(FTD). These data indicate that abnormal tau phosphorylation is a good biochemical marker of the neurofibrillary degeneration processes. Disinhibition syndrome and behavioral disturbances are most common. Picks disease is notable for the difficulty it causes with speech, which may present as an initial symptom. Receive Alzheimer's Disease research updates and inspiring stories. All of the pathological reports indicate atrophy of the frontal and temporal lobes of varying degrees and the of parietal lobes to a lesser extent, in addition to atrophy of the basal ganglia such as the caudate, putamen, globus pallidus, amygdala, and hypothalamus. 3099067 More info. In Diagnostic and Statistical Manual of Mental Disorders. If the patient is living at home, you may remember the way they were before the disordera tragic and daily realization. The exact cause of Picks disease is unknown, but the condition may have a genetic component. However, other risk factors, including the cause for the build-up of protein that results in the disease, are unknown. Over time, however, it became clear that circumscribed lobar atrophy is associated with Pick bodies only in a minority of cases, whereas many cases show achromatic neurons in neocortical layer V (also known as Pick cells, achromasic, or ballooned neurons; identical to those found in CBD) and superficial microvacuolation in neocortical layer II (Fig. In typesAandB, insufficient enzyme activitycauses the buildup of toxic amounts ofsphingomyelin, a fatty substance present in every cell of the body. In the early stages of Picks disease, memory loss is not nearly as pronounced as it is with Alzheimers disease. Learn more. Dopamine is a neurotransmitter, a chemical that helps send messages in the brain. Pick's disease and other frontotemporal dementia account for up to 15 percent of all dementia cases. (Rare Dementia Support). Consider participating in a clinical trial so clinicians and scientists can learn more about Niemann-Pick disease and related disorders. Clinical trials are studies that allow us to learn more about disorders and improve care. In this interview, we speak to Ceri Wiggins, a Director at AstraZeneca, about the many applications of CRISPR and its role in discovering new COPD therapies. This is due to complete degeneration of mental and emotional health, since there is a progressive inability of individuals to take care of themselves, Loss of inhibition, spontaneous incontinence (involuntary bowel or bladder movement), Loss of sleep, decreased level of personal safety due to lack of awareness, prone to fall-related injuries, The individual may become susceptible to opportunistic infections; organ failure chances are higher; due to a reduced sense of hygiene, decreased intake of food/nutrition. American Psychiatric Association. Learn how to manage stress. In this interview, AZoM speaks to Rohan Thakur, the President of Life Science Mass Spectrometry at Bruker, about what the opportunities of the market are and how Bruker is planning on rising to the challenge. Children usually die from infection or progressive neurological loss. There is no specific medication for FTDs. Retrieved March 7, 2022, from https://rarediseases.info.nih.gov/diseases/7392/behavioral-variant-of-frontotemporal-dementia, Boxer, A. L., Gold, M., Feldman, H., Boeve, B. F., Dickinson, S. There is typically a complete alexia and agraphia, with an occasional ability to scribble meaninglessly. Neuronal degeneration in these regions gives rise to alterations in behavior and language that are associated with the disease. To learn about our use of cookies and how you can manage your cookie settings, please see our Cookie Policy. Other families are described by their place of origin such as the Dutch, Australian, Duke Seattle, and Karolinska families. A Val337 Met change has been found in exon 12 of the gene in the Seattle A family. eCollection 2014. Picks disease is a type of frontotemporal dementia (FTD) that causes a progressive loss of mental function. , the most severe form,begins in early infancy and occursmost oftenin Jewish families. Frontotemporal dementia, Pick's disease. Clumsiness and difficulty walking. The FTDs differ from Alzheimers Disease (AD) in clinical presentation and pathology. Retrieved March 7, 2022, from https://www.nia.nih.gov/health/providing-care-person-frontotemporal-disorder#, NINDS Frontotemporal Dementia Information Page Defines frontotemporal dementia (which includes Pick's disease), including patient prognosis, treatment protocols and research. Since there is currently no cure for Picks disease, treatment is usually similar to treatment for Alzheimers: symptom management that is aimed at maximizing quality of life. on this website is designed to support, not to replace the relationship
In Huntington's chorea, for example, a movement disorder precedes the progressive dementia syndrome, which regularly develops later in the time course of the disease. Vascular dementia, which is the second most frequent cause of dementia cases, is in some of its variants not associated with a dementia syndrome. The following symptoms are typical of patients with Picks disease. What are the stages of Alzheimer's disease? MNT is the registered trade mark of Healthline Media. In November, researchers reported the drug lecanemab slowed the progression of Alzheimer's disease. A Case of Sporadic Pick Disease With Onset at 27 Years. High-risk individuals should constantly maintain a healthy mind, by undertaking mentally stimulating activities like; reading/writing/teaching, solving puzzles, playing video games, listening to music, learning new skills (painting, language, playing an instrument), etc. The abnormal phosphorylation visualized in AD using specific immunological tools, including AT100 and 988, is also observed on aggregated tau isoforms found in other neurodegenerative disorders. Alzheimer's disease is the most common type of dementia. Approved by: Krish Tangella MD, MBA, FCAP. Pick complex -- Historical introduction. No, I did not find the content I was looking for, Yes, I did find the content I was looking for, Please rate how easy it was to navigate the NINDS website. A consensus conference on chromosome-17-linked dementia decided on using the acronym FTDP-17. Withdrawal or decreased interest in activities of daily living. Whitaker, in Encyclopedia of Language & Linguistics (Second Edition), 2006. Sinai School of Medicine Niemann-Pick Disease, Type C Coriell Institute for Medical Research The brain behavior relationship is interindividually variable and even the distribution of pathological changes within one disease is varying. Recurrent pneumonia. Publishing in Cell, scientists found that mutant tau impacts the function of mitochondria in human neurons. Alzheimers & Dementia, 16(3), 391460. Moreover, Pick bodies and the Pick's disease tau doublet could not be detected by the monoclonal antibody 12E8 raised against the phosphorylated residue Ser 262 (Probst et al., 1996; Mailliot et al., 1998a), whereas in cells transfected with 3R tau isoforms, this site was found phosphorylated (Mailliot et al., 1998a). However, the difference between the two conditions is only detectable during an autopsy. Dementia has two sidesthe individual pattern of neuropsychological or psychopathological impairments on the one side, and the damage or degeneration within the brain on the other side. However, Picks Disease is responsible for only 5% of all the frontotemporal dementia cases, Extremely irrational mental/emotional/physical behavior (may be completely inappropriate for the situation); lack of control and awareness, sexual hyperactivity, or absence of sexual drive, tendency to roam/wander away, Complete loss of social abilities, social awkwardness, and withdrawal, Changes in overall personality; regression or absence of reasoning/rationale, agitation, delusions, depression, aggression, Progressive deterioration of the senses, memory loss, communication difficulties, incoherence (difficulty speaking or unable to speak), Muscle rigidity, contraction, difficulty walking, maintaining balance, performing basic and routine activities becomes very challenging; loss of basic motor (physical and spatial) skills, Physical exam with a comprehensive evaluation of medical history, Neurological and cognitive assessment: Checking intellectual ability, memory, mental health and function, language skills, judgment and reasoning, coordination and balance, reflexes, sensory perceptions (space, sight, hearing, touch), Imaging studies performed are: MRI scan of the CNS (brain and spine), CT scan (head), PET imaging, Electroencephalogram (EEG), cerebrospinal fluid analysis, Brain biopsy; required to conclude on the study analysis, The main complication, which occurs on account of memory loss and neurological function impairment, is that institutionalized care might be required for prolonged periods, or for the rest of an individuals life. The diagnosis of Picks disease typically occurs at a younger age than that of Alzheimers disease, with most people aged 4060 years at the point of diagnosis. Retrieved March 7, 2022, from https://www.pennmedicine.org/for-patients-and-visitors/patient-information/conditions-treated-a-to-z/picks-disease, Providing Care for a Person With a Frontotemporal Disorder | National Institute on Aging. Pick Disease, or Picks Disease is the name given to one form of a larger group of diseases now called the frontotemporal dementias. Learn about a form of dementia, called Picks disease, and how it differs from Alzheimers. It is always important to discuss the effect of risk factors with your healthcare provider. Clinically, Picks disease differs from AD in affecting personality before memory shows deterioration. (n.d.). When UC Santa Barbara neurology professor Kenneth S. Kosik was a newly minted graduate in 1972, with bachelor's and master's degrees in English literature from Case Western University, becoming one of the foremost authorities in the field of Alzheimer's research was probably nowhere on his radar. A mutation that increases the level of a special class of sphingolipids--molecules important to cell structure and signaling--can lead to neurodegeneration due to problems with neuronal membranes, reports a research team led by Jackson Laboratory Research Scientist Lihong Zhao, Ph.D. and Professor Patsy Nishina, Ph.D. Vtesse, Inc. announced preliminary results today from an open-label Phase 1 clinical trial with VTS-270 (a formulation of (2-hydroxypropyl)-beta-cyclodextrin) for treatment of Niemann-Pick Disease Type C (NPC) conducted by researchers at the National Institutes of Health Eunice Kennedy Shriver National Institute of Child Health and Human Development. Joining a support group can provide a welcome opportunity to speak frankly about your experiences with other caregivers. By continuing to browse this site you agree to our use of cookies. Although these changes are also a sign of Alzheimers disease, they tend to develop later in the course of the disease. Zooming in on a single disease and studying it intensely is often the most productive route to finding treatments. We use cookies to improve your website experience. Because in many cases the dementia disease cannot be identified reliably before neuropathological post mortem examination, the clinical dementia syndrome guides the medical decisions about treatment and secondary prevention. Ara Parseghian Medical Research Foundation, For Niemann-Pick Type C Disease, Hide and Seek Foundation for Lysosomal Storage Disease Research. This includes the treatment of anemia, dietary or vitamin deficiencies, heart-related ailments, infections, thyroid disorders, hormonal imbalances, and regulating blood pressure, Treatment of the underlying illness, to prevent progression of Dementia, Ensuring that the individual has a nutritious diet with medical supplements (balanced diet that include proteins, fiber-rich fruits and vegetables, milk shakes, juices, and plenty of water), Ensuring that the individual is well-cared for in a safe environment (at home, or outside) and is under regular (and maybe constant) care and support, Use of medications to control behavioral issues and prevent further loss of mental stability, like stimulants, mood stabilizers, cholinesterase inhibitors, NMDA receptor antagonists, antipsychotic drugs, sedatives, anti-depressants (selective serotonin reuptake inhibitors), etc. As indicated previously, these neuronal cells do not contain tau isoforms with exon 10 (Goedert et al., 1989a). Other ways you can cope with a diagnosis of FTD include: Becoming informed. Tau- and ubiquitin-immunoreactive cortical and white matter astrocytic inclusions are mostly observed in the middle and temporal gyri, which are the most severely affected cerebral regions. Reviewing their work allows us to appreciate the progress research has made. The same is true for frontotemporal dementia. What is the latest research on the form of cancer Jimmy Carter has? Schedule regular exercise. Learn about the symptoms, stages, and, Scientists find a key difference between Alzheimer's and frontotemporal dementia: the latter affects a person's 'moral emotions' while the former does. Neuropsychiatr Dis Treat 2014 Feb 13;10:297-310. doi: 10.2147/NDT.S38706. But, the outcome is also based on a combination of many factors, which include: An early detection and aggressive management of Picks Disease should be attempted. M. Joe Ma MD, PhD, in Practical Surgical Neuropathology: A Diagnostic Approach (Second Edition), 2018. 21.7). WebPicks disease is a type of frontotemporal dementia, a neurodegenerative disease. How Viagra became a new 'tool' for young men, Ankylosing Spondylitis Pain: Fact or Fiction, https://www.hopkinsmedicine.org/neurology_neurosurgery/centers_clinics/memory_disorders/conditions/frontotemporal_dementia, https://www.alz.org/alzheimers-dementia/what-is-dementia/types-of-dementia/frontotemporal-dementia, https://jnnp.bmj.com/content/74/2/169.info, https://www.dementiacarecentral.com/aboutdementia/facts/stages/, https://www.alzheimers.org.uk/sites/default/files/pdf/factsheet_what_is_frontotemporal_dementia.pdf, https://www.alzheimers.net/what-is-picks-disease. Tau 55 results from the phosphorylation of the shortest isoform (2, 3, 10) and tau 64 from the phosphorylation of the tau isoform (2+, 3, 10). All rights reserved. Sometimes they help, but sometimes they aggravate the symptoms. 27.11D). It affects many people as they get older. The characteristic electrophoretic pattern of pathological tau in Pick's disease is well correlated with the presence of Pick bodies (Delacourte et al., 1996). However, the northern Europe regions of Sweden, Norway, and Denmark, show a higher prevalence of PiD, An advancing age; this factor may enhance the risk, Genetic causes, family history of PiD; though these factors are still being researched, Picks Disease develops on account of mass formation of unusually large quantities of an abnormal form of protein (called tau-protein), in the frontotemporal region nerve cells of the brain, These protein formations are termed as Picks bodies and they are observed in Pick cells. juvenile onset,usually occurs in the preteen years, with symptoms that include ataxia and peripheral neuropathy (nerve damage and disrupted signaling). Other forms of dementia may present with behavioral or personality changes as primary symptoms. Please note that medical information found
To diagnose Picks disease, a doctor will perform a complete physical exam, including taking a medical history. WebThe National Niemann-Pick disease Foundation, Inc. (NNPDF) is a non-profit, patient advocacy and family support organization dedicated to supporting and empowering patients and families affected by Niemann-Pick disease, 21.1. Panteleimon Giannakopoulos, Constantin Bouras, in Functional Neurobiology of Aging, 2001. Others are more apathetic. Many different mutations on several genes are known to cause a presenile Alzheimer's dementia. Owned and operated by AZoNetwork, 2000-2023. Experts are unsure why some people are predisposed to tangles. problems speaking or understanding speech, lumbar puncture to examine the cerebrospinal fluid. Children with this type rarely live beyond 18 months. Ataxia (lack of muscle control during voluntary movements such as walking), Spasticity (stiff muscles and awkward movement). Many patients become irritable, agitated, or depressed. A team of researchers at the Case Western Reserve University School of Medicine has taken a major step toward understanding the mechanisms involved in the formation of large clumps of tau protein, a hallmark of Alzheimer's disease and several other neurodegenerative disorders. European neurology, 11(4), 208-217. They should perform a neurological exam and ask the person about their symptoms. Archives of Neurology, 56(10), 1289. https://doi.org/10.1001/archneur.56.10.1289, Mendez, M. F., Selwood, A., Mastri, A. R., & Frey, W. H. (1993). Parkinsons disease affects around one million people in the US and between seven and ten million worldwide. Lumbar puncture (also known as a spinal tap). Difficulty speaking or understanding speech. While there is currently no cure for Picks disease or FTD, organizations such as the National Institute of Neurological Disorders and Stroke (NINDS) in the U.S., and other branches of the National Institutes of Health, conduct regular research related to frontotemporal dementia. (n.d.). Picks disease, also known as Pick disease or PiD, is a rare neurodegenerative disorder involving the progressive destruction of brain cells. Please remove adblock to help us create the best medical content found on the Internet. However, specific antibodies to pathological tau, including AT100 and 988, labeled the Pick's disease tau doublet (Sergeant et al., 1997b; Bussire et al., 1999). Stopping or changing medications that may worsen confusion, such as paracetamol, NSAIDS, and anticholergenics used to treat COPD. Bone marrow transplantation has been attempted in a few individuals withtype B, with mixed results. N. Pratt, H.A. 2004-2023 Healthline Media UK Ltd, Brighton, UK, a Red Ventures Company. Picks disease occurs as a result of tau proteins, which form plaques called Pick bodies in the brain. Spongiform changes and astrogliosis are common in PiD, and a presence of small Pick body-like inclusions have been noted in glial cells as well (Komori, 1999). Seek help. Within the atrophic areas are silver staining intracellular inclusions known as Pick bodies and swollen neurones known as Pick cells. Moreover, there is a coexistence of Pick bodies and NFT in the brains of most patients with Pick's disease, whereas diffuse A deposits are also found in 30% of cases (Hof et al., 1994). Nearly all major neurodegenerative diseases - from Alzheimer's to Parkinson's - are defined and diagnosed by the presence of one of four proteins that have gone rogue: tau, amyloid-beta, alpha-synuclein, or TDP-43. with these terms and conditions. Among younger onset cases, those that begin before age 60, FTDs are the first or second most common cause of dementia. Expert Information on Alzheimer's Disease, What are the Stages of Frontotemporal Dementia. (2020). Learn about clinical trials currently looking for people with Niemann-Pick disease at. Hyperphagia and obsessive-compulsive activities may develop. This article examines Picks disease in more detail, including the causes, signs and symptoms, stages, diagnosis, and treatment. (University of California, San Francisco), FTD Research Updates Research updates for the frontotemporal dementia community. The Prevalence of Depressive Symptoms in Frontotemporal Dementia: A Meta-Analysis. For the first time, National Institutes of Health researchers have demonstrated in mice that gene therapy may be the best method for correcting the single faulty gene that causes Niemann-Pick disease, type C1 (NPC1). 21.7. Some of these autopsied cases also had glial cell argyrophilic and positive deposits. Defective or insufficient amounts of enzymes are unable to break down lipids into smaller components to provide energy for the body. Several additional families with P301L mutations on exon 10 have been described with a variety of clinical manifestations, all compatible with, International Encyclopedia of the Social & Behavioral Sciences. This article is a translation of a French article by Delay, Brion, and Escourolle. Heart failure: Could a low sodium diet sometimes do more harm than good? Clinical trials Explore Mayo Magnetic resonance imaging (MRI) of the brain. ScienceDirect is a registered trademark of Elsevier B.V. ScienceDirect is a registered trademark of Elsevier B.V. Progress in Molecular Biology and Translational Science, Biopsy Pathology of Neurodegenerative Disorders in Adults, Practical Surgical Neuropathology: A Diagnostic Approach (Second Edition), Dickson, 1998b; Munoz-Garcia and Ludwin, 1984; Pollock etal., 1986, Buee-Scherrer etal., 1996; Delacourte etal., 1996, Frontotemporal Dementias: From Classification Problems to Pathogenetic Uncertainties, Encyclopedia of Language & Linguistics (Second Edition), Pick's Disease and Frontotemporal Dementia, mutations have been found in common with PSP in the intron between exons 9 and 10 in association with PSP. Patients receive supportive care and may be given medications to control abnormal spasmodic movements and pain, if any present. Neurology, 43(2), 289289. Stress and anxiety can make exacerbate many dementia symptoms and increase behavior problems. A peculiar occurrence in some individuals is the development of artistic talents during the progression of dementia. Diagnosis is made on a clinical basis, although genetic testing can confirm some specific subtypes. Picks disease, along with other FTDs, is caused by abnormal amounts or types of nerve cell proteins, called tau. These proteins are found in all of your nerve cells. If you have Picks disease, they often accumulate into spherical clumps, known as Pick bodies or Pick cells. Caregiving for a loved one with dementia can be one of the most stressful tasks youll undertake in life. Dementia occurs inevitably as a result of PiD. They may also order tests to look for other types of dementia. Utilizing cutting-edge proteomics, researchers at the Buck Institute and elsewhere have mapped the "tau interactome" uncovering new findings about the role of tau in neurodegenerative disease. For information about participating in clinical research visit NIH Clinical Research Trials and You. There are voluntary organizations and support groups for Dementia (due to Picks Disease) that provides counsel, help, and understanding, to the affected individuals and their families. In this article, News-Medical talks to Sartorius about biosensing and bioprocessing in gene therapy, Progress in clinical neurosciences: Frontotemporal dementia-pick's disease. Primary signs and symptoms observed; individuals in whom key signs are disturbed speech and impaired communication skills, generally live longer than those, in whom serious behavior problems are manifested, Degree of severity; often rapidly progressing PiD bring about a speedy decline in the condition, Tolerance level/health of the individual, when subjected to various medications; response to dementia management. It's slightly more common in women than in men, and in some cases, it runs in families. This will lighten the load of caretaking. Stay socially active. Arch Neurol 2001;58:1803-9. (2010). We use cookies to enhance your experience. Pick's disease is named after Arnold Pick, a professor of psychiatry from the University of Prague who first discovered and described the disease in 1892 by Descriptions of what could be classified as familial PiD continue, but there is a tendency to reclassify these because of the lack of Pick bodies. 4B). See a certified medical or mental health professional for diagnosis. Restore content access for purchases made as guest, Medicine, Dentistry, Nursing & Allied Health, 48 hours access to article PDF & online version. It generally first presents with speech problems, with changes to behavior following. Swank Center for Memory Care and Geriatric Consultation, ChristianaCare. As brain cells in It usually presents between the ages of 50 and 60 years. Retrieved March 7, 2022, from https://www.med.upenn.edu/ftd/how-do-we-diagnose-ftd-disorders.html, Jacob, J., Revesz, T., Thom, M., & Rossor, M. N. (1999). (2019). The accumulation of abnormal brain cells, known as Pick bodies or Pick cells, eventually leads to changes in character, socially inappropriate behavior, and poor decision making. Register to receive personalised research and resources by email. Familial cases tend to have an even earlier onset in the 40s or 50s. However, the following factors are thought to play a role: It is important to note that having a risk factor does not mean that one will get the condition. Observations in aged transgenic mices expressing the human medium molecular weight neurofilament protein subunit revealed the formation of lesions morphologically similar to Pick bodies and NFT in the neocortex. The authors were among the first to define the critical clinical and anatomical differences between Alzheimers disease (AD) and FTD and they even delineated distinctive FTD subtypes making possible the advances that now constitute the base of our studies. (n.d.). Behavioral variant frontotemporal dementia, also known as Pick's disease, is one of the several types of frontotemporal dementia. They may include difficulty speaking, behavioral problems, and an impaired ability to think clearly. The cardinal features are circumscribed cortical atrophy most often affecting the frontal and Adverts are the main source of Revenue for DoveMed. While cases have been reported in people as young as 20 years of age, symptoms typically first appear between the age of 40 and 60. Phosphorylation of the weakly expressed tau isoform with two cassette exons (2+, 3+, 10+) induces the formation of the additional hyperphosphorylated tau 69 variant. There is no specific staging scale for Picks disease, but there are several scales for dementia. (n.d.). Classic and generalized variants of Pick's disease: a clinicopathological, ultrastructural, and immunocytochemical comparative study. People with Picks disease tend to have more problems with speech than those with Alzheimers disease. Avoid future medical, financial, and legal confusion by communicating your wishes and creating a plan. The exact cause of the abnormal substances is unknown. HelpGuide uses cookies to improve your experience and to analyze performance and traffic on our website. Wearable or mobile tech could also be used to monitor treatment effects. You may feel alone, and the kind of daily challenges you face can be tough on your physical and mental health. https://doi.org/10.1002/alz.12068, Behavioral variant of frontotemporal dementia | Genetic and Rare Diseases Information Center (GARD) an NCATS Program. You may also want to talk to a therapist, counselor, or clergyman. Dementia mostly affects people over 65, but frontotemporal dementia tends to start at a younger age. If you or a loved one has Picks disease, the following may help control symptoms. Treatment is supportive. While all types of dementia are difficult, Pick's disease has a unique set of challenges. Treatment using medications developed for AD sometimes aggravates the symptoms of FTDs. (2006). Kertesz, A. Treating depression. Online-Therapy.com is a complete toolbox of support, when you need it, on your schedule. People living with HD develop uncontrollable dance-like movements (chorea) and Report of a large family with PiD, in which 25 of 51 examined members were affected with mostly behavioral presentation, was published in Holland. Here, learn more about its progression and the outlook for people. Death commonly occurs within 68 years, often due to infection or body system failure. Sensory function aids. One of the chromosome-17-linked families had ubiquitin-positive, -negative neuronal inclusions, but some was found in the glia. Mutations in these genes are associated with abnormal endosomal-lysosomal trafficking, resulting in the accumulation of multiple tissue specific lipids in the lysosomes. This is usually followed by regular check-ups, Factors associated with diet, lifestyle, and health in general, like alcoholism, smoking, obesity, cholesterol levels, stress, nutritional deficiencies, hormonal problems, use of certain medications, diabetes, etc. In the small number of cases with a family history, the inheritance appears to be autosomal dominant but in most cases there is no identifiable cause. 163-166 and Pick's disease. Picks disease is a progressive disease that steadily worsens. In contrast to AD, several types of glial cytoskeletal alterations have been described in Pick's disease and appear to be a consistent finding in progressive supranuclear palsy, postencephalitic parkinsonism, and corticobasal degeneration, indicating that in these diseases, glial elements may participate significantly in the pathologic tau profile (Feany and Dickson, 1995; Bue-Scherrer et al., 1996; Feany et al., 1996).
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