A case of hereditary motor and sensory neuropathy type III with a decrease in unmyelinated fibers

We report a 3-year-old girl with hereditary motor and sensory neuropathy type III with a decrease in unmyelinated fibers. There have been few reports of such cases. The present findings suggest the possibility that the primary lesion in this disease is in the axons. We consider that more attention should be paid to the changes in unmyelinated fibers and axons in further studies on this disease.     

The cytochromes P-450 concentrations in microsomes of liver, kidney and duodenal mucosa of the camel, sheep and goat

The concentration of microsomal cytochromes P-450, and of protein in the homogenate, cytosol and microsomes were measured in the liver, kidney and duodenal mucosa of healthy well-fed male and female camels, sheep and goats. For comparison, data from the liver of male and female rats were also obtained. The protein concentrations in the tissues of adult animals were broadly similar in the four species. The concentration of cytochromes P-450 was highest in the liver, followed by the kidney, then the duodenal mucosa in all the species. No cytochromes P-450 were detected in the tissues of immature (less than 1 mo) male goats, whereas the female goat had the highest concentrations of these enzymes in the liver and kidney when compared with the respective tissues in the other species studied. Males had higher activity of cytochromes P-450 than females in the three tissues, except in the duodenal mucosa of sheep, where males had lower activity than females. In camel liver and sheep kidney, the amount of cytochromes P-450 were similar in the two sexes. The present results suggest that the mature female goat is the species best equipped to handle xenobiotics which are detoxified by the cytochromes P-450 and other drug metabolizing enzymes in diseased or malnourished animals is suggested as these two conditions are known to modify drug metabolizing enzymes.     

Ketotifen treatment of adverse reactions to foods: clinical and immunological effects

Fifteen patients with cutaneous signs and symptoms caused by adverse reactions to foods were treated in an open trial with ketotifen for 4 to 6 weeks. Seven subjects were allergic and 8 had food intolerance. Each patient was treated with a single dose of ketotifen daily: 2 mg half an hour before going to sleep. Clinical improvement was achieved in 6 out of 7 allergic patients and in 6 out of 8 patients with food intolerance. Since several drugs have been demonstrated to have an influence on immune response, the in vitro effects of ketotifen on some immunological parameters were also studied. Ketotifen showed a significant inhibitory effect on autologous mixed lymphocyte reaction responsiveness.     

Syrup of ipecac availability: before and after a poisoning

A retrospective review was conducted of 1,230 human poison exposures in which syrup of ipecac was administered to determine the availability of this emetic. Ipecac was available in 41.1% of the homes, while 42.5% obtained it from the pharmacy. Eight and two tenths percent were referred to a health care facility, 2.9% obtained ipecac from a neighbor, 2.3% went to an emergency room prior to calling the poison center, and 3% obtained ipecac from other sources. A randomly selected sample of 150 of these 1,230 cases were contacted 6 months after their initial call to the poison center to determine any changes in the availability of syrup of ipecac in the home. Although almost 30% more homes had syrup of ipecac than previously, 22.2% of homes still did not have ipecac available, despite the previous poisoning experience. Greater effort should be made during follow-up to educate the public regarding ipecac and its use.     

Familial achalasia associated with adrenocortical insufficiency, alacrima, and neurological abnormalities

We report on two brothers with achalasia, adrenocortical insufficiency, alacrima, short stature, microcephaly, ataxia, optic atrophy, and developmental delay. The parents and three sibs are unaffected. Achalasia, adrenocortical insufficiency, and alacrima comprise a recently characterized familial multisystem disorder of unknown cause. Achalasia has also been described in association with microcephaly and mental retardation in one family and ataxia, optic atrophy, and mental retardation in another. The above reports and these sibs may represent variants of a single pleiotropic recessive gene. We suggest that abnormalities of the central nervous system are a manifestation of the achalasia, adrenocortical insufficiency, alacrima syndrome.     

Properties of neuronal activity related to a visual reaction time task in the monkey prefrontal cortex

1. Quantitative properties of neuronal activity related to a visual reaction time task were studied in the monkey prefrontal cortex. The task consisted of an initial waiting phase (3.0-s period), a warning phase (green lamp, a variable period of 1.5-3.5 s), a go phase (red lamp), and a reward phase. 2. A total of 189 task-related neurons showed 233 changes in discharge rates during the warning (n = 86), GO (n = 103), and reward (n = 44) phases of the task. Most of the task-related neurons (145/189, 77%) showed changes during only one of the task phases, and were designated W (warning phase)-type (n = 42), GO (go phase)-type (n = 59), and RE (reward phase)-type (n = 44) neurons. The remainder (n = 44, 23%) showed changes during both the warning and the go phases, and were designated WG (warning and go phase)-type neurons. In each phase, onset latencies, peak latencies, and decay times of each change were measured and compared. 3. The changes during the warning phase (n = 86) were separated into three groups based on decay time; that is, phasic changes (n = 31), phasic-tonic changes (n = 23), and tonic changes (n = 32). Onset latencies and peak latencies were homogeneously distributed, and there were no clear groupings, although phasic and phasic-tonic changes tended to show shorter latencies than tonic changes. 4. The changes during the go phase (n = 103) did not show distinct differences, either in terms of decay time or of latency. The changes during the go phase showed various degrees of coupling to both the visual go signal (GS) and lever-release hand movement. To quantitate the coupling, a value to indicate the degrees of coupling (coupling index) was calculated. The changes coupled more strongly to the GS (cue coupled), those coupled more closely to the lever release (movement coupled), and intermediate changes could be distinguished from each other. The cue-coupled changes showed shorter latencies from the time onset of the GS than the movement-coupled changes, and the intermediate changes showed intermediate latencies. The decay time and the duration of the intermediate changes were longer than those of the cue-coupled changes and the movement-coupled changes. 5. The properties of WG-type neurons were compared with those of W-type and GO-type neurons.(ABSTRACT TRUNCATED AT 400 WORDS)