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Dysphagia in Generalized Dystonia following Deep Brain Stimulation

nia with the worsening of the cervical and oromandibular dystonias. Repeat videofluoroscopy showed mild improvements, mostly in terms of swallowing delay. The delay with thin liquids was less prominent, and no episodes of airway invasion were observed. However, mastication did not seem significantly improved and continued to be laborious. FT reported he had essentially continued his usual diet since the previous visit, but he consumed a smoothie mixed with a nutritional supplement at lunch by straw. His weight was stable (although he did not return back to his baseline weight) and he felt like he could sit in the lunch room with his coworkers again and be part of the meal.

70.7 Key Points

●

Patients with dystonia have unique, individual presentations of dysphagia and dysarthria, but impaired mastication eﬃciency and bolus control can lead to significant oropharyngeal dysphagia.

●

DBS treatment may impact some symptoms (i.e., mobility) more eﬀectively than others (i.e., speech and swallowing).

●

Community-dwelling patients require a nuanced, realistic approach to develop strategies and recommendations for swallowing that promote safety and e ﬃciency of swallowing, as well as the patient’s personal goals.

References

[1] Hu W, Stead M. Deep brain stimulation for dystonia. Transl Neurodegener.

2014; 3(1):2

[2] Barkmeier-Kraemer JM, Clark HM. Speech-language pathology evaluation

and management of hyperkinetic disorders aﬀecting speech and swallowing function. Tremor Other Hyperkinet Mov. 2017; 7:489

[3] Troche MS, Brandimore AE, Foote KD, Okun MS. Swallowing and deep brain

stimulation in Parkinson’s disease: a systematic review. Parkinsonism Relat Disord. 2013; 19(9):783–788

[4] Isaias IU, Alterman RL, Tagliati M. Deep brain stimulation for primary gener-

alized dystonia: long-term outcomes. Arch Neurol. 2009; 66(4):465–470

279

Speech Therapy Telepractice Program for a Client with Aphasia

71 Speech Therapy Telepractice Program for a Client with Aphasia

Judy P. Walker

71.1 Introduction

Telepractice is the service delivery method of using technology to provide rehabilitation services at a distance. The purpose of this case study is to demonstrate a successful speech therapy telepractice program for a client with aphasia.

71.2 Clinical History and Description

CP was a 68-year-old, right-handed female who was referred for speech therapy telepractice services 6 months following a left hemisphere stroke. At the time of her stroke, a computed tomography (CT) scan revealed a left middle cerebral artery (MCA) cerebrovascular accident (CVA) in the parietal and temporal lobe regions, which was accompanied by severe fluent aphasia and mild oral dysphagia. CP was seen for inpatient speech therapy, followed by outpatient services for 5 months and reportedly made improvements in receptive and expressive language and functional communication using an iPad. Past medical history was significant for diabetes, hypertension, and bilateral carpal tunnel syndrome. CP was a high school graduate and retired bookkeeper. She lived at home with her 92-year-old mother and had two supportive daughters: one lived close by and the other in a nearby state.

71.3 Clinical Testing

CP was evaluated in person in her home to determine her current level of f unctioning and to assess her computer skills as well as her equipment and Internet access to ensure that she was appropriate for telepractice services. One daughter and CP’s mother were present during the evaluation. CP was alert and ambulatory with no evidence of motor diﬃculties. Although she appeared to understand questions, CP had diﬃculty verbally conveying thoughts and frequently requested that her daughter or mother provide responses to questions.

Pure tone hearing screening presented at 250, 500, 1000, 2000, and 4000 Hz was within normal limits (WNL) bilaterally. CP wore glasses for corrected visual acuity. An oral per ipheral examination was WNL. No evidence of motor speech diﬃculties was observed. Speech intelligibility was 100%. CP was currently eating a regular oral diet and her daughter confirmed that CP swallowed without diﬃculty. Attention and executive functioning were observed to be WNL. Language was assessed using the Western Aphasia Battery-Revised (WAB-R), cated an aphasia quotient of 48 (scale of 0–100), indicating severe aphasia (▶ Table 71.1). Auditory comprehension was

and results indi-

moderately impaired for sentences of increased length and complexity beyond simple subject + verb + direct object grammatical constructions. Verbal expression was fluent and severely impaired at word/sentence level. Spontaneous speech consisted primarily of jargon, containing numerous semantic paraphasias and neologisms with periodic recognizable automatic expressions (e.g., I don’t know, don’t worry). CP benefited from a combination of phonemic cueing and writing the first letters of words to assist with word finding. Repetition was moderate/severely impaired for sentences of increased length and complexity. Reading comprehension was moderately impaired beyond simple sentence construc tions. Writing was moderate/severely impaired at word/sentence level. CP frequently used a writing compensatory strategy to assist with lexical access when speaking.

Table 71.1 Comparisons of CP’s pre- and posttherapy raw scores and aphasia quotients from the Western Aphasia Battery-R

Subtest Pretherapy

Spontaneous speech

●

Information content 4/10 6/10

●

Fluency 7/10 7/10

Auditory comprehension

●

Yes/no questions 53/60 57/60

●

Word recognition 43/60 48/60

●

Sequential commands 34/80 41/80

Repetition 34/100 40/100

Naming

●

Object naming 17/60 26/60

●

Word fluency 2/20 3/20

●

Sentence completion 6/10 6/10

●

Responsive speech 6/10 8/10

Aphasia quotient 48 57.2

Reading

●

Comprehension of sentences 10/40 12/40

●

Reading commands 7/20 9/20

●

Word–object matching 6/6 6/6

Writing

●

Writing on request 4/6 5/6

●

Written output 6/34 15/34

●

Writing to dictation 0/10 0/10

Source: Kertesz.

Note: Interpretation of the WAB-R aphasia quotient is as follows: 0 to 25 = very severe; 26 to 50 = severe; 51 to 75 = moderate; 76 and above = mild.

results

Posttherapy results

280

Speech Therapy Telepractice Program for a Client with Aphasia

The ASHA Functional Communication Sk ills for Adults (ASHA

was completed with the assistance of CP’s daughter

FACS) as a standardized measure of functional communication (▶ Table 71.2). Additionally, the Walker Functional Checklist was completed to quantify functional communication abilities in activities of daily living (ADLs) unique to CP (▶ Table 71.3). Cumulatively, CP was able to communicate basic needs and

planning. She had greater diﬃculty in reading and writing required for functional tasks. CP’s daughter reported that CP periodically used nonverbal cues such as gestures, facial expres-

sion, and tone of voice to assist with conveying thoughts. CP also reportedly retrieved objects or attempted to write words related to the topic of discussion to assist in conveying the message, which was very time-consuming.

communicate with some help in social situations and in daily

Table 71.2 Comparison of CP’s pre- and posttherapy total and mean scores from the ASHA Functional Communication Skills for Adults (ASHA FACS)

Domain Total score Mean score

Pretherapy Posttherapy Pretherapy Posttherapy

Social communication 127/147 127/147 6.04 6.04

Communication basic needs

Reading/writing 50/70 54.5/70 5 5.5

Daily planning 27/35 33/35 5.4 6.6

Overall communication index mean score

Source: Frattali et al.

Note: Interpretation of the ASHA FACS mean score range is as follows: 7 = does independe ntly; 1 = does not do at all.

Table 71.3 Comparisons of pretherapy and posttherapy ratings of CP’s ability to complete activities of daily living (ADLs) that were unique to her using an adapted version of the Walker Functional Checklist for people with aphasia

Walker functional checklist

ADL tasks Accuracy Impaired cognitive and linguistic processes

Pretherapy

Easy

●

Housework + + X Gesture

●

Cooking + + X Gesture

●

Yardwork + + X Gesture

●

Self-care + + X Gesture

Moderate

●

Home

– ± X Gesture/ repair requests

●

Bank – + X X X X Gestur e

●

Walmart – + X X X X Gesture

●

Shaw’s – + X X X X Gesture

●

Hannaford – + X X X X Gesture

●

Wendy’s – + X X X X Gesture/iPad

●

Driving/

– ± X X X X Gesture/ directions

Difficult

●

Talking on

– ± X X iPad phone

●

Explaining

– ± X X Gesture/ day

Source: Walker.

Note: Interpretation of the accuracy ratings are as follows: (+) independent, (±) somewhat independent, (–) dependent.

Aud. comp., auditory comprehension; Comp. strategy, compensatory strategy; Ex. fun., executive functioning; Verb. exp., verbal expression;

48/49 49/49 6.8 7

5.81 6.2

Posttherapy

Sensory Motor Atten-

tion

Ex. fun. Aud.

comp.

Verb. exp.

Read Write Comp.

strategy

writing

writing/iPad

281

Speech Therapy Telepractice Program for a Client with Aphasia

71.4 Questions and Answers for the Reader

1. Based on CP’s history, select one area that you would not assess in your evaluation. a) Language. b) Functional communication. c) Dysphagia. d) Hearing and vision. e) Verbal reasoning and problem-solving.

Answer: e is correct. Since this patient has language deficits, the validity of results for assessing verbal reasoning and problem-solving would be in question. This patient may not fully understand questions due to auditory comprehension deficits and may not be able to answer questions due to verbal expression deficits.

a is incorrect. The primary complaint for this patient is language diﬃculties associated with a left hemisphere stroke. Therefore, you want to evaluate all language modalities to determine her level of functioning in each modality.

b is incorrect. The patient repor tedly was able to use strategies to assist with functional communication. You want to determine what strategies the patient uses and how successful she is at using these strategies to communicate.

c is incorrect. Any patient with neurological damage may have diﬃculty with swallowing. Therefore, it is essential to determine whether a patient has a safe swallow as part of any routine assessment of patients who have had strokes. This patient reportedly had an oral dysphagia upon admission to the hospital, which should be addressed in your evaluation.

d is incorrect. Hearing and visual acuity are the first stages in auditory and reading comprehension. Unless hearing and visual acuity are addressed, a clinician will not be able to ascertain whether deficits in auditory and reading comprehension may be attributed to an acuity rather than a language problem.

2. Considering CP’s site of lesion as indicated in the CT findings,

the results of the evaluation, and behavioral observations, what type of aphasia does CP exhibit? a) Broca’s. b) Anomic. c) Global. d) Wernicke’s. e) Transcortical motor.

Answer: d is correct. CP’s presentation is consistent with this type of aphasia. Wernicke’s aphasia is typically associated with a lesion to the posterior superior temporal gyrus and can be described as a fluent aphasia. Although auditory comprehension is often impaired for words and sentences, verbal expression is the hallmark characteristically described as utterances with relatively intact syntax but containing numerous semantic paraphasias and neologisms, which are devoid of meaning. Repetition tends to mimic verbal expression.

a is incorrect. CP’s presentation is not consistent with this type of aphasia. Broca’s aphasia is associated with lesions to the frontal lobe and can be described as a nonfluent aphasia. Auditory comprehension for simple sentences is relatively good, but verbal expression and repetition consists of halting, telegraphic

utterances containing a preponderance of nouns, and verbs, but missing function words.

b is incorrect. CP’s presentation is not consistent with this type of aphasia. Although anomic aphasia is often associated with lesions to the temporal and parietal lobes, this mild fluent aphasia is described primarily as diﬃculty with word finding with relatively preserved auditory comprehension and repetition.

c is incorrect. CP’s presentation is not consistent with this type of aphasia. Global aphasia is associated with large perisylvian lesions with very severe deficits in all modalities. These patients have diﬃculty understanding simple words, and verbal expression is often limited to repetitive automatic utterances.

e is incorrect. CP’s presentation is not consistent with this type of aphasia. Transcortical motor aphasia is associated with a frontal lobe lesion and has similar language characteristics as a Broca’s aphasia, except repetition is relatively good compared to verbal expression.

3. Speech therapy telepractice is recommended for CP. What

would be considered a negative prognostic indicator for improvement in language functioning from speech therapy

telepractice services in this case? a) Patient motivation. b) Time post onset. c) Severity of stroke. d) Family support. e) Medical history.

Answer: c is correct. The strongest predictor of short- and longterm outcome is severity of stroke. CP had a relatively large MCA stroke in the temporal and parietal lobes and continued to demonstrate persistent moderate to severe language deficits 6 months after the stroke.

a is incorrect. Motivation is a key factor in making progress

during recovery. CP was very motivated to engage in speech

y telepractice sessions. She completed all homework

therap assignments, which facilitated carryover of skill development between sessions.

b is incorrect. Most improvement of language occurs within the first few months and patients tend to plateau after 1 year where only smaller gains can be seen. CP is 6 months post onset, which is still within the spontaneous recovery time frame.

d is incorrect. The patient had a strong multigenerational support system living in her home or close by who assisted her within and between sessions. One of CP’s daughters, who lived further away, remotely joined sessions from her home computer assisted with homework assignments from a distance.

e is incorrect . Diabetes and hypertension are controllable risk factors for stroke.

4. You will see this patient for two 1-hour speech therapy

telepractice sessions per week. What is the best treatment approach to use with this patient given her deficits and current living situation?

a) Functionally oriented treatment approach that will focus

only on improving compensatory strategies for communication.

b) Impairment-based treatment approach that will only

target therapy goals for improving linguistic processes.

282

Speech Therapy Telepractice Program for a Client with Aphasia

c) Combined impairment-based and functionally oriented

approaches that will target improving linguistic processes while simultaneously teaching the patient compensatory strategies for communication.

Answer: c is correct. A combined approach that will help the patient recover language while simultaneously providing her with a means to communicate in everyday life is the most comprehensive type of treatment program for this patient.

a is incorrect. Although the brain is the most plastic during the first month following a stroke, spontaneous recovery can still occur up to a year, at which time patients tend to plateau. Therefore, it is essential to assist the brain in recovering neural networks that are associated with linguistic processes during this crucial time through language therapy.

b is incorrect. While it is crucial to treat linguistic processes to assist in language recovery, this patient is living at home and in her community. For reasons involving safety and quality of life issues, this patient needs to have ways to communicate when language is not available. For instance, CP had diﬃculty talking on the phone. So, her house telephone was equipped with a direct dial to the police station and automatic address message to alert authorities in case of an emergency.

71.5 Description of Disorder and Recommended Treatment

At 6 months following left MCA CVA, CP presented with moderate Wernicke’s aphasia. Auditory comprehension was moderately impaired at word/sentence level. Verbal expression was fluent and moderate/severely impaired at word level. Spontaneous speech was characterized primarily by neologistic jargon embedded in sentences, which appeared to have some semblance of grammar and also contained the periodic presence of automatic utterances. Repetition was moderate/severely impaired at sentence level. Reading was moderately impaired at sentence level. Writing was severely impaired at word level. Hearing and visual acuity were WNL. Motor speech, swallowing, attention, and executive functioning were WNL. Functionally, CP’s communication deficits interfered with her ability to perform ADLs independently within and outside of the home environment. Within the home, CP had established compensatory strategies of using writing, inconsistent gesturing, and limited use of her iPad to assist her in conveying her wants/needs/ ideas to her daughter and mother. They reported that CP was successful in communicating her thoughts less than 50% of the time.

Speech therapy telepractice was recommended two times a week for 6 months. The therapy focused on using an impairment-based approach linguistic processes combined with a functionally oriented approach means possible in ADLs that were unique to her. Prognosis for improvement of linguistic abilities was guarded based on the severity of the CVA and severity of residual deficits. Prognosis for improvement of functional communication was good based on CP ’s intact cognitive abilities, motivation, and family support. She was an excellent candidate for telepractice speech therapy given her advanced computer skills.

to improve CP’s ability to communicate using any

to directly target the improvement of

71.6 Outcome

The impairment-based treatment goals targeted (1) auditory comprehension at a sentence and conversational level, (2) verbal expression at word and sentence levels using a combined grapheme–phoneme cueing strategy to aid in the lexical access of nouns and verbs related to func tional contexts, (3) reading and writing at word or sentence level as related to functional contexts. The functionally oriented treatment goals targeted strategies to compensate for language deficits, such as gestures, writing words, and using smart technology, which assisted CP in conveying her thoughts within functionally based ADLs.

Speech therapy telepractice sessions were provided fr om the speech therapy telepractice lab o n the University of Maine campus using a secure interactive video conferencing system that connected CP from her home computer to a computer in the campus lab through high-speed Internet access. Each computer was equipped with high-definiti on audio and video to ensure the highest quality connection and service delivery. The system allowed for adaptation of the impairment-based treatment approach through desktop presentations of custommade materials/activities, which were developed from photos and videos taken at CP’s home and within her community. An example can be seen in Video 71.1, whe re the student clinician worked with CP on a verbal expression, sentence-building activity related to driving using a combined grapheme–pho neme cueing strategy to aid in word retrieval. Since CP drove independently, CP’s daugh ters were concerned that she would not have the language skills to verbally convey information about her locatio n should she become lost. The telepract ice platform allowed for the desktop presentation of photos related to driving and the use of eTools to enable both t he clinician and CP to simultaneously write on the desktop while using the combined phoneme–grapheme cueing strategies to assist with lexical access. Each singular photo slide was followed by a slide containing an audio file of the first sound accompanied by the first letter of the target word, then another slide containing a n audio file of the spoken word associated with the written word. Although they were not used in the activit y, these additional slides were embedded in the activity for CP to practice at home when the clinician was not present to provide her with the grapheme–phoneme cues and confirmation of the correct words. The telepractice platform also supported a functionally oriented treatment approach through sharing websites and browsers to create highly enriched activities that targeted CP’s ADLs within virtual environments i n her home and community. As shown in Video

71.2, the aforementioned sentence-building activity was expanded into a supported conversation activity

to create a virtual environment of CP’s neighborhood

Earth and community. CP was asked to give directions for driving her typical route to a local restaurant while being supported by the clinician and her daughter to provide clarifying information. These types of therapy activities were used to improve CP’s language and communication ab ilities so she could suc- cessfully converse about ideas, places, and things in her world. When CP had achieved criteria for language and functional communication goals within each activit y of daily living, the clinician addressed the functional checkl ist to identify a nother activity of daily living. New sets o f therapy materials were

using Google

283

Speech Therapy Telepractice Program for a Client with Aphasia

Video 71.1 Example of sentence-building activity related to driving. Video 71.2 Example of a supported conversation activit y related to

driving using Google Earth.

developed with the aid of CP and her family members who

●

took photos and videos of CP within that specific activity of daily living.

At the end of 6 months of therapy, CP was reevaluated using

the WAB-R and the ASHA FACS. As shown in ▶ Table 71.1 and

▶ Table 71.2, CP made improvements in linguistic and func-

Several prognostic indicators must be taken into consideration when determining a prognosis for a person with aphasia.

●

Telepractice technology is a viable and highly creative service delivery model for providing successful treatment programs for people with aphasia.

tional communication abilities. Her WAB-R aphasia quotient increased from a 48 to 57.2 with specific areas of improvement especially noted in auditory comprehension, verbal expression in producing words that contained greater meaning, along with written output. Functional communication also improved especially in areas related to reading/writing and daily planning. Additionally, CP made significant progress in achieving therapy goals as they related to ADLs that were unique to her. As shown

Suggested Readings

[1] Towey MP. Speech telepractice: installing a speech therapy upgrade for the

21st century. Int J Telerehabil. 2012; 4(2):73–78

[2] Walker JP. University of Maine, Speech Therapy Telepractice and Technology

Program Manual. University of Maine Faculty Monographs. Book 220. 2015. http://digitalcommons.library.umaine.edu/fac_monographs/220

in ▶ Table 71.3, CP gained greater independence in communicating wants and needs using combined language and functional communication strategies in several easy and moderate ADLs that were previously problematic. She continued to require assistance with communication in diﬃcult ADLs. CP and her family reported great satisfaction with the successful outcome of the speech therapy telepractice program and stated that they would strongly recommend this program to other individuals with aphasia.

71.7 Key Points

●

Classification of aphasia is determined through consideration

References

[1] Kertesz A. Western Aphasia Battery-Revised. San Antonio, TX: Pearson; 2006 [2] Frattali C, Thompson C, Holland A, Wohl C, Ferketic M. Functional Assessment

of Communication Skills for Adults. Rockville, MD: American Speech-Language-Hearing Association; 1995

[3] Walker JP. Functional outcome: a case for mild traumatic brain injury. Brain

Inj. 2002; 16(7):611–625

[4] Galletta EE, Barrett AM. Impairment and functional interventions for aphasia:

having it all. Curr Phys Med Rehabil Rep. 2014; 2(2):114–120

[5] Kagan A. Supported conversation for adults with aphasia: methods and re-

sources for training conversation partners. Aphasiology. 1998; 12(9):851– 864

[6] Google Earth [computer program]. Version 7.1.2.2041. Mountain View, CA:

Google; 2013

of neuroimaging findings, diagnostic results, and behavioral observations.

●

Several important factors regarding language, functional abilities, and patient environment must be taken into consideration when designing a comprehensive evaluation for aphasia.

284

Linguistic and Cognitive Deficits in Autoimmune Encephalopathy

72 Linguistic and Cognitive Deficits in Autoimmune Encephalopathy

Alejandro E. Brice and Jamie Swartz

72.1 Introduction

Autoimmune encephalopathies (AEs) occur when antibodies attack cell surfaces or synaptic proteins with widespread and/or focal nervous system involvement. AE can aﬀect patients of all ages with some preference for young children, women, and young adults, and they can develop with or without concurrent tumors. With AE, all areas of cognition can be impaired with the most common clinical symptom consisting of a rapid and progressive dementia that can be mistaken for Creutzfeldt–Jakob disease.

and can be associated

72.2 Clinical History

SJ was a 32-year-old woman admitted to the emergency room with bilateral arm and leg numbness, heaviness, and tingling sensations in addition to confusion. She reported a bandlike sensation around her upper arm (like a tight strap) and her leg numbness was more pronounced on the left side. Blood tests revealed antinuclear antibody, a test for autoimmune disorders, of 1:40, which was not significant. SJ also presented with decreased protein S, which is associated with a blood disorder and increased risk of thrombosis (e.g., formation of a clot in a blood vessel). At that time, SJ was diagnosed with an undiﬀerentiated connective tissue disorder (i.e., where the body attacks its own tissues) and was treated with steroids. Four years later, SJ returned to the emergency room after suﬀering altered mental state (e.g., three episodes of lost consciousness causing motor vehicle accidents). She was disoriented and presented with short-term memory loss, inability to express herself, and slowed processing.

inability to spell the word “world” backward. SJ scored 25/30 on the MMSE. In addition, the Montreal Cognitive Assessment

(MoCA), tered. The MoCA consists of a 30-point scale and measures the following: (1) memory recall; (1) visuospatial abilities; (3) phonemic fluency; (4) executive function; (5) verbal abstraction; (6) attention, concentration, and working memory; (7) serial subtraction; (8) confrontation naming; (9) sentence repetition; and (10) orientation to time and place. A score of 26 or greater is considered normal. cognitive abilities. She had diﬃculty with drawing a clock with the numbers outside of the circle and with attention and repetition issues (i.e., reading list of digits and letters and repeating them forward and backward). SJ could not remember items even with a multiple choice format. She was eventually diagnosed with encephalopathy of unknown origin.

netic resonance imaging (MRI) indicated ovoid (egg-shaped) T2 (imaging indicating abnormally high water content) hyperintensity (patches of damaged cell tissue that shows up as white spots on the MRI, sometimes indicative of multiple sclerosis, dementia, or diabetes) in the frontal lobe. Also, a punctuate lesion in the parietal lobe was found. These lesions were not identified during her prior workup. Due to her autoimmune hypercoagulable state, SJ had a series of subsequent pulmonary emboli. After treatment with high-dose steroids, the immediate pulmonary symptoms began to dissipate; however, it took several months for full recovery.

an assessment similar to the MMSE, was adminis-

SJ scored 25/30, suggestive of altered

SJ underwent a comprehensive medical workup. The mag-

72.4 Questions and Answers for the Reader

72.3 Clinical Testing

SJ reported that she experienced disorientation, memory loss, word-retrieval diﬃculties, and problems with pragmatic contextaswellasdiﬃculties with focus and concentration. The Rey Auditory Verbal Learning Test (R AVLT),

and an examination of verbal fluency (semantic and let-

(BNT), ters) were administered by a licensed psychologist. SJ was not seen by a speech-language pathologist at that time as she was functional in activities of daily living including her work. The psychologist reported that verbal, immediate memory from the RAVLT scores were low average to average; verbal delayed memory from the R AVLT scores were low average to average and recognition cues did not substantially improve recall. Confrontation naming from the BNT was within normal limits (BNT = 54/60). Letter and semantic fluency scores were reported to be borderline to low average. No alexia or agraphia was reported.

SJ was also assessed by a neurologist using the Mini-Mental State Examination (MMSE) time, but not context; diﬃculty with serial sevens (counting down from 100 using 7, i.e., 100 –93–86–79–72–65,…); and

and oriented to person, place, and

Boston Naming Test

1. AEs are part of a spectrum of inflammatory central nervous system disorders. Three types of AEs exist, which are:

a) Encephalopathies without cancer, encephalopathies with

cancer, and Hashimoto’s encephalopathies.

b) Encephalopathies without cancer, paraneoplastic ence-

phalopathies, and peripheral nervous system vasculitis.

c) Encephalopathies without cancer, paraneoplastic ence-

phalopathies, and central nervous system vasculitis.

d) Paraneoplastic encephalopathies, Hashimoto’s encephalo-

pathies, and central nervous system vasculitis.

Answer: c is correct. The three types of encephalopathies consist of those without cancer, those pertaining to metabolic eﬀects of cancer and changes produced in t issues remote from a tumor or its metastases, and inf lammation of central nervous system blood vessels.

a is incorrect. Hashimoto’s encephalopathy is one type under those with cancer. Encephalopathies with cancer are paraneoplastic encephalopathies, where the body’s immune system is fighting cancer; it is a systemic disturbance associated but not directly related to the cancer.

285

Linguistic and Cognitive Deficits in Autoimmune Encephalopathy

b is incorrect. The third type of encephalopathy involves the

central nervous system and not the peripheral nervous system.

d is incorrect. Hashimoto’s encephalopathy is one type under

those with cancer.

2. SJ scored poor to low average on tests. These tests included: a) Focus and divided attention, short-term memory, visual

memory, and delayed memory.

b) Focus and divided attention, sustained attention, short-

term memory, verbal short-term memory, delayed memory, letter and semantic memory, visuoconstruction skills, and nonverbal reasoning.

c) Focus and divided attention, sustained attention, short-

term memory, verbal short-term memory, delayed memory, letter and semantic memory, and visual short-term memory.

d) Focus and divided attention, sustained attention, short-

term memory, verbal short-term memory, delayed memory, letter and semantic memory, visual short-term memory, and time- and set-sequencing tasks.

Answer: b is correct. SJ scored low on items concerning attention, memory, nonverbal reasoning, and visuoconstruction skills. These deficits are typical of AE and associated dementias.

a is incorrec t. SJ also scored low on sustained attention; verbal short-term memory, letter, and semantic memory were also aﬀected; she also scored impaired on time- and set-sequencing tasks.

c is incorrect. Visual short-term memory was average. In addition, verbal short-term memory, delayed memory, letter and semantic memory, visuoconstruction skills, time sequencing, set sequencing, and nonverbal reasoning were also aﬀected.

d is incorrect. Time- and set-sequencing tasks were borderline.

3. Encephalopathies aﬀect _____ areas of cognition and mem-

ory loss is present in about _____ of patients with AEs. a) Some; one-third. b) Some; one-half. c) All; one-third. d) All; one-half.

Answer: d is correct. Memory is aﬀected in one-half of all AEs and nearly all aspects of cognition are aﬀected.

a is incorrect. AEs aﬀect all areas of cognition and about one half of patients exhibit memory loss.

b is incorrect. AEs aﬀect all areas of cognition.

c is incorrect. About one-half of patients exhibit memory loss.

4. AEs can aﬀect:

a) Patients of all ages. b) Preference for children, young women, and young adults. c) Preference for young men. d) a and b. e) a and c.

Answer: d is correct. AEs can aﬀect anybody; however, there is a preference for children, young adults, adults, and women.

a is incorrect. AEs also show preference for children, young women, and young adults.

b is incorrect. AEs can aﬀect patients of all ages.

c is incorrect. AEs show preference for children, young

women, and young adults.

e is incorrect. AEs show preference for children, young

women, and young adults.

72.5 Description of Disorder and Recommended Treatment

AEs are part of a spectrum of inflammatory central nervous system disorders that may include limbic encephalitis, which is noted by subacute onset, memory loss, altered senses, seizures, possible personality changes, and/or personality or mood changes. memory loss present in about half the patients with AEs. Cognitive communication disorders in AEs may include diﬃculties with memory, attention, organization, problem-solving, and executive functioning. (i.e., phonologic, morphologic, syntactic, semantic, pragmatic).

cer, but with serologic evidence of autoimmunity including Hashimoto’s encephalopathy; (2) paraneoplastic encephalopathies including syndromes caused by the body’s immune system fighting cancer; and (c) central nervous system vasculitis. Hashimoto’s encephalopathy along with other encephalopathy etiologies are often misunderstood. “diagnosis of an autoimmune encephalopathy is diﬃcult and often inferred from serologic, electroencephalopathy, and spinal fluid findings” (p. 879).

onset, headaches, mild hyperthermia, and frequent cerebrospinal fluid (CSF) pleocytosis (an elevated number of lymphocytes or white blood cells in the CSF). Electroencephalograms are more than likely to be abnormal showing focal slow activity often associated with epileptic activity.

gist and presented with flaccid dysarthria (low muscle tone) and generalized weakness on the left side of her face a nd body. Flaccid dysarthria is a lower motor neuron disorder, or a disorder causing disruption in the communication between neurons in the spinal cord and skeletal muscles in the arms, legs, chest, face, throat, and tongue. SJ was clinically diagnosed with myasthenia gravis, an autoimmune disease that leads to fluctuating muscle weakness and fatigue. Voluntary muscle weakness is the main symptom; muscles become weaker with continued physical activity. Dysphagia (diﬃculty swallowing), dysarthria (slurred speech), and breathing diﬃculties can also be outcomes.

MMSE, and the MoCA, indicated low-to-average performance (BNT = 54/60; MMSE 25/30; MoCA = 25/30). In addition, the neurologist and psychologist both reported that SJ scored poorto-low average on tests regarding attention. This was true in tests of focused and/or divided attention. SJ answered quickly and inaccurately in the poor to borderline range on tasks of sustained attention. Short-term memory scores were low average to average, with diﬃculty in verbal short-term memory tasks opposed to visual short-term memory, which was average. Delayed memory scores were also low average to average and cues did not significantly enhance results. Letter and semantic

Encephalopathies aﬀect all areas of cognition,2with

All areas of language may be aﬀected

Three types of AEs exist: (1) encephalopathies without can-

Caselli et al.10stated that

However, AEs display common symptoms consisting of rapid

SJ was subsequently evaluated by a speech-language patholo-

Results of naming and memory tests, that is, the BNT, the

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Linguistic and Cognitive Deficits in Autoimmune Encephalopathy

verbal fluency scores were also low average to average. Visuoconstructional skills were borderline due to poor planning. SJ scored impaired to borderline on a time-sequencing and setshifting task. Borderline scores were also present on complex reasoning tasks. Nonverbal reasoning was low average to average. Verbal fluency was borderline. According to the neurologist, SJ presented with “mild subcortical dysfunct ion” with frontoparietal location consistent with lesions found in the front and left parietal areas as revealed by repeated MRI studies.

Cognitive-language therapy was not formally provided as SJ was functional in both activities of daily living and work activities. SJ did not receive conventional speech-language therapy. However, SJ’s sister was a speech-language pathologist and oﬀered strategies, including word naming tasks, reading, writing, word memory tasks, auditory attention tasks, divided attention tasks, working memory tasks, and cognitive speed

11–14

tasks.

72.6 Outcome

Four months after the onset of symptoms, SJ reported that her episodes of confusion and cognitive issues ceased. After a 3-day course of high-dose Solu-Medrol (a corticosteroid), symptoms dramatically improved, that is, sustained attention and recall and retrieval of information. SJ reported near immediate improvement of memory and attention-related skills. SJ continued treatment (methotrexate and intravenous immunoglobulin) for myasthenia gravis and undiﬀerentiated connective tissue disorder, both autoimmune disorders. In addition, SJ received anticoagulation therapy for autoimmune blood coagulation dysfunction. SJ reported that tasks of attention when presented sequentially and slowly have the highest degree of success. Short-term and delayed memory tasks continue to be facilitated by the use of calendars, note-taking applications and programs, and recording of sequentially based tasks. Activities undertaken systematically and slowly have a higher rate of completion and success. SJ’s cognitive function improved; her pre- and postintervention performances, as measured by the BNT and the MMSE, were as follows: (1) BNT preintervention =54/60 and postintervention = 60/60, and (2) MMSE preintervention =25/30 and postintervention = 30/30.

72.7 Key Points

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AEs can aﬀect patients of all ages.

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Treatment may consist of corticosteroid drug intervention along with cognitive-language therapy.

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Compensatory strategies may include note-taking applications, recording events on a sequential basis, systematically

slowing down tasks for higher completion rates, and/or use of calendars.