Agus Agnetti Masc 68 años Paresia braquicrural derecha de 15 hs de evol Atc ataxia cerebelosa dx 2010 dismetría voz escandida trast deglutorios req sonda postrado epilepsia ID: 573617
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Slide1
Caso en 5 minutos
Agus
AgnettiSlide2
Masc 68 años.
Paresia
braquicrural
derecha de 15 hs de
evol
Atc
: ataxia
cerebelosa
dx
2010, dismetría, voz escandida,
trast
deglutorios (
req
sonda), postrado, epilepsia
Ex
fco
: disartria, ataxia, foco BC derecho moderado
TC de otra institución: normal
Interpretación: ACV
isqco
fuera de ventanaSlide3Slide4
Signo de la Cruz – Hot Cross Bun
AMC - CSlide5
AMS
Enf
neurodegenerativa progresiva
Inicio en la adultez
Agrupa a 3 entidades:
-
Sx
autonómicos
Shy
Drager
(MSA –A) Sx cerebelosos Atrofia olivopontocerebelosa (MSA – C)Sx piramidales Degeneración estriatonígrica (MSA – P)Slide6
Criterios DxSlide7
RM
Hallazgos generales:
Protuberancia – BR achatados (sagital)
Atrofia
vermiana
/ hemisferios cerebelosos
MSA-C
Atrofia de cerebelo, olivas y
protuberancia /
dism
de
diam
AP de protuberanciaHiperintensidad cruciforme en protuberacia ("hot cross bun" sign)MSA-P↓ T2 y atrofia del putamen dorsolateral (GRE)± línea ↑
T1 y T2 putamen lateralFDG PET : ↓ metabolismo en putamenSlide8
Axial T2WI MR through the lower
pons
in the same patient confirms
cerebellar
atrophy. The most striking feature is the cruciform region of
hyperintense
signal (white open arrow) within the atrophic
pons
, the "hot cross bun" sign.
Axial T2WI MR through the lower
pons
in the same patient confirms
cerebellar atrophy. The most striking feature is the cruciform region of hyperintense signal (white open arrow) within the atrophic pons, the "hot cross bun" sign.AMS - CSlide9
Signo de la Cruz
-
H
iperintesidad
cruciforme
protuberancial,
ppalmente
en
sec
T2 axial.
- Debido a perdida selectiva de fibras
mielinicas
transversas pontocerebelosas, neuronas del rafe medio y fibras pontinas transversas, con indemnidad de los haces corticoespinales y tegmentum pontino. - CARACTERÍSTICO pero No patognomónica de AMS, ya que se ha presentado en Ataxia cerebelosas tipo 2 y 3, Parkinsonismo secundario a vasculitis del tallo cerebral
, enfermedad de Creutzfeldt-Jakob, y Leucoencefalopatía por HIV
Slide10
Signo del Santiaguiño
Signo de la cruz + atrofia del vermis y hemisferios
cerebelosos
:
Cruz en la protuberancia: cuerpo
Atrofia vermiana: colaHemisferios
cerebelosos
: siluetaSlide11
Axial T2WI MR also demonstrates that the
putamina
appear small and
hypointense
, with a thin peripheral rim of high signal intensity (white open arrow). The white matter appears quite normal compared to the overall degree of
sulcal
and ventricular enlargement.
Axial T2WI MR also demonstrates that the
putamina
appear small and
hypointense
, with a thin peripheral rim of high signal intensity (white open arrow). The white matter appears quite normal compared to the overall degree of
sulcal and ventricular enlargement.AMS - PSlide12
Muchas gracias