Gorlin syndrome: unusual manifestations in the sella turcica and the sphenoidal sinus.
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Adult↗
Biomedical subjects
Y Numaguchi
Publications and source records attributed to Y Numaguchi.
Repeat intra-arterial papaverine for recurrent cerebral vasospasm after subarachnoid haemorrhage.
We assessed the prevalence of recurrent vasospasm following failure of intra-arterial papaverine and the efficacy of repeat intra-arterial infusions of papaverine for control of recurrent vasospasm. Of 24 patients treated with intra-arterial papaverine for vasospasm following aneurysm surgery, 12 did not improve clinically after the initial treatment; 9 received second or third infusions on consecutive days; 6 received only a second infusion; and 3 received a third. Superselective infusion into the intracranial arteries was performed in all nine cases. Despite angiographic improvement after the initial or second infusions, all nine patients showed varying degrees of recurrent vasospasm at the time of the second or third treatment. Within 24 h of a second infusion, three of the six patients had significant clinical improvement, and one of these showed marked improvement soon after a third infusion. Our preliminary results suggest that repeat papaverine infusion may be a way of controlling recurrent or recalcitrant vasospasm.
Adult↗
Phenotypic variability of CADASIL and novel morphologic findings.
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a non-arterio-atherosclerotic, non-amyloidotic arteriopathy affecting preferentially the small arteries and arterioles of the brain. The morphologic hallmark is the presence of a characteristic granular alteration of the arterial media that ultrastructurally corresponds to the accumulation of electron-dense material surrounding the smooth muscle cells. Although the presence of this granular osmiophilic material (GOM) was originally described as limited to brain vessels, identical electron microscopic findings have been demonstrated in the media of peripheral tissue arteries, allowing for a pathologic diagnosis of the disease by a simple skin, muscle or nerve biopsy. We report some atypical features identified in our CADASIL patients that broaden the phenotypic expression of this disease. Firstly, we identified a cortical infarct in an otherwise typical CADASIL patient. Secondly, we observed GOM in skin arteries of a 30-year-old man with hemiplegic migraine, the son of a woman who had died with CADASIL. This confirms that it may be possible to diagnose the disease at a preclinical stage by the ultrastructural evaluation of peripheral tissue biopsy material, particularly for individuals for whom there is a supporting family history. Thirdly, ultrastructural examination of the skin, and subcutaneous and striated muscle of an unrelated and apparently sporadic patient with neuropathologic and neuroradiologic evidence of CADASIL in meningeal and cerebral vessels failed to reveal diagnostic lesions in peripheral arteries. Thus, the possibility of a false-negative pathologic diagnosis in patients with a clinicoradiologic diagnosis of CADASIL, if one relies solely on a peripheral tissue biopsy, does exist. Additionally, we have identified heat shock proteins (Hsp70 and alphaB crystallin) and ubiquitin in the vascular myocytes of affected arteries. B crystallin also seemed to be deposited extracellularly, which suggests that GOM also might be immunoreactive for alphaB crystallin.
Adult↗
Moyamoya disease in the United States.
The epidemiology and radiological features of Moyamoya disease (MMD) in the US were investigated. This study encompassed 98 cases; 26 were newly collected from eight US institutions and 72 were previously reported in the US literature. The patients ranged in age from 6 months to 67 years with age peaks in the first, third and fourth decades. MMD was seen in various ethnic groups and females were more commonly involved (71%) than males. A specific etiology could not be determined in most cases but arteriosclerosis and use of oral contraceptives were occasional associations. On angiography and/or magnetic resonance angiography (MRA), carotid arterial stenosis or occlusion was seen bilaterally in 95 cases (97%) and unilaterally in three. On MR or MRA, internal carotid steno-occlusive lesions were well demonstrated in all cases but Moyamoya collateral vessels (MMVs) were visualized in only 65% of the patients. MMVs in the basal ganglia and thalami were best demonstrated on T1 weighted images. Parenchymal lesions were seen in all patients and were often bilateral. With advances in MR techniques and increasing awareness of diagnostic guidelines, MMD will be diagnosed more frequently than before in the US.
Adolescent↗
A case of amiodarone-induced thyrotoxicosis successfully treated with prednisolone.
We describe a patient who was treated with amiodarone for ventricular arrhythmia based on arrhythmogenic right ventricular dysplasia and who subsequently developed severe amiodarone-induced thyrotoxicosis. Discontinuation of amiodarone resulted in sustained ventricular tachycardia, which was successfully treated with a DC electrical shock, and subsequently atrial fibrillation, leading to brain embolism due to occlusion of the left middle cerebral artery. Combination treatment with amiodarone and prednisolone was effective both in reducing the serum concentration of thyroid hormones and in improving the patient's general condition. As the use of amiodarone becomes more widespread, treatment with prednisolone for this kind of thyrotoxicosis, which is resistant to conventional treatment, will be required increasingly frequently because iodine overload of the thyroid gland persists for some time after discontinuation of amiodarone treatment.
Adult↗
Prevalence of activated protein C resistance in acute myocardial infarction in Japan.
To investigate the possibility that activated protein C (APC) resistance due to the factor V could be an important predisposing factor in acute myocardial infarction (AMI), we have retrospectively examined the prevalence of APC resistance with protein C, protein S and antithrombin III deficiency and antiphospholipid antibody syndrome in AMI patients (< or = 50 years) admitted to our hospital over the past 7 years. Forty-seven patients were enrolled in the study. We divided the patients into two groups, warfarin group (group A) and a non-warfarin group (group B). APC resistance is defined as when the APC ratio is below or equal to the cut-off value 2. APC resistance was not detected in either group. The prevalence of an APC ratio below or equal to 2.5 was 16.7% (1 case) in group A and 24.4% (10 cases) in group B. The prevalence of protein C deficiency was 5.0% (2 cases) in group B. Two cases (5.0%) in group B had protein S deficiency. Antithrombin III deficiency was not detected in either group. The prevalence of antiphospholipid antibody syndrome measured by APTT was 40.4% (19 cases). We compared the AMI patients with 97 healthy volunteers (< or = 50 years old) without any thromboembolic events or bleeding tendency in their past history. No significant difference were found between these groups and the volunteers. APC resistance is a major cause of venous thromboembolism in Europe and the United States, while in Japan it is believed to be a minor cause of arterial thromboembolism.
Adult↗
Radiosurgery for cerebral arteriovenous malformations: assessment of early phase magnetic resonance imaging and significance of gadolinium-DTPA enhancement.
PURPOSE: To evaluate the initial changes within the nidus of arteriovenous malformations (AVMs) and to assess the reaction to the brain tissue surrounding AVMs after radiosurgery by serial magnetic resonance (MR) imaging. METHODS AND MATERIALS: Twenty-one patients, treated using 60Co gamma knife unit with cerebral AVMs, were retrospectively evaluated. Forty-seven follow-up MR images of the 21 patients were performed including 10 patients with two or more serial gadolinium enhanced studies (Gd-MR). Two or more sequential MR angiographies (MRA) were obtained in 13 patients. Three-dimensional (3D) time-of-flight MRA and two-dimensional (2D) phase contrast MRA were used in 13 patients for evaluating the flow changes of AVMs. The follow-up period after radiosurgery ranged from 3 to 30 months (average 10.8 months) and the interval time of MRI ranged from 34 days to 13 months (average 4.9 months). RESULTS: Reduction of nidus size was observed in 14 of 21 patients (67%) between 4 to 13 months on spin echo (SE) images. Complete obliteration was observed on SE images in 4 of these 14 patients; three were confirmed by conventional angiography. New hyperintense areas surrounding the nidus on T2s-weighted images (T2WI) developed in 9 of the 14 patients who showed nidus reduction between 5 to 17 months after radiosurgery; in three patients, size of the hyperintense area started to decrease between 6 to 7 months after its appearance. Probable radiation necrosis of pons developed in one patient 26 months after radiosurgery. The irradiated area within the AVM nidus was significantly enhanced in 8 of the 10 patients who underwent Gd-MR. The degrees of enhancement within the nidus increased with time in 7 of the 10 patients. Overall, total enhancement of irradiated areas was observed in four of the 10 patients; in three of the four, the enhancement decreased in size and degree, indicating nidus reduction. In three patients who had a partial volume irradiation within the nidus, the enhancing areas corresponded with the exact portions of irradiated volume. The nidus reduction was observed in 7 of the 13 patients on MRA during 5 to 13 months after radiosurgery. MRA was more useful compared to SE images in four of the seven patients in evaluating the size change of AVM nidus, feeding arteries, and draining veins. CONCLUSION: Magnetic resonance imaging and MRA were useful in assessing the progress of nidus reduction. T2-weighted imaging was sensitive to radiation-induced reaction in and around the AVM nidus. The enhancement within the AVM nidus on Gd-MR may represent the initial sign of nidus reduction and demonstrates the exact location of irradiation in the nidus. The changes of the enhancement pattern are presumed to represent the processes of nidus reduction and irradiated reaction within the AVM nidus.
Adolescent↗
Detection of intracranial internal carotid artery and middle cerebral artery vasospasm following subarachnoid hemorrhage.
Little is known about the accuracy of transcranial Doppler (TCD) sonography in detecting intracranial internal carotid artery (IICA) and middle cerebral artery (MCA) vasospasm. TCD was performed in 49 patients with subarachnoid hemorrhage to evaluate 90 IICAs and 87 MCAs during the vasospasm period. When a mean velocity of at least 90 cm/sec was used to indicate IICA vasospasm, there were 11 positive, 42 negative, 4 false-positive, and 33 false-negative results. Sensitivity was 25% and specificity was 93%. When a mean velocity of at least 120 cm/sec was used to indicate MCA vasospasm, there were 15 positive, 45 negative, 3 false-positive, and 24 false-negative results (15 operator errors). Sensitivity was 38.5% and specificity was 93.7%. When the diagnostic criterion was changed to at least 130 cm/sec, specificities were 100% (IICA) and 96% (MCA) and positive predictive values were 100% (IICA) and 87% (MCA). The authors conclude that TCD accurately detects IICA and MCA vasospasm when flow velocities are at least 130 cm/sec. However, its sensitivity may be underestimated and the importance of operator error, overestimated.
Adult↗
Detection of vasospasm by transcranial Doppler sonography. The challenges of the anterior and posterior cerebral arteries.
Little information exists on the utility of transcranial Doppler sonography (TCD) in detecting anterior (ACA) and posterior cerebral artery (PCA) vasospasm following subarachnoid hemorrhage. During the period at risk for vasospasm, 53 patients with subarachnoid hemorrhage who had technically adequate TCD performed within 24 hours of cerebral angiography, allowing evaluation of 87 ACAs and 84 PCAs, were studied. ACA and PCA vasospasm were defined by mean blood flow velocities of at least 120 cm/sec and at least 90 cm/sec, respectively. For detection of ACA vasospasm, sensitivity was 18% and specificity was 65%. For PCA vasospasm, sensitivity was 48% and specificity was 69%. False-positive findings for occlusion accounted for 12 (92%) of 13 ACA of false-positive results and 7 (37%) of 19 PCA false-positive results, and were most often due to anatomical factors and operator error or inexperience. After exclusion of both true-positive and false-positive findings for occlusion and changes in the diagnostic criterion to at least 130 cm/sec for ACA vasospasm and at least 110 cm/sec for PCA vasospasm, specificity improved for both types of vasospasms (100 and 93%, respectively). However, the sensitivity of TCD to detect ACA and PCA vasospasm is limited by a variety of anatomical, technical, and other factors. It is concluded that TCD is highly specific in detecting both ACA and PCA vasospasm on arteries that can be insonated.
Adult↗
Unusual manifestation of a vein of Galen malformation: value of CT angiography.
We describe a neonate with aneurysmal dilatation of the vein of Galen with arteriovenous fistulous sites located at the superior vermian vein. Helical CT angiography was useful for evaluating the anomalous vessels.
Aneurysm↗
Infarcted spinal schwannoma: an unusual MR finding.
We present a case of infarcted schwannoma of the thoracic spine in a patient with acute cord compression. MR images did not enhance after contrast administration, which is highly atypical for schwannoma, but was in keeping with the pathologic findings in this case.
Acute Disease↗
MRI of silicone vitreous ocular implants with phantom correlation.
We imaged two patients who had silicone oil injected their vitreous cavities. The images demonstrated an increased chemical shift artifact. We developed and tested an in vitro silicone oil model in an attempt to explain this. To our knowledge, this artifact has not been previously described. We constructed a phantom using medication cups containing water, lard and two types of silicone. Background control baths of water and lard were used to simulate different local environments. The phantoms were imaged with T1-and T2-weighted and T1-weighted fat suppression sequences. The artifacts were due to the pronounced chemical shift of silicone in relation to intraconal fat. Our model accurately reproduced our clinical images. Spectral analysis of the silicone model revealed different resonating frequencies for fat/silicone, thereby explaining the pronounced chemical shift artifact.
Artifacts↗
Posterior fossa glioblastoma multiforme: MR findings.
PURPOSE: To characterize the MR findings of glioblastoma multiforme in the posterior fossa. METHODS: MR studies of nine patients with surgically proved posterior fossa glioblastoma multiforme were retrospectively evaluated. MR characteristics studied included tumor location, signal intensity, enhancement pattern, and presence of intratumoral hemorrhage, as well as presence of secondary hydrocephalus or metastatic spread. RESULTS: The tumors were located in the median portion of the cerebellum or brain stem in eight cases. Six extended into the fourth ventricle. Hydrocephalus was seen in four cases. Six cases demonstrated decreased T1- and increased T2-weighted signal intensities. Three cases demonstrated mixed signal intensities suggesting intratumoral hemorrhage. All of the eight patients who received contrast showed moderate to marked heterogeneous ringlike enhancement suggesting intratumoral necrosis. Multicentric/multifocal lesions or extraaxial metastases were identified in three of the nine cases, and there was extracranial extension into the cervical region in one case. CONCLUSION: Glioblastoma multiforme is a rare tumor in the posterior fossa. Differentiating it from metastatic tumor or malignant astrocytoma was difficult. However, combination of heterogeneous and ringlike enhancement, midline location, poorly defined margin, tumoral hemorrhage, concomitant multicentric/multifocal lesions, and extraaxial or extracranial metastasis may be clues for the prospective diagnosis of glioblastoma multiforme.
Adult↗
Intraarterial papaverine infusion for cerebral vasospasm after subarachnoid hemorrhage.
PURPOSE: To evaluate the techniques and efficacy of intracranial intraarterial papaverine infusion for symptomatic vasospasm after subarachnoid hemorrhage caused by aneurysm rupture. METHODS: Papaverine was infused on 19 occasions in 14 patients, 6 hours to 2 days after spasm became apparent clinically. Sixty vascular territories were treated. Infusion was made into the supraclinoid internal carotid artery 20 times, cavernous internal carotid artery once, selective A1 anterior cerebral artery 8 times, M1 middle cerebral artery 7 times, and basilar artery 3 times. Papaverine doses ranged from 150 to 600 mg and exceeded 400 mg on 8 occasions. RESULTS: Angiographic improvement occurred in 18 (95%) of the 19 treatment sessions: results were excellent in 3 sessions, moderate in 8, and mild in 7. The best angiographic results often were obtained with superselective infusion, although angiographic results did not always correlate with clinical response. Seven (50%) of the 14 treated patients showed dramatic acute clinical improvement within 24 hours of papaverine therapy, and there was no clinical evidence of recurrent vasospasm in these patients. Recurrence of angiographic vasoconstriction was demonstrated in three patients; one showed marked clinical improvement after a second treatment. There were no episodes of systemic hypotension in any of the cases. Monocular blindness developed in one patient because of papaverine infusion near the ophthalmic artery. CONCLUSIONS: Papaverine was effective in dilating narrowed arteries in most patients with symptomatic vasospasm caused by subarachnoid hemorrhage. This series showed encouraging clinical results with no recurrence of neurologic deterioration in those patients who responded well to papaverine. Superselective infusion appears to be indicated in some cases for adequate papaverine delivery.
Adult↗
Spinal epidural abscess with gadolinium-enhanced MRI: serial follow-up studies and clinical correlations.
We reviewed serial MRI with and without gadolinium-DTPA in eight patients with spinal epidural abscess and correlated the findings and the clinical manifestations. In four patients, diffuse abscesses spanned four vertebral bodies or more; the others had focal abscesses associated with osteomyelitis and/or diskitis. In three of the four patients with diffuse abscesses, MRI (NCMRI) showed diffuse encasement of the subarachnoid space. Contrast-enhanced MRI (CEMRI) demonstrated linear enhancement surrounding unenhanced pus. In the four patients with focal abscesses, CEMR delineated the inflammatory process more clearly than NCMR. On follow-up studies, decrease in abscess size and better visualization of the subarachnoid space correlated with clinical improvement in both diffuse and focal abscesses. Despite clinical improvement, contrast enhancement persisted in the disk or epidural space of three patients, and was thought to represent chronic granulomatous change or postsurgical scar. CEMR is very valuable for the initial diagnosis of an epidural abscess, particularly if it involves lengthy segments. During follow-up, CEMR can document responses to therapy, and provide information for determining appropriate treatment.
Abscess↗
Transcranial Doppler detection of vertebrobasilar vasospasm following subarachnoid hemorrhage.
BACKGROUND AND PURPOSE: Transcranial Doppler sonography is of established value in the detection and monitoring of middle cerebral artery vasospasm. Little information exists on the utility of transcranial Doppler for detection of posterior circulation vasospasm. METHODS: Cerebral angiography and conventional hand-held transcranial Doppler sonography were compared to determine sensitivity and specificity of transcranial Doppler for detection of vertebral and basilar artery vasospasm. RESULTS: Of 59 consecutive subarachnoid hemorrhage patients with transcranial Doppler angiogram correlations, 42 underwent posterior circulation angiography to evaluate 64 vertebral arteries and 42 basilar arteries during the period of risk for vasospasm and had technically adequate transcranial Doppler examinations within 24 hours of the angiogram. A mean flow velocity of 60 cm/s and above was indicative of both vertebral and basilar artery vasospasm. For the vertebral artery, there were 7 true-positive test results, 42 true-negatives, 6 false-positives (unknown cause in 3, increased collateral flow in 1, adjacent vessel vasospasm in 1, hyperperfusion in 1), and 9 false-negatives (anatomic in 7, operator error in 2). Sensitivity was 44% and specificity was 87.5%. For the basilar artery, there were 10 true-positives, 23 true-negatives, 6 false-positives (unknown cause in 4, hyperemia/hyperperfusion in 1, increased collateral flow in 1), and 3 false-negatives (operator error in 2, tortuous vessel course in 1). Sensitivity was 76.9% and specificity was 79.3%. When the diagnostic criterion was changed to > or = 80 cm/s (vertebral artery) and > or = 95 cm/s (basilar artery), all false-positive results were eliminated (specificity and positive predictive value, 100%). CONCLUSIONS: Our data suggest that transcranial Doppler has good specificity for the detection of vertebral artery vasospasm and good sensitivity and specificity for the detection of basilar artery vasospasm. Transcranial Doppler is highly specific (100%) for vertebral and basilar artery vasospasm when flow velocities are > or = 80 and > or = 95 cm/s, respectively.
Adult↗
Melanoma arising in a cervical spinal nerve root: report of a case with a benign course and malignant features.
A 20-year-old caucasian woman with a 5-year history of right arm, neck, and back pain sought treatment when an automobile accident (4 months before admission) exacerbated her pain. Magnetic resonance imaging revealed an intra- and extradural mass compressing the spinal cord at the C5-C6 level. It also extended into and widened the neural foramen, mimicking a neurofibroma. A single cafe-au-lait spot was discovered in the inguinal region. A two-staged surgical resection was performed on an apparent hemorrhagic C6 nerve root mass. The mass exhibited diagnostic features of a malignant melanoma histologically, immunocytochemically, and ultrastructurally. A search for a primary lesion outside the nervous system or other metastases during an 8-year period from the onset of symptoms has been negative. The patient's chronic history, evidence of neural foraminal enlargement, and the absence of other malignant melanoma lesions or subsequent metastases indicates that this lesion may be a primary melanoma of the nerve root with a benign course.
Adult↗
Varix of the eyelid: a unique CT finding.
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Aged↗