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2020, Number 2

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Rev Cubana Neurol Neurocir 2020; 10 (2)

Possible variant of type II Pascual Castroviejo Syndrome

Sotolongo PEM, Soto LA, Castillo RL, Morales AA, Delgado ZFÁ, Durán PM

Language: Spanish
References: 18
Page: 1-15
PDF size: 311.66 Kb.


ABSTRACT

Objective: To describe the clinical and imaging characteristics in a patient with a possible variant of type II Pascual Castroviejo syndrome.
Clinical case: A 44-year-old female patient with spastic congenital hemiparesis, congenital microcephaly, mild intellectual disability, structural focal epilepsy (mesial region of the temporal lobe), and a flat hemangioma port wine stain on the hand. Skull magnetic resonance imaging allowed to diagnose schizoencephaly with open lip or type 2, with bilateral frontal-parieto-temporal polymicrogyria and left periventricular heterotopias. After reaching the therapeutic dose of carbamazepine, the patient improved and her attacks were spaced out in such a way that they occurred approximately once or twice a year. In addition, she began rehabilitative treatment for her spastic hemiparesis.
Conclusions: The association of cutaneous hemangioma and abnormalities in the development of the central nervous system in this patient suggested the diagnosis of Type II Pascual Castroviejo Syndrome. However, in this case, the highest number of brain malformations was contralateral to the hand hemangioma. There was no malformation in the posterior cranial fossa, which differs from most of the published cases.


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