Clinical, cerebrospinal fluid and neuroimaging findings in COVID-19 encephalopathy: a case series

ObjectiveTo describe the clinical, neurological, neuroimaging and cerebrospinal fluid (CSF) findings associated with encephalopathy in patients admitted to a COVID-19 tertiary reference center. MethodsWe retrospectively reviewed records of consecutive patients with COVID-19 evaluated by a consulting neurology team from March 30, 2020 through May 15, 2020. ResultsFifty-five patients with confirmed SARS-CoV-2 were included, 43 of whom showed encephalopathy, and were further divided into mild, moderate and severe encephalopathy groups. Nineteen patients (44%) had undergone mechanical ventilation and received intravenous sedatives. Eleven (26%) patients were on dialysis. Laboratory markers of COVID-19 severity were very common in encephalopathy patients, but did not correlate with the severity of encephalopathy. Thirty-nine patients underwent neuroimaging studies, which showed mostly non-specific changes. One patient showed lesions possibly related to CNS demyelination. Four had suffered an acute stroke. SARS-CoV-2 was detected by RT-PCR in only one of 21 CSF samples. Two CSF samples showed elevated white blood cell count and all were negative for oligoclonal bands. In our case series the severity of encephalopathy correlated with higher probability of death during hospitalization (OR = 5.5 for each increment in the degree of encephalopathy, from absent (0) to mild (1), moderate (2) or severe (3), p<0.001). ConclusionIn our consecutive series with 43 encephalopathy cases, neuroimaging and CSF analysis did not support the role of direct viral CNS invasion or CNS inflammation as the cause of encephalopathy.

Screening for cognitive impairmentin late onset depression in a Brazilian sample using the BBRC-EDU / Rastreio de comprometimento cognitivo na depressão de início tardio em uma amostra brasileira usando o BBRC-EDU

ABSTRACT: Depression and dementia are the most prevalent neuropsychiatric disorders in the elderly population. Alzheimer's disease is the leading cause of dementia in most countries, being responsible for more than half of all dementia cases. Late-onset depression is a frequent cause of cognitive decline in the elderly. Differentiating between cognitive impairment secondary to depression and incipient dementia poses a challenge in the clinical setting. Objective: To evaluate the performance of elderly depressed patients using the BBRC-Edu. Methods: We studied 25 patients with late onset depression (mean age: 73.6 y (6.6); schooling: 9.1 y (5.7)) and 30 patients with mild AD (mean age 76.6 y (5.4); schooling: 7.5 y (7.1)), who were compared to a control group of 30 healthy elderly (mean age 73.8 y (5.8); schooling: 9.1 y (5.4)) using the CERAD and BBRC-Edu batteries. Results: For the CERAD battery, depressed patients performed better than AD patients on all tasks (p<0.0001) except for Constructional Praxis (p>0.05), and performed poorer than controls on verbal fluency (animals) and Word List Recall tasks (p<0.0001). For the BBRC-Edu, depressed patients performed better than AD patients on all tasks (p<0.0001) except for Digit Span (direct order) (p=0.076) and Incidental Memory (p>0.05), and performed worse than controls on Learning (second presentation) and verbal fluency (fruits) tasks (p<0.0001). Conclusion: Overall performance on the BBRC-Edu allowed differentiation of controls and depressed patients from AD patients.

RESUMO: Depressão e demência são os transtornos neuropsiquiátricos de maior prevalência na população idosa. A doença de Alzheimer é a principal causa de demência na maioria os países, sendo a responsável por mais da metade dos casos de demência. Depressão de início tardio é uma causa frequente de declínio cognitivo no idoso. A diferenciação entre transtorno cognitivo secundário à depressão e demência em fase inicial é um desafio na prática clínica. Objetivo: Avaliar o desempenho de pacientes idosos deprimidos usando a Bateria Breve de Rastreio Cognitivo (BBRC-Edu). Métodos: Estudamos 25 pacientes com depressão de início tardio, (idade média 73,6 (6,6); escolaridade: 9,1 (5,7)) e 30 pacientes com DA leve (CDR=1), (idade média 76,6 (5,4); escolaridade: 7,5 (7,1)). O desempenho de ambos os grupos foi comparado a um grupo controle de 30 idosos sadios (idade média 73,8 (5,8); escolaridade: 9,1 (5,4)). Resultados: Na Bateria CERAD pacientes deprimidos tiveram desempenho melhor que pacientes com DA em todos os testes (p<0,0001) exceto para Praxia Construcional (p>0,05), e desempenho pior que os controles na fluência verbal (animais) e Recordação da Lista de Palavras (p<0,0001). Na BBRC-Edu, pacientes deprimidos tiveram melhor desempenho comparados aos pacientes com DA em todos os testes (p<0,0001) exceto para Spam de Dígitos (ordem direta) (p=0,076) e Memória Incidental (p>0,05), e tiveram desempenho pior que os controles na Aprendizagem (segunda apresentação) e na fluência verbal (frutas) (p<0,0001). Conclusão: A Bateria Breve de Rastreio Cognitivo permite a diferenciação de controles e pacientes com transtorno depressivo, de pacientes com doença de Alzheimer.

Dementia in Fragile X-associated Tremor/Ataxia Syndrome / Demência na síndrome de tremor-ataxia associada ao X-Frágil

Fragile X-associated tremor/ataxia syndrome (FXTAS) is a cause of movement disorders and cognitive decline which has probably been underdiagnosed, especially if its prevalence proves similar to those of progressive supranuclear palsy and amyotrophic lateral sclerosis. We report a case of a 74-year-old man who presented with action tremor, gait ataxia and forgetfulness. There was a family history of tremor and dementia, and one of the patient?s grandsons was mentally deficient. Neuropsychological evaluation disclosed a frontal network syndrome. MRI showed hyperintensity of both middle cerebellar peduncles, a major diagnostic hallmark of FXTAS. Genetic testing revealed premutation of the FMR1 gene with an expanded (CGG)90 repeat. The diagnosis of FXTAS is important for genetic counseling because the daughters of the affected individuals are at high risk of having offspring with fragile X syndrome. Tremors and cognitive decline should raise the diagnostic hypothesis of FXTAS, which MRI may subsequently reinforce, while the detection of the FMR1 premutation can confirm the condition.

A síndrome de tremor-ataxia associada ao X-frágil (FXTAS) é uma causa de distúrbios do movimento e de declínio cognitivo que provavelmente tem sido subdiagnosticada, especialmente se a sua prevalência for realmente similar às da paralisia supranuclear progressiva e esclerose lateral amiotrófica. Relatamos um caso de um homem de 74 anos que se apresentou com tremor de ação, ataxia de marcha e esquecimento. Havia história familiar de tremor e de demência e um de seus netos era mentalmente deficiente. A avaliação neuropsicológica demonstrou uma síndrome frontal. A ressonância magnética (RM) revelou hiperintensidade de ambos os pedúnculos cerebelares médios, um critério maior para o diagnóstico de FXTAS. Os testes genéticos confirmaram a presença da pré-mutação do gene FMR1, com uma repetição (CGG)90. O diagnóstico de FXTAS é importante para o aconselhamento genético porque as filhas dos indivíduos afetados tem alto risco de ter uma criança com síndrome do X-frágil. A presença de tremores e declínio cognitivo deve levantar a hipótese diagnóstica de FXTAS, que poderá ser reforçada pela RM e confirmada pela presença da pré-mutação do gene FMR1.