Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 55-years-old woman had left neck pain and headache, dizziness, left Horner's sign, left abducens palsy, diplopia, left peripheral facial palsy, left loss of hearing, left tinnitus, left paralysis of vocal cord and soft palate, dysphagia, left limb ataxia, truncal ataxia, disturbance of temperature and pain sensation over Th10 on the right involving the right face. Left vertebral angiography revealed tapering occlusion of the left vertebral artery. Right vertebral angiography showed normal angiogram of the basilar artery and bilateral anterior inferior cerebellar arteries. MRI disclosed infarcts in the left lateral inferior pons, left lateral medulla, and cerebellum of territories in the anterior inferior cerebellar artery and posterior inferior cerebellar artery. T2 weighted image showed septum (intimal flap) in the left vertebral artery. This is the very rare case of lateral inferior pontine syndrome and lateral medullary syndrome due to the vertebral artery dissection.
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PMID:[A case of vertebral artery dissection with lateral inferior pontine syndrome and lateral medullary syndrome]. 921 27

Lyme borreliosis is spirochetal disease that frequently affects the nervous system months or years after infection giving rise to a varied clinical picture named neuro-borreliosis. We report a case of 47 year old female with progressing hearing loss, tinnitus, paraparesis and ataxia. The disease was beginning six month earlier by weakness of the lower limbs associated with hearing loss. The patient did not remember to be exposed to ticks and did not recall the presence of erythema migrans, arthritis or other systemic signs. CSF was with mononuclear pleocytosis and protein concentration over 600 mg%. Firstly patient was unsuccessfully treated like encephalomeningitis with tbc etiology. Next cerebrospinal fluid analysis showed presence of antibodies against Borrelia burgdorferi in IgG and IgM class. IgG antibodies in serum were founded. Audiometry electric responses from brain stem showed sensorineural hearing loss. Therapy with ceftriaxone was successful. Because negative tick bile history and many signs from different parts of CNS, relationship of this spirochetal infection and severe otolaryngological and neurological disease was firstly difficult to recognition.
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PMID:[Lasting hearing loss in the course of neuro-borreliosis]. 959 44

A 67-year-old lady with breast cancer developed diplopia, tinnitus, nausea and vertigo within 2 weeks, followed 2 months later by severe truncal ataxia. Opsoclonus was never observed. She had anti-Ri antibodies and improved substantially after tumor resection and radiation.
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PMID:[Reversible paraneoplastic cerebellar symptoms. An example of anti-Ri syndrome]. 967 76

We have treated 14 patients with glomus tumor during the 4 years (of 1993 to 1997) using Leksell Gamma Knife radiosurgery. The male: female ratio was 1:3.7, and the mean age 48.6 years (range 22-75 years). The mean tumor volume was 5.5 cm3 (range 0.7-11.3 cm3). The mean maximum dose was 37.4 Gy (range 20-44 Gy). The mean margin dose was 19.4 Gy (range 10-25 Gy). In 3 patients, infrabasal spread of the tumor could not be delineated on peroperative stereotactic CT scans. As a result, this portion of the tumor was treated in 2 patients at a second stage using stereotactic MRI. Follow-up in 11 patients ranged from 6 to 42 months (mean 20.5 months). Hearing on the affected side was further impaired in 3 patients. Tinnitus, vertigo and ataxia improved in 3 patients, headache and nausea in 2 patients. Angiography after radiosurgery was performed in 3 patients. In one patient 12 months after the radiosurgery, pathological vascularisation had completely disappeared. In another patient pathological vascularisation was still present 22 months after the first stage, despite two-stage radiosurgery, although the tumor volume decreased 30%. In the last patient, vascularisation and tumor volume partially decreased 12 months after radiosurgery. The volume of the tumor decreased in 4 patients. No change in tumor volume has been observed in any of the other patients to date. Radiosurgery proves to be a safe treatment for glomus tumor with no acute morbidity. Because of its naturally slow growth rate, up to 10 years follow-up will probably be necessary to establish the therapeutic effectiveness of radiosurgery for glomus tumor.
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PMID:Leksell gamma knife radiosurgery of the tumor glomus jugulare and tympanicum. 978 46

Instillation of gentamicin in the middle ear to cure severe Meniere's disease is of considerable value in the control of vertigo. The drug causes diminished endolymph production and, most often, a peripheral vestibular loss. After vestibular rehabilitation, led by a physiotherapist, the ataxia should be resolved as a result of central compensation. Fifteen to twenty percent of the patients experience an added hearing loss in the treated ear, although this percentage is reduced if the treatment is given as a single instillation on 2 consecutive days. Using this strategy, the mean added hearing loss for the treated group is zero. There is no correlation between the pretreatment hearing level and the added hearing loss.
Int Tinnitus J 1997
PMID:Gentamicin Cures Vertigo, but What Happens to Hearing? 1075 75

The study case was a 66-year-old man who had bilateral neurosensory hearing impairment due to brain stem infarctions. He noticed mild hearing loss, frequent vertigo and tinnitus. About one month later, his hearing took a sudden turn for the worse, and he suffered from dysarthria, dysphagea and abasia. Neurological examination revealed pseudobulber palsy, left hemiparesis, cerebeller ataxia, disturbance of pain and temperature sensation on the right face and left side of the body. Brain stem auditory evoked potentials (BAEPs) showed a delayed small wave V with the abscence of previous waves on the right side and no significant waves on the left side. Brain magnetic resonance images (MRI) revealed infarctions in the bilateral middle cerebellar peduncles, including in the right lateral portions of pons, and the right lower pontine base. We believe that not only peripheral, but also central auditory pathways adjacent to infarctions were damaged. Magnetic resonance angiography (MRA) showed severe stenosis or occlusion of left vertebral artery and basilar artery. We concluded that hypoperfusion of the vertebrobasilar artery territories caused ischemia of the cochlear nerve and the auditory tracts in the brain stem, which resulted in bilateral hearing loss.
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PMID:[A case of brain stem infarction with bilateral hearing loss]. 1076 46

There are many vascular and neoplastic diseases as well as normal variants that produce the vestibulocochlear symptoms of pulsatile tinnitus, hearing loss, dizziness, and ataxia. Magnetic resonance imaging may be diagnostic, and magnetic resonance angiography/magnetic resonance venography have added to the ability of magnetic resonance to image vascular abnormalities. The extent of neoplasms is accurately assessed and complication of vascular lesions are clearly seen. However, detailed vascular anatomy requires high-quality selective angiography. This enables optimal treatment planning. Endovascular therapeutic intervention has a major role to play in conjunction with surgery of skull base lesions and may be curative in certain conditions, avoiding major surgical procedures. The interventionalist, however, must have an excellent knowledge of the external carotid circulation and all of its potential communications with the internal circulation to avoid serious embolic complications.
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PMID:Role of magnetic resonance imaging and diagnostic and interventional angiography in vascular and neoplastic diseases of the skull base associated with vestibulocochlear symptoms. 1079 1

The authors report an autosomal dominant episodic ataxia that is clinically distinct from the other episodic ataxias. Vestibular ataxia, vertigo, tinnitus, and interictal myokymia are prominent; attacks are diminished by acetazolamide. Linkage analyses of markers flanking the EA1 and EA2 loci demonstrate genetic exclusion from the other autosomal dominant episodic ataxias. The authors suggest EA3 for periodic vestibulocerebellar ataxia and EA4 for the disorder described here.
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PMID:An autosomal dominant disorder with episodic ataxia, vertigo, and tinnitus. 1167

A quantitative analysis of two rat syndromes of myoclonus are presented, modeling myoclonic epilepsy and postanoxic myoclonus. Like the human conditions, both of the models benefit therapeutically from drugs that act on the serotonin system. The rat model of myoclonic epilepsy is associated with a profound loss of serotonin throughout the brain (except in the striatum) and is generated by an oscillator that is synchronized around the midline. The rat model of posthypoxic myoclonus does not demonstrate a significant reduction in serotonin in any location of its brain and is generated by a non-oscillating circuit in the medulla. Although some forms of myoclonic epilepsy may benefit from serotonin drugs because they are caused by a decrease in brain serotonin, our data indicate that posthypoxic myoclonus is not caused by a decrease in the serotonergic innervation of any region of the brain. That the raphe nuclei do not degenerate after global brain ischemia was noted by C. David Marsden in a discussion of the histologic findings of three of his human cases of posthypoxic myoclonus (page 117 of reference 10) and led him to question the hypothesis that posthypoxic myoclonus was due to a loss of serotonin neurons. Our data confirm his observation in the rat, but also indicate that density of serotonin fibers and terminals throughout the brain is not reduced by the brain ischemia that produces posthypoxic myoclonus. It remains to be determined whether the physiologic responsiveness of serotonin neurons is altered by global brain ischemia and whether changes in serotonin release or serotonin receptor properties are associated with posthypoxic myoclonus. The stability of the serotonin system in posthypoxic myoclonic rats is remarkable when one considers the wide range of disorders that is produced by the prolonged brain ischemia. The inability of the most severely posthypoxic myoclonic rats to perform 7-Hz tongue protrusions indicates substantial physiologic disruption of brainstem motor function. Moreover, the posthypoxic myoclonic rat suffers from ataxia, seizures, retrograde amnesia, and impaired ability to learn. The wide spectrum of these deficits is sharply constrasted by its apparently intact serotonin system. We have identified the inferior olive as a locus that may generate the rhythmic components of tremor and myoclonus in syndromes that are truly associated with a dramatic loss of brainstem serotonin. Serotonin acts within the inferior olive to constrain its rhythmic firing. Without intraolivary serotonin, olivary neurons are predisposed to oscillate continuously, providing a substrate upon which sustained rhythmic spiking may be superimposed. It is clear that such unconstrained rhythmicity produces synchronized whole-body tremor at 10 Hz (33, 41-43). The effects of serotonin to suppress olivocerebellar rhythmicity are mediated by postsynaptic 5-HT2 receptors that reduce the magnitude of the low-threshold calcium conductance, IT. It is notable that dysregulation of this conductance has been associated with hyper-rhythmic states in the thalamus underlying cognitive disorders ranging from depression to tinnitus (49), indicating a common mechanism underlying a variety of neurologic conditions. The identification of a specific brainstem locus (inferior olive), serotonin receptor 5-HT2, and ionic current IT involved in a form of rhythmic myoclonus may provide multiple clues toward which future pharmacotherapies can be directed.
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PMID:The serotonin hypothesis of myoclonus from the perspective of neuronal rhythmicity. 1196 57

Aneurysms of the distal anterior inferior cerebellar artery (AICA) are rare; fewer than 100 cases have been reported. The authors detail their experience with four cases and present endovascular as well as microsurgical management options. The medical records and neuroimaging studies obtained in four patients who were treated at a single institution were reviewed. Clinical presentations, neuroimaging and intraoperative findings, and clinical outcomes were analyzed. There were three men and one woman; their mean age was 43 years. Two patients presented with acute subarachnoid hemorrhage (SAH), and two presented with ataxia and vertigo (one with tinnitus, the other with hearing loss). Angiographic studies demonstrated aneurysms of the distal segment of the AICA. In one patient with von Hippel-Lindau syndrome and multiple cerebellar hemangioblastomas, a feeding artery aneurysm was found on a distal branch of the AICA. Three of the patients underwent successful surgical obliteration of their aneurysms, one by clipping, one by trapping, and one by resection along with the tumor. The fourth patient underwent coil embolization of the distal AICA and the aneurysm. All patients made an excellent neurological recovery. Patients with aneurysms in this location may present with typical features of an acute SAH or with symptoms referable to the cerebellopontine angle. Evaluation with computerized tomography, magnetic resonance (MR) imaging, MR angiography, and digital subtraction angiography should be performed. For lesions distal to branches coursing to the brainstem, trapping and aneurysm resection are viable options that do not require bypass. Endovascular obliteration is also a reasonable option, although the possibility of retrograde thrombosis of the AICA is a concern.
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PMID:Distal anterior inferior cerebellar artery aneurysms. Report of four cases. 1229 56


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