RESUMO

Abstract Neurosarcoidosis (NS) is a rare subtype of sarcoidosis with a poor prognosis and diverse clinical presentations that often poses a diagnostic and therapeutic challenge. We describe the case of a 53-year-old male with an initial diagnosis of lingual sarcoidosis, who subsequently developed ataxia and rapidly progressive cognitive im pairment. A lumbar puncture revealed hypoglycorrha chia, hyperproteinorrachia, lymphocytic pleocytosis, and elevated IL-6 levels (600 pg/ml). Cerebrospinal fluid flow cytometry showed an elevated CD4 lymphocyte con centration and a CD4+/CD8+ ratio of 3.91, indicative of NS. Brain MRI showed hyperintense periventricular and subcortical lesions on FLAIR/T2 resembling progressive multifocal leukoencephalopathy (PML), although nega tive PCR for JC virus ruled out the differential diagnosis. Following a favorable evolutionary course with cortico steroid pulses, the patient relapsed with normotensive hydrocephalus, treated with immunosuppressants and ventriculoperitoneal shunting with a good response to date. This case underscores the importance of maintain ing a high index of suspicion for NS in individuals with sarcoidosis and neurologic symptoms. In these cases, ce rebrospinal fluid biomarkers such as IL-6 and CD4+/CD8+ ratio are essential to guide the diagnosis. Furthermore, it highlights that hydrocephalus is a rare complication and requires a multidisciplinary approach, including medical and neurosurgical treatment.

Resumen La neurosarcoidosis es un subtipo raro de sarcoidosis con mal pronóstico y diversas presentaciones clínicas que a menudo plantea un reto diagnóstico y terapéutico. Describimos el caso de un varón de 53 años con diagnóstico inicial de sarcoidosis lingual, que posterior mente desarrolló ataxia y deterioro cognitivo de rápida evolución. Una punción lumbar reveló hipoglucorraquia, hiperproteinorraquia, pleocitosis linfocítica y niveles elevados de IL-6 (600 pg/ml). La citometría de flujo del líquido cefalorraquídeo mostró una concentración ele vada de linfocitos CD4 y un cociente CD4+/CD8+ de 3.91, indicativo de neurosarcoidosis. La RM cerebral evidenció lesiones hiperintensas periventriculares y subcorticales en FLAIR/T2 que se asemejaban a una leucoencefalopa tía multifocal progresiva (LMP), aunque la PCR negativa para el virus JC descartó el diagnóstico diferencial. Tras un curso evolutivo favorable con pulsos de corticoides, el paciente recayó con hidrocefalia normotensiva, tratada con inmunosupresores y derivación ventriculoperitoneal con buena respuesta hasta la fecha. Este caso subraya la importancia de mantener un alto índice de sospecha de neurosarcoidosis en individuos con sarcoidosis y síntomas neurológicos. En estos casos, los biomarcadores del líquido cefalorraquídeo tales como la IL-6 y el cociente CD4+/CD8+ son esenciales para orientar el diagnóstico. Además, destaca que la hidro cefalia es una complicación poco frecuente y requiere un abordaje multidisciplinario, que incluya tratamiento médico y neuroquirúrgico.

RESUMO

Many studies found that increased arterial stiffness is significantly associated with the presence and progression of Coronary Calcium Score (CCS). However, none so far have used machine learning algorithms to improve their value. Therefore, this study aims to evaluate the association between carotid-femoral Pulse Wave Velocity (cfPWV) and CCS score through computational clustering. We conducted a retrospective cross-sectional study using data from a cardiovascular risk screening program that included 377 participants. We used an unsupervised clustering algorithm using age, weight, height, blood pressure, heart rate, and cfPWV as input variables. Differences between cluster groups were analyzed through Chi-square and T-student tests. The association between (i) cfPWV and age groups, (ii) log (CCS) and age groups, and (iii) cfPWV and log(CCS) were addressed through linear regression analysis. Clusters were labeled post hoc based on cardiovascular risk. A "higher-risk group" had significantly higher left (0.76 vs. 0.70 mm, P < 0.001) and right (0.71 vs. 0.66 mm, P = 0.003) intima-media thickness, CCS (42 vs. 4 Agatston units, P = 0.012), and ascending (3.40 vs. 3.20 cm, P < 0.001) and descending (2.60 vs. 2.37 cm, P < 0.001) aorta diameters. Association with age appeared linear for cfPWV and exponential for log (CCS). The progression of the log (CCS) and cfPWV through age groups was steeper in the "higher-risk group" than in the "lower-risk group". cfPWV strongly correlated with CCS, and CCS progression over cfPWV differed among clusters. This finding could improve PWV as a "gate-keeper" of CCS testing and potentially enhance cardiovascular risk stratification.