Cases reported "Nystagmus, Pathologic"

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1/10. 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.
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2/10. 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.
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keywords = physical
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3/10. Horizontal canal type BPPV: bilaterally affected case treated with canal plugging and Lempert's maneuver.

    A 54-year-old woman complained of positional vertigo. During 3 months' observation, the patient showed mostly geotropic or apogeotropic nystagmus due to right canalolithiasis or cupulolithiasis, however, she sometimes showed nystagmus which suggested left horizontal canalolithiasis. We suspected that she suffered from bilateral horizontal canal type benign paroxysmal positional vertigo (BPPV) and performed Lempert's maneuver for both directions, however, they were ineffective. She underwent canal plugging for right horizontal canal. After surgery she showed no positional nystagmus of right horizontal canal origin. However, apogeotropic nystagmus of the left horizontal canal origin was still observed. This nystagmus changed to geotropic nystagmus and finally disappeared following Lempert's maneuver for the left side. Bilateral horizontal canal BPPV is difficult to be resolved, probably because physical treatment for one side would move debris to the cupula in the other canal. Canal plugging combined with Lempert's maneuver to the other side is one treatment option for intractable bilateral horizontal canal BPPV.
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4/10. Mietens-Weber syndrome: two new patients and a review.

    In 1966, Mietens and Weber reported four out of six siblings from a consanguineous couple with growth failure, dislocation of the head of the radii, bilateral flexion contracture of the elbows, short ulnae and radii, bilateral corneal opacities, horizontal and rotational nystagmus, strabismus, small, pointed nose and mild to moderate mental retardation. Since then, only three other cases have been reported. We report on two new cases, a pair of female twins aged 9 years. The patients were born after an uneventful, normal pregnancy, to young and non-consanguineous parents. After birth, physical findings included horizontal nystagmus and dislocation of both elbows because of abnormally short radii and ulnae in both twins. Further clinical examinations showed moderate psychomotor delay with marked language compromise. Karyotypes were normal in both girls. A review of the literature reveals that the Mietens-Weber syndrome is an uncommon disorder with a probable autosomal recessive pattern of inheritance. To our best knowledge, including the two cases reported here, only nine cases have been observed so far. The finding of congenital nystagmus and radii dislocation in a patient with mental retardation is probably nonrandom and is highly suggestive of Mietens-Weber syndrome.
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keywords = physical
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5/10. Impairment of vertical motion detection and downgaze palsy due to rostral midbrain infarction.

    We present two cases with acute onset of vertical gaze palsy, mainly consisting of impaired downgaze and apraxia of downward head movements, together with neuropsychological deficits (hypersomnia, impaired attention and disorders of memory and affective control). CT and MRI revealed bilateral post-ischaemic lesions in the dorsomedial thalamus and the mesodiencephalic junction, dorsomedial to the red nucleus, thus being restricted to the territory of the posterior thalamosubthalamic paramedian artery, which includes the region of the rostral interstitial nucleus of the medial longitudinal fascicle as the main premotor nucleus for the generation of vertical saccades. In our patients, oculographic examination with electro-oculography and magnetic search coil recording showed severe impairment of downward more than upward saccades and only minor deficits of vertical pursuit and the vestibulo-ocular reflex. Visual functions were normal, with one exception: a psychophysical test of motion perception revealed a significant deficit in the detection of vertical movements. This could be due to a central adaptive mechanism which, in order to minimize oscillopsia, might elevate thresholds for vertical motion perception in cases of vertical gaze palsy. As an alternative explanation, lesions within the midbrain tegmentum could have damaged subcortical visual pathways involved in motion perception.
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keywords = physical
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6/10. cerebellar ataxia and opsoclonus as the initial manifestations of myoclonic encephalopathy associated with neuroblastoma.

    cerebellar ataxia and opsoclonus were the initial manifestations of an associated neuroblastoma in a 20-month-old girl. Two months after the initial symptomatology, a physical examination revealed an abnormal mass palpable left to the midline. Urinary catecholamines were within normal limits. The child's neurological findings improved immediately after surgery, and steroid treatment and the follow-up on her after 2 years revealed normal general and neurological development. The syndrome of myoclonic encephalopathy including cerebellar ataxia, myoclonus and opsoclonus, and its relationship to neuroblastoma is reviewed. Failure to recognize this association can result in delays in both diagnosis and treatment and could be fatal.
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7/10. Positional nystagmus.

    Positional nystagmus may be persistent or transitory. Of the persistent forms, type I refers to nystagmus changing direction in different head positions and type II to nystagmus beating in a single direction. Both types may denote either peripheral or central abnormality. Transitory, or paroxysmal, positional nystagmus is classified as type III, and the history and physical and electronystagmographic features of its accompanying vertigo and nystagmus permit subdivision into typical and atypical forms. Guidelines are given for the differentiation of typical and atypical forms. In the great majority of cases type III positional nystagmus denotes a harmless inner ear disturbance, but the occasional serious intracranial lesion is also causative. Most instances of the latter, but not all, will fit in the atypical group. A case is reported of vermis metastasis with presenting features of postural vertigo and paroxysmal downbeat positional nystagmus.
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keywords = physical
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8/10. Vestibular dysfunction in a child with embryonic exposure to accutane.

    Children with a history of embryonic exposure to Accutane (isotretinoin) are at great risk for major physical malformations, brain malformations, and decreased intelligence. A case is presented of a 4-year 7-month-old black male with a history of embryonic exposure to Accutane who was born with embryopathy that includes bilateral major ear deformities. The child has a significant bilateral conductive hearing loss, and, in addition, a left sided sensorineural loss. Vestibular function testing revealed evidence of peripheral and central vestibular dysfunction. A course of diphenhydramine hydrochloride and Donnatal (phenobarbital, hyoscyamine sulfate, atropine sulfate, and scopolamine hydrobromide) significantly alleviated the symptoms of vestibular dysfunction. Otologic management of these children should include clinical documentation of the external deformities, evaluation of cochlear function, and early auditory habilitation. Vestibular function should also be evaluated in all children with a history of embryonic exposure to isotretinoin.
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keywords = physical
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9/10. Co-existing post-stapedectomy fistula and acoustic neuroma.

    A 66 year old woman had light-headedness and ataxia which increased slowly despite tissue seal of a post-stapedectomy oval window fistula. The cause was shown to be a large acoustic neuroma, ipsilateral to the side of the fistula. The history, physical, and laboratory findings are presented.
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keywords = physical
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10/10. Saccadic visual suppression and down-beat nystagmus: a case of arnold-chiari malformation.

    A case is reported in which visual disturbances associated with the sole physical finding of downbeat nystagmus led to investigation of a patient for a foramen magnum lesion. The arnold-chiari malformation was suggested by air study and confirmed at surgery. It is suggested that the phenomenon of saccadic suppression was responsible for the patient's visual disability. This was unaccompanied by any of the other neurological manifestations of the arnold-chiari malformation. Cases presenting in this manner, with only visual disturbances, may run the risk of being unrecognized for some time as a result.
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keywords = physical
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