Retracción palpebral de causa neurológica: Presentación de tres pacientes, interpretación patogénica

R, Luis Cartier; S, Jorge Guzmán; F, Renzo Pasquali

doi:10.4067/s0034-98872017000200016

Cited by 2 publications

(2 citation statements)

References 8 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Pueden presentar "visión borrosa" que en ellos sugiere ceguera cortical. Algunos pacientes con pérdida de la visión presentan sobre-apertura palpebral, que define la "mirada hierática" de la ECJ 29 . El diagnóstico suele confirmarse con la aparición de mioclonías de cara y/o extremidades, generalmente plurisegmentarias.…”

Section: Formas Clínicas De La Enfermedad De Creutzfeldt-jakobunclassified

Enfermedad por priones, encefalopatía espongiforme humana y enfermedad de Creutzfeldt-Jakob

Cartier-Rovirosa

2019

Rev. méd. Chile

View full text Add to dashboard Cite

The infectious protein or prion (PrPSC) is a transmissible and replicable polypeptide, which arises from an abnormal folding of the PrP protein, by unknown mechanisms and without changes in the primary sequence of its amino acids. Its new spatial disposition arises from the substitution of its alpha helices by beta bands, which increase its structural stability, avoiding its complete proteolysis, resulting in a residual accumulation of prions. These prions induce the misfolding of normal PrP protein, generating their exponential increase, leading to a disturbance of neuronal homeostasis which results in the development of the fatal spongiform encephalopathy of the Creutzfeldt-Jakob disease (CJD). This is the most prevalent human prion disease, and 90% of cases are sporadic, suggesting the endogenous genesis of prions. There are different types of prions, identified based on the genetic variance of codon 129 amino acids of the prion protein. Meteonin (M) and Valine (V)), associated with the result of their enzymatic proteolysis, define prions type 1 (21 kDa) and type 2 (19 kDa). The Classical form of CJD produced by MM1 prion occurs in 70% of the cases. The Cerebellar form originated by the VV2 prion occurs in 15% of cases, the form with Kuru plates, associated with the prion MV2 occurs in 5%, and the Vacuolar, related to the MM2 prion occurs in 4%. CJD is always characterized by behavioral, motor, cognitive, and vision alterations and by findings in magnetic resonance imaging, electroencephalogram and cerebrospinal fluid that define each clinical and neuropathological form.

show abstract

Section: Formas Clínicas De La Enfermedad De Creutzfeldt-jakobunclassified

Enfermedad por priones, encefalopatía espongiforme humana y enfermedad de Creutzfeldt-Jakob

Cartier-Rovirosa

2019

Rev. méd. Chile

View full text Add to dashboard Cite

show abstract

“…La supraversión de la mirada parece en lo esencial una perturbación del fenómeno de Bell, donde lo normal es que los ojos se eleven al cerrarlos, en estos pacientes los ojos se elevan al abrir los párpados, además la elevación ocular se asocia a una persistente apertura palpebral, configurando un trastorno oculomotor cuya fisiopatogenia parece depender de áreas prefrontales 10 . La persistente apertura palpebral es asimilable a la "retracción palpebral", alteración observada en la enfermedad de Parkinson y en la enfermedad de Creutzfeldt-Jakob, de definido origen cortical 11 . Además, en esta paciente los estímulos nociseptivos desencadenaban crisis extensoras de las extremidades y un simultáneo cierre de los párpados con descenso de los globos oculares y un fenómeno de rebote con elevación ocular a la apertura palpebral.…”

Section: Comentariounclassified

Supraversión de la mirada en intoxicación mortal por bupropión

2018

Rev. méd. Chile

View full text Add to dashboard Cite

Upgaze or sustained elevation of the eyes, is an alteration of ocular motility initially described in hypoxic coma. We report a 65-year-old woman admitted with hypotension and alteration of sensorium due to the ingestion of 9.5 g of Bupropion. She presented two seizures of short duration, without epileptic activity on the EEG. She had a persistent asynchronous myoclonus in extremities, tachycardia and prolonged Q-t. She suffered a cardiac arrest caused by asystole, which recovered quickly in five minutes. At that moment, upgaze appeared, associated with a persistent ocular opening, which persisted for days, but finally disappeared, without remission of coma. A magnetic resonance imaging done at the eighth day, showed hyperintensity of the oval center and corpus callosum which disappeared in a new imaging study done 30 days later, where images of hypoxia in the basal nuclei and cortex appeared. The patient died forty seven days after admission. Up-gaze is an ominous oculomotor alteration linked to an important but incomplete damage in the cerebral cortex, a condition that perverts some sequences of the ocular opening, reversing the Bell phenomenon and producing eyelid retraction.

show abstract

Retracción palpebral de causa neurológica: Presentación de tres pacientes, interpretación patogénica

Abstract: (Rev Med Chile 2017; 145: 264-268)

Cited by 2 publications

References 8 publications

Enfermedad por priones, encefalopatía espongiforme humana y enfermedad de Creutzfeldt-Jakob

Enfermedad por priones, encefalopatía espongiforme humana y enfermedad de Creutzfeldt-Jakob

Supraversión de la mirada en intoxicación mortal por bupropión

Contact Info

Product

Resources

About