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1.
OBJECTIVE: To describe the appearance and frequency of skeletal abnormalities associated with the Marinesco-Sj?gren syndrome of cerebellar ataxia, congenital cataracts, mental and physical retardation and myopathy. SUBJECTS AND METHODS: Seventeen individuals affected with the disorder, of common ancestry and marked consanguinity, were found in an isolated area in southwest Alabama; 11 were available for radiologic examination of parts of the skeleton. The range and frequency of skeletal abnormalities thus demonstrated were tabulated. RESULTS AND CONCLUSION: A constellation of cranial and extracranial skeletal abnormalities-including a small posterior fossa, spinal abnormalities, gracile bones, elbow and hip valgus and asymmetric metacarpal and metatarsal shortening- can lead the radiologist or orthopaedist to suggest the diagnosis, especially if appropriate neurological/neuroradiological findings also are present.  相似文献   

2.
Marinesco-Sj?gren syndrome is rarely reported in the Middle East. This is the 2nd report of Marinesco-Sj?gren syndrome in an Arab family. The clinical features of 2 affected brothers are described. Electrophysiological studies of the 2 patients showed primarily myopathic changes, whereas sural nerve biopsy revealed segmental demyelination and axonal degeneration. The role of tissue biopsy and the relationship to different electrophysiological studies are discussed. Both patients were noticed to have abnormally short lateral 3 metatarsals, a feature not present in other healthy members of the family. We suggest that this feature should be considered part of the syndrome profile.  相似文献   

3.
Forty-two cases of medulloblastoma of posterior fossa in children are presented in this paper. Of the children, 28 were males and 14 females; their age ranged from 1.5 to 12 (mean 7.3) years. The foci found were in vermis (36) and cerebellar hemispheres (6). All of these children presented symptoms and signs of obviously increased intracranial pressure, and 31(77%) of them had the signs of cerebellar functional deficits. CT scan showed severe obstructive hydrocephalus in 39 cases. 42 children with posterior fossa medulloblastoma underwent surgical resection. Three died of postoperative prespiratory and circulatory failure, 39 made good recovery and received craniospinal radiotherapy and adjunct chemotherapy. Up to now, none died. Surgical intervention and operative procedures were emphasized particularly. Radiotherapy and chemotherapy were discussed also.  相似文献   

4.
Carotid and vertebral artery dissections typically occur in young adults after major trauma, although they can arise spontaneously or after trivial injury. Many patients with carotid dissections have minor symptoms such as a subject bruit or Horner's syndrome. Cephalic pain is also frequent and often inaugural in carotid dissection. However, extracranial dissection is a well recognised cause of ischaemic stroke. The diagnosis of dissection was based on angiographic findings. Noninvasive imaging also allows prompt and reliable diagnosis. Our goal was to demonstrate the spectrum of neuroradiologic (CT, MR and angiographic) findings in craniocervical arterial dissection and compare the diagnostic utility of CT, MR, MR angiography. Clinical data imaging studies, and outcome were reviewed and compared with the results in four patients with carotid artery dissection.  相似文献   

5.
PURPOSE: To characterize transient intraspinal subdural enhancement (potentially mimicking the subarachnoid spread of tumor) seen on MR images in some children after suboccipital craniectomy for posterior fossa tumor resection. METHODS: Radiologic and medical records of 10 consecutive children who had MR imaging for spinal staging after resection of posterior fossa tumor during a 9-month period were reviewed retrospectively. In addition, one case with similar findings of intraspinal enhancement on spinal staging MR images obtained at another institution was included in the review. RESULTS: Intraspinal enhancement thought to be subdural was seen in four of 10 patients undergoing spinal staging MR imaging 6 to 12 days after surgery. In these four patients, MR studies 50 to 18 days later, without intervening treatment, showed resolution of the abnormal enhancement. A fifth patient (from another institution) with similar intraspinal enhancement underwent CT myelography 4 days later, which showed no subarachnoid lesions. No metastases have developed in any of these five patients during the 2.5- to 3.5-year follow-up period. conclusions: From analysis of the MR appearance and on the basis of prior myelographic experience, we suggest an extraarachnoid, probably subdural, location of this enhancement. Awareness of this phenomenon will reduce the rate of false-positive diagnoses of metastatic disease. Preoperative spinal staging should be considered for patients undergoing suboccipital craniectomy.  相似文献   

6.
PURPOSE: To determine the frequency of cerebellar and cerebral abnormalities on brain imaging studies in children with congenital ocular motor apraxia. METHODS: Brain imaging studies were performed in 19 children with typical congenital ocular motor apraxia who were in the care of a visual impairment program at a children's hospital. Independent clinical review categorized the subjects as having partial (n = 10) or expanded (n = 9) congenital ocular motor apraxia on the basis of extent of associated speech or neurodevelopmental problems. Fifteen CT studies and 13 MR examinations of the brain performed in these children were reviewed independently by two pediatric neuroradiologists. Radiologic findings were agreed on by consensus. RESULTS: Cerebellar abnormalities were found in 12 of 19 cases. The cerebellar vermis was small in 10 children. A small cerebellar vermis was the only abnormality in five of 10 children with partial congenital ocular motor apraxia and in two of nine children with expanded congenital ocular motor apraxia. Among seven children with a small vermis examined with high-resolution MR imaging, the inferior portion of the vermis was preferentially involved in each case. Of these seven subjects, none of four with partial congenital ocular motor apraxia but two of three with expanded congenital ocular motor apraxia had an abnormality of the superior portion of the vermis. Miscellaneous supratentorial lesions affecting both gray and white matter were found in six subjects. Five of the 19 children had normal imaging findings. CONCLUSION: Inferior vermian hypoplasia is the most common abnormality in children with congenital ocular motor apraxia.  相似文献   

7.
OBJECTIVE: CT is often used after plain films to evaluate fractures of the tibial plateau. Because MR imaging can show associated soft-tissue injuries as well as fractures, we hypothesize that MR is superior to CT for imaging these injuries. Accordingly, we compared the efficacy of MR imaging and CT in 22 patients with tibial plateau fractures. SUBJECTS AND METHODS: CT with two-dimensional reconstruction and MR examinations were performed in 22 patients with tibial plateau fractures. The images were interpreted by four radiologists and two orthopedic surgeons. Findings on CT scans and plain films were used to determine the configuration of the fractures and to classify them according to the Schatzker system. This was done with findings on MR images and plain films at a separate session. The MR images were also evaluated for ligamentous and meniscal injuries. A qualitative side-by-side comparison of two-dimensional CT scans and MR images for depiction of fracture configuration was done. Imaging results were correlated with observations from physical examinations in all patients and with surgical findings in 12 patients. RESULTS: All of the six types of fractures of the Schatzker classification were observed in this series. Comparison of two-dimensional CT reconstructions and MR images for depiction of fracture configuration revealed that the two techniques were equal in 14 patients, MR imaging was superior to CT in five patients, and CT was superior to MR imaging in three patients (who had very complex and comminuted fractures). In addition, MR imaging showed 12 complete ligamentous tears and 15 partial ligamentous tears in 15 (68%) of the 22 patients. MR showed meniscal injuries in 12 (55%) of the 22 patients. CONCLUSION: MR imaging was equivalent or superior to two-dimensional CT reconstruction for depiction of fracture configuration in most patients. In addition, MR showed significant soft-tissue injuries. We believe that MR imaging is the preferable imaging technique for most patients with fractures of the tibial plateau.  相似文献   

8.
The authors used magnetic resonance imaging to measure gray and white matter volumes in cerebellar hemispheres and 4 vermian regions in 61 normal control (NC) men aged 23–72 years, 25 men with uncomplicated alcoholism (ALC), and 8 men and 1 woman with alcoholic Korsakoff's syndrome (KS). NC and ALC took quantitative gait and balance tests. Gray but not white matter volume declined with normal age in both hemispheres and anterior–superior vermis. ALC had gray but not white matter cerebellar hemisphere volume deficits, whereas KS had deficits in both tissue types. ALC and KS had gray and white matter volume deficits in anterior superior but not posterior inferior vermis. ALC had a 1 SD ataxia deficit, significantly and selectively correlated with white matter volume in anterior superior vermis. Regional distribution but not severity of cerebellar volume deficits is similar in alcoholic individuals whether or not complicated by KS and relates to ataxia. (PsycINFO Database Record (c) 2010 APA, all rights reserved)  相似文献   

9.
We describe two infants in whom rhombencephalosynapsis was diagnosed with MR imaging in vivo. In contrast to Dandy-Walker malformation, the vermian maldevelopment in this anomaly is characterized by an absence of the anterior vermis and a deficiency of the posterior vermis. The cerebellar hemispheres are fused. In an attempt to identify the pathogenesis of these anatomic manifestations, we question the traditional concept of the embryologic development of the cerebellar primordium.  相似文献   

10.
BACKGROUND AND PURPOSE: The purpose of our study was to determine whether MR studies in the neonatal period are predictive of the neuroradiologic sequelae and clinical outcome in premature and term infants with perinatal brain injury. METHODS: Thirty subjects (15 premature and 15 term infants) with abnormalities revealed by initial MR studies were reexamined approximately 1 year after birth with both MR imaging and a neurologic assessment. All initial MR studies were performed between 35 and 45 weeks corrected age in premature infants and within 28 days of life in term infants. The initial MR studies were evaluated for deep gray matter involvement, hemispheric parenchymal change, intracranial hemorrhage, and periventricular signal and/or morphologic changes. These MR findings were compared with the follow-up MR findings and with the neurologic outcome. RESULTS: The development of cerebral palsy in premature infants was related to the following initial MR findings: subependymal hemorrhage associated with parenchymal destruction, periventricular signal alteration with irregularity of the ventricular wall, and widespread cerebral infarction. These MR findings were predictive of the subtypes of cerebral palsy. In term asphyxiated infants, T2 signal alterations of the deep gray matter rather than T1 shortening and diffuse involvement of the hemispheres were predictive of an unfavorable outcome. Both in term and premature infants, focal hemispheric parenchymal lesions alone (including infarction and intracerebral, subdural, intraventricular, and subarachnoid hemorrhage) did not produce poor outcomes. CONCLUSION: MR studies performed at or near term in either premature or term infants with perinatal brain damage are effective in predicting both late neuroradiologic and clinical outcome.  相似文献   

11.
We present four cases with combined hypoplasia of the cerebellum and the ventral pons-pontocerebellar hypoplasia (PCH). PCH represents an autosomal recessive neurodegenerative disorder with fetal onset. The disease is rare, with less than 20 cases having been reported. The main findings of PCH and the inclusion criteria for our cases can be summarised as progressive microcephaly from birth, pontocerebellar hypoplasia documented by MRI and marked chorea, which may change, later in childhood, to more dystonic patterns. The cerebral cortex becomes progressively atrophic. Motor and mental development are delayed, and epilepsy, mainly tonic-clonic seizures, is frequent. The MRI features in all of our cases were: (1) Hypoplastic cerebellum situated close to the tentorium. The hypoplastic cerebellum has a reduced number of folia, in contrast to the normal number of thin folia in simple cerebellar atrophy. (2) The cerebellar hemispheres are reduced to bean-like or wing-like structures. The cerebellar hemispheres appear to 'float' in the posterior fossa. (3) Markedly hypoplastic ventral pons. (4) Slight atrophy of the supratentorial gyral pattern. (5) Dilated cerebromedullary cistern and fourth ventricle. (6) Delayed myelination of the white matter. (7) No significant disorganisation of brain architecture and no severe corpus callosum defect.  相似文献   

12.
PURPOSE: To document the accuracy of CT and MR of the sella turcica for detecting adrenocorticotropic hormone-secreting adenomas in Cushing disease. METHODS: The radiologic findings of the sella turcica prior to transsphenoidal surgery are reviewed in 141 patients who had biochemical evidence of pituitary-dependent Cushing disease. Axial thin-collimation CT scans with sagittal and coronal reformations before and after contrast enhancement were obtained in 125 patients. Seventy-eight patients had MR examinations with a 1.5-T superconducting magnet. In 11 of the patients gadolinium-enhanced MR scans were also obtained. The preoperative interpretation of the imaging studies was correlated with the surgical findings and patients follow-up. RESULTS: The sella turcica was enlarged in 43 cases (30%). In 125 patients reformatted or direct coronal thin-collimation CT scans were available. Seventy-eight of the patients had MR. In the 12 patients with pituitary macroadenomas, the accuracy of CT (n = 10) and MR (n = 10) in respect to detection of the lesion was 100%. Of the 98 microadenomas assessed by CT, 47 (48%) were directly depicted as distinct hypodense lesions. In only 31 of 73 cases (42%), however, could CT predict the precise anatomic location and extent of the lesions. Only patients in whom the hypercortisolism was corrected by later surgery were considered for the correlation analysis. Of the 52 microadenomas assessed by MR, 28 (53%) were directly depicted as distinct lesions of reduced signal intensity on T1-weighted images, and in only 21 of 41 cases (52%) did MR show good correlation to the surgical findings. Some degree of partially empty sella was found in 22% of the patients. CONCLUSIONS: Although both the sensitivity and the diagnostic accuracy of imaging methods of the sella turcica have been considerably improved in comparison with previous reports, they still provide only a minor contribution to the diagnosis and differential diagnosis of Cushing syndrome.  相似文献   

13.
OBJECTIVE: The purpose of this report is to describe the clinical history, treatment, pathology, and imaging in two cases of rare CNS infection caused by free-living amebas. The Naegleria fowleri and Acanthamoeba species cause primary amebic meningoencephalitis and granulomatous amebic encephalitis, respectively. We describe the neuroimaging findings of a case involving a nonspecific cerebral edema pattern in primary amebic meningoencephalitis and a case involving focal enhancing lesions in granulomatous amebic encephalitis. CONCLUSION: Primary amebic meningoencephalitis and granulomatous amebic encephalitis have a grave prognosis and, although rare, should be considered in the differential diagnosis for patients who present with appropriate histories and imaging findings, including nonspecific brain edema on CT in primary amebic meningoencephalitis and focal punctate enhancing lesions in the posterior cranial fossa on T1-weighted MR imaging in granulomatous amebic encephalitis.  相似文献   

14.
We describe MR findings in patients with Minamata disease who have been followed for a long time. All patients examined were affected after daily eating of a large quantity of methylmercury-contaminated seafood, from 1955 to 1958, and showed typical neurological findings. On MR images, the visual cortex, the cerebellar vermis and hemispheres, and the postcentral cortex are significantly atrophic in Minamata disease. The visual cortex is slightly hypointense on T1-weighted images and hyperintense on T2-weighted images, probably representing the pathologic changes of status spongiosus. MRI can demonstrate the lesions located in the calcarine area, cerebellum, and postcentral gyri, which are probably related to three of the characteristic manifestations of this disease: the constriction of the visual fields, ataxia, and sensory disturbance, respectively.  相似文献   

15.
PURPOSE: To report Adie syndrome as the initial sign of primary Sj?gren syndrome. METHODS: Case report. RESULTS: Adie syndrome was associated with necrotizing gingivitis and xerostomia. Antibodies against Ro (SS-A) were present. Prednisone and antimalarial drugs were ineffective in treating Adie syndrome but improved the necrotizing gingivitis. CONCLUSION: Search for Sj?gren syndrome is mandated in patients with Adie syndrome. The latter condition is likely related to ganglionitis, a mechanism responsible for peripheral nervous system involvement in primary Sj?gren syndrome.  相似文献   

16.
BACKGROUND: Patients with cerebellar hematomas may appear stable but may worsen suddenly. Whether certain clinical or CT scan findings predict worsening is not known. METHODS: We reviewed clinical and neuroimaging data in 72 patients with cerebellar hematomas at the Mayo Clinic from 1973 through 1993 to identify predictive features for neurologic deterioration. Patients presenting in coma and patients with vascular malformations or malignancies were excluded. Data were analyzed using chi-square or Fisher's exact test, with calculation of odds ratios with 95% confidence intervals. Multivariate logistic regression analysis was performed on appropriate variables. RESULTS: Thirty-three patients (46%) deteriorated, with a decrease in level of consciousness, new brainstem signs, or worsened motor response on the Glasgow Coma Scale. Clinical and neuroradiologic predictors for neurologic deterioration at p < 0.05 were admission systolic blood pressure greater than 200 mm Hg, pinpoint pupils and abnormal corneal or oculocephalic reflexes, hemorrhage extending into the vermis, hematoma size more than 3 cm in diameter, brainstem distortion, intraventricular hemorrhage, upward herniation, and acute hydrocephalus. Multivariate analysis demonstrated that hemorrhage located in the vermis (p = 0.03) and acute hydrocephalus (p = 0.0006) on admission CT scanning independently predicted deterioration. CONCLUSION: Patients with a cerebellar vermian hematoma or acute hydrocephalus are at high risk for neurologic deterioration. These patients should be carefully monitored and are more likely to require consideration for neurosurgical intervention.  相似文献   

17.
OBJECTIVE: We undertook this study to use MR imaging to determine the frequency of injury to the posterior ligament complex of the thoracolumbar spine in patients who have undergone acute thoracolumbar trauma. SUBJECTS AND METHODS: Sixty-eight patients with varying severity of thoracolumbar trauma were examined prospectively. The majority of injuries were related to motor vehicle accidents. The second most common cause was falls. Patients were examined with plain radiography and MR imaging. In addition to conventional MR imaging sequences consisting of T1-weighted and fast spin-echo T2-weighted sagittal and axial images, a fat-suppressed T2-weighted sagittal sequence was performed. The findings were correlated with surgery in six cases and with follow-up clinical examination that included physical examination and conventional anteroposterior and lateral radiographs. RESULTS: Posterior ligament complex injury was detected in 53% (n = 36) of all patients. Such injury was most common in patients with flexion-distraction (n = 15) and patients with dislocation fracture (n = 4). Of the patients with dislocation fracture, all had posterior ligament complex injury. Of the 24 patients with burst fractures, posterior ligament complex tear occurred in 42% (n = 10). Of the 23 patients with compression fractures, 26% (n = 6) had posterior ligament complex tear. Injury to the interspinous ligaments occurred with decreasing frequency in patients with injury to the supraspinous ligament, flaval ligaments, posterior longitudinal ligament, and anterior longitudinal ligament. Surgical findings correlated with MR imaging in all six patients who underwent surgery. CONCLUSION: Injury to the posterior ligament complex, which is often encountered in patients with burst and compression fractures, can be reliably revealed by MR imaging.  相似文献   

18.
A 67-year-old woman had had a neck mass for 10 years, which recently increased in size. Sonographic, CT, and MR examinations showed a mass in the carotid and posterior spaces (triangle) extending from below the submandibular gland to the supraclavicular fossa, displacing the common carotid artery and the sternomastoid anteriorly. The mass was solid, noncalcified with lobulated outlines, hypoechoic on sonograms, of soft-tissue density on CT scans, isointense on T1-weighted MR images, hyperintense on T2-weighted MR images, and enhanced mildly after injection of contrast material on CT and MR studies. Histologic examination revealed a carcinoma showing thymiclike differentiation, a rare tumor of the neck and thyroid gland.  相似文献   

19.
OBJECTIVE: We assessed the usefulness of chest radiographs for predicting whether high-resolution CT scans obtained with the patient prone would be valuable in assessing suspected diffuse lung disease. MATERIALS AND METHODS: In 100 consecutive patients undergoing high-resolution CT, findings on plain chest radiographs were classified as normal, possibly abnormal, or abnormal. CT scans obtained with the patient supine were assessed for the presence and distribution of lung abnormalities without knowledge of the plain radiographic classification. A second review of the CT scans was done with equal numbers of scans obtained with the patient prone and with the patient supine. The usefulness of the CT scans obtained with the patient prone for detecting lung disease was determined and related to the plain radiographic classifications. RESULTS: High-resolution CT scans obtained with patients prone were helpful in excluding or confirming posterior lung abnormalities in 10 (28%) of 36 patients who had normal findings on chest radiographs, five (28%) of 18 patients who had possibly abnormal findings on chest radiographs, and only two (4%) of 46 patients who had abnormal findings on chest radiographs. The proportion of patients who benefited from high-resolution CT scans obtained with the patient prone was significantly lower among the patients with abnormal findings on chest radiographs than among the patients with normal (p = .008) or possibly abnormal (p = .02) findings on chest radiographs. The two patients with abnormal findings on radiographs in whom CT scans obtained with the patient prone were helpful had minimal radiographic abnormalities. CONCLUSION: In patients with suspected diffuse lung disease, obtaining high-resolution CT scans with the patient prone may be useful when chest radiographs show normal findings, possibly abnormal findings, or minimal abnormalities indicative of diffuse lung disease. However, such scans are of little value in patients whose radiographs show abnormalities indicative of diffuse lung disease.  相似文献   

20.
OBJECTIVE: This study describes the technique and efficacy of split calvarial graft cranioplasty for the reconstruction of retrosigmoid/suboccipital defects following surgery for acoustic neuromas. STUDY DESIGN: A prospective study of the technique of split calvarial graft cranioplasty, its postoperative healing, and incidence of postoperative headache. METHODS: The technique requires splitting of the craniotomy bone flap into outer and inner table bone grafts. The combination of both bony grafts allows the coverage of a wider area of posterior fossa dura. This technique was used in 18 patients. All patients were followed for a minimum of 6 months. Eleven of 18 patients were followed for 1 year or longer. Four patients had three-dimensional computed tomography of their skull and area of split calvarial bone graft. RESULTS: One of 18 patients had a persistent disabling headache at 1 year postoperatively. A natural contour of the retrosigmoid area was achieved in all patients. Three-dimensional computed tomography scan, obtained 6 months postoperatively, showed total coverage of the retrosigmoid area and fusion of the bone flap to the surrounding skull. CONCLUSION: The technique of split calvarial grafting of posterior fossa defects is a feasible, safe, and effective way of separating the nuchal musculature and posterior fossa dura. The technique also allows the restoration of the contour and bony covering of the retrosigmoid area. The technique is a simple alternative to other types of cranioplasties aimed at reducing the incidence of postoperative headache in patients with acoustic neuromas.  相似文献   

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