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Annals of African Medicine
Annals of African Medicine Society
ISSN: 1596-3519
Vol. 5, No. 4, 2006, pp. 206-208
Bioline Code: am06049
Full paper language: English
Document type: Research Article
Document available free of charge

Annals of African Medicine, Vol. 5, No. 4, 2006, pp. 206-208

 en Chiari I Malformation: A Missed Diagnosis
Shehu, B. B.; Ismail, N. J.; Mahmu, M. R. & Hassan, I.


Chiari I malformation is a complex congenital malformation of the hindbrain, characterized by herniation of the cerebellar tonsils through the foramen magnum into the cervical spinal canal. There may be mild caudal displacement and flattening or kinking of the medulla with an associated small posterior fossa.
We present a case of a 30 year old man who presented with a 2 year history of worsening nystagmus and ataxia with associated occipital headache and diplopia. Examination showed a young man with horizontal and vertical nystagmus, he had truncal ataxia and Rhomberg's sign was positive. Diagnosis was missed by several clinicians during the 2 year period. Repeated brain CT scans were normal. Brain MRI confirmed the diagnosis of Chiari I malformation and cervical syringomyelia. He had decompressive occipital craniectomy durotomy and duroplasty. The patient recovered fully from ataxia headache and diplopia but still has residual nystagmus.

Chiari I malformation, syringomyelia, posterior fossa, craniectomy, missed diagnosis

Shehu, B. B.; Ismail, N. J.; Mahmu, M. R. & Hassan, I.


La malformation Chiari I est une malformation congenitale complexe du cerveau posterior, characterisée par l'hernie de l'amygdale du cervelet dans le trou occipital et le canal spinal cervical. Il peut y'avoir le deplacement caudal, applatissement ou tortillement de la moelle allongée associé ã une petite fosse posterieur.
Nous presentons un adult de 30 ans qui s'est presenté avec un nystagme progressive, l'ataxie associés à des. cephallées occipitales et la diplopie pour deux ans. L'examen de ce jeune homme revèle du nystagme horizontale et verticale, l'ataxie troncale avec le signe de Rhomberg present. Le diagnostique etait manqué durant ces deux années par plusieurs cliniciens. Des scanner repetés du cerveau etaient normaux. Le M.R.I. du cerveau a confirmé le diagnostique de la malformation Chiari I, ainsi que la syringomyelie cervicale. Il a eu une craniotomy decompressive occipitale (fosse cerebelleuse) durotomy ainsi que duroplastie. Le patient a eu une convallescence parfaite avec l'abolition de l'ataxie, les cephallées et la diplopie mais a encore du nystagme residuel.

Mots Clés
Malformation Chiari I, craniotomie de la fosse, cerebelleuse, diagnostique manqué

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