Cases reported "Nystagmus, Pathologic"

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1/72. Disorders in cerebellar ocular motor control. II. Macrosaccadic oscillation. An oculographic, control system and clinico-anatomical analysis.

    A distinctive cerebellar ocular motor disorder, macrosaccadic oscillation, evolved simultaneously with an acute cerebellar syndrome in 4 patients, 2 with haemorrhagic metastatic melanoma deep in the vermis, a third with a presumed cerebellar haematoma and a fourth with focal demyelinating disease. Ocular oscillations were conjugate, horizontal, symmetrical, occurred in bursts of several seconds duration, had amplitudes of 30 degrees to 50 degrees, and were evoked whenever the patient attempted to shift visual fixation or pursue a moving target. Photo-electric recordings in one patient with tumour defined features of this disorder of saccadic eye movement: (i) oscillation was composed of saccades, (ii) frequency was 2 Hz, (iii) bursts occurred with amplitude first increasing and then decreasing, (iv) intervals between beginnings of saccades averaged 260 ms and (v) eye position did not exhibit systematic drift during the intersaccadic period. These features documented the inreased gain and instability of the visually guided saccadic system. By using increased feed-forward gain in a sampled-data control model we simulated the pattern of macrosaccadic oscillation. We belive that the acute loss of the calibrator function of the cerebellum accounts for the gain abnormality underlying macrosaccadic oscillation.
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2/72. Neuro-ophthalmic, radiographic, and pathologic manifestations of adult-onset alexander disease.

    A 61-year-old woman had a 3-year history of imbalance. Eye movement studies revealed square-wave jerks, gaze paretic nystagmus, rebound nystagmus, impaired smooth pursuit, impaired optokinetic nystagmus, and abnormal fixation suppression of vestibular nystagmus. A brain magnetic resonance imaging study showed extensive areas of increased signal from the middle cerebellar peduncles and dentate nuclei, which enhanced with gadolinium. Histopathological analysis of a needle biopsy specimen of the left cerebellar peduncle revealed diffuse gliosis in the presence of symmetrically distributed areas of demyelination. There were associated Rosenthal fibers. Clinicopathologic correlation supported a diagnosis of alexander disease. An adult patient with a history of progressive imbalance, ocular motility abnormalities consistent with cerebellar and/or brainstem dysfunction, and diffuse, symmetric hyperintense magnetic resonance imaging signals in brainstem and cerebellar white matter should suggest a diagnosis of adult-onset alexander disease.
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keywords = wave
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3/72. Hypotonia, congenital nystagmus, ataxia, and abnormal auditory brainstem responses: a report on the first white patient.

    A white Italian boy, aged 5 years and 8 months, is reported with failure to thrive, hypotonia, truncal ataxia, psychomotor retardation, and congenital horizontal pendular nystagmus with only waves I and II on auditory brainstem responses. Our patient's clinical picture resembles that previously reported in 10 male Oriental patients. He did not manifest spastic diplegia by the age of 2 years, as did the subjects reported in the literature, but knee-jerk hyperreflexia was evident at the most recent clinical reevaluation. Serial brain MRI studies revealed a cystic brain lesion and peritrigonal hyperintensities with no brainstem abnormalities. To date, no other child with a similar syndrome has been described either in europe or in America. The clinical features of this condition are consistent and characteristic. A definitive diagnosis is achieved by demonstrating the absence of all waves following wave I or wave II on auditory brainstem responses as early as 3 months of age. Due to the predominance of males, the occurrence in siblings, the early age at onset, the non-progressive course, and the characteristic auditory brainstem response findings, the syndrome may have a genetic origin and be attributable to a dysgenetic brainstem lesion.
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4/72. 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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5/72. Anti-labyrinthine antibodies in a patient with relapsing polychondritis.

    Relapsing polychondritis is a rare inflammatory disease that causes destruction of cartilaginous tissue in various anatomical regions. We report here about a 55-year-old female patient with relapsing polychondritis that involved the right auricle, both audiovestibular organs and both eyes. The patient presented with persisting inflammation of the right auricle, sudden lower-frequency hearing loss, acute moderate vertigo with nausea and mild ocular symptoms. Immunofluorescence assays were used for the detection of antibodies against the cochlea and the vestibular organ and demonstrated the presence of circulating antibodies against the audiovestibular organ. No staining for anti-corneal IgG was detected. Improvement of clinical disease was achieved by treatment with systemic steroids and vasodilator drugs, and long-term medication with low-dose corticosteroids.
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6/72. The incidence and waveform characteristics of periodic alternating nystagmus in congenital nystagmus.

    PURPOSE: To investigate the incidence and waveform characteristics of periodic alternating nystagmus, (PAN) in congenital nystagmus (CN). methods: In a prospective study, 18 patients with CN without associated sensory defects agreed to undergo eye movement documentation using binocular infrared oculography. Two of the 18 had a diagnosis of suspected PAN before entering the study. The patients sat in a dimly lit room and viewed an LED (4 min in diameter) located in the primary position, at a distance of 100 cm. During an 8-minute recording, patients were read a story of neutral interest to hold attention at a constant level. PAN was defined as a left-beating nystagmus, a transition phase, a right-beating nystagmus, and a final transition phase; the sequence was then repeated. RESULTS: Seven of the 18 patients had PAN (median cycle: 223 seconds, range 180-307 seconds). The periodicity of the cycles for each adult patient was regular, although the phases within a cycle were often asymmetric. Six of the seven patients had an anomalous head posture (AHP), and in five the AHP was in only one direction. Except for one patient, the PAN waveforms had an increasing slow-phase velocity in at least one phase of the cycle; in the other phase they were linear. CONCLUSIONS: The occurrence of PAN in CN is not as rare as previously thought and can be missed because of the long cycles and the use of only one AHP. The AHP was dependent on, and could be predicted from, the waveforms containing the longest foveation times. Although the waveforms and foveation times may differ among the phases of the PAN cycle, the periodicity of the cycle was usually regular and therefore predictable. Identification of PAN is essential in cases in which surgical treatment is considered for correction of AHPs.
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ranking = 52.497416913944
keywords = wave
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7/72. 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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keywords = wave
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8/72. Ocular motor signs in an infant with carbohydrate-deficient glycoprotein syndrome type Ia.

    PURPOSE: To document the evolution of ocular motor abnormalities in an infant with carbohydrate-deficient glycoprotein syndrome. methods: Case report. An infant with carbohydrate-deficient glycoprotein syndrome type 1a underwent magnetic resonance imaging and infrared eye movement recording. RESULTS: A 10-month-old male with carbohydrate-deficient glycoprotein syndrome type Ia had rapid horizontal oscillations of the eyes when startled or awakened from sleep. Clinical examination confirmed this finding and disclosed congenital ocular motor apraxia with a reduced vestibulo-ocular reflex. Infrared eye movement recording showed ocular flutter and square wave jerks superimposed on a horizontal pendular nystagmus. magnetic resonance imaging showed diffuse cerebellar hypoplasia. CONCLUSION: Carbohydrate-deficient glycoprotein syndrome type Ia can be associated with multiple cerebellar eye signs including ocular flutter, square-wave jerks, and congenital ocular motor apraxia.
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ranking = 13.124354228486
keywords = wave
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9/72. 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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10/72. Spontaneous reversal of nystagmus in the dark.

    AIM: To report five children with horizontal jerk nystagmus in whom eye movement recordings in the dark revealed a spontaneous reversal in the direction of the nystagmus beat. Three patients were blind in one eye and were diagnosed as having a manifest latent nystagmus (MLN), and two patients had strabismus and congenital nystagmus (CN). methods: eye movements were recorded using DC electro-oculography with simultaneous video recording, including infrared recording in total darkness. RESULTS: Four patients had decelerating velocity slow phase jerk nystagmus when recorded under natural lighting conditions; the fifth case had accelerating velocity and linear slow phase jerk nystagmus. Under absolute darkness, nystagmus reversed in direction of beat with a mixture of linear and decelerating velocity slow phase waveforms. One child with unilateral anophthalmos could wilfully reverse the beat direction of his nystagmus by trying to look with his blind eye in the light and dark. CONCLUSIONS: These observations support the theory that LN/MLN beat direction is determined by the "presumed" viewing eye and may be consciously controlled. The spontaneous reversal of beat direction in the dark suggests eye dominance is predetermined. Eye movement recordings identified mixed nystagmus waveforms indicating that CN (accelerating velocity slow phases) and LN/MLN (linear/decelerating velocity slow phases) coexist in these subjects.
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keywords = wave
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