Neurocognitive profile of patients with X-linked dystonia-parkinsonism.

X-linked dystonia-parkinsonism (XDP) is a debilitating movement disorder endemic to the Panay Island, Philippines. Most studies focus on motor symptoms, hence we reviewed the neurocognitive profile of XDP patients. Neurocognitive testing of XDP patients focused on five domains: general intellectual functioning, episodic memory, language, attention and executive function, and affect. Twenty-nine genetically confirmed patients were included. Twenty-six (89.6%) had impairments in one or more domains, while only three had no impairment in any domain. Attention and executive function was the most commonly affected domain (n = 23, 79.3%). Deficits in general intellect, episodic memory, attention and executive function and affect were seen in our subset of XDP patients. The striatal pathology affecting the frontostriatal circuitry mandating these cognitive processes is mainly implicated in these impairments. The results of our study provided further evidence on the extent of cognitive impairment in XDP using a select battery of neurocognitive tests.

Utility of phrenic nerve ultrasound in amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder affecting the upper and lower motor neurons causing progressive weakness. It eventually involves the diaphragm which leads to respiratory paralysis and subsequently death. Phrenic nerve (PN) conduction studies and diaphragm ultrasound has been studied and correlated with pulmonary function tests in ALS patients. However, PN ultrasonography has not been employed in ALS. This study aims to sonographically evaluate the morphologic appearance of the PN of ALS patients. Thirty-eight ALS patients and 28 normal controls referred to the neurophysiology laboratory of two institutions were retrospectively included in the study. Baseline demographic and clinical variables such as disease duration, ALS Functional Rating Scale-Revised score, and ALS region of onset were collected. Ultrasound was used to evaluate the PN cross-sectional area (CSA) of ALS and control subjects. The mean PN CSA of ALS patients were 1.08 ± 0.39 mm on the right and 1.02 ± 0.34 mm on the left. The PN CSA of ALS patients were significantly decreased compared to controls (p value < 0.00001). The PN CSA of ALS patients was not correlated to any of the demographic and clinical parameters tested. This study demonstrates that ALS patients have a smaller PN size compared to controls using ultrasonography.

Serotonin syndrome in a Parkinson disease patient after intake of an ethanol-containing homeopathic medication.

Serotonin syndrome is due to excess serotonin in the nervous system. We document a case of an elderly Parkinson disease patient who has been neurologically stable on rasagiline and escitalopram for 1 year but developed serotonin syndrome after intake of an ethanol-containing homeopathic medication. The patient presented with seizures, autonomic dysfunction and neuromuscular hyperexcitability. Maintenance medications were discontinued, hydration, sedation and respiratory support were provided with resolution of the symptoms. The combination of escitalopram and ethanol, both metabolized by the cytochrome P450 enzyme system can lead to serotonin syndrome. Our case highlights the importance of drug interactions in patients taking several medications. Additionally, the intake of medicines, may it be conventional or homeopathic medicine, without the guidance of a trained and competent physician, may lead to serious consequences for the patient.

Generalized chorea associated with subcortical leukoaraiosis of Binswanger type: a case report.

Binswanger disease (BD) involves injuries to the brain small vessels, resulting to gradually progressive subcortical ischemia. This disorder manifests with dementia, gait abnormalities, upper motor signs and parkinsonism, and presents as extensive, confluent, bilateral cerebral white matter hyperintensities in the MRI. Cases of BD typically manifests with vascular risk factors, such as hypertension and multiple strokes. We report a unique case of a Filipino patient whom we have diagnosed with BD presenting with no cardinal signs of parkinsonism, but with generalized choreiform movement disorder and without a history of hypertension and symptomatic strokes. To our knowledge, this is the first report presenting an adult patient with subcortical leukoaraiosis of Binswanger type associated with a hyperkinetic movement disorder.

Incidence, risk factors, and outcomes of unplanned extubation in adult patients in a resource-limited teaching hospital in the Philippines: a cohort study. / Incidência, fatores de risco e desfechos da extubação não planejada em pacientes adultos em um hospital de ensino com recursos limitados nas Filipinas: um estudo de coorte.

OBJECTIVE: We aimed to determine the incidence, risk factors, and outcomes of unplanned extubation among adult patients. METHODS: We conducted a prospective cohort study of adult intubated patients admitted to the charity wards of a government tertiary teaching hospital in the Philippines. Patients managed in both intensive care and nonintensive care settings were included. Patients were followed-up until discharge or until seven days postextubation. RESULTS: The outcomes of the 191 included patients were planned extubation (35%), unplanned extubation (19%), death (39%), and discharge against advice (7%). Competing risk regression showed that male sex (Crude OR: 2.25, 95%CI: 1.10 - 4.63) and age (Crude OR 0.976, 95%CI: 0.957 - 0.996) were significant baseline factors. The night shift (Crude OR: 24.6, 95%CI: 2.87 - 211) was also consistently associated with more unplanned extubations. Among postextubation outcomes, reintubation (unplanned extubation: 61.1% versus planned extubation: 25.4%), acute respiratory failure (unplanned extubation: 38.9% versus planned extubation: 17.5%), and cardiovascular events (unplanned extubation: 8.33% versus planned extubation: 1.49%) occurred significantly more often among the unplanned extubation patients. Admission in an intensive care unit was not associated with a lower risk of unplanned extubation (Crude OR 1.15, 95%CI: 0.594 - 2.21). CONCLUSION: Many intubated patients had unplanned extubation. Patients admitted in nonintensive care unit settings did not have significantly higher odds of unplanned extubation.

OBJETIVO: Determinar a incidência, os fatores de risco e os desfechos da extubação não planejada em pacientes adultos. MÉTODOS: Conduzimos estudo prospectivo de coorte de pacientes adultos intubados admitidos em ala de atendimento gratuito em um hospital governamental terciário de ensino nas Filipinas. Incluíram-se tanto pacientes em cuidados de terapia intensiva quanto fora dela. Os pacientes foram seguidos até a alta ou até o sétimo dia após a extubação. RESULTADOS: Os desfechos dos 191 pacientes incluídos foram: extubação planejada (35%), extubação não planejada (19%), óbito (39%) e alta a pedido (7%). A regressão de riscos competitivos demonstrou que o sexo masculino (OR bruta de 2,25; IC95% 1,10 - 4,63) e a idade (OR bruta: 0,976; IC95%: 0,957 - 0,996) foram fatores basais significantes. O turno da noite (OR bruta: 24,6; IC95%: 2,87 - 211) também teve associação consistente com maior ocorrência de extubação não planejada. Dentre os desfechos após a extubação, ocorreram significantemente mais, entre os pacientes com extubação não planejada, reintubação (extubação não planejada, com 61,1%, versus extubação planejada, com 25,4%), insuficiência respiratória aguda (extubação não planejada, com 38,9%, versus extubação planejada, com 17,5%) e eventos cardiovasculares (extubação não planejada, com 8,33%, versus extubação planejada, com 1,49%). A admissão à unidade de terapia intensiva não se associou com risco menor de extubação não planejada (OR bruta de 1,15; IC95% 0,594 - 2,21). CONCLUSÃO: Muitos pacientes intubados tiveram extubação não planejada. Os pacientes admitidos em outras unidades, que não a de terapia intensiva, não tiveram tendências mais elevadas de extubação não planejada.

Incidência, fatores de risco e desfechos da extubação não planejada em pacientes adultos em um hospital de ensino com recursos limitados nas Filipinas: um estudo de coorte / Incidence, risk factors, and outcomes of unplanned extubation in adult patients in a resource-limited teaching hospital in the Philippines: a cohort study

RESUMO Objetivo: Determinar a incidência, os fatores de risco e os desfechos da extubação não planejada em pacientes adultos. Métodos: Conduzimos estudo prospectivo de coorte de pacientes adultos intubados admitidos em ala de atendimento gratuito em um hospital governamental terciário de ensino nas Filipinas. Incluíram-se tanto pacientes em cuidados de terapia intensiva quanto fora dela. Os pacientes foram seguidos até a alta ou até o sétimo dia após a extubação. Resultados: Os desfechos dos 191 pacientes incluídos foram: extubação planejada (35%), extubação não planejada (19%), óbito (39%) e alta a pedido (7%). A regressão de riscos competitivos demonstrou que o sexo masculino (OR bruta de 2,25; IC95% 1,10 - 4,63) e a idade (OR bruta: 0,976; IC95%: 0,957 - 0,996) foram fatores basais significantes. O turno da noite (OR bruta: 24,6; IC95%: 2,87 - 211) também teve associação consistente com maior ocorrência de extubação não planejada. Dentre os desfechos após a extubação, ocorreram significantemente mais, entre os pacientes com extubação não planejada, reintubação (extubação não planejada, com 61,1%, versus extubação planejada, com 25,4%), insuficiência respiratória aguda (extubação não planejada, com 38,9%, versus extubação planejada, com 17,5%) e eventos cardiovasculares (extubação não planejada, com 8,33%, versus extubação planejada, com 1,49%). A admissão à unidade de terapia intensiva não se associou com risco menor de extubação não planejada (OR bruta de 1,15; IC95% 0,594 - 2,21). Conclusão: Muitos pacientes intubados tiveram extubação não planejada. Os pacientes admitidos em outras unidades, que não a de terapia intensiva, não tiveram tendências mais elevadas de extubação não planejada.

ABSTRACT Objective: We aimed to determine the incidence, risk factors, and outcomes of unplanned extubation among adult patients. Methods: We conducted a prospective cohort study of adult intubated patients admitted to the charity wards of a government tertiary teaching hospital in the Philippines. Patients managed in both intensive care and nonintensive care settings were included. Patients were followed-up until discharge or until seven days postextubation. Results: The outcomes of the 191 included patients were planned extubation (35%), unplanned extubation (19%), death (39%), and discharge against advice (7%). Competing risk regression showed that male sex (Crude OR: 2.25, 95%CI: 1.10 - 4.63) and age (Crude OR 0.976, 95%CI: 0.957 - 0.996) were significant baseline factors. The night shift (Crude OR: 24.6, 95%CI: 2.87 - 211) was also consistently associated with more unplanned extubations. Among postextubation outcomes, reintubation (unplanned extubation: 61.1% versus planned extubation: 25.4%), acute respiratory failure (unplanned extubation: 38.9% versus planned extubation: 17.5%), and cardiovascular events (unplanned extubation: 8.33% versus planned extubation: 1.49%) occurred significantly more often among the unplanned extubation patients. Admission in an intensive care unit was not associated with a lower risk of unplanned extubation (Crude OR 1.15, 95%CI: 0.594 - 2.21). Conclusion: Many intubated patients had unplanned extubation. Patients admitted in nonintensive care unit settings did not have significantly higher odds of unplanned extubation.

Quality of life and Parkinson's disease: Philippine translation and validation of the Parkinson's disease questionnaire.

The 39-item Parkinson's Disease Quality of Life Questionnaire (PDQ-39) is the most widely used self-administered questionnaire assessing the quality of life of Parkinson's disease (PD) patients. We translated the PDQ-39 into the Filipino language as the Philippine-PDQ-39 (P-PDQ-39) and used it to assess the quality of life of Filipino patients with PD. The original PDQ-39 was translated into Filipino using forward and backward translation by independent bilingual translators and used among 100 consecutively sampled PD patients. Baseline demographic data, disease duration and levodopa equivalent dose were collected. Internal consistency was measured using Chronbach's α. The following rating scales and questionnaires were correlated with P-PDQ-39 through Pearson's correlation coefficient to assess construct validity: modified Hoehn & Yahr Stage, Hospital Anxiety and Depression Scale-Pilipino (HADS-P), Unified Parkinson's Disease Rating Scale (UPDRS) Parts I-III, and Non-motor symptom Assessment Scale for Parkinson's Disease (NMSS). The mobility and activities of daily living domains of the P-PDQ-39 were well correlated with the Hoehn & Yahr stage of the patients and the UPDRS II and III scores emphasizing the value of motor symptoms in quality of life. Non motor symptoms in PD were also important determinants of quality of life as demonstrated in the correlation of HADS-P and NMSS Total scores with the P-PDQ 39 domains. The P-PDQ-39 is a valid assessment tool for assessing the quality of life of Filipino-speaking PD patients.

Paediatric non-ketotic hyperglycaemic hemichorea-hemiballismus.

Non-ketotic hyperglycaemic hemichorea-hemiballismus (NHHH) is commonly seen among elderly Asian women with type 2 diabetes mellitus. Here, we present a case of a 16-year-old Filipina with type 1 diabetes mellitus who is poorly compliant to her medications and subsequently developed right hemichorea-hemiballismus (HH). She was initially admitted with hyperglycaemia but was negative for ketonuria or metabolic acidosis. Neuroimaging showed bilateral lentiform nuclei and left caudate hyperdensities on CT and T1-weighted hyperintensity on MRI. Blood glucose was controlled with insulin. Haloperidol and clonazepam were started for the HH with gradual resolution of symptoms in 6 weeks. This is the fifth reported case of NHHH seen among the paediatric age group. NHHH in the paediatric population is clinically and radiographically similar to NHHH seen among adults. Correction of hyperglycaemia results in clinical improvement and radiographic resolution of lesions but persistent cases may necessitate specific treatment targeted towards the abnormal movements.