Cases reported "Nystagmus, Pathologic"

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1/59. Voluntary nystagmus associated with accommodation spasms.

    BACKGROUND: Voluntary nystagmus has been recognized as a pendular, rapid, conjugate, primarily horizontal, benign eye movement initiated and maintained by voluntary effort. CASE: A 10-year-old Japanese girl presented with voluntary nystagmus associated with accommodation spasms. Her chief complaints, intermittent blurred vision, headache, and soreness of the eyes, were thought to be related to the voluntary nystagmus and accommodation spasms. FINDINGS: The waveform of the nystagmus appeared pendular, the frequency was 13-15 Hz, and the amplitude was 3-5 degrees. Scanning laser ophthalmoscopic video images clearly demonstrated vertical and torsional components in addition to the horizontal eye movements. Her refraction was unstable, varying between -0.5 diopters (D) and -5.5 D, and the recording of the accommodometer increased to -12.0 D when nystagmus was initiated. CONCLUSIONS: This may be a unique form of voluntary nystagmus that consists of horizontal, vertical, and rotational components associated with accommodation spasms. observation of this patient continues, without any further treatment or examination.
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ranking = 1
keywords = spasm
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2/59. Nystagmus mimicking spasmus nutans as the presenting sign of bardet-biedl syndrome.

    PURPOSE: To investigate the nystagmus of twin brothers presenting with spasmus nutans later diagnosed as bardet-biedl syndrome. methods: The twins presented at the age of 14 months with a presumed diagnosis of spasmus nutans. They were followed clinically and with quantitative electro-oculographic eye movement recordings until the age of 6 years. RESULTS: polydactyly, truncal obesity, mild delay in cognitive development, visual acuity of 20/100, attenuated retinal vessels and pale disks, and bilaterally almost extinguished scotopic and photopic electroretinograms were found in both brothers. They had fine, fast, pendular, disconjugate, intermittent, oblique nystagmus. No head nodding was observed. CONCLUSION: As described in patients with other retinal diseases such as achromatopsia and congenital stationary night blindness, nystagmus of patients with bardet-biedl syndrome can mimic spasmus nutans.
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ranking = 6805.9681458455
keywords = spasmus nutans, spasmus, nutans, spasm
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3/59. Preservation of the brainstem auditory evoked potential in non-convulsive status epilepticus.

    Brainstem auditory evoked potentials (BAEPs) were recorded from a patient simultaneously experiencing non-convulsive generalized status epilepticus (NGSE). Waves I, III and V were normal but all subsequent waves were absent. This finding indicates that structures within the brainstem adjacent to the generators for the BAEP are likely not affected by NGSE and also illustrates the resilient nature of the BAEP. This is the first report of the recording of an evoked potential during a naturally occurring generalized seizure.
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ranking = 0.036933813865166
keywords = seizure
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4/59. Central nystagmus induced by deep-brain stimulation for epilepsy.

    PURPOSE: The goal of the present study was to describe the localization of central nystagmus induced as a side effect of electrical deep-brain stimulation for epilepsy. methods: Bilateral deep-brain stimulating electrodes were inserted in the centromedian nucleus of the thalamus to control seizures in a patient with intractable epilepsy. RESULTS: Cathodal high-frequency stimulation through the deepest contact of each electrode elicited cycles of slow ipsiversive conjugate eye deviations, each followed by rapid contralateral jerks. The involved electrode contacts were situated at the mesodiencephalic junction just inferior to the centromedian nucleus of the thalamus and rostral to the superior colliculus. Right-sided stimulation evoked left beating nystagmus and left-sided stimulation evoked right beating nystagmus. Stimulation through other electrode contacts did not induce nystagmus. electronystagmography showed the nystagmus to have constant velocity slow phases. CONCLUSIONS: A central nystagmogenic area exists in humans that appears to be homologous to the nucleus of the optic tract, a region described in nonhuman primates to play a role in the generation of optokinetic nystagmus.
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ranking = 0.036933813865166
keywords = seizure
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5/59. Epileptic nystagmus: electroclinical study of a case.

    Epileptic nystagmus (EN) is a rare form of nystagmus that occurs only during epileptic seizures. We report a case in which EN was first noted in an 8 year-old boy. Neuro-imaging was normal. Sharp waves from the left occipital lobe characterised the interictal EEG. Ictal video-EEG showed the aspects of electric seizures during clinical manifestations e.g. nystagmus. Ambulatory EEG displayed numerous diurnal and nocturnal seizures, but exclusively in REM sleep. After two weeks of sodium valproate treatment, the seizures, EEG focus spikes and nystagmus, as well as the squint, disappeared. During a two-year follow-up the child had no further seizures, EEG was normal and the school performance was unaffected. This case has the main features of benign epilepsy, although there are unusual features such as epileptic nystagmus, permanent squint, reduction of EEG paroxysmal abnormalities during NREM, and the presence of seizures during REM sleep. The observation and the significance of EN are discussed with reference to the literature.
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ranking = 0.221602883191
keywords = seizure
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6/59. Epileptic nystagmus: a case study video-EEG correlation.

    Epileptic nystagmus (EN) is an uncommon phenomenon characterized by repetitive and rapid saccades, in association with epileptic discharges. We present a critical video-EEG recording in a patient with occipital seizures that appeared clinically as EN. The subject, male, 70 years-old, was examined because of generalized tonic-clonic seizures, preceded by left cephalic version. These were controlled using i.v. PHT, but partial seizures persisted, which we recorded using video-EEG. Clinically, we observed episodes of left conjugate deviation of the eyes, accompanied by horizontal nystagmoid movements, with a rapid leftward component and visual hallucinations. The patient did not lose consciousness. Ictal EEG: spike rhythm in the posterior right occipito-temporal region extending to adjacent and contralateral regions, followed by post-discharge of slow waves. The video-EEG was interpreted as partial oculo-clonic status epilepticus of right temporo-occipital origin. Cranial MRI: old, bilateral hemorrhaging occipital contusions associated with previous cranial injury. The picture persisted for two days, and disappeared with administration of CBZ 600 mg/d. Our patient's nystagmus seemed to be related to the critical activity recorded in the right occipito-temporal region. The co-existence of visual hallucinations and the video-EEG correlation support this possibility. This phenomenon is probably due to epileptic activation of the cortical center of saccadic movements, with a rapid phase of nystagmus, contralateral to the focus, and a slow ipsilateral phase in association with a defect in the gaze-fixing system ("leaky neural integrator") [published with videosequences].
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ranking = 0.1108014415955
keywords = seizure
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7/59. Epileptic monocular nystagmus.

    The authors describe a case of epileptic monocular nystagmus in a cognitively intact adult with normal vision. Focal seizures originated in the occipital lobe contralateral to the involved eye, and an associated structural lesion was thought to represent a forme fruste of sturge-weber syndrome. It is hypothesized that the seizure discharge either activated a cortical saccade region and caused simultaneous supranuclear inhibition of ipsilateral eye movement or triggered monocular eye movement commands.
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ranking = 0.073867627730332
keywords = seizure
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8/59. Opisthotonic posturing with neuromuscular irritability attributable to 4-aminopyridine ingestion by a healthy pediatric patient.

    INTRODUCTION: 4-aminopyridine (4-AP) is a potassium channel blocker used to increase muscle strength in the treatment of demyelinating diseases such as multiple sclerosis. We describe a case of ingestion by an 8-month-old child that resulted in severe but transient symptoms. CASE REPORT: An 8-month-old boy was found with greenish saliva, and a capsule with green 4-AP powder was missing. On arrival to an emergency department, he was jittery, tachycardic, and tachypneic. Activated charcoal, a cathartic, and midazolam (0.5 mg/kg) were administered before transfer to a tertiary pediatric hospital. On arrival, the infant remained tachycardic and tachypneic. His eyes deviated upward and he was noted to have 3 deep tendon reflexes bilaterally. He was administered 0.9% normal saline (20 mL/kg) for a wide pulse pressure with low diastolic blood pressure. The patient developed dramatic opisthotonic posturing and vermiform tongue fasciculations. The symptoms responded well to repeated intravenous doses of benzodiazepines. In this case, we used 2 doses of lorazepam (0.05 mg/kg each). During opisthotonic posturing, an electroencephalogram performed in the intensive care unit revealed no evidence of seizure activity. Within 20 hours after admission, the patient became asymptomatic. CONCLUSION: This case is, to our knowledge, the first documented pediatric 4-AP ingestion. Clinical signs and symptoms are described as well as the response to therapy with benzodiazepines. The electroencephalogram performed while the patient was symptomatic was negative for seizures.
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ranking = 0.073867627730332
keywords = seizure
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9/59. Epileptic nystagmus and its possible relationship with PGO spikes.

    A simultaneous video-EEG on a waking 6-year-old male revealed rapid horizontal and then vertical eye movements and 10 sec later showed ictal rhythms maximal on the occipital areas, quickly spreading to all other areas. A second ictal event during wakefulness was very similar to the first. During sleep interictal discharges were seen from the right frontal-temporal area and one more ictal event was noted. This latter seizure in the NREM sleep record did not show any eye movements, but showed ictal activity on the right frontal-temporal area, which later became generalized. We propose that the interictal discharges on the right frontal-temporal area likely arose from the amygdala, which activated the pontine nuclei responsible for PGO (ponto-geniculo-occipital) spikes and the rapid eye movements seen in our patient. The PGO spikes activated the occipital areas, which then showed clear ictal rhythms to complete the sequence of events. This case demonstrates a sequence of rapid eye movements without ictal patterns, followed by seizure rhythms, but may still be an example of "epileptic nystagmus," assuming that the eye movements arose from an ictal activation of the deep subcortical portion of the PGO system.
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ranking = 0.073867627730332
keywords = seizure
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10/59. Latent nystagmus and acquired pendular nystagmus masquerading as spasmus nutans.

    SUMMARY: We used ocular motility recordings to identify the characteristics of a rare combination of conjugate, horizontal jerk, and pendular nystagmus in a 9-year-old boy. The clinical diagnoses were amblyopia, left esotropia, congenital nystagmus, and an apparently uniocular pendular nystagmus that mimicked spasmus nutans. Ocular motility recordings revealed an unusual latent/manifest latent nystagmus, pendular nystagmus with characteristics of an acquired nystagmus, and uniocular saccades. The ocular motor data identified clinically unrecognized types of nystagmus and suggested that the pendular nystagmus was acquired in infancy rather than as a result of failure to develop good vision or binocularity. The presence of uniocular saccades adds to the mounting evidence that individual control for each eye exists in humans.
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ranking = 4861.4058184611
keywords = spasmus nutans, spasmus, nutans, spasm
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