Stroke related to cerebral amyloid angiopathy: the significance of systemic vascular disease.

A retrospective postmortem analysis of 25 cases of cerebral amyloid angiopathy (CAA) in the setting of Alzheimer's disease or senile dementia of the Alzheimer type (AD/SDAT) is reported. Seven patients experienced clinically significant cerebral infarcts or hemorrhages or both. There was no statistically significant difference in the incidence of infarcts or hemorrhages in hypertensive and nonhypertensive patients. Hypertension does not appear to be an additional risk factor in the causation of cerebral infarct or hemorrhage associated with CAA in the setting of AD/SDAT. Just over half of patients with CAA and significant ischemic and/or hemorrhagic brain lesions showed atherosclerosis of the circle of Willis, sometimes in the context of severe disseminated atheromatous disease.

Eosinophilic fasciitis with porphyria cutanea tarda and progressive destructive arthritis.

The course of arthritis with eosinophilic fasciitis is poorly documented. We report a patient in whom polyarthritis preceded the clinical appearance of eosinophilic fasciitis and progressed over a 14-year period to a destructive process with inflammatory and degenerative characteristics. Porphyria cutanea tarda and transient extramedullary pancytopenia complicated this patient's disease.

Central pontine myelinolysis: a complication of hyponatremia or of therapeutic intervention?

We report four cases of central pontine myelinolysis (CPM) that illustrate important features of the disorder. The condition is described mainly in the neurological literature and, to our knowledge, is not discussed in the forensic science journals. This disorder must be recognized and understood by the forensic science expert who addresses issues of liability. In cases of multiple motor deficits and death with a history of hyponatremia, CPM must be included in the differential diagnosis. Careful examination of the pons and adjoining structures must be performed. Myelin stains are advisable. The association of CPM with major illnesses, hyponatremia and the correction of hyponatremia by intravenous saline infusions is discussed.

Saccular aneurysm associated with segmental duplication of the basilar artery. A morphological study.

Saccular aneurysm associated with segmental duplication (also called "fenestration") of the basilar artery is an anomaly that results from an embryonic fault. Reports of the treatment of the aneurysmal component have only recently appeared in the neurosurgical literature, and little has been written on the morphology of this anomaly. This study answers the need for information about its structure to the extent permitted by the examination of a single specimen. The specimen was obtained at postmortem examination. A cast of its interior features was made with a synthetic rubber. After the cast was removed, the entire anomaly was serially sectioned for histological study. Defects in the wall of the basilar artery were seen microscopically at each end of the fenestration. At the extensive proximal defect, a saccular aneurysm arose that bulged into the window between the two limbs of the segmental duplication and also presented dorsally and ventrally. It had fatally ruptured. The manner in which the fenestration was formed by intraluminal septa was also revealed.

Bilateral diaphragmatic paralysis as a possible paraneoplastic syndrome from renal cell carcinoma.

Bilateral diaphragmatic paralysis is rare. We describe a patient with bilateral diaphragmatic paralysis who died 18 months after initial presentation and who was found to have renal cell carcinoma. At autopsy, no intrathoracic tumor was found that would explain the diaphragmatic paralysis. We believe that this may represent a paraneoplastic syndrome caused by renal cell carcinoma.

Central nervous system involvement as a presenting feature of acute monocytic leukemia: preceding leukemia by 12 months.

This report describes a patient who presented with a diffuse malignant central nervous system (CNS) process composed of cells with monocytic differentiation one year before the development of acute monocytic leukemia. A similar presentation has been documented for acute lymphocytic leukemia, but to the best of our knowledge, this is the first report to detail a diffuse CNS malignancy as the presenting symptom for acute monocytic leukemia.

Septate bifrontal ependymal cyst. Case report.

The authors describe the case of a septate ependymal cyst involving both frontal lobes. The clinicopathological features, treatment, and results of previously reported cases are reviewed, and the etiology and pathogenesis of these cysts discussed.

Peripheral neuropathy associated with monoclonal gammopathy. Studies of intraneural injections of monoclonal immunoglobulin sera.

A causal relationship between paraproteinemia and neuropathies has been suggested. We studied three patients with chronic sensorimotor polyneuropathy associated with plasma cell dyscrasia and monoclonal gammopathies (IgGK, IgMK, IgA lambda). Sural nerve biopsies showed mild (2 cases) to moderate loss of myelinated fibers (1 case). Teased single fiber studies showed segmental demyelination-remyelination in two patients. Direct immunofluorescence demonstrated immune deposits of the myelin sheath of the same specificity as the serum paraprotein, IgGK (1 of 3 cases). Treatment with prednisone, melphalan or chlorambucil, and plasmapheresis resulted in remission (1 case), partial improvement (1 case), or had no effect (1 case), although reduction of monoclonal immunoglobulin occurred in all. To investigate the role the paraproteins might play in the pathogenesis of the neuropathy, patients' serum was injected intraneurally into rat sciatic nerves. None of the animals developed weakness, slowing of in vitro conduction of sciatic nerve, or significant evidence of demyelination by light- or electron-microscopy or teased single fiber studies 48 hours postinjection. Similar injections of rabbit serum with experimental allergic neuritis (EAN) produced focal segmental demyelination. Our studies employing an in vivo bioassay technique failed to establish antimyelin activity of monoclonal immunoglobulin sera.

Disulfiram neuropathy: a neurofilamentous distal axonopathy.

Disulfiram is used to treat alcoholism and is known to cause peripheral neuropathy: few reports of biopsied human nerves have revealed axonal degeneration and loss of myelinated fibers. We studied a 22-year-old woman with severe sensorimotor neuropathy following treatment with disulfiram for 6 months. Histologic studies of the sural nerve revealed a neurofilamentous axonopathy with rare enlarged axons distended by neurofilaments. Disulfiram is converted enzymatically to carbon disulfide, which causes neurofilamentous distal axonopathy in animals. Similar changes in human nerve after disulfiram administration suggest that carbon disulfide is the toxic agent.