Jane thinks she needs new eyeglasses because she’s having trouble reading and seeing objects right in front of her. Joe’s personality has dramatically changed, with the formerly genteel man making impulsive purchases and saying rude things. And Jack has begun to make things up, telling his family members grandiose stories they know aren’t true.

What do all three have in common? You might never guess that each is suffering from dementia—a devastating condition that most people associate with memory loss.

It’s true that declining thinking and reasoning skills, including memory loss, are signature characteristics of the “Big 3”—Alzheimer’s disease, vascular dementia and Lewy body disease—which account for about 90% of all dementia cases.

However, the red flags suffered by the other 10% of dementia patients are surprising to many people because they deviate from “typical” symptoms, especially when the disease first develops. Dementias that don’t fit the norm… 

• Posterior cortical atrophy (PCA). Visual problems—such as blurry vision, difficulty reading and/or problems with depth perception and identifying objects—can signal PCA, which often strikes in one’s mid-50s to early-60s. But these vision issues aren’t caused by an eye condition. Instead, they stem from shrinkage in the back of the brain, where the occipital lobes, which control vision, are located.

PCA is actually a visual variant of Alzheimer’s or frontotemporal dementia (see below), with the brain no longer properly interpreting what the eyes are seeing. Other symptoms, including diminished memory, reasoning and other cognitive skills, can occur at the same time as the visual disturbances or can come on after a year or two.

How it’s diagnosed: If a person is having vision issues, a visit to the eye doctor (an ophthalmologist or optometrist) can determine if he/she needs glasses or contacts or a new prescription. An eye doctor will also test for macular degeneration, cataracts and other eye problems.

If there are no issues with vision, the patient should see a neurologist, who can perform an exam and order imaging tests. When PCA is present, structural brain scans such as a CT or an MRI will likely show shrinkage in the brain’s occipital lobes. These scans can also rule out other potential causes of symptoms, such as a stroke or tumor.

Treatment approaches: The same medications that may temporarily boost brain cell function in people with Alzheimer’s can also help some people with PCA. These drugs include cholinesterase inhibitors, such as donepezil (Aricept), rivastigmine (Exelon) or galantamine (Razadyne), as well as memantine (Namenda), which blocks a particular brain receptor to enhance the effectiveness of synapses.

To make daily life easier and safer, PCA patients can ask a certified geriatric care manager (usually a social worker or nurse) to assess their homes. To find one in your area, contact the Aging Life Care Association at AgingLifeCare.org.

Examples of what helps: Removing clutter…labeling drawers and items…installing better lighting…and making glass doors and windows more visible with stickers.

• Frontotemporal dementia (FTD). Changes in personality, behavior, language or movement may point to FTD, which accounts for about 5% of all dementia cases. Often diagnosed between one’s mid-40s and early-60s, FTD is caused by progressive nerve cell loss in the frontal lobes, an area in the front of the brain responsible for cognitive functions including problem solving, memory and judgment, and the temporal lobes, which are involved in short-term memory and emotion.

Uncharacteristic and even disruptive behavior changes are often the first noticeable symptoms of FTD, with patients becoming more irritable, impulsive, euphoric or compulsive. As the disease progresses, people with a language variant of FTD will eventually become mute or have more trouble speaking or understanding others. Memory loss is also a later feature of FTD.

How it’s diagnosed: In addition to assessing clinical symptoms, a neurologist will use imaging tests such as CT or MRI scans, which may show wasting in the brain’s frontal and temporal lobes, to help diagnose FTD. Additionally, about one-third of FTD cases are inherited, so genetic testing can be performed in those with a family history.

Treatment approaches: The medications most often used by Alzheimer’s patients don’t always ease FTD symptoms—and may actually worsen them. But other medications, including antipsychotics and antidepressants, can target specific symptoms as they arise. For example, some of the selective serotonin reuptake inhibitor (SSRI) antidepressants, such as citalopram (Celexa), may help behavioral symptoms, such as irritability.

Behavioral programs run by hospitals, dementia centers or psychologists can also help FTD patients and give their families the information they need to support their loved ones. Consult The Association for Frontotemporal Degeneration (TheAFTD.org) for information.

• Korsakoff syndrome. This disorder is triggered by a severe thiamine (vitamin B-1) deficiency, typically stemming from alcohol abuse or bariatric surgery. Because thiamine is needed to help brain cells convert glucose to energy, its absence causes brain damage that can lead to bizarre symptoms (see below).

Those with Korsakoff syndrome have a type of short-term memory loss (anterograde amnesia) that prevents them from learning new information and remembering recent events. Also, they may speak coherently and appear normal but “confabulate”—that is, they make up things they can’t recall and may even believe their made-up stories.

How it’s diagnosed: Since Korsakoff syndrome is typically preceded by a condition known as Wernicke encephalopathy—an acute, life-threatening brain reaction to lack of thiamine—it’s easier to diagnose. Wernicke encephalopathy is characterized by impaired walking, confusion and abnormal eye movements. With the presence of these symptoms, a thiamine blood test can be used to support diagnosis.

Treatment approaches: Oral thiamine supplements will help about one-quarter of those with Korsakoff syndrome to recover (although it may take weeks or months). For others, thiamine deficiency causes permanent brain damage. These patients may need institutional care if their impairment is severe.

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