Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0028738 (nystagmus)
 7,431 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Seventy-nine out of 172 patients with abnormal horizontal or vertical optokinetic nystagmus had a specific condition or disease that was diagnosed following a complete ENG. Ocular vertigo, MS, motion sickness, brain stem lesions, cerebellar tumors, cerebellar atrophy, parietal lobe tumors, and Harada syndrome were the diagnoses made. Optokinetic asymmetry--horizontal, vertical, or a combination of these--was the most consistent finding in these conditions (Table). In six patients with cerebellar tumor, abnormal findings on OPK testing suggested the need for further evaluation when physical and neurologic examinations, brain scans, and EEG were normal. Surgery confirmed the diagnosis of cerebellar tumor with these patients. This study suggested the importance of using five progressively faster drum speeds in both the horizontal and vertical planes. All patients who had proven or strongly suspected MS had eye-speed fatigability as the drum speed increased, that is, as the drum speed increased, the eye speed decreased. It is suggested that optokinetic studies be included in the ENG. Vertical asymmetry, although not as common as horizontal, should be included in that 14 patients had only this finding.
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PMID:The value of including optokinetic nystagmus testing in electronystagmography. 88 71

Disorders of pigmentation can result from either an abnormal number of melanocytes, as in nevus of Ota and vitiligo, or an abnormal amount of melanin production, as in albinism. Melanin-producing cells are found in the skin, mucous membranes, uveal tract, and retinal pigment epithelium of the eye and the stria vascularis of the inner ear. Thus, many of the hereditary or congenital pigmentary disorders of the skin are associated with similar pigmentary abnormalities in the eye, such as iris heterochromia or changes in pigmentation of the fundus; however, more commonly, the associated eye finding is a defect in ocular motility, i.e., strabismus and nystagmus, suggesting a concomitant defect in neurologic development. In albinos, the observed neurologic abnormality in the visual pathway and foveal hypoplasia are hypothesized to be related directly to the lack of melanin in the pigment epithelium during development. In acquired disorders of pigmentation, in particular, vitiligo, Vogt-Koyanagi-Harada syndrome, and onchocerciasis, there is a frequent association with uveitis, suggesting an inflammatory cause for the cutaneous pigmentary changes.
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PMID:Ocular manifestations of pigmentary disorders. 161 18

A 45-year-old Japanese man, who had had bilateral visual disturbance due to Vogt-Koyanagi-Harada (VKH) disease 17 years before entry, was admitted to this hospital because of headache, vertigo and vomiting. On examination at entry, no abnormalities except for poliosis, patches of vitiligo on his left shin, sunset glow fundus, and positional nystagmus with Frenzel glasses were found. Laboratory data other than leukocytosis and elevated level of gamma-GTP were normal and the results of brain CT scan were within normal limits. On the following day, diplopia was developed and the neurological symptoms including loss of bilateral visual acuity, Horner's syndrome on the right side, right facial palsy, bilateral sensorineural hearing disturbance, palsy of the soft palate on the right side with swallowing difficulty, and dissociated sensory disturbance on the right face and the upper and lower extremities on the left side appeared with a few days. He couldn't get up. The cerebrospinal fluid (CSF) was clear and had pleocytosis with normal sugar content. The protein, immunoglobulin G and myelin basic protein (MBP) were elevated but the tests for oligoclonal band and antiviral antibodies were negative. Brain CT scan showed low density areas in right cerebellar hemisphere and in left putamen without abnormality with contrast material and evoked potentials were normal. Prednisolone was prescribed and his symptoms were subsided but his gait remained ataxic. Magnetic resonance imaging (MRI) 4 months later showed an atrophy of the lower half of bilateral cerebellar hemisphere supplied by posterior inferior cerebellar artery, suggesting cerebellar infarction, and high intensity areas on T2 image in bilateral cerebral white matters, basal ganglia, and left cerebral peduncle.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Wallenberg's syndrome in a case of Vogt-Koyanagi-Harada disease]. 261 5

Harada's disease is an uncommon autoimmune disease affecting melanocytes located in the uvea, inner ear, skin, hair, and meninges. A classification of Harada's disease presenting with aural symptoms, especially vertigo, was devised on the basis of otoneurological findings. Peripheral vestibular dysfunction, such as staggering in the stepping test, rotato-horizontal or horizontal nystagmus, and diminished labyrinthine function in the caloric nystagmus test, was observed in the vast majority of these patients. Harada's disease with such features was designated as the peripheral type, and was further divided into vestibular, cochleo-vestibular, and cochlear subtypes. Patients only rarely presented with symptoms or signs of central nervous system involvement, and Harada's disease with such features was designated as the central type.
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PMID:Otoneurological observation and classification of Harada's disease presenting with aural symptoms, especially vertigo. 761 Aug 44

The eye movements of 15 patients with Vogt-Koyanagi-Harada (VKH) syndrome were analysed. Transient dizziness occurred in 60% of the patients. Eight patients (53%) exhibited horizontal jerk nystagmus that was completely inhibited by visual fixation. Eight cases (53%) exhibited the elevation of vestibulo-ocular reflex (VOR) gain in darkness. Six of the patients with nystagmus had defective smooth pursuit movements with reduced gain on the side ipsilateral to the nystagmus. Saccadic and optokinetic eye movements were intact, and visual vestibular interaction was also normal. Impaired eye movements in VKH syndrome may be caused by asymmetry of the vestibular function secondary to the labyrinthine inflammation.
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PMID:Vestibulo-ocular reflex abnormality in Vogt-Koyanagi-Harada syndrome. 781 75

It has been reported that normal hearing is rare in patients with severe inner ear vestibular malformations [Kokai H, Oohashi M, Ishikawa K, Harada K, Hiratsuka H, Ogasawara M et al. Clinical review of inner ear malformation. J Otolaryngol Jpn 2003;106(10):1038-44; Schuknecht HF. Mondini dysplasia. A clinical pathological study. Ann Otol Rhinol Laryngol 1980;89(Suppl. 65):1-23; Jackler RK, Luxford WM, House WF. Congenital malformations of the inner ear: a classification based on embryogenesis. Laryngoscope 1987;97:2-14; Phelps PD. Congenital lesions of the inner ear, demonstrated by tomography. Arch Otolaryngol 1974;100:11-8]. A 37-year-old woman had combined dysplasia of the posterior and lateral semicircular canals (PSCC, LSCC) with normal cochlear development and normal hearing in both ears. She had complained of dizziness for 8 months. High resolution computed tomography (CT) showed hypogenesis of the bony labyrinth in both ears. Bilateral PSCC and LSCC dysplasia and dilatation of the vestibule were detected. Magnetic resonant imaging (MRI) revealed that the deformity of the PSCC was more severe than the LSCC. Although the caloric test of the left ear elicited no nystagmus and there was reduced response in the right ear, the horizontal vestibulo-occular reflex (VOR) was present. Her dizzy sensation disappeared within 3 months without special treatment. The dizziness attack might have been caused by a temporary breakdown of her peripheral vestibular system.
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PMID:Congenital inner ear malformations without sensorineural hearing loss. 1791 22

Vogt-Koyanagi-Harada (VKH) disease is an idiopathic, multisystem autoimmune disorder characterized by bilateral, diffuse granulomatous uveitis associated with neurological, audiovestibular, and dermatological manifestations. The purpose of this study is to investigate vestibular functions in patients with VKH disease. A total of 43 patients with VKH disease in Hokkaido University Hospital were enrolled in this study. Subjective symptoms such as dizziness or vertigo and the results of various vestibular examinations including nystagmus testing, caloric testing, and vestibular-evoked myogenic potential (VEMP) testing were investigated. Eight of 42 patients (19.0%) complained of subjective vestibular symptoms. On the other hand, 12 of 28 patients (42.9%) showed nystagmus, and 7 of 15 patients (46.7%) showed unilateral or bilateral weakness in the caloric test. VEMP testing was performed for 16 patients. Seven (43.8%) and 8 (50.0%) patients were evaluated as abnormal in cervical VEMP and ocular VEMP testing, respectively. The rate of detection of nystagmus was significantly higher than that of subjective symptoms. As vestibular dysfunction in patients with VKH disease cannot be detected through history taking alone, nystagmus testing, caloric testing, and VEMP testing should be performed to evaluate vestibular functions associated with VKH disease. It is considered that abnormal VEMP findings are associated with otolith organ dysfunction.
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PMID:Evaluation of Vestibular Functions in Patients with Vogt-Koyanagi-Harada Disease. 2908 Aug 87

Management of head tilt in infantile nystagmus syndrome (INS) is a challenge. In this case report, we have described successful management of right-sided head tilt in a child with INS by operating on three oblique muscles (superior oblique anterior tenectomy in the right eye, Harada-Ito procedure in the left eye, and inferior oblique recession in the left eye). The child had complete correction of head tilt without causing any cyclovertical strabismus or torsional diplopia postoperatively.
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PMID:Management of head tilt in infantile nystagmus syndrome: A case report. 3143 5