International Journal of Health Sciences and Research Vol.10; Issue: 12; December 2020 Website: www.ijhsr.org Case Report ISSN: 2249-9571

Palilalia, Paligraphia and Progressive Non fluent in a patient with Progressive Supranuclear Palsy

Vandana.V.P

Additional Professor, Department of Speech Pathology and Audiology, G-14, OPD Block, National Institute of Mental Health and Neurosciences, Bangalore 560029

ABSTRACT

Recent studies have demonstrated that a subgroup of patients with progressive supranuclear palsy (PSP) may be associated with progressive apraxia of speech (AOS), nonfluent aphasia (PNFA), or both. We describe the cognitive-linguistic characteristics of patient with progressive supranuclear palsy with co-occurring progressive non fluent aphasia, palilalia, paligraphia and obsessive compulsive disorder symptoms. He presented with history of behavioral disturbance for six years and progressive difficulty in speaking for the last two years. Behavioral abnormalities were manifested in the form of aggressiveness, suspicion towards wife, using abusive language, apathy, withdrawn behavior, impairment in short term memory, sleep disturbances, tactile hallucinations and repetition of the same action. Spontaneous speech was characterized by significant palilalia and writing was characterized by paligraphia which had worsened over the last two years. Auditory comprehension and naming was good. He also demonstrated extrapyramidal features and mild bradykinesia.

Key words: Progressive supranuclear palsy, Progressive nonfluent aphasia, Palilalia, Paligraphia

INTRODUCTION patients with advanced Parkinson’s disease There have been few descriptions of [10]. Palilalia is generally associated with the association of progressive supranuclear bilateral lesion and palsy (PSP) with progressive nonfluent Parkinson’s disease [12], aphasia (PNFA) and apraxia of speech [1-5]. [13,14] , progressive supranuclear palsy [15,16], Karnik et al [6] described a 64-year-old right hemisphere damage [17]. Literature also woman with frontotemporal lobar reports of neuroleptic-induced palilalia degeneration, progressive non-fluent (Garcia, 1990). There is sparse literature on aphasia, obsessive compulsive disorder the progression of speech and language (OCD) and progressive supranuclear palsy. features in PSP patients having PNFA [19]. There is no reported evidence of the The co-occurrence of progressive occurrence of palilalia and paligraphia with supranuclear palsy, nonfluent aphasia, PSP. Critchley [7] was the first to report OCD, palilalia and paligraphia is rare and palilalia in five patients with post we report such a case highlighting the encephalitic Parkinsonism, one patient with speech-language findings. pseudobulbar palsy and in another patient with emotional lability. Palilalia and CASE HISTORY paligraphia are also reported in patients post Mr. MR. is a 51-year old male with road traffic accident and following history of behavioral disturbances and cerebrovascular accident [8-10]. It was also progressive difficulty in speaking since 49 reported in a patient with idiopathic years of age. Behavioral issues started as Parkinsons disease [11] and in 15 out of 24 aggressiveness, and suspicion and of his

International Journal of Health Sciences and Research (www.ijhsr.org) 122 Vol.10; Issue: 12; December 2020 Vandana.V.P. Paligraphia and progressive non fluent aphasia in a patient with progressive supranuclear palsy. wife. He was apathic and withdrawn for the in writing, reading, language and behavioral last two years. He also had progressive issues lead to loss of job, reduced social difficulty in speech characterized by interaction and inability to participate in compulsive repetition of syllables and leisure activities. The onset and course of words and subtle difficulty in word illness was ascertained from his brother with retrieval. Writing was characterized by whom he was currently staying. paligraphia and micrographia. Since 5 months prior to admission his sibling Evaluation noticed impaired attention, repeating same Neurological examination six years action or words, difficulty in remembering after onset of behavioral issues and 2 years recent events, inappropriate smiling, after onset of speech problem revealed muttering, compulsive face washing and relatively young onset speech disturbance, bathing. He started eating at normal rate but perseveration, palilalia, naming impairment, later on ate very fast and often spilled food. relatively preserved comprehension, He also presented with altered sleep wake delusion, aggression, emotional lability, cycle and singing, laughing and muttering to apathy, extrapyramidal features and mild self. He had slowness of movements, bradykinesia. Fig 1. represents writing reduced facial expressions and mild tremors sample of the patient demonstrating in hands since last six months. Difficulties Paligraphia and micrographia

Fig 1: writing sample of the patient with paligraphia and micrographia

Basic ADLs were good, but disorder with progressive nonfluent aphasia instrumental activities of daily living (PNFA) variant of frontotemporal dementia. comprising of telephone usage, managing It was opined that it may persist as PNFA or money, going out independently and leisure may evolve into progressive supranuclear time activities were affected. The UPDRS palsy with or without apraxia of speech. III Speech score in the medication OFF stage was 3, characterized by marked Speech and language evaluation impairment and difficulty to understand Speech and language manifestations speech. consisted of a progressive course in the last There was no apraxia (verbal or 3 months as described by the patient and his nonverbal). He had impaired fronto brother. On examination the patient temporal functions, severe impairment of presented with compulsive repetitions of response inhibition with significant speech syllables (30 to 40 times and sometimes disturbance. MRI showed significant frontal more than 100 reiterations) in spontaneous and anterior temporal atrophy with more speech, naming and responsive speech asymmetry on left side. It also indicated (propositional tasks) with occasional perisylvian, cingulate asymmetry with L>R repetition of words and phrases. Reiterations atrophy consistent with PNFA. Minimental were also seen while counting, reciting state score was 23/30. The patient was names of months and to a lesser extent diagnosed as progressive extrapyramidal while reciting prayer (non propositional

International Journal of Health Sciences and Research (www.ijhsr.org) 123 Vol.10; Issue: 12; December 2020 Vandana.V.P. Paligraphia and progressive non fluent aphasia in a patient with progressive supranuclear palsy. tasks). It was accompanied by reduced palilalia (Figure 2). The speech samples loudness and increased rate and were were audio recorded using a Sony MZ-55 reduced when the patient was less anxious digital recorder and were transferred to and when attention was focused on speech. Computerized Speech Laboratory (CSL- The disfluencies were more characteristic of 4500) at a sampling rate of 44.1 kHz.

Figure 2: Spectrogram of the word /kurangan/ .

The word was completed in a total significantly more disfluent than oral of 23 seconds. There were 9 syllable trains reading, repetition and singing. He could each with approximately 12 reiterations of read fairly well without much dysfluencies. phoneme /k/ before producing the complete He had occasional . Writing was word (Total 108 reiterations in 20 seconds). characterized by similar errors as in It is noticeable that the first syllable train of speaking with involuntary repetitions and /k/ was for 2 sec, 2nd to 8th syllable train of difficulty in terminating writing (paligraphia /k/ was for 1.5 seconds, 9th syllable train and micrographia), with repetitive writing of was for 1 sec. The complete word was the first syllable of the word, word and uttered in 4 seconds (between the 23rd and phrase reiterations. He had fair semantic 27th second). association scores in the Indian semantic The percentage of reiterations in a battery (26 on 42), good naming in the 256 word passage in reading was 35.56% picture naming test (38/40). He had good (91 on 256) and in sentence repetition was category sorting abilities, but performed 20.31% (52 on 256). The reiterations were poor on category fluency. His poor category the same in the medication ON and OFF fluency performance however has to be states. No family history of was interpreted in view of his severe palilalia. reported. As a result of his increasingly In the Western Aphasia Battery unintelligible speech Mr.M.R avoided (WAB; Kertesz, 1980) he had a score of 7 in conversations and other social interactions information content (correct responses to 4 and the speech difficulty had rendered him of 6 items), 2 in fluency (single words and unable to continue his job. effortful, agrammatic and telegraphic).He No significant dysarthria or scored 54 on 60 for yes/no questions and swallowing difficulty was reported. There auditory word recognition, 30 on 80 for were no inconsistent responses, phoneme sequential commands, 42 on 100 for distortions or metathesis suggestive of repetition, and 59 on 100 for naming. His apraxia of speech, except for minimal aphasia quotient was 6.9 and cortical groping while saying multisyllabic quotient was 13.8. These scores were utterances. Spontaneous speech was indicative of transcortical motor aphasia. In

International Journal of Health Sciences and Research (www.ijhsr.org) 124 Vol.10; Issue: 12; December 2020 Vandana.V.P. Paligraphia and progressive non fluent aphasia in a patient with progressive supranuclear palsy.

March 2017, his speech and language DISCUSSION deficits worsened with clear signs of We report the clinical findings of a nonfluent progressive aphasia, mild case who presented with features of agrammatism in reading, difficulties in nonfluent aphasia, palilalia, paligraphia and writing and repetition. He made errors obsessive-compulsive disorder (OCD). (distortions, metathesis etc) while repeating Expressive language was agrammatic, longer sentences. He also exhibited deficits auditory comprehension and naming was in understanding complex grammatical good and he had relatively preserved sentences. semantic and cognitive functions. The The oral peripheral mechanism patient was classified as progressive evaluation based on Frenchay Dysarthria nonfluent aphasia according to the FTLD Assessment [20], revealed no difficulty in international criteria [21]. The patient also reflex, respiration, lip, jaw and palatal had minimal hypokinetic dysarthria. The function. He had only limited change in pathological features were suggestive of volume. His voice deteriorated with the progressive supranuclear palsy (PSP).There length of speech. The movement of lips in is very little information in the literature conversation was minimal with poor about the evolution of extrapyramidal acoustic representation. The findings were features in PSP, especially when it presents suggestive of mild hypokinetic dysarthria. initially as PNFA. Based on the above observations and Our patient presented with findings he was diagnosed to have palilalia, behavioral issues and progressive difficulty paligraphia and progressive nonfluent in language expression with characteristic aphasia secondary to progressive features of progressive nonfluent aphasia supranuclear palsy. He was advised to (PNFA). The association of PSP and PNFA undergo neuropsychological evaluation, has been reported by Boeve et al. [1] and cognitive-linguistic stimulation, monitoring Mochizuki et al. [2]. Boeve et al. [1] reported and pacing of speech. He was also of 71-year-old man with anomia and counselled regarding the use of alternative grammatical errors. He initially had and augmentative communication. The symptoms of Parkinsonism and AOS. His patient was advised speech therapy and he language deficits and AOS worsened after 3 attended the same twice weekly for 2 weeks. years and pathological examination The goals were to reduce his speech rate and confirmed PSP. They noticed that he did not reduce the number of palilalic utterances have any typical features of PSP. Mochizuki using pacing board and hand tapping et al. [2] reported of a 64-year-old man with techniques. Though these external cues were reduced expressive language and preserved useful for few seconds, speech coordination auditory comprehension in the absence of could not be maintained for spontaneous PSP or Parkinsonian symptoms. He had speech, it was characterized by significant paraphasias at 67 years, and dysphagia and palilalia. Hence therapy was refocused on postural instability at 73 years. Pathological improving his functional speech by using examination later revealed PSP. Karnik et alternative and augmentative modes for al. [6] reported of a 64-year-old woman with word and sentence level communication OCD and effortful speech who was later (text-to-speech) and communication confirmed to have PSP. Josephs et al. strategies for care giver. He was advised by (2006) reported that 10% of patients with the neurologist to undergo repetitive PSP had associated AOS ad PNFA in their transcranial stimulation for reducing the cohort of 100 autopsy-confirmed cases. palilalia. They opined that the extrapyramidal features may be seen in the later stages of PSP and may not be as severe as in typical PSP. Dysarthria (hypokinetic) was minimal

International Journal of Health Sciences and Research (www.ijhsr.org) 125 Vol.10; Issue: 12; December 2020 Vandana.V.P. Paligraphia and progressive non fluent aphasia in a patient with progressive supranuclear palsy. in our patient along with mild underlying mechanisms of palilalia. The extrapyramidal symptoms. motor hypothesis has equated the Palilalia can be described as reiterations to freezing episodes seen in involuntary repetitions of phonemes, Parkinsons disease. They said that both the syllables, words or phrases just uttered by disorders have similar characteristics with the patient. Our patient had reiterations difficulty in inhibiting or shifting from one ranged from 56 to 108 phoneme and movement to next [25]. Freezing is also syllable repetitions in spontaneous speech. accompanied by an increase in frequency Palilalia may also be characterized by and reduced amplitude as movement reduced volume and intelligibility towards progresses [26]. Cognitive hypothesis for the end of repetitions in a repetition train reiterations by Levelt [27] and Ackermann and may even become aphonic (palilalie and Ziegler [28] claimed a deficit at aphone). Some studies have reported an prearticulatory level. The fact that the increasing rate of repetition of words and iterations are more in propositional than phrases [22] , akin to the festinating gait in nonpropositional speech and the frequent patients with parkinson’s disease [23]. John association of word finding difficulties with Van Borsel et al. [11] opined that the reiterations (with reiterations acting as durational variability in palilalia may be fillers till the complex word is uttered) and indicative of a variable motor program. increase in reiterations with increased They reported more fade-in repetitions linguistic demands are supportive of the (repetition of part of word or phrase before deficit at the prearticulatory level. Speech uttering the word or phrase; suggestive of characteristics in our patient showed difficulty in initiation of speech) in involvement at the motor as well as automatic speech tasks and fade-out cognitive level. reiterations (suggestive of speech inhibition Alfredo Ardila et al. [9] noticed problems) in propositional speech in their speech task based differences in the severity patients with motor vehicle accident and of palilalia, with palilalia being present in cerebrovascular disease. Kent et al. [24] also spontaneous speech alone compared to reported fade-in repetitions in their patient verbal fluency and naming tasks. They with palilalia. The findings for our patient discussed that this may be due to the are suggestive of problems with initiation different brain regions underlying the and progression of speech as is evident from production of these tasks; with spontaneous the fade-in repetitions, which was more speech being modulated mainly by left pronounced in propositional tasks. No actual frontal areas [29] and verbal fluency and durational measurements were made in our naming by left temporal areas [30]. There are study, but perceptually an increasing rate of mixed opinions regarding the effect of reiterations could be perceived. Kent et al. propositional and nonpropositional speech [24] opined that among the subgroups of on severity of palilalia. Kent et al. [24] patients with palilalia, an increasing rate proposed a clear distinction by stating that may be indicative of extrapyramidal propositional speech (conversation, naming) involvement, a constant rate suggestive of is affected more severely than an invariant motor program and temporal nonpropositional speech (reading aloud, variability and ‘expanded structure reciting verse or prayer, telling days of repetition’ or repetition of progressively week, months of year, letters of alphabet, larger units indicative of involvement of counting). Benke and Butterworth [31] stated new motor instructions. MRI of our patient that palilalia was similar in both tasks. was suggestive of extrapyramidal and Spontaneous speech was spared in patients frontotemporal involvement. with palilalia [12], whereas repetition was A motor and cognitive hypothesis affected. In our patient, the reiterations were has been proposed to understand the

International Journal of Health Sciences and Research (www.ijhsr.org) 126 Vol.10; Issue: 12; December 2020 Vandana.V.P. Paligraphia and progressive non fluent aphasia in a patient with progressive supranuclear palsy. seen more in propositional than automatic 11 out of 18 people with nonfluent PPA and speech tasks. AOS [36]. There have been varying contentions with respect to the locus of reiteration and Management effect of phonological factors on palilalia. Management strategies included Lebrun [32] reported that patients with caregiver education regarding the evolving palilalia repeated words and phrases as disease process and functional deficits and opposed to phoneme and syllable repetition the associated neurological, in developmental stutterers. Van Borsel, neuropsychiatric and speech-language and Van Coster, & Van Lierde [33] found that cognitive decline. They were also explained words and phrases in the final position were about communication strategies, cognitive- more often repeated in contradistinction to linguistic stimulation and pacing for the dysfluencies seen in patients with reducing speech rate. They were also developmental stuttering. In contrast, advised regarding low technology Lapointe and Horner [34] reported that 55% alternative and augmentative of reiterations were seen in the beginning of communication (AAC) (communication a sentence. They also reported differences in books, communication boards and cards, palilalia severity based on phonological pantomime and gesture), and other high- environment with words starting with tech AAC devices (text-to-speech software). consonant clusters, plosives, affricates and They were also advised about informing labiodentals most affected and those with relatives and close friends regarding vowels and linguadentals least affected. The providing enough time to the patient for reiterations were mainly phoneme and communication. syllable repetitions in our patient and were seen at any loci of the sentence. Reiterations CONCLUSION were seen irrespective of the type of vowels The present study was undertaken to or consonants. describe the characteristics of palilalia and Palilalia co-occurred with paligraphia in a patient with PNFA and PSP. paligraphia in our patient. The patterns of The findings of the present study suggest reiterations were similar in spoken as well that it may be more appropriate to define as written language. Ardila et al. [9] reported palilalia as reiterations that are indicative of a case of severe palilalia with transient problems with the initiation of words and paligraphia and speculated that the co- phrases. It also indicates that the severity of occurrence of both symptoms may be palilalia may vary with respect to indicative of a broader disorder than proportionality and length of the utterances. peripheral speech defect alone. Palilalia was The results have to be viewed by keeping in more pronounced than paligraphiain their mind that palilalia can be subjected to patient and there was no paligraphia after increased interindividual variability. It is three weeks. also not yet clear if palilalia shows a Our patient did not have AOS or constant pattern over time. Changes in the phonological errors, but did show pattern of palilalia under the influence of distortions and metathetic errors with medication also needs to be investigated. increased length of the sentence in the repetition task. Increasing errors with REFERENCES sentence length may be ascribed to the 1. Boeve B, Dickson D, Duffy J, et al. demands of auditory processing on the Progressive nonfluent aphasia and sensorimotor aspects of speech motor subsequent aphasic dementia associated control in patients with AOS [35]. Mr.MR with atypical progressive supranuclearpalsy pathology. Eur Neurol. 2003; 49:72–78. had minimal hypokinetic dysarthria. 2. Mochizuki A, Ueda Y, Komatsuzaki Y, et Hypokinetic dysarthria was reported only in al. Progressive supranuclear palsy

International Journal of Health Sciences and Research (www.ijhsr.org) 127 Vol.10; Issue: 12; December 2020 Vandana.V.P. Paligraphia and progressive non fluent aphasia in a patient with progressive supranuclear palsy.

presenting with primary progressive neurodevelopmental disorders: a aphasia: clinicopathological report of an comprehensive review. Child Adolesc autopsy case. Acta Neuropathologica. 2003; Psychiatry Ment Health. 2017; 11: 59 105:610–614. 15. Uyama E, Katahira T, Okada H, et al. 3. Josephs KA and Duffy,JR. Apraxia of Palilalia associated with progressive speech and nonfluent aphasia: a new clinical supranuclear palsy. Rinsho marker for corticobasal degeneration and Shinkeigaku 1989; 29(5):617-21. progressive supranuclear palsy. Current 16. Kluin KJ, Foster NL, Berent S, Gilman S. Opinion in Neurology. 2008; 21:688–692. Perceptual analysis of speech disorders in 4. Josephs KA, Boeve BF, Duffy JR et al. progressive supranuclear palsy. Neurology. Atypical progressive supranuclear palsy 1993; 43 (1) underlying progressive apraxia of speech 17. Horner J & Massey W. Progressive and nonfluent aphasia. Neurocase. 2005; 11, dysfluency associated with right hemisphere 283–296. disease. Brain and Language. 1983; 18, 71- 5. Santos-Santos, MA, Mandelli ML, Binney 85. RJ, et al. Features of Patients With 18. Garcia C. Neuroleptic-induced palilalia. A Nonfluent/Agrammatic PrimaryProgressive currently undescribed side effect. Aphasia With Underlying Progressive Nervenarzt1.990; 61(11):676-83. Supranuclear Palsy Pathology or 19. Josephs KA, Duffy JR, Strand EA, et al. Corticobasal Degeneration. JAMA Neurol. Clinicopathological and imaging correlates 2016; 73 (6):733-742. of progressive aphasia and apraxia of 6. Karnik NS, D’Apuzzo M, Greicius M. speech. Brain. 2006; 129:1385–1398. Nonfluent progressive aphasia, depression, 20. Enderby, Pamela M. Frenchay Dysarthria and OCD in a woman with progressive Assessment. San Diego, Calif.: College-Hill supranuclear palsy: neuroanatomical and Press, 1983. neuropathological correlations. Neurocase. 21. Neary D, Snowden J, Gustafson L, et al. 2006; 12:332–338. Frontotemporal lobar degeneration: A 7. Critchley M. On Palilalia.. J Neurol consensus on clinical diagnostic criteria. Psychopathol. 1927; 8 (29): 23–32. Neurology. 1998; 51: 1546–1554. 8. Van Borsel J, Schelpe L, Santens P, et al. 22. Yasuda Y, Akiguchi I, Ino M, Nabatabe H, Linguistic features in palilalia: two case Kameyama M. Paramedian thalamic and studies. Clin Linguist Phon. 2001; 15: 663– midbrain infarcts associated with Palilalia. 677. Rinsho shinkeigaku = Clinical neurology. 9. Ardila A., Rosselli’M., Surloff, C and 1990; 29(2):186-90. Buttermorel, J. Transient Paligraphia 23. Boller F, Albert M, and Denes F. Palilalia. Associated with Severe Palilalia and British Journal of Disorders of Stuttering: A Single Case Report. Communication. 1975; 10, 92- 97. Neurocase. 1999; 5, 435-440. 24. Kent RD, Horner J. and Andrews I. A case 10. Benke T. Palilalia and Repetitive Speech: report of atypical palilalia: Linguistic and Two Case Studies. Brain and Language. acoustic features of recurrent utterances. 2001; 78, 62–81. Journal of Medical Speech-Language 11. Van Borsel J, Bontinck C., Coryn M. Pathology. 1997; 5, 233± 249. Acoustic features of palilalia: A case study. 25. Fahn S. The freezing phenomenon in Brain and Language. 2007; 101: 90–96. parkinsonism. in Negative motor 12. Ackerman H, Ziegler W, Oertel W. Palilalia phenomena. Ed Fahn S, Hallett M, Lüders H as a symptom of L-dopa induced O, et al. Lippincott-Raven, Philadelphia, hyperkinesias. J Neurol Neurosurg 1995. Psychiatry. 1989; 52:805–807. 26. Ackermann H, Gröne BF, Hoch G, et 13. Comings DE and Comings BG. A al. Speech freezing in Parkinson's disease: a controlled study of Tourette syndrome. I. kinematic analysis of orofacial movements Attention-deficit disorder, learning by means of electromagnetic disorders, and school problems. Am J Hum articulography. Folia Phoniatr. 1993; 45: Genet. 1987; 41(5): 701–741. 84–89. 14. Cravedi E, Deniau E, Giannitelli M et al. 27. Levelt WJM. Speaking: from intention to Tourette syndrome and other articulation. 1989, MIT Press, Cambridge.

International Journal of Health Sciences and Research (www.ijhsr.org) 128 Vol.10; Issue: 12; December 2020 Vandana.V.P. Paligraphia and progressive non fluent aphasia in a patient with progressive supranuclear palsy.

28. Ackermann H, & Ziegler W. Acoustic brain damage. Journal of Fluency analysis of vocal instability in cerebellar Disorders.1996; 21, 137–146. dysfunctions. 1994; 103(2), 98-104. 34. LaPointe LL, & Horner J. Palilalia: a 29. Luria AR. Cognitive development: Its descriptive study of pathological reiterative cultural and social foundations. Trans M. utterances. Journal of Speech and Hearing Lopez-Morillas & L. Solotaroff. Harvard U Disorders.1981; 46, 34–48. Press, 1976. 35. McNeil MR, Robin DA, & Schmidt RA. 30. Benson DF, & Ardila A. Aphasia: A clinical Apraxia of speech: Definition and perspective. Oxford University Press, 1996. differential diagnosis. In MR. McNeil 2nd 31. Benke T, Butterworth B. Palilalia: two case Ed, Clinical management of sensorimotor reports and several implications. Brain speech disorders, 249–268). New York, Lang, 1994, 47: 368–370. NY:Thieme, 2009. 32. Lebrun Y. Repetitive phenomena in aphasia. 36. Ogar JM., Dronkers NF, Brambati SM et al. In G. Blanken, J. Dittman, H. Grimm, J. et Progressive nonfluent aphasia and its al. (Eds.), Linguistic disorders and characteristic motor speech deficits. pathologies. An international handbook, Alzheimer Disease and Associated 225–238.Berlin/New York: Waltger de Disorders. 2007; 21(Suppl.), S23–S30. Gruyter, 1993. 33. Van Borsel J, Van Coster R, & Van Lierde How to cite this article: Vandana.V.P. K. Final sound and final part-word Paligraphia and progressive non fluent aphasia repetitions in a 9-year-old boy with focal in a patient with progressive supranuclear palsy. Int J Health Sci Res. 2020; 10(12):122-129.

******

International Journal of Health Sciences and Research (www.ijhsr.org) 129 Vol.10; Issue: 12; December 2020