Chiari malformation is characterized by caudal displacement of the cerebellar tonsils that penetrate into the spinal canal through the foramen magnum, achieving reach the atlas or axis. Typically is seen in young adults. In some cases scoliosis and Syringomyelic cavities may occur. The authors present as far as they know the first case in the literature with long term follow-up, of a caucasian woman with an unusual form of cerebellar atrophy and Chiari Type I malformation, suffering from weakness in his upper and lower extremities with rapidly progression. The patient was successfully treated with suboccipital decompression and C1 laminectomy.

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[Type I Chiari Malformation Associated With Cerebellar Atrophy. Case Report]

The first one with clinical picture of acute respiratory insuficiency and the second one with vestibular and mild cerebellar syndrome and headache. In both cases the neurological examination demonstrated the presence of "downbeating nystagmus". We emphasize the value of neurological semiology, determining a correct complementary evaluation and effective treatment. Caso 1. Caso 2. Tipo IV: Hipoplasia severa ou aplasia de cerebelo, associada a fossa posterior com pequeno tamanho 1,3,4,5,10,


Revisión bibliográfica sobre la sintomatología cognitiva en la malformación de Chiari tipo I

The authors report 17 cases of symptomatic Chiari type II malformation occurring in two distinct age dependent population. In Group I, 13 neonates and infants in the first year of life presented with cranial nerve and brain stem dysfunction characterized by vocal cord paralysis, apnea, dysphagia and laryngeal stridor. In Group II, 4 patients developed signs and symptoms after the first year of life. In this group, the presentation was more insidious and included neck pain and cerebellar manifestations. The surgical treatment consisted initially in shunt implantation or revision and when there was no improvement, posterior fossa decompression was performed. The response to the surgical treatment differed considerably between the two groups: older patients improved promptly after surgery and there was no casualties; in newborn and infants, especially those under 6 months of age, the mortality rate was The authors stress that prompt diagnosis and surgical intervention should be performed in order to produce a favorable outcome.


2016, Número 1

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