Ｄａｎｄｙ‐Ｗａｌｋｅｒ症候群の１例

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  • A Case of Dandy-walker Syndrome

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Most cases of the Dandy-Walker syndrome (DWS) develop symptoms before the age of two. In this report, we present a rare case of DWS, the patient having been in good health until the age of 13 when she suddenly developed intracranial hypertension.<BR>A 13-year-old girl visited our clinic complaining of headache, vomiting and diplopia. Paralysis of the bilateral abducent nerves was present. CT examination demonstrated a large posterior fossa cyst communicating with the fourth ventricle, 'inverted Y' configuration, absence of cerebellar vermis and symmetrical enlargement of ventricular system. Angiography, pneumoventriculography and RI-ventriculography confirmed the diagnosis of DWS.<BR>An equilibrium test revealed direction-changing, opposing, vertical-positioning nystagmus with almost normal findings on the caloric test, OKP and ETT.<BR>We theorized that the cerebellar vermis takes part in the occurance of direction-changing, opposing, vertical-positioning nystagmus and that a slowlygrowing, expansive cyst of the posterior cranial fossa does not have important effects upon OKP, ETT or the occurance of cerebellar symptoms.

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