Initial Evaluation Of A Rapidly Progressive Dementia
RPDs are rather rare and can be difficult to diagnose. Establishing the time course through a thorough history is a critical first step to the evaluation of an RPD. It is important to rule out delirium in any initial evaluation. The first symptoms of a dementia can help with diagnosis. This is particularly true of the neurodegenerative dementias, which often start in specific neuroanatomic regions.13,14 Autoimmune encephalopathies early on often affect the limbic system and, thus, typically present with memory loss or behavioral changes. Viral encephalopathies or acute demyelinating encephalomyelitis may be preceded by a flulike illness. The evaluation of an RPD typically occurs in stages in which several tests are performed in parallel. For an initial RPD evaluation, recommended blood, urine, CSF, imaging, and other tests are shown in Table 7-3.15 Not all of these tests will need to be done, depending on the clinical scenario and the results of tests that have already returned.
At What Point Do Dementia Patients Need 24 Hour Care
When living at home is no longer an option
There may come a time when the person living with Alzheimers disease or dementia will need more care than can be provided at home. During the middle stages of Alzheimers, it becomes necessary to provide 24hour supervision to keep the person with dementia safe.
What Are The Signs Of End Stage Dementia
Experts suggest that signs of the final stage of Alzheimers disease include some of the following:
- Being unable to move around on ones own.
- Being unable to speak or make oneself understood.
- Needing help with most, if not all, daily activities, such as eating and self-care.
- Eating problems such as difficulty swallowing.
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How Does Peanut Butter Detect Alzheimers
The researchers discovered that those who had an impaired sense of smell in the left nostril had early-stage Alzheimers. They noted that the participants needed to be an average of 10 centimeters closer to the peanut butter container in order to smell it from their left nostril compared to their right nostril.
Differential Diagnosis: Diseases Conditions To Rule Out
The initial differential diagnosis for rapidly progressive cognitive decline includes vasculopathy, toxic metabolic encephalopathy, infectious encephalomyelitis, malignancy, and neurodegenerative and psychiatric causes.
Although this patients myoclonus, progressive encephalopathy, and waxing and waning alertness would be an atypical presentation for stroke, this should be ruled out first because of the potential for rapid, irreversible ischemia to neural tissue. If diagnosed correctly and early enough, acute stroke can be treated either with antiplatelet therapy for ischemic stroke or with surgical or endovascular management for hemorrhagic stroke.
Drug-induced encephalopathies due to lithium, amitriptyline, and baclofen, heavy metal intoxication , and metabolic encephalopathies such as Wernicke-Korsakoff syndrome and vitamin B12 deficiency are all possible causes of rapidly progressive dementia. Given the patients abrupt transition off fluoxetine before admission, serotonin syndrome is also a consideration.
Common causes of chronic cognitive decline include neurodegenerative diseases such as Alzheimer disease and frontotemporal dementia , and dementia associated with movement disorders, such as Parkinson disease, Lewy body dementia, and Huntington disease. Although cognitive decline typically occurs over years in these diseases, atypical presentations can lead to rapidly progressive dementia in 15% to 25% of cases and thus should be considered in such cases.
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The Progression And Stages Of Dementia
Dementia is progressive. This means symptoms may be relatively mild at first but they get worse with time. Dementia affects everyone differently, however it can be helpful to think of dementia progressing in ‘three stages’.
The progression and stages of dementia
Making A Probable Diagnosis
As such, a positive RT-QuIC assay is a highly weighted component of the scoring systems used to make the probable diagnosis of sCJD . However, RT-QuIC can be less sensitive in some molecular subtypes of sCJD, so a negative test does not necessarily rule out the disease. In those cases, 14-3-3 protein results, clinical presentation, and characteristic findings on MRI and EEG can aid the diagnosis.
Criteria for probable diagnosis of sporadic CJD
It should be noted that the most recent World Health Organization guidelines for diagnosis of CJD do not take RT-QuIC into account, and thus it may be considered out of date.
High levels of myelin basic protein and oligoclonal Ig G bands are CSF findings useful to diagnose demyelinating disorders such as multiple sclerosis. Amyloid beta is a protein essential to the pathogenesis of Alzheimer disease, implicated in free radical-induced oxidative stress. Neuron-specific enolase is a marker that has great utility in the evaluation of both small cell and non-small cell lung cancers, stroke and brain injury, neuroendocrine tumors, and neuroblastoma.
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Why Might Dementia Progress Quickly
Alzheimers disease typically has a slow and gradual progression, whereas people affected by vascular dementia tend to show periodic, step-wise impairments in function. However, many factors have an impact on the development of dementia. An individuals genetic heritage will play a role, as does their general, physical health. People with cardiovascular disease or diabetes, especially if they are poorly controlled, are at risk of a faster deterioration. People who are frail with low immunity and recurrent infections are also vulnerable. Young-onset dementia tends to progress more rapidly. People who develop dementia between the ages of thirty and fifty, appear to live two years less than those whose dementia is diagnosed later in life.
Most cases of sudden confusion and rapidly progressing dementia in an elderly person are due to delirium caused by infection. Urinary infections and pneumonia can trigger acute confusion that comes on quickly, causing people to be incoherent, muddled and disorientated. Agitation, aggression and odd behaviour are also common. The good news is that the symptoms of delirium can be reversed when the infection is appropriately treated.
How Quickly Does Dementia Progress
The speed at which dementia progresses varies a lot from person to person because of factors such as:
- the type of dementia for example, Alzheimers disease tends to progress more slowly than the other types
- a persons age for example, Alzheimers disease generally progresses more slowly in older people than in younger people
- other long-term health problems dementia tends to progress more quickly if the person is living with other conditions, such as heart disease, diabetes or high blood pressure, particularly if these are not well-managed
- delirium a medical condition that starts suddenly .
There is no way to be sure how quickly a persons dementia will progress. Some people with dementia will need support very soon after their diagnosis. In contrast, others will stay independent for several years.
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Autoimmune Encephalitis And Other Potentially Treatable Causes Of Rpd
AE represents an increasingly recognized common cause of RPD .
Apart from the relevant diagnostic issue of the exclusion of prion disease , CSF and blood NfL have been explored in AE as easily accessible tools for disease monitoring, prognosis, and response to therapy. Although data are scanty and inconsistent, some findings deserve to be mentioned. In analogy with other RPDs, patients with AE typically showed higher CSF and/or blood NfL values than controls or reference intervals . However, concentrations in the normal range have also been reported .
Interestingly, patients on active disease/progression, those on recovery and/or after disease treatment, and those with a stable clinical picture seem to show increased, decreased, or steady levels, respectively . Given the good correlation between CSF NfL and the disability grade , these data suggest a role for NfL as a marker of disease activity in AE.
CSF NfL concentration also predicted long-term outcomes in patients with AE . However, most studies showed no associations between NfL values, CSF parameters, and MRI variables , reflecting a possible discrepancy between clinical severity and radiological or biochemical features in these conditions .
On a significant issue, all the studies mentioned above merged patients with several subtypes of AE, raising concerns about a possible selection bias that may have influenced the results discussed above.
How Does Rpd Differ From Other Types Of Dementia
Dementia, especially of Alzheimers type, typically develops slowly over many years and is often diagnosed in the late stages of the illness. RPD, on the other hand, is different as it progresses much more quickly. However, compared to Alzheimers disease and vascular dementia, RPD tends to be rare, often challenging to diagnose, and can develop at a greater rate.
The course of RPD is variable and depends on the cause. For example, the average age of onset of sporadic Creutzfeldt-Jakob disease is 6070 years. Those affected may initially experience fatigue, sleep disturbances, and decreased appetite, all of which may occur weeks before the onset of nervous system dysfunction. Behavioural changes can also be an early sign of disease.
As shown below, a number of conditions in addition to CJD can cause RPD and functionally impair a person at a faster rate than other forms of dementia.
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What Happens In Rapidly Progressive Dementia
The presentation and progress of RPD will vary between individuals. People affected will usually develop problems with their memory, thought processes and communication. Many people also have personality or behavioural changes and mood disturbance. Movement changes can also occur as a result of the brain cell injury.
Some forms of RPD are treatable and, if diagnosis is made quickly, early symptoms may be reversed. Regrettably, for other causes of the condition, there are no available cures. There is an inevitable increase in symptoms and decline in function. Sadly, within months or years, the rapidly progressing dementia will cause failure of all body systems and death.
Rapid Cognitive Decline And Myoclonus In A 52
- Release date: October 1, 2021
- Expiration date: September 30, 2022
A 52-year-old woman presented to the emergency department in Las Vegas, NV, with progressively worsening altered mental status for the past 2 weeks. A history of symptoms was obtained from the patients sister, with whom she was staying. The sister reported that during the week before presentation, the patient had episodes of confusion, intermittent blank staring, blurred vision, bilateral incoordination of the upper and lower extremities, difficulty following commands, and decreased verbal communication. The patient had also been holding her left hand in a fist. Two days before presentation, the patient experienced a significant decline in mentation and had multiple episodes of urinary incontinence, which she never had before. During a period of lucidity in the emergency department, the patient denied having fever, chills, nausea, vomiting, chest pain, shortness of breath, abdominal pain, dysuria, or headache.
The patient had a history of major depressive disorder treated with fluoxetine until 8 days before presentation, when she was switched to escitalopram by an outpatient psychiatrist owing to onset of the psychomotor symptoms. The patient had no prior blood transfusions or surgeries and no known drug allergies.
1. Which of the following would be an atypical cause of this patients rapidly progressive cognitive decline?
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How Is An Rpd Diagnosed
RPD can be difficult to diagnose, so it is often necessary to see a doctor who specializes in these conditions. The doctor might ask about the patients progression of symptoms, any similar illnesses in biological relatives or any recent possible exposures . The doctor may request some laboratory testing, such as blood, urine and cerebrospinal fluid brain imaging and/or an electroencephalogram . The information gathered by the physician and tests might help to determine the cause of disease.
Biomarker Values Throughout The Disease Course Of Genetic Prion Disease
Any future treatment aimed to prevent prion disease cannot prescind from the identification and longitudinal evaluation of pre-symptomatic PRNP mutation carriers. The significant heterogeneity of the age at onset in the prion disease spectrum combined with the rarity of the disease strongly limits the use of clinical onset as an outcome in preventive clinical trials. In this regard, identifying a biomarker of proximity to disease onset might be critical to overcome the issue .
To date, only two studies explored neurofilament dynamics in cross-sectional and/or longitudinal samples from pre-symptomatic and symptomatic PRNP mutation carriers. One reported no difference in CSF and/or blood NfL levels between pre-symptomatic PRNP mutation carriers and healthy controls and no temporal increase in longitudinal blood and CSF samples in pre-symptomatic cases . Similarly, in the study of Thompson et al. , NfL concentrations did not differ between pre-symptomatic PRNP mutation carriers with samples collected more than 2 years before symptom onset and those of controls. However, the marker increased progressively in the longitudinal evaluation from 2 years from onset to the symptomatic phase. In summary, the available data on this aspect are, to some extent, controversial. They potentially reflect a common selection bias, namely, the inclusion of subjects with several different PRNP mutations and heterogeneous clinical progression rates.
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What Is The Treatment Of Rpd
Some forms of RPD are treatable, particularly if they are detected at an early stage. The potential for treatment depends on the type of RPD that is diagnosed. For example, for RPD caused by cancer, hormone imbalance or vascular disease, treatment of the underlying cause may help treat the dementia symptoms also. For some cases of RPD, particularly due to CJD, there are treatments available which are effective in reducing distress, maintaining comfort, and improving quality of life.
Familiarity with surroundings and enhanced levels of accessibility can help someone with dementia to continue living independently. You can find out more about making a home dementia-friendly here. Moreover, specific guides to RPD for both healthcare providers and affected individuals have been developed that contain key information and links to additional resources and media .
Infective And Neoplastic Causes
Many CNS infections can cause cognitive decline. CSF results help to differentiate these, including CSF protein and white cell count and viral PCR , cytomegalovirus , EpsteinBarr virus and enterovirus). Against this in our patient, however, is the relatively subacute onset and lack of systemic features including fever. However, neurotropic viruses, such as enterovirus 71, can present with a subacute cognitive decline, which can be fatal. Also, measles can cause cognitive decline in elderly patients and mycoplasma can cause encephalitis. In immunocompromised patients, CMV and EBV infections can cause encephalitis and it would be important to know the patient’s HIV status. There can also be a dementia-like presentation in Lyme disease.
Chronic meningitis due to cryptococcal or tuberculous infection or leptomeningeal metastases may present with dementia and ataxia and can cause intracranial hypertension, either with , or without ventricular enlargement .
Neurological Whipple’s disease is also possible. Patients present with cognitive decline, psychiatric symptoms, seizures and oculopalatal myoclonus. MRI should show one or more multinodular enhancing lesions. The CSF serology for Tropheryma whipplei is not always positive, and brain biopsy might be needed for diagnosis.
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Why Test For Prion Disease
CSF analysis can provide additional data if the diagnosis of CJD is uncertain. RT-QuIC monitors for formation of amyloid in real time after adding disease-associated prion protein from the patient to recombinant prion protein. The mixture is shaken vigorously, exploiting the ability of PrPSc to induce misfolding of recombinant prion protein, forming aggregates. The formation of the aggregates is monitored in real time using a fluorescent dye, thioflavin T. Currently, the National Prion Disease Pathology Surveillance Center at Case Western Reserve University in Cleveland, OH, is the only facility in the United States that performs the RT-QuIC assay.
The 14-3-3 protein is believed to be a marker of massive neuronal disruption and leakage of brain proteins into the CSF. The protein biomarker total tau , another marker of neuronal death, has been found to be elevated in CSF in patients with sCJD, with 1 study showing it to be a more specific but less sensitive test than the 14-3-3 protein assay.
Causes Of A Rapidly Progressive Dementia
There are clearly other disorders which cause rapidly progressive dementia, but I am dismissing many on the basis of the normal MRI and CSF and the lack of encephalopathic features. There are peculiar chronic encephalitic illnesses, such as Rasmussens encephalitis and Landau-Kleffner syndrome, which are predominantly diseases of childhood and adolescence, although they can present in later life. Cerebral vasculitis can be a great mimic, is a great favourite in clinicopathological conferences, and can present focally. In this case, however, there are no stroke or multiple sclerosis-like episodes, and the MRI was normal with no oligoclonal bands on CSF, so I think that it is almost inconceivable.
I also considered Hashimotos encephalopathy. It tends to affect young to middle aged women. It usually begins abruptly, and typically causes an encephalopathic illness. There can be focal signs, and headaches are common. The CSF is usually abnormal. Thyroid function can be normal, so it is imperative to check for thyroid autoantibodies. The progressive unremitting course in this patient is very much against this as a diagnosis.
Whipples disease is a possibility, especially given the history of weight loss. These patients also tend to be encephalopathic. Movement disorders, including oculomasticatory myorhythmia, and brain stem symptoms, are described in this condition.
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Could It Be Rapidly Progressive Dementia
RPD can be difficult to diagnose. However, accurately diagnosing these conditions is critical in order to identify any treatable causes and protect against further brain cell damage. An early hospital assessment by a specialist can help pick up problems so that, where available, appropriate treatment can be initiated. Cancers, infections, toxins and autoimmune conditions could all cause a fast decline in mental function, as well as the more common neurodegenerative causes of dementia such as Alzheimers, strokes and Parkinsons disease.