The ability of patients with Parkinson's disease to recognize masked faces during the COVID-19 pandemic / A capacidade dos pacientes com doença de parkinson de reconhecer rostos mascarados durante a pandemia de covid-19

ABSTRACT. Patients with Parkinson's disease (PwP) have face recognition difficulties. Objective: This study aimed to evaluate the difficulties of PwP in recognizing masked faces during the COVID-19 pandemic. Methods: A total of 64 PwP, 58 age-matched older healthy controls (OHCs), and 61 younger healthy controls (YHCs) were included in the study. The Benton Face Recognition Test - short form (BFRT-sf) and the 13-item questionnaire on face recognition difficulties due to masks during the pandemic developed by the authors were applied to all three study groups. Results: Both the PwP and OHC groups scored worse in BFRT-sf when compared with the YHC group (p<0.001 and p<0.001, respectively). The number of those who had difficulty in recognizing people seen every day and the number of those who asked people to remove their masks because they did not recognize them were higher in the PWP group (p=0.026 and p=0.002, respectively). The number of individuals who looked at the posture and gait of people when they did not recognize their masked faces and those who stated that this difficulty affected their daily lives were higher in the OHC group (p=0.002 and p=0.009, respectively). The number of participants whose difficulty in recognizing masked faces decreased over time was higher in the YHC group (p=0.003). Conclusions: The PwP group demonstrated similar performance to their peers but differed from the YHC group in recognizing masked faces. Knowing difficulties experienced by elderly people in recognizing people who are masked can increase awareness on this issue and enhance their social interaction in pandemic conditions through measures to be taken.

RESUMO. Pacientes com doença de Parkinson (PcP) têm dificuldades de reconhecimento facial. Objetivo: Avaliamos as dificuldades de PcP em reconhecer rostos mascarados durante a pandemia de COVID-19. Métodos: Incluímos 64 PcP, 58 controles saudáveis ​​mais velhos (CSVs) pareados por idade, 61 controles saudáveis mais jovens (CSJs) no estudo. O Benton Face Recognition Test-short form (BFRT-sf) e o questionário de 13 itens sobre dificuldades de reconhecimento facial devido a máscaras durante a pandemia desenvolvido pelos autores foram aplicados a todos os três grupos de estudo. Resultados: Ambos os grupos PcP e CSV tiveram pior pontuação no BFRT-sf quando comparados com o grupo CSJ (p<0,001 e p<0,001, respectivamente). O número daqueles que tiveram dificuldade em reconhecer as pessoas atendidas todos os dias e o número daqueles que pediram para as pessoas retirarem suas máscaras por não as reconhecer foram maiores no grupo PcP (p=0,026 e p=0,002, respectivamente). O número de indivíduos que olharam para a postura e marcha das pessoas quando não reconheceram seus rostos mascarados e aqueles que afirmaram que essa dificuldade afetou seu cotidiano foi maior no grupo CSV (p=0,002 e p=0,009, respectivamente). O número de participantes cuja dificuldade em reconhecer rostos mascarados diminuiu ao longo do tempo foi maior no grupo CSJ (p=0,003). Conclusões: O grupo PcP demonstrou desempenho semelhante aos seus pares, mas diferiu do grupo CSJ no reconhecimento de rostos mascarados. Conhecer as dificuldades vivenciadas pelos idosos em reconhecer as pessoas mascaradas pode aumentar a conscientização sobre essa questão e potencializar sua interação social em condições de pandemia por meio de medidas a serem tomadas.

The ability of patients with Parkinson's disease to recognize masked faces during the COVID-19 pandemic.

Patients with Parkinson's disease (PwP) have face recognition difficulties. Objective: This study aimed to evaluate the difficulties of PwP in recognizing masked faces during the COVID-19 pandemic. Methods: A total of 64 PwP, 58 age-matched older healthy controls (OHCs), and 61 younger healthy controls (YHCs) were included in the study. The Benton Face Recognition Test - short form (BFRT-sf) and the 13-item questionnaire on face recognition difficulties due to masks during the pandemic developed by the authors were applied to all three study groups. Results: Both the PwP and OHC groups scored worse in BFRT-sf when compared with the YHC group (p<0.001 and p<0.001, respectively). The number of those who had difficulty in recognizing people seen every day and the number of those who asked people to remove their masks because they did not recognize them were higher in the PWP group (p=0.026 and p=0.002, respectively). The number of individuals who looked at the posture and gait of people when they did not recognize their masked faces and those who stated that this difficulty affected their daily lives were higher in the OHC group (p=0.002 and p=0.009, respectively). The number of participants whose difficulty in recognizing masked faces decreased over time was higher in the YHC group (p=0.003). Conclusions: The PwP group demonstrated similar performance to their peers but differed from the YHC group in recognizing masked faces. Knowing difficulties experienced by elderly people in recognizing people who are masked can increase awareness on this issue and enhance their social interaction in pandemic conditions through measures to be taken.

Pacientes com doença de Parkinson (PcP) têm dificuldades de reconhecimento facial. Objetivo: Avaliamos as dificuldades de PcP em reconhecer rostos mascarados durante a pandemia de COVID-19. Métodos: Incluímos 64 PcP, 58 controles saudáveis ​​mais velhos (CSVs) pareados por idade, 61 controles saudáveis mais jovens (CSJs) no estudo. O Benton Face Recognition Test-short form (BFRT-sf) e o questionário de 13 itens sobre dificuldades de reconhecimento facial devido a máscaras durante a pandemia desenvolvido pelos autores foram aplicados a todos os três grupos de estudo. Resultados: Ambos os grupos PcP e CSV tiveram pior pontuação no BFRT-sf quando comparados com o grupo CSJ (p<0,001 e p<0,001, respectivamente). O número daqueles que tiveram dificuldade em reconhecer as pessoas atendidas todos os dias e o número daqueles que pediram para as pessoas retirarem suas máscaras por não as reconhecer foram maiores no grupo PcP (p=0,026 e p=0,002, respectivamente). O número de indivíduos que olharam para a postura e marcha das pessoas quando não reconheceram seus rostos mascarados e aqueles que afirmaram que essa dificuldade afetou seu cotidiano foi maior no grupo CSV (p=0,002 e p=0,009, respectivamente). O número de participantes cuja dificuldade em reconhecer rostos mascarados diminuiu ao longo do tempo foi maior no grupo CSJ (p=0,003). Conclusões: O grupo PcP demonstrou desempenho semelhante aos seus pares, mas diferiu do grupo CSJ no reconhecimento de rostos mascarados. Conhecer as dificuldades vivenciadas pelos idosos em reconhecer as pessoas mascaradas pode aumentar a conscientização sobre essa questão e potencializar sua interação social em condições de pandemia por meio de medidas a serem tomadas.

Patients with cerebrotendinous xanthomatosis diagnosed with diverse multisystem involvement.

Cerebrotendinous xanthomatosis (CTX) is a lipid storage disease caused by deficiency of sterol 27-hydroxylase enzyme encoded by CYP27A1 gene. This multicenter, cross-sectional descriptive study aimed to document clinical characteristics of CTX patients of different ages, clinical presentations of early-diagnosed patients, and responses to short-term chenodeoxycholic acid (CDCA) treatment. Seven of 11 CTX patients were diagnosed in childhood. Three patients (27%) had neonatal cholestasis, seven (63%) patients had a history of frequent watery defecation started in infantile period, and eight (72.7%) patients had juvenile cataract. Four patients in the adult age group had pyramidal signs and parkinsonism symptoms. The mean Mignarri score at diagnosis was significantly lower in the pediatric patients (267.8 ± 51.4) than in the adult patients (450.0 ± 64.0, p = 0.001). No significant difference was determined between pediatric patients and adult patients regarding plasma cholestanol concentration at diagnosis (p = 0.482). The frequency of defecation decreased with treatment in six children, who had diarrhea at admission. Compared to pretreatment values, patients' body weight and standardized body mass index significantly increased at the 12th month of treatment. In conclusion, Mignarri scores are lower in the pediatric patients than in adult patients since the most determinative signs of the CTX disease are not apparent yet in the childhood. The disease is frequently overlooked in routine practice as the disease presents itself with different clinical combinations both in adults and in children. CTX is potentially a treatable disease; thereby, enhanced awareness is critically important for early diagnosis particularly in children.

Holmes' Tremor with Shoulder Pain Treated by Deep Brain Stimulation of Unilateral Ventral Intermediate Thalamic Nucleus and Globus Pallidus Internus

A 21-year-old male was admitted with severe right arm and hand tremors after a thalamic hemorrhage caused by a traffic accident. He was also suffering from agonizing pain in his right shoulder that manifested after the tremor. Neurologic examination revealed a disabling, severe, and irregular kinetic and postural tremor in the right arm during target-directed movements. There was also an irregular ipsilateral rest tremor and dystonic movements in the distal part of the right arm. The amplitude was moderate at rest and extremely high during kinetic and intentional movements. The patient underwent left globus pallidum internus and ventral intermediate thalamic nucleus deep brain stimulation. The patient improved by more than 80% as rated by the Fahn-Tolosa-Marin Tremor Rating Scale and Visual Analog Scale six months after surgery.