Golgi study on brain of macular mutant mouse as a model of Menkes kinky hair disease

Summary This study was undertaken to elucidate, using the Golgi method, the neuropathological change in the brain of the macular mutant mouse, whose hemizygote (Ml/y) is considered to be a model of Menkes kinky hair disease (MKHD). The hemizygote mice gradually lost weight after 10 days of age and died with emaciation and seizure around day 15. The normal littermate (+/y) was well developed. In the cerebrum, the arborization of pyramidal neurons in the layer V of the Ml/y was the same as that in the +/y on day 10. However, development of arborization in the Ml/y was delayed in comparison with that in the +/y on days 12 and 14. Purkinje cells with several somal sprouts were observed in the cerebellum in both the Ml/y and +/y on day 7. The somal sprouts in the +/y had regressed gradually by day 12, while they were still in the anterior and middle lobes of the Ml/y on day 14. Additionally, the trunks of Ml/y stem dendrites became thicker and a cactus formation was recognized on the branching portion of the dendrites on day 14. Arborization of these abnormal Purkinje cells was distinctly poor compared with that in the +/y. These results suggest that the growth of the neurons is delayed in the Ml/y and simultaneously their cytoskeletal developments are disturbed, especially in the Purkinje cells. There is a close similarity in many respects to the neuropathological change in MKHD.     

Symptomatic HIV infection of the CNS in a patient without clinical evidence of immune deficiency

Major depression with psychotic features, dementia, and focal neurologic abnormalities appeared in a Haitian man without AIDS or other syndromes of immune compromise. Neurologic evaluation, including brain biopsy, was nondiagnostic, but CSF culture revealed human immunodeficiency virus (HIV).     

In vivo electrochemical demonstration of potassium-evoked monoamine release from rat cerebellum

In vivo electrochemical methods were employed to study the potassium (K⁺-evoked release of monoamines from the cerebellum of the chloral hydrate anesthetized rat. K⁺-evoked releases were elicited using micropipette-Nafion-coated graphite epoxy electrode arrays in the granule/Purkenje cell layer, molecular layer, and white matter. These recorded releases were generally found to be reversible, moderately dose-dependent, and reproducible. However, the temporal dynamics of the releases were different for the cell layer versus molecular layer records. Releases were infrequently observed in cerebellar white matter, an area which is relatively devoid of monoamine containing terminals. The signals recorded from the cell and molecular layers were significantly attenuated by pretreatment with nomifensine, a potent catecholamine reuptake blocker, significantly prolonged the K⁺-evoked signals observed in both the granule/Purkenje cell and molecular layers. These data, taken together with earlier reports on the electrophysiological responses to activation of cerebellar noradrenergic inputs, support the conjecture that in vivo electrochemical recording methods have the sensitivity and spatial resolution for studies of functional monoamine release from brain regions that have a diffuse or laminated monoamine innervation.     

Hyperprolactinaemia and sexual function in the male

Although an uncommon cause of sexual dysfunction, hyperprolactinaemia is almost invariably associated with loss of libido and impotence in men. The finding of hyperprolactinaemia should prompt a systematic search for an underlying cause, particularly prolactin-secreting pituitary adenomas; in this condition, sexual dysfunction may antedate other complications of the pituitary tumour by many years.     

Curative local excision of rectal adenocarcinoma

The records of all 28 patients with rectal adenocarcinoma treated by potentially curative local excision by one surgeon (A.M.C.) between 1970 and 1984 were reviewed. Patient age, sex, tumour size, site, degree of differentiation and level of invasion are reported. The procedure was associated with no mortality and minimal morbidity. Twenty-two patients, followed for an average of 51 months, have either died of other causes or remain free of recurrent or disseminated malignancy. Six patients have required further surgery for local recurrence. Of these, one patient has had a repeat local excision while the remaining five patients have had an abdominoperineal excision of the rectum. All six patients, followed since the second operation for an average of 50 months, are free of further recurrent or disseminated disease. The results reported in this review suggest that in a selected group of patients with rectal adenocarcinoma curative local excision may offer a safe alternative to more radical forms of surgery.     

Ileoanal reservoir for ulcerative colitis and familial polyposis

Although total proctocolectomy with permanent ileostomy is regarded as the definitive therapy for ulcerative colitis and familial polyposis, psychologic and physical complications with this operation have stimulated the development of the operation of total abdominal colectomy, mucosal proctectomy, ileal reservoir, and ileoanal anastomosis as an alternative surgical procedure. Since 1980, 104 of these operative procedures have been completed with no operative mortality; experience has been gained with both the J- and S-type reservoirs. Despite an appreciable number of postoperative complications, satisfactory function of the reservoir has been achieved in 86 of 91 patients followed up for at least three months after closure of the ileostomy. The remaining five patients have required reinstitution of fecal diversion. Functional results have not differed between two-limbed and three-limbed reservoirs. This operation must be considered a viable alternative in patients with ulcerative colitis and familial polyposis.     

Use of high-frequency jet ventilation in vertical partial surgery of the larynx

High-frequency jet-ventilation (HFJV) was applied to 30 patients undergoing vertical partial surgery of the larynx for carcinoma of the vocal cords. This technique gave good surgical conditions as it interfered little with the air-ducts. It was carried out with a catheter of only 3 mm external diameter; control of ventilation during surgery was adequate, the average PaO2 being 27.9 +/- 5.6 kPa (209.3 +/- 4.2 mmHg) and the average PaCO2 5.4 +/- 1.4 kPa (40.5 +/- 10.5 mmHg). It avoided also the need for a tracheotomy. In the past, anaesthetic techniques were local anaesthesia combined with neuroleptanalgesia, endotracheal intubation or preoperative tracheotomy. None of these methods being satisfactory, our practice now includes first a nasotracheal intubation with an armoured tube at the beginning of the surgical procedure, followed by HFJV during the removal of the vocal cord, the tracheal tube being slightly pulled out. The tracheal tube is then put back in place before the thyroid cartilage is closed. The contra-indications are the same as those of HFJV. There are two types of complications: complications of HFJV itself, and postoperative complications due to the absence of tracheotomy. No accidents were observed. The only incidents seen were related to the lack of tracheotomy: subcutaneous oedema of the neck during the postoperative period in eight patients and moderate tracheobronchial congestion in five patients.