【语音】11-12-额颞叶痴呆101-Frontotemporal dementia

 

Case 1: At the grocery store, an 83-year-old woman fills her cart with items to donate to a food bank. When she realizes that the store does not have donation bins, the woman exits the store without paying and wheels the food-filled cart to a donation center across the street.

Case 2: Another woman likes to wander through the neighborhood where she successfully enters several homes. Although she is arrested for trespassing and the authorities warn her not to return, the 59-year old persists.

Are these behaviors crimes? Is it still a crime if the person has a brain disease?

"Among the middle-aged and elderly, the sudden appearance of criminal behavior may be an early sign of brain illness."

In the March 2015 issue of JAMA Neurology, research published by UCSF Memory and Aging Center (MAC) faculty and others shows a potential link between neurodegenerative disease and new-onset criminal behavior. The authors studied reported criminal conduct in patients with frontotemporal dementia (FTD) and Alzheimer’s disease (AD) and concluded that the sudden appearance of illicit behavior in adults might be an early indicator of brain disease.

Madeleine Liljegren, MD, PhD candidate at Lund University in Sweden and the paper's first author commented, “The consequences for these individuals are many and often devastating. Because of their aberrant behavior, they could get fired from work, their spouse may divorce them and they may even end up in jail. It is important that society and healthcare professionals pay attention and react to these deviant behaviors and help the affected persons to see a doctor.”

Patients diagnosed with FTD showed the highest frequency of criminal behaviors, while those with AD had the lowest rate. FTD patients were the most commonly reported group to authorities for their crimes.

If an adult trespasses or steals, but has FTD, what are the appropriate legal repercussions? When cognitive tests show normal function, these patients become especially vulnerable within the legal system.

Figure：Executive functions help people judge and plan behavior and are located in the frontal lobes, one of the areas hardest hit by frontotemporal dementia.-(http://medschool.ucsf.edu/features/examining-link-between-early-stage-dementia-and-criminal-behavior)

Although Alzheimer disease is the most common and well-known type of dementia, frontotemporal dementia accounts for up to 10% of dementia cases.In fact, in patients under age 65 years, its prevalence is almost equal to that of Alzheimer disease. Unfortunately, frontotemporal dementia is a steadily progressive disease that causes a faster decline than Alzheimer disease, with the average patient surviving only 4 to 8 years after diagnosis.

Frontotemporal dementia also may have strong genetic components. A recent study showed that 10% to 25% of patients have an identifiable autosomal dominant inheritance pattern. All subtypes are due to abnormal deposition and aggregation of pathologic proteins within neural tissues. All subtypes are due to abnormal deposition and aggregation of pathologic proteins within neural tissues. As the disease progresses, these cytoplasmic and nuclear protein inclusions build up and cause neuronal loss and eventual atrophy of the frontal and temporal lobes, either symmetrically or asymmetrically.

• Frontotemporal dementia is a neurodegenerative disorder that affects behavior, language, and personality while sparing early memory loss.
• Because Alzheimer disease and psychiatric conditions are more recognized, frontotemporal dementia frequently is misdiagnosed.
• Medications for Alzheimer disease are not approved for treating patients with frontotemporal dementia; however, providing support services to patients and family members is an important part of management.

The two main clinical subtypes, differentiated by presentation, are behavioral variant and primary progressive aphasia. Behavioral variant presents primarily with significant changes in behavior and personality. Primary progressive aphasia is further broken down into three categories, all of which present primarily with speech impairment and language difficulties.

Diagnostic criteria for behavioral variant frontotemporal dementia (most common subtype)

Possible—presence of three of the following six symptoms:

•  1.Behavioral disinhibition (socially inappropriate behavior, impulsivity, loss of manners):  An impulsive job change or a new childlike sense of humor may be the first indication of pathology. As more of the patient’s frontal lobe is affected, behavioral disinhibition worsens. Patients begin to exhibit socially inappropriate behavior and fail to recognize social cues (A social cue can either be a verbal or non-verbal hint, which can be positive or negative. These cues guide conversation and other social interactions. A few examples of social cues include: facial expression. vocal tone). This lack of social awareness often causes embarrassment for family members. One study even suggests that new criminal behavior in previously law-abiding citizens could be a warning sign. The researchers found that up to one-third of patients had criminal records for offenses such as violent behavior, theft, public indecency, and inappropriate sexual advances.

•  2.Apathy or inertia (loss of interest or motivation, decreased initiation of behavior):  Patients may become either more impulsive or more withdrawn than their norms. Outright apathy and disinterest can develop, which are understandably often misdiagnosed as depression. The loss of sympathy and empathy is one of the most difficult symptoms of behavioral variant frontotemporal dementia for family members to understand. Patients will become uncaring toward family members and even pets. They may lash out, say hurtful things, and detach themselves emotionally. Decreased libido is a frequent symptom.

•  3.Loss of sympathy or apathy

•  4. Perseverative or compulsive behavior (repetitive movements, repeating words): Later in the disease, repetitive movements and speech patterns also can develop

•  5. Hyperorality (binge eating, change in food preference, consumption of inedible objects):  Patients may exhibit binge eating; eating to excess at meals; and increased consumption of sweets, alcohol, or tobacco. Other frequent symptoms are placing inedible objects in the mouth, eating only one type of food, or scratching or picking at the skin or lips.

•  6. Dysexecutive neuropsychological profile (decreased executive function)

Probable—meets criteria for possible, plus:

•  Significant functional decline

•  Imaging findings consistent for frontotemporal degeneration (MRI, CT, or PET)

Definite—meets criteria for possible or probable, plus one of the following:

•  Histopathological evidence on biopsy or at autopsy

•  Known genetic pathogenic mutation

 Diagnostic comparison of frontotemporal dementia and Alzheimer disease

Diagnostic criteria for PRIMARY PROGRESSIVE APHASIA

1.Progressive nonfluent aphasia is the most common variant of primary progressive aphasia and manifests as anomia(忘名症), or trouble finding words and naming objects. Patients often have halting speech with pronunciation and grammar errors. Patients whose disease is mild usually can compensate by using alternative words or simplifying their speech. Although comprehension and repetition are spared early on, these abilities begin to deteriorate as the disease progresses.

2.Semantic（语义） dementia presents as more of a difficulty in language comprehension with a relatively normal fluency. These patients also have trouble finding words and naming objects. They have additional problems with remembering meanings of words, and often compensate by using vague language.13 They may also have visual agnosia, or trouble finding words for visual images.4 Visual agnosia can be tested by showing patients pictures of commonly recognized animals, such as lions or giraffes, and asking them to identify the animal.

3.Logopenic phonological（语音）aphasia is a rare subtype of primary progressive aphasia. These patients have significant impairments in speech quality, talking slowly and having difficulties with word retrieval. Unlike patients with other subtypes, these patients have significant trouble with sentence repetition.

An MRI is necessary to rule out a focal lesion in anyone presenting with word-finding and comprehension difficulty, decreased fluency, and/or motor speech difficulty. However, if an MRI reveals focal atrophy rather than a lesion, consider primary progressive aphasia.5

The four most commonly diagnosed dementias—Alzheimer disease, vascular dementia, dementia with Lewy bodies, and frontotemporal dementia—together account for more than 90% of all cases of dementia.

Tx

Proper neurologist refer once suspect this diagnosis

Unfortunately, standard medications used to treat Alzheimer disease have proven ineffective in treating frontotemporal dementia.

For now, pharmacologic treatment is reserved for managing behavioral symptoms. Selective serotonin reuptake inhibitors have been the most effective for decreasing impulsivity, repetitious behaviors, and sexual disinhibition. Trazodone and some antipsychotics also may be beneficial for aggressive or easily agitated patients, though the antipsychotics must be prescribed with caution due tothe increased incidence of extrapyramidal adverse reactions in patients with frontotemporal dementia.Use of these medications in patients with frontotemporal dementia is off-label and should be prescribed thoughtfully for targeted symptoms, with ongoing reevaluation, dose adjustment, or discontinuation as indicated by patient response. Open communication between clinicians and caregivers works best to attain each patient's ideal medication regimen.

From JAAPA

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