Abnormalities of ocular motility in myotonic dystrophy

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Abnormalities of ocular motility in myotonic dystrophy.

Are the oculomotor disturbances in myotonic dystrophy (MD), i.e. reduced smooth pursuit (SP) gain and reduced saccadic peak velocity (PV), of muscular or central origin? To answer this question the following two approaches were used. (i) The performance of SP was compared with the patient's ability to suppress the vestibulo-ocular reflex (VOR) visually (VOR suppression; VOR-S). In the latter ta...

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Ocular motor myotonic phenomenon in myotonic dystrophy.

OBJECTIVE To detect disconjugate ocular motor abnormalities and a possible extraocular muscle myotonic phenomenon in patients with myotonic dystrophy (MyD). METHODS The magnetic scleral search coil technique was used to record monocularly the small (25 degrees ) and large (50 degrees ) saccades, which were paced to two interstimulus intervals (ISIs), one short (1 s), the other long (5 s). The...

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[Management of atrioventricular conduction abnormalities in myotonic dystrophy].

Sudden death is a serious problem in patients with myotonic dystrophy. Atrioventricular blocks (AVBs) and tachyarrhythmias are regarded as a cause of the cardiac sudden death. The increase of QRS width and the deviation of electrical axis are rather important because the main lesion of AVB is located in His-Purkinje system, while the prolongation of PR interval attracts our attention for AVB. T...

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Laboratory abnormalities in patients with myotonic dystrophy type 2.

BACKGROUND Myotonic dystrophy type 2 (DM2) is a recently discovered adult muscular dystrophy. Similar to DM1, this disease causes progressive debilitating weakness, clinical myotonia, and early cataracts and is thought to cause widespread physiologic dysfunction of multiple organ systems. OBJECTIVE To analyze and compile the laboratory abnormalities of patients with DM2. DESIGN Baseline DM2...

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Multimodal Evoked Potential and Eye Movement Abnormalities in Myotonic Dystrophy

By means of multimodal evoked potentials (EPs) we evaluated the central nervous system (CNS) involvement in 25 subjects suffering from myotonic dystrophy (MD): brainstem auditory evoked potentials (BAEPs), middle-latency auditory evoked potentials (MLAEPs) and somatosensory evoked potentials (SEPs) from upper limb were performed on all the subject, whereas only the 19 patients, whose clinical o...

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ژورنال

عنوان ژورنال: Brain

سال: 1997

ISSN: 1460-2156

DOI: 10.1093/brain/120.10.1907