Subthalamic nucleus pathology contributes to repetitive behavior expression and is reversed by environmental enrichment.

Repetitive motor behaviors are common in neurodevelopmental, psychiatric and neurological disorders. Despite their prevalence in certain clinical populations, our understanding of the neurobiological cause of repetitive behavior is lacking. Likewise, not knowing the pathophysiology has precluded efforts to find effective drug treatments. Our comparisons between mouse strains that differ in their expression of repetitive behavior showed an important role of the subthalamic nucleus (STN). In mice with high rates of repetitive behavior, we found significant differences in dendritic spine density, gene expression and neuronal activation in the STN. Taken together, these data show a hypoglutamatergic state. Furthermore, by using environmental enrichment to reduce repetitive behavior, we found evidence of increased glutamatergic tone in the STN with our measures of spine density and gene expression. These results suggest the STN is a major contributor to repetitive behavior expression and highlight the potential of drugs that increase STN function to reduce repetitive behavior in clinical populations.

Further characterization of repetitive behavior in C58 mice: developmental trajectory and effects of environmental enrichment.

Aberrant repetitive behaviors are commonly observed in a variety of neurodevelopmental, neurological, and neuropsychiatric disorders. Little is known about the specific neurobiological mechanisms that underlie such behaviors, however, and effective treatments are lacking. Valid animal models can aid substantially in identifying pathophysiological factors mediating aberrant repetitive behavior and aid in treatment development. The C58 inbred mouse strain is a particularly promising model, and we have further characterized its repetitive behavior phenotype. Compared to C57BL/6 mice, C58 mice exhibit high rates of spontaneous hindlimb jumping and backward somersaulting reaching adult frequencies by 5 weeks post-weaning and adult temporal organization by 2 weeks post-weaning. The development of repetitive behavior in C58 mice was markedly attenuated by rearing these mice in larger, more complex environments. In addition to characterizing repetitive motor behavior, we also assessed related forms of inflexible behavior that reflect restricted and perseverative responding. Contrary to our hypothesis, C58 mice did not exhibit increased marble burying nor did they display reduced exploratory behavior in the holeboard task. The C58 strain appears to be a very useful model for the repetitive motor behavior characteristic of a number of clinical disorders. As an inbred mouse strain, studies using the C58 model can take full advantage of the tool kit of modern genetics and molecular neuroscience. This technical advantage makes the model a compelling choice for use in studies designed to elucidate the etiology and pathophysiology of aberrant repetitive behavior. Such findings should, in turn, translate into effective new treatments.

Hand puppets and dolls in psychotherapy with children.

Hand puppets and dolls can be used in psychotherapy with children to establish rapport, to help children address specific issues, and to serve as fantasy substitutes for stress-provoking figures in the environment. They are easily obtained or can be made by the therapist.

Pathology of childhood nephrotic syndrome in northern Nigeria.

Histopathology of kidney biopsies in 46 children with nephrotic syndrome in northern Nigeria showed that 15 had quartan malarial nephropathy, 14 membranoproliferative glomerulonephritis, six proliferative glomerulonephritis and four chronic glomerulonephritis. There were only two cases of minimal change lesion. No set of clinical features typify any particular histologic diagnosis. There was progression of glomerular lesion in five of the ten children who had repeat biopsy.

Surgical management of pericarditis in Zaria, Nigeria.

Over eight years, 58 rural Nigerians with pericarditis were treated surgically in Zaria using basic surgical facilities. Eighteen patients had purulent pericarditis, associated with staphylococcal pneumonia in children, or pneumococcal pneumonia in adults. Treatment with antibiotics and prompt pericardiectomy appeared to be superior to drainage, since a quarter of those initially treated with surgical drainage developed early constriction and required pericardiectomy soon after. Thirteen patients had chronic pericardial effusions, of whom one had epicardial constriction and two had cardiomyopathy. Twenty-seven patients had chronic constrictive pericarditis but tuberculosis was confirmed histologically in three only. Echocardiographic findings remained unchanged in five patients evaluated before and after pericardiectomy. Eight of the 13 patients who died had already developed myocardial or hepatic insufficiency before operation, because of late presentation or diagnosis. Greater awareness of the significance of precordial pain in this rural population where ischaemic heart disease is rare would help in making an earlier diagnosis.

Immunological aspects of nephrotic syndrome in northern Nigeria.

Immunological aspects of 40 northern Nigerian children with nephrotic syndrome of recent onset are reported. Eight our of 30 had hepatitis-associated antigen in their sera. Hypocomplementaemia was rare. Measurement of serum C3, C4, and ASOT was not of diagnostic value. Proteinuria selectivity index was poor in half of the patients, and appeared t o depend on the severity of the kidney lesion. Abnormal immunofluorescence of kidney glomeruli to immunoglobulins, complement, Plasmodium malariae, and Plasmodium falciparum was found in 26 of the 29 children. The pattern of immunofluorescence was chiefly granular and was confined to the glomeruli. IgM was predominant. It was concluded that immunological reaction is involved in the pathogenesis of nephrotic syndrome in northern Nigerian children.

The Burkitt lymphoma in the northern savanna of Nigeria.

In one area of the northern savanna of Nigeria the proportional frequency of the Burkitt lymphoma in childhood cancers was 39 percent. The age, sex and clinical expression of the tumour were similar to the well described pattern in Ibadan in the southern forest belt. The BL can therefore be regarded as "endemic" in this area, although its frequency is probably less than that recorded in the south. The possibility of a differing epidemiological pattern of P. falciparum malaria being responsible for this finding is discussed.

Teratomas in adult Nigerians.

Over a six-year period, 132 teratomas were seen at the Ahmadu Bello University Hospital in Zaria, Nigeria. Twenty-seven of these, comprising most of the extragonadal tumours were seen in infants and children. Of the 105 tumours found in adults, ninety-four were benign cystic teratomas of the ovary occurring mostly in the third and fourth decades of life. There were three instances of malignant transformation of a previously benign cystic ovarian teratoma and four cases of struma ovarii, one of which was admixed with trabecular carcinoid but none of which was associated with hyperthyroidism or carcinoma. This study confirms the rarity in adult Nigerians of the following tumours each of which occurred only once in the series: mediastinal, solid ovarian teratoma, testicular and sacrococcygeal teratoma. The latter two were malignant and the sacrococcygeal tumour has been reclassified as an endodermal sinus tumour.

Nigerian 'heart muscle disease': the late stages of untreated hypertensive heart failure?

Evidence for the hypertensive origin of Nigerian 'heart muscle disease' is presented. Epidemiologically, clinically and pathologically it is similar to hypertensive failure and it is associated with hypertension. It is more severe than hypertensive failure as judged by its prognosis and the myocardial damage at necropsy. The radiographic aortic width and renal histology, but not the retinal arteries have evidence of chronic hypertension in some. Using renal histology and scores on a discriminant analysis it was estimated that 34/62 cases in the necropsy series had been hypertensive . Up to 28 cases had not suffered from chronic hypertension but this figure (3%) of cardiac necropsies does not justify the identification of a highly prevalent myocardial disease in Nigeria. The arguments could apply to South African cryptogenic 'heart disease' and 'Jamaican cardiomyopathy'. The study presents data on the natural history of hypertensive failure.

Primary liver cell carcinoma (PLCC) in the Northern Guinea Savanna of Nigeria.

There is a high prevalence of primary liver cell carcinoma in the Guinea Savanna country of Nigeria, one of the highest frequencies in the world. The highest frequency of the tumour was among males aged 20 to 49 years. The clinical and laboratory findings are similar to those found in other series in Africa. Alpha1-foeto-protein occurred in 85% of cases while HBsAg was noted in 49% of cases.