Neuroepithelial cysts presenting with movement disorders: two cases

The authors present two cases of movement disorders caused by neuroepithelial cysts and highlight their management. Neuroepithelial cysts are ependymal or epithelial lined fluid collections of unknown etiology within the central nervous system parenchyma with no obvious ventricular or subarachnoid connection. Most cysts are asymptomatic, however, some present with seizures, mass effect, or rarely with movement disorders.

The first patient, a 27-year-old female, presented with progressive weakness, dystonic posturing, tremor, ballismus and choreoathetotic movements of her right upper extremity. Her symptoms improved after stereotactic drainage of a neuroepithelial cyst in her basal ganglia but recurred within a year. The second case, a 56-year-old female, presented with diplopia, nystagmus, gait imbalance and hemiparesis. Her symptoms improved after stereotactic drainage of a midbrain neuroepithelial cyst. The cyst reaccumulated over the next few years and she became symptomatic with left arm tremor and facial weakness. Aspiration was again performed with symptomatic improvement for nine months. Her tremor recurred and a cyst access device was placed stereotactically. She improved and has remained stable for over a year.

Simple stereotactic drainage of neuroepithelial cysts has a high recurrence rate. The authors recommend considering placement of a drainage device to facilitate aspiration of the cyst fluid during follow-up, if needed.