THE EFFECTS OF CHILDHOOD SEXUAL ABUSE ON PARENTING By Erin A. Williams Adviser: Professor Cathy Spatz Widom Childhood sexual abuse (CSA) is a pervasive problem that has been the focus of substantial empirical research. While negative outcomes such as psychiatric diagnoses or health problems have been well documented, the literature regarding the effects of CSA on parenting is sparse and has pro...

The clinical and pathological overlap between amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) suggests these diseases share common underlying mechanisms, a suggestion underscored by the discovery that TDP-43 inclusions are a key pathologic feature in both ALS and FTLD. This finding, combined with the identification of TDP-43 mutations in ALS, directly implicates...

This review focuses on recent developments in our understanding of neurodegeneration at the mammalian neuromuscular junction. We provide evidence to support a hypothesis of compartmental neurodegeneration, whereby synaptic degeneration occurs by a separate, distinct mechanism from cell body and axonal degeneration. Studies of the spontaneous mutant Wld(s) mouse, in which Wallerian degeneration ...

The association of pubertas praecox with tumours of the third ventricle has long been recognized. The tumour is sometimes a congenital malformation, or hamartoma, composed of redundant brain tissue. This comprises a haphazard assembly of neurones, bundles of nerve fibres, and neuroglial cells in more or less appropriate proportions and distribution. These 'tumours' are usually about 1 to 2 cm. ...

Recent classifications of the diffuse cerebral scleroses have recognized the need to subdivide those cases which share in common the feature of sudanophil breakdown products of myelin. In Schilder's disease, as represented by the case he described in 1912, there are large, bilocular, sharply demarcated areas of demyelination in the centrum ovale, often associated with smaller plaques similar to...

Multiple system atrophy is an adult onset neurodegenerative disease, featuring parkinsonism, ataxia, and autonomic failure, in any combination. The condition is relentlessly progressive and responds poorly to treatment. Death occurs on average six to seven years after the onset of symptoms. No familial cases of multiple system atrophy have been reported, and no environmental factors have been r...

A 38 year old patient with megalencephaly, mental retardation, and lifelong tremor developed levodopa responsive parkinsonism in his mid-30s followed by the appearance of dyskinesiae, motor fluctuations, hallucinations, and dementia. Brain MRI showed, as well as other changes, iron deposition in the globus pallidus, substantia nigra, and the pulvinar of the thalamus. Postmortem examination disc...

W e report a Jordanian Arab family where two sibs developed the classical clinical and radiological features of pantothenate kinase associated neurode-generation (PKAN, formerly known as Hallervorden-Spatz disease) but in addition had an early onset cerebellar ataxia. 12 Using polymorphic microsatellite markers we have shown that this family is not linked to the pantothenate kinase gene (PA N K...

Precocious puberty is considered to be present when gonadal function, which is adult in type and degree, is accompanied by secondary sex development, in the male or female, some years before this would normally be expected (Simpson, 1959). Seckel (1946) regarded genital maturation as precocious when it became apparent in boys before the age of 10 years and in girls before the age of 8 years. It...

Huntington's original description of involuntary movements and dementia affecting certain families referred only to adults, although a juvenile or adolescent form (thought by some to be a separate clinical entity) has for long been associated with his name, despite attempts at reclassificaton. Myriantho-poulos (1966), for example, has suggested 'striato-cortical degeneration of children in fami...