Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0028738 (nystagmus)
7,431 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This study maps the eye movements evoked by microstimulations in the medulla of 9 alert cats. Trains of square waves (20 microA amplitude, 0.2 msec duration, 200 Hz) were delivered through glass-covered tungsten microelectrodes (0.5-1 M omega). Movements of both eyes were recorded by the magnetic field/eye coil technique. Stimulation of the prepositus hypoglossi nucleus (PH) evoked nystagmus with ipsilaterally-directed slow phases followed by after-nystagmus with contralaterally-directed slow phases. Stimulation of the medial vestibular nucleus (MVN) induced nystagmus whose slow phases were more often ipsilaterally-directed but at time contralaterally-directed. At nearly all sites where we stimulated the reticular formation underlying prepositus and vestibular nuclei (from P4.5 to P12), we recorded versional conjugate movements. They were most often ipsilaterally-directed. Some microstimulations in the region of the medial longitudinal fasciculus evoked recentering eye movements: regardless of the initial position of the gaze (to the left as well as to the right), microstimulations given at the same place induced a movement of both eyes toward their neutral position. The amplitude of this movement was proportional to the eccentricity of the pre-stimulation position of the gaze.
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PMID:Eye movements evoked by microstimulations in the brainstem of the alert cat. 279 72

Wolfram's syndrome is defined by the association of diabetes mellitus, diabetes insipidus, optic atrophy and nerve deafness. Other neurological anomalies, such as ataxia, nystagmus, tonic pupil, dizziness, dysarthria, dysphagia and epilepsy are rarely described and tend to appear later than the primary manifestations. We describe a patient with Wolfram's syndrome whose magnetic resonance image (MRI) of the head showed brainstem and cerebellar atrophy years before the appearance of clinical signs of brainstem disfunction. We conclude that alterations in MRI precede neurological symptoms by several years in Wolfram's syndrome.
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PMID:[Wolfram's syndrome: correlation of clinical signs and neurological images]. 769 38

Wolfram's syndrome, also known as DIDMOAD syndrome, includes juvenile diabetes mellitus and optic atrophy variously associated with diabetes insipidus and deafness. We describe the neurological findings in 5 patients with Wolfram's syndrome. All patients had a neurological examination and were subjected electrophysiological and brain imaging including CT scan and, in one patient, MRI. There were two pairs of brothers and a sporadic case with paternal consanguinity suggesting recessive inheritance. Neurological abnormalities were found in four patients including dysarthria, seizures, anosmia, nystagmus, ataxia and changes in the electroencephalograms, electroretinograms and evoked potentials. In contrast with previous reports, four patients had abnormal brain CT scan with prominent atrophy of the brainstem. In the patient studied with NMR, severe brainstem and cerebellar atrophy was found. These neuroradiological findings are reminiscent of those described in olivopontocerebellar atrophy and are in agreement with previous pathological studies. We conclude that Wolfram's syndrome includes phenotypical manifestations of olivopontocerebellar atrophy. This reinforces the opinion that olivopontocerebellar atrophy is a nonspecific syndrome of varied causes.
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PMID:[Neurologic manifestations in Wolfram's syndrome]. 833 58