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Malformaciones congnitas del SNC:
Hidrocefalia, agenesia del cuerpocalloso.
Orellana Cullar, Jota Walter
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DEFINICIN
Hidrocefalia congnita:
Crecimiento progresivo ventricular por acmuloanormal de LCR, que aparece desde el primer da de
vida, por consecuente con aparicin inutero.
J. Volpe. Neurology of the New Borne. 3a edicion, 31-35, 1994
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Produccin de lquidocefalorraqudeo
El lquido cefalorraqudeo es producido por el plexocoroides. A travs de los agujeros de Magendie y deLuschka fluye hasta el cuarto ventrculo. Finalmente
es absorbido en los cuerpos de Pacchioni y en lasvellosidades aracnoideas.
Velocidad de produccin en los adultos 0.33ml/min = 475 ml en 24 h.
Velocidad de produccin en los nios 0.35 ml/min= 504 ml en 24 h.
HJ Lpez. Hidrocefalia congnita asociada a Sndrome de Dandy-Walker. Rev Mex Ped; 67; 77-82; 2000.
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Produccin de liquido cefalorraqudeo
El volumen total de LCR es aproximadamente unos 50 ml en ellactante, la mayor parte se encuentra fuera de los ventrculos.
El lquido una vez absorbido pasa hacia el seno sagital, y deall pasa a la sangre. Una vez en la sangre, es llevado y filtradopor los riones e hgado en la misma manera que cualquierotro fluido orgnico.
Nelson. Tratado de pediatra. Ed McGrawHill.16 Ed. P 1971, 2001
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Causas de hidrocefalia congnita
Estenosis acueductal 33%
Mielomeningocele (Arnodl Chiari) 28%
Hidrocefalia comunicante 22%
Malformacin Dandy Walker 7% Otros 10%
J. Volpe. Neurology of the New Borne. 3a edicion, 31-35, 1994
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Cuadro clnico Permetro ceflico aumentado.
Separacin de suturas craneales.
Somnolencia, rechazo progresivo a la va oral.
Anormalidades oculares (atrofia ptica yestrabismo).
Disfuncin del tronco enceflico (pobre
succin, vmitos, broncoaspiracin). Muerte por herniacin transtentorial o
transforaminal.
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AGENESIA DEL CUERPO CALLOSO
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AGENESIS OF CORPUS CALLOSUM
Agenesis of the corpus callosum (ACC) is a rare birth defect (congenital
disorder) in which there is a complete or partial absence of the corpuscallosum.
The development of the fibers that would otherwise form the corpuscallosum become longitudinally oriented within each hemisphere andform structures called Probstbundles.
In addition to agenesis of the corpus callosum, other congenital callosaldisorders include
1. hypogenesis (partial formation),
2. dysgenesis (malformation) of the corpus callosum,
3. hypoplasia (underdevelopment) of the corpus callosum.
Diagnosis
Callosal disorders can be diagnosed only through a brain scan
They may be diagnosed through an MRI, CT scan, prenatal ultrasound,or prenatal MRI
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AGENESIS OF CORPUS CALLOSUM
Cause Agenesis of the corpus callosum is caused by disruption to
development of the fetal brain between the 3rd and 12th weeksof pregnancy.
In most cases, it is not possible to know what caused anindividual to have ACC or another callosal disorder.
However, research suggests that some possible causes mayinclude
chromosome errors, inherited genetic factors,
prenatal infections or injuries, prenatal toxic exposures,
structural blockage by cysts or other brain abnormalities metabolic disorders.
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AGENESIS OF CORPUS CALLOSUM
Signs and symptoms Vary greatly among individuals
Some characteristics common in individuals with callosal disorders include
Research : shown to have some cognitive disabilities (difficulty in complex problemsolving) and social difficulties (missing subtle social cues), even when theirintelligence quotient is normal.
Other characteristics sometimes associated with callosal disorders includeseizures, spasticity, early feedingdifficulties and/or gastric reflux, hearing
impairments, abnormal head and facial features, and mental retardation.
1. Poor motor coordination,2. Delays in motor milestones such as sitting and walking,3. Delayed toilet training,4. Chewing and swallowing difficulties
5. Vision impairments,6. Hypotonia
7. Low perception ofpain, .
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AGENESIS OF CORPUS CALLOSUM
Associated syndromes ACC can occur as an isolated condition or in combination with other cerebral
abnormalities, including Arnold-chiari malformation, Dandy-walkersyndrome,
Andermannsyndrome(motor and sensory neuropathy) Schizencephaly (clefts or deep divisions in brain tissue) Holoprosencephaly (failure of the forebrain to divide into lobes.)
Girls may have a gender-specific condition called Aicardi'ssyndrome, whichcauses severe mental retardation, seizures(infantile spasms), abnormalities inthe vertebra of the spine, and lesions(lacunae) on the retina of the eye.
ACC can also be associated with malformations in other parts of the body,such as midline facialdefects.
The effects of the disorder range from subtle or mild to severe, depending on
associated brain abnormalities.
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