The role of biopsy in vasculitis of the central nervous system.
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Biomedical subjects
Publications and source records attributed to J E Parisi.
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PURPOSE: To test the accuracy of fast spin-echo (FSE) imaging versus that of double-echo conventional spin-echo (CSE) imaging in identification of the increased hippocampal signal intensity associated with mesial temporal sclerosis (MTS). MATERIALS AND METHODS: Thirty-four subjects who subsequently underwent anterior temporal lobectomy for intractable seizures and in whom the presence or absence of MTS was ascertained with certainty were imaged with CSE and FSE. Three blinded reviewers evaluated the first and second CSE images (CSE1 and CSE2) and the FSE images. RESULTS: CSE1 imaging had lower accuracy than FSE (P = .038) and CSE2 (P = .006) imaging. CSE2 imaging was slightly more accurate than FSE imaging (P = .048). Contrast-to-noise ratios were lower for CSE1 imaging than for CSE2 or FSE imaging (P < .001). CONCLUSION: The FSE sequence evaluated was more time efficient than CSE imaging but slightly less accurate. Therefore, substitution of this sequence for a CSE sequence seems unwarranted.
Biologically malignant pilocytic astrocytomas are rare. Precise morphologic criteria permitting their identification and clinically meaningful distinction from more ordinary pilocytic astrocytomas have not been developed. The histologic grading schemes applied to fibrillary astrocytomas--ones based on nuclear atypia, increased cellularity, mitotic activity, endothelial proliferation, and necrosis--are thought not to correlate with prognosis in pilocytic astrocytomas. Nonetheless, these features were systematically sought in 107 cerebellar pilocytic astrocytomas, four of which were histologically malignant. The clinical, morphologic, and flow cytometric features of these cases were studied. The incidence of histologic malignancy occurring spontaneously in otherwise typical pilocytic astrocytomas was 0.9%; that occurring after radiation was 1.8%. The four histologically malignant tumors occurred in two males and two females, ages 6 to 18 years. All showed increased mitotic activity (three to five mitoses per high-power microscopic field [250x]). Endothelial proliferation and necrosis were present in three and two cases each. DNA ploidy analysis showed the spontaneously occurring malignant-appearing tumors to be aneuploid, whereas both previously radiated tumors were tetraploid; 5% to 11% of cells were in S phase. The appearance of histologic malignancy in pilocytic astrocytoma is rare and less reliably correlated with prognosis than in patients with fibrillary astrocytomas. Essential features of such tumors include a high mitotic index and perhaps a high percentage of cells in S phase.
We performed a retrospective study of 53 consecutive "nonlesional" temporal lobectomy patients to assess the relative utility of MRI versus interictal single-photon emission computed tomography (SPECT) in this patient population. We compared the seizure lateralizing properties of MRI and SPECT using multiple blinded expert reviewers for both SPECT and MRI with a test-retest reviewer paradigm and measurements of hippocampal volume from MRI. The criterion standard for seizure lateralization was satisfactory postoperative seizure control (n = 43). The rate of correct seizure lateralization was significantly greater for MRI than for SPECT (p < or = 0.01), and the rate of incorrect lateralization was significantly less for MRI than for SPECT. The most accurate MRI measure was hippocampal volume measurements, which correctly lateralized the seizures in 86.0% of cases. The correct lateralization rate for SPECT was 45.4%. The MRI and SPECT studies tended to be noncomplementary with respect to seizure lateralization, and SPECT was likely to give an incorrect or indeterminate result in patients who were not lateralized by MRI. Concordant MRI-EEG lateralization was a strong predictor of satisfactory postoperative seizure control, while no relationship between postoperative seizure control and SPECT findings was present.
With reference to vascular malformations, the term cavernous has architectural as well as histologic connotations. It refers to a compact pattern of growth wherein no intervening brain parenchyma is evident, as well as to the histological nature of the vessels, which are hyaline and collagenous in appearance, lacking the microscopic features of arteries or veins. Historically, cavernous angioma has been defined as exhibiting both features. Twenty-five patients with neurological symptoms and neuroimaging abnormalities who underwent surgery for cerebral vascular malformations between 1987 and 1990 satisfied the following study criteria: their lesions were angiographically occult and both magnetic resonance imaging (MRI) and histological sections were available for review. The patients' ages ranged from 4 to 49 years (mean, 30 years), the male to female ratio being 1:2. Two thirds of the lesions were supratentorial in location and all were intraparenchymal. All patients had clinical improvement after resection. In 24 of the 25 lesions, the vascular channels were histologically cavernous in nature; one inadequate specimen precluded classification. Three demonstrated a purely compact or cavernous pattern, 20 a mixed cavernous and racemose pattern, and one a purely racemose pattern. The authors conclude that 1) histologically cavernous lesions are the commonest form of occult vascular malformation; 2) a purely compact or cavernous architectural pattern is uncommon, most lesions showing a partially racemose architecture; 3) some histologically cavernous malformations possess a capillary component; 4) clinical growth of cavernous malformations may have its basis in intraluminal thrombosis and subsequent recanalization; 5) the T2-weighted MRI pattern of cavernous malformations varies, the most common being a multifocal hyperintense center surrounded by a hypointense ring; 6) the MRI pattern reflects the histological appearance; 7) since no thrombosed arteriovenous malformations were encountered, such lesions must be rare; 8) in that the pathophysiological hallmark of a cavernous lesion is recurrent thrombosis and hemorrhage, a resolving hematoma cannot always be distinguished from a cavernous lesion; 9) MRI is the examination of choice in evaluating occult vascular malformations; and 10) microsurgical excision is a satisfactory method of treatment.(ABSTRACT TRUNCATED AT 250 WORDS)
Proliferating cell nuclear antigen, an auxiliary protein of deoxyribonucleic acid polymerase-delta, has been shown to be a reliable marker of nuclear deoxyribonucleic acid synthetic activity. We applied a monoclonal antibody to proliferating cell nuclear antigen to a series of serial stereotactic biopsies from patients with glioblastoma multiforme and found the proliferative activity to vary relative to biopsy location within or surrounding the solid tissue component of the tumor. Twenty-seven trajectories in 26 patients were analyzed, each consisting of two to five sequential 10 x 1.5 mm core biopsies (mean = 3). The proliferative index (PI) was greatest in those cells located at the solid tumor-infiltrated parenchyma interface. PI values were significantly lower in those biopsy cores located proximal (within infiltrated parenchyma) and distal (within solid tumor tissue) to the solid tumor-infiltrated parenchyma interface (median PI values, proximal to distal: 0.38, 0.66, 5.45 solid tumor-infiltrated parenchyma interface], 0.39, 0.09%). The mean PI values were significantly lower in neoplastic cells samples from regions of peripheral hypodensity on computed tomographic scans compared with those sampled from contrast-enhancing regions (0.9 and 3.91%, respectively). There was no significant difference in the mean PI values of neoplastic cells sampled from regions of contrast enhancement or central hypodensity (3.91 and 4.31%, respectively).
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.
Cranial CT features of two patients with intracerebral hemorrhages in the setting of sepsis and disseminated intravascular coagulation are reported. Multiple predominantly subcortical hemorrhages were seen. This pattern of cerebral hemorrhage should raise suspicion of disseminated intravascular coagulation as an underlying cause.
Normative bases for the Wechsler Adult Intelligence Scale-Revised (WAIS-R) and the Wechsler Memory Scale-Revised (WMS-R) recently were extended through age 97 with the publication of norms that permit computation of MAYO IQs for the WAIS-R and MAYO Memory Indices for the WMS-R. This study compares traditional Wechsler summary scores with their corresponding MAYO values in a clinical sample. MAYO summary scores are correlated highly with traditional Wechsler values, and concordance rates are also strong. Despite their similarities MAYO summary scores and traditional Wechsler values are not interchangeable. Cautions are discussed relative to the use of both MAYO and traditional Wechsler IQs and Memory Indices.
Neurological involvement in Wegener's granulomatosis was studied by reviewing the charts of 324 consecutive patients in whom the diagnosis was made at the Mayo Clinic. One hundred nine patients (33.6%) had neurological involvement. Peripheral neuropathy occurred in 53; cranial neuropathy, in 21; external ophthalmoplegia, in 16; cerebrovascular events, in 13; seizures, in 10; cerebritis, in 5; and miscellaneous involvement, in 25. The mean age and sex ratio were similar in the patients with and those without neurological involvement. Among the patients with peripheral neuropathy, 42 had mononeuropathy multiplex; 6, distal symmetrical polyneuropathy; and 5, unclassified peripheral neuropathy. Multiple mononeuropathy was a major presenting symptom in 8 patients. A significantly higher percentage of patients with peripheral neuropathy, compared to those without peripheral neuropathy, had kidney involvement (p < 0.001). The second, sixth, and seventh cranial nerves were most frequently affected. Multiple cranial nerves were affected in 8 patients. Unusual neurological manifestations in the miscellaneous group were spastic paraparesis, temporal arteritis, Horner's syndrome, and papilledema.
Articulatory and language impairment heralded rapidly progressive motor neuron disease in 7 patients aged 54 to 77 years. One patient had a family history of a similar disorder. Severe nonfluent aphasia developed in all 7 patients and 4 were anarthric within a year. Other cognitive domains were impaired, yet 2 patients lived alone until 1 month before their deaths. Four died within 2 years. Abnormalities were found on electromyography, computed tomography, magnetic resonance imaging, single-photon emission computed tomography, and electroencephalography. Neuropathological examination in 3 patients showed bilateral hemispheric atrophy with neuronal loss and gliosis predominantly of superficial cortical layers. Pigmented and hypoglossal nuclei were relatively preserved. At all spinal levels there was degeneration of corticospinal tracts and loss of anterior horn cells with gliosis. Rapidly progressive aphasic dementia and motor neuron disease are a distinctive clinical entity whose nosology is poorly understood.
We performed a prospective study using preoperative magnetic resonance imaging to identify hippocampal formation atrophy in 15 consecutive patients with intractable partial epilepsy who had undergone a stereotactic resection of an extrahippocampal temporal lobe foreign-tissue lesion. A stereotactic lesionectomy was performed in all patients, i.e., only the imaging-defined lesion itself was resected. Hippocampal formation atrophy was identified in three of the 15 patients. Neuroimaging-detected hippocampal formation atrophy has been shown to be a reliable marker of moderate to severe mesial temporal sclerosis. All patients with hippocampal formation atrophy had an unfavorable operative outcome. Pathological examination of the hippocampus in one patient with neuroimaging-identified hippocampal formation atrophy who subsequently received an anterior temporal lobectomy revealed mesial temporal sclerosis. Nine of the 12 patients without hippocampal formation atrophy experienced a significant reduction in seizure tendency after lesionectomy. The surgically excised hippocampus in one patient without hippocampal formation atrophy who later underwent a temporal lobectomy showed no significant neuronal loss. Results of this study have modified the surgical approach taken at this institution in patients with temporal lobe lesional epilepsy. Patients with magnetic resonance imaging-defined dual pathology now undergo a temporal lobectomy which includes resection of the hippocampus and the foreign-tissue lesion.
Between January 1973 and October 1991 at the Mayo Clinic, 5 of 345 patients with Wegener's granulomatosis initially had features suggestive of giant cell arteritis and subsequently were found to have biopsy-proven vasculitis of the temporal artery. All five patients were older than 60 years of age and had jaw claudication, sudden loss of vision, severe headache with or without diplopia, or polymyalgia rheumatica at the time of initial examination. The erythrocyte sedimentation rate was high at the time of onset of symptoms in four patients (and unavailable in one patient). A temporal artery biopsy specimen revealed giant cell arteritis in one patient and non-giant cell arteritis in four patients. All five patients subsequently had pulmonary and renal lesions characteristic of Wegener's granulomatosis, with typical histopathologic features on biopsy or positive cytoplasmic staining antineutrophil cytoplasmic antibodies. Thus, overlapping features of giant cell arteritis and Wegener's granulomatosis do occur in some patients.
BACKGROUND: Atrial myxoma is uncommon and may be associated with brief isolated ischemic events. We describe a patient with atrial myxoma and an incompletely evaluated ischemic stroke followed 1 year later with almost complete brain necrosis. SUMMARY OF REPORT: A 63-year-old woman presented with a rapidly progressive illness resulting in coma within 24 hours from multiple myxomatous emboli. Cranial computed tomographic scan demonstrated multiple hypodensities in both cerebellar and cerebral hemispheres. Postmortem examination revealed virtually complete brain necrosis, multiple peripheral artery embolizations, embolization in the coronary arteries with acute myocardial infarction, and tumor emboli in the spleen and kidneys. CONCLUSIONS: Although uncommon, atrial myxoma may present as a fatal multiple organ embolization that includes both anterior and posterior circulation territories of the brain.
Four patients with Wegener's granulomatosis (WG) presented with unique neurologic manifestations. On initial presentation, these patients had multiple mononeuropathy, multiple cranial neuropathy, myelopathy, and cerebritis. This series demonstrates that WG can present with a spectrum of neurologic manifestations.
We describe a family in which cutaneous malignant melanoma or cerebral astrocytoma, or both, developed in eight members over three generations. Other malignancies also occurred with a lesser frequency. In two patients with both malignant melanoma and astrocytoma, the brain tumor followed the diagnosis of melanoma by a period of 2 and 10 years and was the primary cause of morbidity and mortality. The findings in this family may represent a newly described genetic disorder.
The clinical, radiological, and pathological features of nine cases of intracranial parenchymal schwannoma are described. The clinical course in four patients 23 years of age or younger mirrored the indolent nature of this neoplasm. Imaging studies included computerized tomography in eight patients and magnetic resonance imaging in three. The lesions were well demarcated with only mild surrounding edema. Five tumors were deep within the temporoparieto-occipital region, three were in the cerebellum, and one lay peripherally in the parietal lobe. Over two-thirds of the nine tumors were either cystic (five) or contained areas of cystic degeneration (two). One lesion was frankly hemorrhagic. A variety of imaging characteristics and contrast enhancement patterns were observed, including those of a cyst with a mural nodule and peripheral enhancement. Of the four solid neoplasms, two enhanced homogeneously while the other two demonstrated heterogeneous enhancement. Six tumors were resected totally. The follow-up period ranging from 2 months to 2 years has shown no recurrences. Microscopically, immunohistochemically, and ultrastructurally, the tumors were indistinguishable from peripheral schwannomas. A possible mechanism underlying the histogenesis of these rare lesions is discussed. The importance of recognizing this tumor is stressed, particularly in younger patients, given its benign nature, radiological resemblance to other tumors such as pilocytic astrocytoma, and favorable response to resection.
Acoustic neuromas account for 75 to 80 percent of cerebellopontine angle tumors and 8 to 10 percent of all intracranial neoplasms. These tumors arise from the Schwann cell sheath that surrounds the peripheral portion of the facial, cochlear, superior, and inferior vestibular nerves. Theoretically, a schwannoma may arise from Schwann cells anywhere along these nerves from the glial-Schwann cell junction to the end organ. Clinically, however, they most frequently originate from the vestibular nerves, occasionally from the facial, and only rarely from the cochlear nerve. This investigation utilized light microscopy, immunochemistry, and digital optical morphologic analysis to determine the distribution of Schwann cells along the facial and vestibulocochlear nerves in an attempt to explain the observed distribution of tumors, and to evaluate currently accepted theories. No direct correlation between the distribution of Schwann cells and the observed distribution of schwannomas was found. Thus, this study did not confirm the currently held theories regarding schwannoma distribution in these nerves.