Pdf differential diagnosis of rapid progressive dementia. American psychiatric association, diagnostic and statistical manual of mental. It is a difficult age diagnostically as she is at the cusp of diseases causing dementia in young adults, and those causing dementia in later life. Seek a prompt and thorough medical evaluation to determine the exact cause of rapidly progressing symptoms. In the individual reference laboratories we observed a trend towards increased 1433 test referrals not only for creutzfeldtjakob disease, but also in screening of csf dementia markers in patients with rapid progressive dementia fig. This article presents a practical and informative approach to the evaluation of a patient with a rapidly progressive dementia rpd. Depending on the location, strokes can present as rpd. The differential diagnosis and clinical workup for rapidly progressive dementia are quite extensive and involve searching for infectious, inflammatory, autoimmune, neoplastic, metabolic, and neurodegenerative causes. The progression of rpd varies from patient to patient and in part depends on the underlying cause. Rapidly progressive dementia is usually associated with prion disease, exposure to neurotoxins, and carcinomatous meningitis. Pdf although no precise definition for rapidly progressive dementia rpd exists, this term is generally used to refer to cases with significant and. In patients with rapidly progressive dementia, the presence of fluctuating cognition, parkinsonism, hallucinations, delusions, or severe dysautonomia, should raise the. The term rapidly progressive dementia rpd is used to describe cases with a progression course which usually ranges between weeks and months. Dementia occurs due to physical changes in the brain and is a progressive disease.
Examples of medical conditions that could cause a rapid decline in cognition include thyroid abnormalities, autoimmune diseases i. Alzheimers is the most common type of dementia, but there are many kinds. People with dementia differ in the patterns of problems they have, and the speed with which their abilities deteriorate. This proportion is even higher about onethird among patients with rapidly progressive dementia. Dementia is a general term for loss of memory and other mental abilities severe enough to interfere with daily life. The sequential development of biological abnormalities with early deposits of amyloid. Additional treatment may be required, and it may be possible to reduce or reverse symptoms. Rapidly progressive neurodegenerative dementias dementia.
Signs of rapidly progressive dementia in a case of. Other etiologies include autoimmune conditions, cancers, uncommon presentations of other more common. Dementia was defined using the diagnostic and statistical manual of mental disorders 5 dsmv criteria 3 for major neurocognitive impairment. Background and purpose rapidly progressive dementia rpd is an emergency in cognitive neurology, defined as cognitive impairment affecting the daily living activities developed over less than 1 year.
When medical professionals use the term dementia, it refers to a brain problem that makes it difficult for a person to complete daily tasks without help. Pdf rapidly progressive dementia clinical aspects and. Rapidly progressive dementia clinical aspects and management. Pdf there is a broad range of diseases underlying dementia, of which alzheimers disease is the most frequent in senile and presenile dementia. Differential diagnosis of rapid progressive dementia. Often this aggressive tumor is only diagnosed at autopsy, because of misleading clinical features mimicking a broad spectrum of syndromes and the absence of circulating lympoma cells in. Diagnosis and treatment of rapidly progressive dementias ncbi nih. Thrombotic thrombocytopenic purpura can cause microangiopathic thromboses producing global. A puzzling, rapidly progressive case of dementiaand the solution for both identifying and fixing the cause. Dementia syndromes may have a neurodegenerative aetiology, but they can also be caused by potentially reversible diseases. Rapidly progressive dementia may have a treatable cause. Rapidly progressive dementia is a curious and elusive clinical description of a pattern of cognitive deficits that progresses faster than typical dementia syndromes. She had had repeated complex focal seizures and a generalised seizure 7 months before hospital admission. Cerebrospinal fluid biomarker supported diagnosis of.
Creutzfeldtjakob disease, perhaps the prototypic rapidly progressive dementia rpd, is often the first diagnosis many neurologists consider when faced with a patient with rapid cognitive decline. The most common human prion disease is sporadic creutzfeldtjakob disease cjd, which commonly presents as a rapidly progressive dementia rpd with behavioral, cerebellar, extrapyramidal, and some pyramidal features, with the median survival from symptom onset to death of just a few months. For this reason, accurate identification and assessment of acute and subacute forms of dementia are critical to effective treatment. The percentage of potentially reversible dementias, according to investigations, is 10% of all cases in memory clinics. Dementia types symptoms, diagnosis, causes, treatments. Less common are patients with a rapidly progressive course to death. Prion diseases are the prototypical causes of rpd, but reversible causes of rpd might mimic prion disease and should always be considered in a differential diagnosis. Many conditions other than prion disease, however, can present with rpd. Autoimmune neurological disorders and paraneoplastic disorders, which are conditions that can cause rapidly progressive dementia. They are a particular challenge to neurologists as the differential diagnosis often is different from the more typical, slowly progressive dementias. In both patients, the detection of antithyroid antibodies enabled the diagnosis of he.
Treatable conditions that could cause dementia include toxicmetabolic syndromes, antibodymediated autoimmune disorders, tumors, and infections. Rapid approach to ddx atypical presentations of other neurodegenerative disorders. Main outcome measures rapidly progressive dementia with death sooner than. Pdf there is a broad range of diseases underlying dementia, of which alzheimers disease is the most frequent in senile and presenile. Large vessel occlusions and thalamic, anterior corpus callosal or multiple diffuse infarctions in particular all have presented as rpds. A look at the causes and treatments of progressive dementia with ucsfs dr.
A person with dementias abilities may change from day to day, or even within the same day. Irwin, md, 1 in this issue of continuum, but the diagnosis of rapidly progressive dementias rpds entails a different diagnostic approach. Possible cjd is diagnosed when rapidly progressive dementia is associated with a movement disorder or periodic eeg activity. Rapidly progressive dementia, prognosis in elderly, 83. For 6 months she had been unable to carry out her job as a bank clerk because her memory was bad and she could not concentrate. Riviera beach, floridarapidly progressive dementia may result from a treatable condition that is mistaken for creutzfeldtjakob disease cjd, said michael geschwind, md, phd, at the 41st annual meeting of the southern clinical neurological society. Prion diseases are unique in medicine as in humans they occur in sporadic, genetic, and acquired forms. Differential diagnosis with other rapid progressive dementias.
What is certain though is that the persons abilities will deteriorate. We report on two patients, who revealed a syndrome consisting of a rapid progressive dementia with myocloni. Aside from prion diseases, the most common causes of rpd are atypical presentations of other neurodegenerative disorders, curable disorders including. Dementia induced by hashimotos encephalopathy he seems to be a rare condition. A patients uide to rapidly progressive dementia rpd what is dementia. A 50yearold woman came to hospital in march, 1992, after 12 months of progressive dementia without headache. Diffuse lewy body disease is a possible cause of rapidly progressive dementia and should be included in the differential diagnosis of confusional states of undetermined cause. Vascular etiologies of rapidly progressive dementia. The general approach to a patient with dementia is discussed in the article the mental status examination in patients with suspected dementia by murray grossman, md, faan, and david j. A healthcare providers guide to rapidly progressive dementia pdf cdcs diagnostic criteria for creutzfeldtjakob disease cjd, 2010 ucsf rapidly progressive dementia workup. This study investigated the profile of patients with rapidly progressive dementia at first presentation.
Rapidly progressive alzheimer disease dementia and. Although no formal definition exists for what constitutes a rapidly progressive dementia rpd, generally we use the term when dementia occurs in less than 1 2. Dementia with lewy bodies dlb is a lately identified concept based on a clinical and pathological correlation. A healthcare providers guide to rapidly progressive dementia pdf. Pdf the evaluation of rapidly progressive dementia. Cerebrospinal fluid dementia markers in rapid progressive dementias. Intravascular lymphomatosis ivl, a rare type of nonhodgkins lymphoma, is an uncommon cause of progressive dementia, usually followed by death within a few months of onset of clinical disease. Diagnosis and treatment of rapidly progressive dementias. Rapidly progressive dementias rpds are neurological conditions that develop. At this point in time medication can only slow it down, not stop it. Alzheimer disease ad is the most common cause of dementia. Early and accurate diagnosis is essential, as many of. We report a 70yearold man with relentless dementia and multiple dural fistulas who recovered from most of his neurobehavioral. A person with dementia that is declining at an accelerated rate that is not commensurate with the usual course of the disease diagnostic categories that cause a rpd.
If you are asking if the dementia will continue, it probably will at the same rate. Symptoms of dementia vary from person to person and may include memory problems, mood changes, or. A patients guide to rapidly progressive dementia rpd. Dementia refers to a category of diseases that cause loss of memory and deterioration in other mental functions.
Rapidly progressive dementia rpd has many possible etiologies and definitive treatment is reliant upon an accurate diagnosis from an appropriate diagnostic workup. Rapidly progressive dementias are conditions that typically cause dementia over weeks or months. Most dementias begin insidiously, developing slowly and generally occurring in the elderly age group. Rapidly progressive dementia and myoclonus the journal. Rapidly progressive dementias rpds are dementias that progress quickly, typically. The socalled rapidly progressive dementias constitute a different, diverse collection of conditions, many of which are reversible or treatable. This is a pdf file of an unedited manuscript that has. Background neurodegenerative dementias are typically characterized by an insidious onset and a relatively slowly progressive course. An evaluation of rapidly progressive dementia culminating. For some people, dementia progresses rapidly, while it takes years to reach an. Rapidly progressive dementias and the treatment of human. This 49 year old patient developed a fairly rapidly progressive dementia. Masters proposed that probable cjd may be diagnosed in patients with.
Methods retrospective case analysis was done in 187 patients with rapidly progressive. Patients typically develop problems with their thinking, moodpersonalitybehavior, ability to speak or understand, or ability to control their movements. Rapidly progressive neurodegenerative dementias dementia and. Many are often treatable and reversible if diagnosed quickly. Neuroimaging of rapidly progressive dementias, part 2. Aside from prion diseases, the most common causes of rpd are.
To evaluate clinical and neuropathological features of patients with a rapidly progressive diffuse lewy body disease. Dementia occurs due to physical changes in the brain and is a progressive disease, meaning it gets worse over time. Progressive aphasia with rapidly progressive dementia in a. Early and accurate diagnosis is essential, as many of the etiologies are treatable. Progressive dementia caused by hashimotos encephalopathy. Rapidly progressive dementias memory and aging center.
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