A 50yearold woman came to hospital in march, 1992, after 12 months of progressive dementia without headache. Broadrange polymerase chain reaction identified mycobacterium neoaurum as the possible pathogen. An evaluation of rapidly progressive dementia culminating. This abated, but she developed a rapidly progressive dementia approximately 2 years after that and died 12 months later. Jul 30, 2008 because many rapidly progressive dementias are treatable, it is paramount to evaluate and diagnose these patients quickly. Less common are patients with a rapidly progressive course to death. Rapidly progressive dementia is a syndrome caused by numerous disease entities. Although rapidly progressive dementia rpd syndromes are often considered to be synonymous with cjd, these syndromes have a broad differential diagnosis, which. Large vessel occlusions and thalamic, anterior corpus callosal or multiple diffuse infarctions in particular all have presented as rpds. Dementia with lewy bodies dlb is a lately identified concept based on a clinical and pathological correlation. Antibodylgi 1 autoimmune encephalitis manifesting as. 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. Rapidly progressive dementias are conditions that typically cause dementia over weeks or months. Rapidly progressive dementia and myoclonus the journal.
Rapidly progressive neurodegenerative dementias dementia. Diagnostic challenges in rapidly progressive dementia. He developed a rapidly progressive dementia 2 years after that and died 11 months later. Imaging can demonstrate the atrophy for which this condition is named in half of. Rapidly progressive dementias memory and aging center. An evaluation of rapidly progressive dementia culminating in. A 75yearold lady presented with 2 months history of rapidly progressive illness dominated by dementia, unsteady gait and slurring of speech. Antibodylgi 1 autoimmune encephalitis antilgi1 ae has increasingly been recognized as a primary autoimmune disorder with favorable prognosis and.
Depending on the location, strokes can present as rpd. A 66yearold woman was transferred from an outside hospital for evaluation of her rapidly. Prion diseases are caused by abnormal prions, microscopic infectious agents made of protein. This 49 year old patient developed a fairly rapidly progressive dementia. Pdf rapidly progressive dementia clinical aspects and. A look at the causes and treatments of progressive dementia with ucsfs dr. Other etiologies include autoimmune conditions, cancers, uncommon presentations of other more common. For some people, dementia progresses rapidly, while it takes years to reach an. Rapidly progressive dementia and myoclonus the journal of.
These more rapidly progressive dementias often have clinical characteristics that. Accurate diagnosis is crucial as substantial proportion of these diseases is highly treatable. Dengue infection can cause various effects on the central and peripheral nervous systems. A case of enteroviral meningoencephalitis presenting as. Rapidly progressive dementia geschwind 2008 annals of. Neuroimaging of rapidly progressive dementias, part 2. In some patients, however, cognitive, motor or behavioral symptoms may emerge and progress over the course of weeks to months. Alzheimer disease ad is the most common cause of dementia. Thrombotic thrombocytopenic purpura can cause microangiopathic thromboses producing global. Methods retrospective case analysis was done in 187 patients with rapidly progressive. Rapidly progressive dementia and ataxia in an elderly man.
Rapidly progressive dementia rpd is usually associated with creutzfeldtjakob disease, a fatal condition. A puzzling, rapidly progressive case of dementia and the solution for both identifying and fixing the cause. 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. Early and accurate diagnosis is very important because.
There are multiple etiologies that could potentially cause the subacute neurological decline in rapidlyprogressive dementias. Jan 21, 2016 a look at the causes and treatments of progressive dementia with ucsfs dr. 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. For this reason, accurate identification and assessment of acute and subacute forms of dementia are critical to effective treatment.
Progressive aphasia with rapidly progressive dementia in a 49. Clues that are helpful in identifying patients with an autoimmune dementia can be summarized within a triad of. Using validated, commercially available enzymelinked immunosorbent assay kits, we measured cerebrospinal fluid csf nfl, total tau ttau. Early and accurate diagnosis is essential, as many of the etiologies are treatable. They are a particular challenge to neurologists as the differential diagnosis often is different from the more typical, slowly progressive dementias.
These individuals do not have the mental retardation syndrome, which is caused by the fmr1 gene full mutation 200 cgg repeats. 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. Many conditions other than prion disease, however, can present with rpd. She had had an 18 month history of difficulty finding names, with speech hesitancy. Later she developed spontaneous jerky movements of limbs, rigidity of all four limbs and became bedbound, incontinent and unresponsive. Pathologic findings showed granulomatous inflammation with caseation necrosis, foreign bodytype giant cells, and proliferative endarteritis with vascular occlusions. Early and accurate diagnosis is very important because many causes of rpds can be treated. Rapidly progressive dementia rpd has many possible etiologies and definitive treatment is reliant upon an accurate diagnosis from an appropriate diagnostic workup. Objective to characterize patients with a neurodegenerative disease and a rapidly progressive course to death design, setting, and patients using a text word search for rapid and. Diagnosis and treatment of rapidly progressive dementias. We present the case of a 64yearold woman with rapidly progressive dementia with seizures following dengue infection.
Rapidly progressive dementia and unilateral jerky movements. In contrast with more common dementing conditions that typically develop over years, rapidly progressive dementias can develop subacutely over months, weeks, or even. Although rapidly progressive dementia rpd syndromes are often considered to be synonymous with cjd, these syndromes have a. Early and accurate diagnosis is essential, as many of. For this reason, prompt identification and assessment of acute and subacute forms of dementia are critical to effective treatment. Creutzfeldtjakob disease cjd is a form of spongiform encephalopathy which is caused by an abnormal protein called prion. 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. The socalled rapidly progressive dementias constitute a different, diverse collection of conditions, many of which are reversible or treatable. Dementia refers to a decline in more than one brain function that interferes with daily life. Neurofilament light chain protein nfl is a surrogate biomarker of neurodegeneration that has never been systematically tested, either alone or in combination with other biomarkers, in atypicalrapidly progressive neurodegenerative dementias nds.
While memory loss is a common symptom, others include difficulty with problem solving, controlling emotions, personality changes, agitation and possibly hallucinations. 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. This article presents a practical and informative approach to the evaluation of a patient with a rapidly progressive dementia rpd. These more rapidly progressive dementias often have clinical characteristics. Current advances in the understanding of immunemediated diseases allow the diagnosis of previously unrecognized treatable rpds. The majority of cases are due to neurodegenerative diseases andor vascular disease with relatively gradual progressive clinical courses. In a retrospective study, 23% of patients diagnosed as rapidly progressive dementia had frontotemporal dementiamotor neurone disease, and 9% had alzheimers disease. Rapidly progressive dementia is a curious and elusive clinical description of a pattern of cognitive deficits that progresses faster than typical dementia syndromes.
Rapidly progressive dementia geschwind 2008 annals. Patient 16 was hospitalized for herpes simplex keratitis and cellulitis and experienced a 1week episode of confusional psychosis. The term rapidly progressive dementia rpd is used to describe cases with a progression course which usually ranges between weeks and months. Rapidly progressive alzheimer disease dementia and. Most neurodegenerative diseases such as frontotemporal degeneration or alzheimers disease have a slow, gradual progression over several years. Prions cause a number of diseases in a variety of mammals, including bovine spongiform encephalopathy bse or mad cow disease in cattle and scrapie in sheep there are three different subtypes of prion disease categorized by how the disease is contracted.
Rapidly progressive dementia poses a variety of challenges to the treating clinician, not only due to the speed of disease progression, but. Vascular etiologies of rapidly progressive dementia. Rapidly progressive dementias and the treatment of human. Rapidly progressive dementias rpds are dementias that progress quickly, typically over the course of weeks to months, but sometimes up to two to three years. She had had repeated complex focal seizures and a generalised seizure 7 months before hospital admission. Progressive aphasia with rapidly progressive dementia in a. Dementia, as a clinical syndrome, has many potential causes. Irwin, md, 1 in this issue of continuum, but the diagnosis of rapidly progressive dementias rpds entails a different diagnostic approach.
Because many rapidly progressive dementias are treatable, it is paramount to evaluate and diagnose these patients quickly. Dementia occurs due to physical changes in the brain and is a progressive disease, meaning it gets worse over time. First, there are complications of medical conditions that can cause a subacute decline, including metabolic, infectious, inflammatory, neoplastic or vascular conditions. Rapidly progressive dementia with hypoglycorrhachia. Primary and secondary causes of rapidly progressive dementia, divided by etiology.
The term is used to describe a collection of signs. 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. Direct neurotropism and immunological mechanisms are responsible for most such neurological manifestations. A puzzling, rapidly progressive case of dementiaand the solution for both identifying and fixing the cause. 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. Background neurodegenerative dementias are typically characterized by an insidious onset and a relatively slowly progressive course. Dementia is common among the elderly, affecting about 7% of people age 65 or older, but it is not a normal part of aging. Frontotemporal dementia, albeit generally with quicker symptom progression than ad, is a rarer form of rapidly progressive dementia. Neurodegenerative alzheimers disease corticobasal degeneration dementia with lewy bodies familial spastic paraparesis frontotemporal lobar degeneration motor neuron disease progressive supranuclear palsy prion disease i. Anti leucinerich glioma inactivated 1 lgi1 encephalitis is a rare autoimmune encephalitis ae, characterized by acute or subacute cognitive impairment, faciobrachial dystonic seizures, psychiatric disturbances and hyponatremia.
Prions cause a number of diseases in a variety of mammals, including bovine spongiform encephalopathy bse or mad cow disease in cattle and scrapie in sheep. Most dementias begin insidiously, developing slowly and generally occurring in the elderly age group. This study investigated the profile of patients with rapidly progressive dementia at first presentation. Neuroimaging of rapidly progressive dementias, part 1. The fragile xassociated tremorataxia syndrome fxtas is a progressive neurologic disorder that may affect carriers of premutations of the fmr1 gene 55 to 200 cgg repeats, mainly men older than 50 years of age. However, dementia can be rapidly progressive with time courses measured in only weeks or a few months. Rapidly progressive dementia poses a variety of challenges to the treating clinician, not only due to the speed of disease progression, but also due to the poor prognosis if intervention is delayed.
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