Encefalitis letárgica. La epidemia en los albores de la neurología / Encephalitis lethargica. The epidemic at the dawn of neurology

La encefalitis letárgica es un cuadro neurológico con una variada gama de manifestaciones clínicas en el ámbito neurológico y también en el psiquiátrico. El cuadro se ha presentado de manera epidémica en brotes que han seguido a los de la gripe. El último brote acaecido a comienzos del siglo XX lo describió en profundidad Constantin von Economo. La epidemia notificada inicialmente en Europa y luego en Norteamérica se presentó también en otras latitudes, incluyendo Chile. Así, las descripciones de Lea-Plaza, Tello, Iturra, Cienfuegos y otros médicos chilenos dieron cuenta del cuadro en Chile con toda la complejidad que también tuvo en Europa. El origen sigue siendo un misterio, aunque la evidencia creciente de que fuera autoinmune gana fuerza con los hallazgos de la tecnología médica actual. En este trabajo presentamos el cuadro, privilegiando la riqueza clínica y la belleza de las descripciones realizada por los médicos de la época en que esta enfermedad se presentó

Lethargic encephalitis is a neurological illness that shows a wide range of symptoms and signs, including neurological and psychiatric spectrum. It presented in an epidemic way, following influaenza relapses. The last relapse started at the beginning of 20th century and it was deeply described by Constantin von Economo. The illness described first in Europe and North America, was described in many others countries including Chile. There were beautiful descriptions by Chilean physicians like Lea-Plaza, Tello, Iturra and Cienfuegos. Their works showed the complexity of the illness like European physicians did too. The etiology is still unknown; however growing evidence about autoinmune aetiology is gaining force with the use of actual medical technology. In this work, we show encephalitis lethargica, focusing in clinical picture, the beauty of medical descriptions that physicians did at this date

Plasma urate in REM sleep behavior disorder.

BACKGROUND: Rapid eye movement (REM) sleep behavior disorder (RBD) is associated with a high risk of developing Parkinson's disease (PD). Higher urate levels are associated with a lower risk of PD. We conducted a study to evaluate plasma urate levels in patients with RBD and their role in the development of PD. METHODS: We evaluated plasma urate levels in a cohort of 24 patients with idiopathic RBD. Patients were divided into 2 groups according to the presence or absence of PD. Other known markers of the risk of developing PD, such as olfaction testing, and substantia nigra (SN) hyperechogenicity, were evaluated in the 2 groups. RESULTS: No differences were observed regarding age, years of evolution of the RBD, SN hyperechogenicity, or plasma urate levels between the 2 groups. In patients without PD, there was a positive correlation between years of evolution of RBD and the levels of uric acid (R(2) =0.88). Patients without PD and those who had more than 5 years of RBD exhibited higher levels of uric acid than patients with PD (P=0.02). CONCLUSIONS: Higher levels of plasma urate were associated with a longer duration of RBD without converting to PD. Future prospective studies would be needed to confirm this finding. Disorder Society

[Evaluation of olfaction in healthy subjects using the Sniffing Sticks battery]. / Valores normales de olfato, hiposmia y anosmia en población chilena sana según la batería "Sniffin Sticks"

BACKGROUND: Olfaction dysfunction is linked to neurodegenerative diseases, therefore the evaluation of this function is becoming important AIM: To evaluate olfaction in healthy participants. MATERIAL AND METHODS: We evaluated 44 healthy males and 55 females, aged 21 to 89 years with the Sniffing Sticks battery to determine normal values in Chilean population. During the test, participants must correctly identify 12 different odors. RESULTS: Normal olfaction, hyposmia and anosmia were defined. An age related decline in olfaction was observed, especially among males aged 59 years or more. CONCLUSIONS: This study provides age and gender specific normal values for the Sniffing Sticks battery.

Valores normales de olfato, hiposmia y anosmia en población chilena sana según la batería "sniffin sticks" / Evaluation of olfaction in healthy subjects using the sniffing sticks battery

Background: Olfaction dysfunction is linked to neurodegenerative diseases, therefore the evaluation of this function is becoming important Aim: To evaluate olfaction in healthy participants. Material and Methods: We evaluated 44 healthy males and 55 females, aged 21 to 89 years with the sniffing sticks battery to determine normal values in Chilean population. During the test, participants must correctly identify 12 different odors. Results: Normal olfaction, hyposmia and anosmia were defined. An age related decline in olfaction was observed, especially among males aged 59 years or more. Conclusions: This study provides age and gender specific normal values for the sniffing sticks battery.

Transcranial sonography in the discrimination of Parkinson's disease versus vascular parkinsonism.

Cerebro-vascular disease is a well-known cause of parkinsonism. However, so far, there are no standardized clinical criteria that allow the diagnosis with accuracy and reliability. Rather, there is a great symptom overlap between idiopathic Parkinson's disease, other causes for parkinsonism, and vascular parkinsonism. Therefore, additional instruments are necessary to improve diagnostic accuracy. Transcranial sonography of brain parenchyma (TCS) has become a broadly applied tool in the diagnosis of Parkinson's disease, and secondary and atypical parkinsonian syndromes. In contrast to patients with idiopathic Parkinson's disease, patients with vascular parkinsonism in general show no hyperechogenicity of the substantia nigra. In contrast to a number of patients with atypical parkinsonian syndromes, also the basal ganglia are usually normoechogenic on TCS. A more specific approach to vascular parkinsonism includes the Doppler or duplex technique in order to show stenosis of vessels. Therefore, the combination of TCS and Doppler/duplex imaging might help to improve diagnosis of vascular parkinsonism transcranial.

Clinical and genetic analysis of a Chilean family with early-onset autosomal dominant Alzheimer's disease.

Autosomal dominant early-onset Alzheimer's disease (ADEOAD) is associated predominantly with mutations in the genes that codify for presenilin 1 (PSEN1). Only a few ADEOAD families have been reported from Latin America. This is an extended Chilean pedigree affected by ADEOAD along 4 generations. The age of onset of dementia was between 38 and 42 years. Early manifestations were anxiety and depression. Mutation analysis revealed a heterozygous G to C transversion at position 438 of the mRNA in PSEN1 in all affected members. This is the first report of a Chilean family with ADEOAD to include mutation analysis.