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