Challenges Faced by Rural Health Care Providers Caring for Parkinson's Disease Patients in Neighbor Islands of Hawai'i.

When compared to their urban counterparts, rural regions have worse health outcomes and more challenges in health care access. As the only island state in the US, Hawai'i's unique geographic layout may magnify these disparities. However, there are limited publications on the impact of urban-rural disparities in health care in Hawai'i. The study team aimed to identify the challenges rural health care providers face when managing treatment of Parkinson's disease (PD), a complex disease. A self-administered survey was sent to 247 eligible providers who practiced in Hawai'i and prescribed PD medications from 2017-2019. The survey assessed: provider's comfort level in PD management; utilization and accessibility of health care services; perspective on barriers to PD care; and perspective on telemedicine. Providers were categorized into O'ahu providers (OP, urban) and neighbor island (Hawai'i, Kaua'i, and Maui) providers (NIP, rural). The final sample size was 44 providers (18% response rate). NIP were significantly less likely than OP to report access to social workers (P=.025), geriatric services (P=.001), and psychologist/psychiatrist/mental health professionals (P=.009). There were no statistical differences in: criteria used for PD diagnosis, resources utilized for PD education, and comfort in prescribing PD medications. The findings show that NIP are just as engaged and capable in providing PD care as OP. However, NIP encounter more limitations to accessibility, which can affect the quality of PD care that their rural patients receive. Further research is needed to understand how these limitations affect health-related outcomes in PD as well as other chronic diseases.

The Prevalence of Hospitalized Parkinson's Disease Patients in All Case Hospitalization among Different Race/Ethnic Subgroups in Hawaii.

Background: Little is known about the epidemiology of Parkinson's disease (PD) patients in Native Hawaiian Or Other Pacific Islander (NHPI) and Asian American (AA) subgroups. Objective: To determine if the prevalence of hospitalized PD patients is different across age groups and racial/ethnic subgroups in Hawaii. Methods: We conducted a retrospective analysis of Hawaii statewide registry (2016-2020) hospitalization data for patients who were 50 years or older. PD patients were identified using an ICD 10 code: Parkinson's Disease (G20) as their primary/secondary hospitalization discharge diagnosis code. Demographic and clinical characteristics among racial/ethnic subgroups (White, Japanese, Filipino, Chinese, NHPI, or Other) were compared. Results: Of 146,844 total hospitalized patients (n = 429,879 records), 1.6% (n = 2,401) had a PD diagnosis. The prevalence of hospitalized PD patients was 2.3% among Japanese and Chinese, followed by 1.7% for Whites, 1.2% for Filipinos and was lowest for NHPI with 0.9% (p < 0.001). As patient's age increased, the prevalence of hospitalized PD patients increased, with 80-84 years old for the highest age range (3.4%). The prevalence of hospitalized PD patients at 80-84 years old varied across the race/ethnic subgroups (Chinese 4.3%, Japanese 4.0%, Whites 3.7%, Filipinos 2.5%, NHPI 2.3%). Conclusions: The prevalence of hospitalized PD patients among all case hospitalizations were lower for NHPI and Filipino compared to that of Japanese, Chinese, and Whites. As patients' age increased, the prevalence of hospitalized patients with PD increased, but less so in NHPI and Filipino groups. Further research is warranted to understand the reason for these observed differences among racial/ethnic subgroups.

Racial disparities in hospitalization characteristics among Native Hawaiians, Pacific Islanders and Asian American subgroups with Parkinson's disease.

BACKGROUND: Differences among Native Hawaiians/Pacific Islanders (NHPI) and Asian American (AA) subgroups have not been adequately studied in Parkinson's disease (PD). OBJECTIVE: To determine differences in demographics, comorbidities, and healthcare utilization among NHPI, AA subgroups, and White hospitalized PD patients. METHODS: We conducted a retrospective cross-sectional analysis of Hawai'is statewide registry (2016-2020). Patients with PD were identified using ICD10 code G20 and categorized as White, Japanese, Filipino, Chinese, NHPI, or Other. Variables collected included: age, sex, residence (county), primary source of payment, discharge status, length of stay, in-hospital expiration, Charlson Comorbidity Index (CCI) and Deep Brain Stimulation (DBS) utilization. Bivariate analyses were performed: differences in age and CCI were further examined by multivariable linear regression and proportional odds models. RESULTS: Of 229,238 hospitalizations, 2428 had PD (Japanese: 31.3 %, White: 30.4 %, Filipino: 11.3 %, NHPI: 9.6 %, Chinese: 8.0 %). NHPI were younger compared to rest of the subgroups [estimate in years (95 % CI): Whites: 4.4 (3.0-5.8), Filipinos: 4.3 (2.7-5.9), Japanese: 7.7 (6.4-9.1), Chinese: 7.9 (6.1-9.7), p < 0.001)]. NHPI had a higher CCI compared to White, Japanese, and Chinese (p < 0.001). Among AA subgroups, Filipinos were younger and had a higher CCI compared to Japanese and Chinese (p < 0.001). There were no significant differences in DBS utilization among subgroups. CONCLUSIONS: NHPI and Filipinos with PD were hospitalized at a younger age and had a greater comorbidity burden compared to other AAs and Whites. Further research, ideally prospective studies, are needed to understand these racial disparities.

Differences Among Native Hawaiian, Asian, and White Patients with Progressive Supranuclear Palsy.

BACKGROUND: Most studies of progressive supranuclear palsy (PSP) have been conducted in White populations. OBJECTIVE: The objective of this study was to identify whether differences exist for patients with PSP among Whites, East Asians (EAs), and Native Hawaiians/Pacific Islanders (NHPIs) in Hawaii. METHODS: We conducted a single-center, retrospective study of patients meeting Movement Disorder Society probable PSP criteria (2006-2021). Data variables included age of onset and diagnosis, comorbidities, and survival rate. Variables were compared across groups using Fisher's exact test, Kruskal-Wallis rank sum test, and log-rank tests. RESULTS: A total of 94 (59 EAs, 9 NHPIs, 16 Whites, and 10 Others) patients were identified. Mean age ± standard deviation (in years) of symptom onset/diagnosis were both youngest in NHPIs (64.0 ± 7.2/66.3 ± 8.0) followed by Whites (70.8 ± 7.6/73.9 ± 7.8), then EAs (75.9 ± 8.2/79.2 ± 8.3) (P < 0.001). Median survival from diagnosis was significantly lower (P < 0.05) in NHPIs (2 years) compared with EAs (4 years) and Whites (6 years). CONCLUSIONS: There may be racial disparities for PSP, and studies are needed to identify genetic, environmental, and socioeconomic contributions. © 2023 International Parkinson and Movement Disorder Society.

Utilization and gender disparities of Deep Brain Stimulation surgery amongst Asian Americans, Native Hawaiians, and Other Pacific Islanders with Parkinson's disease in Hawai`i.

INTRODUCTION: Despite its efficacy in Parkinson's disease (PD) management, Deep Brain Stimulation (DBS) is underutilized in sociodemographic minorities. Previous investigations of racial disparities in PD aggregated Asian American (AA) and Native Hawaiian or other Pacific Islander (NHPI) populations into a single category; however, these groups have significant health differences. We sought to characterize the PD population in Hawai`i and the use of DBS among AA subgroups and NHPI patients to elucidate potential sociodemographic and clinical disparities. METHODS: Retrospective chart review of PD patients who received DBS from 2002 to 2021 was conducted at The Queen's Medical Center on Oahu, Hawai`i. Hawai`i PD admissions from 2016 to 2020 were collected from Laulima Data Alliance database. We compared the characteristics of DBS patients, total PD admissions, and Hawai`i census data. Alpha level of < 0.05 determined statistical significance. We did a subgroup analysis of white, AA and NHPI subgroups within the patients who underwent DBS. RESULTS: Analysis included 4215 PD admissions and 74 DBS surgeries. Compared to census data, Whites (OR: 1.67; p < 0.0001) and AA (OR: 1.18; p < 0.0001) were overrepresented in total PD admissions; whereas NHPI (OR: 0.64; p < 0.0001) and Blacks (OR: 0.17; p < 0.0001) were underrepresented. Overall, males received DBS more than females. All NHPI patients who received DBS were male, despite 37.65 % of total NHPI PD admissions being female (p = 0.0049). Most DBS patients were AA (45.95 %), followed by Whites (43.24 %), and NHPI (10.81 %). CONCLUSIONS: NHPI and Black PD patients were disproportionately underrepresented in the Hawai`i PD population. All NHPI receiving DBS were male. These racial and gender disparities must be explored in future studies to achieve health equity and improved quality of care in a culturally sensitive manner.

What Getting COVID-19 Finally at This Stage in the Pandemic Taught Me About Compassion to Others and Myself.

This essay discusses what the author learned when she had COVID-19.

Difference in rural and urban Medicare prescription pattern for Parkinson's disease in Hawai'i.

Background: Medical management of Parkinson's Disease (PD) is becoming complex. Increasing evidence suggests that patients have better outcomes when they are managed by neurologists. However, access to neurologists can be limited in rural areas. Analysis of prescription pattern can provide insight into access gap rural patients face. Methods: This retrospective observational study used National Medicare Provider Utilization and Payment Data: Part D Prescriber Public Use Files from 2013 to 2018. Query was made for levodopa, dopamine agonists and other antiparkinsonian medications. The data elements obtained included drug name, number of prescribers, prescriber specialty, number of claims, number of standardized 30-day Part D prescriptions, and number of Medicare beneficiaries in the state of Hawai'i. Individual prescribing providers were categorized as urban or rural based on their cities of practice. Prescription patterns of urban and rural providers in Hawai'i as well as difference in provider specialty were compared, using standardized 30-day prescriptions as the primary measure of utilization. Results: Practice patterns differed between rural and urban areas. In rural Hawai'i, Rytary, Rotigoitne and selegiline were rarely prescribed. Levodopa percentage was higher in urban Hawai'i. In urban Hawai'i, 74.4% of the prescriptions were provided by movement disorders and general neurologists. In rural Hawai'i, 25.1% of the prescriptions were written by neurologists and 74.9% by general practitioners. Conclusions: In the state of Hawai'i, there is an urban-rural access gap to neurologists as evidenced by Medicare prescription pattern. Further study is needed to understand the reasons for rural-urban differences in prescription patterns and their impact on outcomes.

PET-positive extralimbic presentation of anti-glutamic acid decarboxylase antibody-associated encephalitis.

Anti-glutamic acid decarboxylase (GAD) antibody-associated autoimmune encephalitis has been reported mostly as limbic encephalitis. Only few cases with extralimbic involvement are reported with limited investigation. Here, we report an extensive investigation with MRI, PET, and pathological examination. A 66-year-old Japanese female with a history of hypothyroidism, colon cancer, pheochromocytoma, and thymoma-associated myasthenia gravis presented with generalised tonic-clonic seizures. MRI showed multiple hyperintense lesions and PET showed hypermetabolic lesions in the brain. Biopsy showed non-specific gliosis, microglial proliferation, and perivascular lymphohistiocytic infiltrates. Various neuronal antibodies were negative, except for anti-GAD antibody. Anti-GAD antibody-associated encephalitis is an increasingly recognised CNS disease. Pathophysiology of this encephalitis is unclear. While PET showed hypermetabolic lesions, the biopsy showed non-specific changes. The treatments may include immunosuppressants, IVIg, and plasma exchange. One should consider to measure this antibody, in addition to others, when autoimmune encephalitis is suspected [Published with video sequences] .

Mutations in the gene PRRT2 cause paroxysmal kinesigenic dyskinesia with infantile convulsions.

Paroxysmal kinesigenic dyskinesia with infantile convulsions (PKD/IC) is an episodic movement disorder with autosomal-dominant inheritance and high penetrance, but the causative genetic mutation is unknown. We have now identified four truncating mutations involving the gene PRRT2 in the vast majority (24/25) of well-characterized families with PKD/IC. PRRT2 truncating mutations were also detected in 28 of 78 additional families. PRRT2 encodes a proline-rich transmembrane protein of unknown function that has been reported to interact with the t-SNARE, SNAP25. PRRT2 localizes to axons but not to dendritic processes in primary neuronal culture, and mutants associated with PKD/IC lead to dramatically reduced PRRT2 levels, leading ultimately to neuronal hyperexcitability that manifests in vivo as PKD/IC.

Leptomeningeal metastases from solid tumors (meningeal carcinomatosis).

Leptomeningeal metastases (LM) are increasingly recognized as a devastating complication of solid tumors. Improved treatment of primary malignancy and advances in diagnostic imaging have led to an apparent increase in the number of patients diagnosed with LM. Unfortunately, therapeutic options remain limited. Radiotherapy is used to treat bulky tumor and provide symptomatic relief. Intrathecal chemotherapy benefits a selected subset of patients. The challenge to the future is to delineate the molecular mechanisms underlying LM and to develop novel therapeutic or prophylactic modalities to combat LM.

Finger and face representations in the ipsilateral precentral motor areas in humans.

Several human neuroimaging studies have reported activity in the precentral gyrus (PcG) ipsilateral to the side of hand movements. This activity has been interpreted as the part of the primary motor cortex (M1) that controls bilateral or ipsilateral hand movements. To better understand hand ipsilateral-PcG activity, we performed a functional MRI experiment in eight healthy right-handed adults. Behavioral tasks involved hand or lower face movements on each side or motor imagery of the same movements. Consistent with the known M1 organization, the hand contralateral-PcG activity was centered at the "hand-knob" portion of the PcG; face contralateral-PcG activity was localized ventrolateral to it. Hand ipsilateral-PcG activity was identified in most subjects. However, converging results indicated that this ipsilateral PcG activity was situated in Brodmann's area 6 in both hemispheres. The hand ipsilateral-PcG zones were active not only during hand movements but also face movements. Moreover, the hand ipsilateral-PcG zones revealed substantial imagery-related activity, which also failed to differentiate the hand and face. Statistical analyses confirmed poor effector selectivity of the hand ipsilateral PcG activity during both movement and imagery tasks. From these results, we conclude that the hand ipsilateral-PcG activity in healthy adults probably corresponds to a part of the ventral premotor cortex. In contrast, available evidence suggests that M1 contributes to controlling the ipsilateral hand in children and patients after stroke recovery. It appears that within the human PcG, there are two parallel systems potentially capable of controlling ipsilateral hand movements: ventral premotor cortex and M1. These two systems may be differentially influenced by developmental or pathologic changes.

The gene for paroxysmal non-kinesigenic dyskinesia encodes an enzyme in a stress response pathway.

Paroxysmal non-kinesigenic dyskinesia (PNKD) is characterized by spontaneous hyperkinetic attacks that are precipitated by alcohol, coffee, stress and fatigue. We report mutations in the myofibrillogenesis regulator 1 (MR-1) gene causing PNKD in 50 individuals from eight families. The mutations cause changes (Ala to Val) in the N-terminal region of two MR-1 isoforms. The MR-1L isoform is specifically expressed in brain and is localized to the cell membrane while the MR-1S isoform is ubiquitously expressed and shows diffuse cytoplasmic and nuclear localization. Bioinformatic analysis reveals that the MR-1 gene is homologous to the hydroxyacylglutathione hydrolase (HAGH) gene. HAGH functions in a pathway to detoxify methylglyoxal, a compound present in coffee and alcoholic beverages and produced as a by-product of oxidative stress. Our results suggest a mechanism whereby alcohol, coffee and stress may act as precipitants of attacks in PNKD. Stress response pathways will be important areas for elucidation of episodic disease genetics where stress is a common precipitant of many common disorders like epilepsy, migraine and cardiac arrhythmias.

Exercise-induced dystonia as a preceding symptom of familial Parkinson's disease.

Paroxysmal exercise-induced dystonia can occur with Parkinson's disease (PD), and in rare cases, this can also be the presenting symptom. We report on 2 second cousins (no known consanguinity) who presented with paroxysmal exercise-induced dystonia who later developed clinical features of PD. Although autosomal recessive inheritance was suggested, and the dystonic features further suggest parkin as a possible cause, sequencing for parkin mutations was negative and this family may represent a genetic variant of PD. Further genotype-phenotype studies in this and similar families may give clues to pre-symptomatic symptoms in PD and may reflect a particular phenotype of interest for genetics studies in the future.

The pathogenesis and treatment of optic disc swelling in neurosarcoidosis: a unique therapeutic response to infliximab.

OBJECTIVE: To review the pathogenesis and treatment of optic disc swelling in neurosarcoidosis, including a novel therapeutic response to infliximab. DESIGN AND SETTING: Case reports from an inpatient neurology service. PATIENTS: A 35-year-old woman presented with headache, chronic visual loss, papilledema, and optic atrophy, characteristic of chronic intracranial hypertension. Magnetic resonance imaging showed bifrontal cerebral edema with en plaque frontal pachymeningeal enhancement. Her visual loss progressed despite conventional therapies. The use of the tumor necrosis factor alpha antagonist infliximab maintained functional vision in her right eye. A 57-year-old woman presented with bilateral, subacute, painful visual loss and unilateral papillitis consistent with optic neuritis. Her visual loss responded rapidly to intravenous corticosteroids. The funduscopic examination findings in both patients prompted further clinical investigation, culminating in the diagnosis of neurosarcoidosis. CONCLUSION: Understanding the multiple etiologic mechanisms that produce optic disc swelling in sarcoidosis can help neurologists tailor treatment for patients with neurosarcoidosis who present with this symptom.

Epilepsy in Pregnant Women.

For women of childbearing potential with epilepsy, seizures should be controlled with the smallest dosage of anti-epileptic drug (AED). Treatment with monotherapy should be achieved, if possible. The possibility of AED withdrawal should be considered in appropriate clinical setting prior to conception, and the AED treatment should be optimized prior to conception. Many pregnancies are unplanned, underscoring the need for constant vigilance in streamlining the treatment regimen. Prenatal counseling becomes particularly important, in order that both the physician and patient have open communication and realistic expectations about the course and outcome of a potential pregnancy. All women of childbearing potential with epilepsy should be informed about the known rates of teratogenicity of AEDs, possibility of increased seizure frequency during pregnancy, and the risks of the pregnancy and labor. All of the conventional AEDs are associated with an increased risk of major and minor anomalies in the offspring and are categorized as US Food and Drug Administration class C or D. Polytherapy increases this risk. Valproic acid and carbamazepine are each associated with an increased risk of neural tube defects, and should be avoided by women with a family history of spina bifida. This combination should be avoided, if possible. When a woman with epilepsy presents with pregnancy, a monotherapy regimen should not be changed if the seizures are well controlled. Reducing the number of AEDs can be considered in case of polytherapy, if the seizures are well controlled. If seizures are poorly controlled, adequate seizure control is the primary goal. Serum AED levels should be documented prior to conception, and within each trimester. More frequent monitoring may be necessary in case of poorly controlled seizures. If seizures have occurred during pregnancy, therapeutic AED levels should be documented in the late third trimester, prior to delivery. Phenytoin levels should also include an unbound fraction ("free" level); other unbound AED levels are not generally available. The dose adjustment should be made taking the whole clinical picture into account. Vitamin K 10 mg per day orally should be administered in the last 4 weeks of pregnancy for women taking hepatic enzyme-inducing AEDS (phenytoin, phenobarbital, primidone, carbamazepine, topiramate, and oxcarbazepine). The newborn should receive vitamin K 1 mg intravenously or intramuscularly regardless of maternal AED exposure.