Intravenous gammaglobulin therapy in recurrent acute disseminated encephalomyelitis.
Explore the source record for details and available documents.
Adrenal Cortex Hormones↗
Biomedical subjects
D Enzmann
Publications and source records attributed to D Enzmann.
Brainstem multiple sclerosis in an 11-year-old child presenting as acute disseminated encephalomyelitis.
Multiple sclerosis and acute disseminated encephalomyelitis are demyelinating disorders of the central nervous system that can present initially as an acute focal demyelinating syndrome. We report an 11-year-old girl who initially presented with intractable vomiting and hypertension and later developed a subacute onset of focal neurologic (brainstem) signs. Magnetic resonance imaging (MRI) demonstrated a large solitary demyelinating lesion of the brain stem consistent with acute disseminated encephalomyelitis. Because of the morbidity associated with biopsy and its questionable value in the course of management of this patient, she was treated empirically with aggressive supportive measures and high-dose corticosteriod therapy. She had near full recovery, with only minimal neurologic sequelae. Six months later, she presented with similar focal neurologic signs, and a new lesion was found on MRI. Because of the separation of her two episodes in time and central nervous system location, a diagnosis of multiple sclerosis was made. Herein, we used this patient to illustrate the difficulty in distinguishing acute disseminated encephalomyelitis from multiple sclerosis in patients who present initially with an acute focal demyelinating syndrome.
Brain Stem↗
Repeated treatment with chimeric anti-CD4 antibody in multiple sclerosis.
We treated 21 multiple sclerosis patients with two to four doses of cM-T412, a chimeric monoclonal antibody against the CD4 antigen found on helper/inducer T lymphocytes. The mean number (+/- standard error) of circulating CD4 lymphocytes decreased from 888 (+/- 81) cells/mm3 at baseline to 246 (+/- 18) after treatment. At 1 year after the last treatment, the CD4 count had recovered to only 335 (+/- 32). The antibody had no effect on CD8 lymphocytes, B lymphocytes, or other leukocytes. Side effects were minimal. Despite the prolonged depletion of CD4 lymphocytes, no opportunistic infections occurred. Only 1 patient had a possible allergic reaction. Most patients were clinically stable, but a few progressed. We conclude that repeated treatment with cM-T412 is effective in reducing the number of circulating CD4 lymphocytes and has no limiting side effects.
Adult↗
Phase 1 clinical trial of chimeric monoclonal anti-CD4 antibody in multiple sclerosis.
We conducted an open trial of cM-T412, a chimeric monoclonal anti-CD4 antibody, in 29 patients with MS. This antibody caused a prompt and long-lasting depletion of circulating CD4 (helper/inducer) lymphocytes. The mean (+/- SE) CD4 count for the group decreased from 870 (+/- 66) cells/mm3 at baseline to 76 (+/- 11) 3 hours after treatment, and then increased to 425 (+/- 38) at 1 month after treatment and 475 (+/- 39) at 6 months after treatment. Numbers of CD8 (cytotoxic/suppressor) lymphocytes, B lymphocytes, granulocytes, and monocytes changed transiently but showed no significant long-term effects. The most common side effects were headache, nausea, myalgia, fever, and tachycardia occurring in the first few hours after treatment. No serious or unexpected infections or other significant adverse effects occurred. Kurtzke EDSS scores remained stable, and MRI scans showed less contrast enhancement 1 week after treatment. We conclude that treatment of MS patients with cM-T412 chimeric anti-CD4 antibody is well tolerated at the doses tested and produces a long-lasting, selective depletion of CD4 lymphocytes.
Adult↗
Consensus: diagnosis and management of neurocysticercosis in children.
Explore the source record for details and available documents.
Adult↗
1H spectroscopic imaging using a spectral-spatial excitation pulse.
Excellent water suppression is required to perform in vivo 1H spectroscopic experiments. However water suppression is difficult due to both B0 and RF inhomogeneities. These inhomogeneities are particularly troublesome in spectroscopic imaging experiments where water suppression is required throughout some large region of interest. In this paper, we propose the use of spectral-spatial excitation pulses for such experiments. These two-dimensional pulses are shown to provide water suppression that is insensitive to a range of B0 and RF variations while simultaneously providing spatial localization. Experimental results including images (with voxel volumes ranging from 3.4 to 1.5 cc) of various brain metabolites from both a normal volunteer and a patient with a metastatic lung carcinoma are presented.
Brain↗
Spectroscopic imaging with multidimensional pulses for excitation: SIMPLE.
Proton spectroscopy and spectroscopic imaging in the human brain require the elimination of both water and lipid signals. Strong lipid signals from subcutaneous fat are usually eliminated by confining the excited volume to lie wholly within the skull. Water suppression, however, can be difficult due to both B0 and RF inhomogeneities, which are particularly troublesome in imaging experiments where a relatively large region-of-interest (ROI) is typical. In this paper, we discuss the use of multidimensional selective-excitation pulses (e.g., pulses that are simultaneously selective along two axes) to both define the ROI and provide the necessary water suppression. Pulse sequences providing three-dimensional localization along with water suppression that is insensitive to a range of B0 and RF inhomogeneities are described. Spectra and spectroscopic images (voxel volume = 3.4 cc. acquisition time = 38 min) of various 1H metabolites from a patient with an astrocytoma show clear differences between normal and cancerous tissues and demonstrate the ability of these techniques to be used in vivo.
Astrocytoma↗
Magnetic resonance imaging in MELAS syndrome.
MELAS syndrome is a distinct clinical entity belonging to a group of mitochondrial encephalomyopathies characterized by the tetrad of myopathy, encephalopathy, lactic acidosis, and stroke-like episodes. Computed tomography (CT) and magnetic resonance (MR) findings are reviewed in a patient with MELAS. Serial CT studies demonstrated multiple "migrating" infarcts in various stages of evolution involving primarily the posterior temporal and occipital regions. MR was more sensitive than CT in demonstrating the number and extent of cortical lesions in this disease entity.
Acidosis, Lactic↗
Short TR, variable flip angle, gradient echo scans of the cervical spine: comparison of 2DFT and 3DFT techniques.
A prospective study of 16 patients was performed to compare quantitatively a contiguous single slice 2DFT version with a 3DFT version of a short TR, variable flip angle, gradient echo (GRASS) pulse sequence. The 3DFT GRASS scans had higher signal-to-noise ratios (SNR) of cord and CSF compared to the single slice 2DFT GRASS scans. The 3DFT GRASS scans, however, had lower CSF-cord and CSF-disc contrast than the single slice 2DFT version. The 3DFT GRASS sequence demonstrated comparable contrast only on the end slices of an imaging volume suggesting influence of an entry phenomenon. The lower CSF-cord and CSF-disc contrast of the 3DFT GRASS technique diminished its usefulness in the diagnosis of cervical disc disease compared to the single slice 2DFT GRASS technique. Two different slice thicknesses (3 mm and 5 mm) were investigated with the 2DFT GRASS technique and found to be comparable although the 3 mm scans had sharper disc and dural margins because of less partial volume artifact.
Cervical Vertebrae↗
Improved MR images of the brain with use of a gated, flow-compensated, variable-bandwidth pulse sequence.
Peripheral gating and first-order flow compensation were compared for their ability to improve the quality of second-echo (echo time = 80 msec) brain images obtained with a T2-weighted spin-echo sequence. The contrast-to-noise ratios (C/Ns) for interfaces between brain and cerebrospinal fluid, gray and white matter, and lesion and white matter were measured; the C/N was highest for the combination of gating and flow compensation. This combination of motion compensation also reduced motion artifacts more than did either technique alone. Further improvement in C/N was sought by using a variable-bandwidth pulse sequence, which was compared to a conventional spin-echo sequence. The variable-bandwidth technique increased the C/N of the second-echo image by 27%. The combined use of gating, flow compensation, and the variable-bandwidth option produced high-resolution brain images with a single excitation and retained flexibility in number of sections, number of echoes, and echo times.
Brain↗
Magnetic resonance appearance of slow flow vascular malformations of the brainstem.
Twelve patients with slow flow brain stem vascular malformations had magnetic resonance scans performed on a 1.5 Tesla scanner using T1 (TR = 400-800 ms, TE = 25 ms) and T2 (TR = 2000 ms, TE = 20, 25, 80 ms) weighted scans. Eight patients (70%) had solitary brainstem vascular malformations while 4 (30%) had multiple lesions both supra and infratentorially. Five patients had venous angiomas demonstrated by angiography and MR. There were 26 lesions seen on MR of which only 14 were identified by CT. These lesions demonstrated a specific MR image pattern indicative of subacute or chronic parenchymal hematoma. It was characterized by an iso to hyperintense central signal surrounded by a well defined thin band of low signal intensity. The central zone could appear to be single or multilocular. In multilocular lesions the hemorrhages within different cells could be of different ages. One patient did not have findings of a chronic hematoma but exhibited only low signal secondary to calcification and a venous angioma. None of these lesions had surrounding edema. Despite the variable etiologies of slow flow vascular malformations of the brainstem, their MRI manifestations seem to indicate a final common pathway of chronic hematoma. These lesions are quite distinct from MS or tumor, the usual clinical considerations in the differential diagnosis.
Adolescent↗
Positive rolandic sharp waves in the EEG of the premature infant.
Ninety-seven EEGs from 30 premature infants found to have multifocal white matter necrosis on ultrasound (US) or autopsy were reviewed retrospectively. Twenty infants had intraparenchymal echodensities on US that developed into cystic lesions, a finding consistent with periventricular leukomalacia; 8 had intraparenchymal hemorrhages; and 2 had white matter necrosis at autopsy. Four of these infants had no intraventricular hemorrhage. Positive sharp waves in the central (rolandic) regions (PRS) were identified in 22 of these 30 infants (73%) and in 0 of 30 age-matched controls (p less than 0.001). The presence of PRS on the EEG of the premature infant has a high correlation with white matter necrosis rather than with intraventricular hemorrhage. In all cases, this EEG pattern was present prior to the development of cavitations when echodensities were present on US.
Brain↗
The natural history of subependymal germinal matrix hemorrhage.
A prospective study of 377 premature infants (less than or equal to 1500 gm) was undertaken to delineate the natural history of subependymal/intraventricular hemorrhage (S/IVH) and its complications using ultrasound (US) and computed tomography (CT). Low grade (I, II) S/IVH had a low mortality while higher grades (III, IV) still had elevated mortality rates. The addition of intraparenchymal hemorrhage (IPH) to S/IVH incrementally increased the incidence of death and other complications, suggesting IPH hemorrhage should be categorized separately. When a specific day could be identified, S/IVH had its onset in the first 7 days of life with peak incidence occurring on day 3. S/IVH appeared to be an event limited to less than 24 hours in all but 5% of infants in whom progression of hemorrhage was documented over a 24-hour period. The mortality rate of these progressive hemorrhages was high, 50%. The benign phenomenon of late S/IVH was detected in 5% of infants. These hemorrhages were clinically silent and of minor severity. Several complications of S/IVH were detected. Hydrocephalus was a significant complication only for higher grades of S/IVH. When present, severe hydrocephalus had an early onset and reached a maximum at around 3 weeks of age. "Atrophic change" of a cerebral hemisphere was detected in 30% of all S/IVH infants, while this was not seen in nonS/IVH infants. This "atrophic" abnormality had a marked predilection for the left hemisphere, independent of the site of the S/IVH. Periventricular leukomalacia (PVL) was documented by US in 2% of infants and could be detected in the first week of life. PVL presented in the first week of life as an echogenic lesion which developed "cystic" changes at approximately 3-4 weeks of age. This complication should be categorized separately from S/IVH.
Atrophy↗
Hybrid intravenous digital subtraction angiography of the carotid bifurcation.
A hybrid digital subtraction angiography technique and noise-reduction algorithm were used to evaluate the carotid bifurcation. Temporal, hybrid, and reduced-noise hybrid images were obtained in right and left anterior oblique projections, and both single- and multiple-frame images were created with each method. The resulting images were graded on a scale of 1 to 5 by three experienced neuroradiologists. Temporal images were preferred over hybrid images (average score = 3.2 and 2.4, respectively). The percentage of nondiagnostic examinations, as agreed upon by two readers, was higher for temporal alone than temporal + hybrid (4 and 1, respectively). In addition, also by agreement between two readers, temporal + hybrid images significantly increased the number of bifurcations seen in two views (87%) compared to temporal subtraction alone (64%).
Aged↗
Specific CT findings in Krabbe disease.
Specific computed tomography (CT) findings in four patients with biochemically proven Krabbe disease included symmetric increased attenuation in the cerebellum, brainstem, thalami, caudate nuclei, and corona radiata before and in conjunction with decreased attenuation of white matter followed by atrophy at a later stage. Familiarity with the CT findings in the acute phase of Krabbe disease may assist clinicians in limiting the differential diagnosis and requesting appropriate laboratory tests.
Brain↗
Cryptic vascular malformations involving the brainstem.
Six patients with angiographically cryptic vascular malformations involving the brainstem were examined with computed tomography (CT). The clinical and CT findings of cryptic vascular malformations of the brainstem are described and distinguished from those of brainstem glioma and multiple sclerosis. Calcification within a brainstem lesion that displays relatively little mass effect and shows little contrast enhancement, particularly when associated with a long history of waxing and waning brainstem symptoms, should suggest a vascular malformation.
Adult↗
The changing angiographic appearance of an arteriovenous malformation after subarachnoid hemorrhage.
The changing angiographic appearance of a cerebral arteriovenous malformation (AVM) illustrated hemodynamic changes that can occur following subarachnoid hemorrhage and antifibrinolytic therapy. Decreased size of this lesion suggested thrombosis of the AVM. This appearance actually represented a transient, vasospastic phenomenon which reversed with time. Although the AVM underwent significant changes acutely, little changed in the long term.
Adult↗
Computed tomography of rabbit V2 carcinoma after intraarterial contrast enhancement.
Changes in X-ray attenuation of rabbit V2 carcinoma, as determined by computed tomography (CT) scanning, were assessed following the intraarterial and intravenous administration of a water soluble contrast agent. After intraarterial contrast medium administration, a significant increase in CT attenuation values occurred in both blood and V2 carcinoma, and the attenuation values remained above control values for at least 120 min. A similar, although less pronounced, increase in VA attenuation values occurred following intravenous contrast medium administration. Comparison of V2 CT enhancement values after intraarterial and intravenous administration suggested that tumor enhancement was greater after intraarterial administration.
Animals↗