"A lady of few words": Review of literature and report of a case of progressive nonfluent aphasia type of frontotemporal dementia

Thomas Gregor Issac; Sadanandavalli Retnaswami Chandra

doi:10.4103/2348-9995.195675

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CASE REPORT

Year : 2016 | Volume : 3 | Issue : 2 | Page : 164-167

"A lady of few words": Review of literature and report of a case of progressive nonfluent aphasia type of frontotemporal dementia

Thomas Gregor Issac¹, Sadanandavalli Retnaswami Chandra²
¹ Department of Clinical Neurosciences, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India
² Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India

Date of Web Publication

13-Dec-2016

Correspondence Address:
Thomas Gregor Issac
No. 13, New Kabini Hostel Complex, National Institute of Mental Health and Neurosciences, Bengaluru - 560 029, Karnataka
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/2348-9995.195675

Abstract

Frontotemporal dementia (FTD) is a clinically and pathologically heterogeneous syndrome. It can be classified into three clinical syndromes depending on the early and predominant symptoms: A behavioral variant FTD and two language variants namely, semantic dementia, and progressive nonfluent aphasia (PNFA) which are very rare and elude diagnosis. We report the case of an elderly homemaker who came to our institute with features of cognitive decline and behavioral problems with language deficits in the form of nonfluent speech, impaired vocabulary to three pairs of words, agrammatism, and impaired single sentence comprehension with corroborative magnetic resonance imaging findings. PNFA is a rare clinical variant of FTD and often underdiagnosed. It should be considered in elderly patients who apart from having cognitive decline, behavioral problems, and absent insight also develop limited vocabulary, especially using a set of nouns to express themselves. Speech therapy and behavioral therapy in the initial stages can be of utility.

Keywords: Frontotemporal dementia, language, progressive nonfluent aphasia

How to cite this article:
Issac TG, Chandra SR. "A lady of few words": Review of literature and report of a case of progressive nonfluent aphasia type of frontotemporal dementia. J Geriatr Ment Health 2016;3:164-7

How to cite this URL:
Issac TG, Chandra SR. "A lady of few words": Review of literature and report of a case of progressive nonfluent aphasia type of frontotemporal dementia. J Geriatr Ment Health [serial online] 2016 [cited 2023 Jun 4];3:164-7. Available from: https://www.jgmh.org/text.asp?2016/3/2/164/195675

Introduction

Frontotemporal dementia (FTD) is a clinically and pathologically heterogeneous syndrome under 65 years of age with an estimated point prevalence is 15-22/100,000 and incidence of 2.7-4.1/100,000. ^[1] Original cases were described by Alzheimer and Pick more than a century ago and later found to have tau-positive inclusions. ^[2] FTD can be classified into three clinical syndromes depending on the early and predominant symptoms: A behavioral variant FTD (bvFTD) and two other rarer variants namely, semantic dementia and progressive nonfluent aphasia (SD and PNFA). The most common form of FTD is the behavioral variant which is characterized by insidious onset of personality changes, behavioral abnormalities, and poor insight. ^[3],[4] Orbitobasal involvement leads to some of the primary symptoms encountered in this disorder. These include disinhibition, poor impulse control, antisocial behavior, anger episodes, and varied stereotypies. Examples of stereotypical, or ritualized behaviors, include insisting on eating the same food at exactly the same time daily, organic orderliness like in arranging articles in particular places unlike in the premorbid self, cleaning the house in precisely the same order, or simple repetitive behaviors such as foot-tapping. Ritualized acts may also include the use of a "catch-phrase" and a change in food preference in terms of preference for sweets. A patient's decline in social conduct can include breaches of interpersonal etiquette and tactlessness. Disinhibition can often lead to verbally inappropriate sexual comments and gestures which are discomforting to the caregiver along with social incontinence. Echolalia and perseveration may be present. ^[5] The most common cognitive deficit in bvFTD is an impairment of executive function or working memory, which is indicative of frontal and prefrontal cortex involvement. Other frequently encountered cognitive abnormalities include attentional deficits, poor abstraction, difficulty shifting mental set, and perseveration. Orbitofrontal atrophy seen in FTD patients correlates with impulsivity and disinhibition. Involvement of the medial frontal cortex is mainly associated with response inhibition and obsessive behaviors. ^[3],[4]

Behavioral disturbances are very rarely described in language variants of FTD especially PNFA as compared to SD in literature. These patients demonstrate a profound impairment for semantic knowledge due to atrophy of the left temporal lobe. Patients lose the ability to name and understand words and to recognize the significance of faces, objects, and other sensory stimuli. Working memory and autobiographical memory, at least for the recent past, tend to be preserved. ^[4],[5]

Patients with PNFA often are females and present with decreased verbal output, slow effortful speech, impaired production and comprehension of grammar (agrammatism), and motor speech deficits with retained comprehension for single words and often normal reading skills unlike other variants of FTD. Unlike the behavioral variant wherein patients had early disengagement with poor insight in majority, patients with PNFA had more of speech difficulties with noticeable decreased verbal output and decreased ability in language processing with more of gestural communication. The neuroanatomical correlates are also significantly different in the various clinical forms and contribute to the varied symptomatology in the initial stages of the disease. In patients with PNFA type of FTD disease, the left ventral inferior frontal cortex is affected, resulting in impaired grammatical processing. ^[4],[5],[6] PNFA patients usually present with apraxia of speech with or without expressive agrammatism. The single-word comprehension and object knowledge are relatively preserved; fluency is impaired and often there is phonological dyslexia whereas behavioral symptoms are less common. ^[7],[8] This type of FTD is mainly a tauopathy though association with trans DNA binding protein aberration is also reported. ^[9],[10] These conditions are seldom evaluated in detail but a proper assessment of these conditions will reveal more about the physiology of speech production and regression during development as most of the speech is learnt in childhood following imitation and repetition of others orofacial movements and sound production with available vocabulary though grammatical and phonemic corrections come later on. Change in the language abilities can help in clinically identifying and delineating the particular variant of dementia and determining the structural correlates as well.

Case Report

A 62-year-old homemaker came to our institute with features of cognitive decline and behavioral problems for the past 1½ year duration. Her illness started as becoming aloof and showing disinterest in daily household activities. There was decreased interest in cooking and interacting with her family members especially grandchildren to whom she was previously very much attached. Later her relatives noticed that she became easily irritable with occasional anger outbursts for trivial issues, unlike her premorbid self. Gradually, she started repeating questions and could not recognize close relatives and neighbors. In spite of this, she was able to dress, bath and navigate inside her house without any confusion. Gradually she started having decreased verbal output often resorting to gestures. Her vocabulary dwindled and she started using same pair of words again and again. Her vocabulary became restricted to the following six words "mud-water," "chair-table," and "inside-outside" which she would interchangeably use for whatever asked to her including these terms also as responses to tell her name and address. It was also noted that for any reply she would use a combination of a pair of these words only. After 9 months of onset of her illness, she started showing disinhibition in terms of improperly dressing, using vulgar terms in public, showing stimulus-bound behaviour in terms of wearing a blouse over the one she was wearing and utilizing crayons, pens and paper sheets belonging to her grandchildren, tendency to wander, emotional incontinence and became incontinent to both urine and stools without any regret for the same. Her appetite also increased with a preference for carbohydrate rich food items and sweets (food faddism) and occasionally drinking sunflower oil. She was evaluated in local hospital and diagnosed as bipolar disorder and managed conservatively with antipsychotics and benzodiazepines without any significant improvement. Even though the patient had initial features of apathy and disinterest, the features of depression was ruled out clinically by the absence of features of hopelessness, worthlessness, and guilt. There were no features of terminal insomnia rather the patient had interrupted sleep. Her insight into the illness was very poor. Gradually, she became incontinent for both stool and urine with loss of language faculties suggestive of organicity rather than depression. There was also no history for attempts of self-harm elicited from the bystanders. Her routine blood investigations such as hemoglobin, total, and differential counts, platelet counts and red cell indices were within normal limits and specific investigations to rule out acquired, and reversible causes of dementia such as estimation of Vitamin B12 levels, thyroid profile, HIV/venereal disease research laboratory status were noncontributory. Neuropsychological profiling showed abnormality in the domains of attention (poor performance in digit forward and backward tests and color trails), working memory (poor performance in color trails 1 and 2), verbal learning and memory (as assessed by Auditory Verbal and Learning test) as well as inability in response inhibition and error correction (Stroop test, color trails 2 and Wisconsin Card Sorting test) suggesting involvement of the frontal and temporal lobe structures. Constructional apraxias and dressing apraxias were not observed indicating sparing of the parietal lobes. Magnetic resonance imaging demonstrated atrophy of the inferior frontal and temporal lobes with asymmetrical widening of the left sylvian fissure, and knife cut atrophy of the anterior temporal and inferior frontal gyrus indicating PNFA-FTD [Figure 1]a-c. She was started on memantine, fluoxetine and symptomatic management for incontinence with tolterodine. Fluoxetine was added in this case because there is evidence that patients with FTD show serotoninergic deficits which appears largely to be related to postsynaptic defects although rarely presynaptic deficits have also been reported. ^[10] Selective serotonin reuptake inhibitors (SSRIs) have been used with some success in many cases. ^[11] SSRIs have been used to treat compulsions, ritualistic behaviors, carbohydrate cravings, anxiety, agitation, and behavioral symptoms in patients with FTD. ^[11] Nonpharmacological approach consisted of patient and caregiver education though the patient had very poor insight. Furthermore, the caregiver was advised regarding maintenance of sleep hygiene, strict control of dietary sugars along with some yoga exercises and meditation specifically designed for such patients which was also provided. This consisted more of pranayama and relaxation exercises. Speech therapy and behavioral therapy was also initiated. She is currently continent with decreased agitation and anger, but her vocabulary still remains the same after a year of follow-up. Speech therapy is being continued but the vocabulary has not increased. Neuropsychological assessment after 1 year revealed mild improvement in attention (as evidenced by better performance in digit forward test) but no improvement in other domains. Parietal lobe functions seemed to remain unaltered at this point.

Figure 1: Magnetic resonance imaging demonstrating. (a) Dilated temporal horn, left > right (b) Knife cut atrophy of anterior temporal lobe with perisylvian atrophy. (c) Lateral and medial temporal atrophy

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Discussion and conclusion

Even though PNFA is seen in only 20% of the patients with FTD, it often begins by deficits in speech or language and social decorum is almost always intact with some patients progressing to bvFTD. ^[3],[4] The words used interchangeably in pairs for communication by the patient are also only nouns and pronouns whereas verbs are completely omitted. Speech and language assessment revealed impaired fluency, articulatory errors, agrammatism with very simple sentence or word comprehension but impaired complex sentences, abnormal sentence repetition, and phonological dyslexia indicating her regression of vocabulary skills as well. Our patient thus had breakdown of syntactic and phonological components of language with nonfluent speech characteristic of PNFA-FTD similar to that described in literature. ^[12] Only few cases have been described especially from the Indian subcontinent. ^[13],[14] A patient with progressive speech difficulty, naming difficulty, and poor comprehension was described in Indian literature. ^[12],[14] Neologistic jargon aphasia and agraphia is also described in two patients with primary progressive aphasia variant of FTD. ^[13],[14] Unlike in cases reported in world literature our case is unique in the fact that apart from other typical cases of PNFA which will usually begin with nonfluent aphasia, agrammatism related errors, phonemic paraphasias, and rarely anomias with associated stuttering or oral apraxias, impaired repetition, alexia, agraphia, early preservation of word meaning and late mutism, the index case had initial loss of word output, relative mutism with loss of vocabulary evidenced by restriction of vocabulary to only three pairs of words and later behavioral changes. ^[14] A second such similar case was seen by the authors recently. Thus, the hitherto rare but not uncommon occurrence of PNFA has to be considered in elderly patients especially females who apart from having cognitive decline, behavioral problems, and absent insight also develop limited vocabulary, especially using a set of nouns to express themselves. Speech therapy can be of significant initial use, but as the condition worsens, use of appropriate gestures for communication or sign language would be useful.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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