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Biomedical subjects

I Gottlob

Publications and source records attributed to I Gottlob.

At least 37 records · Page 2Linked to original sources
PubMed2001

Possible role of corticosteroids in nervous system plasticity: improvement in amblyopia after optic neuritis in the fellow eye treated with steroids.

OBJECTIVE: Amblyopia consists of reduced visual function in the absence of organic disease, caused by deficient visual stimulation, most commonly due to squint or refractive error. Amblyopia is thought to be reversible up until the age of approximately 8 years (critical period) and is usually treated with occlusion of the fellow eye. There is recent evidence for visual system plasticity extending beyond the critical period, supported by reports of improvement in visual acuity in the amblyopic eye after loss of vision in the contralateral eye. This suggests that the adult visual system exhibits sufficient plasticity to allow such improvement. We describe here improvement in visual acuity in three amblyopic patients after they received high-dose intravenous glucocorticoids for optic neuritis in the contralateral eye. METHODS: Clinical and neurological evaluation added. RESULTS: In all cases, the improvement was sustained, even after the recovery from the optic neuritis. CONCLUSIONS: Because steroids affect neural plasticity, we hypothesize that they facilitate and enhance visual improvement in amblyopia, a quality that may be tested in future controlled trials.

Adult↗
PubMed2000

Periodic alternating nystagmus in two children with a similar, unusual phenotype.

The report describes two unrelated male children, aged 6 and 8 years, respectively, with congenital periodic alternating nystagmus, congenital strabismus, microcephaly with cortical and cerebellar hypoplasia, mental retardation, low stature, and bat ears. Karyotypes were normal. Neuropediatric and ophthalmologic examinations, radiologic imaging of the brain, and laboratory analyses were performed to exclude other causes of periodic alternating nystagmus, such as ataxia-telangiectasia, acquired disease of the caudal brainstem or the cerebellum, albinism, or loss of vision resulting from cataract or vitreous hemorrhage. The similar morphologic and clinical features of both patients raise the possibility that they have an identical syndrome.

Body Height↗
PubMed2000

Nystagmus.

This report reviews the recent contributions to the field of pathologic nystagmus. The classification and nomenclature of nystagmus with onset in infancy is controversial. Because there are differences in nystagmus forms between patients with idiopathic nystagmus and those with nystagmus associated with afferent sensory defects, a distinction between these two nystagmus types is proposed. The distinctions are also helpful for clinicians, because these separate entities imply different diagnostic evaluations and visual prognosis. Recent studies have confirmed that periodic alternating nystagmus is detected more easily if the patient is evaluated for a longer time period and occurs more commonly than previously thought. Psychophysical investigations indicate that extraretinal signals play an important role in suppression of oscillopsia in infantile nystagmus. Genetic analysis recently has allowed identification of genes of X-linked idiopathic nystagmus and achromatopsia.

Evoked Potentials, Visual↗
PubMed2000

Arnold-Chiari malformation and nystagmus of skew.

The Arnold-Chiari malfomation is typically associated with downbeat nystagmus. Eye movement recordings in two patients with Arnold-Chiari malfomation type 1 showed, in addition to downbeat and gaze evoked nystagmus, intermittent nystagmus of skew. To date this finding has not been reported in association with Arnold-Chiari malfomation. Nystagmus of skew should raise the suspicion of Arnold-Chiari malfomation and prompt sagittal head MRI examination.

Adult↗
PubMed1999

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.

Bardet-Biedl Syndrome↗
PubMed1999

[Eye movement abnormalities as a sign for the diagnosis in Niemann-Pick disease type C].

BACKGROUND: Eye movement abnormalities in familial mental retardation syndrome should lead to the suspicion of a storage disorder, including Niemann Pick disease type C, Gaucher's disease, abetalipoproteinemia and Wilson's disease. The eye movement abnormalities in our two patients were suggestive of Niemann Pick disease type C, characterized by initial loss of voluntary vertical eye movements and subsequent loss of horizontal eye movements, with preservation of the vestibulo-ocular response. The characteristics of eye movements in storage disorders are different. In Gaucher's disease a progressive horizontal gaze palsy, in abetalipoproteinemia a particular type of internuclear ophthalmoplegia with nystagmus of the adducting eye and in Wilson's disease slowing of saccades may be observed. PATIENTS: We evaluated two mentally retarded sisters with unclear diagnosis at the age of 34 and 27 years. At the age of 24 and 21 a vertical gaze palsy led to the diagnosis of Parinaud syndrome. RESULTS: At the time of our examination both sisters were unable to perform voluntary horizontal or vertical saccades or pursuit eye movements. The vestibulo-ocular reflex was present in all directions. Optokinetic nystagmus and convergence were absent. This clinical picture led us to a suspicion of Niemann-Pick disease type C, confirmed by the presence of sea-blue histiocytes in the bone marrow biopsy. CONCLUSION: These cases demonstrate that the pattern of eye movement disorders in some syndromes associated with mental retardation can give important clues in the determination of the diagnosis.

Adult↗
PubMed1999

The detection, prevention, and rehabilitation of amblyopia.

Some of the past year's important papers on amblyopia are reviewed. Preschool screening for amblyopia is still controversial in regard to its effectiveness, the best age, and the most cost-effective method. In Sweden, where screening for amblyopia is well established, the prevalence of deep and moderate amblyopia has been markedly reduced. Several studies in the past year underscored the importance of anisometropia as a risk factor for amblyopia. Clear evidence for the importance of early treatment of unilateral cataracts in the first 8 weeks of life has been shown. Cytidine-5'-diphosphocholine administration has been shown to positively influence amblyopia and represents, in addition to levodopa, another promising pharmacologic treatment.

Amblyopia↗
PubMed1999

Rod monochromatism and blue cone monochromatism: pupillary, accommodative and convergence reactions to darkness.

PURPOSE: To elucidate the mechanism of paradoxical pupillary constriction to darkness (PPCD) and the clinical characteristics facilitating this phenomenon. METHODS: Six rod monochromats, three blue cone monochromats, with three obligate BCM carriers, and ten age matched controls were studied. Pupillary responses, refractions and eye positions were measured with an infrared refractometer, with and without background room lighting while being simultaneously recorded on VHS (infrared) video tape from onset of darkness. RESULTS: Only rod monochromats displayed typical paradoxical pupillary responses. Blue cone monochromats and obligate BCM carriers showed reduced pupillary contraction, compared to controls, but no paradoxical pupillary reaction. Changes neither in accommodation nor convergence were found during paradoxical pupillary constriction to darkness in our rod monochromats. CONCLUSIONS: Paradoxical pupillary constrictions to darkness were seen in rod monochromats but were found not to be age or gender related. This constriction was accompanied neither by accommodation nor convergence changes. No significant differences in pupillary responses to darkness were observed in BCMs nor their carriers, although pupillary dilation to darkness seemed slightly impaired as compared to normals.

Accommodation, Ocular↗
PubMed1999

Visual development in preterm and full-term infants: a prospective masked study.

PURPOSE: To compare development of visual acuity and binocular vision in preterm and full-term infants in a prospective study that used testers masked to subject's gestational age. METHODS: Seventy-nine healthy full-term infants, mean gestational age 40 weeks, and 18 low-risk preterm infants, mean gestational age 33 weeks, were examined biweekly between the 44th and 54th weeks of postmenstrual age. Ocular alignment, convergence, fusion, grating acuity, and onset of optokinetic nystagmus (OKN) were assessed at each examination. RESULTS: The mean postnatal ages of onset of ocular alignment, convergence, fusion, grating acuity to 1.6 cycles per degree, and OKN from temporal to nasal and nasal to temporal were, respectively, 5, 7, 7, 11, 6, and 9 weeks for the full-term and 12, 13, 14, 18, 13, and 16 weeks for the preterm infants. The mean postmenstrual ages of onset for the corresponding parameters were 46, 48, 48, 51, 46, and 50 weeks for full-term and 46, 47, 48, 52, 47, and 49 weeks for preterm infants. The onset of all parameters was earlier in full-term infants than in preterm infants of the same postnatal age (P < or = 0.0001). However, no differences were found when the parameters were compared at postmenstrual ages. CONCLUSIONS: Additional visual experience of preterm infants does not influence development of visual acuity or binocular vision during the first months of life as measured from the time of conception.

Aging↗
PubMed1998

The development of visual pursuit during the first months of life.

BACKGROUND: There are few previous investigations of smooth pursuit in infants. The aim of our study was to quantify visual pursuit in infants between 1 day and 16 weeks of age. METHODS: Eye movements of 97 healthy infants between 1 day and 16 weeks of age were recorded one to seven times with infrared photo-oculography. For stimulation of visual pursuit a square of 9.4 deg of visual angle with vertical gratings moved horizontally at a constant velocity of 7.5 deg/s. RESULTS: In the first 2 weeks of life, segments of smooth pursuit were measured with a maximum velocity of 7.93 deg/s, with a maximum gain of 1.06 and a maximal duration of 3.16 s. In sequential recordings no significant increases of velocity, gain or duration were found. However, the total time the subjects followed the stimulus with smooth plus saccadic pursuit increased significantly with age (from a median of 39.0% to a median of 61.5% of examination time). CONCLUSION: This study clearly demonstrates that smooth pursuit is already present in the first week of life. We found no significant increase in velocity, gain and duration of smooth pursuit segments in the first 16 weeks of life with our recording technique. However, the total pursuit time, reflecting attention, increased with age. The ocular machinery to drive pursuit appears to be in place at birth and seems not to be influenced by increased attention in the first months of life.

Aging↗
PubMed1998

Nystagmus.

This article reviews some of the past year's important papers, with emphasis on early onset and acquired neurological nystagmus. Advances in understanding the mechanisms of suppression of oscillopsia, the evolution of nystagmus, and the treatment of periodic alternating nystagmus and of nystagmus in albinism have been made in early-onset nystagmus. Successful pharmacological treatment for acquired neurological nystagmus has been demonstrated with the gamma aminobutyric acid agonist gabapentin and with memantine, a glutamate antagonist.

Excitatory Amino Acid Antagonists↗
PubMed1997

A case of Erdheim-Chester disease with orbital involvement.

The Erdheim-Chester disease is a rare idiopathic, systemic, histiocytic disorder. To our knowledge, ocular involvement has been reported in only 16 cases. We describe a 55-year-old man who had symmetrical exophthalmos and several skin nodules on the arms and trunk. A magnetic resonance imaging scan confirmed the presence of bilateral, intraconal, retrobulbar tumors. An examination of the histopathologic features of orbital and skin biopsy specimens revealed xanthogranulomatous infiltrate with Touton giant cells. Further systemic investigations showed bone and retroperitoneal involvement. Three years later, multiple eyelid xanthelasmas developed in the patient. These findings are consistent with the diagnosis of the Erdheim-Chester disease. The patient's condition is stable under therapy with low-dose corticosteroids. His survival is longer than usually described in the literature.

Bone Diseases↗
PubMed1997

Infantile nystagmus. Development documented by eye movement recordings.

PURPOSE: To report on the development of infantile nystagmus in a patient's first year of life. METHODS: A case study using consecutive photo-oculographic and electro-oculographic eye movement recordings in the subject ranging between 1 and 12 months of age. RESULTS: Although no nystagmus was present at 5 weeks of age, square-wave jerks were recorded at 7 weeks, and a small pendular nystagmus was recorded at 8 weeks. At 10 weeks of age, evaluation revealed predominantly larger jerk-type nystagmus with increasing and decreasing exponential velocities of the slow phase. After 14 weeks of age, the nystagmus became smaller and was predominantly pendular. Between 7 and 12 months of age, binocular electro-oculography recordings showed conjugate pendular nystagmus typical of infantile nystagmus. CONCLUSION: This is the first report documenting that, at least in some forms of infantile nystagmus, eye movement abnormalities are not present at birth. Before the development of the typical pattern of infantile nystagmus waveforms (that is, conjugated pendular or jerk-type nystagmus with increasing exponential velocity slow phases), saccadic abnormalities (square-wave jerks) and jerk-type nystagmus with increasing as well as decreasing velocities were observed.

Electrooculography↗
PubMed1996

Head and eye movements in children with low vision.

BACKGROUND: Early childhood nystagmus may herald blindness, brain tumors, benign idiopathic motor nystagmus or spasmus nutans. Nystagmus unique to low vision was sought. METHODS: Videotapes and head/ eye movements of 18 congenitally visually impaired subjects were analyzed. RESULTS: Nystagmus of congenitally visually impaired subjects was characterized as small horizontal or vertical movements superimposed on larger oscillations. Small and large nystagmus movements were of pendular or jerk type. Slow-phase jerk nystagmus had increasing, constant and decreasing velocities. Fast-phase nystagmus changed direction periodically. Pendular nystagmus was out of phase and evolved to jerk nystagmus in two subjects. All subjects displayed head nodding, and one stabilized gaze with head movements. CONCLUSION: Eye movement recordings allow characterization and differentiation of subjects with nystagmus and low vision from other nystagmus forms.

Child↗
PubMed1996

Elevation deficit caused by accessory extraocular muscle.

PURPOSE: To report an elevation deficit, which was caused by an accessory extraocular muscle, in a 6-year-old boy. METHODS: Computed tomography and magnetic resonance imaging were used to confirm an accessory, fusiform, well-defined, solid structure in the retrobulbar space. RESULTS: A supernumerary intraconal muscle was detected between the annulus of Zinn and the posterior part of the left globe. CONCLUSION: This rare anomaly may represent an atavistic retractor bulbi muscle.

Child↗
PubMed1996

Vertical Duane's retraction syndrome.

PURPOSE: We report three patients with a rare variant of Duane's retraction syndrome. METHODS: Eye movements were recorded by electro-oculography. One patient underwent electro-myography. RESULTS: All patients showed V-incommitance and twitch abduction on vertical saccades. Electromyography showed lateral rectus muscle firing activity during upgaze and downgaze. CONCLUSION: Our patients showed synergistic innervation between the lateral rectus muscle and ipsilateral vertical acting muscles documented by electromyography.

Adolescent↗