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Biomedical subjects

J K Curé

Publications and source records attributed to J K Curé.

At least 19 recordsLinked to original sources
PubMed2001

Cervical spinal cord compression and the Hoffmann sign.

Little information exists about the ability of the Hoffmann sign to predict cervical spinal cord compression. The objective of this study was to determine the correlation between the Hoffmann sign and cervical spinal cord compression in a consecutive series of patients seen by a single spine surgeon. All new patients with complaints related to their cervical spine were included. Hoffmann sign was elicited by flicking the nail of the middle finger. Any flexion of the ipsilateral thumb and/or index finger was considered positive. All imaging studies were reviewed for spinal cord compression. Cord compression was defined as flattening of the AP diameter of the spinal cord coexisting with obliteration of CSF around the cord compared to normal levels. Of 165 patients, 124 patients had imaging of their spinal canal. Review by the spine surgeon found sensitivity of the Hoffmann sign relative to cord compression was 58%, specificity 78%, positive predictive value 62%, negative predictive value 75%. 49 studies were also read by a "blinded" neuroradiologist, the sensitivity was 33%, specificity 59%, positive predictive value, 26%, negative predictive value 67%. Although attractive as a simple method of screening for cervical spinal cord compression, the Hoffmann sign, in the absence of other clinical findings, is not in our experience a reliable test.

Cervical Vertebrae↗
PubMed2000

Lovastatin therapy for X-linked adrenoleukodystrophy: clinical and biochemical observations on 12 patients.

X-linked adrenoleukodystrophy (X-ALD) is a progressive demyelinating disorder whose neurological signs and symptoms can manifest in childhood as cerebral ALD or in adulthood in the form of a progressive myelopathy (AMN). The consistent metabolic abnormality in all forms of X-ALD is an inherited defect in the peroxisomal beta-oxidation of very long chain (VLC) fatty acids (>C(22:0)) which may in turn lead to a neuroinflammatory process associated with demyelination of the cerebral white matter. The current treatment for X-ALD with Lorenzo's oil aims to lower the excessive quantities of VLC fatty acids that accumulate in the patients' plasma and tissues, but does not directly address the inflammatory process in X-ALD. We have previously demonstrated that lovastatin and other 3-HMG-CoA reductase inhibitors are capable of normalizing VLC fatty acid levels in primary skin fibroblasts derived from X-ALD patients. Lovastatin can block the induction of inducible nitric oxide synthase and proinflammatory cytokines in astrocytes, microglia, and macrophages in vitro. In a preliminary report, we demonstrated that lovastatin therapy can normalize VLC fatty acids in the plasma of patients with X-ALD. Here we report our clinical and biochemical observations on 12 patients with X-ALD who were treated with lovastatin for up to 12 months. Our results show that the high plasma levels of hexacosanoic acid (C(26:0)) showed a decline from pretreatment values within 1 to 3 months of starting therapy with 40 mg of lovastatin per day and stabilized at various levels during a period of observation up to 12 months. The percentage decline from pretreatment values varied and did not correlate with the type of ALD gene mutation (point mutation versus gene deletion). In 6 patients, in whom red cell membrane fatty acid composition was studied, a mean correction of 50% of the excess C(26:0) was observed after 6 months of therapy suggesting sustained benefit. In a few patients who discontinued lovastatin therapy plasma C(26:0) levels reverted to pretreatment values suggesting a cause and effect relationship between these events. Two patients dropped out of the study claiming no clinical benefit, 1 was withdrawn due to adverse effects, and an adult patient with cerebral involvement died during the study. A 10-year-old boy with severe cerebral involvement showed worsening of his neurological status. All patients with AMN remained neurologically stable or showed modest subjective improvement. All patients who did not have Addison's disease at the time of enrollment maintained normal adrenal function throughout the study. The implications of our findings for developing an effective therapy for X-ALD are discussed.

Adrenoleukodystrophy↗
PubMed1997

Cystlike white matter lesions in tuberous sclerosis.

PURPOSE: To investigate the presence of small cystlike structures in the cerebral hemispheric white matter on MR images of patients with tuberous sclerosis. METHODS: The MR images of 18 consecutive patients with tuberous sclerosis were reviewed retrospectively. RESULTS: Eight of the 18 patients were found to have cystlike structures in the cerebral white matter. The signal intensity of these lesions was isointense with cerebrospinal fluid on T1-, proton density-, and T2-weighted images. Four patients were imaged with a fluid-attenuated inversion recovery sequence, which in each case also showed fluid-type signal in these areas. Three of the patients had CT for correlation, and these scans supported the diagnosis of cystic lesions. Cysts ranged in number from one to 12 per patient and were usually smaller than 1 cm. The most common location was adjacent to the occipital horn or trigone of the lateral ventricle (six of eight patients). Less frequent sites were near the frontal horns, in the corpus callosum, and in the deep white matter near the body of the lateral ventricle. Cysts in five patients were either immediately adjacent to a cortical tuber or in the center of a white matter dysplastic lesion. A cyst in one patient had septa, and none of the cysts enhanced. CONCLUSIONS: Cystlike structures in the cerebral hemispheric white matter were seen on the MR images of 44% of 18 patients with tuberous sclerosis. Whether these findings represent cystic degeneration of dysplastic tissue or are unrelated to the disease process of tuberous sclerosis is unknown. More than one pathogenesis may exist.

Adult↗
PubMed1996

Petrous carotid canal stenosis in malignant osteopetrosis: CT documentation with MR angiographic correlation.

PURPOSE: To study the association between petrous carotid canal (PCC) and internal carotid artery (ICA) stenoses in patients with malignant osteopetrosis. MATERIALS AND METHODS: Mean and minimum PCC diameters obtained from cranial computed tomographic (CT) scans in 20 patients were compared with similar measurements in 52 control subjects. ICA caliber, evaluated with magnetic resonance (MR) arteriography, was correlated with age and PCC dimensions. RESULTS: There was a statistically significant difference between patient and control PCC diameters. There was a strong positive correlation between age and PCC diameter in the control subjects, but only a weakly positive correlation in the patients. One or both ICAs were stenotic on all patient MR arteriograms. MR angiographic stenosis grade correlated positively with age but not with PCC diameters. CONCLUSION: PCC and ICA stenoses occur frequently in patients with malignant osteopetrosis. Bony overgrowth or a "persistent fetal state" may produce the PPC stenoses. The findings do not support progressive PCC narrowing in these patients.

Adolescent↗
PubMed1996

MR of nasolabial cysts.

We report the MR imaging findings in two cases of nasolabial cysts. Demonstration of their extraosseous location with cross-sectional imaging should prevent confusion with maxillary cysts and obviate unwarranted dental or maxillary surgery.

Adult↗
PubMed1995

Magnetic resonance imaging in patients with low-tension glaucoma.

OBJECTIVE: To study diagnoses and anatomic findings found on magnetic resonance imaging in patients with low-tension glaucoma. PATIENTS: We included in this study magnetic resonance images of 20 consecutive patients with low-tension glaucoma. We individually matched each patient with low-tension glaucoma to a control with normal ocular findings who had magnetic resonance imaging for reasons unrelated to the visual pathway. DESIGN: We studied axial and coronal images of the orbit and optic nerve with digitizing software (Image-Pro Plus, Media Cybernetics, Silver Spring, Md). Statistical evaluation was with a Wilcoxon Signed Rank Test for anatomic findings and a McNemar Test for diagnosis. RESULTS: We found no difference between groups in the optic nerve diameter or length, the carotid artery area, or the distance from the optic nerve to the carotid artery (P > .05). Left optic nerve area was greater in the control patients than patients with low-tension glaucoma (P = .026). The prevalence of intracranial abnormalities, including meningioma, aneurysm, and arteriovenous abnormality, was similar between groups (P > .05). However, diffuse cerebral small-vessel ischemic changes were found more in patients with low-tension glaucoma (n = 8) than control patients (n = 1) (P = .0196). CONCLUSIONS: This study proposes a hypothesis that cerebral small-vessel ischemia is more common in patients with low-tension glaucoma and potentially reflects indirectly a vascular cause of the optic nerve head damage at least in a subgroup of patients. Importantly, further research still is required to provide direct evidence for a vascular cause involved in low-tension glaucoma.

Aged↗
PubMed1995

Nonneoplastic intracranial cysts and cystic lesions.

This article reviews the gamut of cystic lesions, unrelated to neoplastic disease, that are found in the brain, subarachnoid space, and ventricles. The utility of MRI in diagnosing these entities is shown.

Arachnoid Cysts↗
PubMed1995

MR of cystic aberrant cervical thymus.

The clinical, MR imaging, surgical, and histologic findings in two cases of cystic aberrant cervical thymus were reviewed. Aberrant cervical thymic tissue was sharply circumscribed and extended deep to the posterior pharyngeal wall at the level of the piriform sinus in both cases. Both lesions had cyst contents that were of increased signal intensity on T1-weighted images. Solid components displayed MR signal characteristics identical to normal thymus.

Child↗
PubMed1994

Normal and variant anatomy of the dural venous sinuses.

The normal and variant anatomy of the cerebral veins and dural venous sinuses is poorly understood by many radiologists. Beginning with a discussion of cerebral venous anatomy, this review illustrates clinically pertinent anatomy of the cerebral sinovenous system. Various methods of imaging cerebral veins and dural venous sinuses are described. Techniques and pitfalls of MR venography are emphasized.

Cerebral Angiography↗
PubMed1994

Congenital and acquired abnormalities of the dural venous sinuses.

The imaging features of a variety of dural venous sinus (DVS) abnormalities are reviewed. Congenital and heritable diseases affecting the DVS, tumor-related sinus compression, and traumatic injuries of the DVS are discussed. The causes, clinical manifestations, and imaging findings in cerebral sinovenous thrombosis are described, and pertinent imaging techniques and pitfalls are illustrated. Etiologic theories about the formation of dural arteriovenous malformations are discussed and their imaging features are demonstrated.

Arteriovenous Fistula↗
PubMed1992

Encephalocraniocutaneous lipomatosis and the Proteus syndrome: distinct entities with overlapping manifestations.

We have studied three children with cutaneous (epidermal nevi), subcutaneous (lipomas, plantar skin thickening), vascular (hemangioma, lymphangioma), skeletal (osteoma, exostosis, localized hypertrophy), and neurological (hydrocephaly, lissencephaly, partial agenesis of the corpus callosum) developmental defects associated with the Proteus syndrome and related hamartoneoplastic conditions. We compared our findings in these three patients with those of 50 others with Proteus syndrome and nine with encephalocraniocutaneous lipomatosis (ECCL) reported in the literature. We found that Proteus syndrome and ECCL have distinct identities even though some clinical manifestations are shared by both and a few patients have manifestations of both conditions.

Bone Neoplasms↗
PubMed1990

Variables that affect the expansion rate and outcome of small abdominal aortic aneurysms.

Seventy-three patients with small (less than 6 cm in diameter) abdominal aortic aneurysms (AAAs) were selected for nonoperative management and followed up with sequential ultrasound size measurements. Fifty-four men and 19 women, 51 to 89 years of age (mean 70 years), had an initial mean AAA size of 4.1 cm (anteroposterior) x 4.3 cm (lateral) diameter, with a calculated elliptic cross-sectional area of 14.3 cm2. After a mean of 37 months of follow-up, AAA area increased at a mean rate of 20% per year (3 cm2 yr; 0.4 to 0.5 cm/yr diameter). Expansion rate was not affected by initial aneurysm size. During follow-up, only 3 patients (4%) required urgent operation (1 died), 26 patients (36%) died of non-AAA causes, and 26 patients (36%) underwent elective AAA repair because of progressive size increase (1 died). Elective operations were performed at the rate of 10% per year, when mean AAA size had increased to 22 cm2 (5.1 cm in diameter). Multiple regression analysis of clinical parameters available at presentation indicated that subsequent elective AAA repair was predicted by younger age at diagnosis and larger initial aneurysm size. As anticipated, patients who underwent surgery had more rapid aneurysm expansion (5.3 cm2/yr) compared with patients who did not undergo surgery (1.6 cm2/yr; p less than 0.05). This difference was caused by more rapid expansion during later follow-up intervals among patients selected for operation and was not predicted by the change in aneurysm size observed during initial ultrasonographic follow-up. Final aneurysm size was predicted by initial size, duration of follow-up, and both systolic and diastolic pressure.(ABSTRACT TRUNCATED AT 250 WORDS)

Actuarial Analysis↗
PubMedDate not supplied

Cranial MR imaging of osteopetrosis.

BACKGROUND AND PURPOSE: The purpose of this study was to describe the cranial MR imaging manifestations of osteopetrosis. These features have not previously been reported in the literature. METHODS: Cranial MR studies, obtained with a uniform imaging protocol, were reviewed in 47 patients with osteopetrosis. Thirty-four patients had autosomal recessive (malignant) osteopetrosis (AROP), seven had intermediate osteopetrosis (IOP), and six had either type I or type II autosomal dominant osteopetrosis (ADOP I or II). The prevalence of abnormalities was tabulated and compared with the specific osteopetrosis variants. RESULTS: All patients with osteopetrosis had thickening and sclerosis of the calvaria. Ventriculomegaly, tonsillar herniation, proptosis, and dural venous sinus stenosis were observed in the majority of patients with AROP and ADOP I. Optic nerve sheath dilatation occurred in many of the patients with AROP and in all patients with ADOP I. Acquired cephaloceles were also observed only in these two groups. Optic nerve atrophy and optic canal stenosis were observed in a majority of patients with AROP, IOP, and ADOP II. Middle ear fluid was prevalent in AROP and IOP, present in over half the patients in each group. Features seen most prevalently, or exclusively, in AROP included stenosis of the internal carotid and vertebral arteries and extramedullary hematopoiesis. CONCLUSION: The cranial MR imaging features of osteopetrosis are both shared and unique among the various subtypes of the disease. The specific cranial and intracranial manifestations reflect the predominant calvarial or skull base patterns of bone thickening. The unique features seen in patients with AROP probably reflect the early age of onset and the greater severity of this form of the disease.

Adolescent↗
PubMedDate not supplied

Auditory dysfunction caused by multiple sclerosis: detection with MR imaging.

We reviewed the MR examinations of 167 patients who presented over a 3-year period with a chief symptom of hearing loss and/or tinnitus. In 14 of these patients the only MR abnormality was the presence of multiple parenchymal high-signal foci on T2-weighted images. Nine of the 14 had clinical evidence of multiple sclerosis; the remaining five had no clinical evidence of multiple sclerosis. Lesions in the auditory pathways, potentially responsible for the patients' symptoms, were identified in only five cases. We recommend T2-weighted images of the whole brain in addition to T1-weighted images of the internal auditory canals and cerebellopontine angles in patients with hearing loss. In some patients, lesions found at higher levels in the periventricular white matter may provide the only clue to the origin of auditory abnormalities.

Adult↗