Subacute spongiform encephalopathy (Creutzfeldt-Jakob disease) with amyloid angiopathy.

A case is reported of Creutzfeldt-Jakob disease associated with amyloid infiltration of cerebral vessels. The duration of progressive dementia was only 4 months. Neuritic plaques were not a feature of the pathology. This report emphasises the association of spongiform encephalopathy with the presence of amyloid in the brain.     

Subacute spongiform encephalopathy and its relation to Jakob-Creutzfeldt disease: report on six cases

An account is given of six cases of Jakob-Creutzfeldt disease confirmed at necropsy. The rapid fatal outcome within three months was typical of the variety designated subacute spongiform encephalopathy (Nevin-Jones). The characteristic EEG changes were found to be of great value in making an early diagnosis and, together with akinetic mutism and myoclonus, constituted an easily recognizable picture. Treatment with idoxyuridine was ineffective in one case in which electronmicroscopy of brain biopsy material supported the diagnosis.     

Electron microscopic study of experimental acute hypertensive encephalopathy

Summary Electron microscopic examinations were carried out in the brains of cats with acute arterial hypertension produced by intermittent compression of the descending aorta.The ultrastructural changes included swelling of the astrocytic processes, dilatation of the endoplasmic reticulum, and slight enlargement of the extracellular space of the white matter. Swelling of the mitochondria with disruption of the cristae was sometimes observed. The extracellular space of the gray matter showed no expansion.The changes in the ultrastructure of the central nervous system in hypertensive and ischemic encephalopathy were found to be very similar. This finding is discussed with special regard to the pathogenesis of acute hypertensive encephalopathy.Supported by research grant NB 03754-06 from the National Institute of Neurological Diseases and Blindness.     

Subacute spongiform encephalopathy with periodic paroxysmal activities: clinical evolution and serial EEG findings in 20 cases

Evolution of both clinical and EEG abnormalities was analyzed in 20 (16 pathologically confirmed) patients suffering from subacute spongiform encephalopathy with periodic paroxysmal activities (PPA) on the EEG. Illness duration was less than 4 and greater than 17 months in 65% and 10% of cases, respectively. All data but EEG were utilized to assess 3 conventional clinical stages in 20 patients. The early clinical stage was characterized by gradual presentation of gait disturbances, mental deterioration, sensory or autonomic disorders. In contrast with other reports, no PPA were observed in 10 EEG recordings from 7 patients examined at the early clinical stage. Both clinical and EEG findings were not in contrast with a hypothetic subcortical onset of disease. Similar to recent data in the literature, early PPA appeared within 12 weeks of disease evolution in 88% of patients who underwent EEG recordings in the first 3 months of disease. Nonetheless, these early PPA always occurred at an intermediary stage, when our patients showed a marked worsening of the clinical picture. Focal, segmental and/or generalized myoclonic jerks were observed in 15%, 53% and 100% of cases at prodromal, intermediary and terminal stages respectively. Different kinds of PPA were observed: bi-tri-phasic periodic complexes (PC), periodic complexes with multiphasic configuration (PPC) and periodic polyspiking discharges (PPD). Abnormal "pacing" of PC by slowly repeated flashes was found in 4 patients presenting visual hallucinations or cortical blindness. Burst-suppression activity was frequently found at the terminal stage in decorticate patients.     

Electron microscopic studies on the lamprey meninges

Lamprey meninges were revealed to consist of four layers. The I (outermost) layer was made up of densely packed collagen fibrils and microfibrils with fibrocytes interposed between them. It contained a vascular network (epidural vascular plexus). The II layer was a cellular layer, consisting of two parts: the outer, membranous and the inner, spongy part. The membranous part was composed of two sheets of type 1 cells which were held together by desmosomes, gap junctions and tight junctions. Continuous basal laminae were found on both the outer and the inner surface of the membranous part. The spongy part consisted of type 2 cells, collagen fibrils and microfibrils, which were loosely arranged. Type 2 cells were joined together with each other, or with type 1 cells, by gap junctions. These cells lacked a basal lamina covering. The III layer was a peculiar thick layer, being mostly occupied by fine filamentous matrix substance with scattered round cells, fibroblasts and microfibrils. The IV (innermost) layer was a thick fibrous one, consisting of two parts: the outer, vascular and the inner, loose part. The former consisted of a vascular network (perineural vascular plexus) and thick bundles of microfibrils which frequently contained a dense filamentous core. The latter was composed of loosely arranged collagen fibrils. The inner part of the I layer (inside the epidural vascular plexus) was considered to correspond to the true dura, and the II and IV layer to the arachnoid and pia in the meninges of higher animals, respectively. Characteristically the subarachnoid space was absent in lamprey meninges, and instead a peculiar “gelatinous” layer, III layer, was present between the II and IV layer.     

Nuclear inclusions in subacute spongiform encephalopathy

Summary Type A inclusions were observed in glial cells from necropsy material from a case of subacute spongiform encephalopathy. Their origin and aetiological significance are obscure.

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Zusammenfassung Kerneinschlüsse vom Typ A wurden in Gliazellen eines Autopsiefalles von subakuter spongiöser Encephalopathie beobachtet. Ihre Genese und ätiologische Bedeutung sind unklar.