Frontotemporal Dementia: Identification and Management

Leah Wilfong, MS, AGPCNP-BC; Nancy E. Edwards, PhD, ANP-BC; Karen S. Yehle, PhD, FAHA; Karla Ross, MSN, ANP

Disclosures

Journal for Nurse Practitioners. 2016;12(4):277-282. 

In This Article

FTD

FTD is the spectrum of non-AD dementias characterized by focal atrophy of the frontal and anterior temporal lobes of the brain.[3] The 2 major clinical presentations of FTD are categorized as behavioral and aphasic (language variant). The prominent subtype, behavioral variant FTD, results in frontal lobe deterioration, which can elicit behavioral, emotional, and personality changes.[3] The most common aphasic subtype consists of primary progressive aphasia.[13] In the aphasic subtype, the temporal lobe atrophy results in decreased speech production and loss of meaning of words, people, and objects.[3] The diagnostic criteria for both subtypes are summarized in Table 2.

Behavioral Variant FTD

Behavioral variant FTD (bvFTD) has a subtle onset with steadily progressive deterioration in behavior and/or cognitive functioning.[4] It accounts for nearly 60% of all FTD cases.[2] Although gender susceptibility has not been fully established for FTD, in certain subtypes, such as bvFTD, male predominance has been noted.[7] To meet the criteria for diagnosis, 3 of the following 6 symptoms described must be present. These symptoms should not be isolated or rare events, but perpetual in nature.

The first symptom is behavior disinhibition, which encompasses 1 or more of the following: socially inappropriate behavior; loss of manners; and impulsive actions.[4] This may be reported as kissing or grabbing strangers, inappropriate sexual acts or propositions, public urination, swearing, and disregard for personal space or hygiene. Poor financial judgment and impulsivity may manifest as shoplifting, excessive buying sprees, and grabbing food or objects belonging to others.[4]

Second, apathy results in loss of interest, motivation, initiation, or engagement in previously satisfying activities.[4] Family members may note a disinterest in appearance, grooming, and daily care activities. The third symptom is loss of sympathy or empathy.[4] Family members and caregivers may report social disinterest with a lack of eye contact and warmth, or indifference regarding other's feelings in response to hurtful comments or physical distress. The fourth symptom includes compulsive or ritualistic behavior. This may be seen as simple repetitive gestures including tapping, rubbing, or picking at skin or clothing.[4] More complex behaviors resembling obsessive-compulsive disorder, including excessive cleaning, counting, repetitively completing a task, and rereading the same book, may be exhibited.[4] Verbal repetition is noted in asking the same question or repeating words and phrases.

Dietary changes and hyperorality may also be displayed as excessive compulsions to place items in their mouth, particularly inedible objects.[4] Carbohydrate and sweet cravings are common, in addition to binge behavior with food, alcohol, or cigarettes. The final symptom involves deficits in executive tasks, such as difficulty planning, problem-solving, and trouble grasping various concepts. Episodic memory, word recall, and judgment of spatial positions for the most part remain intact until later phases.[4]

Language Variant FTD

Language variant FTD accounts for almost 40% of cases of FTD.[2] The most frequent subtype, primary progressive aphasia,[13] results in gradual progressive language deterioration with a subtle onset. Difficulty with naming objects and persons and expressing oneself is common.[13] As the disease progresses, difficulties in reading and writing develop, with less and less language use, until the individual becomes virtually unable to communicate.[13]

Language alterations are exhibited as a loss of the ability to conceptualize words, such as calling all winged animals birds. Language may lack proper grammar; speech becomes hesitant and slow, requiring additional effort. Further complications with word finding culminate in a decreased ability to produce speech, often leading to muteness. Aphasia represents the most pertinent deficit and remains the primary cause for limitations in daily living, such as the inability to communicate via telephone.[13]

A key distinction from AD is that learning, memory, and perceptual-motor functions remain undisturbed early on, although other cognitive functioning tasks, such as planning and attention, may decline with disease progression.[14] The cause of aphasia must exclude cerebrovascular accident, brain tumor, and other neurocognitive or medical disorders. Recurrent deficits must also not be attributed to psychiatric influence or substance use.

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