Non-Alzheimer’s Dementia syndromes
Not all Dementia is Alzheimer’s disease. Here are some tips for recognizing Non-Alzheimer’s Dementia syndromes.
TIPS FOR RECOGNIZING NON-ALZHEIMER’S DEMENTIA SYNDROMES
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Alzheimer’s disease is a common problem that is appropriately garnering both public and professional attention. As attention to Alzheimer’s increases, however, there is a risk that other causes of dementia-like illnesses will be overlooked, including the 10-15% of these cases that may be reversible. Examples of dementia-like illnesses that may be confused with Alzheimer’s disease are shown in Table 1.
There are four features of dementia-like illnesses that should raise a concern about causes other than Alzheimer’s disease. They include:
- Speed of onset,
- Age of onset,
- The patient’s cognitive and neurological profile, and
- Medical history might indicate a non-Alzheimer’s cause.
Contents
Non-Alzheimer’s Dementia syndromes
Speed of Onset and Progression
The more rapid the onset, the less likely a dementia-like condition is Alzheimer’s disease. Examples of conditions confused with Alzheimer’s, but which have a more rapid onset or progression, range from delirium to mercury toxicity (Table 1).
Age at Onset
The younger the age at onset, the greater the chance of a non-Alzheimer’s dementia. This is particularly true if there is no family history of early-onset Alzheimer’s.
Clinical Profile
Alzheimer’s disease is a slowly progressive disorder that begins by affecting memory and gradually affects other domains including executive skills and naming.
Anosognosia (lack of awareness of one’s disability) may also develop, as may personality changes. But, gait and non-neurological functions are usually spared. Patterns different than this should raise the possibility of a non-Alzheimer’s dementia.
Medical History
A patient’s medical history and medications should be considered. Many medical conditions, ranging from cerebrovascular disease to drug side effects, can cause a dementia like illness that is confused with Alzheimer’s.
Table 1. Examples of Dementia Like Illnesses that Can Be Confused with Alzheimer’s Disease | |||
Speed of Onset | |||
Underlying Cause | Acute | Subacute | Chronic |
Degenerative | Delirium | ALS-dementia with hypoventilation | See Table 2 |
Infectious | Viral encephalitis | Fungal meningitis | Neurosyphilis |
Inflammation | Disseminated Encephalomyelitis | Paraneoplastic syndrome | Autoimmune encephalopathy |
Neoplastic | Obstructive hydrocephalus | Glioblastoma | Orbritofrontal meningioma |
Nutritional | Wernicke-Korsakoff | Vitamin B12 deficiency | |
Psychiatric | Acute psychosis | Inadequately controlled psychosis | Severe depression |
Toxic | Drug or alcohol intoxication | Mercury toxicity | Polypharmacy |
Traumatic | Acute head injury | Subdural hematoma | Chronic traumatic encephalopathy |
Vascular | Acute stroke | Disseminated intravascular coagulation | Vascular dementia |
Reversible Imitators of Alzheimer’s Disease
Although reversible causes account for only 10-15% of cases of dementia-like illnesses, it is essential not to overlook them. The following cases illustrate real patients who were labeled as having Alzheimer’s dementia, but who subsequently turned out to have a reversible medical condition.
Case 1: Drug Toxicity
A 72-year-old man with a history of bipolar disorder presented with a 7-month history of memory loss that was preceded by two years of impaired gait. He scored 18/30 on a Mini-Mental State Exam (MMSE). He was on a stable dose of lithium for years but blood work showed an elevated level. After 3 days off lithium his cognitive function and gait returned to normal.
Case 2: Infection
A 66-year-old man with diabetes developed progressive memory and gait difficulties over two months. Brain imaging showed ventricular enlargement suggesting normal-pressure hydrocephalus, but the patient failed to improve after serial spinal taps. Spinal fluid cultures grew Coccidioides immitis that responded to fluconazole, and his cognition and gait returned to normal.
Case 3: Depression
A 74-year old woman developed personality changes, apathy, and difficulty with memory during the year following the death of her husband. Over the year she withdrew from social activities, and she reported difficulty participating in conversation and was sleeping poorly. Her MMSE score was 23/30, but she didn’t seem to put effort into answering the questions. Her score on the Geriatric Depression Scale (GDS) was 23/30, indicating moderate-severe depression. A trial of antidepressant medication was initiated and over a period of two months, the patient’s cognitive status returned to normal.
Comment
All three cases were atypical for Alzheimer’s disease because the onset of symptoms was too rapid. In addition, cases 1 and 2 involved a recent-onset gait disorder, which is an indicator that Alzheimer’s disease is not the cause of a patient’s cognitive decline. In case 3, the patient had depression, an important diagnosis to exclude because it can sometimes mimic the cognitive impairment seen in dementia.
Non-Reversible Causes of Dementia
Although Alzheimer’s disease is the most common and well-known form of dementia, accounting for about 70% of cases, several other degenerative neurological disorders can cause irreversible dementia and are often confused with Alzheimer’s disease. The most common of these disorders is vascular dementia (now called vascular cognitive impairment), which accounts for about 15-20% of dementia cases and is often mixed with Alzheimer’s. Other common syndromes include Lewy body dementia and frontotemporal dementia, each accounting for about 10% of cases. Key characteristics of these syndromes are shown in Table 2. Although not reversible, recognizing these conditions allows for a more accurate prognosis and more effective use of symptom-modifying medications.
Table 2. Non-Reversible Dementia Syndromes That May Be Misdiagnosed as Alzheimer’s Disease | |
Syndrome | Key Features |
Vascular Dementia |
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Lewy Body Dementia |
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Fronto-Temporal Dementia |
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References and Resources
- Caselli RJ, Tariot PN. Alzheimer’s Disease and Its Variants. New York, NY: Oxford University Press; 2010.
- Graus F, Titulaer MJ, Balu R, et al. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol. 2016;15(4):391-404.
- Kawas CH, Kim RC, Sonnen JA, Bullain SS, Trieu T, Corrada MM. Multiple pathologies are common and related to dementia in the oldest-old: the 90+ study. Neurology. 2015; 85(6): 535-542.
- Porter VR, Avidan AY. Clinical overview of REM sleep behavior disorder. Semin Neurol. 2017; 37(4):461-470.
National Editorial Board: Theodore M Johnson II, MD, MPH, Emory University; Jenny Jordan, PT, DPT, Sacred Heart Hospital, Spokane, WA; Jane Marks, RN, MS, FNGNA, Johns Hopkins University; Josette Rivera, MD, University of California San Francisco; Jean Yudin, CRNP, University of Pennsylvania
Interprofessional Associate Editors: Carleigh High, PT, DPT; David Coon, PhD; Marilyn Gilbert, MS, CHES; Jeannie Lee, PharmD, BCPS; Marisa Menchola, PhD; Francisco Moreno, MD; Linnea Nagel, PA-C, MPAS; Lisa O’Neill, DBH, MPH; Floribella Redondo; Laura Vitkus, MPH, CHES
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