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Frontotemporal Dementia Disorders

Published on: March 15, 2012

by Dementia and Alzheimers

While Alzheimer’s disease accounts for approximately 70% of dementia cases, frontotemporal dementia is another type of dementia that affects approximately 50,000-60,000 Americans. FTD is an umbrella term that includes several related disorders, including the following: Behavioral Variant FTD (bvFTD)

More than 50% of FTD cases are of the behavioral variant type. The most common symptoms of bvFTD are behavioral or emotional challenges, such as overeating compulsively, an emotionally-detached demeanor, socially inappropriate responses, hyper-sexuality in conversation and thought, apathy and selfishness.

Some individuals with bvFTD also experience movement difficulties similar to Parkinson’s disease, including decreased facial expressions, muscle stiffness, weakness and rigidity. Memory loss does not usually develop until the disease has significantly progressed. Diagnosis is often delayed because initially, it may be thought that the individual is just being rude, selfish, depressed or acting out of character.

The prognosis for bvFTD is poor. Average life expectancy from onset of symptoms is approximately nine years. Treatment may consist of attempting to use non-drug behavior strategies to curb specific behaviors. Some physicians prescribe antidepressants called selective serotonin reuptake inhibitors (SSRIs), which may help with some of the obsessive-compulsive behaviors such as hoarding or overeating.

Primary Progressive Aphasia (PPA)

Individuals with PPA can often perform intricate tasks but have difficulty with speech or language. For example, they may be able to build a complicated house but not be able to express themselves well verbally or understand what others are trying to communicate to them. Initial symptoms of PPA include difficulty recalling a specific word or substituting a closely related word such as “take” for “tack.”

Other symptoms develop as the disease progresses, such as increasing difficulty in speaking and not understanding words that are read or spoken to them. Individuals often become completely mute.

On average, about five years after these initial symptoms involving language, PPA begins to affect memory and other cognitive functions. Interestingly, more men than women experience PPA.

PPA can be subdivided into three categories:
Semantic PPA: Individuals lose the ability to say certain words, and their ability to recognize others may decline.
Agrammatic PPA: Individuals have difficulty forming complete sentences. For example, they may be able to speak using nouns and verbs but not be able to connect them with words like “to” and “from.” As agrammatic PPA progresses, individuals may struggle with forming any words and have trouble with swallowing and muscle control.
Logopenic PPA: Individuals may experience difficulty locating the correct words to speak but retain the ability to understand what others are saying to them.

Progressive Supranuclear Palsy (PSP)

PSP was identified in 1964 as a specific disease and is sometimes referred to as Steele-Richardson-Olszewski Syndrome, named after the scientists who identified it. PSP’s initial symptoms include poor balance, unsteady gait in walking and unexplained falls. Memory problems, emotional outbursts, depression, apathy and irritability are other early symptoms.

Eye movement problems are one of the hallmark symptoms of PSP. Because of the lesions that develop in the brain, it becomes difficult to aim the eyes correctly. For example, a person may not be able to maintain eye contact during a conversation. An accurate diagnosis of PSP often is not reached until it progresses to the stage when the eyes begin to function poorly. Until then, it is often misdiagnosed as Parkinson’s.

PSP is different from Parkinson’s in several ways. One significant difference is that individuals with PSP often stand straight up or even lean back (causing the risk for falling backwards), while those with Parkinson’s often lean forward and bend over.

Treatment options for PSP are limited. Some patients experience temporary improvement in their symptoms with the use of medications like Sinemet, a prescription drug traditionally prescribed to treat Parkinson’s. There also has been some improvement in motor and movement symptoms with Elavil, an antidepressant, though some patients report decreased balance when taking this drug.

The prognosis for PSP is poor. Life expectancy may be up to 12 years after initial symptoms, and death usually results from a complication of PSP such as pneumonia. It is estimated that approximately 20,000 Americans have PSP.

Corticobasal Degeneration (CBD)

CBD, also known as corticobasal ganglionic degeneration, is a rare neurological disorder that shares several symptoms with Parkinson’s. Early symptoms include slow or clumsy movements, tremors or shakiness, muscle weakness and stiffness. The individual with CBD often initially has symptoms only on one side of the body. Later, as the disease progresses, both sides are affected.

CBD also affects the ability to speak and understand what others are saying, and can affect short-term memory and the ability to do mathematical calculations. Behavioral components such as compulsions, socially inappropriate behaviors and repetitive actions may also be present.

As with the other disorders in the frontotemporal dementia group, treatment of CBD is limited. Medications that may help relieve some of the symptoms include cognitive enhancers usually prescribed for Alzheimer’s patients, such as cholinesterase inhibitors. Other individuals show some improvement in their physical abilities with medications like Sinemet. Physical, occupational and speech therapy may also be ordered by the physician.

CBD is a progressive disease that often renders its victims immobile after about five years; within 10 years, the person may succumb to complications such as pneumonia or another infection.

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BMC Neurology

  • Case report
  • Open Access
  • Open Peer Review

Progression of logopenic variant primary progressive aphasia to apraxia and semantic memory deficits

  • 1 Email author ,
  • 2 ,
  • 3 ,
  • 4 ,
  • 5 and
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BMC Neurology201313:158

https://doi.org/10.1186/1471-2377-13-158

©  Funayama et al.; licensee BioMed Central Ltd. 2013

  • Received: 4 April 2013
  • Accepted: 30 October 2013
  • Published:




Open Peer Review reports

Abstract

Background

Due to the nature of neurodegenerative disorders, patients with primary progressive aphasia develop cognitive impairment other than aphasia as the disorder progresses. The progression of logopenic variant primary progressive aphasia (lvPPA), however, has not been well described. In particular, praxic disorders and semantic memory deficits have rarely been reported.

Case presentations

We report three patients in the initial stage of lvPPA who subsequently developed apraxia in the middle stage and developed clinically evident semantic memory deficits in the advanced stages.

Conclusions

The present case series suggests that some patients with lvPPA develop an atypical type of dementia with apraxia and semantic memory deficits, suggesting that these cases should be classified as a type of early-onset Alzheimer’s disease.

Keywords

  • Logopenic variant primary progressive aphasia
  • Apraxia
  • Semantic memory deficit
  • Alzheimer’s disease

Background

Primary progressive aphasia (PPA) is an initial clinical presentation of degenerative dementia, which is characterized by three variants of progressive language disorder: non-fluent/agrammatic, semantic, and the newly recognized logopenic subtypes [ 1 ]. The clinical presentations of the subsequent and advanced stages of non-fluent/agrammatic primary progressive aphasia (naPPA) and semantic variant primary progressive aphasia (svPPA) have been well documented. The later behavioral changes in naPPA are similar to those of frontotemporal dementia and include a decline in social interpersonal conduct, impairment in regulation of personal conduct, emotional blunting, and loss of insight [ 2 ]. Patients with svPPA also demonstrate behavioral changes such as loss of sympathy and empathy, narrowed preoccupations, and parsimony [ 2 ]. In short, patients with these variants of PPA may develop social behavioral problems in the advanced stages. However, the progression of logopenic variant primary progressive aphasia (lvPPA), the third variant of PPA, has not been well described.

The notable clinical characteristics of patients with lvPPA in the early stage are length-dependent impaired repetition, phonological errors, and anomia. However, the characteristics of patients with lvPPA in the middle and later stages have not been fully studied. As a case series study, Gorno-Tempini et al. 2008 [ 3 ] reported that patients with lvPPA showed relative sparing of their functional status 5 years after the onset of symptoms. Caffarra et al. 2013 [ 4 ] presented a patient with lvPPA who developed jargon aphasia as a late feature. Rogalski et al. 2011 [ 5 ] suggested that the unique linguistic features of lvPPA in the early to middle stage may lose their distinctiveness from other types of PPA as the degeneration worsens. Thus, cognitive impairment other than aphasia has rarely been reported during the deterioration process in lvPPA. However, due to the nature of neurodegenerative disorders, patients with lvPPA inevitably develop cognitive impairment other than aphasia as the disorder progresses.

In the present study, we describe three patients with lvPPA in the early stage who later presented with apraxia in the middle stage and then showed clinically evident semantic memory deficits in the advanced stages. We focused on the clinical and neuropsychological features during cognitive decline in these three patients with lvPPA.

Case presentations

Patient 1

A right-handed woman with 16 years of education began to experience progressive word-finding difficulty at the age of 56 years old. She had been an efficient housewife for more than 30 years. She had shown no previous abnormal neurological or psychiatric abnormalities. She had no family history of dementia. She was referred to our hospital for evaluation of speech difficulties 1 year after onset of the aphasia. Her cranial nerves, motor systems, sensory systems, and coordination systems were normal, and she showed no extrapyramidal signs. Brain magnetic resonance imaging (MRI) and electroencephalography (EEG) were normal. Her laboratory examinations were normal including levels of vitamins, folic acid, and thyroid hormones. The absence of human immunodeficiency virus, syphilis, collagen disease markers, anti-phospholipid antibody, and antineutrophil cytoplasmic antibody-associated vasculitis was noted. Her cerebrospinal fluid was normal. We evaluated the patient with 99mTc-ethylcysteinate dimer single photon emission computed tomography (Tc-99 m ECD SPECT), which was analyzed with an easy Z score imaging system (eZIS) [ 6 ] (Figure  1 ). The image demonstrated hypoperfusion in the bilateral temporo-parietal areas, in particular, the left temporo-parietal junction and left middle and inferior temporal gyri.

Figure 1
Figure 1

Brain SPECT eZIS analysis of case 1. This series of scans showed relative hypoperfusion mainly in the bilateral temporo-parietal areas.

Patient 1 showed word-finding difficulty and occasional phonological paraphasia (Table  1 ), for example, saying ‘dansha’ for ‘densha’ (train). Comprehension of single words was nearly intact, whereas comprehension of complex sentences was impaired. In addition to spoken language, her comprehension of written language was also impaired for complex sentences. Her writing skills were also impaired. Although grammar and articulation were preserved, her repetition was limited to two words. Her forward digit span was a maximum of three digits. For the backward digit span, she was unable to give any correct answers even for two digits. Non-word repetition deteriorated as the number of morae increased, with 14 correct answers of 14 non-words with two morae, 12 correct of 14 with three morae, 8 correct of 14 with four morae, and 4 correct of 14 with five morae. Her repetition performance including the word-length effect was highly suggestive of a verbal short-term memory deficit. Her ability to calculate was also impaired.

Table 1

Demographics and initial linguistic assessment

 

Case 1

Case 2

Case 3

Age at onset, years/gender

56/F

53/M

51/M

Education (years)

16

12

12

Years of follow-up

9

7

10

Clinical Dementia Rating total (0–3) for the first 2 years

0.5

0.5

0.5

Forward digit span

3

2

3

Backward digit span

None

None

2

Non-word repetition

Word-length effect

Word-length effect

None

Apraxia of speech

Not found

Not found

Not found

Dysarthria

Not found

Not found

Not found

Phonological paraphasias

Occasional

Frequent

Frequent

Confrontation naming in SLTA, % correct

65

40

35

Word repetition in SLTA, % correct

100

80 (due to paraphasia)

80 (due to paraphasia)

Sentence repetition in SLTA, % correct

20

20

0

Auditory single-word comprehension in SLTA, % correct

100

100

100

Auditory complex sentence comprehension commands in SLTA, % correct

30

0

20

SLTA: Standard Language Test of Aphasia.

However, her orientation and episodic memory were well preserved. Insight, judgment, behavior, concern with hygiene, and effectiveness in performing customary daily activities were also preserved. She continued to live independently. She was partly aware of her aphasia and was frequently frustrated by her predicament. Diagnosis of lvPPA was made according to the clinical features and neuroradiological findings.

Her aphasia gradually worsened, with additional problems starting at age 58 (Table  2 ). She began to complain of difficulty using electrical appliances such as a landline phone, cell phone, TV remote control, hot water dispenser, and coffee maker. She clearly understood what these appliances are and what they are used for, but she was not sure how to use them. She was unable to drive a car as she did not know how to manipulate the many buttons, the pedals, the steering wheel, and the levers. She was not sure how to heat her bath. Mild ideomotor apraxia of the left hand was found when asked to imitate the examiner’s gesture.

Table 2

Clinical course of apraxia and semantic memory impairment following symptom onset and initial radiological assessment

 

Case 1

Case 2

Case 3

Ideomotor apraxia

2 years later

2 years later

4 years later

Conceptual apraxia

4 years later

5 years later

8 years later

Episodic memory deficit

4 years later

3 years later

7 years later

Inability to recognize family members or relatives

7 years later

5 years later

9 years later

Pica

7 years later

6 years later

9 years later

Relative hypoperfusion on SPECT

Bilateral temporo-parietal area

Bilateral temporo-parietal area

Bilateral temporo-parietal area

SPECT: single photon emission computed tomography.

At age 59, her inability to use electrical appliances extended to tools for everyday life. She was no longer able to use a nail clipper or open a suitcase. At age 60, she presented with remarkable semantic errors when using tools. When she tried to boil water, she used a pan instead of a kettle. She tried to unlock her car door with a remote control for the air conditioner. She brushed her teeth with a comb.

With the Standard Language Test of Aphasia (SLTA) in Japanese [ 7 ], her correct naming decreased from 65% at age 58 to 10% at age 60. Although her comprehension of single words remained 100% even at age 60, her comprehension of command sentences fell from 30% at age 58 to 0% at age 60. To study her ability to use tools, she was given 20 tools for use in everyday life, such as scissors and comb, and asked to show how they are used. She used all 20 objects correctly at age 59. However, her performance worsened to 17 correct of 20 at age 60, 10 correct of 20 at age 61, and 5 correct of 20 at age 62. Errors were found mainly in how to manipulate and even how to hold an object in the later stage.

It was not until age 60 that she showed episodic memory deficits and behavioral changes such as excitement and aggression. At age 61, her ability to copy the interlocking pentagons of the Mini-Mental State Examination [ 8 ] was poor, and the number of correct answers in the Benton Visual Retention Test [ 9 ] was 2 out of 10 designs, suggesting that she had visuospatial deficits and/or visual memory deficits. She could not perform the motor series Luria test of the frontal assessment battery [ 10 ] with her right hand, suggesting her frontal function had also deteriorated. At age 63, she was unable to recognize her husband. She began to frequently eat non-edible objects such as diapers.

Patient 2

A right-handed man with 12 years of education experienced a gradually progressive speech disorder and difficulty in using a personal computer at age 53. Although he had worked for a manufacturing company for 35 years after high school, he became unemployable in his capacity as a factory worker. Subsequently he found a job as a janitor, but he was fired within a year because of his word-finding difficulty. At age 54, he was referred to our hospital for evaluation of his problems. He had no previous medical or psychiatric history. Regarding his family history, one of his older brothers had been diagnosed with probable Alzheimer’s disease. His physical and neurological examination revealed no remarkable findings. His blood test and serum chemistry, which covered many of the same items as in Patient 1, were normal. His EEG was normal, but brain MRI showed enlargement of the bilateral sylvian fissures. N-isopropyl-p[123I] iodoamphetamine (IMP) SPECT with three-dimensional stereotactic surface projections (3D-SSP) analysis [ 11 ] showed relative hypoperfusion in the bilateral temporo-parietal areas, extending from the inferior parietal lobule to the temporo-parietal junction to the middle and inferior temporal gyri.

His main difficulties were related to word finding and frequent phonological paraphasias (Table  1 ). Furthermore, he frequently presented with phonological retrieval difficulty, as he spoke fragmentary syllables as in ‘tochi’ for ‘tomodachi (friend)’, and ‘sen’ for ‘sensha’ (military vehicle). No anarthria or dysarthria were noted. His grammar was preserved. His forward digit span was limited to two digits, and he was unable to perform the backward digit span. In non-word repetition, a word-length effect was noticed, with 11 correct answers of 14 with two morae, 6 correct of 14 with three morae, 3 correct of 14 with four morae, and 1 correct of 14 with five morae. These clinical findings suggested a verbal short-term memory deficit. Comprehension of single words remained intact with 100% correct in the SLTA, whereas comprehension of complex sentences was severely impaired. His reading abilities and his writing skills were also poor. However, orientation and episodic memory were intact at this time. No behavioral abnormalities were noted. He continued to live alone independently. He was partly aware of his aphasia and was easily irritated by his word-finding difficulty. Diagnosis of lvPPA was made according to the clinical and neuroradiological findings.

At age 55, ideomotor apraxia of both hands was observed when asked to imitate the examiner’s gesture (Table  2 ). The phenomenon of body parts as objects was frequently noted. Apathy became gradually evident, and his family sometimes had to supervise his daily living.

At age 56, he became forgetful and sometimes became lost. He was also unable to open and shut off the gas valve. He had particular difficulty in using electrical appliances in daily life. At age 57, his speech rate had significantly slowed down due to difficulty with lexical and phonological retrieval. His comprehension of single words gradually worsened, and he scored 80% correct on the SLTA. His reading and writing skills also deteriorated to the point that he was unable to write his own name. His ability to copy the interlocking pentagons of the Mini-Mental State Examination was poor, and the number of correct answers in the Benton Visual Retention Test was only 1 out of 10 designs, suggesting that he had visuospatial deficits and/or visual memory impairment.

At age 58, he presented with “TV sign” [ 12 ], in which he thought that people on TV were in his room, and he tried to speak to them. He was unable to use almost all the tools of everyday life. He tried to use a toothbrush to comb his hair. He would occasionally shout inexplicably. He was no longer able to recognize his relatives. At age 59, he started to eat waste thread and rubbish with crumbs on the floor.

Patient 3

A right-handed man with 12 years of education noticed progressive speech difficulties at age 51. At age 52, he was no longer able to read the clock and found himself sometimes unable to comprehend long sentences. Although he had been employed as a salesman in an apparel business for more than 30 years, he was fired due to his aphasia. He had no previous medical or psychiatric problems. He had no family history of dementia. His physical and neurological examinations were normal. The patient underwent numerous laboratory tests, which were normal, including blood tests and serum chemistry. His EEG was normal. Brain MRI showed enlargement of the bilateral sylvian fissures. Brain IMP SPECT 3D-SSP analysis showed relative hypoperfusion in the bilateral temporo-parietal areas.

One year after symptom onset, examination showed progressive aphasia with word-finding difficulties, frequent phonological paraphasia, and mild word deafness (Table  1 ). Speech repetition was limited to a single word. Non-word repetition was almost impossible to execute due to phonological paraphasia. His phonological paraphasia for repetition or word retrieval involved omission, substitution, rearrangement, and addition of speech sounds. His forward digit span was limited to three digits, and his backward digit span was a maximum of two digits. His articulation and grammar were preserved. Comprehension of both spoken and written words was intact. Comprehension of complex sentences, however, was impaired in both auditory and visual conditions. His writing skills were also impaired. He was somewhat aware of his aphasia. Although he was often irritated by his aphasia, he continued to live a nearly independent life. His clinical features and neuroradiological findings led to a diagnosis of lvPPA.

Over the years, his aphasia slowly but definitely worsened. With SLTA, correct naming decreased from 35% at age 52 to 0% at age 55. Comprehension of single words also fell from 100% at age 52 to 70% at age 55. The characteristics of his paraphasia at this point were occasional neologistic jargon and remarkable phonological addition errors. For example, he said ‘takenokokonoko’ for ‘takenoko (bamboo)’, a phenomenon that can also be regarded as logoclonia. Cognitive deficits in addition to aphasia became evident at age 54 and progressively deteriorated. At age 55, he began to have problems in daily life. He forgot how to use electrical appliances. He developed ideomotor apraxia of both hands when instructed to demonstrate how to use tools or to imitate the examiner’s actions. He became partly dependent on his family.

At age 58, most of his speech consisted of logoclonia such as “sonnnanananananana” and “sirorourorororo” and he was no longer able to comprehend most single words. He was deeply frustrated by his aphasia and sometimes became aggressive. At age 59, he started to eat food with his fingers because he was no longer able to use chopsticks or a spoon. Other tools he was unable to use included a shaver and a toothbrush. He seemed to understand what these tools were used for when his choice for each action was accompanied with the proper tools. However, at age 59, he gradually became confused with what to use for a desired action. He confused toothpaste with shaving cream. His ability to copy the interlocking pentagons of the Mini-Mental State Examination was poor, and the number of correct answers in the Benton Visual Retention Test was only 2 out of 10 designs, suggesting that he had visuospatial deficits and/or visual memory impairment.

At age 60, he started to glare at everyone, as he was unable to recognize family members. He would try to use and manipulate whatever objects he saw. However, he never understood what these objects were and sometimes put them in his mouth and tried to chew them. In addition, he ate cat food several times.

Case summary

Although all three cases exhibited some cognitive impairment other than aphasia at initial presentation such as acalculia, inability to use a computer, and inability to read a clock, their main symptom was limited to the language impairment of lvPPA, which began in their 50s. One of the three cases had a family history of probable Alzheimer’s disease. Their Clinical Dementia Rating scale [ 13 ] remained at 0.5 for the initial 2 years of their illness. Their aphasia progressively worsened, and each patient showed progressive cognitive impairment, which became evident in 2 or more years after the onset of symptoms. The characteristics of their subsequent cognitive impairment were apraxia, which extended from ideomotor (praxis production deficits) to conceptual apraxia (impaired knowledge for tool-action or tool-object associations as well as mechanical knowledge). These apraxias presumably accounted for the patients’ difficulties in using electrical appliances. Episodic memory deficits as well as visuospatial deficits became apparent several years after the onset of symptoms. Lastly, clinically evident semantic memory deficits were noted, including a lack of recognition of their family members or relatives. Their primary affected areas involved a large portion of the bilateral temporo-parietal lobes, extending from the inferior parietal lobule to the temporo-parietal junction to the middle and inferior temporal gyri.

Conclusions

The present case series provides new insight into the progression of lvPPA. The three right-handed patients with lvPPA developed language decline with subsequent apraxia, followed by clinically evident semantic memory deficits. The results suggested that some patients with lvPPA develop an atypical type of dementia with apraxia and semantic memory deficits.

The clinical course of lvPPA in this study differs from the other variants of PPA, i.e., naPPA and svPPA, which result in profound social and behavioral problems. In contrast, in the present cases, the activities of their social lives were well preserved until the later stage. The patients also had some awareness of their illness. Although semantic memory deficits were noted, similar to the deficits in svPPA, the clinical courses of the two variants were quite different from each other. In particular, apraxia was a prominent feature in the present lvPPA cases, including ideomotor and conceptual apraxia along with difficulties in using home electrical appliances.

The outcomes of the present cases differed from those of previous lvPPA reports. Although a previous report described almost pure aphasia [ 3 ], the present cases developed further cognitive decline. Several possibilities could be postulated to account for the different clinical courses. First, the duration of the follow-up period of the present cases was longer than for the previous report. Gorno-Tempini et al. [ 3 ] acknowledged that extensive follow-up was not available in most patients with lvPPA that they examined. Given the nature of degenerative disorders, a variety of types of cognitive impairment in addition to aphasia inevitably develops in the advanced stages. Second, the affected brain regions may have been different. The main lesions in our cases extended to the bilateral temporo-parietal areas, whereas the previous cases showed only a unilateral left lesion. In addition, the affected area in our cases involved a large portion of the temporo-parietal areas, and may have involved a larger area than that of previous reports.

Apraxia has been reported in degenerative diseases. Although apraxia in corticobasal ganglionic degeneration is well known, other types of degenerative diseases are also regarded as involving apraxia [ 14 , 15 ], in particular, Alzheimer’s disease. Ideomotor apraxia [ 16 20 ] and conceptual apraxia [ 21 24 ] have frequently been noted in Alzheimer’s disease. In contrast, in svPPA or Pick’s disease, apraxia has been documented in only two case reports [ 25 , 26 ]. Bilateral hypoperfusion on SPECT is not suggestive of corticobasal ganglionic degeneration, which is characterized by striking asymmetric atrophy and/or hypoperfusion. Moreover, the present cases had no signs of parkinsonism, which rules out the possibility of corticobasal ganglionic degeneration. Instead, the onset of Alzheimer’s disease may reasonably account for the apraxia observed in our cases.

Although semantic memory deficits are a hallmark of svPPA, these deficits have been described in Alzheimer’s disease as well [ 27 31 ]. Some researchers have pointed out that semantic memory deficits in Alzheimer’s disease can be regarded as a continuum beginning with conceptual apraxia [ 23 , 24 ]. According to Dumont et al. 2000 [ 23 ], the representations of action-semantic and semantic knowledge of objects are often simultaneously disrupted in Alzheimer’s disease, suggesting that conceptual apraxia and semantic memory deficits are closely related. Falchook et al. 2012 [ 24 ] shared a similar view, suggesting that semantic taxonomic relationships correlate with performance on tests for conceptual apraxia in people with Alzheimer’s disease. The coexistence of conceptual apraxia and semantic memory deficits in our cases is highly suggestive of the onset of Alzheimer’s disease.

The areas of the brain that are primarily affected in the present cases may account for the development of apraxia and semantic memory deficits. The main location of lesions in lvPPA, but not naPPA or svPPA, involves parietal areas, which have been associated with various types of apraxia [ 32 ]. Primary progressive ideomotor apraxia in degenerative diseases is also considered to involve posterior parietal lesions [ 33 ]. From an anatomical point of view, lvPPA may develop into or be accompanied by apraxia during the course of the illness. The extension of the affected area to the middle and inferior temporal gyri, which was found in our cases, is thought to be related to semantic memory deficits. Recent neuroimaging studies have demonstrated that not only the anterior temporal area [ 34 ], but also the middle and inferior temporal gyri, are crucial for semantic memory [ 35 , 36 ]. The different neural substrates for semantic memory may reflect category-specific semantic systems. Although previous cases with deficits for living things often resulted from anterior temporal lobe damage [ 37 ], deficits for non-living things or man-made objects have been reported after left temporo-parietal damage [ 38 ]. From a lesion study involving lexical retrieval, words for living things are associated with the left anterior temporal lobe, whereas those for tools are associated with the left posterior temporal lobe [ 39 ]. Neuroimaging studies have also demonstrated that the left posterior middle temporal gyrus is particularly important for perceiving and knowing about tools and their functions [ 40 ]. Because apraxia is substantially related to tools, the conceptual apraxia observed in our cases may have resulted from posterior temporal lobe damage. In this context, semantic memory deficits in patients with lvPPA may involve tools more than living things.

The clinical pictures of the present cases most likely suggest Alzheimer’s disease. The primary locations of the lesions are also consistent with lesions in Alzheimer’s disease, which involve the lateral temporo-parietal areas [ 41 43 ] as well as the medial temporal and medial parietal cortex. Furthermore, recent neuropathological studies have shown that most patients with lvPPA have Alzheimer’s disease [ 3 , 44 , 45 ]. As the conditions of the patients reported here deteriorated relatively rapidly to the point that severe semantic memory deficits were noticed in their late 50s or early 60s, a type of early-onset Alzheimer’s disease [ 46 , 47 ] is suggested, which often features aphasia or apraxia rather than episodic memory deficits as an initial symptom, followed by relatively rapid deterioration.

Some limitations must be considered when interpreting the present cases. First, we did not perform brain autopsies to confirm the clinical diagnoses. Second, the observation that patients with lvPPA developed apraxia and semantic memory deficits may not be surprising, because patients with localized degeneration of parietal regions can show apraxia, and patients with Alzheimer’s disease often develop semantic memory deficits in the advanced stages. However, the unique, previously undocumented progression from lvPPA to apraxia and semantic deficits may be a typical course of deterioration in patients with lvPPA and is quite different from those with svPPA or naPPA. This information may be important in clinical practice.

In conclusion, the present case series suggested that some patients with lvPPA develop an atypical type of dementia with apraxia and semantic memory deficits, indicating that these cases could be classified as a type of early-onset Alzheimer’s disease.

Consent

Written informed consent was obtained from the patients and their family members for publication of this case series and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.

Abbreviations

lvPPA: 

Logopenic variant primary progressive aphasia

naPPA: 

Non-fluent/agrammatic variant primary progressive aphasia

svPPA: 

Semantic variant primary progressive aphasia

PPA: 

Primary progressive aphasia

MRI: 

Magnetic resonance imaging

EEG: 

Electroencephalography

Tc-99 m ECD SPECT: 

99mTc-ethylcysteinate dimer single photon emission computed tomography

eZIS: 

Easy Z score imaging system

SLTA: 

Standard Language Test of Aphasia

IMP SPECT: 

N-isopropyl-p[123I] iodoamphetamine single photon emission computed tomography

3D-SSP: 

Three-dimensional stereotactic surface projections.

Declarations

Authors’ original submitted files for images

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Competing interests

The authors declare that they have no competing interests.

Authors’ contributions

MF acquired case data, designed the study, and drafted the manuscript. YN, YY, and FY acquired case data. MM and MK supervised the study and helped to draft the manuscript. All authors read and approved the final manuscript.

Authors’ Affiliations

(1)

Department of Neuropsychiatry, Ashikaga Red Cross Hospital, Ashikaga-City, Japan
(2)

Department of Rehabilitation, Edogawa Hospital, Tokyo, Japan
(3)

Department of Neurology, Edogawa Hospital, Tokyo, Japan
(4)

Daigo Hospital, Kyoto, Japan
(5)

Department of Neuropsychiatry, Keio University School of Medicine, Tokyo, Japan

References

  1. Gorno-Tempini ML, Hillis AE, Weintraub S, Kertesz A, Mendez M, Cappa SF, Ogar JM, Rohrer JD, Black S, Boeve BF, Manes F, Dronkers NF, Vandenberghe R, Rascovsky K, Patterson K, Miller BL, Knopman DS, Hodges JR, Mesulam MM, Grossman M: Classification of primary progressive aphasia and its variants. Neurology. 2011, 76: 1006-1014. 10.1212/WNL.0b013e31821103e6. View Article PubMed PubMed Central Google Scholar
  2. Neary D, Snowden JS, Gustafson L, Passant U, Stuss D, Black S, Freedman M, Kertesz A, Robert PH, Albert M, Boone K, Miller BL, Cummings J, Benson DF: Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology. 1998, 51: 1546-1554. 10.1212/WNL.51.6.1546. View Article PubMed Google Scholar
  3. Gorono-Tempini ML, Brambati SM, Ginex V, Ogar J, Dronkers NF, Marcone A, Perani D, Garibotto V, Cappa SF, Miller BL: The logopenic/phonological variant of primary progressive aphasia. Neurology. 2008, 71: 1227-1234. 10.1212/01.wnl.0000320506.79811.da. View Article Google Scholar
  4. Caffarra P, Gardini S, Cappa S, Dieci F, Concari L, Barocoo F, Ghetti C, Ruffini L, Prati GD: Degenerative jargon aphasia: unusual progression of logopenic/phonological progressive aphasia?. Behav Neurol. 2013, 26: 89-93. View Article PubMed Google Scholar
  5. Rogalski E, Cobia D, Harrison TM, Wieneke C, Weintraub S, Mesulam M: Progression of language decline and cortical atrophy in subtypes of primary progressive aphasia. Neurology. 2011, 76: 1804-1810. 10.1212/WNL.0b013e31821ccd3c. View Article PubMed PubMed Central Google Scholar
  6. Matsuda H, Mizumura S, Nagao T, Ota T, Iizuka T, Nemoto K, Kimura M, Tateno A, Ishiwata A, Kuji I, Arai H, Homma A: An easy-Z score imaging system for discrimination between very early Alzheimer’s disease and controls using brain perfusion SPECT in a multicentre study. Nucl Med Commun. 2007, 28: 199-205. 10.1097/MNM.0b013e328013eb8b. View Article PubMed Google Scholar
  7. Hasegawa T, Kishi H, Shigeno K, Tanemura J, Kusunoki T: Three-dimensional structure in language tests of aphasia. Folia Phoniatr (Basel). 1985, 37: 246-258. 10.1159/000265805. View Article Google Scholar
  8. Sugishita M, Hemmi I, JADNI: Validity and reliability of Mini Mental State Examination-Japanese (MMSE-J): a preliminary report. Japanese J Cogn Neurosci. 2010, 12: 186-190. Google Scholar
  9. Takahashi T: The revised visual retention test (Benton visual retention test, Japanese Version). 1965, Sankyobo Kyoto: Sankyobo Google Scholar
  10. Kugo A, Terada S, Ata T, Ido Y, Kado Y, Ishihara T, Hikiji M, Fujisawa Y, Sasaki K, Kuroda S: Japanese version of the frontal assessment battery for dementia. Psychiatry Res. 2007, 153: 69-75. 10.1016/j.psychres.2006.04.004. View Article PubMed Google Scholar
  11. Shidahara M, Inoue K, Maruyama M, Watabe H, Taki Y, Goto R, Okada K, Kinomura S, Osawa S, Onishi Y, Ito H, Arai H, Fukuda H: Predicting human performance by channelized Hotelling observer in discriminating between Alzheimer’s dementia and controls using statistically processed brain perfusion SPECT. Ann Nucl Med. 2006, 20: 605-613. 10.1007/BF02984658. View Article PubMed Google Scholar
  12. Nagaratnam N, Irving J, Kalouch H: Misidentification in patients with dementia. Arch Gerontol Geriatr. 2003, 37: 195-202. 10.1016/S0167-4943(03)00045-1. View Article PubMed Google Scholar
  13. Hughes CP, Berg L, Danziger WL, Coben LA, Martin RL: A new clinical scale for the staging of dementia. Br J Psychiatry. 1982, 140: 566-572. 10.1192/bjp.140.6.566. View Article PubMed Google Scholar
  14. Leiguarda RC, Merello M, Nouzeilles MI, Balej J, Rivero A, Nogues M: Limb-kinetic apraxia in corticobasal degeneration: clinical and kinematic features. Mov Disorder. 2003, 18: 49-59. 10.1002/mds.10303. View Article Google Scholar
  15. Zadikoff C, Lang AE: Apraxia in movement disorders. Brain. 2005, 128: 1480-1497. 10.1093/brain/awh560. View Article PubMed Google Scholar
  16. Della Sala S, Lucchelli F, Spinnler H: Ideomotor apraxia in patients with dementia of Alzheimer type. J Neurol. 1987, 234: 91-93. 10.1007/BF00314108. View Article PubMed Google Scholar
  17. Ceccaldi M, Poncet M, Gambarelli D, Guinot H, Billé J: Progressive severity of left unilateral apraxia in 2 cases of Alzheimer disease. Rev Neurol (Paris). 1995, 151: 240-246. Google Scholar
  18. Giannakopoulos P, Duc M, Gold G, Hof PR, Michel JP, Bouras C: Pathologic correlates of apraxia in Alzheimer disease. Arch Neurol. 1998, 55: 689-695. 10.1001/archneur.55.5.689. View Article PubMed Google Scholar
  19. Foundas AL, Macauley BL, Raymer AM, Maher LM, Rothi LJ, Heilman KM: Ideomotor apraxia in Alzheimer disease and left hemisphere stroke: limb transitive and intransitive movements. Neuropsychiatry Neuropsychol Behav Neurol. 1999, 12: 161-166. PubMed Google Scholar
  20. Kato M, Meguro K, Sato M, Shimada Y, Yamazaki H, Saito H, Yamaguchi H, Yamadori A: Ideomotor apraxia in patients with Alzheimer disease: why do they use their body parts as objects?. Neuropsychiatry Neuropsychol Behav Neurol. 2001, 14: 45-52. PubMed Google Scholar
  21. Ochipa C, Rothi LJ, Heilman KM: Conceptual apraxia in Alzheimer’s disease. Brain. 1992, 115: 1061-1071. 10.1093/brain/115.4.1061. View Article PubMed Google Scholar
  22. Schwartz RL, Adair JC, Raymer AM, Williamson DJ, Crosson B, Rothi LJ, Nadeau SE, Heilman KM: Conceptual apraxia in probable Alzheimer’s disease as demonstrated by the Florida action recall test. J Int Neuropsychol Soc. 2000, 6: 265-270. 10.1017/S1355617700633015. View Article PubMed Google Scholar
  23. Dumont C, Ska B, Joanette Y: Conceptual apraxia and semantic memory deficit in Alzheimer’s disease; two sides of the same coin?. J Int Neuropsychol Soc. 2000, 6: 693-703. 10.1017/S1355617700666079. View Article PubMed Google Scholar
  24. Falchook AD, Mosquera DM, Finney GR, Williamson JB, Heilman K: The relationship between semantic knowledge and conceptual apraxia in Alzheimer disease. Cogn Behav Neurol. 2012, 25: 167-174. 10.1097/WNN.0b013e318274ff6a. PubMed Google Scholar
  25. Fukui T, Sugita K, Kawamura M, Shiota J, Nakano I: Primary progressive apraxia in Pick’s disease: a clinicopathologic study. Neurology. 1996, 47: 467-473. 10.1212/WNL.47.2.467. View Article PubMed Google Scholar
  26. Schumann G, Halsband U, Kassubek J, Gustin S, Heinks T, Juengling FD, Hüll M: Combined semantic dementia and apraxia in a patient with frontotemporal lobar degeneration. Psychiatry Res. 2000, 100: 21-29. 10.1016/S0925-4927(00)00065-2. View Article PubMed Google Scholar
  27. Giffard B, Desgranges B, Nore-Mary F, Lalevée C, de la Sayette V, Pasquier F, Eustache F: The nature of semantic memory deficits in Alzheimer’s disease. New insights from hyperpriming effects. Brain. 2001, 124: 1522-1532. 10.1093/brain/124.8.1522. View Article PubMed Google Scholar
  28. Giffard B, Desgranges B, Eustache F: Semantic memory disorders in Alzheimer’s disease: clues from semantic priming effects. Curr Alzheimer Res. 2005, 2: 425-434. 10.2174/156720505774330582. View Article PubMed Google Scholar
  29. Duarte LR, Syssau A, Jiménez M, Launay M, Terrier P: Deficit of access or storage: semantic memory processing in Alzheimer disease. Can J Aging. 2007, 26: 227-239. 10.3138/cja.26.3.227. View Article PubMed Google Scholar
  30. Rogers SL, Friedman RB: The underlying mechanisms of semantic memory loss in Alzheimer’s disease and semantic dementia. Neuropsychologia. 2008, 46: 12-21. 10.1016/j.neuropsychologia.2007.08.010. View Article PubMed Google Scholar
  31. Leyhe T, Saur R, Eschweiler GW, Milian M: Clock test deficits are associated with semantic memory impairment in Alzheimer disease. J Geriatri Psychiatry Neurol. 2009, 22: 235-245. 10.1177/0891988709335798. View Article Google Scholar
  32. Liepmann H: Uber Stroungen des Handelns bei Gehirnkranken. 1905, Berlin: Karger Google Scholar
  33. Buxbaum LJ, Giovannetti T, Libon D: The role of the dynamic body schema in praxis: evidence from primary progressive apraxia. Brain Cogn. 2000, 44: 166-191. 10.1006/brcg.2000.1227. View Article PubMed Google Scholar
  34. Lambon Ralph MA, Ehsan S, Baker GA, Rogers TT: Semantic memory is impaired in patients with unilateral anterior temporal lobe resection for temporal lobe epilepsy. Brain. 2012, 135: 242-248. 10.1093/brain/awr325. View Article PubMed PubMed Central Google Scholar
  35. Visser M, Embleton KV, Jefferies E, Parker GJ, Ralph MA: The inferior, anterior temporal lobes and semantic memory clarified: novel evidence from distortion-corrected fMRI. Neuropsychologia. 2010, 48: 1689-1696. 10.1016/j.neuropsychologia.2010.02.016. View Article PubMed Google Scholar
  36. Visser M, Jefferies E, Embleton KV, Lambon Ralph MA: Both the middle temporal gyrus and the ventral anterior temporal area are crucial for multimodal semantic processing: distortion-corrected fMRI evidence for a double gradient of information convergence in the temporal lobes. J Cogn Neurosci. 2012, 24: 1766-1778. 10.1162/jocn_a_00244. View Article PubMed Google Scholar
  37. Lambon Ralph MA, Lowe C, Rogers TT: Neural basis of category-specific semantic deficits for living things: evidence from semantic dementia, HSVE and a neural network model. Brain. 2007, 130: 1127-1137. View Article PubMed Google Scholar
  38. Gainotti G: What the locus of brain lesion tells us about the nature of the cognitive defect underlying category-specific disorders: a review. Cortex. 2000, 36: 539-559. 10.1016/S0010-9452(08)70537-9. View Article PubMed Google Scholar
  39. Damasio H, Grabowski TJ, Tranel D, Hichwa RD, Damasio AR: A neural basis for lexical retrieval. Nature. 1996, 380: 499-505. 10.1038/380499a0. View Article PubMed Google Scholar
  40. Beauchamp MS, Martin A: Grounding object concepts in perception and action: evidence from fMRI studies of tools. Cortex. 2007, 43: 461-468. 10.1016/S0010-9452(08)70470-2. View Article PubMed Google Scholar
  41. Fukuyama H, Harada K, Yamauchi H, Miyoshi T, Yamaguchi S, Kimura J, Kameyama M, Senda M, Yonekura Y, Konishi J: Coronal reconstruction images of glucose metabolism in Alzheimer’s disease. J Neurol Sci. 1991, 106: 128-134. 10.1016/0022-510X(91)90249-7. View Article PubMed Google Scholar
  42. Rabinovici GD, Seeley WW, Kim EJ, Gorno-Tempini ML, Rascovsky K, Pagliaro TA, Allison SC, Halabi C, Kramer JH, Johnson JK, Weiner MW, Forman MS, Trojanowski JQ, Dearmond SJ, Miller BL, Rosen HJ: Distinct MRI atrophy patterns in autopsy-proven Alzheimer’s disease and frontotemporal lobar degeneration. Am J Alzheimers Dis Other Demen. 2007, 22: 474-488. View Article PubMed PubMed Central Google Scholar
  43. Buckner RL, Sepulcre J, Talukdar T, Krienen FM, Liu H, Hedden T, Andrews-Hanna JR, Sperling RA, Johnson KA: Cortical hubs revealed by intrinsic functional connectivity: mapping, assessment of stability, and relation to Alzheimer’s disease. J Neurosci. 2009, 29: 1860-1873. 10.1523/JNEUROSCI.5062-08.2009. View Article PubMed PubMed Central Google Scholar
  44. Leyton CE, Villemagne VL, Savage S, Pike KE, Ballard KJ, Piguet O, Burrell JR, Rowe CC, Hodges JR: Subtypes of progressive aphasia: application of the international consensus criteria and validation using β-amyloid imaging. Brain. 2011, 134: 3030-3043. 10.1093/brain/awr216. View Article PubMed Google Scholar
  45. Ahmed S, de Jager CA, Haigh AMF, Garrard P: Logopenic aphasia in Alzheimer’s disease: clinical variant or clinical feature. J Neurol Neurosurg Psychiatry. 2012, 83: 1056-1062. 10.1136/jnnp-2012-302798. View Article PubMed Google Scholar
  46. Boller F, Forette F, Khachaturian Z, Poncet M, Christen Y: Heterogeneity of Alzheimer’s disease. 1992, Berlin, New York: Springer-Verlag View Article Google Scholar
  47. Yesavage JA, Brooks III JO, Taylor J, Tinklenberg J: Development of aphasia, apraxia, and agnosia and decline in Alzheimer’s disease. Am J Psychiatry. 1993, 150: 742-747. View Article PubMed Google Scholar
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© Funayama et al.; licensee BioMed Central Ltd. 2013

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Contents

  • Semantic Variant Primary Progressive Aphasia (Semantic Dementia)

  • Nonfluent/Agrammatic Primary Progressive Aphasia (Progressive Non-fluent Aphasia)

  • Logopenic variant of Primary Progressive Aphasia (Logopenic Progressive Aphasia)

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  • Logopenic variant of Primary Progressive Aphasia (Logopenic Progressive Aphasia)

Logopenic variant of Primary Progressive Aphasia (Logopenic Progressive Aphasia)

Overview

There are several forms of aphasia, or a disorder of language, that can gradually worsen over time.  These are not due to head trauma, stroke, cancer or other observable secondary changes in the brain, and hence appear to be intrinsic disorders of brain functioning – as such, we refer to this class of conditions as “primary.” 

Clinical Features of the Logopenic Variant of Primary Progressive Aphasia (lvPPA)

  • Impaired repetition
  • Naming difficulty

Several primary features characterize the logopenic variant of Primary Progressive Aphasia (PPA).  One characteristic feature is profound difficulty with repetition and very short-term memory.  Thus, these individuals have difficulty repeating brief phrases, and have difficulty repeating a short sequence of numbers.  Individuals with the logopenic variant of PPA also have difficulty with naming objects.  Word comprehension tends to be relatively preserved, and the naming deficit in logopenic variant PPA appears to be due instead to difficulty retrieving the lexical form of the word. 

Other Clinical Features of the Logopenic Variant of Primary Progressive Aphasia

In addition to difficulty with repetition and naming, individuals with the logopenic variant of PPA can have other deficits.  Among these may be difficulty with fluent speech, or impaired comprehension of single words (although comprehension of objects is well preserved).  Because of this variability, this among the most difficulty of PPA conditions to diagnose. 

Over time, many individuals with the logopenic variant of PPA can develop a disorder of episodic memory.  Due to difficulty with learning and remembering, individuals can repeat themselves in the course of a conversation, ask the same question repeatedly during the course of a day, and forget events and conversation that recently occurred.  Disorientation in place and time can emerge. 

MRI Studies of the Logopenic Variant of Primary Progressive Aphasia

Detailed MRI studies of the logopenic variant of PPA show subtle atrophy in the core language regions of the left hemisphere.  This includes the frontal lobe, parietal lobe, and temporal lobe.  Over time, the atrophy can involve the hippocampus, an area deep in the temporal lobe that is important for memory.

Causes of the Logopenic Variant of Primary Progressive Aphasia

In about 70% of individuals with the logopenic variant of PPA, inspection of the brain at autopsy reveals the accumulation of amyloid plaques and tau neurofibrillary tangles.  These are the microscopic characteristics of Alzheimer’s disease.  These individuals with the logopenic variant of PPA thus can be thought to have an unusual variant of Alzheimer’s disease that begins by affecting the language regions of the brain.  Eventually, as the condition progresses, it may begin to affect the memory regions that are typically involved in most cases of Alzheimer’s disease.  In the remaining 30% of cases, microscopic inspection of the brain reveals an accumulation of tau inclusions in the brain cells that are often seen in the non-fluent/agrammatic variant of PPA. 

Cerebrospinal Fluid Studies to help diagnose the Logopenic Variant of Primary Progressive Aphasia

It is possible to identify the cause of the logopenic variant of PPA by inspection of the cerebrospinal fluid (CSF).  This is the fluid that bathes the brain and spinal cord, and thus contains some of the proteins that are found in the brain.  Rather than performing a brain biopsy to study the brain during life, a lumbar puncture (also known as a spinal tap) can be performed. 

Treatments for the Logopenic Variant of Primary Progressive Aphasia

There are two kinds of treatments available for individuals with the logopenic variant of PPA.  First, medication treatments can be borrowed from among those available for Alzheimer’s disease.  These may help slow down the decline in memory and language.  Second, individuals with the logopenic variant of PPA can take advantage of behavioral and speech therapies and improve their own naming and comprehension skills through practice in normal conversations.  Some individuals benefit from a picture dictionary.  Communication efficacy is enhanced when conversing in a familiar environment at a slow speech rate, supplemented by gestures.  Computer devices can hold prerecorded phrases and sentences that the patient can use to assist communication.  Memory can be supplemented by developing the habit of writing down key appointments in a calendar book. 

In addition to performing more detailed studies to characterize patients with the logopenic variant of PPA, scientists at the Penn FTD Center have developed techniques to study the CSF, perform detailed analyses of MRI scans, and to relate language, MRI and CSF characteristics together to provide an accurate diagnosis of patients with PPA so that the most effective treatments can be applied. 

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