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Sarcoidosis Presenting as an Intramedullary Spinal Cord Lesion

Published online by Cambridge University Press:  24 February 2017

F.B. Maroun ,
F.J. O'Dea ,
G. Mathieson ,
G. Fox ,
G. Murray ,
J.C. Jacob ,
R. Reddy  and
R. Avery
F.B. Maroun*
Affiliation:
Division of Neurosurgery, Neurology, Departments of Radiology and Pathology, Memorial University of Newfoundland, Health Sciences Centre, St. John's, Newfoundland, Canada
F.J. O'Dea
Affiliation:
Division of Neurosurgery, Neurology, Departments of Radiology and Pathology, Memorial University of Newfoundland, Health Sciences Centre, St. John's, Newfoundland, Canada
G. Mathieson
Affiliation:
Division of Neurosurgery, Neurology, Departments of Radiology and Pathology, Memorial University of Newfoundland, Health Sciences Centre, St. John's, Newfoundland, Canada
G. Fox
Affiliation:
Division of Neurosurgery, Neurology, Departments of Radiology and Pathology, Memorial University of Newfoundland, Health Sciences Centre, St. John's, Newfoundland, Canada
G. Murray
Affiliation:
Division of Neurosurgery, Neurology, Departments of Radiology and Pathology, Memorial University of Newfoundland, Health Sciences Centre, St. John's, Newfoundland, Canada
J.C. Jacob
Affiliation:
Division of Neurosurgery, Neurology, Departments of Radiology and Pathology, Memorial University of Newfoundland, Health Sciences Centre, St. John's, Newfoundland, Canada
R. Reddy
Affiliation:
Division of Neurosurgery, Neurology, Departments of Radiology and Pathology, Memorial University of Newfoundland, Health Sciences Centre, St. John's, Newfoundland, Canada
R. Avery
Affiliation:
Division of Neurosurgery, Neurology, Departments of Radiology and Pathology, Memorial University of Newfoundland, Health Sciences Centre, St. John's, Newfoundland, Canada
*
Rm. 5202, Health Sciences Centre, St. John's, NF, Canada A1B 3V6
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Abstract:

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Objectives:

Meige syndrome is a movement disorder that includes blepharospasm and oromandibular dystonias. Its etiology may be idiopathic (primary) or it may arise secondary to focal brain injury. Acute respiratory distress as a feature of such dystonias occurs infrequently. A review of the literature on Meige syndrome and the relationship between dystonias and respiratory compromise is presented.

Methods:

A 60-year-old woman suffered a cerebral anoxic event secondary to manual strangulation. She developed progressive blepharospasm combined with oromandibular and cervical dystonias. Neuroimaging demonstrated bilateral damage localized to the globus pallidus. Years later, she presented to the emergency department in intermittent respiratory distress associated with facial and cervical muscle spasms.

Results and conclusions:

Increasing frequency and severity of the disorder was noted over years. The acute onset of respiratory involvement required intubation and eventual tracheotomy. A partial therapeutic benefit of tetrabenazine was demonstrated.

Résumé:

RÉSUMÉ:Objectifs:

La moelle épinière est impliquée dans la sarcoïdose chez seulement 0.43% des patients atteints de cette maladie. Il y a habituellement des lésions systémiques avant l'apparition de lésions de la moelle épinière. Nous présentons un cas de lésion sarcoïdosique intramédullaire isolée, ce qui représente un défi diagnostique et thérapeutique. Nous revoyons le cas et nous présentons une revue de la littérature en soulignant le mode de présentation, le diagnostic et le traitement.

Méthodes:

Nous revoyons le cas d'un patient porteur d'un granulome sarcoïdosique isolé de la moelle épinière cervicale. L'histoire médicale pertinente et les données de l'examen physique ont été tirés du dossier du patient ainsi que d'une entrevue avec le patient. Nous présentons l'investigation biochimique, radiologique et anatomopathologique.

Résultats et conclusions:

Quatorze patients porteurs d'une sarcoïdose localisée uniquement à la moelle épinière ont été rapportés dans la littérature. La chirurgie a un rôle à jouer dans le diagnostic et la corticothérapie constitue la base du traitement.

Type
Case Report
Information
Canadian Journal of Neurological Sciences , Volume 28 , Issue 2 , May 2001 , pp. 163 - 166
Copyright
Copyright © The Canadian Journal of Neurological 2000

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