Document Detail


Opsoclonus-myoclonus syndrome in patients with locked-in syndrome: a therapeutic porthole with gabapentin.
MedLine Citation:
PMID:  20511483     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Patients with locked-in syndrome, although fully conscious, have quadriplegia, mutism, and lower cranial nerve paralysis. The preservation of vertical gaze and upper eyelid movements usually enables them to interact with the environment through an eye-coded communication. However, locked-in syndrome may be complicated by the development of an opsoclonus-myoclonus syndrome that may represent an additional impediment to communication. We evaluated whether off-label treatment with gabapentin could help patients with locked-in syndrome and opsoclonus-myoclonus symptoms regain voluntary control of full eye movements. A mechanism responsible for gabapentin-induced improvement has been also hypothesized. In this study, 4 patients presenting with locked-in syndrome complicated by opsoclonus-myoclonus syndrome were continuously treated with gabapentin up to 1200 mg/d. The treatment resulted in a rapid and long-lasting resolution of opsoclonus-myoclonus symptoms without adverse effects. After 2 weeks, patients showed voluntary attempts to communicate through eye blinking and thereafter regained voluntary control of full eye movements. This event enabled them to regain a communication channel with relatives and physicians and to start using eye-controlled brain-computer interfaces. Because of its effectiveness in restoring eye movement control, gabapentin opened a communicative porthole in the patients' lives. Since opsoclonus may be related to disorders of the inhibitory control of saccadic burst neurons by pontine pause cells, we hypothesize that gabapentin acts as a regulator of saccadic circuits.
Authors:
Francesca Pistoia; Massimiliano Conson; Marco Sarà
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Mayo Clinic proceedings. Mayo Clinic     Volume:  85     ISSN:  1942-5546     ISO Abbreviation:  Mayo Clin. Proc.     Publication Date:  2010 Jun 
Date Detail:
Created Date:  2010-05-31     Completed Date:  2010-06-18     Revised Date:  2011-03-03    
Medline Journal Info:
Nlm Unique ID:  0405543     Medline TA:  Mayo Clin Proc     Country:  United States    
Other Details:
Languages:  eng     Pagination:  527-31     Citation Subset:  AIM; IM    
Affiliation:
Post-Coma Intensive and Rehabilitation Care Unit, Hospital San Raffaele, Cassino, Italy. pistoiafrancesca@hotmail.com
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MeSH Terms
Descriptor/Qualifier:
Aged
Amines / administration & dosage,  therapeutic use*
Cyclohexanecarboxylic Acids / administration & dosage,  therapeutic use*
Eye Movements / drug effects
Female
Humans
Male
Middle Aged
Nonverbal Communication
Opsoclonus-Myoclonus Syndrome / complications*,  drug therapy*
Quadriplegia / complications*
gamma-Aminobutyric Acid / administration & dosage,  therapeutic use*
Chemical
Reg. No./Substance:
0/Amines; 0/Cyclohexanecarboxylic Acids; 56-12-2/gamma-Aminobutyric Acid; 60142-96-3/gabapentin
Comments/Corrections
Comment In:
Mayo Clin Proc. 2010 Jun;85(6):508-11   [PMID:  20511480 ]

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