Experimental Gaze Palsies in Monkeys and Their Relation to Uman Pathology

Henn, Volker; Lang, W.J.; Hepp, K.; Reisine, H.

doi:10.1093/brain/107.2.619

Cited by 134 publications

(49 citation statements)

References 29 publications

(24 reference statements)

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“…There has been one report of extensive Purkinje cell loss affecting most of the cerebellar cortex, including the flocculus, in SCA6 patients (Gomez et al 1997). Our neuro-otological findings suggested lesions in the vermis, flocculus, paraflocculus or nodulus with sparing of the paramedian pontine reticular formation, and these findings were consistent with the purely cerebellar disorders of eye movements in previous reports (Zee et al 1981;Henn et al 1984;Fetter et al 1994;Moschner et al 1994;Buttner and Grundei 1995;Buttner et al 1998;Burk et al 1999;Lin and Young 1999). Further studies will be necessary to confirm the relationship between anatomical lesions and the neuro-otological features of SCA6, especially the relationship between DPN and lesions of the flocculus or nodulus.…”

Section: Neuro-otological Aspectsupporting

confidence: 92%

A clinical and genetic study in a large cohort of patients with spinocerebellar ataxia type 6

et al. 2004

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In order to clarify the clinical and genetic features of SCA6, we retrospectively analyzed 140 patients. We observed an inverse correlation between the age of onset and the length of the expanded allele, and also between the age of onset and the sum of CAG repeats in the normal and the expanded alleles. The ages of onset of four homozygous patients correlated better with the sum of CAG repeats in both alleles rather than with the expanded allele calculated from heterozygous SCA6 subjects. Clinically, unsteadiness of gait was the main initial symptom, followed by vertigo and oscillopsia, and cerebellar signs were detected in nearly 100% of the patients. In contrast, extracerebellar signs were relatively mild and infrequent. The results of neuro-otological examination performed in 22 patients suggested the purely cerebellar abnormalities of ocular movements in nature. There was a close relationship between downbeat positioning nystagmus (DPN) and positioning vertigo, which became more common in the later stage. We conclude that total number of CAG repeat-units in both alleles is a good parameter for assessment of age of onset in SCA6 including homozygous patients. In addition, clinical and neuro-otological examinations suggested that SCA6 is a disease with predominantly cerebellar dysfunction.

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Section: Neuro-otological Aspectsupporting

confidence: 92%

A clinical and genetic study in a large cohort of patients with spinocerebellar ataxia type 6

et al. 2004

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“…That is, the saccadic eye movement is encoded by the temporal discharge of burst neurons [35]. Experimental or clinical lesions of the PPRF and riMLF abolish or slow saccades [7,11,12]. Most patients with PSP eventually show slow [24] or absent vertical saccades, and neuropathological studies have demonstrated involvement of the region in which riMLF lies [6,9].…”

Section: Discussionmentioning

confidence: 99%

Slow vertical saccades in the frontotemporal dementia with motor neuron disease

et al. 2008

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“…Upward extension of the lesion can be responsible for horizontal gaze palsy, but some previous reports indicated that there was also vertical gaze palsy. 6 ' 8 An animal experimental study 9 suggested the relation of vertical saccade palsy to the lower pontine lesion. Bilateral damage of the caudal paramedian pontine reticular formations (PPRFs) ventral to the abducens nuclei leads to a severe disruption of vertical rapid eye movements in it.…”

Section: Discussionmentioning

confidence: 99%

Bilateral medial medullary infarction with oculomotor disorders.

Toyoda

Hasegawa

Yonehara

et al. 1992

Stroke

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Background and Purpose:We describe the first case of bilateral medial medullary infarction demonstrated by magnetic resonance imaging. We discuss the relation between this lesion and the oculomotor signs that were observed clinically.Case Description: A 71-year-old man initially presented with pure motor hemiparesis, which progressed to complete quadriplegia. He also developed nearly complete vertical and horizontal ophthalmoplegia. Magnetic resonance imaging revealed upper medial medullary infarctions bilaterally that extended to the pontomedullary junction.Conclusions: We propose that the vertical oculomotor disorders resulted from involvement of the oculomotor system in the caudal brain stem, especially the caudal paramedian pontine reticular formations on both sides. (Stroke 1992;23:1657-1659 KEY WORDS • cerebral infarction • cerebrovascular disorders • magnetic resonance imaging • medulla oblongata • ocular disease I nfarction of the medial medulla oblongata has been rarely reported, even during the era of magnetic resonance imaging (MRI), a technology that can detect small lesions in the posterior fossa.1 Because of the rarity of this syndrome, clinical features such as disturbance of oculomotor control are still controversial. We report the first case, to our knowledge, in which bilateral medial medullary infarction was clearly demonstrated by MRI and discuss the relation between this anatomic lesion and the oculomotor signs appearing in this patient.Case Report A 71-year-old man with a history of hypertension and smoking awoke with a right hemiparesis. He was admitted to our hospital the day after symptoms began. On admission, his pulse rate was 80 beats per minute, blood pressure was 202/110 mm Hg, and respirations were 18 per minute and regular. He was alert, with normal higher cortical function. His speech was dysarthric. The visual fields were intact; pupils were equal, with a normal light reflex; and ocular movements were full and smooth, without nystagmus. A mild right hemiparesis was present involving the face and tongue. The rightsided deep tendon reflexes were slightly increased. There were no sensory deficits to touch, pain, temper-

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Experimental Gaze Palsies in Monkeys and Their Relation to Uman Pathology

Cited by 134 publications

References 29 publications

A clinical and genetic study in a large cohort of patients with spinocerebellar ataxia type 6

A clinical and genetic study in a large cohort of patients with spinocerebellar ataxia type 6

Slow vertical saccades in the frontotemporal dementia with motor neuron disease

Bilateral medial medullary infarction with oculomotor disorders.

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