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

Two cases of neurocysticercosis from Maharaj Nakorn Chiang Mai Hospital were reported with autopsy and surgical findings. The autopsy case was a 14-year-old girl who had a single cyst at the cisterna ambiens of the pineal region, causing hydrocephalus and severe brain edema accompanied with increased intracranial pressure and psychiatric problems but no definite localizing signs. The surgical case was a 35-year-old man with symptoms of increased intracranial pressure, localizing signs of ataxia of the left lower extremity and hearing loss in the right ear. Computed brain tomography showed a posterior fossa cyst with obstructive hydrocephalus. The cyst and the tissues from CP angle region were removed and sent to the Department of Pathology. Histopathologic appearance of both cases showed cysticercosis.
 ...
PMID:Cysticercosis in the posterior cranial fossa. 774 77

Cerebellar infarcts have been neglected for a long time and are now shown well by CT and especially MRI. Some infarcts involve the full territory supplied by a cerebellar artery. They are frequently complicated by edema with brain stem compression and supratentorial hydrocephalus, requiring at times emergency surgery, and are often accompanied by other medullary, medial pontine, mesencephalic, thalamic and occipital infarcts. On the other hand, partial territory infarcts are usually confined to the cerebellum and have a benign outcome with total recovery or minimal disability. They are more common than full territory infarcts. However, clinical presentations are similar to those full territory infarcts, differing mainly by the lack of drowsiness or unconsciousness. The main symptoms are vertigo, headache, vomiting, unsteadiness of gait and dysarthria. Signs include ipsilateral limb dysmetria, ipsilateral axial lateropulsion, ataxia and dysarthria. Vertigo is more severe and rotary in posterior inferior cerebellar artery territory infarcts, whereas dysarthria and ataxia are prominent in superior cerebellar artery territory infarcts. A few brain stem signs are sometimes added. In these territorial cerebellar infarcts, cardioembolism is the most common cause. Atherosclerotic occlusion comes next, involving the intracranial part of the vertebral artery and, less frequently, the lower basilar artery, both locations inaccessible to surgery. Other causes are artery to artery embolism from a vertebral artery origin stenosis, or the aortic arch, in situ intracranial branch atherosclerotic occlusion, and vertebral artery dissection. Border zone cerebellar infarcts occur in one third of the cases. They are small cortical or deep infarcts. They have the same symptoms and signs as territorial infarcts except for more frequent postural symptoms occurring over days, weeks or months after the ischemic event. The infarcts mainly have a thromboembolic mechanism, and sometimes have a hemodynamic mechanism: 1) focal cerebellar hypoperfusion due to large artery occlusive disease in more than half the cases, 2) small or end (pial) artery disease due to hypercoagulable state (thrombocythemia, polycythemia, hypereosinophilia, disseminated intravascular coagulation), arteritis or intracranial atheroma, and 3) rarely systemic hypotension due to cardiac arrest.
 ...
PMID:[Cerebellar infarctions and their mechanisms]. 809 Oct 85

Small asymptomatic cysts of the pineal gland represent a common incidental finding in adults undergoing computerized tomography or magnetic resonance (MR) imaging or at postmortem examination. In contrast, large symptomatic pineal cysts are rare, being limited to individual case reports or small series. The authors have reviewed 24 cases of large pineal cysts. The mean patient age at presentation was 28.7 years (range 15 to 46 years); 18 were female and six male. Presenting features in 20 symptomatic cases included: headache in 19; nausea and/or vomiting in seven; papilledema in five; visual disturbances in five (diplopia in three, "blurred vision" in two, and unilateral partial oculomotor nerve palsy in one); Parinaud's syndrome in two; hemiparesis in one; hemisensory aberration in one; and seizures in one. Four lesions were discovered incidentally. Magnetic resonance imaging typically demonstrated a 0.8- to 3.0-cm diameter mass (mean 1.7 cm) with homogeneous decreased signal intensity on T1-weighted images, increased signal intensity on T2-weighted images, and a distinct margin. Hydrocephalus was present in eight cases. The cysts were surgically excised via an infratentorial/supracerebellar approach (23 cases) or stereotactically biopsied (one case). Histological examination revealed a cyst wall 0.5 to 2.0 mm thick comprised of three layers: an outer fibrous layer, a middle layer of pineal parenchymal cells with variable calcification, and an inner layer of hypocellular glial tissue often exhibiting Rosenthal fibers and/or granular bodies. Evidence of prior hemorrhage, mild astrocytic degenerative atypia, and disorganization of pineal parenchyma were often present. Postoperative follow-up review in all 24 cases (range 3 months to 10 years) revealed no complications in 21, mild ocular movement deficit in one, gradually resolving Parinaud's syndrome in one, and radiographic evidence of a postoperative venous infarct of the superior cerebellum with ataxia of 1 week's duration in one. Of the patients referred for study, the cysts were most often initially misdiagnosed as a pineocytoma in eight and a pilocytic astrocytoma in three. Only two patients were correctly diagnosed as having pineal cysts. This stresses the importance of recognizing the histopathological spectrum of pineal cysts, as well as correlation with radiographic findings, if a correct diagnosis is to be attained.
 ...
PMID:Symptomatic glial cysts of the pineal gland. 811 58

The morbidity associated with gross total removal of pediatric posterior fossa tumors is well recognized although it is rarely isolated from other factors that comprise the management morbidity for these tumors. This study reviews (1) the operative and postoperative complications in 105 patients and (2) the neurological morbidity in a subset of 91 patients undergoing gross total removal of their tumor between 1982 and 1992. Gross total removal was achieved in 102 patients with a single procedure. Two patients with residual tumor underwent early repeat craniotomy for excision and 1 is being followed without repeat resection. Intra- and postoperative complications occurred in 33 patients and included hematoma requiring craniotomy (3), gastrointestinal hemorrhage (2), hydrocephalus requiring shunt placement (9), wound problems (4), and pseudomeningocele formation requiring additional treatment (5). Delayed onset hydrocephalus requiring shunting occurred in 2 patients and spinal deformity in 4 patients. Worsening of preoperative deficit (new cranial nerve palsies, worsening ataxia, bulbar dysfunction including apnea, mutism and seizures) occurred in 41% of patients operated on for primitive neuroectodermal tumors (PNET) (14/34), 53% of ependymomas (10/19), and 30% of astrocytomas (15/50). No patient who had a choroid plexus tumor was worsened by the procedure. Complete recovery of new postoperative deficits occurred in 14% of PNET (2/14), 50% of ependymoma (5/10) and 47% of astrocytoma (7/15), most often within 6 months of the procedure. Residual neurological morbidity, due to persistence of preoperative symptoms or due to deficits that occurred as the result of the surgical procedure, was assessed in a subgroup of 91 patients followed for an average of 48 months (2-147 months). This assessment did not include morbidity due to adjuvant therapy. Sixty-two percent of patients continued to exhibit abnormal cerebellar or bulbar signs. Forty-three percent of the total population exhibited limitation in function due to residual deficit. Only 38% of patients were both functionally normal and had a normal neurological examination at last follow-up.
 ...
PMID:The surgical and natural morbidity of aggressive resection for posterior fossa tumors in childhood. 814 78

Third ventricular ependymomas are rare tumors that have not been specifically examined. Four cases of these tumors are reported. The presenting symptoms included headache, ataxia, vertigo, and Parinaud's syndrome. All the patients underwent computed tomographic scanning and cerebral angiography, followed by craniotomy and microsurgical resection of the tumor. In addition, all patients had or developed symptomatic obstructive hydrocephalus requiring shunting procedures. Three of the patients are alive with a follow-up of 4 to 12 years. It is remarkable that these tumors are so rare, given that the ependymal surface area of the third ventricle is greater than that of the fourth. The management of these tumors should include aggressive surgical resection, radiation therapy, and cerebrospinal fluid diversion.
 ...
PMID:Ependymomas of the third ventricle. 817 98

Six patients with achondroplasia and symptoms suggestive of cervicomedullary junction compression are reviewed; these included three females and three males, with an average age of 8 years (range 7 months to 30 years). The mean duration of symptoms prior to intervention was 1.9 years. Symptoms included occipitocervical pain, ataxia, incontinence, apnea, and respiratory arrest. Radiological investigations consisted of plain films with flexion and extension views, pluridirectional tomography, thin-section computerized tomography, and magnetic resonance imaging. Typical findings included marked foramen magnum stenosis, ventrolateral cervicomedullary junction compression secondary to central and paramesial basilar invagination, and dorsal cervicomedullary junction compression secondary to ligamentous hypertrophy and invagination of the posterior atlantal arch. All patients underwent posterior fossa decompression and atlantal laminectomy. Surgery consistently revealed marked dorsal and paramesial overgrowth of the rim of the rim of the foramen magnum, with thickening and invagination of the atlantal posterior arch and a dense fibrotic epidural band resulting in dorsal cervicomedullary compression. Intraoperative ultrasonography was used to determine the extent of decompression required. Three patients required duraplasty. Three patients had concurrent hydrocephalus, two of whom had undergone ventriculoperitoneal shunting prior to surgical decompression of the posterior fossa. One patient developed a pseudomeningocele postoperatively, requiring serial lumbar punctures before it resolved. No patient developed craniovertebral instability following decompression. Improvement or resolution of symptoms was noted in all patients, with an average follow-up period of 4.8 years. Thus, cervicomedullary compression in patients with achondroplasia can be successfully treated with dorsal decompression of the craniovertebral junction. Dense epidural fibrotic bands are frequently noted in these cases and must be aggressively released to ensure satisfactory decompression.
 ...
PMID:Cervicomedullary compression in achondroplasia. 820 26

Radiological and clinical analysis was performed in 5 patients with posterior fossa dural arteriovenous fistulas (DAVFs) with isolated sinus segment due to sinus thrombosis, and their patho physiological, diagnostic, and therapeutic concerns were discussed. Patients ranged in age from 36 to 73 years old with a mean of 57 years and included 2 males and 3 females. Two patients presented with ataxia, one with ataxia and bruit, one with atypical facial pain, and one with parietal dysfunction. One patient had a history of hemorrhagic event. DAVFs were located at transverse-sigmoid sinus (3 cases), superior petrosal sinus (1 case), and straight sinus (1 case). They were fed by many branches of external carotid artery including middle meningeal artery, ascending pharyngeal artery, posterior auricular artery, occipital artery, meningeal branches of vertebral artery and posterior cerebral artery, and meningohypophyseal trunk of internal carotid artery. Shunt flow drained into contralateral transverse-sigmoid or supratentorial sinuses via the isolated venous segment through markedly dilated cortical and/or deep venous systems, which caused altered normal venous drainage pattern and venous hypertension. Transarterial embolization in multiple stages (mean 3.4) using n-butyl cyanoacrylate (NBCA) could alleviate symptoms in all cases. DAVFs were almost totally obliterated in 3 patients. Further embolization in one case, and surgical excision in one case were planed because of some residual dilated cortical venous drainage. Posterior fossa DAVFs with isolated sinus segment accompany markedly dilated cortical and/or deep venous systems. They could cause cerebellar, brainstem, or cranial nerve dysfunctions, and sometimes present distant supratentorial symptoms or hydrocephalus due to abnormal venous drainage and venous hypertension.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:[Posterior fossa dural arteriovenous fistula with isolated sinus segment]. 821 93

Infarction of the cerebellum comprises about 1.5 percent of all strokes. The symptoms are initially very similar to those of benign labyrinthitis, and the diagnosis is easy to miss. There are three major causes of clinical deterioration and death: Expansion of the infarct to the brainstem, swelling of the infarcted cerebellum with compression of the brainstem, and hydrocephalus. Surgical intervention may be lifesaving if hydrocephalus develops. Five patients admitted to our department are described. All described sudden vertigo or dizziness, which in three of them was accompanied by headache. All had ataxia. In four this was ipsilateral to the infarction and in one bilateral. Two had slight hemiparesis and plantar inversion. Two patients developed hydrocephalus. One was operated with external drainage. It is important to have this diagnosis in mind in all patients with acute labyrinthine symptoms. Acute CT scanning should be carried out if the patient becomes less conscious.
 ...
PMID:[Cerebellar infarctions]. 823 75

A 9-week-old Jack Russell terrier with progressive ataxia had histopathological lesions consistent with neuroaxonal dystrophy. Gross observation revealed absence of the septum pellucidum, hypoplasia of the corpus callosum and marked bilateral hydrocephalus. Light microscopy of the CNS revealed extensive axonal swellings principally in the gray matter of the brain stem where the sensory nuclei were most affected. Ultrastructurally, spheroids were identified as axonal terminals and probably dystrophic boutons; they were characterized by accumulations of membrane bound bodies. Clinical and morphological findings are similar to those identified in human infantile neuroaxonal dystrophy (Seitelberger's disease).
 ...
PMID:Neuroaxonal dystrophy in a Jack Russell terrier pup resembling human infantile neuroaxonal dystrophy. 846 99

Four cases of isolated fourth ventricle in neurocysticerocosis are reported (three after ventriculoperitoneal shunting, and one after hydrocephalus without shunting). Diagnosis was made based on computed tomography (CT) and/or CT cisternogram/ventriculogram. Three patients underwent fourth ventricle drainage (two died and the third remains well). The fourth patient refused surgical treatment and continues to have severe ataxia. Diagnosis, pathophysiology, and treatment of this entity in neurocysticercosis are discussed.
 ...
PMID:Isolated fourth ventricle in neurocysticercosis: pathophysiology, diagnosis, and treatment. 848 50


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>