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Congenital cerebellar cortical degeneration in Holstein cattle in Southern Brazil.

A congenital progressive cerebellar disorder is described in Holstein calves. The clinical signs were progressive and were characterized by ataxia, hypermetria, a wide stance and fine head tremors. When the affected cattle were forced to run, the signs were exacerbated, leading to epileptiform attacks. Histological lesions consisted of a very selective cerebellar cortical degeneration, almost exclusively affecting the Purkinje cells. The disease affected 6 out of 200 Holstein calves from the same bull. However, results of mating tests of the bull with his daughters and granddaughters suggested that it was not hereditary (p = 0.0062) although an environmental-genetic interaction could not be ruled out.

Animals↗

[Orbicularis oculi reflex in computerized tomography verified lesions of the posterior cranial fossa].

The blink reflex elicited by electrical stimulation was studied in 60 patients with lesions of the brainstem and cerebellum (ischemic lesions, non-traumatic bleedings or tumors), which were diagnosed by computertomography. The relationship between localized morphological changes in computertomography and changes in blink reflex has been studied. 46 patients (77%) had a pathologic blink reflex. Usually the blink reflex was normal in patients with a tumor of cerebellum. In bleedings or tumors of the brainstem a good relationship existed between localization and extension of the lesion and the pathological pattern of the blink reflex. In patients with thrombosis of the basilar artery no responses or single delayed responses to the electrical stimulation were found. A less good relationship was observed in ischemic lesions of the brainstem. This is interpreted as indicating possible multilocalization of vascular lesions in the brainstem. Nearly half of the patients with ischemic lesions also had supratentorial lesions. The influence of these lesions on the pattern of the blink reflex will be discussed.

Adolescent↗

Magnetic resonance imaging in "typical" and "late onset" Friedreich's disease and early onset cerebellar ataxia with retained tendon reflexes.

MRI makes it possible to study the in vivo brain and spinal cord morphology of patients with hereditary ataxia. We performed T1- and T2-weighted studies in eleven patients with Friedreich's disease (FD), five with "late onset" FD and ten with early onset cerebellar ataxia with retained tendon reflexes (EOCA). Cervical cord atrophy was constant in FD and "late onset" FD and often associated with atrophy of the cerebellum and of the brainstem; T2-weighted studies showed posterior column degeneration in the cervical cord. The most frequent finding in EOCA was cerebellar atrophy, pure or associated with cervical cord or brainstem atrophy; the cerebellar atrophy was marked in a few cases and was related to disease duration.

Adolescent↗

Visual control of balance in cerebellar and parkinsonian syndromes.

The role of vision in the control of balance in patients with Parkinson's disease (PD) and cerebellar disease (CD) was studied by measuring body sway with eyes open, closed, and in response to visual stimuli generated by discrete lateral displacements of a moveable room which enclosed the subjects. In response to room movement, normal subjects swayed by an amount intermediate between sway with eyes open and eyes closed and their response attenuated on repetition of the movement, a process depending on shifting from predominantly visual to proprioceptive control. CD patients swayed more than controls with eyes open or closed and as shown by high 'Romberg quotients' (eyes closed/eyes open sway ratio) were able to use visual information to control much of their unsteadiness. CD patients had a normal attenuation of response to repetition of the room movement. PD patients had normal sway with eyes open or closed but their responses to room movement were abnormal, being proportionately larger and failing to attenuate during successive stimuli. The results indicate that cerebellar lesions seem largely to spare the visuopostural loop and also spare the ability to shift from a visual to a proprioceptive control of postural sway. In contrast, the findings in PD suggest that the visuopostural loop is hyperactive and that its influence cannot easily be de-emphasized when visual information is misleading. The latter finding suggests that basal ganglia participation in posture is concerned with the reweighting of the various sensorimotor loops controlling posture in the process of adapting to novel situations.

Adaptation, Physiological↗