Continuous quality improvement (CQI) in neuropathology.

Continuous quality improvement activities are mandated by the Joint Commission on Accreditation of Healthcare Organizations and the College of American Pathologists. In a recent Needs Assessment Survey of neuropathologists undertaken by the College of American Pathologists, in cooperation with the American Association of Neuropathologists, neuropathologists indicated the need for information regarding continuous quality improvement for neuropathology laboratories. This article suggests activities that may be incorporated in a continuous quality improvement program, including suggested monitors in surgical and autopsy neuropathology, as well as an example of a continuous quality improvement plan.

Alpha 1-antitrypsin in cerebrospinal fluid of patients with neurologic diseases.

Proteases and their inhibitors have been implicated in the pathogenesis of neuroimmunologic diseases, particularly multiple sclerosis (MS). We measured the immunochemical level and functional activity of alpha 1-antitrypsin (AAT) in cerebrospinal fluid (CSF) and serum in patients with MS and other neurologic diseases. Increases in the immunochemical level of AAT in CSF correlated directly with disturbances in the blood-brain barrier, as reflected by the ratio of albumin in CSF to that in serum. The AAT activity in CSF directly correlated with the immunochemical level when all patients were compared. However, the AAT activity in patients with inflammatory diseases tended to be decreased relative to the immunochemical level, suggesting inactivation of AAT in these disorders. The AAT activity was not significantly altered in patients with MS, despite reports of increased protease activity in active MS.

Cystic prolactinoma: a variant of "transitional cell tumor" of the pituitary.

Pituitary tumors with features of both Rathke's cleft cysts and adenohypophysial adenomas are extremely rare and have been termed "transitional cel tumors." The ultrastructural features of one of these tumors has been reported, demonstrating cells with features transitional between those of adenohypophysial cells and Rathke's cleft cells, along with other cells with the distinctive features of either cell type. We report a case of a prolactin-secreting "transitional cell tumor" with similar light-microscopic and ultrastructural features. Additionally, the transitional cells in our case exhibited abundant intracellular mucin production. This represents a variant of "transitional cell tumor" of the pituitary gland.

Agarose electrophoresis and immunonephelometric quantitation of cerebrospinal fluid immunoglobulins: criteria for application in the diagnosis of neurologic disease.

The cerebrospinal fluid (CSF) IgG index, the CSF to serum albumin ratio, and electrophoresis on agarose gel of CSF and serum were evaluated retrospectively for their usefulness in the differential diagnosis of multiple sclerosis (MS). Standardized procedures were adopted for grading the intensity of oligoclonal banding and for the certainty of diagnosis of MS. One hundred and forty-nine patients were studied, including 23 with definite multiple sclerosis (MS), 12 with probable MS, 20 with possible MS, 20 with inflammatory neurologic disease, 65 with noninflammatory neurologic disease, and nine with no neurologic disease. The CSF IgG index and the CSF to serum albumin ratio were calculated from nephelometric measurements of serum and CSF IgG and albumin. The intensity of oligoclonal banding was graded relative to the density of the prealbumin band. Eighty-eight per cent of cases of definite MS had distinct oligoclonal bands, and an equal number had an elevated IgG index. These tests were not specific for MS, however, since 50% of cases of inflammatory neurologic disease and 5% of those with noninflammatory neurologic disease had an elevated IgG index. Similarly, 48% of cases with inflammatory disease and 25% with noninflammatory disease had oligoclonal bands. However, only patients with definite MS (21%) or possible MS (4%) had prominent oligoclonal bands whose density was greater than or equal to that of prealbumin, together with a CSF IgG index greater than 1.50. This combination of findings therefore may enhance the level of suspicion of MS. By contrast, an isolated increase in the CSF to serum albumin ratio may suggest a diagnosis other than MS.

Suture granuloma simulating bladder neoplasm.

A large suture granuloma rarely may occur long after surgery using nonabsorbable suture. We report a case in which such a lesion simulated a bladder wall neoplasm.

Interactions between nucleus centrum medianum and gigantocellular nociceptive neurons.

Cells in nucleus reticularis gigantocellularis (NGC) and nucleus centrum medianum (CM) are known to respond to tooth pulp stimulation, which is a nociceptive trigeminal stimulus. We examined the effect of stimulation of CM on this class of neurons in NGC. Thirty-nine percent of the 57 neurons tested were antidromically activated by stimulation of the ipsilateral CM, while 28% of the 40 neurons tested were antidromically activated from the contralateral CM. In addition, the spontaneous activity of seven NGC neurons was altered by stimulation of the ipsilateral CM, while five cells were affected by stimulation of the contralateral CM. These data suggest a complex and reciprocal interaction between neurons in NGC and CM.

Response of cells in feline nucleus centrum medianum to tooth pulp stimulation.

Nucleus centrum medianum (CM) has been implicated in the processing of nociceptive information based on behavioral, physiological and clinical data. Although the responses of neurons in CM have been extensively studied using spinal stimuli, few data are available concerning their responses to trigeminal stimuli. This investigation was designed to determine the responses of cells in CM to tooth pulp stimulation, which has been demonstrated to be an effective and relatively specific trigeminal nociceptive stimulus. Responses were recorded from 378 neurons in 38 pentobarbital-anesthetized cats. Most cells responded at a latency of 5 to 40 msec with one to six spikes for each stimulus. Cells in the dorsal portion of CM tended to respond only to maxillary teeth, while cells in the ventral portion of the nucleus tended to respond only to mandibular teeth. Cells in the central portion of CM generally responded to both maxillary and mandibular teeth. Cells in CM generally responded to both ipsilateral and contralateral teeth, without a preference for one side. These data differ from those reported using spinal stimulation, since a topographic organization has not been identified with spinal stimulation.

Neuronal receptive field properties in feline nucleus reticularis gigantocellularis.

Nucleus reticularis gigantocellularis (NGC) has been shown, using both behavioral and physiological techniques, to be involved in the processing of nociceptive information. However, previous studies of the receptive fields of NGC neurons have utilized only innocuous stimuli. Thus, while neurons in NGC may play an important role in nociception, the receptive field properties of these cells remain to be defined. This investigation was designed to determine the receptive field properties of neurons in NGC using nociceptive and innocuous stimuli. Receptive fields were determined for 127 neurons in NGC. Eighty-seven percnet of the NGC neurons studied responded exclusively to noxious stimuli, while 13% also responded to innocuous stimuli. None of the neurons studied responded exclusively to innocuous stimuli. The receptive fields of most NGC neurons (63%) were large, discontinuous, and bilaterally symmetrical. Eighty-one percent of NGC neurons received convergent inputs from both spinal and trigeminal systems. These receptive field properties differ from those previously reported using only innocuous stimulation.

Hemangioendothelioma of the neuraxis: an ultrastructural study.

Hemangioendotheliomas are vascular tumors that rarely involve the neuraxis. However, when they do, they must be differentiated from the other neoplastic vascular lesions that more commonly involve the central nervous system (CNS). We describe the ultrastructural features of three hemangioendotheliomas of the neuraxis. These lesions are characterized by the proliferation of endothelial cells around vascular lumina and of pericytes outside of the endothelial basal lamina. These features differ from those of other vascular tumors of the CNS. Based on these observations, we consider hemangioendothelioma to be a distinct entity among the neoplasms of vascular origin in the CNS.

Glioblastoma multiforme occurring 13 years after treatment of a medulloblastoma.

Although medulloblastoma is said to be capable of astrocytic differentiation, the development of an astrocytic neoplasm in association with a medulloblastoma is extremely rare. We report the light and electron microscopic features of a supratentorial glioblastoma that occurred 13 years after radiation therapy for a cerebellar medulloblastoma. The diverse ultrastructural features of the glioblastoma may reflect the multipotentiality of the cells in medulloblastoma. An alternate interpretation is that the glioblastoma represents a radiation-induced neoplasm after therapy for a medulloblastoma.

Supratentorial subarachnoid epithelial cyst: case report and ultrastructural study.

Supratentorial epithelial cysts are rare entities that have evoked controversy regarding their pathogenesis. Recent ultrastructural studies have failed to resolve this controversy. We present a case report and an ultrastructural study of such a cyst. The cyst wall was lined by columnar epithelial cells with prominent cilia and microvilli at their apical surface. They were joined by well-developed junctional complexes and contained abundant intracytoplasmic, electron-dense, granular material, some of which was membrane-bound. These ultrastructural findings suggest that epithelial cysts are derived from elements of the embryological tela choroidea.

Intraventricular primary cerebral neuroblastoma in adults: report of three cases.

Primary cerebral neuroblastoma most often occurs in the cerebral parenchyma in children. When it occurs in adults, it is also described as an intraparenchymal neoplasm. We report three cases of primary cerebral neuroblastoma in adults presenting as an intraventricular mass.

Granular cell tumor of the pituitary gland associated with diabetes insipidus.

A hypophysectomy was performed for palliation of metastatic carcinoma of the breast in a 62-year-old woman who also had diabetes insipidus. At operation a neurohypophyseal mass was discovered; it proved to be a granular cell tumor. The diabetes insipidus resolved postoperatively.

Carcinoid tumor in a presacral teratoma associated with an anterior sacral meningocele: case report and review of the literature.

We report a case of a presacral teratoma containing a malignant carcinoid component associated with an anterior sacral meningocele that presented in a 35-year-old woman. The clinical, radiographic, and pathological features of these rare tumors, of presacral meningoceles, and of the hereditary presacral teratoma syndrome are discussed.

Primary lymphoma of the central nervous system diagnosed by computed tomographic scan-directed needle biopsy with a frozen section immunoperoxidase technique.

Primary lymphoma of the central nervous system is an uncommon neoplasm that requires definitive diagnosis because it is potentially treatable. We report a case in which the diagnosis of lymphoma was unsuspected and was made by needle biopsy with computed tomographic scan direction. A frozen section immunoperoxidase technique demonstrated monoclonality. When combined with a compatible morphological appearance, this confirmed the diagnosis of lymphoma. This case demonstrates the importance of this immunochemical method in the rapid diagnosis of central nervous system lymphomas.

Benign hemangioendothelioma involving the central nervous system: "strawberry nevus" of the neuraxis.

Benign hemangioendothelioma is rarely included in discussions of vascular neoplasms involving the central nervous system, whereas it is a well-defined entity outside the neuraxis. We present four cases of benign hemangioendothelioma, including an infantile hemangioendothelioma with venous drainage into the transverse dural sinus, an intracranial hemangioendothelioma adherent to the falx cerebri, a spinal epidural hemangioendothelioma causing spinal cord compression, and a parasellar hemangioendothelioma. Although hemangioendothelioma resembles hemangioblastoma in some ways, it is a distinct entity differing from the other vascular lesions involving the central nervous system.

Intraventricular fiberscopic observations in adult hydrocephalic patients.

A flexible ventriculofiberscope was used to diagnose intraventricular pathological conditions in eight adult hydrocephalic patients. In each case the entire procedure was recorded on videotape for documentation, review, and teaching. Four representative cases are presented to illustrate the use and value of this technique.

Traumatic neuropathology.

The neuropathology of trauma is reviewed based on the mechanism of injury. Pathology is divided into primary and secondary injury, based on the relationship to the time of injury. It is further divided by mechanism, with primary impact injury including skull fracture, epidural hematoma, brain contusion and laceration, and intracerebral hemorrhage; primary inertial injury including subdural hematoma, diffuse axonal injury, and diffuse vascular injury; and secondary injury including hypoxia/ischemia, brain swelling, infection, and increased intracranial pressure. The neuropathology of child abuse is also reviewed.

Muscle biopsy.

OBJECTIVE: To provide practical guidelines for handling muscle biopsies to be submitted for outside processing and consultation. DESIGN: Review each step of handling a muscle biopsy and provide alternative means of tissue processing when feasible. RESULTS: Practical guidelines are presented for specimen selection, performance of needle biopsy, preparation of tissue for electron microscopy, preparation of tissue for shipping, clinical information to be provided, and submission of tissue for molecular and biochemical studies. CONCLUSIONS: Muscle biopsy samples to be sent to referral centers can be properly handled to avoid the problems noted by the Neuropathology Needs Assessment of the College of American Pathologists.

Proximal myotonic myopathy: clinical, neuropathologic, and molecular genetic features.

The primary genetic abnormality in myotonic dystrophy (DM) is an expansion of the CTG trinucleotide repeat on chromosome 19q. Recently, patients with similar clinical features, but without this genetic alteration, have been designated as proximal myotonic myopathy (PROMM). We describe two additional cases of PROMM, both of whom presented with clinical features suggestive of myotonic dystrophy. The patients had electromyographic (EMG) evidence of myotonia, normal cardiac evaluation, and no cataracts. Genetic analysis of peripheral blood leukocytes revealed no expansion of the trinucleotide repeat by polymerase chain reaction (PCR) and Southern blot analysis. Muscle biopsies in both cases were significant with features suggestive of myotonic dystrophy, such as large numbers of fibers containing multiple internal nuclei, occasional nuclear chains, and fiber atrophy, although sarcoplasmic masses and ring fibers were absent. These cases illustrate the clinical and neuropathologic findings of PROMM and underline the importance of correlating these aspects with genetic studies in patients with myotonic muscle disorders.