11/198. 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.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
12/198. Periodic alternating nystagmus and congenital nystagmus: similarities in possibly inherited cases.In this paper, 2 patients, a daughter with periodic alternating nystagmus and a mother with congenital fixation nystagmus, are presented, and the similarities of the two disorders are discussed, not only in the eye movements, but also in their underlying abnormalities. The literature was reviewed, and the link between periodic alternating and congenital fixation nystagmus, which had been suggested based on circumstantial evidence, may be confirmed by our cases. The possible evidence of inheritance suggests that the two disorders share common abnormalities in visual processing.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
13/198. Malignant pilocytic astrocytoma in the medulla oblongata: case report.A 27-year-old woman visited our hospital with chief complaints of abducens nerve palsy and cerebellar symptoms. On computerized tomographic scanning and magnetic resonance imaging, a tumor with strong enhancement was found on the dorsal side of the medulla oblongata. A tumor was excised by suboccipital craniotomy and C1 laminectomy. Histologically, many Rosenthal fibers together with pilocytic tumor cells were found in some regions, but a very high Ki-67 labeling rate accompanied by cells with nuclei of irregular size and giant cells was observed in other regions. The tumor was diagnosed as malignant pilocytic astrocytoma originating from pilocytic astrocytoma by transformation. The biological behavior of pilocytic astrocytoma is obscure in several respects. We report our experience of a case of malignant pilocytic astrocytoma that developed in the brain stem and progressed extremely rapidly.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
14/198. Successful chemotherapeutic treatment of diencephalic syndrome with continued tumor presence.A 7-month-old infant with typical features of diencephalic syndrome (DES) associated with a hypothalamic mass, most probably a glioma, was treated with chemotherapy. The tumor showed clear shrinkage, but after more than 2 years regrowth was noted. During the treatment period the child regained normal growth and became free of symptoms. As radiation therapy, especially at a young age, has significant adverse effects and a neurosurgical approach to the diencephalic region also has the potential to cause significant sequelae, a chemotherapeutic option, when it exists, is preferred. Thus, in an infant in whom a glioma is suspected to be the cause of the DES, based on the clinical picture and the neuroimaging appearance, chemotherapy should be considered the primary therapeutic modality. Even if its effect is temporary, its use is well justified. The most appropriate treatment protocol still needs to be determined.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
15/198. Periodic alternating nystagmus and vestibulo-spinal system facilitating activity.OBJECTIVE: Periodic alternating nystagmus has been associated with the instability of the velocity storage mechanism, which is known to play an important role in both the vestibulo-oculomotor and the optokinetic systems. In the present study we looked for a possible spinal equivalent to PAN. methods AND RESULTS: In 3 PAN patients, the h-reflex amplitude proved to be slightly but significantly influenced by nystagmus direction, in that it was greater when the nystagmus was beating toward the stimulation side. CONCLUSIONS: This finding suggests that projections from velocity storage may play a role not only in the ocular motor but also in assisting postural stability through the vestibulo-spinal system.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
16/198. Downbeat nystagmus associated with intravenous patient-controlled administration of morphine.IMPLICATIONS: This case documents a patient who developed dizziness with downbeating nystagmus while receiving a relatively large dose of IV patient-controlled analgesia morphine. Although there have been case reports of epidural morphine with these symptoms and signs, this has not been previously documented with IV or patient-controlled analgesia morphine.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
17/198. Osteopetrorickets: case report.We report the case of a baby girl who presented with rickets at 3 months. At the age of 5 months she was readmitted because of nystagmus and a diagnosis of osteopetrosis was made on the basis of clinical and radiological findings. rickets is a paradoxical feature of osteopetrosis resulting from inability to maintain a normal calcium-phosphorus balance. In our patient the onset of rickets before other symptoms of osteopetrosis suggests a primary defect. Conclusion: It is possible that patients with osteopetrosis and rickets (osteopetrorickets) represent a different mutation like the osteopetrosis mouse, which is the only animal mutation with rickets.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
18/198. Downbeating nystagmus and postural hypotension due to basilar invagination.Downbeating nystagmus is an involuntary vertical rhythmic eye movement with the fast component in the downward direction. The sign indicates a craniocervical disorder. The most common cause is the arnold-chiari malformation, followed by cerebellar degeneration. Basilar invagination is a rare cause of downbeating nystagmus. However, with appropriate treatment its prognosis is good. Here, we report a case of basilar invagination which presented with downbeating nystagmus and postural hypotension. A 31 year-old Thai male patient had a 20 year history of postural hypotension. He had recurrent pneumonia and cough-induced syncope a year before admission. He complained of symptoms of an acute febrile illness and a productive cough. The physical examination showed high grade fever, postural hypotension and medium crepitation in the right upper lobe. The neurological examination showed downbeating nystagmus, atrophy and fasciculation of the right side of the tongue, atrophy of the right sternocleidomastoid muscle, mild weakness of the extremities and generalized hyperreflexia. The cervical spine X-ray revealed upward displacement of the vertebral bodies of C1 and C2, with a mild narrowing of the space between C1 and the occiput. The CT-myelogram and MRI showed upward displacement of C1 with overriding of the dens over the anterior lip of the foramen magnum; this also compressed the medulla. syringomyelia was seen at the C1-C5 level. We report a patient who presented with postural hypotension, recurrent pneumonia and downbeating nystagmus due to basilar invagination. The symptoms were aggravated by cough which caused an increase in intracranial pressure. This resulted from medulla compression in the foramen magnum by the first cervical spine. The treatment of choice was surgical decompression.- - - - - - - - - - ranking = 2keywords = ms (Clic here for more details about this article) |
19/198. 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.- - - - - - - - - - ranking = 2keywords = ms (Clic here for more details about this article) |
20/198. A liberatory maneuver for the treatment of horizontal canal paroxysmal positional vertigo.OBJECTIVE: The purpose of this study was to determine the effectiveness of a new liberatory maneuver in the management of the geotropic variant of horizontal canal paroxysmal positional vertigo (HC-PPV). STUDY DESIGN: Case review. SETTING: Outpatient clinic. patients: The diagnosis of HC-PPV was based on the history of recurrent sudden crisis of vertigo associated with bursts of horizontal geotropic paroxysmal nystagmus provoked by turning the head from the supine to either lateral position. The patients were 11 men and 21 women ranging in age from 30 to 85 years (average 55.43 years), and the average duration of symptoms was 7.68 days. INTERVENTIONS: All patients were treated with a liberatory maneuver based on the hypothesis that the syndrome is caused by the presence of free-floating dense particles inside the endolymph of the posterior arm of the semicircular horizontal canal. The maneuver favors their outmigration into the utricle. patients were reexamined immediately after the treatment and again 2 days later. MAIN OUTCOME MEASURE: The treatment outcome was considered as responsive when, after one or more liberatory maneuvers, the clinical signs of PPV disappeared at the end of physical therapy. RESULTS: The liberatory maneuver resulted in a complete remission of the positioning vertigo and nystagmus in all patients after the first session. CONCLUSIONS: This approach represents a simple and effective approach to the management of the geotropic form of HC-PPV.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
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