Chiari I Malformation: A Missed Diagnosis
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 poster...
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| Published in: | Annals of African medicine Vol. 5; no. 4 |
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| Main Authors: | , , , |
| Format: | Journal Article |
| Language: | English |
| Published: |
Annals of African Medicine Society
23-05-2007
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| Subjects: | |
| Online Access: | Get full text |
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| Summary: | 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.
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. |
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| ISSN: | 1596-3519 |