James W. Schmidley

Brain injury, edema, and vascular permeability changes induced by oxygen‐derived free radicals

We studied the cerebral effects of oxygen-derived free radicals generated from the xanthine oxidase/ hypoxanthine/ADP-Fej3+ system. Xanthine oxidase/hypoxanthine/ADP-Fe3+ solution (0.1 ml) was infused into caudate putamen, and brain was frozen rapidly in situ. Brain water and sodium content increased concomitant with decreased potassium content at 24 hours and 48 hours after the infusion. The degree of brain edema and injury depended on the dose of xanthine oxidase. Spongy neuropil and neuronal cytoplasmic vacuoles were seen at 2 hours, with an infiltration by polymorphonuclear leukocytes at 24 hours, followed by lipid-laden macrophages and reactive astrocytes. Leakage of fluorescent dye into neuropil was seen at 2 hours, but not later. These data suggest that oxygen-derived free radicals damage endothelial cells of the blood-brain barrier; the brain injury is characterized by edema and by structural damage of neurons and giia.

Syphilitic polyradiculopathy in an HIV‐positive man

We report a case of syphilitic lumbosacral polyradiculopathy in an HIV-positive, 22-year-old bisexual man with a recent history of secondary syphilis treated with intramuscular penicillin. He presented with rapidly progressive pain and weakness, and muscle wasting in the legs. CSF was under increased pressure and showed a marked pleocytosis (1,130 cells/mm3), hypoglycorrhachia (19 mg/dl), and very elevated protein (1,000 mg/dl). Serum and CSF VDRL serologies were positive. In the legs, nerve conduction studies and needle EMG were consistent with an asymmetric lumbosacral polyradiculopathy with active denervation. His clinical state, CSF, and electrophysiologic studies all improved promptly and markedly after intravenous penicillin. This report documents an uncharacteristically aggressive case of neurosyphilis accompanied by marked changes in the CSF in an HIV-positive patient. While the immunologic effects of HIV and syphilis in combination are not yet fully understood, the cellular immunity defect associated with HIV may alter the natural history of syphilis in patients with concomitant infection, producing unusually aggressive forms or atypical presentations of neurosyphilis.

Delayed postanoxic demyelination and arylsulfatase‐A pseudodeficiency

We report a patient with delayed postanoxic demyelination who had pseudodeficiency of arylsulfatase A, reducing his enzyme activity to 10 to 30% of normal. This may have implications regarding the pathogenesis of postanoxic demyelination.

Agitated confusional states in patients with right hemisphere infarctions.

Patients with infarctions in the territory of the right middle cerebral artery (RMCA) some- times present with an agitated confusional state. We reviewed clinical data on 46 patients with RMCA infarcts and compared neurologic findings in patients with and without agitated confusion. Neither of the two patients presenting with agitated confusion showed obvious localizing neurologic signs; subtle motor, visual field and sensory deficits referable to the infarcted regions were present, but difficult to elicit because of the mental state. In contrast, all but one of the patients without agitated confusion had prominent motor and sensory signs. Infarction of the RMCA territory may cause agitated confusion in patients without prominent localizing signs; the initial neurologic findings may suggest a metabolic encephalopathy. Howev- er, the possibility of a cerebrovascular cause should not be dismissed in confused and agitated patients who have no definite lateralizing signs. Stroke Oct 15, No 5, 1984

The noncerebrovascular complications of chiropractic manipulation.

We describe a patient who suffered cervical spine, spinal cord, and nerve root injury as a result of chiropractic spinal manipulation, and we review medical literature concerning the noncerebrovascular complications of spinal manipulation.

Infantile X‐linked ataxia and deafness A new clinicopathologic entity?

We describe an X-linked disorder of the CNS, characterized by onset, in infancy, of hypotonia, ataxia, sensorineural deafness, developmental delay, esotropias, and optic atrophy, and by a progressive course leading to death in childhood. Pathologically, neuron loss and gliosis of the dentate nucleus and inferior olive are conspicuous; involvement of the cerebellar cortex is less prominent. In the proband, the red nucleus, dorsal motor nucleus of the vagus, and central auditory pathways were severely affected. The mother of the proband, now 33, has self-limited episodes of ataxia, and cerebellar atrophy for which no other cause is apparent. The unique heredity, pathology, and clinical picture distinguish this entity from previously described inherited or metabolic ataxias.

Abundant, uniquely oriented endoplasmic reticulum in capillaries of the CNS: demonstration using reduced-osmium and glucose-6-phosphatase cytochemistry.

In the endothelial cells of capillaries in the rat CNS, we have observed abundant, circumferentially oriented, smooth, membrane-bound profiles, found just beneath, and parallel to, the abluminal plasmalemma. These structures are seen particularly well in tissue exposed to potassium ferricyanide-reduced OsO4. We studied these structures cytochemically, using glucose-6-phosphatase as a marker for endoplasmic reticulum, and acid phosphatase as a marker for the Golgi-associated endoplasmic reticulum containing lysosomal enzymes (GERL). We found that they contained glucose-6-phosphate hydrolyzing activity but did not contain acid phosphatase activity. Comparable structures were not seen in the continuous capillaries of skeletal muscle. Based on their morphology and content of glucose-6-phosphate hydrolyzing activity, we conclude that these structures are uniquely oriented smooth endoplasmic reticulum, which is much more abundant in capillaries of the CNS than in other continuous capillaries. The function of this distinctive feature of the CNS capillary is not known.

Transient cerebral ischemia: Pathophysiology

Abstract The clinical features of TIAs in the anterior and posterior cerebral circulations are fairly uniform and usually easily recognized. The pathogenesis of TIAs is varied, complex, and incompletely understood. The importance of TIAs rests on the fact that they may serve as dramatic warnings of an impending stroke. Depending on the type and cause of a TIA, medical therapy, alone or in combination with surgical intervention, can postpone or sometimes prevent permanent neurologic deficit. It is the task of the clinician first to determine which among many mechanisms—vascular, cardiac hematologic, or circulatory—is responsible for transient neurologic symptoms in an individual patient, and then to supply appropriate therapy as prophylaxis against a stroke.

Delayed postanoxic demyelination registry

To the Editor: We read with interest the report by Gottfried et al. describing a second patient with delayed postanoxic demyelination and arylsulfatase A pseudodeficiency.1 Taken together with our initial case,2 the evidence supporting a causal association between relative deficiency of arylsulfatase A …