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At least 19 recordsLinked to original sources

Speech disorders in cerebellar disease.

The areas of cerebellar damage most commonly associated with dysarthria were sought by reviewing the clinical, radiographic, surgical, and autopsy findings in patients with nondegenerative cerebellar disease. Case histories on 162 patients with focal cerebellar lesions were reviewed. All but 15 of the patients underwent surgery, and 28 had autopsies. Thirty-one of the 122 patients with adequate descriptions of speech had dysarthria. Twenty-two of these 31 dysarthric patients had exclusively or predominantly left cerebellar hemisphere disease; 7 had right hemisphere disease; and 2 had vermal disease. Only 19 of 41 patients with exclusively or predominantly left hemisphere disease had had normal speech before surgery. Dysarthria developed in isolated cases following cerebellar resections extending into the paravermal segments of the left hemisphere. There was no correlation between the extent of vermal damage and development of abnormal speech. Cerebellar speech function was most commonly affected with damage to the superior portion of the left cerebellar hemisphere.

Adult

Acoustic characteristics of dysarthria associated with cerebellar disease.

The speech of five individuals with cerebellar disease and ataxic dysarthria was studied with acoustic analyses of CVC words, words of varying syllabic structure (stem, stem plus suffix, stem plus two suffixes), simple sentences, the Rainbow Passage, and conversation. The most consistent and marked abnormalities observed in spectrograms were alterations of the normal timing pattern, with prolongation of a variety of segments and a tendency toward equalized syllable durations. Vowel formant structure in the CVC words was judged to be essentially normal except for transitional segments. The greater the severity of the dysarthria, the greater the number of segments lengthened and the degree of lengthening of individual segments. The ataxic subjects were inconsistent in durational adjustments of the stem syllable as the number of syllables in a word was varied and generally made smaller reductions than normal subjects as suffixes were added. Disturbances of syllable timing frequently were accompanied by abnormal contours of fundamental frequency, particularly monotone and syllable-falling patterns. These dysprosodic aspects of ataxic dysarthria are discussed in relation to cerebellar function in motor control.

Aged

Gaze failure, drifting eye movements, and centripetal nystagmus in cerebellar disease.

Three abnormalities of eye movement in man are described which are indicative of cerebellar system disorder, namely, centripetally beating nystagmus, failure to maintain lateral gaze either in darkness or with eye closure, and slow drifting movements of the eyes in the absence of fixation. Similar eye movement signs follow cerebellectomy in the primate and the cat. These abnormalities of eye movement, together with other signs of cerebellar disease, such as rebound alternating, and gaze paretic nystagmus, are explained by the hypothesis that the cerebellum helps to maintain lateral gaze and that brain stem mechanisms which monitor gaze position generate compensatory biases in the absence of normal cerebellar function.

Adult

Chronic mania in childhood: case report of a possible association with a radiological picture of cerebellar disease.

The case-history of a woman with psychiatric symptoms since childhood is reported, the best label for which was that of "chronic mania". Cerebellar atrophy was suspected on radiological grounds. The literature on chronic mania in childhood and on cerebellar damage and mental changes is reviewed and the view is put forward that cerebellar dysfunction may play a part in the production of some psychiatric symptoms.

Adult

Prevention of virus-induced cerebellar diseases by defective-interfering lymphocytic choriomeningitis virus.

Defective-interfering (DI) lymphocytic choriomeningitis virus (LCMV) prevented disease in the central nervous system produced by standard LCMV. Standard LCMV injected into Lewis rats two days after birth produced a disorder distinguishable clinically by weight loss and ataxia and histologically by infiltration of mononuclear cells and necrosis of the cerebellum. Concurrent injection of DI LCMV with standard LCMV prevented the disease and markedly reduced the synthesis of standard LCMV and of viral antigens in the brain. Because inhibition of viral synthesis occurred early (day 3) after infection and because no interferon activity could be demonstrated, it was concluded that the interference effect was likely due to DI virus-mediated homologous interference. Other experiments showed that DI LCMV blocked viral antigen synthesis in culture. The curtailed production of viral antigens and cytolytic standard virus by DI virus may play a role in control of acute and persistent viral infections.

Animals

[Oculomotor signs in cerebellar disease shown in ataxia telangiectasia (Louis Bar) (author's transl)].

Disturbances of the eye movements are described in 2 brothers with ataxia telangiectasia (Louis Bar): pathological smooth pursuit and command movements of the eyes (hypometry) with a preseved doll's phenomenon, increased reaction times of voluntary saccades, failure of gaze holding, gaze nystagmus, altered optokinetic nystagmus, and distinct convergence defect. These oculomotor defects are the result of cerebellar lesions. The E.N.G.-findings in pathologically changed pursuit movements are in accordance with those disturbances of eye movements shown by Westheimer and Blair in cerebellectomized monkeys.

Animals

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

The rebound phenomenon of Gordon Holmes.

Contrary to a prevalent belief, the rebound phenomenon of Gordon Holmes is not a sign of cerebellar disease. It is elicited by having the patient attempt to move a limb against resistance. When the resistance is suddenly removed, the limb normally moves a short distance in the desired direction and then rebounds (jerks back in the opposite direction). Gordon Holmes pointed out that the rebound phenomenon is (1) present in normal libs, (2) exaggreated in spastic limbs, and (3) absent in limbs affected by cerebellar disease. An awareness of Holmes's observations may be helpful in the diagnosis of both cerebellar disease and spasticity.

Animals

[True and false cerebellar oculomotor manifestations (author's transl)].

The oculomotor perturbations of cerebellar affections are numerous. Their localizing value is however extremely variable: It is amongst the abnormalities of refixation that one can find the most typical cerebellar feature: such as dysmetria, macrosquare wave jerks, macrosaccadic oscillations only be seen in cerebellar disease. The ocular static of cerebellar patients is often abnormal, and affected by square wave jerks and pendular nystagmus. However, the abnormalities of cinetics are not specific.

Ataxia

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

Sodium valproate in the treatment of cerebellar disorders.

Because of the high concentrations of gamma-aminobutyric acid (GABA) in the cerebellar cortex and nuclei, an attempt was made to enhance GABAergic transmission in patients with cerebellar disease. Maximum tolerated doses of sodium valproate, a drug which inhibits the degradation of GABA, failed to influence cerebellar deficits in a double blind crossover study on six patients.

Cerebellar Diseases

[Cerebellar dysarthria (author's transl)].

Dysarthria due to involvement of the cerebellum represents a facultative symptom of cerebellar disease. The mechanisms by which such dysarthrias are produced are unknown but may result from pathologic involvement of the entire cerebellum. The symptoms of cerebellar dysarthria in children and in adults are described in two case histories. Characteristic speech defects or dysarthrophony occur. In addition to speech pathology, computer tomography of the skull is useful in obtaining both differential diagnosis and an estimate of prognosis. The goal of treatment should be the improvement of muscle defects of tonus and coordination needed for speech, and can employ both physio- and ergotherapy.

Adult

A physiological approach to motor disorders.

Spasticity in man is presented as a disinhibition of spinal cord mechanisms, the responses to stretch depending on the interaction of the reflex effects of group Ia with those of group II afferent fibres. The reflex responses to muscle stretch and shortening in Parkinson's disease do not depend on an abnormality of spinal reflex mechanisms. The superimposition of physiological tremor or alternating tremor in rigidity produces the classical cog-wheel sensation. The phase lead of the action tonic stretch reflex was found to be reduced in patients with athetosis and cerebellar disease, thus diminishing damping of unwanted movements. The more complex transmission characteristics of the action tonic stretch reflex of normal man are absent in patients with spasticity and cerebellar lesions, presumably due to interference with long-loop pathways. In normal subjects gain of the reflex loop increases with voluntary contraction but in spasticity gain remains high irrespective of contraction level.

Athetosis