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Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The value of evoked potentials in studying conduction in the somatosensory pathway was assessed in patients with various neurological disorders. In patients with multiple sclerosis (MS) abnormalities of the cervical response (N14) were found particularly in longstanding cases but also in the early stages of the disease, even in patients without sensory symptoms or signs, and were reversible in some patients. The cortical response was also abnormal in some cases but the two were not always affected together. In Friedreich's ataxia both the cervical and cortical responses were usually abnormal. Subclinical abnormalities of the cervical responses were found in some patients with hereditary spastic paraparesis or mixed forms of spinocerebellar ataxia. The cervical responses were also abnormal in patients with peripheral neuropathy and cervical radiculopathy, and in some patients with brain-stem or thalamic lesions. Cervical and cortical responses were normal in the lateral medullary syndrome, whereas the cortical response was markedly abnormal in patients with high brain-stem or cerebral hemisphere vascular lesions. Cortical and subcortical responses were abnormal in some patients with stereotactic thalamic lesions. Enhanced cortical responses were found in patients with lesions at different levels in the CNS. The most marked enhancement was observed in patients with familial myoclonic epilepsy. Lesser degrees were found in some patients with MS, progressive supranuclear palsy, thalamic lesions, brain-stem encephalitis and syringomyelia. Enhanced responses were usually found in patients with minimal or no clinical sensory involvement. It is postulated that this type of abnormality results from an interference to the inhibitory mechanisms which normally operate at various levels in the somatosensory pathway. It is concluded that evoked potential studies are a valuable adjunct to the clinical evaluation of sensation, and that they may provide useful information on the pathophysiology of conduction in the somatosensory pathway.
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PMID:The contribution of evoked potentials in the functional assessment of the somatosensory pathway. 22 50

Atlantoaxial instability is a relatively frequent finding in Down syndrome, but syringomyelia is a rare symptom. A four-year-old girl with Down syndrome was noted to have one year history of torticollis and progressive motor deterioration presenting with abnormal gait, ataxia or quadriparesis for the past three months. She was admitted because of acute urinary retention. Physical examination showed distended urinary bladder, hyperreflexia, bilateral ankle clonus and the existence of Babinski's signs. The x-ray films of the cervical spine showed widening between the joint space of atlas and odontoid processes. Magnetic resonance imaging revealed C1-C2 subluxation with syringomyelia. After bladder training and cervical orthosis, the symptoms improved.
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PMID:Atlantoaxial subluxation and syringomyelia in Down syndrome: report of one case. 130 27

A patient with a 2-year history of progressive ataxia and tingling in the right hand had prominent facial myokymia. Magnetic resonance imaging revealed syringomyelia and syringobulbia. After successful syringosubarachnoid shunting, the patient died of massive pulmonary embolism. Postmortem examination revealed a syrinx involving the spinal cord and lower half of the medulla; neither the facial nucleus nor facial nerve fibers were directly involved. We hypothesize that interruption of aberrant corticobulbar fibers in the medulla produced disinhibition of a rhythmic neural generator in the facial nucleus.
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PMID:Facial myokymia in syringobulbia. 218 81

Herniation of the hindbrain occurs when the lowest parts of the cerebellum and sometimes part of the medulla are moved downwards through the foramen magnum, a pressure difference acting across the foramen magnum moulding the tissues into a plug. It is suggested that the clinical course in both adults and babies with spina bifida may be explained by the hindbrain hernia acting as a valve.The term 'Chiari Type I deformity' is commonly used for an abnormality in which the tonsils and lowermost parts of the cerebellar hemispheres are prolapsed through a normal foramen magnum. Acute herniation may occur as a result of space-occupying lesions. Chronic herniation may be morphologically identical although it tends to be more severe. Sometimes it will produce few symptoms which often may be delayed so that the original causative lesion may not be apparent. Causes include bone softening, tumour, or previous meningitis. Birth injury is probably the commonest cause of the deformity, which presents clinically in adults.In infants with severe forms of spina bifida a hindbrain herniation is present. This abnormality may be called 'Chiari Type II deformity' or Arnold-Chiari deformity and is an intra-uterine abnormality in which the fourth ventricle and medulla are grotesquely herniated before they are properly developed and the foramen magnum is enlarged.The commonest clinical presentation of Chiari Type I deformity is syringomyelia, which is usually not diagnosed until adult life. Other presentations include syringobulbia, headache, oscillopsia, attacks of giddiness, lower cranial nerve palsies, and ataxia. Particularly characteristic are cough headache and cough syncope. Syringomyelia and syringobulbia in particular may be irreversible by the time they are diagnosed. Nevertheless, surgical decompression may be successful in relieving symptoms of headache, cough syncope, and long-tract compression; most cases of syringomyelia show some improvement and in others progression of the disease is arrested. Operative techniques for hindbrain herniation are discussed.Chiari Type II deformity is probably responsible for the progression of hydrocephalus after birth in the majority of babies with spina bifida. Measurement of pressure in the cerebrospinal fluid above and below the foramen magnum shows that intermittent pressure difference is commonly present at times of neurological deterioration. Surgical decompression of the hernia in adults allows correction of the valvular effect, which may be monitored by pressure measurements. In babies the associated hydrocephalus is usually so gross that it requires separate treatment, but pressure monitoring may be of value in assessing the state of the disease.
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PMID:Chronic herniation of the hindbrain. 701 51

A 28-year-old male was admitted to our hospital with the complaints of numbness of the left upper limb and gait disturbance. Neurological examination disclosed slight left dysmetria, truncal ataxia and sensory disturbance at the dermatome of C8 and Th1. CT and MRI scans showed a large mass lesion in the left posterior fossa, ventricular dilatation, cavum septi pellucidi and cavum Vergae, empty sella, cervical syringomyelia and left tonsilar herniation. The tumor which attached to cerebellar tentorium was totally removed and a histological diagnosis of meningothelial meningioma was made. Postoperative MRI scan demonstrated a disappearing of syringomyelia with the improved tonsillar herniation. Association of syringomyelia with brain tumor is relatively rare, so its pathogenesis was discussed.
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PMID:[Syringomyelia associated with tentorial meningioma]. 754 27

Osteogenesis imperfecta (OI) is a heritable disorder of bone development caused by defective collagen synthesis. Basilar invagination is an uncommon but devastating complication of this disease. The authors present a comprehensive strategy for management of craniovertebral anomalies associated with OI and related osteochondrodysplasias. Twenty-five patients with congenital osteochondrodysplasias (18 OI, four Hajdu-Cheney syndrome, and three spondyloepiphyseal dysplasia) and basilar invagination were evaluated between 1985 and 1995. The male/female ratio in this cohort was 1:1. The mean age at presentation was 11.9 years (range 13 months-20 years). Fourteen patients (56%) presented during adolescence (11-15 years of age). Symptoms and signs included headache (76%), lower cranial nerve dysfunction (68%), hyperreflexia (56%), quadriparesis (48%), ataxia (32%), nystagmus (28%), and scoliosis (20%). Four patients (16%) were asymptomatic. Seven (28%) had undergone previous posterior fossa decompression; one had also undergone ventral decompression. Imaging findings included basilar invagination (100%), ventral brainstem compression (84%), hydrocephalus (32%), hindbrain herniation (28%), and syringomyelia/syringobulbia (16%). Patients with hydrocephalus underwent ventricular shunt placement. Reducible basilar invagination (40%) was treated with posterior fossa decompression and occipitocervical fusion. Those with irreducible ventral compression (60%) underwent transoral-transpalatopharyngeal decompression followed by occipitocervical fusion. All patients improved initially. However, basilar invagination progressed radiographically in 80% (symptomatic in 24%) despite successful fusion. Prolonged external orthotic immobilization with the modified Minerva brace afforded symptomatic improvement and arrested progression of the deformity. The mean follow-up period was 5.9 years (range 1.1-10.5 years). Ventral brainstem compression in OI should be treated with ventral decompression, followed by occipitocervical fusion with contoured loop instrumentation to prevent further squamooccipital infolding. Despite fusion, however, basilar invagination tends to progress. Prolonged immobilization (particularly during adolescence) may stabilize symptoms and halt further invagination. This study represents the largest series to date addressing craniovertebral anomalies in OI and related congenital bone softening disorders.
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PMID:Basilar invagination in osteogenesis imperfecta and related osteochondrodysplasias: medical and surgical management. 917 Nov 73

The Chiari malformation, condition characterized by herniation of posterior fossa contents below the level of foramen magnum coexists very often with solitary or multiple syringomyelic cysts in spinal cord. The authors, on the basis of current opinions presented in literature and an own case present considerations concerning pathogenesis, operative procedures and prognostic factures which are of value in prediction of long-term outcome in patients which Chiari type I-Syringomyelia complex. The presence or absence of three preoperative signs as: muscular atrophy, ataxia and scoliosis facilitate, with confidence of 95%, prediction of long-term postoperative result. Preoperative incidence of all three above signs is much less favourable for outcome. In the presented case all unfavourable signs were observed preoperatively and, despite of good MRI-result, long-term outcome was only fair.
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PMID:[Type I Chiari malformation coexisting with syringomyelia: pathogenesis, treatment choice and prognostic factors for remote outcome in the light of own case]. 1035 45

The Chiari malformations are a group of central nervous system defects that involve the posterior cranial fossa. The clinical complaints range from headache, sensory changes, vertigo, ataxia, and imbalance to hearing loss. In patients with Chiari malformation, only those with type I malformation were born grossly normal, the neurological dysfunction developing gradually as they grew up, which spurred our interest. From August 1990 to September 1998, seven patients received surgical intervention at Kaohsiung Medical College Hospital under the impression of Chiari malformations. There were 4 males and 3 females, with age ranging from 7 years to 41 years. All these patients were diagnosed via magnetic resonance imaging and surgical findings revealed a hypoplasic vermis. Among them, 4(57%) were cranial nerve plasy, 2(29%) nystagmus, 6(100%) syringomyelia, 7(100%) scoliosis and one (14%) with hydrocephalus. Cerebellar ataxia and upper limbs weakness was noted in 5(71%) patients. Our surgical interventions included suboccipital craniectomy and dural opening(7/7), C1 posterior arch decompression (7/7), C4,5 laminectomy (1/7), and syringostomy(4/7). There was one mortality in a 41 y/o male with progressively lower cranial nerve(V, VII, IX) impairment as well as poor lung compliance. Other patients improved in their symptoms after follow-up periods of 6 months to 6 years. Our study shows that 1) surgical decompression was indicated in all patients with brain stem and lower cranial nerve dysfunction as well as central cord syndrome caused by syringomyelia; 2) a better result was achieved in those patients who received adequate nervous system decompression; and 3) much attention should be payed to cases with lower cranial nerves XI, X, XII impairment, because complications such as choking and aspiration pneumonia can happen.
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PMID:Surgical outcome of Chiari I malformations--an experience sharing and literature review. 1063 63

The Chiari I congenital malformation is characterized by caudal displacement of the cerebellar tonsils through the cervical canal. Although this malformation is often asymptomatic, coexisting syringomyelia can result in neurologic symptoms. We report a case of progressive ataxia with brainstem dysfunction in an adolescent female manifesting a severe Chiari I malformation with syrinx. Chiari decompression 4 years after initial presentation led to rapid improvement in most of her long-term symptoms. This case demonstrates the importance of consideration of Chiari I with syringomyelia in the differential diagnosis of progressive ataxia and brainstem symptoms.
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PMID:Chiari I malformation with syrinx. 1458 Jun 64

The authors report on the case of a patient evaluated for Valsalva maneuver-induced headache, dizziness, and ataxia. Neuroimaging revealed a Chiari I malformation without syringomyelia. A history of idiopathic hypertension was noted. After posterior fossa decompression, pathologically elevated blood pressure was absent, and at 24-month follow-up evaluation the patient remained normotensive. Although seemingly rare, this case illustrates that some patients with tonsillar ectopia may exhibit elevated blood pressure. Clinicians should consider hindbrain herniation a rare cause in idiopathic hypertension.
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PMID:Cessation of chronic hypertension after posterior fossa decompression in a child with Chiari I malformation. Case report. 1475 49


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