1/36. 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.- - - - - - - - - - ranking = 1keywords = malformation (Clic here for more details about this article) |
2/36. 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 = 0.25keywords = malformation (Clic here for more details about this article) |
3/36. Continuous vertical pendular eye movements after brain-stem hemorrhage.Electro-oculographic studies are reported in a 33-year-old man with bilateral horizontal gaze palsies and continuous pendular eye movements in the vertical plane secondary to hemorrhage from a pontine arteriovenous malformation. The effects of pharmacologic and physiologic stimuli on the movements are described.- - - - - - - - - - ranking = 0.25keywords = malformation (Clic here for more details about this article) |
4/36. Periodic alternating nystagmus associated with arnold-chiari malformation.The arnold-chiari malformation type I is a rare congenital condition characterized by herniation of the cerebellar tonsils into the upper cervical spinal tract. Various otoneurological manifestations occur in patients with the disease, which has usually been associated with a downbeat type of nystagmus. In this paper a case with this condition is reported presenting with periodic alternating nystagmus (PAN), which is a rare type of nystagmus observed in diseases of the central nervous system of different pathologies. The patient had a complete otoneurological examination and vestibular function testing. diagnosis was established by using magnetic resonance imaging (MRI).- - - - - - - - - - ranking = 1.25keywords = malformation (Clic here for more details about this article) |
5/36. Positional down beating nystagmus in 50 patients: cerebellar disorders and possible anterior semicircular canalithiasis.OBJECTIVES: To clarify the clinical significance of positional down beat nystagmus (pDBN). methods: A discussion of the neuro-otological findings in 50 consecutive patients with pDBN. RESULTS: In 38 patients there was evidence of CNS disease (central group) but in 12 there was not (idiopathic group). In the CNS group, presenting symptoms were gait, speech, and autonomic dysfunction whereas in the idiopathic group patients mostly reported positional vertigo. The main neurological and oculomotor signs in the CNS group were explained by cerebellar dysfunction, including 13 patients with multiple system atrophy. In patients with multiple system atrophy with a prominent extrapyramidal component, the presence of pDBN was helpful in the differential diagnosis of atypical parkinsonism. No patient with pDBN had the arnold-chiari malformation, a common cause of constant down beat nystagmus (DBN). In the idiopathic group, the pDBN had characteristics which suggested a peripheral labyrinthine disorder: vertigo, adaptation, and habituation. In six patients an additional torsional component was found (concurrently with the pDBN in three). Features unusual for peripheral disorder were: bilateral positive Dix-Hallpike manoeuvre in nine of 12 patients and selective provocation by the straight head-hanging manoeuvre in two. CONCLUSION: It is argued that some patients with idiopathic pDBN have benign paroxysmal positional vertigo (BPPV) with lithiasis of the anterior canal. The torsional component may be weak, because of the predominantly sagittal orientation of the anterior canal, and may not be readily seen clinically. Nystagmus provocation by bilateral Dix-Hallpike and straight head-hanging may be explained by the vertical upwards orientation of the ampullary segment of the anterior canal in the normal upright head position. Such orientation makes right-left specificity with the Dix-Hallpike manoeuvre less important than for posterior canal BPPV. This orientation requires a further downwards movement of the head, often achieved with the straight head-hanging position, to provoke migration of the canaliths. The straight head-hanging manoeuvre should be carried out in all patients with a history of positional vertigo and a negative Dix-Hallpike manoeuvre.- - - - - - - - - - ranking = 0.25keywords = malformation (Clic here for more details about this article) |
6/36. Otorhinolaryngologic manifestations in Chiari malformation.The Chiari malformation causes herniation of the cerebellar amygdalae through the foramen magnum, resulting in the descent of the brain stem and/or traction on the lower cranial pairs. It is important for otolaryngologists to recognize Chiari malformations as part of the differential diagnosis of balance disorders, because patients may initially exhibit symptoms related to the vestibular system, including ataxia, nystagmus, or vertigo. We report 2 cases.- - - - - - - - - - ranking = 1.5keywords = malformation (Clic here for more details about this article) |
7/36. Subarcuate venous malformation causing audio-vestibular symptoms similar to those in superior canal dehiscence syndrome.OBJECTIVE: To present a patient with symptoms similar to those of superior canal dehiscence syndrome due to another cause. STUDY DESIGN: Case report. SETTING: University hospital, tertiary referral center. PATIENT: The 65-year-old woman had suffered for 4 years from hearing loss, tinnitus, and pressure-induced vertigo. INTERVENTION: Audio-vestibular testing, high-resolution computed tomography, and magnetic resonance angiography. MAIN OUTCOME MEASURE: The superior canal dehiscence syndrome is caused by failure of normal postnatal bone development in the middle cranial fossa leading to absence of bone at the most superior part of the superior semicircular canal. The typical features for this syndrome are sound and pressure-induced vertigo with torsional eye movements, pulse synchronous tinnitus and apparent conductive hearing loss in spite of normal middle ear function. We present a patient with very similar symptoms and findings, who instead had a superior canal dehiscence close to the common crus. Neuroradiologic findings suggested that the dehiscence was related to a venous malformation. CONCLUSIONS: Symptoms and findings suggesting superior canal dehiscence syndrome can have a different cause.- - - - - - - - - - ranking = 1.25keywords = malformation (Clic here for more details about this article) |
8/36. arnold-chiari malformation.arnold-chiari malformation is a congenital malformation of the skull near the foramen magnum in which the cerebellum and the medulla are caudally displaced. This herniation of the brainstem causes down-beat nystagmus and oscillopsia, the most commonly presenting sign and symptom, respectively. Differential diagnoses for the arnold-chiari malformation include, but are not limited to, demyelinating disease, tumor, and vascular disorders. Symptoms will generally worsen with time and may even be brought on during exercise or valsalva maneuvers. A correct diagnosis can lead to timely surgical intervention which can improve the quality of eye movements. Treatment generally involves the surgical decompression of the surrounding spinal tissue.- - - - - - - - - - ranking = 1.75keywords = malformation (Clic here for more details about this article) |
9/36. Symptomatic arnold-chiari malformation and cranial nerve dysfunction: a case study of applied kinesiology cranial evaluation and treatment.OBJECTIVE: To present an overview of possible effects of arnold-chiari malformation (ACM) and to offer chiropractic approaches and theories for treatment of a patient with severe visual dysfunction complicated by ACM. CLINICAL FEATURES: A young woman had complex optic nerve neuritis exacerbated by an ACM type I of the brain. INTERVENTION AND OUTCOME: Applied kinesiology chiropractic treatment was used for treatment of loss of vision and nystagmus. After treatment, the patient's ability to see, read, and perform smooth eye tracking showed improvement. CONCLUSION: Further studies into applied kinesiology and cranial treatments for visual dysfunctions associated with ACM may be helpful to evaluate whether this single case study can be representative of a group of patients who might benefit from this care.- - - - - - - - - - ranking = 1.25keywords = malformation (Clic here for more details about this article) |
10/36. Resolution of periodic alternating nystagmus after decompression for Chiari malformation.CASE REPORT: A 20-year-old female presented with horizontal jerk nystagmus, blurred vision, severe headaches, unsteady gait, and paresthesia. magnetic resonance imaging revealed Chiari malformation I. Symptoms resolved after decompression of the posterior cranial fossa and removal of the right cerebellar tonsil. COMMENTS: Conditions associated with acquired periodic alternating nystagmus and ocular disorders associated with Chiari malformation are discussed. To our knowledge, this case is the first to report the resolution of periodic alternating nystagmus after neurosurgical decompression.- - - - - - - - - - ranking = 1.5keywords = malformation (Clic here for more details about this article) |
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