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Neuroepithelial Cysts Presenting with Movement Disorders: Two Cases

Published online by Cambridge University Press:  02 December 2014

Navraj S. Heran ,
Caglar Berk ,
Constantine Constantoyannis  and
Christopher R. Honey
Navraj S. Heran
Affiliation:
Surgical Centre for Movement Disorders, Division of Neurosurgery, University of British Columbia, Vancouver, BC, Canada
Caglar Berk
Affiliation:
Surgical Centre for Movement Disorders, Division of Neurosurgery, University of British Columbia, Vancouver, BC, Canada
Constantine Constantoyannis
Affiliation:
Surgical Centre for Movement Disorders, Division of Neurosurgery, University of British Columbia, Vancouver, BC, Canada
Christopher R. Honey
Affiliation:
Surgical Centre for Movement Disorders, Division of Neurosurgery, University of British Columbia, Vancouver, BC, Canada
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Abstract

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Background:

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.

Case Reports:

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.

Conclusion:

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.

Résumé:

RÉSUMÉ:Introduction:

Les auteurs présentent deux cas de désordres du mouvement causés par des kystes neuroépithéliaux et discutent de leur traitement. Les kystes neuroépithéliaux sont des cavités remplies de liquide, tapissées de cellules épendymales ou épithéliales d'étiologie inconnue dans le parenchyme du système nerveux central, sans connexion ventriculaire ou sous-arachnoïdienne évidente. La plupart des kystes sont asymptomatiques, mais certains patients présentent des crises convulsives, un effet de masse ou rarement un désordre du mouvement.

Observation:

La première patiente, une femme âgée de 27 ans, a consulté pour une faiblesse progressive, une posture dystonique, du tremblement, du ballisme et des mouvements choréoathétosiques du membre supérieur droit. Ses symptômes se sont améliorés après drainage stéréotaxique d'un kyste neuroépithélial situé au niveau des noyaux gris centraux, mais elle a présenté une récidive des symptômes en dedans d'un an. Le deuxième cas, une patiente âgée de 56 ans, a consulté pour diplopie, nystagmus, déséquilibre à la marche et hémiparésie. Ses symptômes se sont améliorés après drainage stéréotaxique d'un kyste neuroépithélial du mésencéphale. Le kyste s'est reformé de nouveau au cours des années et elle a présenté du tremblement du bras gauche et de la faiblesse des muscles faciaux. Une nouvelle aspiration a été effectuée avec amélioration des symptômes pendant neuf mois. Son tremblement a réapparu et un accès au kyste a été implanté par stéréotaxie. Son état s'est amélioré et elle est demeurée stable pendant plus d'un an.

Conclusions:

Le drainage stéréotaxique simple de kystes neuroépithéliaux comporte un taux de récidive élevé. Les auteurs recommendent de considérer la mise en place d'un dispositif de drainage pour faciliter l'aspiration du liquide contenu dans le kyste, au besoin.

Type
Case Report
Information
Canadian Journal of Neurological Sciences , Volume 30 , Issue 4 , November 2003 , pp. 393 - 396
Copyright
Copyright © The Canadian Journal of Neurological 2003

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