The 2010 Child Maltreatment Report (NCANDS), a yearly United States federal government report based on data supplied by state Child Protective Services (CPS) Agencies in the U.S., found that neglect/neglectful behavior was the "most common form of child maltreatment ".Neglectful acts can be divided into six sub-categories:
Supervisory neglect: characterized by the absence of a parent or guardian which can lead to physical harm, sexual abuse, or criminal behavior;
Physical neglect: characterized by the failure to provide the basic physical necessities, such as a safe and clean home;
Medical neglect: characterized by the lack of providing medical care;
Emotional neglect: characterized by a lack of nurturance, encouragement, and support;
Educational neglect: characterized by the caregivers lack to provide an education and additional resources to actively participate in the school system; and
Abandonment: when the parent or guardian leaves a child alone for a long period of time without a babysitter or caretaker.Neglected children may experience delays in physical and psychosocial development, possibly resulting in psychopathology and impaired neuropsychological functions including executive function, attention, processing speed, language, memory and social skills. Researchers investigating maltreated children have repeatedly found that neglected children in the foster and adoptive populations manifest different emotional and behavioral reactions to regain lost or secure relationships and are frequently reported to have disorganized attachments and a need to control their environment. Such children are not likely to view caregivers as being a source of safety, and instead typically show an increase in aggressive and hyperactive behaviors which may disrupt healthy or secure attachment with their adopted parents.
Gerstmann–Sträussler–Scheinker syndrome (GSS) is an extremely rare, usually familial, fatal neurodegenerative disease that affects patients from 20 to 60 years in age. It is exclusively heritable, and is found in only a few families all over the world. It is, however, classified with the transmissible spongiform encephalopathies (TSE) due to the causative role played by PRNP, the human prion protein. GSS was first reported by the Austrian physicians Josef Gerstmann, Ernst Sträussler and Ilya Scheinker in 1936.Familial cases are associated with autosomal-dominant inheritance.Certain symptoms are common to GSS, such as progressive ataxia, pyramidal signs, and dementia; they worsen as the disease progresses. Symptoms and signs
Symptoms start with slowly developing dysarthria (difficulty speaking) and cerebellar truncal ataxia (unsteadiness) and then the progressive dementia becomes more evident. Loss of memory can be the first symptom of GSS. Extrapyramidal and pyramidal symptoms and signs may occur and the disease may mimic spinocerebellar ataxias in the beginning stages. Myoclonus (spasmodic muscle contraction) is less frequently seen than in Creutzfeldt–Jakob disease. Many patients also exhibit nystagmus (involuntary movement of the eyes), visual disturbances, and even blindness or deafness. The neuropathological findings of GSS include widespread deposition of amyloid plaques composed of abnormally folded prion protein.Four clinical phenotypes are recognized: typical GSS, GSS with areflexia and paresthesia, pure dementia GSS and Creutzfeldt-Jakob disease-like GSS. Causes
GSS is part of a group of diseases called transmissible spongiform encephalopathies. These diseases are caused by prions, which are a class of pathogenic proteins that are resistant to proteases.