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Biomedical subjects

J C Kaufmann

Publications and source records attributed to J C Kaufmann.

At least 37 records · Page 2Linked to original sources

Oligoclonal bands in multiple sclerosis: clinical-pathologic correlation.

Although oligoclonal banding is a characteristic feature of MS spinal fluid, some patients do not show this abnormality. Four of 18 consecutive patients with autopsy-proven MS had no oligoclonal bands (OB) in CSF during life or at postmortem. Patients with OB had numerous plasma cells in meninges and plaques (confirmed in sections stained for cytoplasmic immunoglobulin). The four patients without bands had few or no identifiable plasma cells. Therefore, lack of OB correlates with both inactivity of plaques and absence of plasma cells in plaques or meninges. This is another form of heterogeneity in MS.

Adult↗

Staining procedure to aid in assessment of bucrylate histotoxicity in tissue sections.

Isobutyl-2-cyanoacrylate (bucrylate) is commonly used as a material for therapeutic embolization. Histologic sections of embolized tissue routinely stained with hematoxylin-eosin show the bucrylate as a translucent material. We outline a staining procedure that demonstrates bucrylate in tissue sections. It is useful in routinely prepared paraffin-embedded tissue, and it is also effective after embolized tissues have been processed with petroleum ether rather than xylene. This stain may be effectively utilized to assess harmful tissue reactions to bucrylate.

Arteriovenous Malformations↗

Fibrosarcoma complicating irradiated pituitary adenoma.

Eight years after radiation therapy (5000 rads of 60Co) for a pituitary adenoma, a patient developed a sellar fibrosarcoma. The tumor had an aggressive growth pattern: it infiltrated the optic nerve, sphenoidal air sinus, hypothalamus, and both cavernous sinuses, where compression of the left internal carotid artery resulted in a massive hemispheric infarction. Surgery was ineffective in arresting rapid growth of the lesion; death occurring 5 months after onset of symptoms.

Adenoma, Chromophobe↗

Atheromatous emboli in mural capillaries of carotid endarterectomy specimens.

Seventy-eight carotid endarterectomy specimens were reviewed microscopically for the presence of mural capillary atheromatous emboli. Twelve specimens (15%) were found to contain these emboli. An attempt to correlate this finding with clinical and radiologic data yielded no evidence of an increased predisposition to recurrent transient ischemic attacks or stroke in the affected patients even in the absence of ulceration. Nevertheless, further attention must be given to defining the drainage patterns of such embolized mural capillaries and elucidating their role, if any, in the pathophysiology of stroke.

Adult↗

Astrocytoma following scalp radiotherapy in infancy.

A 24 year old woman with a 15 year history of intractable seizures underwent resection of a small low grade astrocytoma of the right mesial frontal lobe. The tumor arose beneath a benign scalp nevus that had been treated in infancy with radium patches and focal x-irradiation. Neuropathological changes within the tumor were compatible with radiation injury. Meningiomas and sarcomas complicate cranial irradiation but the evidence that gliomas do so is less convincing. Our observations support an association between radiation exposure and the subsequent development of glial tumors.

Adult↗

Computed tomography in temporal lobe epilepsy.

Forty patients who had intractable temporal lobe epilepsy had surgery for treatment of their seizures following study by computed tomography (CT). Fifteen of 19 patients with tumors had good correlation between CT and pathological findings. Another group of patients had macroscopic changes that were demonstrated on CT and verified at surgery. Sixteen patients had microscopic pathological diagnoses including cytoarchitectural abnormalities, Chaslin gliosis, and ectopic neurons. Six of these had enlarged temporal horns on the ipsilateral and five on the contralateral side to the electroencephalographic-pathological abnormality. In the investigation of temporal lobe epilepsy we believe that CT, especially with the newer generation scanners, records tumors and other larger macroscopic changes but that temporal horn size is unreliable in predicting the abnormal side if only microscopic changes are present.

Adolescent↗

Ocular involvement in thrombotic thrombocytopenic purpura: the angiographic and histopathological features.

We report two cases of thrombotic thrombocytopenic purpura (TTP). The first case illustrates the clinical findings and fluorescein angiographic features of this disease, notably the serous macular detachments and focal areas of choriocapillaris occlusion. The second case demonstrates a more severe case of TTP, with neurologic complications that led to death; histopathological examination of the eyes showed serous retinal detachments and choriocapillaris occlusion, with electron microscopic evidence of intravascular fibrin-platelet thrombi.

Adult↗

Iron deposits and multiple sclerosis.

A recent publication described autopsy samples from five brains in which positive staining for iron was observed as surrounding demyelinated plaques. Because this has not been previously reported, autopsy material from 13 patients who were being followed up at the Multiple Sclerosis (MS) Clinic, University Hospital, The University of Western Ontario, London, Ontario, was reviewed. A total of 32 paraffin-embedded blocks containing demyelinated plaques of varying age were sectioned and stained using a standard acid ferrocyanide technique (Peris' test) for iron. Microscopic examination failed to detect the presence of significant positive staining either within or surrounding the areas of demyelination. Positive histochemical staining for iron identifies hemosiderin, the presence of which suggests either iron overload or remote hemorrhage. Support for these two processes in the pathogenesis of MS could not be provided from our material.

Autopsy↗

'Foreign' particles in encephalic vascular malformations.

Among 84 surgically resected arteriovenous malformations (AVMs), four were found to have refractile foreign particles lined by endothelium in the vessel walls. In three cases, this was associated with granuloma formation. The particles were probably introduced during cerebral angiography. They represent a not unusual pathologic finding, are relatively more common in AVMs than in other neurosurgically excised lesions, and may have clinical significance, especially if abundant.

Adult↗

Gerstmann-Sträussler-Scheinker disease with coincidental familial onset.

A family with Gerstmann-Sträussler-Scheinker disease had coincidental clinical onset in three members of two generations, a phenomenon suggesting a common source of a transmissible agent. A regular dietary supplement in this family was home-bred rabbit. The clinical picture, although generally similar to that in previous accounts, included the unusual findings of visual loss (one patient) and sensory loss (one patient), and dementia was not apparent until late in the illness in two patients. Pathological examination of a cerebellar cortical biopsy specimen from one patient and postmortem tissue from two patients revealed multicentric amyloid plaques located in cerebral and cerebellar cortex, basal ganglia, and white matter with degeneration of corticospinal, dorsal spinocerebellar, dentatorubral, and geniculocalcarine tracts and dorsal columns. Spongiform change was focal and confined to the superficial cerebral cortical layers.

Creutzfeldt-Jakob Syndrome↗

Lymphomatoid granulomatosis involving the central nervous system: complication of a renal transplant with terminal monoclonal B-cell proliferation.

A 33-year-old man, an immunosuppressed renal allograft recipient, developed fever of unknown etiology, multiple pulmonary densities, space-occupying lesions in the liver, confusion, disorientation, and seizures with cerebral infarcts. At autopsy, changes classical of active lymphomatoid granulomatosis (LYG) were found in the brain but those studied in the lungs and liver were in the healing phase. In addition, one cerebral focus in the right temporo-occipital lobe showed a lymphomatous transformation. Immunofluorescence studies done on this focus showed monoclonal IgG-lambda cytoplasmic staining in the perivascular infiltrate. Immunoperoxidase studies confirmed the monoclonal nature of this focus but showed a polyclonal pattern in the other foci. This finding is discussed along with the relationship between LYG and lymphoma.

Adult↗

The histologic effect of intraventricular injection of metrizamide.

A patient with sudden apoplexy and coma was found to have hydrocephalus on computed tomographic scan, and metrizamide was instilled into the ventricles. Subsequent autopsy disclosed a brainstem infarct secondary to a primary dissecting aneurysm of the basilar artery, and a histologic picture of encephalitis in the walls of the lateral and third ventricles. Metrizamide may cause pathologic changes that must be recognized for the correct interpretation by the pathologist of tissue sections previously exposed to metrizamide. In the present case, the changes were seen 11 days after exposure of the tissue to metrizamide, consonant with the time course of similar changes observed in animals. The localization of the cellular infiltrates to the Virchow-Robin spaces corroborates evidence from the literature that metrizamide enters brain parenchyma via these spaces.

Adult↗

Childhood brain tumors presenting as chronic uncontrolled focal seizure disorders.

Sixteen of 35 patients (46%) 21 years old or less who underwent surgical resection of a longstanding epileptic focus harbored tumors. The median duration of the seizure disorder prior to operation was 6.0 years for patients with and 7.6 years for those without tumor. Among the 35 patients, tumor was more common when intelligence and results of neurological examination were each normal, a plausible cause for uncontrolled seizures was lacking, and persistent focal delta activity occurred in a majority of electroencephalograms (EEGs). The type of seizures and the distribution of EEG spikes failed to distinguish patients with tumor from those without. Multifocal EEG spikes appeared in a majority of each group.

Adolescent↗

Selective spinal cordectomy: clinicopathological correlation.

The operation of selective spinal cordectomy is rarely performed. The cases of 10 paraplegic patients who underwent this procedure are presented. Their clinical indications were varied, including posttraumatic syringomyelia, uncontrollable leg spasticity, posttraumatic spontaneous neurogenic leg pain, and repair of a myelomeningocele gibbus. The cordectomy specimen was available for pathological examination in nine cases. Specimens removed from or near the site of spinal cord trauma showed extensive gliosis, leptomeningeal fibrosis, and schwannosis. This latter feature possibly represents an attempt at spinal cord repair by the dorsal root ganglion cells. Four specimens contained a syrinx with features including a thick gliotic wall and enlarged Virchow-Robin spaces, features that have been implicated in the pathogenesis of posttraumatic syringomyelia. Clinical results in the patients with syringomyelia and uncontrollable leg spasticity have been excellent. Cordectomy did not provide permanent relief in the patients with neurogenic leg pain. The authors conclude that selective spinal cordectomy is a valuable procedure for specific indications.

Adult↗

Pathology of arteriovenous malformations embolized with isobutyl-2-cyanoacrylate (bucrylate). Report of two cases.

There is controversy as to the possible toxic effects of isobutyl-2-cyanoacrylate (bucrylate) when this substance is used for purposes of therapeutic embolization. Two cases are presented in which cerebral arteriovenous malformations were resected, one 42 days and the other a year after bucrylate embolization. In both, pathological examination revealed a brisk intimal foreign-body giant-cell reaction wherever bucrylate was present in a vessel, along with chronic inflammation in the vessel walls and adjacent brain parenchyma. The findings are discussed in the light of other observations on the histotoxicity of bucrylate.

Adult↗

Improved technique for cutting brain slices.

When two disposable serological pipettes with an external diameter of 1 cm are placed on the cutting surface on either side of the brain and the knife rides across them, it will cut even slices precisely 1 cm thick. Both coronal and horizontal slices can be cut as well as oblique slices for comparison with computerized tomographic scan roentgenograms although for the latter the initial cut will have to be made freehand. For practical purposes, brain slices obtained by this simple technique are as good as those made with a mechanical jig or electric machine and are superior to those made freehand.

Brain↗

Vertebrobasilar artery insufficiency in rheumatoid atlantoaxial subluxation.

Cervical myelopathy has become commonly recognized as a complication of rheumatoid atlantoaxial subluxation. A small group of patients with atlantoaxial subluxation may have intermittent symptoms associated with change of head position and which are due to vertebral artery compression. Two such cases are reported, one with necropsy findings of infarction in the area supplied by the vertebrobasilar system. The pathogenesis of the symptomatology and infarction is discussed.

Aged↗

Isolated spinal cord arteritis.

This patient presented as a subacute progressive cervical myelopathy and the differential diagnosis included cervical spondylotic myelopathy and intramedullary mass. Microscopically, vascular lesions plus a patchy myelomalacia indicated a vasculitis. However, there was no suggestion of a generalized vasculitis at autopsy and the only supporting laboratory study was a raised erythrocyte sedimentation rate. It would seem that a vasculitis similar to polyarteritis nodosa or other collagen disease may be confined to the spinal cord.

Arteritis↗