Cultural Considerations in Fahr's Syndrome: A Case Report

CASE REPORT

Our patient was an Ethiopian woman in her 50s with a history of mild cognitive impairment thought to be due to early-onset Alzheimer's dementia and failed treatment with paroxetine and donepezil. She was evaluated in the emergency department for evaluation of acute altered mental status including auditory hallucinations, delusions, and paranoia. Workup was significant for hypokalemia and an incidental finding of bilateral basal ganglia calcifications and cerebellar densities noted on imaging. The patient was admitted for further neuropsychiatric evaluation.

Psychiatry consultation noted impairment in her short-term memory, with all other cognitive testing refused by the patient. She had unusual ideation, was hyper-religious and paranoid, was somewhat on a delusional level, and responded to internal stimuli. The patient was medically admitted for 6 days for severe electrolyte replacement, and then discharged to the psychiatric service for further evaluation of persistent altered mental status with possible psychosis.

On inpatient psychiatry admission, she was started on mirtazapine and olanzapine with the tentative diagnosis of neurocognitive disease with significant paranoid/psychotic symptoms. Because of minimal symptomatic improvement, olanzapine was transitioned to risperidone. Further workup throughout admission identified a urinary tract infection, for which she was treated with 3 days of ceftriaxone, at which time the patient's mentation improved but still remained confused. She was started on mirtazapine, rivastigmine, and risperidone for baseline dementia, but her clinical picture remained unclear.

Diagnosis of the patient's neurocognitive dysfunction danced between a primary psychotic disorder (ie, schizophrenia), early-onset dementia, or Fahr's syndrome due to basal ganglia calcification. The rarity of Fahr's syndrome further complicated this discussion as its clinical picture and treatment options remain unclear.1–4 The ultimate challenge was deciphering the patient's clinical presentation through cultural and language barriers between patient and clinician. In this case, there was a significant barrier between an Ethiopian patient who spoke only Amharic (the official language of Ethiopia) and an American physician who spoke only English. For example, although not the only cognitive assessment available, the Montreal Cognitive Assessment is widely used to screen for mild cognitive impairment but does not seem to have cross-cultural applicability. Even with a translator, the language barrier made it difficult for us to clearly explain the directions of each section, resulting in confusion and frustration in a patient who may already be having cognitive impairment. Not only that, but the sole purpose of the assessment was difficult to explain as the abstract concept of assessing cognition with various mental exercises does not directly translate from one culture to another. On the second attempt, the patient's demeanor became more enthusiastic and motivated when she began to connect the pictures (lion, rhinoceros, and camel) in the “Naming” section to animals that she explained she had previously encountered in Africa. In patients already with potential cognitive impairment or mental illness, the language and cultural barriers seem to discourage and demoralize their efforts to share their suffering with care providers. Despite the knowledge of the patient originating from Ethiopia, much of her care plans and assessments focused on her presenting symptoms and results from physical and cognitive examinations.

Although it is possible that her lack of responses to certain tests like the Montreal Cognitive Assessment could be assessed as signs of dementia or psychosis, there is also the possibility that the patient simply could not understand what was being asked of her. Notably, the patient was much more reserved and softer spoken with care providers. Even though her English was advanced, she got quickly frustrated when she was unable to express her thoughts and feelings adequately. When her family first came to visit, she was more talkative and conversational when she was able to express herself freely in her native language.

DISCUSSION

Health of patients is directly intertwined with the quality of care given by their provider and sensitivity to their respective cultural background. Yet, many psychiatrists, and other providers alike, find difficulty in integrating a culture-centered approach into clinical practice and navigating the challenges when they arise.5 However, a recent study shows that people who identified as Black or African American or have depression are significantly more likely to place importance on the need for providers to share or understand their culture.6 Failure of a clinician to actively engage and consider patients' unique cultural backgrounds has been linked to worsened health outcomes, threats to informed consent, and distrust among cultural groups and the health care system.5,7,8 Solely language concordance in primary care settings has shown to create a better patient experience, leading to increased compliance to treatment plans and improved follow-up care.9 Actually, the use of interpreter services in interactions with patients with language barriers contributes to increased patient satisfaction and overall improved patient care.10,11 Health care professionals should seek to address the gap between cultural competence and clinical practice, especially with regard to language barriers, which also serve to assist the clinician in gaining better insight into the social determinants of health in the context of each patient.5,12 These barriers have been linked to misattribution of psychiatric symptoms, diagnoses, gaps in communication during patient interviews, and quality of care when present.13,14

Cultural considerations in psychiatry, a challenging paradigm to put into practice, can significantly influence neuropsychiatric diagnoses and patient care.15 Disparities between patient and physician should be addressed in each interaction to help create a bridge. Cultural competence training has been an effective tool to help bridge this gap in the past; however, it is evident that these cultural disparities persist in patient care today.5,16

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