Cases reported "Ocular Motility Disorders"

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1/10. Complete bilateral horizontal gaze paralysis disclosing multiple sclerosis.

    Two women presented with bilateral internuclear ophthalmoplegia evolving in a few days to complete bilateral horizontal gaze paralysis. Convergence and vertical eye movements were normal. Cerebral MRI showed a few small white matter lesions in the lateral ventricle regions, and, at the brainstem level, a single, small, bilateral lesion affecting the posterior part of the medial pontine tegmentum and responsible for the clinical syndrome. The condition gradually improved in both patients, following a similar progression as at the onset: improvement first involved the adduction movements in both eyes, whereas bilateral abduction paresis still persisted for a few weeks, before complete recovery of eye movements. Bilateral damage to the medial longitudinal fasciculus and subsequent lateral extent of damage to the region of the two abducens emerging fibres may explain the clinical findings. In both cases, the cause was probably multiple sclerosis.
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keywords = sclerosis
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2/10. Ocular contrapulsion in multiple sclerosis: clinical features and pathophysiological mechanisms.

    The objective was to describe in multiple sclerosis, a cerebellar eye movement syndrome that resulted from an acute episode of inflammatory demyelination. Contrapulsion is an ocular motor disturbance characterised by a triad of (1) hypermetric saccadic eye movements in a direction opposite from a precisely localised lesion within a specific white matter pathway, the uncinate fasciculus, at the level of the superior cerebellar peduncle (SCP); (2) hypometric saccades towards the side of the lesion; (3) oblique saccades directed away from the side of the lesion on attempted vertical saccades. Infrared oculography was used to demonstrate the characteristic features of contrapulsion in two patients with multiple sclerosis. brain MRI showed lesions within the region of the uncinate fasciculus and superior cerebellar peduncle in both patients. Eye movement recordings showed saccadic hypermetria away from the side of the lesion and saccadic hypometria towards the side of the lesion. The hypometria decomposed into a series of stepwise movements as the eye approached the target. Oblique saccades directed away from the side of the lesion were seen on attempted vertical saccades.In conclusion, ocular contrapulsion can be seen in patients with multiple sclerosis and results from a lesion in the region of the SCP, involving the uncinate fasciculus.
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keywords = sclerosis
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3/10. Ocular flutter associated with a localized lesion in the paramedian pontine reticular formation.

    Ocular flutter is a rare horizontal eye movement disorder characterized by rapid saccadic oscillations. It has been hypothesized that it is caused by loss of "pause" neuronal inhibition of "burst" neuron function in the paramedian pontine reticular formation (PPRF); however, there have been no imaging studies confirming such anatomical localization. We report the case of a woman with an acute attack of multiple sclerosis associated both with ocular flutter and a circumscribed pontine lesion, mainly involving the PPRF on magnetic resonance imaging. As she recovered from the attack, both the midline pontine lesion and the ocular flutter dramatically improved. This case is the first clear evidence that at least some cases of ocular flutter are due to lesions involving the PPRF.
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keywords = sclerosis
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4/10. multiple system atrophy with macrosquare-wave jerks.

    Macrosquare-wave jerks consist of high-amplitude saccadic intrusions occurring mainly during fixation that are observed in several disease entities, e.g., multiple sclerosis or parkinsonian syndromes, but very rarely in patients with multiple system atrophy. We present here a patient with multiple system atrophy exhibiting the oculomotor feature of macrosquare-wave jerks.
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ranking = 0.2
keywords = sclerosis
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5/10. Unilateral reverse ocular bobbing caused by tuberous sclerosis.

    A female aged 12 months with developmental delay and left-sided reverse ocular bobbing (rapid deviation of the eye upward and a slow return to the horizontal position) was observed. At birth, an upper left gingival mass, pathologically diagnosed as a benign granular cell tumour, was removed. On computed tomography (CT), left middle cerebral pedincular and midpontine lesions were seen. Magnetic resonance imaging revealed additional white matter hamartomas, corticosubcortical tubers, and subependymal nodules. The patient was re-examined at 36 months. Her general developmental quotient was equivalent to 23 months of age. Third cranial nerve functions and auditory brainstem response were normal. Her abnormal eye movements were still present. Reverse bobbing is usually observed in patients who are unconscious and who have significant pontine pathology and disruption of the reticular formation. This case is the first tuberous sclerosis-related ocular bobbing case to our knowledge and is interesting as the causative lesion was relatively mild, and ocular horizontal movements were preserved.
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ranking = 1
keywords = sclerosis
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6/10. Minor head trauma and isolated unilateral internuclear ophthalmoplegia.

    Internuclear ophthalmoplegia is a syndrome that develops due to a lesion of the medial longitudinal fasciculus. This lesion is mostly caused by multiple sclerosis (usually bilaterally), and only rarely by head injury. A case is presented of unilateral internuclear ophthalmoplegia as an isolated sequel of minor head trauma, which eventually resolved. A 40-year-old woman with isolated internuclear ophthalmoplegia secondary to closed head trauma with anatomical lesions of the mesencephalon in the region of medial longitudinal fasciculus is described. A minor contusion was detected by magnetic resonance imaging. diplopia resolved in 5 months. In conclusion, internuclear ophthalmoplegia should be considered in the differential diagnosis in patients with recent head injuries showing adduction impairment. The connection between the clinical picture and anatomical lesions is visualized by magnetic resonance imaging.
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ranking = 0.2
keywords = sclerosis
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7/10. Bilateral horizontal gaze palsy in multiple sclerosis.

    Bilateral horizontal gaze palsies are rare. In this case report, we present a 28-year-old woman with bilateral horizontal gaze palsies due to involvement of both paramedian pontine reticular formations (PPRFs) by multiple sclerosis (MS) plaques.
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keywords = sclerosis
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8/10. Involuntary ocular deviations and generalized dystonia in multiple sclerosis. A case report.

    A man with chronic relapsing multiple sclerosis developed involuntary ocular deviations, blepharospasm, torticollis, and chorea of the right arm and head during relapses precipitated by urinary tract infections. An MR scan showed lesions adjacent to the right caudate and lentiform nuclei.
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ranking = 1
keywords = sclerosis
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9/10. multiple sclerosis presenting as Parinaud syndrome.

    The authors report a patient presenting with a 1-week history of paresis of upward gaze as his initial manifestation of demyelinating disease. They stress that: 1) multiple sclerosis can present as Parinaud syndrome (paralysis of upward gaze); and 2) it is important to rule out a non-contour-deforming intraaxial midbrain lesion if a posterior third ventricle or pineal region mass is not identified on screening studies in these patients.
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ranking = 1
keywords = sclerosis
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10/10. Familial childhood primary lateral sclerosis with associated gaze paresis.

    Three children from consanguineous parents began losing the ability to walk in late infancy. Despite chronically progressive weakness leading to wheelchair dependence by adolescence and later loss of motor speech production, intellect remained preserved. Examination revealed upper motor neuron findings of pseudobulbar palsy and spastic quadriplegia, without dementia, cerebellar, extrapyramidal or sensory signs. In addition they exhibited a diffuse conjugate saccadic gaze paresis, especially severe on down-gaze. CT and MRI scans of brain and spinal cord, EEGs, visual and brainstem auditory evoked potentials, CSF examinations, enzyme assays for lysosomal storage diseases, blood amino acids and urine organic acids were all normal. Cortical somatosensory evoked potentials were poorly configured in two of the patients, though they had normal central conduction. EMG showed no signs of denervation. Nerve conduction studies showed normal peripheral motor and sensory conduction velocities. Transcranial magnetic stimulation of the brain elicited no motor-evoked potentials. Despite the lack of neuropathological confirmation, the clinical course and neurophysiologic data strongly support the diagnosis of a familial (autosomal recessive) primary lateral sclerosis (PLS).
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ranking = 1
keywords = sclerosis
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