S. Mark Sumi

Giant axonal neuropathy caused by industrial chemicals: neurofilamentous axonal masses in man.

Symmetrical polyneuropathy developed in two patients after they had been in contact with acrylamide and methyl n-butyl ketone, respectively. In sural nerve biopsy material from both patients, electron microscopy showed frequent focal axonal swellings containing masses of neurofilaments. Some axons undergoing axonal degeneration also were seen. These morphologic features are identical to those produced in experimental animals after exposure to these chemicals and are similar to those found in n-hexane neuropathy and in the three reported cases of giant axonal neuropathy. Sural nerve biopsy is an important diagnostic test in identifying cases of peripheral neuropathy caused by these chemicals.

Presenile Alzheimer disease Amyloid plaques in the cerebellum

Seven of 24 patients with pathologically proved Alzheimer disease had amyloid plaques resembling kuru plaques in the cerebellum. In all seven patients, the dementia was presenile in onset, there was a positive family history of the disease in five patients, and other neurologic symptoms were present in five. Similar plaques have been described in one of two patients with familial Alzheimer disease from whom spongiform encephalopathy was induced in subhuman primates after intracerebral inoculation of brain tissue. These observations suggest that some cases of familial Alzheimer disease may be clinically distinct from senile dementia and may not be genetic in origin.

Cerebrospinal fluid and Serum creatine kinase BB activity after out‐of‐hospital cardiac arrest

In patients resuscitated from out-of-hospital cardiac arrest, neurologic outcome was compared with creatine kinase isoen-zyme BB activity (CKBB) in cerebrospinal fluid (CSF) in 20 patients and in serum in 52 patients. CSF CKBB was 2 units Der liter or less in patients with complete neurologic recovery but was significantly elevated in patients without neurologic recovery (mean, 55 units per liter) or with incomplete neurologic recovery (mean, 7 units per liter). Serum CKBB was detected more than 6 hours after cardiac arrest in only 4% of patients with complete neurologic recovery but in all patients without neurologic recovery. These results demonstrate a relationship between CSF and serum CKBB and neurologic outcome after cardiac arrest.

A simple method of rapid freezing adequately preserves brain tissue for immunocytochemistry, light and electron microscopic examination

A simple and reproducible method for cryo-preservation of brain tissue from patients with Alzheimers disease is described. Fresh brain slices (1 cm thick) obtained less than 6 h postmortem are placed in sealed plastic bags, sandwiched between 0.3-cm-thick aluminium sheets, and frozen by placing the entire “sandwich” between layers of dry ice pellets. The frozen brain slices are stored at −85 °C. Specific anatomic areas can be retrieved at any time for light and electron microscopic, immunocytochemical, autoradiographic and neurochemical studies.

Sudanophilic lipid accumulation in astrocytes in periventricular leukomalacia in monkeys.

SummarySudanophilic lipid accumulation is a characteristic feature of periventricular leukomalacia (PVL) of infants. At least two types of lipid-containing cells have been identified, one being the macrophage, the other the “pre-myelin glial cell”. A third type of lipid-containing cell has been seen in two monkeys with spontaneous PVL. Electron microscopically this cell appears to be an astrocyte. This probably represents a reaction of the astrocyte to hypoxia and may be the equivalent of the “hypertrophic” astrocytes found in human infants.

Effects of Drugs, Narcotics and Toxins on the Chemical Maturation of the Infant Brain

The remark by Norman (1958) that “the act of birth marks no particular milestone in the development of the human brain” has made it essential that embryologists, neonatologists, pediatricians, pathologists and others work together to bridge gaps created by professional but artificial time-based periods of study. Lemire et al. (1975) have argued that “malformations of the nervous system” can much more easily be considered as part of the spectrum of “diseases of the developing nervous system.”