Student Rush University Western Springs, Illinois, United States
Disclosure(s):
Lila R. Zlotowski, n/a: No financial or non-financial relationships to disclose
Hannah Olenec, n/a: No financial or non-financial relationships to disclose
Abstract: The present case presentation focused on describing cognitive-linguistic functioning in a patient with herpes simplex (HSV) encephalitis developing into N-methyl-D-aspartate receptor (NMDA) encephalitis. The patient presented with severe receptive and expressive non-fluent aphasia, consistent with the diagnosis of Broca’s aphasia. Assessment and treatment considerations for this patient were discussed.
Summary of Presentation : Introduction N-methyl-D-aspartate (NMDA) receptor encephalitis is an autoimmune disease that can cause a range of neurological and behavioral deficits including autonomic dysfunction, psychosis, altered consciousness, seizures, dyskinesia, and cognitive-linguistic functioning (Dalmau et al., 2019; Jones et al., 2012). NMDA receptor encephalitis can be triggered by teratomas and the herpes simplex virus (HSV) infection (Hu et al., 2021). Despite the growing number of cases, there is limited knowledge regarding the consequences after the development of NMDA receptor encephalitis post-HSV infection (Jones et al., 2012). In particular, the presentation and management of cognitive-linguistic consequences post NMDA receptor encephalitis is not well understood. It is imperative for speech-language pathologists (SLPs) to increase awareness about this condition and the common sequelae associated with it in order to provide effective clinical services and maximize a patient's functional outcomes. The present case describes cognitive-linguistic changes in an otherwise healthy young adult English-Spanish bilingual male with HSV encephalitis developing into NMDA receptor encephalitis.
Aims We aim to describe the patient’s complex medical progression, cognitive-linguistic assessments administered (including interpreter involvement), and rehabilitation goals during their hospital stay. Given the patient's background and the evolving nature of their diagnosis, we discuss future clinical recommendations with a particular emphasis on cognitive-linguistic symptom management.
Case Description The patient presented to the hospital with initial symptoms of altered mental status, seizures, agitation, and increased sleeping and was initially diagnosed with HSV encephalitis. The patient received speech and language therapy services in the inpatient rehabilitation setting where the Western Aphasia Battery Revised - Bedside (WAB-B; Kertesz, 2007) was administered. Findings indicated severe Broca’s aphasia characterized by language deficits in semantic, phonological, morphological, and syntactic domains as observed during spontaneous speech, repetition, and naming tasks. The patient made minimal progress throughout his five-day stay in inpatient rehabilitation. Further cerebrospinal fluid (CSF) testing was conducted which tested positive for NMDA receptor antibodies. The patient was subsequently sent back to acute care where he underwent three days of intravenous immunoglobulin (IVIg) treatment. After treatment he was assessed using the WAB-B, the results of which indicated Broca’s aphasia, although less severe than when he was in inpatient rehabilitation. Specifically, the patient was able to produce longer and more complex utterances, more accurately repeat words and phrases, and name objects with fewer cues and paraphasias. The long-term goal for the patient was to participate in complex conversations by improving their receptive and expressive language abilities. Accordingly, short-term goals in both inpatient rehabilitation and acute care focused on naming, automatic speech tasks, following commands, and answering yes/no questions. The patient’s cognitive-linguistic functioning was noted to gradually improve over time, and he was discharged one week after his acute care stay. The patient’s bilingual status indicated a need for an interpreter, according to hospital policy. The patient’s mother advocated for his fluency in English; the patient did not show signs of attempting to speak Spanish, and cognitive impairments contraindicated the need for an interpreter. Thus, interpreter services were only used during the first treatment session in inpatient rehabilitation.
Results and Discussion Given the ongoing uncertainty regarding the patient’s diagnoses, many members of the medical team were unsure of the appropriate treatment plan. The patient's diagnoses changed from an initial diagnosis of HSV encephalitis to NMDA receptor encephalitis within a matter of weeks. During the course of the complicated diagnostic process, SLPs were critical in monitoring patient's cognitive-linguistic symptoms with the evolving diagnoses. The WAB-B served as a quick and appropriate assessment that helped the SLPs characterize the nature of language deficits (i.e., severe nonfluent aphasia) and identify the problematic linguistic domains including deficits in naming, repetition, and complex sentence productions (Kertesz, 2007). Further testing incorporating novel and more detailed assessments of linguistic and extralinguistic cognitive skills would provide a more comprehensive picture of the patient's abilities. It is imperative for clinicians to focus on treating the patient's symptoms, regardless of changes in the underlying diagnosis (American Speech-Language-Hearing Association, 2016). Based on the current research regarding the HSV to NMDA encephalitis transition, the prognosis for this patient was good. Recovery from NMDA encephalitis is a multistage process that occurs in the reverse order of symptom presentation and there remains a chance of relapse for up to several years after disease and symptom resolution (Jones et al., 2012). As per most recent reports, two months after treatment and discharge, the patient continues to present with encephalitis and a recurrence of seizures, likely indicative of relapse. Relapse most likely occurs in instances where immunotherapy may not have been used (Jones et al., 2012). Thus, the patient will likely require a longer regimen of medical treatment to reach his baseline level of functioning (Jones et al., 2012). Given the patient was bilingual, testing their linguistic ability in both languages is warranted to gain a comprehensive understanding of how encephalitis is likely to affect language processing. Further, an interpreter was utilized during inpatient rehabilitation to communicate with the mother but was not used in acute care, as the SLP was a certified Spanish speaker. Additional investigation into the patient’s Spanish and English competency and consistent use of interpreter services could aid in creating an appropriate outpatient treatment plan to be used in the future (Johnson, 2020).
Conclusion and Future Considerations We present a case of a young adult with NMDA receptor encephalitis secondary to HSV encephalitis. Encephalitis of any type often directly impairs cognitive-linguistic functioning. Thus, patients who experience neurological symptoms as a consequence of NMDA receptor encephalitis, following the resolution of HSV encephalitis, should be evaluated and monitored closely. SLPs are important members of the interdisciplinary team whose responsibilities include monitoring patient progress through chart review and administering appropriate and evidence-based assessments and treatments for individuals with such evolving encephalitis diagnoses. Continuous research and education are imperative for clinicians to improve current knowledge regarding new diagnoses. Future considerations include further deliberation of the use of detailed cognitive-communication assessments and additional treatment activities, including those available for bilingual patients, and careful consideration of interpreter use throughout the rehabilitation process.
References American Speech-Language-Hearing Association. (2016). Scope of practice in speech-language pathology [Scope of Practice]. Available from www.asha.org/policy/. Dalmau, J., Armangué, T., Planagumà, J., Radosevic, M., Mannara, F., Leypoldt, F., Geis, C., Lancaster, E., Titulaer, M., Rosenfeld, M., & Graus, F. (2019). An update on anti-NMDA receptor encephalitis for neurologists and psychiatrists: Mechanisms and models. The Lancet Neurology, 18(11), 1045-1057. https://doi.org/10.1016/S1474-4422(19)30244-3 Hu, S., Lan, T., Bai, R., Jiang, S., Cai, J., & Ren, L. (2021). HSV encephalitis triggered anti-NMDAR encephalitis: A case report. Neurological Sciences, 42(3), 857–861. https://doi.org/10.1007/s10072-020-04785-9 Jones, K. C., Benseler, S. M., & Moharir, M. (2012). Anti-NMDA receptor encephalitis. Neuroimaging Clinics of North America, 23(2), 309-320. http://dx.doi.org/10.1016/j.nic.2012.12.009 Johnson, L. W. (2020). Factors influencing aphasia assessment for bilingual adults. In Cases on Communication Disorders in Culturally Diverse Populations (pp. 196-216). IGI Global. Kertesz, A. (2007). Western Aphasia Battery - Revised. San Antonio, Texas: Psychological.
Learning Objectives:
At the completion of this activity, participants should be able to:
Describe how HSV and NMDA encephalitis directly impair cognitive-linguistic functioning.
Describe the importance of treating the symptoms at hand regardless of the diagnosis.
Discuss the value of the interprofessional team in monitoring patient progress and determining utility of interpreter services.