sample was relatively high. It is not possible to reliably identify high risk patients for selective MRI indication on the basis of history and clinical features. It may be prudent to consider routine MRI for all patients with TN to exclude structural lesions.

Adult↗

Purkinje cell degeneration, a new neurological mutation in the mouse.

A new autosomal recessive mouse mutation, Purkinje cell degeneration (pcd), is described. Mutants exhibit a moderate ataxia beginning at 3 to 4 weeks of age. The ataxia results from postnatal degeneration of virtually all cerebellar Purkinje cells beginning around 15 to 18 days of age and progressing rapidly over the next 2 weeks. In addition to the cerebellar disease there is slow progressive degeneration in the retina (photoreceptor cells) and olfactory bulb. Also, adult males have abnormal sperm.

Animals↗