Stanley M. Aronson

Diffuse intracytoplasmic ganglionic inclusions (Lewy type) associated with progressive dementia and quadriparesis in flexion.

Intraganglionic inclusion bodies of presumably non-viral ongm have been associated with a range of defined neurologic disturbances, including myoclonus epilepsy (1), Picks disease (2), and Parkinsons disease (3). In each of these entities the inclusions present a pattern of characteristics (histochemical and histologic properties, topographical distribution and size) which permits a degree of diagnostic differentiation. In the Parkinsonian states, the inclusion is typically intracytoplasmic, and most frequently encountered in the pigmented neurons of the mesencephalic and rhombencephalic nuclei (4). Two elderly patients with progressive dementia associated with quadriparesis in flexion, and without any Parkinsonian stigmata, have been studied, demonstrating at autopsy widely disseminated intracytoplasmic ganglionic inclusions morphologically and histochemically indistinguishable from those initially described by Lewy in Parkinsons disease (paralysis agitans).

Familial centronuclear myopathy: a clinical and pathological study.

RECENTLY, Spiro and associates1 reported an isolated case of a hitherto undescribed myopathy, characterized by the presence of central nuclei in as many as 85% of the skeletal muscle fibers, without associated degenerative changes. We have observed the same pathological features in muscle biopsies of two sisters suffering from slowly progressive muscular weakness and wasting. Similar changes were present focally in the biopsied muscle obtained from the clinically normal mother of these patients. These cases apparently represent a hereditary, slowly progressive myopathy which is morphologically distinct from other myopathies. It is the purpose of this report to present and discuss the clinical and pathological features of this disease.

Intramedullary nerve fiber and Schwann cell proliferation within the spinal cord (schwannosis).

w Raymond was apparently the first to call attention to clusters of Schwann cells proliferating within the substance of the spinal cord in a case of syringomyelia.’ Subsequent authors have considered this lesion, noting its association with a variety of nervous system diseases, particularly those involving the spinal Koeppen and associates surveyed the published reports of intramedullary Schwann cell and nerve fiber proliferation and tabulated 61 cases, finding most to be associated with local disorders such as syringomyelia, compressive lesion, trauma, or neighboring neoplasm.’ Russell and Rubinstein described spinal subpial Schwann cell foci in close apposition to a root schwannoma and regarded them as developmentally displaced cells.’ They had also noted such foci in the neighborhood of spinal ependymomas. These authors employed the term “schwannosis” to describe these spinal cord cell collections, stating they had been encountered only in conjunction with various neoplastic and chronic degenerative disorders of the cord. In the present paper we report the frequency of focal intramedullary nerve fiber and Schwann cell proliferation, or so-called schwannosis, in an unselected autopsy series from a general hospital.

Gangliogliomatous maturation in cerebellar medulloblastoma

SummaryTwo cases of gangliogliomatous maturation of cerebellar medulloblastomas are presented in support of Bailey and Cushings concept of the medulloblast as a bipotential element capable of both neurogenesis and gliogenesis. The presence of mature but atypical neuronal and glial elements was demonstrated histologically by specific procedures. The suggestion is made that cellular maturation in cerebellar medulloblastomas, as in extracranial neuroblastomas, may be more frequent in females than in males.ZusammenfassungZwei Fälle von cerebellarem Medulloblastom mit gangliogliomatöser Ausreifung werden beschrieben, welche die Bailey und Cushingsche Auffassung vom Medulloblasten als ein bipotentiales Element, das der Neurogenese und Gliogenese fähig ist, unterstützen. Reife aber atypische Nervenzellen und gliöse Elemente sind durch spezielle Methoden darstellbar. Es wird angenommen, daß eine celluläre Reifung in cerebellaren Medulloblastomen, ähnlich wie in extracranialen Neuroblastomen, häufiger bei weiblichen als bei männlichen Personen auftritt.

Cardiac Disorders Predisposing to Embolic Stroke

Data from 4,558 consecutive complete necropsies in patients 16 years of age or older were analyzed to ascertain the influence of certain variables upon the frequency of embolic encephalomalacia. Embolic lesions of the central nervous system occurred more commonly in the female, regardless of age, race, diabetic status, nutritional status or heart size, partly because the cardiovascular lesions responsible for embolic dissemination were more common in women. While affecting only a small percentage of patients at any age, cerebral embolism assumes more importance in strokes of young adulthood. Embolic infarcts demonstrate a predilection for cerebral and cerebellar cortical locations via the long circumferential arterial branches and tend to spare the parasagittal perforating branches supplying the brain stem and diencephalic nuclei. Certain cardiac states predispose to cerebral embolism. Patients with endocarditis show the greatest risk of embolic lesions, although patients with rheumatic valvular disease of the left heart and mural thrombi of the left ventricle produce numerically more cases of embolic encephalomalacia because these conditions occur far more often than the endocardidites.