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At least 163 records · Page 9Linked to original sources
PubMed2003

The MAZ protein is an autoantigen of Hodgkin's disease and paraneoplastic cerebellar dysfunction.

Probing a cerebellar expression library with TrAb sera from patients with Hodgkin's disease and paraneoplastic cerebellar degeneration resulted in the isolation of MAZ (myc-associated zinc-finger protein). Eleven of 19 TrAb sera and 16 of 131 controls reacted with MAZ, indicating a significant, although not specific, association between Tr and MAZ immunities (p < 0.001). Interestingly, 9 of 16 positive control patients also had cerebellar dysfunction. Purified MAZ antibodies reacted with Purkinje cells. In neuronal cells, MAZ interacts with DCC (Deleted in Colorectal Cancer product), the receptor for netrin-1, a neuronal survival factor. These findings suggest epitope spreading between the Tr antigen and the MAZ-DCC complex and offer a possible model of immune-mediated cerebellar disease.

Autoantibodies↗
PubMed2000

Phonological grouping is specifically affected in cerebellar patients: a verbal fluency study.

OBJECTIVES: Recent clinical and functional neuroimaging evidence points towards a cerebellar role in verbal production. At present it is not clear how the cerebellum participates in language production. The aim was to investigate the influence of cerebellar lesions on verbal fluency abilities with specific focus on the verbal searching strategies employed by patients with cerebellar damage. METHODS: Twenty five patients with focal or degenerative cerebellar disease and 14 control subjects were tested in a timed verbal fluency task requiring word production under forced (phonemic or semantic) conditions. To analyse the verbal searching strategy employed, semantic and phonemic cluster analyses were also performed. RESULTS: Performances of cerebellar patients were comparable with those of controls in the semantic task; conversely their performances were significantly impaired when tested in the letter task. Cluster analysis results showed that the verbal fluency impairment is linked to specific damage of phonemically related retrieval strategies. CONCLUSION: Cerebellar damage impairs verbal fluency by specifically affecting phonemic rule performances while sparing semantic rule ones. These findings underline the importance of the cerebellar computing properties in strategy development in the linguistic domain.

Adult↗
PubMed1989

Anticerebellar antibodies in neurologically normal patients with ovarian neoplasms.

Several groups of investigators have confirmed the occurrence of antibodies to Purkinje and other cerebellar neuronal populations in the serum and spinal fluid of patients with paraneoplastic cerebellar degeneration. Although this antibody response suggests that paraneoplastic cerebellar degeneration may have an autoimmune basis, it is not known what role anticerebellar antibodies play in the pathogenesis of this disorder or whether the presence of antibodies invariably results in cerebellar injury. We identified 3 patients with ovarian malignancies in whom high titers of circulating anticerebellar antibodies were present without clinical evidence of cerebellar disease. We followed these patients clinically and serologically until their deaths from their neoplasms. All 3 patients remained neurologically normal. In 2 of the patients, anticerebellar antibodies persisted at high titer. CSF obtained from 1 of these patients postmortem did not contain detectable levels of anticerebellar antibody, but histopathologic examination of her cerebellum revealed patchy loss of Purkinje cells. In the 3rd patient, antibody titers fell with removal of the primary tumor and chemotherapy but did not rise with tumor recurrence. Indirect immunofluorescence did not reveal anticerebellar antibodies in the serum or CSF of other patients with neoplasms, patients with other cerebellar disease, or normal controls. The present study demonstrates that patients with ovarian malignancies may occasionally develop antibodies that react with cerebellar neuronal antigens and can maintain this antibody response for protracted periods of time without clinically evident cerebellar injury. Tumor recurrence may not be accompanied by rise in titers of anticerebellar antibodies.

Aged↗
PubMed1994

Impairments of precision grip in two patients with acute unilateral cerebellar lesions: a simple parametric test for clinical use.

A precision grip task was used to test two patients with acute unilateral cerebellar lesions due to a stroke and to compare performance of the affected and the unaffected hand. The results show, that grip force development is slowed and coordination of grip and lift force impaired by the lesion. Changes in shape and peak levels of grip force rate indicate a contribution of cerebellar function to the programming of precision grip. This method should be suited to monitor the course of cerebellar disease.

Cerebellar Diseases↗
PubMed1992

The coordination of posture and voluntary movement in patients with cerebellar dysfunction.

Postural adjustments associated with the task of rising on tiptoes were investigated in a reaction time paradigm in 10 normal subjects and 18 patients with cerebellar disorders. Cerebellar dysfunction was due to either degenerative cerebellar disease, tumor, or ischemia. Displacements of the center of foot pressure (CFP) were recorded. The task, accomplished by the triceps surae muscle (executional activity, mean latency of 411 ms), is mechanically effective only if the center of gravity has been shifted forward in advance. To this effect, a phasic burst of preparatory EMG activity in the tibialis anterior normally occurs at a mean latency of 163 ms, shifting the center of gravity forward. Shortly thereafter, activity of the quadriceps femoris (175 ms) extends the knee and aids the forward shift of the center of gravity. Different aspects of this motor sequence were disturbed in individual patients: Latencies of preparatory and executional activity were uncorrelated in 15 of the 18 patients. Executional (n = 16) or preparatory (n = 13) EMG activity was tonic instead of phasic. Latencies of either preparatory or executional EMG activities or both were prolonged (n = 10). The time interval between motor preparation and execution was increased (n = 9). The trial-to-trial variability of biomechanical parameters and EMG latency was increased. Preparatory EMG activity in the quadriceps was entirely missing (n = 9), resulting in knee bending at the unsuccessful attempt to rise on tiptoes. Patients who were most severely affected had no preparatory activity at all (n = 2), and therefore were unable to perform the task.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1997

Rediscovery of an early concept.

The study of the cerebellum has been dominated by interest in its role in movement and motor control. From the earliest days of the neuroscientific era, however, clinical reports and physiological and behavioral investigations have suggested that overt motor dysfunction is but one manifestation of cerebellar disease. The nature of cerebellar involvement in autonomic, sensory, and cognitive functions has been investigated for many years, and possible mechanisms that could subserve this relationship have been specifically addressed. This work has not been incorporated into the mainstream of neuroscience or clinical neurological thinking. This chapter traces the history of these early investigations that demonstrated the need to revise the notion that cerebellar function is confined to the motor realm. The collaboration across disciplines and the advances in the methods and concepts of contemporary neuroscience have facilitated the maturation of this field of inquiry. The "new" story of the cerebellum and cognition, in fact, represents the evolution of a century-old revolutionary concept.

Europe↗