Spinocerebellar Atrophy Type-3 with Chiari Malformationin a Young Man: A Case Report

Chiari malformations are a group of congenital anomalies which involve the hindbrain and the cervical spinal canal.

Here, we describe a patient who presented with acute diplopia and gait unsteadiness which was first deigned with Chiari malformation type-1. However due to progression of the ataxia the full neurologic evaluation was considered which established the diagnosis of spinocerebellar ataxia type 3 (Machado-Joseph-Disease).

We aim to highlight the importance of careful examination in order to avoid misdiagnosis of even rare diseases.